5 6337055981314245059

OPHTHALMOLOGY CLINICS
for
Postgraduates
OPHTHALMOLOGY CLINICS
for
Postgraduates
Editors
Prafulla Kumar Maharana MD DNB

Assistant Professor
Dr Rajendra Prasad Centre for Ophthalmic Sciences
All India Institute of Medical Sciences (AIIMS)
New Delhi, India
Namrata Sharma MD
Professor
New Delhi, India
Atul Kumar MD FAMS

Professor and Chief
New Delhi, India
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Ophthalmology Clinics for Postgraduates
First Edition: 2017
ISBN: 978-93-86322-89-0
Dedicated to
My parents, Mr Devendra Maharana and Mrs Binadini Maharana
—Prafulla Kumar Maharana
My parents, Dr Ramesh C Sharma and Mrs Maitreyi Pushpa

My husband, Dr Subhash Chandra; and, Daughter, Vasavdatta
—Namrata Sharma
My late parents, Mr Sanat Kumar and Mrs Swarna Kumar

My wife, Mrs Parul Kumar; and, Children, Aman and Arshi
—Atul Kumar
Contributors
Atul Kumar MD FAMS Bijnya Birajita Panda MS FICO (UK)

Professor and Chief Consultant
Dr Rajendra Prasad Centre for Ophthalmic Sciences Kar Vision Eye Hospital
All India Institute of Medical Sciences Bhubaneswar, Odisha, India
New Delhi, India
Brijesh Takkar MD FICO FAICO

Adarsh Shashni MD Senior Research Associate
Senior Resident Dr Rajendra Prasad Centre for Ophthalmic Sciences
Dr Rajendra Prasad Centre for Ophthalmic Sciences All India Institute of Medical Sciences
All India Institute of Medical Sciences New Delhi, India
New Delhi, India
Deepali Singhal MD
Aditi Dubey MBBS MS (Ophthal) Senior Resident
Assistant Professor Dr Rajendra Prasad Centre for Ophthalmic Sciences
Department of Ophthalmology All India Institute of Medical Sciences
Gandhi Medical College New Delhi, India
Bhopal, Madhya Pradesh, India
Amar Pujari MD Devesh Kumawat MD DNB

Senior Resident Senior Resident
Dr Rajendra Prasad Centre for Ophthalmic Sciences Dr Rajendra Prasad Centre for Ophthalmic Sciences
All India Institute of Medical Sciences All India Institute of Medical Sciences
New Delhi, India New Delhi, India
Ashish Markan MBBS Devika S Joshi MS

Junior Resident Fellow
Dr Rajendra Prasad Centre for Ophthalmic Sciences Narayana Nethralaya
All India Institute of Medical Sciences Bengaluru, Karnataka, India
New Delhi, India
Dewang Angmo MD DNB MNAMS FRCS

Ashutosh Kumar Gupta MS
Assistant Professor
Graded Specialist, Ophthalmology
Military Hospital
All India Institute of Medical Sciences
Agra, Uttar Pradesh, India
New Delhi, India
Aswini Kumar Behera MD

Junior Resident Dhaval Patel MD
Dr Rajendra Prasad Centre for Ophthalmic Sciences Consultant
All India Institute of Medical Sciences Centre for Sight
New Delhi, India Vadodara, Gujarat, India
viii Ophthalmology Clinics for Postgraduates
Dheepak Sundar MD Jyotiranjan Mallick MS (Ophthal)

Dr Rajendra Prasad Centre for Ophthalmic Sciences Department of Ophthalmology
New Delhi, India Bhubaneswar, Odisha, India
Divya Agarwal MBBS Karthikeya R MD DNB

Junior Resident Senior Resident
D Satyasudha MBBS Mandeep S Bajaj MD

Junior Resident Professor
Esha Agarwal MD Manpreet Kaur MD

Namrata Sharma MD
Harathy Selvan MBBS
Professor
Junior Resident
New Delhi, India
New Delhi, India
Neelima Aron MD DNB FICO

Harika Regani MBBS Senior Resident
Junior Resident Dr Rajendra Prasad Cenre for Ophthalmic Sciences
New Delhi, India
Patil Mukesh Prakash MBBS MD FICO

Jeewan S Titiyal MD Senior Resident
Professor Dr Rajendra Prasad Centre for Ophthalmic Sciences
New Delhi, India
Prafulla Kumar Maharana MD DNB

Jennil Shetty MBBS Assistant Professor
DNB Resident Dr Rajendra Prasad Centre for Ophthalmic Sciences
LV Prasad Eye Institute All India Institute of Medical Sciences
Bhubaneswar, Odisha, India New Delhi, India
Contributors ix
Prakhar Goyal MBBS DNB Rajesh Sinha MD

Consultant Professor
Goyal Eye Hospital Dr Rajendra Prasad Centre for Ophthalmic Sciences
Narnaul, Haryana, India All India Institute of Medical Sciences
New Delhi, India
Pranayi Behera MS
Senior Resident Ritika Mukhija MBBS
Department of Ophthalmology Junior Resident
All India Institute of Medical Sciences Dr Rajendra Prasad Centre for Ophthalmic Sciences
Bhubaneswar, Odisha, India All India Institute of Medical Sciences
New Delhi, India
Pranita Sahay MD
Senior Resident Ritu Nagpal MD
Dr Rajendra Prasad Cenre for Ophthalmic Sciences Fellow Cornea
All India Institute of Medical Sciences LV Prasad Eye Institute
New Delhi, India Hyderabad, Telangana, India
Prateek Kakkar MD DNB FICO FAICO Ruchita Falera MD DNB FICO

Priyanka Ramesh MBBS Ruchir Tewari MD

Junior Resident Senior Resident
Pulak Agrawal MD Sagnik Sen MBBS

Senior Resident Junior Resident
Raghav Ravani MD Sandeep Gupta MS DNB MNAMS

Senior Resident Associate Professor
All India Institute of Medical Sciences Armed Forces Medical College
New Delhi, India Pune, Maharashtra, India
Rajesh Pattebahadur MD Sapna Raghuwanshi MS

Senior Resident Assistant Professor
Department of Ophthalmology Department of Ophthalmology
All India Institute of Medical Sciences LN Medical College
Bhopal, Madhya Pradesh, India Bhopal, Madhya Pradesh, India
x Ophthalmology Clinics for Postgraduates
Saranya Devi K MD DNB FICO Talvir Sidhu MD DNB

Shipra Singhi MD Tushar Agarwal MD

Consultant Professor
ASG Eye Hospital Dr Rajendra Prasad Centre for Ophthalmic Sciences
Jodhpur, Rajasthan, India All India Institute of Medical Sciences
New Delhi, India
Shorya Vardhan Azad MS

Vaishali Ghanshyam Rai MBBS DNB
Assistant Professor
Senior Resident
Department of Ophthalmology
New Delhi, India
Bhopal, Madhya Pradesh, India
Shreyas Temkar MD DNB FICO FAICO Varsha Varshney MBBS MS

New Delhi, India Jodhpur, Rajasthan, India
Swati Phuljhale MD Yamini Attiku MBBS

Assistant Professor Junior Resident
Preface
“The whole art of medicine is in observation”.

—Sir William Osler
Postgraduate exam is one of the most difficult and stressful milestones in the medical profession.
It entails tremendous amount of stress on the candidates who appear for the exam. The never-ending
knowledge of ophthalmology makes it quite difficult to revise all the cases from the standard textbooks
before the exams. Further, the presentation of cases—long case or short case—is completely in a different
format from what is given in the standard textbooks. Most of the time, the candidate fails to present the
case properly in spite of good theoretical knowledge. The primary reason for this is that the format in
which a topic is discussed in textbooks is completely different from that required for a practical exam.
This book attempts to present the important topics in a format that is exactly same as required
in the practical exams. The primary focus is on history taking and proper clinical examination of the
cases. Every effort has been made to describe the procedures of clinical examinations in such a way that
the candidate can perform these examination techniques accurately in front of the examiner. Special
emphasis has been given to the differential diagnosis of the cases, which is often a favorite question
amongst the examiner. Clinical photographs of the cases as well as the important signs and investigation
findings have been provided that will help the students for better understanding of the cases. The cases
are being selected after discussing with experts in this field as well as candidates who have recently
appeared in various postgraduate exams. Each chapter ends with a section on viva-voce questions that
will help the candidates to mentally prepare for the viva before the final exam. In addition, a chapter on
instruments has been included which is invariably a part of all postgraduate practical exams. The editors
and all the contributors have made sincere efforts to make things simple and concise and to facilitate
quick and thorough revision. However, it must be remembered that this is not a substitute to standard
textbooks. The readers are encouraged to read standard textbooks before going through this book.
This will make their understanding and application of this book more rewarding.
The editors wish best of luck to the students for their exams!
Prafulla Kumar Maharana

Namrata Sharma
Atul Kumar
Contents
1. OCULOPLASTY 1
Long Cases
• Proptosis 1
Varsha Varshney, Amar Pujari, Mandeep S Bajaj
• Lid Tumors 9
Sapna Raghuwanshi, Amar Pujari, Mandeep S Bajaj
• Ptosis 15
Aditi Dubey, Amar Pujari
• Contracted Socket 25
Varsha Vashney, Aditi Dubey, Amar Pujari
• Blow out Fracture of Orbit 30
• Thyroid-associated Ophthalmopathy 35
Aditi Dubey, Prafulla Kumar Maharana
• Lacrimal Gland Tumors 44
Varsha Vashney, Amar Pujari
Short Cases
• Congenital Ptosis 52
• Ectropion 56
Aditi Dubey, Bijnya Birajita Panda
• Entropion 60
Aditi Dubey, Ritu Nagpal
• Blepharophimosis, Ptosis, Epicanthus Inversus Syndrome 63
Amar Pujari, Aditi Dubey
• Sebaceous Gland Carcinoma 67
Amar Pujari, Sapna Raghuwanshi
• Pyogenic Granuloma 71
Sapna Raghuwanshi, Bijnya Birajita Panda
• Lagophthalmos 72
Varsha Varshney, Ritu Nagpal
xiv Ophthalmology Clinics for Postgraduates
• Dermoid Cyst 77
Shipra Singhi, Deepali Singhal
• Orbital Hemangioma 82
Aditi Dubey, Ritika Mukhija, Rajesh Pattebahadur
• Coloboma of Eyelid 86
Shipra Singhi, Amar Pujari
2. CORNEA AND CONJUNCTIVA 90
Long Cases
• Corneal Ulcer 90
Shipra Singhi, Tushar Agarwal, Prafulla Kumar Maharana, Namrata Sharma
• Keratoconus 106
Prafulla Kumar Maharana, Sapna Raghuwanshi, Manpreet Kaur, Namrata Sharma
• Corneal Stromal Dystrophy 117
Shipra Singhi, Divya Agarwal, Prafulla Kumar Maharana, Rajesh Sinha
• Fuchs’ Endothelial Corneal Dystrophy 128
Sapna Raghuwanshi, Ritu Nagpal, Prafulla Kumar Maharana, Namrata Sharma
• Acute Graft Rejection 134
Ritu Nagpal, Vaishali Ghanshyam Rai, Prafulla Kumar Maharana, Manpreet Kaur
Short Cases
• Keratoglobus 141
Prafulla Kumar Maharana, Sapna Raghuwanshi, Namrata Sharma
• Pellucid Marginal Degeneration 144
Sapna Raghuwanshi, Manpreet Kaur, Namrata Sharma
• Band-shaped Keratopathy 148
Manpreet Kaur, Sapna Raghuwanshi, Ritu Nagpal
• Spheroidal Degeneration 150
Sapna Raghuwanshi, Neelima Aron, Namrata Sharma
• Congenital Hereditary Endothelial Dystrophy 153
Prafulla Kumar Maharana, Vaishali Ghanshyam Rai, Manpreet Kaur
• Iridocorneal Endothelial Syndrome 157
Vaishali Ghanshyam Rai, Neelima Aron, Prafulla Kumar Maharana
• Peters’ Anomaly 160
Shipra Singhi, Neelima Aron, Manpreet Kaur
• Limbal Dermoid 163
Shipra Singhi, Deepali Singhal, Neelima Aron
Contents xv
3. GLAUCOMA 171
Long Cases
• Primary Open Angle Glaucoma 171
Dewang Angmo, Vaishali Ghanshyam Rai, Ritika Mukhija
• Primary Angle Closure Glaucoma 178
Vaishali Ghanshyam Rai, Talvir Sidhu, Dewang Angmo
Short Cases
• Sturge-Weber Syndrome 185
Vaishali Ghanshyam Rai, Ritika Mukhija, Dewang Angmo
• Buphthalmos 188
Vaishali Ghanshyam Rai, Dewang Angmo
• Neovascular Glaucoma 192
Jennil Shetty, Talvir Sidhu
• Angle-recession Glaucoma 197
Prakhar Goyal, Divya Agarwal, Talvir Sidhu
• Steroid-induced Glaucoma 200
Vaishali Ghanshyam Rai, Talvir Sidhu
• Pseudoexfoliation Glaucoma 203
4. RETINA 208
Long Cases
• Vitreous Hemorrhage 208
Shipra Singhi, Brijesh Takkar
• Central Retinal Vein Occlusion 216

Ashish Markan, Esha Agarwal, Brijesh Takkar
• Branch Retinal Vein Occlusion 223

Pulak Agrawal, Shorya Vardhan Azad
• Proliferative Diabetic Retinopathy 229

Brijesh Takkar, Dhaval Patel, Rajesh Pattebahadur
• Nonproliferative Diabetic Retinopathy 236

Dhaval Patel, Brijesh Takkar, Rajesh Pattebahadur
• Retinitis Pigmentosa 244
Jyotiranjan Mallick, Prafulla Kumar Maharana
xvi Ophthalmology Clinics for Postgraduates
• Macular Hole 252
Vaishali Ghanshyam Rai, Brijesh Takkar
• Retinal Detachment 260
Pranayi Behera, Rajesh Pattebahadur, Raghav Ravani
• Age-related Macular Degeneration 272

Shipra Singhi, Raghav Ravani, Brijesh Takkar
• Intermediate Uveitis 285
Raghav Ravani, Karthikeya R, Harathy Selvan, Atul Kumar
• Choroidal Melanoma 291
Karthikeya R, Raghav Ravani, Prateek Kakkar, Atul Kumar
Short Cases
• Cherry-red Spot 300
Rajesh Pattebahadur, Brijesh Takkar
• Central Serous Chorioretinopathy 303
Vaishali Ghanshyam Rai, Raghav Ravani
• Diabetic Macular Edema 308
Brijesh Takkar, Dhaval Patel
• Epiretinal Membrane 314
Dhaval Patel, Brijesh Takkar
• Fundal Coloboma 318
D Satyasudha, Ruchir Tewari, Atul Kumar
• Giant Retinal Tear 325
• Posterior Segment Cysticercosis 329
Harika Regani, Karthikeya R, Yamini Attiku, Atul Kumar
• Cataract in Silicone Oil-filled Eyes 332
Sagnik Sen, Esha Agarwal, Raghav Ravani, Atul Kumar
• Silicone Oil-induced Secondary Glaucoma 337
Ashish Markan, Esha Agarwal, Raghav Ravani, Atul Kumar
• Posterior Dislocated Lens 341
• Stargardt Disease 346
Aswini Kumar Behera, Ruchir Tewari
• Traumatic Retinal Detachment 350
Priyanka Ramesh, Shreyas Temkar, Dheepak Sundar, Atul Kumar
Contents xvii
5. NEURO-OPHTHALMOLOGY AND STRABISMUS 356
Long Cases
• Third Cranial Nerve Palsy 356
Adarsh Shashni, Shipra Singhi
• Sixth Cranial Nerve Palsy 364
Shipra Singhi, Swati Phuljhale
• Fourth Cranial Nerve Palsy 371
Swati Phuljhale, Shipra Singhi, Patil Mukesh Prakash
• Optic Neuritis 377
Ritu Nagpal, Adarsh Shashni
• Esodeviation 384
Shipra Singhi, Ritika Mukhija, Adarsh Shashni
• Exodeviation 395
Shipra Singhi, Adarsh Shashni
Short Cases
• Duane Retraction Syndrome 400
Shipra Singhi, Saranya Devi K
• Ocular Myasthenia Gravis 403
6. LENS 409
Long Cases
• Zonular Cataract 409
Manpreet Kaur, Ashutosh Kumar Gupta, Jeewan S Titiyal
• Ectopia Lentis 414
Ruchita Falera, Manpreet Kaur, Prafulla Kumar Maharana
Short Cases
• Lenticonus 421
Manpreet Kaur, Prafulla Kumar Maharana, Jeewan S Titiyal
• Posterior Polar Cataract 424
Manpreet Kaur, Devika S Joshi, Jeewan S Titiyal, Sandeep Gupta
• Microspherophakia 428
Manpreet Kaur, Devika S Joshi, Prafulla Kumar Maharana
xviii Ophthalmology Clinics for Postgraduates
• Posterior Capsular Opacification 431

Prafulla Kumar Maharana, Manpreet Kaur
• Traumatic Cataract 435
Deepali Singhal, Ruchita Falera, Manpreet Kaur
7. INSTRUMENTS 440
• Ophthalmic Instruments 440
Pranita Sahay, Devesh Kumawat
Index463
CHAPTER
1
Oculoplasty
LONG CASES
PROPTOSIS
Varsha Varshney, Amar Pujari, Mandeep S Bajaj
INTRODUCTION hemangioma and Graves’ disease] or induced

astigmatism due to globe compression or
Proptosis is defined as an abnormal forward exposure keratopathy.
protrusion of one or both eyeballs with respect • Rare presentation includes diplopia, restricted
to the orbit. Among adults, the usual distance ocular motility, redness/pain/discharge due to
from the lateral orbital rim to the corneal apex associated exposure keratopathy.
is approximately 16–21 mm. 1,2 Proptosis is
said to be present when following criteria are
present. History of Present Illness
1. Protrusion more than 22 mm beyond the Following points must be noted while examining a
orbital rim. case of proptosis.
2. An asymmetry of more than 2 mm between the zz Age of onset: Age of onset can point towards the
eyes. probable diagnosis as shown in Table 1.
Proptosis is one of the common topics given zz Nature of onset
as a long case in exams. —— Sudden (hours to days): It suggests
inflammatory and infective process,

HISTORY trauma (orbital emphysema, fracture of the
medial orbital wall, orbital hemorrhage)
Chief Complaint or rupture of ethmoidal mucocele.
A case of proptosis usually presents with following —— Gradual (over many months to years): It
complaints: suggests tumors, lymphoma—prolifera

zz Protrusion of one or both the eyes. tive disorders.
zz Loss of vision: It indicates optic nerve zz Progression
compression/involvement [by intrinsic lesions —— Slow continuous: It suggests tumor,
of optic nerve such as meningiomas or optic however, gradual progression with

nerve gliomas or external compression, e.g. sudden increase in proptosis can harbor
tumors located in the orbital apex, such as a malignant transformation
2 Ophthalmology Clinics for Postgraduates
Table 1 Causes of proptosis based on the age of onset

Newborn Children Young adults Middle age Senile
• Orbital • Rhabdomyosarcoma • Thyroid • Pseudotumor • Malignant and
cellulitis • Hemangioma ophthalmopathy • Endocrine metastatic
• Orbital • Dermoid cyst • Pseudotumor • Malignant tumor of orbit
neoplasm • Orbital cellulitis • Orbital cellulitis lymphomas/leukemias • Pseudotumor
• Optic nerve glioma • Osteomas • Optic nerve sheath • Leukemia
• Craniosynostosis • Infiltrative meningiomas • Lymphomas
lymphomas tumors • Mucocele • Sarcomas
—— Rapid progression: It indicates infection/ Table 2 Common causes of bilateral proptosis

inflammation/hemorrhage/malignant
transformation Pathology Etiologies
—— Intermittent: It intermittent proptosis can Inflammations • Thyroid orbitopathy
be due to following causes • Wegener’s granulomatosis
Periodic orbital edema • Idiopathic inflammatory
Recurrent orbital hemorrhage/ pseudotumor
chocolate cyst and highly vascular • Myositis
tumors • Sarcoidosis
Increases during attacks of common
• Sjögren’s syndrome
cold/upper respiratory infections– Neoplasia • Lymphoma
lymphangioma • Leukemia
Postural (associated with bending • Metastatic carcinoma
forward) or with Valsalva suggests • Optic nerve glioma
orbital varices. Vascular lesions Arteriovenous shunts
Increases on crying capillary heman Varix
giomas in young children
zz Pain: Depending upon presence of pain
proptosis can be with deficient orbital roof such as congenital
—— Painful: Infective, acute inflammations, meningocele or meningoencephalocele, and
chocolate cyst, orbital hemorrhages traumatic or operative hiatus.
—— Painless: Tumor, endocrinopathy (Pain
can be there in some malignant tumors History of Past Illness

that show perineural spread, such as
A careful past history can point towards the
adenoid cystic carcinoma of the lacrimal
provisional diagnosis. Following points must be
gland).
asked in history of past illness:
zz Laterality
• Systemic inflammatory disease such as thyroid
Unilateral: Unilateral proptosis is seen in
disorder, sarcoidosis
tumors, cysts, and vascular anomalies
• Malignancy—Lungs, breasts, prostate
Bilateral: The different causes of bilateral
• Trauma
proptosis are summarized in Table 2.
• Periocular tumors
zz Special characteristics: Rarely, patient can give
a history of feeling the pulsation within the
orbit or periorbital area. An example of such Past Surgical History
cases include; AV malformation, carotico- Prior periorbital surgery or history of surgery
cavernous fistula and saccular aneurysm for intraocular malignancy such as malignant
of ophthalmic artery or due to transmitted melanoma might point to the possibility of orbital
cerebral pulsations in conditions associated extension or metastasis.
Oculoplasty 3
EXAMINATION —— Nonaxial/Eccentric:
Down and out: Dermoid, dermoli
General Examination/Specific Systemic poma, frontal and ethmoidal muco
Examination cele, meningocele
Down and in: Lacrimal gland tumor,
Look for signs of Graves’ disease/Sarcoidosis/Any
malignancy/Any infective foci. Dermoid
Upwards: Carcinoma of maxillary
sinus, lacrimal sac tumors, Lym

Ocular Examination phoma, maxillary sinus tumor, meta
Following points must be noted in ocular static tumors
Outwards: Lesion of anterior
examination:
ethmoidal sinus, nasopharyngeal
tumor, lymphangioma (Fig. 2),
Visual Acuity
lethal midline granuloma, Metastatic
In general, visual acuity is not affected with tumors, secondary tumor
orbital diseases except in cases with optic nerve
compression, refractive changes due to pressure
on back of the eyeball or exposure keratopathy.
Eyeball
A case of proptosis should be examined under
following headings:
A. Inspection
zz Head posture, facial asymmetry, shape of the
skull
zz Protrusion of the eye: In unilateral cases, there
will be obvious disparity between the two
eyes. In bilateral cases, some difficulty may be
there in early cases of proptosis. Following two
methods are useful in detection of proptosis: Fig. 1: Axial proptosis due to optic nerve glioma
1. Naffziger’s method: Relative protrusion can
be observed by simply standing behind
a seated patient and gazing downward
(tangentially) toward the chin from the
forehead to assess protrusion of the eye
beyond the orbital rim.
2. Worm’s eye view: It is similar to Naffziger’s
method but the difference is that the
examiner examines up from below with
the patient’s head-tilted back.
zz The direction of proptosis: The direction of
proptosis can indicate the probable etiology.
The direction can be following:
—— Axial: Thyroid related ophthalmopathy,
Glioma of optic nerve (Fig. 1), Optic

nerve sheath, meningioma, cavernous Fig. 2: Abaxial proptosis due to medial orbital
hemangioma. lymphangioma
Medial displacement: Dermoid cyst, zz Orbital thrill/Pulsation: Place your index and
lacrimal fossa tumors, and cysts, middle finger over the orbit (with closed lids)
sphenoid wing meningioma and observe following
zz Laterality: Unilateral or bilateral —— True pulsation: Finger will raise and
zz Ocular motility: Ocular motility disturbance separated

can be due to: —— Transmitted pulsation—finger will raise
—— Involvement of the rectus or oblique only

muscles directly zz Swelling/mass around the eyeball: If present
—— Affecting nerve supply of rectus or oblique its size, site, shape, consistency, fixity (skin/
muscles bone/muscle), signs of inflammation (Rubor—
—— From restriction of the orbital fascial redness; Color—raised temperature, check
connective tissue septae with your back surface of hand; Dolor—pain/
zz Eyelids: Lids can be affected by direct tenderness; Tumor—swelling; Functionless—
involvement of the levator muscle or the loss of function), and overlying skin changes
third cranial nerve, or because of associated must be noted carefully.
proptosis. Examination of lid is especially zz Regional lymph nodes: Palpate the subman
important in thyroid-associated eye disease. dibular and preauricular lymph nodes.
The various lid signs and their terminology has —— Medial and central lower lid (along the
been described in Table 3. facial vein), medial upper lid, central

zz Periorbital inflammation: Look for inflam upper lid and medial canthus drains into
matory signs (erythema, edema, chemosis, the submandibular lymph nodes
dilated vessels) of the periorbital structures —— Lateral upper lid and lateral lower lid
that can be associated with infections and drain into the preauricular parotid lymph
acute inflammatory diseases. nodes.
zz Nose/roof of mouth (sinus disease or when zz Orbital rim/margin with index finger to
intranasal source is suspected) and neck (for look for any mass, or erosion (malignancy),
goiter) irregularity (previous trauma)
zz Valsalva: Orbital varix or highly vascular zz Reducibility of the mass: Check for reducibility
lesions such as capillary hemangiomas will of the mass lesion. It can provide a clue about
enlarge with increases in arterial pressure. This the probable etiology, e.g. vascular tumors,
can be elicited with the Valsalva maneuver or schwannoma are reducible masses but
by asking the patient to bend forward. lacrimal gland tumor, pseudotumor are not
zz Lagophthalmos, Bell’s phenomena, and reducible.
exposure keratopathy zz Infraorbit al/supraorbit al anesthesi a :
Perineural invasion by a tumor can result in
B. Palpation pain, numbness, paresthesia in infraorbital or
It should be carried out to confirm the findings of supraorbital area. Adenoid cystic carcinoma
inspection and also for following: (ACC) of the lacrimal glands often presents with
zz Retropulsion: In a retropulsion test, thumb/ orbital pain and paresthesia, since this type of
two fingers/palm is used to gently push on tumor is frequently associated with perineural
the globe through the upper eyelid to know spreading (Remember, the lacrimal nerve
compressibility/resistance of the tumor. While is the smallest of the three branches of the
hemangioma, lymphangioma, orbital varices ophthalmic division of the trigeminal nerve. It
give a soft consistency; optic nerve glioma passes through the lacrimal gland then pierces
may give a firm consistency. Resistance to the upper lid and supplies the supraorbital
retropulsion suggests retrobulbar tumor or area. It provides sensory innervations for the
thyroid ophthalmopathy. lacrimal gland, conjunctiva, and the lateral
Oculoplasty 5
Table 3 Lid signs in proptosis

Sign Description
Abadie’s sign Elevator muscle of upper eyelid is spastic
Ballett’s sign Paralysis of one or more extraocular muscle (EOM)
Beck’s sign Abnormal intense pulsation of retina's arteries
Boston’s sign Jerky movements of upper lid on lower gaze
Cowen’s sign Extensive hippus of consensual pupillary reflex
Dalrymple’s sign Upper eyelid retraction
Enroth’s sign Edema especially of the upper eyelid
Gifford’s sign Difficulty in eversion of upper lid
Goldzieher’s sign Deep injection of conjunctiva, especially temporal
Griffith’s sign Lower lid lag on upward gaze
Hertoghe’s sign Loss of eyebrows laterally
Jellinek’s sign Superior eyelid folds is hyperpigmented
Joffroy’s sign Absent creases in the forehead on upward gaze
Jendrassik’s sign Abduction and rotation of eyeball is limited also
Knies’ sign Uneven pupillary dilatation in dim light
Kocher’s sign Spasmodic retraction of upper lid on fixation
Loewi’s sign Quick mydriasis after instillation of 1:1000 adrenaline
Mann’s sign Eyes seem to be situated at different levels because of tanned skin
Means’ sign Increased scleral show on upgaze (globe lag)
Moebius’s sign Lack of convergence
Payne/Trousseau sign Dislocation of globe
Pochin’s sign Reduced amplitude of blinking
Rieseman’s sign Bruit over the eyelid
Movement’s cap Eyeball movements are performed difficultly, abruptly and incompletely
phenomenon
Rosenbach’s sign Eyelids are animated by thin tremors when closed
Saiton’s sign Frontalis contraction after cessation of levator activity
Snellen-Rieseman’s sign When placing the stethoscope's capsule over closed eyelids’ a systolic murmur
could be heard
Stellwag’s sign Incomplete and infrequent blinking
Suker’s sign Inability to maintain fixation on extreme lateral gaze
Tellas’s sign Inferior eyelid might be hyperpigmented
Topolanski’s sign Around insertion areas of the four rectus muscles of the eyeball a vascular band
network is noticed and this network joins the four insertion points.
von Graefe’s sign Upper lid lag on downgaze
Wilder’s sign Jerking of the eye on movement from abduction to adduction
upper eyelids and adjacent supraorbital area, zz Locate the orbital notch with patients eyes
and partly the zygomaticotemporal area just closed (the deepest points on orbital rim) on
adjacent to the orbital rim. Hence, in case the temporal side of the orbital rim near the
ACC, these areas must be checked for sensory lateral canthus.
abnormalities). zz The prisms or the mirrors are slide across the
bar to adjust the footplates to fit on the orbital
C. Auscultation rim. The exophthalmometer is opened so that
Auscultation is done with the help of a stethoscope, the grooves are placed in the orbital notch.
over eyeball and temporal area, to look for bruit. zz The separation of the exophthalmometer
A bruit can be seen in cases of AV malformation. (baseline reading) is very important and must
always be noted and the exophthalmometer
D. Transillumination must always be set at that separation on future
It is helpful in evaluating anterior orbital or repeated readings.
lesions. It is usually performed using a penlight. zz The patient is then asked to open the eyes and
Interpretation is based on the opacity of the mass look straight ahead.
relative to surrounding tissues as follows: zz Look into the exophthalmometer, the red lines
zz Bright: The area lights up more than the should overlap to avoid the parallax. Look
surrounding tissues, e.g. cyst filled with clear into the mirrors located at each end of the
liquid, Dacryops exophthalmometer. Now note the millimeter
zz Equal: The area lights up to the same degree as mark corresponding to the corneal apex
surrounding tissues, e.g. lipoma. position on the scale and the readings on the
zz Indeterminate: The area seems darker than
cross bar for baseline reading.
surrounding tissues to a variable degree, e.g.
inclusion cyst, dermoid cyst Limitations: There are several limitations to
zz Dark: The area is clearly opaque, creating a Hertel’s exophthalmometer. The readings are
shadow effect. Aneurysm, e.g. solid tumors unreliable in presence of poor fixation, uncooper
(lacrimal gland tumors), osteoma. ative patients with convergence or repeated head
zz Remember some clinicians interpret trans movements. In addition, in presence of depressed/
illumination test as positive or negative; in that fractured lateral orbital rim, it cannot be done.
case first two categories can be considered
positive while the last two as negative trans Luedde’s exophthalmometer: It is a transparent
illumination test plastic ruler which is thicker than the normal ruler.
It has several advantages over an ordinary plastic
E. Exophthalmometry ruler such as, the reading starts from the apex
The most common type exophthalmometer used and the apex fits the orbital notch accurately. It is
in clinical practice is Hertel’s exophthalmometer. more accurate than Hertel’s exophthalmometer in
Other Exophthalmometers used in clinical practice presence of facial asymmetry.
are Luedde scale, Naugle exophthalmometer, Naugle exophthalmometer: It uses fixation points
and Gormaz exophthalmometer. However, in the slightly above and below the superior and inferior
absence of any of these an ordinary transparent orbital rims (cheekbones and forehead). Naugle
ruler can be used to measure proptosis. The exophthalmometer measures the difference
measurement, in Hertel’s exophthalmometer,
in proptosis between the two eyes rather than
is done from the lateral orbital rim to the
absolute measure with the Hertel method. It is
anterior corneal surface. A difference of greater
preferred in presence of an orbital fracture or after
than 2 mm between an individual patient’s
lateral orbitotomy.
eyes suggests proptosis. Procedure—following
points must be considered while using Hertel’s Interpretation: The normal range is 12–21 mm.
exophthalmometer. A difference of > 2 mm between the two eyes is
zz The patient and the examiner must be at the significant. In pediatric age group the readings
same level, eye to eye. may vary depending upon the age; <4 years old
Oculoplasty 7
(13.2 mm), 5–8 years old (14.4 mm), 9–12 years old Choroidal folds and opticociliary shunts may
(15.2 mm) and 13–17 years old (16.2 mm). be seen in patients with meningiomas. In cases
Conjunctiva: Examine carefully (with a slit-lamp) with cavernous sinus thrombosis retinal edema,
for following: exudates and engorged retinal veins can be seen
zz Dilated or tortuous blood vessels, chemosis, due to increased venous pressure.
dilated lymphatics-vascular malformation,
inflammation DIFFERENTIAL DIAGNOSIS
zz Hyperemia over the insertions of the horizon Based upon history and clinical examination
tal rectus muscles—one of the earliest sign of the differential diagnosis has to be made. The
thyroid eye disease commonly seen diseases in clinical practice
zz Corkscrew shaped tortuous dilated episcleral includes:
vessels-high-flow AV malformations or a zz Vascular (cavernous fistula, cavernous
carotid cavernous fistula hemangiomas)
zz Subconjunctival mass—anterior extension of zz Endocrine (thyroid eye disease)
a deeper orbital tumor, such as a lymphoma zz Inflammatory (orbital cellulitis, orbital
or intraocular melanoma with extraskeletal inflammatory disease, orbital cysticercosis)
orbital extension (a dark subconjunctival zz Neoplastic (ON glioma, ON meningiomas,
mass) lacrimal gland tumors, frontal sinus mucocele)
Cornea: Look for signs of exposure keratopathy.
Fluorescein staining can reveal early stage of INVESTIGATIONS
exposure keratopathy showing multiple punctate
defects. The corneal sensation must be checked in The line of investigation depends on upon the
all cases of proptosis. differential diagnosis arrived. Following tests must
Sclera/Iris: Careful examination is carried out to be done routinely in a case of proptosis.
look for signs of inflammation and any nodules
zz Thyroid function tests,
zz Complete blood count (CBC), peripheral
Pupil: Examination of pupil is extremely important smear (leukemia/lymphoma), erythrocyte
since it often provides the first clue of a probable sedimentation rate (ESR), blood sugar
optic nerve involvement. The presence of relative
afferent pathway defect (RAPD) must be ruled
Specific Tests
out in all cases. Any intraconal mass compressing
optic nerve or any tumor intrinsic to optic nerve Imaging Technique
(optic nerve glioma) can lead to RAPD.
Noninvasive techniques
Intraocular pressure (IOP): If possible IOP should zz Plain X-rays: It is often the initial radiological
be recorded in different gaze (ideally in al gazes examination, especially when other modalities
and at least in primary and superior) especially are not available. Commonly done exposures
in cases of suspected thyroid ophthalmopathy (in are in the Caldwell view, the Water’s view,
case of thyroid ophthalmopathy variable IOP in a lateral view and the Rhese view (for optic
upgaze can be an early sign due to an involvement foramina). The findings of orbital diseases in
of inferior rectus muscle). The IOP may vary due to X-ray include enlargement of orbital cavity,
the restricted mobility of extraocular muscles. calcification, hyperostosis and enlargement of
Lens: Lens is usually clear except the effect of optic foramina.
aging. zz Ultrasonography: It is a nonradiational
noninvasive, completely safe and extremely
Vitreous: Usually normal except in cases of valuable initial scanning procedure for orbital
intraocular tumor. lesions. In the diagnosis of orbital lesions, it
Fundus: Carefully look for signs of globe is superior to computed tomography (CT)
compression such as venous engorgement, scanning in actual tissue diagnosis and can
choroidal folds, and papilledema or optic atrophy. usually differentiate between solid, cystic,
infiltrative and spongy masses. The limitations the size and extent of the lesion that helps in
of USG are; limited ability to evaluate orbital proper surgical planning.
bones, periorbital sinuses, and the orbital apex zz Carotid angiography: Useful in cases of pul
due to poor distance penetration. satile proptosis and in those associated with a
zz Computed tomography scanning: It is extremely bruit or thrill. It helps to identify the location
helpful in determining the location and size of and extent of ophthalmic artery aneurysms, AV
an orbital mass. A combination of axial and malformations. It is also helpful in identifying
coronal cuts enables a three-dimensional the feeding vessels thus, for planning surgery
visualization. In addition to globe, extraocular in cases of vascular orbital tumors.
muscles, and optic nerves visualization it
can show areas adjacent to the orbits such as Histopathology
orbital walls, cranial cavity, paranasal sinuses
The exact diagnosis of many orbital lesions cannot
and nasal cavity. Mass lesions in the orbit
be made without the help of histopathological
usually appear as an abnormal density within
studies which can be accomplished by following
the typically low-density orbital fat. The lesion
techniques:
may be well defined with sharp borders (e.g. zz Fine-needle aspiration biopsy (FNAB): Quick,
cavernous hemangioma), or infiltrative with reliable, and relatively accurate. The biopsy
diffuse borders (e.g. pseudotumor). CT can aspirate is obtained under direct vision (when
give information about adjacent bone erosion there is an obvious mass)or guided by CT/USG
(suggestive of malignancy), remolding or fossa using a 23-gauge needle.
formation (encapsulated benign lacrimal zz Incisional biopsy: The scope of incisional
gland tumor such as pleomorphic adenoma, biopsy in the diagnosis of orbital masses is
encapsulated malignant lacrimal gland tumor, not clearly defined. It is often contraindicated
orbital dermoid), or displacement of adjacent due to the risk of tumor seeding or increased
bony orbital walls. Its main disadvantage is the risk of malignant transformation (e.g. benign
inability to distinguish between pathologically lacrimal gland tumor).
soft tissue masses which are radiologically zz Excisional biopsy: It is the procedure of choice
isodense. In addition, the risk of radiation especially when the mass is well encapsu
exposure (in contrast to MRI) is always there. lated or circumscribed. It is performed by
Examples of few findings on CT in cases of orbitotomy.
proptosis includes.
zz Magnetic resonance imaging (MRI): MRI is very
CLASSIFICATION/STAGING/SCORING
sensitive for detecting differences between
normal and abnormal tissues. Advantages of It depends upon the individual disease.
MRI over CT includes:
—— Better soft tissue visualization, especially
MANAGEMENT
in the region of the orbital apex, optic
canal, and cavernous sinus. The management depends upon the individual
—— Various fat suppression techniques diseases.
allow the visualization of gadolinium-
enhanced lesions that is often difficult to
see the normally high signal-generating VIVA QUESTIONS
orbital fat.
—— Better tissue differentiation
Q.1. Explain pseudoproptosis.
—— No radiation exposure.
Ans. Pseudoproptosis is either the simulation
of an abnormal prominence of the eye
Invasive procedures or a true asymmetry that is not caused
zz Orbital venography: Useful in orbital varix. by a mass, a vascular abnormality, or an
It confirms the diagnosis and also outlines inflammatory process.
Oculoplasty 9
Causes are multiple and include: • Orbital varies

Enlarged globe Transmitted
• Myopia • Congenital failure in the development
• Trauma of roof of the orbit, e.g. encephalocele/
• Glaucoma encephalomyelocele
Asymmetric orbital size • Traumatic or operative hiatus in orbit
• Congenital roof resulting in the formation of
• Postradiation meningocele
• Postsurgical
Q.4. The 6 P’s of the orbital history and
Asymmetric palpebral fissure physical examination are
• Contralateral ptosis
Ans. Useful in the diagnostic process
• Lid retraction
• Pain
• Facial nerve paralysis
• Proptosis
• Lid scar, ectropion, entropion
• Progression
Extraocular muscle abnormalities
• Palpation
• Postsurgical muscle recession
• Pulsation
• Paralysis or paresis
• Periocular changes
Contralateral enophthalmos
• Contralateral orbital fracture Q.5. Few facts about orbit dimensions.
• Contralateral small globe Ans. • Volume—30 cc
• Contralateral cicatricial tumor • Horizontal entrance width—40 mm
Physiological proptosis: It is proptosis in • Height at orbital rim—35 mm
infants due to the fact that orbital cavities • Orbital depth (rim to the optic strut)—
do not attain their full size so rapidly as the 45–55 mm
eyes. • Orbital segment of optic nerve—25 mm
Q.2. Most common cause of proptosis.
Ans. • Unilateral proptosis: Thyroid eye disease REFERENCES
• Bilateral proptosis: Thyroid eye disease
1. Albert DM, Miller JW, Azar DT. Albert
Q.3. Causes of pulsatile proptosis. & Jakobiec’s Principles and Practice of
Ans. True vascular Ophthalmology. 2008.
• Carotid cavernous fistula 2. Bowling B. Kanski’s Clinical ophthalmology:
• AV fistula in the orbit between ophtha A systematic approach, 8th edn. Edinburgh:
lmic artery and orbital vein, in the neck— Elsevier, 2015.
Carotid artery and jugular vein
• Highly vascular orbital tumor
LID TUMORS
Sapna Raghuwanshi, Amar Pujari, Mandeep S Bajaj
INTRODUCTION The common periocular malignancies are basal

cell carcinoma (BCC), squamous cell carcinoma
Lid tumors can arise from the epidermis, dermis, (SCC), sebaceous gland carcinoma (SGC), and
or adnexal structures of the eyelid. Malignant malignant melanoma. In western literature, basal
lesions are common around the eyes, because cell carcinoma constitute around 85% of the cases
many are induced by sun exposure or develop because of fair complexion. Whereas in India
from sun-related benign lesions. Typically, all three major variants constitute around 33%
most of these are small and slowly growing. each.1,2
HISTORY zz Ulceration of the lid margins

zz Loss of eyelashes
Demography zz Distortion of eyelid margin
Commonly, present in 5th to 6 decade of life with zz Ectropion/retraction (secondary due to lid
site of involvement: growth or skin contracture)
zz Involvement pattern in basal CC (lower eyelid, zz Increasing pigmentation of eye lid margins
caruncle, upper eyelid, lateral canthus) (Fig. 1) zz Palpebral preauricular lymph nodes
zz Involvement pattern in sebaceous CC (upper zz Dilated blood vessels (telangiectasias).
eyelid, lower eyelid) (Fig. 2)
zz Involvement pattern in squamous CC (lower History of Present Illness
eyelid, caruncle, upper eyelid) (Fig. 3).
Following points must be noted for a lid tumor:
zz Onset: Common lid tumors are insidious in
Chief Complaints onset
A case of lid tumor can present with presents with zz Progression: Progression is usually slow
following complaints: zz Any deterioration of vision
zz Slow, generally painless growth on lid margins zz Diplopia or restriction of eye movements
(frozen globe)
zz Any other swelling around the head and neck
region (preauricular and cervical lymph node
for metastasis)
zz Systemic features of metastasis.
Past History
Predisposing factors: Following are the pre
disposing factor for lid tumours:
zz Ultraviolet exposure (UV-B)
zz Ionizing radiation
zz Arsenic exposure
zz Psoralen plus ultraviolet A (PUVA)
zz Human papilloma virus
Fig. 1: Pigmented basal cell carcinoma of zz Genetic diseases (albinism, xeroderma
the left lower eyelid pigmentosum risk factor for BCC)
Fig. 2: Sebaceous gland carcinoma of Fig. 3: Squamous cell carcinoma of the eyelid with
the left upper eyelid extensive periorbital involvement
Oculoplasty 11
zz Recurrent Chalazion (could be sebaceous cell zz Impression cytology: After drying the area
carcinoma) to be examined, nitrocellulose filter paper
zz Previous surgery for eyelid malignancies is applied, and then firmly presses with
zz Topical chemotherapy. Goldmann tonometer head. Peel off paper
with forceps and place in appropriate fixative
Important Examination Findings solution for examination.
zz Fine needle aspiration cytology: In case of large
zz Eyelid: Careful examination of the eyelid for
tumor as a preliminary modality or in cases of
signs of lid malignancy
lymph node involvement.
zz Eyelid should be examined carefully for
depth or plane of involvement, lid margin
involvement, and medial and lateral canthal Imaging
involvement. Computed tomography is indicated in cases with
zz Bulbar and palpebral conjunctiva: In cases of postseptal extension to know the extent of orbital
sebaceous cell carcinoma to look for “Pagetoid spread and bony involvement. PET and SPECT in
spread” (Intraepithelial spread of tumor cells). cases to study the status of sentinel lymph nodes.
zz Cornea: Again in case of sebaceous cell
carcinoma
STAGING
zz Look for scleral invasion and globe integrity:
In cases where the tumor is extending beyond Staging is required to plan the treatment strategy
the orbital septum. and prognosis. The most common staging system
for carcinoma of the eyelid is the TNM system.
DIAGNOSIS The following information applies for all three
common eyelid carcinomas, but melanoma of the
The best way to confirm the diagnosis is excision eyelid is staged in the manner as skin melanomas.
biopsy. TNM stands for tumor, nodes, and metastasis.
This describes the size of the primary tumor, the
Histopathology number and location of any regional lymph nodes
metastatic foci anywhere in the body.
zz BCC: The tumor cells arise from the basal
layer of the epidermis. The cells proliferate
downwards thus exhibiting palisading at the
MANAGEMENT
periphery. Surgical Excision with Standard Frozen
zz Squamous cell carcinoma: The tumor arises Section Control
from the squamous layer of epidermis, well
differentiated tumors show characteristic This technique is performed by noting the clinical
keratin ‘pearls’. boundaries of the tumor edges and excising an
zz Sebaceous cell carcinoma: The tumor shows additional 3 mm cuff of normal-appearing tissue
irregular lobules consisting of sheets of followed by histopathological assessment of the
cells with varying degrees of sebaceous excised specimen for tumor free margins.
differentiation. The malignant cells show
foamy, multivacuolated cytoplasm, secondary Mohs’ Micrographic Surgery
to intracytoplasmic lipid.
This technique involves removal of the gross mass
of the tumor plus a small peripheral margin of
Biopsy Methods normal tissue. A thin layer of tissue, approximately
zz Incision biopsy: Where only a part of the 2 mm thick, is further excised from the entire base
tumor is removed using either blade or biopsy and edges of the wound. The initial specimen is
punch. divided into 4–7 µm thick portions on glass slides;
zz Excision biopsy: Here the entire tumor is the edges are marked with different colored dyes
removed. to maintain orientation. Frozen sections are
obtained from the under surface and skin edge zz Patients in whom surgery will result in
of each specimen. Locations of residual tumor extensive disfigurement with potential loss of
are marked on a map and only those areas are useful ocular function.
re-excised. Surgical resection is continued until Complications of radiotherapy includes:
there is a microscopically proven tumor-free —— Skin atrophy
plane. The defect is then reconstructed by the —— Ectropion
oculoplastic surgeon. —— Entropion
—— Nasolacrimal duct stenosis
Cryosurgery —— Keratitis
—— Conjunctival keratinization
It is useful for small lesions, but less effective for —— Cataract
larger and deeply invasive tumors. —— Loss of eyelashes
—— Globe perforation
Contraindications of Cryosurgery zz Chemotherapy: Used in cases of systemic

zz Involvement of the conjunctival fornix involvement
zz Fixation of tumor to periosteum zz Others like photodynamic therapy and CO 2
zz Sensory or motor denervation LASER treatment are rarely used.
zz Cold intolerance—cryoglobulinemia or cold
urticaria PROGNOSIS
zz Deeply pigmented skin
zz Indistinct margins Following are the poor prognostic factor:
zz Diameter of lesion >10 mm zz Duration of symptoms >6 months
zz Sclerosing or multicentric type. zz Vascular and lymphatic infiltration
zz Orbital extension
zz Poor tumor differentiation
Complications
zz Multicentric origin intraepithelial carcinoma
zz Depigmentation tous changes of the conjunctiva, cornea, or
zz Hyperpigmentation skin
zz Eyelid notching zz Location in the upper eyelid
zz Hypertrophic scar zz Tumor diameter >2 cm
zz Pseudoepithelial hyperplasia zz Location on central face or ears
zz Ectropion zz Long-standing presence prior to initial
zz Punctal and canalicular stenosis treatment
zz Lash loss. zz Incomplete excision
With conjunctival involvement: The adjuvant zz Aggressive subtype
methods followed are zz Perineural or perivascular involvement
zz Local application of cryotherapy zz Recurrent tumor:
—— Basal cell carcinoma (BCC) of the eyelid
zz Topical mitomycin C application.
rarely spreads to lymph nodes or other
Extensive disease: Exenteration for orbital involve organs, so the prognosis for this type of
ment and extended exenteration with radical neck tumor is usually very good.
dissection for metastatic disease beyond the orbit —— Squamous cell carcinoma (SCC) can be
with lymph node involvement. more aggressive than BCC and can spread
Radiotherapy: to the orbit, lymph nodes or other organs.
Indication of radiotherapy includes following: However, the prognosis is good if SCC of
zz Inoperable disease the eyelid is detected early and can be
zz Multiple medical problems completely removed.
zz Elderly patients unable to tolerate surgical —— The mortality rate (the number of people
resection who die from the disease each year) for

Oculoplasty 13
sebaceous gland carcinoma of the eyelid Q.2. Signs of lid malignancy

is about 5%–10%. However, sebaceous Ans. Following are the signs:
gland tumors are often not diagnosed • Destruction of lid architecture
early and have a high rate of recurrence • Ulceration of the lid margins
and spread (metastasis). • Loss of eyelashes
• Distortion of eyelid margin
• Ectropion/retraction (secondary due to
VIVA QUESTIONS lid growth or skin contracture)
• I ncreasing pigmentation of eye lid
Q.1.
Key points of individual malignancies. margins
Ans.
Basal cell carcinoma: • Palpebral preauricular lymph nodes

• It is the common type of eyelid tumour. • Dilated blood vessels (telangiectasias)

• Usually affects adult population.
Q.3. Differentiation of three lid malignancies

• Commonly involve the lower eyelid due
based on clinical findings
to exposure to sunlight.
Ans. See Text
Inherited conditions predisposing to BCC:
• Albinism Q.4. Sentinel lymph node biopsy
• Xeroderma pigmentosum Ans. Sentinel lymph node (SLN) biopsy is
• Basal cell nevus syndrome or Gorlin used for identifying the microscopic
syndrome nodal metastasis from a malignant tumor.
• Bazex syndrome Tumors may preferentially spread to a first
• Rombo syndrome draining or “sentinel” lymph node before
Sebaceous gland carcinoma (SGC) they spread to distant sites.3
(Sebaceous gland carcinoma, Meibomian Preoperatively: 99mTc-Sulfur colloid
gland carcinoma)[also see short case]: (t1/2 6 hours) will be injected around the
Important clinical points: tumor followed by hybrid SPECT/CT
• These arise from the Meibomian glands is performed to locate sentinel lymph
in the eyelid. nodes (Preauricular, intraparotid and
• It is more often in elderly women than submandibular).
men and often present late due to less Intraoperatively: In the first setting based
malignant course. on the previous SPECT/CT images maximal
• These tumors commonly arise from the radioactive counts were identified by hand
upper eyelid followed by the lower eyelid held gamma probe, followed by injection
the caruncle and bulbar conjunctiva. of 1% isosulfan blue dye perilesionally
The upper eyelid is more prone due to followed by gentle massage to augment
more number of Meibomian glands in lymphatic drainage. An incision will be
the upper eyelid (20–25) as compared to made over the area of highest radioactive
(15–20) in lower eyelid. count and lymph nodes are dissected. In
• S GC can be multifocal due to peculiar second setting eyelid tumors are excised
pattern of spread called “Pagetoid and reconstruction done.
spread” where there is an intraepithelial
Q.5. Map biopsy
spread of the tumor.
Ans. See text
• T he gross appearance resembles
yellowish nodular mass Q.6. Reconstruction of the eyelid defects.
• May resemble blepharoconjunctivitis. Ans. Lid reconstruction:
• Can involve the orbit and regional lymph Anterior lamella reconstruction:
nodes. • Primary closure
• Full thickness skin grafts • Tarsoconjunctival graft.

• Musculocutaneous flap

Full thickness lid defect—See Table 1.
• Advancement flap
• Transposition flap Q.7. Different histological types of BCC
• Rhombic flap. Ans. See Table 2
Posterior lamella reconstruction Q.8. Different lid manifestation of Malignant
• Buccal mucosa (preferred) Melanoma
• Hard palate mucosa Ans. See Table 3
Table 1 Full thickness lid reconstruction

Size of the lid defect Repair
<25% Direct closure
25–50% Direct closure with cantholysis
33–66% Semicircular flap (Tenzel flap) alone or along with the periosteal flap
50–75% Cutler Beard (Upper eyelid defect)
Hughes procedure (Lower eyelid defect)
75–100% Lower eyelid (Tarsoconjunctival flap with skin grafting)
Upper eyelid (Median forehead flap with mucus membrane grafting
Table 2 Lid manifestation of BCC

Nodular-ulcerative Pigmented Morphea or sclerosing Superficial Fibroepithelioma
• Most common • Similar to the • Least common • Scaling • Pedunculated or
lesion noduloulcerative • Flat, indurated, yellow- patch with a sessile smooth,
Pink or pearly type in pink plaque with raised pearly pink nodule.
papule or nodule morphology ill-defined borders border • Arise on the trunk
• Overlying • Brown or black • Aggressive and may • Arise on the rather than the
telangiectatic pigmentation invade the dermis trunk rather eyelid
vessels present • More common in deeply than the
• Central ulceration dark complexion • Invade into the eyelid
with rolled border persons paranasal sinuses and
‘rodent ulcer’ orbit
• Mimic
blepharoconjunctivitis
Table 3 Lid manifestation of malignant melanoma

Lentigo maligna Superficial spreading
melanoma melanoma Nodular melanoma Acral lentiginous melanoma
Slowly expanding Plaque with irregular • Blue-black nodule Occurs on the palms, soles, and
pigmented, flat, outline, variable with normal distal phalanges as well as on the
nonpalpable, tan to pigmentation surrounding skin mucous membranes
brown macule with • May be non-
irregular borders pigmented
Oculoplasty 15
REFERENCES 3. Lokdarshi G, Vuthaluru S, Pushker N, Kumar

R, Kashyap S, Mathur S. Sentinel lymph
1. Albert DM, Miller JW, Azar DT. Albert node biopsy in malignant eyelid tumor:
& Jakobiec’s principles and practice of hybrid single photon emission computed
ophthalmology; 2008. tomography/computed tomography and dual
2. Bowling B. Kanski’s Clinical Ophthalmology: dye technique. Am J Ophthalmol. 2016;162:
A Systematic Approach, 8th edn. Edinburgh: 199-200.
Elsevier; 2015.
PTOSIS
INTRODUCTION For example:

—— Associated double vision points towards
Drooping of the eyelid is known as ptosis or 3rd cranial nerve palsy/aberrant regene-
blepharoptosis. It is a common pathology seen in ration.
all age group. It is given as both long and short case —— Alteration in an amount of ptosis with jaw
in the examination. The most important part in a movements (jaw-winking phenomenon)
case of ptosis is its examination. can be seen in Marcus Gunn syndrome.
—— Difficulty in deglutition is seen in oculo
HISTORY pharyngeal muscular dystrophy.

—— Limitation of ocular motility can be seen
Chief Complaints in myasthenia gravis and Kearns-Sayre

A case of ptosis can present with following syndrome.
—— Worsening of ptosis as the day progresses
complaints:
zz Adult patient commonly complaints of is seen in myasthenia gravis (Levator
drooping of the upper lid. palpebrae superioris (LPS) weakness
zz In children, parents often complaints of an compensated for by Müller’s muscle
affected eye being smaller in comparison to in myasthenia, which fatigues with
another eye. progression of the day)
—— Associated recurrent allergic conjunctivi
zz Diminution of vision is not a common
tis can cause mechanical ptosis.
complaint and amblyopia is associated only
with severe ptosis due to stimulus deprivation.
Past History
The prevalence of amblyopia is around 12% to
20 % in cases with severe congenital ptosis.1-4 Following points must be noted in history in a
ptosis case:
zz Recurrent episodes of ptosis can be seen
History of Present Illness
in recurrent 3rd nerve palsy, e.g. ischemic
It is important to note about following points: neuropathy associated with diabetes or
zz The age of onset and duration (congenital or hypertension.
acquired) zz Contact lens wear (ill-fitting contact lens may
zz Progression of ptosis (chronic progressive cause blepharospasm and small palpebral
external ophthalmoplegia) aperture which may be confused with
zz Any head position (e.g. chin lift) ptosis)
zz Associated symptoms can often indicate the zz Drug intake such as neostigmine can point
underlying cause. towards the possible diagnosis.
zz Trauma (lid or facial trauma mat cause scarring zz Pseudoptosis: Ipsilateral microphthalmos
or LPS damage) to rule out traumatic ptosis or contralateral lid retraction can lead to
zz Medical conditions, e.g. myasthenia gravis, pseudoptosis.
myotonia, muscular dystrophies, diabetes and zz The presence of strabismus must be ruled out
hypertension. by Cover-Uncover test.
zz Spectacle use or amblyopia therapy during zz Extraocular muscle function should also be
childhood is important when there is asso assessed in primary and secondary gazes.
ciated vision loss. The presence of motility abnormalities is
seen in congenital conditions such as double
Past Medical History elevator palsy (combined superior rectus and
LPS muscle maldevelopment), congenital
Diabetes mellitus (DM), hypertension, bleeding
oculomotor palsy and acquired conditions
diathesis (important for surgery), recurrent stye/
such as ocular or systemic MG, chronic
chalazion/vernal keratoconjunctivitis (mechanical
progressive external ophthalmoplegia,
ptosis), any neurological disorder must be ruled
oculopharyngeal dystrophy, and oculomotor
out.
palsy with or without aberrant regeneration.
Past Surgical History Eyelid: Following points must be noted:

zz If ptosis is unilateral (Figs 1 and 2) or bilateral
Peribulbar block for any intraocular surgery, zz Signs of previous trauma such as eyelid scar
cataract surgery can lead to ptosis. Any previous zz Mechanical causes of ptosis such as eyelid
squint surgery or ptosis surgery must be recorded. tumors, multiple chalazia, giant papillae
zz Lagophthalmos—important for surgical
Birth History planning
Such as pregnancy, delivery, neonatal period, and zz Pupillary reaction—pupillary involvement can
early development are important in congenital be seen in cases of 3rd CN palsy and Horner’s
ptosis. Birth history such as instrument/forceps syndrome
delivery can lead to ptosis point towards the cause zz The position of the lids should be noted in the
of ptosis. different position of gaze. Variability of ptosis
in the different position of gaze is an indication
of aberrant regeneration after third-nerve
Family History
palsy.
Positive family history can be present in zz Note also the speed of saccades: slow saccades
blepharophimosis syndrome. are indicative of myopathic muscles.
EXAMINATION
Visual acuity: Visual acuity and refractive error
must be assessed in all cases of congenital or
childhood ptosis in order to identify and treat the
child with concomitant amblyopia. Amblyopia
can result from anisometropia, high astigmatism,
strabismus or occlusion of a pupil. Amblyopia
occurs in approximately 12–20% of patients
with congenital ptosis.
Facial symmetry and orbit: Look for following:
zz Head posture: Chin elevation is seen in cases of
bilateral moderate to severe ptosis.
zz Frontalis overaction: Raised eyebrows to
compensate for ptosis. Fig. 1: Simple severe congenital ptosis before surgery
Oculoplasty 17
the formation of the upper eyelid crease.

High, duplicated or asymmetric creases may
indicate an abnormal position of the levator
aponeurosis. The upper eyelid crease is
8–9 mm in males and 9–11 mm in females.
The crease is usually elevated in patients
with involutional ptosis and is often shallow
or absent in patients with congenital ptosis.
The height of the crease on the normal side
should be measured and compared to the
ptotic eyelid in downgaze. In patients, when
more than one lid crease is present, the most
prominent one should be considered.
zz Levator Palpebrae Superioris (LPS) function
Fig. 2: Simple severe congenital ptosis after surgery Berke’s Method (lid excursion): LPS function
is estimated by measuring the upper eyelid
excursion from downgaze to upgaze with
zz Variation in the amount of ptosis with frontalis muscle function negated. Fixating
extraocular muscle or jaw muscle movements the brow with digital pressure minimizes
(synkinesis) should be noted. Synkinesis may contributions from accessory elevators of the
be seen in Marcus Gunn jaw-winking ptosis, eyelids such as the frontalis muscle. Failure
aberrant regeneration of the 3rd CN or the VII to negate the influence of the frontalis muscle
CN, and some types of Duane syndrome. results in an overestimation of LPS function.
LPS function can be graded according to
Measurement of Ptosis Beard’s classification
—— Normal: >15 mm
zz Margin-reflex distance 1 (MRD1): It is the —— Good: 12 –14 mm
distance from the upper eyelid margin to the —— Fair: 5–11 mm
corneal light reflex in the primary position. It —— Poor: <4 mm
is the single most important measurement in
describing the amount of ptosis. The MRD1 is Putterman’s method: This is carried out by the
also checked in the reading position. measurement of the distance between the middle
zz Margin-reflex distance 2 (MRD2): The MRD2 of upper lid margin to the 6’o clock limbus in
is the distance from the corneal light reflex to extreme upgaze. This is also known as the margin
the lower eyelid margin. MRD2 is a measure of limbal distance (MLD). Normal is about 9.0 mm.
lower eyelid retraction (or scleral show). Assessment in children:
zz Palpebral aperture: The vertical interpalpebral zz Assessment of LPS function in small children
fissure is measured at the widest point between is a difficult task, as the child allows no formal
the lower eyelid and the upper eyelid. This evaluation. Following methods may help
measurement is taken with the patient fixating zz The presence of lid fold and increase or
on a distant object in primary gaze. Normally, decrease in its size on a movement of the
the palpebral fissure height in males, is eyelid gives us a clue to the LPS action.
7–10 mm, and in females, it is 8–12 mm. zz The presence of anomalous head posture like
Remember the sum of the MRD1 and the MRD2 the child throwing his head back suggests a
should equal the vertical interpalpebral fissure poor LPS action.
height. zz Iliff test: This test can be performed in the first
zz Margin crease distance (MCD): It is the year of life to evaluate the levator function. The
distance from the upper eyelid crease to the upper eyelid of the child is everted as the child
eyelid margin. The insertion of fibers from looks down. If the levator action is good, lid
the LPS muscle into the skin contributes to reverts on its own.
Lagophthalmos: The patient should be assessed are present in some acquired and congenital
for lagophthalmos and if it is present, the degree conditions associated with ptosis (e.g. Horner
should be noted, checking head position, chin syndrome, cranial nerve III palsy). Miosis that
elevation, brow position, and brow action in is most apparent in dim illumination is seen in
attempted upgaze. Lagophthalmos and poor tear Horner syndrome and mydriasis is seen in some
film quantity or quality may predispose a patient cases of 3rd CN palsy.
to complications of ptosis repair such as dryness Fundus: Fundus examination after mydriasis is
and exposure keratitis. essential for any concomitant fundus abnormality.
Bell’s phenomenon: it is an upward and outward Rest of the findings such as iris, lens, sclera and
movement of the eye when an attempt is made IOP are usually within normal limits.
to close the eyes. Bell’s phenomenon is a normal
defense reflex. DIFFERENTIAL DIAGNOSIS
zz Demonstration: Ask the patient to close the eye
forcibly (as if the patient wants to sleep). The A case of ptosis must be differentiated from
examiner then lifts the patient’s upper eyelid pseudoptosis. Pseudoptosis is apparent eyelid
manually. In a patient with a normal Bell’s drooping due to ocular or adnexal diseases should
phenomenon, the globe will rotate upwards be differentiated from true ptosis. On elevating
and outwards and the eyelid will cover the the ptotic lid, the other eyelid droops slightly in
cornea. true ptosis while remains at the same level in
zz Significance: If a patient does not have a pseudoptosis. Causes of pseudoptosis includes
good Bell’s phenomenon, a cautious ptosis following:
correction should be undertaken to prevent zz Unilateral:
—— Hypertropia
subsequent corneal exposure, especially when
—— Enophthalmos
planning for sling surgery.
—— Microphthalmia
zz Bell’s phenomenon is graded into three
—— Anophthalmia
grades:
—— Phthisis bulbi
1. Good: Less than one-third of cornea visible
—— Superior sulcus defect
2. Fair: One-third to one-half of the cornea
—— Dermatochalasis
visible
3. Poor: More than one-half of cornea visible zz Contralateral:
—— Upper eyelid retraction
zz Inverse Bell’s phenomenon: If the cornea is
—— Proptosis
not in upgaze or if it moves to other position
—— Buphthalmos
of gaze, such as downgaze on closing the eyes
than it is called inverse Bell’s.
INVESTIGATION
Cornea A case of ptosis usually does not need any special
zz The corneal sensation must be checked in all investigation other than routine tests done before
cases. A normal corneal sensation is essential surgery. Investigations like visual field may be
for normal blink reflex and prevention of required in special situations as discussed under
exposure keratitis the following surgery. Viva questions.
zz Quantity and quality of the tear film must
be documented in the initial examination. CLASSIFICATION
Schirmer test, tear break-up time (TBUT) and Based on the onset
Tear meniscus must be recorded in all cases of zz Congenital ptosis: Ptosis present since birth.
ptosis. Dry eye syndrome is a contraindication It can be further categorized into following:
for ptosis surgery; especially sling surgeries as —— Congenital simple ptosis
it may cause corneal damage postoperatively. —— Complicated
Pupil: Pupillary examination is important in With oculomotor abnormalities
the evaluation of ptosis. Pupil abnormalities With blepharophimosis syndrome

Oculoplasty 19
Synkinetic ptosis zz The difference from normal in bilateral cases

- Marcus Gunn Jaw Winking gives the amount of ptosis.
- Misdirected third nerve ptosis
zz Acquired ptosis: True acquired ptosis is the
result of some disturbance of the upper lid
retractors, the levator or Müller’s muscle, or
both, and is best classified according to its
primary cause, which includes; mechanical
(Fig. 3), myogenic, neurogenic, and aponeu
rotic. Differentiating points between congeni
tal and acquired ptosis are given in Table 1
Bases on pathogenesis: As shown in Table 2.
STAGING/SCORING
The amount of ptosis can be determined by:
zz The difference in MRD 1 of the two sides in
unilateral cases Fig. 3: Right upper eyelid mechanical ptosis
Table 1 Differences between congenital and acquired ptosis

Parameters Congenital ptosis Acquired ptosis
MRD 1 Mild-to-severe ptosis Mild-to-severe ptosis
Upper eyelid crease Weak or absent crease in normal position Higher than normal crease
LPS function Reduced Near normal
Downgaze Eyelid lag (lid lag sign) Eyelid drop
Palpebral aperture Greater in downgaze Less in downgaze
Abbreviations: MRD, margin-reflex distance; LPS, levator palpabrae superioris
Table 2 Classification of ptosis based on pathogenesis

Type Mechanism Example
Myogenic • Maldevelopment of elevators • Congenital simple ptosis
• Myopathic conditions involving the LPS or • Blepharophimosis syndrome
myoneural junction. • Double elevator palsy
• Direct damage to LPS or myoneural • Congenital ocular fibrosis syndrome
junctions • Chronic progressive external
ophthalmoplegia
• Oculopharyngeal dystrophy
• Muscular dystrophy
• Traumatic
Neurologic • Dysfunction of the third cranial nerve • Oculomotor nerve palsy
• Dysfunction of sympathetic innervation to • Horner’s syndrome
Müller’s muscle • Myasthenia gravis
• Aberrant regeneration after oculomotor • Marcus Gunn jaw-winking phenomenon
nerve palsies
Contd…
Contd…
Type Mechanism Example
Aponeurotic • Dehiscence in the central part of the • Involutional (aponeurosis dehiscence)
aponeurosis • Post-traumatic
• Disinsertion of the aponeurosis from the • Post-surgical blepharochalasis
tarsus • Chronic, recurrent edema
• Thinning and stretching, termed • Pregnancy
attenuation or rarefaction, of the • Chronic ocular inflammation
aponeurosis attenuation or rarefaction, • Rigid contact lens wear
of the aponeurosis
Mechanical • Excessive weight • Dermatochalasis
• Eyelid mass
• Giant papillae/VKC
• Multiple chalazion
• Orbital mass
• Scarring
Grading VIVA QUESTIONS

zz Mild ptosis—2 mm or less
zz Moderate ptosis—3 mm Q.1. What are visual problems due to ptosis?
zz Severe ptosis—4 mm or more. Ans. • Ptosis is a common cause of reversible
peripheral visual loss. Although, the
MANAGEMENT superior visual field is most often
The aim of the surgery is to lift the ptotic lid involved, central vision can also be
above the pupillary aperture when the eyes are affected. Patients with ptosis complain
in the primary position. The height of the two of difficulty with reading because the
lids regardless of whether the ptosis is unilateral ptosis worsens in downgaze. Ptosis has
or bilateral should be equal. There should also also been shown to decrease the overall
be adequate mobility of the lid when blinking, amount of light reaching the macula
a normal lid fold and no diplopia. The surgical and, therefore, can reduce visual acuity,
procedures and their indications are as follows: especially at night.
zz Fasanella Servat Operation • The restricted peripheral visual field is
—— Mild ptosis (<2 mm or less)
a contraindication for issuing of driving
—— Levator action >10 mm
license and jobs requiring broader fields
—— Well defined lid fold—no excess skin
in western countries. Visual fields less
zz Levator resection
—— Mild/moderate ptosis
than 10 degrees in the better eye with the
—— Levator action ≥ 4 mm
best correction are considered as legal
zz Brow suspension ptosis repair blindness.
—— Severe ptosis
—— Levator action <4 mm

Q.2. What is the normal position of UL?
—— Jaw-winking ptosis or blepharophimosis Ans. The vertical interpalpebral fissure is
syndrome measured at the widest point between
The treatment of congenital ptosis has been the lower eyelid and the upper eyelid.
described in detail in chapter congenital ptosis Normally, the UL should cover 1/6th or
(short case). The treatment of acquired ptosis 2 mm of the cornea and lower lid should
depends upon the cause. just touch the limbus.
Oculoplasty 21
Q.3.
Classification of ptosis Q.9. How to demonstrate and grade Marcus
Ans.
Ptosis can be classified as following: Gunn Jaw winking?
• Based on onset: Congenital or acquired.
Ans. Synkinesis is best demonstrated by having
• Based on etiopathogenesis: Myogenic,
the patient move the jaw to the opposite
aponeurotic, neurogenic, mechanical side of the ptotic eye, but widely opening
and traumatic. the mouth or moving the jaw forward
The most common type of congenital would also elevate the eyelid.
ptosis results from a poorly developed Grading of Marcus Gunn jaw-winking
levator muscle LPS (myogenic). The most phenomenon is based on the amplitude of
common type of acquired ptosis is that lid movement:
caused by stretching or disinsertion of • Mild—2 mm or less
the levator aponeurosis (aponeurotic). • Moderate—3–6 mm
Q.4. Differentiate between congenital and • Severe—7 mm or more
acquired ptosis Q.10. What are the other ancillary tests that
Ans. See Table 1 should be done in ptosis?
Q.5. What is blepharophimosis epicanthus Ans. • V isual field testing with the eyelids
inversus ptosis syndrome (BPES)? untaped (in the natural, ptotic state)
Ans. Details in short cases. and taped (artificially elevated) helps
determine the patient’s level of functional
Q.6. What are Bells phenomenon and its visual impairment. Comparison of the
grading? taped and untaped visual fields gives
Ans. See Examination an estimate of the superior visual field
Q.7. The importance of Hering’s law in ptosis improvement that can be anticipated
Ans. In cases with bilateral ptosis and with one following surgery.
side having marked ptosis compared to the • Pharmacologic testing may be helpful
other side, following surgical correction in confirming the clinical diagnosis
on the greater ptotic side, the side with of Horner syndrome, myasthenia
minimal ptosis may droop more. This is gravis. Fluctuating ptosis that seems
due to the Hering’s law. This is important to worsen with fatigue or prolonged
to predict the postoperative results of ptosis upgaze, especially when accompanied
surgery. Patient has to be warned that the by diplopia or other clinical signs of
contralateral eye may droop following systemic MG.
correction of the greater ptotic lid to avoid • P henylephrine test: One drop of
any postoperative unrealistic expectation phenylephrine 2.5% is installed in the
by the patient. upper fornix, stimulates the alpha-
receptors in Müller’s muscle, causing its
Q.8. What should be the sequence of surgery
contraction and hence lid elevation. It
if ptosis and strabismus coexist?
is possible to demonstrate the potential
Ans. Since correction of the strabismus may
outcome of surgery to a patient with a
relieve the ptosis, strabismus surgery
positive response to phenylephrine. One
should be performed before treatment
can also unmask a coexisting ptosis in the
of ptosis. An exception may be made for
so-called normal eye, which appeared
cosmetically acceptable strabismus for
normal due to increased LPS stimulation
which the only ptosis needs to be treated.
(Hering’s law).
If the patient has horizontal strabismus
with ptosis, surgery for both strabismus Q.11. The significance of pupillary examination
and ptosis can be performed at the same in ptosis.
sitting because the result of one is unlikely Ans. • A small pupil (miotic) indicates Horner’s
to influence the result of the other. syndrome
• A large pupil might be a sign of third- • S

ingle-fiber electromyogram (EMG:
nerve palsy. 100% sensitivity) and muscle biopsy are
• In aberrant third-nerve palsy, the size of other more invasive tests that are helpful
a pupil may change in different position in identifying the site of pathology in
of gaze. myopathic and myasthenic ptosis.
Q.12. What is MRD3? Q.14. Postsurgical ptosis?
Ans. MRD3 is the distance from the ocular Ans. The incidence of ptosis after cataract
light reflex to the central UL margin when surgery has been reported to be as high as
the patient looks in extreme upgaze. In 13%.3 Although it can be seen following
unilateral ptosis, the difference between any intraocular surgery, it is often seen
normal and abnormal MRD3 multiplied by following cataract and vitreoretinal surgery.
3 approximately shows the amount of LPS It can be transient/acute ptosis that resolves
that must be resected. after surgery or chronic/persistent ptosis
Q.13. What are the bedside tests to rule out that persists after surgery
myasthenic ptosis? Etiopathogenesis: Postsurgical ptosis can be
Ans. • Fatigability: Ask the patient to look up due to the following:
and down for about 1 min to induce • Myogenic due to the process of injecting
fatigue or sustained upgaze for 1 min anesthetic into the muscle or myotoxic
will achieve the same result. Progressive effects of the anesthesia
ptosis will ensue in a myasthenic patient. • Aponeurotic: Due to use of a bridle suture
MRD1 is measured before and after or rigid lid speculum
these fatigue tests. Note that myasthenic • Neurogenic: Due to the prolonged effects
patients might also develop diplopia of anesthetic on the neuromuscular
with this test. junction, causes transient neurogenic
• Cogan’s twitch sign: The patient is first ptosis
asked to look down for 15 s. A small • Mechanical: May be due to edema or
upshoot of the eyelid is noted as a hematoma formation in the eyelid
myasthenic patient then moves back to • Traumatic: Due to blunt or sharp trauma
the primary position. to the levator aponeurosis
• Ice-pack test: Apply an ice-filled glove Prevention: Prevention of postsurgical
to the affected eye for 10 min. In a ptosis is an essential part of the modern
myasthenic patient, the ptosis improves ocular surgery.
by 2 mm or more. • Topical anesthesia eliminates all
Tests to confirm Myasthenia: problems with local anesthesia including
• Edrophonium chloride test or Tensilon hematoma and edema of the eyelid and
test: This test is done in doubtful myotoxic effects on the levator.
cases where an acquired ptosis due • Use of ocular massage and compression
to Myasthenia Gravis is suspected. In decreases the amount of eyelid edema
adults, 2 mg of edrophonium is injected and hematoma formation.
slowly in 15–30 seconds. The needle • L imit surgical time and thus eyelid
is left in situ and the remaining 8 mg is complications secondary to ocular
injected slowly if no untoward incident inflammation or compressive effects of
is observed within 1 minute. The effect prolonged use of a lid speculum.
occurs in 1–5 minutes and if myasthenia • D isuse of bridle sutures or a rigid
is the cause, ptosis improves after speculum
edrophonium injection. • Superior approach to surgery have a
• Acetylcholine receptor antibody test when greater risk compared to a temporal
positive has 100% specificity. approach
Oculoplasty 23

Treatment: After a thorough examination Surgical correction may be difficult, requir
in which the etiology is determined, one ing frontalis sling procedures.
must decide whether to intervene. In most
Q.17. What is aponeurotic ptosis?
cases, postsurgical ptosis resolves with
time, and therefore observation is the most Ans. It is the most common form of acquired
prudent form of intervention. This form of ptosis. It results from stretching or dehi
ptosis typically improves within six months. scence of the levator aponeurosis or disin
Ptosis that does not resolve is typically sertion from its normal position. Common
secondary to aponeurotic dehiscence; this causes are involutional attenuation or
is readily repaired surgically. repetitive traction on the eyelid, which
may occur with frequent eye rubbing or
Q.15. What is traumatic ptosis? prolonged use of rigid contact lenses. It
Ans. Trauma to the levator aponeurosis or the can also occur due to intraocular surgery
LPS muscle may also cause ptosis through or eyelid surgery. The characteristic sign
myogenic, aponeurotic, neurogenic, or is a high or an absent upper eyelid crease
mechanical defects. Eyelid lacerations secondary to upward displacement or loss
exposing preaponeurotic fat indicate that of the insertion of LPS fibers into the skin.
the orbital septum has been transected Thinning of the eyelid superior to the upper
and suggest the possibility of damage to the tarsal plate can also be there. LPS function
levator aponeurosis exploration of the LPS in aponeurotic ptosis is usually normal
muscle or aponeurosis is indicated in these (12–15 mm) and worsens in downgaze.
patients if LPS function is diminished or
ptosis is present. Orbital and neurosurgical Q.18. Describe neurogenic ptosis.
procedures may also lead to traumatic Ans. Congenital neurogenic ptosis is caused by
ptosis. The ophthalmologist normally innervational defects during embryonic
observes the patient for 6 months before development. It is rare and commonly
considering surgical intervention. associated with congenital 3rd cranial
nerve palsy, Horner syndrome or Marcus
Q.16. What is myogenic ptosis? Gunn Jaw-Winking syndrome. It manifests
Ans. Congenital myogenic ptosis: This type of as ptosis together with an inability to
ptosis is due to dysgenesis of the leva elevate, depress, or adduct the globe. The
tor palpebrae superioris (LPS) muscle. pupils may also be dilated.
Congenital ptosis caused by maldevelop • Congenital Horner syndrome is a mani
ment of the LPS muscle is characterized festation of an interrupted sympathetic
by decreased LPS function, eyelid lag, nervous chain. It is associated with mild
and sometimes, lagophthalmos. The ptosis, miosis, anhidrosis, and decreased
upper eyelid crease is often absent or pigmentation of the iris on the involved
poorly formed, especially in cases of more side. Decreased sympathetic tone to the
severe ptosis. Congenital myogenic ptosis inferior tarsal muscle in the lower lid (the
associated with a poor Bell’s phenomenon analog of the Müller’s muscle), results
or with vertical strabismus may indicate in elevation of the lower eyelid. This
concomitant maldevelop m ent of the phenomenon is called as lower eyelid
superior rectus muscle (double elevator reverse ptosis. The combined upper and
palsy, or monocular elevation deficiency). lower eyelid ptosis decreases the vertical
Acquired myogenic ptosis: It is uncommon interpalpebral fissure and may confuse
and results from localized or diffuse mus with enophthalmos. The pupillary miosis
cular diseases such as muscular dystrophy, is most apparent in dim illumination.
chronic progressive external ophthalmo • Marcus Gunn Jaw-Winking syndrome
plegia, MG, or oculopharyngeal dystrophy. (Figs 4 and 5) is the most common form
Fig. 4: Right-sided severe complicated ptosis Fig. 5: Right-sided severe complicated ptosis with
improvement in ptosis after chewing movement
of congenital synkinetic neurogenic It can be caused by a congenital abnormality,

ptosis. such as a plexiform neurofibroma or
• S ome forms of Duane retraction syn hemangioma, or by an acquired neoplasm
drome also cause elevation of a ptotic such as a large chalazion, skin carcinoma,
eyelid with the movement of the globe. or orbital mass. Postsurgical or post-
Acquired neurogenic ptosis results from traumatic edema can also cause temporary
interruption of normally developed mechanical ptosis.
innervation and is most often secondary
to an acquired III-N palsy, to an acquired REFERENCES
Horner syndrome, or MG. Less common
1. Brad Bowling. Kanski’s Clinical Ophthalmology:
causes of acquired neurogenic ptosis
A systematic approach, 8th edn. Edinburgh:
include myotonic dystrophy, chronic
Elsevier; 2015.
progressive external ophthalmoplegia, 2. Albert DM, Miller JW, Azar DT. Albert &
Guillain-Barré syndrome, and oculo Jakobiec’s Principles and Practice of Ophthal
pharyngeal dystrophy. Botulinum toxin mology; 2008.
injection in the forehead or orbital region to 3. Oral Y, Ozgur OR, Akcay L, Ozbas M, Dogan
ameliorate benign essential blepharospasm OK. Congenital ptosis, and amblyopia. J Pediatr
can also lead to this form of ptosis. Ophthalmol Strabismus. 2010;47:101-4.
4. Skuta GL, Cantor LB, Weiss JS. Basic and
Q.19. What is mechanical ptosis? Clinical Science Course Orbit, Eyelids, and
Ans. Mechanical ptosis usually refers to the Lacrimal System American Academy of
condition in which an eyelid or orbital mass Ophthalmology. Section 7 San Francisco,
weighs or pulls down the upper eyelid. 2011–2012.
Oculoplasty 25
CONTRACTED SOCKET
Varsha Vashney, Aditi Dubey, Amar Pujari
INTRODUCTION zz Cicatrizing conjunctival diseases may lead to

progressive forniceal shortening
The contracted socket is defined as the shrinkage zz Chronic inflammation and infection that
and shortening of all or a part of orbital tissues might have led to the surgery
causing a decrease in depth of fornices and orbital zz Mutilating trauma to eye in which evisceration
volume ultimately leading to inability to retain might have done to prevent sympathetic
prosthesis. it is characterized by extensive loss of ophthalmia.
conjunctival surface area, deep cicatrix formation,
atrophy of the orbital fat, fornix contraction and
Volume redistribution leading to post-enucleation
Surgical History
syndrome. History of prior surgery; such as enucleation/
In examinations, it can be given as a long or evisceration, multiple socket reconstruction and
short case. the details of the procedure can identify the reason
for contracted socket.
HISTORY
History of Systemic Illness
Chief Complaints
History of hypertension, diabetes mellitus, vas
Patient is usually an adult with history previous cular diseases and any previous cerebrovascular
enucleation or evisceration surgery for any cause accident must be noted carefully. Any neurological
with or without prosthesis with complaints of: event may indicate the intracranial spread of the
zz Poor cosmetic appearance disease, for which the surgery have been done,
zz Repeated extrusion of prosthesis such as infection or malignancy.
zz Previously fitting prosthesis not fitting now
zz The patient sometimes may be a child with a
EXAMINATION
history of the underdeveloped socket.
Systemic Examination
History of Present Illness Most of the time the surgery is carried out to
Note about following: remove an intraocular malignancy in an advanced
zz Age at onset: To differentiate between congen stage, e.g. stage E retinoblastoma. Thus, a detailed
ital and acquired anophthalmic socket systemic examination is carried out to rule out
zz Preceding surgery: Type of surgery performed any systemic metastasis, especially the nervous
and its details from the record if available system.
zz Progression: Whether the implant or prosthesis
was fitting earlier, and if the implant size has Ocular Examination
been changed over time.
Ocular examination includes following:
zz Eyelids
History of Past Illness —— Eyelid notches and abnormalities need to
Past history may give a clue about the probable be looked out for. In longstanding cases
cause, so following points must be noted in past there may be stretching and lengthening
history: of the lower lid which would need to be
zz Ocular insult like chemical or radiation injury tackled simultaneously.
may be present —— Eyelid closure needs to be looked for too.
zz Panophthalmitis requiring evisceration —— The lower eyelid should be evaluated
zz Severe ischemic ocular disease for laxity. Lash position and lid margin
position should be noted, as entropion —— Palpation of the socket is done to for the
can indicate socket contracture. presence or absence of an implant and
—— The superior sulcus should be checked the position of the implant should be
for deepening and symmetry with the noted.
opposite side. The upper eyelid position —— Prosthesis: With the prosthetic in place
should be noted for ptosis, and levator the patient should be evaluated for fit,
function should be evaluated. size and its appearance with respect
zz Assessment of the socket: The following to the fellow eye. The movement of the
parameters are evaluated: prosthetic should be evaluated compared
—— General appearance and symmetry to the other eye. The prosthetic can then
compared to the other normal side should be removed and evaluated for type, size,
be noted carefully. smoothness and cleann ess.
—— Area of the socket: The area is assessed
particularly the depth of the fornices. The INVESTIGATIONS

depth of the fornices can be calculated by
inserting a blunt lacrimal probe into each zz Microbiological investigations to rule out any
of the fornices and noting down the length infection.
to which it can be inserted. The inferior zz Radiological investigation to look for any
fornix is the most important as it has to fracture in orbit or search of buried implant.
support the prosthesis. The other fornices
also need to be adequate to ensure the GRADING
prosthesis fitting.
—— The volume of the socket: The volume is
zz Gopal Krishna classification
assessed by noting the relative depth of The soft tissue sockets were divided into five
the socket compared to the fellow eye. grades for the sake of convenience in the
Another practical way of assessing the management of contracted sockets.
—— Grade-0: Socket is lined with the healthy
volume is to inject saline into the socket
drop by drop till it overflows. The superior conjunctiva and has deep and well-
sulcus deformity and presence of ptosis formed fornices.
—— Grade-I: Socket is characterized by the
are also indicators of volume loss.
—— Dry or wet socket: Look for any discharge
shallow lower fornix or shelving of the
lower fornix. Here the lower fornix is
from the socket. There should be no
converted into a downwards sloping shelf
active discharge from the socket. Dry
which pushes the lower lid down and out,
fibrotic conjunctiva indicates a poorly
preventing retention of an artificial eye.
vascularized socket.
Common causes are physical injuries,
—— Movements: The movements of the
endophthalmitis, and retinoblastoma.
muscles are looked for. In a case of dermis —— Grade-II: Socket is characterized by the
fat grafting, suturing the muscles to the loss of the upper and lower fornices.The
graft ensures better survival. common causes are physical injuries,
—— The tone of the orbicularis and tarsal sulci.
endophthalmitis, panophthalmitis, and
—— Cicatricial bands and degree of contrac
retinoblastomas.
ture within the socket. —— Grade-III: Socket is characterized by the
—— Associated any bony contracture must be
loss of the upper, lower, medial and lateral
checked for. fornices. Common causes are chemical
—— Look for signs of inflammation, excessive
injuries and panophthalmitis.
mucus, giant papillary conjunctivitis —— Grade-IV: Socket is characterized by the
under the upper eyelid and pyogenic loss of all the fornices and reduction of
granulomas. a palpebral aperture in horizontal and
Oculoplasty 27
vertical dimensions. Common causes are classify and eliminate any precipitating factors
chemical injuries and panophthalmitis. leading to contracture.
—— Grade-V: In some cases, there is the G eneral considerations before socket
recurrence of contraction of the socket reconstruction:
after repeated trial of reconstruction. zz Informed consent must be obtained
Common causes are thermal and zz The prognosis and aim of surgery must be well
chemical injuries of the eye. explained
zz Byron Smith classification zz In cases, oral mucosa grafting is planned the
Socket contraction may also be graded as patient should have mouthwashes started at
follows: least 2 weeks prior to the surgery.
—— Mild: Includes grade I and II where only zz Ensure the that the socket is free of any
one fornix is involved and there is a infection.
shortening of the posterior lamella of the zz Mild contracted socket: This can usually be
lids. managed by deepening the inferior fornix with
—— Moderate: Includes grade III where fornix formation sutures.
both superior and inferior fornices are zz Management of moderate-to-severe contracted
involved. socket: These cases are usually managed with
—— Severe: Comprises of cases in which all a graft. Grafts that can be used for socket
fornices are involved along with phimosis
of palpebral aperture.
—— Malignant contracted socket: It is the most
Table 2 Mechanism of contracted socket
severe variety of contracted socket and Etiology Factors
associated bony contraction, resulting Etiology • Alkali burns, Radiation therapy
from severe trauma or multiple surgeries. related leading to severe damage to the
zz Morphological classification: Guibor has socket and fibrosis
classified clinically contracted socket into Surgery • Fibrosis from the initial injury
4 morphological types as shown in Table 1. related • Poor surgical techniques: Extensive
dissection of the orbital tissue.
TREATMENT (TABLE 2) • No implant or undersized implant: In
children the absence of the stimulus
The primary aim of management is to create of either eyeball or implant can lead
a socket so as to maintain a prosthesis with a to bony contraction as well.
good cosmetic appearance. Before commencing • Excessive sacrifice of the conjunctiva
a definitive therapy, it is necessary to identify, and Tenon’s capsule
• Traumatic dissection within the
socket leading to scar tissue
Table 1 Morphological classification of • Multiple socket operations
contracted socket
Site • Poor vascular supply
Type Examples related • Severe ischemic ocular disease in
Anophthalmic Most common seen after the past
contracted socket enucleation and evisceration • Cicatrizing conjunctival diseases
surgery • Chronic inflammation and infection
Ophthalmic Following chemical and Implant • Undersized implant
contracted socket irradiation injury and • Implant migration
prosthesis • Implant exposure
Microphthalmic In association with
related • Not wearing a conformer/prosthesis:
contracted socket microphthalmos and
Confirmer keeps the fornices
microcornea
stretched and prevents fornicial
Hypoplastic Congenital under development shallowing
contracted socket of bony socket • Ill-fitting prosthesis
reconstruction include mucosa, split skin, membrane as against mucous membrane. It is

and dermis—fat grafts. The socket needs to cheap and easily available and has no significant
be healthy and vascularized for the grafts to complications associated with it.
take up. For mucous membrane grafts, mucus zz Treatment of dry socket: The socket is lined
can be taken from buccal cavity (lip or cheek), with a split-thickness skin graft in these cases.
rectum or vagina. The buccal cavity is preferred The skin graft is placed around an orbital
as it is easy to access. mould with the epithelial surface towards
zz Management severe contracted socket: it and perforations are made in the graft.
These cases usually require both area and The mould is sutured into the socket. After
volume replacement thus a composite graft the 1-month graft is split open in the area of
is required. The commonly used graft is the palpebral fissure. The mould is kept for at least
dermis fat graft wherein the fat provides the 4 months after which a permanent prosthesis
volume and the dermis provides the surface is placed.
area of the socket. The graft is taken from zz Management of recalcitrant cases: A socket
the hip. Although autogenous dermis fat that has undergone multiple unsuccessful
orbital implantation is an effective means of operations and has excessive scar tissue is
orbital reconstruction, there is a 30% chance unlikely to benefit from further repair. For such
of atrophy of at least half of the graft volume sockets exenteration of the eyelid and residual
when it is implanted in an avascular socket. socket material to create a cavity into which
Introducing a pedicle flap into the orbit as a a prosthesis is fitted, can be done. Optical
vascular bed for an autogenous dermis fat graft methods to improve the appearance includes
may increase the prospect of graft survival, as spectacle prosthesis or smoked lenses, plus or
well as supply additional volume to fill the minus lenses to magnify a micro-ophthalmic
socket. Temporalis muscle graft is supplied by socket to minimize buphthalmic socket,
a superficial temporal artery, a branch of an prisms to change the apparent horizontal or
external carotid artery and it can be used as a vertical position of malpositioned prosthesis
pedicle graft. or socket.
zz Treatment of moist socket: Partial-thickness
mucous membrane grafts are more susceptible VIVA QUESTIONS
to shrinkage and contracture. Full thickness
mucous membrane contracts less and may Q.1. What are the causes of contracted socket?
be obtained with minimal postoperative Ans. Causes of contracted socket can be
complications at the donor site. However, congenital or acquired.
mucosal contracture and submucosal scar • Congenital: Conditions such as micro
formation increase with the size of the oral phthalmos (Fig. 1) or congenital anoph
mucous membrane harvested and mucous thalmos (Fig. 2) usually lead to a con
membrane lacks the rigidity needed for tracted socket as the stimulus of the
grafting the palpebral surface. A full thickness eyeball is essential for healthy growth of
mucous membrane graft is obtained from oral the orbit.
mucosa of cheeks and lips (Most common), • Acquired: Acquired causes are described
hard palate, preputial skin, the skin of labia. here:
The graft should be 40–50% larger than anti – Enucleation without implant: A poorly
cipated to allow for subsequent contracture done enucleation, particularly without
with healing. It is helpful to harvest the graft at implant can lead to a contracted
the beginning of the procedure so that it can be socket. This is more so in children as
soaked in antibiotic solution before use. in the absence of the stimulus of either
Amniotic membrane can also be used instead eyeball or implant, there is a bony
of the mucosa. It has less patient morbidity, contraction as well. The implant needs
faster recovery and better fitting of a prosthesis. to be carefully selected, both in terms
No contracture is observed with an amniotic of size and material.
Oculoplasty 29
Fig. 1: Unilateral microphthalmia with Fig. 2: Bilateral anophthalmia with contracted socket
contracted socket
– Delay in use of conformer: In both • S ecure closure of all layers over the
enucleation and evisceration pro implant without tension or superior
cedures, conformer should be fitted displacement of the inferior fornix
immediately. This keeps the fornices • Avoidance of ill-fitting or roughened
stretched and prevents fornicial prosthesis as it may cause a more rapid
shallowing. The conformer should be contracture, symblepharon formation
on the correct side, adequate size and and total abandonment of prosthesis
have multiple holes to allow flushing • E limination of any source of chronic
and drainage of secretions. infection that may arise from lid margin,
– Trauma: Extensive lacerations of socket, canaliculi, lacrimal sac, chemical
the lids and orbital tissue can lead or thermal injury
to tissue loss and fibrosis resulting • Identification of conjunctival cicatrizing
in socket contraction. Injuries with diseases like pemphigoid, Stevens-
alkali/acid can also cause fibrosis. Johnson syndrome
– Radiotherapy: Post-operative radio • Avoidance of oversized prosthesis so as to
therapy for retinoblastoma can cause prevent migration of the implant into the
fibrosis and a grossly contracted inferior fornix and thereby obliteration of
socket. These sockets are usually inferior cul-de-sac.
poorly vascularized and difficult to • Conformer: A conformer should always
reconstruct. be placed at the end of the surgery. This is
• Infection: Socket/implant infection replaced by the prosthetic eye 4-6 weeks
can lead sloughing of the conjunctiva later. If the socket has undergone prior
and shortening of the fornices. irradiation, chemical, or thermal injury,
Q.2. What precautions should be taken to the conformer has to be left for a much
prevent contracted socket ? longer time.
Ans. Socket contraction should be prevented as • Radiotherapy if required, should be used
far as possible by taking some precautions with fractionation of dose.
at the primary surgery. Q.3. What are the characteristics of an ideal
• Proper dissection at the time of initial orbital implant?
procedure Ans. Ideal orbital implant should be:
• P reserving as much conjunctiva and • Lightweight
Tenon’s capsule as possible during • Nonantigenic
enucleation • Inert
• Biocompatible but not prosthesis. For example, dermis

• Affordable fat graft, mucous membrane graft.
• Mimic the motility of the normal globe Porous implants are presently the material
• Minimum complications like infection, of choice as vascularization leads to
extrusion and migration. the integration of implants. But porous
implants are significantly more expensive
Q.4. Types of orbital implants
than acrylic and silicon implants.
Ans. Presently all implants are broadly classified
as: Q.5. Post-enucleation socket syndrome/
• Nonintegrated implants: Are the ones volume deficient socket.
which are nonporous and are not Ans. Typically seen the following enucleation
integrated and have no direct muscle and characterized by following:
attachment, e.g. silicon and acrylic • Enophthalmos
implants. • An upper eyelid sulcus deformity
• Semi-integrated implants: Allow attach • Ptosis or eyelid retraction
ment of muscle in tunnels on the anterior • Laxity of the lower eyelid
surface for better motility. Examples • A backward tilt of the ocular prosthesis
include Allens, Iowa, etc. • Unhappy with cosmetic appearance.
• Biointegrated implants: These allow
fibrovascular ingrowth in the porous BIBLIOGRAPHY
channels and result in direct biologi
1. Krishna G. Contracted sockets-I (Aetiology
cal integration with orbital contents.
and types). Indian J Ophthalmol. 1980;28:
Examples include hydroxyapatite,
117-20.
porous polyethylene implants, alumi 2. Nesi FA, Lisman RD, Levine MR. Evaluation
num oxide, alpha sphere. and current concepts in the management of
• B iogenic implants: An autograft or anophthalmic socket. In: Smith’s ophthalmic
allograft of natural tissue with direct bio plastic and reconstructive surgery, 2nd edn.
logical integration with orbital structures Mosby. 1998. pp. 1079-124.
BLOW OUT FRACTURE OF ORBIT

INTRODUCTION very thin, the medial orbital wall is also prone

to fracture, either in isolation or in association
Orbital injury forms an important aspect of ocular with a fracture of the orbital floor or other facial
trauma. The blowout fracture is the most common bones.
type of orbital fracture. The term pure orbital In examinations, it can be given as a long case.
blowout fracture is used to describe fracture of
the orbital floor, the medial wall or both, with
HISTORY
an intact bony margin. The term impure orbital
blowout fracture is used when such fractures Chief complaints: It depends upon the duration
occur in conjunction with a fracture of the orbital following trauma, after which the patient presents.
rim. The most common site for orbital blowout When the patient presents immediately following
fracture is the posteromedial aspect of the orbital trauma the complaints, include:
floor medial to the infraorbital neurovascular zz Eyelid ecchymosis (Fig. 1)/periorbital hema
bundle where the maxillary bone is very thin toma: Usually present but signs may be absent
(0.25–0.50 mm). As the lamina papyracea is also as seen in the ‘white-eyed blowout fracture’.
Oculoplasty 31
EXAMINATION
Systemic examination: General condition of a
patient and another nonocular injury should
be checked. Life-threatening injuries must be
taken care of first before proceeding for ocular
examination.
Ocular examination: Following points must be
noted in a case of orbital fracture.
Visual acuity: Visual acuity at presentation has
medico-legal importance in ocular trauma cases.
Uncorrected as well as best corrected visual acuity
must be noted in all cases of trauma.
Fig. 1: Orbital floor fracture
zz Subcutaneous emphysema: If a blowout Orbit

fracture communicates with an air-filled sinus zz Palpate orbital rim to look for deformity and
it may result in emphysema. Commonly seen crepitus. Subcutaneous emphysema with
in medial orbital wall blowout fractures. It may crepitus seen in fractures communicating with
result in palpable crepitus. Patients should be air-filled sinuses. The malar eminences should
advised not to blow their nose be palpated and any depression noted. The
zz Proptosis of variable degree can also be seen patient should be asked to open and close his
initially due to orbital edema and hemorrhage mouth to rule out pain or trismus that may be
zz Other complaints such as epistaxis, pain, associated with a zygomatic complex fracture.
loss of vision can be there depending on the zz Paresthesia or sensory loss over ipsilateral
damage to adjacent structures. lower lid, cheek and upper lip pathognomonic.
zz Diplopia can be there due to muscle entrap- Neurosensory loss occurs in the area supplied
ment by the infraorbital nerve. This occurs because
zz Small or inward displacement of an eyeball the fracture extends along the infraorbital
(e nophthalmos) can be there due to soft tissue groove or canal injuring the infraorbital
prolapse into adjacent sinuses. nerve. These sensory defects tend to resolve
zz In cases, where the presentation is delayed, the spontaneously with time but may get
complaints are usually of a small or shrunken aggravated by surgery in the area.
eyeball, limitation of movements or diplopia. zz Ocular motility: Full orthoptic assessment
should be performed in nine positions of gaze.
History of Present Illness Limitation of ocular motility can occur due to
A detailed history regarding mode of injury should following causes:
—— Entrapment of orbital contents such as
be taken to assess the mechanism and extent
of an injury. In cases of delayed presentation, connective tissue, septa, extraocular
progression must be noted carefully. The decision muscle (inferior rectus most commonly)
for surgical intervention largely depends upon the within the fracture.
—— Hematoma/edema in the orbital fat
course of the symptoms over time.
adjacent to the fracture.
Past history: Past history of poor vision in the —— Hematoma or contusion of the extraocular
affected eye, repeated trauma should be noted. muscle itself.
Systemic history: Past history of DM, hypertension, —— Palsy of an extraocular muscle due to
bleeding disorders and neurological problems neuronal damage.

must be noted. This will be helpful while planning —— Volkmann’s ischemic contracture of an
surgery, especially under general anesthesia. entrapped extraocular muscle.

—— Inferior rectus muscle leads to motility fracture. X-ray shows bone discontinuity in the
restriction especially in upgaze (vertical orbital floor with herniation of soft tissue in
diplopia. maxillary antrum seen as ‘hanging drop’ sign.
Eyelid: Pseudoptosis occurs due to loss of support.
zz Computerized tomography (CT) scanning: CT
Look for other signs of trauma such as laceration gives the detailed visualization of bony and
or scar. soft tissue injury where entrapment of muscle
can be appreciated (Fig. 2). Coronal sections
Conjunctiva: Subconjunctival hemorrhage and are particularly useful and can show antral soft
conjunctival chamois may be there. tissue densities, such as prolapsed orbital fat,
Cornea: Trauma can lead to corneal abrasion, extraocular muscle or hematoma.
corneal laceration. zz Magnetic resonance imaging (MRI): Can be
utilized when there is the need for greater
Iris and anterior chamber: Trauma may be
soft tissue evaluation. MRI is insufficient in
associated with iritis, miosis or mydriasis,
assessing the bony structures and therefore
hyphema.
needs to be combined with CT.
Pupil: Presence of relative afferent pupillary defect
(RAPD) indicates optic nerve injury that needs
urgent intervention in the form of pulse steroid
MANAGEMENT
therapy. Treatment includes both medical and surgical
Lens: Trauma may be associated with subluxation, management:
dislocation of lens. zz Medical management/Observation: It con
sists of oral antibiotics, analgesics and a
Fundus: Look for effects of trauma such as dialysis, short course of oral prednisone. Oral steroid
tear, retinal detachment or vitreous hemorrhage. benefits the patient by reducing the edema of
the orbit and muscle. This also may allow for
Special Tests a more thorough assessment of the relative
contribution to enophthalmos or entrapment
zz Hertel exophthalmometer: Exophthalmometry from the fracture versus that from edema.
is done to document enophthalmos. Medical management is indicated in following
zz Force duction test (FDT): FDT is useful in conditions:
determining whether dysmotility is restrictive —— No significant enophthalmos (<2 mm)
or paralytic. In a blow out fracture with inferior —— Lack of marked hypo-ophthalmos
rectus, entrapment FDT is ‘positive’ indicating
a mechanical cause.
zz Force generation test (FGT): In testing force
generation, the muscle insertion is grasped
and the patient is asked to look into the
muscle’s field of action. A paretic muscle will
feel weak when compared with the fellow eye.
zz Diplopia charting: With red green glass,
diplopia charting with streak light shows
diplopia worsening in upgaze.
zz Hess screen or Lee screen tests were done
document the muscle involved.
INVESTIGATIONS
zz Plain X-rays: Easily available and cost-effective
imaging modality. Water’s view is the most
useful projection for detecting an orbital floor Fig. 2: Fracture of orbital floor on CT Scan
Oculoplasty 33
—— Absence of an entrapped muscle or tissue The orbital floor can be approached through
—— Fracture less than 50% of the floor following ways:
—— No diplopia
Subciliary approach: Incision given 2–3 mm below
—— The patient must be advised to avoid nose
the lash line. It has the advantage of better scar
blowing, to avoid creating or worsening camouflage. The disadvantage is postoperative
orbital emphysema. Nasal decongestants ectropion and lower lid retraction.
can be used if not contraindicated. Ice
packs may be applied for initial 48 hours Subtarsal approach: Incision below tarsal plate
to reduce the pain and tissue edema. over orbital rim. The advantage is it gives direct
zz Surgical treatment access to the floor with good exposure. The
Surgical intervention is indicated in following disadvantage is it leads cosmetically unacceptable
cases: scar.
—— Immediate intervention: Transconjunctival approach: Incision given in
Diplopia present with CT evidence of lower fornix 3 mm below the tarsal plate and can
an entrapped muscle or periorbital be combined with a lateral canthotomy for better
tissue and associated with a non- exposure. The advantage is it gives no visible scar.
resolving oculocardiac reflex: brady
Transantral approach: Orbital floor reached via
cardia, heart block, nausea, vomiting
the maxillary sinus using Caldwell-Luc incision. It
or syncope
is a difficult technique and it is not a favored by an
“White-eyed blow-out fracture.”
ophthalmologist.
Young patients (<18 years), history
of periocular trauma, little ecchy Endoscopic approach: Trans-maxillary and trans-
mosis or edema (white eye), marked nasal endoscopies have been described which
extraocular motility vertical restric eliminate the need for eyelid incisions and gives
tion, and CT examination revealing an improved visualization of fractures. However, it is
orbital floor fracture with entrapped difficult and often clumsy.
muscle or perimuscular soft tissue The orbital floor is reinforced with either
Early enophthalmos/hypoglobus autogenous or synthetic implant (Table 1). The
causing facial asymmetry. surgeon should size the implant to cover the
zz Within 2 weeks: Patients with diplopia are defect adequately and to prevent displacement
usually observed for 2 weeks. If the diplopia or extrusion later. While cutting the implant, it
resolves with a small fracture evident on CT, no should be tapered posteriorly to fit the orbital floor
surgical intervention is required. It is advisable configuration.
to wait for 2–3 weeks for resolution of orbital
edema/hematoma. However, surgery is VIVA QUESTIONS
indicated in the following scenario:
—— Symptomatic diplopia with positive Q.1. What is a pure and impure blowout
forced ductions, evidence of an entrapped fracture?
muscle or perimuscular soft tissue on Ans. A pure orbital blowout fracture is used to
CT examination, and minimal clinical describe fracture of the orbital floor, the
improvement over time. medial wall or both, with an intact bony
—— Large floor fracture causing latent orbital margin. Impure orbital blowout
enophthalmos fracture is used when such fractures occur
—— Significant hypo-ophthalmos in conjunction with a fracture of the orbital
—— Progressive infraorbital hypoesthesia rim.
Surgical principle: The basic steps of surgery Q.2. Most common site for the blowout frac
includes assessing the orbital floor, releasing ture of orbit?
the soft tissue and muscle entrapment and Ans. The posteromedial aspect of the orbital floor
strengthening the floor with use of implants. medial to the infraorbital neurovascular
Table 1 Examples of implant materials used in orbital floor repair

Implant material Advantage Disadvantage
Membranous bone Autogenous • Morbidity at donor site
• Extended operation time
• Resorption unpredictable
Cartilage Autogenous • Morbidity at donor site
• Extended operation time
• Resorption unpredictable
Titanium mesh Biocompatible stable • Foreign material that remains in the body
• Combination with bone recommended
Porous polyethylene Easy to shape and handle, Foreign material that remains in the body
(Medpore) sheets Biocompatible stable
Silicon sheet Easy-to-handle and cheap Extrusion rates higher
Silastic sheet (Teflon) Easy-to-shape and handle Foreign body reaction and extrusion
common
bundle where the maxillary bone is very with midfacial fracture as in tripod or Le
thin (0.25–0.50 mm) is the most common Fort type III. Clinically patient has gross
site. enophthalmos, inferior displacement of
Q.3. What is the etiopathogenesis of blowout the globe (hypoglobus), deep superior
fracture? sulcus, eyelid asymmetry and diplopia.
Ans. There are two theories to explain the Q.5. What are the features of medial wall
possible mechanism of orbital fracture: fracture?
• Retropulsion theory/hydraulic theory: Ans. Blowout fracture of a medial wall is much
States that the backward displacement less common than a floor and usually seen
of the globe caused by a blunt non- along with nasoethmoid fractures, rather
penetrating object raises the intraorbital than as an isolated entity. Horizontal
pressure sufficiently to fracture the diplopia is usually the primary complaint
posteromedial orbital floor and/or the when medial orbital tissues are involved.
lamina papyracea of the ethmoid. However, a vertical or oblique component
• Buckling theory/transmission theory: A can also be found in such cases.
transient deformation of the orbital rim Q.6. What is ‘white-eyed’ blowout fracture?
transmits the force of injury directly to Ans. The bones of a child’s orbit are more elastic
the orbital wall. During the course of than adults. Thus, injury in children causes
injury, the force that is transmitted to more anteroposterior buckling creating a
bony walls of the orbit may also cause fracture with overlapping segments. This
concussion ocular trauma leading to leads to ‘trapdoor-type’ fracture where
angle recession, hyphema, vitreous prolapsed orbital tissue is caught in the
hemorrhage, commotio retinae, etc. fracture site leading to severe motility
Q.4. Expanded orbit syndrome restriction and diplopia in absence of
Ans. Multiple fractures in and around the marked congestion or ecchymosis. The
orbit may lead roomy orbit with extensive condition is also called the ‘white-eyed’
prolapse of orbital tissues. This expansion blowout fracture. It is seen in orbital
can be seen in orbital fracture along blowout fracture in children.
Oculoplasty 35
Q.7. Boundaries of the orbital floor and floor fracture. Diplopias in primary gaze
contents and in downgaze (functional gaze) are
Ans. • The adult orbital floor is formed by the more troublesome. Such cases will require
maxillary, zygomatic bones anteriorly muscle surgery. To correct diplopia in
and palatine bones posteriorly. down gaze ‘Reverse Knapp procedure’
• Orbital floor measures about 35–40 mm performed placing medial and lateral recti
anteroposteriorly and it is the shortest behind inferior rectus muscle. Fresnel
of all the walls. It forms the roof of the prisms can be employed in selective
maxillary sinus. cases.
• The floor of the orbit contains infra Q.10. How to treat postoperatively cosmetically
orbital groove that forms infraorbital unacceptable enophthalmos?
foramen. Infraorbital nerve, a branch of Ans. A repeat surgery with adequate size
the maxillary division of trigeminal nerve orbital implant, if the downward sinking
passes through the groove, providing of an eye along with enophthalmos is
sensory innervations to the ipsilateral unacceptable to patient, may have to be
orbital floor, mid face, and posterior done. The pseudo ptosis can be corrected
upper gingival area. The infraorbital with mullerectomy, which will increase
artery, a branch of the maxillary artery, palpebral height.
and the infraorbital vein also are found
within the infraorbital groove. BIBLIOGRAPHY
Q.8. What are common complications of floor 1. Burnstine MA. Clinical recommendations
repair surgery? for repair of isolated orbital floor fractures:
Ans. • Intraoperative bleeding An evidence-based analysis. Ophthalmology.
• Residual or new-onset diplopia 2002;109(7):1207-10.
• Extraocular muscle dysfunction 2. Principles and Practice of Ophthalmology:
• Postoperative neuralgia Albert and Jakobeic, Vol. 6, 2nd edition, Pgs.
• Residual enophthalmos 5303-9.
• Implant extrusion 3. Smith B, Regan WF. Blowout fracture of the orbit
• Possible loss of vision. (mechanism and correction of internal orbital
fracture). Am J Ophthalmol. 1957;44:733-9.
Q.9. How to treat persistent diplopia? 4. Yadav P, Pushker N, Bajaj M, Chandra M, Shrey
Ans. Some patients may have persistent diplo D, Lohiya P. Orbital blow out fracture. DOS
pia even after adequate surgical repair of Times. 2008;8(14):2.
THYROID-ASSOCIATED OPHTHALMOPATHY
Aditi Dubey, Prafulla Kumar Maharana
INTRODUCTION examinations. In TED, it is important to record

a proper history and elicit the signs associated
Thyroid associated ophthalmopathy (TAO) is the with it.
most common cause of proptosis in adults. TAO
is a self-limiting autoimmune disease associated HISTORY
mainly with hyperthyroidism, but also with
hypothyroid and euthyroid states. Although it can Demography
affect any age, most commonly presents during It can affect any age group. Patients with TAO are
the fourth and fifth decades of life. It is one of more likely to be female by a 2:1 ratio, following the
the most important long case given in practical usual predominance of autoimmunity in women.
However, male Graves’ disease are at the same, zz Females are having more predominance in
if not higher, risk of TAO development, which is thyroid eye disease (2:1). However, males
usually of a more severe form and occurs at a more presents with more severe disease and usually
advanced age than in their female counterparts. at a later age.
Asians are having a lower likelihood of developing zz Patient may be in hyperthyroid or hypothyroid
the disease than Europeans.1,2 state, but 5 to 10% cases are euthyroid at the
time of presentation.
Chief Complaints
A case of TAO can present with following Past History
complain: zz History of diabetes mellitus (DM), hyper
zz Excessive lacrimation, a gritty sensation, tension, asthma, thyroid abnormality should
discomfort, and photophobia are often present be noted.
in early course of the disease. zz In a case of diagnosed TAO, past history of
zz Bilateral upper lid retraction is most common steroid, radiotherapy, orbital decompression
presenting feature of the thyroid eye disease. or any thyroidectomy must be recorded.
zz Bilateral proptosis can also be the presenting
feature. Personal History
zz At times it may be detected accidentally or
Personal history of alcohol intake, smoking,
Referred by the endocrinologist.
tobacco chewing or any other if present should
zz Decrease in visual acuity can be a presenting
be noted, because smoking is considered as
feature when it is associated with Optic nerve
important risk factor for the TED.
compression, exposure keratopathy or induced
astigmatism due to globe compression.
zz Patients may also complain of general EXAMINATION
symptoms like weight loss, sweating, heat Examination of a case of TAO is similar to that of
intolerance, weakness, fatigue and palpitation a case of proptosis. Salient features of TAO are
along with ophthalmic complains. described here.
Approximately 5 to 10% of Graves’ orbitopathy
patients are euthyroid at presentation and some of
them may not have a history of thyroid dysfunction.
Around 40% of patients with TAO, the signs of the General examination may show the signs of
eye disease occur simultaneously with the first hyperthyroidism such as tachycardia, fine
symptoms of hyperthyroidism. hand tremor, warm and sweaty skin, pretibial
myxedema, finger clubbing, alopecia and vitiligo.
A detailed examination of cardiovascular system,
respiratory system, gastrointestinal system and
The history of present illness must include all the Central nervous system has to be done as these
points described in the chapter of proptosis. systems gets affected by Graves’ disease.
zz The patient of TED present with the bilateral
upper lid retraction and or exophthalmos. A
careful history can reveal history of irritation,
Ophthalmic Examination
foreign body sensation, watering and recur The ocular examination is similar to a case of
rent lid edema in past that is often ignored by proptosis. Important points to note are described
the patient. As the disease progresses the full below:
blown picture of TED develops. zz Proptosis is usually axial.
zz Cigarette smoking has been considered the zz Globes are mostly aligned.
strongest risk factor for developing TED. zz The most characteristic signs are eyelid
Hence, a detailed history of smoking must be erythema and swelling, caruncular and
asked. conjunctival injection and edema (Fig. 1).
Oculoplasty 37
Fig. 1: Severe bilateral proptosis due to Fig. 2: Thyroid eye disease with disproportionate
thyroid eye disease proptosis and inferior dystopia in left eye
zz Some patients may have restriction of the

eye movements leading to squint. The most
commonly affected muscle is inferior rectus
followed by medial, superior, levator and
lateral rectus (Fig. 2). The muscles affected
results in ocular misalignment, diplopia.
Strabismus is also common, and it often
presents as hypotropia or esotropia.
zz Eyelid: Various lid signs which can be seen in
thyroid ophthalmopathy (Table 1). All signs
may not be present in a single patient. Lid
retraction, also known as Dalrymple’s sign,
occurs most commonly as lid sign, in about
37–92% of patients (Fig. 3). Fig. 3: Thyroid eye disease with lid retraction
zz Eyelashes are usually normal.
zz Conjunctiva may show mild congestion due zz Pupillary reaction may or may not be normal.
the dry eye caused by the excessive evapora
Presence of RAPD or APD suggests optic nerve
tion of the tears due to the lid retraction. compression.
zz Inflammation along recti muscle (tendonitis) zz Variable intra ocular pressure (IOP) can be
can be an early sign of the disease. there in different gazes due to restrictive
—— Cornea may have exposure keratopathy.
myopathy. An IOP rise more than 8 mm Hg on
Causes of exposure keratopathy include: upgaze is significant and warrant treatment.
Inadequate eyelid closure leading
Glaucoma can also result from decreased
to excessive moisture loss as a episcleral venous outflow.
consequence of proptosis and eyelid zz Lens do not show any specific changes.
dysfunction. zz Fundus examination may show signs of
Diminished tear production resulting
globe compression. Compressive optic
from lacrimal gland infiltration. neuropathy occurs in <5% of patients with
Lagophthalmos from proptosis. thyroid ophthalmopathy resulting in slowly
Loss of Bells phenomenon from progressive visual loss. It occurs due to
inferior rectus infiltration. compression from the oversized recti and
Table 1 Lid signs in proptosis

Sign Description
Abadie’s sign Elevator muscle of upper eyelid is spastic
Ballett’s sign Paralysis of one or more extraocular muscle (EOM)
Beck’s sign Abnormal intense pulsation of retina's arteries
Boston’s sign Jerky movements of upper lid on lower gaze
Cowen’s sign Extensive hippus of consensual pupillary reflex
Dalrymple’s sign Upper eyelid retraction
Enroth’s sign Edema especially of the upper eyelid
Gifford’s sign Difficulty in eversion of upper lid
Goldzieher’s sign Deep injection of conjunctiva, especially temporal
Griffith’s sign Lower lid lag on upward gaze
Hertoghe’s sign Loss of eyebrows laterally
Jellinek’s sign Superior eyelid folds is hyperpigmented
Joffroy’s sign Absent creases in the forehead on upward gaze
Jendrassik’s sign Abduction and rotation of eyeball is limited also
Knies’ sign Uneven pupillary dilatation in dim light
Kocher’s sign Spasmodic retraction of upper lid on fixation
Loewi’s sign Quick mydriasis after instillation of 1:1000 adrenaline
Mann’s sign Eyes seem to be situated at different levels because of tanned skin
Means’ sign Increased scleral show on upgaze (globe lag)
Moebius’s sign Lack of convergence
Payne/Trousseau sign Dislocation of globe
Pochin’s sign Reduced amplitude of blinking
Rieseman’s sign Bruit over the eyelid
Movement’s cap Eyeball movements are performed difficultly, abruptly and incompletely
phenomenon
Rosenbach’s sign Eyelids are animated by thin tremors when closed
Saiton’s sign Frontalis contraction after cessation of levator activity
Snellen-Rieseman’s sign When placing the stethoscope's capsule over closed eyelids’ a systolic murmur
could be heard
Stellwag’s sign Incomplete and infrequent blinking
Suker’s sign Inability to maintain fixation on extreme lateral gaze
Tellas’s sign Inferior eyelid might be hyperpigmented
Topolanski’s sign Around insertion areas of the four rectus muscles of the eyeball a vascular band
network is noticed and this network joins the four insertion points.
von Graefe’s sign Upper lid lag on downgaze
Wilder’s sign Jerking of the eye on movement from abduction to adduction
Oculoplasty 39
orbital fat causing compartment syndrome at zz Postsurgical lesion—Recession of the inferior

the apex of orbit. It is characterized by decrease rectus muscle, Repair of a blowout fracture
in vision, color vision, contrast sensitivity and zz Facial nerve lesion.
relative afferent papillary defect. Visual loss
may progress undetected due to insidious INVESTIGATIONS
onset and subtlety of neuropathy. Risk factors
for optic neuropathy include: Following investigations are carried out in a case
—— Older age of TAO:
—— Smoking
zz CBC, Thyroid function test [Tri-iodothyronine
—— Male gender (T3), Free thyroxin (T4), Serum thyroid-
—— Significant strabismus with mild stimulating hormone (TSH), Thyrotrophic
proptosis. receptor antibodies (TRAB), ESR].
zz Visual field: Central and inferior arcuate zz Ultrasonography (USG): On cross-section,
scotoma and generalized constriction may be there is an increase in thickness of the
seen in advanced cases ophthalmopathy. extraocular muscles. USG of the globe and the
orbit can help in visualization of the tendinous
DIFFERENTIAL DIAGNOSIS intersections. This also helps to differentiate
between active and inactive disease. By
The diagnosis often straight forward. The comparing the muscle thickness, ultrasound
characteristic signs and systemic signs are difficult may help in confirming the diagnosis in
to miss. However, in early course of the disease unilateral cases. It also helps in differentiating
following diseases may mimic TAO associated diseases presenting with similar
zz Orbital myositis clinical features.
zz Nonspecific orbital inflammatory disease zz Computed tomography (CT) scan: Typical
(NSOID) radiological features seen on CT are muscle
zz Myasthenia gravis belly enlargement that is classically described
zz Chronic progressive external ophthalmoplegia as ‘tendon sparing’, an increase in orbital fat
zz Carotid-cavernous fistula volume, and crowding of the optic nerve at the
zz Specific inflammatory orbitopathy orbital apex in severe cases. It helps in assessing
zz Orbital tumors the relationship between the optic nerve and
zz Lid retraction, characteristic of TAO can also muscles at the apex that helps in planning for
be seen in number of other diseases as given the surgical intervention, if needed. CT is more
here. sensitive than MRI in identifying enlarged
extraocular muscles. As a standard, 2 mm cuts
Upper Lid Retraction should be requested for, along with coronal
and axial slices. Orbital fat is imaged in CT as a
zz Congenital
black, low-density area that contrasts with the
zz Neurologic disorders: Midbrain disease,
higher-density image of extraocular muscles
Hydrocephalus
and the optic nerve. CT scans allow for better
zz Post-surgery: Ptosis surgery, lid reconstruction
delineation of the bony orbit and therefore are
zz Marcus Gunn phenomenon, Faulty regenera
invaluable in planning orbital decompression.
tion of cranial nerve III
zz Magnetic resonance imaging: Demonstrates
zz Parinaud’s syndrome
fusiform rectus enlargement and orbital
zz Sympathomimetic drugs
fat expansion. It assesses water content in
zz Cirrhosis.
the muscles that correlates with the active
inflammation. In the active phase, the
Lower Lid Retraction extraocular muscles appear isointense in
zz Idiopathic senile flaccidity of lower lid T1-weighted images and hyperintense in T2
zz Post-traumatic impairment weighted images; where as in the chronic
zz Congenital abnormality phase, they appear hypointense on T2-images.
zz Visual field testing is important for detecting Table 2 NO-SPECS classification of thyroid
early damage to the optic nerve due to apical associated ophthalmopathy
crowding around the optic nerve. The changes
on visual fields are reversible if the crowding Class Grade Clinical features
is relieved early, either surgically or medically. 0 N – No signs symptoms
Usually, the patterns of visual field loss vary, 1 O – Only signs
the most common being central, paracentral
and/or inferior. 2 S – Soft-tissue involvement
O Absent
A Minimal
CLASSIFICATION B Moderate
C Marked
Different classification system have been
proposed, however there is no consensus on the 3 P – Proptosis
O <23 mm
best way to classify TAO:
A 23–24 mm
zz NO-SPECS classification
B 25–27 mm
—— Proposed by Werner et al. and adopted
C ≥28 mm
by the American Thyroid Association
(Table 2) 4 E – Extraocular muscle involvement
—— Based upon clinical presentation
O Absent
A Limitation of motion in extremes
—— Limitation: Relies on subjective evalua
B of gaze
tion, does not take into account the C Evident restriction of movement
severity of manifestations, Patient may fall Fixed eyeball
into more than 1 particular class, may not
5 C – Corneal involvement
progress in an orderly fashion from class
O Absent
1 to class 6 and is relatively insensitive to
A Stippling of cornea
subtle changes, hence less preferred. B Ulceration
zz RELIEF classification of soft tissue signs and C Clouding
symptoms
6 S – Sight loss
R – Resistance to retropulsion
O Absent
E – Edema of conjunctiva and caruncle
A 20/20 – 20/60
L – Lacrimal gland enlargement B 20/70 – 20/200
I – Injection over the horizontal rectus muscle C <20/200
insertions
E – Edema of the eyelids
F – Fullness of the eyelids
and pain with ocular movement. In
addition, if the patient has been examined
Staging/Scoring
within the 3 months prior, additional
zz Clinical activity score (CAS): points may be given for decreased visual
—— It is one of the widely utilized grading acuity, worsened diplopia, and increased
system described by Mourits and proptosis compared with that visit.
colleagues —— TAO is considered active in patients with
—— It attempts to identify patients with active a CAS of 3 or more out of 7 (if no previous
disease who are likely to respond to assessment is available), or 4 out of 10 on
medical therapy. the complete scale.
—— The CAS is generated by the addition of —— This scale has a specificity of 86%,
1 point for the presence of each the follow sensitivity of 55%, positive predictive
ing features: Chemosis, eyelid swelling, value of 80%, and negative predicative
eyelid erythema, conjunctival erythema, value of 64% for predicting the activity of
caruncular swelling, pain in primary gaze, the disease.
Oculoplasty 41
—— Limitation: It is subjective (depends on Vision: 1 point

both patient and practitioner) and it fails Inflammation/congestion: 10 points
to account for active improvement or Strabismus: 6 points (diplopia: 3
worsening of the disease. points plus restriction: 3 points)

zz European Group on Graves’ orbitopathy Appearance/exposure: 3 points.
(EUGOGO): It is one of the commonly used A global severity grade, with maxi
scoring systems. It recommends the following mum score is 20 points, is the sum of
classification of patients with thyroid each of the involved systems graded
ophthalmopathy.3 independently:
Mild GO: They usually present with one or zz Vision (V): Evaluates the visual problems,
more of the following signs: especially due to associated dysthyroid optic
—— Minor lid retraction (< 2 mm)
neuropathy. It is assessed through visual
—— Mild soft tissue involvement
acuity, pupillary reflexes, color vision, visual
—— Exophthalmos < 3 mm (above the normal
fields, optic nerve examination, and visual
range for the race and gender) evoked potentials.
—— Transient or no diplopia
zz Soft tissue inflammation/congestion (I)
—— Corneal exposure responsive to lubricants
evaluation is graded according to the worst
Moderate-to-severe GO: These patients usually score for the eye or the eyelid with the
have any one or more of the following: Inflammatory Index as shown in Table 3.
—— Lid retraction >2 mm
Patients with moderate inflammatory index
—— Moderate or severe soft tissue involvement
(less than 4 of 10) are managed conservatively.
—— Exophthalmos >3 mm above normal for
Patients with high scores (above 5 of 10) or
race and gender with evidence of progression (as documented
—— Inconstant or constant diplopia
Sight-threatening GO:
—— Patients with dysthyroid optic neuropathy
Table 3 VISA inflammatory index
and/ or corneal breakdown.
Sign or symptom Score
—— Other infrequent conditions are ocular
globe subluxation, severe forms of frozen Caruncular 0: Absent

eye, choroidal folds, and postural visual edema 1: Present
darkening. Chemosis 0: Absent
—— This category warrants immediate 1: Conjunctiva lies behind the
intervention. gray line of the lid
—— As a rule of thumb, it is considered that 2: Conjunctiva extends anterior
all patients who do not have a mild or to the gray line of the lid
a sight-threatening ophthalmopathy Conjunctival 0: Absent
present a moderate-to-severe disease. redness 1: Present
zz VISA scoring: Lid redness 0: Absent
—— Developed by Dolman and Rootman
1: Present
and adopted with modifications by the
Lid edema 0: Absent
International Thyroid Eye Disease Society
1: Present but without
(ITEDS). redundant tissues
—— It is based on symptoms (subjective) and
2: Present and causing bulging
signs (objective) inputs. in the palpebral skin, including
—— Four severity parameters are analyzed:
lower lid festoon
V (vision); I (inflammation/congestion);
Retrobulbar ache
S (strabismus/motility restriction); and • At rest 0: Absent; 1: Present
A (appearance/exposure). • With Gaze 0: Absent; 1: Present
—— Each feature is considered and graded
independently Diurnal variation 0: Absent; 1: Present

on subsequent visits) in the inflammation are thyroid status (every 4–6 weeks) is imperative
offered a more aggressive therapy. in the initial phases of treatment when changes
zz Strabismus/motility restriction (S) is docu in thyroid status are expected.
mented by three aspects:
1. Diplopia that is graded from 0 to 3 (0 = no
Treatment of Ophthalmopathy
diplopia, 1 = diplopia with horizontal or
vertical gaze, 2 = intermittent diplopia in Treatment should follow the sequence of
straight gaze, and 3 = constant diplopia in (V-I-S-A), i.e. 1st take care of visual disturbance
straight gaze). then ISA (of VISA scoring).
2. Ocular ductions are measured to the
nearest 5° in four directions using the Treatment Options
corneal light reflex technique. Any change
of ≥ 12° in any direction can be considered zz Supportive measures:
—— Artificial tears: Lubricant eye drops
progression.
3. Ocular restriction can be graded from 0 during the day and Lubricant ointments
to 3 based on the range of ductions (0 = at night-time
—— Sunglasses: To avoid photophobia
duction > 45°, 1 = 30–45°, 2 = 15–30°, and 3
—— Patients with symptomatic diplopia-
<15°) quantified by prism cover testing.
zz Appearance/exposure (A) Fresnel prisms or occlusion therapy
—— Symptoms include appearance concerns —— Botulinum toxin injection may be
(such as bulging eyes, eyelid retraction, considered for upper lid retraction
and fat pockets) and those derived —— Topical adrenergic blocking agents
from ocular exposure (such as gritting such as 5% guanethidine sulfate drops

sensation, photophobia, dryness, and transiently improve mild eyelid retraction
secondary tearing). but not of much use
—— Signs include measurements of eyelid —— Cool compresses
retraction (millimeters from the pupillary —— Head elevation: To reduce periorbital
light reflex to the lid margin); scleral show edema.

(millimeters from the limbus to the lid zz Medical management:
margin); levator palpebrae superioris Corticosteroids: Systemic steroids are
function; lagophthalmos (incomplete indicated in patients with severe inflammation
eyelid closure); and proptosis with the or compressive optic neuropathy. Intravenous
Hertel exophthalmometer. Signs of glucocorticoids is required for patients with
corneal exposure are best assessed with advanced thyroid-associated orbitopathy.
the slit-lamp microscope and may include Intravenous glucocorticoids seem to be associ
punctate epithelial erosions, ulcerations, ated with higher success rate and better toler
and, in severe cases, corneal thinning and
ability as compared to oral glucocorticoids.
risk of perforation.
Steroid-sparing immunosuppressive drugs:
The VISA and CAS were designed to determine
Cyclosporine and Methotrexate, Intravenous
the clinical activity. In comparison, the NO SPECS
administration of immunoglobulin, Tumor
and EUGOGO classification assess the clinical
severity. Both VISA (particularly in US) and necrosis factor-α blockers and anti-CD20
EUGOGO (European countries) are currently used monoclonal antibodies (rituximab) have been
for deciding upon treatment and also monitoring found useful. However, these are inferior to
response to treatment.3-5 steroids as monotherapy and considered only
when steroid is contraindicated.
MANAGEMENT zz Radiation therapy:
Acts by a nonspecific anti-inflammatory
Treatment of Thyroid Gland Dysfunction effect. RT is effective in patients who have
It is the most important aspect of treatment of active eye disease with recent progression and
thyroid ophthalmopathy. Frequent monitoring of ineffective in inactive stages of the disease.
Oculoplasty 43
The Lymphocytes infiltrating the orbit have zz Very severe TED: Very sever TED should be
high radio sensitivity. Usually a dose of 20 Gy treated with 1 g methylprednisolone IV daily
is given per eye fractionated over a 2-week for three consecutive days, repeated after
period. However, RT can be associated with one week, followed by an oral tapering dose.
transient exacerbations of inflammation, When there is clinical deterioration, urgent
hence simultaneous glucocorticoids must orbital decompression should be considered.
be started. Although, the evidence regarding Indications for surgical decompression
the efficacy of radiation therapy in the includes:
management of TAO is limited, it is still one of —— Patients with active disease who have
the widely used treatment modality. refractory or progressing corneal ulcer.

zz Surgical management —— A stretched optic nerve
Orbital decompression: It is indicated in —— Prevention of further corneal damage
cases with compressive optic neuropathy not —— Cosmetic in acceptability.
improving with medical treatment. It enlarges In-orbital decompression, part of the bony
the existing space of the orbit by partial walls is removed to provide more room for the
removal of bony walls and periosteum. The extraocular muscles and orbital fat. Associated
most commonly done decompression involves diplopia usually requires surgery of the extraocular
the posteromedial wall followed by floor and muscles. But after the orbital decompression
lateral wall. surgery, diplopia surgery should be postponed till
effect of the previous if established. Eyelid surgery
Treatment such as lengthening (in case of upper eye lid
retraction) may be a final step in the rehabilitation
It depends upon the stage and severity: of the patient with TED.
zz Mild TED: Only supportive therapy is required.
Progression from mild-to-moderate-to-severe
TED occurs in about 15%. The side effects of VIVA QUESTIONS
immunosuppressive treatment or radiation
do not weigh against the expected beneficial Q.1. What are the risk factors for TAO?
effects. Ans. Genetics: The TED is considered be an
zz Moderate-to-severe TED: Moderate-to-severe autoimmune disease because of its clinical
TED is defined as: no threat to vision but association with Graves disease, an
sufficient impact on daily life to justify the risks associated condition known to be caused
of immunosuppression. Corticosteroids are by anti-thyrotropin receptor antibodies
the treatment of choice with a response rates (TRAb).
up to 80%. Intravenous prednisolone treat Tobacco smoking: Smoking is the risk
ment is recommended because it has better factor most consistently linked to either
results compared with high-dose oral therapy development or deterioration of TAO.
and it is associated with less side effects such Overall, more than 40% of smokers either
as diabetes or weight gain. Prior to starting developed or worsened TAO, which was
high dose steroid possible contraindications almost double the rate of non smokers.
for high-dose prednisone treatment, such as Cigarette smoke extract increase produc
gastrointestinal ulcer disease, severe osteo tion by orbital fibroblasts of glycosamino
porosis, latent tuberculosis or hepatitis B or C glycans, hydrophilic macromolecules that
positivity, uncontrolled diabetes/hypertension accumulate in TAO orbital tissues.
must be ruled out. The cumulative dose of Therapy for TED with RAI: TAO HAS 15%
prednisolone should not exceed 8 g in one and 39% risk for development or progres
course of therapy. However, the exact dose sion after RAI therapy for hyperthyroidism.
of prednisolone that yields satisfactory The majority of patients developing TED
therapeutic effect without adverse events is after RAI treatment had mild and transient
not exactly known. disease requiring no treatment.

Thyroid dysfunction: Both hyper- and which is associated with orbital fibrosis,
hypothyroidism have been shown in glycosaminoglycan deposition and
multiple reports to be associated with enlarged extraocular muscles. There are
increased risk for development or usually no active inflammatory episodes
deterioration of TAO. in this phase.

Thyroxine and tri-iodothyronine levels:
Some studies have suggested that circul REFERENCES
ating tri-iodothyronine (T3) or thyroxine
(T4) may also be associated with GO. 1. Stan MN, Bahn RS. Risk Factors for Development
or Deterioration of Graves’ Ophthalmopathy.
Q.2. What is Rundles curve/natural course of THYROID. 2010;20:7.
TAO ? 2. Bhatt R, Nelson CC, Douglas RS. Thyroid-
Ans. Rundle conceptualized two distinct phases associated orbitopathy: Current insights
for TED, which is graphically represented into the pathophysiology, immunology and
in his famous ‘Rundle’s curve’. Rundles management. Saudi Journal of Ophthalmology.
curve represent the natural course of TED. 2011;25:15-20.
It helps in understanding and managing 3. Barrio-Barrio J, Sabater AL, Bonet-Farriol E,
TED. It has two stages: Velázquez-Villoria Á, Galofré JC. Graves’
1. An initial active inflammatory phase Ophthalmopathy: VISA versus EUGOGO
which is associated with by periorbital Classification, Assessment, and Management.
J Ophthalmol. 2015;2015:249125.
erythema and edema, conjunctival
4. Bahn RS. Graves’ ophthalmopathy. The New
chemosis, orbital inflammation and
England Journal of Medicine. 2010;362:
congestion, associated with upper lid 726-38.
retraction, proptosis, and occasionally 5. Dolman PJ. Evaluating Graves’ orbit
diplopia. The inflammatory phase opathy. Best Practice and Research Clinical
typically lasts for a period between 6 and Endocrinology and Metabolism. 2012;26:
24 months 229-48.
2. This is followed by a quiet, minimally
inflammatory chronic fibrotic phase
LACRIMAL GLAND TUMORS

Varsha Vashney, Amar Pujari
INTRODUCTION are inflammatory, and the other half is neoplastic.

Out of the neoplastic group, half are the aggressive
The lacrimal gland is situated in the superotem adenoid cystic carcinoma (ADCC) variety.1,2
poral orbit and it consists of 2 lobes, the orbital In exams, it can be given as a long case.
lobe, and the much smaller palpebral lobe.
The palpebral lobe can be visualized in the
superior fornix on lid eversion but not the orbital HISTORY
lobe. Thus, any pathology that affects the orbital
lobes only may be missed for a long period of time.
Demography
Lacrimal gland tumors account for about 10–15% Lacrimal gland tumors are seen more frequently in
of all orbital tumors.1 The clinician should consider the third to fourth decade of life (may present from
the axiom: “Half and a half; then half again.” childhood to old age), and the second bimodal
Approximately half of all lacrimal fossa masses peak is in the teenage years.
Oculoplasty 45
Chief Complaints Surgical History

The presentation varies from patients who are History of surgical removal of similar mass may
asymptomatic but have a slight fullness in the be there (recurrence is found in pleomorphic
temporal upper lid to those who present with frank adenoma). Incomplete excision of pleomorphic
proptosis, diplopia, and an encroaching mass adenoma can lead to relentless recurrences
lesion. and malignant transformation (Fig. 1). Thus the
previous history of biopsy (such as incisional or
History of Present Illness needle) is important in such cases.
All points as described in section proptosis
must be recorded carefully while taking history. History of Systemic Illness
In addition following points must be noted: The past general medical history may elicit
zz History of a long-standing (>1–2 years), non- important diagnostic information. For example, a
infiltrating lacrimal gland lesion suggests history of breast cancer might suggest metastasis.
a benign tumor, such as a pleomorphic A history of systemic inflammatory disease such
adenoma. as sarcoidosis should raise concern for a related
zz A shorter history suggests either an inflamma orbital inflammatory process.
tory or a malignant process.
zz Pain most commonly is seen with inflammatory EXAMINATION
lesions of the lacrimal gland, but adenoid
cystic carcinomas and other malignancies also Systemic Examination
can present with pain secondary to perineural
A detailed general examination is carried out to
or bony involvement.
rule out any systemic metastasis. Preauricular
zz Malignant lesions characteristically present
lymphadenopathy from regional metastasis in
with a subacute course of proptosis and
malignant lesions must be ruled out. Signs of
temporal sensory loss in the distribution of the
primary elsewhere in the body must be looked for.
lacrimal nerve in one-third of patients.
zz Limitation of eye movement, diplopia, and
diminished visual acuity can be seen with Ocular Examination
Large tumors due to distortion of the globe by Following points must be noted in ocular
the firm tumor mass. examination:
zz Benign lesions commonly present with zz Visual acuity: Diminished visual acuity can be
painless inferonasal globe displacement seen with rapidly progressive lesions. Patients
and fullness of the superotemporal lid and
orbit. Old photographs may be helpful in
establishing the duration of displacement.
zz Acute onset of a painful, erythematous,
indurated eyelid suggests inflammation.
zz Other symptoms that may present include
facial asymmetry noted by friends, epiphora,
exposure symptoms.
History of Past Illness

Past ocular history may uncover an episode of
trauma, prior periorbital surgery, or periocular
tumors that could relate to the present illness.
A history of intraocular malignancy such as
malignant melanoma might point to the possibility
of orbital extension or metastasis. Fig. 1: Adenoid cystic carcinoma of lacrimal gland
with induced hyperopia from an orbital mass zz Posterior segment: (Slit-lamp biomicroscopic
may show a significant asymmetric refractive examination using a 90D/78D lens and
error. indirect ophthalmoscopy)
zz Eyeball: Displacement of the globe with or —— Vitreous, optic disc and macula: Are
without proptosis can occur. The displacement usually normal.

is characteristically nonaxial with inferomedial —— Choroidal folds may be present, resulting
globe displacement. from globe indentation by an orbital mass.

zz Ocular balance and ductions: Binocular
patients should be examined for latent or INVESTIGATIONS
manifest ocular deviations and the approxi Following investigating modalities are used in a
mate extent of uniocular ductions in the four suspected case of lacrimal gland tumor:
cardinal positions estimated. A forced duction
(traction) test under topical anesthesia High-resolution Computed
will assist differentiation of neurological Tomography (HR-CT)
from mechanical causes of restricted eye
movements. Likewise, retraction of the globe zz Benign tumors: Pleomorphic adenomas
during an active duction suggests fibrosis of appear as well-defined, sometimes nodular
the ipsilateral antagonist muscle, this being a and non-homogeneous lesions that show
common sign with chronic orbital myositis. moderate enhancement with intravenous
zz Lids: An S-shaped contour to the upper lid contrast. Palpebral lobe tumors lie anterior
(the lateral half of the eyelid lies lower than to the orbital rim, whereas expansion of the
the medial half ) is characteristic for lacrimal lacrimal fossa with preservation of intact
gland lesions, but it is relatively nonspecific to cortical bone is seen in most cases of orbital
the type of tumor. A firm, rubbery, nontender lobe adenoma. Molding of the lacrimal gland
mass can be seen with either benign or fossa on CT scan is a hallmark of benign
lymphoproliferative lesions. growth. Discrete calcification may also be
zz Complete examination of mass should be present in a minority of cases, and indentation
done (as discussed in chapter proptosis) of the globe is common with larger tumors.
which includes size, shape, site, margins, zz Malignant tumors: These are poorly defined
edges, consistency, mobility, adherence to margins, with infiltration into surrounding
overlying skin and underlying bone, color tissues, and bone. Calcification occurs in
of skin, temperature of skin, reducibility and about one-third of carcinomas but is diffuse
compressibility, increase in size with valsalva compared to pleomorphic adenomas. In
maneuver , pulsations, and trans illumination. contrast to the hard pleomorphic adenomas
zz Conjunctiva, cornea: Signs of inflammation that flatten the globe, rapidly growing and
such as congestion and chemosis may be softer lesions (such as carcinoma and
present with dacryoadenitis, tumors or lymphoma) tend to mold to its surface.
infiltration of the lacrimal gland. A “salmon
patch” subconjunctival lesion may be present Histologic Findings
and is characteristic of lymphoma. Signs Histologic examination of pleomorphic adenomas
of exposure keratopathy (punctate defects reveals evidence of both epithelial and mesenchy
or epithelial defect with infiltrates) may mal differentiation. The proliferation of benign
be present. A reduced Schirmer’s test may epithelial cells usually is arranged in a double
indicate towards an inflammatory lesion (e.g. layer to form lumens. Stromal differentiation can
Sjögren syndrome). be seen in the formation of bone and cartilage.
zz Pupils: Usually the pupillary reaction is ADCC are derived from duct cells, and they
normal. RAPD may be present if the mass is form spaces into which basement membrane—
compressing or infiltrating the optic nerve. like material is deposited. This confers a cribriform
zz Anterior segment: Usually normal. Raised IOP or “Swiss cheese” appearance to the tissue, which is
may be present due to globe compression. characteristic of ADCC.
Oculoplasty 47
Immunohistochemistry zz Painless, progressive, slow growing

zz Well-circumscribed mass with absence of
This may be helpful in distinguishing between bony destruction
inflammatory, benign, and malignant lympho zz Remove with pseudo capsule intact to decrease
proliferative lesions. Immunohistochemistry is risk of recurrence or malignant transformation
a laboratory modality that uses special markers zz Histopathologically, comprised of two cell
to demonstrate the presence of specific antigens components: Benign epithelial cells arranged
in target tissues. Benign inflammatory lesions in double layer forming ducts stellate spindle
(pseudotumor) have a polyclonal morphology, cells contained in loose stroma
whereas the lymphoid lesions tend to be
monoclonal.
Myoepithelioma
DIFFERENTIAL DIAGNOSIS zz Rare tumor with biological behavior similar to
that of a pleomorphic adenoma
The differential diagnosis of a lacrimal gland mass zz Five subtypes: Spindle, plasmacytoid, epithe
has been described in Table 1. lial, clear and mixed.
Key features [also See Table 2].
Oncocytoma
Benign Tumors zz Rare tumor secondary to metaplasia of
ductular cells (epithelial origin).
zz Pleomorphic adenoma: It has following
zz Large, eosinophilic cells rich in mitochondria
important features:
“Warthin Tumor” (Cystadenolymphoma)
zz Most common intrinsic lacrimal gland lesion
zz Commonly presents as an epithelial neoplasm
of the salivary glands (lacrimal gland is an
unusual location).
Table 1 Differential diagnosis of lacrimal gland zz Epithelial columnar cells arranged in solid
mass nests or lining cystic spaces.
Non neoplastic Neoplastic
Dacryops/ Lymphoproliferative diseases Malignant Tumors
dacryoadenitis • Benign lymphoid hyperplasia
Adenoid Cystic Carcinoma
Dermoid cysts • Atypical lymphoid hyperplasia
Hemangioma • Malignant lymphoma zz Bimodal distribution with peak incidences in
Amyloid Benign tumors second and fourth decades of life
• Pleomorphic adenoma (benign zz Periocular pain (severe pain due to perineural
mixed tumor) spread), mild ptosis, proptosis, downward and
• Benign fibrous histiocytoma inward displacement of the globe
• Oncocytoma zz Bony erosion, bone destruction, and soft tissue
• Myoepithelioma calcification on CT
• Cystadenoma zz High mortality rate (intra-arterial cytoreduc
Malignant tumors tive chemotherapy may improve survival)
• Adenoid cystic carcinoma zz Sheets of epithelial cells arranged in solid or
• Malignant mixed tumor cribriform pattern resembling a glandular
(carcinoma expleomorphic structure is characteristic.
adenoma)
• Adenocarcinoma
Primary Adenocarcinoma
• Mucoepidermoid carcinoma
• Squamous cell carcinoma zz Rare tumor with clinical findings similar to
• Acinic cell carcinoma adenoid cystic carcinoma.
• Malignant oncocytoma zz Pleomorphic, mitotically active cells arranged
• Lung and breast metastases in sheets and cords.
Table 2 Summary of major lacrimal gland tumors

Histopathologic
Types of lesion Clinical features Imaging features features Treatment
Pleomorphic Painless, CT—round to oval Two morphologic Modified lateral
adenoma (benign progressive, slow- well circumscribed cell components: orbitotomy and
mixed tumor) growing mass on mass in the lacrimal benign epithelial excision using an
Most common superotemporal fossa, with bony cells arranged in extraperiosteal
epithelial tumor area of upper expansion and a double forming approach for the
eyelid, excavation, no bony ducts and stellate lateral portion
Nontender, firm, destruction. spindle cells For an anteriorly
well-contoured USG—round to oval contained in a situated palpebral
mass. mass with medium loose stroma. lobe tumor, isolated
Variable proptosis, to high reflectivity Epithelial cells in dacryoadenectomy
Diplopia loss of and regular internal the stroma can via a transcutaneous
vision rare structure undergo metaplasia or transconjunctival
with cartilaginous, approach
fibrous or myxoid
characteristics
Oncocytoma Rare Large, eosinophilic Complete surgical
affects elderly cells rich in excision
females mitochondria
caruncle most
common site
Cystadenoma Rare Similar to those Epithelial columnar Complete excision
(Warthin’s tumor) Clinical of a pleomorphic cells arranged in of the globular
characteristic adenoma solid nests or lining cystic mass with
is similar to cystic spaces. preservation of the
pleomorphic Often contains an thin capsule
adenoma exudative fluid
component and a
lymphoid infiltrate
with focal follicular
organization
Adenoid cystic Periocular pain, Globular lacrimal Sheets of epithelial En bloc, excision
carcinoma mild ptosis, gland mass with cells arranged of the orbit and its
proptosis, brow irregular borders, in either solid or contents, including
numbness and bony erosion, bone cribriform patterns the orbital roof, the
diplopia. destruction and soft with spaces into lateral wall, the lids,
Rapid progression tissue calcification. which basement and the anterior
symptoms are Contiguous membrane portion of the
typically present tumor extension to like material is temporalis
for 6 months, and adjacent area deposited. muscle where the
almost always less (Swiss-Cheese) zygomatico frontal
than one year and zygomatico-
temporal nerves
extend.
Adjunctive
postoperative
radiotherapy
Contd…
Oculoplasty 49
Contd…
Histopathologic
Types of lesion Clinical features Imaging features features Treatment
Malignant mixed The average age at Similar to adenoid Histopathologically, Complete surgical
tumor diagnosis is cystic carcinoma, the malignant resection
50 years may show a bilobed component may be Mortality is high
This tumor may appearance attached to and arise
arise de novo, from the benign
because of malig mixed aspect of the
nant transforma tumor, yielding a
tion following an bilobed appearance
incomplete exci-
sion of a benign
adenoma, or as
malignant trans-
formation years
after diagnosis of a
presumed benign
adenoma
Mucoepidermoid Rarely Similar to adenoid Epidermoid and Excision with or
carcinoma Locally aggressive cystic carcinoma mucus-secreting without adjuvant
Average age at cells arranged in a radiotherapy.
presentation of pattern of cords and Advanced stage has
49 years islands. a worse prognosis,
Male: Female 2:3 The mucus-secreting require exenteration
cells and cystoid and radiotherapy
spaces within the
specimen stain
positively with
mucicarmine, alcian
blue stains and
Periodic acid-Schiff
reaction
Carcinosarcoma Carcinosarcoma Considered in the Management
may arise from differential diagnosis requires complete
a pleomorphic of a lacrimal gland excision of the lesion
adenoma mass, if sarcomatous
components are
encountered
on histologic
examination
Pleomorphic Adenocarcinoma TREATMENT3-5 (TABLE 3)

(Malignant Mixed Tumor)
Pleomorphic Adenoma
zz May arise de novo, as consequence of malig
nant transformation following incomplete It should be excised intact with a cuff of normal
excision of benign adenoma or as malignant tissue. Palpebral lobe tumors can be resected
transformation of a presumed benign through an upper lid skin crease incision
adenoma. Stallard-Wright incision (anterior orbitotomy).
zz Well circumscribed, pseudo capsulated. Orbital lobe tumors can be approached through
Table 3 Difference between pleomorphic adenoma and adenoid cystic carcinoma

Features Pleomorphic adenoma Adenoid cystic carcinoma
Pain Painless Periocular pain often sever
Course Slow-growing mass Fast growing
Proptosis Variable Proptosis with downward and inward
displacement of the globe
Associated Decreased vision and diplopia rare Brow numbness is characteristic, diplopia,
features mild ptosis
Palpation Non-tender, firm, well contoured mass Firm irregular bordered mass
Histological Benign epithelial cells arranged in a double Sheets of epithelial cells arranged in either
features layer forming ducts and stellate spindle cells solid or cribriform patterns (Swiss-Cheese
contained in a loose stroma pattern) that mimic glandular structure
Radiological • Round to oval well-circumscribed mass in • Globular lacrimal gland mass with borders
features the lacrimal fossa that are irregular
• Bony expansion and excavation but no • Associated with bony erosion, bone
bony destruction destruction and soft tissue calcification
• The posterior edge of the lesion typically • Tumor extension toward the medial orbit,
exhibits a curved contour that molds to the apex and the temporalis fossa
adjacent orbital bone
a lateral orbitotomy approach. It is important followed by external beam radiation therapy

to avoid any intraoperative spillage during (EBRT). ADCC is often extensive and requires
surgery. Thus, a buffer of normal tissue should orbital exenteration or midfacial resection. EBRT
always be maintained around the tumor mass. delay the growth or recurrence of the tumor.
If intraoperative spillage of cells occurs through Brachytherapy (locally implanted radioactive
cautery and lavage of the operative field has to be plaques seeds) may also give results similar to
done. If the periosteum is already destroyed, the EBRT. Chemotherapy does not have a recognized
breach should be treated by strict surgical isolation role in the treatment of adenoid cystic carcinoma.
and Cyanoacrylate glue may be applied to minor The malignant mixed tumor is treated with
capsular breaches during surgery. Excision of the local excision followed by irradiation.
orbital lobe alone, with preservation of palpebral Metastatic tumor carries a poor prognosis.
lobe, reduces the incidence of dry eye and The target is to provide palliative therapy through
secondary corneal disease. orbital irradiation or chemotherapy.
Biopsy (other than excision) should not be
attempted in any suspected case of pleomorphic VIVA QUESTIONS
adenoma. If it has been inadvertently biopsied, the
biopsy tract and the tumor should be meticulously Q.1. Most common lacrimal gland tumors.
excised since recurrent pleomorphic adenoma is Ans. • P leomorphic adenomas account for
typically infiltrative and may need extensive tissue almost all benign tumors of the lacrimal
resection or exenteration. gland.
• Adenoid cystic carcinoma is the most
common (76%) malignant epithelial
Adenoid Cystic Carcinoma
tumor.
It depends upon the extent of the tumor. For • Carcinoma arising in pre-existing pleo
tumors that are localized to the orbit excision of morphic adenoma (malignant mixed
the tumor and adjacent tissues should be done. tumor) is the second most common
Advanced tumors require surgical resection malignancy of the lacrimal gland.
Oculoplasty 51
• L
ymphoma accounts for about 10–14% cystic carcinomas was 47%. This number
of all lacrimal gland masses and may be reduces to 20% after 13 years and 22% after
part of a systemic disease. 15 years. Most cases recur within 2 years of
Q.2. Where do you find a “salmon patch treatment. Intracranial spread can occur
lesion”? due to the propensity of perineural invasion
Ans. Salmon patch subconjunctival lesion is that results in the death of the majority of
a characteristic non-tender firm reddish patients.
fleshy mass of conjunctival lymphoma.
It may be an extension of orbital or REFERENCES
intraocular lymphoma. Only 25% patients
of orbital/adnexal lymphoma have 1. Albert DM, Miller JW, Azar DT. Albert
conjunctival involvement. Most commonly, and Jakobiec’s principles and practice of
ophthalmology; 2008.
it is confused with chronic follicular con
2. Bowling B. Kanski’s clinical ophthalmology:
junctivitis. Histologically, these are non-
Hodgkin’s lymphoma of low-grade B-cell Elsevier, 2015.
variety. 3. von Holstein SL, Coupland SE, Briscoe
Q.3. Differential diagnosis of a lacrimal fossa D, Le Tourneau C, Heegaard S. Epithelial
mass. tumors of the lacrimal gland: a clinical,
Ans. Refer to differential diagnosis. histopathological, surgical and oncological
survey. Acta Ophthalmol. 2013;91(3):195-206.
Q.4. How do you differentiate pleomorphic doi: 10.1111/j.1755-3768.2012.02402.x. Epub
adenoma from adenoid cystic carcinoma? 2012 Apr 4. Review.
Ans. See Table 3. 4. Bernardini FP, Devoto MH, Croxatto JO.
Q.5. What is “Swiss cheese” pattern of ADCC? Epithelial tumors of the lacrimal gland: an
Ans. It refers to the microscopic picture of update. Curr Opin Ophthalmol. 2008;19(5):
ADCC. Closely packed small, densely 409-13. Review.
stained cells aggregate around large ovoid 5. Alkatan HM, Al-Harkan DH, Al-Mutlaq
M, Maktabi A, Elkhamary SM. Epithelial
spaces containing hyaline or mucin. This
lacrimal gland tumors: A comprehensive
resembles the Swiss cheese pattern and
clinicopathologic review of 26 lesions with
hence the description. radiologic correlation. Saudi J Ophthalmol.
Q.6. Prognosis of ADCC. 2014;28(1):49-57.
Ans. The prognosis is poor in cases of ADCC. The
overall 5 year survival rate of all adenoid
SHORT CASES
CONGENITAL PTOSIS
INTRODUCTION
Drooping of the eyelid is known as ptosis. It may be
present at birth, or it may develop later in life. If a
droopy eyelid is present at birth or within the first
year of life, the condition is called congenital ptosis.
In most cases of congenital ptosis, the problem is
isolated and does not affect the vision.1-3
Congenital ptosis can be given as a short case
in exams.
HISTORY
All pediatric patients presenting with either
unilateral droopy eyelid or bilateral droopy eyelids Fig. 1: Simple severe congenital ptosis
need a thorough history and examination (kindly
see the section of ptosis, long case). History of Drug or Allergic Reactions
A history of drug or allergic reactions may be
Chief Complaint helpful. Allergic reactions can result in eyelid
edema and droopy eyelid.
Parents usually bring the child with the history of
drooping of the eyelid (Fig. 1) or narrow palpebral
fissure since birth. History of Trauma
Orbital wall fractures (pseudoptosis with enoph
History of Present Illness thalmos) or IIIcranial nerve palsy from trauma
may result in ptosis.
The onset, progression, and other associated
abnormalities such as deviation of eyes, nystagmus,
face turn and any relation to the amount of ptosis EXAMINATION
to jaw movement. A case of congenital ptosis must be evaluated in
detail as described in the chapter of long case
Medical History ptosis. In cases of congenital ptosis following
should be taken care of:
A careful medical history regarding malignancy
zz Visual acuity: Risk of amblyopia is there in
should be obtained. Metastatic or primary orbital
case of severe ptosis. The amblyopia can occur
tumors can result in malpositioning of the eyelid.
due to occlusion amblyopia or rarely due to
astigmatism induced by the compression of
Family History the droopy eyelid.
A patient with a strong family history of congenital zz Refractive error and cycloplegic refraction
ptosis may not need an extensive work-up. should be recorded in all cases.
Family photographs can help determine onset or zz In infants, make sure that the baby can fixate
variability of the ptosis. and follow objects with each eye individually.
Oculoplasty 53
zz The patient should be evaluated for strabismus zz New onset of third cranial nerve palsy with or
(misalignment). without other neurologic findings
zz Serial external photographs of the eyes and the zz Globe displacement with either enophthalmos
face may be included in the patient’s record for or proptosis.
documentation. Differential diagnosis/classification/manage
zz Tear function should be evaluated. ment has been covered in detail in the long case,
zz Corneal sensitivity should be tested (if ptosis part.
possible) may be a difficult test in young
pediatric patients. MANAGEMENT
zz The pupillary size and the iris color differences
between the eyes should be examined for The following points are considered while per
Horner syndrome. forming the surgery.
zz Palpebral fissure distance.
zz Lid position in downgaze (the ptotic lid Timing of Surgery
appears higher in downgaze).
It is advisable to wait till 4-5 years of age for surgical
correction when the tissues are mature enough
Levator Function to withstand the surgical trauma and a better
Measurement of levator function in small children assessment and postoperative care is possible
is a difficult task, as the child allows no formal due to improved patient co-operation.2,3 Urgent
evaluation. The presence of lid fold and increase surgery is indicated in children with severe ptosis
or decrease its size on the movement of the eyelid developing amblyopia. In such cases, sling surgery
gives us a clue to the levator action. The presence is done.
of anomalous head posture like the child throwing
his head back suggests a poor levator action. Surgical Approach
Iliff Test It is based on whether the:

zz Ptosis is unilateral or bilateral
This test can be performed in the first year of life to zz Severity of ptosis
evaluate the levator function. The upper eyelid of zz Levator action
the child is everted as the child looks down. If the zz Presence or absence of abnormal ocular
levator action is good lid reverts on its own. motility, jaw-winking phenomenon or
blepharophimosis syndrome.
INVESTIGATION
A routine case does not require any specific Aim of Surgery
investigations except those required for general Target is to lift the ptotic lid above the papillary
anesthesia if surgery planned (hemoglobin, aperture when the eyes are in the primary position.
urea, creatinine, bleeding and clotting time). The height of the two lids regardless of whether the
Neuroimaging (MRI or CT) is indicated in ptosis is unilateral or bilateral should be equal.
following conditions: There should also be adequate mobility of the
zz If history not consistent and onset not clear lid when blinking, a normal lid fold and no
zz Other neurologic findings along with ptosis are diplopia.
present
zz Orbital wall fracture suspected with history of
Surgical Procedure
trauma
zz Visible or palpable lid mass The choice of surgery is given below:
zz Suspected orbital tumors (e.g. lymphoma, zz Fasanella-Servat operation
leukemia, rhabdomyosarcoma) —— Mild ptosis (<2 mm or less)
zz New onset of Horner syndrome with or without —— Levator action >10 mm
other neurologic findings —— Well-defined lid fold-no excess skin

Table 1 Berke’s criteria for levator resection

Degree of ptosis Levator function Amount of levator resection Ideal preoperative correction
1.5–2 mm (mild) Good (8 or more) Small (10–13) Under correct by
Good (8 or more)—usual Moderate (14–17) 1–3 mm
3 mm (moderate) Fair (5–7) Large (18–22) Match the level of normal lid
Poor (rare, 4 or less) Maximal (23 or more) and correct ptosis fully
4 or more (severe) Fair (Sometimes, 5–7) Super maximal (27 or more) Over correct by 1–2 mm
Poor (usual, 4 or less) Frontalis sling
zz Levator resection Table 2 Putterman’s criteria for amount of

—— Mild/moderate/severe ptosis
levator resection
—— Levator action ≥4 mm
zz Brow suspension ptosis repair Levator action Recommended lid placement

—— Severe ptosis 2–4 mm 1 mm above the limbus
—— Levator action <4 mm
5–7 mm 1 mm below the limbus
—— Jaw-winking ptosis (along with LPS
8 mm or more 2 mm below the limbus
excision) or blepharophimosis syndrome.
In cases with bilateral congenital ptosis,
simultaneous bilateral intervention in the two
eyes is needed. However, in cases where gross • B lepharophimosis syndrome (BPES):
asymmetry exists between the two eyes, the eye Short palpebral fissures, congenital
with a greater ptosis is operated first and the other ptosis, epicanthus inverses, and tele-
eye is operated after 6–8 weeks when the final canthus.
correction of the operated eye can be assessed. • Third cranial nerve palsy
Levator resection is the most commonly • Horner syndrome: Ipsilateral findings
performed procedure, there are different criteria of mild ptosis, miosis, and anhidrosis
to determine the amount of levator resection characterize this syndrome.
required. The two important and widely used • Marcus Gunn jaw-winking syndrome:
guidelines are Berkes (Table 1) and Puttermans The motor nerve to the external pterygoid
(Table 2) method. muscle is misdirected to the ipsilateral
levator muscle. Lid elevation occurs with
Contraindications to Surgery mastication or with the movement of the
jaw to the opposite side.
Ptosis surgery is relatively contraindicated in • Birth trauma
presence of following: • Periorbital tumor: Neuroblastoma, plexi
zz Poor orbicularis muscle function (lagoph form neuromas, lymphomas, leukemias,
thalmos and corneal exposure) rhabdomyosarcomas, neuromas, neuro
zz Loss of blink reflex fibromas, or other deep orbital tumors
zz Loss of corneal sensitivity may produce ptosis and proptosis.
zz Significant dry eye • Kearns-Sayre syndrome: Progressive
zz Poor Bell’s phenomena. external ophthalmoplegia, heart block,
retinitis pigmentosa, and central nervous
VIVA QUESTIONS system manifestations. This condition
begins in childhood but is rarely present
Q.1. What are the causes of congenital ptosis? at birth.
Ans. Following are important causes of • Myotonic dystrophy.
congenital ptosis: • Blepharochalasis: Infiltrative processes
• Idiopathic that thicken the lids and produce ptosis.
Oculoplasty 55
• M yasthenia gravis: A defect at the neuro (usually associated with severe ptosis)
muscular junction. an inferior rectus recession at times
• Pseudotumor of the orbit: Ptosis due to combined with superior rectus resec
inflammation and edema of the eyelid. tion is carried out on the affected side
• Pseudoptosis: Less tissue in the orbit (e.g. as the first procedure. To correct the
unilateral smaller eye, fat atrophy, blow ptosis levator resection with bilateral
out fracture) produces the appearance brow suspension is done later. Knapp’s
of ptosis secondary to the decreased procedure may be done for ptosis
volume of orbital contents. associated with double elevator palsy
where lateral and medial rectus tendons
Q.2. Describe the pathology in congenital
are transplanted to the area of superior
ptosis.
rectus insertion. This does not cause
Ans. Histologically, the levator muscles of
significant limitation of adduction or
patients with congenital ptosis are dystro
abduction. Ptosis is corrected 3 months
phic. The levator muscle and aponeurosis
later.
tissues appear to be infiltrated or replaced
• B lepharophimosis syndrome: The
by fat and fibrous tissue. In severe cases,
following sequence is followed:
little or no striated muscle can be identified – Y-V plasty mustard’s double “Z”
at the time of surgery. This suggests plasty with transnasal wiring is done
that congenital ptosis is secondary to as a primary procedure. This gives
local developmental defects in muscle a good surgical result both in terms
structure. Congenital ptosis may occur of correction of telecanthus as well
through autosomal dominant inheritance. as deep placement of the medial
Common familial occurrences suggest that canthus. The results are long lasting.
genetic or chromosomal defects are likely. – Brow suspension is carried out
Q.3. Complications of the sling surgery. 6 months after the first procedure for
Ans. Complications associated with the frontalis correction of ptosis.
suspension procedure for congenital ptosis • Marcus Gunn ptosis: Mild cases of
repair include the following: jaw winking where the jaw winking is
• Granuloma minimal can be treated satisfactorily by
• Lid asymmetry Fasanella-Servat operation while severe
• O vercorrection with exposure kerato- cases or cases where jaw winking is
pathy prominent, require bilateral resection of
• Undercorrection levator aponeurosis and terminal levator
• Infection. with fascia lata brow suspension.
• Misdirected third nerve ptosis: In cases
Q.4. What is the prognosis after surgery? of misdirected third nerve ptosis
Ans. The repair of congenital ptosis can produce where treatment is imperative levator
excellent functional and cosmetic results. resection with bilateral fascia lata
Careful observation and treatment, ambly sling is the procedure of choice. Ptosis
opia ptosis can be treated successfully. associated with third nerve palsy is
Patients who require surgical intervention, difficult to manage because of poor
50% or more may require repeat surgery in bell’s phenomenon. A crutch glass may
8–10 years following the initial surgery. be prescribed or a conservative sling
Q.5. Management of complicated ptosis. surgery may be performed.
Ans. The management of simple congenital
ptosis associated with other anomalies is REFERENCES
described below: 1. Bernardini FP, Devoto MH, Priolo E. Treatment
Ptosis with oculomotor abnormalities: In
• of unilateral congenital ptosis. Ophthalmology.
cases, with superior rectus involvement 2007;114(3):622-3.
2. Shields M, Putterman A. Blepharoptosis 3. Yilmaz N, Hosal BM, Zilelioglu G. Congenital

correction. Curr Opin Otolaryngol Head Neck ptosis and associated congenital malformations.
Surg. 2003;11(4):261-6. J AAPOS. 2004;8(3):293-5.
ECTROPION
Aditi Dubey, Bijnya Birajita Panda
INTRODUCTION Schirmer’s test: (to rule out dry eye).

Syringing and Jones test I and II (to rule out
Ectropion is characterized by an eversion or lacrimal passage obstruction).
outward turning of the eyelid margin away from
the globe. It is a commonly encountered eyelid
malposition. It is characterized by rotation of lid
Tests for Ectropion
margin outwards resulting in its fall away from the Pinch Test
globe.1 To make things worse, the constant wiping To determine the amount of lid laxity. If the lid can
and rubbing of eyes irritated by the epiphora be pulled more than 6 mm away from the globe,
further aggravates the condition. The underlying the lid is lax. If the medial and lateral canthal
factor may vary in each case and an appropriate tendons are lax as well, the lid can be pulled away
identification of the type of ectropion and the up to 20–25 mm.
factor responsible for its occurrence are important
in choosing the correct surgical intervention.2,3
Snap-back Test
In exams, it is usually given as a short case or
spot case. Downward traction is applied to the lower lid and
then released the lid should revert to its normal
position, without the aid of a blink in a normal
HISTORY
person. However, when laxity is present, the lid is
Chief Complaints not opposed to the globe.
Watering, irritation, grittiness, foreign body Grading of lid laxity according to snap-back test
sensation or chronic red eye. Symptoms are caused zz Normal: The lid returns to its position
by ocular exposure and inadequate lubrication. immediately on release
zz Grade 1: Approximately 2–3 sec
zz Grade 2: 4–5 sec
Past History zz Grade 3: >5 sec but returns to position on
Facial palsy, lid trauma, ocular allergy and previous blinking
lid surgery should be taken. zz Grade 4: Continues to hang down
EXAMINATION Inferior Lid Retractor Laxity

Retractor weakness can be demonstrated by
The routine examination is carried out and the
observing the lower lid as the patient looks down.
conjunctiva, cornea, and anterior chamber are
Reduction in inferior movement on down gaze
examined for any signs of inflammation.
and a deep inferior fornix occurs due to laxity or
Eyelid: There is outward turning of the lid margin. loss of retractor attachment in this area.
There may be signs of chronic blepharitis.
Conjunctiva: Keratinization and hypertrophy. Medial Canthal Tendon (MCT) Laxity
Cornea: Changes secondary to exposure may be The lateral excursion of the inferior punctum is
present. measured by pulling the lid laterally. The punctum
Oculoplasty 57
lies lateral to the caruncle at rest and should not absent nasolabial fold and drooping of the angle
be displaced more than 1–2 mm with lateral lid of the mouth should also be looked for.
traction. If pulling on the medial canthus allows
the punctum to be stretched, it suggests MCT is CLASSIFICATION
lax. Laxity is graded as following:
zz Mild: Up to the limbus Ectropion can be classified as following:
zz Moderate: Up to the pupil zz Congenital ectropion: Rare, associated with
zz Severe: Beyond the temporal pupillary border. congenital epiblepharon.
zz Acquired ectropion: Can be further classified
as following on the basis of pathogenesis:
Lateral Canthal Tendon (LCT) Laxity —— Involutional ectropion: It is the most
The lateral canthal angle should be evaluated common variety. Multiple factors are
with the lid at rest. The lateral canthus should responsible for its development e.g.
have an acute angular contour and should lie horizontal lid laxity, medial canthal
1–2 mm medial to the lateral orbital rim. A rounded tendon laxity, etc. which are all normal
appearance of the canthus indicates laxity. The aging changes of the lid (Fig. 1).
lateral part of the lid if pulled medially should —— Cicatricial ectropion: Lid margin is
not result in more than 1–2 mm movement of the pulled away from the globe due to the
lateral canthal angle, in absence of laxity. shortage of skin e.g. congenital shortage
(Fig. 2), trauma, burns, cicatrizing skin
Position of the Lacrimal Puncta tumors, allergies, etc. it may be unilateral
or bilateral/localized or generalized
Punctum alone can be everted or the whole lid may depending on the cause.
be everted. In a normal lid, the inferior punctum is
directed posteriorly against the globe and should
not be visible without pulling the lid downward.
Direction of the punctum away from the globe is
the earliest sign of medial lid ectropion and can be
graded as follows:
zz Mild: Puncta are not opposed to the globe on
looking up.
zz Moderate: Puncta are not opposed to the globe
even in primary gaze.
zz Severe: Palpebral conjunctiva and fornix are Fig. 1: Senile ectropion
exposed.
Cicatricial Skin Changes

Vertical shortening of the anterior lamella—like
signs of repair of lid laceration or scar of excision
of the tumor should be looked for.
Orbicularis Muscle Weakness

The facial nerve must be examined to rule out
ectropion due to paralysis of the seventh nerve.
Lagophthalmos and reduced force of contraction
on forced eyelid closure demonstrate orbicularis
muscle weakness. Other signs of facial palsy Fig. 2: Paralytic ectropion due to tight skin in
such as brow ptosis, loss of forehead wrinkles, a collodion baby
—— Mechanical ectropion: Tumor or cyst near zz Mild ectropion with an excess of skin:
the lid margin mechanically pulling down Modified Kuhnt-Szymanowski procedure:
the lid. blepharoplasty with a base up lateral triangle
—— Paralytic ectropion: VII N palsy resulting and excision of full thickness wedge of lid
in sagging and downward displacement beneath the blepharoplasty flap.
of paralyzed orbicularis muscle. zz Moderate ectropion—generalized or affecting
lateral lid:
GRADING Lateral tarsal strip: In this, a horizontal
incision is made and inferior crus of LCT is cut,
Grade of Orbito-lid Apposition triangular portion of the temporal lid is resected
zz Grade 0: With normal lid-globe apposition sparing tarsus and the tarsal strip is sutured
zz Grade 1: With punctal eversion with a mattress suture in a superotemporal
zz Grade 2: With partial lid eversion and scleral direction to the periosteum. Smith and Lisman
show propose an alternative method where after
zz Grade 3: With conjunctival hyperemia and excising the anterior lamella of the lid on
thickening temporal aspect the periosteum is exposed in
zz Grade 4: As for grade 3 with exposure keratitis. a superotemporal direction and the tarsal strip
is sutured to it (Fig. 3).
Grades of Ectropion zz Marked ectropion: Double wedge resection
with lateral tarsal strip
zz Grade 1: Punctal eversion zz Extreme ectropion: Temporalis muscle transfer
zz Grade 2: Eversion of sharp posterior lid margin Involutional ectropion affecting only the
zz Grade 3: Palpebral conjunctival exposure medial aspect
zz Grade 4: Exposure of the fornix. zz Only punctal eversion present and no lid laxity:
Medial conjunctivoplasty
TREATMENT zz Horizontal lid laxity is present but MCT is
not lax: Lazy–T (Excision of tarsoconjunctiva
Factors considered for selection of surgery for
combined with full thickness wedge excision
ectropion:
of lid)
zz Basic cause of ectropion
zz Horizontal lid laxity which is due to MCT
zz Secondary mechanisms coexisting with the
laxity: MCT plication or resection depending
basic pathology
on severity of laxity.
zz Grade of ectropion
zz Identify the defects in various components of
lid lower Management of Cicatricial Ectropion
zz Excess lid skin Correction of a cicatricial ectropion requires
zz Laxity of LCT/MCT and its severity lengthening of the cutaneous surface and
zz Shortening of posterior lamella comprising of
tarsoconjunctiva
zz Any mass lesion in lid causing ectropion
zz Any scarring whether localized or generalized
zz Systemic disease causing scarring of tarso-
conjunctiva.
Surgical Management of Ectropion

Involutional Ectropion
zz Mild-to-moderate ectropion mainly affecting
lateral lid: Full thickness pentagonal wedge Fig. 3: Cicatricial ectropion (Figure 1) after surgery
resection of the lid. (lateral tarsal strip procedure)
Oculoplasty 59
correction of any associated factors-resection • Lower lid retractors disinsertion or laxity

of subcutaneous cicatrix or horizontal lid
Q.2. Congenital ectropion.
lengthening. Following surgeries have been
described: Ans. Congenital ectropion is a rare entity. It is
zz Localized: Z-plasty (Elschnig’s operation) more often associated with blepharophi
zz V- Y operation mosis syndrome (BPES Type 2), Down
zz Severe or generalized cicatricial ectropion: Full- syndrome or ichthyosis. In rare cases,
thickness skin grafting. congenital ectropion occurs as an isolated
finding. It is caused by a vertical insuffi
ciency of the anterior lamella of the eyelid.
Management of Paralytic Ectropion Complications include chronic epiphora
Management involves giving support to the lower and exposure keratitis. Management is as
lid or strengthening of the lower lid. Support can follows:
be given medially, laterally or to the lower lid as a • Mild congenital ectropion—no treat-
whole. In long standing cases associated with cheek ment.
ptosis, a cheek lift/mid-face lift may be necessary. • S evere and symptomatic—horizontal
Following surgeries have been described: tightening of the lateral canthal tendon
zz Only medial ectropion: Medial canthoplasty and vertical lengthening of the anterior
zz Generalized lid laxity: MCT plication + lateral lamella by means of a full-thickness skin
canthal sling graft.
zz Medial with MCT laxity: MCT resection. A complete eversion of the upper eyelids
occasionally occurs in premature infants
Management of Mechanical Ectropion transiently due to orbicularis slippage/
lamellar slippage, inclusion conjunctivitis,
Masses near the lid margin causing the ectropion anterior lamellar inflammation or shortage,
should be excised. Excision should be as vertical or Down syndrome. Treatment includes
as possible and it is important to avoid scar topical lubrication, short-term patching of
formation/ skin shortening. eyes, full-thickness sutures or a temporary
tarsorrhaphy.
VIVA QUESTIONS Q.3. What are the disadvantages of lid
resection procedures?
Q.1. Pathogenesis of involutional ectropion. Ans. • Does not correct the underlying physio
Ans. Ectropion involves the lower lid more com logical abnormality
monly than the upper lid. There is decreased • Causes lid notching
resilience and increased laxity of periocular • Causes lid shortening
tissues due to age-related microinfarction • Causes loss of meibomian glands.
and secondary atrophy. This inadequate
support and effect of gravity cause more REFERENCES
pronounced stretching of the lower lid
increasing the burden on suspensory 1. Bosniak SL, Zilkha MC. Ectropion. In: Smith`s
Ophthalmic plastic and reconstructive surgery,
canthal tendons and resulting in ectropion.
Mosby; 2nd edn; 1998. pp. 290-307.
Primary abnormality is laxity of the lateral
2. Bergin DJ. Anatomy of the eyelids, lacrimal
canthal tendon. Other contributory factors system, and orbit. In: McCord CD, Tanenbaum,
include: Nunery WR (Eds). Oculoplastic surgery. 3rd
• Horizontal lid laxity edn. New York: Raven press; 1995. pp. 51-84.
• Medial canthal tendon laxity 3. Robinson FO, Collin RO. Ectropion. In: Yanoff
• Punctal malposition M, Duker JS (Eds). Ophthalmology, 2nd edn.
• Vertical tightness of the skin India: Mosby. 2006;1:676-83.
ENTROPION
Aditi Dubey, Ritu Nagpal
INTRODUCTION zz Lid laxity:

—— The pinch test: To determine the amount
Entropion is a condition in which eyelid turns of lid laxity. If the lid can be pulled more
inward. This causes the eyelashes or the eyelid than 6 mm away from the globe, the lid
margin to rub against the eyeball and results in is lax. If the medial and lateral canthal
irritation, watering, redness, keratitis and even tendons are lax as well, the lid can be
corneal perforation. It may occur at any age but pulled away up to 20–25 mm.
occurs primarily because of advancing age. It can —— There may a hump on lower eyelid due to
be missed easily and thus one should specifically overriding of preseptal orbicularis over
look at the eyelid to diagnose it.1-3 the pretarsal part.
In exam, it can be given as a short case. —— It is often associated with an absence of
the downward excursion of the eyelid in

HISTORY down gaze due to the weakness of the
lower lid retractors. Excursion of the lower
Chief Complaints
lid in down gaze usually 3–4 mm—loss of
Patients with entropion commonly complain of: movement indicates retractor weakness/
zz Foreign body sensation disinsertion.
zz Frequent eye infections zz Snap-back test: Perform this test by pulling the
zz Red eyes lower lid away and down from the globe for
zz Watering. several seconds. If the lid resumes position,
note the time required for the lid to return to its
Past History original position without the patient blinking.
The snap-back test provides a good idea of
History of onset is important to rule out congenital
relative lower lid laxity. Lids with normal laxity
component.
immediately spring back to original position;
Age of patient: In children, congenital entropion the longer this takes, the more laxity is present.
is rare and should be definitely differentiated from —— Grades
epiblepharon. Normal: Lid returns immediately on
History of ocular trauma, facial burn, Stevens- release

Johnson syndrome is important for cicatricial Grade 1: Approximately 2–3 sec
entropion. Grade 2: 4–5 sec
History of ocular surface irregularity or any

other painful ocular pathology (acute spastic
entropion).
EXAMINATION
zz Lid margin is found in-turned (Fig. 1).
Depending upon the degree of in turning it can
be divided into three grades.
—— Grade I: Only the posterior lid border is
in rolled
—— Grade II: Entropion includes in turning
up to the intermarginal strip

—— Grade III: The whole lid margin including
the anterior border is in turned. Fig. 1: Entropion causing keratopathy

Oculoplasty 61
Grade 3: >5 sec but returns to position

on blinking
Grade 4: Continues to hang down
zz Medial canthal laxity test: Perform this test by

pulling the lower lid laterally from the medial
canthus. Measure displacement of the medial
punctum. Greater distance equates to more
laxity. Normal displacement ranges from only
0–1 mm.
—— Grades
Mild—up to the limbus
Moderate—up to the pupil
Severe—beyond the temporal

pupillary border.
Fig. 2: Entropion with eyelash rubbing cornea
zz Lateral canthal laxity test: Perform this test by
causing keratopathy
pulling the lower lid medially from the lateral
canthus. Measure displacement of the lateral
canthal corner. Greater distance equates to zz Lacrimal system patency assessment: Done by
more laxity. Normal displacement ranges from syringing, dye disappearance test and jones
only 0–2 mm. Assign grades on a scale from dye test I & II.
0–4 (0 = normal laxity, 4 = severe laxity).
zz Bell phenomenon: Instruct patient to attempt CLASSIFICATION
eye closure while the examiner holds lids Entropion can be classified into congenital,
open. If eyes move up, the test indicates a involutional, cicatricial and acute spastic.1-3
positive result for Bell phenomenon. zz Congenital entropion: Occurs due to hyper
zz Orbicularis muscular tone: Ask the patient to trophy of the anterior lamella. Mostly mild,
squeeze eyes shut. Note how much worse the resolves with time.
entropion is immediately after opening. zz Involutional entropion: An age-related
—— Grades of orbicularis muscle tone
condition caused by the laxity of tarsus, its
Grade 0 = no paralysis
medial and lateral canthal tendons, lower lid
1 = weak
retractors, along with the over-riding of the
2 = normal
orbicularis oculi muscle.
3 = overactive
zz Cicatricial entropion: Occurs due to scarring
4 = spastic
and shortening of the posterior lamella due to
zz The digital eversion test can be done to
chemical injury, infection or Stevens-Johnson
distinguish cicatricial component: Observe
syndrome.
directly by everting the lids. It can also be zz Acute spastic entropion: Follows ocular
ascertained by pulling the lid superiorly if it
irritation or inflammation.
does not reach 2 mm above lower limbus; lid
is vertically deficient.
Most Common Types of Entropion
zz Slit-lamp examination: To look for corneal
status and other evidence of dryness, zz Lower lid—involutional entropion
punctuate keratopathy due to blepharitis, zz Upper lid—cicatricial entropion.
meibomitis, trichiasis, foreign bodies, corneal
scarring (Fig. 2) and dry eyes. MANAGEMENT
zz Fluorescein staining: This test is essential when
looking for signs of corneal damage. It can
Nonsurgical Management
detect damage from lashes or lid skin rubbing In cases before proceeding to the definitive surgery
on the cornea. these medical management plans can be followed.
zz Schirmer test: To rule out other causes of zz Eyelid taping to the malar eminence
dryness. zz Injecting Botox into the orbicularis muscle.
Surgical Management • S
urgery (Hotz procedure): Minimal
ellipse of skin and orbicular is excised
Senile Entropion
from the medial two-thirds of the lower
zz Rotational sutures lid. Skin is fixed to the lower edge of the
zz Lateral tarsal strip tarsus.
zz Lower eyelid retractor reinsertion.
Q.2. Differentiate between congenital entro
Spastic Entropion pion and epiblepharon?
Ans. See Table 1.
First treat the underlying condition that might
infection or irritation of ocular surface. Followed Q.3. Involutional entropion.
by either rotational sutures can be passed or Botox Ans. • Often seen in elderly patients,particularly
can be injected. women.
• C auses great discomfort as well as
Cicatricial Entropion problems with clear vision due to
zz Tarsal fracture constant watering.
zz Transverse blepharotomy with marginal • The appropriate procedure depends on
rotation the degree of entropion, keratinization,
zz For severe cases, posterior lamellar lengthen and distortion of the lid margin and
ing using mucous membrane grafting. eyelashes and analysis of posterior
lamellar shortening and scarring.
Surgery Names • For cases with mild to moderate degree
of cicatrization—tarsal wedge resection
zz Weiss procedure (transverse blepharotomy
or the tarsal fracture procedure.
with everting sutures)
• In severe or recurrent cases: Posterior
zz Quickert everting sutures
lamellar grafting procedure to lengthen
zz Jones retractor plication.
the posterior lamella.
VIVA QUESTIONS Q.4. Causes of cicatricial entropion.

Ans. • Trachoma
Q.1. Congenital entropion. • Acid and alkali burn
Ans. • Extremely rare • Ocular pemphigus
• Inversion of entire tarsus and lid margin • Leprosy
• Epiblepharon and horizontal tarsal kink • Severe membranous conjunctivitis
are to be differentiated • Stevens-Johnson syndrome
Table 1 Differences between congenital entropion and epiblepharon

Features Epiblepharon Congenital entropion
Extrafold of skin Fold of skin overlapping the lid margin is Absent
present medially
Occurrence Common Rare
Lid affected Lower lid Both
Lid margin Not turned inwards Entire lid margin turned inward
Direction of eye lashes Straight up and lie flat against the cornea Turned inward
On pulling down the skin Lashes turn out but the margin of the lid Lid margin also pulls away from
remains in apposition to the globe the globe
Treatment Spontaneous resolution May require surgical correction
Oculoplasty 63
Q.5. Causes of spastic entropion. • D ose and procedure: 2.5 units of botu
Ans. • Senile linum toxin are injected at two or three
• Ocular surface disorder, e.g. dry eye places below the lower lid margin.
• Enophthalmos Injected directly into the muscle by
• Loss of orbital fat pinching it taking care not to go deep
• Tight bandage to avoid trauma to extraocular muscles
• Enucleation socket (especially medially as it may cause
Management of acute spastic entropion inferior oblique muscle paresis and lead
• Treatment of the underlying cause-break to diplopia).
the irritation-entropion cycle. • Disadvantage: May take around 4–7 days
• Taping of the inturned eyelid to evert the for its effect to appear. Short-term, not a
margin, various suture techniques afford permanent solution.
temporary relief for most patients. Q.7. The material of choice for the spacer graft
• A dditional definitive surgical repair for entropion correction.
to correct the underlying involutional Ans. • Hard palate mucosal graft
changes. • Donor sclera
• I n selected cases, botulinum toxin • Buccal mucous membrane
injection can be used to paralyze the • Amniotic membrane.
overriding preseptal orbicularis muscle.
Q.6. Role of botulinum toxin in management REFERENCES
of entropion
1. Richard R Tenzel. Orbit and Oculoplastics
Ans. • Works very well in correcting spastic Textbook of Ophthalmology. 1993;4:3.1-3.12.
entropion. 2. Charles K Beyer, Machule, Gunter K. Atlas of
• Also, in selected cases of involutional Ophthalmalmic Surgery. Von Noorden. First
entropion with significant preseptal Indian Edition. 1990;1:1.6-1.14.
muscle override when the patient is not 3. Albert and Jacobeic Principles and Practice of
willing for surgery or is bedridden and Ophthalmology. 2000;4:3491-2.
not fit for surgery.
BLEPHAROPHIMOSIS, PTOSIS, EPICANTHUS INVERSUS SYNDROME

Amar Pujari, Aditi Dubey
INTRODUCTION HISTORY
Chief Complaints
First described by Komoto in 1921, blepharo
phimosis-ptosis-epicanthus inversus syndrome The parents bring the child with anyone or a
(BPES) is a dominantly inherited disorder combination of the following complaints:
characterized by the presence of above features
zz Drooping of eyelid,
zz Abnormal head posture, (chin up due to ptosis)
(i.e. blepharophimosis, ptosis, and epicanthus)
zz Small size of eyeball
at birth. The main findings of this disorder are zz Abnormal eyelid
eyelids that are abnormally narrow horizontally zz Diminution of vision: Blurring of vision is
(blepharophimosis), a vertical fold of skin from the related to refractive error, astigmatism.
lower eyelid up either side of the nose (epicanthus zz Absence of eyeball
inversus), and drooping of the upper eyelids zz Bluish colored swelling (in case of crypto-
(ptosis). It can be given as a short case in the phthalmos)
examination. zz Epiphora (due to displaced tear ducts).
Past History refraction is a must in all such cases since the

significant refractive error may be seen in almost
A careful past history should be taken for any:
1/3rd of these cases and if untreated can lead to
zz Perinatal and pregnancy history
amblyopia. Amblyopia can also occur due to
zz Family history of congenital eyelid colobomas
stimulus deprivation (in a case of severe ptosis)
or other congenital anomalies, especially facial
but rare.
(e.g. cleft lip/palate)
zz History of other birth defects Head posture: To compensate for the ptosis,
zz Pediatric review of systems affected person assumes a characteristic posture
zz Facial asymmetry with the head tilted backward, the brow furrowed,
zz Hearing loss and the chin arched upward. Frontalis overaction
zz Recurrent infection may be there (eyebrows are increased in their
zz Menstrual history (in case of late presentation). vertical height and they are drawn up into a
pronounced convex arch).
Past Surgical History
Eyeball: The eyeball may show microphthalmos,
The previous history of ocular surgery (attempts to anophthalmos, cryptophthalmos. The palpebral
correct any of the lid deformity or squint) may or fissure is reduced in both horizontal and vertical
may not be present. dimension. The normal horizontal fissure length
in adults is 25–30 mm whereas in this syndrome
EXAMINATION it is usually 20–22 mm. Telecanthus is seen in
the majority of patients. This refers to a lateral
displacement of the inner canthi leading to
Features frequently observed in both BPES type I a widening of the intercanthal distance. The
and type II are a broad nasal bridge, low-set ears, interpupillary distance (IPD) is usually normal.
and a short philtrum. The patient may have esotropia, divergent
This condition is sometimes associated with strabismus or nystagmus.
ovarian failure although breast development is
often normal. Secondary sexual characteristics Eyelids: Eyelids are often covered by the smooth
are usually normal in both BPES type I and type II. skin without eyelid folds and deficient amounts
In BPES type I, menarche is usually normal, of skin in both eyelids may be found (Fig. 1).
followed by oligomenorrhea and secondary Frequently, the upper and lower lacrimal puncta
amenorrhea. are displaced laterally or duplication of puncta can
be seen.
Other malformations that can be seen includes
(also known as BPES Plus):
zz Contractures
zz Low nose bridge, micrognathia, microcephaly
zz Ear abnormalities: Incomplete ear develop
ment/Cupped ears, posteriorly rotated ears
zz Infertility in females, premature menopause,
primary gonadal failure
zz Reduced muscle tone—only early in life
zz Mental defects, severe psychomotor retarda
tion, growth retardation
zz Genitourinary malformations, Cryptorchi
dism, syndactyly.
Ocular Examination
Visual acuity: It is variably impaired depending Fig. 1: Blepharophimosis epicanthus
on the severity of ptosis, astigmatism. Cycloplegic inversus syndrome
Oculoplasty 65
Blepharoptosis literally means a falling of before age 40. Primary ovarian insufficiency can
the lids. The palpebral fissure is abnormally lead to difficulty conceiving a child (subfertility) or
small in the vertical dimension. It is caused by a complete inability to conceive (infertility).
the absence or impairment of the function of
the levator palpebrae superioris muscle and is BPES Type II
usually bilateral and symmetrical [Kindly see the Both affected females and males transmit this
examination part of ptosis in chapter ptosis for a variant. It is not associated with female infertility.
detailed examination of ptosis]. Both types are inherited as an autosomal
Dysplastic eyelids: Eyelids are often covered by dominant trait. There is complete penetrance
the smooth skin without eyelid folds and deficient (100%) in type I and slightly reduced (96.5%)
amounts of skin in both eyelids may be found. penetrance in type II. Both types I and II include the
The upper eyelid margin may show ‘S’ shaped eyelid malformations and other facial features.1-3
curve. The lower lid margin usually has an
abnormal concavity downwards, particularly DIFFERENTIAL DIAGNOSIS
laterally where an ectropion might occur. Trichiasis
Differential diagnosis includes those conditions
can also occur in BPES.
in which ptosis or blepharophimosis are a
Epicanthus inversus: A small skin fold that arises major feature (see Table 1 for features of these
from the lower lid and runs inwards and upwards syndromes)
characterizes it. Associated with this are an zz Congenital simple ptosis
increased length of the medial canthal ligament zz Ptosis with external ophthalmoplegia
and a lack of the normal depression seen at the zz Noonan syndrome
internal canthus. zz Marden-Walker syndrome
Conjunctiva: It is usually normal. zz Schwartz-Jampel syndrome
zz Dubowitz syndrome
Cornea: It may show micro cornea occasionally. zz Smith-Lemli-Opitz syndrome.
Corneal sensation should be checked.
The characteristic combination of signs usually
Lens/Sclera/Iris/Fundus: Usually normal. clinches the diagnosis.
INVESTIGATIONS MANAGEMENT
Diagnosis of the disease is straightforward based Management requires the input of specialists
on the clinical signs. Molecular genetic testing including a clinical geneticist, pediatric ophtha
and specific laboratory studies are indicated in lmologist, oculoplastic surgeon, (pediatric or adult)
cases of associated syndromes. A pelvic ultrasound endocrinologist, reproductive endocrinologist,
examination and measurement of bone mineral and gynecologist.
density are indicated if at ovarian insufficiency is Management of BPES is primarily surgical if
suspected. indicated. However, any refractive error must be
corrected to avoid amblyopia. The indication of
CLASSIFICATION surgery is moderate to severe ptosis, amblyopia,
trichiasis which may cause the corneal lesion,
Two types of BPES have been described: cosmesis, strabismus. Care should be given to treat
associated amblyopia.
BPES Type I The timing of eyelid surgery is controversial; it
It is the more common type, in which males involves weighing the balance of early surgery to
transmit the syndrome only and affected females prevent deprivation amblyopia and late surgery to
are infertile. It is associated with an early loss of allow for more reliable ptosis measurements, the
ovarian function (primary ovarian insufficiency) latter of which provides a better surgical outcome.
in women, which causes their menstrual periods Furthermore, ptosis surgery is hampered by the
to become less frequent and eventually stop dysplastic structure of the eyelids. The surgical
Table 1 Syndromes associated with BPES

Association Inheritance Features
Hereditary congenital AD Ptosis
ptosis 1 (PTOS1)
Hereditary congenital XL Ptosis
ptosis 2 (PTOS2)
Ohdo blepharophimosis AD Blepharophimosis, ptosis, mental retardation, congenital heart
syndrome defects, teeth abnormality (hypoplastic teeth)
3MC syndrome 1 (Michels AD Blepharophimosis, ptosis, epicanthus inversus, corneal
syndrome) abnormality, Cleft lip/palate, skeletal abnormalities
Ptosis with external AR Ptosis, ophthalmoplegia, miosis. Decreased accommodation
ophthalmoplegia
Noonan syndrome AD Ptosis, short stature, heart defects, clotting abnormalities
Marden-Walker syndrome AR Ptosis, blepharophimosis, growth retardation, mental retardation
Schwartz-Jampel AR Intermittent ptosis, blepharophimosis, telecanthus, cataract, short
syndrome stature, skeletal anomalies, muscle hypertrophy
Dubowitz syndrome AR Ptosis, blepharophimosis, lateral telecanthus, short stature,
intellectual disability, immunodeficiencies
Smith-Lemli-Opitz AR Ptosis, epicanthus, cataract, growth retardation, intellectual
syndrome disability, genitourinary, cardiac, gastrointestinal anomalies
KANSL1-related AR Developmental delay, intellectual disability, long face, high
intellectual disability forehead, ptosis, blepharophimosis, large low-set ears, bulbous
syndrome nasal tip, pear-shaped nose, cardiac septal defects, seizures,
cryptorchidism
Abbreviations: AD, autosomal dominant; AR, autosomal recessive; XL, X-linked; BPES, blepharophimosis-ptosis-
epicanthus inversus syndrome
management is traditionally performed in two

stages and involves a medial canthoplasty for
correction of the blepharophimosis, epicanthus
inversus, and telecanthus at about the age of
4–5 years and correction of the ptosis about
9–12 months later. Early surgery may be necessary
for amblyopia.
Epicanthus fold and telecanthus: The various
procedures for correction of epicanthus fold and
telecanthus includes: double Z or Y-Z plasties
(Fig. 2), Transnasal wiring of the medial canthal
tendons. If the epicanthal folds are small, a Y-V
canthoplasty is traditionally used; if the epican
thal folds are severe, a double Z-plasty is used. Fig. 2: Blepharophimosis, ptosis, epicanthus inversus
An alternate technique for medial canthoplasty syndrome (Figure 1 patient) after bilateral Y-V plasty
has been described recently using the skin and bilateral sling surgery
redraping method, which has a simple flap design,
Oculoplasty 67
less scarring, and the effective repair of epicanthus Q.2. When should the BPES be repaired?
inversus and telecanthus.3 Ans. See management part.
Ptosis: Generally, it is corrected with brow Q.3. What are the syndromes associated with
suspension procedure. Super-maximal resection ptosis?
and frontalis suspension is the preferred method Ans. See Table 1.
as it leads to a good cosmetic outcome as well as to
an improved muscle function. Q.4. Classify BPES.
Although traditional management of blepha Ans. Already given in classification.
rophimosis syndrome includes medial cantho Q.5. Genetics of BPES.
plasty between the ages of 3 and 5 years, followed
Ans. Both types are caused by mutations in the
by ptosis correction about 6 months later, patients
FOXL2 gene. The FOXL2 gene provides
with severe ptosis may need early surgery to prevent
instructions for making a protein that
amblyopia. Traditional multiple surgeries may
is active in the eyelids and ovaries. The
delay the amblyopia management and influence
FOXL2 protein is likely involved in the
the visual outcome. Thus, many surgeons suggest
development of muscles in the eyelids.
correction of ptosis first, even at a very early age,
to prevent amblyopia. Soft-tissue medial canthal
and lateral canthal surgery can wait until the face REFERENCES
is grown.3 Treatment of associated abnormalities: 1. Alao MJ, Lalèyè A, Lalya F, Hans Ch,
It includes management of ovarian failure, Abramovicz M, Morice-Picard F, Arveiler B,
hormone replacement therapy, and embryo Lacombe D, Rooryck C. Blepharophimosis,
cryopreservation. Management of amblyopia (i.e. ptosis, epicanthus inversus syndrome with
with/without spectacle wear/contact lens must translocation and deletion at chromosome
be continued after surgical intervention to obtain 3q23 in a black African female. Eur J Med Genet.
optimal results. 2012;55:630-4.
2. Batista F, Vaiman D, Dausset J, Fellous M, Veitia
Patient education: Genetic consultation is highly RA. Potential targets of FOXL2, a transcription
recommended, especially for patients with factor involved in craniofacial and follicular
associated syndromes. development, identified by transcriptomics.
Proc Natl Acad Sci USA. 2007;104:3330-5.
3. Beckingsale PS, Sullivan TJ, Wong VA, Oley
VIVA QUESTIONS C. Blepharophimosis: a recommendation
for early surgery in patients with severe
Q.1. What is the sequence of surgeries for ptosis. Clin Experiment Ophthalmol. 2003;31:
ptosis and epicanthus in BPES? 138-42.
Ans. See management part.
SEBACEOUS GLAND CARCINOMA

Amar Pujari, Sapna Raghuwanshi
INTRODUCTION usually occurs between fifth and ninth decades

of life, women’s are more affected than man.1-3
Sebaceous gland carcinoma (SGC) is third most It mostly involves the upper lid (Fig. 1).
common eyelid malignancy of the eyelids. Most In exams, it can be given as a long case or
sebaceous carcinomas arise from the meibomian short case.
glands, Zeiss gland, and Moll gland of the eyelid
zz Prolonged use of thiazide diuretics

zz Older age (5th to 9th decade, average age
60–69 years)
zz Female sex (55–57% cases are females).
EXAMINATION
Ocular Examination
Eyelids: Eyelid may have a nodule with following
features:1
zz Slowly enlarging, firm, and painless mass
affecting the tarsal plate or the eyelid
margin(Since the meibomian gland is buried
deep in the tarsus, initially the tumor will
Fig. 1: Sebaceous gland carcinoma form a firm mass, and may be misdiagnosed
as a chalazion. As the tumor invades more
superficially, a yellowish cast may be visualized
HISTORY
through the skin).
Kindly see the section of lid tumors also. zz Location
—— Upper lid: 60–70%, most common site,
Chief Complaint involved two to three times more fre

quently than the lower lid (Fig. 1). This is
Patient may present with the following complaint:
due to the presence of a greater number
zz Slowly enlarging, firm, and painless mass
of meibomian glands in the upper lid
or nodule at the lid margins (most common
(50 glands in upper eyelids, 25 in lower
presentation)
approximately).
zz Yellowish nodule at eyelid margin or caruncle
—— Caruncle: 5–11%
zz As chronic blepharoconjunctivitis (irritation,
—— Eyebrow: 2%
redness or foreign body sensation)
—— Simultaneous involvement of both lids:
zz Recurrent chalazion
6–8%
zz Chronic blepharitis with loss of cilia
zz Lesions may also exhibit varying degrees of
zz Skin or conjunctival ulcer
yellow coloration/yellowish cast due to the
zz Advanced cases may present as eyelid mass
presence of lipid within the mass.
with a destruction of marginal cilia and lid
zz Lesions originating from the Zeis glands
architecture
appear as small, yellowish nodules located at
zz Proptosis due to local invasion (anterior orbital
the eyelid margin anterior to the gray line. At
mass or lacrimal gland tumor).
times it may form a papilloma or cutaneous
horn.
Past History zz Tumors arising from sebaceous glands of the
A careful history about the onset, progression, caruncle usually appear as a subconjunctival,
association with pain must be recorded (similar multilobulated, yellow mass.
to that described under long case section for Lid zz Eyelids are diffusely thickened.
tumors) zz Skin appears indurated, usually skin is
The risk factors of sebaceous cell carcinoma movable on the mass until late stages
must be ruled out in history such as. zz Small telangiectasia’s over the mass
zz Recurrent chalazion zz Loss of eyelashes (Disruption of eyelid
zz Previous radiotherapy architecture and lash loss can occur as the
zz Chronic blepharoconjunctivitis tumor destroys lash bulbs)
zz Immunosuppression zz Rarely can present as anterior orbital mass or
zz Asian race lacrimal gland tumor.
Oculoplasty 69
Enlarged lymph nodes: Submandibular, sub conjunctiva. Map conjunctival biopsies were taken
mental, preauricular and cervical. in all four quadrants.1-3
Conjunctiva: Conjunctival inflammation, superior
MANAGEMENT
limbic keratoconjunctivitis can be seen.
zz Nodular sebaceous carcinoma without
Cornea: Superficial keratitis may be present if pagetoid involvement: Lesion can be removed
tumor cells invade corneal epithelium. by following techniques:
Other findings are usually within normal —— Full thickness eyelid resection with frozen
limits. section control of margins
—— Mohs’ micrographic technique: Excision of
DIFFERENTIAL DIAGNOSIS the visible tumor, with a 5-mm margin of

clinically normal tissue on either side.
The following disease must be differentiated from zz Nodular sebaceous carcinoma of one eyelid
the sebaceous cell carcinoma with evidence of pagetoid spread:
zz Blepharoconjunctivitis —— Excision of the nodular lesion with
zz Blepharitis conjunctive and superficial keratectomy/
zz Chalazion cryotherapy of the lesion.
zz Superior limbic keratoconjunctivitis —— Mitomycin C (MMC) can be tried to
zz Basal cell carcinoma control conjunctival tumor. MMC 0.4%
zz Squamous cell carcinoma. four times per day for a week and then
The characteristic features of sebaceous were medication free for 1 week; this
cell carcinoma that help in differentiating from cycle was repeated until resolution of
these lesions are yellowish discoloration and malignancy.
telangiectatic blood vessels on the surface.At —— If bulbar conjunctiva is involved exten
times, it is often difficult to differentiate, especially sively by tumor and reconstruction is not
in early cases and excisional biopsy is the best way possible: Exenteration is recommended
to arrive at a conclusion. —— If both upper and lower eyelids are
involved by tumor without an involvement

INVESTIGATION of the conjunctiva: Remove both eyelids
and reconstruct the defect.
A case of SGC needs following investigations. —— Concomitant involvement of eyelids and
Routine tests like: Complete blood count, renal conjunctiva: It requires exenteration.
and liver function tests. zz Orbital disease with no metastasis: Orbital
exenteration
Metastatic work-up: X-ray chest/USG abdomen/ zz Ocular disease with lymphatic metastasis:
CECT: Brain and orbit. Mass resection/exenteration, radical neck
FNAC: lymph node (lipid stains). dissection, and postoperative radiation
Conjunctival impression biopsy (for pagetoid zz Systemic chemotherapy may is required in the
spread). management of metastatic disease; however,
Map biopsy: Sebaceous gland carcinoma must be there is little in the literature on the efficacy
confirmed by a full-thickness wedge biopsy of the of postsurgical chemotherapy for metastatic
affected eyelid. Because of multicentric spread, disease.
multiple biopsy specimens should be taken from
the adjacent bulbar and palpebral conjunctiva PROGNOSIS
and the other ipsilateral eyelid to form a map zz Poor compared to BCC.
of the extent of tumor spread across an ocular zz Five year mortality
1-3
surface. After histologic confirmation of sebaceous —— Early disease: 15% (6–30%)
1-3
carcinoma, the surgeon must consider the extent —— Metastatic disease: 50–67%
of possible pagetoid involvement of the bulbar zz Five year recurrence rate: 9–36%1-3
VIVA QUESTIONS Risk factors for subclinical spread include:

• Duration of symptoms >6 months
Q.1. Histopathology. • Vascular and lymphatic infiltration
Ans. Dysplasia and anaplasia of the sebaceous • Orbital extension
lobules in the meibomian glands are seen • Poor tumor differentiation
in SGC, associated with the destruction of • Multicentric origin intraepithelial car
tarsal and adnexal tissues. As the neoplastic cinomatous changes of the conjunctiva,
nodule enlarges, it may erupt toward the cornea, or skin
eyelid skin to initiate the intraepidermal • Location in the upper eyelid.
growth phase, wherein the sebaceous cells
spread diffusely throughout the epidermis. Q.3. Prognostic factors.
This ‘pagetoid’ epidermal invasion is a Ans. • Site:
distinctive feature of sebaceous carcinomas. – Lower lid tumor (better) > Upper lid
Specific characteristics on histopathology tumor >Both upper and lower lids
• Stains with oil red O and Sudan IV (worst)
• Cytoplasm is frothy and vacuolated – Conjunctival tumor: Metastatic
• C ells are larger vesicular and have disease
prominent nucleoli. • Tumor size: Increased size, worse
Histopathologic subtypes: Lobular (lobules prognosis >10 mm
of sebaceous architecture), comedocarci • Delay in diagnosis: Delay >6 months, bad
noma (characterized by a central necrotic prognosis
core), papillary (papillary projections and • Histology:
areas of sebaceous differentiation), and – Vascular invasion
mixed (features of any subtype). It can – Lymphatic invasion
also be described as well differentiated, – Highly infiltrative pattern
moderately differentiated, and poorly – Poor differentiation
differentiated. – Pagetoid spread
– Multicentric origin
Q.2. Metastasis. – Orbital invasion.
Ans. An invasive, potentially lethal tumor,
SGC may cause an extensive local Q.4. What is Muir-Torre syndrome?
destruction of eyelid tissues. It carries Ans. • It is an autosomal dominant condition
a risk of metastasis to preauricular and in which there are sebaceous (oil gland)
submandibular lymph nodes or may skin tumors in association with internal
spread hematogenous to distant sites. It cancer.
may invade locally into the globe, the orbit, • The most common organ involved is the
the sinuses, or the brain. The frequencies of gastrointestinal tract, with almost one-
the spreads are:1-3 half of the patients having colorectal
• Direct extension: Orbit, lacrimal glands— cancer. The second most common site is
6–17% cancer of the genitourinary tract.
• Lymphatic: Regional nodes—17–28% • Following skin lesions are associated
• Hematogenous: Rare, <1%, lungs, liver, with this syndrome
skull, and brain – Sebaceous adenomas
Pagetoid spread: Intraepithelial or intra – Sebaceous epitheliomas
epidermal spread of malignant cells similar – Sebaceous carcinoma
to that observed in Paget’s disease of the – Keratoacanthoma
nipple or extramammary Paget’s disease. – Squamous cell carcinoma
Spread along skin or conjunctiva, producing – Multiple follicular cysts
individual cell clusters are characteristic. • It is now thought to be a hereditary non
It is seen in almost 47% of the cases. polyposis colorectal cancer syndrome
Oculoplasty 71
due to mutations in the DNA mismatch REFERENCES

repair genes MSH2 or MLH1.
1. Wali UK, Al-Mujaini A. Sebaceous gland
Q.5. Sebaceous carcinoma in systemic carcinoma of the eyelid. Oman J Ophthalmol.
disease. 2010;3(3):117-21.
Ans. Sebaceous gland carcinoma is seen in 2. Mulay K, Aggarwal E, White VA. Periocular
association with following: sebaceous gland carcinoma: A comprehensive
• Familial retinoblastoma after radio- review. Saudi J Ophthalmol. 2013;27(3):159-65.
therapy 3. Albert DM, Miller JW, Azar DT. Albert
• Immunosuppression in HIV disease & Jakobiec’s principles and practice of
• Muir–Torre syndrome. ophthalmology; 2008.
PYOGENIC GRANULOMA
Sapna Raghuwanshi, Bijnya Birajita Panda
INTRODUCTION1,2 EXAMINATION
Pyogenic granuloma is an inflammatory vascular Eyelids: Raised, red, smooth surfaced lesions with
response of the tissue that usually occurs after a a narrow base (Fig. 1).
previous insult, typically either inflammatory or
Conjunctiva: If conjunctiva is involved con
trauma. It is the most common acquired vascular
junctival inflammation may be present (Fig. 1).
lesion to involve the eyelids. It also involves the
Other findings are usually within normal
conjunctiva. The name is a misnomer because this
limits.
lesion is neither pyogenic nor granulomatous.
In exams, it can be given as a short case.
DIFFERENTIAL DIAGNOSIS
HISTORY zz Kaposi’s sarcoma—slow growing, in immuno
compromised patient in contrast to fast
Chief Complaints growing pyogenic granuloma and associated
The patient may present with following: risk factors
zz Rapidly growing mass over eyelids and
conjunctiva
zz Lesion readily bleeds with minor contact
zz Pain (associated with superficial ulceration).
Past History
Following points must be noted in history
zz Minor trauma
zz Surgery (Limbal surgery for pterygium,
squamous cell carcinoma, phthisis, squint
surgery)
zz Chalazion
zz Microbial infection
zz Pterygium
zz Chemical burns. Fig. 1: Pyogenic granuloma
zz Intravascular papillary endothelial hyperplasia zz Topical or intralesional corticosteroids: It is

zz Squamous papilloma better to give a short course of steroid therapy
zz Conjunctival lymphoma before proceeding with surgery.
zz Ocular lymphangiectasia.
REFERENCES
HISTOPATHOLOGY
1. Brad Bowling. Kanski’s Clinical ophthalmology:
Lesion consists of a mass of granulation tissue, A systematic approach, 8th edn. Edinburgh:
prominent capillaries, and acute and chronic Elsevier, 2015.
inflammatory cells. 2. Albert DM, Miller JW, Azar DT. Albert
& Jakobiec’s principles and practice of
MANAGEMENT ophthalmology; 2008.
zz Surgical excision
LAGOPHTHALMOS
Varsha Varshney, Ritu Nagpal
INTRODUCTION
Lagophthalmos is a condition in which the eyelids
do not close to cover the eye completely. The term
lagophthalmos actually comes from the Greek
word for hare (lagoos) and derives from a myth
that hares sleep with their eyes open.1,2 In exams,
it can be given as a short or spot case. A normal,
healthy eye is covered by a film of tears that
protects the surface and washes away dust and
particles. Dry eyes that result from lagophthalmos
are not only uncomfortable but are also subject
to injury or infection from foreign objects landing
in and abrading the eye surface. Left untreated,
lagophthalmos can lead to permanent loss of vision. Fig. 1: Normal lid opening
HISTORY
Chief Complaints
Lagophthalmos patients commonly complain of
inability to close lids completely (Figs 1 and 2).
Associated features are:
zz Foreign body sensation
zz Increased tearing
zz Photophobia
zz Pain may be worse in the morning due to
increased corneal exposure and dryness
during sleep
zz Blurry vision, which results from unstable tear
film Fig. 2: Lagophthalmos on lid closure in a case (Figure 1)
Oculoplasty 73
zz In cases of advanced keratopathy and corneal zz Bell’s phenomenon.

ulceration, the symptoms and presentation zz Scar marks of previous trauma or surgery or
may be severe. infections may be present.
Conjunctiva: Diffuse or ciliary congestion may
History of Past Illness be there in presence of dry eye and exposure
zz The recent history of trauma or surgery keratopathy.
involving the head, face or eyelids should be Cornea: Following points must be recorded:
documented with special attention to fractures zz Corneal sensitivity by applying a wisp soft
to the skull base (a petrous portion of the cotton to the unanesthetized cornea and
temporal bone) or mandible that could affect comparing the blink reaction with that of the
facial nerve. fellow eye.
zz Past infections should be reviewed, with zz Fluorescein staining with cobalt blue filter to
particular attention to any history of herpes find out the presence of punctate epithelial
zoster infection. erosions or abrasions. Pay particular attention
zz It is also important to document any past to the inferior cornea where lid excursion ends.
symptoms suggestive of thyroid disease zz Tear breakup time.
or obstructive sleep apnea (Floppy eyelid zz Any epithelial defects or corneal ulcers should
syndrome). be carefully documented.
zz Schirmer test: The Schirmer test is used to
History of Systemic Illness assess tearing function. The degree of tearing
History of systemic diseases like diabetes can be compared between the paralyzed and
(diabetic neuropathy), any neurological disorder normal sides.
(polio, Guillain-Barré syndrome, leprosy), Pupils: Pupillary reflexes are usually normal
cerebrovascular accidents. unless other cranial nerves are involved.
Anterior segment: Normal chamber depth and
EXAMINATION contents.
Systemic Examination Posterior segment: Slit-lamp biomicroscopic
examination using a 90D/78D lens and indirect
Complete neurological examination should be
ophthalmoscopy—usually normal.
performed.
Ocular Examination INVESTIGATIONS

Visual acuity: Usually, normal. The blurring of Blood Investigations
vision may be present due to tearing or dryness. To rule out any systemic disease or infection
Eyeball: Usually, normal. following tests can be performed:
Lids: Ask the patient to look down and gently
zz CBC, blood sugar
close both eyes. Lagophthalmos is present when
zz Thyroid function test
space remains between the upper and lower eyelid
zz HIV ELISA/western blot
margins in extreme downgaze. Document the
zz VDRL/RPR
degree of lagophthalmos by measuring this space, The tests advised must be based on the history
in millimeters, with a ruler. In addition, following and clinical findings.
points must be recorded carefully.
zz Record the blink rate as well as the complete Radiological Investigations
ness of the blink. Preferably gadolinium-enhanced MRI: To rule out
zz Carefully test ocular motility and the strength any neurological causes such as fracture damaging
of the orbicularis oculi muscle. The latter can the nerve, mass compressing the nerve, ischemic
be assessed by evaluating the force generated areas involving facial nerve origin (geniculate
on attempted eyelid closure. ganglion).
Conduction Testing and Electromyography arriving at a diagnosis. Following points must be

kept in mind:
The tests are most useful when performed
zz Most common cause is Bell’s palsy.
3–10 days after the onset of paralysis. Comparison
zz If another cranial nerve, motor, or sensory
to the contralateral side helps to demonstrate
symptoms are present, then other neurologic
the extent of nerve injury and has prognostic
diseases should be considered (e.g. stroke,
implications. Nerve conduction responses are
Guillain-Barré syndrome, basilar meningitis,
abnormal if a difference of 50% in amplitude
cerebellar pontine angle tumor).
between the paralyzed and normal side is detected;
zz Symptoms associated with seventh nerve
a difference of 90% between the 2 sides suggests a
neoplasm include slowly progressive paralysis,
poorer prognosis.
facial hyperkinesis, severe pain, recurrent
Electroneurography palsy, and other cranial nerve involvement.
zz Cerebellopontine tumors may affect the
It is a physiologic test that objectively measure seventh, eighth, and fifth cranial nerves
the difference between potentials generated simultaneously.
by the facial musculature on both sides of the zz Patients with a progressive paralysis of the
face in response to a supramaximal electrical facial nerve lasting longer than 3 weeks should
stimulation of the facial nerve. be evaluated for neoplasm.
zz Recurrent ipsilateral facial paralysis must raise
Electrodiagnostic Testing the suspicion of a tumor of the facial nerve or
Measures the facial nerve degeneration indirectly. parotid gland. Tumors in the temporal bone,
If a patient does not reach 90% degeneration within such as facial nerve neuromas, meningiomas,
the first 3 weeks of the onset of paralysis, some hemangiomas, and malignant primary and
studies suggest that the prognosis is excellent, metastatic lesions, should be considered as
with over 80–100% of the patients recovering with well.
excellent function. zz If a patient reports the sudden onset of hearing
loss and severe pain with the onset of facial
Brainstem Auditory Evoked Response (BAER) paralysis, Ramsay Hunt syndrome must be
It may be obtained in patients with peripheral considered. Typically, these patients will also
facial nerve lesions and other neurologic have an erythematous vesicular rash involving
involvement. This test measures the transmission the ear canal, auricle, and/or oropharynx.
of response through the brainstem and is effective Bilateral cases: Bilateral simultaneous Bell palsy is
in detecting, notably, retrocochlear lesions. a rare (<1% of that of unilateral facial nerve palsy).
Examples include Guillain-Barré syndrome,
Blepharokymographic Analysis sarcoidosis, Lyme disease, meningitis (neoplastic
A high-speed eyelid motion-analysis system, has or infectious), or bilateral neurofibromas (in
been used to evaluate movement of the eyelids. patients with neurofibromatosis type 2).
The computer-based analysis may prove helpful
in diagnosing Bell palsy, predicting prognosis, TREATMENT
and evaluating response to therapeutic measures
such as placement of a gold weight in the affected Medical Treatment and Supportive Care for
upper eyelid (used in cases in which spontaneous Corneal Exposure
recovery has been limited). Nonpreserved artificial tears should be
All these investigations must be conducted in administered frequently (at least four times per
consultation with a neurologist. day) in order to supplement the patient’s tear
film. Ointments can be applied to the cornea
DIFFERENTIAL DIAGNOSIS once at bedtime or throughout the day in
Lagophthalmos can be due to a variety of causes, cases of severe corneal exposure. Prophylactic
careful history and neuroimaging often help in antibiotics (preferably nonepitheliotoxic such
Oculoplasty 75
as chloramphenicol 0.3%) can be added to the as well as migration and/or extrusion of the gold
regimen. In addition, following measures can be weight, are its limitations. In cases of allergy to
used: gold, platinum may be used.
zz Moisture goggles also may be used.
zz Punctal plugs may be helpful if dryness of the Upper Eyelid Retraction and
cornea is a persistent problem. Levator Recession
zz Infectious corneal ulcers should be treated
with appropriate antibiotic therapy. The recession of the upper eyelid retractors (levator
zz Patching the eye in the night time with simple and Müller’s muscles) is a useful procedure in
micropore or a Frost suture for temporary patients with lagophthalmos related to upper
protection of the cornea can also be helpful. eyelid retraction from thyroid ophthalmopathy.
zz Botulinum toxin can be injected trans Also, a combination of full-thickness skin grafts,
cutaneously or subconjunctivally at the upper advancement flaps, tarsal-sharing procedures
border of the tarsus to paralyze the levator and release of scar bands can be performed on
muscle to produce complete ptosis and to patients with lagophthalmos from cicatricial or
protect the cornea. postsurgical lid shortening.
Tarsorrhaphy Lower Eyelid Tightening and Elevation

Laxity of the lower eyelid may occur in conditions
A temporary tarsorrhaphy performed if recovery
such as facial nerve palsy and floppy eyelid
of the eyelid closure is expected within a few
syndrome. A tightening procedure such as a lateral
weeks. In most cases, the cornea can be protected
tarsal strip will improve apposition of the lower
adequately by suturing the lateral one-third of
eyelid to the globe and decrease tearing. This is also
the eyelids together. Ideally, a small opening
helpful in cases where upper eyelid restructuring
remains so that the patient can retain useful vision,
procedures fail.
the health of the cornea may be assessed and
lubrication or antibiotic therapy can be applied to
the eye. Other Surgical Procedures
A permanent tarsorrhaphy performed if a In cases of severe lagophthalmos various other
protracted clinical course is expected. If the patient procedures have been described such as elevation
regains useful function of the orbicularis oculi of the midface using a variety of materials
muscle, the adhesions can be lysed. The limitation such as autogenous fascia slings, temporalis
of tarsorrhaphy is poor cosmetic appearance. muscle transposition/transfer, nerve grafts and
anastomoses, palpebral springs, soft tissue
Gold Weight Implantation repositioning and suborbicularis oculi fat lifts.
Gold weights can be implanted into the upper

eyelid to treat paralytic lagophthalmos. It enhances VIVA QUESTIONS
eyelid closure in a gravity-dependent fashion.
Gold is considered an ideal substance because it Q.1. What are the ocular symptoms and signs
is inert and it is not visible through the thin skin of Bell’s palsy?
of the eyelid. Gold weights range from 0.6 to 1.6 g Ans. Bell palsy is the most common cause
(in 0.2-g increments). The appropriate weight is of uni l ateral facial paralysis. Ocular
chosen preoperatively by taping weights of varying manifestations have been described in
sizes onto the external lid above the tarsus and Table 1. Two-thirds of patients complain
observing the closing and opening of the lids. about epiphora which is due to punctal
Properly chosen, the ideal weight will allow full eversion and the reduced function of the
closing and the opening of the lids while avoiding orbicularis oculi in transporting the tears
ptosis in primary gaze. Gold weight implantation (fewer tears arrive at the lacrimal sac, and
is usually well-tolerated. However, astigmatic shift, overflow occurs).
Table 1 Ocular manifestations of Bell’s palsy Q.3. Relevant anatomy of facial nerve and
eyelid.
Early Late
Ans. Facial nerve: The facial nerve (seventh
• Lagophthalmos • Narrow palpebral fissure-
cranial nerve) innervates both the frontalis
• Paralytic generalized mass contracture
muscle, which raises the eyebrow and the
ectropion of the of the facial muscles(after
lower lid several months) orbicularis oculi muscle, which closes the
• Tear overflow • Aberrant regeneration of eyelids. In addition, the 7th nerve innervates
• Brow ptosis the facial nerve with motor the muscles of the facial expression such
• Upper eyelid synkinesis zygomaticus muscles, which elevate the
retraction • Reversed jaw winking- cheeks as well as the corrugator supercilii
• Dry eye—poor twitching of the corner and procerus muscles, which depress the
tear distribution of the mouth or dimpling eyebrow.
• Corneal of the chin occurring Eyelids: The upper and lower eyelids
exposure, simultaneously with each
contain seven structural layers. Beginning
erosion, blink
anteriorly, these comprise (1) skin and
infection, and • Crocodile tears—tearing with
ulceration (rare) chewing subcutaneous tissue, (2) orbicularis oculi
muscle, (3) orbital septum, (4) orbital fat,
Q.2. Discuss etiology of lagophthalmos. (5) muscles of retraction, (6) tarsus and (7)
Ans. Lagophthalmos can occur due to a patho conjunctiva. Damage to or degeneration of
logy in the facial nerve or in the lid. The any of these tissues may inhibit good eyelid
different causes are summarized in Table 2. closure.
Table 2 Causes of lagophthalmos

Pathology Etiology Factors/mechanism
Facial Trauma Fractures to the skull base (petrous portion of the temporal bone) or
nerve mandible
Neurosurgical procedures
Bell’s palsy Acute viral infection or reactivation of herpes simplex virus
Tumors Acoustic neuromas in the cerebellopontine angle
Metastatic lesions
Cerebrovascular Blockage of anterior inferior cerebellar artery
accidents
Infectious, immune- Lyme disease, chickenpox, mumps, polio, Guillain-Barré syndrome, leprosy,
mediated causes diphtheria and botulism
Mobius’ syndrome Characterized by cranial nerve palsies (sixth and seventh cranial nerve),
motility disturbances, limb anomalies and orofacial defects
Eyelids Cicatrices Chemical or thermal burns, ocular cicatricial pemphigoid, Stevens-
Johnson syndrome, mechanical trauma
Eyelid surgery Excessive removal of eyelid skin or muscle; blepharoplasty, tumor excision
Overcorrection in ptosis repair
Proptosis Exophthalmos of one or both globes may inhibit eyelid closure
Enophthalmos Posterior displacement of the eye may affect eyelid apposition and closure
Causes include orbital blowout fractures; orbital fat atrophy (trauma,
infection, inflammation, aging, scleroderma, HIV-AIDS); phthisical eye;
scirrhous carcinomas
Floppy eyelid Severe laxity and flexibility of the superior and inferior tarsal plates
syndrome
Oculoplasty 77
Q.4. Discuss treatment options for Bell’s

Surgical options: Surgical options for Bell
palsy? palsy include the following: Facial nerve
Ans. Treatment options for Bell’s palsy includes decompression, subocularis oculi fat
following: (SOOF) lift, Implantable devices (e.g. gold
Pharmacologic therapy: The most widely weights) placed into the eyelid, tarsor
accepted treatment for Bell palsy is cortico rhaphy, transposition of the temporalis
steroid therapy. However, the use of muscle, facial nerve grafting, direct brow
steroids is still controversial because most lift.
patients recover without treatment. The
recommended dose of prednisone for the
treatment of Bell palsy is 1 mg/kg or 60 mg/ REFERENCES
day for 6 days, followed by a taper, for a total 1. Pereira MV, Glória AL. Lagophthalmos. Semin
of 10 days. Ophthalmol. 2010;25(3):72-8.
Antiviral agents: Such as Acyclovir (Zovirax) 2. Vásquez LM, Medel R. Lagophthalmos after
and Valacyclovir (Valtrex) have shown facial palsy: current therapeutic options.
limited benefit. Ophthalmic Res. 2014;52(4):165-9.
DERMOID CYST
Shipra Singhi, Deepali Singhal
INTRODUCTION zz Diminution of vision: Blurring of vision is

related to size and nerve compression and
A dermoid cyst (epidermal dermoid cyst) is an the presence of complications. It is usually
epithelial-lined structure with dermal appendages progressive and painless.
in its wall and keratin and hair in its lumen. It can zz Sometimes patient may present with
be found in any subcutaneous location but more Symptoms consist of the rapid onset of
than 80% are located in the region of the head, with unilateral pain, redness and watery discharge
the majority in the eyelid and orbital area, usually due to a ruptured cyst.
superotemporally near the zygomaticofrontal zz Intermittent increase in size during chewing
suture. indicated extension to temporalis muscle
It can be given as a short case or spot case in of deep dermoid. However this mode of
exam. presentation is very rare.
In adults, dermoids may become symptomatic
HISTORY for the first time and grow considerably over a
Chief Complaint year. Based on this fact, some conclude that these
lesions may be dormant for many years or have
The presenting feature depends upon the location
intermittent growth.
of dermoid. It can present in following manner:
zz Painless fullness of upper eyelid or a mass
lesion, most commonly at lateral orbital rim History of Present Illness
can be the presenting feature in an Anteriorly The onset, progression, association with pain and
located dermoid. any preceding events such as trauma must be
zz A posteriorly located dermoid can present with noted. Dermoid cyst usually has a insidious onset,
Painless, progressive proptosis, and diplopia. painless and progresses slowly over a period of
zz Rarely there can be associated ptosis and years. Rapid onset of unilateral pain, redness and
limitation of the movement. watery discharge suggests a ruptured cyst.
Past History —— Occasionally, subconjunctival droplets of

fat are seen. In some cases, a secondary
A careful past history should be taken of any mass
fistula between the cyst and the skin may
(tumor), nerve paresis, infection, trauma and
allow the contents of the cyst to drain
ocular inflammatory diseases.
intermittently.
Past Surgical History zz Rarely the dermoid is incompletely separated
from the skin surface and presents as a
Previous history of any intraocular surgery should chronically inflamed and discharging sinus.
be enquired. zz Dermoid cyst can also be associated with
motility deficits and diplopia when the extent
EXAMINATION is large.
Ocular examination: Ocular examination should Eyelids: When dermoid is located anteriorly it can
include following: lead to ptosis.
Visual acuity: Visual acuity is variably impaired Anterior segment: Generally normal. Occasionally,
depending on the site of involvement, size subconjunctival droplets of fat are seen in case of
and compression of nerve and the presence of rupture of cyst.
complications. Tonometry: IOP is usually unaffected.
Eyeball Fundus: There may be compressive neuropathy
zz Proptosis is usually nonaxial. Extension into or choroidal folds especially in cases of posterior
intracranial fossae is possible if the frontal or dermoid.
sphenoid bones are involved. Temporal fossa
involvement is rare but reported; this may DIFFERENTIAL DIAGNOSIS
result in intermittent proptosis associated
with chewing, as positional changes of the Differential diagnosis depends upon the location
temporalis muscle during chewing transmit of the dermoid:
pressure to the lesion and, hence, to the zz Lateral anterior dermoid
—— Lacrimal gland mass
orbit.
—— Lipodermoid
zz Mass: A mass lesion due to dermoid cysts have
—— Teratoma
following characteristics:
—— Plexiform neurofibroma
—— Firm in consistency
—— Margins are smooth

zz Medial anterior dermoid
—— Mucocele
—— Non-tender
—— Encephalocele
—— Mobile preseptal masses without fixity to
skin or muscle zz Cyst with spontaneous rupture

—— Orbital cellulitis
—— Superotemporal quadrant is the most
—— Orbital pseudotumor
common site, less commonly, the supero-
nasal quadrant is affected.
zz Deep dermoid with mass effect
—— Orbital tumors
—— Many of them have variable periosteal
—— Thyroid ophthalmopathy.
attachment near the underlying frontozy
gomatic or frontoethmoidal sutures.
—— Occasionally the dermoid will pass into or
INVESTIGATION
through defects in the neighboring bone Classic dermoid cysts located at the frontozy
and may communicate intracranially. gomatic suture whose posterior aspect can be
—— A dermoid cyst can rupture spontane palpated may be diagnosed clinically without
ously or with trauma, inciting an intense imaging. Medial lesions require imaging to rule
inflammatory response in the orbital soft out an encephalocele or mucocele before surgical
tissues. This response may be limited to excision. Deep orbital lesions also require imaging
injection of the conjunctiva or may be for diagnostic purposes and to help with surgical
severe and mimic orbital cellulitis. planning.
Oculoplasty 79
CT Scan iatrogenic rupture. Leakage of the cystic contents

into the orbit can result in significant inflammation
A dermoid cyst typically has a hyperdense wall and and recurrence, while lesions removed in their
a hypodense cavity which remains non-enhancing entirety rarely recur.
with contrast. The central cavity may appear The surgical approach depends upon
heterogeneous as a result of keratin and other the location of the lesion. An anterior orbital
cystic debris. CT imaging is especially useful in epidermal dermoid cyst can be removed through
delineating bony changes such as smooth pressure anterior orbitotomy (superior eyelid crease
erosion (scalloped) near the affected suture, clefts, incision). A posterior orbital epidermal dermoid
and full-thickness bony channels, seen in as much cyst can be removed through lateral orbitotomy.
as 85% of cases. A dumbbell cyst have a typical The use of a cryo-probe can help in the delivery of
appearance with a component on either side of the cyst intact in these cases. Deeper lesions are
the bone and a bony communication between approached based upon their location in the orbit
them. and relationship to adjacent structures. Intracranial
extension requires a multi-disciplinary surgical
MRI approach for complete excision.
Great care should be taken to remove the cyst
The lesions are generally hypointense on
with the capsule intact, using meticulous dissection
T1-weighted imaging with respect to fat and are
at the site of the attachment of the cyst to the bony
best visualized using fat-suppression techniques. It
sutures. If the cyst is accidentally ruptured at the
appears as a well defined, round to ovoid structure
time of surgery, copious irrigation and attempted
of variable size. The lesions tend to be hyperintense
removal of the cyst remnants should be done.
on T2-weighted imaging. These lesions typically
In the rare case of dermoid cyst at the orbital
do not enhance with contrast due to lack of blood
apex, an orbital deroofing procedure may be
vessels in the cyst. MRI has the advantage of not
necessary. If the cyst is too large to remove intact,
exposing the patient to radiation, hence extremely
its contents can be aspirated in order to facilitate
useful in pediatric age group.
removal. Recurrence can develop after incomplete
excision.
Ultrasonography (USG)
In ruptured cyst:
Ultrasound characteristics of dermoid cysts zz Systemic steroid therapy
include a smooth contour and variable echo- zz Systemic NSAID therapy (aspirin, ibuprofen).
genicity.
CLASSIFICATION
Color Doppler imaging 1. Epidermal dermoid cyst—anterior or deep
Color Doppler imaging of dermoid cysts shows 2. Conjuctival dermoid cyst.
no intralesional blood flow, which can help
differentiate them from hemangioma and rhabdo- Conjunctival Dermoid Cyst
myosarcoma.
Occasionally, an otherwise typical dermoid
cyst is lined by nonkeratinizing epithelium with
MANAGEMENT features of conjunctival epithelium. This is called
a conjunctival dermoid cyst.
A small, asymptomatic orbital epidermal dermoid
cyst requires no immediate treatment. In many
Incidence
cases, however, the cyst slowly enlarges or ruptures,
and eventually requires treatment. The treatment A conjunctival dermoid cyst is lined by conjunctival
of orbital dermoid cysts is surgical excision. The epithelium. It accounts for about 5% of dermoid
primary goal of excision is to remove the dermoid cysts that occur in the orbit, with the other 95%
with the cyst wall intact without causing an being of epidermal origin.
Clinical Features Q.2. Compare epidermal and conjunctival

dermoid cyst?
Conjunctival dermoid cyst is probably congenital
Ans.
but often it is not evident until childhood or
sometimes later in life. Epidermal dermoid Conjunctival dermoid
It occurs in the superonasal aspect of the
• Age of onset: Anterior • Age of onset: Deeper
orbit usually and presents as firm or fluctuant
dermoids typically dermoids may present
subcutaneous mass. present in first decade in adolescence or
• It accounts for about adulthood
Investigation 95% of dermoid cyst • It accounts for about
With CT or MRI, a conjunctival dermoid cyst has that occur in the orbit 5% of dermoid cysts
that occur in the orbit
features similar to an epidermal dermoid cyst.
However, it is more likely to be situated in the • Histopathology: • Histopathology
orbital soft tissues in the anterior and nasal aspect Dermoid cyst is • Histopathologically,
of the orbit, usually without contact to bone. lined by keratinizing conjunctival dermoid
stratified epithelium. cyst is lined by non
• It contains dermal keratinizing epithe
Histopathology appendages such as lium which contains
Histopathologically, conjunctival dermoid cyst hair shafts, sebaceous goblet cells. Like the
is lined by nonkeratinizing epithelium which gland, and occasional epidermoid dermoid
contains goblet cells. Like the epidermoid dermoid sweat glands cyst, it contains dermal
cyst, it contains dermal appendages such as hair appendages such as
hair shafts, sebaceous
shafts, sebaceous gland, and occasional sweat
gland, and occasional
glands.
sweat glands
• Site: Superotemporal • S ite: Superonasal
Management
usually usually
A conjunctival dermoid cyst is usually symptomatic • Imaging: With CT or
• I maging: On CT, a
when diagnosed and is best managed by surgical dermoid typically has MRI, a conjunctival
excision. a hyperdense wall dermoid cyst has
Either a conjunctival or skin incision supero and a hypodense features similar to an
nasally is generally used because it is usually cavity which remains epidermal dermoid
located superonasally in the anterior orbit, non-enhancing with cyst. However, it
In a skin approach , an eyelid crease incision in contrast. The central is more likely to
the upper eyelid is recommended. cavity may appear be situated in the
heterogeneous as a orbital soft tissues
result of keratin and in the anterior and
VIVA QUESTIONS other cystic debris. nasal aspect of the
An estimated 85% of orbit, usually without
Q.1. What is the epidemiology of dermoid? dermoids are associated contact to bone
Ans. Epidemiology: with such bony
• Congenital choriostoma changes as smooth
• Account for 3–8% of orbital tumors in pressure erosion near
children the affected suture,
• The dermoid cyst becomes the most clefts, and full-thickness
bony channels
common noninflammatory space-
• On MRI: Hypointense on
occupying lesion of the orbit
T1-weighted imaging;
• In the Wills Eye Hospital pathology hyperintense on T2-
series, dermoid cyst accounted for 46% weighted imaging
of childhood orbital lesions and for 89%
of all cystic lesions. Contd…
Oculoplasty 81
Contd… Q.6. What is the most common location?

Ans. Children
• Treatment: Anterior • T reatment: Anterior
orbitotomy (superior orbitotomy— • The most common location is in the
eyelid crease incision) conjunctival or skin superior temporal aspect of the orbit. The
incision superonasally second most common location is in the
• In a skin approach, an superior nasal aspect of the orbit.
eyelid crease incision • L esions located superotemporally are
in the upper eyelid is generally smooth, firm subcutaneous
recommended masses attached to the orbital rim in the
region of the zygomaticofrontal suture.
Q.3. Differentiate between anterior and deep • The mass is generally less than 1 cm in
dermoid cyst. diameter, nontender, and oval in shape.
Ans. Little displacement of the globe usually
occurs.
Anterior dermoid Deep dermoid • Orbital dermoid cysts are not attached to
• A
ge of onset: Anterior • A
ge of onset: Deeper the skin, which helps differentiate them
dermoids typically dermoids may present from sebaceous cysts. The cyst usually is
present in first decade in adolescence or tethered to the periosteum of the bone
adulthood near suture lines, including the sinuses
• Suture involved: • S uture involved: or intracranial cavity.
– Lateral: Frontozygo Sphenozygomatic Adults
matic suture or sphenoethmoidal • The cysts are palpated less easily and
– Medial: Frontoeth suture have more vague borders. They are more
moidal or likely to displace the globe and may
frontolacrimal sutures erode their way into adjacent structures.
• S ymptoms: Painless • S ymptoms: Painless, • Dystopia: A larger dermoid cyst can cause
fullness of upper progressive proptosis, downward and medial displacement of
eyelid, most diplopia the globe.
commonly at lateral • Motility deficits
orbital rim • Diplopia
• S igns: Subcutaneous, • S igns: Proptosis, Anterior lesions: typically present in the
mobile nodule, most motility deficit, first few years of life as smooth, well-
commonly located inferior or superior circumscribed, subcutaneous, painless
at frontozygomatic displacement of globe masses.
suture • Site: The most common location for
• T reatment: Anterior • T reatment: Lateral the anterior dermoid cyst is at the
orbitotomy (superior orbitotomy superolateral aspect of the orbit at the
eyelid crease incision) frontozygomatic suture, as seen in the
case described here. Medial lesions occur
Q.4. What is choriostoma? less frequently and often arise from tissue
Ans. Choristoma is a mass of histologically sequestered in the frontoethmoidal or
normal tissue present at abnormal location. frontolacrimal sutures. If there is no
orbital extension, the posterior aspect of
Q.5. What is difference between choriostoma the mass may be palpable.
and teratoma? • P tosis: Because of their anterior location,
Ans. While choristoma is a mass of histologi these lesions do not usually cause globe
cally normal tissue present at abnormal displacement, but they can cause visually
location, teratoma is a mass of neoplastic significant ptosis if they grow to a large
tissue at abnormal location. enough size.
Deep lesions are more insidious, and inflammatory response in the orbital soft
• Site: often develop at the sphenozygo tissues. This response may be limited to
matic or sphenoethmoidal suture. injection of the conjunctiva or may be
• P roptosis: Their presence is usually severe and mimic orbital cellulitis.
declared by mass effect on surrounding • Occasionally, subconjunctival droplets
structures: Patients with deep lesions of fat are seen. In some cases, a secondary
may present in late adolescence or fistula between the cyst and the skin may
adulthood with painless, progressive allow the contents of the cyst to drain
proptosis, intermittently.
• D umbbell dermoids: Dermoids may • While this is rarely the first presenting
also straddle the orbital bones (most sign for an anterior dermoid, it may
commonly the lateral orbital wall) be the first presenting sign of a deep
such that they have both an anterior dermoid.
lobe and a deeper orbital lobe. These Complications
so-called “dumbbell” dermoids must be • Rupture of the cyst
imaged to assess the extent of the orbital • Orbital cellulitis
component before excision. • Recurrent cyst
Rupture • Compressive neuropathy
• A dermoid cyst can rupture spontane • Amblyopia
ously or with trauma, inciting an intense • Strabismus.
ORBITAL HEMANGIOMA
Aditi Dubey, Ritika Mukhija, Rajesh Pattebahadur
INTRODUCTION Examination
Orbital hemangiomas are of two types—capillary Inspection
hemangioma and cavernous hemangioma.
Superficial hemangiomas are confined to the
Capillary hemangioma is the most common pri
dermis, pink-purple mass lesion with mulberry
mary benign tumor of orbit in children (infancy).
appearance or dimpled texture and increases
Cavernous hemangioma is more common in
on crying or Valsalva. Deep orbital lesions may
adults (20–30 years), usually women.1,2
present with axial/non-axial proptosis.
CAPILLARY HEMANGIOMA
Palpation
History
Soft, nontender, nonpulsatile, ill-defined mass over
Chief Complaints the eyelid which may have an orbital extension.
Mass over eyelid present since birth or appear in
the first few weeks of birth. Auscultation
No bruit or pulsation heard.
History Deep orbital lesions may present with hypero
Usually parents complain of bluish or pink mass pia, optic nerve edema (due to compression),
over eyelid present since birth with enlarging with retinal striae, raised intraocular pressure and
age. In addition, there will be a history of increases strabismus.
in size on crying.
Oculoplasty 83
Classification Modalities of Treatment

zz Superficial or simple: Involves the skin and zz Small lesion < 2 mm thickness—laser
appear as a bright red, soft mass with a zz Superficial or preseptal lesion—intralesional
dimpled texture. steroid [betamethasone (4 mg/mL) 1–2 mL or
zz Preseptal or subcutaneous: Dark blue/purple triamcinolone (40 mg/mL) 1–2 mL at different
soft ill-defined non-tender mass (Fig. 1). site repeat after 2 months]. Adverse effects
increases on crying and Valsalva nonpulsatile of steroid injection include skin necrosis,
no bruit. subcutaneous fat atrophy, orbital hemorrhage
zz Deep: Located deeper within the orbit may and rarely central retinal artery occlusion.
present merely as a progressively enlarging zz Deep/orbital lesions—systemic steroids
mass without any overlying skin change (D/D zz Systemic beta-blockers (inhibit angiogenesis
Rhabdomyosarcoma should be ruled out). and acts as vasoconstrictor)
zz Surgical excision may be considered for lesions
Management that are smaller, subcutaneous or refractory to
Normally the course of capillary hemangioma is steroids
as follows: zz Interferon-α (however, it has significant
zz Rapid growth up to 6–12 months systemic adverse effects and poorly tolerated)
zz 30% spontaneous resolution by 3 years zz Radiation therapy has also been used, but it
zz 70% spontaneous resolution by 7 years has the potential to cause cataract formation,
Most lesions will regress spontaneously, bone hypoplasia, and future malignancy.
therefore; observation, refractive correction and
amblyopia therapy are the first line of management. CAVERNOUS HEMANGIOMA
Treatment should be deferred until it is clear that
the natural course of the lesion will not lead to the Chief Complaints
desired result. Cavernous hemangioma—slowly progressive
proptosis (growth may accelerate during
Indication for Treatment pregnancy).
zz Amblyopia secondary to astigmatism, ptosis,
and anisometropia. History
zz Exposure keratopathy Cavernous hemangiomas are usually seen in
zz Optic nerve compression adults presenting as progressive proptosis,
zz Severe disfigurement or cosmetic blemish sometimes decreased visual acuity may be present
zz Infection. due to compressive optic neuropathy.
Examination
Examination is similar to a case of proptosis due
to an intraconal tumor. It usually leads to axial
proptosis.
Course and Management of

Cavernous Hemangioma
zz This lesion rarely resolve spontaneously
zz Observation: If asymptomatic
zz An indication of treatment: 1. Symptomatic
lesion (lesion compromising ocular function);
2. Gradually enlarging
Fig. 1: Capillary hemangioma zz Treatment: Surgical excision.
VIVA QUESTIONS • M affucci syndrome: multiple skin and

visceral hemangiomas associated with
Q.1. Classify orbital hemangioma. enchondromas
Ans. • Cutaneous • High output heart failure associated with
• Purely preseptal fast growing visceral hemangiomas.
• P reseptal with orbital involvement • P HACES syndrome: Posterior fossa
(extraconal) malformations–hemangiomas–arterial
• P reseptal with orbital involvement anomalies–cardiac defects–eye abnor
(extraconal + intraconal). malities–sternal cleft and supraumbilical
raphe syndrome
Q.2. What are the risk factors for capillary
hemangioma? Q.6. What are the other vascular malfor
mations of the orbit?
Ans. Premature infants and newborns whose
Ans. • Hemangiopericytoma
mothers had chorionic villus sampling.
– The uncommon lesion, well
Q.3. What is the most common location of encapsu l ated, hypervascular and
hemangioma? hypercellular.
Ans. Capillary hemangioma: Predilection for the – Appear in middle age.
superonasal quadrant of the orbit and the – Resemble cavernous hemangiomas
medial upper eyelid, may involve skin over on both CT and MRl, but they appear
face some patients may have cutaneous bluish intraoperatively.
and visceral hemangiomas. – Histologically composed of plump
Cavernous hemangioma: Extraconal and pericytes that surround a rich capillary
retrobulbar. network, microscopically “benign”
lesions may recur and metastasize,
Q.4. Histopath olo g y an d imag ing in
whereas microscopically “malignant”
hemangioma?
lesions may remain localized.
Ans. Shown in Table 1.
– Treatment—complete excision
Q.5. What are the systemically associated syn because they may recur, undergo mali-
dromes with capillary hemangioma? gnant degeneration, or metastasize.
Ans. • K
asabach Merritt syndrome: Triad of • Lymphatic malformation
hemangioma, decrease coagulation – Also known as lymphangiomas.
factors and thrombocytopenia. Asso – Due to vascular dysgenesis.
ciated with rapidly expanding visceral – Become apparent in the first decade
hemangiomas. of life.
Table 1 Differentiating features between capillary and cavernous hemangioma

Parameters Capillary hemangioma Cavernous hemangioma
Histopathology Tumor composed of small Lesions are well encapsulated and composed of
anastomosing channels without large cavernous spaces containing red blood cells
true encapsulation with walls of the spaces containing smooth muscle
B scan For extention of disease and Well encapsulated mass lesion with cavernous fluid
anatomical relations (blood) filled spaces
CT scan Homogeneous enhancing soft Homogeneously slowly enhancing, well-
tissue mass ± extraconal extension encapsulated mass
with fingerlike projections
MRI scan Fine intralesional vascular channels Small intralesional vascular channels with slowly
and high blood flow flowing blood, i.e flow voids. Chronic lesions may
contain radiodense phleboliths
Oculoplasty 85
– Occurs in the orbit, conjunctiva, – Diagnosis: Contrast-enhanced rapid

eyelids, oropharynx, or sinuses. spiral CT during a Valsalva maneuver
– Contain both venous and lymphatic showing characteristic enlargement
components. of the engorged veins. Phleboliths
– May enlarge during URTIs and pre may be present on imaging.
sent with sudden proptosis caused – Treatment: Conservative
by spontaneous intralesional hemor- – Biopsy: Avoided because of the risk of
rhage. hemorrhage.
– Histology: Characterized by large, not – Surgery: Reserved for the relief of
encapsulated, serum-filled channels significant pain or for cases in which
that are lined by flat endothelial cells. the venous malformation causes
– MRI: Pathognomonic features multi vision-threatening compressive
ple grapes like cystic lesions with a optic neuropathy. Complete surgical
fluid-fluid layering of the serum and excision is difficult. Intraoperative
red blood cells. Venography shows no embolization of the lesion may aid
arterial or venous connection. surgical removal.
– Management: Surgical intervention • Arteriovenous malformations
should be deferred unless vision is – High-flow developmental anomalies
affected, due to the risk of hemorrhage. due to vascular dysgenesis.
A subtotal resection is generally – Composed of anastomosing arteries
needed to avoid sacrificing important and veins without an intervening
structures. Orbital hemorrhage is capillary bed.
allowed to resorb spontaneously; – Sign: Dilated corkscrew episcleral
but if optic neuropathy or corneal
vessels.
ulceration threatens vision, aspiration
– Treatment: Selective occlusion of the
of blood through a hollow-bore needle
feeding vessels followed by surgical
or by open surgical exploration can be
excision of the malformations
attempted.
(complication -arterial hemorrhage).
• Venous malformations
– Also known as orbital varices.
– Low-flow vascular lesions due to REFERENCES
vascular dysgenesis. 1. Albert DM, Miller JW, Azar DT. Albert
– Clinical features: Enophthalmos at & Jakobiec’s Principles and Practice of
rest (when the lesion is not engorged), Ophthalmology; 2008.
proptosis when the patient’s head 2. Brad Bowling. Kanski’s Clinical ophthalmology:
is dependent or after a Valsalva A systematic approach, 8th edn. Edinburgh:
maneuver). Elsevier; 2015
COLOBOMA OF EYELID
Shipra Singhi, Amar Pujari
INTRODUCTION Past Surgical History

An eyelid coloboma is a full-thickness defect of Previous history of ocular surgery may or may not
the eyelid.1,2 The word coloboma comes from the be present.
Greek word that means, “Curtailed”. Lid coloboma
occurs due to a delayed fusion of mesodermal EXAMINATION
components of frontonasal and maxillary Systemic Examination
processes of the face. It is caused by the failure
It may be associated with multiple systemic
of fusion of the mesodermal lid folds. Although
anomalies.
an eyelid coloboma can occur in many locations,
zz Cardiovascular abnormalities, facial hemi
the most common position is at the junction of
atrophy, atresia of the external auditory
the medial and middle third of the upper lid.3-5
meatus, accessory auricles, nevus flammeus,
In exams, it can be given as a short or spot case.
neurofibromatosis, preauricular appendages,
and pre-tragal fistulas can be there.One-third
HISTORY of cases associated with Goldenhar’s syndrome
(triad of peribulbar dermoid, preauricular
Chief Complaint
appendages, and pre-tragal fistulas).
A case of coloboma usually presents with cosmetic zz Facial defects that may be associated with
issues due to the defect (notching) in the eyelid. It eyelid colobomas, include a less prominent
can be associated with following: supraorbital margin, and a bifid nose.
zz Absence of eyeball zz Among the syndromes that may include eyelid,
zz Bluish colored swelling (in case of crypto- colobomas are Goldenhar, Treacher Collins,
phthalmos), Delleman, Fraser and nano palpebral lipoma
zz Small size of eyeball coloboma syndrome.
zz Drying of eyes
zz Diminution of vision: Blurring of vision is Ocular Examination
related to corneal opacity, exposure kerato
Visual acuity: Is variably impaired depending on
pathy, cataract and choroidal coloboma. It is
associated abnormalities such as limbal dermoid.
usually present since birth which may progress
and painless in origin. Eyeball: Microphthalmos, anophthalmos, eury-
zz Diplopia due to restriction blepharon, cryptophthalmos, lagophthalmos and
zz Painless mass (associated limbal dermoid) esotropia can be there.
zz Foreign body sensation/irritation. Extraocular movement: Duane’s retraction
syndrome may be an associated feature.
Past History Eyebrow: Loss of eyebrow hair may be seen.
A careful past history should be taken of any— Eyelids: Eyelid colobomas have following features
zz Perinatal and pregnancy history zz Most commonly triangular with the base at the
zz Family history of congenital eyelid colobomas eyelid margin.
or other congenital anomalies, especially facial zz It is usually located on the medial half of the
(e.g. cleft lip/palate) upper eyelid or lateral half of the lower eyelid.
zz History of other current birth defects zz They are usually unilateral, generally located
zz Pediatric review of systems, hearing loss, at the medial one-third of the upper eyelids
cardiovascular disease, facial asymmetry (90%), and may vary from a small notch to
zz History of progressive corneal problems. complete defects of the eyelid.
Oculoplasty 87
Iris: Coloboma (key hole)—may be typical or

atypical, complete or incomplete, partial or total.
Intraocular pressure (IOP): It is usually normal
Fundus: There may be choroidal coloboma, retinal
detachment due to choroidal coloboma and
hypoplastic disc.
CLASSIFICATION
Lid coloboma can be due to following:
zz Congenital lid coloboma (isolated or
syndromic)
zz Acquired lid coloboma (traumatic or post-
Fig. 1: Surgical eyelid coloboma surgical).
Depending upon the associations, it is
classified as following:
zz Upper eyelid coloboma (Fig. 1) is more
common than lower lid coloboma and may be Isolated Coloboma
associated with Goldenhar syndrome. zz Coloboma associated with cornea palpebral
zz Lower eyelid coloboma: Lower lid colobomas adhesions
are more commonly associated with facial —— Complete: No discernable eyelid differen
clefts. Treacher Collin syndrome is usually tiation and the eyes are completely
associated with this. covered with skin.
Lacrimal system: Obstruction proximal to the —— Incomplete: A skin fold devoid of tarsus
lacrimal sac and lacrimal stenosis can be there. covers the medial aspect of the palpebral
aperture, significant cornea palpebral
Conjunctiva: Symblepharon, absence of an upper
adhesions, lower fornix, and lateral upper
eyelid fornix and malformation of the caruncle
eyelids usually spared.
can be seen. Conjunctival traction bands are
—— Abortive type/congenital symblepharon
common (present in a third of eyelid colobomas).
variant: True coloboma of variable sizes
These bands are highly amblyogenic owing to
with a diverse range of cornea palpebral
strabismus. Forced duction testing (FDT) is often
adhesions, lower fornix, and lateral upper
positive in such cases of restriction.
eyelids usually spared.
Cornea: Following anomalies can be seen: zz Simple coloboma: Coloboma not associated
zz Exposure keratopathy with cornea palpebral adhesions.
zz Corneal opacities
zz Limbal dermoid: Yellowish-white, solid, Syndromic Variants
vascularized, elevated nodules straddling the
corneal limbus. Size may vary ranging from 2 zz Fraser syndrome
to 15 mm in diameter. Corneal dermoid occur zz Goldenhar syndrome
as single lesions mostly but may be multiple, zz Rare syndromes: Manitoba oculotrichoanal
and they may be unilateral or bilateral, the synd rome, Ablepharon-macrostomia syn
former being the more common. drome, Nasopalpebral lipoma-coloboma
zz Dellen formation may occur syndrome, Amniotic band sequence, Oculo
zz Cicatrization. ectodermal syndrome, Neurocutaneous
syndromes, CHARGE (coloboma, heart
Lens: Cataract (anterior polar) and subluxation of defect, atresia choanae, retarded growth and
the lens may be there. development, genital abnormality, and ear
Sclera: Epibulbar dermoid tumor can be there. abnormality) syndrome
GRADING Small defects: If the defect in the upper eyelid

involves less than one-third of the margin, and
Lid coloboma can be graded as follows (Nouby)3 well managed with topical lubrication, then
zz Grade 1: Coloboma without cryptophthalmos. surgery may be delayed until later in childhood.
zz Grade 2: Coloboma with abortive crypto- This surgery may require a lateral canthotomy
phthalmos. and/or superior cantholysis to rotate or advance
zz Grade 3: Coloboma with complete crypto- adjacent tissue to prevent excessive tension on
phthalmos. the wound.The edges of the defect are freshened
zz Grade 4: Classic cryptophthalmos (absence of with sharp incisions, and the precise anastomosis
all eyelid structures and complete coverage of is performed. The lid margin is brought together
eye by skin). using a 2-layer approximation of the tarsus and the
zz Grade 5: Severe cryptophthalmos (with severe skin. Lateral cantholysis and placement of near-
deformity of the nose and ectropion of the far, far-near sutures may be necessary to minimize
upper lip). horizontal tension.
Moderately-sized defects: Larger defects, a Tenzel
INVESTIGATION semicircular rotational flap may be used for
defects involving approximately one-third of the
The diagnosis of a lid coloboma requires a direct
eyelid margin.
clinical examination. Specific laboratory studies
are generally indicated in associated syndromes Large defects: If the defect is larger than one-half
that may include following: of the upper eyelid, other surgical procedures
zz X-ray of spine—for hemivertebra or scoliosis should be used. The various surgeries that can
zz ECG, echocardiography—for cardiac defect be performed include a free transconjunctival
zz MRI of brain graft from the contralateral upper eyelid can be
zz Complete blood count taken, modified Hughes procedure (for lower lid
zz Renal function test coloboma), modified Cutler-Beard procedure
zz Audiometry for hearing assessment. (upper lid coloboma), rotational flap from cheek
(Mustard’s technique).
Prognosis: Prognosis is excellent to good in eyelid
MANAGEMENT
coloboma, depending on the size of the lesion and
Medical Management the speed of therapy.
Corneal protection is the primary goal in the Patient education: Genetic consultation is highly
medical treatment of eyelid colobomas. Medical recommended, especially for patients with
therapy includes artificial tears and ointment and associated syndromes, such as Treacher Collins
Bedtime patching. syndrome, which is autosomal dominant with
variable penetrance and expressivity.
Surgical Management
VIVA QUESTIONS
Indication of surgery includes:
zz Exposure keratitis Q.1. When should the coloboma of eyelid be
zz Trichiasis which may cause corneal lesion. repaired?
zz Cosmesis Ans. This depends on the size of the defect
zz Amblyopia and on the presence of corneal exposure.
zz Strabismus. If the defect of the eyelid is small and not
The initial evaluation of an upper eyelid associated with corneal exposure, surgery
coloboma consists of measuring the size of the can be delayed until the age of 3–4 years,
eyelid margin defect and comparing it with the when there is an increased amount of
overall length of the horizontal palpebral fissure. eyelid tissue is available for repair. In a case
The surgical procedure used depends on the size of large defect, surgery should be done as
and the location of the defect. soon as possible to avoid corneal lesions.
Oculoplasty 89
Q.2. What is the difference between lower and Table 2 Treacher Collins syndrome
upper eyelid coloboma?
Structure
Ans. See Table 1. Clinical features
affected
Table 1 Difference between upper lid and lower Eyes • Antimangloid slant of palpebral
lid coloboma fissures
• Coloboma of lower eyelid
Upper eyelid coloboma Lower eyelid coloboma
• Hypoplasia of lower eyelid
More common Less common • Hypertelorism
Usually isolated Usually syndromic Ears • Microtia
association • Conductive hearing loss
Occur at the junction Occur most frequently • Stenosis or complete atresia
of the inner and middle at the junction of the • External ear abnormalities
thirds middle and lateral thirds Face • Hypoplasia of facial bones
Tend to be full thickness Tend to be partial (mandibular or zygomatic arch) or
thickness involving complete absence of zygomatic arch
preferentially the • Dental malocclusion
anterior lamella • Microstomia
• High arched palate, cleft palate
Have normal adjacent Adjacent lid margins
• Clonal atresia
lid margins may be abnormal
• Nasal dorsus parrot like shape
Usually not associated Usually associated with
Others • Malformations associated with
with facial clefts. facial clefts
heart, kidney, vertebral column and
Often associated with Usually not extremities
cryptophthalmos • Obstructive sleep apnea.
Q.3. What are the clinical findings in Treacher • Syndactyly

Collins syndrome? • Genital anomalies
Ans. See Table 2. • Sibling with Fraser syndrome.
Minor characteristics:
Q.4. What are the clinical findings in
• Alterations of the nose
Goldenhar syndrome?
• Alterations of the ears
Ans. • L imbal dermoid (bilateral in 25% of
• Alterations of the larynx
cases)
• Oral clefts (cleft lip and/or palate)
• Eyelid coloboma
• Umbilical hernia
• Preauricular appendages/skin tags
• Renal agenesis (unilateral or bilateral)
• Microtia or anotia of external ear can
• Skeletal anomalies.
be associated with hearing loss with or
without middle ear malformation
• Vertebral abnormalities (butterfly verte REFERENCES
brae or hemivertebrae) 1. Casey TA. Congenital colobomata of the eyelids.
• C ongenital heart disease (numerous Trans Ophthalmol Soc UK. 1976;96:65-8.
anomalies have been reported) 2. Collin JR. Congenital upper lid coloboma. Aust
• Central nervous system abnormalities NZ J Ophthalmol. 1986;14:313-7.
(hydrocephalus, intracranial lipomas, 3. Online Mendelian Inheritance in Man (OMIM)
cranial nerve dysgenesis and mental database, http://www.ncbi.nlm.nih.gov/omim
retardation have been described). (accessed 5 May 2013).
4. Pearson AA. The development of the eyelids.
Q.5. What are the clinical findings in Fraser Part I. External features. J Anat. 1980;130(1):
syndrome? 33-42.
Ans. Major characteristics: 5. Sevel D. A reappraisal of eyelid development.
• Cryptophthalmos Eye. 1988;2:123-9.
CHAPTER
2
Cornea and Conjunctiva
LONG CASES
CORNEAL ULCER
Shipra Singhi, Tushar Agarwal, Prafulla Kumar Maharana, Namrata Sharma
INTRODUCTION the host immunity. The predisposing factors are

summarized in Table 1.
Any breach in the continuity of an epithelial Sudden onset and rapid progression is generally
surface is called an ulcer. However, a corneal ulcer associated with bacterial corneal ulcers such as
is better defined as an epithelial defect associated Staphylococcus aureus, Pseudomonas aeruginosa
with superficial tissue loss along with variable and Pneumococcus species. Gradual onset and an
grades of inflammation. Corneal ulcer can be indolent course is commonly seen in ulcers caused
given as a long case in exams. A careful history and by fungi and parasites (Acanthamoeba) and few
examination often clinches the diagnosis. bacteria such as Moraxella, coagulase negative
Staphylococcus, Nocardia species and atypical
HISTORY Mycobacteria.
Chief Complaints In Acanthamoeba, keratitis course can be
variable (gradual or rapid) and it may be associated
The common presenting symptoms in a case of
with a prolonged course with remissions.
corneal ulcer are:
zz Pain
Pain
zz Diminution of vision
zz Redness, watering, discharge, foreign body The occurrence of pain in corneal ulcer can be
sensation minimal to excruciating. The type of causative
zz Photophobia. organism and depth of the ulcer influence the
severity of the pain.
History of Present Illness zz Superficial corneal ulcers are more painful
than deep corneal ulcers due to rich sensory
Following points must be recorded in history: nerve supply in the superficial cornea.
zz Acanthamoeba keratitis usually presents with
Onset and Progression excruciating pain due to associated radial
The onset of corneal ulcer depends on the keratoneuritis. The pain is usually out of
predisposing factor, virulence of the organism and proportion to the objective clinical findings.
Cornea and Conjunctiva 91
Table 1 Predisposing factors in cases of corneal

zz A sudden relief in pain in a case of corneal
ulcers ulcer may be indicative of perforation of the
corneal ulcer.
Predisposing
factors Examples
Redness and Photophobia
Ocular Trauma: Mycotic (vegetative) and
Acanthamoeba keratitis Corneal ulcer is usually associated with circum-
ciliary congestion or a combination of conjunctival
Contact lenses: Pseudomonas and and circumciliary congestion. Photophobia can
Acanthamoeba keratitis
be severe due to irritation of the anterior ciliary
Lid and adnexal infections: Pneumo nerves.
coccus keratitis (dacryocystitis) and
actinomyces (canaliculitis)
Discharge
Abnormality in lids such as
Most of the corneal ulcers are associated with
trichiasis, coloboma, ectropion,
entropion, lagophthalmos, discharge, which may be watery (in viral or
exophthalmos, proptosis, small bacterial corneal ulcer), mucopurulent or
blepharitis and meibomitis purulent (bacterial ulcer). Corneal ulcers caused
by Pseudomonas are associated with a greenish-
Ocular surface disease
yellowish discharge. A membranous discharge
Allergic eye disorders is seen with keratitis caused by Corynebacterium
Bullous keratopathy diphtheria.
Topical medications (topical
corticosteroids, honey, prolonged Decreased Visual Acuity
use of topical antibiotics) Loss of vision depends upon the severity and
Prior ocular surgery [pterygium location of the ulcer. Central corneal ulcers
surgery, keratoplasty, (caused by Pseudomonas species, Staphylococcus
photorefractive keratectomy and aureus and Fusarium species) are associated with
laser in situ keratomileusis (LASIK)] significant loss of visual acuity. The visual acuity
Systemic Diabetes mellitus may not be severely affected in small, peripheral
Sjögren’s syndrome ulcers (e.g. early cases of Acanthamoeba keratitis
Steven-Johnson syndrome where only epithelium is affected).
HIV Other factors that can reduce visual acuity
Advanced malignancies include the presence of an associated pupillary
Connective tissue disorders membrane, hypopyon, cataract, glaucoma and
Alcoholics endophthalmitis.
Extremes of age
Measles
Malnutrition
Smoking History of trauma, contact lens use, allergic eye
Occupational Farmers (mycotic keratitis) disorders, topical medication use, prior ocular
Animal handlers (Listeria keratitis) surgery or systemic disease (as mentioned in
Gardners Table 1) has to be noted carefully.
In contrast reverse is true for fungal ulcers Family History

where pain may be completely absent in spite Family history may be there in cases of connective
of an advanced corneal ulcer. tissue disorders.
Past Surgical History keratoconjuctivitis may be associated with

pseudomembrane formation.
Recent surgery can be a predisposing factor for
zz Foreign bodies can be a cause for non-healing
infective keratitis (see Table 1).
corneal ulcer.
EXAMINATION Discharge: Characteristic of discharge can provide
a clue about the probable diagnosis such as:
General Examination/Specific Systemic zz Watery—viral or small bacterial corneal ulcer
Examination zz Mucoid—bacterial corneal ulcer
A thorough general examination must be carried
zz Mucopurulent—Pseudomonas and Gono-
out to look for following: coccus
zz Potential source of infection
zz Frankly purulent—severe bacterial corneal
zz Connective tissue disorders ulcer
zz Immunocompromised states.
zz Corneal ulcers caused by Pseudomonas
are associated with a greenish-yellowish
discharge. A membranous discharge is seen
Ocular Examination
with keratitis caused by Corynebacterium
Visual acuity: Visual acuity may be reduced in diphtheriae.
cases where the ulcer is located in the center of
the cornea or due to presence of corneal edema, CORNEA
massive hypopyon or associated endophthalmitis.
The endothelium and associated anterior chamber Ulcer
inflammation (cell, flare, hypopyon, or fibrin) Following parameters must be noted:
should not be overlooked. zz Location: The location of ulcer gives an
Eyeball: Look for presence of any lagophthalmos, indication about the probable cause, visual
or proptosis/exophthalmos. Blepharophimosis prognosis and the initial choice of antibiotics.
can be there in presence of severe inflammation. The location can be
—— Central: Staphlococcus aureus (Fig. 1),
Lid: Look for trichiasis, coloboma, entropion,
Pseudomonas, Fusarium
lid lag, ectropion and blepharitis that may be
—— Paracentral: Staphlococcus aureus,
predisposing factors.
Pseudomonas, Fusarium
Lacrimal sac: Look for dacryocystitis (can be —— Peripheral: Coagulase –ve Staphylococcus
associated with pneumococcal corneal ulcers) aureus, M. tuberculosis, Herpes simplex

and canaliculitis (associated with Actinomyces
keratitis). Regurgitation, syringing, and probing
must be done in all cases to rule out any potential
source of infection.
Conjunctiva: The bulbar conjunctiva and the
upper and lower tarsal conjunctiva should be
examined for the presence of:
zz Any follicles, papillae, for diseases like vernal
catarrh and atopic conjunctivitis
zz Discharge
zz Erythema, cicatrization, keratinization, sug
gestive of poor ocular surface, severe dry eye
and limbal stem cell deficiency
zz Membrane, pseudomembrane formation:
Membranous conjunctivitis is seen with kera
titis caused by Corynebacterium diphtheria.
Gonococcal, pneumococcal and Haemophilus Fig. 1: Central corneal ulcer
—— Superior: Ulcer associated with Shield zz Margins of ulcer: Margin can be

ulcer (VKC) or foreign body (FB) in sulcus —— Well-defined: Seen in healing infectious
subtarsalis [common in children] ulcer or sterile ulcer

—— Inferior: Ulcers associated with exposure —— Punched out: In cases of neurotrophic
keratopathy. ulcer
zz Size: Record the size of the ulcer along the two —— Indistinct: Seen in cases of progressive
largest meridians. (Two axes where the extent ulcer

is maximum that can be vertical and horizontal —— Hyphae or feathery: Characteristic of
or two oblique axes). Micrometer present fungal corneal ulcer

in slit-lamp can be used to record the size of —— Overhanging: Mooren’s ulcer
the corneal ulcer. Recording the baseline size zz Base: Usually the base of the ulcer is filled with
is important as it helps in grading as well as necrotic slough. A dry looking ulcer suggests
monitoring of therapy. fungal corneal ulcer.
The size of the epithelial defect and the zz Depth: Depth of the ulcer is best measured
size of the infiltration should be measured with the use of a slit-lamp. It helps in grading
separately. They should be measured of the ulcer and deciding the initial treatment
separately as their sizes may not be similar (in protocol. A deep-seated ulcer (>50%) or
corneal abscesses corneal epithelium may be descemetocele (Fig. 3) may warrant the
intact). The epithelial defects is best examined initiation of systemic antibiotics.
using a slit-lamp with cobalt blue light after zz Surrounding area: Look for presence of satel
staining the cornea with fluorescein dye. lite lesions (characteristic of fungal ulcer),
Infiltration may be single or multiple and corneal scars (ghost scars, in recurrent viral
may be of varying sizes depending on the keratitis).
organism involved, severity and duration of zz Pigmentation: Pigment producing fungi such
the infection. Look for satellite infiltrates in as curvularia, alternaria can give a distinct
fungal corneal ulcers. color to the ulcer due to pigment production
zz Shape: Shape of the ulcer can give a clue about (Fig. 4).
the probable cause. Examples include: zz Vascularization: Appearance of new vessels
—— Dendritic, amoeboid or geographic (Fig. 5) is a sign of healing keratitis. Superficial
shape—viral keratitis or deep corneal vascularization of varying
—— Ring shaped ulcer: Acanthamoeba (Fig. 2), extent may be seen in cases of infectious
Staphylococcus keratitis. A quadrant wise record of corneal
—— Oval—neurotrophic ulcer vascularization should be made.
Fig. 2: Ring shaped ulcer in Acanthamoeba Fig. 3: Descemetocele

Fig. 4: Corneal ulcer with pigment production Fig. 6: Perforated corneal ulcer
surrounding cornea becomes hazy and grossly

edematous appearing like a ground glass. Clearing
of surrounding corneal edema after the initiation
of medical therapy is an early sign of resolution of
the ulcer.
Corneal Thinning/Perforation
The ulcer should be closely monitored for the
development of corneal thinning, descemetocele
and perforation (Fig. 6). In the presence of shallow
anterior chamber and low intraocular pressure,
a Seidel’s test should be performed in all cases.
Severe corneal thinning and perforation warrant
Fig. 5: Appearance of new vessels in a case of immediate surgical therapy (glue or tectonic patch
healing keratitis graft).
Other Findings
Corneal Sensations Look for other findings that may give a clue
Corneal sensations should be measured with the about the possible cause for ulcer, such as foreign
help of a cotton wisp or esthesiometer (Cochet- bodies, exposed or broken sutures, signs of corneal
Bonnet esthesiometer). In cases of herpetic dystrophies, previous corneal inflammation
keratitis, neuropathic keratitis and cases with (thinning, scarring, or neovascularization), and
diabetes mellitus, the corneal sensations are signs of previous corneal or refractive surgery.
decreased. Fluorescein or rose Bengal staining may
provide additional information, such as the
Surrounding Cornea presence of dendrites, pseudodendrites, loose or
exposed sutures, and epithelial defects.
The cornea surrounding the lesion may be,
clear or hazy due to edema depending on the
virulence of the organisms. Candida tends to Documentation
cause localized lesions with distinct borders and The documentation of the corneal ulcer may be
minimal surrounding edema. Some organisms done using color clinical photographs or using
like Pseudomonas produce ulcers in which the detailed schematic drawings.
Color Photographs of the cornea and dots represent punctate epithe

lial keratopathy, small lines depict filaments and
In all cases of corneal ulcer a colored photograph
shaded outline demonstrate epithelial defects.
of the diffuse as well as the slit section of the
Green color is also used to depict the location of
cornea should be taken. Measurements should be
lens and vitreous opacities/hemorrhage, etc.
made of the maximum diameter of the ulcer and
Clinical features of specific keratitis have been
the meridian perpendicular to it. Apart from this
described in Table 2.
infiltrate, size should also be measured in same
manner.
Sclera
Schematic Drawings One should look for scleral inflammation,
ulceration, nodules or ischemia. Any involvement
Following color-coding is used to depict a corneal
of the sclera should be recorded as this helps
ulcer:
in prognosticating the case as well as in the
Black—outline of the corneal limbus, indicates
management protocol. Scleral involvement
scars resulting from keratitis, degeneration and
warrants the use of systemic antimicrobial
foreign bodies. Blue—designates edema, small
agents. Sclerokeratitis usually occurs in cases
dots for epithelial edema or circles for lakes of fluid
of immunologic disorders and Acanthamoeba
within the stroma, and wavy lines to depict the
keratitis.
folds in Descemet’s membrane. Brown indicates
melanin or iron pigmentation including pupil
and iris. Red—is used to depict blood vessels and Anterior Chamber
rose Bengal staining. Red wavy lines indicate Mild flare to severe hypopyon (Fig. 7) formation
subepithelial vessels, straight lines indicate deep maybe there. Size of hypopyon should be
stromal vessels and dotted lines indicate ghost measured using slit-lamp micrometer. Hypopyon
vessels. The wavy lines of superficial vessels and its characteristics are helpful in establish
begin outside the limbus circle, whereas straight ing the etiological diagnosis (Table 3). In order
lines of stromal vessels begin at the margin of to test the mobility of the hypopyon, following a
the circle. Solid shading depicts hemorrhage and slit-lamp examination, the patient is asked to lie
red dots indicate area stained by rose Bengal. supine for 10 minutes and a slit-lamp examination
Orange—denotes inflammation and presence of is then done. In case of the fixed hypopyon
white blood cells, which may be in the following (Fig. 8), there is no change in position of hypopyon
forms—stromal infiltrate, hypopyon or keratic as demonstrated by the height of hypopyon. In case
precipitates. Green—indicates fluorescein staining of the mobile hypopyon, there is actual movement
Table 2 Clinical features of specific keratitis

Disease specific Clinical features
Bacterial keratitis Well-defined infiltrate with moderate inflammation in anterior chamber
Fungal keratitis Dry looking ulcer with feathery margins, satellite lesions, ring ulcer, endothelial
plaque, pigmentation in dematiaceous keratitis
Acanthamoeba keratitis Epithelial haze with pseudodendrites, radial perineuritis, ring ulcer
Microsporidiosis Multifocal punctate raised epithelial lesions with clear underlying stroma
Viral keratitis Dendrites, geographic ulcer, annular stromal edema with KPs
Pseudomonas keratitis Rapidly sloughing ulcer, ring ulcer, with evident corneal edema in the uninvolved
cornea, rapid melting with perforation of cornea
Microsporidiosis Multifocal punctate raised epithelial lesions with clear underlying stroma
Atypical bacteria Cracked wind shield corneal ulcer, minimal changes in surrounding cornea,
minimal reaction in anterior chamber
Fig. 7: Hypopyon Fig. 8: Fixed hypopyon
pressure (IOP) in cases of corneal ulcer. Secondary

Table 3 Characteristics of hypopyon
glaucoma may be present in some cases. Hypotony
Characteristics Probable diagnosis may be present in case of corneal perforation.
Central Pneumococcal corneal ulcer
Lens
Hemorrhagic Pneumococcal corneal ulcer,
herpes simplex viral keratitis Cataract formation may be there or pigment
deposit may be there.
Mobile Bacterial corneal ulcer
Fixed/immobile Fungal corneal ulcer Posterior Segment
Sterile Behçet’s syndrome Usually, it is not possible to view the vitreous and
retina in case of corneal ulcer due to the presence
and the upper level or height of the hypopyon of hazy cornea. However, if the ulcer is small and
decreases. peripheral, slit-lamp biomicroscopy may be done
to visualize the anterior one-third of the vitreous.
Iris Additionally, an indirect ophthalmoscopy may
be performed to check for the involvement of the
Variable degrees of uveal inflammation can occur posterior segment.
with infectious keratitis. Synechiae formation
maybe there. Presence of new vessels on iris
(rubeosis iridis) can also be seen in cases of Fellow Eye
prolonged inflammation. If the ulcer perforates, One should also examine the fellow eye as
uveal prolapse may occur and this may later form bilateral involvement may be there (usually in
a corneoiridic scar. immunological cases).
Pupil
GRADING OF CORNEAL ULCER
Any abnormality in the pupil size, its shape and
location should be recorded. In presence of severe Kindly refer to Table 4.
inflammation iris may be atonic.
Intraocular Pressure Based on history and clinical examination a provi
Digital tonometry in the experienced hands is the sional diagnosis can be made. The differentiating
most practical method of assessing intraocular features are described in Table 5.
INVESTIGATIONS methodology should encompass techniques that

allow for the recognition of as large a number of
Microbiological Diagnosis of offending organisms as possible.
Infectious Keratitis
Collection of samples: The samples should be
Owing to the considerable overlap in the clinical collected at the initial presentation before the
appearances of corneal ulcers due to various start of antimicrobial therapy. The treatment
microorganisms, a standard basic laboratory can be initiated based on the results of smear
examination. Samples for diagnosis of corneal
ulcer can be following:
Table 4 Grading of corneal ulcer
zz Eyelid swab—not of much use
Feature Mild Moderate Severe zz Conjunctival swab—not of much use
Size of <2 2–5 >5 zz Corneal scraping—most important
ulcer (mm) zz Contact lens, contact lens case and solution—
Depth of <20 20–50 >50 must in contact lens user
ulcer (%) zz Anterior chamber paracentesis (hypopyon)—
deep ulceration or when insufficient material
Infiltrate
is present.
• Density • Dense • Dense • Dense
• Extent • Superficial • E xtension • D
eeper
up to mid than mid Corneal Scrapings
stroma stroma Instruments
Scleral Not Not May be Kimura’s spatula is traditionally used to collect
involve involved involved involved scrapings from a corneal ulcer though Bard-
ment Parker blade number 15, 26 gauge needle,
Table 5 Differential diagnosis of corneal ulcer

Parameters Bacterial Fungal Viral Acanthamoeba Atypical bacteria
Risk factors Blepharitis, Trauma with Past history of viral Contact lens Prior corneal
dacryocystitis, vegetative matter, infection use, swimming surgery
trichiasis, con indiscriminate
tact lens use, use of topical
ocular surface antibiotic or
disorder steroid
Symptoms Severe Indolent course, Like bacterial, may Waxing and Indolent
symptoms, signs more than be associated with waning course, course, failure
rapid symptoms recurrences severe pain to respond
progression to routine
antibiotics
Sign Epithelial Feathery margins/ Epithelial keratitis Pseudoden- Cracked wind
defect, hyphate edges, (punctate, dendritic, drites, radial shield corneal
infiltration, rough and dry geographic, marginal, keratoneuritis, ulcer, minimal
mobile texture, satellite neurotrophic), stromal ring shaped changes in
hypopyon, lesions, gray/ keratitis (immune ring infiltrate surrounding
anterior brown pigmenta of wessely, stromal cornea, minimal
chamber tion, immune neovascularization), reaction
reaction, ring of wessely, endothelitis, uveitis, in anterior
mucopurulent collar button decreased corneal chamber
discharge configuration, sensation, periocular
fixed hypopyon lymphadenopathy
hypodermic needle, platinum spatula are also made on a slide, which may not be sterile. More
used. Cotton swabs are not recommended for recently, calcium alginate swabs moistened with
collection of corneal material (may interfere with trypticase soy broth provides another method of
fungal filament interpretation). collecting corneal specimens for yielding higher
number of bacteria as well as fungi compared
Technique to platinum spatula. However, one limitation is
calcium itself may act as an antibacterial agent.
Topical anesthesia drop (0.5% proparacaine) Scrapping has to be done with utmost care in cases
is applied. A lid speculum is applied gently to of severe keratolysis, descemetocele and deep
separate the lids. Any slough or mucus debris stromal keratitis.
must be removed gently. Multiple scraping of
the ulcer base and margins is done under topical Inoculation
anesthesia (0.5% proparacaine hydrochloride
is proffered since it is the least bactericidal Solid agar media (Table 6) are inoculated on
compared to other anesthetic agents like 4% ligno the surface making multiple “C” shaped marks
caine hydrochloride or tetracaine) with the aid of without cutting the agar. In the liquid media, the
a slit-lamp or operating microscope. Streptococci spatula or blade is swirled to allow the sample to be
pneumoniae is more readily found at edge of transferred. In the case of thioglycollate broth and
the ulcer whereas Moraxella is more likely to be Sabouraud’s dextrose agar (SDA) deep inoculation
present at the ulcer base hence both base and of the medium is ensured by transferring the
margin have to be scrapped. Several scrapings sample to a swab tip and dropping the swab in the
are collected and used in a sequence to prepare tube allowing it to settle at the bottom.
smears and inoculate culture media. The blade or
spatula may be reused when a sterile medium has Routine Smears and Stains
been streaked. However, a blade must be changed Gram stain (most common), Kinyoun stain
(spatula should be flamed) when a smear has been (Cold carbol Fuchsin) or Giemsa stain (Table 7)
Table 6 Common culture media for various organisms

Culture medium Growth Incubation temperature
Blood agar plate Aerobic bacteria 35°C
Facultative anaerobic bacteria
Fungi
Chocolate agar plate Aerobic bacteria 35°C
Facultative anaerobic bacteria
Neisseria
Haemophilus
Moraxella
Thioglycollate broth Aerobic bacteria 35°C
Anaerobic bacteria
Sabouraud’s dextrose agar plate with antibiotic Fungi Room temperature
Nocardia
Brain heart infusion broth plate with antibiotic Fungi Room temperature
Nocardia
Cooked meat broth Anaerobic bacteria 35°C
Thayer martin blood agar plate Neisseria 35°C
Lowenstein-Jensen media Mycobacteria species 35°C with 3–10% CO2
Middlebrook-Cohn agar Mycobacteria species 35°C with 3–10% CO2
Nocardia
Table 7 Different stains used for smear examination

Type of stain Organism visualized Color of the organism
Gram stain Bacteria Gram positive-purple
Gram negative-pink
Acridine orange Bacteria, Fungi, Acanthamoeba Yellow-orange
Calcofluor white Fungi Bright green
Acanthamoeba cysts Bright green
Acanthamoeba trophozoites Reddish orange
Acid fast Mycobacteria Pink
are employed to study the corneal material which Cultures

is spread as a thin layer on several clean glass
Identification of bacteria may be accomplished
slides within an area defined with a wax pencil
within 48 hours along with its antibiotic
on the reverse. In the preparation of wet mounts
susceptibility pattern. In some cases, the pathogen
such as potassium hydroxide (KOH), lactophenol
may be recognized in 12–15 hours. Standardized
cotton blue or calcoflour white, the scrapings
disk diffusion or dilution techniques should be
can be placed on the slide in a demarcated area
utilized for antibiotic susceptibility testing of
and covered with a drop of the solution followed
bacteria. The majority of fungi causing keratitis can
by a coverslip. Special stains and media may be
be detected on SDA within 72 hours. Aspergillus
included whenever usual procedures have yielded
and Fusarium species grow on blood agar, SDA
negative results.
and brain-heart infusion broth within 48 hours.
However, appreciably characteristic colonies
Interpretation develop after 1–2 weeks. Hence, culture media
Staining methods yield a rapid result and help to should be observed for at least 2 weeks before they
determine the initial choice of an antimicrobial are considered negative. Non-nutrient agar (NNA)
agent. KOH wet preparation—a 10–20% solution of is the standard medium used with an overlay of
KOH is used to visualize fungal elements in corneal Escherichia coli for the growth of Acanthamoeba.
scrapes. Owing to the chitin in their cell wall, The specimen is simply touched to the surface of
fungal filaments and cysts of Acanthamoeba are the plate without streaking or breaking the surface.
clearly delineated in a homogeneous background Two plates may be inoculated for incubation at
of corneal tissue digested by KOH. Its sensitivity 25°C and 37°C since some species do not grow
ranges from 90% to 99%. Gram stain is utilized to at the higher temperature and the plates are
identify bacteria, fungi as well as Acanthamoeba. examined for trophozoites and cysts directly under
It has been reported to yield an accuracy of the microscope (100 X). Trophozoites may be seen
60–75% in identifying the responsible organisms. in 24–48 hours. They move and cover the entire
Calcofluor white (CFW) is a fluorescent brightener plate surface on further incubation and turn into
with great affinity for certain polysaccharides such cysts. The plates should be observed for at least
as cellulose and chitin, thus providing the basis 7 days.
for demonstration of fungal cell walls as well as Special media, selective and non-selective,
cysts of Acanthamoeba species. The preparation is may be indicated in certain clinical situations.
viewed under fluorescence microscope. The cysts Lowenstein-Jensen medium is used when
of Acanthamoeba and fungal filaments appear mycobacterial infection is suspected. Nocardia
bright apple green in a corneal scraping stained organisms can grow, though slowly, on blood agar
with CFW. Acanthamoeba trophozoites and as well as other bacterial media.
bacteria such as Nocardia and Actinomyces do not Remember: Aerobic cultures of the corneal
stain with CFW. specimens should be held for 7 days, anaerobic
cultures for 7–14 days and Mycobacterial and Biopsy from below a lamellar flap can be
fungal cultures for 4–6 weeks before being reported considered for a midstromal lesion such as
as no growth. infectious crystalline keratopathy, or a deep
stromal lesion such as fungal keratitis.
Interpretation of Culture Results
Interpretation of the culture results should be Anterior Chamber Paracentesis
made with regard to the clinical situation, the This procedure is rarely indicated in the diagnostic
adequacy of the sample and the possibility of evaluation of a patient with a corneal ulcer.
contamination by organisms present on the skin, However, this procedure may be indicated in
eyelids and conjunctiva. Positive culture rates vary the instance where keratomycosis is strongly
from 40% to 73%. suspected clinically, yet corneal scrapings and
An isolate is more likely to be considered biopsy have been negative, the damage to the
significant if it is consistent with the clinical signs cornea is progressive and a hypopyon is present
plus: or increasing.
zz The same organism is grown on more than one
media Confocal Microscopy
zz Confluent growth of a known ocular pathogen In vivo confocal microscopy (IVCM) is a non
in one solid medium or invasive in vivo diagnostic method for microbial
zz Growth in one medium of an organism with keratitis. It has been successfully used to
positive smear results or growth of same distinguish some unusual pathogens such as
organism in liquid media. Acanthamoeba cysts or fungal hyphae. With new
generation, IVCM can help in initiating anti-fungal
Jones Criteria for Positive Culture therapy and in monitoring the response to therapy.
zz Clinical signs of infection plus isolation of
bacteria (10 or more colonies) on one solid Newer Methods
medium and one additional medium, or The need for rapid diagnosis has led to modifi
zz Isolation of organism (any detectable growth) cation of various conventional techniques
on any two solid media or and introduction of new techniques such as
zz Isolation of organism in one medium in the immunohistochemistry, fluorescent microscopy,
presence of a positive smear. enzyme immunoassays, radioimmunoassay,
When the culture results are negative, anti polymerase chain reaction (PCR) and molecular
biotic treatment can be suspended for 24 hours biology. Most ocular infections can now be
and rescraping is done, following which repeat diagnosed by these modern techniques within
cultures are sent and examined. 1–6 hours.
Corneal Biopsy Ultrasonography

Corneal biopsy is indicated if infectious keratitis It is done in cases of corneal haze for fundus
is suspected clinically and twice repeated evaluation. In cases of perforated corneal
microscopic evaluation of smears and culture ulcer, endophthalmitis, choroidal and retinal
results are negative and no clinical improvement detachment should be excluded.
is noted on the initial broad-spectrum antibiotic
therapy. In addition, in certain cases of deep MANAGEMENT
mycotic keratitis and intrastromal abscesses a
corneal biopsy is indicated. Management of Bacterial Keratitis
A partial-thickness trephination employing a Bacterial keratitis should be treated as an ocular
trephine of sufficient size, to guarantee adequate emergency due to its rapid progression and
material for the laboratory, is required. Care is disastrous complications. Initially an empirical
taken to avoid the visual axis as far as possible. antibiotic therapy (combination therapy) is started
to cover for both Gram+ve and Gram-ve zz Increase in size of anterior chamber reaction
organisms. Standard therapy is a combination of: zz Non-healing of epithelial defect
zz Fortified cefazoline 5% or 10% + tobramycin zz Progressive stromal thinning
1.3% The clinical response should be the first
zz Fluroquinolone (moxifloxacin 0.5% or gatiflox guideline of therapy, although in non-healing
acin 0.3%) + tobramycin 1.3%. ulcer, in vitro sensitivity should be given due
Monotherapy can be considered in cases attention. In vitro sensitivity should be correlated
where the ulcer is in periphery and is small with in vivo response before considering change
(<3 mm). For monotherapy, fluoroquinolones in therapy as inadequate frequency, poor
are preferred (ofloxacin 0.3%, ciprofloxacin penetration, stromal lysis, and necrotic debris
0.3%, gatifloxacin 0.3% or moxifloxacin 0.5%). covering the site of infection can be the causes of
Comparison of fluoroquinolones and fortified eye nonresponsiveness of ulcer to therapy.
drops is given in Table 8. The aminoglycoside antibiotics used in
The frequency of instillation of drops depends fortified drops are gentamycin and tobramycin.
upon the severity, but it is usual to start half-hourly They give an excellent gram-negative coverage
drops all through 24 hours for most patients. and are active against staphylococci and some
A loading dose of a drop every 5 minutes for streptococci but not against pneumococci. The
the first 30 minutes is used in severe ulcers. most commonly used cephalosporin in fortified
The frequency is reduced based on the clinical drops is cefazolin. It gives good coverage for non-
response described below. penicillinase producing Gram –positive bacteria.
Adjunctive therapy is required to alleviate
Favorable signs: the symptoms that includes cycloplegic drugs,
zz Stabilization and no progression and improved antiglaucoma drugs, tear supplements and topical
symptoms corticosteroids. Management in specific bacterial
zz Reduced activity at infiltrate margins/blunting keratitis has been described in Table 9.
of ulcer edges Indication of systemic antibiotic in bacterial
zz Resolution of infiltration keratitis includes following:
zz Progressive healing of epithelial defect zz Perforated/impending perforation in corneal
zz Reduction in adjacent stromal inflammatory ulcer
reaction and anterior chamber reaction zz Postperforating injury
zz Reduction in hypopyon zz Scleral involvement
zz Vascularization. zz Endophthalmitis
Unfavorable signs: Signs that suggest poor zz Highly virulent organisms—Neisseria, Hemo-
response to therapy includes following: philus
zz Deterioration in symptoms The steroid for corneal ulcer trial (SCUT)
zz Increase in size and density of infiltration found no significant difference in 3 month BCVA
between patients receiving topical corticosteroid
Table 8 Comparison of fluoroquinolones and or placebo as adjunctive therapy in the treatment
fortified eye drops of bacterial corneal ulcers. No apparent increased
risk of corneal perforation was observed with the
S.
use of corticosteroids.
No Fluoroquinolones Fortified drops
1. Cheaper More expensive
Management of Fungal Keratitis
2. Readily available Need to be prepared
The major group of anti-fungal agents available
3. Stable Preferable to refrigerate
includes the following:
4. Shelf life of 1 month Shelf life of 1 week zz Polyenes: Natamycin, nystatin, amphotericin B
5. Less toxic More toxic zz Azoles: Fluconazole, itraconazole, voricona
6. Poor coverage of gram- Better coverage zole, posaconazole, ravuconazole
positive organisms with zz Fluorinated pyrimidines: Flucytosine
older generations zz Echinocandins: Caspofungin, micafungin
Table 9 Management of specific bacterial keratitis

Organism Topical Systemic
Methicillin resistant Staphylococcus Vancomycin 50 mg/mL Vancomycin 2 g/day
Severe Pseudomonas keratitis Ceftazidime (50 mg/mL) Ceftazidime (1–2 g/day I/V or I/M)
Mycobacterium fortuitum—chelonae Amikacin 40–100 mg/mL Clarithromycin 500 mg BD
Nocardia Amikacin 40–100 mg/mL Trimethoprim/sulfamethoxazole
or (10–20 mg/kg/day) I/V
Trimethoprim (16 mg/mL)
sulfamethoxazole (80 mg/mL)
injectable
Table 10 Sensitivity of different anti-fungals (MIC values of different genera in microgram/mL)
Fungus Voriconazole AMB Fluconazole Natamycin
Candida 0.08–0.016 0.25–0.5 0.12–0.5 3.12–12.5
Aspergillus 0.25–0.5 1–2 >256 3.12–25
Fusarium 1–4 1–2 >256 1.56–6.25
Abbreviations: AMB, amphotericin B; MIC, minimum inhibitory concentration
The standard treatment followed includes the zz Large ulcer (>6 mm diameter)
following step wise approach: zz Post-penetrating keratoplasty
zz Topical antifungals: 5% natamycin (drug of zz Scleral involvement
choice) is given hourly at daytime, 2 hourly at zz Endophthalmitis.
bed time Commonly used systemic antifungals
zz Taper after 4–7 days interval depending upon include ketoconazole (200 mg bd), fluconazole
clinical response. (200 mg bd), itraconazole (100 mg bd) and vori
zz Surface debridement helps to remove slough conazole (200 mg bd). The systemic antifungals
and reduce load of infection. Benefits are can cause a number of adverse reactions, hence
controversial but still preferred by most monitoring of blood glucose, blood pressure and
clinicians. liver function test has to done regularly.
zz If worsening (after 14 days of treatment)—
add 0.15% amphotericin B drops or 1% Targeted Drug Delivery
voriconazole. Also review the culture report.
About 20% of fungal ulcers are refractory to
zz Therapy duration is 3–4 weeks. Complete
medical therapy. In such cases targeted drug
resolution often require 4–8 weeks.
delivery (providing the drug where it is needed the
Unlike antibacterials, the antifungals suffers most) is a useful alternative before proceeding for
from the limitations of poor ocular penetration, surgery. The different modalities are intracameral,
poor bioavailability, epithelial toxicity and poor intracorneal or intrastromal drug delivery.
commercial availability. The sensitivity of currently The different agents used are amphotericin B
available antifungals are summarized in Table 10. (5–7.5 µg/0.1 mL/5% dextrose) and voriconazole
The indications of systemic antifungals in fungal 50–100 µg/0.1 mL.
keratitis are following:
Perforated/impending perforation corneal Management of Viral Keratitis
zz
ulcer
zz Severe deep ulcer (involving >2/3 stromal The different antivirals available includes
depth) acyclovir 3% ointment, vidarabine 3% ointment,
Table 11 Treatment protocol for viral keratitis

Type of keratitis Topical acyclovir 3% Topical corticosteroids Comments
Epithelial keratitis 5 times for 1 week Contraindicated
3 times for 2–3 weeks
Epithelial + stromal 5 times for 1 week After 1 week Taper both by 6–8 weeks
3 times for 2–3 weeks Pred acetate 2–4 hourly
Necrotizing and non- 5 times for 1 week Pred acetate 2–4 hourly Systemic ACV 400 mg
necrotizing stromal HSK 3 times for 2–3 weeks 5 times one week
3 times for 1 week
trifluorothymidine 1% and idoxuridine 1%. The Epithelial Removal and

dose and duration of antivirals (acyclovir) has Anterior Lamellar Keratectomy
been described in Table 11. Remember if a true
ulceration persists after 14 days of treatment, one This is useful in cases of fungal keratitis. Regular
must distinguish between a neurotrophic ulcer and debridement of the base of the ulcer helps in
persistent infectious epithelial keratitis. A neuro elimination of organisms and necrotic material.
trophic ulcer has smooth borders and lacks the This procedure facilitates penetration of anti
scalloped edges of infectious epithelial keratitis. fungal drugs. This can be done under topical
If the lesion is persistent infectious epithelial anesthesia leaving a margin of 1–2 mm at the
keratitis, resistance to the antiviral medication limbus with a number 15 Bard-Parker blade.
must be considered, and an alternative medication Anterior lamellar keratectomy helps in removal
can be initiated. The readers are advised to refer to of the thick mat of fungal filaments on the cornea
a standard textbook for treatment of viral keratitis and facilitates increased drug penetration in
in detail. cases of dematiaceous fungal filaments. Anterior
stromal corneal infiltrates can also be ablated with
phototherapeutic keratectomy.
Surgical Management
Surgical treatment is indicated in cases of
Conjunctival Flaps
impending perforation, perforated corneal ulcer
and nonhealing corneal ulcer (to reduce microbial Conjunctival flaps help in achieving a stable
load). The decision to surgically intervene in a case epithelial surface in cases of persistent or recur
of active infectious keratitis should be made after rent epithelial defects and progressive ulceration
proper evaluation of the clinical progress. Surgery especially in viral keratitis. In advanced cases of
helps to eliminate or reduce the microbial load and corneal ulcer, where the only aim is to save the
in providing tectonic support to the globe where globe, a Gunderson’s flap is done where the entire
the integrity is threatened as in cases of thinning surface is covered with a conjunctival flap.
or perforation of the cornea. The different surgical
options and various modalities of treatment avail Tissue Adhesives
able in such cases are:
zz Removal of epithelium and anterior lamellar Tissue adhesive (cyanoacrylates) helps in sup
keratectomy porting corneal thinning and sealing corneal
zz Conjunctival flaps (Gunderson’s flap) perforation up to 2 mm. In addition, cyanoacrylate
zz Patch graft adhesive is bacteriostatic for Gram-positive
zz Tissue adhesives with bandage contact lens bacteria. Necrotic stroma or epithelium and other
(BCL), therapeutic penetrating keratoplasty debris must be removed from the base of the ulcer
zz Deep anterior lamellar keratoplasty before the adhesive is applied. A BCL is fitted after
zz Collagen crosslinking. the application. The adhesive is left in place until
it loosens spontaneously, or the bed becomes Q.2. What are the signs of healing and non
vascularized or keratoplasty is performed. healing corneal ulcer ?
Patch graft: The different types of patch graft that Ans. Already discussed in the text.
can be done includes following: Q.3. What is the interpretation of culture
zz Tenon’s patch graft: For peripheral ulcers, results and describe Jones criteria?
inexpensive, seals the defect by the fibroblastic Ans. Already discussed in the text.
response of the tenon’s tissue.
Q.4. What are the indications of corneal
zz Multilayered amniotic membrane graft
biopsy in the corneal ulcer?
(AMG): In cases of severe thinning or small
Ans. • If infectious keratitis is suspected clini
perforations.
cally and twice repeated microscopic
zz Tectonic patch graft: A small patch of corneal
evaluation of smears and culture results
graft, in perforations 3–5 mm in size.
are negative.
Therapeutic penetrating keratoplasty: A full • No clinical improvement is noted on
thickn ess graft is performed in perforations the initial broad-spectrum antibiotic
≥5 mm. The results of keratoplasty in acutely therapy.
infected or inflamed eyes are relatively poor, the • Certain cases of deep mycotic keratitis
risk of rejection and glaucoma is greater especially and intrastromal abscesses.
in larger grafts. In all these cases at least 0.5 mm
of clear tissue all around the infected area is to be Q.5. What is the grading of corneal ulcer?
excised to decrease the incidence of recurrence. Ans. Already discussed in the text.
Postoperative antimicrobial treatment is to be Q.6. What does the HEDS recommends about
continued. In fungal keratitis, postoperative viral keratitis?
topical steroids are to be used with caution. Ideally, Ans. • In HSV stromal keratitis—topical anti
steroids should be avoided until the culture report virals + steroids are less likely to fail
(suggesting free margins) is available or at least for treatment.
10–14 days. Surgery when performed with 8 mm or • No beneficial effect of systemic acyclovir
smaller diameter donor grafts have better results is there in HSV stromal keratitis
than larger grafts. Hence, penetrating keratoplasty • Oral acyclovir is beneficial in prevention
is to be considered early when fungal ulcers do of stromal keratitis/iritis in epithelial
not respond to antifungal medication. The results HSV keratitis
of penetrating keratoplasty for Acanthamoeba • Oral acyclovir is beneficial in preventing
keratitis are poor and surgery is to be considered the blinding sequele of HSV iridocyclitis
only in patients with gross corneal thinning or • Prophylaxis is beneficial in recurrent
perforation. ocular HSV or stromal HSV (400 mg bd)
Collagen crosslinking (CXL): CXL has direct [Note: Also refer to Table 12]
bactericidal activity (by oxidative damage) to the Q.7. What are the indications of systemic
pathogens and also cross linked corneas become therapy in case of corneal ulcer?
more resistant to degrading enzymes of organisms. Ans. Already discussed in the text.
Several studies have shown its effectiveness in
refractory keratitis. Q.8. What are the indications of surgical
intervention in case of corneal ulcer?
Ans. Already discussed in the text.
VIVA QUESTIONS
Q.9. Different stains used in keratitis.
Q.1. What is the role of hypopyon in etio Ans. • A modification of Gomori methenamine
logical diagnosis of corneal ulcer? silver stain may be helpful for the
Ans. Already discussed in the text. identification of fungal elements and
Table 12 Herpetic eye disease (HEDS)

Study group Intervention Recommendation
HEDS-1
Stromal keratitis not on Topical prednisolone phosphate Faster resolution and fewer treatment
steroids; on trifluridine TFT failures
Stromal keratitis on Oral acyclovir 400 mg 5 times a day No added benefit
steroids and TFT
HSV iridocyclitis on Oral acyclovir 400 mg 5 times a day Fewer patients recruited but potential
steroids benefits noted
HEDS-2
HSV epithelial keratitis trial Oral acyclovir 400 mg 5 times a No benefit in preventing subsequent
day for 3 weeks stromal keratitis/iridocyclitis
Acyclovir prevention trial Oral acyclovir 400 mg bd Reduced the risk of any form of ocular
herpes by 41% and stromal keratitis by 50%
Ocular HSV recurrence Studied the association between No association noted
study psychological and other forms of
stress with HSV recurrence
Abbreviation: TFT, trifluridine
Acanthamoeba cysts in corneal scrapings. Q.11. Antimicrobials for acanthamoeba kera-

Fungi and Acanthamoeba cysts stain titis.
black on a light green background. Ans. • B iguanide: PHMB 0.02% or chlorhexi
• The periodic acid-schiff (PAS) stain may dine 0.02%
also be used to visualize fungal elements as • Diamidine: Propamidine 0.1% or hexam-
well as Acanthamoeba especially in tissue idine 0.1%
sections. • Others: Miconazole and clotrimazole
• L actophenol cotton blue stain, which 1–2% suspension
is generally used for the microscopic • Usually a combination of buguanide and
examination of fungal cultures, has been diamidine is used and the treatment has
effectively used for the demonstration of to be continued for at least 6 months.
fungal elements and Acanthamoeba cysts
in corneal scrapings. BIBLIOGRAPHY
• Z iehl-Neelsen stain or its modification 1. Gupta N, Tandon R. Investigative modalities
(Kinyoun’s stain) is indicated for the in infectious keratitis. Indian J Ophthalmol.
detection of Mycobacteria and Nocardia 2008;56(3):209-13.
species, respectively. 2. Krachmer JH, Mannis MJ, Holland EJ. Cornea.
2nd edition. Philadelphia: Elsevier, Mosby.
Q.10. Prophylaxis for postpenetrating kerato 2005;1:955.
plasty herpetic keratitis. 3. Vajpayee RB, Namrata S. Corneal ulcers:
Ans. Oral acyclovir 400 mg bd for 1 year is diagnosis and management, 1st edition. Jaypee
prescribed. Brothers Medical Publishers (P) Ltd; 2008.
KERATOCONUS
Prafulla Kumar Maharana, Sapna Raghuwanshi, Manpreet Kaur, Namrata Sharma
INTRODUCTION History of Present Illness

Keratoconus is a disorder characterized by pro The onset and progression of the disease is
gressive corneal steepening (usually asymmetrical characteristic. The onset is usually at puberty. The
noninflammatory), most typically inferior to the disease has a rapid progression stage until the age of
center of the cornea, with eventual corneal thin 30 years. The rate of progression plateaus after this.
ning, induced myopia, and irregular astigmatism. After the age of 40 years the disease progression
It is the most common corneal ectatic disorder usually stops. The onset in Indian eyes may occur
seen in clinical practice. In postgraduate exam, earlier especially in cases with associated vernal
it can be given as a long case. keratoconjunctivitis (VKC). It is important to know
whether the keratoconus is progressive or not. In
HISTORY case of progression, the patient can be advised
to undergo corneal collagen crosslinking (CXL).
Epidemiology/Demography The best way to document progression is serial
Prevalence of keratoconus is about 54.5 cases per topography taking into consideration the change
100000.1-3 Keratoconus occurs in people of all in keratometry. However, progressive deterioration
races. There is no significant gender predilection. of BCVA, progressive decrease in corneal thickness
Keratoconus usually occurs bilaterally. Unilateral and a previously CL tolerant patient becoming
cases can occur but are rare (in the range of CL intolerant are certain other clinical clues of
2–4%).1-3 The age at onset is usually around the keratoconus progression.
age of puberty. It is more prevalent in the Asian
countries than in the West. Asian patients present Past History
at a younger age compared to the western world. Following past history must be recorded carefully.
Chief Complaints Contact Lens Wear

A case of keratoconus can present with following: If the contact lenses have not been fitted properly,
zz Progressive visual blurring and/or distortion the constant pressure or continual injury can lead
due to associated irregular astigmatism. It to scarring, its role in keratoconus progression is
may be associated with photophobia, glare, controversial. In addition, a better best-corrected
monocular diplopia, and ocular irritation. visual acuity (BCVA) with CL indicates good
zz Frequent change of glasses—the irregular prognosis after keratoplasty.
astigmatism is often difficult to correct with
glasses hence the patient keeps on visiting
Eye Rubbing
different optometrists.
zz Rarely a case may present with symptoms of Mechanical epithelial trauma leads to release of
associated diseases such as recurrent attacks cytokines that have a role in corneal weakening
of itching, eye rubbing (vernal keratocon and ectasia. In addition, rubbing can cause
junctivitis) and keratoconus is discovered mechanical trauma to the keratocytes and
during examination. increased hydrostatic pressure in the eye. Chronic
zz Often manifests during the late teens or early eye rubbing can lead to orbital fat atrophy that
twenties, then progresses slowly for the next often gives a clue about the cause of keratoconus
decade or two as the cornea scars and becomes in cases where a clear cut history of eye rubbing is
more elongated. not there.
Topography EXAMINATION
The patient might have been already a case of Visual Acuity
diagnosed keratoconus and the patient might have
undergone corneal topography several times. In Uncorrected visual acuity and BCVA must be
that case a serial recording of the keratometry, assessed in all cases. Refraction must be attempted
central corneal thickness (CCT), and thinnest in all such cases:
pachymetry must be done. Remember an increase
zz Scissoring of the red reflex on retinoscopy is
in keratometry by 1 D over a period of one year one of the earliest sign of keratoconus.
suggests progression and such cases require CXL
zz In presence of irregular astigmatism, visual
(few clinicians consider an increase of 0.5D per acuity with rigid gas permeable (RGP) lenses
6 months). may provide the BCVA. This is important
before surgical planning to know the visual
potential.
Ocular Surgery
Keratoconus can occur secondary to ocular Facial Appearance/Orbit
surgeries such as LASIK and radial keratotomy
(RK). Hence, any past refractive surgery must be Look for sign of orbital fat atrophy/oculo-digital
enquired. In few cases, a previous history of CXL sign suggestive of chronic eye rubbing.
maybe there.
Eyelid
Past Medical History Look for signs of allergic conjunctivitis. In
Keratoconus can be associated with certain ocular advanced keratoconus Munson’s sign, a V-shape
and systemic disorders. A careful history must be deformation of the lower eyelid when the eye is in
taken to rule out these disorders. downward position, can be elicited.
Ocular associations:
zz Floppy eyelid syndrome Conjunctiva
zz Leber’s congenital hereditary optic neuropathy Look for presence of papillae in tarsal conjunctiva.
zz Cone-rod dystrophy In India, keratoconus is often associated with VKC
zz Corneal granular dystrophy or allergic conjunctivitis. Signs of VKC include
zz VKC papillae, trantas dots (gelatinous thickening of
zz Refractive surgery limbus), limbal nodule, pigmentation and ropy
zz Trauma. discharge.
Systemic associations:
zz Down syndrome Cornea
zz Atopy-bronchial asthma, angioneurotic
The slit-lamp examination reveals following
edema, Marfan syndrome
signs.1-3
zz Mitral valve prolapse
zz Corneal thinning: The thinnest part of the
zz Rosacea.
cornea is usually located outside the visual
axis, and corneal thinning is a common sign
Family History preceding ectasia. Thinning is most commonly
A three-generation pedigree chart must be seen inferiorly (Fig. 1) or inferotemporally.
prepared. An autosomal dominant mode of zz Corneal ectasia: An eccentrically located
inheritance with variable expression has been ectatic protrusion of the cornea is noted in
suggested for keratoconus. Between 6 and 18% of keratoconus. The apex is usually inferior to
patients with keratoconus have a positive family a horizontal line through the pupillary axis
history.1-3 (Fig. 1). Corneal thinning from one-half to
Fig. 1: Ectatic protrusion of the cornea with thinning Fig. 3: Vogt’s striae
hemosiderin (Iron deposits) arc or circle line

seen around the cone base. The ring is formed
from hemosiderin pigment deposited deep
in the epithelium from the tear film onto the
cornea as a result of severe corneal curvature
changes induced by the disease and/or
due to modification of the normal epithelial
slide process. This ring is brown in color and
best appreciated with the cobalt blue filter
using a broad, oblique beam.1
zz Vogt’s striae: These are fine vertical lines
produced by compression of Descemet’s
membrane (Fig. 3), which tend to disappear
when physical pressure is applied on the
Fig. 2: Corneal scar cornea digitally or by gas permeable contact
lens wear. The lines are seen in the deep stroma
one-fifth of normal thickness is observed and Descemet’s membrane and are parallel to
in the apex of the protrusion. Three types of the axis of the cone.
cones can be seen in advanced keratoconus. zz Prominent corneal nerves: The increased
The round or nipple-shaped cone is more visibility of corneal nerves results from the
common. It is of smaller diameter. The center outward bowing and thinning of the ectatic
lies mostly inferonasally. These corneas are cornea.
more easily fit with contact lenses. The oval or zz Superficial and deep corneal opacities.
sagging cone is larger and lies predominantly zz Increased intensity of the corneal endothelial
inferotemporally. This cone is more often reflex: An endothelial reflex may appear at
associated with hydrops, scarring, and contact the peak of the cone due to the increased
lens fitting problems. When the ectasia concavity of the posterior corneal surface.
involves >75% of the cornea it is termed as a An annular dark shadow separates the bright
globus type of cone. reflex of the cone from the reflex of the
zz Corneal scarring (Fig. 2) corneal periphery. This shadow results from
zz Fleischer ring—in moderate and advance cases total internal light reflection induced by
of keratoconus, a Fleischer’s ring is a partial the corneal ectasia. It is best demonstrated
or complete annular line commonly seen at using the direct ophthalmoscope in a dilated
the base of the cone. This line is nothing but a pupil.
zz Sub-epithelial fibrillary lines: Bron et al. has reflex resembling a drop of honey or oil (sign of
described, white subepithelial fibrillary lines “Charleux”), in the reflection of the red reflex
in concentric bundles lying just inside the from a direct ophthalmoscope. This is one of the
Fleischer’s ring.1 These are best seen under earliest sign of keratoconus.
high magnification with a broad, oblique
slit beam. The pattern is characteristic of DIFFERENTIAL DIAGNOSIS
keratoconus and occurs in approximately one-
third of patients with this disease.1 Kindly see Table 1.
zz Corneal hyperesthesia can be detected early
in the course of the disease. Later the cone CLASSIFICATION
becomes relatively less sensitive. Keratoconus is classified based on morphology,
zz Rizzuti phenomena: This is demonstrated by disease evolution, ocular signs and index-based
a penlight shining on the temporal side of systems.
cornea or parallel to the iris plane. Normally,
the light rays illuminate the nasal limbal area. Morphology
In mild keratoconus, the ectatic cornea focuses
the light sharply inside the nasal limbus. In Classically, keratoconus has been classified into:
more advanced states, the light is focused at
zz Nipple: The cone has a diameter ≤5 mm, round
the nasal limbus and beyond. It is important morphology and is located in the central or
to remember that this response can also be paracentral cornea, more commonly in the
elicited in patients with refractive errors. inferonasal corneal quadrant. Correction with
zz Breaks in Descemet’s membrane have been contact lenses is normally relatively easy.
described in severe keratoconus, causing acute
zz Oval: The cone has a diameter >5 mm
stromal edema, known as hydrops (Fig. 4), and a paracentral to peripheral location,
sudden vision loss and significant pain. more commonly in the inferotemporal corneal
quadrant. Contact lens correction is more
Fundus Examination difficult.
zz Globus: The cone is located throughout 75% of
Fundus evaluation after mydriasis is essential for the cornea. Contact lens correction is a difficult
any concomitant fundus abnormality. challenge, except in very limited cases.
Charleux Sign Disease Progression

With a dilated pupil and a lens + 6 D positioned Amsler proposed the first keratoconus classifica
in front of the eye one can appreciate a dark tion based on the disease evolution.2,3 The details
reflex in the area of the cone with a central bright of classification is given in Table 2.
Curvature
Keratoconus is classified into following:
zz Mild: <45 D
zz Moderate: 45–52 D
zz Advanced: >52 D
zz Severe: >62 D.
INVESTIGATIONS
Keratometry
Keratometry mires in keratoconus are commonly
steep, highly astigmatic, irregular, and often
Fig. 4: Corneal hydrops appear egg-shaped (rather than circular or oval).
Table 1 Differential diagnosis of keratoconus

Characteristics Keratoconus PMD Keratoglobus TMD
Frequency Most common Less common Rare Rare
Laterality Usually bilateral Bilateral Bilateral Bilateral
Age at onset Puberty 20–40 years Usually at birth Middle-aged to elderly
Thinning Inferior Inferior band Maximum in Superior cornea
paracentral 1–2 mm wide periphery
CCT Reduced Usually normal May be normal Usually normal
Protrusion Thinnest at apex Superior to band Generalized Superior cornea
of thinning
Rizutti’s phenomenon Present Absent Present Absent
and Munson’s sign
Fleischer ring Present Sometimes None Absent
Scarring Common Only after Mild Superior cornea with
hydrops vascularization, lipid
deposition and inflammation
Vogt’s striae Common Sometimes Sometimes Absent
Abbreviations: CCT, central corneal thickness; PMD, pellucid marginal degeneration; TMD, Terrien’s marginal
degeneration
Table 2 Amsler-Krumeich classification of keratometry may be normal. Some patients

keratoconus with keratoconus do not exhibit these signs. The
disadvantages of keratometry in keratoconus is, it
Stage Description provides information about central 3 mm of cornea
1. Eccentric corneal bulging only, it is not useful in irregular astigmatism. Few
Myopia and/or astigmatism <5D clinicians perform central keratometry, followed
Corneal radius ≤48D by keratometry with the patient in upward gaze, to
Vogt’s striae identify the steepening in the inferior cornea but
No central opacity it is often difficult and inconclusive.
2. Myopia and/or astigmatism >5 D but <8 D
Corneal radius ≤53 D Videokeratography (VKG)
No central opacity
This is based on the Placido disk principle. It
Pachymetry ≥400 μ
provides qualitative contour information. In early
3. Myopia and/or astigmatism >8 D but <10 D cases, there will be an isolated area of smaller
Corneal radius >53 D ring spacing and distortion. As the keratoconus
No central corneal opacity worsens, the cornea becomes steeper; the
Pachymetry 200–400 μ ring spacing decreases overall and becomes
4. Refraction not possible increasingly irregular. Its disadvantage is it does
Corneal steepening >55.00 D not give accurate information about posterior
Corneal scarring curvature (cannot detect early keratoconus) and
Pachymetry <200 μ corneal thickness.
Inability to superimpose the central keratometric Orbscan

rings suggests irregular corneal astigmatism, It uses the principle of scanning slit combined
a hallmark of keratoconus. In early cases, with a Placido system. It provides reliable data
on anterior and posterior elevation and best-fit treated with keratoplasty. Other surgical treatment
sphere and a corneal pachymetry map. However, options include intracorneal rings segments,
the posterior curvature maps are based on corneal cross-linking, intra-ocular lens implants
assumptions and may not be 100% accurate. In or a combination of these.4-6
addition, it requires patient fixation for accuracy
of data that is difficult at times and data of central Nonsurgical Management
cornea (near point of fixation) is not accurate.
zz Spectacles: Spectacles are normally used in
early cases of keratoconus only. As the disease
Pentacam progresses, irregular astigmatism develops
This device uses a rotating Scheimpflug camera. and adequate visual acuity cannot be achieved
It provides reliable measurement of anterior with this type of visual correction.
and posterior corneal elevation and accurate zz Contact lens: Different contact lenses used for
measurement of corneal thickness. Pentacam treatment of keratoconus are soft toric lenses,
differs fundamentally from the Orbscan by the standard bicurved hard lenses, custom-back
way in which it takes image slices of the cornea. toric lenses, piggyback systems, hybrid lenses
The Orbscan takes vertical image slices that are (made of combined hard lens with a soft skirt),
separated from one another and have no common scleral lenses, and mini-scleral lenses.
point. Thus, the Orbscan cannot re-register for any —— Rigid gas permeable lenses: Rigid gas
eye movement that occurs while it is capturing the permeable (RGP) corneal lenses are
images. The Pentacam maintains the central point the lenses of first choice for correcting
(the thinnest point) of each meridian. Thus, during the irregular astigmatism. The aim is to
the examination, the software can re-register these provide the best vision possible with the
central points and eliminate the eye movement. maximum comfort so that the lenses can
This single feature makes the Pentacam’s be worn for a long period. The different
measurements 10 times more accurate. Thus, it fitting strategies of gas permeable contact
is largely independent of patient fixation with lenses are as follows:
better repeatability than orbscan. In addition, the Apical clearance: In apical clearance
central corneal curvature can be measured more fitting there is no bearing or touch in
accurately compared to orbscan. the apical area and the lens bearing
is in the periphery. Advantages
Pachymetry are reduced risk of scarring, whorl
keratopathy and erosions; the limita
Both ultrasonic and optical based devices
tion is that tightening at the periphery
(ASOCT) can be used to measure the pachymetry.
can hamper tear exchange and the
Measurement of corneal thickness is useful for
edge of the lens can come into the
diagnosis, documenting progression, and planning
visual axis, especially in cases with
treatment (see treatment section).
advanced ectasia. This strategy is very
rarely used now-a-days.
Ocular Response Analyzer Apical touch fitting technique is
The ocular response analyzer allows keratoconus characterized by providing primary

diagnosis and classification by assessing corneal lens support on the apex of the
hysteresis and resistance. cornea, in which the central optic
zone of the lens actually touches or
MANAGEMENT “bears on” the central cornea. The
advantage is better quality of vision
The treatment of keratoconus varies depending on but the problem is there can be heavy
the disease severity. Early cases are managed with bearing on the cornea resulting in
spectacles, mild to moderate cases are managed corneal scarring and intolerance over
with contact lenses, and severe cases can be long-term use.
The three-point touch fitting tech fluid before insertion in the eye. Treatment
nique is the most popular technique. has a high success rate when measured by the
In three-point touch fitting the ability to achieve satisfactory fit and impact
lens bearing is shared between the on VA. PROSE treatment can be an alternative
apex and the midperipheral cornea to PKP for patients with corneal ectasia who
that minimizes the risk of apical are CL intolerant. The BOSP is a fluid-filled
scarring. These lenses provide good scleral CL. These lenses rest on the sclera and
vision, better comfort and prolonged do not touch the cornea. There is a constant
wearing time and are hence the most pool of tears over the cornea, which acts as
preferred type of lenses. a liquid corneal bandage and avoids any
zz Piggyback systems: Consisting of the fitting a friction between the posterior surface of the
rigid gas permeable on top of a soft contact CL and the corneal apex. In addition, these
lens. The soft contact lens is used to improve lenses mask corneal surface astigmatism and
wearing comfort and provide a more regular improve best-corrected VA. Thus these lenses
area for the gas permeable contact lenses to are extremely useful in patients with advanced
sit, whereas the gas permeable contact lens is ectasia where the patients are intolerant to CL,
primarily used for providing adequate visual or immediate surgery is not possible, or when
acuity. the patient refuses surgery. The limitation of
zz Hybrid contact lenses (such as soft perm, the use of scleral lenses is high cost, reduced
solotica and synergeyes): Hybrid CL contain a tear exchange and difficult insertion-removal,
RGP center with a soft skirt. New-generation which require considerable practice. Overall,
hybrid CL provides higher oxygen permeability studies have shown a good outcomes with
and greater strength of the RGP/hydrogel these lenses.2,3
junction. These lenses are fitted with no or
minimal apical touch in the central cornea. The Surgical Management
lenses can be fitted on cones of any severity Current surgical options include:
but the problem with these lenses is they zz Corneal transplantation: Penetrating kerato
can cause hypoxia-related changes such as plasty, deep anterior lamellar keratoplasty.
vascularization and central corneal clouding. zz Intra-corneal ring segment insert: Intacs,
However, these lenses have not been widely Ferrara rings.
accepted as the current designs, because zz UVA/riboflavin corneal cross linkage (C3R).
they are generally more expensive than gas zz Thermokeratoplasty.
permeable lenses, do not normally provide zz Lenticular refractive surgery: Refractive lens
improved visual correction and wearing exchange with toric intraocular lenses, toric
comfort in comparison with gas permeable phakic intraocular lenses.
contact lenses.
zz Rose K Lenses (Rose K, Rose K2 XL and Rose Penetrating keratoplasty (PKP): PKP in kerato
K2 IC) are multicurve lenses with a small conus in comparison to other indications is
optical zone that snugly fits over the cone. The considered low risk in terms of graft rejection,
Rose-K CL provides greater comfort, better graft survival and postoperative complications.
quality of vision and requires less chair time The success rate is 90–95%. Visual recovery takes
in cases with keratoconus. The Rose K2 IC is several weeks/months, with full stabilization not
a large diameter, intralimbal lens that can be occurring until a year, after which time the sutures
used for large or oval cones. can be removed.
zz Scleral lenses: These lenses rest on the sclera Deep anterior lamellar keratoplasty (DALK):
and do not touch the cornea and limbus, DALK has several advantages over PKP (Table 3).
leaving a clear area between the CL and the In keratoconic eyes, the corneal endothelium
cornea. The advantages are good centration, is usually intact; with good cell counts even
stability and improved VA. The PROSE is a after cases of acute hydrops, hence DALK is the
non-fenestrated scleral CL that is filled with procedure of choice. The major disadvantage is
Table 3 Comparison of deep anterior lamellar keratoplasty (DALK) and penetrating keratoplasty (PKP)
Parameter DALK PKP
Indication Stromal opacification with healthy Both endothelial failure and stromal
endothelium opacification
Visual rehabilitation Early Delayed
Quality of vision Poor than PKP Best
Interface haze Affects vision None
Higher order abberations More Less
Postoperative astigmatism Less More
Wound strength Better Poor
Open sky procedure None Risk of expulsive hemorrhage
Intraocular surgery None Complications can occur
Tensile strength Better Poor
Steroid use Early taper Prolonged
Donor criteria Not stringent even nonoptical grade Only optical grade
can be used
Single donor multiple use Possible Not possible
Graft rejection Low-risk High-risk
Technique Difficult Easy
Learning curve Steep Less steep
corneal stromal rejection and migration of host residual stroma and interface haze. In addition, it
keratocytes to replace donor keratocytes resulting is a very time consuming process.7
in recurrence of the disease in graft. However,
stromal rejection can never lead to graft failure and Air-assisted DALK
recurrence in graft is extremely rare. Air-assisted lamellar keratoplasty involves
The goal of DALK is to achieve a depth of injection of air into the corneal stroma that helps
dissection as close as possible to DM. Various agents to achieve dissection as close as possible to DM.
have been used to create a plane of separation Archila first described the technique of air assisted
between DM and the deep stromal layers. These deep lamellar keratoplasty. Over a period of time,
include air, fluid, viscoelastic, microkeratome and many modifications of air-assisted DALK were
a femtosecond laser. The common techniques of tried. The big-bubble technique was described by
DALK are described below: Anwar and Teichmann and it is the most widely
used technique of DALK.7
Layer-by-layer Manual Dissection Big bubble DALK: The basic step of this technique
In this technique, after an initial partial trephina involves injecting air into the corneal stroma
tion of variable depth ranging from 50% to 70% of deep into a groove, which is created by trephining
corneal thickness, the stroma is removed using 60–80% of the stromal thickness. The air infiltrates
either a crescent knife or various types of lamellar the potential space between the deep stromal
dissectors. This is followed by layer-by-layer stromal layer and DM. The air anterior to DM creates a
removal, which is repeated multiple times to reach dome-shaped detachment of DM, which is then
as close as possible to the DM. The major limitations identified by a ring visible with the microscope.
of this technique are poor visual outcomes due to Once a plane of separation is achieved, the stromal
tissue can be easily excised. The main advantage Diamond-knife Assisted DALK
of this technique is that the quality of vision
Vajpayee et al. have described a new technique
achieved is as good as PK. However, the learning
of DALK that is easy to perform, provides visual
curve associated with this technique is very steep.
outcomes comparable to those of big-bubble
Often its repeatability is uncertain even with the
DALK, and can be performed in cases of extreme
most experienced surgeons. Inadvertent DM
corneal thinning or corneal scars. The essential
perforation can occur at any stage of the surgery.7
steps of this technique involve the use of a
diamond knife set at a depth of 30 μ less than the
Viscoelastic-assisted DALK pachymetry reading, to make a 2.0 mm incision
Melles et al. described a technique that uses a at the 11–12 o’clock position. This incision is then
viscoelastic injection rather than air to achieve extended circumferentially and centripetally to
a cleavage plane between DM from stroma. The take out the anterior stromal lamella, leaving a
depth of stromal dissection is guided by the “air to thin stromal bed. The authors found comparable
endothelium” interface which is seen by a specular outcomes to the big bubble DALK.4,7
light reflex localized at the tip of the blade. Once Epikeratophakia: It involves removing the corneal
the plane is achieved, the superficial stroma is epithelium from the host and then sewing onto
removed using trephine and lamellar dissection.7 the corneal stromal bed a previously cryolathed
lenticule of donor cornea. The procedure has
Hydrodelamination generally resulted in less favorable outcomes than
This technique was described by Sujita et al. In PKP with reports of failure of re-epithelialization,
this technique, saline solution is injected into poor BSCVA, stromal and lenticule inflammation
the cornea, which enhances the identification and opacification and interface haze. It is rarely
and removal of the deep stromal fibers. An performed now-a-days.
initial partial thickness corneal trephination is Intracorneal ring segment inserts (intacs and
done up to approximately 2/3 of the thickness ferrara rings): The technique consist of the
using vacuum trephines. However, it is difficult implantation of one or two polymethyl metha
to achieve an actual cleavage plane over DM by crylate segments in the corneal stroma to flatten
hydrodelamination.7 the central cornea and improve visual acuity,
contact lens tolerance and delay the need for
Femtosecond-assisted DALK corneal graft. It acts by its Arc-Shortening effect. It
is commonly used to treat mild to moderate cases
The femtosecond laser (FSL) computer-guided
of keratoconus, as normal corneal transparency
cuts allows precise, accurate and reproducible
and a minimum corneal thickness of 450 µm at the
placement of incisions at desired depths in the
site of the incision are required.
corneal stroma. Hence, it can be used to create
the initial cut at the desired depth to inject air Three types of rings are available: Intacs which
for the successful formation of big bubbles. In have a hexagonal cross-section and are placed
addition, it can be used to create corneal incisions more peripheral than ferrara rings which are
with customized graft edges and lamellar planes triangular/prismatic in shape. Recently, Intacs SK
for both donor and recipient corneas. Thus, FSL (SK—severe keratoconus) has been introduced for
can be utilized for creating customized graft host use in more severe forms of corneal ectasia. It has
interfaces, such as mushroom or Zigzag shaped two significant design modifications—a smaller
DALK. The greatest advantages are its accuracy of inner diameter of 6.0 mm compared with 6.8 mm
forming the bubble at the desired corneal depth of the standard intacs; and an elliptical cross
and its positive refractive outcomes due to the section compared with a hexagonal cross section
successful alignment of the donor and recipient of the standard Intacs.
zigzag or mushroom configurations. However, the The rings are inserted into the posterior stroma
major limitation is the cost and availability.7 (about 75% of corneal depth at the incision site)
in a quick outpatient technique performed under stable disease with good spectacle corrected visual
topical anesthesia. The circular intralamellar acuity.
pockets for the rings are created either using a
specially designed vacuum lamellar dissector or VIVA QUESTIONS
with the femtosecond laser. It is assumed that they
push out against the ectatic curvature peripherally Q.1. Refractive surgery in keratoconus.
flattening the peak of the cone centrally and Ans. Keratoconus as a contraindication to
returning the cornea to a more spherical shape. corneal surgical procedures such as laser
Intracorneal ring technology does not offer in situ keratomileusis (LASIK), photo
a cure for the condition but can very often refractive keratectomy (PRK), laser
produce a marked improvement in unaided and epithelial keratomileusis (LASEK), excimer
best corrected visual acuity and allow eyes to be laser phototherapeutic keratectomy (PTK).
corrected with spectacles and/or soft rather than
rigid lenses. Q.2. Complications associated with kera-
toconus.
Corneal collagen crosslinking with riboflavin Ans. Complications of keratoconus include cor-
(C3R) or corneal cross linkage (CXL): CXL using neal hydrops and corneal perforation.
riboflavin (vitamin B2)/ultraviolet A (UVA) [370 nm] Corneal hydrops is characterized by
light is a therapeutic modality that can halt and corneal edema due to seepage of aqueous
stabilize the keratoconic process. It increases humor through a tear in the Descemet’s
the corneal rigidity and biomechanical stability. membrane (DM). Corneal hydrops has
The success rate varies between studies but also been reported with PMD, TMD,
overall 60–70% cases shows some stabilization keratoglobus and post-LASIK ectasia. If
after CXL.2,4 The procedure involves removing not treated, resolution usually takes a long
the corneal epithelium in a 6–7 mm diameter time and occurs by endothelial sliding over
central zone followed by riboflavin 0.1% a period of 2–4 months. Medical manage
solution application and corneal radiation with ment consists of topical hypertonic drops,
ultraviolet-A light at 370 nm. Ultraviolet-A light topical steroids, prophylactic antibiotic
radiation activates riboflavin generating reactive drops and antiglaucoma medications.
oxygen species that induce covalent bonds However, persistent edema can cause
between collagen fibrils in the corneal stroma. complications such as corneal neovascular
The irradiation level at the corneal endothelium, ization, infection and corneal perforation.
lens and retina is significantly smaller than the Surgical intervention is often performed
damage threshold. It has been recommended not to shorten the duration of the disease.
to perform this technique in corneas thinner than Intracameral injection of air/isoexpansile
400 µm as toxic reactions could take place in the gases (C3F8/SF6) is the most commonly
corneal endothelium. In such cases hypotonic performed procedure. In the presence of
CXL have been tried with variable success. a large DM detachment or stromal clefts,
The CXL is largely safe except for the risk of ASOCT guided intrastromal drainage with
keratitis. No long-term problems in terms of loss of stab incisions; compressive sutures and
transparency of the cornea or lens have occurred even penetrating keratoplasty may have to
and endothelial counts have been unchanged be performed.
postoperatively. In addition, this technique has
been successfully used in combination with Q.3. What is Munson’s sign?
other surgery techniques, such as corneal ring Ans. See text.
segments. Q.4. What is Rizzuti’s sign?
Refractive lens exchange: Refractive lens exchange Ans. See text.
and toric phakic intraocular lens insertion may Q.5. What is posterior keratoconus?
be of some benefit in correcting myopia and Ans. Posterior keratoconus refers to a congenital
astigmatism in selected eyes with early/mild/ corneal anomaly in which the posterior
corneal surface protrudes into the stroma. Q.11. Topographic patterns in normal cornea.
It usually occurs in a localized area, but Ans. The topographic patterns of both the eyes
may be more diffuse. It is usually sporadic, of an individual often show mirror-image
unilateral, and is nonprogressive. Bilateral symmetry. This phenomenon is called
and familial cases do occur but are less enantiomorphism. Topographic patterns
frequent. The anterior corneal contour is seen in a normal eye are following; round,
usually unaffected. Frequently, scarring oval, superior steepening, inferior steepen
occurs in the stroma anterior to the ing, symmetric bow tie, symmetric bow tie
Descemet’s bulge. Scarring at the level of with skewed axes, asymmetric bow tie with
Bowman’s membrane and thinning of DM inferior steepening, asymmetric bow tie
with excrescences has been reported on with superior steepening, asymmetric bow
histopathology. It is considered a variant tie (AB) with skewed radial axes (SRAX)
of corneal mesenchymal dysgenesis. and irregular. Skewing of more than 30° is
Treatment usually is not necessary, described as significantly abnormal.
although occasionally keratoplasty is Q.12. What is KISA index?
indicated. Ans. Rabinowitz/Rasheed’s described KISA%
Q.6. Forme fruste KC (FFKC). to diagnose keratoconus. KISA% index is
Ans. The diagnosis of KC is a clinical one that is usually applied to the axial map. It uses four
aided by topography, while the diagnosis indices on the topography. It is calculated
of FFKC is topographic. It is a subclinical as:
disease and is not a variant of KC. Cornea (K) × (I – S) × (AST) × (SRAX) × 100
specialists define FFKC in two ways: KISA% =
300
1. Few consider FFKC is a normal cornea • K -central keratometric value in excess of
with the fellow eye is having keratoconic 47.2 D (i.e. K-47.2). If value is less then or
or there is a family history of KC. equal to 47.2, it is replaced by 1.
2. Few consider FFKC is an abnormal • I-S or inferior-superior asymmetry
cornea. Corneal topography or corneal • AST calculated from (Sim K1-Sim K2)
hysteresis (ORA) or both are abnormal • SRAX is calculated from 180—the angle
but there are no obvious clinical signs of between two steep axis above and below
keratoconus. the horizontal meridian (smaller of the
Q.7. Hypotonic CXL. two angles). To amplify any abnormality,
Ans. It has following features: the value 1 was substituted in the
• Used for thin corneas <400 μ (320–400 μ) equation whenever a calculated index
• Iso-osmolar uses riboflavin 0.1% solution has a value of <1
in 20% dextran while hypo-osmolar uses KISA% >100% is considered as highly
riboflavin 0.1% solution in 0.9% NaCl suggestive of keratoconus.
• Corneal thickness increases (hypotonic
solution) thus allows for safe CXL REFERENCES
• Results variable. 1. Krachmer JH, Feder RS, Belin MW. Keratoconus
and related non-inflammatory corneal thinning
Q.8. Difference between PMD and kerato-
disorders. Surv Ophthalmol. 1984;28:293-322.
conus. 2. Krachmer JH, Mannis MJ, Holland EJ (Eds).
Ans. See Table 1. Cornea. 2nd edition. Philadelphia: Elsevier,
Q.9. Systemic association and keratoconus. Mosby. 2005;1:955.
Ans. See discussion part. 3. Maharana PK, Dubey A, Jhanji V, Sharma
N, Das S, Vajpayee RB. Management of
Q.10. Amsler-Krumeich classification. advanced corneal ectasias. Br J Ophthalmol.
Ans. See Table 2. 2016;100(1):34-40.
4. American Academy of Optometry. Keratoconus. Keratoconus: A review. Contact Lens & Anterior
2008. pp. 1-13. Eye. 2010;33:157-66.
5. Espandar L, Meyer J. Keratoconus: Overview 7. Maharana PK, Agarwal K, Jhanji V, Vajpayee
and update on treatment. Middle East Afr J RB. Deep anterior lamellar keratoplasty
Ophthalmol. 2010;17:15-20. for keratoconus: a review. Eye Contact
6. Mi g u e l R o m e ro -Ji m é n e z a, Ja c i n t o Lens. 2014;40(6):382-9. doi: 10.1097/
Santodomingo-Rubido b, James S. Wolffsohn c. ICL.0000000000000076. Review
CORNEAL STROMAL DYSTROPHY

Shipra Singhi, Divya Agarwal, Prafulla Kumar Maharana, Rajesh Sinha
INTRODUCTION zz Photophobia: Photophobia is mild in case of

granular corneal dystrophy, type 1 (GCD1).
The corneal dystrophies are a group of non- More in case of GCD2 and macular dystrophy.
inflammatory, inherited, bilateral disorders of the zz Glare: Due to diffraction of light by the
cornea characterized by pathognomonic patterns opacities.
of corneal deposition and morphological changes zz Foreign body sensation: Due to recurrent
which are slowly progressive and not related to erosion or lesions extending up to epithelium.
environmental or systemic factors. The stromal zz Color haloes: Due to the deposits and corneal
corneal dystrophies primarily affect the stroma. edema.
Over time they often extend into the anterior zz Recurrent corneal erosion: Presents with mild
corneal layers and some may affect Descemet’s to extreme irritation, and discomfort that is
membrane and the endothelium. In exams, worse in the morning. It may be associated
corneal dystrophy can be given as both long and with severe pain due to epithelial defect and
short case.1-3 fluctuating vision or blurred vision due to
irregular astigmatism (uneven surface). These
are uncommon with GCD, common with MCD
HISTORY and frequent with LCD.
Demography zz Systemic features: Rarely, especially in cases
with LCD2, the patient may be referred from
Usually the patients are young adults, typically a physician with systemic symptoms. The
<45 years of age. The disease is usually bilateral, various systemic symptoms include dry, itchy
however the presentation may be unilateral.1-3 skin, laxity of the facial skin, edema over
feet, breathlessness, severe mask-like facial
Chief Complaints paresis with gradual onset of facial drooping,
protruding lips and pendulous ears (due to
Patient presents with following complaints: amyloid deposition and secondary muscular
zz Blurring of vision: Blurring of vision is rare dysfunction).
before the 5th decade of life in case of granular zz Usually asymptomatic in early stage and may
corneal dystrophy, (GCD) type 1. It appears be detected accidentally.
as early as 3 years of age in case of granular
corneal dystrophy type 2 and between 3 and History of Present Illness
9 years of age in case of macular corneal
dystrophy (MCD). Lattice dystrophy may Following points must be noted in present illness:
present as progressive loss of vision in first
decade in LCD1 and in 3rd or 4th decade in Age of Onset
LCD2. Schnyder’s Crystalline Dystrophy (SCD) zz Blurring of vision is rare before the 5th decade
is rare before 4th decade. of life in case of granular corneal dystrophy,
type 1. It appears as early as 3 years of age Past Surgical History

in case of granular corneal dystrophy, type 2
History of prior surgery; such as phototherapeutic
and between 3 and 9 years of age in case of
kerectomy (PTK), penetrating keratoplasty (PKP)
macular corneal dystrophy. Lattice dystrophy
or deep anterior lamellar keratoplasty (DALK)
may present as progressive loss of vision in
must be recorded carefully.
first decade in LCD1 and in 3rd or 4th decade
in LCD2. The onset of visual loss is insidious
and progressive in all types of stromal Past Medical History
dystrophy. A careful systemic history is required to rule out
zz Recurrent erosion can occur in all types of hypertension, diabetes and cardiac abnormality,
stromal dystrophy but it is most commonly renal failure, skin disease, and neuropathy.
seen in LCD. The onset of recurrent corneal Multiple systems may be affected in LCD2.
erosions (RCE) is in 1st–2nd decade in cases
of LCD, in cases of GCD can occur in early Family History
stages but episodes are usually mild and rare
Pedigree chart should be drawn to know the
(More patients with GCD2 or avellino corneal
hereditary pattern. Autosomal dominant pattern
dystrophy experience recurring erosions than
is found in LCD and GCD and autosomal recessive
patients with typical GCD1). MCD usually
in MCD.
presents with blurring of vision but RCE can
occur in 2nd to 3rd decade.
zz Corneal opacities in MCD usually first appear EXAMINATION
in adolescence but may become apparent Systemic Examination
anytime from early infancy to the sixth
decade of life. Affected individuals usually Systemic features associated with LCD2 are as
experience severe visual impairment before follows:
the fifth decade of life, usually in 2nd to 3rd zz Dry, itchy skin
decade, once opacities have coalesced and zz Laxity of the facial skin
the entire stroma becomes cloudy. Onset of zz Intermittent proteinuria (nephrotic syn-
corneal changes in lattice corneal dystrophy dromes)
type I usually occurs in the first decade of life, zz Severe mask—like facial paresis with gradual
although patients may remain asymptomatic onset of facial drooping, protruding lips and
for years. Signs of lattice dystrophy most often pendulous ears (due to amyloid deposition
appear in early childhood and become more and secondary muscular dysfunction)
prominent into the 2nd and 3rd decades. In zz Cranial and peripheral neuropathy
case of GCD the opacities usually appears in zz Peripheral polyneuropathy affects mainly
1st to 2nd decade but becomes symptomatic senses of vibration and touch.
only in 3rd to 4th decade. zz Carpal tunnel syndrome.
zz Autonomic disturbance includes orthostatic
Progression: Stromal dystrophies are progressive hypotension, cardiac conduction abnormal
diseases. To begin with the lesions are localized to ities, and dysfunction of perspiration.
stroma. With time the lesions progressively involve
the other layers too. The progression is relatively Ocular Examination
faster in MCD followed by LCD when compared
to GCD. Visual acuity: Uncorrected as well as corrected
visual acuity (VA) must be recorded in all cases.
This is important for planning treatment.
Past History
Eyeball: Lagophthalmos can be present in LCD2.
Similar episodes of recurrent corneal erosions
Lids: Dermatochalasis can be present in LCD2.
associated with pain and redness in past may be
there. Conjunctiva: Usually normal.
Cornea: On slit-lamp biomicroscopy following usually grouped into three basic morphologic
signs must be noted: types: drop-shaped, crumb-shaped, and ring-
zz Corneal size: Usually normal. shaped. The deposits can resemble crushed
zz Corneal shape: Keratoconus can be found breadcrumbs or snowflakes or popcorn or
in avellino as well as granular dystrophy Christmas tree. The overall pattern is ray- or disk-
otherwise normal. shaped. The granules are primarily located in the
zz Corneal opacity. central cornea. Initially, the stroma between the
zz Epithelium/anterior cornea: Shows atrophy opacities remains clear (Fig. 3). As the disease
and degeneration of basal epithelial cells and progresses individual lesions increase in size and
focal thinning or loss of Bowman layer. number and coalesce. Lesions extend into the
zz Stroma. deeper and more peripheral stroma but 2–3 mm
GCD1: In early stage of the disease fine dots and of the peripheral cornea usually remain free of
radial lines are seen in anterior stroma, these dots deposits. In more advanced disease, the inter
are opaque on focal illumination and translucent vening cornea develops a diffuse, ground-glass
on retoillumination (Figs 1 and 2). Opacities are appearance. Corneal sensation is variably affected.
GCD2: The three characteristic clinical signs of
avellino corneal dystrophy must be noted:
zz Anterior, stromal, discrete gray-white granular
deposits
zz Mid to posterior stromal lattice lesions
zz Anterior stromal haze.
Lattice lesions develop after the granular
deposits appear. With increasing age, the granular
lesions become larger and more prominent and
often coalesce to form linear opacities, especially in
the inferior cornea. The lattice lesions also become
more prominent with age (Fig. 4). Initially, they
are found in the mid and deep stroma and later
involve the entire stroma. The stromal haze is
seen only in patients with advanced granular and
Fig. 1: Multiple opacities resembling popcorn with lattice opacities and becomes more prominent
clear intervening cornea in a case of granular dystrophy with age.
Fig. 2: Multiple opacities resembling crushed bread Fig. 3: Multiple scattered opacities with clear inter
crumbs with clear intervening cornea in a case of vening cornea in a case of early granular dystrophy
granular dystrophy
MCD: In the early stages of the disease, ground- and can lead to RCE, glare and photophobia.
glasslike haze in the central and superficial There can be associated guttae. This opacification
stroma is seen. The epithelium is usually spared. usually involves the entire thickness of the cornea
With progression small, multiple, gray-white, by the second decade of life. The corneal thickness
pleomorphic opacities with irregular borders are is reduced.
seen (Fig. 5). The opacities are more superficial LCD1: In the early stages of the disease discrete
and prominent in the central cornea and are ovoid or round subepithelial opacities, anterior
deeper and more discrete in the periphery. The stromal white dots, and small refractile filamentary
intervening area between the opacities is hazy and lines may appear (Fig. 6). With progression of
gives a ground glass appearance. In later stages, disease the lesions can appear as small nodules,
the stroma is diffusely involved, Descemet’s dots, threadlike spicules, or thicker, radially
membrane takes on a gray appearance and careful oriented branching lines. The lines can extend
slit-lamp examination may show deposits over into deep stroma and may opacify. The lattice
Descemet’s membrane. When the opacities grow lines are typically refractile with a double contour
anteriorly, the corneal surface becomes irregular and a clear core on retroillumination (Fig. 7).
Fig. 4: Pleomorphic opacities with hazy intervening Fig. 6: Lattice lesions along with granular deposits in a
area between the opacities giving a ground glass case of avellino corneal dystrophy
appearance in a case of macular dystrophy
Fig. 5: Multiple anterior stromal white dots and small Fig. 7: Lattice lines are typically refractile with a
filamentary lines in a case of Lattice dystrophy double contour and a clear core on retroillumination
The filaments are opaque with irregular margins. zz ASOCT (Anterior segment OCT): Depth of the
They are radially oriented with dichotomous lesions can be determined by ASOCT. This is
branching near their central terminations. The extremely important for planning of surgery.
lines overlap one another, creating a latticework ASOCT can also provide the details about
pattern. The stroma between the lines and dots is the anterior segment structure and corneal
clear initially. In extreme cases, vascularization thickness.
may be present. Central corneal sensitivity also can zz Specular microscopy: It is done to evaluate
be decreased. In more advanced stages, the lattice corneal endothelium in cases of MCD.
depositions may also exhibit autofluorescence with A healthy endothelium is necessary for any
slit-lamp illumination using the cobalt-blue filter. lamellar keratoplasty. Involvement of endo
In LCD2 the fine lattice lines extend to the limbus. thelium requires a full thickness graft.
In LCD3 the lattice lines appears thick, and ropy zz Confocal microscopy:
and without any RCE. Lattice dystrophy Type IIIA —— GCD1: Hyper-reflective opacities.
has been described with similar corneal changes, —— GCD2: Findings are a combination of
but with recurrent erosions. A late onset lattice GCD1 and LCD. Reflective, breadcrumb-
dystrophy, Type IV, with deep stromal opacities like round deposits with well-delineated
has also been described. borders or highly reflective, irregular
zz Descemet membrane/Endothelium: trapezoidal deposits are present in the
—— Unaffected in GCD and LCD.
anterior stroma (similar to GCD1). Linear
—— In advanced stage of MCD descemet
and branching deposits with changing
membrane gets opacified and endothelial reflectivity are observed (similar to
guttate changes occur. LCD).
zz Corneal sensations: Corneal sensations are —— MCD: Blurred limited accumulations of
reduced in LCD and MCD and in advanced light reflective material are located in the
cases of GCD anterior part of the corneal stroma.
zz Corneal vascularization: It can occur in —— LCD1: Linear and branching structures
advanced cases of LCD. in the stroma with changing reflectivity
zz Corneal thickness: It is normal in GCD and
and poorly demarcated margins. Lines
LCD. Reduction of corneal thickness occurs in
must be differentiated from other similar
MCD.
images
Rest of the anterior segment is usually normal. —— LCD2: Prominent deposits, presumably
Tonometry: Increased IOP can be seen in case of amyloid, are seen contiguous to basal
LCD2 which may be associated with POAG. epithelial cells and stromal nerves. In
severely affected corneas, sub-basal and
Posterior segment: Usually normal. In case of
stromal nerves are reduced or absent.
media haze due to corneal opacity USG is done to
Anterior stroma shows fibrosis and
evaluate posterior segment.
abnormal extracellular matrix. Thick
Examination of fellow eye is important: Dystrophy anterior and mid-stromal filaments
is a bilateral disease. corresponding to lattice lines and thin
undulating structures are visible.
zz Corneal biopsy: When a corneal transplant
INVESTIGATIONS
is performed, the specimen is submitted for
zz Axial length, keratometry: All these cases may histopathology evaluation. Corneal biopsy is
undergo keratoplasty and cataract extraction the gold standard for confirming the diagnosis.
with intraocular lens implantation may be Various histochemical stains are specific for
required on a later date. Thus AL and Km must stromal dystrophies such as:
be done in all these cases. —— LCD: Congo red-pink to orange staining.
zz CCT (Central corneal thickness): It is reduced Dichromism-alternating red and green

in cases of MCD. color when viewed in green light through
polarized filter. Birefringence-yellow DIFFERENTIAL DIAGNOSIS

green color against black background
The differentiating features are summarized in
viewed through 2 rotating filters.
Table 1.
—— MCD: Stained with PAS, colloidal
iron, and alcian blue. Colloidal iron CLASSIFICATION OF CORNEAL
stain shows abnormal aggregation of
DYSTROPHY
glycosaminoglycan at sub-epithelial
Bowman’s and endothelial layer. The corneal dystrophies are classified as
—— GCD: Amorphous hyaline deposits stains following:2-5
with Masson trichrome as bright red
deposits. Congo red may show some foci Epithelial and Subepithelial Dystrophies
of amyloid in GCD2. zz Epithelial basement membrane dystrophy
zz Genetic analysis: It is not required routinely (EBMD)—majority degenerative, some C1
[kindly see the Viva section for details]. zz Epithelial recurrent erosion dystrophy (ERED)
zz Polymorphic amyloid degeneration (LCD2). C4 (Smolandiensis variant)
Table 1 Differential diagnosis of corneal stromal dystrophy

Feature Granular dystrophy Macular dystrophy Lattice dystrophy
Age of onset of lesions Ist decade Ist decade Ist decade
Age at presentation 30–40 3–9 10–20
(years)
Common presentation Loss of vision in advanced MC loss of vision MC recurrent erosion
cases (40–50 years) (10–30 years), erosion (10–20 years), loss of vision
(20–30 years)
Heredity Autosomal dominant Autosomal recessive Autosomal dominant
Opacity Breadcrumbs or Small, multiple, gray- Lattice lines-refractile with a
snowflakes or popcorn white, pleomorphic double contour and a clear core
opacities with sharp opacities with on retroillumination
border irregular borders Refractile tiny lines and dots,
Subepithelial spots in early
stages
Intervening stroma Clear Hazy Hazy
Deeper extension DM, endothelium free DM, endothelium DM, endothelium free
involved
Extension to limbus Usually absent Present Usually absent
Corneal sensation Reduced in late stages Reduced early Reduced early
Corneal thickness Normal Thinned Normal
Characteristic Masson trichrome Periodic acid—schiff, Periodic acid—schiff, congo red,
histochemical stains Colloidal iron, alcian thioflavine–T (fluorescence),
blue, metachromatic crystal violet (metachromasia),
dyes positive birefringence and
dichroism
Material accumulated Hyaline Glycosaminoglycans Amyloid
Abbreviations: DM, Descemets membrane; MC, most common
zz Subepithelial mucinous corneal dystrophy Granular corneal dystrophy, type 3

(SMCD) C4 (RBCD) = (Reis-Bücklers) C1
zz Mutation in keratin genes zz Macular corneal dystrophy (MCD) C1
zz Meesmann corneal dystrophy (MECD) C1 zz Schnyder corneal dystrophy (SCD) C1
zz Lisch epithelial corneal dystrophy (LECD) C2 zz Congenital stromal corneal dystrophy (CSCD)
zz Gelatinous drop-like corneal dystrophy C1
(GDLD) C1 zz Fleck corneal dystrophy (FCD) C1
zz Posterior amorphous corneal dystrophy
Bowman Layer Dystrophies (PACD) C3
zz Reis-Bücklers corneal dystrophy (RBCD)— zz Central cloudy dystrophy of francois (CCDF)
granular corneal dystrophy type 3 C1 C4
zz Thiel-Behnke corneal dystrophy (TBCD) C1, zz Pre-Descemet’s corneal dystrophy (PDCD) C4
potential variant C2
zz Grayson-Wilbrandt corneal dystrophy Descemet’s Membrane and
(GWCD) C4.
Endothelial Dystrophies
Stromal Dystrophies zz Fuchs’ endothelial corneal dystrophy (FECD)
zz TGFBI corneal dystrophies C1, C2 or C3
—— Lattice corneal dystrophy zz Posterior polymorphous corneal dystrophy
Lattice corneal dystrophy, TGFBI type (PPCD) C1 or C2
(LCD) zz Congenital hereditary endothelial dystrophy 1
Classic lattice corneal dystrophy (CHED 1) C2
(LCD1) C1, variants (III, IIIA, I/IIIA, zz Congenital hereditary endothelial dystrophy 2
IV) are C1 (Table 2) (CHED 2) C1
Lattice corneal dystrophy, gelsolin zz X-linked endothelial corneal dystrophy
type (LCD2) C1, this is not a true (XECD) C2.
corneal dystrophy but is included
here for ease of differential diagnosis Evidential Categories for IC3D Classification
—— Granular corneal dystrophy C1
Granular corneal dystrophy, type 1 zz Category 1 (C1): A well-defined corneal

(classic) (GCD1) C1 dystrophy in which the gene has been mapped
Granular corneal dystrophy, type 2 and identified and specific mutations are
(granular-lattice) (GCD2) C1 known.
Table 2 Differentiating features between LCD1, LCD2, and LCD3

Features Type I Type II (Meretoja) Type III
Inheritance AD AD AR
Age of onset <10 years 20–35 years >40 years
Visual acuity Poor after age 40 Good until age 65 Impaired after age 60
Recurrent erosions Frequent Infrequent None
Cornea • Numerous delicate lines • Few thick lines • Thick lines
• Many amorphous deposits • Few amorphous deposits
• Periphery clear • Extend to periphery
Systemic involvement None Systemic amyloidosis (skin, None
arteries and other organs)
Face Normal Facial paresis and Normal
blepharochalasis after age 40
Abbreviations: AD, autosomal dominant, AR, autosomal recessive; LCD, lattice corneal dystrophy
zz Category 2 (C2): A well-defined corneal Surgical Management

dystrophy that has been mapped to one or It depends on the depth of the opacities (Table 3)
more specific chromosomal loci, but the and described below:
gene(s) remains to be identified.
Superficial opacities: For superficial opacity
zz Category 3 (C3): A clinically well-defined
following options are available:
corneal dystrophy in which the disorder zz Epithelial scraping
has not yet been mapped to a chromosomal zz Superficial keratectomy
locus. zz Lamellar keratoplasty
zz Category 4 (C4): This category is reserved for zz Phototherapeutic keratectomy (PTK) with the
a suspected new, or previously documented, argon-fluoride excimer laser.
corneal dystrophy, where the evidence for it
Deep stromal lesions and significant visual loss:
being a distinct entity is not yet convincing. Deep anterior lamellar keratoplasty (DALK) or
penetrating keratoplasty. The comparison between
TREATMENT the two techniques have been described in Table 3.
Medical Management MCD
Medical management consists of following: zz Keratoplasty is required earlier in MCD.
zz Photophobia: Tinted cosmetic lenses for zz DALK carries a higher risk of failure due to
photophobia endothelial involvement (often there is sub-
zz Recurrent corneal erosion: Patching, hypertonic clinical involvement of endothelium without
agents, artificial tears, or a therapeutic contact any clinical evidence). Hence, few surgeons
lens. prefer PKP to DALK.
Table 3 Comparison of deep anterior lamellar keratoplasty (DALK) and penetrating keratoplasty (PKP)
Parameter DALK PKP
Indication Stromal opacification with healthy Both endothelial failure and
endothelium stromal opacification
Visual rehabilitation Early Delayed
Quality of vision Poor than PKP Best
Interface haze Affects vision None
Higher order aberrations More Less
Postoperative astigmatism Less More
Wound strength Better Poor
Open sky procedure None Risk of expulsive hemorrhage
Intraocular surgery None Complications can occur
Globe strength Better Poor
Steroid use Early taper Prolonged
Donor criteria Not stringent even nonoptical Only optical grade
grade can be used
Single donor multiple use Possible Not possible
Graft rejection Low-risk High-risk
Technique Difficult Easy
Learning curve Steep Less steep
Abbreviations: DALK, deep anterior lamellar keratoplasty; PKP, penetrating keratoplasty
zz Recurrence less common, appear 1.5–11 years Q.2. Describe different types of histochemical
after surgery. The earliest site of recurrence is stains?
graft host junction. Ans. See investigation section.
Q.3. Differentiate between GCD, MCD and,
GCD
LCD?
zz PTK: Used for superficial lesion and recurrence Ans. See Table 1.
zz Endothelium is usually uninvolved hence;
DALK is preferred over PKP in deeper involve Q.4. Differentiate between dystrophy and
ment. The success rate for PKP is around 85% degeneration?
at 1 year. Ans. See Table 4.
zz Recurrence rate is higher than MCD, can recur
between 1 and 19 years. Q.5. What is BIGH3 gene?
Ans. Keratoepithelin (Transforming growth
LCD factor, beta-induced, 68kDa) is a protein
which in humans is encoded by the TGFBI
zz Endothelium is usually uninvolved hence;
gene (initially called BIGH3, BIG-H3),
DALK is preferred over PKP in deeper
locus 5q31. Keratoepithelin produced in
involvement.
zz Recurrence is very common, usually occurs superficial epithelial cells and it has a role in
2–14 years after surgery. modulating cell adhesion. Mutation of this
gene causes accumulation of this product
in abnormal deposits. This is associated
VIVA QUESTIONS
with several corneal dystrophy such as
Q.1. Differentiate between LCD1, LCD2, and GCD1, Lattice dystrophy, Avellino, and
LCD3? Reis-Buckler (remember the mnemonic
Ans. See Table 2. GLARe]
Table 4 Difference between dystrophy and degeneration

Dystrophy Degeneration
A condition where cells have some inborn defects A condition where normal cells of tissue undergo
due to which pathological changes may occur during some pathological changes under influence of some
passage of time abnormal circumstances
Family history positive Family history absent
Hereditary (except: cogan’s) No heritance pattern
Usually bilateral and symmetrical Usually unilateral; if bilateral asymmetrical
Onset—early life, slowly progressive Onset-middle life or later, progressive
Located centrally Located peripherally or at least eccentrically
To begin with affect a particular layer of cornea Usually not restricted to a single layer
No vascularization (Except LCD) May be accompanied by vascularization
No role of environmental factors Environmental factors have a role in pathogenesis
Usually not associated with any ocular disease May be secondary to some ocular disease
Not associated with systemic disease May be associated with systemic disease
Examples—Meesman’ dystrophy, lattice dystrophy, Examples—BSK, SND, spheroidal deg
Fuch’s dystrophy
Abbreviations: BSK, band shaped keratopathy; LCD, lattice corneal dystrophy; SND, Salzmann nodular
degeneration
Q.6. Classify MCD? and a femtosecond laser. The common

Ans. MCD is classified into three phenotypic techniques of DALK are described below:6
variants based on the reactivity of the serum • L ayer-by-layer manual dissection: In
and corneal tissue to an antibody that this technique, after an initial partial
recognizes sulfated epitopes on antigenic trephination of variable depth ranging
keratan sulfate (AgKS). from 50%–70% of corneal thickness, the
• Type I has no detectable antigenic stroma is removed using either a crescent
keratan sulfate knife or various types of lamellar dis
• Type IA the serum lacks detectable anti sectors. This is followed by layer-by-
genic keratan sulfate, but the keratocytes layer stromal removal, which is repeated
react with antibodies to keratan sulfate. multiple times to reach DM as close as
• Type II: All the abnormal accumulations possible. The major limitations of this
react positively with AgKS and the serum technique are poor visual outcome due
has normal or lower levels of AgKS. to residual stroma and interface haze.
• Clinically they are indistinguishable from In addition, it is a very time consuming
each other. process.
Q.7. Associated ocular and systemic findings • Air-assisted DALK: Air-assisted lamellar
with LCD2: keratoplasty involves injection of air
Ans. Ocular into the corneal stroma that helps to
• Corneal hypothesia achieve dissection as close as possible
• D ermatochalasis (due to amyloid to DM. Archila first described the
deposition and secondary muscular technique of air assisted deep lamellar
dysfunction) keratoplasty. Over a period of time, many
• Lagophthalmos modifications of air-assisted DALK were
• POAG tried. The big-bubble technique was by
Systemic Anwar and Teichmann and it is the most
• Dry, itchy skin widely used technique of DALK.
• Laxity of the facial skin Big bubble DALK: The basic step of this
• Intermittent proteinuria (nephrotic syn- technique involves injecting air into
dromes) the corneal stroma deep into a groove,
• C ardiac conduction abnormalities, which is created by trephining 60–80% of
orthostatic hypotension, perspiration the stromal thickness. The air infiltrates
dysfunctions the potential space between the deep
• S evere mask-like facial paresis with stromal layer and DM. The air anterior to
gradual onset of facial drooping, DM creates a dome-shaped detachment
protruding lips and pendulous ears (due of DM, which is then identified by a ring
to amyloid deposition and secondary visible with the microscope. Once a plane
muscular dysfunction). of separation is achieved, the stromal
tissue can be easily excised. The main
Q.8. Advantage of lamellar keratoplasty over advantage of this technique is that the
full thickness penetrating keratoplasty quality of vision achieved is as good as PK.
Ans. See Table 3. However, the learning curve associated
Q.9. Different techniques of DALK? with this technique is very steep. Often its
Ans. The goal of DALK is to achieve a depth repeatability is uncertain even with the
of dissection as close as possible to DM. most experienced surgeons. Inadvertent
Various agents have been used to create DM perforation can occur at any stage of
a plane of separation between DM and the surgery.
the deep stromal layers. These include • Viscoelastic-assisted DALK: Melles et al.
air, fluid, viscoelastic, microkeratome described a technique that uses a
viscoe lastic injection rather than air technique involve the use of a diamond
to achieve a cleavage plane between knife set at a depth of 30 μ less than
DM from stroma. The depth of stromal the pachymetry reading, to make a
dissection is guided by the “air to 2.0 mm incision at the 11–12 o’clock
endothelium” interface which is seen by position. This incision is then extended
a specular light reflex localized at the tip circumferentially and centripetally to
of the blade. Once the plane is achieved, take out the anterior stromal lamella,
the superficial stroma is removed using leaving a thin stromal bed. The authors
trephine and lamellar dissection. found comparable outcomes to the big
• Hydrodelamination: This technique bubble DALK.
was described by Sujita et al. In this Q.10. Genetics of stromal dystrophies.
technique, saline solution is injected Ans. • G ranular dystrophy-AD TGFBI (5q31)
into the cornea, which enhances the corneal dystrophy.
identification and removal of the deep • Macular dystrophy (MCD): Autosomal
stromal fibers. An initial partial thickness recessive, chromosome 16 (16q22.1), a
corneal trephination is done up to mutation in a new carbohydrate sulfo
approximately 2/3 of the thickness using transferase gene (CHST6) has been
vacuum trephines. However, it is difficult identified as the cause of macular
to achieve an actual cleavage plane over dystrophy.
DM by hydrodelamination. • Lattice dystrophy: TGFBI gene-related
• F emtosecond-assisted DALK: The dystrophy with two different types, both
femtosecond laser (FSL) computer- representing Category 1 (C1).
guided cuts allows precise, accurate and Lattice corneal dystrophy type II is not a true
reproducible placement of incisions at corneal dystrophy. It part of the systemic
desired depths in the corneal stroma. disorder familial amyloid polyneuropathy
Hence, it can be used to create the initial Type IV (Finnish type), also known as
cut at the desired depth to inject air for Meretoja’s syndrome. Nearly all cases are
the successful formation of big bubbles. bilateral, progressive and usually inherited
In addition, it can be used to create as an autosomal dominant trait and it is
corneal incisions with customized graft due to single amino acid substitution in the
edges and lamellar planes for both donor plasma protein gelsolin, the consequence
and recipient corneas. Thus, FSL can be of a single nucleotide guanine to adenine
utilized for creating customized graft host change on chromosome 9q 32-34. Lattice
interfaces, such as mushroom or Zigzag corneal dystrophy type III is an autosomal
shaped DALK. The greatest advantages recessive disorder. The gene has not yet
are its accuracy of forming the bubble at been mapped. Lattice corneal dystrophy
the desired corneal depth and its positive type IIIA—has an autosomal dominant
refractive outcomes due to the successful pattern, and the clinical findings are due to
alignment of the donor and recipient a mutation at 5q31(Pro501Thr; Ala622His;
zigzag or mushroom configurations. His626Ala). The lattice corneal dystrophy
However, the major limitation is the cost type IV is associated with a Leu527Arg
and availability. mutation in the βig-h3 and is a dominant
• Diamond-knife assisted DALK: Vajpayee form of late-onset, deep lattice dystrophy.3-5
et al. have described a new technique of Q.11. Dystrophies associated with kerato-
DALK that is easy to perform, provides conus.
visual outcomes comparable to those of Ans. Keratoconus associated with other corneal
big-bubble DALK, and can be performed dystrophies.
in cases of extreme corneal thinning or In a study by Cremona et al. 51 patients
corneal scars. The essential steps of this manifested typical signs and topographic
evidence of keratoconus associated with Holland EJ. Cornea, 2nd edition. Philadelphia:
another corneal dystrophy.7 These dystro Elselvier Mosby; 2005.
phies were 3. Dighiero P, Niel F, Ellies P, et al. Histologic
• Fuchs dystrophy (most common)—52.9% phenotype-genotype correlation of corneal
• Anterior basement membrane dystro- dystrophies associated with eight distinct
phy—25.5% mutations in the TGFBI gene. Ophthalmology.
• P osterior polymorphous dystrophy 2001;108:818-23.
—13.8% 4. Dighiero P, Drunat S, D’Hermies F, et al. A novel
variant of granular corneal dystrophy caused
• Combination of Fuchs dystrophy and
by association of 2 mutations in the TGFBI
anterior basement membrane dystrophy
gene-R124L and DeltaT125-DeltaE126. Arch
—5.8%
Ophthalmol. 2000;118:814-8.
• Granular dystrophy—2% 5. Mashima Y, Yamamoto S, Inoue Y, et al.
Few case reports have reported keratoconus Association of autosomal dominantly inherited
in association with macular dystrophy and corneal dystrophies with BIGH3 gene mutations
avellino dystrophy also. in Japan. Am J Ophthalmol. 2000;130:516-7.
6. Maharana PK, Agarwal K, Jhanji V, Vajpayee
REFERENCES RB. Deep anterior lamellar keratoplasty for
1. Brad Bowling. Kanski’s Clinical ophthalmology: keratoconus: a review. Eye Contact Lens.
A systematic approach, 8th edition. Edinburgh: 2014;40(6):382-9.
Elsevier; 2015. 7. Cremona FA, Ghosheh FR, Rapuano CJ, Eagle
2. De Sousa LB, Mannis MJ. The Stromal RC Jr, Hammersmith KM, Laibson PR, Ayres BD,
Dystrophies. In: Krachmer JH, Mannis MJ, Cohen EJ. Cornea. 2009;28(2):127-35.
FUCHS’ ENDOTHELIAL CORNEAL DYSTROPHY

Sapna Raghuwanshi, Ritu Nagpal, Prafulla Kumar Maharana, Namrata Sharma
INTRODUCTION Asian countries compared to the western world.

Females are affected more than males (corneal
Fuchs’ endothelial corneal dystrophy (FECD) is guttae 2.5 times and corneal edema 5.7 times
the most common corneal endothelial dystrophy more than males; overall 4:1 ratio).1
seen in clinical practice. It is characterized by
bilateral, noninflammatory, progressive loss of Chief Complaints
corneal endothelium that ultimately results in
corneal decompensation and loss of vision. In Presenting features may depend upon the stage of
exams, it is given as a long case. Most of the time the disease. Patient may presents with following
complaints:
it may be a case of corneal decompensation
zz Early stage: Blurring of vision (initially in
following cataract surgery with evidence of FECD
morning gradually improving as the day
in the other eye or it may be a case of operated
passes)
corneal graft in one eye for corneal decompensa
zz As the disease progresses: Mild blurring due
tion with evidence of FECD in the other eye.
to stromal edema, glare, and colored halos
around lights (due to corneal edema) can
HISTORY occur.
Demography
zz Late stages: Loss of vision, recurrent attacks of
redness and pain due to epithelial edema and
The FECD is a slowly progressive disease affecting bullae rupture.
persons between 5th and 7th decade. The onset of zz Advanced stages: Loss of vision but without any
the disease occurs around one decade earlier in pain or photophobia due to corneal scarring.
History of Present Illness Cornea: The findings in cornea depends upon the
stage of the disease.
The natural course of the disease is quite
zz Stage 1 (stage of corneal guttae)
characteristic. The onset is gradual. In early
—— Central corneal guttae: It appears as
stages there is blurring of vision in morning that
gradually improving as the day passes. As the tiny dark spots (Fig. 1) on the posterior
disease progresses the blurring of vision becomes corneal surface on direct illumination.
persistent and symptoms of pain, photophobia, Specular reflection also reveals dark spots
watering may appear due to epithelial edema. and disruption of regular endothelial
There may be repeated exacerbations of the mosaic. In retroillumination the guttae
symptoms associated with episodes of epithelial appears as dewdrops.
—— As the disease progresses, guttae spread
bullae formation-rupture-healing cycle. In
advanced cases a pannus or fibrosis forms that peripherally and coalesce centrally along
leads to resolution of symptoms. with pigment dusting on the endothelium.
This characteristically gives the appear
Past History ance of “beaten metal appearance”.
—— As the disease progresses the Descemet’s
Past medical history must include diseases such
as diabetes mellitus, hypertension, tuberculosis, membrane becomes thickened and
bronchial asthma. These diseases may not be irregular.
related directly to FECD but are important for
zz Stage 2 (stage of corneal stromal edema)
—— Corneal edema initially appears in the
surgical planning.
posterior stroma, which is best seen with
Past Surgical History sclerotic scatter as a fine gray haze.
—— Vertical wrinkles or striae in Descemet’s
Quite often a case may present with persistent
membrane appear due to swelling of the
corneal edema following cataract surgery and
corneal stroma.
a careful examination of the other eye reveals
—— Progressive stromal edema results in a
signs of FECD. Thus history of any recent
ground-glass opacification with marked
intraocular surgery must be noted. In addition,
the postoperative best corrected visual acuity is thickening of the central cornea (Fig. 2).
an important parameter while considering for
zz Stage 3 (stage of corneal epithelial edema)
—— Multiple epithelial microcysts that may
keratoplasty and its visual prognosis.
coalesce to form bullae (Fig. 3).
—— Rupture of bullae leads to epithelial
EXAMINATION
erosions and fingerprint lines following
General Examination healing of such lesions.
A thorough general examination must be carried
out to look for any systemic condition that may
need attention before surgical planning.
Ocular Examination
Visual acuity: It depends on the stage of the
disease and severity of corneal edema (see chief
complaint).
Eyeball: Usually normal.
Eyelid: Usually normal. There may be blepharo
spasm in presence of corneal epithelial defect as
a consequence of ruptured bullae.
Conjunctiva: Conjunctival congestion and watery
discharge can be there in presence of epithelial
defect. Fig. 1: Central corneal guttae in stage 1 of FECD
Fig. 2: Progressive stromal edema with ground-glass Fig. 4: Stromal scarring in stage 4 FECD
opacification in FECD stage 2
A case of FECD must be differentiated from
following:
zz Hassall-Henle bodies
zz Central herpetic disciform keratitis-KP present
zz Aphakic or pseudophakic bullous keratopathy
zz Congenital hereditary endothelial dystrophy
(CHED)
zz I r i d o c o r n e a l e n d o t h e l i a l s y n d ro m e
(Chandler’s syndrome) (ICE)
zz Posterior polymorphous corneal dystrophy
(PPCD).
Fig. 3: Bullae formation and progressive stromal Hassall-Henle Bodies

edema in stage 3 FECD They are present in 70% of the population over
40 years old. Resembles guttae of FECD but are
zz Stage 4 (stage of scarring) located only in the peripheral cornea and are not
—— Avascular subepithelial fibrous scarring associated with progressive visual loss or corneal
occurs between the epithelium and edema.
Bowman’s membrane. Scarring leads to
resolution of symptoms but visual acuity Central Herpetic Disciform Keratitis
further deteriorates due to irregularity of Differentiating features are:
corneal surface (Fig. 4). zz Presence of keratic precipitates (KP’s)
—— Peripheral superficial corneal neovas-
zz Respond to steroids
cularization. zz Presence of scarring (herpetic footprints).
Anterior chamber/sclera/iris/pupil/fundus are
usually within normal limit. Shallow AC with Aphakic or Pseudophakic Bullous
angle closure glaucoma have been reported in few Keratopathy
cases. Following are the differential features:
Lens: Carefully examine the lens for presence of zz History of cataract surgery often with signs of
cataract. Detection of cataract is important for complicated surgery
management of FECD. zz Other eye will be normal.
CHED/ICE/PPCD cells (polymegethism), CV values

between 0.22 and 0.31 are considered
See Table 1.
normal. CV values from 0.32 to 0.40
are elevated, and CV values above
INVESTIGATION
0.40 are abnormal
zz Specular microscopy: Specular microscopy can Percentage of hexagonal cells (HEX)—
reveal following changes: in a normal endothelium, more

—— Qualitative parameters-pleomorphism than 60% of the endothelial cells are
(variation in shape, indicates disruption hexagonal. In FECD it is <60%
in the regular hexagonal pattern of the zz Corneal pachymetry: Measurement of central
endothelium), polymegathism (variation corneal thickness is important for diagnosis
in size, indicates injury to endothelium) (in doubtful and early cases) and planning of
—— Quantitative parameters: treatment.
Reduced endothelial cell density in zz Confocal microscopy: In presence of corneal
mm2 edema, confocal microscopy is the best tech
Increased coefficient of variation nique for evaluation of corneal endothelium
(CV)-CV represents the degree of zz Anterior segment optical coherence tomography
variation in the sizes of the endothelial (ASOCT): It can provide the details of anterior
Table 1 Differential diagnosis of FECD

Parameters FECD PPMD CHED (type 1) ICE
Age of onset 40s to 50s Teens to 20s Birth to 10 years Young adult
Laterality Bilateral Bilateral Bilateral Unilateral
Sex predilection F>M F=M F=M F>M
Heredity AD AD AD No
Basic defect Attenuation and Epithelialization of Mutation of solute Abnormal
reduced number of endothelium carrier family 4, proliferation of
endothelial cells sodium borate endothelium
transporter member
11–SLC4A11
Corneal findings Guttae, stromal Vesicles, bands, Marked corneal Fine, guttae-
thickening, epithelial diffuse opacities, thickening and like changes,
edema, sub-epithelial plaques at opacification, ‘hammered
fibrosis Descemet’s endothelium rarely silver’
membrane visible
Other ocular Increased intraocular Iris atrophy/ Usually none Iris atrophy,
abnormalities pressure, narrow corectopia, broad iris nodules,
angles peripheral synechiae, glaucoma in
glaucoma 25% 80–100%
Progression Progressive Minimal Progressive Relentless
Specular microscopy Polymorphism Focal change, endo Not possible Diffuse
Polymegathism thelial cells usually changes, ICE
Decreased enlarged but count is cell
endothelial cell count usually normal
Abbreviations: CHED, congenital hereditary endothelial dystrophy; FECD, Fuch’s endothelial corneal dystrophy;
ICE, Irido-corneal endothelial dystrophy; PPCD, posterior polymorphous corneal dystrophy
chamber in presence of an edematous cornea. Endothelial Keratoplasty

In addition corneal thickness and level of
Currently the treatment of choice. The different
scarring can also be detected.
techniques of endothelial keratoplasty are as
zz Ultrasonography (USG): USG is done to rule follows:
out any posterior segment pathology before zz Descemet’s stripping endothelial keratoplasty
proceeding for keratoplasty. (DSEK): The donor tissue (consisting of
DM-endothelium complex and some stroma)
MANAGEMENT is prepared using manual technique.
The management of FECD includes following:2,3 zz Descemet’s stripping automated endothelial
keratoplasty (DSAEK): Similar to DSEK
Medical Management except the donor tissue is prepared using a
zz Corneal edema microkeratome.
—— Topical hypertonic saline solutions and
zz Descemet’s membrane endothelial keratoplasty
ointments: It artificially raises the osmo (DMEK): Donor tissue is consist of only
lality of the tear film and dehydrates DM-endothelium complex and no stroma.
the cornea by drawing fluid from the Technically difficult than DSAEK but visual
epithelium and anterior stroma. results are better than DSAEK.
—— Dehydration of the cornea by a blow dryer
in the morning or throughout the day can

Penetrating Keratoplasty
decrease the symptoms in early stages. A full thickness graft is indicated in presence of
—— Cycloplegics and nonsteroidal anti- corneal scar or the surgeon lacks the expertise.
inflammatory agents are useful in
diminishing corneal pain from bullous FECD with Cataract
keratopathy. Senile cataract is commonly seen in cases of FECD
—— Reduction of intraocular pressure: Use
due to the common age group affected. It is often
of intraocular pressure-lowering medi a dilemma to decide if only cataract surgery is
cations may reduce corneal edema in enough or the patient needs a triple procedure.
patients with elevated or even normal Although there are no universal guidelines, most
intraocular pressure. corneal surgeons follow following approach:
zz Recurrent erosion zz Only Cataract with IOL
—— Bandage contact lenses: Use of therapeutic 2
—— Specular count >1000 cells/mm
so contact lenses help in relieving the —— CCT <600 μm
pain from recurrent epithelial erosions, —— Good corneal clarity to allow cataract
while decreasing irregular astigmatism surgery
in cases that have progressed to bullous zz Triple procedure
keratopathy —— Specular count <800 cells/mm
2
—— Anterior stromal puncture/amniotic
—— CCT >640 μm
membrane graft/phototherapeutic kera —— Corneal clarity is not enough to allow
tectomy/anterior stromal puncture/ cataract surgery
conjunctival flaps have been described In cases where the values of CCT and endo
for symptomatic relief of bullous kera thelial counts are in between, the decision is
topathy when the visual prognosis is poor. individualized and based on surgeons experience.
However, these procedures are rarely
required in FECD, where the visual
VIVA QUESTIONS
potential is often good.
Q.1. Association of FECD: FECD has been
Surgical Management associated with following:
The different surgical options in a case of FECD Ans. • Axial hypermetropia, shallow anterior
are described below: chamber and angle closure glaucoma
Table 2 Early and late onset FECD

Early FECD Late FECD
ICD category Category 1 Category 2/3
Genetics Mutation in the gene for the alpha 2 AD, many with no inheritance pattern
chain of collagen VIII (COL8A2-Q455K) on 13pTel-13q12.13/18q21.2-q21.32/
chromosome 1 p34.3-p32 possible SLC4A11
Onset First decade 4th–5th
Retroillumination Fine, patchy distribution of corneal Coarse and distinct corneal guttae
guttae
Specular microscopy Small, shallow guttae Larger guttae
DM Considerably thicker than late —
Sex distribution F=M F>M
Abbreviations: AD, autosomal dominant; DM, descemets membrane; F, female; FECD, Fuchs’ endothelial
corneal dystrophy; M, male
• Keratoconus • At birth: 4000–5000

• I ncreased prevalence of age-related • 10–19 years: 2,900–3,500 cells/mm2
macular degeneration (controversial) • 20–29 years: 2,600–3,400 cells/mm2
• Increased rate of cardiovascular disease • 30–39 years: 2,400–3,200 cells/mm2
(controversial) • 40–49 years: 2,300–3,100 cells/mm2
Q.2. Causes of corneal guttae. • 50–59 years: 2,100–2,900 cells/mm2
Ans. Corneal guttae may be seen in the following • 60–69 years: 2,000–2,800 cells/mm2
cases: • 70–79 years: 1,800–2,600 cells/mm2
• Interstitial keratitis: Focal gutta formation • 80–89 years: 1,500–2,300 cells/mm2
without corneal edema The normal rate of endothelial loss is
• Macular dystrophy approximately 0.6% per year.
• Posterior polymorphous dystrophy
• Pseudo-guttae (appears as gutta but REFERENCES
these are transient, due to edema of the 1. Albert DM, Miller JW, Azar DT. Albert
endothelial cells, and disappear with and Jakobiec’s principles and practice of
resolution of the underlying condition) ophthalmology; 2008.
—trauma, intraocular inflammation, 2. Brad Bowling. Kanski’s Clinical ophthalmology:
infection, toxins and thermokerato- A systematic approach, 8th edition. Edinburgh:
plasty. Elsevier; 2015.
3. Weisenthal RW, Streeten BW. Descemet’s
Q.3. What is difference between early and late
Membrane and Endothelial Dystrophies. In:
onset FECD? Krachmer JH, Mannis MJ, Holland EJ (Eds).
Ans. Kindly see Table 2. Cornea. St. Louis, Mo. Mosby; 2011.
Q.4. Normal endothelial cell count and rate of
endothelial loss.
Ans. The normal endothelial cell count as per
age is as follows:
ACUTE GRAFT REJECTION

Ritu Nagpal, Vaishali Ghanshyam Rai, Prafulla Kumar Maharana, Manpreet Kaur

Corneal graft rejection can be defined as zz The onset is usually acute and progresses if
development of graft edema in conjunction with not treated. Graft rejection after PKP usually
inflammatory signs in a graft that has been clear occurs after an average period of 8 months.
for at least 2 weeks in a primary graft and one Most cases occur within one year. If the
week in a regraft. It is an immunological response symptoms are there from day one, it may be a
of the host to the donor corneal tissue without case of primary graft failure.
regard to the effect of the response on graft zz It is important to enquire about the best vision
survival. Immune rejection of the transplanted gained after the corneal graft. This helps in
cornea is the major cause of graft failure in the deciding for a re-graft on a later date.
intermediate and late postoperative period. It is
primarily of three types; epithelial, stromal and Past History
endothelial rejection. Endothelial rejection is
the most common and clinically important type A careful past history must be taken to rule out
of rejection. Approximately 30% of eyes with the risk factors responsible for graft rejection.
penetrating keratoplasty experience at least one Following history is important in a case of graft
episode of graft rejection and about 5–7% lead to rejection
eventual graft failure. Around 12% of graft rejection zz History of ocular surface diseases and treat
cases in patients with good prognostic keratoplasty ment should be asked to the patient, e.g.
and 40% in complicated cases have been reported Ocular surface diseases, such as severe dry eye,
to lead to subsequent graft failure.1-3 severe chemical burns, radiation burns, ocular
pemphigoid, Steven-Johnson syndrome,
neuroparalytic disease etc.
HISTORY zz Past history of herpes simplex keratitis and
Demography recurrent episodes should be asked.
zz Past history of any ocular surgery, e.g. pene
Several clinical series have shown that a younger
trating keratoplasty, excimer laser photothera
recipient conferred a higher risk of rejection
peutic keratectomy (can also trigger a corneal
probably due to a robust immune system.
graft rejection episode).
Recipients younger than 40 years are at a higher
zz Past history of glaucoma and antiglaucoma
risk for graft rejection.
medications taken.
zz History of a previous graft failure, especially if
Chief Complaints the failure was a result of an allograft rejection.
A case of acute graft rejection presents with One study demonstrated that the rate of
following symptoms: graft failure secondary to allograft rejection
zz Redness increased from 8% in patients with no history
zz Sensitivity to light of previous transplantation to 40% in patients
zz Vision loss with two or more previous grafts.
zz Pain. zz Details of the surgery: Look for following if the
These characteristic symptoms are present in detailed record of the surgery is available:
approximately 70% of the cases while 30% cases —— Presence of corneal vascularization at the
are asymptomatic and diagnosed during routine time of surgery—puts the graft at high risk
follow-up. for graft failure
—— Concomitant vitrectomy with PKP— —— An epithelial rejection line, also known

a twofold increased risk of graft failure as Krachmer’s line, in the graft from the
—— Concurrent intraocular inflammation host graft junction without edema and
—— Donor endothelial count keratic precipitates/infiltrate can be seen.
—— Presence of anterior synechiae in host. It is usually seen in epithelial rejection.
All these factors are risk factors for graft An epithelial line stains with fluorescein
rejection and subsequent graft failure. or Rose Bengal. The epithelium behind the
rejection line may appear hazy, irregular
Past Medical History and is replaced by recipient epithelium.
Superficial epithelial infiltrates appear
A case of acute graft rejection needs aggressive
near the suture lines which progress
steroid therapy. Hence any systemic history that
centrally. These are known as Kaye’s
is a contraindication for systemic therapy must
dots. It usually subsides in 6–10 days but
be rule out. A careful history of diabetes mellitus,
may last several weeks. Both Krachmer’s
hypertension, peptic ulcer disease, tuberculosis,
line and Kayes dots are seen in epithelial
osteoporosis and any neuropsychiatric disorder
rejection commonly. However, they can
must be ruled out.
be present in endotheial rejection also.
—— Keratic precipitates: They can appear as
EXAMINATION scattered deposits or can form a distinct
General/Systemic Examination line known as the Khodadoust line
(Fig. 1). This line tends to migrate from
Carefully look for any systemic contraindication the peripheral cornea to the central
for steroid therapy. cornea and many a times its origin can be
traced to a loose suture or donor corneal
Ocular Examination vessel. Khodadoust line is the hallmark of
zz Visual acuity: Best corrected visual acuity graft rejection.
(BCVA) must be recorded at the base line. —— Differential edema: Endothelial rejection
Monitoring BCVA on a daily basis can indicate is often associated with stromal edema
the response to pulse steroid therapy. overlying the areas that have been
zz Eyeball/Eyelids are looks normal. However, lid traversed by the endothelial rejection line
edema can be there in an acutely inflammed while the areas ahead of the line are clear
eye. Also look for any findings such as (Figs 2 and 3).
ectropion, entropion, meibomian disease,
trichiasis that may put the graft at risk.
zz Conjunctiva: Circumciliary congestion is
characteristically seen in corneal graft rejec
tion. Often, it is the earliest sign of rejection
reaction and can occur before clinical appear
ance of cellular infiltrates in the cornea or the
anterior chamber.
zz Cornea:
—— Cellular infiltration of the cornea as dis
crete subepithelial infiltrates reminiscent

of those seen in epidemic keratoconjun
ctivitis. These small (0.2–0.5 mm), hazy
infiltrates are usually scattered in the
central cornea and occur exclusively in Fig. 1: Khodadoust line on the endothelium in a
the donor tissue but not the peripheral case of acute graft rejection following penetrating
recipient tissue. keratoplasty
Fig. 2: Differential stromal edema in a case of Fig. 4: Failed graft after an episode of
acute graft rejection acute graft rejection
Fig. 3: Acute graft rejection with stromal thickening Fig. 5: Decompensated corneal graft with
and edema stromal thickening after acute graft rejection
—— Deep corneal vascularization should be tonometry. A Mackay-Marg principle based

noted. tonometer (Tonopen) can give more reliable
—— Staining: Cornea must be stained to values.
look for presence of epithelial defect or
recurrence of a herpetic keratitis. DIFFERENTIAL DIAGNOSIS
—— In delayed presentation the entire
graft becomes edematous (Fig. 4) with A case of acute corneal graft rejection must be
significantly increased graft thickness differentiated from graft failure, sterile/infectious
(Fig. 5). endophthalmitis, epithelial down growth and
zz Anterior chamber (AC): AC flare and cells can recurrent herpetic keratitis. The differentiating
be noted which indicates elevated levels of points are summarized in Table 1.
protein in the aqueous humor due to leakage
from the uveal vasculature. INVESTIGATIONS
zz Intraocular pressure (IOP) raised IOP can
both be a consequence or cause for corneal Pachymetry
graft rejection. In edematous cornea IOP may Increased central corneal thickness (CCT) is often
be falsely low with Goldmann’s applanation the first indicator of endothelial dysfunction.
Table 1 Differential diagnosis of acute corneal graft rejection

Condition Features
Late graft failure • Gradual onset of graft edema
• Not associated with signs of inflammation such as AC cells/flare or
keratic precipitates
• No Khodadoust line or differential edema
Sterile/infectious endophthalmitis • Inflammatory signs are severe
• Presence of hypopyon
• Presence of infiltrates in vitreous
Epithelial down growth • Clumps of cells like material in the anterior chamber
• Cells larger than that of cells of inflammation
• These cells does not respond to corticosteriod therapy
• Presence of white membrane over anterior surface of iris
• Associated increased intraocular pressure that is unresponsive to
medical therapy
Recurrent herpetic keratatis • KP’s are restricted to host cornea only
• History of previous herpetic keratitis
• Absence of Khodadoust line
• Characteristic shape
• Response to topical antiviral therapy
CCT can be measured with the help of ultrasonic Steroids

pachymeter, orbscan, pentacam or anterior
Topical Steroids
segment OCT (ASOCT). Serial monitoring of CCT
is important for monitoring the response to steroid A case of acute graft rejection can be managed on
therapy. outpatient basis but admission to hospital for the
first few days of treatment is helpful monitoring
Specular Microscopy compliance. Aggressive administration of potent
topical corticosteroids with good intraocular
It can reveal the reduced endothelial cell count penetration (such as prednisolone acetate 1% or
that further reduces each episode of graft rejection. prednisolone phosphate 1% eye drop) aborts most
attacks of acute graft rejection. Commonly used
Confocal Microscopy treatment regime is:
In presence of severe graft edema, where specular zz 1 hourly–3 days
microscopy is not possible, confocal microscopy zz 2nd hourly–15 days
can reveal the endothelial changes. zz 4 times–2 months
zz 3 times–2 months
zz 2 times–3 months
Ultrasonography (USG) zz Once–4 months
In presence of severe corneal edema USG is Topical steroids are effective in epithelial
helpful in ruling out sterile endophthalmitis. rejection, stromal rejection and mild-moderate
endothelial rejection.
MANAGEMENT
Most episodes of allograft rejection can be reversed Systemic Steroids
if prompt and aggressive treatment is initiated. The zz Severe episodes of endothelial rejection needs
treatment of choice is steroids. systemic steroid therapy.
zz Pulse steroid therapy is more effective than —— Topical cyclosporine A is available as 2%

oral steroids for the treatment of a rejection in castor oil or 1% in artificial tears 4 times
episode. A single pulse (500 mg intravenous daily
methylprednisolone) in a single dose is more —— Systemic
effective and better tolerated than daily oral Recommended dosage is 15 mg/kg/
prednisolone. Repeating the dose at 24 or day for 2 days followed by 7.5 mg/
48 hours after the initial dose does not add kg/day for 2 days then adjusted
any advantage. to maintain trough blood levels of
zz Intravenous dexamethasone (100–200 mg) 100–200 mg/L for 6 months after
single dose has been found to be equally reversal of acute rejection episode
efficacious as methylprednisolone and thus Close monitoring of blood pressure,
may be used as an alternative in patients who renal function including serum

are nonaffording. creatinine and liver function test
zz In severe cases of rejection, topical predniso Recently studies suggest topical cyclosporin A
lone acetate 1% hourly, one dose of pulsed (CsA) as well as oral CsA have not been found
intravenous methylprednisolone (500 mg), to reduce the risk of allograft rejection.
and oral prednisone at 1 mg/kg/day for 5 days zz Mycophenolate mofetil
—— MMF acts by inhibiting inosine mono
is recommended.
zz The IV steroid therapy beyond 8 days of the phosphate dehydrogenase required for
onset of symptoms may not add any benefit to proliferation of T- and B-lymphocytes
—— Dose 750 mg bd
intense topical steroid therapy. In a study by
—— Renal, hepatic and bone marrow function
Hills et al. when patients were treated within
8 days of the onset of symptoms, the survival must be monitored
—— Recent studies suggest that oral MMF
rate of grafts was 92% versus 55%.
is effective in the prevention of allograft
rejection in high risk keratoplasties.
Supportive Therapy —— Unlike CsA, therapeutic drug monitoring
Anti-glaucoma medications have to be given when is not required which significantly reduces
IOP is raised. Cycloplegics can reduce pain by the cost of treatment.
zz Azathioprine
relieving ciliary spasm. Topical lubricating drops
—— 1–2 mg/kg/day orally
are useful in presence of sutures and associated
—— It reduces the need of systemic cortico
epithelial defect.
steroids, and, thus, reduces the systemic
complications expected by high-dose
Immunosuppressive Therapy corticosteroids.
—— Renal, hepatic and bone marrow function
Immunosupressive therapy is not required
must be monitored
routinely. Probable indications includes high zz Tacrolimus (FK-506)
risk graft and cases where long-term steroid use —— Macrolide immunosuppressant with
is contraindicated or causing complications. a mechanism of action similar to CsA,
Following immunosupressive therapy have been but 10–100 times more potent than the
tried with variable success. latter. It inhibits calcineurin by binding to
zz Cyclosporine A: CsA is a powerful immuno immunophilin or FK-506 binding protein
suppressive agent which binds to an (FKBP). Topical (ointment 67 or drops)
intracellular protein called cyclophilin and as well as systemic tacrolimus has shown
inactivates calcineurin. The inactivation of to be promising as a prophylactic agent
calcineurin inhibits IL-2 and lymphokine against corneal graft rejection.
production, thus limiting the activity if CD4+ —— A dose of 0.16 mg/kg/day.
and CD8+ lymphocytes. —— Renal function must be monitored.

zz Other agents: Rapamycin/Sirolimus, anti- rejection. These eyes are more prone
lymphocyte monoclonal antibodies have been for rejection.
tried with variable success. • Intraoperative factors:
– Large graft: Graft is nearer to limbal
vessels
VIVA QUESTIONS
– Eccentric graft: Proximity to limbal
Q.1. What are the risk factors for acute corneal vessels
graft rejection? – Small graft: Less endothelial cell
Ans. • Donor factor transferred
– The method and duration of storage – Iris adhesion at graft host junction:
of the donor cornea and nature of Immune cells through iris vasculature
donor button cutting. get exposed to antigens
– Pretreatment of donor tissue with – Recent anterior segment surgery:
ultraviolet radiation may reduce the Associated inflammation brings more
chances of development of rejection. immune cells
• Host factor – Anterior vitrectomy
– Vascularization of the host cornea: – Full thickness graft > lamellar graft
Deep stromal vascularization of the • Postoperative factors
host cornea of two or more quadrants – Corneal epithelial breach
classifies as a high-risk cornea. CCTS – Exposed suture knots, loose suture: By
has defined vascularization of the host inciting vascularization
bed in 2 or more quadrants extending – Postoperative uveitis
at least 2 mm into the stroma as a – Postoperative glaucoma
risk factor associated highly with the – Synechiae between iris and graft host
rejection of the corneal grafts. junction.
– Regraft: A cornea with a previously
Q.2. What are the measures you can take to
failed graft due to any cause is con
sidered to be at high risk. prevent graft rejection?
– Herpes simplex virus keratitis: Active Ans. • P reoperative measures: Reducing the
or healed HSV keratitis considered antigenic load of donor tissue.
as high risk for graft rejection. The – Use the central corneal graft
increases risk is due to the vasculariza – Removal of the donor epithelium
tion associated with HSV keratitis. – Exposure to ultraviolet light
– Ocular surface diseases, such as – Depletion of local macrophages—
severe dry eye, severe chemical burns, subconjunctival injection of clodro
radiation burns, ocular pemphigoid, mate liposomes which alters delayed
Steven-Johnson syndrome, and type hypersensitivity
neuroparalytic disease, are also asso – Pretreatment of the graft with hyper
ciated with poor prognosis for the baric oxygen and use of heterologous
corneal graft. antibody treated corneal button.
– Young patients and bilateral graft • I ntra-operative factors: Meticulous
have more chances of graft rejection surgical technique, including of avoiding
due to active immune system. decentration of the recipients bed cut,
– Pediatric patients: The immune optimal suturing, and good graft-host
system of children is more active than apposition
that of adults and due to rapid wound • Postoperative measures: Controlling or
healing suture becomes loose early, alleviating the host immune response to
both these factors along with the the foreign donor tissue. Steroids are the
inability of the child to communicate best option for prophylaxis against graft
timely leads to an increases risk of rejection. Long-term (12–18 months) of
topical steroids have a better rejection • P

resenting symptoms are minimal such
free graft survival. as mild irritation, photophobia and
rarely mild blurring of vision.
Q.3. What is a relation between corneal
vascularity and graft rejection? Q.5. Unusual manifestations of graft rejection.
Ans. • Low-risk—avascular Ans. Rarely acute graft rejection can present
• M edium risk: Vascularization 1–2 with following (without the characteristic
quadrants. presentation described in history section).
• High-risk: Vascularization 3 or more • Raised IOP due to engorgement and or
quadrants. edema of TM
• Acute epithelial defect along with ocular
Q.4. Graft rejection following endothelial
inflammation. This type of unusual
keratoplasty.
presentation is usually seen in young
Ans. Graft rejection in EK differs from rejection
patients.
following PKP in following ways:
• L ower rates of rejection compared to
cases with PKP probably due to the lower REFERENCES
antigenic load.
1. Hill JC. High risk corneal grafting. Br J
• The incidence of graft rejection is around
Ophthalmol. 2002;86:945.
7.5%.
2. Hill JC, Ivey A. Corticosteroids in corneal graft
• Rejection episodes are less severe with rejection. Double versus single pulse therapy.
high rates of reversibility and low rates of Cornea. 1994;13:383-8.
graft failure 3. Panda A, Vanathi M, Kumar A, Dash Y, Priya
• One-third of patients with graft rejec S. Corneal Graft Rejection. Surv Ophthalmol.
tion after endothelial keratoplasty are 2007;52(4):375-96.
asymptomatic
SHORT CASES
KERATOGLOBUS
Prafulla Kumar Maharana, Sapna Raghuwanshi, Namrata Sharma
INTRODUCTION Rarely a case may present with sudden loss of

vision, pain, conjunctival injection, photophobia
Keratoglobus is a rare noninflammatory corneal and glare, typically in cases of acute hydrops.
thinning disorder characterized by generalized
thinning and globular protrusion of the cornea
(Fig. 1). Nearly all cases are bilateral. The onset
EXAMINATION
is often at birth with minimal or no progression. Systemic Examination
However, both congenital and acquired forms
Keratoglobus can be associated with connective
have been reported, and may be associated with
tissue disorders; hence, a thorough systemic
various other ocular and systemic syndromes
examination must be carried out (especially
including the connective tissue disorders. In
look for blue sclera, joint hypermobility, skeletal
exams, it is given as a short case.
abnormalities, hearing loss, abnormal dentition,
high-arched palate). The diseases that have been
HISTORY reported to be associated with keratoglobus are
Chief Complaints described in Table 1.
Patient may present with following complaints:
zz Blurring of vision due to irregular astigmatism
Ocular Examination
(most common presentation) The ocular examination includes following:
zz Itching, watering (if associated with atopy, zz Eyeball: Usually normal.
VKC) zz Eyelid: Usually normal.
zz Corneal perforation, either spontaneous or zz Conjunctiva: Usually normal, however signs of
following minimal trauma VKC can be there.
zz At times diagnosed incidentally and may be zz Cornea: Corneal thinning is characterized
completely asymptomatic. by the presence of limbus-to-limbus corneal
thinning (Fig. 2) with globular corneal
Table 1 Associations of keratoglobus

Connective • Ehlers–Danlos syndrome type VI
tissue disorders • M arfan syndrome
• Rubinstein-Taybi syndrome
• Osteogenesis imperfecta
Hereditary • Leber’s congenital amaurosis
ocular • Posterior polymorphous
disorders dystrophy
Acquired • Vernal keratoconjunctivitis
ocular • Chronic marginal blepharitis
disorders • Idiopathic orbital inflammation
Fig. 1: Globular corneal protrusion and corneal
• Post-traumatic
thinning in a case of keratoglobus
protrusion (Fig. 3). Usually the thinning is Anterior chamber: It is usually deep.
greatest in the corneal periphery or mid Iris/pupil/lens/fundus/IOP: All these findings
periphery initially but with progression, are usually within normal limits. However, retinal
limbus-to-limbus thinning occurs. changes of associated diseases such as Leber’s
Prominent folds and areas of thickening may be congenital amaurosis may be there.
present in Descemet’s membrane. Other corneal
parameters, however, are normal, including a
normal corneal diameter that is an important
criterion in differentiating it from conditions such The differentiating features from other corneal
as buphthalmos. ectatic disorders are summarized in Table 2. In
Sclera: Usually scleral thinning or “blue sclera” addition, it must be differentiated from following:
is present, especially in association with connec
tive tissue disorders and is most apparent over the Megalocornea
ciliary body. It creates a “blue halo” around the It is nonprogressive symmetric enlargement of the
limbus. cornea (greater than 12 mm in horizontal diameter)
Fig. 2: Limbus-to-limbus corneal thinning Fig. 3: Globular corneal protrusion with

normal corneal diameter
Table 2 Differential diagnosis

Pellucid marginal Terrien’s marginal
Parameters Keratoconus degeneration Keratoglobus degeneration
Frequency Most common Less common Rare Less common
Laterality Usually bilateral Bilateral Bilateral Bilateral
Age at onset Puberty 20–40 years Usually at birth 20–40 years
Thinning Inferior Paracentral Inferior band 1–2 mm Usually starts superiorly
wide, greatest in then progress
periphery circumferentially
Protrusion Thinning at apex Superior to band of Generalized
thinning
Iron line Fleischer ring Sometimes None None
Scarring Common Only after hydrops Mild Only after hydrops
Striae Common Sometimes Sometimes Sometimes
without a significant change in corneal thickness Corneal Topography (Orbscan/

or contour unlike keratoglobus where corneal Videokeratography/Pentacam)
diameter is normal but thickness is reduced.
It shows diffuse thinning and irregular astigmatism
with irregular power distribution. In advanced
Congenital Glaucoma cases, it is often difficult to perform corneal
It is associated with elevated intraocular pressure, topography.
a cloudy cornea, changes in the optic disc or
generalized enlargement of the eye that are absent MANAGEMENT
in keratoglobus.
The management consists of following:
INVESTIGATIONS
Conservative Management
The diagnosis of keratoglobus is essentially a
Often the patient maintains a good visual acuity
clinical one owing to the characteristic clinical
till late. Thus observation is the rule in early cases.
findings. Following investigations are done in a
These patients are advised to use protective eye
case of keratoglobus if the diagnosis is doubtful
wear (e.g. polycarbonate glasses), and avoidance of
and while planning treatment.
contact sports owing to the high risk of perforation.
Visual rehabilitation is achieved through refractive
Ultrasonic Pachymetry correction for high myopia. Spectacles are
It would show reduced corneal thickness. normally used in early cases of keratoglobus only.
Table 3 Treatment options for keratoglobus

Surgical procedure Advantage Disadvantage
Large diameter (limbus-to-limbus) Covers the thinned part and • Increased rejection due to
PKP suturing is easy proximity of limbal vasculature
• Limbal stem cell damage
• Damage to Angle structure
Epikeratoplasty • Good tectonic stability • Limbal stem cell disruption
• Corneal flattening effect • Persistent epithelial defect
Epikeratoplasty with 360 host • No limbal stem cell disruption, Poor visual outcome due
peripheral intrastromal tucking • No angle structure disruption to interface opacities and
intraepithelial cysts
Tuck-in lamellar keratoplasty • Good tectonic stability • Technically difficult
• No limbal stem cell disruption • Interface haze
• No angle structure disruption
Pentacam-based deep anterior LK Advantages of lamellar graft Technically demanding
Corneoscleral rim (Buttress over • Technically easy Temporary measure
thinned corneal periphery for • Allows for delay in further
tectonic stability) surgical intervention
Epikeratoplasty/tectonic LK Better visual outcome • Two stage procedure
followed by 2nd stage PKP • Two donor corneas required
Abbreviations: PKP, penetrating keratoplasty; LK, lamellar keratoplasty

As the disease progresses, irregular astigmatism zz Higher chance of perforation while performing
develops and adequate visual acuity cannot be lamellar graft due to limbus-to-limbus thinning.
achieved with this type of visual correction. The various treatment treatment options for
keratoglobus are summarized in Table 3.1
Contact Lens
Various types of contact lenses (CL) are available VIVA QUESTIONS
for treatment of keratoglobus such as scleral
lenses, rigid gas permeable (RGP) lenses, reverse Q.1. Difference between congenital and
geometry hydrogel lenses and large diameter aquired keratoglobus
inverse geometry RGP lenses. Ans. Remember keratoglobus is almost always
a congenital disease. Recently, it has
Surgical Management been reported to be associated with few
acquired diseases (Table 1). The acquired
Surgery in keratoglobus is difficult because of
types are more severe with a higher chance
following:
of perforation from trivial trauma. Few
zz Large graft is required to include the thinned
authors consider these aquired forms as
periphery and large graft as such is a risk factor
nothing but severe variants of keratoconus
for graft rejection.
only.
zz Owing to the fragility of the thinned cornea at
periphery placement of sutures is difficult and
often leads to cut through or ‘cheese-wire’. REFERENCE
zz Proximity of the graft to limbus can lead to 1. Wallang BS, Das S. Keratoglobus. Eye.
increased chance of graft rejection. 2013;27:1004-12.
PELLUCID MARGINAL DEGENERATION

Sapna Raghuwanshi, Manpreet Kaur, Namrata Sharma
INTRODUCTION Chief Complaints

Pellucid marginal corneal degeneration (PMCD) Patient may present with following symptoms:
is a bilateral, peripheral corneal ectatic disorder zz Blurring of vision due to marked against-the-
characterized by a band of thinning 1–2 mm in rule astigmatism (most common presentation)
diameter extending from 4 o’clock to 8 o’clock zz Frequent changes of glasses
position in the inferior cornea. The area of zz Rarely a case may present with sudden
thinning is separated from limbus by a 1–2 mm loss of vision, pain, conjunctival injection,
width of normal thickness cornea. Atypical cases photophobia and glare typically in cases of
can present with superior thinning or thinning acute hydrops.
beyond 4 o’clock to 8 o’clock hour, but these
cases are rare. In exams, PMCD can be given as Past History
a short case. Past history of spectacle or contact lens (CL) use
must be noted carefully.
HISTORY
EXAMINATION
Demography
The disease is almost always bilateral without any Ocular Examination
gender predilection. The onset is often between Eyeball/eyelid/conjunctiva is usually within
second to fifth decade of life. normal limits.
Cornea: On slit lamp biomicroscopy following Unlike keratoconus the protruding cornea
signs may be present. is of normal thickness. When viewed from
zz Corneal thinning: A band of thinning 1–2 mm the side, the inferior-central cornea in PMD
in width, typically in the inferior cornea, typically shows the side-profile contour of a
extending from the 4 o’clock to 8 o’clock “beer-belly”.
position is present (Figs 1 and 2). Between zz Stromal scars: Scarring can be present at the
the area of thinning and limbus there is superior aspect of the thinned area and can
usually a 1–2 mm width of cornea with normal extend into the mid stroma.
thickness. Unlike Terrien’s marginal degenera zz Descemet’s folds: These are occasionally
tion, there is no scarring, lipid deposition, or seen concentric to the inferior limbus, may
vascularization. disappear with external pressure.
zz Corneal protrusion: The area of ectasia is zz Bowman’s layer: It may be normal or focal
just superior to the area of thinning (Fig. 3). disruption within the area of corneal thinning
can be there.
zz In case of previous attack of hydrops, corneal
scarring and vascularization of the inferior
cornea can be seen.
Other ocular findings are usually within
normal limits.
A case of PMCD must be differentiated from
peripheral corneal thinning disorders such as
Terrien’s marginal degeneration, Mooren’s ulcer
and Furrow degeneration. The differentiating
features are summarized in Table 1. Advanced
keratoconus and rarely keratoglobus can also be
confused with PMCD. The differentiating features
Fig. 1: Inferior corneal thinning extending from the are summarized in Table 2.
4 o’clock to 8 o’clock position
Fig. 2: Inferior corneal thinning extending from the Fig. 3: Corneal ectasia superior to
4 o’clock to 8 o’clock position with a 1–2 mm width of area of thinning in PMD
corneal with normal thickness between the area of
thinning and limbus
Table 1 Differential diagnosis of pellucid marginal corneal degeneration

Pellucid marginal Terrien's marginal Senile Furrow
Features corneal degeneration corneal degeneration Mooren's ulcer degeneration
Age at onset Second to fifth decade Middle-aged to elderly Adult to elderly Elderly
Laterality Bilateral Bilateral Either Bilateral
Gender M=F M>F M>F M=F
Astigmatism Common Common Sometimes Absent
Thinning Inferior band Superior cornea Starts within lid Occurs within arcus
1–2 mm wide fissure
Inflammation Absent May be present Present Absent
Epithelial defect Absent Usually absent Present Absent
Vascularization Absent Crosses area of Peripheral edge Absent
thinning of thinning
Lipid deposition Absent Common; central to Rarely Absent
thinning
Perforation Can occur Can occur Can occur Never
Abbreviations: M, male; F, female
Table 2 Differential diagnosis of pellucid marginal degeneration

Characteristics Keratoconus PMD Keratoglobus
Frequency Most common Less common Rare
Laterality Usually bilateral Bilateral Bilateral
Age at onset Puberty 20–40 years Usually at birth
Thinning Inferior paracentral Inferior band 1–2 mm wide Maximum in periphery
CCT Reduced Usually normal May be normal
Protrusion Thinnest at apex Superior to band of thinning Generalized
Rizzuti’s phenomenon Present Absent Present
and Munson’s sign
Fleischer ring Present Sometimes None
Scarring Common Only after hydrops Mild
Striae Common Sometimes Sometimes
Abbreviations: PMD, pellucid marginal degeneration; TMD, Terrien’s marginal degeneration
INVESTIGATIONS mid-periphery, inferior oblique corneal meridians

in a classic “crab-claw”, “butterfly” or “kissing doves”
Corneal Topography appearance. It shows the presence of superior flat
Topography characteristically shows inferior tening with against-the-rule astigmatism superiorly
peripheral steepening extending into the and with-the-rule astigmatism inferiorly.
MANAGEMENT normal central cornea, no risk of rejection or

interface haze, better wound strength, and
Spectacles: Spectacles are normally used in early shorter visual rehabilitation period. However,
cases of PMD only. As the disease progresses, postoperative unstable astigmatism is an
irregular astigmatism develops and adequate issue due to persistent tension at the sutured
visual acuity cannot be achieved with this type of wound. Various modifications have been
visual correction. described to improve the outcome of wedge
Contact lens: Newer generation CL such as ROSE resection, such as wedge resection followed
K, scleral lenses [Prosthetic Replacement of the by complete (limbus-to-limbus) or partial
Ocular Surface Ecosystem (PROSE) and Boston host lamellar dissection and corneal wedge
ocular surface prosthesis (BOSP)] and hybrid resection combined with paired, opposed
lenses (such as the Soft Perm-gas-permeable clear corneal penetrating relaxing incisions.
lens center and a hydrogel skirt) have shown The relaxing incisions prevent the astigmatic
promise in PMCD in early studies. These lenses drift seen following wedge resection.
offer improvements in the VA and good stability
so they could be an option for patients who have
failed conventional treatments before considering VIVA QUESTIONS
surgery.
Q.1. How to differentiate between keratoconus
and PMCD
Surgical Treatment
Ans. Kindly see Table 1.
zz Large-diameter or eccentric penetrating kerato
Q.2. How to differentiate between PMCD and
plasty: A large diameter PKP is done so as
TMCD
to include the thinned out periphery. The
Ans. Kindly see Table 2.
problems with such grafts are an increased risk
of rejection due to proximity to the limbus and Q.3. What are the difficulties in performing
severe postoperative astigmatism associated PKP in PMCD?
with a decentered graft. Ans. There challenges in performing PKP cases
zz Combined lamellar keratoplasty (LK) with of PMCD are as follows:
penetrating keratoplasty (PKP): LK with PKP • Due to involvement of paracentral and
can be done in the same setting or as a two- peripheral cornea a large graft with
stage procedure (LK followed by PKP 6 months increased proximity to limbus is required
later). The large diameter lamellar graft that increases the chances of graft
provides the tectonic support to the weakened rejection.
peripheral host cornea while a central small • Extreme corneal thinning makes suturing
diameter full thickness graft can provide difficult and increases the chances of
excellent visual outcome. intraoperative DM perforation.
zz Crescentic lamellar keratoplasty: A ‘match
and patch’ lamellar graft procedure is done. BIBLIOGRAPHY
Precise lamellar dissection of the recipient
bed is done to acheive vertical margins and 1. Feder RS, Gan TJ. Noninflammatory ectatic
disorders. In: Krachmer JH, Mannis MJ, Holland
an even stromal bed depth. A lamellar donor
EJ. Cornea. St. Louis, MO: Mosby; 2011.
undersized by 0.25–0.5 mm is then sutured to
2. Jinabhai A, et al. Pellucid corneal marginal
the recipient bed that results in flattening and degeneration: A review. Contact lens anterior
reduction of ectasia. eye, doi:10.1016/j.clae.2010.11.007.2011.
zz Crescentic or wedge excision: This technique is 3. Maharana PK, Dubey A, Jhanji V, Sharma N,
useful when the ectasia is confined to a small Das S, Vajpayee RB. Management of advanced
sector of periphery. It has several advantages corneal ectasias. Br J Ophthalmol. 2015.
over a corneal graft such as; preservation of pp.1-7.
BAND-SHAPED KERATOPATHY
Manpreet Kaur, Sapna Raghuwanshi, Ritu Nagpal
INTRODUCTION EXAMINATION
Band shaped keratopathy (BSK) is a slowly Systemic Examination
progressive, usually painless, corneal degeneration
A careful systemic examination is carried out to
characterized by deposition of calcium across
rule out any systemic association (see Table 1).
the cornea at the level of Bowman’s membrane,
epithelial basement membrane and stroma.
BSK is divided into two forms calcific and non Ocular Examination
calcific form. Eye ball: It is usually normal. Blepharophimosis
Its examination is especially important as it is may be present in cases of epithelial erosion or
given as short case in examination. elevated nodules associated with inflammation.
CHIEF COMPLAINT Eyelid: Lid edema may be present if BSK is asso

ciated with ocular inflammation.
Patients present with following complaints:
Conjunctiva: It may be normal or signs of
Early stage: Usually asymptomatic previous disease may be present. Ciliary as well as
Late stage: May present with the following conjunctival congestion can be there in presence
symptoms: of epithelial defect.
zz Blurring of vision
Cornea: On slit lamp examination following signs
zz Foreign body sensation
must be noted.
zz Tearing zz Most of the times the opacity begins in the
zz Photophobia.
form of peripheral interpalpebral calcification
at the 3 and 9 o’clock positions (Fig. 1).
PAST HISTORY zz In the peripheral form, sharply demarcated
BSK may be associated with several diseases, peripheral edge of the opacities separated
(Table 1) hence a thorough history must be taken from the limbus by a lucent zone is seen. This
to rule out these disorders. zone is either due to the lack of Bowman’s
Table 1 Associations of band shaped keratopathy

Ocular disease Hypercalcemia Systemic diseases Chemicals Familial
• Chronic uveitis • Hyperparathyroidism • Discoid lupus • Mercury fumes • Fanconi's
• Phthisis bulbi • Hypophosphatasia • Gout • Phosphate disease
• Long-standing • Sarcoidosis • Tuberous containing drops • Ichthyosis
glaucoma • Renal failure sclerosis • Intraocular silicone • CHED
• Interstitial (e.g. Fanconi’s syndrome) • Norrie’s disease oil
keratitis • Excessive vitamin D • Congenital • Viscoelastic
• Dry eye and (e.g. oral intake, band • Thiazides
corneal exposure sarcoidosis, and keratopathy • Lithium
syndromes osteoporosis) • Still’s disease • Vitamin D toxicity
• Spheroidal • Multiple myeloma • Uremia
keratopathy • Milk alkali syndrome
• Keratoprosthesis • Metastatic carcinoma to
• Trachoma bone
• Viscoelastics • Idiopathic
• Paget’s disease
Lens: Complicated cataract may be present in case

of uveitis.
Other findings are usually within normal
limits.
INVESTIGATION
Following investigations should be done in BSK to
rule out underlying systemic disorders that affect
the calcium homeostasis:
zz Serum calcium
zz Serum phosphorus
zz Serum uric acid
zz Renal function measurements
Fig. 1: Interpelpebral calcification
zz Parathyroid hormone (PTH)
zz Angiotensin-converting enzyme (ACE) levels.
MANAGEMENT
The most important part is to recognize and treat
the underlying causes. The indications for corneal
intervention in a case of BSK are:
zz Central band keratopathy contributing to
reduced vision
zz Mechanical irritation because of calcific
deposits causing discomfort and foreign-boy
sensation.
Following are the surgical options for BSK:
zz Mechanical debridement: If calcium plaque is
thick, it is scraped off the cornea with forceps.
zz Superficial keratectomy: Superficial calcium is
Fig. 2: BSK in CHED scraped off the corneal surface with a no. 15
scalpel blade until a sufficiently clear cornea
layer at the periphery or from the buffering can be observed.
capacity of the limbal vessels, which prevent zz Chelation: After application of topical
precipitation of calcium. anesthesia and placement of an eyelid
zz In chronic ocular inflammation, the band may speculum, all the epithelium overlying the
start centrally. calcium deposits is removed with a sponge
zz Gradual central spread to form a complete or a no. 15 blade to allow penetration of
limbus-to-limbus band like chalky plaque the EDTA. EDTA (0.05 mol/L) is applied to the
containing the small lucent holes represen subepithelial calcification by surgical sponges
ting penetrating corneal nerves is seen in late or directly using a corneal trephine as a well.
stage. The chemical reaction takes several minutes
zz Advanced lesion may become nodular to occur (5–30 minutes depending on the
and elevated which may lead to epithelial severity). Then all of the calcium can be
breakdown (Fig. 2). removed using blunt dissection with cellulose
sponges or with gentle scraping using a blunt
Iris: Festooned pupil, muddy iris, and posterior spatula.
synechiae may be present in case of uveitis. zz Phototherapeutic keratectomy (PTK): The
Anterior chamber: Emulsified silicon oil may be basic steps of PTK include removal of corneal
present in anterior chamber. epithelium with hockey stick spatula; ablation
of superficial corneal tissue with excimer laser Q.2. Pathogenesis of BSK.

and placement of bandage contact lens. The Ans. Following mechanism are involved in the
complications of PTK include hyperopic shift, deposition of calcium in cornea:
cornel haze, glare and myopic shift. • Corneal exposure-calcium deposition
zz Anterior lamellar keratoplasty (ALK): When occurs primarily in the exposed part of
the opacities extend into deeper stroma cornea due to precipitation left as tears
and the visual potential is good, ALK is per evaporate.
formed. Visual prognosis is generally good • Alteration of tear film osmolarity.
after ALK. • Elevation of pH due to corneal tissue
zz Amniotic membrane transplantation (AMT): metabolism.
AMT is used as an adjunct to augment con • Increase in concentration of calcium and
ventional surgical and chemical removal of phosphate.
band keratopathy by replacing the damaged
Q.3. Histopathology of BSK.
basement membrane. It facilitates healing
and provides long-term stability to the corneal Ans. Calcium is deposited as the hydroxyapatite
epithelium. salt in the epithelial basement membrane,
basal epithelium, and Bowman’s mem
brane. The deposits are usually extracel
VIVA QUESTIONS lular, although hypercalcemia may cause
intracellular epithelial accumulation.
Q.1 What is Still’s syndrome?
Ans. Still’s disease is also known as systemic-
onset juvenile idiopathic arthritis. Still’s BIBLIOGRAPHY
triad consists of complicated cataract, 1. Albert DM, Miller JW, Azar DT. Albert
glaucoma and BSK. It is characterized by & Jakobiec’s Principles and Practice of
high spiking fevers, salmon-colored rash Ophthalmology; 2008.
that comes and goes, and arthritis and
hepatospleenomegaly.
SPHEROIDAL DEGENERATION
Sapna Raghuwanshi, Neelima Aron, Namrata Sharma
Spheroidal degeneration (SD) is a corneal Epidemiology/Demography
degeneration characterized by the appearance SD is commonly seen in cases associated with
in the cornea, and sometimes in the conjunctiva, high UV exposure and/or reflected light such
of translucent, golden brown, spheroid deposits as observed in desert, ocean and snow-covered
in the superficial stroma. It is known by many regions. Thus, it is more common in males and
different names including Bietti’s nodular cor persons involved in outdoor activities. It is a
neal degeneration, Labrador keratopathy, climatic disease of elderly and the incidence rises with age.
droplet keratopathy, degeneratio corneae sphae
rularis elaioides, corneal elastosis, fisherman’s Chief Complaints
keratitis, keratinoid corneal degeneration, and The presenting feature depends upon the severity
chronic actinic keratopathy. Spheroidal degenera of the disease:
tion and climatic droplet keratopathy (CDK) are zz Grade 1 (nodules in periphery): Usually
the most commonly used terms. asymptomatic (diagnosed incidentally during
routine corneal examination)
zz Grade 2 (nodules encroaching pupillary axis): association with pinguecula. The spherules darken
Blurring of vision (6/30) with age, progressing from a lighter yellow to a
zz Grade 3 (involvement of visual axis): Blurring brownish-yellow color.
of vision (<6/60) Cornea: On slit lamp biomicroscopy following
zz Grade 4 (raised elevated nodules): Pain, signs must be noted.
photophobia, redness, foreign body sensation. zz Clear to yellow-gold spherules seen in the
CDK is slowly progressive, painless, asym subepithelium, within Bowman’s, or in the
metric, may be unilateral or bilateral and is more superficial corneal stroma. The droplets
common in males. appear oily (Fig. 1).
zz Initially the spherules appear at the limbus
Past History in the interpalpebral zone at 3 o’clock and
A careful past history can identify the predisposing 9 o’clock. Following grades of the primary
factor. Most cases are idiopathic and follow form have been noted:
—— Grade I: Initially SD begins as a gray haze
ing are the risk factors for secondary spheroidal
degeneration. in the superficial cornea, close to the
zz Ultraviolet light exposure nasal and temporal margins of the cornea
zz Drying of the cornea and repeated corneal but usually separated from them by a
trauma clear zone. On retroillumination, the haze
zz Corneal scars after keratitis, trachomatous can be resolved into small gray deposits,
keratopathy or trauma which look like ‘droplets’, immediately
zz Lattice corneal dystrophy beneath the epithelium. The deposits are
zz Glaucoma restricted to a nasal and temporal strip in
zz Microtrauma including sand, dust, wind, and each cornea but must be present at both
drying margins of the corneas.
—— Grade II: As the disease progresses, the
zz Herpes keratopathy.
deposits extend towards central cornea
into the optical axis. The disease is
EXAMINATION
restricted to the interpalpebral area of
Systemic Examination cornea.
—— Grade III: Characterized by central
One must look for chronic signs of sun damage
involvement over the pupil, sufficiently
on the skin in addition to routine systemic
dense to reduce vision to any degree. In
examination.
this stage, exposed band of the cornea
appears as ground glass.
Ocular Examination
Eye ball: It is usually normal.
Eyelid: If it is associated with inflammation, lid
edema may be presents. Features of associated
disease such as trichiasis (trachoma) may also be
there.
Conjunctiva: Signs of chronic sun exposure such
as keratinization, pigmentation, pinguecula, or
pterygium can be there. In addition, findings of
related disease such as conjunctival scar, herbert’s
pits must be looked for. If the nodules involve
the conjunctiva (type 3), clear to yellow-gold
spherules are seen interpalpebrally in the 3 o’clock
and 9 o’clock positions. The spherules are generally Fig. 1: Yellow-gold spherules in spheroidal
smaller and less numerous. They may be found in degeneration
3. Type 3: It is usually bilateral, involves the

conjunctiva too and may associated with
types 1.
MANAGEMENT
The management of CDK includes following:
Medical Management
It consists of artificial tear substitute, both in
the form of drops and ointments. In presence
of inflammation, topical steroid can be added.
Central nodules with recurrent erosion can benefit
Fig. 2: Coalescence of droplets to form from application of bandaged contact lens (BCL).
corneal nodules
Surgical Management
—— Grade IV: In this stage, the droplets The surgical management consists of following
coalesce to form large corneal nodules options:
(Fig. 2). These nodules may cause corneal
Conjunctival lesions: These nodules can be
epithelial defect.
directly excised.
zz In type 2, the spherules may be diffuse or begin
centrally. There can be associated corneal Central corneal lesions: These lesions can be
scarring and neovascularization. A clear managed in following ways:
interval is observed between the spherules zz Superficial keratectomy with or without
and neovascularization. amniotic membrane grafting (AMG):
Superficial keratectomy can be done using
Sclera/Anterior chamber/Iris: All these structures
a simple no. 15 blade or a crescent knife.
are usually within normal limits.
After keratectomy, a BCL is placed until
Lens: Since the age groups as well as the risk factors the epithelium heals. Application of AMG
are similar to that of senile cataract, these cases improves healing, decreases scarring and
are often associated with cataract. vascularization.
Fundus: The fundus may be normal or signs of age zz Excimer laser phototherapeutic keratectomy
related macular degeneration may be seen. (PTK): If the nodules cannot be removed
In addition, careful examination must be done completely by superficial keratectomy or
to rule out any complication such as: surface irregularity persists after keratectomy
zz Sterile ulceration, descemetocele or perforation than PTK has to be done. PTK is useful only
zz Corneal scar, macular or leucomatous for lesions extending up to 100 μm depth.
zz Recurrent corneal epithelial defect. Preoperative corneal thickness must be done
before proceeding with PTK. Postoperative
corneal haze and hyperopic shift are the major
CLASSIFICATION
complications associated with PTK.
CDK is classified into three basic types: zz Lamellar keratoplasty (LK)/anterior lamellar
1. Type 1 or primary form: It is usually bilateral, therapeutic keratoplasty (ALTK): LK is done
not associated with other ocular pathology. in cases where there is corneal scar and visual
2. Type 2 or secondary spheroidal degeneration: it potential is good.
may be unilateral or bilateral and is associated zz Penetrating keratoplasty: Penetrating kerato
with other ocular pathology. plasty is rarely required in cases of CDK.
careful examination shows that they lack the

VIVA QUESTIONS
granular quality and deep purple color of
Q.1. Histopathology of spheroidal degene- calcium crystals but rather are amorphous
ration. centrally homogeneous deposits.
Ans. In spheroidal degeneration deposits appear Q.2. What are the other names of spheroidal
as extracellular mauve colored amorphous degeneration?
globules, which may coalesce to form Ans. Already given in introduction section.
larger masses in Bowman’s membrane.
The globules are often confluent. These
BIBLIOGRAPHY
globules are made up of a protein material
with elastotic features. Histopathology—It 1. Chang RI, Ching S. Corneal and Conjunctival
is very easy to confuse these globules with degenerations. In: Krachmer JH, Mannis MJ,
calcification especially when they are in Holland EJ (Eds). Cornea, 2nd edition. Vol 1.
the cornea near Bowman’s layer. However, Philadelphia: Elsevier, Mosby. 2005;1:955.
CONGENITAL HEREDITARY ENDOTHELIAL DYSTROPHY

Prafulla Kumar Maharana, Vaishali Ghanshyam Rai, Manpreet Kaur
INTRODUCTION Age at onset is extremely important. Both types

are usually bilateral, symmetric, slowly progressive,
Congenital hereditary endothelial dystrophy but they differ in their age at onset. While CHED 1
(CHED) is a corneal endothelial dystrophy. CHED is usually evident in the 1st decade ( first year or in
is a disorder characterized by bilateral, symmetric, the second year of life) CHED 2 is usually evident
noninflammatory corneal clouding that is usually at birth or within the early postnatal period. Also
present at birth. There are two main forms, CHED 1 presence of nystagmus almost always suggests
and CHED2, the latter being more severe. CHED 2.
It is given as short case in postgraduate/DNB/
Diploma examination.
EXAMINATION
HISTORY Systemic Examination
Chief Complaints A complete examination is a must, preferably by a
Presenting complaints depend on the type of pediatrician.
CHED.
Child’s parents present with the following Ocular Examination
complaints.
Visual acuity: Often difficult to record, preferential
looking test should be performed whenever
CHED 1 possible.
zz Corneal clouding
Eye ball: Usually the size of eyeball is normal but if
zz Photophobia
it is associated with glaucoma then size of eyeball
zz Tearing
is increased.
zz Blurring of vision.
Eyelid: Usually normal. In presence of corneal
CHED 2 edema blepharophimosis may be there.
zz Nystagmus Conjunctiva: Usually normal but if it is associated
zz Blurring of vision. with glaucoma then congestion may be present.
Cornea: On slit lamp biomicroscopy following DIFFERENTIAL DIAGNOSIS

signs may be present.
zz Initially corneal clouding is seen (Fig. 1) The differential diagnosis of both types of CHED
zz Diffuse gray-blue ground-glass appearance are summarized below:
of cornea (Fig. 2). The corneal opacification
extends to the limbus without any clear zones. CHED1
zz Corneal thickness is increased two to three zz PPCD
times than normal and often greater than zz FECD
1 mm centrally (Fig. 3). zz Iridocorneal endothelial syndrome.
zz Epithelial surface is irregular.
zz Discrete white dots may also be seen in the CHED2
stroma.
zz In some areas where stromal opacification is zz Congenital glaucoma
less dense, Descemet’s membrane appears zz Congenital rubella
gray and on specular reflection may have a zz Forceps injury
peau d’orange texture. zz Peters’ anomaly
zz A fine corneal pannus may be seen. zz Mucopolysaccharidoses
Other ocular findings are usually within zz Congenital rubella.
normal limits. Mucopolysaccharidoses: Following features
differentiate it from CHED.
zz Corneal clouding is not present at birth and
usually develops within the first few years of
life
zz Cornea is not thickened
zz Systemic stigmata of MPS are typically present.
A urinalysis or corneal biopsy will usually
identify the abnormal metabolic product to
confirm the diagnosis.
Congenital glaucoma:
zz Increased intraocular pressure in glaucoma
zz Increase in corneal diameter
zz Haab’s striae
Fig. 1: Corneal clouding in CHED
zz Buphthalmos
All these features are absent in CHED.
Congenital rubella: In contrast to CHED, there is
episcleral injection, nuclear cataract, raised IOP,
posterior synechiae, miosis, chorioretinopathy.
Fig. 2: Diffuse gray-blue ground-glass appearance of Fig. 3: Increased corneal thickness (>1 mm) in CHED
cornea in CHED
Forceps injury: Edema is transient, localized, —— Later the onset better the prognosis
overlying the break in Descemet’s membrane and —— Most commonly done procedure.
unilateral in contrast to CHED. A double linear zz Descemet-stripping automated endothelial
scar in Descemet’s membrane at the rupture edges keratoplasty (DSAEK) (Fig. 5):
can be seen once the edema resolves. Compared to PK, DSEK offers the advantage
Peters anomaly: Other associated anterior seg of:
—— Faster visual recovery
ment abnormalities would be there.
—— No risk of complications of open globe
Congenital hereditary stromal dystrophy (CHSD):
surgery such as expulsive hemorrhage
zz Normal corneal thickness —— Less corneal astigmatism, less aberration
zz No edema but opacity that is full-thickness —— Relatively less chance of graft rejection
with feathery clouding of the stroma.
(although controversial)
PPCD, ICE and early-onset FECD: Especially —— Preservation of corneal tectonic stability.
when the patient presents late; the differentiating

features are summarized in Table 1.
INVESTIGATIONS
zz VER: For visual potential assessment.
zz Pachymetry: Corneal thickness increases 2–3
times.
zz Ultrasonography: For posterior segment
evaluation.
zz Specular/Confocal: Difficult to perform.
MANAGEMENT
zz Penetrating keratoplasty (Fig. 4):
—— Success rate 40–75% Fig. 4: Penetrating keratoplasty in CHED
Table 1 Differences between PPCD, ICE, FECD and CHED

Parameters PPCD FECD CHED 1 ICE
Onset Teens to 20s 40–50, early onset in 1st decade Young adults
1st–3rd decade
Hereditary AD AD AD No
Corneal Vesicles bands Guttae Marked Fine, guttae-like
findings Diffuse opacities Stromal thickening corneal changes, hammered
Corneal steepening Epithelial edema thickening and silver sppearance
(rare) Subepithelial fibrosis opacification
Other ocular Broad peripheral Narrow angles – Glaucoma
abnormalities synechiae Iris atrophy/corectopia
Specular Vesicles, bands, mosaic Polymorphism Often not Diffuse changes, ICE
microscopy Endothelial cells usually Polymegathism possible cell
enlarged but count may Decreased
be normal endothelial cell count
Abbreviations: PPCD, posterior polymorphous corneal dystrophy; FECD, Fuchs endothelial corneal dystrophy;
CHED 1, congenital hereditary endothelial dystrophy; ICE, iridocorneal endothelial dystrophy; AD, autosomal
dominant.
Table 2 Difference between CHED type-1 and 2

Parameters CHED1 CHED2
Inheritance AD AR
Gene 20p11.2–q11.2 20p13–12,
SLC4A11 gene
Corneal First decade At birth
clouding (Initial few (First week to
years of life) 6 months)
Nystagmus Absent Present
Amblyopia Absent Present
Photophobia More common Less common
Fig. 5: Descemet Stripping Automated Endothelial and tearing
Keratoplasty (DSAEK) in CHED Subepithelial More common Less common
fibrosis
with some
Disadvantage: calcification
—— Poor visibility
—— Risk of clear lens damage in phakic eye

Spheroidal More common Less common
degeneration
—— DM scoring is difficult.
If patients maintain good fixation with normal Abbreviations: CHED, congenital hereditary
alignment, surgery may be delayed; loss of endothelial dystrophy; AD, autosomal dominant;
fixation or development of nystagmus should AR, autosomal recessive.
lead to prompt intervention.
zz CHED associated with glaucoma:
—— Trabeculotomy
endothelium. Subsequent endothelial cell
—— Combined trabeculectomy with trabe-
death may lead to loss of barrier function
culectomy and progressive corneal edema.
—— Combined trabeculectomy with trabe
Q.2. Complication of CHED.
culectomy with subconjunctival collagen
Ans. • Subepithelial fibrosis/corneal pannus
matrix.
• Amblyopia
• Glaucoma
VIVA QUESTIONS • BSK
Q.3. What is Harboyan syndrome?
Q.1. Pathogenesis Ans. It is an autosomal recessive disease with
Ans. Mutations in the SLC4A11 (solute carrier CHED 2 and sensorineural deafness
family 4, sodium borate transporter, (CDPD).
member 11) gene is seen in CHED2 which
encodes a membrane-bound sodium borate Q.4. What is the difference between CHED 1
cotransporter. The transporter regulates and CHED 2?
intracellular boron concentration regulate Ans. See Table 2.
the growth and terminal differentiation
of neural crest cells. Loss of function of BIBLIOGRAPHY
this transporter may affect the normal 1. Weisenthal RW, Streeten BW. Descemet’s
restrictive pattern of corneal endothelial membrane and endothelial dystrophies. In:
synthesis and secretion that leads to failure Krachmer JH, Mannis MJ, Holland EJ. Cornea.
of growth regulation during the terminal St. Louis, MO: Mosby; 2011.
differentiation and reorganization of the
IRIDOCORNEAL ENDOTHELIAL SYNDROME

Vaishali Ghanshyam Rai, Neelima Aron, Prafulla Kumar Maharana
INTRODUCTION zz In the advanced stages of the syndrome,

symptoms of blurred vision and pain may
The iridocorneal endothelial (ICE) syndrome
persist throughout the day.
describes a group of disorders characterized by
zz Patients also may present with a chief
abnormal corneal endothelium that is responsible
complaint of an irregular shape or position of
for variable degrees of iris atrophy, secondary
the pupil (corectopia), or they may describe a
angle-closure glaucoma in association with
dark spot in the eye.
characteristic peripheral anterior synechiae (PAS),
and corneal edema. It can be given as a short case
in postgraduate exams. EXAMINATION
The following three clinical variations within Cornea
the ICE syndrome have been distinguished pri
marily on the basis of changes in the iris (Table 1). zz Corneal edema (Fig. 1) with abnormal
1. Progressive essential iris atrophy. corneal endothelium may be seen on specular
2. Chandler syndrome. reflection.
3. Cogan-Reese syndrome.
HISTORY
Epidemiology/Demography
The syndrome affects individuals between 20 and
50 years of age. It occurs more often in women
and is almost always unilateral. Glaucoma is
present in approximately half of all cases.
Chief Complaints
ICE can present in following ways:
zz Most common presentation is unilateral pain
and DOV which may be worse in the morning
and improves later in the day. Fig. 1: Corneal edema in ICE
Table 1 Iridocorneal endothelial syndrome: Variants1

Progressive (essential) Cogan-Reese
Parameters iris atrophy Chandler’s syndrome syndrome
Corneal endothelium abnormal Yes, may be subclinical Yes Yes
(slit lamp or specular microscopy)
Corneal edema Variable–late Present–early Present
Peripheral anterior synechiae Present Present Present
beyond Schwalbe’s line
Iris surface change Present Present Present
Iris atrophy Marked Minimal Variable
Iris nodules Appears late Appears late Appears early
Ectropion uveae Present Rare Present
Glaucoma Present Present Present
Fig. 2: Hammered-silver appearance on posterior Fig. 3: Iris atrophy in ICE

surface of cornea
zz A fine hammered-silver or beaten metal DIFFERENTIAL DIAGNOSIS

appearance on posterior surface of cornea
(Fig. 2) can be seen, similar to that of Fuchs’ Corneal Disorders
dystrophy. The two main differential diagnoses for corneal
endothelial disorders are posterior polymorphous
Iris
dystrophy (PPMD) and Fuchs endothelial dystro
zz Chandler syndrome usually presents with phy (Refer Table 1).
corneal edema and minimal iris alterations like zz PPMD is a bilateral disorder with autosomal
iris stromal atrophy (Fig. 3) and corectopia. dominant inheritance. Specular microscopic
zz Progressive iris atrophy is characterized by findings are different in two entities.
marked atrophy of the iris, associated with zz Fuchs endothelial dystrophy does not have the
variable degrees of corectopia and ectropion
anterior chamber angle or iris features seen in
uveae. The hallmark of progressive iris atrophy
ICE syndrome. Specular microscopic findings
is hole formation in the iris, which occurs in
are similar in both conditions.
two forms: stretch holes and melting holes.
zz Cogan-Reese syndrome is characterized by any
degree of iris atrophy, but the predominant Iris Disorders
feature is the presence of multiple, pigmented, Iris disorders that could be confused with ICE
pedunculated iris nodules. syndrome include Axenfeld-Rieger syndrome,
aniridia and iris melanosis.
IOP zz Axenfeld-Rieger syndrome has clinical and
In early stage, IOP may be in normal range with histopathologic similarities to ICE syndrome
minimal corneal edema. But in late stage, high IOP but differs in being bilateral and congenital.
with marked corneal edema can be noted. The iris and angle alterations in Axenfeld-
Rieger syndrome are due to retention and
Gonioscopy contraction of a primordial endothelial
Peripheral anterior synechia (PAS) on gonioscopy, layer, whereas changes in ICE syndrome
usually extending to or beyond Schwalbe’s line, is are secondary to migration and subsequent
another clinical feature common to all variations contraction of abnormal corneal endothelial
of the ICE syndrome. cells.
Other ocular structures are usually within zz Aniridia is usually bilateral and congenital
normal limits. absence of the iris tissue.
zz Iris melanosis should be differentiated from TREATMENT

Cogan-Reese syndrome. Iris melanosis is
typically bilateral and familial. Glaucoma is
zz Medical treatment: Aqueous suppressant
uncommon. medications are effective in controlling the
IOP. Corneal edema may often be controlled
using hypertonic saline solutions.
INVESTIGATIONS zz Surgical treatment: If the IOP level remains
Specular Biomicroscopy uncontrolled despite medical treatment,
filtration surgery is indicated.
zz Specular microscopic appearance of corneal
endothelial cells in the ICE syndrome
demonstrates the pleomorphism in size and VIVA QUESTIONS
shape, dark areas within the cells, and loss of
clear hexagonal margins. Q.1. Clinical variants of ICE.
zz Endothelial changes studied by specular Ans. See Table 1
microscopy are typical: in early stages, a
rounding off of cell angles and intracellular Q.2. What is an ICE cell?
blackout areas can be seen; in former stages, Ans. The endothelial mosaic, on specular micr
black out areas increase and there is a oscopy, may contain a typical ICE cell
disruption of the regular mosaic. in which the hexagonal borders are lost,
zz Fellow eye specular microscopy is important, a light or dark area is seen within, and
which is normal in ICE syndrome whereas reversal of the usual normal light/dark
abnormal or similar to other eye in Fuchs pattern occurs. Oval dark and light bodies
dystrophy. within cell boundaries and smaller round
zz Less role in ICE syndrome with marked corneal structures with either a bright or dark
edema. appearance near cell centers are thought to
zz ICE cells—in which the hexagonal borders are be endothelial cell nuclei and blebs of the
lost, a light or dark area is seen within, and apical cell membrane. Epithelialization of
reversal of the usual normal light/dark pattern the endothelial cells are thought to be the
occurs. Although it is characteristic but not histological correlate of the ICE cell seen
necessary for the diagnosis. on specular microscopy. These cells are
usually seen in Chandler’s syndrome.
Pachymetry Q.3. What is Iris nevus syndrome?
Increased central corneal thickness (CCT) in Ans. It is considered to be a variant of Cogan-
involved eye due to corneal edema whereas Reese syndrome. It is characterized by loss
normal in fellow eye. of surface architecture of the iris resulting
in a matted appearance, ectropion uvea,
heterochromia, PAS, corneal edema, and
Confocal Microscopy
unilateral glaucoma.
zz Important diagnostic tool in case of corneal
edema to study corneal endothelium
REFERENCE
morphology.
zz Confocal microscopy may be used to diagnose 1. Carpel EF. Iridocorneal endothelial syndrome.
the ICE syndrome by demonstrating epithelial- In: Krachmer JH, Mannis MJ, Holland EJ.
like endothelial cells with hyperreflective Cornea. St. Louis, MO: Mosby; 2011.
nuclei. Normal in fellow eye.
PETERS’ ANOMALY
Shipra Singhi, Neelima Aron, Manpreet Kaur
INTRODUCTION can present at any time. However, most commonly

it develops soon after birth.
Peters’ anomaly is a developmental abnormality
of the cornea, usually present at birth and is
characterized by a central white opacity (leukoma)
EXAMINATION
with a lucent periphery. Most cases are sporadic General Examination
and bilateral in 80% of cases. It is associated
Detailed systemic examination of a child is
with glaucoma, presumably due to abnormal
required to rule out any cardiac disease, central
development of aqueous humor drainage
nervous system anomaly, deafness or delayed
structures. Approximately 50–70% of the patients
development. Peters’ plus syndrome refers to
with Peters’ anomaly will develop glaucoma,
patients with Peters’ anomaly associated with cleft
which is frequently present at birth.
lip and palate, short stature, abnormal ears, and
In exams, it can be given as a short case.
mental retardation. General examination must be
done carefully to rule out all these associations.
HISTORY
Chief Complaints Ocular Examination
zz Parents may complain of unilateral or bilateral Most of the time the examination has to be done
white opacity or hazy eyes of the child since under general anesthesia.
birth. Visual acuity: Child may have poor fixation to light
zz Photosensitivity or lacrimation since birth and may resist occlusion of better eye.
may be there.
zz Infant with cardiac disease or neurological Eye ball: Microphthalmos is common with Peters’
anomaly may be referred by Pediatrician to anomaly.
ophthalmologist to rule out Peters’ anomaly Cornea: Following findings can be there:
(Peters’ plus syndrome). zz Bilateral central leukomatous corneal opacity
with lucent periphery is characteristic. Look
Perinatal History for central or paracentral corneal edema or
central or paracentral corneal opacity with a
A detailed antenatal history, history of delayed
lucent periphery. The size and density of the
development milestones and history of cardiac
central defect is variable. It can range from
disease, central nervous system anomalies or deaf
a small, faint opacity to a dense leukoma
ness should be asked as there are many systemic
precluding visualization of the anterior
associations with Peters’ anomaly.
chamber.
zz Anterior chamber: AC depth may be shallow or
History of Present Illness irregular.
Corneal edema may be present early in the course zz Iris: Iris strands arising from collarette attached
of the disease and may persist or recur in the face to periphery of corneal opacity is an important
of elevated intraocular pressure. The edema may sign. Iris coloboma can be seen.
progressively resolve as peripheral endothelial zz Lens: Look for lenticular adhesion to posterior
cells migrate over the posterior defect, leaving an corneal surface which is a sign of type II Peters’
overlying corneal scar. Corneal edema may recur anomaly. Look for presence of cataract that is
later in life secondary to the natural attrition of frequently seen in cases of Peters’ anomaly.
already compromised endothelial cells. Glaucoma Cataract can be from a primary lens anomaly
develops in approximately 50–70% of patients and or due to a secondary change as the lens is
pushed forward against the cornea. The lens The urinalysis shows the abnormal metabolic
may touch or be adherent to the cornea, but product. Systemic features of the underlying
can also be in its normal position and yet be cause will be there.
cataractous. zz Congenital hereditary endothelial dystrophy
Gonioscopy: Using direct gonioscopy look for (CHED): The cornea is usually diffusely hazy as
peripheral anterior synechiae or angle anomalies opposed to centrally hazy in Peters’ anomaly.
which is the main cause of glaucoma in Peters’ Corneal thickness is 2–3 times increased as
anomaly. compared to PA.
IOP: Raised IOP may be there when associated

glaucoma is there. Glaucoma is seen in 50–70% of Table 1 Causes of congenital corneal
the cases. Glaucoma in PA can appear at any age opacities—STUMPED classification
but it is most commonly seen soon after birth. Category Disease Subcategories
Posterior segment: Look for choroidal coloboma, S Sclerocornea
persistent hyperplastic primary vitreous, and optic T Tears in • Congenital glaucoma
nerve hypoplasia which can be seen in cases of PA, Descemet's • Birth trauma
although rarely. membrane
U Ulcer • Herpes simplex virus
DIFFERENTIAL DIAGNOSIS • Bacterial
Other causes of central corneal opacities in infants • Neurotropic
(Table 1) and the differentiating points (Table 2) M Metabolic • Mucopolysacchari-
are summarized below: (Rarely doses
zz Congenital glaucoma: Breaks in Descemet’s present at • Mucolipidoses
membrane (Haab’s striae) as well as birth) • Tyrosinosis
buphthalmos are common. P Posterior • Peters’ anomaly
zz Birth trauma: Birth trauma is usually unilateral corneal • Posterior keratoconus
and should demonstrate breaks in Descemet’s defect • Staphyloma
membrane. Only corneal edema will be there E Endothelial • Congenital hereditary
without any opacity characteristic of PA. dystrophy • Posterior poly-
zz Mucopolysaccharidoses: The cornea is usually morphous corneal
diffusely hazy with no stromal thickening dystrophy
as opposed to centrally hazy with stromal • Congenital stromal
edema and clear periphery in case of PA. corneal dystrophy
Table 2 Differential diagnosis of congenital corneal opacity

Peters’ anomaly Sclerocornea Dermoids CHED PPCD
Central Diffuse full-thickness Yellowish-white Diffuse corneal Diffuse corneal
leukomatous corneal opacity, vascularized elevated edema edema (less than
opacity with encroaching from nodules that may contain CHED)
normal peripheral periphery. hair follicles, sebaceous
cornea Center relatively clear and sweat glands, smooth
and skeletal muscle,
nerves, blood vessels,
bone, cartilage, and teeth
Lens normal or Usually normal Usually normal Usually normal Usually normal
abnormal
Bilateral Bilateral but Unilateral Bilateral Bilateral
asymmetric
CLASSIFICATION VIVA QUESTIONS

zz Type I: Consists of a central or paracentral
Q.1. Ocular anomalies associated with Peters’
corneal opacity with iris strands that arise from
anomaly.
the collarette and attach to the periphery of the
Ans. Chorioretinal coloboma, iris coloboma,
opacity.
persistent hyperplastic primary vitreous,
zz Type II: It has lens adherence to the posterior
microphthalmos, and optic ner ve
cornea. Type I usually is unilateral, while type
hypoplasia.
II frequently is bilateral.
zz Peters plus syndrome: Characterized by Peters’ Q.2. Systemic associations with Peters’
anomaly in association with cleft lip/palate, anomaly.
short stature, abnormal ears, and mental Ans. • K rause-Kivlin syndrome (inheritance
retardation. is autosomal recessive): The systemic
associations of Peters’ anomaly include
MANAGEMENT short stature, facial dysmorphism,
developmental delay, and delayed
Treatment of Corneal Opacity skeletal maturation.
It depends upon the location and size of corneal • The Peters’-plus syndrome consists of
opacity. In addition the presence of glaucoma also Peters’ anomaly cleft lip and palate,
need to be considered. short stature, abnormal ears, and mental
zz Small central opacity and a clear lens: A large retardation.
peripheral iridectomy (Optical iridectomy) Q.3. Histopathological findings of cornea in
may permit a formed retinal image. Mydriatic Peters’ anomaly.
therapy in cases where the opacity is not large Ans. There are abnormalities in all layers of the
can be considered in an effort to reduce the cornea which include:
likelihood of amblyopia, while awaiting a more • Disorganized epithelium
definitive procedure. • Loss of Bowman’s layer
zz Large central corneal opacity and a clear lens: • Stromal edema at the affected area
Penetrating keratoplasty may be required to • An abrupt absence or marked attenuation
clear the visual axis. of the endothelium and Descemet’s
zz Corneal opacity with cataract: A triple membrane underlying the corneal
procedure or a cataract surgery with IOL with opacity. Peripheral to the opacity, the
pupilloplasty (when CO is small) can be done endothelium is normal.
in such cases. The success rate of penetrating
keratoplasty in PA is between 22% and 83%. Q.4. What are the risk factors for graft
Prognosis is poor in presence of glaucoma and rejection in penetrating keratoplastomy
in type 2 PA. in cases of Peters’ anomaly?
Ans. Anterior synechiae, co-existing glaucoma,
Treatment of Glaucoma large corneal grafts and central nervous
system abnormalities.
zz Topical antiglaucoma medications and oral
carbonic anhydrase inhibitors are effective in Q.5. Genetics of PA
few cases. Most cases require surgery for IOP Ans. Most cases are sporadic; however, auto
control. somal recessive and autosomal dominant
zz Surgical treatment such as trabeculectomy, pedigrees have also been reported rarely.
tube-shunt procedures, or cyclodestructive Genetic mutations in four genes have been
procedures are done in cases of glaucoma described in PA. These are
refractory to medical management. 1. PAX6 gene associated with aniridia.
2. PITX2 genes associated with Axenfeld- BIBLIOGRAPHY

Rieger syndrome. 1. Parikh M, Alward WLM. Axenfeld-Rieger
3. FOXC1 genes associated with Axenfeld- syndrome and Peters’ anomaly. In: Krachmer
Rieger syndrome. JH, Mannis MJ, Holland EJ. Cornea. St. Louis,
4. CYP1B1 gene associated with primary MO: Mosby; 2011.
congenital glaucoma.
LIMBAL DERMOID
Shipra Singhi, Deepali Singhal, Neelima Aron
INTRODUCTION lesions may be dormant for many years or have

intermittent growth.
Limbal dermoids are benign congenital tumors zz Painless mass (cosmetic deformity)
that contain choristomatous tissue (tissue not zz Foreign body sensation/irritation
found normally at that site). They appear most zz Drying of eyes
frequently at the inferior temporal quadrant of the zz Diminution of vision: Blurring of vision is
corneal limbus. However, they may occasionally related to size, astigmatism and involvement
present entirely within the cornea or may be of visual axis. It is usually progressive and
confined to the conjunctiva. They may contain a painless.
variety of histologically aberrant tissues, including zz Diplopia due to mechanical restriction of
epidermal appendages (Fig. 1), connective tissue, ocular movements.
skin, fat, sweat gland, lacrimal gland, muscle,
teeth, cartilage, bone, vascular structures, and
neurologic tissue, including the brain. Malignant
Past History
degeneration is extremely rare. It can be given as a A careful past history should be taken of:
short case or spot case in exam. zz Mass
zz Facial asymmetry
Chief Complaints zz Hearing loss
In adults, dermoids may become symptomatic zz Cardiovascular disease
for the first time and grow considerably over a zz Infection
year. Based on this fact, some conclude that these zz Trauma
zz Ocular inflammatory diseases.

Previous history of intraocular surgery may or may
not be present.
EXAMINATION
Cardiovascular abnormalities, facial hemiatrophy,
atresia of the external auditory meatus, accessory
auricles, nevus flammeus, neurofibromatosis.
preauricular appendages, and pretragal fistulas
should be examined if present.
Fig. 1: Limbal dermoid with epidermal appendages One-third of cases associated with Goldenhar’s
such as hair syndrome. It is a non-familial syndrome that
presents with a classic triad of epibulbar dermoids, bilateral, the former being the more common.
preauricular appendages, and pretragal fistulas. Dermoids can be central and often appear to
have satellite lesions.
Ocular Examination zz Dellen formation may occur.
zz Anterior staphyloma can be present.
Visual acuity: It is variably impaired depending on zz Aniridia may be present.
the site of involvement, pupil encroachment size zz Anterior segment dysgenesis—may be present
and astigmatism.
in severe cases.
Cycloplegic refraction: Irregular astigmatism- zz Lens involvement can occur. Congenital
compound hypermetropic. cataract or aphakia may be there.
Amblyopia zz Neuroparalytic keratitis can occur.
zz Anisometropic or strabismic amblyopia
zz Stimulation deprivation amblyopia. Tonometry
Eye ball IOP usually normal.
zz Microphthalmos
zz Anophthalmos Gonioscopy
zz Upper eyelid coloboma
Posterior corneal protrusion, synechiae or pig
zz Lower eyelid coloboma.
mentation, dermoid involving ciliary body.
Lacrimal system: Lacrimal stenosis
Fundus: There may be:
Extraocular movement: Duane’s retraction zz Irido fundal coloboma
syndrome. zz Hypoplastic disc.
Slit Lamp Biomicroscopy Complications

Anterior Segment zz Recurrent dermoid
zz Dellen formation
zz Limbal dermoid: Yellowish-white, solid, zz Amblyopia
vascularized, elevated nodules straddling the zz Strabismus.
corneal limbus (Fig. 2). Size may vary ranging
from 2 mm to 15 mm in diameter. Corneal
dermoids occur as single lesions mostly but
may be multiple, and they may be unilateral or Differential diagnosis of dermoids (Table 1).
Others
zz Ectopic lacrimal gland
zz Lymphoma
zz Dermolipoma
zz Corneal scar (infection or trauma)
zz Pterygium, atypical
zz Foreign body granuloma
zz Epibulbar dermoid
zz Episcleral osteoma
zz Juvenile xanthogranuloma.
INVESTIGATIONS
The diagnosis of a limbal dermoid requires a direct
clinical examination. Specific laboratory studies
Fig. 2: Limbal dermoid are generally not necessary.
Table 1 Differential diagnosis of dermoids

Dermoids Corneal keloids CHED Peters’ anomaly Sclerocornea
Yellowish-white Chalky white Diffuse corneal Corneal opacity Loss of transition
vascularized elevated solid masses edema bilaterally; + iridocorneal between cornea
nodules with glistening cornea is not adhesions with and sclera
gelatinous vascularized; there or without lens
texture are never hair abnormality (position
follicles present or transparency)
Inferotemporal at the Inheritance may Denser corneal Cornea plana
limbus junction be recessive or opacity commonly
dominant associated
May contain hair follicles, Most frequently Peripheral
sebaceous and sweat bilateral cornea more
glands, smooth and opacified than
skeletal muscle, nerves, central cornea
blood vessels, bone,
cartilage, and teeth
Usually unilateral Surface
vascularization
Can be central but do
not involve the most
peripheral cornea (leaving
a definite sclerocorneal
junction) and often appear
to have satellite lesions
Imaging Studies with an anterior segment high resolution

B-scan (ultrasound biomicroscopy) to
zz MRI:
—— Some dermoids may appear to extend into
assess for involvement of Descemet’s
the conjunctival fornix or lateral canthus. membrane. These steps are necessary in
—— These lesions may contain connective
order to plan for the appropriate surgical
tissue that entangles with the orbital approach. Meticulous biomicroscopic
fat and muscle tissue belonging to the ultrasound examination is needed to
extraocular muscles. improve the depth of corneal penetration
—— Radiologic imaging with an MRI can for sound waves, since studies have
be useful in identifying such lesions, demonstrated that dermoids produce
especially if surgical management is strong sound attenuation, reducing
being considered. the visibility of deep corneal structures
—— Biopsy is not necessary except in rare and in particular Descemet’s membrane.
instances when the diagnosis is doubtful. zz Anterior segment OCT:
—— Anterior segment OCT is done to deter
zz UBM: To determine the depth of the corneal
tissue involvement. mine the depth and posterior extension of
—— Ultrasound biomicroscopy may serve the lesion.
as a useful diagnostic adjunct for limbal zz Histologic findings:
dermoids. Additionally, it may be helpful —— Limbal dermoids contain choristomatous
in delineating the extent of these lesions. tissue, including epidermal appendages,

—— Clinical examination may be done under adipose and lacrimal gland tissue, smooth
general anesthesia in pediatric along and striated muscle, cartilage, brain, teeth,
and bone. Lymphoid nodules and vascular Surgical Management

elements also have been reported. The zz Shave and excision
surface of the dermoids consists of corneal zz Shave and excision with lamellar keratoplasty
or conjunctival epithelium. The lesion
(LK)
may be cystic or solid. zz Shave and excision with patch graft
zz Shave and excision penetrating keratoplasty
Systemic Work-up with relaxing corneal incisions
zz X-ray of spine-for hemivertebra or scoliosis zz Shave and excision with corneal-limbal scleral
zz ECG donor graft transplantation
zz Echocardiography—for cardiac defect zz Shave and excision with amniotic mem
zz MRI of brain brane graft/limbal stem cell allograft/
zz Complete blood count pericardial graft—improves postoperative
zz Renal function test reepithelialization, prevents postoperative
zz Audiometry for hearing assessment. scarring, and protects the limbal stem cells.
zz Use of mitomycin C may improve the results.
MANAGEMENT zz It can reduce recurrences by the inhibition
of fibroblast proliferation at the level of the
Indications for Surgery episclera. Therefore, the use of mitomycin C
zz Cosmetic deformity can be beneficial in the treatment of limbal
zz Involvement of the visual axis dermoids in matters of postoperative compli
zz Regular or irregular astigmatism cations like formation of pseudopterygium.
zz Amblyopia zz Management of amblyopia: Occlusion treat
zz Strabismus ment, chemical penalization with/without
zz Dellen formation spectacle wear/contact lens (in unilateral
zz When the lesion becomes progressive and cases) must be continued after surgical
starts to increase in size or cause irritative excision to obtain optimal results if the surgery
symptoms. is done at a younger age.
In a small, asymptomatic orbital epidermal
dermoid cyst no immediate treatment is required. Prognosis of Surgical Outcome
Medical Management Extent of

zz Medical management is generally reserved for corneal
Depth involve-
grade I dermoids which are smaller lesions in
of ment from Surgery
terms of diameter and height involve limbus to be Progno-
zz Inducing only mild astigmatism of <1 D with Grade ment % (mm) planned sis
minimal surface irregularity
I <50 <3 Excision Excellent
zz Parents report relatively good compliance
(Ex)
with spectacle correction. Essentially small
asymptomatic grade I limbal dermoids should II <50 3–5 Excision Good
>50 <3 Excision
not be removed because they may lead to
+ lamel-
postoperative scarring and development
lar kera-
of pseudopterygium. It is recommended toplasty
that these children undergo close clinical (LK)
observation with serial examinations in the
III <50 5.1–7 Excision Fair
office, not only to monitor stability but also to >50 3–5 Ex/Ex+LK
provide reassurance for parents.
zz Occlusion therapy. Contd…
Contd… Contd…
Extent of Grade 1 (limbal or
corneal epibulbar) Grade 2 Grade 3
Depth involve-
Single Variable depth Entire anterior
of ment from Surgery
of stromal segment is
involve limbus to be Progno-
extension involved
Grade ment % (mm) planned sis
Inferotemporal Does not Associated
IV <50 >7 Excision Guarded
limbus involve abnormalities:
>50 5.1–7 +LK
Descemet’s Microphthal-
Excision
membrane or mos, posterior
+LK
the corneal segment
V >50 >7 Excision Poor endothelium abnormalities
+LK
It may enlarge
(especially at
Complications of Surgery puberty)
zz Residual vascularization Superficial
zz Corneal scar One-third of
zz Persistent epithelial defect cases associated
zz Pseudopterygium formation with Goldenhar's
zz Ocular perforation syndrome:
zz Recurrent dermoid. Nonfamilial; triad of
epibulbar dermoids,
Corneal Choristoma Classification preauricular
appendages, and
Stargardt Scheme pretragal fistulas
Grade I Microphthalmos, no involvement of lens Other abnormalities:
Grade II Lens involvement Coloboma of
Grade III Cornea only the lids, aniridia,
Grade IV Limbal dermoid microphthalmos,
anophthalmos,
neuroparalytic
Corneal Choristoma Classification keratitis, lacrimal
Mann’s Scheme stenosis, Duane's
syndrome,
Grade I Limbal or epibulbar dermoid cardiovascular
Grade II Superficial abnormalities,
Grade III Anterior segment involvement with or facial hemiatrophy,
without microphthalmos atresia of the
external auditory
Classification meatus, accessory
auricles, nevus
Grading of Dermoids flammeus, and
neurofibromatosis
Grade 1 (limbal or
epibulbar) Grade 2 Grade 3
VIVA QUESTIONS
Most frequent type Much larger Most severe
type
Q.1. What is the epidemiology of dermoid?
Small (5 mm in Covers part or Very rare Ans. Epidemiology:
diameter) entire central • Congenital choristoma
corneal surface • Account for 3–8% of orbital tumors in
Contd… children
• T he dermoid cyst becomes the most 75 of 1016 such lesions were documented
common noninflammatory space- to be epibulbar choristomas, with more
occupying lesion of the orbit. than 80% of lesions noted to be located
• In the Wills Eye Hospital pathology temporally and inferiorly. In another study
series, dermoid cyst accounted for 46% at the Wilmer Eye Institute of Pathology,
of childhood orbital lesions and for 89% choristomas comprised 33% of all epibulbar
of all cystic lesions. lesions in individuals younger than 16 years
Q.2. What are the common variants of ocular of age. This study showed that these lesions
dermoids? may sometimes be associated with other
Ans. There are two main dermoid types that ocular findings, including scleral/corneal
occur on or around the eyes. First, an orbital staphyloma, aniridia, congenital aphakia,
dermoid is typically found in association cataract, and microphthalmia.
with the bones of the eye socket (closure of The pattern of inheritance is quite
embryonic sutures). Second, an epibulbar variable in epibulbar choristomas. They
dermoid is found on the surface of the can be autos omal dominant, recessive,
eye. There are two typical locations for an X-linked, or multifactorial.
epibulbar dermoid. One of the locations Q.4. What is Goldenhar syndrome?
is at the junction of the cornea and sclera Ans. The characteristic features of Goldenhar
(limbal dermoid). The second location of syndrome (also known as Oculo-
an epibulbar dermoid is on the surface of Auriculo-Vertebral spectrum, craniofacial
the eye where the lids meet in the temporal dysostosis, or first and second branchial
corner (towards the ear). arch syndrome) are summarized in Table 2
Q.3. What is the inheritance pattern and (Figs 3 and 4)
site of involvement of ocular epibulbar Differential diagnoses for Goldenhar syn
dermoids? drome (especially the facial abnormalities):
Ans. A study by Nevares et al. indicates that the • Treacher Collins syndrome
majority (76%) of ocular dermoids occur • Romberg disease (hemifacial atrophy)
at the inferotemporal bulbar location of seen later in life could have a similar
the eye, with the other 22% reported to appearance to hemifacial microsomia
occur superotemporally. In a study by • Craniosynostosis
the Armed Forces Institute of Pathology, • Hemifacial microsomia.
Table 2 Goldenhar syndrome

Epidemiology Signs
• Sporadic, no documented • Limbal dermoids (bilateral in 25% of cases)
inheritance pattern • Eyelid colobomas
• No proven environmental insult • Preauricular appendages/skin tags
during pregnancy (medication, • Microtia or anotia of external ear, can be associated with hearing loss
infection, or otherwise) with or without middle ear malformation
• Males affected 2:1 compared to • Vertebral abnormalities (butterfly vertebrae or hemivertebrae)
females • Congenital heart disease (numerous anomalies have been reported)
• Incidence between 1 in 3,000 and • Central nervous system abnormalities (hydrocephalus, intracranial
5,600 live births lipomas, cranial nerve dysgenesis and mental retardation have been
described)
Contd…
Contd…
Symptoms Treatment
The syndrome is almost always • Large eyelid colobomas resulting in exposure keratopathy may
diagnosed early in life, before there require surgical repair
is any complaint of symptoms by • Spectacle
the infant patient. Symptoms could • Superficial keratectomy may be required to excise large limbal
include: dermoids causing occlusive or astigmatic amblyopia or exposure
• Double vision (motility restriction • Cleft lip and palate will require surgical repair, if present
or strabismus) • Severe underdevelopment of the mandible may require
• Dry eye (exposure due to reconstruction, perhaps with the aide of a bone graft (i.e. from the rib)
coloboma or large dermoid) • In cases of microtia or other ear defect, external ear reconstruction
is generally done between 6 and 8 years of age and is a multistage
process
• Further facial reconstruction may be required
• Cardiac defects (ventricular or atrial septal defect, other) are treated
accordingly
• If the facial or tongue malformation is severe, speech therapy may be
indicated
Fig. 3: Limbal dermoid with preauricular tags Fig. 4: Goldenhar syndrome with limbal dermoid and
ear anomalies
BIBLIOGRAPHY 5. Burillon C, Duran L. Solid dermoids of the

limbus and the cornea. Ophthalmologica.
1. American Academy of Ophthalmology. 1997;211:367-72. [PubMed]
Basic and Clinical Science Course. Amercian 6. Cohen J, Schanen NC. Branchial cleft anomaly,
Academy of Opthalmology; 2012. (Series 6). congenital heart disease, and biliary atresia:
2. Ash JE. Epibulbar tumors. Am J Ophthalmol. Goldenhar complex or Lambert syndrome?
1950;33:1203-19. [PubMed] Genet Couns. 2000;11(2):153-6.
3. Bayraktar S, Bayraktar ST, Ataoglu E, Ayaz A, 7. Goldenhar M. Associations malformatives de
Elveli M. Goldenhar’s syndrome associated l’oeil et l’oreille, en particulier le syndrome
with multiple congenital abnormalites. J Trop dermoide épibulbaire-appendices auriculaires-
Pediatr. 2005;51(6):377-9. fistula auris congenita et ses relations avec la
4. Beck AE, Hudgins L, Hoyme HE. Autosomal dysostose mandibulo-faciale. J Genet Hum.
dominant microtia and ocular coloboma: 1952;1:243-82.
new syndrome or an extension of the oculo- 8. Gorlin RJ, et al. Oculo-auriculovertebral
auriculo-vertebral spectrum? Am J Med Genet dysplasia. J Pediatr. 1963;63:991-9.
A. 2005;1;134(4):359-62.
9. Grossniklaus HE, Green WR, Lukenbach M, 14. Mohan M, Mukherjee G, Panda A. Clinical
et al. Conjunctival lesions in adults: a clinical evaluation and surgical intervention of limbal
and histopathological review. Cornea. 1987;6: dermoid. Indian J Ophthalmol. 1981;29:69-73.
78-116. [PubMed] [PubMed]
10. Mann I. Developmental abnormalities of the 15. Nevares RL, Mulliken JB, Robb RM. Ocular
eye. Cambridge, UK: Cambridge University dermoids. Plast Reconstr Surg. 1988;82:959–64.
Press; 1937. [PubMed]
11. Mann I. In: Developmental abnormalities of the 16. Oculoauriculovertebral dysplasia. Online
eye. 2nd edition. Mann I (Ed). Philadelphia, PA: mendelian inheritance in man. www.ncbi.nlm.
Lippincott; 1957. nih.gov/entrez/dispomim.cgi?id=164210.
12. Mansour AM, Barber JC, Reinecke RD, Wang 17. Schaefer, Bradley G, Olney A, Kolodziej P.
FM. Ocular choristomas. Surv Ophthalmol. Oculoauriculo-vertebral spectrum. ENT—Ear,
1989;33:339-58. [PubMed] Nose & Throat Journal. 1998;77:17-8.
13. Mattos J, Contreras F, O’Donnell FE., Ring 18. Singer SL, Haan E, Slee J, Goldblatt J. Familial
dermoid syndrome. A new syndrome of auto hemifacial microsomia due to autosomal
somal dominantly inherited, bilateral, annual dominant inheritance. Case reports. Aust Dent
limbal dermoids with corneal and conjunctival J. 1994;39(5):287-91.
extension. Arch Ophthalmol.1980;98:1059-61. 19. Stoll C, Viville B, Treisser A, Gasser B. A family
[PubMed] with dominant oculoauriculovertebral.
CHAPTER
3
Glaucoma
LONG CASES
PRIMARY OPEN ANGLE GLAUCOMA

Dewang Angmo, Vaishali Ghanshyam Rai, Ritika Mukhija
INTRODUCTION
Glaucoma is a chronic, degenerative optic
neuropathy which may or may not be associated
with raised intraocular pressure (IOP). The
glaucomas are classified by the appearance of the
iridocorneal angle into two broad categories open
angle (Fig. 1) and closed angle. In open-angle
glaucoma (OAG) the iridocorneal angle is open
(unobstructed) and normal in appearance but
aqueous outflow is diminished.1 It may be of primary
or secondary type. Primary (no other associated
disease) open-angle glaucoma (POAG) includes
both adult-onset disease (occurring after 40 years of
age) and juvenile-onset disease (occurring between Fig. 1: Open angles on gonioscopy
the ages of 3 and 40 years of age). Secondary Abbreviation: TM, trabecular meshwork
(secondary to some other disease in eye) OAG
include those associated with pseudoexfoliation leading cause of blindness in the United States
or pigment dispersion syndrome.1 Primary open and the leading cause of blindness among black
angle glaucoma case is commonly kept in practical Americans. In India it accounts for almost half of
examination as long case.2 the cases (5.8% of blindness in India is attributable
to glaucoma). Gender predilection is controversial,
HISTORY most study reports no predilrection.1,2
Chief complaints: POAG can present in following
Epidemiology ways:
Primary open angle glaucoma (POAG) primarily zz Commonly an incidental finding on ocular
affects persons >40 years of age, which is the second examination.
zz POAG has no associated symptoms or other and diabetic patients usually show more
warning signs before the development of association with POAG.
advanced visual field loss. zz Vasospastic diseases: Migraine, Raynaud’s
zz Patient may present with eye pain and redness syndrome may be associated with increased
and gradually diminishing vision for distance. incidence of glaucoma.
zz Other complaints can be blurred vision with or zz Hemodynamic crisis: Acute blood loss (post-
without color halos, frequent change of glasses partum hemorrhage, ruptured abdominal
or early morning or afternoon blurred vision aneurysm, severe trauma, stroke) can cause
with or without heaviness in eyes depending severe systemic hypotension, destabilizing the
on IOP peak. ocular blood flow and increase the optic nerve
damage.
History of Present Illness zz Endocrine: Diabetes and thyroid may have
History must include the onset and progression of an increased risk of glaucoma. Cushing’s
vision loss. In addition, following points must be syndrome cause endogeneous release of
noted: corticosteroids.
zz Brief history of patient’s previous records to get zz It is very important to ask about cardiovascular
baseline IOP. disease, renal diseases and bronchial asthma
zz Patient who is already on treatment for before deciding on antiglaucoma treatment.
glaucoma, it is important to know how many zz History of disorders which cause endogeneous
medications he/she is using and whether this release of corticosteroids, e.g. Cushing’s
treatment has sufficiently controlled IOP and syndrome must be enquired.
visual field loss.
zz Patient’s previous visual fields record should Drug History
be checked to know the progression of zz History of use of ocular and systemic
glaucoma. medications, especially if patient is already on
zz Any other record such as OCT, GDx. systemic beta-blockers (topical beta-blockers
would work sub-optimally) must be enquired.
History of Past Illness zz Known local or systemic intolerance to ocular
Past history of ocular trauma is important to rule or systemic medications.
angle recession glaucoma. zz History of long-term use of corticosteroid in
any form like eye drop, nasal spray, systemic
Surgical History is important to rule out steroid induced
glaucoma.
History of previous ocular surgery like cataract zz Long-term systemic use of steroids following
surgery, retinal surgery, trabeculectomy, any major organ transplantation surgery, e.g
penetrating keratoplasty or any laser procedure liver or kidney transplant surgery.
such as peripheral iridotomy (PI) must be zz long-term topical use of steroids following
recorded. keratoplasty or chronic allergic keratitis, e.g.
vernal keratoconjunctivitis.
History of Systemic Illness zz History of systemic use of topiramate needs to
zz Hypertensive, thyroid diseases and diabetic rule out.
patients are at increased risk of developing
zz Any hypersensitivity reaction to anti-glaucoma
POAG. medications both topical and sulpha allergy.
zz Cardiovascular disease: Systemic hypertensive
(HTN) has weak association with glaucoma. Family History
Beta-blockers given for systemic HTN can Relatives of POAG patients are at higher risk for
reduce IOP and so a patient who is on systemic developing glaucoma. The severity and outcome
beta blockers, topical ones should be avoided of glaucoma in family members, including history
as the first line therapy. Thyroid diseases of visual loss from glaucoma is also important.
Glaucoma 173
EXAMINATION IOP elevation, such as angle recession, pigment

dispersion, peripheral anterior synechiae, angle
General Examination/Specific Systemic neovascularisation (Fig. 1).
Examination
Optic disc and retinal nerve fiber layer: It is best
Detailed systemic examination to rule out neuro done using +90D or +78D lens with slit lamp
fibromatosis, Sturge-Weber syndrome, carotid- under dilated pupils. Following points must be
cavernous fistula, thyrotoxicosis exophthalmus remembered:
which cause elevated episcleral venous pressure zz Early findings include enlargement of the optic
and secondary open angle glaucoma must be disc cup, deep cup, thinning or saucerizing of
done. the neural rim (Fig. 2), disc hemorrhages, and
peripapillary atrophy (common at beta zone)
Ocular Examination (Fig. 3).
Eyeball: Usually looks normal. zz The diagnosis of glaucoma should be strongly
considered if vertical CD ratio ≥ 0.7 or
Lids: Usually normal. In patient who is already
asymmetric cupping between two eyes is > 0.2.
on anti-glaucoma medications like Prostaglandin
zz Changes at lamina cribriosa—laminar dot
analogues look for hyperpigmentation of lid
sign.
margin and long eyelashes.
Conjunctiva: All anti-glaucoma medications can
cause some form of conjunctival toxicity so always
look for conjunctival congestion. This congestion
is more in inferior quadrant.
Cornea: The cornea is typically normal in POAG.
Ocular hypertension has a higher incidence of
increased central corneal thickness (CCT) hence
CCT measurement is important. Thinner CCT
corneas are at higher risk for POAG. IOP is also
affected by CCT. Fluorescein (Na fluorescein
1%) staining with cobalt blue light examination
of cornea is advisable to look for corneal surface
toxicity caused by some antiglaucoma medications.
Pupils: The pupils are examined for direct and Fig. 2: Enlargement of the optic disc cup, thinning or
consensual light reflex. Relative afferent pupillary saucerizing of the neural rim and peripapillary atrophy
defect indicates advanced glaucoma.
Anterior segment: A slit-lamp biomicroscopic
examination of the anterior segment can
provide evidence of physical findings associated
with narrow angles, corneal pathology, or a
secondary mechanism for elevated IOP such as
pseudoexfoliation, pigment dispersion, iris and
angle neovascularization, or inflammation.
IOP: IOP is measured in each eye, preferably
using a Goldmann applanation tonometer before
gonioscopy or dilation of the pupil. Time of day
should be recorded because of diurnal variation.
Gonioscopy: The diagnosis of POAG requires
careful evaluation of the anterior-chamber angle Fig. 3: Advanced glaucoma with near total cupping,
to exclude angle closure or secondary causes of thinned neural rim and peripapillary atrophy
zz Changes at neuroretinal rim—look for focal or zz Other ocular side effects due to steroid use, e.g.
diffuse defect in neuroretinal rim which does posterior subcapsular cataract.
not obey ISNT rule. Angle recession glaucoma: Following points helps
zz Vascular changes—nasalization of retinal in differentiation:
vessels, bayoneting of vessels, baring of zz Past history of ocular trauma
circumlinear vessels. zz Angle recession on gonioscopy
zz Changes in retinal nerve fiber layer (RNFL)— zz Other signs may be seen as phacodonesis,
look for focal or diffuse defect in RNFL using iridodonesis, traumatic cataract or retinal tear
red-free illumination (green filter). zz Usually unilateral.
Look for other abnormalities of fundus that
might account for visual field defects, e.g. optic INVESTIGATIONS
nerve pallor, tilted disc, disc drusen, optic disc
pits, optic nerve hypoplasia, neurological disease, Following investigations are done in a case of
macular degeneration, and other retinal disease. POAG:
Diurnal variation in IOP: IOP needs to be
DIFFERENTIAL DIAGNOSIS recorded every 3 hourly for 24 hours, important for
zz Diagnose early cases of glaucoma
Ocular hypertension: Following points help in zz Assess pre-treatment baseline IOP (highest
differentiation:
recorded IOP before the diagnosis of glaucoma
zz High IOP > 21 mm Hg on 2 consecutive
without any medication)
occasions with applanation tonometry zz Pick up nocturnal rise of IOP
zz Normal optic disc and neuro-retinal rim (NRR) zz Helpful in timing of anti-glaucoma medica-
zz Normal visual field
tions
zz Open angles on gonioscopy zz Assess maximum-minimum variation of IOP-
zz Absence of any other ocular disease causing
IOP difference of 8 mm Hg or more between
raised IOP.
any two reading is significant.
Pigmentary glaucoma: Following points helps in Pachymetry: To estimate central corneal thickness
differentiation: and correction of IOP accordingly.
zz Younger age group 20–30 years
zz Pigments dispersion on corneal endothelium, Perimetry: Usually automated perimetry to
lens surface document visual field loss.
zz Mid-peripheral iris transillumination defect Retinal nerve fiber layer thickness: Retinal nerve
zz Wide open angles with dark broad pigmenta fiber layer (RNFL) assessment by optical coherence
tion of trabecular meshwork on gonioscopy. tomography (OCT), GDx and heidelberg retinal
tomogram (HRT).
Pseudoexfoliation glaucoma: Following points
helps in differentiation: Fundus photography: For documentation of optic
zz Higher IOP and greater 24 hours IOP nerve head changes.
fluctuation
zz Exfoliation material deposits on corneal endo MANAGEMENT
thelium, at pupillary margin, on lens surface,
upon trabecular meshwork on gonioscopy. Medical Treatment
zz Diffuse loss of NRR zz Target IOP: In every diagnosed case of
zz Greater visual field loss glaucoma, target IOP should be calculated to
zz Steroid induced glaucoma—following points halt or prevent further glaucomatous damage
helps in differentiation to optic nerve and visual field loss progression.
zz History of corticosteroid intake in any form zz Start with one or two medication based on
zz History of endogenous disease causing target IOP (see viva section). Beta-blockers
increased blood corticosteroid levels and prostaglandin (PG) analogues are often
zz Usually bilateral but can be unilateral also the first choice (See Table 1).2
Glaucoma 175
Table 1 Commonly used antiglaucoma medications

Ocular side Systemic side
Class Mechanism Drugs Dose effects effects
Prostaglandin Increase in Latanoprost HS Conjunctival Minimal; may
analogues uveoscleral (Xalatan)—0.005%, hyperemia, be related to
(prostamide) outflow of travoprost lengthening headaches
aqueous humor (Travatan)—0.004%, and darkening
tafluprost of eyelashes,
(Zioptan)—0.0015%, brown
bimatoprost discoloration of
(Lumigan)—0.01%, the iris, uveitis,
0.03% macular edema
Unoprostone
(Rescula)—0.15%
β-adrenergic Reduction of Timolol (0.5/0.25%), OD or BD Irritation and Contraindicated
blockers aqueous humor levobunolol, dry eyes in patients with
production carteolol, asthma, chronic
metipranolol, pulmonary
betaxolol obstructive
disease, and
cardiac failure,
bradycardia
α-adrenergic Initial reduction Brimonidine TID some Irritation, dry CNS effects and
agonists of aqueous (0.1/0.15%), times BD eyes, allergic respiratory arrest
humor with Apraclonidine reaction in young children
subsequent (contraindicated
effect of <2 years); caution
increase in in patients
outflow with cerebral
or coronary
insufficiency,
postural
hypotension,
renal or hepatic
failure
Carbonic Reduction of Dorzolamide (2%), TID some Irritation, dry Oral form may
anhydrase aqueous humor brinzolamide (1%), times BD eyes, burning be associated
inhibitors production acetazolamide (oral) sensation with with paresthesia,
topical agents nausea, diarrhea,
loss of appetite
and taste,
lassitude, or renal
stones
Cholinergic Increase in Pilocarpine Usually QID, Irritation, Ciliary spasm
agonists aqueous humor (0.5/1.0/2.0 %), but may vary induced myopia leading to
outflow carbachol and decreased headaches in
vision due to young patients
ciliary spasm
zz Adjuvant treatment with neuroprotectives like zz Patients whose conjunctiva is so scarred from
N-methyl D-aspartate (NMDA) receptors previous surgery that filtering surgery with
like memantine; Alpha 2 adrenergic agonists antimetabolites is at high risk for failure.
like brimonidine; Calcium channel blocking
agents, e.g. Flunarizine, nimodipine, anti Newer Nonpenetrating Glaucoma Surgery
oxidants or nitric oxide synthetase inhibitors zz Viscocanalostomy
can be considered. zz Nonpenetrating deep sclerectomy
zz Follow every 3 months in mild to moderate zz Sinusotomy
visual field loss cases and 1 monthly in zz Canaloplasty.
advanced cases. Every patient should be
checked for compliance and tolerance to anti
VIVA QUESTIONS
glaucoma medications. IOP with Goldmann
applanation tonometry should be recorded Q.1. What are the most important parameters
at each follow-up to monitor IOP control and on clinical examination that lead you to
target IOP with medication. investigate the patient for open angle
zz Visual fields with standard automated glaucoma?
perimetry should be done every year in ocular Ans. • Raised intraocular pressure (IOP)
hypertension with high-risk cases, every • Optic disc cupping:
6 monthly in mild to moderate cases while – A suspicious disc with increased
every 3 monthly in advanced cases to monitor vertical cup-to-disc (CD) ratio more
progression of disease and modification in than or equal to 0.7
treatment accordingly. – Asymmetric cupping between two
Laser trabeculoplasty: In patients who cannot eyes >0.2
or will not use medications reliably due to cost, – A diffuse or focal thinning of the
memory problems, difficulty with instillation, or neuroretinal rim which does not obey
intolerance to the medication. the ISNT rule (normally the inferior
rim is thickest followed by superior,
nasal and temporal), and a retinal
Filtration Surgery: Trabeculectomy
nerve fiber layer defect in red free
Indication light.
• Field changes suggestive of glaucoma
zz Patients from rural areas where follow up is
• Additional risk factors, e.g. age, myope,
likely to be difficult
diabetes mellitus, hypertension, thyroid
zz Patients with baseline IOP of more than 40 mm
disease, family history of glaucoma.
Hg where even maximal medical therapy will
be unsuccessful Q.2. What is the importance of DVT in POAG
zz Patients who have lost one eye due to glaucoma management?
at presentation Ans. Diurnal variation is very important in a
zz Failure to control IOP after maximal tolerated glaucoma patient both for
medical therapy • Diagnosis—to establish baseline IOP,
zz Patient develops side-effects of antiglaucoma determine magnitude of IOP fluctuation
treatment and the timing of peak IOP.
zz IOP not at target on 3 topical medications or • M anagement—to establish diurnal
progression of visual fields despite maximum control with ocular hypotensive drugs
medical therapy and maintain IOP below target with a
zz Poor compliance to medical therapy. fluctuation of less than 5 mm Hg, instill
medicines at a time to cover peak IOP
Glaucoma Drainage Devices spikes.
Q.3. Define target IOP and how you will
Indication calculate target IOP?
zz Patients who have failed filtering surgery with Ans. Target IOP may be defined as a pressure,
antimetabolites rather a range of intraocular pressure levels
Glaucoma 177
within which the progression of glaucoma preferentially in upper and lower poles of
and visual field loss will be delayed or disc.
halted. It is calculated depending on Q.6. Why Goldmann’s applanation tonometry
severity of glaucomatous optic damage. should be done before gonioscopy and
The severity of glaucoma damage can be before dilatation of pupils?
estimated using the following scale: Ans. • D uring gonioscopy angle of anterior
• Mild: Characteristic optic nerve abnor chamber opens up due to pressure over
malities consistent with glaucoma and cornea, which results in reduction of IOP
a normal visual field as tested with and applanation tonometry performed
standard automated perimetry. after gonioscopy gives low IOP than
• Moderate: Characteristic optic nerve correct IOP.
abnormalities consistent with glaucoma • By dilatation of pupil there is transient
and visual field abnormalities in one rise in IOP 4–5 mm Hg that also gives
hemifield and not within 5° of fixation. high IOP than actual IOP on applanation
• S evere: Characteristic optic nerve tonometry if performed on dilated pupils.
abnormalities consistent with glaucoma Q.7. Which are pre-perimetric diagnostic
and visual field abnormalities in both tools of glaucoma?
hemifields and loss within 5° of fixation Ans. Pre-perimetric diagnostic tools can defect
in at least one hemifield. early glaucomatous damage in RNFL even
Depending on severity of glaucomatous before that damage can be located on
optic disc damage, American Association perimetry. These are GDxVCC, HRT, OCT
Ophthalmology has given guidelines to and SWAP.
estimate target IOP as follow: Q.8. What is the pathogenesis of primary open
• In Mild damage cases—30% reduction of angle glaucoma?
IOP from baseline IOP Ans. • Genetics
• I n advanced damage cases—40% – MYOC: Myocilin (GLYC1A, chromo
reduction of IOP from baseline IOP some 1), associated with juvenile
• I n ocular hypertension cases—20% open angle glaucoma and ≈4% of
reduction of IOP from baseline IOP adults with POAG
• I n normal tension glaucoma (NTG) – OPTN: Optineurin (GLYC1E, chromo
cases—30% reduction of IOP from some 10)
baseline IOP. – Other loci: GLYC1B, GLYC1C
Q.4. What are the qualitative evaluation and • Pressure dependent (mechanical factors)
quantitative evaluation of optic nerve – Increased IOP→compression and
head in case of glaucoma? backward bowing of lamina cribrosa
Ans. Qualitative evaluation →obstruction of axoplasmic transport
• Contour of the neuroretinal rim →ganglion cell death
• Optic disc hemorrhage • Ischemic factors/pressure independent
(esp significant for NTG)
• Parapapillary atrophy
– Vascular perfusion compromise
• Bared circumciliary vessels
(DM, HTN, migraine, Raynaud’s
• Appearance of retinal nerve fiber layer.
phenomenon
Quantitative evaluation
– Abnormal coagulability
• Optic disc size (vertical disc diameter)
– Nocturnal hypertension, significant
• Cup disc ratio
blood loss
• Rim disc ratio. • Neurodegenerative factors
Q.5. Which cup disc ratio is important hori – Primary ON damage leads to gluta
zontal or vertical and why? mate release, which interacts with cell
Ans. The vertical cup disc ratio is more important receptors that leads to an increase in
since early neuroretinal rim loss occurs intracellular calcium levels
– This triggers cell death via apoptosis Refractive surgeries reduce the CCT and
and leads to further release of therefore the IOP measurements in such
glutamate and a vicious cycle occurs. patients would be falsely low.
Q.9. What is the role of central corneal
Q.10. Classify antiglaucoma drugs, their
textbook (CCT) in ocular hypertension common side effects.
(OHT)/open angle glaucoma (OAG)? Ans. Refer to any Standard Textbook (see
Ans. CCT measurement is important before Table 1).
diagnosing glaucoma and deciding the
management, while it is not helpful
in predicting risk of progression of
REFERENCES
existing glaucoma. CCT influences IOP 1. Kwon YH, Fingert JH, Kuehn MH, Alward WL.
measurement by applanation tonometry Primary open-angle glaucoma. N Engl J Med.
and therefore it is important to note CCT in 2009;12;360(11):1113-24.
every patient being evaluated for glaucoma. 2. Weinreb RN, Aung T, Medeiros FA. The
CCT can be broadly categorised as: Thin pathophysiology and treatment of glaucoma: a
(<500 μm), Normal and Thick (>570 μm). review. JAMA. 2014;14;311(18):1901-11.
PRIMARY ANGLE CLOSURE GLAUCOMA

Vaishali Ghanshyam Rai, Talvir Sidhu, Dewang Angmo
INTRODUCTION of vision with redness in eye with severe eye

pain associated with ipsilateral headache with
Primary angle closure glaucoma (PACG) is usually nausea and vomiting.
allotted in practical examination as a long case.
Primary angle closure (PAC) is appositional or
synechial closure of the anterior-chamber angle
caused by pupillary block. The angle closure may Following must be enquired:
or may not be associated with elevated intraocular zz History of symptoms precipitated by
pressure (IOP) or glaucomatous optic neuro watching television, darkened room, reading,
pathy, and may occur in either an acute or chronic pharmacological mydriasis.
form. This entity does not include secondary zz Brief history of patient’s previous records to
forms of angle closure induced by other causes,
get baseline IOP.
e.g. subluxed lens.
zz Patient who is already on treatment for
HISTORY glaucoma is important to know how many
medications he/she is using and whether
Chief Complaints
these treatment has sufficiently controlled IOP
A case of PACG can present in following ways: and visual field loss.
zz Majority of patients with angle closure zz Patient’s previous visual fields record should
glaucoma are asymptomatic. be checked to know the progression of
zz Blurred vision or smoke filled room
glaucoma.
zz Some patient present acutely with color halos
around lights due to corneal edema, aching
eye or brow pain and/or eye redness. History of Past Illness
zz Patient with acute angle closure attack Ask for previous use of glasses. Hypermetropic
presents usually with unilateral diminution patients are at higher risk to develop PACG.
Glaucoma 179
History of Systemic Illness EXAMINATION

zz Diabetic or hypertensive patients who need General examination/specific systemic examina
frequent dilated fundus examination, need tion is carried out to look for any contraindication
special attention to rule out PAC or PACG to antiglaucoma medications.
since shallow anterior chamber depth may
develop acute angle closer attack on dilation Ocular Examination
of pupil.
zz It is very important to ask about cardiovas zz Eyeball: Usually looks normal. Small eye ball in
cular disease, renal diseases and bronchial case of hypermetropia.
asthma before deciding on antiglaucoma zz Lids: Usually normal. In patient who is already on
treatment. anti-glaucoma medications like prostaglandin
analogues look for hyperpigmentation of lid
margin and long eyelashes.
Family History zz Conjunctiva:
Relatives of PACG patients are at higher risk for —— In case of acute angle closure attack,
developing glaucoma. The severity and outcome marked circumciliary congestion can be
of glaucoma in family members, including history noted.
of visual loss from glaucoma is also important. —— Patients of chronic angle closure glau-
coma who already on anti–glaucoma

Drug History treatment, always look for conjunctival
congestion as all antiglaucoma medica-
zz History of use of ocular and systemic tions can cause some form of conjunctival
medications. toxicity. This congestion is more in infe-
zz Known local or systemic intolerance to ocular rior quadrant.
or systemic medications. zz Cornea: Following signs can be seen.
zz History of drugs which induce angle closure —— In acute angle closure attack, unilateral
attack needs to be asked. Such drugs include: epithelial and stromal cornea edema due
—— Anticholinergic agents (topical, e.g. atro-
to raised IOP.
pine, cyclopentolate, and tropicamide, —— In chronic cases, Krukenberg spindle
or systemic, e.g. antihistamine, antipsy- (pigment distribution over the inferior
chotic (especially antidepressants), anti- corneal endothelium).
parkinsonian, atropine, and gastrointesti- zz Anterior chamber:
nal spasmolytic drugs). —— Anterior chamber is shallow (Fig. 1).
—— Adrenergic agents (topical, e.g. epineph-
The Van Herick technique is useful
rine and phenylephrine, or systemic, e.g. for estimating the peripheral anterior
vasoconstrictors, central nervous system
stimulants, bronchodilators, appetite
depressants, and hallucinogenic agents).
zz Specific questioning includes asking about
the use of topical or systemic medication (e.g.
sulfonamides, topiramate, phenothiazines)
that may induce angle narrowing and sub
sequent symptoms that suggest intermittent
angle-closure attacks should be enquired.
Surgical History
History of previous ocular surgery like trabe
culectomy or any eye laser like iridotomy must be
asked for. Fig. 1: Shallow anterior chamber
Fig. 2: Patchy iris stromal atrophy Fig. 3: Segmental iris atrophy
chamber depth. In PACG cases, peripheral

anterior chamber is shallow that can
be graded by Van Herick technique
(discussed later in viva questions). When
the peripheral anterior chamber depth
is less than one fourth of the corneal
thickness, the anterior chamber angle
may be potentially occludable.
—— Anterior-chamber inflammation sugges
tive of a recent or current attack.

zz Iris: Following points must be looked for
—— In acute attack, iris bombe is usually
present due to pupillary block.

—— In recent PACG attack, iris whorling
(sectoral infarction of the iris sphincter) Fig. 4: Glaukomflecken

or patchy iris stromal atrophy is usually
present (Fig. 2).
—— Mid-dilated pupil is common in acute or (typically a Goldmann tonometer) before
recent PACG attack. gonioscopy. Measuring central corneal
—— Signs of previous angle-closure attacks thickness should be postponed until
are PAS, segmental iris atrophy (Fig. 3), resolution of an acute attack.
posterior synechiae, irregular pupil. —— In acute attack, IOP is usually very high
zz Lens: In previous angle closure attack, look (50–100 mm Hg).

for glaukomflecken (small gray-white anterior —— In chronic PACG, IOP elevation may be
subcapsular or capsular opacities (Fig. 4) in intermittent.

the pupillary zone, due to infarction of lens zz Gonioscopy:
fibers). Lens thickness might be increased. An —— Gonioscopy of both eyes should be
intumescent lens can be there in angle closure performed on all patients in whom angle
attack. Vogt’s Triad: Sectoral iris atrophy, closure is suspected.
Krukenberg spindle and glaucomflecken is —— This is best performed using first a two-
characteristic of PACG. mirror Gonio lens (e.g. Goldmann) to

zz Intraocular pressure (IOP): avoid artifactual distortion of the angle
—— IOP is measured in each eye, preferably caused by inadvertent pressure on the
using a contact applanation method cornea.
Glaucoma 181
Since primary narrow angles and PAC tend to be
bilateral, the observation of a wide-open angle
in the fellow eye suggests a diagnosis other than
PAC.
zz Plateau iris syndrome:
—— The peripheral iris is forced into the angle
by anterior rotation of the ciliary body or

anteriorly positioned ciliary processes.
—— Angle closure attack may be precipitated
after dilatation of pupil even in presence

of patent peripheral iridotomy.
zz Neovascular glaucoma:
Fig. 5: Gonioscopy shows presence of PAS —— Neovascularisation of iris or angles
hallmark sign
—— This is required to evaluate the angle zz Inflammatory causes of angle closure (e.g.
anatomy, appositional closure, and posterior synechiae, iris bombé).
presence of primary angle closure (PAS) zz Iridocorneal endothelial syndrome.
(Fig. 5). zz Ciliary body engorgement or suprachoroidal
—— Compression (indentation) gonioscopy effusion caused by systemic medications (e.g.
with a four-mirror or similar lens is topiramate, sulfonamides, phenothiazines).
particularly helpful to evaluate for
zz Ciliary body engorgement associated
appositional closure versus synechial with retinal vascular occlusion or scatter
(panretinal) photocoagulation.
angle closure and for extent of PAS.
zz Anterior suprachoroidal effusions (e.g.
—— Various grading systems including Scheie,
congestion, edema, displacement).
Shaffer, and Spaeth have been proposed zz Aqueous misdirection (ciliary block)
for the recording of gonioscopic findings. syndrome after incisional or laser surgery (e.g.
These gonioscopic grades provide an following peripheral iridectomy).
index of the likelihood of angle closure. zz Lens-induced angle closure (e.g. phacomor-
zz Fundus examination: phic or subluxed).
—— For patients with PAC or narrow angle who zz Developmental disorders (e.g. nanophthal-
are not in an acute attack, pupil dilation mos, retinopathy of prematurity, persistent
is contraindicated until iridotomies have hyperplastic primary vitreous).
been performed. zz Iris or ciliary body mass lesions or cysts.
—— Although a dilated examination may not
be advisable in patients with anatomic CLASSIFICATION

narrow angles or angle closure, an
attempt should be made to evaluate the Recently angle closure glaucoma has been
fundus and optic nerve using the direct classified into three categories (Table 1).
(1) Primary angle closure (PAC); (2) Primary angle
ophthalmoscope or biomicroscope with
closure glaucoma (PACG); (3) Primary angle
+78D or +90D.
closure suspect (PACS); depending upon the
—— In acute attack, optic nerve head may
presence of following:
look hyperemic and edematous in early zz Iridotrabecular contact (>180°)
stage, the disc then became pale and zz Elevated IOP
glaucomatous cupping can be observed zz PAS
after 9 to 10 days. zz Glaucomatous optic neuropathy.
Table 1 Classification and management of angle closure glaucoma

Characteristics PACS PAC* PACG**
Iridotrabecular contact + + +
(>180°)
Elevated IOP – +/– +/–
PAS – +/– +/–
Glaucomatous optic – – +
neuropathy
Treatment Close observation with LPI+ medical or surgical LPI+ medical or surgical
serial gonioscopy or LPI*** therapies to control IOP therapies to control IOP
Abbreviations: IOP, intraocular pressure; PAC, primary angle closure; PACG, primary angle closure glaucoma;
PACS, primary angle closure suspect; PAS, peripheral anterior synechiae, LPI, laser peripheral iridotomy.
*For a diagnosis PAC either elevated IOP or PAS, one of them must be positive, along with Iridotrabecular
contact (>180°).
**For a diagnosis of PACG either elevated IOP or PAS, one of them must be positive, along with Iridotrabecular
contact (>180°) and optic nerve damage.
***LPI should be considered taking into consideration of symptoms suggestive of intermittent angle closure,
systemic medications that may predispose to pupillary block, need for frequent pupillary dilation or lack of
reliable access to healthcare.
INVESTIGATION zz Topical steroids qid.

zz Preventive laser peripheral iridotomy (LPI)
zz Visual field analysis by automated perimetry should be done in fellow eye as early as
to document visual field loss. possible.
zz Ultrasound biomicroscopy (UBM): It can help zz Once IOP is controlled and corneal edema
to elucidate the underlying mechanism of clears a LPI should be performed.
angle closure in most cases, including plateau
iris syndrome and iridociliary cysts, thereby
allowing the appropriate treatment to be given.
Management of Primary
zz Anterior segment optical coherence tomography Angle Closure Suspect
(AS-OCT): A comparison with gonioscopy has Management options include LPI or close
found that it may be superior in its ability to observations with regular follow for IOP checking,
detect angle occludability. gonioscopy and disc evaluation. Indication for LPI
in PACS includes:
MANAGEMENT zz Patient who needs frequent dilatation, e.g.
diabetic, hypertensive age-related macular
Medical Management degeneration (ARMD)
zz Patient who had previous angle closure attack
Management of acute angle closure attack:
in one eye, prophylactic peripheral iridotomy
zz Acetazolamide (250–500 mg) oral stat, then 125
(PI) for fellow eye
to 250 mg tid/qid until symptoms subside or zz Hyperopic patient
IV Mannitol 1.5g/kg of body weight of 20% 200 zz Patient who is unlikely to come for regular
mL over 30 minutes then oral Acetazolamide
follow-up.
250 mg qid.
zz Topical pilocarpine 2% stat, then qid can be
given but after inflammation control. Surgical Treatment
zz Analgesics and antiemetic as required. A patient whose IOP does not come under control
zz Topical beta-blockers eye drop BD. with PI and medications, and those with fairly
Glaucoma 183
advanced disease will require filtering surgery. Q.4 What are the indications for laser
The application of antifibrotic agents such as peripheral iridotomy in PACS?
5-Fluorouracil (5-FU) and Mitomycin C (MMC) Ans. • Patient who needs frequent dilatation,
results in greater success and lowers IOP following e.g. diabetic, hypertensive, ARMD
trabeculectomy. • Patient who had previous angle closure
attack in one eye, prophylactic PI for
VIVA QUESTIONS fellow-eye
• Hyperopic patient
Q.1. How do you grade peripheral anterior • P atient who is unlikely to come for
chamber depth (PACD) on slit lamp? regular follow-up.
Ans. By Van Herick’s method
• Grade 0: Iridocorneal contact Q.5. Explain technique to perform laser
• Grade 1: PACD < 1/4 corneal thickness peripheral iridotomy (LPI)?
• Grade 2: PACD = 1/4 corneal thickness Ans. The procedure of LPI involves following
• Grade 3: PAC = 1/4–1/2 corneal thickness • B efore LPI: Pilocarpinie eye with 1%
• Grade 4: PACD ≥ 1 corneal thickness pilocarpine then anesthetize eye with
Grade 0,1 and 2—suspicious of angle 0.5% proparacaine.
closure • LPI: Abraham’s type of contact lens is
applied. This lens has a +55 D, peripheral
Q.2. Which are the risk factors for developing button over a routine contact lens. This
PACG? lens helps in the following way:
Ans. Following are risk factors for PACG – It stabilizes the eye and prevents
• Patient factors: undue movements.
– Advancing age – It helps to open the eye and keep the
– Female gender lids retracted during the procedure.
– Asian or Inuit descent – It smoothens out the corneal surface.
– Family history of angle closure – It provides peripheral view, which is
• Ocular factors: highly magnified.
– Shallow anterior chamber – It helps to reduce the axial expansion
– Narrow angle of plasma, which reduces the
– Relative anterior location of iris-lens unnecessary spread of the damage.
diaphragm – It increases the power density of the
– Hyperopia [increased lens thickness, spot.
small corneal diameters and short – Gives pressure to prevent the bleed
axial length (AL)] from increasing.
Q.3. What are the mechanisms of angle • Site of LPI: The iridotomy site should be
closure? in the peripheral third of the iris, just
Ans. • Pupil block (most common) anterior to the arcus. A crypt or a thinned
• Abnormalities anterior to iris: area of the iris is recommended. Most
– PAS ophthalmologists place the iridotomy
– ICE syndrome between 11 o’clock and 1 o’clock, where
– Neovascular glaucoma the lids superiorly cover it.
• Abnormalities of iris and ciliary body: • Size of LPI: Iridotomy be at least 200 μm
– Cysts thick peripheral iris in size. The preferable size is 500 μm in
– Peripheral iris roll diameter.
• Abnormalities of lens: • E nd point: Once the iridotomy is
– Thick intumescent lens complete one can notice a sudden gush
– Subluxated lens of aqueous or outflowing of the pigment
• Abnormalities posterior to lens: Malig- from the posterior to the anterior
nant glaucoma. chamber along with sudden deepening
of the anterior chamber. The presence • Visual symptoms

of retro-illumination may be looked • Corneal decompensation.
for after a few weeks of laser iridotomy, Rare complications of LPI are:
however it is not a sure sign of total • Aqueous misdirection
penetration. Visualization of the anterior • Recurrent herpetic keratouveitis
lens capsule confirms LPI. • Retinal and subhyaloid hemorrhage
• Parameters for LPI: In Indian patients • Choroidal and retinal detachment after
brown irides, LPI can be performed with argon LPI
a neodymium: yttrium-aluminum-garnet • Stage I macular hole.
(Nd:YAG) laser, using the following
settings: Q.7. Risk of progression of PACS to PAC/
– Power—4–8 mJ PACG.
– Pulses/burst—1–3 Ans. The reported rates of developing AACC
– Spot size—fixed range from 6 to 10%, and rates of PAC or
• Monitoring and follow-up post LPI: At PACG are 17–35%.
1 hour after completion of LPI, the IOP Q.8. The International Society of Geography
should be checked to make sure that it did and Epidemiology of Ophthalmology
not increase significantly (i.e. IOP has not classification of angle closure glaucoma.
increased by 8 mm Hg or more and that Ans. Classified PAC disease into PAC suspect
IOP does not exceed 30 mm Hg). Topical (PACS), closure (PAC) and glaucoma
prednisolone acetate 1% is given 4 times (PACG) based on intraocular pressure
a day for 5–7 days. Topical beta-blocker (IOP), gonioscopy findings, disc, and visual
is added in cases of PACS or continue field examination.
antiglaucoma medications if patient is
already on anti-glaucoma medications.
BIBLIOGRAPHY
At 1 week, the patient is seen to monitor
IOP, to confirm the patency of the 1. Emanuel ME, Parrish RK 2nd, Gedde SJ.
iridotomy site, and to check for any Evidence-based management of primary angle
significant intraocular inflammation. closure glaucoma. Curr Opin Ophthalmol.
2014;25(2):89-92.
Q.6. What are the complications of LPI? 2. Kashiwagi K, Abe K, Tsukahara S. Quantitative
Ans. Common complications are: evaluation of changes in anterior segment
• Postoperative intraocular pressure spike biometry by peripheral laser iridotomy using
• Anterior uveitis newly developed scanning peripheral anterior
• Iris bleeding and hyphema chamber depth analyzer. Br J Ophthalmol.
• Focal cataract 2004;88:1036-41.
• Posterior synechiae
Glaucoma 185
SHORT CASES
STURGE-WEBER SYNDROME
Vaishali Ghanshyam Rai, Ritika Mukhija, Dewang Angmo
Sturge-Weber syndrome (SWS) is a rare congenital Systemic Examination
neuro-oculocutaneous disorder present at
Sturge-weber syndrome (SWS) can involve
birth. It is also known as encephalotrigeminal
central nervous system (CNS) as well skin hence;
angiomatosis. The ocular component manifests
a thorough examination of both the system is
as glaucoma and vascular malformations of the
required. SWS is called tri-symptomatic when
conjunctiva, episclera, choroid, and retina. The
the skin, eye, and CNS involvement is there.
hamartoma occurring in SWS arises from vascular
Similarly, it is called bisymptomatic when the
tissue and produces a characteristic ipsilateral port-
skin and CNS or the skin and eye are affected; and
wine hemangioma of the skin along the trigeminal
monosymptomatic when the skin or the CNS is
distribution. Classic SWS comprises the triad of
affected.4
Port-wine facial telangiectasia (nevus flammeus)
Specific examination to rule out nervous
in the distribution of the trigeminal nerve that
system involvement such as hemispheric motor or
respects the vertical midline, ipsilateral glaucoma, sensory defects, and intellectual deficiency has to
and intracranial angiomata.1-3 Glaucoma occurs be done. Dermatological examination may reveal
in approximately one half of the cases (30–70%) in a characteristic Port-wine hemangioma (dilated,
which the Port-wine stain involves the ophthalmic telangiectatic cutaneous capillaries) of the skin
and maxillary divisions of the trigeminal nerve.4-6 along the trigeminal distribution (Fig. 1).
HISTORY Ocular Examination

Chief Complaints Eyeball: Usually normal.
A case of SWS can present with following:
zz Commonly referred to ophthalmologist from
general practitioner or neurologist to rule out
ocular manifestation of SWS.
zz Heaviness and dull aching pain around eye
zz Progressive peripheral visual field loss.
History
Parents usually give history of presence of
unilateral port wine hemangioma of skin along the
trigeminal distribution since birth. The angiomata
are present at birth and are usually unilateral,
although bilateral cases also occur. History of
seizures generally in infancy is usually present. Fig. 1: Bilateral facial angiomatosis
Eyelids: A unilateral port–wine hemangiomas of MANAGEMENT

the lid skin long the trigeminal distribution
(maxillary and/or ophthalmic division) can be The treatment includes following:
seen.
Medical Treatment
Conjunctiva: A dense episcleral vascular plexus
and occasional ampulliform dilatations of Antiglaucoma agents may suffice to control
conjunctival vessels is common on the site of the glaucoma that occurs in later life, whereas
cutaneous lesion. the infantile form usually requires surgical
intervention. Beta-blockers, alpha-adrenergic
Cornea: Usually normal. Corneal edema can be agonists or carbonic anhydrase inhibitors can be
there in presence of high IOP. used as mono therapy or in combination to achieve
Anterior chamber: When the glaucoma is target IOP. One should avoid prostaglandin
congenital abnormalities of the chamber angle analogues in cases of SWS (where episcleral
similar to other forms of congenital glaucoma venous pressure is already raised) since it can
can be there. When glaucoma occurs later in life; cause anterior uveal effusion.
at that time, it is associated with a more normal-
appearing anterior chamber angle. Surgical Treatment
Fundus: Good indirect ophthalmoscopy should The surgical options include following:
be performed to look for choroidal hemangiomas. Goniotomy: It is first choice, as chance of
Also, look for retinal edema and retinal intraoperative choroidal effusion is not associated
detachment, which are usually associated with with goniotomy.
choroidal hemangiomas. A 90 D stereoscopic disc
Combined trabeculotomy: Trabeculectomy may
evaluation should be performed to document cup-
improve the chances of success, by treating both
disc ratio, neuroretinal rim status and any retinal
possible sources of elevated IOP that anterior
nerve fiber layer defect.
chamber angle anomaly as well as elevated
IOP: In unilateral cases, IOP is >21 mm Hg in episcleral venous pressure.
ipsilateral eye in around 50% of patients with facial
Trabeculectomy: Chances of intraoperative
port–wine stain and normal in contralateral eye.1-3
choroidal effusion and expulsive choroidal
While in bilateral facial port-wine stain, IOP is
hemorrhage are more with trabeculectomy. It
>21 mm Hg in both eyes.
is preferable to perform one or more sclerotomy
Gonioscopy: In infants, look for developmental before trabeculectomy to reduce chances of
anomalies or neovascularization of angles of intraoperative complications.
anterior chamber. In adults, usually angles of Glaucoma drainage device: Another surgical
anterior chamber look normal. approach to reduce pressure in these patients
while minimizing intraocular complications.
INVESTIGATIONS Cyclophotocoagulation: Performed in patients
Visual fields: A standard automated perimetry to with refractory glaucoma or in patients with
document visual field defect should be performed. high risk of intraoperative or postoperative
complications (choroidal expulsive hemorrhage
Pre-perimetric test: OCT, GDxVCC or HRT to or choroidal detachment) after glaucoma filtration
document any early glaucomatous damage of surgery and when the visual potential is poor.
RNFL is advisable in cases of SWS with borderline
IOP and no evidence of glaucoma.
VIVA QUESTIONS
MRI brain/CT head: To look for cortical
calcifications, which can be appreciated as double Q.1. What is the cause of glaucoma in SWS?
densities or railroad tracks. Ans. The cause differs according to the onset.
Glaucoma 187
Table 1 Differentiating feature between congenital glaucoma and glaucoma

due to Sturge-Weber syndrome (SWS)
Congenital glaucoma Glaucoma due to SWS
Bilateral Usually unilateral, rarely bilateral
Absence of port-wine stain Ipsilateral port-wine stain
Other systemic involvement rare Neurological involvement common
• I n infants or children (approximately with the distribution of PWS along

60% of the cases) cause of raised IOP is various branches of the trigeminal nerve.
anomalies of anterior chamber angles. Involvement of both V1 and V2 carries
• In late-onset glaucoma (approximately the highest risk of glaucoma while
40% of the cases), raised IOP is due to involvement of only V2 distribution
increased episcleral venous pressure. carries the lowest risk.
• Remember the incidence of glaucoma • The treatment of PWS is pulsed dye
increases when the port-wine stain laser photocoagulation, which causes
involves the eyelid. It is usually ipsilateral irreversible damage to blood vessels but
to the lesion but can also manifest spares other components of the skin.
bilaterally.5,6 Multiple treatment session is required.
Q.2. How would you differentiate secondary The side effects are minimal. However,
glaucoma due to SWS and congenital 100% clearance of the skin discoloration
glaucoma? is not possible.
Ans. Differentiating feature between congenital
glaucoma and glaucoma due to SWS are REFERENCES
given in Table 1.
1. Phelps CD. The pathogenesis of glaucoma in
Q.3. Explain port-wine stain (PWS). Sturge-Weber syndrome. Ophthalmology. 1978;
Following points must be remembered 85:276.
about PWS: 2. B o a rd R J, S h i e l d s M B . C o m b i n e d
Ans. • It is a well delineated red macule present trabeculotomy-trabeculectomy for the
at birth management of glaucoma associated with
• W ith increasing age, it gets darker Sturge-Weber syndrome. Ophthalmic Surg.
and thicker and many small and large 1981;12:813.
dark nodules can grow on the surface, 3. Agarwal HC, Sandramouli S, Sihota R, et al.
resembling pyogenic granulomas. Sturge-Weber syndrome: management of
• It typically presents in the V1 and V2 glaucoma with combined trabeculotomy-
distributions of the trigeminal nerve. trabeculectomy. Ophthalmic Surg. 1993;24:399.
4. Awad AH, Mullaney PB, Al-Mesfer S, et al.
• The upper eyelid is more frequently
Glaucoma in Sturge-Weber syndrome. J AAPOS.
affected than the lower.
1999;3:40-5.
• In cases where PWS is present bilaterally, 5. van Emelen C, Goethals M, Dralands L, et al.
the likelihood of having SWS is higher Treatment of glaucoma in children with
than unilateral cases. Sturge-Weber syndrome. J Pediatr Ophthalmol
• R arely ipsilateral nasal and buccal Strabismus. 2000;37:29-34.
mucosa may also be involved on the side 6. Amirikia A, Scott IU, Murray TG. Bilateral diffuse
of PWS. choroidal hemangiomas with unilateral facial
• The severity of associated neurological nevus flammeus in Sturge-Weber syndrome.
deficits and glaucoma is often correlated Am J Ophthalmol. 2000;130:362-4.
BUPHTHALMOS
INTRODUCTION zz Cornea: Increase in corneal diameter (Fig. 2).

A horizontal corneal diameter >12 mm gives a
Buphthalmos is a term that is applied to congenital high index of suspicion for the disease. Corneal
glaucomas with enlargement of globe that appear edema with horizontal breaks in Descemet’s
during the first 3 years of life. The incidence of membrane, i.e. Haab’s striae should be noted.
buphthalmos is 1:3300 in India and 1:10, 000– In severe cases, acute hydrops may be seen.
1:20, 000 in western world.1,2 It is bilateral in up zz Anterior chamber: Usually deep.
to 65–80% of cases. Around 90% of cases appear zz Iris: Usually normal, although it may have
to be sporadic, 10% of cases appear to be a strong stromal hypoplasia with loss of the crypts.
familial component.1,2 zz Posterior segment: Examination of optic nerve
head is important to look for asymmetric
HISTORY cupping between two eyes or >0.3 CDR.
Chief Complaints
zz Infant is usually referred to an ophthalmologist
from pediatrician due to corneal cloudiness.
zz Parents of infant may complaint of large eye
ball, lacrimation and or blepharospasm.
zz Triad of epiphora, photophobia and blepharo
spasm is the most common presentation.
Family History
Similar complaint in other offspring is important
due to familial inheritance. The chance of a second
child having the disease is approximately 3%, and
it may be as high as 25% if two children have the
disease. Fig. 1: Clinical photograph of a case of
congenital glaucoma showing bilateral buphthalmos
OCULAR EXAMINATION
zz Visual acuity: Child may have severe
photophobia due to corneal edema and breaks
in Descemet’s membrane with torch light
while checking fixating and following light.
The enlargement of the globe with elevated
IOP during the first 3 years of life creates a
myopic shift in the refractive error.
zz Eyeball: Enlarged eyeball is common
(Fig. 1), which occurs because the immature
and growing collagen that constitutes the
cornea and sclera in the young eye still
responds to increased intraocular pressure
(IOP) by stretching.
zz Sclera: Bluish discoloration of sclera due to Fig. 2: Clinical photograph of a case showing
stretching can be seen. right eye buphthalmos
Glaucoma 189
INVESTIGATIONS cyclocryotherapy or laser cyclophotocoagula

tion may lower the IOP profoundly.
zz IOP: The IOP in normal infants is in the range zz Penetrating keratoplasty: Permanent corneal
of 11–14 mm Hg using Tonopen or hand- scarring may persist after normalization of the
held Goldmann tonometer, IOP >14 mm Hg IOP, in those cases penetrating keratoplasty
in bilateral cases or >5 mm Hg difference can be indicated.
between two eyes in unilateral or asymmetric zz Endothelial keratoplasty: Recently Descemet
case should be considered for diagnosis of stripping automated endothelial keratoplasty
Buphthalmos. has gained popularity for treatment of
zz Gonioscopy: Evaluation of the anterior endothelial dysfunction associated with
chamber angle is essential for the accurate buphthalmos.
diagnosis of congenital glaucoma. It is done • Tube surgery: Initial results are encouraging.
under anesthesia using an infant Koeppe • Non penetrating Sx: Rarely done.
goniolens. The iris inserts anteriorly compared
to the normal infant angle. The stroma of the
peripheral iris is hypoplastic, unpigmented, VIVA QUESTIONS
and has a scalloped appearance.
zz Barkan’s membrane: It refers to a membrane Q.1. What is normal corneal diameter in
formed due to incomplete resorption of infants and when would you suspect
mesodermal tissue across the anterior chamber buphthalmos?
angle, referred to as the Barkan’s membrane. Ans. Normal horizontal corneal diameter in
Although its existence is controversial it infants is 10–10.5 mm, increases from 0.5
forms the basis of the surgical procedure of to 1.0 mm during the first year of life. A
goniotomy which results in cleaving of the horizontal corneal diameter >12 mm gives
membrane to increase aqueous flow.1,2 a high index of suspicion for the disease.
zz Monster vessels: Normally, the angle is
usually devoid of blood vessels. In congenital Q.2. What is differential diagnosis of
glaucoma loops of blood vessels from the buphthalmos?
major arterial circle may be seen above the iris Ans. Differential diagnosis of buphthalmos
surface and referred as monster vessels and depending on ocular signs are:
this phenomena is called Loch Ness Monster • Corneal edema or clouding
phenomenon.1,2 – Congenital hereditary endothelial
d y s t ro p h y — c o r n e a t h i c k n e s s
TREATMENT increased two/three times normal,
corneal enlargement is typically
Treatment of buphthalmos is mainly surgical. absent. Corneal clouding is often
zz Goniotomy: It is surgical choice where cornea symmetrical with no descemet breaks
is clear so that angle of anterior chamber can or corneal scarring.1
be visualized clearly. – Mucopolysaccharidoses—absence
zz Trabeculotomy: Where cornea is hazy, of elevation of IOP and corneal
trabeculotomy is the surgical procedure of enlargement.
choice. – Sclerocornea—the opaque corneal
zz Filtration surgery: Cases where goniotomy tissue extends onto the cornea.
and trabeculotomy failed, filtration surgery – Obstetric birth trauma (“forceps
trabeculectomy or drainage implant is injury”)—See Table 1.
an option. It is usually combined with • Epiphora and/or red eye
trabeculotomy (Trab+Trab). – Nasolacrimal duct obstruction
zz Cycloablative procedures: Cases in which – Ophthalmia neonatorum/Conj
conventional glaucoma surgery has failed unctivitis (viral, Chlamydial, bacterial)
to control IOP cycloablative procedures, e.g. – Corneal epithelial defect, abrasion
Table 1 Differentiation of birth trauma and congenital glaucoma

Features Birth trauma Congenital glaucoma
Corneal diameter Normal Large (buphthalmos)
IOP Normal High
Photophobia No Yes
Onset of corneal edema after birth Immediate Weeks to months later
Clearing of edema Spontaneous After reducing IOP
Tears in Descemet’s membrane Vertical or oblique Horizontal or concentric to the limbus
Eye affected Left more Equal
Soft tissue injuries May be there Absent
• Photophobia • Secondary
– Conjunctivitis – Systemic disorders
– Uveitis ♦ Chromosomal abnormalities
• Corneal enlargement ♦ M etabolic disorders (Lowe’s
– Axial myopia syndrome, Zellweger’s syndrome)
– Megalocornea (X-linked or sporadic) ♦ P hakomatoses (Sturge-Weber
– Microphthalmic fellow eye. syndrome)
– Ocular developmental disorders
Q.3. How to differentiate between Haab’s
♦ Anterior segment dysgenesis
striae and force injury?
♦ Aniridia
Ans. In case of forcep injury, Descemet’s
♦ Congenital ectropion uvea
membrane tears are usually vertical or
♦ Nanophthalmos
oblique but these tears are horizontal in
– Ocular diseases—retinoblastoma,
congenital glaucoma (i.e. Haab’s striae).
retinopathy of prematurity (ROP),
[also See Table 1]
persistent hyperplastic primary
Q.4. Is buphthalmos reversible? vitreous (PHPV), trauma, uveitis.
Ans. Cupping of the optic nerve head proceeds
Q.6. What are the issues in management of
more rapidly in infants than in adults and
congenital glaucoma?
is more likely to be reversible if the pressure
Ans. Following are the issues in management of
is lowered early enough. The cupping
congenital glaucoma:
appears to be caused by incomplete
• A ssessing etiology and inheritance of
development of connective tissue in the
congenital glaucoma
lamina cribrosa, which allows compression
• Managing systemic problems of secondary
or posterior movement of the optic disc
congenital glaucoma
tissue in response to elevated IOP, with an
• Deciding type of surgery
elastic return to normal when the pressure
– Goniotomy–clear cornea
is lowered.
– Trabeculotomy or Trabeculotomy +
Q.5. Write about classification of congenital Trabeculectomy
glaucoma. – Valve implant
Ans. • Primary • Managing associated ocular problems—
– Congenital refractive errors, corneal opacity, cataract,
– Infantile squint, amblyopia
– Juvenile • Counselling of parents.
Glaucoma 191
Table 2 Hoskins’ anatomic classification of the developmental glaucomas

Group Major category Sub-category Variants Examples
I Isolated Flat iris insertion • Anterior insertion
trabeculodysgenesis • Posterior insertion
Malformation of trabecular • Mixed insertion
meshwork in absence of
Concave (wraparound)
iris or corneal anomalies
iris insertion
Unclassified
II Iridotrabeculodysgenesis Anterior stromal Hypoplasia Can be seen in
Trabeculodysgenesis with defects of the iris Axenfeld’s, Rieger’s,
iris anomalies and Peters’ anomalies
Hyperplasia Sturge-Weber
syndrome with
glaucoma
Anomalous iris vessels • Persistence of
tunica vasculosa
lentis
• Anomalous
superficial vessels
Structural anomalies • Holes
• Colobomas
• Aniridia
III Corneotrabeculodysgenesis Peripheral corneal Axenfeld’s anomaly
Trabeculodysgenesis with defects
congenital corneal defects.
Midperipheral corneal Rieger’s anomaly
Usually associated with iris
defects
anomalies
Central corneal defects Peters’ anomaly
Abnormalities of Microcornea or
corneal size megalocornea and
their associations
Q.7. Give details of Hoskin’s anatomic REFERENCES

classification of the developmental
glaucomas. 1. Moore DB, Tomkins O, Ben-Zion I. A review of
primary congenital glaucoma in the developing
Ans. See Table 2. Developemental anomalies
world. Surv Ophthalmol. 2013;58(3):278-85.
of anterior segment is the hallmark of
Review.
congenital glaucoma. It may involve one 2. Krishnadas R, Ramakrishnan R. Congenital
or more of the angle structures such as the glaucoma—A brief review. Journal of Current
trabecular meshwork, the iris, and/or the Glaucoma Practice. 2008;2(2):17-25.
cornea. Hoskin’s classification is based 3. Hoskins HD Jr, Shaffer RN, Hetherington J Jr.
on this. It is very useful for planning the Anatomical classification of the developmental
treatment as well as prognostication of the glaucomas. Arch Ophthalmol. 1984;102:1331.
cases.3
NEOVASCULAR GLAUCOMA
Jennil Shetty, Talvir Sidhu
INTRODUCTION zz Common in post vitrectomized eyes of

proliferative diabetic retinopathy especially if
Neovascular glaucoma (NVG) is a severe form of
the eye is having untreated retinal detachment.
secondary glaucoma characterized by proliferation
of fibrovascular tissue in the anterior chamber
such as iris and angles. It is a potentially blinding EXAMINATION
clinical condition, where delayed diagnosis
or poor management can result the treatment
involves management of both the elevated The underlying cause of retinal ischemia is
intraocular pressure (IOP) and the underlying associated with several systemic diseases such
cause of disease.1,2 as diabetes, hypertension. A thorough systemic
examination is carried out to look for complications
HISTORY of these diseases.
Chief complaints: A case of NVG usually presents
with:
Ocular Examination
zz Pain, redness and decrease of vision in the Lid: Normal or edematous if there is acute rise
affected eye. of IOP in the affected eye as in angle closure
zz Depending on the cause of the neovascular glaucoma stage.
ization patient may give a history of dimness
Conjunctiva: May be congested if IOP is grossly
of vision preceding pain and redness for few
raised.
weeks or months.
Cornea: Clear or hazy due to epithelial edema
History of Present Illness depending on the IOP.
Dimness of vision may precede the history of pain Iris: Presence of neovascularization can be seen
and redness by few weeks to months depending (Fig. 1). A careful examination of the iris and angle
on the cause of the neovascularization. Usually the of the anterior chamber is essential, before the
loss of vision in the affected eye is of sudden onset pupil is dilated and any drops put in the eye. Once
but may be of gradual onset as well depending on the pupil is dilated, it may not be easy to find the
the underlying cause. At the time of presentation NV.1 During the early stages, neovascularization
patient may or may not have regained the lost of the iris (NVI) is essentially at the pupil margin
vision. and is very fine and delicate in character (Fig. 1).
At times new vessels may be seen in angles (NVA)
History of Past Illness on gonioscopy, while NVI is yet to appear (Fig. 2).
Neovascular glaucoma (NVG) can be associated Anterior chamber (AC): The AC often shows the
with recurrent attacks of angle closure glaucoma presence of flare and sometime a few cells. There
hence; similar episodes of dimness of vision in the may be presence of hyphema also.
past may be present. Pupil: Following signs can be seen:
zz Fine randomly oriented superficial vessels
Past Surgical History near pupillary margin.
zz Patients may have history of cataract surgery. zz Presence of ectropion uveae.
More often, it is associated with complicated zz Pupillary reaction may be sluggish or absent or
cataract surgeries resulting in posterior there may be relative afferent pathway defect
capsular dehiscence or leaving the patient (RAPD) depending upon the extent of optic
aphakic. nerve damage.
Glaucoma 193
Fig. 1: Neovascularization of iris Fig. 2: Neovascularization of angle
Table 1 Stages of neovascular glaucoma

Stage Characteristic NVI/NVA Symptoms Other signs IOP Gonioscopy
Prerubeosis Predisposing Absent Absent Laser photometry Normal Open angle
stage ocular/ or fluorescein iris
extraocular angiography is helpful
condition is in detecting early
present leakage of iris vessels
Pre- Rubeosis iridis Present Absent Usually none Normal Open angle
glaucoma stage with early
stage NVA
Open angle Florid Florid Present Cells, flare, hyphema Elevated Open angle
glaucoma rubeosis with with florid
stage leaky vessels NVA
Angle Contraction of New Present Loss of visual acuity Persistently Variable PAS
closure fibrovascular vessels Ectropion uveae, flat high Sometime
glaucoma membrane in become smooth, glistening (> 60 mm) complete
stage the angle less appearance of the iris. angle
apparent Conjunctival congestion closure
Corneal edema
Cells, flare, hyphae
Optic disc changes
Abbreviations: IOP, intraocular pressure; NVI, neovascularization iris; NVA, neovascularization of angles;
PAS, peripheral anterior synechiae
IOP: Markedly raised in angle closure stage. Optic nerve hypoplasia (ONH) damage depends
upon the duration and severity of raised IOP.
Lens: Pseudophakia with or without posterior
It is important to remember that the disease
capsular dehiscence or aphakia are risk factors for
process progresses through four stages and the
NVI.
symptoms and signs depend upon the stage
Fundus: Evidence of retinal ischemia in the at which the patient presents. The stages and
form of central retinal vein occlusion (CRVO), the associated symptoms and signs have been
proliferative diabetic retinopathy (PDR), central described in Table 1.
retinal artery occlusion (CRAO), etc. maybe there.
DIFFERENTIAL DIAGNOSIS Anti-VEGF agents: As an adjunctive treatment

with PRP.
Normal iris vessels: In some eyes normal iris
vessels are seen easily, particularly in blue eyes
that may be mistaken for NVI or even angle NV
Medical Management
when the vessels are seen near the root of the iris.1 Medical management consists of following:
Following points may help in differentiating: Antiglaucoma drugs: Mainstay of medical
zz Iris vessels are present in stroma but new treatment is to reduce aqueous production with
vessels are superficial. topical beta-blockers, alpha-2 agonists and with
zz Iris vessels are radial in arrangement, unlike topical and/or oral carbonic anhydrase inhibitors.
irregularly arranged new vessels. Mannitol (hyperosmotic agents) may also be
zz Sizes of iris vessels are uniform, new vessels required in cases of acute rise in IOP. Topical
are of varying sizes. prostaglandin analogues and Miotics are better
zz Branching of new vessels is absent in iris avoided as they may increase ocular inflammation.
vessels.
zz New vessels are leaky as is found in fluorescein Anti-inflammatory drugs: Topical steroids and
angiography or fluorophotometry. cycloplegics are recommended to reduce the
In the open angle stage, neovascular glaucoma inflammation that is often present.
may have to be differentiated from other glaucoma Antiangiogenic drugs: Several studies propose the
of acute onset such as angle closure glaucoma and usefulness of anti-VEGF agents as an adjunct to
glaucoma with uveitis. NVG can be differentiated traditional treatments such as PRP and additional
easily by the presence of rubeosis iridis in spite of surgery. Anti-VEGF (intracameral or intravitreal, or
the fact that eyes with uveitis may have dilated iris both simultaneously) have been used in following
vessels. circumstances.
In angle closure stage, when new vessels are zz As an adjunct to panretinal photocoagulation
less apparent the condition has to be differentiated (PRP) or bevacizumab alone when visibility
from other causes of iris distortion and peripheral of the posterior segment is difficult due to
anterior synechia, e.g. old trauma or iridocorneal opacities of the media (e.g. hemorrhage).
endothelial (ICE) syndrome. zz Intracameral injection of bevacizumab
may provide additional strategy for treating
MANAGEMENT rubeosis iridis in NVG. bevacizumab is well
tolerated, effectively stabilizes NVI, and
The treatment of NVG includes identifying the controls IOP when used alone and at an early
underlying etiology and its timely and adequate open angle stage of NVG.
treatment to prevent the development and zz In advanced cases of NVG, it can be used as
progression of NVG. Once NVG develops and IOP a therapeutic window before PRP or surgical
is high, the target is to control high IOP and prevent intervention (usually 1 week before but can be
optic nerve damage in addition to treatment of within 14–48 hour also). It decreases the risk of
underlying etiology. failure, hemorrhage and inflammation.
zz In cases where PRP is not possible due to poor
Prophylactic Treatment retinal view, intravitreal bevacizumab can
Panretinal photocoagulation: Mechanism is be given followed by Trabeculectomy with
uncertain, but probably it acts by reducing oxygen Mitomycin C.
demand. PRP is able to reverse IOP elevation in
the open angle glaucoma stage and in early angle Remember
closure stage where synechial angle closure is not zz Bevacizumab (most reported anti-VEGF in
more than 270° yet. NVG) cause regression of the NVI within 24
Trans-scleral panretinal cryotherapy or anterior to 48 hours following intravitreal injection
retinal cryopexy (ARC): When cloudy media whereas NVI starts regressing post PRP by
preclude PRP. 2 weeks and is complete by 4–6 weeks.3
Glaucoma 195
Most studies report similar dose for intravitreal

VIVA QUESTIONS
zz
and intracameral use (1.25 mg/0.05 mL).

zz Medical management with anti-VEGF Q.1. Name important causes of neovascular
along with retinal ablation can control the glaucoma.
IOP in the open angle stage of NVG only; in Ans. • Diabetic retinopaty: Most common cause
advanced stage with synechial, angle closure of NVI. In fact 1/3rd of rubeotic cases
surgical intervention for IOP lowering is often have diabetic retinopthy.
required. • R etinal vascular occlusive diseases:
Second commonest cause—ischemic
Surgical Management central retinal vein occlusion (CRVO),
central retinal artery occlusion (CRAO),
The type of surgery depends upon level of IOP, branch retinal vein occlusion (BRVO),
presence of active or regressed NVI, prior laser or branch retinal artery occlusion (BRAO),
anti-VEGF treatment, prior intraocular surgeries, sickle cell retinopathy.
degree of inflammation, stage of disease, degree • E xtraocular diseases: Carotid artery
of angle closure, severity of glaucomatous optic disease, ocular ischemia, giant cell
neuropathy and visual potential. 3 Following arteritis, pulseless disease, and carotid-
options are available: cavernous fistula.
zz Filtration surgery: Success rate of trabe • Assorted retinal diseases: Retinopathy of
culectomy are poor if performed alone. It is prematurity (ROP), retinal detachment
usually combined with use of intra-operative (RD), Eale’s disease, Coat’s disease,
mitomycin C (MMC). Chance of success persistent hyperplastic primary vitreous
increases significantly when combined (PHPV), Norrie’s disease.
with preoperative bevacizumab and/or PRP • Trauma
(success rate may improve up to 95%).3 • Ocular neoplasms: Malignant melanoma,
zz Glaucoma drainage device surgery: Glaucoma retinoblastoma, and optic nerve glioma.
drainage devices (GDDs) are often considered • O cular inflammatory diseases: Chronic
as a primary surgical procedure in the uveitis, endophthalmitis, sympathetic
management of NVG where there is a high risk ophthalmia and Vogt Kayanagi Harada’s
for failure of conventional filtering surgery. disease (VKH).
Various drainage devices like Molteno • O cular surgery: Cataract extraction
implant ; Baerveldt implant and Ahmed especially in diabetics, vitrectomy,
glaucoma valve have been used in manage retinal detachment surgery.
ment of NVG and shown comparable results Note: Of all these causes three most
to trabeculectomy. common causes are diabetic retinopathy,
zz Cyclodestructive procedure: These procedures ischemic CRVO and ocular ischemic
syndrome. In Indian set-up, chronic angle
are indicated in cases with refractory NVG
closure glaucoma is also an important
with poor visual prognosis. Trans-scleral
cause of NVG.
cyclophotocoagulation (TSCPC) with non
contact neodymium:yttrium-aluminum- Q.2. Where does neovascularization start?
garnet (Nd:YAG) (TSCPC) or semiconductor Ans. At pupillary margin, from capillaries of
diode laser cyclophotocoagulation (DLCP) minor arterial circle.
have proven useful in treatment of such cases. Q.3. In what percentage of cases of neovascular
Repeat treatment may be required to maintain glaucoma neovascularization at the
good control of IOP.3 angle (NVA) may be found in absence of
zz Other surgeries: Endoscopic cyclophotocoa NVI at pupillary margin?
gulation, intravitreal injection crystalline tri Ans. In approximately 12%.1,3
amcinolone acetonide, injection of silicon oil Q.4. How can we differentiate between normal
during revision of vitrectomy after unsuccess iris vessels and new vessels?
ful vitreous surgery in diabetics. Ans. Following points helps:
• I ris vessels are present in stromal but in acute- onset glaucoma. Rubeosis
new vessels are superficial. iridis in this stage is more florid and is
• Iris vessels are radial in arrangement, often associated with anterior chamber
unlike irregularly arranged new vessels. inflammatory reaction. Due to fragile
• Sizes of iris vessels are uniform, new nature of the new vessels, a hyphema
vessels are of varying sizes. can also present at this stage sometimes.
• Branching of new vessels is absent in iris Gonioscopy shows an open angle but
vessels. with more intense neovascularization.
• New vessels are leaky as is found in fluo- • Angle-closure glaucoma stage: Heavy
rescein angiography or fluorophotometry. neovascularization and extensive peri
Q.5. How do you identify NVA on gonioscopy? pheral anterior synechia. Most patients
Ans. New vessels extend from iris root across the present or are detected at this stage. In
ciliary body and sclera spur; arborize over this stage, the contraction of fibrovas
the trabecular meshwork. cular membrane in the angle leads to
progressive synechial angle closure,
Q.6. What is hundred-day glaucoma? ectropion uveae and flat, smooth, glisten
Ans. NVG occurring after 3 months following ing appearance of the iris. Gonioscopy
CRVO. reveals varying degrees of peripheral
Q.7. Describe the stages of NVG. anterior synechiae or complete angle
Ans. Stages of NVG are as follows (See Table 1) closure may be present at this stage. The
• P re-rubeotic stage—In patients with IOP is usually very high and can go up to
proliferative diabetic retinopathy and 60 mm Hg. Conjunctival congestion and
ischemic CRVO, neovascularization corneal edema are frequently present.
must be looked for carefully under Glaucomatous optic nerve damage is
high magnification on the iris and often moderate to advance. Visual acuity
in the angle of the anterior chamber may also be severely affected.
(neovasculariztion of angle—NVA) at Few authors include a regression stage
every visit. The iris should be examined characterized by total synechial angle
before dilatation of the pupil and closure and less visible vessels.
pupillary margins and margins of
Q.8. How does surgery influence the occur
iridotomy should be carefully looked for
rence of neovascularization?
new vessels.
Ans. Crystalline lens, posterior capsule, vitreous
• P re-glaucoma stage/rubeosis iridis:
all act as mechanical barriers for angiogenic
Variable amounts of neovascularization
factors liberated by ischemic retina to reach
(rubeosis) can be found at pupillary
anterior chamber. Vitrectomy, cataract
margin, iris surface and in the angle.
surgery especially with disruption of
Characteristic features of this stage
posterior capsule removes this mechanical
are normal IOP, unless pre-existing
barrier and an increased risk of NVG.
concomitant POAG/PACG is present.
Patients are usually asymptomatic at this Q.9. What are the theories behind neovascul
stage unless the underlying condition ogenesis in NVG?
produces symptoms like field loss due to Ans. Following factors play an important role in
CRVO or decreased vision due to vitreous NVG:
hemorrhage or macular ischemia in • Retinal hypoxia: Stimulus for release of
diabetic retinopathy (DR). angiogenic factors.
• Open angle glaucoma stage: New vessels • Angiogenesis factors: VEGF with its
spreading and fibrovascular tissue several isoforms, angiogenin, and platelet
covering angle. At this stage, IOP begins derived endothelial growth factor, TGF
to rise and stays elevated. In some cases, beta, TNF alfa, vascular endothelial
the IOP may rise suddenly resulting growth factor.
Glaucoma 197
• Vaso-inhibitory factors: Vitreous and lens 2. Saito Y, Higashide T, Takeda H, Ohkubo S,

may be potential source of these factors; Sugiyama K. Beneficial effects of preoperative
explains why NVG is more common intravitreal bevacizumab on trabeculectomy
following lensectomy and vitrectomy. outcomes in neovascular glaucoma. Acta
Ophthalmol. 2010;88:96-102.
3. Sharma P, Agarwal N, Choudhry RM.
REFERENCES
Neovascular glaucoma: A review. Delhi J
1. Hayreh SS. Neovascular Glaucoma. Prog Retin Ophthalmol. 2016;26:170-5.
Eye Res. 2007;26(5):470-85.
ANGLE-RECESSION GLAUCOMA
Prakhar Goyal, Divya Agarwal, Talvir Sidhu
Angle recession glaucoma is a secondary open General Examination/Specific Systemic
angle glaucoma that is associated with blunt Examination
trauma to eye. Angle recession is a tear between
Look for any signs of trauma especially scars
the longitudinal and circular muscles of the ciliary
around the eye.
body. It is a gonioscopy diagnosis. Post-traumatic
hyphema is strongly associated with angle
recession (60–90%).1,2 Ocular Examination
Examination will show features of trauma along
HISTORY with angle recession which is diagnosed on
gonioscopy.
Chief Complaints
Eyeball: Look for any associated signs of trauma
The patient may present with following: such as orbital fracture, enophthalmos, periocular
zz Deep set pain, redness and gradually scars.
diminishing vision for distance (when
Lid: In case of early presentation lid edema or lid
associated with glaucoma).
laceration may be present due to blunt trauma.
zz Onset can be immediately after injury or
Those presenting late may show scar of eyelid
months to years after blunt trauma.
repair.
zz May be completely asymptomatic.
Conjunctiva: May be normal in delayed presenta
History of Past Illness tion. Presence of subconjunctival hemorrhage can
be seen in acute cases.
Patients presenting late with complaints of deep
seated ocular pain or other symptoms of raised Cornea: Following points must be noted:
intraocular pressure will give past history of trauma
zz Stromal edema and pigment deposition
weeks or months back. The case may present on endothelium, blood staining of the
years after the trauma, when glaucoma occurs. endothelium may be there in early onset cases.
Although eye trauma invariably occurs before
zz Longstanding cases, no abnormality can be
angle recession, it is common to have forgotten seen.
details of the injury or even the entire episode after Sclera: Partial or complete scleral tear may be
a number of years have passed. associated in early presentation.
Anterior chamber (AC): Following points must be

noted.
zz Deep and irregular AC depth may be seen
zz Hyphema in early onset.
Iris: Look for other manifestations of blunt trauma
such as:
zz Iridodialysis (D shaped pupil)/cyclodialysis
zz Iris sphincter tears
zz Iridoschisis.
Pupil: Signs of trauma such as corectopia and
traumatic mydriasis can be there.
IOP: IOP is raised when presents with glaucoma.
A reduced IOP may be seen in early cases due to Fig. 1: Widening of the ciliary body band due to
ciliary shock/ciliary shutdown. retrodisplacement of iris root
Lens: Look for previous signs of trauma such as:
zz Subluxated or dislocated lens. DIFFERENTIAL DIAGNOSIS
zz Cataract may be present in some cases due to
associated trauma. Although diagnosis of angle recession glaucoma is
zz Vossius ring. evident on gonioscopy and optic disc examination
other differential diagnosis of unilateral glaucoma
Vitreous: Vitreous hemorrhage may be present as should be considered like:
a consequence of trauma. zz Pseudoexfoliation glaucoma
Fundus: Following signs of trauma must be looked zz Neovascualr glaucoma
for: zz Lens particle and phacolytic glaucoma should
zz Retinal dialysis, retinal detachment, sub- be considered and differentiated from angle
retinal hemorrhages. recession.
zz Patients will long-standing raised IOP will
show glaucomatous optic nerve changes and MANAGEMENT
other features as seen in POAG.
The treatment of angle recession glaucoma can be
Gonioscopy: In acute cases gonioscopy examina divided into following:
tion should be deferred for at least 4–6 weeks post
acute injury. Gonioscopy signs: Medical Management
zz Gonioscopy examination using 4-mirror
In acute cases treatment should be directed at
Gonio lens shows asymmetry of angle recess
lowering IOP and reducing inflammation by use
if compared to nontraumatized eye or to
of aqueous suppressants like topical B-blockers-
other quadrants of same eye. Simultaneous,
Timolol (0.5%) BD or/and alpha 2 agonists like
bilateral Koeppe gonioscopy is the most useful
apraclonidine or brimonidine (0.2%). Topical
technique to detect subtle angle recession.
cycloplegics like atropine and steroids should be
zz Widening of the ciliary body band due to retro
given for relief of pain and to reduce inflammation
displacement of iris root is the most impor
and possibly risk of secondary hemorrhage.
tant gonioscopic findings of angle recession
(Fig. 1).
zz Prominent scleral spur. Laser Trabeculoplasty
zz Irregular and darker pigmentation of angle. It has found to be ineffective in angle in most
zz Peripheral anterior synechiae. cases due to distortion of angle anatomy and
Glaucoma 199
TM scarring. Nd:YAG laser trabeculopuncture has Q.6. What are the 7 rings of trauma?
found to be effective in some cases as shown in Ans. This often refers to the seven commonly
some studies where TM was intact on gonioscopy. injured intraocular structures following
contusion injury:
Filtration Surgeries • Iris sphincter tear
• Iridodialysis
Trabeculectomy is effective in controlling
• Angle recession
IOP when used with antimetabolites however
• Cyclodialysis
success rate is lower as compared to POAG. Use
• Tear in trabecular meshwork
of glaucoma drainage devices has also limited
• Z onular dialysis, subluxation or
benefits in angle recession glaucoma.
dislocation of lens
• Retinal dialysis or tears.
VIVA QUESTIONS Q.7. What is the risk of glaucoma in angle
recession?
Q.1. What is the incidence of angle recession Ans. • Glaucoma is seen in only 5.5% (7 to 10%)
after blunt trauma? of patients with angle recession.1-3
Ans. Angle recession is reported to occur in 20 • A n increased risk of glaucoma
to 94% of eyes after blunt trauma. It is often development was found if the angle
masked initially due to the presence of recession exceeded 180°.
concomitant hyphema, which results from • Two peaks in incidences of glaucoma
shearing of the anterior ciliary arteries.1 is seen, less than 1 year and at least
10 years after trauma. A 3.4% incidence
Q.2. What are the chances of getting angle
of glaucoma after ocular contusion
recession in a case of traumatic
has been reported during a 6-month
hyphema?
follow-up and up to 10% during the
Ans. Angle recession may occur in 85% (range 71
10 years after trauma.1,2
to 100% of eyes) of patients with traumatic
hyphema.1,2 Q.8. How to differentiate angle recession from
cyclodialysis?
Q.3. What is the mechanism of angle Ans. Ciliary muscle is torn between the
recession? longitudinal and circular layers in angle
Ans. Close globe injury causes anteroposterior recession. The longitudinal or meridional
globe compression with equatorial scleral ciliary muscle remains attached. This
expansion, limbal stretching, and posterior distinguishes recession from cyclodialysis,
displacement of the lens/iris diaphragm. where the entire ciliary body including the
This may lead to the angle recession. longitudinal muscle is detached.
Q.4 What is the mechanism of glaucoma in
angle recession? REFERENCES
Ans. Mainly due to trabecular damage and not 1. Girkin CA, McGwin G Jr, Long C, Morris R, Kuhn
the recession itself. F. Glaucoma after ocular contusion: a cohort
study of the United States eye injury registry.
Q.5. What are the sources of traumatic
J Glaucoma. 2005;14:(6):470-3.
hyphema?
2. Kaufman JH, Tolpin DW. Glaucoma after
Ans. • Major arterial circle and branches of the traumatic angle recession: a ten-year
ciliary body (MC >90% cases) prospective study. Am J Ophthalmol. 1974;78:
• Choroidal arteries (rare) (4):648-54.
• Ciliary body veins (very rare) 3. Sihota R, Sood NN, Agarwal HC. Traumatic
• Iris vessels at the pupillary margin or in glaucoma. Acta Ophthalmol Scand. 1995;73(3):
the angle (very rare). 252-4.
STEROID-INDUCED GLAUCOMA
Vaishali Ghanshyam Rai, Talvir Sidhu
INTRODUCTION zz Conjunctiva and sclera—usually normal

zz Cornea—long-term topical steroid may cause
A certain percentage of the general population increased corneal thickness or corneal ulcers.
responds to repeated instillation of topical zz Angle of anterior chamber—normal depth and
corticosteroids with a variable increase in the contents.
intraocular pressure (IOP). Certain people do zz Pupil—topical steroids use can cause
manifest this response to chronic steroid therapy, mydriasis.
whether given by the topical, systemic, or zz Lens—usually appears normal or may show
periocular route, and the IOP elevation can lead posterior sub capsular cataract that is also a
to glaucomatous optic atrophy and loss of vision. side effect long-term use corticosteroid.
Such a condition is referred to as steroid induced zz Vitreous—normal
glaucoma. Following 4–6 weeks of topical steroid zz Fundus—glaucomatous cupping of optic
administration, about 5% of the population will nerve.
demonstrate a rise in IOP of more than 16 mm Hg zz IOP—with Goldmann’s applanation tonometer
and 30% a rise of 6–15 mm Hg.1 may be normal due to discontinuation of
steroid therapy in past. IOP may be high in
HISTORY cases of intravitreal steroid implants or patient
Chief Complaints currently on any steroid medication.
zz Gonioscopy with four mirror Gonio lens
The clinical presentation and onset is highly
reveals open angles in all quadrants.
variable. Patient may present with pain and
gradually diminishing vision for distance or may
be completely asymptomatic. DIFFERENTIAL DIAGNOSIS
Primary Open Angle Glaucoma
Past History
zz Usually bilateral with high IOP. Steroid
Patient may give history of long-term use of induced glaucoma can be unilateral or
corticosteroids in any form like topical, systemic bilateral depending upon steroids used with
or local applications in past. History of intravitreal normal or high IOP.
triamcinolone acetonide (IVTA) or slow release zz History of steroid intake
intravitreal implant of dexamethasone like ozurdex —— Uveitic glaucoma
is also important. —— Normal tension glaucoma—usually
Risk factors for steroid induced glaucoma include: bilateral with thin cornea with normal
zz Patients with primary open angle glaucoma IOP
(POAG) —— Glaucomatocyclytic crisis—usually uni
zz Family history of POAG lateral with other uveitis signs like circum
zz Children below 10 years ciliary congestion, keratic precipitates on
zz High myopia corneal endothelium, anterior chamber
zz Diabetes mellitus reaction with raised IOP.
zz Connective tissue disorder, e.g. rheumatoid —— Primary juvenile open angle glaucoma.
arthritis.
MANAGEMENT
EXAMINATION zz Stop the responsible steroid medication, in
Ocular examination: majority of cases raised IOP comes to normal
zz Eyeball—normal levels within few weeks to months.
zz Eyelids—usually normal, or may show eyelid zz In refractory cases with advanced glaucoma
skin atropy or ptosis with topical steroids tous optic nerve, damage management would
Glaucoma 201
be cessation steroid and start antiglaucoma zz Prevention of steroid induced glaucoma

medication to reduce IOP. —— By regular monitoring of patients on
zz Glaucoma following IVTA needs to be steroids for IOP, check every 2 weeks
managed with antiglaucoma medication till —— Avoid topical steroids wherever possible
the intravitreal steroid crystals resolve, i.e. by using alternatives

6 months —— Use of lower potent steroids like
zz Intractable glaucoma following intravitreal fluorometholone or loteprednol.
steroid depot may be treated by removal
of depot through pars plana vitrectomy
VIVA QUESTIONS
combined with trabeculectomy.
zz Substitute potent steroid with lower potency Q.1. Define steroid induced glaucoma.
steroid where complete cessation of steroid Ans. Steroid induced glaucoma is a form of open
for medical condition is not possible. Lower angle glaucoma occurring as an adverse
potency steroids like phosphate forms pre effect exogenous corticosteroid therapy
dnisolone and dexamethasone, rimexolone, or excess endogenous production of
loteprednol etabonate or fluorometholone can glucocorticoids.
be used to substitute potent steroids.
zz Steroids can also be substituted with non- Q.2. What is an average time taken for
steroidal anti-inflammatory drugs like IOP rise in different routes of steroid
diclofenac, nepafenac or ketorolac. administration?
zz Other steroid sparing agents are immuno Ans. See Table 1.
suppressant like tacrolimus ointment or Q.3. Explain the mechanism of steroid
cyclosporine for vernal keratoconjunctivitis induced glaucoma.
or methotrexate can be used in uveitis or Ans. Corticosteroids cause IOP elevation by
systemic conditions. increasing the outflow resistance and
zz Laser trabeculoplasty: Argon laser trabeculo- thereby decreasing the facility of aqueous
plasty or selective laser trabeculoplasty can be outflow. The main mechanisms of steroid
considered where medical treatment failed to induced glaucoma are as follow:
control the IOP or patient is intolerant to med- • Stabilization of lysosomal membranes
ical treatment or in cases where filtering sur- and leading to accumulation of
gery is precluded due to systemic condition. polymerized glycosaminoglycan (GAG).
zz Trabeculectomy: With or without anti- • A lteration of the composition of the
metabolites is indicated in patients whom extracellular matrix through which aque
both medical or laser treatment fail to control ous flows, thereby increasing resistance
IOP. to outflow.
Table 1 Average time taken for IOP rise in different routes of steroid administration
Route Average dose Average time taken for IOP rise
Oral 25 mg hydrocortisone/day 1 year
50 mg prednisolone /day 2–15 months
Inhalational Most of steroid inhalers 3 months
Pulse steroids 140 mg repeated 4 weekly 6 months
Dermatological Betamethasone cream 0.1% 3 months
Topical QID doses of potent steroid 2–6 weeks
IVTA 4 mg 4–8 week
Posterior sub tenon 40 mg of triamcinolone acetonide 5–9 weeks
• I ncreased production of collagen, Table 2 Becker and Armaly studies

elastin, laminin and fibronectin within
trabecular meshwork and resulting Parameters Becker Armaly
in increased in trabecular meshwork Frequency QID TDS
resistance. Duration 6 weeks 4 weeks
• Inhibition of phagocytosis activity of
endothelial cells lining the trabecular Parameter Final IOP IOP change
meshwork and leading to accumulation Type of responder
of debris in the trabecular meshwork. Low <20 mm Hg <6 mm Hg
• Decreased production of extracellular
Intermediate 20–30 mm Hg 6–15 mm Hg
proteinase like fibrinolytic enzymes, stro
molysin and matrix metalloproteinases. High >31 mm Hg >15 mm Hg
• Inhibition of the production of outflow
enhancing prostaglandins such as
PGF 2α.
Table 3 Intraocular pressure elevation in
Q.4. Name gene associated with steroid different type of steroid
induced glaucoma.
Mean intraocular
Ans. Many genes associated with steroid
pressure rise
induced glaucoma are myocin, optineurin, Type of steroid (in mm Hg)
antichymotrypsin, pigment epithelium-
derived factor, cornea-derived transcript Dexamethasone 0.1% 22
6, prostaglandin D2 synthase, decorin, Prednisolone 1.0% 10
insulin-like growth factor binding protein 2, Dexamethasone 0.005% 8
ferritin light chain and fibulin-1C. Myocilin
Fluoromethalone 0.1% 6
gene (previously known as the trabecular
meshwork inducible glucocorticoid Hydrocortisone 0.5% 3
response or TIGR gene) is the most studied Tetrahydrotriamcinolone 0.25% 2
amongst these.1,2
Q.5. Name two landmark studies done on
steroid induced glaucoma. What findings Q.7. Intraocular pressure elevation in
were demonstrated in those studies? different type of steroid
Ans. Becker and Armaly studies. The findings Ans. See Table 3.2
have been described in Table 2.
Q.6. Low potency steroid and risk of glaucoma. REFERENCES
Ans. Different low potency steroids are
1. Jones R 3rd, Rhee DJ. Corticosteroid-induced
phosphate forms prednisolone 0.1% and ocular hypertension and glaucoma: a brief
rimexolone 1%, loteprednol etabonate review and update of the literature. Curr Opin
0.5% or fluorometholone 0.1%. Risk of Ophthalmol. 2006;17:163-7.
glaucoma after use of low potency steroid 2. Kersey JP, Broadway DC. Corticosteroid-
has not been studied in detail. However, the induced glaucoma: a review of the literature.
general agreement is it is very less. Eye (Lond). 2006;20(4):407-16.
Glaucoma 203
PSEUDOEXFOLIATION GLAUCOMA
INTRODUCTION zz Conjunctiva—may be normal or ciliary con

gestion can be there in presence of raised IOP.
Pseudoexfoliative syndrome (PXF) is a systemic zz Cornea—small whitish powdery flakes or
condition characterized by the deposition of white
clumbs may be found on corneal endothelium.
powdery or fluffy material within the anterior
Early corneal endothelial decompensation
segment of the eye including lens, angle, pupil and
is common with PXF syndrome (Fuchs like
cornea. The deposits most notable on the anterior
keratopathy). Corneal guttata may also be
lens capsule. PXF can cause secondary open angle
found the exact cause of which is not known.
glaucoma which is more aggressive in its clinical
Pigment can be observed dispersed on the
course with high IOP at onset, progresses at a
endothelium. The pigment is believed to arise
faster rate and responds poorly to medical therapy,
from disruption of iris pigment epithelium
compared to primary open angle glaucoma
secondary to frictional interaction with PXF
(POAG). Pseudoexfoliative glaucoma is seen in up
material on the lens capsule.2
to 50% of eyes with PXF.1,2
zz Anterior chamber (AC): AC may be shallow in
pseudoexfoliation syndrome. Small pigments
HISTORY
can be appreciated floating in anterior chamber.
Epidemiology PXF syndrome has been associated with a
disrupted blood-aqueous barrier. Testing
Pseudoexfoliative syndrome (PXF) is seen
with a flare meter demonstrated markedly
worldwide with a high prevalence in Scandinavian
increased flare in comparison with primary
countries. Incidence varies between 1.5% to 27%.
open angle glaucoma.2 The zonulopathy can
Incidence increases with age. Commonly seen after
also lead to anterior displacement of the
the age of 40 years. Few studies suggests a female
lens, that is, a phacomorphic narrowing, with
preponderance however it is still debated.1,2
intermittent pupillary block.
zz Iris and pupil: Iris and pupil examination can
Chief Complaints reveal following signs:
Patient may present with following: —— Small flecks of PXF material deposits can
zz Pain and redness usually unilateral be seen on pupillary margin which is

zz Gradually diminishing vision for distance hallmark of PXF syndrome.
zz Patient may be completely asymptomatic. —— There may be flakes deposits on iris crypts
The majority of patients are asymptomatic, and folds.

and PXF is often an incidental finding. —— Trans-illumination defect can be
appreciated in pupillary margin resulting
EXAMINATION from atrophic and/or fibrotic changes in
the iris sphincter muscle. The pupillary
Ocular Examination margin of the iris is also affected with
zz Eyeball—usually normal or may be deep loss of the pupillary ruff. These changes
seated as patients with pseudoexfoliation collectively result in what is described as
are usually present in 7–8th decade. Relative a “moth eaten” pupil margin.2
anterior microphthalmos (normal axial length —— Look for iridodonesis which is common
but anterior segment is smaller) may also be in PXF syndrome.

there. —— Sphincter muscle degeneration and
zz Eye lid—normal. posterior synechiae are also seen.

zz Pupil: Usually poor mydriasis (atrophic and/or zz IOP: Measured with Goldmann applanation
fibrotic changes in the iris sphincter muscle) tonometer shows >21 mm Hg. Usually mean
and asymmetric pupil is seen in PXF. IOP in PXF patient is more than that of POAG
zz Lens: Following signs can be seen: cases. 40% of pseudoexfoliation syndrome
—— Whitish powdery ring deposit on anterior patients will develop glaucoma or ocular
lens capsule is more consistent and hypertension.
diagnostic sign of PXF (Fig. 1). zz Gonioscopy: Shows characteristic increased
—— Target sign: The deposition is observed pigmentation of trabecular meshwork, more
in 3 distinct zones; a central zone of prominent in superior quadrant and usually
material deposition; clear intermediate unilateral. Dark, dense and uneven wavy
zone (secondary to iris excursion rubbing pigmentation along the Schwalbe’s line
the PXF material off ); a peripheral zone (Sampaolesi’s line) is also seen in PXF. This
of PXF material (Figs 2 and 3) outside of finding is not exclusive to PXF (also seen in
this intermediate zone. The central zone pigment dispersion syndrome and chronic
may be absent in 20% of cases of PXF and inflammation).2 In 9–18% angle is occludable
the peripheral granular zone can only in pseudoexfoliation syndrome.2
be observed with dilation. 2 Clinically
three distinct zones can be found on
anterior lens capsule on dilated pupil, two
concentric rings of powdery deposits with
central translucent zone.
—— Cataractous lens, nuclear sclerosis is more
common with PXF. Asymmetric nuclear

cataract formation can be there.
—— Look for phacodonesis.
—— In advanced stage there can be subluxated
or dislocated cataractous lens due to weak

zonules.
zz Fundus: Unilateral glaucomatous cupping
of optic disc with diffuse neuroretinal rim
damage if media is clear. In cases of mature
cataract needs to get B-scan biometry to see Fig. 2: Peripheral zone of PXF material
optic nerve head and retina status. over lens capsule
Fig. 1: Whitish powdery ring deposit on anterior lens Fig. 3: Peripheral ring of target sign
capsule and pupillary margin
Glaucoma 205
DIFFERENTIAL DIAGNOSIS MANAGEMENT

A case of PXF has to be differentiated from following: The medical management – similar to that of POAG
like beta-blockers, alpha adrenergic agonists,
Pigmentary Glaucoma carbonic anhydrase inhibitors or prostaglandin
analogs. Mono therapy is usually insufficient to
zz Common in young age group. control IOP in PXG so combination therapy is
zz Usually bilateral. advisable.
zz Transpupillary defect is common in mid-
Argon/selective laser trabeculectomy is
peripheral area of iris.
successful and well–established procedure for
zz Prominent uniform dark pigmentation band of
reducing IOP in PXG associated with open angles.
trabecular meshwork is characteristic feature
Trabeculectomy is indicated when medical
of pigmentary glaucoma.
or laser therapy has failed to obtain target IOP or
when there is progressive of glaucoma.
True Exfoliation
Combined trabeculectomy and cataract
zz Characterized by thin clear membrane like surgery is indicated in PXG with intractable IOP
material separating from anterior lens capsule. with cataract. Common complications anticipated
zz Glaucoma is infrequent associated with true during combined surgery in PXG are (also See
exfoliation. Table 1).
zz Underlying pathology can be anterior uveitis zz Poor dilation of pupil
or exposure to UV rays. zz Zonular dialysis and vitreous loss
zz Dislocation or decentration IOL
Primary Amyloidosis zz Corneal decompensation
zz Postoperative ocular inflammation.
zz Generalized systemic disorder with numerous
ocular manifestation along with glaucoma.
zz Bilateral ocular involvement is common. VIVA QUESTIONS
zz Fine whitish powdery deposits throughout eye
is characteristic. Q.1. What is Sampaolesi’s line?
POAG: Differentiation table in viva questions. Ans. In PXG cases on gonioscopy a dark, dense
and uneven wavy pigmentation along the
PACG: Can be differentiated by characteristic Schwalbe’s line is known as Sampaolesi’s
clinical signs. line.
Uveitic glaucoma: AC reaction, and other signs of Q.2. What are the complications anticipated
uveitis are absent. during cataract surgery in PXG cases?
Ans. • Poor dilation of pupil
INVESTIGATIONS • Zonular dialysis and vitreous loss
• Dislocation or decentration IOL
zz Diurnal variation of IOP is valuable in • Corneal decompensation
evaluating the true magnitude of IOP reduction • Heightened postoperative inflammation
zz Baseline perimetry • Postoperative IOP elevation
zz RNFL imagining • Late intraocular lens implant decen
zz Specular biomicroscopy shows reduced tration and prolapse into the posterior
endothelial cells count segment.
zz A-scan biometry for axial length and
keratometry Q.3. How do you differentiate between PXG
zz B-scan ultrasonography in case of hazy media and primary open angle glaucoma?
to evaluate retina. Ans. See Table 2.
Table 1 Difficulties and precaution during cataract surgery

Difficulty Precaution
Poorly dilating pupil • Atropine
• Stretch the pupil with instruments
• Iris hooks
• Pupil expansion rings
Endotheliopathy • Visco-adaptive (Healon 5 or Visco-dispersive viscoelastic (Viscoat)
• Ashrinoff’s soft-shell technique
Weak zonules • Chopping techniques or prolapse the lens nucleus out of the capsular bag
during phaco
• Traditional extracapsular surgery
• Capsular tension ring (CTR)
• 3-piece intraocular lens implant (IOL)
Postoperative inflammation • Aggressively with postoperative steroids and perhaps for a longer duration
Postoperative pressure spikes • Postoperative Diamox
Capsular phimosis • Nd-YAG laser, placing relaxing incisions in the anterior lens capsule at the
4 cardinal positions
Table 2 Differentiation between PXG and POAG

POAG PXG
Age of onset 40–50 years >60 years
Vision loss Less marked Marked due to presence of nuclear sclerosis
IOP > 21 mm Hg High IOP >40 mm Hg
Laterality Bilateral Usually unilateral
Anterior segment Usually normal White powdery deposits on pupillary margin, on lens
capsule with weak zonules. Subluxated or dislocated
lens can be seen
Anterior chamber Normal May be normal or shallow
Flare may be present
Gonioscopy Open angle Marked blotchy pigmentation of trabecular
meshwork. Sampaolesi’s line is characteristic
Disc Focal or diffuse RNFL defect Usually diffuse RNFL defect
depending on early or late
presentation
Severity of glaucoma Less More
Response to therapy Good Poor
Surgery May be required Often required
Q.4. Cause of zonular weakness in PXF. • A

ccumulation of PXF material at the
Ans. The exact cause is not known, however, the origin of the zonules on pre-equatorial
possible mechanisms include following:2 regions of the lens disrupts zonular
• PXF material directly induce zonular architecture.
damage
Glaucoma 207
Table 3 Differential diagnosis

Parameters Pigment dispersion Pseudoexfoliation
Demographics 30–50 years 60 years
Men Men and women
Related to myopia Related to aortic aneurysms (abnormal basement
White race membrane)
Scandinavian countries
Pathomechanism Posterior bowing of the iris Systemic disease of abnormal basement membrane
Constant rubbing of posterior (skin, viscera, eyes)
iris and zonules Secretion of amyloid like material (oxytalon) in AC
Release of pigments Deposit in trabeculum and zonules
Trabecular block Trabecular block
Clinical features Krukenberg’s spindle White powdery deposits on pupillary margin
Deep AC with poterior bowing (pseudoexfoliative material), on lens capsule (Target
of iris (reverse pupillary block) sign) with weak zonules.
Iris atrophy in periphery of Iris Subluxated or dislocated lens can be seen
Pigment deposit on lens Poorly dilating pupil
(Zentmayer’s line) Iris atrophy at edge of pupil margin
Gonioscopy Heavily pigmented angle Marked blotchy pigmentation of trabecular
Queer iris pigmentation meshwork.
Sampaolesi’s line is characteristic
Pseudoexfoliative material
• I ntrinsic differences between normal REFERENCES

and PXF zonules because PXF zonules
are composed of modified forms of 1. Stamper R, Lieberman M, Drake M. Becker-
zonular fibers. Shaffer’s Diagnosis and Therapy of the
Glaucomas, 8th edition. New York, NY: Mosby;
Q.5. How do you differentiate between PXG 2009. pp. 239-65.
and pigment dispersion syndrome 2. Desai MA, Lee RK. The medical and surgical
(PDS)? management of pseudoexfoliation glaucoma.
Ans. See Table 3. Int Ophthalmol Clin. 2008;48(4):95-113.
CHAPTER
4
Retina
LONG CASES
VITREOUS HEMORRHAGE
INTRODUCTION worse in the morning as blood has settled to

the back of the eye, covering the macula.
Vitreous hemorrhage (VH) is defined as the zz Hemorrhage that is more significant can cause
presence of extravasated blood within the space visual fields defect or scotomas.
outlined by the internal limiting membrane of the
retina posteriorly and laterally, the nonpigmented
epithelium of the ciliary body laterally and the
lens zonular fibers and posterior lens capsule Presentation can be unilateral or bilateral. It may
anteriorly.1 The incidence of VH is seven cases per be rapidly progressive. Preceding events such as
100,000, which makes it one of the most common trauma, Valsalva maneuver, recent surgery or
causes of acutely or subacutely decreased vision. recent retinal laser therapy must be recorded.
Such cases are a common reason for surgery and Many a times the patients would give history
a favorite for long case. During work-up, focus suggestive of the cause of VH, e.g. recurrent
should be on identifying the cause of VH. pain and redness s/o uveitis or VF loss s/o RVO/
glaucoma or central scotoma in age-related
HISTORY macular degeneration (ARMD). Scotomas remain
fixed in the field whereas generally floaters move
Chief Complaint with eye movement.1
The symptoms of VH are varied but usually include:
zz Early or mild hemorrhage cases presents with History of Past Illness
as floaters, which patient describes as cobwebs, History of trauma, diabetes, hypertension, tumor
ring shadow, multiple insects moving in front (hemangioma, retinoblastoma, melanoma, retinal
of eye, smoke signals, dark clouds or even a red angioma), valsava maneauver, shaken baby
hue. syndrome, venous occlusion, bleeding disorder,
zz In severe cases, sudden onset painless leukemia, ocular surgery (glaucoma surgery,
unilateral visual loss is the usual complain RD surgery, cataract surgery), laser (panretinal
(MC presentation). Patients often say vision is photocoagulation), vasculitis, chest compression,
Retina 209
pseudotumor cerebri must be asked as it may Lid

provide a clue to the underlying cause.
Usually normal.
Recurrent VH may point towards Eales’
disease, proliferative diabetic retinopathy (PDR),
Conjunctiva
or bleeding diathesis. History from the fellow eye
may also be suggestive of ocular predisposition to Multiple hemorrhages in conjunctiva may be
VH. In cases trauma it is necessary to classify as per there. Conjunctival microvascular abnormality
BETTS, to prognosticate as such cases can often be in sickle cell disease “the comma sign” is
medico legal. pathognomonic but its pathogenesis remains
obscure. In such cases, corkscrew vessels are
Family History seen. Similarly, in heart disease conjunctiva may
Family history of diabetes, hypertension, bleeding reveal vascular tortuosity or in rare cases bulbar
disorder, leukemia, tumor, and vacuities must be telangiectasia.
recorded.
Cornea
Past Surgical History May be large and corneal thinning may be there
A detailed past ocular surgery must be noted. in myopes.
Medical History Sclera

Medical history of diabetes mellitus, systemic Scleral thinning may be there (blue sclera in
hypertension, drug intake, sickle cell disease, collagen vascular disease).
anemia, bleeding diathesis and cerebral stroke
can give a valuable clue even before ocular Anterior Chamber
examination. Keratic precipitates, cell and flare may be present
in the inflammatory diseases. Deep AC is seen in
EXAMINATION
in high myopic eyes. There may be signs of angle
Systemic Examination closure glaucoma (ACG).
Systemic examination is important in a case of
VH to rule out the above-mentioned causes. It is Iris
especially important in cases of vascular occlusion Iridodialysis, neovascularisation of iris (NVI)
since there may be some cardiology or associated or angle (NVA) may be there. This is extremely
intracranial events that need immediate attention important, as it is prognostic and diagnostic.
by a general physician. Similarly, it is not uncom-
mon to discover signs of hematological disorder in Pupil
patients with unexplained VH. It becomes more
Presence of relative afferent pupillary defect
important when the disease or causation cannot
points unequivocally to an underlying retinal
be localized to the eye.
detachment, retinal vascular occlusion, and large
Ocular Examination macular lesion or optic nerve disease.
Visual Acuity Intraocular Pressure

Recording of VA of either eye is necessary. An intraocular pressure (IOP) less than 9 mm Hg
The status of the other eye often guides the or more than 22 mm Hg needs to be investigated
management plan. and explained. Hypotonic globe would suggest
retinal detachment, wound leak, or an open globe
Eyeball injury (occult or obvious). Raised IOP could be due
Usually normal. May be large in case of high to neovascular glaucoma, hemolytic glaucoma,
myopia. Proptosis may be in cases of tumor (axial corticosteroid usage, or tumor invasion under
in cavernous hemangioma). conditions of VH.
Gonioscopy by a posterior vitreous detachment (retrohyaloid

or subhyaloid hemorrhage) are also considered
Neovascularization of angle (NVA) can be seen in VH.1 Blood within Berger’s space settles down
cases of PDR, angle recession or cyclodialysis may and forms a crescent-shaped pool with the
be there in case of blunt trauma. hyaloideo-capsular ligament as its inferior border.
Hemorrhage into the Canal of Petit- also has a
Lens crescent-shaped superior border, which is also
Following points must be noted subluxation, formed by the hyaloido-capsular ligament. Blood
dislocation, zonular dialysis, phacodonesis and in Cloquet’s canal-outlines its inferior border
traumatic cataract presence of all this points and, Blood in the retrohyaloid space—generated
towards trauma as the underlying cause of VH. by a vitreous detachment (retro- or subhyaloid
hemorrhage) can collect as a meniscus at the
Anterior Vitreous inferior vitreoretinal demarcation. A clinically
similar appearance is caused by hemorrhage
Presence of vitreous pigments or Shaffer’s sign into the space between the internal limiting
commonly seen in presence of retinal detachment membrane and the nerve fiber layer (subinternal
(RD) but rarely also in cases of trauma. Presence limiting membrane hemorrhage). In the latter
of RBC’s or hemosiderin pigments can be typically type of hemorrhage, the blood is under tension
there. Importantly, retrolental cells may also be and does not shift with changes in the position
seen, their presence however is not specific for of the patient’s head, as observed in subhyaloid
uveitis. hemorrhage. Subinternal limiting membrane
hemorrhage has been described in penetrating
Fundus ocular injury, Terson’s syndrome, anemia, valsalva
Posterior vitreous detachment (PVD) can cause maneuver-induced retinopathy, shaken baby
VH. If PVD is there scleral, depression is manda syndrome, retinal macroaneurysm, diabetic
tory to rule out a peripheral retinal break. It is retinopathy, and branch retinal vein occlusion.1
important to remember that an acute PVD with In contrast to the above-mentioned types of
VH has an up to 70% incidence of retinal tears, hemorrhage into defined vitreous spaces, bleeding
compared to low incidence in acute PVD without into the vitreous gel (intravitreal hemorrhage)
VH. Other findings to look for includes NVE, shows no characteristic borders and rapidly clots.
NVD, wet ARMD, DR, familial exudative vitreo- Visual acuity with vitreous VH and retained
retinopathy (FEVR), retinopathy of prematuring macular function is primarily determined by the
(ROP), Retinal vasculitis, signs of trauma prolife location and density of the hemorrhage. Only
rative sickle cell retinopathy, venous occlusion, small amounts of blood are necessary to cause
retinal macroaneurysm, choroidal melanoma, a substantial reduction in visual acuity. Around
and angioma. Presence of fresh red hemorrhage 12.5 pL of diffuse blood in a 5 mL aphakic or 10 pL
mixed with old bleed is suggestive of recurrent of diffuse blood in a 4 mL phakic vitreous cavity
bleeding, and indicates retinal neovasculopathy. may decrease the visual acuity to hand motions.1
In old hemorrhage, one should look for presence
of fibrotic clots. Presence of diffuse whitish opa Natural Course of Vitreous Hemorrhage
city should indicate possibility of other causes of
There are certain unique biochemical features
media opacity, other than blood. Specific fundus
of the vitreous in catabolism of bloodlike—rapid
features of individual diseases may be there
clot formation, slow lysis of fibrin, persistence of
(see viva section).
intact red blood cells for months and lack of early
polymorphonuclear response, extracellular lysis
Different Forms of Vitreous Hemorrhage of red blood cells, spontaneous clearance is more
Hemorrhage into the Berger’s space (retrolental common in diseases, which have no recurrent
space of Erggelet) and the Canal of Petit (except bleeding, syneresis of vitreous gel, and in elderly
for the Canal of Hannover) or in a space generated and aphakic patients. The VH does not clear as
Retina 211
spontaneously in patients with diabetic retinopa- Differential Diagnosis

thy, longstanding VH, with an ochre membrane
(the accumulated red cells and red cell debris These are causes of media opacity and true for old
suspended in and mixed with vitreous collagen). white VH:
Complications that can occur must be looked for zz Vitritis
such as hemosiderosis bulbi, retinal detachment, zz Amyloidosis
glial and fibrovascular proliferation and glaucoma zz Lymphoma
(ghost cell/hemolytic/hemosiderotic), hyphema, zz Asteroid hyalosis
and staining of ocular structures. zz Vitreous degeneration
zz Leukemic vitreous infiltration.
Fellow Eye
Evaluation of the fellow eye can often help in INVESTIGATIONS
diagnosis of VH. Common conditions of the fellow
eye that help speculate cause of VH in the affected Systemic
eye include diabetic retinopathy, hypertensive Based on the disorder suspected.
retinopathy, ARMD, myopic changes, lattice,
vitreous condensation, retinal white without
pressure (WWOP) changes, retinal tear, retinal Lab Tests
hole, giant retinal tear, peripheral retina breaks/
retinal detachment, retinal vasculitis (including Blood sugar, complete hemogram, coagulation
Eales’ disease), ocular ischemic syndrome, venous profile, erythrocyte sedimentation rate (ESR),
occlusions, familial exudative vitreoretinopathy C-reactive (CRP), peripheral blood smear,
(FEVR), and retinoschisis. We should also look for enzyme-linked immunosorbent assay (ELISA),
any retinal detachment in fellow eye. venereal disease research laboratory (VDRL),
Mantoux carotid Doppler scan, ECG, chest X-ray,
Fundus Findings in Eales’ Disease echocardiography.
Retinal phlebitis—characterized by mid-
peripheral venous dilation, perivascular exudates FFA/ICG
along the peripheral veins, and superficial retinal
hemorrhages. Vascular sheathing ranges from thin Once media is partially or totally clear angiography
white lines limiting the blood column on both can be done for deciding on management or
sides to segmental heavy exudative sheathing. diagnoses.
Peripheral nonperfusion—Fine solid white
lines retaining configuration of normal retinal
vasculature as a remnant of obliterated large
vessels, sharply demarcated junction between
the anterior peripheral nonperfusion and the
posterior perfused retina, vascular abnormalities
at the junction between the perfused and non-
perfused zones such as microaneurysm, veno-
venous shunts, venous beading, and occasionally
hard exudates and cotton-wool spots can be
seen. Neovascularization: NVE, NVD, recurrent
VH, proliferative changes, tractional retinal
detachment (TRD). The macula is usually not
involved, but when it does, it is termed as central
Eales’ disease. In this variant, all mid-peripheral
lesions appear in the posterior pole and cause loss
of vision in the early stage of the disease (Fig. 1). Fig. 1: Central Eales disease. Note sheathing of
Sea fan new vessels may be there.1,2 the major vascular arcade with macular edema
USG X-ray, CT and MRI

Extremely important in management of media Occasionally, computed tomography (CT) and
haze related cases. Multiple scans must be taken magnetic resonance (MR) imaging are performed
including transverse and longitudinal just like on patients with VH, for example, in Terson’s
in a screening USG. If the whole or a part of syndrome to evaluate for intracranial hemorrhage.
the underlying retina is obscured due to VH, Computed tomography does not easily
ultrasound B scans with corresponding A-scan differentiate hemorrhage from the surrounding
is mandatory to detect any associated retinal vitreous. May also be useful in cases of trauma.
detachment/mass lesion. During the scan,
emphasis should be on three sites: the vitreous VER
cavity, vitreoretinal interface and retinochoroidal
It may be useful in cases where visual prognoses
layer. With ultrasound, it is possible to differentiate
is doubtful.
between fresh and clotted hemorrhage. Unclotted
hemorrhage with no cellular clumps may not be
visible ultrasonically. Asteroid hyalosis is one MANAGEMENT
condition that may appear similar to clotted VH As discussed earlier, first part is determining the
on B-scan. It allows determination of the location possible cause of VH, as management involves
and density of the VH, the location and extent systemic consultations accordingly. A case of
of traction membranes and retinal detachment uveitis may need prior treatment with steroids;
(Fig. 2), and the vitreoretinal relationship, all of whereas a case of RD may need urgent surgery.
which may help to predict the visual outcome After establishing the etiology, management of
after vitreous surgery. Status of PVD and its VH should be individualized. Management is done
differentiation from RD is necessary. Rarely retinal in form of observation, laser photocoagulation,
breaks may also be picked up. cryotherapy and pars plana vitrectomy.
Some patients may need repeated retinal The choice depends on several factors. It
evaluation and serial ultrasonography in 7–10 days include patient’s age, the duration of disease, visual
to reaffirm the cause and again rule out any retinal acuity, intraocular pressure, presence or absence of
detachment/retinal break that would warrant an neovascularization of iris, amount of hemorrhage,
early surgery. Typically, patients with acute PVD retinal status, adequacy of photocoagulation if
need repeat USG. done before the onset of hemorrhage, lens status
(phakic or aphakic/pseudophakic) and presence
or absence of posterior vitreous detachment
(PVD).1
Principles of Management
Observation: Fresh VH often clears in days to
weeks to allow evaluation of retina. Serial USGs are
of paramount importance in such cases.
In case of retina attached—In unknown etiology:
In these patients, the patient is asked to rest with
the head in an elevated position and we should
reevaluate after 3–7 days to ascertain the possible
source of hemorrhage. Oral ascorbic acid (Vitamin
C) may be given for faster clearance (though not
Fig. 2: USG showing VH with TRD in a case of PDR. The clinically proven), as there is more liquefaction
membrane persisted on low gain and had poor after and loss of gel structure in eyes with ascorbic acid.
movements. Ruling out RD is necessary as it affects In known etiology—In these patients re-evaluation
visual prognoses severely is done after 3-4 weeks. This group includes
Retina 213
post laser or postvitrectomy recurrent VHs, VH zz Advanced proliferative retinopathy where

in Tersons’ syndrome or after acute PVD and the VH does not resolve in 6–8 weeks after
hemorrhage associated with bleeding diathesis. adequate laser therapy
zz VH with retinal detachment
In retinal detachment : Early surgery is zz VH with giant retinal tear
recommended in VH associated with retinal zz Tractional retinal detachment involving the
detachment. In eyes with attached macula, one
macula
may wait for some days for PVD to occur, as this zz Combined tractional and rhegmatogenous
will enhance the technical ease and improve the
retinal detachments
outcomes of surgery. This includes penetrating zz Severe progressive fibrovascular proliferation
trauma without retained intraocular foreign zz Anterior segment neovascularization with
body (and not associated with infection), fresh
posterior segment opacities
retinal detachment with VH and no PVD, Eales’ zz Dense premacular hemorrhage
disease without PVD and rhegmatogenous zz Ghost cell glaucoma
retinal detachment, VH in closed globe injury zz Macula edema associated with premacular
without retinal detachment. In macula-off retinal
traction
detachment with VH, we should do immediate zz Anterior hyaloid fibrovascular proliferations
surgery. zz Fibrinoid syndrome with associated retinal
Laser photocoagulation: Laser photocoagulation detachment
in proliferative vasculopathies should start as zz VH with retained intraocular foreign body
soon as any part of retina is visible. In some cases, zz VH due to AMD and idiopathic polypoidal
one may start laser therapy using an indirect choroidal vasculopathy (IPCV).
ophthalmoscope delivery system in dense VH
and later on one can shift to slit lamp delivery. Management of Specific Conditions
After partial clearing of hemorrhage, one may
Proliferative Diabetic Retinopathy
visualize a retinal break or avulsed vessel that
can be treated with barrage laser. In media (See Long Case for PDR)
haze due to VH, cataract, corneal edema (as in Eales’ disease: Eales’ disease usually presents with
neovascular glaucoma) or poorly dilating pupil, VH at the time of onset. Sixty-two percent patients
transconjunctival cryopexy mode of laser can be have VH at the time of initial presentation. It can
used for pan retinal photocoagulation or treatment occur due to severe retinal vasculitis or in the
of retinal breaks. Role of preoperative anti-VEGF proliferative stage due to new vessels and traction.
has been discussed in the chapter on PDR. It can be treated with retinal photocoagulation
initially. Early vitrectomy has been advocated,
Anterior retinal cryotherapy (ARC): Has limited with 87% eyes showing improvement in visual
use. Generally not applied. acuity.
Vitrectomy: Early vitrectomy is indicated in Macroaneurysm: VH develops in as many as 30%
situations where the underlying pathology is likely of macroaneurysm. Mostly occurs in women over
to progress fast if left untreated.1 Surgery can be 60 years of age with systemic hypertension. These
delayed in eyes with well-lasered proliferative may resolve spontaneously or may require laser
retinopathy with retina attached. Vitrectomy treatment.
can be deferred till good PVD occurs in eyes
Uveitis: Pars planitis can result in retinal neo
with Tersons’ syndrome, closed globe-injuries,
vascularization and cause VH. Sarcoidosis,
postcataract surgery VH (if not due to peribulbar
Behçets’ syndrome and toxoplasmosis may cause
anesthesia related globe perforation), VH in
VH due to retinal neovascularization whereas
bleeding diathesis, etc.
ocular histoplasmosis syndrome causes VH from
choroidal neovascularization.
Indications of Virectomy IRVAN (idiopathic retinal vasculitis, aneurysms
zz Severe nonclearing VH over 2–3 months. and neuroretinitis) can also present with VH.
Retinal vascular anomalies and tumors: Caver VH in a child), retinoblastoma and leukemia. In
nous hemangioma of the retina and optic disc, infants, disseminated intravascular coagulopathy
capillary hemangiomatosis or juxtapapillary or Terson’s syndrome are causes of VH. Pediatric
vascular hamartomas of the retina, and congenital retinal diseases that can present with VH include
arteriovenous anastomoses can lead to VH. familial exudative vitreoretinopathy retinoschisis,
Parafoveal telangiectasia and Coats’ disease cause high myopia with retinal tears/detachment,
VH rarely. A choroidal melanoma does not cause retinopathy of prematurity, toxocariasis. Early
VH until it reaches a considerable size. surgery is advocated in these eyes to avoid
Sickle cell disease and leukemia: With peripheral amblyopia and anisometropia.
scatter photocoagulation reduces the risk of
VH in Sickle cell hemoglobinopathies. Retinal VIVA QUESTIONS
neovascularization may also develop in chronic
cases of chronic myelocytic leukemia and can Q.1. Describe causes of VH on the basis of age
cause VH. of the patient
Posterior vitreous detachment: Spontaneous VH Ans. The age of the patient can provide clues
can occur with posterior vitreous detachment. An about etiology of VH. For example:
early diagnosis is crucial because a retinal tear is a • Newborn babies—trauma after sponta
common cause of VH. A detailed peripheral retinal neous vaginal delivery (but not after
evaluation with scleral depression is mandatory cesarean delivery), shaken baby syn
to screen for any retinal tears, obscured by VH. drome and retinopathy of prematurity.
B-Scan (sometimes dynamic USG) and A-scan • Young boys—X-linked retinoschisis.
may be helpful to detect the retinal tear and the Children—trauma, retinoblastoma,
traction site. Surgery is indicated in case of retinal leukemia and other coagulopathies.
detachment. • Young healthy adults—Eales’ disease in
Age-related macular degeneration: VH secondary the Indian subcontinent is an important
to age-related macular degeneration results from cause of VH. Retinals tear with or without
a subretinal bleeding due to choroidal neovas associated retinal detachment.
cularization. Ultrasonography shows a highly • In elderly—choroidal neovascular
echogenic subretinal mass temporal to the optic membrane (CNVM) secondary to age-
disc typically, without any choroidal shadowing. related macular degeneration (AMD),
In these patients, the hemorrhage usually resolves proliferative retinopathy associated
spontaneously. If surgery is planned, antivascular with diabetes or retinal vein occlusion,
endothelial growth factor (VEGF) may be given at and rarely due to retinal tears, posterior
the end of surgery. vitreous detachment, melanoma, IPCV,
or systemic anticoagulants.
Miscellaneous: VH may occur in eyes undergoing
intracapsular or extracapsular cataract extraction. Q.2. What are the causes of VH?
Most VHs are mild and clear spontaneously, the Ans. See Table 1. Depending upon the source,
possibility of needle perforation due to local following categories can be seen.
anesthesia should be excluded. VH in Terson’s A. Bleeding from abnormal vessels
syndrome occurs due break through bleeding
i. Retinal vascular disorders that cause
from the internal limiting membrane of the retina
retinal ischemia (due to VEGF, FGF,
and extends into the vitreous cavity. In a Valsalva
IGF–NVD, NVE)
maneuver, increased intravascular pressure causes
• Proliferative diabetic retinopathy
VH due to retinal vein rupture. VH can also occur
• Ischemic retinal vein occlusion
in warfarin or aspirin users. (RVO), more commonly branch
VH in children: One of the most common causes retinal vein occlusion (BRVO)
of VH in children is trauma. Other causes are • Eales’ vasculitis
shaken-baby syndrome (in otherwise unexplained • FEVR
Retina 215
Table 1 Causes of vitreous hemorrhage

Ocular causes Coat’s disease, retinal branch artery malformation, retinopathy of prematurity, ocular
Vascular ischemic syndrome, branch retinal artery occlusion, central retinal artery occlusion,
choroidal vascular aneurysm, retinal vein rupture, retinal neovascularization after
retinectomy, hypertensive uveitis, persistent hyaloid artery, venous stasis retinopathy,
arteriovenous communications of the retina
Inflammatory Retinal vasculitis, Behçet’s disease, sarcoid posterior uveitis, multiple sclerosis with
retinal vasculitis, pars planitis, syphilitic retinitis, dermatomyositis. Systemic lupus
erythematosus, toxocara
Iatrogenic Retinal laser photocoagulation, after scleral buckling, molteno implant surgery,
trabeculectomy, ocular perforation, during peribulbar injection, secondary IOL,
cataract wound neovascularization, penetrating keratoplasty
Tumor Retinoblastoma, cavernous hemangioma of the optic disc, combined retinal-retinal
pigment epithelial hamartoma, vasoproliferative tumors, retinal angioma, retinal
astrocytic hamartoma, choroidal malignant melanoma
Others Senile bullous retinoschisis, juvenile retinoschisis, tearing of retinal pigment epithelium,
Talc retinopathy, Retinitis pigmentosa, extracorporeal membrane oxygenation, trauma
Indirect causes Pseudotumor cerebri, Valsalva retinopathy, chest compression, newborn after vaginal
delivery
Blood disorders Thrombocytopenia, idiopathic thrombocytopenic purpura, hemophilia, pernicious
anemia, disseminated intravascular coagulation disorder, von-Willebrand’s syndrome,
Protein C deficiency, anticoagulant therapy
• P
roliferative sickle cell retino- • P
eripheral exudative hemorrhagic
pathy chorioretinopathy (PEHCR).
• Hematological disorders
ii. Retinal vascular disorders that is not Q.3. What is the role of fellow eye?
associated with retinal ischemia- Ans. See chapter.
• Retinal artery macroaneurysm Q.4. USG differentiation of PVR/RD.
• Retinal angioma Ans. For RD: Persistence at low gain, poor after
• Severe early vasculitis in absence movements, attached to disc, quantitative
of ischemia
method.
B. Rupture of a normal retinal vessel
• Posterior vitreous detachment Q.5. Reasons for early surgery.
• Blunt trauma Ans. Unlasered PDR, RD, IOFB, one-eyed
• Terson syndrome patient, other eye lost to VH, Zone 3 injuries.
• Valsava retinopathy
• Hematological disorder (anemia, REFERENCES
leukemia, coagulation disorder).
1. Spraul CW, Grossniklaus HE. Vitreous
C. Breakthrough bleeding Hemorrhage. Surv Ophthalmol. 1997;42(1):3-
• Choroidal neovascular membrane 39. Review.
(CNVM) 2. Goff MJ, McDonald HR, Johnson RN, Ai E,
• Choroidal melanoma Jumper JM, Fu AD. Causes and treatment of
• I diopathic polypoidal choroidal vitreous hemorrhage. Compr Ophthalmol
vasculopathy (IPCV) Update. 2006;7(3):97-111.
CENTRAL RETINAL VEIN OCCLUSION

Ashish Markan, Esha Agarwal, Brijesh Takkar
INTRODUCTION Patients with partially recovered CRVO may

give history of visual field loss or constriction.
Retinal vein occlusion (RVO) is an obstruction Floaters can rarely occur due to accompanying
of the retinal venous system may involve the vitreous hemorrhage.
central, hemicentral or branch retinal vein. The Decrease in contrast sensitivity, micropsia,
most common etiological factor is compression macropsia, metmorphopsia, scotoma can also be
by adjacent atherosclerotic retinal arteries there due to associated macular edema. Vision
travelling through the same adventitial sheath. recovery is dependent on onset and duration of
Other possible causes are external compression or occlusion.
inflammation of the vein wall. Central retinal vein
occlusion (CRVO) may result from thrombosis
of the central retinal vein when the vein passes
through the lamina cribrosa. The prevalence of Ocular disorders predisposing to crowding/
CRVO is reported to be around <0.1% to 0.4%.1,2 compression at the level of optic disc, and
CRVO is usually a unilateral disease, the risk of systemic disorders predisposing to thrombo-
developing any type of vascular occlusion in fellow embolic disease or disease of the vascular wall
eye is approximately 1% per year, and about 7% are the most commonly identified risk factors.
of persons with CRVO may develop CRVO in the For example, there may be history of open angle
fellow eye within 5 years of onset in the first eye.3,4 glaucoma or angle closure glaucoma, ischemic
optic neuropathy, pseudotumor cerebri, tilted
HISTORY optic nerve head, optic nerve head drusen,
hypermetropia, hypertension, cardiovascular
The CRVO occurs predominantly in elderly diseases, carotid insufficiency, diabetes, bleeding/
population (>65 years), and affects male and thrombotic disorder, leukemia, multiple myeloma,
female equally. These patients may often have sickle cell disease, SLE, HIV, herpes zoster,
ocular or systemic risk factors. syphilis, sarcoidosis. It may also be presented after
retrobulbar block, dehydration and pregnancy.
Chief Complaints There is small risk of past CRVO in the fellow
zz Sudden painless loss of vision eye, ~7% in 5 years.
zz Rarely pain, redness due to neovascular
glaucoma (NVG) or accom p anying high Family History
intraocular pressure (IOP). Family history of risk factors may be there.
History of Present Illness Past Surgical History

Patients usually present with sudden painless
History of ocular surgery, cardiovascular surgery
loss of vision in one eye. Some patients may also
maybe there.
present with gradual decline of vision in cases
of less severe occlusion. Marked deterioration
of visual acuity especially on waking in morning Personal History
can be seen in ischemic CRVO. Patients with non History of smoking, alcohol intake, tobacco use.
ischemic CRVO may have no symptoms and it
may be detected as an incidental finding on a
Drug History
routine ophthalmic examination. It is not rare to
find some of these patients complain of episodes History of intake of oral contraceptive, diuretics,
of amaurosis fugax before the constant blur. and hepatitis-B vaccine must be enquired.
Retina 217
EXAMINATION of final visual outcome. Generally, visual acuity

better than 20/200 is believed to be a sign of good
General examination/specific systemic examina
final prognoses.
tion should be aimed at ruling out the systemic risk
External and anterior segment evaluation can
factors.
reveal signs of systemic risk factors.
Ocular Examination Eyeball: Proptosis in case of tumor (abaxial in
Visual acuity: Visual acuity at the time of presenta- sarcoid)
tion is variable (ischemic vs nonischemic Lid: Signs of other systemic risk factors may be
Table 1), and is an important prognostic indicator present.
Table 1 Types of central retinal vein occlusion (CRVO)

Parameters Nonischemic CRVO Ischemic CRVO
Incidence 80% 20%
Age Young adults and past middle age Past middle age
Symptoms— Vague blurring of vision Marked deterioration of vision especially on waking
vision Normal/>6/60 in morning <6/60
Pupil Normal RAPD present >0.7 log units (on neutral density filter)
Site of occlusion Further back in retrolaminar At or near retrolaminar region
region
Early stages Mild to moderate dilatation of all Marked tortuosity and engorgement of retinal vein
branches of central retinal vein
Retinal Mild to moderate, more in Extensive hemorrhages involving
hemorrhages periphery Periphery and posterior pole
Cotton wool spots Rare Common
Optic disc Hyperemic and may be Marked optic disc edema
edematous
Macula Normal or may show edema Gross hemorrhages and edema
Late Veins—mild to moderately Veins—mild to moderately engorged
ophthalmoscopic engorged Sheathing—frequently seen
finding Sheathing +/– Hemorrhages—none or few
No neovascularization Retina has—aneurysms, neovascularization,
Macula—normal/CME preretinal/vitreous hemorrhages
Optic disc—pale
Macula—degenerative/pigmentary disturbances
FFA <10 disc area of capillary non- >10 disc areas of capillary nonperfusion
perfusion
ERG Minimal or no change Marked reduction of b-wave amplitude <60%
Goldmann No or minimal defects Marked peripheral visual field defects
perimetry
Complications Macular edema, macular Macular edema, macular degeneration, preretinal
degeneration hemorrhages, vitreous hemorrhage, NVE, NVD, NVG
Prognosis Good Poor
Abbreviations: ERG, electroretinogram; FFA, fundus fluorescein angiography; NVE, neovascularization
elsewhere; NVD, neovascularization of the disk; NVG, neovascular glaucoma
Conjunctiva: Multiple hemorrhages in conjunc

tiva may be seen (bleeding disorders).
Cornea: Peripheral ulcerative keratitis (PUK) in
case of SLE, PAN.
Sclera: Nodular scleritis.
Anterior chamber: It may be shallow in patients
with angle closure glaucoma or short axial
length. Keratic precipitates, cells and flare may be
present in the inflammatory diseases (vasculitis,
sarcoidosis, HIV). Flair may be noted in patients
with NVI and presence of hyphema is uncommon.
Gonioscopy: Undilated gonioscopy is essential
to determine the presence of NVA or evidence Fig. 1: Fundus picture of ischemic CRVO with macular
of angle closure. NVA may be present without edema. Four quadrant retinal hemorrhages can be
neovascularization of the iris (NVI) in 12% of eyes.5 seen along with soft exudates and retinal edema
Iris: NVI or NVA may be there. Pupillary margin

should be carefully examined for the presence
of NVI. As later discussed, it is an important sign
deciding on management.
Pupil: Presence of relative afferent pupillary
defect is an ominous sign. It is important to look
for RAPD before pupil dilation whenever one is
suspecting vascular occlusion.
Lens: Generally normal or age-related nuclear
sclerosis. Complicated cataract can be seen in
inflammatory conditions.
IOP: An IOP more than 22 mm Hg needs to be
investigated and explained. Raised IOP could be
due to neovascular glaucoma, underlying open Fig. 2: Fundus picture of CRVO
angle glaucoma. ACG attack around the time of showing venous tortuosity
CRVO is not uncommon. It may be both causative
as well as the effect of CRVO.
Posterior segment (Figs 1 to 3): The typical
clinical constellation in CRVO includes retinal
hemorrhages (both superficial flame shaped and
deep blot type) in all four quadrants with dilated,
tortuous retinal veins (classic “blood and thunder
appearance”). Optic nerve head swelling, cotton
wool spots, splinter hemorrhages, and macular
edema are present to varying degrees. Macular
edema may be accompanied accumulation of sub-
retinal fluid. In severe cases, the retinal thickening
may be 4–5 times the normal. With time, retinal
hemorrhages may decrease or resolve completely
with secondary retinal pigment epithelium Fig. 3: Fundus picture of old CRVO with macular
alteration. An epiretinal membrane may form. edema. Collaterals have developed over the optic disc
Retina 219
Optociliary shunt vessels (disc collaterals) can Systemic Work-up

develop on optic nerve head and are very impor
It is generally not indicated in elderly patient with
tant clue to an old RVO. With time disc pallor
known systemic vascular risk factor for CRVO.
ensues. NVD or NVE may develop. Careful 90 However, routine blood pressure, fasting blood
D examination should be done to differentiate glucose and lipid profile should be checked.
disc collaterals from NVD. The vessels that A cardiac consultation should be done when
comprise NVD are typically of smaller caliber possible.
than optociliary shunt vessels and branch into a Younger patients, patients with bilateral simul
vascular network. Fibrovascular proliferation from taneous vascular occlusion, prior occlusion in the
NVD or NVE may result in vitreous hemorrhage or fellow eye, prior systemic thrombotic disease,
traction retinal detachment. family history of thrombosis require detail evalua
Gross signs of vascular sclerosis may be present tion for hypercoagulable condition as these
as well as clues to systemic disorders discussed persons may be at risk for future, nonocular
before. One should always look for presence of thrombotic events. These investigation include:
atherosclerosis in the retinal arteries. zz Complete hemogram with peripheral smear
Fellow eye should be examined carefully to look
zz Erythrocyte sedimentation rate
for signs of hypertensive retinopathy, diabetic
zz Plasma homocysteine level
retinopathy, vasculitis or signs of vascular occlu
zz Chest X-ray—to rule out TB, sarcoidosis and
left ventricular hypertrophy.
sion. Gonioscopy should be performed to rule
zz C-reactive protein
out occludable angle (vascular occlusion may be
zz Thrombophilia screen—PT, TT, activated
secondary to angle closure glaucoma).
partial thromboplastin time (aPTT), protein
Complications associated with CRVO: A careful C, protein S, activated protein C resistance,
examination must be done to rule out any of these factor V Leiden mutation, lupus anticoagulant,
complications of CRVO. anticardiolipin antibody.
zz Macular edema zz Autoantibodies—ANA, ANCA, anti-DNA
zz Macular ischemia antibody, rheumatoid factor.
zz Neovascular glaucoma zz Serum angiotensin converting enzyme
zz Vitreous hemorrhage zz Treponemal serology
zz Tractional retinal detachment zz Carotid Doppler scan.
zz Optic atrophy.
Ocular Investigation
DIFFERENTIAL DIAGNOSIS zz Fundus fluorescein angiography (FFA):
Fluorescein angiography in CRVO (Fig. 4)
A case of CRVO may have to be differentiated from
following:
zz Ocular ischemic syndrome
zz Diabetic retinopathy
zz Papilledema
zz Radiation retinopathy
zz Retinopathy due to anemia
zz CRAO with CRVO
zz Venous stasis retinopathy.
INVESTIGATIONS
One important part of work-up includes identifying
appropriate risk factors, ocular or systemic. Ocular Fig. 4: Fluorescein angiography picture of CRVO
risk factors enlisted before should be ruled out as showing collateral vessels, severe retinal ischemia and
appropriate. microaneurysms
shows marked delay in arteriovenous transit central visual acuity by minimizing macular
time, blocked fluorescence due to retinal edema, reducing the risk of bleeding into the
hemorrhages, vessel wall staining, areas of vitreous cavity by producing regression of retinal
nonperfusion, collaterals, NVD, NVE and neovascularization, and preventing neovascular
macular edema. Blocked fluorescence of glaucoma. Previously there has been lot of interest
the underlying retinal circulation occur if in relieving the obstruction or bypassing it, but
extensive intraretinal hemorrhages are present none of therapies have proven to be of benefit
especially in the early part of the disease (discussed later).
and therefore FFA may not reveal useful
information. So, best to wait for resolution of Macular Edema
hemorrhages.
FFA is indicated to rule out macular ischemia, Before the advent of intravitreal pharmacotherapy,
to determine the type of CRVO (ischemic vs. observation was the standard of treatment
nonischemic), and to detect NVD and NVE. for macular edema associated with CRVO as
CRVO is said to be nonischemic if capillary recommended by CVOS. In CVOS group M, no
nonperfusion is less than 10 disc areas and significant improvement in visual acuity was
ischemic if capillary nonperfusion is more seen with grid laser as compared to untreated
than 10 disc areas (definition of CVOS). group, though macular edema was decreased
zz Optical coherence tomography (OCT): Optical angiographically. Hence macular grid is not
coherence tomography is useful in the routinely recommended.
assessment of macular edema, and particularly Intravitreal steroids by reducing vascular
in monitoring its course, especially with permeability and inhibiting the expression of
treatment of the edema. It can readily detect the VEGF gene and the metabolic pathway of
cystic spaces, retinal thickening and serous VEGF plays an important role in the treatment
retinal detachments, all of which are rather of macular edema due to CRVO (SCORE trial,
frequent in CRVO. In long-standing cases, GENEVA trial). Ozurdex (sustained release
helps to detect ERM and VMA. intravitreal dexamethasone delivery system)
zz Ultrasonography: If the whole or a part of is also now FDA approved for treatment of
the underlying retina is obscured due to macular edema secondary to CRVO, but it has
vitreous hemorrhage, ultrasound B-scan with complications like cataract and increased IOP.
corresponding A-scan is mandatory to detect Intravitreal antivascular endothelial growth factor
any associated retinal detachment/mass (VEGF) agents are currently the first-line therapy
lesion. Also useful in detecting hypermetropia for macular edema. Various trials have shown
and disc drusens. their efficacy Ranibizumab in CRUISE trial, VEGF
zz Electroretinogram (ERG): This is an objec trap (Aflibercept) in Galileo and Copernicus, and
tive functional test, very useful in the they have now replaced observation established
differentiation of ischemic from nonischemic by CVOS as the standard of care for the treatment
CRVO. In ischemic CRVO, there is reduced b of macular edema associated with CRVO. Anti-
wave amplitude (<60% of normal), reduced b: VEGF drugs are also FDA approved for treatment
a ratio and prolonged b-wave implicit time on of macular edema due to CRVO. A common dosing
the electroretinogram. would be 3 or 6 monthly injections of ranibizumab
zz Perimetry: Visual field (VF) plotting with followed by as required dosage as the shunt vessels
a Goldmann perimeter, helps in the develop and the RVO relieves itself with time,
differentiation of ischemic (defective V4e the need for injections decreases. It is less needed
target) from nonischemic CRVO. in nonischemic CRVO.
MANAGEMENT Ocular Neovascularization

Goals of treatment in CRVO are to identify and Panretinal photocoagulation (PRP) should be
treat predisposing medical condition, to maintain promptly delivered after the development of
Retina 221
NVI/NVA to prevent secondary complications. of the scleral outlet. Some studies have shown
CVOS did not recommend prophylactic PRP in improvement in visual acuity but its use has
ischemic CRVO. PRP in patient without NVI has been abandoned owing to significant risks.
the risk of making future NVI refractory and is not
indicated. Laser should be delivered as anterior as
possible and supplementary cryo may be added as VIVA QUESTIONS
per need.
Prophylactic PRP can how ever be considered Q.1. What are the various risk factors for
in cases of ischemic CRVO where follow-up is not CRVO?
possible in high risk cases. Ans. Refer text.
Anti-VEGF agents results in rapid regression Q.2. What is the pathogenesis of CRVO?
of neovascularization, but these should be used as Ans. • The site of occlusion in CRVO is at or just
temporizing adjunctive measure with subsequent proximal to the lamina cribrosa.
PRP as definitive treatment. • T he central retinal artery and vein
are aligned parallel to each other in a
Role of Vitrectomy common tissue sheath within the retro
laminar portion of the optic nerve and
It is indicated in cases of nonresolving vitreous
they are naturally compressed as they
hemorrhage or tractional retinal detachment
pass through rigid sieve like openings
secondary to retinal neovascularization.
in the lamina cribrosa but they typically
Pars plana vitrectomy with ILM peeling has also
give off branching collateral vessels
been investigated for macular edema secondary to
just before piercing the lamina. These
CRVO and has shown variable results.
vessels may be subject to compression
from increase in intraocular pressure
Other Modalities which causes posterior bowing of lamina
zz Role of systemic anticoagulants in manage cribrosa due to mechanical stretch or
ment of CRVO is unclear, though it does not occlusion of central retinal vein can be
alter the natural course of CRVO it may help to due to compression by an atherosclerotic
prevent nonocular thrombotic events. central retinal artery or it can be primary
zz Role of oral pentoxifylline (vasodilator)/ due to inflammation of the central
hemodilution in management of CRVO is still retinal vein. Hemodynamic alterations
controversial. may lead to thrombus formation in the
zz Recombinant tissue plasminogen activator central retinal vein by Virchow’s triad
(r-tPA) has been administered by several (diminished blood flow, increased blood
routes systemic, intravitreal and by endo viscosity, altered lumen wall).
vascular cannulation of retinal vessels for • O cclusion of both the retrolaminar
treatment of CRVO and has shown variable central retinal artery and central retinal
results. vein posterior to lamina cribrosa and
zz Chorioretinal venous anastomosis between prior to the branching of collateral
nasal branch retinal vein and the choroidal channels from the main trunk is required
circulation have been created using Nd: YAG to produce ischemic CRVO while non-
laser in nonischemic CRVO. It may allow ischemic CRVO is due to occlusion of
transretinal retrograde flow of the venous the central retinal vein at a site further
blood from the eye and may prevent retinal posterior, allowing normal collateral
ischemia. Studies have shown limited visual channels to provide alternative routes of
recovery even after successful anastomosis venous drainage.
due to thrombosis of the treated retinal vein. • Resistance to venous flow, blood stagna
zz Radial optic neurotomy involves transvitreal tion and ischemia stimulates production
incision of the nasal scleral ring to release of VEGF resulting in neovascularization
pressure on the central retinal vein at the level and macular edema.
Q.3. What are the types of CRVO and how to Interpretations: Delaying treatment with
differentiate between them? PRP until the development of NV resulted in
Ans. Refer to Table 1. significant regression of neovascularization
Q.4. What is the risk of nonischemic CRVO and no additional risk of neovascular
converting to ischemic CRVO? glaucoma compared to patients treated
Ans. About 1/3rd cases of nonischemic CRVO with prophylactic PRP.
convert to ischemic CRVO over a period of The study also suggested a follow-up
one year. schedule, which basically advised regular
monthly follow-up for first 6 months and
Q.5. What is the common site of neovascular then tapered follow-up. However, in the
ization in CRVO? day of anti-VEGF treatment, the discussion
Ans. Following are the sites: is rather arbitrary.
• Iris
• NVI develops in about 50% of eyes, usu Q.9. What are the indications for FFA in
ally in 2–4 months (100 days glaucoma). CRVO?
• NVG develops in 1/3rd of cases with NVI. Ans. Refer text.
• Retinal neovascularization is seen in 5% Q.10. When is PRP indicated in CRVO?
of cases. Ans. Refer text.
Q.6. What is ischemic index and what is its Q.11. What is the treatment of choice for
importance? macular edema in CRVO?
Non-perfusion area Ans. Refer text.
Ans. Ischemic index6 =
Total area of retina
Q.12. What are the causes for CRVO of the
An ischemic index of 50% corresponding young?
to about 10 disc areas of retinal capillary Ans. Refer to risk factors and work-up.
nonperfusion was considered the thre
shold for a significant risk of neovascular Q.13. What are the risk factors for development
complications. of NVI in CRVO cases?
Ans. Risk factors of NVI includes:
Q.7. When will you call anterior segment neo • > 10 DD of nonperfusion in the
vascularization significant? posterior pole (greater risk with greater
Ans. When there is NVI of more than 2 clock
nonperfusion)
hours or there is presence of NVA.
• RAPD
Q.8. What are the objectives and conclusion of • Decreased visual acuity
CVOS? • ERG: decreased b: a ratio if <1 (normal
Ans. The main objectives of CVOS study are: 2:1)
• To assess whether grid-pattern photoco • Elevated central retinal venous pressure
agulation therapy will reduce loss of • Duration <1 month.
central visual acuity due to macular
edema secondary to CVO. Q.14. What is CRAO with CRVO?
• To determine whether photocoagula Ans. In this case, the retinal hemorrhages are
tion therapy can help prevent iris scattered and less due to the accompanying
neovascularization in eyes with central RAO. These cases are at very high risk of
vein occlusion (CVO) and evidence of developing NVI, some figures as high as
ischemic retina. 80%.
Its conclusion are: Q.15. Why does CRVO cause more of NVI and
• There is no visual benefit to treating BRVO cause more of retinal new vessels?
macular edema from a CRVO with grid Ans. In CRVO, particularly, ischemic variant,
laser photocoagulation. the ischemia is so severe that the retinal
• There is no benefit of treating ischemic tissue may not be able to respond to VEGF
CRVO with early (prophylactic) PRP. load to develop new vessels. Hence, retinal
Retina 223
new vessels are much less frequent than in 2. Klein R, Klein BE, Moss SE, Meuer SM. The
BRVO. epidemiology of retinal vein occlusion: the
Beaver Dam Eye Study. Trans Am Ophthalmol
Q.16. What is the current role of Laser in CRVO Soc. 2000;98:133-41.
macular edema? 3. Hayreh SS, Zimmerman MB, Podhajsky P.
Ans. As discussed above macular laser has no Incidence of various types of retinal vein
or poor role in treating macular edema. occlusion and their recurrence and demo
Recent studies, RELATE study for CRVO, graphic characteristics. Am J Ophthalmol.
are indicating role of peripheral laser for 1994;117(4):429-41.
treating edema refractory to injections. The 4. The Central Vein Occlusion Study Group.
Natural history and clinical management of
earlier the laser done, the more chance of
central retinal vein occlusion. Arch Ophthalmol.
it being effective. With availability of wide
1997;115(4):486-91.
field FA, targeted laser may be attempted to 5. Browning DJ, Scott AQ, Peterson CB,
knock of the VEGF producing CNP areas. Warnock J, Zhang Z. The risk of missing angle
neovascularization by omitting screening
REFERENCES gonioscopy in acute central retinal vein
occlusion. Ophthalmology. 1998;105(5):776-84.
1. Mitchell P, Smith W, Chang A. Prevalence 6. Magargal LE, Donoso LA, Sanborn GE. Retinal
and associations of retinal vein occlusion in ischemia and risk of neovascularization
Australia. The Blue Mountains Eye Study. Arch following central retinal vein obstruction.
Ophthalmol. 1996;114(10):1243-7. Ophthalmology. 1982;89(11):1241-5.
BRANCH RETINAL VEIN OCCLUSION

Pulak Agrawal, Shorya Vardhan Azad
INTRODUCTION to complete loss of vision. In such cases patient

may have vitreous hemorrhage.
Retinal vein occlusion is the second most common zz Lastly, in certain cases patient may entirely be
retinal vascular disease. Amongst vascular asymptomatic and a peripheral BRVO may be
occlusions, branch retinal vein occlusion (BRVO) detected on routine clinical examination.
is commoner (78%). Most patients are in the 6th
decade of life. However, it can occur in younger History of Present Illness
population. In such cases, systemic investigation
becomes imperative for finding the underlying It includes complete documentation of onset
cause. Risk factors for BRVO are: (see Table 1) duration and progress of visual complaints.
BRVO patient is usually given as long case or spotter. Associated ocular and systemic symptoms
(discussed above) should also be enquired. There
HISTORY may be history of similar episodes in the same or
the other eye, for which patient might have sought
Chief Complaint medical attention.
BRVO most commonly present in an elderly
patient with complaints of:
Medical/Treatment History and
zz Sudden, painless diminution of vision/blurred Past History
vision/onset of field defect. It should include following:
zz Patient may also have metamorphopsia, which zz Detailed history of patient’s systemic status
will indicate towards macular edema. like hypertension, diabetes, hyperlipidemia,
zz Occasionally, patient old occult RVO may hypercholesterolemia, cardiovascular disease
present with sudden onset of floaters, leading and any other drug intake.
zz Young people should be specifically asked EXAMINATION

about previous history of hypercoagulability
(bruises elsewhere in body, blood transfusions Systemic Examination
etc.), HIV or other infectious diseases, use of Detailed systemic examination should be carried
OCPs. out (See Table 1). In patients with VH, other eye
zz Personal history should include smoking, may reveal the predisposition to BRVO.
chewing tobacco, and alcohol consumption.
zz Patient may have received some form of laser Ocular Examination
or intravitreal therapy, which need careful
documentation. Visual Acuity
zz Careful documentation of past ocular It is paramount to note the best-corrected vision
disorders and therapies, e.g. for glaucoma/ with correction, as it would decide the need to
retinal vasculitis, must be obtained. treat. In treated cases, it is important, as it would
Table 1 Risk factors for BRVO

Risk factors What to look
Systemic
Age >60 years
Hypertension Blood pressure
Diabetes mellitus RBS, PPBS, HbA1C
Coagulopathies • Serum homocysteine
• Hyperhomocysteinemia • Screening blood test: Activated protein C resistance
• Factor V leiden mutation • Antiphospholipid antibodies (aPL): Anticardiolipin antibodies
• Antiphospholipid antibody syndrome (aCL) Lupus anticoagulant (LA) Anti-beta 2-glycoprotein-1
• Protein C/S deficiency (anti-B2GP1)
• Free protein C/S antigen.
Inflammatory • HLA B51, Pathergy Test
• Behçets • Serum ACE, Chest X-ray, Chest CT
• Sarcoidosis • C-ANCA, Chest X-ray
• Wegener’s
Infections • Mantoux test, Chest X-ray
• TB • VDRL
• Syphilis • ELISA and Western Blot
• Lyme’s • ELISA
• HIV
Chronic renal failure RFT
Others History
• Smoking
• Oral Contraceptive
• Dehydration
Ocular
Glaucoma Intraocular pressure
Short-axial length Axial length
Abbreviations: VDRL, venereal disease research laboratory; ELISA, enzyme-linked-immunosorbent assay;
RBS, random blood sugar; PPBS, post prandial blood sugar; RFT, renal function test
Retina 225
help in monitoring the response to therapy. In zz As most of the patients are hypertensive, signs
addition, initial visual acuity could be a prognostic of hypertensive retinopathy should be noted.
factor for post intervention success. Splashed tomato and blood and thunder
appearance must be kept in mind in cases of
Ocular Adnexa and Globe RVO.
zz Macular assessment on slit lamp bio
Ocular adnexal skin can be examined for signs
microscopy with +90D lens may reveal
of coagulopathy, collagen vascular disorders and
macular edema seen as elevation of retina with
infections. There is no significant examination
loss of foveal reflex. Long standing cases may
regarding ocular alignment or movements. Lids,
have intraretinal hard exudates and cystic type
eyebrows and eyelashes are in their limits for
of edema.
the age. zz Meticulous inspection of vitreoretinal
interface may show area of vitreomacular
Conjunctiva adhesion or an epiretinal membrane in
Patients with glaucoma may have conjunctival recalcitrant cases.
blebs. Conjunctival hemorrhages may be present zz Mostly BRVO occurs in superotemporal
in coagulopathies. quadrant (Fig. 1), as more arteriovenous (AV)
crossings are present in this area. AV crossing
Cornea should be carefully evaluated as it may reveal
the site of occlusion.
Involvement is rare, except in collagen vascular zz In contrast, a nasal BRVO or a peripheral BRVO
disorders. may even go unnoticed. There will be dilated,
tortuous vessels proximal to the occlusion.
Anterior Chamber Intraretinal flame shaped hemorrhages;
It may be shallow if the patient has a preexisting microaneurysms can be present along the
glaucoma or short axial length. AC should be occluded vessel. Cotton wool spots are also
inspected for signs of uveitis. Similarly, gonioscopy seen.
may reveal NVA in selected cases. zz Old cases may have collaterals, telengiectatic
vessels and neovascularization.
Pupil zz Neovascularization can be present on the
disc or at the junction of perfused and
Rarely neovascularization may be present at
pupillary border. Presence of RAPD may indicate
CRVO or HRVO or AION rather than BRVO. Even
major BRVOs will not have RAPD.
Lens
As patients are elderly, there may be cataractous
changes present in the lens.
Posterior Segment
It requires +90D/78D and +20D examination after
pupillary dilatation.
zz Vitreous is mostly clear except when there
maybe vitreous haze present posthemorrhage
with red blood cells floating in the vitreous.
zz The next important structure for assessment Fig. 1: Clinical photograph of fresh superior-temporal
is the optic disc and the peri-papillary area to BRVO depicting scattered hemorrhages in the area of
rule out signs of glaucoma and NVD and AION. drainage of the major vein and macular hemorrhages
non-perfused retina. After 6–12 months, the factor V leiden mutation, lupus anticoagulant,
acute findings will resolve and there will be anticardiolipin antibody.
venous sheathing and sclerosis. Macular zz Autoantibodies—ANA, ANCA, Anti DNA
edema can also be seen with +90D or +78D. antibody, Rheumatoid factor.
Amsler grid testing: It is important to monitor
zz Serum angiotensin converting enzyme
the response of the patient. Patients at their
zz Treponemal serology
home itself can do it. It can provide objective
zz Carotid duplex imaging
evidence to the patient if there is sudden increase
zz Full blood count.
in metamorphopsia or blurring of vision, so that Ocular investigation: It includes following:
patient can seek medical attention in time. This Fluorescein angiography (FA): Although the
is more important for patients who present with diagnosis of BRVO is purely clinical, FA does
vision of more than 6/12 and hence a follow-up is reveal additional findings that help in treatment
recommended for monitoring any deterioration in and prognostication. FA may not be useful in
vision. acute onset BRVO due to extensive areas of
hemorrhage resulting in block fluorescence and
Major Complications Associated with BRVO masking of underlying features. Therefore, the
zz Macular edema—main cause of low vision best time to do FA would be when substantial
zz Macular ischemia clearing of hemorrhage is seen. The characteristic
zz Neovascularization leading to vitreous findings on FA is delayed filling of the occluded
hemorrhage. NVE develops in 40% of cases. retinal vein, block fluorescence due to intraretinal
zz NVI develops rarely (1%). hemorrhages, microaneurysms, dye extravasation
secondary to macular edema, telangiectatic
DIFFERENTIAL DIAGNOSIS collateral vessels, capillary non perfusion and
retinal neovascularization (Fig. 2).
In patients with NVs or VH or Macular edema, The two most important features to note
differentials are essentially of the same (See on FA are the type macular edema and BRVO.
relevant chapters). For intraretinal hemorrhages, Macular edema may be perfused or nonperfused
differentials include DR, CRVO, AION, CNVM, (ischemic). In perfused, FA would reveal areas of
PEHCR, Coats, Homocysteine disorders, retinitis, macular leakage with a normal foveal avascular
vasculitis, etc. Usually the typical sectoral or zone (FAZ), whereas an ischemic edema may have
quadrantic appearance in a predisposed patient is
the clincher for BRVO.
INVESTIGATIONS
Systemic Investigation
In a person aged more than 60 years, BRVO may
occur as an age related vasculopathy even in the
absence of hypertension. However, routine blood
pressure, fasting blood glucose and lipid profile
should be checked.
In a young person the investigations that
should be done are:
zz Plasma homocysteine level
zz Chest X-ray- to rule out TB, sarcoidosis and left
ventricular hypertrophy.
zz C-reactive protein Fig. 2: Late FA picture showing blocked fluorescence
zz Thrombophilia screen—PT, TT, aPTT, protein in the area of the hemorrhages along with minimal
C, protein S, activated protein C resistance, leakage of the capillary bed
Retina 227
a distorted and enlarged FAZ apart from leakage. Study (BVOS), which recommended grid laser for
Type of edema will help guide our treatment, as perfused macular edema of more than 3 months’
ischemic cases have shown not to benefit from duration with best corrected vision <6/12. They
any intervention. Secondly, it helps us asses the showed 63% of these patients recovered more
whether BRVO is Ischemic, marked by presence than two Snellen’s lines at 3 years follow-up
of >5 disc area of capillary non-perfusion. following laser treatment in comparison to shams
These cases have more chances of developing 36%. However, the visual gain was delayed and
neovascularization and may need to be lasered. somewhat incomplete, and hence treatment
Another recent concept is to map peripheral options hastening recovery were explored.
ischemic areas, which may be responsible for Although various forms of steroids (SCORE trial,
constant production of VEGF leading to chronic GENEVA trial) have been used, their efficacy is less
macular edema. These areas may need targeted than anti-VEGF agents, along with increased risk of
laser ablation to decrease VEGF load. FA is also glaucoma and cataract. The second most important
essential for follow-up. trial was the BRAVO study, which evaluated
efficacy of intra-vitreal ranibizumab in macular
Wide-field Angiography (UWA) edema. They concluded that ranibizumab treated
patients gained more than 3 line improvement in
UWA gives 200 degrees field of vision. A
nearly 60% of patients and maintained it at 2 year
single image helps in delineating the extent of
follow-up (HORIZON trial, not to be confused with
peripheral capillary non-perfusion and can help
the ARMD Horizon trial). Thus, the first line of
in targeted laser. In addition, it can pick up any
treatment is anti-VEGF. Single monthly injection
neovascularization, as dynamic FA may lead to
for first 3 months followed by PRN dosing is
skip areas.
preferred, though the trials gave continuous
monthly injections for 6 months. Laser can be used
Optical Coherence Tomography (OCT) as second line of treatment. Recalcitrant cases
It is a noninvasive and highly informative should be assessed for cause—chronic breakdown
investigation. Even in acute BRVO where FA might of blood-retinal barrier, peripheral ischemia
not be helpful, OCT is minimally affected by or epiretinal membrance (ERM). Most of cases
extensive intraretinal hemorrhages. Characteristic respond well to steroids, others may need targeted
findings may include intraretinal edema, cystoid laser to peripheral ischemic areas or vitrectomy
macular edema, intraretinal hyper-reflectivity for significant ERM. Later, neovascularisation
from hemorrhages, shadowing from edema may occur and complicate further by vitreous
and occasional neurosensory detachment. hemorrhage, which may require vitrectomy
Some studies have also assessed IS-OS junction in nonresolving cases. Recently another anti-
abnormalities in chronic cases. Chronic cases may VEGF Aflibercept has also shown equal efficacy
also show ERM or VMA. with longer duration of action (VIBRANT trial).
It is the most preferred investigation for following Combination treatment of anti-VEGF with laser
up cases of macular edema and response to are ongoing has shown no additional benefit of
therapy. laser with anti-VEGF (RELATE and BRIGHTER
trial).
MANAGEMENT These case scenarios may be encountered.
Systemic condition should be taken care of Condition 1: Patient having BRVO with no macular
properly. Anticoagulants are not of much benefit. edema or neovascularization. FFA shows <5DD of
OCPs and HRTs may be avoided if possible. capillary nonperfusion.
Treatment for BRVO is usually done for its
Rx: Follow up.
complications.
The treatment of BRVO macular edema Condition 2: Patient having macular edema but no
(See Table, also See Chapter on CRVO) until neovascularization. FFA shows <5DD of capillary
recently was guided by the Branch Vein Occlusion nonperfusion.
Rx: Determine the cause of macular edema VIVA QUESTIONS

by fluorescein angiography. If the cause is
leaking capillaries, but the vision is 6/9 or better, Q.1. What are common causes of BRVO? What
observation can be employed with serial follow-up. are risk factors in young patients and how
If the vision is worse than 6/12 or hampers patients will you work up such patient?
activities, Anti-VEGF injection can be given. In Ans. Refer text.
chronic cases or in recalcitrant edema steroid Q.2. What is the pathogenesis of BRVO?
in the form of Triamcinolone (SCORE trial) or Ans. The obstruction occurs at the A/V crossing.
dexamethasone (GENEVA trial) can be given Most of times the artery is above the vein
with the risk of side effects like IOP elevation and can lead to compression of the other
and cataract. In cases of macular ischemia, only as they share the common adventitial
observation can be done and the macular edema sheath. Vitreous has also been implicated,
usually resolves within one year with gain of with greater vitreomacular attachments at
visual acuity. BVOS had suggested a wait period of greater risk for the developing BRVO. Thus
3 months, though current studies do not suggest turbulent flow may lead to endothelial
the same. swelling, increase in vessel wall size
Condition 3: Patient having BRVO with no macular and obstruction. The resulting venous
obstruction leads to elevation of venous
edema or neovascularization. FFA shows >5DD of
pressure that may overload the collateral
capillary nonperfusion.
drainage capacity and lead to macular
Rx: More stringent follow-up, as the chances of edema and ischemia. Increase in venous
developing neovascularization are more in these pressure can also result in rupture of the
cases. vessel wall with intraretinal hemorrhage.
Recently increased VEGF load has shown
Condition 4: Patient having BRVO with no macular
to lead to lessening of endothelial cells
edema but with neovascularization present, either
and increase in permeability of capillaries,
NVE or NVD.
thereby validating the use anti-VEGF.
Rx: According to Branch retinal vein occlusion
Q.3. What is the classification of BRVO?
study, sectoral retinal photocoagulation. Now with
Ans. It is classified based on anatomical
wide field angiography available trend has shifted
location—Major and macular. Major
towards targeted laser photocoagulation with
is when the vessel supplying the entire
repeat fluorescein angiography after 6 months to
quadrant gets affected. Macular is when a
look for any new areas of capillary nonperfusion or macular branch gets affected.
neovascularization and repeat laser. It can also be classified based on area of
Condition 5: Patient having BRVO with macular capillary nonperfusion—Ischemic or Non-
edema and neovascularization. ischemic. Ischemic having more than 5 disc
diameter area of CNP. These cases are more
Rx: Determine the cause of macular edema as to likely to develop neovascularization.
ischemia or leakage. Laser of peripheral capillary
nonperfusion areas has to be done. However, prior Q.4. Which is the most common quadrant for
to that an anti-VEGF intravitreal injection may BRVO and why?
be considered to reduce macular edema in cases Ans. Superotemporal quadrant is the most
of leaking capillaries as laser photocoagulation commonly involved quadrant as superior
increases macular edema. OCT should be done to hemi retina has the most number A/V
document macular edema. This is a current gray crossing changes and superonasal BRVO
zone. In patients with NVs, though BRVO is likely do not present due to lack of symptoms.
to be long standing and so the chronic edema, it Q.5. What is the most common cause of
may be prudent to treat the NVs first followed by defective vision due to BRVO?
sequential/simultaneous and rapid management Ans. The most common cause is macular edema.
of edema. However, it is imperative to rule out on FA
Retina 229
whether it is perfused or nonperfused as it Q.9. Name two landmark clinical trials.

guide the further course of treatment. Ans. BVOS and BRAVO. Refer text.
Q.6. What are the common complications Q.10. What is the first line of treatment for
of BRVO? What are chances of a non- macular edema?
ischemic BRVO to convert into ischemic Ans. Refer text.
BRVO?
Q.11. What are the treatment options in
Ans. The most important complications are mac- recalcitrant macular edema?
ular edema, macular ischemia and neovas- Ans. Refer text.
cularization sequelae. Neovascularization
may progress to vitreous hemorrhage, Q.12. What is the role of laser in management
tractional retinal detachment and neo- of macular edema? Recent trials for the
vascular glaucoma. Patients with macular same?
edema with vision < 6/12 are treated with Ans. Refer text. RELATE and BRIGHTER
above mentioned therapeutic options. (ongoing).
Macular ischemia has not been shown to
Q.13. Compare modalities for managing
benefit from treatment. Neovascularization macular edema in BRVO.
sequelae are expected in 11% of nonisch- Ans. See discussion.
emic and 40% of ischemic BRVO. It can
occur up until 3 years of BRVO, although in BIBLIOGRAPHY
most cases it occurs between 6–12 months.
Laser is recommended only after develop- 1. Hayreh SS, Zimmerman MB. Fundus changes
in Branch Retinal Vein Occlusion. Retina. 2015;
ment of new vessels. There is no additional
35(5):1016-27.
benefit of prophylactic laser in ischemic
2. Jaulim A, Ahmed B, Khanam T, Chatziralli IP.
cases without new vessel formation. Branch retinal vein occlusion: epidemiology,
Q.7. What investigations would you do in pathogenesis, risk factors, clinical features,
a case of BRVO and what would they diagnosis, and complications. An update of the
reveal? literature. Retina. 2013;33(5):901-10.
Ans. Refer text. 3. Rogers SL, McIntosh RL, Lim L, Mitchell P,
Cheung N, Kowalski JW, et al. Natural history
Q.8. When do you treat a patient of BRVO and of branch retinal vein occlusion: an evidence-
what are the treatment options? based systematic review. Ophthalmology.
Ans. Refer text. 2010;117:1094-101.
PROLIFERATIVE DIABETIC RETINOPATHY

Brijesh Takkar, Dhaval Patel, Rajesh Pattebahadur
Diabetic retinopathy is leading cause for vision loss
Chief Complaint
in middle-aged population. Worldwide prevalence
of DR is 34.6% and that of vision threatening DR is Proliferative diabetic retinopathy (PDR) generally
10.2%.1 Broadly, diabetic retinopathy is classified presents in a known case of diabetes mellitus (DM)
into nonproliferative diabetic retinopathy (NPDR), along with established diagnosis of DR under
proliferative diabetic retinopathy (PDR) and follow-up. Nevertheless, it is not uncommon
diabetic maculopathy. It is important that students to see a patient with presentation of PDR on
undergoing examination should be able to first ophthalmic visit. The different presenting
examine and identify the changes of DR and PDR, symptoms depend on the stage and complications
which is harbinger of severe visual loss. of PDR and are as follows:
zz Gradual loss of vision: Due to deterioration of Ocular Examination

DR, macular edema or associated progression
of cataract or tractional papillopathy/ The examination findings are similar to a case
retinopathy of NPDR. Few important points for PDR are
zz Acute loss of vision: When associated with summarized below:
vitreous hemorrhage (VH) or tractional
retinal detachment (TRD) involving macula or Visual Acuity
Diabetic macular edema (DME) Uncorrected visual acuity (UCVA) and best
zz History of floaters: Due to VH (mild) or vitreous corrected visual acuity (BCVA) must be noted. This
degeneration associated with PDR is important for decision-making.
zz Acute pain, redness and loss of vision: Due to
associated neovascular glaucoma (NVG). Eyeball
History of Present Illness Usually normal in such case. In few cases
While taking history of such case the onset squint can be seen due to associated ischemic
and progression of decrease of vision (DOV) is mononeuropathy involving cranial nerve 3rd, 4th
important, because the sudden onset implies or 6th (classically pupil sparing III CN palsy or VIth
the causes like vitreous hemorrhage, retinal CN palsy).
detachment (secondary rhegmatogenous retinal
detachment) whereas the slow onset DOV can be Cornea
caused by the complication like diabetic macular
Following points must be noted:
edema, clinically significant macular edema, etc. zz Corneal hypoesthesia (risk of neurotrophic
keratitis)
History of Past Illness zz Decrease corneal healing (risk of recurrent
See chapters on NPDR and DME for history. corneal erosion and persistent epithelial
defect)
Past Surgical History zz Tear film abnormalities (Dry eye due to
autonomic neuropathy affecting sensory
See chapter on NPDR/DME. nerves associated with tear secretion)
zz Corneal endothelium is usually normal in
Family History clinical examination. However, specular
Family history of DM, hypertension and other risk microscopy significantly higher coefficient
factors for DR. of variation, a decrease in the percentage of
hexagonal cells, and a low figure coefficient.
Personal History
Iris
History of smoking, alcohol intake, tobacco
chewing. Check for neovascularization of iris (NVI).
Pupillary margin are the earliest sites where
neovascularization the NVI. (See chapter on NPDR
EXAMINATION
and DME)
General Examination/Specific Systemic
Examination Pupil
Diabetes is multisystem disorder involving Following points must be noted
causing vasculopathy and neuropathy. So detailed zz Ectropion uvea (the fibrous tissue accom
systemic evaluation of cardiovascular system, panying neovascularization contracts which
respiratory system, central and peripheral nervous caused eversion of posterior pigmented layer
system, renal system should be done to rule out at pupillary margin).
the co-morbidity associated with DM. zz Increase pigment at angles.
Retina 231
zz Difficulty in dilating pupils (manifestation Fundus

of diabetic neuropathy resulting in reduced
Following signs must be noted.
functional innervation of the dilator muscle). zz Early PDR: New vessels at disc or within 1 DD
The maximum pupillary dilatation should
of disc (NVD) or New vessels elsewhere (NVE).
always be noted. In DM the pupils dilates zz High risk PDR: The presence of any three of the
poorly. This is important because every surgery
following four features characterizes DRS.
including cataract and retinal surgery will need
1. Neovascularization (at any location)
the maximum papillary dilatation. Diabetic
2. Neovascularization at the optic disc (NVD)
pupil dilates poorly in response to standard
3. Severe neovascularization:
anticholinergic eye drops (Tropicamide,
– New vessels within one disc diameter
Homatropine and Cyclopentolate that act by
of the optic nerve head (NVD) that are
paralyzing the iris sphincter muscle). This
>1/4–1/3 disc area in size
failure of dilatation is due, atleast in part,
– NVE at least 1/2 disc area in size
to a sympathetic dysfunction related to the
4. Vitreous or preretinal hemorrhage: To
autonomic neuropathy of these patients. The
simplify presence of the following indicates
addition of phenylephrine (which acts directly
need for treatment
by stimulating α-adrenergic receptors on iris
– NVD > 1/3 to 1/4 of disc area
dilator muscle, producing contraction of the
– Any NVD with associated VH
dilator muscle of the pupil), which utilizes the
– NVE greater than 1/2 with vitreous or
denervation super sensitivity (sharp increase
preretinal hemorrhage.
of sensitivity of postsynaptic membranes to
a chemical transmitter after denervation) of Tractional retinal detachment: It is important
the small diabetic pupil, greatly improving the to note if TRD is involving macula and TRD is
mydriatic drug response in diabetic patients. threatening macula (Figs 1 and 2).
zz Argyll Robertson pupils (bilateral small pupils TRD threatening macula: Retinal elevation at
that reduce in size on a near object (they least 4 DD area whose at least some part is within
“accommodate”), but do not constrict when 30° of center of macula or retinal elevation of less
exposed to bright light (they do not “react” to than 4DD, along with one or more vitreo-retinal
light)- an useful mnemonic Accommodation adhesion causing elevation of retina within 30° of
Reflex Present). center of macula in presence of new vessel or fresh
bleed vitreous hemorrhage.
Intraocular Pressure (IOP) TRD involving macula: Vitreo-retinal traction
Raised IOP can be there due to POAG (6% to 11%), along the arcade or disc or retinal traction lines
neovascular glaucoma or ghost cell glaucoma (in
long standing cases of VH) can be seen in DM.
Lens
See chapter on NPDR.
Vitreous following findings can be there:
zz The vitreous in diabetic patients undergoes
abnormal collagen crosslinking and non
enzymatic glycation, which lead to precocious
liquefaction and posterior vitreous detach
ment (PVD).
zz Asteroid hyalosis
zz In cases with secondary RRD along with TRD
will show the tobacco dust (Schaffer’s sign) is Fig. 1: Fibrovascular proliferation on disc and along
seen. arcades can be seen in the fundus photograph
PDR occurred. However, PRP can be done under

following circumstances:
zz Patient has poor DM control with associated
DM complications (nephropathy).
zz The patient will not or cannot be followed
closely
zz Access to health care is difficult
zz Fellow eye is blind from DR
zz Poor patient compliance to follow-up
zz Prior to cataract operation or pregnancy
(controversial)
zz Whenever iris or angle neovascularization is
seen, early PRP should be done irrespective of
Fig. 2: FA picture is suggestive of NVD and NVEs. presence or absence of retinal HRC.
Severe peripheral ischemia and macular ischemia are
also visible High-risk PDR
zz High-risk PDR requires immediate pan
extending through fovea and causing progressive
retinal photocoagulation (PRP) or scatter
vision loss.
photocoagulation
Presence of TTPH, VMT and CSME should
zz Patients with HRC treated with PRP have a
always be noted as it affects treatment management.
50% reduction in risk of severe visual loss. The
Very commonly these attachments are around the
rate of severe visual loss (visual acuity <5/200)
arcades.
was reduced by treatment from 16% in non-
Advanced DR: Also known as end stage DR or treated eyes over 2 years to 6% in treated eyes,
burnt out DR. The retina becomes feature less with a reduction of 57% in DRS.1,2
sclerosed vessels and loss of sheen. There may be zz Following points must be remembered, PRP:
massive TRD and accompanying absolute NVG. —— Does not improve visual acuity
—— May cause worsening macular edema,
DIFFERENTIAL DIAGNOSIS and loss of peripheral vision and night

vision
See chapter on DME and NPDR.
—— Indications for supplementation are
uncertain
INVESTIGATION —— Does not always cause regression of NVD/
(See chapter on DME and NPDR) NVE: Regression of neovascularization

zz UWFA may be very helpful in picking up occurs in 30–55% of eyes after laser
peripheral anterior ischemia with peripheral photocoagulation. Complete regression
new vessels of NVD was found in 29.8% and partial
zz USG should be done in VH. It also helps in regression in 24.5% of eyes at 12 months
prognostication. after treatment in DRS.1,2 Regression is
marked by fibrotic changes.
—— Is also indicated in patients with NVI from
MANAGEMENT
PDR even in the absence of NVD/NVE or
[Also See Chapter on VH, NPDR and DME in presence widespread retinal ischemia
(Table 1)]. and capillary drop-out on fluorescein
angiography
Non-high-risk PDR —— Protocols of Diabetic Retinopathy Clinical
These cases are treated with careful follow-up Research Network (DRCR.Net) have
(at 2–4 month intervals) and prompt pan-retinal attempted management of NVs with
photocoagulation (PRP) if progression to high-risk monthly intravitreal Ranibizumab alone,
Retina 233
Table 1 Comparison of anti-VEGF

Pegaptinib Bevacizumab Ranibizumab Aflibercept
Composition Aptamer (single stand Full length Antibody fragment Recombinant protein
of RNA or DNA antibody combined with Fc
portion of IgG
Molecular 50 kD 149 kD 48 kD 115 kD
weight
VEGF-165 isoform All isoforms of All isoforms of All isoforms of VEGF-A,
VEGF-A VEGF-A VEGF-B, PLGF-1 and 2
Half-life 2 days 5 days 3 days 7 days
Doses 0.3 mcg/0.9 mL 1.25 mg/0.05 mL 0.5 mg or0.3 2 mg/0.05 mg

mg/0.05 mL
Systemic Bronchitis Arterial Acute HTN CVA
side effect Plural effusion thromboembolic CVA HT
Diarrhea events MI
Nausea CHF Facial skin redness
Vomiting Dizziness Itchy skin rash
Carotid artery occlusion Confusion
CVA CVA
TIA
Contact dermatits
HS reaction/
anaphylactic reaction
Abbreviations: RNA, Ribonucleic acid; DNA, Deoxyribonucleic acid; VEGF, Vascular endothelial growth factor;
PLGF, Placental growth factor; CVA, Cerebrovascular accident; TIA, Transient ischemic attacks;
HS, Hypersensitivity; CHF, Congestive heart failure; MI, Myocardial infarction.
but up to 2 years, though there is no zz Long-term: Foveal burn, Macular edema,

benefit in terms on vision in comparison Choroidal neovascularization, PVD, Retinal,
to laser PRP, which is an economically subretinal or choroidal hemorrhage due
easier therapy. to excessive power of laser, Exudative RD,
zz If CSME is also present in addition to high- VH, Increased IOP, Mydriasis and paresis of
risk PDR, combined anti-VEGF therapy and accommodation, Loss of visual field, Loss of
PRP at the first treatment session should be dark adaptation/Nyctalopia, Lens opacities,
considered. In the days of LASER and early Increase in traction detachments.
treatment diabetic retinopathy study (ETDRS),
it was suggested that diabetic macular High-risk PDR—not Amenable to
edema (DME) should be managed prior to
Photocoagulation
PDR to prevent its worsening. However, now
intravitreals are preferred which act on NVs In presence of severe vitreous or pre-retinal
also, making decisions easier. hemorrhage, it may not be possible to deliver
zz Complications of PRP laser photocoagulation adequately. In such
zz Transient side effects: Blurring of vision, cases, retinal cryopexy or vitrectomy has to be
Macular edema, CD, Headache, iritis considered. 3 Reported benefits of peripheral
zz Medium term side effects: Macular edema may retinal cryotherapy include resorption of vitreous
persist for more than 3 months hemorrhages and regression of NVD, NVE,
and NVI. The main complication is the develop VIVA QUESTIONS

ment or acceleration of traction retinal detachment
in 25–38% of eyes.1 Therefore, this treatment should Q.1. Define NVD and NVE.
be avoided in patients with known traction retinal Ans. When neovascularization arise on or
detachment, and all patients must be monitored within 1 disc diameter of the optic disc
carefully. Now days, generally surgery is preferred. they are referred to as neovascularization
Vitrectomy in diabetic patients: Following are the of the disc (NVD). When they arise further
Indications of vitrectomy in PDR: than 1 disc diameter away, they are called
zz Non-clearing Hemorrhage-Vitreous/Sub- neovascularization elsewhere (NVE).
hyaloid/Premacular Q.2. What is the prevalence of retinopathy in
—— The Diabetic Retinopathy Vitrectomy
diabetes?
Study (DRVS) was the landmark Ans. The overall prevalence is about 25%. It is
randomized controlled trial to evaluate 40% in insulin-dependent diabetes mellitus
indications and timing of pars plana (IDDM) and 20% in non-insulin-dependent
vitrectomy for the management of diabetes mellitus (NIDDM).
advanced DR.
—— Early vitrectomy (within 3 months) Q.3. What associated systemic conditions
for treatment of vitreous hemorrhage worsen diabetic retinopathy?
secondary to DR was highly cost-effective Ans. These are—Pregnancy, Hypertension,
in a cost-utility analysis using DRVS Anemia and Renal failure.
results. Q.4. What do you know about the pathogenesis
The benefits of early vitrectomy for
of retinal new vessel formation?
nonresolving severe VH are greater Ans. It is not completely understood and current
for patients with T1DM and lower for theories emphasize the production of
T2DM. angiogenic factors by areas of ischemic
With diffuse or chronic DME, or a
and hypoxic retina. More recently, vascular
thickened or taut posterior hyaloid, endothelial growth factor (VEGF) has
early vitrectomy reduces DME. been isolated from ocular fluid and is
If retinal detachment is present, an
an endothelial cell-specific angiogenic
early vitrectomy is usually suggested. factor whose production is increased
Patients with bilateral severe vitreous
by hypoxia: it has been implicated in
hemorrhage generally should the neovascularization seen in diabetic
undergo vitrectomy in one eye when retinopathy and retinal vein occlusion.2,4
they are medically stable.
zz Tractional retinal detachment not involving Q.5. Enlist non-DR causes of visual loss in
the macula may remain stable for many years. diabetic subjects.
When the macula becomes involved, immedi Ans. These are as follows:
ate vitrectomy is generally recommended. • Trauma and injury
zz Combined tractional and rhegmatogenous RD • Amblyopia
may progress rapidly, and early surgery should • Age-related macular degeneration
be considered in these patients. • R etinal vein and artery occlusion,
zz Anterior segment neovascularization with ischemic optic neuropathy
post segment opacity • Cataract
zz Tractional RD threatening/involving macula • Glaucoma
zz Ghost cell/hemolytic glaucoma • Hypertension (and macroaneurysms)
zz Anterior hyaloid fibrovascular proliferation • Retinal detachment
zz Epiretinal membrane • Optic atrophy
zz Concurrent internal limiting membrane (ILM) • R etinal dystrophies and myopic
peeling may be done for cases with DME. degeneration.
Retina 235
Q.6. PASCAL (Pattern-scanning retinal laser) and en bloc methods of dissection. These
Ans. PASCAL technology is a semi-automated days the role of MIVS cutter as multi-
pattern generation method using short purpose instrument has made surgery
laser pulse durations of typically 20 ms much easier. Meticulous PRP should be
(five times shorter than conventional done and ILM peeling as needed. At the
systems). These laser pulses are delivered end of surgery, again all bleeders must be
in a rapid pre-determined sequence cauterized.
resulting in precise even burn patterns as Q.10. Management of NVG.
well as improved safety, patient comfort, Ans. See glaucoma chapters.
and a significant reduction in treatment
time when compared to single-shot Q.11. What are the specifications for PRP
photocoagulation. Multiple spot lasers are Ans. • 300–500 μm laser spot size depending
similar technology. on type of lens used (for magnification
adjustment)
Q.7. What is the role of anti VEGF injections in • L aser power starts at 100 mW and
surgical management of PDR. increase
Ans. It is helpful in managing unlasered cases • Spot distance half-one spot size apart
posted for surgery to prevent bleeding. It • Total spots around 2000–2500
should be given 1–3 days before surgery. • To be done in 2–3 sittings to avoid
In patients with TRS, however, caution exudative RD, angle closure and CD
should be executed for risks of conversion • Mark boundary and start inferiorly first
to secondary rhegmatogenous RD. Some • Stay 500 μm a far nasal to disc, just
surgeons also leave it at the end of surgery outside/within arcades and always
as it may help in managing concurrent atleast 2 DD away from fovea
DME and prevent rebleed to some extent. • Augmentation may be done as needed.
Q.8. What is Rebleed?
Ans. Up to 20% of patients may have recurrent REFERENCES
VH following PPV. Early causes includes
dispersed hemorrhage, hypotony and port 1. American Academy of Ophthalmology
Retina/Vitreous Panel. Preferred Practice
site bleeds. Late causes include inadequate
Pattern® Guidelines. Diabetic Retinopathy.
laser, poor hyaloidal dissection with retinal
San Francisco, CA: American Academy of
breaks poor systemic status and port site Ophthalmology; 2016. Available at: www.aao.
new vessels. org/ppp.
Q.9. How is diabetic PPV different from other 2. Nentwich MM, Ulbig MW. Diabetic retinopathy-
surgeries? ocular complications of diabetes mellitus.
Ans. After core vitrectomy, the surgeon should World J Diabetes. 2015;6(3):489-99.
3. Eliott D, Lee MS, Abrams GW. Proliferative
cauterize all possible bleeders first and
diabetic retinopathy: principles and techniques
then identify VR adhesions. Vitreoschisis of surgical treatment. In: Ryan SJ (Ed). Retina.
is frequent in such cases. PVD should not 3: 4th edition, Philadelphia: Elsevier Mosby;
be induced before clearing all adhesions 2006:2413–49.
to retina or new vessels. Intraoperative 4. Heng LZ, Comyn O, Peto T, Tadros C, Ng E,
bleeding is very common and the surgeon Sivaprasad S, Hykin PG. Diabet Med. Diabetic
should be prepared for it. Keep suction retinopathy: pathogenesis, clinical grading,
and other parameters low. An old protocol management and future developments.
would involve segmentation, delamination 2013;30(6):640-50. Review.
NONPROLIFERATIVE DIABETIC RETINOPATHY

Dhaval Patel, Brijesh Takkar, Rajesh Pattebahadur
INTRODUCTION —— Type of DM: Screening of DR is done

immediately after diagnosis in Type 2 and
Broadly, diabetic retinopathy (DR) is classified within 5 years of diagnosis in cases of Type
into nonproliferative diabetic retinopathy (NPDR), 1 DM. Typically diabetic macular edema
proliferative diabetic retinopathy (PDR) and (DME) is more common presentation of
diabetic maculopathy. The previous concept of Type 2 DM and PDR is more common in
background retinopathy generally falls under Type 1.1
NPDR. DR is a very common disorder in wards —— Duration of DM: Duration of DM is
and retina clinics. Hence, the chances of getting directly proportional to the risk of DR.
DR as short or long cases are very high. Particularly After 5 years, approximately 25% of Type
the viva revolves around the landmark studies 1 patients will have retinopathy.1 After
done for DR. In India, most of the results seen in 10 years, almost 60% have retinopathy,
western trials have been echoed by the Sankara and after 15 years, 80% have retinopathy.
Nethralaya-Diabetic Retinopathy Epidemiology In the Wisconsin Epidemiologic Study
and Molecular Genetic (SN-DREAMS) study of Diabetic Retinopathy (WESDR),
conducted in southern India. most vision-threatening complications
were present in approximately 50% of
HISTORY Type 1 patients who had the disease for
20 years.1 In the Los Angeles Latino Eye
Chief Complaints
Study (LALES) and in Proyecto VER
It primarily depends upon whether associated (Vision, Evaluation and Research), 18%
maculopathy is present or not. The various modes of participants with diabetes of more
of presentation can be than 15 years’ duration had PDR, with
zz Gradual or acute loss of vision (macular no difference in the percentage with
edema) PDR between those with Type 1 versus
zz History of floaters Type 2 diabetes.1,2 Type 2 patients with
zz Paracentral scotomata duration of diabetes of less than 5 years,
zz Some patients may be asymptomatic if macula 40% of those patients taking insulin and
is spared 24% of those not taking insulin have
zz Detection during DR screening. DR.1 These rates increased to 84% and
53%, respectively, when the duration of
History of Present Illness diabetes was 19 years.1,2 It is generally
Typically, the patient is a known case of diabetes believed that almost all diabetics develop
mellitus (DM) often referred by general physician some forms of DR overtime, whether
for screening. In India, it is not uncommon for the treatable or not.
Ophthalmologists to see a case of DR without a
—— DM control: Poorly controlled DM is
previous diagnosis of DM. highly likely to be associated with DR.
Duration of diabetes and severity of
hyperglycemia is the major risk factor
for developing retinopathy. Remember
Unlike many ocular disorders, this is very HbA1c is the most important parameter
important in a case of diabetic retinopathy. to evaluate control of (DM) over a period
Following points must be noted in history: of 3 months. As per guidelines, HbA1c
zz History of DM: It is very important to note of 7% or lower is the target for glycemic
down the following: control in most patients with DM.
Retina 237
As per the Diabetes Control and Complica If no DR, then at least one examination should
tions Trial (DCCT) for each 10%, decrease be done in every trimester. FA, intravitreal
in the HbA1c the risk of progression of injections and lasers should be avoided if
retinopathy decreases by 39%.1,2 possible. The retinopathy can eventually
—— Insulin use: Recent change from oral subside on its own following delivery.
hypoglycemic agents to insulin can be zz Cardiac disease and neurological disease
associated with a high risk of having
DR. Among the Type 2 patients a higher Past Surgical History
proportion of cases on insulin develops Previous history of any cataract surgery must
DR compared to those not taking insulin be enquired. Effects of cataract surgery on
(40% vs 24% at 5 years and 84% vs 53% the acceleration of the progression of DR are
at 19 years.1 Certain oral hypoglycemic controversial. Some reports suggests that eyes that
agents (OHAs) may in fact precipitate had phacoemulsification had a two-fold increased
DME. risk of developing retinopathy compared with eyes
—— Associated nephropathy: The development
that were not subjected to cataract surgery and
of PDR parallels an increased risk of cataract surgery may deteriorate the progression
nephropathy, myocardial infarction, and/ of diabetic macular edema. 1,2 But the Early
or cerebral vascular accidents.1 However, Treatment Diabetic Retinopathy Study (ETDRS)
the same cannot be said for DME as nearly did not find an association between clinically
all the studies on the topic have failed to significant macular edema and cataract extraction.
find an association between nephropathy
and DME. EXAMINATION
—— History of retinal laser for DR may be
present, type, number of settings must be General Examination/Specific Systemic

noted. Examination
—— History of prior treatment with intravitreal zz A detailed general examination must be done
injections should always be asked for as to rule out other complications of DM such as
leading questions. neuropathy, nephropathy, and diabetic foot.
zz Hypertension: Intensive management of zz Other medical problems (often coexisting with
hypertension may slow retinopathy progression. DM) must be looked for such as:
zz Hyperlipidemia: Management of serum lipids —— Hypertension
may reduce retinopathy progression and —— Smoking
the need for treatment. This has been noted —— Obesity
both in Action to Control Cardiovascular —— Hyperlipidemia
Risk in Diabetes (ACCORD) and Fenofibrate —— Anemia.
Intervention and Event Lowering in Diabetes

(FIELD) Trials.1 Ocular Examination
zz Anemia/use of angiotensin-converting Visual acuity: Reversible refractive errors with
enzyme inhibitor/clotting factors have been changes in glycemic levels even during a day
described to influence the onset of DR, should be kept in mind while performing vision
although the evidence is inconclusive. Overall, testing. Best-corrected visual acuity (BCVA) is
ACE inhibitors were not found to have any often helpful in deciding the treatment modality in
conclusive effect on DR progression.1,2 presence of macular edema.
zz Pregnancy: DR can worsen during pregnancy.
This is due to the physiologic changes of Eyeball: Cranial nerve (CN) palsies (classically
pregnancy as well as the changes in overall pupil sparing III CN palsy or VIth CN palsy as a
metabolic control. During the first trimester, sign of reversible ischemic mono neuropathy).
an eye examination should be performed and Lid: Xanthelasma (suggestive of hyperlipidemia),
subsequent follow-up visits must be scheduled recurrent hordeola and blepharitis in uncontrolled
depending on the severity of retinopathy. diabetes can be seen.
Conjunctiva: There is an increased risk of develop Lens: Look for following:

ing conjunctival bacterial infections. In addition, zz Reduction in accommodative ability.
microaneurysms can be seen in the bulbar zz DM can produce following forms of cataract:
conjunctiva. —— Typically nuclear and cortical cataract
Cornea: Look for following: formation is chronic and progressive

zz Corneal hypoesthesia (due to associated cases.
—— Acute cortical cataract formation with
neuropathy; risk of neurotrophic keratitis)
zz Decrease corneal healing (risk of recurrent profound elevations in blood glucose.
—— Snowflake cataracts, which are white
corneal erosion)
zz Tear film abnormalities (a manifestation of subcapsular opacifications, have been
autonomic neuropathy). described in young Type 1 diabetic
patients. This type of cataract is less
Iris: Check for neovascularization of iris (NVI) that commonly seen today because it is usually
indicates presence of PDR. associated with long-term untreated
Pupil: Look for following: hyperglycemia.
zz Ectropion uvea (the fibrous tissue accom —— Rapid progression of senile cataract
panying neovascularization contracts which occurs in DR.

caused eversion of posterior pigmented layer Vitreous: The vitreous in diabetic patients
at pupillary margin) undergoes abnormal collagen cross-linking and
zz Increase pigment at angles nonenzymatic glycation, which lead to precocious
zz Difficulty in dilating pupils (manifestation liquefaction and posterior vitreous detachment
of diabetic neuropathy resulting in reduced (PVD). Asteroid hyalosis can also occur.
functional innervation of the dilator muscle)
zz Argyll Robertson pupils (bilateral small pupils Fundus: The different changes that can be seen in
that reduce in size on a near object (they DR are summarized in Tables 1 and 2.
“accommodate”), but do not constrict when
exposed to bright light (they do not “react” to Stages of NPDR
light)—an useful mnemonic Accommodation zz Mild NPDR: Microaneurysm (one or more)
Reflex Present). (Fig. 1)
Intraocular pressure (IOP): DR can be associated zz Moderate NPDR: Microaneurysm, retinal
with primary open angle glaucoma (POAG) and hemorrhages (dot and blot), hard exudates,
neovascular glaucoma (NVG). cotton wool spots (CWS), venous beading,
Table 1 Fundus changes in diabetic retinopathy

Clinical sign Pathogenesis Layer of retina affected Effect of treatment
Cotton wool spot Nerve fiber layer ischemic necrosis Nerve fiber layer Persisits
Microaneurysms Secondary to capillary wall Superficial retinal layers Resolves if controlled
outpouching due to pericyte loss
Dot and blot Microaneurysms rupture in the Inner nuclear and outer Resolves gradually
hemorrhages deeper layers of the retina plexiform layers
Flame-shaped Splinter hemorrhages that occur in Superficial layers Resolves gradually
hemorrhage the more superficial nerve fiber layer
Retinal edema Breakdown of the blood-retina Deeper layers Resolves but
and hard exudates barrier, allowing leakage of serum complete resolution
proteins and lipids from the vessels unlikely
Retina 239
Table 2 International diabetic retinopathy severity scale

Disease Severity level Dilated ophthalmoscopy findings
No apparent DR No abnormalities
Mild nonproliferative DR Microaneurysms only
Moderate nonproliferative DR More than “mild” but less than “severe”
Severe nonproliferative DR Any of the following:
20 or more intraretinal hemorrhages in 4 quadrants
Definite venous beading in 2 or more quadrants
Prominent IRMA in 1 or more quadrants and no neovascularization
Proliferative DR One or more of the following:
Definite neovascularization
Preretinal or vitreous hemorrhage
Fig. 1: Mild NPDR with microaneurysm Fig. 2: Moderate NPDR with retinal hemorrhages and
and hemorrhage venous beeding
arteriolar narrowing, intraretinal micro

vascular abnormalities (IRMA) (Figs 2 and 3).
zz Severe NPDR: All of above plus anyone of
the following three (the famous 4:2:1 rule in
ETDRS) (Fig. 4):
1. Blot hemorrhages in 4 quadrants
2. Venous beading in 2 quadrants
3. IRMA in 1 quadrant.
zz Very severe NPDR: More than one of the above
mentioned rules.
Clinically Significant Macular Edema (CSME)

zz Retinal thickening or edema at or within
500 micron of the center of the macula
zz Hard exudates at or within 500 micron of the Fig. 3: Very severe NPDR with retinal hemorrhages,
center of the macula associated with retinal hard exudates, cotton wool spots (CWS) and venous
thickening of the adjacent retina beading
zz Retinal vein occlusion (branch or central)

zz Hemoglobinopathies
zz Anemia or leukemia
zz Ocular ischemic syndrome
zz Radiation retinopathy
zz Idiopathic juxtafoveal telangiectasis
zz Coats’ disease
zz Vasculitis (e.g. sarcoidosis, lupus).
INVESTIGATION
Systemic investigations: Following are specific for
DR:
zz Fasting and postprandial blood sugar test
Fig. 4: Severe CWS and arterial attenuation along with zz Glucose tolerance test
features of NPDR in a case of combined retinopathy zz Hemoglobin A1c
zz Renal function test
zz An area or areas of retinal thickening at least zz Others for the systemic entities listed before.
one disk area in size at least part of which
Fundus photography: Color fundus photography
is within 1 disk diameter of the center of the
is commonly used to document retinal disease
macula.
and its evolution in diabetic patients. It is used
for tracking disease progression and is accepted
Retinovascular Disease
as the best screening method for DR especially in
zz Retinal vein and artery occlusion teleophthalmology.
zz Ischemic optic neuropathy.
Optical Coherence Tomography
Diabetic Papillopathy
zz Noninvasive imaging technique
zz It is a rare cause of bilateral (or sometimes zz It is important in managing diabetic macular
unilateral) disc swelling in patients with type-1
edema (see the short case on DME).
DM.
zz Disc edema is often associated with capillary Following are uses of optical coherence tomo
telangiectasia’s overlying the disc surface. graphy (OCT) in DR:
zz It differs from anterior ischemic optic neuro zz To investigate cases with unexplained visual
pathy (AION) in that there is often bilateral, acuity loss
simultaneous optic nerve involvement. zz To detect areas of vitreomacular traction
zz Visual acuity is often normal. (VMT)
zz The pathogenesis is impaired of blood flow zz To evaluate patients with difficult and/or
causing disc swelling, but not have sufficient questionable examinations for DME
to significantly affect optic nerve function. zz To rule out other causes of macular edema
zz Most cases, the optic disc edema resolves zz To screen a patient with no or minimal diabetic
without residual visual deficit. retinopathy.
Fundus fluorescein angiography (FFA): Following
DIFFERENTIAL DIAGNOSIS are uses of FFA in DR.
The characteristic clinical findings and history of
DM often differentiates DR from other disorders. Diagnosis
However, following disorders must be kept in mind zz Ischemic maculopathy
that can mimic NPDR, especially in unilateral zz Areas of capillary nonperfusion
cases: zz To rule out other causes of macular swelling
zz Hypertensive retinopathy zz Differentiate between new vessels from IRMA
Retina 241
zz To identify suspected but clinically obscure Mild to moderate NPDR without macular edema:
retinal neovascularization Re-examine within 6 to 12 months. Approximately
zz To evaluate unexplained visual loss. 16% in cases of mild and 23% in cases of moderate
NPDR of patients may progress to proliferative
Aid in Treatment stages within 4 years.
zz To guide laser treatment of CSME Mild to moderate NPDR with CSME: If it is center-
zz Delineate fovea and fovea avascular zone involving (ci-CSME) anti-VEGF is the treatment
zz Delineate area of leakage of choice. If it is non-center-involving (nci-CSME)
zz To detect areas of untreated retinal capillary then focal/grid laser surgery guided by the ETDRS
nonperfusion that may be the cause for is the treatment of choice. (Refer to the chapter
persistent retinal or disc neovascularization short case diabetic macular edema for detail on
after previous scatter laser photocoagulation. management of macular edema).
Ultra wide imaging: Instead of the previously used Severe and very severe NPDR: Follow-up patient
7 field fundus imaging, now the focus is shifting very closely (2–4 months). Half of patients with in
towards ultra wide field imaging for monitoring case of severe NPDR 50% of the cases progresses
NPDR. PDR within 1 year, and 15% will develop high-risk
PDR. Similarly in cases very severe NPDR 75% of
Red-free imaging: This is helpful in identifying new
the cases develop PDR within 1 year while 45%
vessels, which may not be visible to the clinician.
will develop high-risk PDR. Therefore, a close
Electroretinogram (ERG): Full field and multifocal follow-up of such cases is required.
ERG are helpful in diagnosing what has been Classic indication of PRP is high risk PDR,
called as diabetic neuroretinopathy. This entity however it may be done even without CSME in
particularly can cause mild/minimal/functional/ special situations such as:
qualitative visual loss in patients without zz Patient is a young insulin-dependent diabetic
maculopathy. (IDDM)
OCT angiography: Role is under evaluation, zz Patient has poor DM control with associated
and current concept involves imaging micro- DM complications (nephropathy)
aneurysms and FAZ changes. zz Fellow eye is blind from DR
zz Family history of blindness from DR
MANAGEMENT zz Poor patient compliance to follow-up
zz Prior to cataract operation or pregnancy.
Following points are important in management of
NPDR:
VIVA QUESTIONS
zz Joint management with family physician or
endocrinologists Q.1. Describe pathophysiology of micro
zz Stress should always be on systemic control aneurysm development.
first, and ocular management later Ans. Normally there is one pericyte per endo
zz Ensure good DM control (good glycosylated thelial cell. Pericytes are contractile cells
hemoglobin levels) that play an important role in microvascular
zz Treat associated systemic disease most autoregulation and maintaining the blood
important among them is maintaining, serum retinal barrier. In people with diabetes,
lipids, and blood pressure. the pericytes die off and are decreased
zz Worsening DR is an indicator of poor systemic in number (SORBITOL accumulation).
control. Their absence weakens the capillaries and
Normal or minimal NPDR (with rare micro permits thin-walled dilatations, which
aneurysms): Follow-up patient and watch for are known as microaneurysms. Naturally,
progression and macular edema. Remember these microaneurysms tend to collapse
within 1 year 5–10% of such patients can progress upon themselves but may leak causing
to advanced stages of DR.1 disease.3
Q.2. Describe pathophysiology of macular cases, NV tends to grow along the posterior
edema development. hyaloid interface especially around the
Ans. Breakdown of the blood–retina barrier is optic nerve (NVD) and periphery (NVE).
an important pathophysiologic feature On fluorescein angiography, NV will
of diabetic retinopathy that leads to the often show late leakage whereas IRMAs
development of macular edema. traditionally do not leak.3
Q.3. How would you differentiate between Q.6. How often would you screen diabetic
microaneurysm and small dot hemor- patients for retinopathy?
rhage? Ans. Type-1 (Insulin-dependent diabetics):
Ans. It is often difficult to distinguish a small 5 years after diagnosis then annually.
dot hemorrhage from a microaneurysm Type 1 diabetes, substantial retinopathy
by ophthalmoscopy alone. On fluorescein becomes apparent as early as 6–7 years
angiography patent microaneurysms will after onset of the disease. In addition, the
fill with dye quickly and then leak, unlike disease is diagnosed early because of its
a small dot hemorrhage that will block severity, hence screening is recommended
fluorescence. beginning 5 years after the diagnosis of
Type 1 diabetes.
Q.4. Describe pathophysiology of dot-blot and
Type-2 (Non-insulin-dependent diabetics):
flame-shaped hemorrhage.
Screening is done at diagnosis and then
Ans. If the hemorrhage is deep (i.e., in the inner
annually. The diagnosis of DM may be
nuclear layer or outer plexiform layer), it
delayed in these cases and when the
usually has a round or oval shape (dot or
diagnosis is made, it is assumed that the
blot hemorrhage). Superficial (nerve fiber
disease might have already been present
layer) hemorrhages, on the other hand,
for last 4–5 years. About 30% of patients
become flame or splinter-shaped. This is
will have some manifestations of DR at
due to the peculiar arrangement of nerve
diagnosis hence screening has to be done
fibers in respective layers. Superficial
immediately after diagnosis.
hemorrhages may be associated with CWS,
Pregnancy (Type 1 or Type 2):
which represent blocked axoplasmic flow
• First examination—soon after conception
in the RNFL following focal ischemia.3
and early in the first trimester.
Q.5. Intraretinal microvascular abnormalities • Follow-up—no retinopathy to mild or
(IRMAs) moderate NPDR: every 3–12 months. If
Ans. These are shunt vessels and appear as severe NPDR or worse is present than
abnormal branching or dilation of existing follow-up: every 1–3 months.
blood vessels (capillaries) within the retina
Q.7. What is the earliest sign of retinal change
that appears to supply the areas of non-
in diabetes?
perfusion. These vessels represent either
Ans. Microaneurysms at posterior pole are the
new vessel growth within the retina or
first clinical signs. An increase in capillary
remodeling of pre-existing vessels through
permeability, evidenced by the leakage
endothelial cell proliferation stimulated
of dye into the vitreous humor after
by hypoxia bordering areas of capillary
fluorescein injection, is the earliest sign of
non-perfusion. When compared to neo-
retinal change in diabetes mellitus.
vascularization (NV) in PDR, IRMAs are
slightly larger in caliber with a more broad Q.8. What are the three questions that ETDRS
arrangement and are always contained addressed and what are the inferences?
to the intraretinal layers. Conversely, NV Ans. • What is the role of aspirin in diabetic
tends to be much finer and delicate in retinopathy?
caliber, and is sometimes more focal in Answer: It neither improves nor worsens
location depending on its severity. In severe retinopathy.
Retina 243
• W hat is the role of initiating early laser • D

irect diffusion of oxygen from chorio
(as compared to DRS high-risk criteria) capillaries through scar to inner retina.
in the management of severe non- • Stimulates endothelial repair process.
proliferative and early proliferative
Q.12. How will you differentiate between
retinopathy?
Diabetic papillopathy and AION?
Answer: Inconclusive. No strong benefit
Ans. See fundus changes above in the chapter.
to early scatter panretinal photocoagu
lation (PRP) was found. Certain clinical Q.13. Preoperative/intraoperative/postopera
circumstances (e.g. poor compliance tive precautions for cataract surgery in
with follow-up examinations, rapid presence of NPDR with CSME.
progression in fellow eye, very poor Ans. Following are the precautions:
cardiorenal status) may justify early Preoperative care
initiation of PRP. • In patients with existing rubeosis, this
• What is the role of laser (PRP or focal should be treated preoperatively with
macular laser, or both) in the manage panretinal photocoagulation.
ment of macular edema? • Laser of macular edema or anti-VEGF
Answer: Traditionally, there is no role injection should be given.
for PRP in treatment of macular edema. • Topical nonsteroidal anti-inflammatory
Macular laser is of benefit, reducing drugs (NSAIDs) can prevent intra-
the risk of moderate visual loss by 50%. operative miosis.
Patients with CSME should be treated. Intraoperative care
However, now research focus is towards • I t is advisable to perform a large
targeted laser for peripheral ischemic capsulorrhexis with a 6 mm optic lens,
areas detected upon wide FA. thus allowing the better visualization of
the fundus for PRP if required.
Q.9. What is international DR severity scale?
• Multifocals are contraindicated as
Ans. A workshop was held in April 2002 in con they reduce the contrast and fundus
junction with the International Congress evaluation is difficult.
of Ophthalmology to develop and build
Postoperative care
broad-based consensus around a clinically
• Patients with diabetes are at slightly
relevant and simplified DR disease severity
greater risk of cystoid macular edema
scale that is summarized in Table 2.
which may be difficult to differentiate
Q.10. What is subthreshold micropulse diode from true diabetic maculopathy (see
laser photocoagulation? DME chapter).
A. Subthreshold: The intent of treatment is to • Incidence of capsular opacification is
lightly affect the retinal pigment epithelium greater in diabetics than in nondiabetics.
alone, restricting the lateral spread of Therefore, a large yttrium aluminum
heat and thus, sparing damage to the garnet (YAG) capsulotomy to improve
overlying photoreceptors and mid-retinal vision or improve visualization of the
interneurons retina may become necessary.
Micropulse: Employs repetitive trains
Q.14. Role of NSAIDS.
(50–500) of laser pulses of short duration
Ans. DM induces inflammatory reactions by
(0.8–5.0 ms) and extending the “off-time”
many mechanisms including, oxidative
between micropulses.
stress NF-κB activation, NOS dysregulation,
Diode laser: Using a longer wavelength
AGEs formation, hypertension, dyslipi
near-infrared 810 nm diode laser.
demia and impaired anti-inflammatory
Q.11. How does laser act in macular edema? pathways. Topical nonsteroidal anti-
Ans. It acts by following mechanism: inflammatory medications are common
• Reduces metabolic need. treatment for DME. These agents are
directed at decreasing intraocular pro San Francisco, CA: American Academy of

staglandin levels, which have been Ophthalmology; 2016. Available at: www.aao.
implicated in the pathogenesis of DME. org/ppp.
Bromfenac perhaps has the highest vitreal 2. Nentwich MM, Ulbig MW. Diabetic retinopathy-
penetration. ocular complications of diabetes mellitus.
World J Diabetes. 2015;6(3):489-99.
3. Heng LZ, Comyn O, Peto T, Tadros C, Ng E,
REFERENCES
Sivaprasad S, Hykin PG. Diabet Med. Diabetic
1. American Academy of Ophthalmology retinopathy: pathogenesis, clinical grading,
Retina/Vitreous Panel. Preferred Practice management and future developments.
Pattern® Guidelines. Diabetic Retinopathy. 2013;30(6):640-50. Review.
RETINITIS PIGMENTOSA
Jyotiranjan Mallick, Prafulla Kumar Maharana
INTRODUCTION macular edema (CME), complicated cataract

or in atypical form of RP.
Retinitis pigmentosa (RP) is a retinal dystrophy zz Acquired tritan color vision defect
affecting the rod photoreceptors, to begin with, zz Noted during sibling screening
and subsequently cones resulting in severe visual zz Noted during fundus evaluation after medical
loss. The prevalence is 1:3500 to 1:4000. The advise.
inheritance pattern is complex. It usually presents
clinically in the second or third decade. Central History of Present Illness
vision is preserved late in the course of the disease. zz The disease usually begins in childhood or
However, earlier loss of central vision may be
adolescence. The initial symptom is difficulty
attributed to cystoid macular edema occurring in dark adaptation. The earlier the onset of
in 10–50% of individuals.1-3 Typically X-linked defective dark adaptation, the more severe is
variants present very early in childhood where as the course of RP.
autosomal recessive (AR) variants present around zz The loss of red cell functioning in the retina
10–12 years of age. Autosomal dominant (AD) of RP patients leads to many glare-related
linked and spontaneous variants present later. In problems. In low light, these patients have
exams, it is usually given as a long case. night blindness. However, in bright light they
can experience “white out” glare. Parents may
HISTORY note an affected child to bump into objects at
night.
Chief Complaint zz Subsequently there occurs constriction of
The usual presenting complaints are following: visual field with preserved central vision until
zz Dark adaptation difficulty (earliest, but patient late in the disease. A careful history can often
often fail to recognize) reveal frequent bumping or frequent accidents
zz Night blindness (most common presentation) while driving vehicle in cases unaware of their
zz Progressive loss of peripheral visual field— constricted field of vision.
rarely the patient can present with tunnel
vision when the central field is the only vision History of Past Illness
left. Past medical and surgical history must include
zz Gradual decrease in visual acuity—central careful history to rule out the systemic associations
visual acuity is usually preserved until the of RP. This is more important in X-linked and AR
end stages of retinitis pigmentosa (RP). Early variants. There may be past history of associations
central acuity loss can occur from cystoid of RP as well.
Retina 245
Family History Ocular Examination

Meticulous family history should be taken and a Visual acuity: The visual acuity is normal in
pedigree should be prepared. A three generations early cases. Early central acuity loss can occur
pedigree chart must be prepared in all such cases. from cystoid macular edema (CME), epiretinal
Effort should be made to identify the inheritance membrane (ERM), and complicated cataract or in
pattern if present. atypical form of RP.
Eyeball: Myopia is a common association of RP
EXAMINATION (22–75% of people with RP have myopia of about
General Examination -2 D).
Detail systemic examination is done to know any Lid/Conjunctiva/Cornea/Sclera: These are

associated sensory neural hearing loss, vestibular usually within normal limits (WNL). There may be
nerve function, ataxia, and speech disorder. RP can fat atrophy of the orbits due to frequent rubbing,
be associated with multiple syndromes; some of typically seen in LCA. Keratoconus may be noted
them and their systemic features are summarized as an association of RP.
in Table 1. Associated sleep disturbance and Iris: Forward shifting of iris lens diaphragm may
headache are common in RP patients.1-3 occur due to zonular weakness.
Table 1 Common forms of syndromic retinitis pigmentosa

Syndrome/disease Characteristic features Etiology
Usher syndrome Bilateral sensory neural hearing loss Most commonly due to a
Lack of development of speech mutations in the MYO7A
Vestibular nerve dysfunction gene on chromosome 11q
Ataxia
Laurence-Moon- Obesity Mutation involving at least
Bardet-Biedl syndrome Mental retardation or mild psychomotor delay six distinct loci
(BBS) Post-axial polydactyly
Hypogenitalism
Renal abnormalities/renal failure
Refsum disease Neuropathy Recessively inherited
Ataxia condition in which the
Deafness patient accumulates
Arrhythmia exogenous phytanic acid
Bassen-Kornzweig Acanthocytosis A malabsorption syndrome
syndrome Malabsorption associated with absence
(abetalipoproteinemia) Ataxia of low-density plasma
lipoproteins or so-called
β-lipoproteins
Freidreich-like Friedreich-like ataxia, dysarthria, hyporeflexia, and Recessively inherited,
ataxia with retinitis decreased proprioceptive and vibratory sensation, as mutation in the
pigmentosa well as markedly decreased serum vitamin E levels α-tocopherol-transfer
protein (α-TTP) gene
Kearns-Sayre syndrome Conduction defects of heart Mitochondrial DNA disorder
External ophthalmoplegia
RP
Pupil: As per traditional teaching, afferent papillary —— The bony spicules are characteristically
defect (APD) does not occur in normal room perivascular. Remember the spicules are
luminance, as light reflex pathway dependent on initially more around the venules. They
melanopsin RPE cells is still functional. Relative appear first in the mid periphery.
afferent pupillary defect (RAPD) suggests zz In advanced cases waxy pallor of the optic disc
underlying optic atrophy. However, RAPD can be is seen (50–55%) (Fig. 2).4
absent in spite of optic atrophy as RP is a bilateral zz Associated cystoid macular edema (CME) is
disorder. seen in 19–70% of cases (Fig. 3).1,4
zz Macula may show atrophy or epiretinal
IOP: Increase in IOP may occur in cases with RP
membrane (ERM) formation.
with glaucoma, open angle glaucoma (OAG) is an
zz Optic disc drusen can be seen in cases of RP.
association.
zz There may be the golden ring around the optic
Lens: Posterior subcapsular cataract (PSC) may disc sign.
occur in some cases (41–53%). Zonular weakness,
although rare, can also occur in RP. Atypical RP
Vitreous: Vitreous degeneration and early In 20–30% of cases of RP the classical triad of
posterior vitreous detachment can occur. Dust- attenuated vessels, bone spicules and waxy pallor
like particles can be seen in the vitreous. These
particles are fine, colorless consisting of free
melanin pigment granules, pigment epithelium,
uveal melanocytes, and macrophage-like cells.
They are found evenly distributed throughout
the vitreous cavity. Observation of these particles
can be helpful in the diagnosis of early RP before
fundus changes are apparent. Retrolental cells
may also be noted, as pars planitis is known to be
associated with RP.
Fundus: Following signs can be seen in a case of
typical RP:
zz Arteriolar narrowing (most common but not
earliest, seen in 90–95% of cases)
zz Pigmentary changes: (Some authors list this as Fig. 1: Fundus photograph showing biny spicules with
the first and most prevalent sign) waxy disc pallor
—— Earliest changes include fine dust-like
intraretinal pigmentation, and loss of

pigment from the pigment epithelium.
—— The pathognomonic pigment clumps in
a characteristic “bone spicule” configura

tion appears subsequently as the
photoreceptor deterioration progresses,
and there is increasing loss of pigment
from the pigment epithelium with intra-
retinal clumping of melanin (Fig. 1).
—— The term bone spicules are used to refer
to the type of small cells that are laid down

in the formation of new bone matrix.
These spicules in bone have a similar
shape to the classic pigment finding in RP, Fig. 2: Fundus photograph in case of RP showing
thus the name. severe vascular attenuation
Retina 247
zz Paravenous RP: In this form the ERG changes

and intraretinal pigment and atrophy of the
pigment epithelium remains confined to
the distribution of the retinal veins in each
eye. Patients may lose central vision but, like
pericentral RP, they retain peripheral vision
into later life.
Syndromic RP
zz RP associated with systemic disorder
zz Usually AR or mitochondrial inheritance
zz Usher syndrome, Kearns-Sayre syndrome,
Bassen-Kornzweig syndrome, refsum disease,
Fig. 3: Macular scarring in a case of RP with CME Bardet-Biedl Syndrome (Table 1).
may not be seen. These forms of RP are known as
atypical forms. Pseudo-RP
zz Retinitis pigmentosa sine pigmento: Fundus
appears normal but there is evidence of photo This term refers to the conditions that mimic
receptor dysfunction in electroretinogram the fundal appearances of retinitis pigmentosa.1-3
(ERG). This can occurs in following conditions:
zz Retinitis punctata albescens: Retinal pigment zz Syphilis (leopard skin retinopathy)
epithelial degeneration appears as presence of zz Congenital rubella
deep white dots at the level of RPE. zz Drug induced: Thioridazine streaks, chloro
zz Sectoral RP: Only one quadrant or one-half quine, quinine and phenothiazine
of the fundus has degenerative changes of zz Laser scars
retinitis pigmentosa. Most commonly, the zz Old retinal detachment
inferior and nasal quadrants are involved and zz Trauma
the involvement is often symmetrical. zz Chronic uveitis
zz Retinitis pigmentosa inversus or pericentric RP: zz Cancer-associated retinopathy.
Changes of RP primarily affects macula and
posterior pole. Thus, it can mimic hereditary Choroideremia
fundus dystrophies. Visual acuity and color
vision are affected early in the course of the zz X-linked disease
disease. It progresses at a slower rate than zz Early stage: Fine pigmentary stippling and
typical RP and in advanced cases, the central atrophy of fundus
vision is severely affected but the peripheral zz Later stage: Patchy RPE and choroidal atrophy
vision is retained. The visual filed defect is near which gradually coalesce.
mid-peripheral scotoma extending from the
5 to 30° isopter in contrast to the 20–40° isopter Gyrate Atrophy of Retina and Choroid
seen in cases of typical RP.
zz Unilateral RP: It is characterized by fundus An AR disorder. Discrete round patches of cho
changes of RP in one eye and no evidence of RP roidal and retinal atrophy occur in midperipheral
in the fellow eye. ERG findings are substantially fundus. Gradual coalescence of the lesions occurs
reduced in the affected eye but normal in the leading to sharply defined scalloped defects. There
fellow eye. The RP progresses in the affected are 10 to 20-fold increase in plasma ornithine
eye while the other eye remain unaffected. level due to ornithine ketoacid aminotransferase
DDs of pseudo RP must be evaluated. deficiency.1
Cone and Rod Dystrophy zz Visual field: In early RP, there is presence of
ring sctoma in midperiphery (20–25° from
In contrast to typical retinitis pigmentosa (known fixation). It usually starts as a group of isolated
as the rod-cone dystrophies), cone rod dystrophies scotomas around 20–25° from fixation, and
reflect the opposite sequence of events, where gradually coalesces to form a partial followed
cone cells are primarily first affected with later loss by a complete ring. The outer edge of the
of rods. Thus, the presenting features are difficulty scotoma expands relatively rapidly, while the
with the clarity of vision, color vision problems inner edge constricts slowly toward fixation.
and light sensitivity. It can occur in association In advanced cases, the inner edge progresses
with Alstrom syndrome, Bardet-Biedl syndrome, to center giving rise to tunnel vision. (It must
neuronal ceroid lipofuscinosis, Joubert syndrome be remembered that when visual acuity is low
and related disorder. Remember few clinicians only Goldman’s visual field is possible and this
term this as inverse RP; but this is better termed is the preferred modality in RP).
as inverse retinal pigmentary dystrophy; in inverse zz Optical coherence tomography (OCT): It is
RP although the lesion starts around macula or done to rule out any macular pathology. This
posterior pole the pathogenesis is same unlike must be done in any case presenting with early
cone rod dystrophy where cones are primarily loss of central vision or acute loss of vision
affected.1 during the course of RP. Choroidal changes
have also been studied in RP, but have been
Leber’s Congenital Amaurosis found to not relate to visual acuity in these
patients.
Following points helps in differentiating from zz Fluorescein angiogram: It shows diffuse
RP:1-3
hyperfluorescence due to RPE window defects.
zz AR inheritance with onset in 1st year life. zz Ultra wide imaging helps in documenting
zz Severely reduced visual function with
peripheral changes.
nystagmus/nystagmoid movements, sluggish zz Color vision and contrast sensitivity may also
pupillary response, photophobia, and
be done for evaluation.
hyperopia.
zz Oculodigital sign: Characterized by orbital
fat atrophy (apparent by the deep sockets CLASSIFICATION
with a prominent eyeball), and development zz Non-syndromic or simple: It does not affect
of keratoconus if the child survives into later
other systems of body. It may occur as AD, AR,
decades. This is because the child repeatedly
X-linked, and digenic forms.
rubs, pokes and presses the eyes to elicit zz Syndromic: Affects other neurosensory
retinal stimulation.
systems of body such as hearing [see Table 1]
zz Initially fundus appears normal but later in zz Systemic: Affects multiple organs of the body.
childhood pigmentary retinopathy changes
develop. Toxoplasma like scars can occur.
zz ERG is nondetectable and severely abnormal STAGING
quite in early stage of the disease. Typical form of RP has been divided into following
zz SECORD (Severe childhood onset retinal three stages:1-3
dystrophy): Similar to LCA but with better
visual acuity than LCA later in life.
Early Stage
INVESTIGATION zz Involvement occurs in first years of life
zz Night blindness
zz ERG: It shows early and severe reduced rod zz Very early minimal visual field defect
response, decreased a and b wave amplitude, zz Normal fundus appearance
prolonged implicit time, reduction in cone zz ERG shows decreased b wave amplitude
response in advanced cases. particularly in scotopic conditions.
Retina 249
Mid Stage as there is risk of teratogenicity. It should not

be given to children <18 years and persons
zz Peripheral visual field defect in day light
with ABCA4 deficiency due to risk of
zz Fundus changes like bony spicule like
accumulation of toxic product A2E. Vitamin
pigmentary changes, attenuated vessels and
E in high dose has been shown to adversely
optic disc pallor.
affect the course of RP and should therefore be
zz ERG: Unrecordable in scotopic conditions and
avoided.
hypovolted cone response
zz Increased intake of docosahexaenoic acid
and lutein-zeaxanthin has shown promising
End Stage results in decreasing progression.
zz Tunnel vision zz Systemic carbonic anhydrase inhibitors in
zz Decreased visual acuity due to cone a dose of 500 mg daily have been shown to
involvement reduce the cystoid macular edema associated
zz Unmasking of choroidal vessels giving the with RP. However, the CME is chronic and often
fundus tessellated appearance difficult to treat. Topical carbonic anhydrase
zz Unrecordable ERG. inhibitor not very effective in treating CME.
zz Intravitreal triamcinolone and anti-VEGF
COMPLICATIONS injections have shown to reduce CME.
zz Cataract extraction is often indicated for
zz Keratoconus decreased vision due to posterior subcapsular
zz Posterior subcapsular cataract cataract especially in cases with less than
zz Open angle glaucoma (3%) 10° of visual field.
zz Occasional intermediate uveitis zz Hyperbaric oxygen therapy to promote pho
zz Exudative vasculopathy often called coats like toreceptor survival, retinal cell transplantation,
disease. calcium channel blockers, neurotrophic
growth factors (CTNF, GDNF, cardiotrophin
MANAGEMENT 1, b FGF, BDNF, topical brimonidine tartrate
0.2%) are various treatment modalities which
Currently there is no definitive treatment for are under trial.
retinitis pigmentosa. The visual prognosis in zz Gene therapy with surgical administration of
advanced cases is usually poor. Various treatment AAV vectors having RPE65C DNA to subretinal
modalities are under trial to slow down the disease space was found to save vision in LCA in an
progression, treatment of complications and animal model. This is an upcoming treatment
improve the visual rehabilitation of RP patients.1-4 under advanced phases of studies in humans.
zz Smoking should be avoided This is likely to benefit LCA/SECORD.
zz Optical aids and light protection: zz Retinal prosthesis like microphotodiode that
—— UV A and UV B blocking sunglasses: capture light and stimulate retina, optic nerve
It reduces the retinal degeneration by and visual cortex are being developed.
reducing short wave length light exposure. zz Psychological and visual rehabilitation is often
Use of CPF 550 lenses filters out 97–99% necessary and patients should be trained to
spectral and UV ray. develop new professional skill.
—— Low vision aids, e.g. magnifiers, closed zz Follow-up: Goldmann visual perimetry and
circuit television are given to people with dilated fundus examination by ophthalmo
decreased central vision. scopy should be done on annually and
—— Wide field bright intensity flashlight biannually. In cases with RP associated with
improves night vision by producing bright CME, more frequent follow-ups are necessary.
wide beam of light. zz Examination of other family members of the
zz Vitamin A palmitate: It is prescribed in a dose affected person is recommended. People
of 15000 IU/day. However, the use is highly who are undergoing family planning should
controversial. Pregnancy is a contraindication have proper genetic counseling regarding the
mode of inheritance and possible affection of • A bite from a Black Mamba and other
the offspring’s and manifestations of RP to snakes
help them taking proper decision. • Mercury poisoning (especially Methyl
mercury).
VIVA QUESTIONS Q.3. Why does bony spicules occur in peri

vascular region? Why there is thinning of
Q.1. Mechanism of glaucoma in RP. blood vessel in RP?
Ans. RP can be associated with both angle Ans. Bony spicules: It is referred to the type
closure and open angle glaucoma. of small cells that are formed during the
• A ngle closure glaucoma: There is formation of new bone matrix. In RP, the
increased zonular instability with appearance of pigment has similar shape
anterior shifting of iris lens diaphragm as spicules in bone.
resulting in angle narrowing and angle Attenuation of vessels: Due to progressive
closure glaucoma. damage of outer retina the vascular
• POAG, NTG and JOAG: attributed to the demand of retina on choriocapillaries and
mutation of gene for retinitis pigmentosa inner retinal arteries decrease.
GTPase regulator-interacting protein 1 Perivascular distribution: In RP due to
(RPGRIP1) on chromosome 14q11. progressive outer photoreceptor and outer
retinal damage the inner retina and the
Q.2. Differential diagnosis of tunnel vision. inner retinal blood vessels lie in contact with
Ans. The loss of peripheral vision with retention RPE. This blood vessel-RPE contact sends a
of central vision gives rise to tunnel vision. trigger signal for bony spicule like pigment
Tunnel vision can be caused by: formation. Progressive attenuation of inner
Common: Glaucoma, retinitis pigmentosa retinal arteries leads to gradual hypoxia
Rare:1-4 and subsequent damage to the cells
• Blood loss (hypovolemia) forming inner blood retinal barrier. The
• Alcohol consumption bony spicule like pigment (along with RPE
• Sustained, i.e. 1 second or more high cells) migrates along the vessels in order to
accelerations, (typically, flying an reform the inner blood retinal barrier. The
airplane with a centripetal acceleration RPE cells seal the vessels with tight junction
of up to or over 39 m/s2 with the head linkage, deposit perivascular extracellular
towards the center of curvature, common matrix and induce fenestrations in the
in aerobatic or fighter pilots) vascular endothelium of the cuffed vessels
• Hallucinogenic drugs (dissociative)/ that leads to the characteristic “spicule”
extreme fear or distress/intense physical pattern.1-4
fight/excitement or extreme pleasure
Q.4. Bionic eye in RP.
(causing a surge of adrenaline in the
Ans. In RP patients who are blind several
body)
prosthesis prototypes have been tested
• Altitude sickness/hypoxia in passenger
that electrically stimulate the inner retina.
aircraft/exposure to oxygen at a partial
These devices can induce phosphenes
pressure above 1.5–2 atmospheres
and can improve performance on some
(producing central nervous system
tests of visual function. However, it needs
oxygen toxicity, called narcosis)
a functional optic nerve and its role in RP
• Pituitary tumors (or other brain tumors
with optic atrophy is unclear.
that compress the optic chiasm)
• A dvanced cataract, during the aura Q.5. Stem cell transplant.
phase of a migraine, intense anger (due Ans. Several studies have demonstrated in vitro
to the body being rapidly flooded with differentiation of embryonic and adult
adrenaline and oxygen) stem cell into retinal cell types. The in vivo
Retina 251
transplantation of these cells as well as fetal

Digenic (very rare): Inherited in pseudo
retinal cells to animal models has given dominant pattern. Simultaneous presence
promising results. Some studies of stem cell of PRPH2 and ROM1.
transplantation on individuals with RP have Prognosis:
shown improvement of visual function. • Best prognosis: AD.
Q.6. Can an RP patient be legally blind inspite • Worst prognosis: XR.
of having good central vision? • AD>Sporadic>AR>XR
Ans. Yes, visual field of <10° is considered as MC mode of inheritance:
blind even when the visual acuity is 6/6. • Sporadic/simplex (40–50%)
Q.7. Role of multifocal ERG. • AD (15–25%)
Ans. Records local response from the macula • AR (5–20%)
and is able to detect residual macular • X-linked (5–15%)
functions in advanced cases. Thus, in cases • Digenic (very rare).
with advanced RP, long-term follow-up and
Q.10. Type of Sunglasses preferred in RP
monitoring of visual function can be done
with multifocal ERG. Ans. Some clinicians prefer orange photo
chromic sunglasses with tinted side shields
Q.8. Full field ERG. or dark amber sunglasses with tinted side
Ans. It stimulates the full visual field and shields. However, no particular type of
records response from the entire retina. It sunglasses has been shown to delay the
is uses traditionally to follow the disease progression of the disease. Sunglasses
progression in RP. should be selected for outdoor use that
Q.9. Genetics and RP. provide maximal comfort to the vision
The different genes associated with RP are without compromising vision.
described below.1-4
Q.11. Causes of completely extinguished ERG.
AD RP: These are mildest forms with some
cases occurring after age 50. RHO (most Ans. • Leber’s congenital amaurosis
common), PRPF 31, PRPH2, RP1, IMPDH1, • Severe retinitis pigmentosa
PRPF8, KLHL7, NR2E3, CRX, PRPF3, • Retinal aplasia
TOPORS, CA4, NRL, ROM1, RP9, RDH12, • Total detachment of retina
SNRNP200, AIPL1, BEST1, PRPF6, RPE 65, • Ophthalmic artery occlusion
GUCA1B, FSCN2, SEMA4A. • Advanced siderosis.
Sporadic: May have favorable prognosis.
Retention of central vision until 6th decade REFERENCES
or later.
1. Fahim AT, Daiger SP, Weleber RG (1993)
AR RP: Usually start in the first decade. Retinitis pigmentosa overview. In: Pagon
USHA2A (most common), ABCA4, PDE6A, RA, Adam MP, Ardinger HH, Wallace SE,
PDE6B, RPE65, CNGA1, BEST1, C20RF71, Amemiya A, Bean LJH, Bird TD, Fong CT,
C80RF37, CLRN1, CNGB1, DHDDS, Mefford HC, Smith RJH, Stephens K, (Eds).
FAM161A, IDH3B, IMPG2, LRAT, MAK, SourceGeneReviews® [Internet]. Seattle (WA):
MERTK, NRL, PDE6G, PRCD, PROM1, University of Washington, Seattle; 1993-2016.
RBP3, RGR, RHO, RLBP1, RP1, SPATA7, 2000 Aug 4 [updated 2013 Mar 21].
TTC8, TULP1, ZNF513, ARL6, NR2E3, EYS, 2. Brad Bowling. Kanski’s Clinical Ophthalmology:
CRB1, CERKL, SAG. A Systematic Approach, 8th edition. Edinburgh:
Elsevier, 2015.
X-linked: Early onset and frequently 3. Albert DM, Miller JW, and Azar DT. Albert
associated with myopia. Least common, but & Jakobiec’s principles and practice of
most severe, usually complete blindness ophthalmology, 2008.
by 3rd or 4th decade. In most of the cases, 4. Hartong DT, Berson EL, Dryja TP. Retinitis
transmission is recessive. RPGR, RP2. pigmentosa. Lancet. 2006;368:1795-1809.
MACULAR HOLE
Vaishali Ghanshyam Rai, Brijesh Takkar

A macular hole (MH) is an anatomic discontinuity The formation of a MH typically evolves over a
of the neurosensory retina that develops in the period of weeks to months. However, it is fre
center of the macula or fovea. Idiopathic macular quently detected when the patient’s symptoms
hole is the most common type seen in clinical change relatively abruptly. Thus, the onset can be
practice. Typically, the patient presents with subacute or acute and the visual acuity progres
metamorphopsia and decreased visual acuity. sively deteriorates. Typically, metamorphopsia
In examinations, MH can be given as a long case would precede significant visual loss.
and needs elaborate work up. Most discussion is
around full thickness idiopathic MH. History of Past Illness
HISTORY Any preceding history of trauma, ocular surgery,

sun or eclipse gazing or use of myopic glasses
Epidemiology/Demography must be recorded. This will help in identifying the
The incidence of MH ranges between 7 and 9 cause of MH. Other eye history may be pertinent
eyes per 100,000 people per year.1-4 Females are in indicating interface anomalies. There may
affected more than males (approximately 2:1). be history of diabetic retinopathy (DR), retinal
Most cases occur in 6th to 7th decade. However, vein occlusion (RVO), or radiation exposure in
young myopes can present in 3th decades of life, secondary cases. History of similar complaint in
as do patients with traumatic MH. The 5-year risk the fellow eye must be enquired, as MH is bilateral
of a patient with a full-thickness macular hole in 10–15% of the cases. Medication use that may
(FTMH) of developing an FTMH in the fellow eye be related to macular cystoid edema (e.g. systemic
is approximately 10–15%.1-4 Rarely, they are with niacin, topical prostaglandin analogs) should be
simultaneous presentation. asked. Lastly, history of glaucoma, cataract or
ARMD must be ruled out as these may coexist with
Chief Complaints
MH and may account for the poor visual acuity.
A case of MH can present in following manners:
zz Asymptomatic-early stages of MH are Systemic History
asymptomatic, especially if the other eye is
normal. Such cases may be detected during Since most of the cases occur in 60–70 years of
fundus examination for other causes, most life coexisting systemic diseases such as Diabetes
commonly cataract due to the age group mellitus (DM), hypertension, bronchial asthma or
affected. any posture-related issues (cervical spondylitis,
zz Late-stages of MH can present with signifi kypho-scoliosis) must be checked. Following MH
cantly reduced visual acuity (VA), metamor surgery the patient may be advised certain posture
phopsia, and loss of central vision with a for few days, hence posture-related problems must
central scotoma. VA is inversely correlated be enquired carefully.
with the size of the MH.
zz Symptoms of vitreo-macular tension (VMT)
EXAMINATION
and vitreous traction—may precede or be
the only presentation in early cases of MH. Systemic Examination
These symptoms include metamorphopsia
(distorted VA), micropsia (a diminution of As discussed earlier coexisting systemic diseases,
objects within the visual field), and photopsia due to the age factor, must be ruled out. In addition,
(flashes of light). any posture-related issues must be looked for.
Retina 253
Ocular Examination zz Typically include central vision loss (with

visual acuity typically measuring 20/25 to
Visual acuity (VA): VA measurement, especially
20/60) and metamorphopsia.
best corrected (BCVA) is extremely important in
cases of MH. A BCVA <6/60 or 20/200 is rare in Stage 2:
MH and in presence of such low acuity other zz Macular hole represents the progression
causes of loss of VA (such as ARMD, cataract or of a foveal pseudocyst to a full thickness
glaucoma) must be ruled out. Poor BCVA is also an dehiscence
indicator of poor prognoses. zz Small opening in the inner layer (<400 µm
Examination should cover all the aspects of diameter) may be either centrally or
the external eye and intraocular structures should eccentrically located
be done to look for secondary causes of MH. Pupil zz Visual acuity 20/25 to 20/80
dilation is of essence for further management. zz May have partially detached operculum.
Lens: MH surgery invariably leads to cataract Stage 3:
formation (almost 100%) hence many surgeons zz Macular hole is a fully developed hole
prefer to perform the cataract surgery during (>400 µm diameter), typically accompanied by
vitrectomy itself. Presence of PSC is especially a rim of thickened and slightly elevated retina.
hampering during the macular surgery. In zz Visual acuity may range from 20/100 to 20/400.
addition, cataract and MH may coexist and few zz Posterior hyaloid is detached from the macula
cases may benefit from only cataract surgery and but remains attached to optic disc.
observation. zz A detached operculum is present on the
posterior hyaloid over the hole and is visible
Posterior segment: Slit-lamp biomicroscopy is clinically or by means of optical coherence
the best technique to evaluate the MH (Fig. 1). tomography.
The MH progresses characteristically through four zz A cuff of subretinal fluid may be detected along
stages (Gass classification).1-4 The clinical findings with intraretinal edema and cysts.
of these stages are described below: zz Drusen-like or yellow deposits may be
Stage 1: Impending macular holes occasionally seen in the base of the hole.
zz Stage 1A: Loss of the foveal depression These deposits represent macrophage activity
associated with a small yellow spot (foveal at the level of the retinal pigment epithelium,
pseudocyst) suggesting chronicity of disease. These
zz Stage 1B: Loss of the foveal depression typically have poor outcomes.
associated with a small yellow ring zz A rim of retinal pigment epithelium hyper/
hypopigmentation is often present at the
junction between edematous or detached
retina and normal-appearing attached retina
in long-standing cases.
zz Epiretinal membranes may be present.
Stage 4
zz A full-thickness hole with a diameter usually
larger than stage 3 (>400 μm in diameter).
zz A complete posterior vitreous detachment
with a Weiss ring.
zz A cuff of subretinal fluid, intraretinal edema,
and cystoid changes are usually present.
zz Drusen-like deposits may be occasionally seen
in the base of the hole.
Fig. 1: Fundus photograph showing old macular hole zz Epiretinal membranes are more frequent.
of the left eye. Large diameter along with pigment zz Visual acuity is more profoundly decreased to
changes the base and disc pallor are noticeable 20/100 to 20/400.
Following macular examination, periphery of retinal vascular tortuosity and compression

must be examined for presence or squeal of and absence of rim of subretinal fluid. Watzke-
anomalous PVD. These patients commonly Allen test and red beam tests are negative in
have VR problems as pathogenesis revolves pseudomacular hole.
around anomalous vitreous traction. In addition,
peripheral lesions would need attention during Lamellar Macular Holes
the surgery, as these patients are especially prone
These are sharply circumscribed, partial-thickness
to peripheral iatrogenic breaks as well. Signs of
defects of the macula usually seen following
trauma or other secondary causes may also be
chronic cystoid macular edema or an aborted
picked up.
FTMH. In contrast, in FTMH a “well” is noted
Traumatic hole: Apart from other history and signs depicting boundaries of the hole. A flat, reddish
of trauma, these holes typically have irregular/ hue-type lesion with intact outer retinal tissue
ragged borders secondary to contusion necroses, characterizes a lamellar macular holes (LMH).
irregular margins and shape, larger (usually Unlike true FTMH, they do not have subretinal
>1000 μm) with underlying pigmentary changes, fluid, drusen-like yellowish deposits in the base
and PVD may be absent. Surrounding retina may of the hole or operculum. Watzke-Allen and laser
appear atrophic. aiming beam tests are negative. LMH do not
progress to full-thickness lesions.
Diagnostic Tests (Help in Differentiating Other lesions that may confuse with MH
Lamellar Holes) includes following:
zz Vitreomacular traction syndrome
Watzke-Allen Test zz Foveal drusen
This test is performed at the slit-lamp biomicro zz Choroidal neovascular membrane
scope by using a fundus/macular lens and placing zz Solar retinopathy
a narrow vertical slit beam through the center of zz Central serous chorioretinopathy
macula. In a FTMH a positive test is noted, that is zz Macular atrophy
the patient detects a break in the bar of light. zz Macular hemorrhage.
All these lesions can be easily differentiated, as
The Laser Aiming-beam Test the characteristic findings of FTMH are absent. In
doubtful case, OCT often confirms the diagnosis.
This is similar to Watzke-Allen test. It is performed
similarly using a macular/fundal lens and placing
a 50-μm laser (Helium Neon) aiming beam INVESTIGATION
through the center of macula. In presence of Optical Coherence Tomography
FTMH, a positive test is observed when the patient
cannot detect the aiming beam within the hole Gold standard tool for diagnosing, staging,
area but is able to detect it in surrounding intact prognosticating, planning for macular hole
tissue. This test is possibly more sensitive and more surgery and following up later (Fig. 2). Several
specific for FTMH. indices have been described based on OCT that
often helps in predicting the prognosis of surgery.
These are described below:1-5
Amsler Charting zz MH minimum diameter also known as the
It is useful in early stages of holes to document clini minimum linear dimension of MH—a smaller
cal progression also to document metamorhopsia. minimum diameter is associated with better
postoperative visual acuity, irrespective of the
DIFFERENTIAL DIAGNOSIS presence/absence of a statistical significance,
eyes with MHs smaller than 400 μm tended to
Pseudomacular Holes have greater visual acuity improvement.
Pseudomacular holes associated with epiretinal zz The basal hole diameter is a linear dimension
membranes can be differentiated by the presence of MH at the level of the retinal pigment
Retina 255
several studies. The visual outcomes is better

in patients with MHI value ≥ 0.5 compared to
a value of <0.5.
zz Diameter Hole Index (DHI): It is calculated by
the ratio of minimum hole diameter and basal
hole diameter. It indicates tangential traction
strength.
zz Tractional Hole Index (THI): It is defined as
the ratio of the hole height to the minimum
diameter. It is another OCT index useful as
a predictor for visual outcome. It indicates
anteroposterior VMT. Larger THI indicates
Fig. 2: EDI OCT of a right eye macular hole showing a stronger AP VMT & weaker tangential traction
large full thickness hole with cystoid spaces and hence a better outcome.
Remember Min hole Diam of <310 μ and THI
epithelium layer. The smaller the basal hole >1.4 are associated with good visual outcome
diameter becomes, the better the postoperative following MH surgery.1,5
visual acuity.
zz Hole form factor (HFF) is the first calculated Fluorescein Angiography
OCT index used as a prognostic factor. The
HFF is the quotient of the summation of the left Fluorescein angiography (FFA) may be a useful
and right arm lengths divided by the basal hole test in differentiating macular holes from
diameter. The HFF is reported to be positively masquerading lesions, such as CME and choroidal
correlated with the postoperative visual acuity. neovascularization (CNV). Full-thickness stage 3
HFF > 0.9 indicates better prognoses. holes typically produce a window defect early in
zz MH height : The hole height is another the angiogram and do not expand with time. No
preoperative OCT parameter, defined as the leakage or accumulation of dye is observed as
greatest distance between the retinal pigment opposed to other lesions.
epithelium layer and the vitreoretinal interface.
Previous studies concluded that there is no TREATMENT
significant relationship between the hole The treatment depends upon the stage of the
height and postoperative visual outcomes, disease (Table 1).1-5
with the exception of one retrospective study zz Stage 1 can be followed up regularly. Only
showing a negative correlation between the about 50% of early stages progress to an FTMH.
hole height and visual acuity more than 5 years In a small number of cases, VMT resolves
after MH surgery. spontaneously without intervention (reported
zz The photoreceptor inner segment/outer incidences are 10–11%).
segment (IS/OS) junction line (now termed the zz Stages 2, 3 and 4 require surgical intervention.
EZ): It is recognized as a hyper-reflective band zz Patients with very good visual acuity may be
by spectral domain OCT imaging. There are followed up.
studies reporting that the preoperative IS/OS zz MIVI-TRUST trials: These have found up to
junction defect length is associated with the 40% success with ocriplasmin for MH closure.
postoperative macular sensitivity and visual However, results are still in initial phase of
acuity. study.
zz Similarly ELM and COST have also been used
as markers for visual prognostication in MH. Surgery for Macular Hole
zz Macular Hole Index (MHI): The MHI is defined
as the ratio of the hole height to the basal Indication
hole diameter and is reported to be positively Life style hampering metamorphopsia or signifi
correlated to the postoperative visual acuity in cant visual acuity loss, usually taken as less than
Table 1 Management of macular hole

Stage Management Follow-up
1-A and Observation 2–4 months with prompt return if new symptoms develop
1-B Monitoring with Amsler grid
2 Vitreoretinal surgery Depending on the outcome of surgery and the patient’s clinical course
Vitreo-pharmacolysis Follow-up at 1 week and 4 weeks, or with new symptoms (i.e. retinal
detachment symptoms)
3 and 4 Vitreoretinal surgery Depending on the outcome of surgery and the patient’s clinical course
6/12 is considered to be the criteria for surgery fiber layer loss during removal. ILM peeling can
by most clinicians. Cautious decisions has to be be done using indocyanine green (ICG), trypan
taken in one eyed patients measuring the risk blue (TP), brilliant blue (BB), and triamcinolone
benefit ratio. Stage 1 holes are usually kept under acetonide (TA), to optimize visualization of
observation. the ILM during surgery. ICG was used initially,
reports of visual field defects and retinal pigment
Aim epithelium abnormalities in the foveal center
Surgical repair of macular holes include relief of all raised concerns for possible toxicity. Importantly,
tangential traction and AP traction, to use vitreous when the surgeon prefers ICG to stain the ILM,
substitute or reverse ILM flap for allowing the the lowest possible concentration with correct
repair process to heal the hole. osmolarity of ICG should be used. Currently ICG
and infracyanine green have fallen out of favor due
Technique to toxicity and stringent use conditions. Trypan
blue was commonly advocated, it being a “real
A standard pars plana vitrectomy (PPV) with ILM vital dye” staining the ERM more than an ILM.
peeling with gas injection is the most commonly However, it required injection under air and a wait
procedure done. Three sclerotomies are created period of 5–8 mins for proper staining. Next BBG
3.5 mm from the limbus for infusion cannula, was introduced which is more active in staining
illumination pipe and vitreous cutter. Using active ILM, though it may also stain ERMs. Steroid
aspiration (150–250 mm Hg), a silicone-tipped crystals typically attach to hyaloids/ERM, rather
suction cannula/vitreous cutter, using suction than actual staining. Combination of Trypan Blue
only is used very effectively for PVD induction— and BBG have been used, and pegylated BBG is
“fish strike sign” or “diving rod sign”. Once the heavier than BBG and may offer better staining.
vitreous is completely detached, vitrectomy is Blood has also been used in combination with
completed. another dye or as sole agent for this purpose.
“Negative staining” is useful in cases where both
Internal Limiting Membrane Peeling ERM and ILM are planned to be removed.
Several studies have reported excellent macular Peeling is carried out using the ILM forceps.
hole closure rates (87–96%) using internal limiting Pinch and peel technique is commonly applied.
membrane (ILM) peeling techniques.1 The ILM act Peel the ILM across the hole to relieve all traction
as a scaffold for cellular proliferation or attachment at the edge of the hole. Few surgeons prefer peeling
of contractile tissue elements that may cause from arcade to arcade. Adequate sized peel is
persistent traction. Thus, failure of the original debated, for usual stage 2–3 holes, I disc diameter
surgery or late reopening of initially successfully (DD) peel usually suffices. For larger holes, up to
closed holes may occur without removal of the 2 DD peel may be done. Total fluid-air exchange
ILM. However, the limitation of ILM peeling is loss is performed, nonexpansile concentration of gas
of its structural role or secondary collateral nerve is exchanged for air.
Retina 257
Postoperatively, prone position is advised for VIVA QUESTIONS AND ANSWERS

3–5 days. There is no clear consensus regarding
duration of facedown positioning to seal macular Q.1. What is stage 0 macular hole?
holes following vitrectomy surgery, but longer Ans. An abnormal vitreofoveal traction observed
positioning may be required for holes larger than on OCT in the contralateral eye of a patient
400 μm or those with inadequate tamponade. with a macular hole is associated with an
Post-ILM peeling OCT features: These may be elevated risk of macular hole formation
early or late. Early features include dimpling of in the contralateral eye. May be present in
inner retina, double or split in nerve fiber layer. extremely high number of contralateral eyes.
Late changes include thinning of retina, ganglion
Q.2. What is a lamellar macular hole?
cell layer and nasalization of fovea.
Ans. A hole in the macular region that is not
full thickness. It may be outer or inner.
Common Complications It may also be an early stage towards
full thickness hole, or an aborted full
zz Nuclear sclerotic cataracts: Almost all cases
thickness hole that may spontaneously
(80–98%) develop cataract within few years of
close. Inner holes usually occur secondary
surgery.1-5 In addition, a closed macular holes
to rupture following CME, like in uveitis
may reopen after cataract surgery and cystoid or DR. Outer holes are seen typically after
macular edema after surgery further increases solar retinopathy, macular dystrophy,
the risk by seven-fold. Thus, some surgeons PFT etc. ERMs typically accompany inner
advocate combining macular hole surgery holes, surgery is controversial and usually
with phacoemulsification and placement of an reserved for few cases.
intraocular lens.
zz Peripheral retinal breaks: Occurs in 3–17% Q.3. Prognosis factor for macular hole.
of macular hole surgeries and most occur Ans. Following are prognostic factors for
inferiorly.1-4 macular hole surgery:
zz Rhegmatogenous retinal detachment: Most • B etter closure rates and better final
visual acuities have been reported when
series report an incidence of 1–5%. The
the duration of symptoms is less than 6
detachment is typically located inferiorly
months.
and caused by tears at the posterior vitreous
• Patients whose macular holes fail to seal
base.1-5
after the first surgery usually have a less
zz Visual field defects: The reported incidence
favorable visual acuity outcome when
is 10–20% of patients. Most believe that this
compared with primary closure.
field loss is caused by either mechanical injury
• Hole size >400 μm is associated with
(such as trauma to the peripapillary retinal
poor prognosis.
vasculature or nerve fiber layer) or dehydra
• P resenting visual acuity: <6/60 is
tion damage to the retina as a result of air
associated with poor prognosis.
streaming from the temporally placed infusion • O CT indices: Smaller base diameter,
cannula during the air-fluid exchange. smaller inner opening size, shorter
zz Enlargement of the hole and late reopening of minimum linear dimension and larger
the hole occurs in 2–10% of the cases.1,2,5 THI are associated with good visual
zz Endophthalmitis: Endophthalmitis has been outcome and hole closure.
reported rarely. • Traumatic holes and those related to
Success rate: Recent reports on acute (less than secondary pathologies have usually poor
6 months) idiopathic macular holes are showing outcomes.
anatomic success rates from 89% to 100% and Q.4. Controversies of macular hole.
improvement in visual acuity of two or more lines Ans. ILM peeling: The role of internal limiting
in 72–95% of patients.1,3,5 membrane (ILM) peeling its necessity,
pref erred technique, and potential results of PPV for IMH but visual recovery
complications, including toxicity of is quicker.
adjuvant agents are far from definitively
Role of vitreous substitute: The major
established. Removal of the ILM increase function of vitreous substitutes (air/SF6/
the rate of macular hole closure and C3F8/silicone oil) is to segregate the hole
perhaps improve the final visual acuity. from fluid filled vitreous cavity, allowing
Closure rates as high as 88–100% have the healing process to occur. Typically,
been reported. 1-3 There is controversy, short acting substitute like SF6 is preferred.
however, as to whether all sizes of macular
Other techniques (adjunctive or sole):
holes require ILM peeling. IMH of <400 μm These include ILM flap techniques
in diameter may close equally with (pedunculated/detached/multilayered,
and without ILM peeling. In addition, etc.), MH tapping, temporal arcuate
ILM peeling can lead to reduced retinal retinectomy, fluid injection at MH base,
sensitivity and microscotomas. Lastly blood in MH well and others. Such
various dyes used to facilitate ILM peeling techniques are usually reserved for holes
(especially ICG) can lead to retinal toxicity. with poor chances of postoperative closure

Duration of face-down positioning: The or those undergoing second surgeries.
ideal duration of positioning (short vs
prolonged) is not well defined. Most Q.5. Types of hole closure.
vitreoretinal surgeons recommend strict Ans. As per postoperative OCT, U, V and W types
face-down positioning for at least 1 week of closures have been seen. U closure has
postoperatively. However prolonged the best outcomes. Closure, as proved
positioning may be required especially in on OCT, would typically occur by 3 days,
large diameter holes. Most holes close by though visual acuity increases slowly.
1 day, up to 80% by 3 days, as proved with Type 1 closure includes U and V. Type 2
face down positioned OCT. closure (W types) indicates poor visual

Surgical adjuvants: The effect of adjuvants outcome, seen in advanced stages and
on MH closure rate is controversial. Most traumatic holes.
surgeons do not currently use adjuvant Q.6. ILM peeling.
agents in the surgical repair of macular Ans. • Techniques for removal of the ILM
holes. involve establishing an elevated edge of

Role of silicone oil: Few surgeon advocates the ILM and then peeling the ILM from
silicone oil tamponade instead of gas around the macular hole. Establishing an
tamponae for a prolonged effect. However, initial edge may be accomplished with
the visual acuity and closure rates are better the use of a barbed microvitreoretinal
among eyes undergoing surgery with gas blade or with Tano diamond dusted
tamponade compared with silicone oil. scraper or with the use of fine intraocular
The use of silicone oil for postoperative end-grasping forceps to ‘pinch’ and
tamponade can be considered for patients elevate the ILM. Peeling is generally
unable to position or when prolonged carried out using a pinch-peel technique
positioning is required. with fine-tipped forceps.

Combined phacovitrectomy or sequential • The ILM peel is most often performed
vitrectomy and phacoemulsification (during in a circular motion around the hole
the first year following vitrectomy): Since (‘maculorhexis’). The ILM is usually peeled
almost all the patient develop cataract to a radius of approximately one disc
following VR surgery in MH few surgeons diameter around the hole.
advocate phacovitrectomy. However, • It work by: Removing residual adherent
There is no clear evidence that combined vitreous cortex remnants on the ILM
phacovitrectomy affects the long-term surface; removing associated fibro-cellular
Retina 259
Table 2 International Vitreomacular Traction Study Group optical coherence tomography (OCT)-based
anatomic classification system for diseases of the vitreomacular interface (VMI)
Anatomic
state Definition Classification
VMA • Evidence of perifoveal vitreous • By size of By presence of
cortex detachment from the attachment area concurrent
retinal surface. • Focal (≤1500 μm) retinal condi
• Macular attachment of the • Broad (>1500 tions (other
vitreous cortex within a 3-mm μm, associated
radius of the fovea. parallel to macular
• No detectable change in foveal RPE and may abnormalities,
contour or underlying retinal include areas of including ARMD,
tissues dehiscence) RVO and DME)
• Isolated
• Concurrent
VMT • Evidence of perifoveal vitreous By size of By presence
cortex detachment from the attachment area of concurrent
retinal surface • Focal (≤1500 μm) retinal
• Macular attachment of the • Broad (>1500 μm, conditions
vitreous cortex within a 3-mm parallel to RPE • Isolated
radius of the fovea and may • Concurrent
• Association of attachment include areas of
with distortion of the foveal dehiscence)
surface, intraretinal structural
changes, and/or elevation of
the fovea above the RPE, but
no full-thickness interruption
of all retinal layers
FTMH Full-thickness foveal lesion that By size (horizontally By presence or By cause
interrupts all macular layers from measured linear absence of VMT • Primary (initiated
the ILM to the RPE width across hole by VMT)
at narrowest point, • Secondary (directly
not ILM) due to associated
• Small (250 μm) disease or trauma
• Medium known to cause
(>250 μm and macular hole in the
400 μm) absence of prior
• Large (>400 μm) VMT)
LMH • Irregular foveal contour
• Defect in the inner fovea (may not have actual loss of tissue)
• Intraretinal splitting (schisis), typically between the outer plexiform and outer nuclear layers
• Maintenance of an intact photoreceptor layer
Macular • Invaginated or heaped foveal edges
Pseudo- • Concomitant ERM with central opening
hole • Steep macular contour to the central fovea with near-normal central foveal thickness
• No loss of retinal tissue
Abbreviations: ERM, epiretinal membrane; FTMH, full-thickness macular hole; ILM, internal limiting membrane;
IVTS, International Vitreomacular Traction Study; LMH, lamellar macular hole; RPE, retinal pigment epithelium;
VMA, vitreomacular adhesion; VMT, vitreomacular traction; ARMD, age-related macular degeneration; RVO,
retinal vein occlusion; DME, diabetic macular edema
collections; removing the rigid and less are applied to the site of the macular hole
compliant ILM (relative to the retina itself ); in hopes of stimulating a cellular reparative
and causing a retinal glial cell proliferation response and hole closure. Examples
that may help macular hole contraction includes autologous serum, autologous
and repair. platelet concentrate, thrombin-activated
Q.7. Macular hole formation, pathogenesis, fibrinogen, thrombin, transforming growth
tangential traction. factor beta-2 (TGFb2), and tissue glue.
Ans. Gass hypothesised that IMHs begin with Q.9. IVMTS Classification.
tangential traction of the prefoveal vitreous Ans. See Table 2.
cortex, which results in a foveal dehiscence
that progresses from foveolar detachment
to a full-thickness IMH. However, more REFERENCES
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OCT) has elucidated that IMHs are initiated Sadda SR, Wiedemann P. Retina, 5th edition.
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The anterior tractional forces acting at the London: Elsevier/Saunders, 2013.
foveola firstly produce an intrafoveal split, 3. Albert DM, Miller JW, Azar DT. Albert
which evolves into a foveal pseudocyst. & Jakobiec’s principles and practice of
Dehiscence of the foveal cyst creates a full- ophthalmology, 2008.
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the cyst roof is observed by the appearance traction and macular hole: a comprehensive
of an operculum within the vitreous gel.1,2 review of pathophysiology, diagnosis, and
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Q.8. Adjunctive therapy in macular hole S1-S21.
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Ans. The role of adjunctive therapy in macular A Systematic Approach, 8th edition. Edinburgh:
hole repair is controversial. The adjuvants Elsevier, 2015.
RETINAL DETACHMENT
Pranayi Behera, Rajesh Pattebahadur, Raghav Ravani
INTRODUCTION 3. Exudative (serous) retinal detachment: This

results from exudation of material into the
Retinal detachment refers to separation of the
subretinal space (Fig. 3) from retinal vessels
neuro-sensory layers of the retina from the
underlying retinal pigment epithelium (RPE). (as in hypertension, central retinal venous
Retinal detachment occurs by 3 basic mecha occlusion, vasculitis, or uveitis, tumors,
nisms and thus is classified into the following infective disorders, chronic rhinosinusitis etc.)
3 main types (Table 1): The RRDs are usually kept as long cases and
1. Rhegmatogenous retinal detachment (RRD) the subsequent discussion particularly revolves
(the most common type): This results when a around it.
hole, tear, or break in the neuronal layer allows
fluid from the vitreous to seep between and HISTORY
separate sensory and RPE layers (Fig. 1).
2. Traction retinal detachment: This results
Chief Complaints
from adhesions between the vitreous gel/ A case of RRD may present with following:
fibrovascular proliferation and the retina zz Flashing lights
(Fig. 2). zz Floaters
Retina 261
Table 1 Types of retinal detachment

Rhegmatogenous Tractional Exudative
History Photopsia, visual Diabetes, penetrating trauma, Malignant hypertension, eclampsia
field defects sickle cell disease and renal failure, uveitis, tumor
Retinal break Present No primary break. May develop No break or coincidental
secondary break
Extent of Extends to ora Does not extend to ora. Gravity dependant
detachment early
Retinal shape Convex Elevated till level of traction, Extremely high convex
concave
Motility Undulating Taut. Peaks to traction point Smoothly elevated bullae
Subretinal fluid Clear Clear. No shift May be turbid. Shift rapidly to
dependant location.
Choroidal mass None None May be present
PVR + – –
Abbreviation: PVR, proliferative vitreoretinopathy
Fig. 1: Ultrawide field pseudocolor image of Fig. 2: Ultrawide field pseudocolor image of
rhegmatogenous retinal detachment with large tear choroiditis with inferior exudative retinal detachment
zz Visual field defect The sudden onset of large floaters in the center
zz Visual loss. of the visual axis may indicate posterior vitreous
detachment (PVD). The patient observes a circular
History of Present Illness floater when the vitreous detaches from its
annular ring surrounding the optic nerve (Weiss
Photopsia: It includes the sensation of a flashing
ring). More serious is the description of hundreds
light related to retinal traction. Typically described
of tiny black specks or multiple small insects
as sensation of falling stars, even when the eyes are
floating in front of the eye, as this is suggestive
closed or in a dark room. A shower of floaters and
of a vitreous hemorrhage, resulting from tear of
vision loss often accompanies it.
a retinal blood vessel caused by a retinal tear or
Floaters: Floaters are a very common visual mechanical traction of a vitreoretinal adhesion.
symptom in the population; thus, distinguishing A few hours after the initial shower of black spots,
their etiology requires eliciting a detailed history. the patient can note cobwebs that result from
Table 2 Disorders associated with retinal

detachment
Familial vitreoretinal disorders
• Familial exudative vitreoretinpathy
• Goldmann-Favre vitreoretinal degeneration
• Familial retinal dialysis
• Stickler syndrome (types I and II)
• Knobloch syndrome
• Enhanced S-cone syndrome
• Autosomal dominant vitreoretinochoroidopathy
• Wagner disease
• Snowflake vitreoretinal degeneration
Hereditary systemic disorders
Fig. 3: Ultrawide field pseudocolor image of
• Marfan syndrome
tractional retinal detachment • Homocystinurea
• Ehler-Danlos syndrome
• Sickling hemoglobinopathies
blood forming irregular clots. Generally, the new
onset of floaters associated with flashing lights is Other causes of RD
highly suggestive of a retinal tear, full thickness or • Infections
otherwise. • Inflammatory conditions
• Toxoplasma
Field defect: Patient may report a black curtain or • Toxocara
shadow in the peripheral visual field, which, over • Pars planitis
a period of few days, may spread to involve the • ROP
entire visual field. Bullous (i.e. large ballooning)
Abbreviations: RD, retinal detachment;
detachments produce dense visual field defects
ROP, retinopathy of prematurity
(i.e. blackness), and flat detachments produce
relative field defects (i.e. grayness). The visual field
defect can be helpful in guessing the probable tuberculosis, tumors and other syndromes
quadrant of detachment. (Table 2) as applicable in the case.
Visual loss: If the retinal detachment (RD) involves
the central visual area or the macula, the complaint Past Surgical History
is sudden onset painless visual loss. Patient may Following points should be noted:
describe this as cloudy vision. The intensity of zz History of vitreous loss during cataract surgery.
the same depends on height and duration of the zz Previous laser capsulotomy.
macular detachment. zz Intraocular foreign body removal.
History of Past Illness Family History

For RRD, one should inquire regarding family It may be helpful in certain cases of familial
history of retinal disorders or degenerations, RD, where retinal degenerations are hereditary.
myopia or prior retinal therapies. History of past Such cases may also be syndromic, e.g. Stickler’s,
intraocular surgeries should always be identified Marfan’s, Wagner’s etc. (Table 2).
in detail (see further), history of trauma should be
asked on leading questions, especially if suggestive
CLINICAL EXAMINATION
by signs.
Systemic history is very essential for tractional Visual acuity: Check visual acuity at near and
retinal detachment (TRD) and exudative RD. distance, correcting for refractive error should be
One may find history of DM, hypertension, noted. Always look for myopia in the fellow eye.
Retina 263
External examination: For signs of trauma (see transparency. A pigmented or nonpigmented line
chapter on traumatic RD). may demarcate the limit of a detachment.
Anterior segment: Look for signs of trauma, sta Specifically for RRD:
bility of lens barrier/status, and media clarity etc. zz Identify the extent of RD, identify fresh or old,
Uveitis, neovascularization of the iris (NVI) may be identify location, types-numbers of retinal
seen in TRD/exudative RD. breaks, grade of PVR, macular status, presence
Pupil Reaction: A fixed dilated pupil may of risk factors. See viva questions for detailed
indicate previous trauma; a positive Marcus- discussion.
Gunn pupil can occur with any disturbance of the zz For exudative RD and TRD, see the differenti
afferent pupillomotor pathway, including retinal ating features Table 1.
detachment. Relative afferent pupillary defect zz Remember the triad of cardinal signs: RAPD,
(RAPD) is more likely to be seen clinically if the gray reflex and hypotony for presumptive
RD is bullous, >2 quadrants and especially if the diagnosis of RRD.
macula is off. zz A thorough fellow eye examination must
Intraocular pressure: A relative hypotony of be done for risk factors of RD, or for signs of
>4–5 mm Hg less than the fellow eye is common. causative etiology of TRD and exudative RD.
If intraocular pressure (IOP) is extremely low,
choroidal detachment may be present. It may be DIFFERENTIAL DIAGNOSIS
raised in Schwartz-Matsuo syndrome in which zz Posterior uveitis/scleritis
RRD is associated with a mild anterior uveitis zz Posterior vitreous detachment
and due to blockage of the angles by parts of
zz Vitreous Hemorrhage
photoreceptors. This is also seen due to retinal
zz Vitreous syneresis
dialysis due to prior blunt trauma in a young man.
zz Thick hyaloid
Vitreous: Look for signs of pigment or tobacco dust zz Vitreous membranes
(i.e. Shaffer sign), which is suggestive for retinal zz Retinoschisis
tears in 70% of cases with no previous eye disease zz Retinal cyst
or surgery, hence a sign of RRD. Other findings that zz Sub retinal exudates
must be looked for includes VH in TRD, RL cells in zz Other differentials of TRD/Exudation
exudative RD and vitreous degeneration in RRD. zz Retinal mass
Fundus examination: Indirect ophthalmoscopy zz CD.
is the definitive means of diagnosing retinal
detachment. Direct funduscopy may detect INVESTIGATION
vitreous hemorrhage and large detachment of the
posterior pole, but it is inadequate for complete zz USG B-SCAN: In presence of media haze USG
examination because of the lower illumination, helps in differentiating the type of membrane,
lack of stereopsis, and limited view of the rule out IOFB, sub retinal mass, CD etc.
peripheral retina. However, following IO, either zz Systemic investigations as needed
90 D assisted biomicroscopy or direct fundoscopy zz Other eye investigations may depend depend
must be done to assess the macular status. All the ing on complete diagnoses
findings must be recorded in a modified Amsler-
Dubois chart (Fig. 4). MANAGEMENT
Obvious detachment is observed as marked
elevation of the retina, which appears gray due to The management for RRD is usually surgical. In
loss of transparency with dark blood vessels that certain poor prognoses cases, surgery may be
may lie in folds. The detached retina may undulate deferred, while in certain other like subclinical RD
and appear out of focus. Shallow detachments are medical management may be opted for. Broadly
much more difficult to detect; thus, comparing for RRD, the surgical options include: Vitrectomy,
the suspected area with an adjacent normal scleral buckling and pneumatic retinopexy
quadrant is helpful to detect any change in retinal (Table 3). See viva questions as to how to choose
Fig. 4: Modified Amsler-Dubois retinal detachment chart with color coding
for best plan. In all cases, fellow eye treatment layers, hence the potential space. Normally,
must be considered as optimum. the hydrostatic pressure of the fluid
The management for TRD may be observation dynamics, mechanical vitreous pressure
or vitrectomy depending on macular status. In all and glycoprotein matrix between these
cases, efforts should be made for identification layers keeps the retina in position.
of the cause of TRD. For exudative RD, either Q.2. How to identify Ora serrata on exami-
observation or management with steroids is done. nation?
Like TRD, efforts should be made to discern the Ans. Ora is the anterior most limit of the retina.
cause and initiate treatment accordingly. See Dentate process, oral bays, meridional
relevant chapters for further discussion. folds, oral bays are present. The choroid
also ends there with beginning of the pars
VIVA QUESTIONS plana.
Q.1. What keeps the retina attached? Q.3. Define vitreous base.
Ans. Embryologically, RPE and the neurosensory Ans. This is a 3–4 mm wide zone of condensed
retina arise from different neuroectodermal and firmly adherent vitreous cortex
Retina 265
Table 3 Surgery for retinal detachment

Scleral buckling VR surgery Pneumatic retinopexy
Indications • RRD (PVR less than C1) • RD with PVR changes • A detachment caused by a
• Inferior retinal breaks • RD with GRT single break, in superior 8 clock
• Retinal dialysis • RD with vitreous hours
• Pediatric RD hemorrhage • The break should not be more
• RD with intraocular FB than 1 clock hour
• Multiple breaks but in
1–2 clock hours of each other
Contra- • Posterior breaks • Bleeding disorders • Break larger than 1 clock hour
indications • Opaque media • Suspected tumors like or multiple breaks in more than
• Vaso-occlusive diseases RB, melanoma one clock hour
like sickle cell anemia • Break in inferior 4 clock hours
• PVR more than C2 • PVR grade C or D
• Patient not able to maintain
the head position
• Severely uncontrolled
glaucoma or recent cataract
surgery
• Hazy media preventing
adequate visualization of retina
Complications • Perforation • Iatrogenic breaks • Incarceration of vitreous
• Rise in IOP • Lens trauma • Subconjunctival gas
• Extrusion • Re detachment • New or missed breaks
• Infection • Secondary glaucoma • PVR
• Diplopia • PVR • Re-detachment
• Anterior segment ischemia • Cataract progression • Persistent sub retinal fluid
• Extrusion of explants • Re-opening of original break
• Epiretinal membrane (ERM) • Vitreous haze
• Recurrent retinal • Sudden rise in IOP
detachment
Benefits Excellent anatomic results, Visualization of the all In-office procedure, minimally
longevity, good visual tears/breaks, removal invasive, reduced recovery time,
outcomes of opacities/synechiae, better postoperative visual acuity
anatomic success in
complicated detachments
Abbreviations: RD, retinal detachment; RRD, rhegmatogenous retinal detachment; PVR, proliferative
vitreoretinopathy; IOP, intraocular pressure; GRT, giant retinal tear; RB, retinoblastoma
straddling either side of ora. It starts Q.5. What are the normally strong adhesions
around 5 mm from limbus. of vitreous?
Ans. Fovea, vascular arcades, optic disc and
Q.4. Enumerate some retinal degenerations
vitreous base (strongest).
not leading to RD.
Ans. Microcystoid (most common), paving Q.6. What is retinal break?
stone, reticular, WWOP. WWOP is consi Ans. Retinal break is full thickness deficiency
dered by some as an optic illusion. in neural retina. Holes, Horseshoe tears
and retinal dialysis are the types. Scleral mechanically stimulating a sensation of
depression may be needed to identify light. Ocular migraine is a differential
peripheral breaks. diagnosis.
Q.7. Which retinal degenerations are asso Q.9. What is the significance of floaters?
ciated with RD? Ans. • Sudden appearance of one large floater
Ans. Lattice: It is the most important degenera near the visual axis is mostly due to PVD
tion. Though it may be seen in up to 8% (Weiss ring)
of the normal population, it is present • Appearance of numerous curvilinear
in up to 50% of RD, and cause of up to opacities within the visual field indicates
20%. It is bilateral in 50%. It can develop vitreous degeneration
HSTs at posterior border or atrophic • Floaters due to vitreous hemorrhage are
holes. Clinically it is seen as cigar shaped, characterized by numerous tiny black
pigmented or nonpigmented peripheral dots, followed by cobwebs as the blood
circumferential lesion, though it may be forms clots. While single floater has
radial also like in Stickler syndrome. It has low (~15%) risk of having a retinal tear,
hyalinized criss cross-vessels, thin retina multiple floaters have higher risk (70%).
with overlying liquefied vitreous, strongly PVD with VH is particularly ominous.
adherent on its posterior border. Q.10. Why is the intraocular pressure (IOP)
Retinoschisis: This is split in retinal layers. decreased in RRDs?
It may be typical (split in OPL) and reti Ans. An eye with rhegmatogenous RD typically
cular (split in NFL layer). Reticular is has decreased IOP and it is due to the
seen in congenital schisis as the X linked following factors:
retinoschisis syndrome. It is characterized • E arly transient pressure drop may
by bicycle maculopathy, vitreous veils, result from inflammation and reduced
pockmarks, snowflakes and is seen hyper aqueous production. A vicious cycle sets
metropes. Some children may develop up between CD and inflammation.
RD or VH rarely. In contrast, degenerative • Prolonged hypotony may be caused by
schisis is seen elderly, in inferior temporal posterior flow, presumably through a
region and may or may not be bilateral. break in the RPE and even anterior PVR
This develops due to coalescence of and CD.
cystoids degeneration. The retinal breaks
Q.11. What is Schwartz-Matsuo syndrome?
may develop in inner layer or outer layer of
Ans. RRD is typically associated with decreased
schisis. While inner breaks do not lead on
IOP. Schwartz described a condition in
to RD, outer layer breaks singularly, or in
which patient presents with unilateral
combination can lead on to RD and should
intraocular pressure elevation, retinal
be treated. Retinoschisis has an absolute
detachment and open anterior chamber
scotoma in contrast to RD, and diagnostic
angle with ‘cells’ in the anterior chamber.
differentiation is the laser uptake test in
Elevated intraocular pressure is often seen
schsis.
in the evening. The detachment is typically
Snailtrack degeneration: White frost caused by a dialysis at the ora serrata or a
like peripheral degeneration, HSTs are break in the nonpigmented epithelium of
uncommon due to less traction. Some the pars plana or pars plicata of the ciliary
believe it to be lesser form of lattice. body. The elevated intraocular pressure is
Q.8. What are flashes due to? usually discovered incidentally at the time
Ans. The perception of flashes or photopsia is of diagnosis of the retinal detachment, and
due to the production of phosphenes by resolves without specific treatment when
pathophysiologic stimulation of retina. the retina is reattached.
During PVD, as the vitreous separates from The proposed hypothesis for raised IOP:
the retinal surface, the retina is disturbed The cells in the aqueous were infact
Retina 267
photoreceptor outer segments rather than HSTs, tessellated or tigeroid fundus,

inflammatory cells. These fragments are diffuse chorio retinal atrophy etc. None
derived from the rods. The peripheral of these however is specific for myopia.
retinal break allows free communication
Q.16. Which are the systemic conditions asso
between the subretinal space and aqueous
ciated with rhegmatogenous RD?
humor. Outer segments then flow into
the aqueous and obstruct the trabecular Ans. See Table 2.
meshwork. Q.17. Why is configuration of SRF important?
Q.12. Why is IOP raised in certain RD’s? Ans. Because SRF spreads in gravitational
Ans. • Chronic low grade uveitis in RDs damage fashion and its shape is governed by
the trabecular meshwork anatomic limits (ora and optic nerve), it can
• In long standing RDs, Rubeosis iridis be used to locate primary break. Knowledge
(NVI) followed by increased IOP due to of Lincoff’s rules is imminent as these rules
NVG. indicate the location of retinal break.
Q.13. What is “tobacco dusting”? Q.18. What are the factors promoting SRF into
the break?
Ans. • Pathognomonic of RRD
Ans. • Ocular movements
• Present in the anterior vitreous phase
• Gravity
• The cells represent macrophages con
• Vitreous traction, at the edge of the
taining shed RPE.
break
Q.14. What is the incidence of retinal detach • PVD.
ment in myopes?
Q.19. What is Lincoff’s rule?
Ans. 40% of all RDs occur in myopes. The reasons Ans. SRF usually spreads in gravitational fashion
for high myopes to have RRD includes and its shape is governed by anatomical
following: limits and location of the primary retinal
• Increased stretch of the retina over the break. If the primary break is located
bigger eye ball superiorly, SRF first spreads inferiorly on
• Incidence of lattice generation is higher the same side of the break and then spreads
• Incidence of PVD is higher superiorly on the opposite side of the
• Macular hole fundus.
• Vitreous loss during cataract surgery • A shallow inferior RD in which SRF is
• Diffuse chorioretinal atrophy. slightly higher on the temporal side

Q.15. What is high myopia, pathological points to a primary break on that side.
myopia? What are the signs of myopia? • A primary break at 6 o’clock will cause
Ans. A refractive error greater than 6D, or axial inferior RD with equal fluid levels.
length >26 mm are typical of high myopia. • In a bullous inferior RD, the primary
• Pathological myopia refers to occurrence break usually lies above the horizontal
of pathological changes in a high myope, meridian.
the most typical being a posterior • If a primary break is in the upper nasal
staphyloma. quadrant, the SRF will revolve around the
• O ther signs of myopia includes disc optic disk and then rise on the temporal
changes like large, pale disc, high CDR, side until it is level with the primary
temporal crescent, disc pit, disc tilt, disc break.
coloboma; macular changes like MH, • A subtotal RD with a superior wedge
foveoschisis, subretinal hemorrhage, of attached retina points to a 1° break
lacquer cracks, foster fuchs spots, CNVM, located in the periphery nearest its
focal atrophy; peripheral changes like highest borders.
retinal degeneration, lattice, paving • W hen the SRF crosses the vertical
stone, atrophic retinal holes, WWOP, midline above the primary break is near
to 12 o’clock the lower edge of the RD • Height of macular detachment.

corresponding to the side of the break. • A
ge >60 years negatively affect visual
Q.20. What is vitreoretinal traction? restoration.
Ans. It is the force exerted on the retina by Q.24. What are the indications for segmental
structures originating in the vitreous. circumferential buckling?
Types: Ans. • Multiple breaks located in one or
• D ynamic: It is induced by rapid eye 2 quadrants and varying distance
movement, where there is a centri • From ora serrata
petal force towards the vitreous cavity. • Anterior breaks
Responsible for retinal tears and rheg • Wide breaks, dialysis and giant tears.
matogenous RD.
• Static: Independent of ocular move Q.25. What are the indications for encircling
ments and plays an important role buckle (360°)?
in pathogenesis of tractional RD and Ans. • Break involving 3 or more quadrants
proliferative vitreoretinopathy. • Extensive RD without detectable breaks
It may be: particularly is eyes with hazy media
– Tangential: Epiretinal fibrovascular • Lattice degeneration, snail track degen
membranes eration involving 3 or more quadrants
– Anteroposterior traction: Contraction • Along with vitrectomy
of fibrovascular membranes • Multiple breaks
– Bridging (trampoline) traction: Con • Pseudophakia, aphakia.
traction of fibrovascular membranes, Q.26. What are the steps in scleral bucking
which stretch from one part of the surgery?
posterior retina to another or between Ans. • Preliminary examination
vascular arcades, which tends to pull • 360° peritomy
the 2 involved points together. • Traction (bridle) sutures around the recti
Q.21. How do you differentiate between the • Inspection of sclera
three types of RD? • Localization and marking of the break
Ans. See Table 1. • Cryotherapy
• Scleral buckling
Q.22. Differentiate clinically between CD and • Drainage of SRF
RD • Intravitreal air or BSS injection followed
Ans. • RD is gray, CD is brown. by reinspection
• RD has undulating motions, while CD • Closure of the peritomy.
may have “jiggly” movements.
• USG shows the typical double peaked Q.27. What are the indications for subretinal
sign of a membrane arising from equator fluid (SRF) drainage?
till ora. Ans. • Difficulty in localization of retinal breaks
in bullous detachments
Q.23. What are the factors governing visual
• Long standing RD as SRF is viscous
function following surgical reattach-
• Bullous RD
ment?
• As part of DACE procedure
Ans. • Macular involvement: If the macula has
• Glaucomatous cyclitis
been involved the prognosis is poorer.
• Resurgeries.
• Duration: Typically a macular detach
ment <7 days is believed to have good Q.28. What are the methods of SRF drainage?
visual prognoses. The patient may Ans. Prang: Here digital pressure is applied
achieve the pre-RD visual acuity. till central retinal artery is occluded and
Detachment beyond 10 days usually had choroidal vasculature is blanched. Then
poor outcomes. full thickness perforation is made with
Retina 269
27-gauge hypodermic needle to drain SRF. • S

ub retinal bleed. Just after drainage, IO
Air is injected to form the globe. must be done to rule this out. If present,
Cut down: Radial sclerotomy is made immediate build of IOP with head tilt
beneath the area of deepest SRF. Mattress must be done. If still bleed reaches
suture may be placed across the lips of the beneath macula, gas injection with
sclerotomy. Prolapsed choroidal knuckle positioning or immediate vitrectomy
is examined with +20D lens for large should be considered.
choroidal vessels. After ruling this out, Q.34. What are the indications for internal
light cautery is applied to knuckle to avoid tamponade in scleral buckling?
bleeding and knuckle is perforated with
Ans. • Superior break
25-gauge hypodermic needle.
• Hypotony
Q.29. What are the advantages of SRF drainage? • Retinal folds
Ans. It provides immediate contact between • Fish mouthing
sensory retina and RPE with flattening • Posterior breaks
of the fovea. If this contact is delayed, the • Sub retinal bleed
stickiness of RPE wears off and adequate • DACE procedure.
adhesion may not occur, resulting in Q.35. Who are the best candidates for pneu
nonattachment of retina. matic retinopexy?
Q.30. What are the precautions taken before Ans. • A detachment caused by a single break,
drainage of SRF? in superior 8 clock hours
Ans. • Examine the fundus to make sure, SRF • T he break should not be more than
has not shifted 1 clock hour
• Avoid vortex vein • Multiple breaks but in 1–2 clock hours of
• IOP should not be elevated (it may cause each other
retinal incarceration). • Free of systemic disease (rheumatoid
Q.31. How to choose site for drainage? arthritis) (who can maintain position)
Ans. Most dependent retinal area, horizontal • Phakic patients
median as it is generally devoid of vortex • Total PVD.
vessels, just behind muscle insertions, Q.36. What are the principles of pneumo-
preferably nasal site to avoid macular retinopexy?
complications. Superior site should be Ans. Intraocular gases keep the retinal break
avoided for the risk of macular bleeds. Cryo closed by the following properties:
sites and break sites to be avoided, draining • Mechanical closure and thus RPE pump
under planned buckle area is suitable. removes excessive SRF
Q.32. How do you know that SRF drainage is • Surface tension
completed? • Buoyancy.
Ans. By the presence of pigments. Q.37. What are the substances used as vitreous
substitutes in RD surgery?
Q.33. What are the complications of SRF
Ans. • Intraocular gases:
drainage?
– Nonexpansile: Air, SF6: Air mixture,
Ans. • Choroidal hemorrhage
C3F8: Air mixture
• Ocular hypotony
– Expansile: SF6, C3F8 (Table 4)
• Iatrogenic break
• SILICON OIL
• Retinal incarceration
• Perfluorocarbon liquids (PFCL)
• Vitreous prolapse
• Damage to long posterior ciliary arteries Q.38. What are different surgical option for
and nerves RRD and compare them?
• Endophthalmitis Ans. See Table 1.
Table 4 Commonly used vitreous substitute

Gas Expansion Non-expansible conc. Average duration Volume used for PR
SF6 2x 20% 10–14 days 0.5 mL
C3F8 4x 12% 30–45 days 0.35 mL
Air Non-expansible – 5–7 days 0.8 mL
Q.39. What is the difference between fresh and Table 5 Features of fresh and old RD
old RD?
Ans. See Table 5. Fresh RD Old RD
• Loss of choroidal pattern • Demarcation lines
Q.40. What is the pathogenesis of traumatic • R etina: Convex • Immobile retina
RD? configuration, corrugations • Very thin atrophic
Ans. See chapter on traumatic RD. • Fluid extending up to ora retina
Q.41. How to draw an RD chart? serrata • Secondary intra-
• Slightly opaque with dark retinal cysts
Ans. See Figure 1.
blood vessels • Tobacco dust
Q.42. What are disadvantages of silicone oil? • Moves freely with eye • Advanced PVR
Ans. The major disadvantage is need for a movements
second surgery for removal. Complications Abbreviations: PVR, proliferative vitreoretinopathy;
include emulsification of oil causing media RD, retinal detachment
opacification, glaucoma, corneal edema,
BSK, cataract amongst others. It has been
noted that aphakic patients do not do well Table 6 Retinal society proliferative
with long-term silicone oil. The silicone vitreoretinopathy classification
oil study suggested use of oil in children Grade
and other patients non-compliant to (Stage) Characteristics
positioning and in eyes with large breaks A Vitreous haze, vitreous pigment clumps
and hypotony. Otherwise, results with C3F8 B Wrinkling of the inner retinal surface,
were comparable. rolled edge of retinal break, retinal
Q.43. What is PVR? stiffness, vessel tortuosity
Ans. Proliferative vitreoretinopathy: This is de- C Full-thickness retinal folds in
differentiation followed by proliferation, C-1 One quadrant
migration and then fibrotic metaplasia C-2 Two quadrants
of progenitor cells like RPE cell, glial cells
C-3 Three quadrants
or muller cells. The most common theory
states that RPE cells exposed to vitreous D Fixed retinal folds in four quadrants
as in an open break are responsible for D-1 Wide funnel shape
the same. They migrate to form epiretinal D-2 Narrow funnel shape (anterior end of
membranes and sub retinal bands. RD is funnel visible by indirect ophthalmoscopy)
not a prerequisite of PVR and it may form in D-3 Closed funnel (optic nerve not visible)
attached retinas as well.
Q.44. What are classification systems for PVR? Q.45. How can PVR be managed?
Ans. Three important classification systems Ans. Medical management for prevention
include retina society classification, includes use of steroids, antineoplastic
silicone oil study classification and then drugs like daunorubucin, use of heparin
the updated retina society classification and other drugs like tetracyclines.
system. See Tables 6 to 9. These have not proven to be beneficial.
Retina 271
Table 7 Silicone study classification system to Surgical management peeling of mature

proliferative vitreoretinopathy membranes, removal of sub-retinal
membranes, using PFCLs and performing
Grade Features relaxing procedures like retinotomies and
A Vitreous haze, vitreous retinectomies.
pigment clumps
Q.46. Discuss prophylactic management of RD.
B Inner retinal wrinkling, rolled Ans. Normally, HSTs should always be
edge of retinal breaks treated. As per theory holes, other lattice
CP degenerations need to be treated only if
there is symptomatic PVD or it is fellow eye
P1: 1 quadrant Starfolds and/or diffuse
(1–3 clock hours) contraction in posterior of a patient. Patients with family history,
retinal and/or subretinal high myopia, undergoing cataract surgery
P2: 2 quadrants or any other predilection need to be
(4–6 clock hours) membrane in posterior retina
P3: 3 quadrants
(7–9 clock hours) Table 8 Updated proliferative vitreoretinopathy
grade classification by machemer
P4: 4 quadrants
(10–12 clock hours) Grade Features
CA Circumferential and/or A Vitreous haze, vitreous pigment clumps,
perpendicular and/or anterior pigment clusters on inferior retina
traction in anterior retina B Wrinkling of inner retinal surface, retinal
A1: 1 quadrant stiffness, vessel tortuosity, rolled and
(1–3 clock hours) irregular edge of retinal break, decreased
mobility
A2: 2 quadrants
CP 1–12 Posterior to equator, focal, diffuse, or
(4–6 clock hours)
circumferential full-thickness folds,
A3: 3 quadrants subretinal strands
(7–9 clock hours)
CA 1–12 Anterior to equator, focal, diffuse, or
A4: 4 quadrants circumferential full-thickness folds,
(10–12 clock hours subretinal strands, anterior displacement
Table 9 Silicone study classification of contraction type in proliferative vitreoretinopathy

Type Contraction Location
number type of PVR Summary of clinical signs
1 Focal Posterior Star fold
2 Diffuse Posterior Confluent irregular retinal folds in posterior retina; remainder of
retina drawn posterior; optic disc may not be visible
3 Subretinal Posterior “Napkin ring” around disc, or “Clothesline” elevation of retina
4 Circumferential Anterior Irregular retinal folds in the anterior retina; series of radial folds more
posteriorly; peripheral retina within vitreous base stretched inward
5 Perpendicular Anterior Smooth circumferential fold of retina at insertion of posterior hyaloid
6 Anterior Anterior Circumferential fold of retina at insertion of posterior hyaloid pulled
forward; trough of peripheral retina anteriorly; ciliary processes
stretched with possible hypotony; iris retracted
informed regarding the same, before opting All membrane removal is done before fluid
for treatment. Methods of prophylaxis may air exchange and SRF drainage.
be laser delimitation or cryotherapy.
Q.49. What are causes of pediatric RD?
Q.47. What is subclinical RD? Ans. Trauma, ROP, FEVR, hereditary syndromes,
Ans. It is a break surrounded by minimal high myopia, coloboma, coats disease,
SRF. Different definitions may be found. IOFB, previous surgery etc.
One definition says SRF <1 DD around
a break no part of which is posterior to Q.50. What are causes of failed surgery?
equator. While another definition says SRF Ans. Major causes include missed/new retinal
<2 DD, no part of which is >1 DD posterior breaks and PVR. Additionally in buckling
to equator. Such patients can be managed optimum break buckle relationship and
by walling off the RD till ora or by laser in vitrectomy optimum tamponade and
delimitation around the SRF. Cryo may be positioning is required. Poor drainage
done in selected cases. and poor retinopexy are other causes of
failure or relapses.
Q.48. What are basic principles of vitrectomy
for RD?
Ans. These include performing a central BIBLIOGRAPHY
vitrectomy, inducing PVD and complete 1. Azad R, Azad SV, Takkar B. Basics of Vitrectomy.
peripheral vitreous dissection followed New Delhi; 2016.
by SRF drainage. This is followed by 2. Kanski and bowling. Clinical Ophthalmology.
retinopexy around all retinal breaks and 7th edition. Chapter 16.
finally a vitreous substitute is inserted. 3. Ryan SJ. Retina. 5th edition Volume 3.
AGE-RELATED MACULAR DEGENERATION

Shipra Singhi, Raghav Ravani, Brijesh Takkar
Age related macular degeneration (ARMD or Chief Complaint
AMD), a degenerative disease of persons above
ARMD can present with
the age of 50 years that is characterized by the
zz Gradual painless loss of vision in eye (dry
following abnormalities in the macula:1
ARMD)
zz Presence of at least intermediate-size drusen
zz Sudden loss of vision (wet ARMD)
(63 μm or larger in diameter)
zz Shadows, distorted vision, difficulty for dis
zz Retinal pigment epithelium (RPE) abnor
cerning colors, decreased contrast, slow
malities such as hypopigmentation or
recovery of visual function after exposure to
hyperpigmentation
bright light, and slow reading may accompany
zz Reticular pseudodrusen
the loss of vision.
zz Presence of any of the following features:
zz Central scotomas—shadows or missing areas
Geographic atrophy of the RPE, choroidal neo
of vision may be present (positive scotoma).
vascularization (exudative, wet), polypoidal
choroidal vasculopathy, or retinal angioma
tous proliferation.
Visual acuity is not a factor in the disease Those with non-exudative macular degeneration
definition or classification scheme. In postgraduate may be asymptomatic or notice a gradual loss
exam, it can be given as a long case. of central vision, whereas those with exudative
Retina 273
macular degeneration often notice a rapid onset Ocular Examination

of vision loss.
Eyebrow: Brow-ptosis may occur due to aging.
History of Past Illness Eyeball: Generally normal.
History of previous anti VEGF injections, laser, Lid: Senile ptosis, dermatochalasis may be present
PDT, low vision aid may be there. AMD is multi (due to aging).
factorial in etiology, and is thought to involve a Conjunctiva: Generally normal.
complex interaction between polygenic, lifestyle
and environmental factors. The various risk factors Cornea: Arcus senilis may be present (due to
should be identified on history and includes aging).
following: Sclera: Usually normal.
zz Age is the major risk factor.
Anterior chamber: Usually normal.
zz Race: Late AMD is more common in white
individuals than those of other races. Iris: Increased risk of AMD in people with blue or
zz Heredity: Family history is important; the risk light iris color compared with those with darker
of AMD is up to three times as high if a first- iris pigmentation.
degree relative has the disease. Pupil: Usually normal.
zz Variants in many genes have been implicated
in AMD risk and protection such as the IOP: Usually normal.
complement factor H gene CFH, which helps Gonioscopy: Usually normal.
to protect cells from complement-mediated
Lens: Senile cataract, another comorbidity is
damage and the ARMS2 gene on chromosome
found due to aging factor.
10. Genes related to lipid metabolism are also
thought to be important. Anterior Vitreous: Liquefied vitreous, mostly age
zz Smoking roughly doubles the risk of AMD. related and may be present.
zz Hypertension and other cardiovascular risk Fundus: Stereoscopic fundus examination is
factors.
the best method for examining a patient with
zz Dietary factors: High fat intake and obesity
suspected choroidal neovascularization (CNV).
may promote.
A fundus contact or non-contact lens in
zz Aspirin may increase the risk of neovascular
conjunction with slit lamp biomicroscopy should
AMD. Though the evidence is limited, if an
be utilized for the exam. For those less comfortable
individual at high risk requires an antiplatelet
with the non-contact fundus macular lenses, a
agent it may be sensible to consider an
fundus contact lens is easiest to use. Following
alternative to aspirin.
findings should be looked for.
zz Other factors: Such as cataract surgery, blue
iris color, high sunlight exposure and female Dry ARMD:
gender are suspected, but their influence zz Drusen: Age-related drusen are rare prior to
remains less certain. the age of 40, but are common by the sixth
decade. Numerous intermediate—large soft
Family History drusen; may become confluent. Drusen is
Family history may be there. positively associated with the size of lesions
and the presence or absence of associated
Past Surgical History pigmentary abnormalities. The distribution
is highly variable, and they may be confined
Rule out any past intravitreal surgery.
to the fovea, may encircle it or form a band
around the macular periphery (Fig. 1). They
EXAMINATION
may also be seen in the peripheral and mid-
General examination/specific systemic exami peripheral fundus.
nation should be carried out to rule out any zz Pigment epithelial abnormalities: Focal
systemic risk factors as described above. hyper- and/or hypopigmentation of the
Fig. 1: Clinical photograph of dry AMD. Both soft and Fig. 2: Clinical photograph of geographic atrophy
hard drusens are present
RPE is associated with a significantly higher

likelihood of progression to late AMD with
visual loss.
zz Sharply circumscribed areas of RPE atrophy
associated with variable loss of the retina and
choriocapillaris atrophy.
zz Geographic atrophy, enlargement of atrophic
areas, within which larger choroidal vessels
may become visible and pre-existing drusen
disappear (Figs 2 and 3). Visual acuity may
be severely impaired if the fovea is involved.
Rarely, CNV may develop in an area of GA.
zz Dystrophic calcification may develop in all
types of drusen.
zz Drusenoid RPE detachment may occur. Fig. 3: FFA of geographic atrophy with
large temporal PED
Drusenoid PED develops from confluent large
soft drusen, and is often bilateral. Shallow zz Signs of CNV include subretinal fluid,
elevated pale areas with irregular scalloped hard exudates, subretinal hemorrhage or
edges. intraretinal hemorrhage, pigmented subretinal
High-risk characteristics of drusen for development lesions, and subRPE fluid.
of CNV include: Soft type, large size, greater than zz Localized subretinal fluid, sometimes with
five in number, confluent, and presence of RPE cystoid macular edema may be present.
stippling, family history of wet AMD or other eye zz Intra- and subretinal lipid deposition, some
wet AMD. These risk factors have been used by times extensive.
the AREDS to formulate a 5 year risk of developing zz Hemorrhage is common, e.g. subretinal, pre
wet AMD. retinal/retrohyaloid, or vitreous (breakthrough
bleed can occur in to vitreous).
zz Serous and/or hemorrhagic detachment of the
Wet ARMD Neovascular
sensory retina or RPE may occur.
CNV: It can be occult or classic CNV (see Table 1). zz Serous PED: An orange dome-shaped elevation
zzIt appears as a gray—green or pinkish-yellow with sharply delineated edges, often with
lesion. Associated medium–large drusen are a a paler margin of subretinal fluid. Multiple
typical finding in the same or fellow eye. lesions may occur. An associated pigment
Retina 275
Table 1 FFA and ICG findings in ARMD

Clinical condition FFA ICG
Classic CNV Well demarcated boundaries, discerned Similar to FFA but less well delineated.
during transit, late leakage often obscuring ICGA demonstrates CNV as a focal
boundaries hyperfluorescent “hot spot” or “plaque”
Occult CNV: Stippled hyperfluorescence Stippled hyperfluorescence
Fibrovascular Irregular elevation of RPE, Boundaries may
PED (FVPED) or may not be well demarcated, Persistent
staining or leakage of fluorescein at 10 minutes
Serous PED A well demarcated oval area of An oval hypofluorescent area with a
hyperfluorescent pooling that increases surrounding hyperfluorescent ring
in intensity but not in area with time; an
indentation (notch) may signify CNV
Drusenoid PED Early diffuse hypofluorescence with patchy Hypofluorescence predominates
relatively faint early hyperfluorescence,
progressing to moderate irregular late staining
Hemorrhagic Dense masking of background fluorescence, Similar to ICG
PED but overlying vessels are visible
Basal laminar Hyperfluorescence early and give an
drusen appearance of “starry night”
Hard exudates Window defects with early hyperfluorecence
and fading of fluorescence in late frames
Soft exudates Early hypofluorescence or hyperfluorescence
with no late leakage
RPE tear/rip The fluorescein angiogram shows blocked
fluorescence in the area of scrolled RPE and
hyperfluorescence in the area without RPE
Idiopathic Hyperfluorescent dilated complexes of Hyperfluorescent nodules and a
polypoidal choroidal vessels (branching vascular network of large choroidal vessels with
choroidopathy networks) that leak in the later phases of the surrounding hypofluorescence appear in
(IPC) angiograms. These dilated complexes look like the early phase. The polyp-like swellings
polyps or grapes rapidly begin to leak. The previously
darker surrounding region becomes
hyperfluorescent by the late phase
Geographical Autofluorescent hyperfluorescent on angio
atrophy graphy due to transmission defect and
staining
RAP FFA is usually similar to occult or minimally ICGA is diagnostic in most cases,
classic CNV, but may show focal intraretinal showing a hot spot in mid and/or late
hyperfluorescence frames, and frequently a perfusing
retinal arteriole and draining venule
(‘hairpin loop’ when linked)
Abbreviations: ARMD, age-related macular degeneration; ICG, indocyanine green; PED, pigment epithelial
detachment; CNV, choroidal neovascularization; IGGA, indocyanine green angiography; FFA, fundus
fluorescein angiograms
band may indicate chronicity. Associated

blood, lipid exudation, chorioretinal foldsor
irregular subretinal fluid may indicate
underlying CNV.
zz Fibrovascular PED is much more irregular in
outline and elevation than serous PED. This is
regarded as a form of wet AMD and should not
be confused with serous/drusenoid PEDs.
zz Hemorrhagic PED: Sub RPE or subretinal
blood is found (Fig. 4).
zz Fibrovascular disciform scar.
zz Retinal pigment epithelial tear/rip: An RPE
tear may occur at the junction of attached and
detached RPE. Tears may occur spontaneously,
following laser (including PDT), or after Fig. 4: Clinical photograph of wet AMD showing
disciform scar with subretinal bleed
intravitreal injection. Older patients and large
irregular PEDs associated with CNV are at
higher risk. A crescent-shaped pale area of
which highly exudative lesions with steep walled
RPE dehiscence is seen, next to a darker area
hemorrhagic pigment epithelial detachments
corresponding to the retracted and folded
are seen most typically adjacent to the optic disc,
flap. An RPE tear is readily identifiable as a
but can occur anywhere within the macula and
sharply-demarcated area of bare choroid with
even outside the macula.
a straight, linear edge. This straight, linear edge
corresponds to the location of the associated
retracted, scrolled RPE. DIFFERENTIAL DIAGNOSIS
zz Retinal angiomatous proliferation (RAP): It Non-exudative macular lesions mimicking AMD:
is an atypical form of neovascular AMD. The A number of conditions feature lesions similar to
presence of small central macular hemor age-related drusen
rhages, sometimes punctiform, associated zz Doyne honeycomb retinal dystrophy (malattia
with edema in an eye with soft drusen, leventinese, autosomal dominant radial
is highly suggestive of RAP in its initial stages. drusen) is an uncommon condition in which
The following lesions suggest RAP in AMD:
fairly characteristic drusen appear during the
—— Small multiple hemorrhages, pre, intra
second or third decades.
or subretinal, normally not observed in zz Pattern dystrophy (PD): It affects the macula
macular neurosensory detachments with
and can be mistaken for nonexudative AMD.
choroidal neovascularization.
The most common types of PD seen are adult
—— Tortuous, dilated retinal vessels, some-
vitelliform macular dystrophy (AVMD) and
times showing retino-retinal anasto-
less commonly butterfly shaped pattern
moses.
dystrophy. Differentiating AVMD from AMD
—— Telangiectasias.
can be difficult. Fundus autofluorecence
—— Microaneurysms.
imaging especially when combined with
—— Sudden disappearance of a retinal vessel
optical coherence tomography is helpful in
that appears to have moved deeper.
distinguishing PD from AMD. Fluorescein
—— Hard exudates around the retinal lesion.
angiography can show a typical ‘corona sign’
Earlier CNV was classified into Type 1—sub
in AVMD, and the branching lines seen in
RPE, Type 2—sub-retinal with sub RPE. RAP now
butterfly shaped PD are associated with a
has been labelled as type 3 CNV.
hyperfluorescence distributed in the area of
Idiopathic polypoidal choroidopathy (IPC): This the deposits, which does not show leakage
is another atypical form of neovascular AMD in throughout the phases of the angiogram.
Retina 277
zz Cuticular drusen, also known as grouped early response to laser photocoagulation or PDT.
adult-onset or basal laminar drusen tend to Current indications include:
be seen in relatively young adults. The lesions —— Diagnosis of CNV prior to committing to
consist of small (25–75 μm) yellowish nodules anti-VEGF treatment; FA should usually
that tend to cluster and increase in number be performed urgently on the basis of
with time and can progress to serous PED. clinical suspicion. To detect the presence
FA characteristically gives a ‘stars in the sky’ of and determine the extent, type, size,
appearance. The condition has been linked to and location of CNV. If verteporfin PDT or
a variant of the CFH gene. laser photocoagulation surgery is being
zz Type 2 membranoproliferative glomerulone considered, the angiogram is also used as
a guide to direct treatment.
phritis is a chronic renal disease that occurs
—— To detect persistent or recurrent CNV
in older children and adults. A minority of
following treatment.
patients develop bilateral diffuse drusen- —— To assist in determining the cause of
like lesions. The CFH gene has again been visual loss that is not explained by the
implicated. clinical examination.
—— As an adjunct to diagnosis of an alternative
Exudative macular lesions mimicking AMD
zz Diabetic maculopathy form of neovascular AMD such as PCV
and RAP.
zz High myopia
—— Localization for extrafoveal photocoagu
zz Inflammatory CNV
lation, or guidance for PDT.
zz Angioid streaks, and chorio retinal inflam —— Monitoring response to therapy.
matory conditions such as presumed ocular
CNVs can be detected and categorized either
histoplasmosis.
as classic or occult, or a combination of the
two, depending on the leakage patterns
INVESTIGATIONS they present at various time points on the
angiogram. This differentiation was imperative
zz Ultrasonography: USG is useful in cases of for laser treatments where well defined margins
media haze for fundus evaluation. for treatment decision were necessary.
zz Fluorescein angiography (FA): This is central Classic CNVM—present as discrete,
to diagnoses and management. See Table 1 for early hyperfluorescence with late leakage of
various findings. FA is used to diagnose CNV dye into the overlying neurosensory retinal
(Figs 5 and 6) and to plan and monitor the detachment. A lacy pattern within the CNVM
Fig. 5: FFA of wet AMD with disciform scar with Fig. 6: Late phase FFA of wet AMD of Figure 5,
subretinal bleed confirms presence of leakage
is most often not observed in exudative AMD. Stereoscopic fluorescein angiography

Only 12% of newly diagnosed patients with is indicated to determine the extent, type,
exudative AMD present with classic CNV. size and location of CNV. ICG is useful when
Occult CNVM—are categorized into 2 assessing patients with macular hemorrhage
basic forms, late leakage of undetermined or suspected of having retinal angiomatous
source and fibrovascular PEDs. proliferative lesions, idiopathic polypoidal
Late leakage of undetermined source choroidopathy, or nonvascularized versus
(LLUS) manifests as regions of stippled or ill- vascularized PEDs.
defined leakage into an overlying neurosensory zz Optical coherence tomography (OCT): High
retinal detachment without a distinct source resolution OCT, such as spectral domain OCT,
focus that can be identified on the early frames is mandatory for diagnosis and monitoring
of the angiogram. response to therapy (Fig. 7). With enhanced
Fibrovascular PEDs present as irregular depth imaging OCT (EDI OCT) and swept
elevation of RPE, which is associated with source OCT choroidal evaluation has opened
stippled leakage into an overlying neurosensory up new windows as has OCT angiography.
retinal detachment in the early and late frames The CNVM can be charted out and response
of the angiograms. Fibrovascular PEDs can be to therapy measured. On OCTA, various types
differentiated from serous PEDs, which show of CNVMS have been seen Sea fan like, spider
more rapid homogenous filling of the lesion like, medusa head pattern, poorly defined
in the early frames without leakage in the late etc. for different findings see Table 2 and
frames of the angiogram. Serous PEDs typically Figures 8 and 9.
show smooth and sharp hyperfluorescent zz Amsler grid: The Amsler grid is a useful test
contours. for detecting the early visual symptoms of
Fig. 7: OCT collage of a patient with PED, CNVM complex and subretinal fluid
Retina 279
Table 2 OCT in ARMD

Clinical condition OCT findings
Classic CNV Classic CNV presents on OCT as a fusiform thickening and disruption of RPE-BM-
CC complex. Some CNV is anterior to it but in contact with it
Occult CNV Occult is characterized by a focal, irregular poorly defined enhanced reflectivity
anterior to choroid. Hyperreflectivity extends from above to below the RPE with
no separation line
CME On OCT images is seen as hyporeflective, dark spaces within retinal tissue
RPE detachments Highly reflective tissue beneath the dome of the RPE detachment (OCT shows
separation of the RPE from the Bruch membrane by an optically empty area. CNV
may be indicated by a notch between the main elevation and a second small
mound)
Double RPE detachments Separated by notch rest similar to RPE detachments
Fibrovascular PED Less uniform than a serous PED; both fluid and fibrous proliferation are shown,
the latter as irregular scattered reflections
Drusenoid PED OCT shows homogeneous hyper-reflectivity within the PED, in contrast to
optically empty serous PED. There is commonly no subretinal fluid
RPE tears Loss of the normal dome shape of the RPE layer in the PED, with hyper-reflectivity
of the folded RPE
PCV Cup-shaped RPE elevations and choroidal angiomatous lesions. Hemorrhagic and
serous detachments of the retina and the RPE also can be seen
RAP Initial signs that correspond to stage 1 (intraretinal vascularization) consist of
a focal area, usually extrafoveal, with increased retinal reflectivity that are not
associated with epiretinal, intraretinal, or subretinal changes or changes in the
retinal thickness. When RAP reaches the subretinal space and merges with the
RPE, a serous detachment of the RPE usually develops (stage 2 or CNV). In well-
developed cases, there may be retinal choroidal anastomoses (stage 3 or CNV)
Drusen • RPE excrescences overlying reflective material consistent with drusen
• Saw-toothed configuration or bunching of the RPE
• Discrete nodular drusen which actually disrupt as opposed to distort the RPE
Outer retinal tubulations Roundish hyporeflective spaces, often around the margin of GA
Outer retinal Basal laminar deposit
corrugations Basal linear deposit
Abbreviations: ARMD, age-related macular degeneration; OCT, optical coherence tomography; PED, pigment
epithelial detachment
exudative AMD in patients with high risk AMD. cause is neovascular AMD. There are limits
Each box on the grid represents one to Amsler grid testing which includes the
degree of visual field. Thus, the Amsler grid cortical completion phenomenon, crowding
tests the central 10° of visual field beyond phenomenon and lack of forced fixation.
fixation. The patient is asked to fixate on zz Preferential hyperacuity perimeter (PHP):
the central black dot and to note whether A newly developed computer-automated,
surrounding lines are wavy, missing or three dimensional, threshold, Amsler grid
obscured by scotomas (dark areas). If these visual field test has been shown to be useful in
findings are present, the patient should be earlier detection of AMD. The central 14° are
instructed to seek attention urgently with his tested in about five minutes.
or her ophthalmologist as it is likely that the
Fig. 8: OCT of a large PED. Note the notches in the PED
Fig. 9: A time domain OCT of a patient with disciform scar and cystoids retinal spaces
zz SLO microperimetry: SLO microperimetry

Table 3 Clinical classification of age-related
have found impaired rod photoreceptor macular degeneration
function and photopic sensitivity respectively
in areas of increased FAF in the junction zone, Definition, based on presence of
which underscores abnormalities associated lesions within two disc diameters
Category of the fovea in either eye
with increased fundus autofluorescence.
zz Fundus autofluorescence: FAF imaging in No apparent • No drusen
patients with GA is characterized by a decreased aging changes • No AMD pigmentary
signal with sharp borders corresponding to the abnormalities
area of atrophy on conventional retinography. Normal aging • Only drupelets
changes • No AMD pigmentary
CLASSIFICATION abnormalities
See Tables 3 to 5. Early age- • Medium drusen (>63 μm
related macular but <125 μm)
Classification of CNVM degeneration • No AMD pigmentary
(AMD) abnormalities
Angiographic
Intermediate • Large drusen (>125 μm)
Terminology used to describe CNV on FA is AMD • Any AMD pigmentary
derived from the Macular Photocoagulation Study abnormalities
(MPS):
zz Classic CNV (20%) fills with dye in a well- Late AMD Neovascular AMD and/or any
geographic atrophy
defined ‘lacy’ pattern during early transit
Retina 281
subsequently leaking into the subretinal space 2. Juxtafoveal: CNV is between 1 micron and
over 1–2 minutes, with late staining of fibrous 199 microns from the foveal center.
tissue. Most CNV is subfoveal, extrafoveal 3. Subfoveal: CNV is under the foveal center.
being defined as ≥200 μm from the center of
the foveal avascular zone on FA. MANAGEMENT
zz Occult CNV (80%) is used to describe CNV
when its limits cannot be fully defined on The management guidelines have been described
FA. Variants are fibrovascular PED and ‘late in Table 6.
leakage of an undetermined source’ (LLUS).
zz Predominantly or minimally classic CNV is Management of Dry AMD
present when the classic element is greater or Most vases need observation only. Prophylaxis for
less than 50% of the total lesion respectively. prevention of complications includes following:
zz Antioxidant supplementation: Age-related
Topographic eye disease study (AREDS), now known as
The location of well-demarcated CNVs was broken AREDS1 and AREDS2 has proven the efficacy
into three categories: of such therapy.
1. Extrafoveal: CNV is 200 microns or more from Indications
the foveal center. —— Extensive intermediate: (≥63–125 μm)
drusen
—— Atleast one large (≥125 μm) drusen
Table 4 Classification of age-related macular
—— GA in one or both eyes
degeneration
—— Late AMD in one eye (greatest benefit in
Non-neovascular (DRY) Neovascular (WET)
AREDS1)
• Drusen • Choroidal AREDS 1 formulation included antioxidant
• Focal hyperpigmentation neovascularization vitamins—500 mg of vitamin C; 400 IU of
• Nongeographic atrophy • Disciform scarring vitamin E; 15 mg of beta-carotene; 80 mg of zinc
• Geographic atrophy oxide and 2 mg of cupric oxide. This formulation
Table 5 AREDS classification

AREDS
Class category Features
No AMD 1 No or few small drusen (<63 μm in diameter). Represented the control group of
(AREDS)
Early AMD 2 Combination of multiple small drusen, few intermediate drusen (63–124 μm
in diameter), or mild RPE abnormalities
Intermediate 3 Any of the following features:
AMD • Numerous intermediate drusen
• At least one large druse (≥125 μm in diameter)
• Geographic atrophy not involving the center of the fovea
Advanced 4 One or more of the following (in the absence of other causes) in one eye:
AMD • Geographic atrophy of the RPE involving the foveal center.
• Neovascular maculopathy that includes the following:
– CNV
– Serous and/or hemorrhagic detachment of the neurosensory retina or RPE
– Retinal hard exudates (a secondary phenomenon resulting from chronic
intravascular leakage)
– Subretinal and sub-RPE fibrovascular proliferation
– Disciform scar (subretinal fibrosis)
Abbreviations: AREDS, age-related eye disease study; CNV, choroidal neovascularization
Table 6 Management of ARMD1

Major category Recommended treatment Diagnoses eligible for treatment
Non-neovascular Observation • Early AMD (AREDS category 2)
AMD • Advanced AMD with bilateral subfoveal
geographic atrophy or disciform scars
Antioxidant supplements as • Intermediate AMD (AREDS category 3)
recommended in AREDS2 reports • Advanced AMD in one eye (AREDS category 4)
Neovascular AMD Aflibercept intravitreal injection 2.0 mg Macular CNV
Bevacizumab intravitreal injection Macular CNV
1.25 mg
Ranibizumab intravitreal injection Macular CNV
0.5 mg
• Less commonly PDT with verteporfin as recommended • Macular CNV, new or recurrent, where the
• Used treat in the TAP and VIP reports classic component is >50% of the lesion and
ments for the entire lesion is ≤5400 μm in greatest linear
neovascular diameter
AMD • Occult CNV may be considered for PDT with
vision <20/50 or if the CNV is <4 MPS disc
areas in size when the vision is >20/50
• Juxtafoveal CNV in select cases
Laser photocoagulation as • May be considered for extrafoveal classic CNV,
recommended in the MPS reports new or recurrent
• May be considered for juxtapapillary CNV
Abbreviations: AMD, age-related macular degeneration; AREDS, age-related eye disease study; CNV, choroidal
neovascularization; MPS, macular photocoagulation study; OCT, optical coherence tomography;
PDT, photodynamic therapy; TAP, treatment of age-related macular degeneration with photodynamic therapy;
VIP, verteporfin in photodynamic therapy
has been shown to reduce the risk of developing zz Zeaxanthin (2 mg)

advanced AMD and the associated visual loss by zz Zinc (25–80 mg; the lower dose may be equally
as much as 25%, over 5 years, in individuals with effective)
moderate to high risk of age-related macular zz Copper (2 mg; this may not be required with
degeneration (AREDS categories 3 and 4). These the lower zinc dose).
findings were accompanied by a 19% reduction
Risk factors modification should be addressed,
in the risk of moderate vision loss (loss of three or
e.g. smoking, ocular sun protection, cardiovas
more lines on the visual acuity chart), at 5 years.
cular, dietary.
However, high zinc doses are potentially
associated with genitourinary tract problems, Beta- An Amsler grid should be provided for home use,
carotene can increase the incidence of lung cancer with advice to self-test on a regular basis, perhaps
in current and former smokers. AREDS2 looked at weekly, and to seek professional advice urgently in
adjusting the beta-carotene and zinc components, the event of any change, when imaging (e.g. OCT,
and also whether additional or alternative supple FA) should be performed to rule out progression
ments could enhance outcomes.2 to neovascular AMD. This might be of increased
Recommended daily supplementation based importance following cataract surgery.
on AREDS2 includes: Provision of low vision aids for patients with
zz Vitamin E (400 IU) significant visual loss and certification as visually
zz Vitamin C (500 mg) impaired if available as this may facilitate access to
zz Lutein (10 mg) social and financial support.
Retina 283
Management of Wet AMD Photodynamic Therapy (PDT)

Anti-VEGF Photodynamic therapy (PDT) is useful for eyes
with subfoveal CNV (see Table 6). Verteporfin is
Indications of anti-VEGF includes following: a light-activated compound preferentially taken
zz All CNV subtypes respond to anti-VEGF up by dividing cells including neovascular tissue.
therapy, but benefit is only likely in the It binds to LDL receptors and upon reaction to
presence of active disease. light produces singlet oxygen residues. It is infused
zz Active disease includes fluid or hemorrhage, intravenously followed by the delivery of laser light
leakage on FA, an enlarging CNV membrane, of a wavelength of 689 nm to the CNV lesion as a
or deteriorating vision judged likely to be due single spot with a diameter 1000 μm larger than
to CNV activity. the greatest linear diameter of the lesion. When
zz An eye with almost any level of vision may activated by diode laser it cause thrombosis.
benefit, although better VA at presentation is
associated with a better visual outcome.
Laser
Anti-VEGF agents: See Table 1 in chapter on
proliferative diabetic retionopathy (PDR). Thermal argon or diode laser ablation of CNV is
now rarely used, though may still be suitable for the
Aflibercept (Eylea) is a recombinant fusion treatment of small classic extrafoveal membranes
protein that binds to VEGF-A, VEGF-B and well away from the macular center, and possibly
placental growth factor (PlGF). The advantage is some cases of PCV and RAP.1
the maintenance regimen consists of one injection
every 2 months in contrast to the monthly
Other Therapies
injections recommended with ranibizumab and
bevacizumab. In addition, it is more efficacious Various treatment modalities have been tried
than Lucentis due to its increased affinity to with variable success. These are—Transpupillary
bind the receptors. The standard dose is 2 mg in Thermotherapy (TTT), Teletherapy (EBRT),
0.05 mL; an induction course of three injections is Brachytherapy (Plaque Radiotherapy), Anecortave
given at monthly intervals. Acetate (an angiostatic steroid), small interfering
Ranibizumab (Lucentis): Ranibizumab is a RNA (siRNA), Vatalanib (inhibitor of all known
humanized monoclonal antibody fragment VEGF receptor tyrosine kinases), Squalamine
developed specifically for use in the eye, though is Lactate (an anti-angiogenic amino sterol derived
derived from the same parent mouse antibody as from cartilage of the dogfish shark, action includes
bevacizumab (see next). It non-selectively binds blockade of cell membrane ion transporters
and inhibits all isoforms of VEGF-A. The usual that regulate cell function by controlling pH and
dose is 0.5 mg in 0.05 mL. metabolism. Sphingomab (monoclonal antibody
targeted against sphingosine-1-phosphate,
Bevacizumab (Avastin): In contrast to ranibi which has been implicated in angiogenesis, scar
zumab, bevacizumab is a complete antibody formation, and inflammation), Volociximab
originally developed to target blood vessel growth (a chimeric monoclonal antibody that inhibits
in metastatic cancer deposits. Its use for AMD the functional activity of a5ß1 integrin, a protein
and other indications is ‘off label’; it is very much found on activated endothelial cells, and prevent
cheaper than ranibizumab and aflibercept. Clinical angiogenesis), Designed ankyrin repeat proteins
trial suggest comparable results to ranibizumab in (DARPins) [genetically engineered antibody
efficacy and safety. The dose of bevacizumab is mimetic proteins that is a potent VEGF inhibitor],
usually 1.25 mg/0.05 mL. Sirolimus (Rapamycin), Infliximab (a monoclonal
Pegaptanib (Macugen): Pegaptanib sodium was antibody that binds and neutralizes tumor
the first anti-VEGF agent approved by regulatory necrosis factor alpha), Eculizumab (Complement
authorities for ocular treatment; the results are Inhibitors), Pigment epithelial-derived growth
similar to outcomes with PDT, and its use is now factor (PEDF), Brimonidine, antioxidant eye
extremely limited. drops, Alprostadil (Choroidal Blood Perfusion
Enhancers), Maculoplasty (overall tissue engine large size, greater than five in number,
ering attempt to reestablish the normal subretinal confluent, and presence of RPE stippling.
anatomy), Macular translocation (moving the Q.4. High-risk characteristics for develop
neurosensory retina of the fovea in one eye with ment of CNV.
recent-onset subfoveal CNV to a new location Ans. Systemic risk factors associated with CNV
before the occurrence of permanent retinal include increased age, Caucasian race,
damage, may allow it to recover or to maintain its smoking. Ocular risk factors associated with
visual function over a healthier bed of RPE-Bruch’s increased risk of CNV include large drusen,
membrane-choriocapillaris complex), gene confluent drusen, hyperpigmentation and
therapy, stem cells. hypertension.
Q.5. Median rate of enlargement of GA?
Management of Poor Vision Ans. GA continues to enlarge over time with
Low vision aid refers to an optical device a median rate of enlargement over a two-
that improves or enhances residual vision by year period of 1.8 MPS disc areas. The
magnifying the image of the object at the retinal prevalence of GA increases with age, being
level. Non-optical aids also work as LVAs as they half as common as CNV at age 75, and more
may help in enhancing the visual performance.3 common than CNV in older age groups.
GA is bilateral in more than half of the
VIVA QUESTIONS people with this condition.
Q.6. What is BIONIC EYE/ARGUS II?
Q.1. What is the rate of progression of CNV? Ans. Designed for patients who are blind due
Ans. • Progress rapidly irrespective of its initial to diseases like retinitis pigmentosa or
location and extent at a mean rate of AMD. Relies on patient having a healthy
18 µm/day. optic nerve and a developed visual cortex.
• Disciform scars with fibrous tissue or The prosthesis consists of—a digital
geographic atrophy represent the end camera built into a pair of glasses—a
stages of both types of CNV. video processing microchip built into a
Q.2. What is Drusen? hand held unit—a radio transmitter on the
Ans. Drusen (singular: druse) are extracellular glasses—a receiver implanted above the
deposits located at the interface between ear—a retinal implant with electrodes on
the RPE and Bruch membrane. The a chip behind the retina. Camera captures
material of which they are composed has a an image Send image to microchip convert
broad range of constituents, and is thought image to electrical impulse of light and dark
to be derived from immune-mediated pixels—Send image to radiotansmitter—
and metabolic processes in the RPE. Their Transmits pulses wirelessly to the receiver
precise role in the pathogenesis of AMD is sends impulses to the retinal implant by a
unclear, but is positively associated with hair thin implanted wire—The stimultaed
size. The earliest pathological changes are electrodes generate electrical signals that
the appearance of basal laminar deposits travel to the visual cortex.1
(BlamD) and basal linear deposits (BlinD). Q.7. What is implantable miniature tele-
BlamD consist of membrano-granular scope?
material and foci of wide spaced collagen Ans. Implantable miniature telescope is
between the plasma membrane and basal implanted into one eye only (typically
lamina of the RPE. BlinD consist of vesicular the non-dominant or poorer seeing eye).
material located in the inner collagenous It generates a 20°–24° field of vision. The
zone of Bruch’s membrane. FDA approved the implantable miniature
Q.3. High-risk characteristics of drusen for telescope (IMT) in 2010 for patient 75 years
development of CNV. and older with stable severe-to-profound
Ans. High-risk characteristics of drusen for vision impairment (20/160 to 20/800)
development of CNV include: soft type, caused by bilateral end-stage AMD.
Retina 285
Q.8. Name some low vision aids. REFERENCES

Ans. Optical devices: Hand held telescopes,
1. American Academy of Ophthalmology
mounted telescopes, intra-ocular LVA
Retina/Vitreous Panel. Preferred Practice
(IO-LVA) NEAR, spectacles—prismatic ½
Pattern ® Guidelines. Age-Related Macular
eyes—bifocals, magnifiers, Degeneration. San Francisco, CA: American
Absorptive lenses: Tinted lenses, photo Academy of Ophthalmology; 2015. Available at:
chromatic lenses, polarization glasses, www.aao.org/ppp.
Filters—corning CPF, younger PLS—visual 2. Age-Related Eye Disease Study Research
field enhancement devices, fresnel prisms, Group. A randomized, placebo-controlled,
gottlieb field expanders, reverse telescopes, clinical trial of high-dose supplementation with
hemianopic mirrors. vitamins C and E, beta carotene, and zinc for
Non-optical: Lighting, contrast enhance age-related macular degeneration and vision
ment, increasing size of object, auditory loss: AREDS report No. 8. Arch Ophthalmol
aids. 2001;119:1417-36.
3. Maniglia M, Cottereau BR, Soler V, Trotter Y.
Electronic magnifiers: CCTV, large print
Rehabilitation Approaches in Macular
computers.
Degeneration Patients. Frontiers in Systems
Orientation and mobility LVA: Canes, guide Neuroscience. 2016;10:107.
dog, electronic orientation devices, GPS
Newer technology LVA: E-readers, smart
phones, tablets.
INTERMEDIATE UVEITIS
Raghav Ravani, Karthikeya R, Harathy Selvan, Atul Kumar
INTRODUCTION Pars planitis forms the majority subset of inter

mediate uveitis, as high as 70–80%.
As per its anatomical definition, intermediate Also note that involvement of vessels in form
uveitis (IU) is defined as an inflammation involv of peripheral vascular sheathing and cystoid
ing the anterior vitreous (hyalitis), ciliary body macular edema (CME) does not change the
(posterior part of ciliary body), and peripheral nomenclature or classification.
retina.1 Intermediate uveitis is usually given as a long
case or a short case in the exams.
NOMENCLATURE
HISTORY
Various names were used to describe the
condition including chronic cyclitis, vitritis, Chief Complaint
cyclochorioretinitis, pars planitis, intermediate The condition may have minimal symptoms.
uveitis, peripheral uveoretinitis, etc.1 Recently, Unlike anterior uveitis, the symptoms are bilateral
as per the SUN (Standardization of Uveitis more than unilateral, and often asymmetrical. Also
Nomenclature, IUSG*) working group’s guide the disease may present with its complications
lines,2 IU refers to the subset of uveitis wherein the like CME, vitreous membranes, glaucoma and
major site of inflammation is vitreous and there is cataract. The usual presenting complaints are:
an associated infection or systemic disease. The zz Floaters: The most common (due to snow balls,
term, pars planitis refers to the “idiopathic” subset membranes, vitreous opacification)
of intermediate uveitis occurring in absence of zz Blurring of vision (chronic, painless—due to
any associated infection or systemic disease. CME, cataract)
*International Uveitis Study Group

zz Loss of vision [sudden, painless due to glau tuberculosis, multiple sclerosis, etc.) Even on
coma, spill over uveitis, secondary rhegmato absence of symptoms, leading questions must be
genous retinal detachment (RD)]—rarely asked for commonly associated disorders as IU
zz Noted during routine fundus evaluation. may be the primary presentation (Flow chart 1).
History of Present Illness Family History

zz Intermediate uveitis tends to affect young Intermediate uveitis though not hereditary has
individuals with a bimodal distribution, i.e. been seen reported in families. It is associated
affecting two age groups, 5–15 years and with HLA DR-15 and HLA DR-51 alleles. IU is
20–40 years, equally affecting males and seen in families with common HLA haplotypes.
females. Some reports from India put it as the Also note that HLA DR-15 is associated with
most common subtype of uveitis in children, multiple sclerosis and thus may suggest a
whereas others report it as the least common. predisposition of it in patients with IU with HLA
zz Unlike anterior uveitis, it has an insidious DR-15 positivity.
onset.
zz The most common presenting symptoms are EXAMINATION
floaters.
zz Associated pain, redness, watering is usually General Examination
absent or may be present in children with Detailed systemic examination is warranted to
associated anterior uveitis. These are usually know any associated conditions like sarcoidosis,
minimal when present and often seen as spill tuberculosis, multiple sclerosis, signs of tick bite
over anterior uveitis. or Lyme disease. The following condition are
zz Patient may have history of similar episodes associated with IU (Flow chart 1).
in the past with remissions or may have a
chronic history without remissions. Ocular Examination
zz Patient may complain of chronic worsening
of vision or a sudden worsening of vision in a Visual acuity: The visual acuity is usually normal
background of long standing history. in early cases. Patient may have vision acuity of
6/6 with floaters being the only complains. Main
cause of decreased visual acuity in IU is develop
History of Past Illness ment of cystoid macular edema (CME). Other
Past medical and surgical history must include causes of dimness of vision include develop
careful history to rule out the systemic associat ment of cataract, dense vitreous membranes/
ions or etiological causes of intermediate uveitis. opacities, epiretinal membrane (ERM), vitreous
There may be past history of associations of hemorrhage from neovascularization, tractional
intermediate uveitis as well (i.e. sarcoidosis, or rhegmatogenous retinal detachment.
Flow chart 1: Flowchart depicting common causes of intermediate uveitis

Retina 287
Eyeball: Squint may be present, but is a con steroid treatment or glaucoma medications (esp.
sequence of complications of disease due to loss cholinergic agents) used in treatment of uveitis
of fusion. and uveitic glaucoma. Retrolental space should be
Lid/conjunctiva/cornea/sclera are usually carefully examined for cells, which is pathognomic
within normal limits (WNL). There may be for present/past anterior vitreous inflammation.
aponeurotic ptosis or in rare cases enophthalmos However, presence of these cells does not indicate
as a side effect of previous treatment in form of active disease. Vitreous haze should be noted and
periocular steroid injections. Scleritis may be graded (Table 2). Increasing vitreous haze is a sign
present, the disease is then called sclerouveitis. of activity and decreasing vitreous haze is a sign of
Keratic precipitates (KPs) may be present, in fact response.
IU is characterized by KPs that may be central Vitreous: Vitreous is the major site of inflamma
rather than the typically inferior Arlt’s triangle KPs tion in intermediate uveitis. Thus inflammatory
of anterior uveitis. cells mainly accumulate in the vitreous cavity in
Anterior chamber: It may show variable number form of retrolental cells, vitreous membranes,
of spill-over anterior chamber cells/flair. These may condense as ‘snow-balls’ or may settle down
should be carefully documented as per SUN inferiorly over retina or pars plana as ‘snow-
classification (Table 1). banking’ leading to variable degrees of vitreous
haze. ‘Snow-balls’ appear as yellow-white con
Pupil: Pupil is circular as there in no or minimal densations in mid-vitreous, especially inferiorly.
anterior chamber reaction, and usually no
synechiae. Pupillary reflexes are usually within
normal limit, unless complicated by retinal Table 1 Grading of anterior chamber cells as per
detachment, which is rare. standardization of uveitis nomenclature
Intraocular pressure (IOP): A rise in IOP may (SUN) workshop (2004) consensus.
occur, especially as a complication in IU due to The cells are counted in a dark back
steroid use or secondary glaucoma. A rise in IOP ground of an undilated pupil in a slit of
in uveitis is also associated with increasing age, 1 mm × 1 mm
duration since diagnosis and active inflammation. Grade Number of cells/field
IOP may be low in case of severe reaction and
0 <1 cells
formation of cyclitic membrane.
0.5+ 1–5 cells
Lens: Cataract may occur in 50–60% of cases and
is one of the causes of decreased vision in IU. 1+ 6–15
Posterior subcapsular cataract is the most common 2+ 16–25
cataract associated, however even anterior sub 3+ 26–50
capsular cataract may be found. Cataract in IU
may be due to chronic inflammation or long-term 4+ >50
Table 2 NEI grading of vitreous haze as adapted by the standardization of uveitis nomenclature (SUN)
working group
Grade Description
4+ Optic nerve head not visualized
3+ Optic nerve head hazily visualized
2+ Retinal vessels visualized better. Optic nerve head hazy
1+ Vitreous cells and haze present but optic nerve head and retinal vessels clearly visualized
0.5+ Some media haze present, nerve fiber layer striations not able to be visualized. But disc and retinal
vessels are seen distinctly
0 No inflammation, normal fundus view
‘Snow-banking’ depicts more severe inflam zz Purified protein derivative skin test (PPD)
mation. ‘Snow-banking’ appears like exudates (Mantoux test)
over the pars plana, especially inferiorly, but may zz Chest X-ray ( to look for evidence of pulmonary
be seen all around. Scleral indentation or use TB or sarcoidosis)
of Goldmann three-mirror contact lens may be zz Serum angiotensin-converting enzyme levels
required to view peripheral retina and pars plana. zz Gallium scan
Sometimes vitreous haze may be severe enough zz MRI brain
to obscure view of the fundus preventing further zz Lyme titers and western blot—if high suspicion
examination. Early posterior vitreous detachment and in endemic areas.
may be seen. The list is very large and should be based on a
tailored work up. Refer to Flow chart 1. In Indian
Fundus: If media clarity permits, examination
subcontinent, TB should be ruled out in all the
of retina must be done carefully in all cases of
cases. Masquaredes should be considered when
intermediate uveitis. The fundus findings in IU
ever applicable.
includes:
zz Tortuosity of vessels
Ocular Investigation
zz Involvement of peripheral retinal vasculature
in form of sheathing (e.g. periphlebitis) zz Ultrasonography (USG) B-scan: To be done to
zz Neovascularization, which is usually peri rule out complications like retinal detachment
pheral near the area of snow-banking in cases where intense inflammation leads to
zz Peripheral vitreous traction and hole for severe media haze preventing fundus evalua
mation due to contraction of inflammatory tion. It may also help to rule out conditions
membranes may be seen masquerading as intermediate uveitis like
zz Retinal detachment has also been reported as intraocular tumors.
a complication of intermediate uveitis
zz Ultrasound biomicroscopy (UBM): To demon
zz Peripheral choroiditis may also be seen, strate pars plana exudates when clinical
though severe chroidal involvement indicates examination is difficult in presence of media
review of clinical diagnosis. opacities.
zz Optical coherence tomography (OCT): To
document presence of cystoid macular edema,
especially in patients with complain of dim
zz Vitreous membranes ness of central vision (Fig 1). It can be used as
zz Vitreous degeneration a guide to therapy. Choroidal thickness should
zz Leukemia also be monitored once treating concomitant
zz Lymphoma choroiditis. Later on, epiretinal membranes
zz Endophthalmitis (ERMs) and tractional changes can also be
zz Panuveitis seen up on studying the virtual reality (VR)
zz Amylodosis interface.
zz Old vitreous hemorrhage. zz Fluorescein angiogram shows presence of
peripheral capillary non-perfusion, peripheral
INVESTIGATION neovascularization, presence of retinal vas
culitis and petaloid (Fig 2) and honeycomb
Systemic Evaluation leakage suggestive of cystoid macular edema
As previously mentioned, many infections and (CME). Fluorescein angiography may also be
systemic conditions are associated with inter helpful for follow-up after cyrotherapy or laser
mediate uveitis (IU). A careful history, ocular photocoagulation treatment.
examination and ancillary/laboratory tests zz Ultrawide imaging helps in documenting
should be carried out to search for the etiology or peripheral changes. Ultrawide field fluore
associated condition. The test may include: scein angiography may also help in docu
zz Complete blood count with differential count menting peripheral CNP areas and presence of
and erythrocyte sedimentation rate (ESR). peripheral neovascularization.
Retina 289
Fig 1: OCT image depicting cystoids macular edema
may not need treatment. In absence of media haze

(less than grade 1), in cases with only few inferior
snow balls and absence of complications like
CME, just meticulous frequent observations may
suffice.
Following are the management options:
zz Step 1: Infections should always be ruled out if
planning for steroids. Periocular steroids may
be considered as initial treatment modality
especially in uniocular conditions. Sub-tenon
injection is the most common route for depot
corticosteroids. Other route for local therapy
includes intravitreal corticosteroid injection.
Fig 2: FA image showing macular leakage of dye in In case of failure of depot steroids and in
CME. Note the petaloid configuration bilateral cases, systemic corticosteroids could
be considered. In cases with CME, periocular
COMPLICATIONS steroids help by maintaining a constant
influx of steroids. Presence of CME should
zz Cataract indicate an alerting sign warranting aggressive
zz Glaucoma therapy as it may lead on to permanent visual
zz Cystoid macular edema loss. Throughout therapy patient should be
zz Epiretinal membrane monitored for ocular and systemic side effects
zz Vitreous hemorrhage of steroids, which can be debilitating at times.
zz Retinal detachment Dietary supplementation for calcium should
zz Rarely, optic disc edema. also be considered.
A more recent advancement is use
MANAGEMENT of sustained release steroid implants like
If an etiology has been identified on investigation, ozurdex and retisert. These have now been
treatment depends on the cause. In cases where approved for ocular use in noninfectious
no specific etiology has been identified, following uveitis. They work well in isolated ocular
are the management options. Mild cases with disease, but complications should be moni
out CME may be managed conservatively by tored. The recently conducted multicenter
observation. A four-step approach for the treat uveitis steroid treatment (MUST) study has
ment of intermediate uveitis was proposed by shown good results with flucinolone implant
Kaplan.3 It should be remembered that every case when compared to systemic therapy.
zz Step 2: Peripheral retinal ablation over snow- • C ataract: Especially posterior subcap
bank near pars plana region may be considered sular cataract
in case of nonresponse to peribulbar steroids. • Epiretinal membrane: May lead to dis
This may be done either using cryopexy tortion of vision and if severe, dimness of
(double-freeze thaw technique) or peripheral vision.
scatter photocoagulation, especially in cases • Media haze due to intense vitritis leading
with peripheral neovascularization. This to vitreous veils/membranes.
may help decrease the risk of bleeding and • Vitreous hemorrhage: Secondary to peri
regression of neovascularization. Recent pheral neovascularization.
studies indicate primary role of cryotherapy as • Retinal detachment: Tractional or rheg
a first measure also. However, this treatment matogenous retinal detachment.
may lead on to temporary worsening of disease Q.3. Other causes of ocular inflammation with
as well. ‘white’ eye?
zz Step 3: Pars plana vitrectomy with induction of Ans. Apart from intermediate uveitis, other
posterior vitreous detachment and peripheral causes include:
laser is helpful in cases resistant to previous • Fuch’s heterochromic iridocyclitis
modalities and in whom immunosuppressive • Juvenile rheumatoid arthritis
therapy may be contraindicated. This is • Posner-Schlossman syndrome
particularly helpful in cases with decreased • Kawasaki disease.
vision due to severe media haze with vitreous
Q.4. Causes of intermediate uveitis?
membrane, in cases with vitreous hemorrhage,
Ans. • Infectious conditions
in cases with nonresponding CME or cases – Tuberculosis (Mycobacterium tuber
complicated with retinal detachment/ERMs. culosis) (especially in endemic coun
zz Step 4: Systemic immunomodulatory therapy tries like India)
is of special benefit in bilateral disease. − Syphilis (Treponema pallidum)
Chronic or frequently relapsing cases may − Toxoplasmosis (Toxoplasma gondii)
benefit due to lesser steroid dependence. − Toxocariasis (Toxocara canis)
Further, volcosporin [Lux Uveitis Multicenter − Lyme’s disease (Borrelia burgdorferi)
Investigation of a New Approach to TrEatment (in endemic areas)
(LUMINATE study)] is showing promise as a − Cat-scratch diseases (Bartonella
steroid sparing agent. henselae, B. quintana)
Note: The treatment plan may not necessarily − Human T-lymphotropic virus type 1
follow the step wise approach and a combination (HTLV-1)
of one or more modalities may be considered as • Noninfectious conditions
per individual cases and to achieve early remission − Sarcoidosis
or decrease the duration of corticosteroids. − Multiple sclerosis
− Intraocular lymphoma
VIVA QUESTIONS • Idiopathic/pars planitis.
Q.5. Describe Kaplan’s four-step manage
Q.1. What is the most common presenting ment for intermediate uveitis?
symptom in case of intermediate uveitis? Ans. It is as described above in the text.
Ans. The patients most commonly present Q.6. Differential diagnoses of IU
with symptoms of floaters in a case of Ans. Causes of media haze: Old vitreous haze
intermediate uveitis. (VH), ocular lymphoma, amyloidosis,
Q.2. What are the causes of vision loss in a endophthalmitis, Candida infections,
case of intermediate uveitis? Propionibacterium acnes endophthalmitis,
Ans. Cystoid macular edema (CME) is the most leukemia, acute retinal necrosis (ARN),
common cause of dimness of vision. Other intense vitritis due to other causes such as
causes include: toxoplasmosis.
Retina 291
Q.7. Discuss steroid implants. REFERENCES

Ans. See above and chapter on DME.
1. Vitale AT, Zierhut M, Foster CS. Intermediate
Q.8. What are the side effects of steroids? Uveitis. In: Foster CS, Vitale AT (Eds). Diagnosis
Ans. Ocular side effects: Cataract, glaucoma, and treatment of uveitis. Philadelphia: WB
activation of viral keratitis (topical), Saunders and Company; 2002. pp. 844-57.
fungal corneal ulcer, central serous 2. Jabs DA, Nusenblatt RB, Rosenbaum JT.
chorioretinopathy. Standardization of uveitis nomenclature (SUN)
working group. Standardization of uveitis
Systemic side effects: Cushingoid state, dia
nomenclature for reporting clinical data.
betes, hypertension, osteoporosis, peptic
Results of the First International Workshop.
ulcer disease, sodium and water retention, Am J Ophthalmol. 2005;140:509-16.
worsening of psychosis, menstrual irregu 3. Kaplan HJ. Intermediate uveitis (pars planitis,
larities, weight gain, thromboembolism, chronic cyclitis): a four-step approach to
muscle weakness, aseptic necrosis of fem treatment. In: Saari KM (Ed). Uveitis Update.
oral head, pancreatitis, easy bruisability, Amsterdam: Experta Medica; 1984. pp. 169-72.
convulsions, etc.
CHOROIDAL MELANOMA
Karthikeya R, Raghav Ravani, Prateek Kakkar, Atul Kumar
INTRODUCTION fundus examination. When symptoms do occur,

they can be variable depending on the tumor
Uveal melanoma is a rare form of melanoma characteristics such as location, size, proximity to
accounting for <3% of the primary malignant macula, proximity to optic nerve and presence or
melanomas in the body. This includes anterior absence of overlying retinal changes. Anteriorly
uveal melanoma (which includes iris melanoma) located masses present later and are usually larger
and posterior uveal melanomas (which include in size and more advanced at the time of diagnosis
ciliary body melanoma and choroidal melanoma). while posteriorly located tumors present early due
In this chapter, we will focus only on choroidal to visual symptoms. Gradual painless diminution
melanoma. Choroidal melanoma is the most of vision is the most common presentation and
common uveal melanoma (90%) and also the is usually due to the presence of an exudative
most common primary intraocular tumor in an retinal detachment or a cystoid macular edema.
adult. It has an incidence of about 5 cases per An intelligent patient can also report a scotoma
million population per year in the west. Like other or a field loss. A subfoveal growth can cause a
melanomas, it is much less commonly seen in central scotoma or can cause refractive error. Very
India as compared to the west. In the west, it is rarely, patients may present with severe eye pain
seen 150 times more frequently in Caucasians as due to involvement of the posterior ciliary nerves
compared to the blacks. or sudden onset of loss of vision due to vitreous
hemorrhage subsequent to the tumor involving a
HISTORY large retinal or choroidal vessel. Photopsias can
occur due to the subretinal location of the tumor.
The presenting clinical features of a case of Presentation with loss of weight and appetite, bony
choroidal melanoma are highly variable.1,2 pain, head ache may suggest an advanced disease
with metastasis.
Chief Complaint
Patients with choroidal melanoma are usually History of Present Illness
picked up incidentally. Symptoms are often absent, Choroidal melanoma usually presents in patients
with the tumor detected by chance on routine over 60 years of age in the west. But in Asian
population it is noted to occur early at around located choroidal melanoma. It is a dilated

45 years. Males are affected slightly more tortuous anterior ciliary artery in the quadrant of
frequently than females. Note the duration and the tumor which supplies the tumor of its blood
progression of symptoms. Rapidly changing supply.
symptoms in a patient with exudative RD may be
Cornea: Corneal edema may be present if there is
a uveitic entity. Eliciting a history of pain, redness
a co-existing glaucoma.
and photophobia is also important for the same
reason. Symptoms in the contralateral eye are also Sclera: Nevus of Ota (Oculo cutaneous melano
less likely to occur—choroidal melanoma is only cytosis) is a risk factor for choroidal melanoma. It
rarely multicentric and extremely rarely bilateral. presents as unilateral slate-gray pigmentation of
the sclera, uvea, orbit, periocular skin, meninges
History of Past Illness etc. Also, look for any scleral thinning/uveal show
which may indicate a scleral extension of the
History of previous medical and surgical illnesses tumor.
are important to document and rule out other
differentials of a choroidal mass or exudative RD Iris/Pupil: Iris nevus is a risk factor for uveal mela
such as tuberculoma, hypertension, metastases, noma (Iris melanoma > choroidal melanoma).
Vogt-Koyanagi and Harada’s disease and other Choroidal melanoma with extension into ciliary
posterior uveitic entities. History of mass lesion body or primary ciliary body melanoma causes
anywhere else is the body should be specifically variety of abnormality in the angle and anterior
sought for to rule out a metastases to the eye of a chamber—irregular anterior chamber depth,
primary elsewhere or a metastases of the choroidal localized peripheral anterior synechiae etc. Rarely
melanoma. Choroidal melanoma is rarely choroidal melanoma with extensive exudative
inherited or familial—it is a sporadic malignancy. RD can cause neovascular glaucoma. Also note
Relevant systemic history should be elicited when iris color—light iris is a risk factor for choroidal
deciding for other differential diagnoses (Table 1). melanoma.
IOP: Choroidal melanoma can cause neovascular
EXAMINATION glaucoma. Ciliary body extension can cause
glaucoma by destruction/infiltration of the trabe
General Examination cular meshwork.
Should include examination of the general condi Lens: Localized cataract can occur in anteriorly
tion of the patient—to look for pallor, icterus, placed tumors which impinge upon the lens or
lymphadenopathy, cachexia, other palpable tumors with ciliary body extension.
masses in the neck or abdomen, pathological
fractures etc. which may indicate distant meta Vitreous: Look for cellular infiltration of the
stases. In the skin examination, look for nevi, anterior vitreous—If present indicates an
atypical nevi, cutaneous melanocytosis and inflammatory pathology.
freckles—all of which are risk factors for the Fundus: A distant direct ophthalmoscopy exami
development of uveal melanoma. nation may reveal a dark shadow in the area of the
mass, a ciliary body mass on the other hand may
Ocular Examination obliterate the red reflex all together. Choroidal
Visual acuity: As discussed above, visual acuity melanomas are usually seen as dome shaped
may be decreased in certain presentations of masses smoothly arising from the underlying
melanoma. choroid varying in size from <3 mm to large tumors
over 15 mm in base diameter. The height of the
Eyeball and lids: Look for proptosis, chemosis and tumor is more important as a differentiating feature
restriction of movements—all of which suggests from benign nevus (Tables 2 and 3). When they
an extraocular extension of the melanoma. break through the bruch’s membrane they take
Conjunctiva: A sentinel vessel might be observed the classically described collar-stud appearance
in cases of ciliary body melanoma or an anteriorly or mushroom appearance. They can arise anywhere
Table 1 Differentiating choroidal melanoma from other mass like choroidal pathologies
Cicrumscribed Congenital Optic disc
Character- Choroidal Choroidal choroidal Choroidal hypertrophy of melanocy- Posterior
istic melanoma Choroidal nevus metastasis hemangioma osteoma RPE toma scleritis
Age/Sex 5th decade and Adoloscence Elderly 30–50 years 10–30 years, Congenital 30–40 years 30–50 years,
above 90% female female
Laterality Unilateral Unilateral 20% bilateral Unilateral 20% bilateral Mostly unilateral Unilateral Unilateral
Location Posterior pole or Posterior to Posterior pole Within 2DD Juxtapapillary/ Peripheral ONH Peripheral/
periphery equator from ONH circumpapillary posterior pole
Symptoms Occasional visual Asymptomtic Occasional Occasional Occasional Asymptomatic Enlarged Pain
loss visual loss visual loss visual loss blind spot
Clinical • Amelanotic Amelanotic • Amelanotic Orange-red, Yellow-white Jet black, rarely Jet black Yellowish or
appear- or darkly to darkly or pale <5 DD, RPE to orange, well amelanotic, well to brown, same color as
ance pigmented pigmented, • Broad based changes and demarcated, defined, flat to fibrillated adjacent RPE,
• Dome/ Flat, <5 DD, with variable SRF + pseudopod ovoid, overlying margin, concentric
mushroom Overlying drusen thickness margins, RPE amelanotic choroidal choroidal
shaped (50–80%), • Overlying changes, lacunae nevus can be folds
• Shifting SRF + Rare: Orange RPE changes vascular spider, associated
• Overlying pigment/SRF/ • Often exten occasional CNV
orange pigment CNV sive SRF
Fluorescein Early hyper Hypofluores- Diffuse late Early prearte- Irregular Hypofluores- Hypofluores- Hyperfluores-
Angio fluorescence cence unless leak rial hyperfluo- fluorescence cence with cence, late cent mottling
graphy with late leakage drusen/RPE rescence of with late hyperfluores- ON hyper- with multiple
and stippling changes large choroi- staining cence of fluorescence pinpoint
dal vessels, lacunae is possible areas of leak-
late staining/ age
leakage
Ultrasono • Low-medium <2 mm thick • Flat/multi- Homogenous • High initial Flat Slight High internal
graphy reflectivity, variable nodular medium- spike (low elevation, reflectivity,
• Kappa sign reflectivity • High irregu- high internal gain) irregular thickened
• Regular internal lar internal reflectivity • Acoustic height sclera and
structure reflectivity shadowing reflectivity choroid, fluid
• Choroidal in subtenon
excavation, space (T-sign)
“hollowing”,
subjacent
Retina
shadowing
293
Table 2 Differentiating a choroidal melanoma from a choroidal nevus

Characteristic Choroidal nevus Choroidal melanoma
Age Any age, discovered on screening 6th or 7th decade
Symptom More likely to be asymptomatic. But can More likely to be symptomatic. Symptoms
cause vision loss due to SRF, CME or CNVM described above
Margins Well defined Poorly defined or abruptly elevated edges
Elevation Tends to be flat (<2 mm) >2 mm
Drusen More likely Less likely
Depigmented halo Characteristic Absent
Orange pigment Less likely More likely
Subretinal fluid Less likely More likely
Overlying RPE Likely Likely
changes
Growth Very slow (0.5 mm in a decade) Rapid (Over 1–2 years)
Table 3 Showing the definitions used in COMS

COMS Apical height Largest basal diameter
Small tumor 1.5–2.4 mm 5–16 mm
Medium sized tumor 2.5–10 mm ≤16 mm
Large sized tumor >10 mm >16 mm
in the fundus either de-novo or from a pre-existing

choroidal nevus. They are usually pigmented in
most of the cases (about 85% of the cases) but
about 15% are amelanotic—which means they do
not show any melanin clinically. The color per-se
could vary from dark brown to tan (Fig. 1). About
5% of the tumors are diffusely infiltrative without
any nodular mass. About 60% of the tumors are
located within 3 mm of the optic disc or fovea.
They could be bilobular or multilobular and could
even be multicentric.
The retinal pigment epithelium (RPE) over
lying the tumor often show stress changes such
as drusen, mottling, areas of atrophy, pigment Fig. 1: A pigmented choroidal melanoma with
epithelial detachment etc. owing to the depriva surrounding subretinal fluid and orange pigmentation
tion of normal choroidal circulation to the RPE over it
due to the presence of the tumor. Orange pig
ment on the melanoma is an important sign to neurosensory retina may also show chronic
differentiate it from the benign choroidal nevus degenerative changes such as cystoid spaces,
and represents the accumulated melanin and schitic cavity and CME.
lipofuscin in the RPE cells after phagocytosis of the Exudative RD is commonly associated with
cellular debris of the melanocytes. The overlying choroidal melanoma and is again an important
Retina 295
differentiating feature from choroidal nevus described four cardinal acoustic hallmarks of
(Table 2). The exudative RD may be so bullous it malignant melanoma: (1) A regular internal
can sometimes hide the tumor under it and appear structure with similar height of the inner tumor
like an RRD. Typically the sub retinal fluid is seen spikes or regular decrease in height (positive
surrounding the mass, and largely spread fluid angle kappa sign); (2) low to medium reflectivity;
indicates a possibility of choroidal metastasis. (3) solid consistency; and (4) echographic sign of
Associated intra and subretinal hemorrhage is vascularization with a fast, spontaneous, conti
generally a sign of development of choroidal nuous, flickering vertical motion of single tumor
neovascular membrane in a long standing spikes.
dormant melanoma. Vitreous hemorrhage and B-mode ultrasonography can help detect
massive subretinal hemorrhage can occur in tumors and detail the internal characteristics in
melanoma owing to erosion of a large retinal/ cases of exudative detachment and hazy media.
choroidal vessel by the tumor or due to necrosis of It can help in measuring the size, extent, spread
a part of the tumor. and in ruling out differentials. The characteristic
findings are: (1) homogenous internal structure
INVESTIGATION with low to medium reflectivity; (2) choroidal
excavation; (3) shadowing of the subjacent
Choroidal melanoma is a clinical diagnosis that is choroidal structures; (4) an acoustically empty
aided by ancillary diagnostic testing. Collaborative zone at the base (acoustic hollowing). A ‘collar
ocular melanoma trial showed that clinical stud’ appearance is highly suggestive of choroidal
evaluation along with fundus photography, melanoma. It will also define the area of RD.
ultrasonography and fluorescein angiography is
99% accurate in diagnosing a choroidal melanoma.
Fluorescein Angiography
Ultrasonography Fluorescein angiography (FA) is not diagnostic
and adds limited information to the diagnosis.
Ultrasonography (Fig. 2) is the most useful investi It helps in ruling out hemorrhagic lesion such
gative modality in the diagnosis of choroidal as a ruptured retinal arterial macroaneurysm
melanoma.3 A standardized ultrasonography has a or peripheral choroidal neovascularization,
diagnostic accuracy of >95%. On A scan, Ossoinig which generally blocks fluorescence. Choroidal
melanomas on fluorescein angiography show their
intrinsic vasculature different from the overlying
retinal vasculature and this is called “dual
circulation”. There may be early stippling and late
leakage and staining. There are no pathognomic
signs on angiography for choroidal melanoma.
Indocyanine Green Angiography

Indocyanine green angiography (ICG) angio
graphy better delineates the extent of mass
since the infrared red waves used penetrate
the RPE well. They can also pick up choroidal
neovascularization. It helps in differentiating a
melanoma from a choroidal hemangioma, the
Fig. 2: Combined ultrasonography A- and B-scan latter shows a wash out of dye.
showing a mushroom shaped mass arising from
the choroid with surrounding subretinal fluid and
Fundus Autofluorescence
homogenous internal reflectivity. A-scan shows
moderate spikes. There is acoustic hollowing towards Intense diffuse or confluent hyperautofluore
the base of the tumor scence is seen in melanoma.
Optical Coherence Tomography phosphatase are the basic screening test since
liver is the most common site of metastasis. If
The OCT (EDI) may be useful in detecting tumors
these enzymes are elevated CT scan of the
<3 mm in size which are difficult to pick up on
abdomen or ultrasonography can be considered.
clinical examination and ultrasonography. It
PET-CT scan should be considered if available
is also helpful in confirming the presence of
as metastasis was believed to be the rule
subretinal fluid in small doubtful lesions. Presence
(Zimmermans hypothesis), even after successful
of subretinal fluid shifts the diagnosis more
excision of the tumor. Other sites of systemic
towards melanoma than towards choroidal nevus.
spread include bone marrow, skin, brain and
Secondary retinal changes like CME, atrophic
lungs.
changes are often evident overlying the lesion.
Recently, due to the enhanced choroidal imaging,
OCT characteristics of melanoma and other DIFFERENTIAL DIAGNOSIS
choroidal tumors have been defined, the focus Since the clinical manifestation and appearance of
being on shape and edge of the tumor, as well as the tumor are highly variable, numerous differen
the internal vascularity. Still like FA, these features tials need to be considered in a case of choroidal
are not conclusive of a differential diagnoses. mass. Table 1 summarizes the differentials in a
tabular form.
CT Scan
Melanomas appear hyperdense on CT scanning MANAGEMENT
with moderate contrast enhancement. It is useful
for evaluation of metastasis. Treatment in a case of choroidal melanoma
depends on the size and extension of the tumor,
Magnetic Resonance Imaging life expectancy of the patient and visual potential of
the eye. Treatment is tailor made for each patient.
For ocular diagnoses, MRI is more useful than CT
scan. On MRI, melanomas (like retinoblastoma)
are hyperintense to vitreous on T1 and hypo Observation
intense to vitreous on T2. It is also useful in Increasingly the trend in ocular oncology is to treat
assessing extraocular spread and in ruling out smaller melanomas to reduce the risk of meta
some of the differentials. stasis. But observation still has a role especially
in tumors <2.5 or 3 mm in height and <10 mm in
Tissue Diagnosis diameter which are very slow growing or in tumors
When clinical methods and non-invasive investi in whom an unequivocal diagnosis of choroidal
gative modalities fail to conclusively prove or rule melanoma could not be reached. Also in patients
out the diagnosis of a melanoma, tissue diagnosis with limited life expectancy or untreatable
by a fine needle aspiration or a transscleral conditions, observation might be a prudent choice.
biopsy or a bimanual biopsy with chandelier
assisted 25-G vitrectomy system may be useful. Enucleation
One should be careful of port/aspiration site Enucleation is the classic treatment modality
spread, and cryotherapy should be considered at for large melanomas or melanomas with poor
the entry sites. Also if diagnoses is proven to be visual potential or extensive spread within and
melanoma, earliest possible further management outside the globe. The COMS also suggested
(like enucleation should) be done to avoid tumor enucleation for “large” tumors (see below). Addi
spread. A lymph node biopsy or that of other site of tional precautions must be taken in the form of
spread may also be done if appropriate. performing an indirect ophthalmoscopy after
draping the parts to confirm the correct eye is
Systemic Investigations enucleated. In large tumors pre-enucleation
It is directed principally towards detecting meta radiotherapy may not provide additional mortality
static spread. Hepatic transaminases and alkaline benefit. If there is extrascleral extension, the entire
Retina 297
tumor should be removed en bloc to reduce the difficult and are indicated in carefully selected
risk of residual disease. All treatment modalities tumors that are too thick for radiotherapy but
developed subsequently to preserve globe and less than about 16 mm in diameter. The results
salvage some useful vision are compared against are difficult to compare as long term follow-up
enucleation in terms of mortality and metastasis. is needed to disprove systemic spread. Orbital
exentration in rarely done for melanoma with
Episcleral Plaque Brachytherapy orbital extension because the disease has generally
Brachytherapy is done by suturing an Iodine-125 reached an advanced stage with extensive meta
or Ruthenium-106 plaque to the sclera over the stasis by this time. Nonconventional approach
area of the tumor. This may be used for medium should be considered where visual/eye ball
sized tumors with some potential for salvaging salvation is possible.
vision and <15 mm in basal diameter and up to
10 mm in height. Survival is similar to that follow Systemic Chemotherapy
ing enucleation. Complications include cataract, Systemic chemotherapy is used only in cases with
papillopathy (with or without disc neovasculariza metastatic disease.
tion) and radiation retinopathy. About 25% of the
patients develop iris neovascularization. VIVA QUESTIONS
External Beam Radiotherapy Q.1. What are the risk factors for choroidal
melanoma?
Melanomas are radio resistant. To radiate them Ans. Risk factors include fair skin, lighter
using conventional external-beam radiotherapy iris color, blonde hair, chronic sun light
techniques would require high radiation dose and exposure, choroidal or iris nevus, nevus
this subsequently leads to severe complications. of Ota, atypical nevus syndrome, multiple
Modern techniques of delivering precise high nevi, exposure to arc welding, uveal
dose radiation like proton beam irradiation and melanocytoma and BRCA-1 associated
stereotactic radiotherapy can be used for tumors protein 1 (BAP1) mutation. The exposure
that are unsuitable for brachytherapy due to their to sunlight is although hypothesized and
large size or posterior location. These techniques believed widely, it has not been proven by
cause minimal damage to the adjacent tissues. epidemiological studies.
Their efficacy is similar to brachytherapy.
Q.2. What is the diagnostic accuracy of ultra
sonography in diagnosing choroidal
Transpupillary Thermotherapy and
melanoma?
Photodynamic Therapy Ans. Combined A- and B-scans have a diag
These modalities can be used to treat smaller nostic accuracy of over 95% in diagnosing
lesions. In some reports, double fluence choroidal melanoma. (COMS showed
(100 J/cm2) and double duration (166s) of PDT that clinical diagnosis which included
have been used. While TTT is more useful in clinical examination, fundus photography
pigmented tumors, PDT is useful in amelanotic and ultrasonography was 99% accurate in
tumors. diagnosing choroidal melanoma).
Q.3. What are the histopathological features
Surgical Management of choroidal melanoma?
Melanomas can be removed through the scleral Ans. Calender in 1931 developed a histopatho
approach or through pars plana vitrectomy. In the logical classification system for uveal
scleral approach, the tumor is removed en-bloc melanomas depending on the most
with surrounding choroid and overlying sclera if prominent cell type in a tumor. This
involved. In the pars-plana approach the tumor is included tumors classified as:
removed in piece meal through a vitreous cutter • Spindle cell subtype A
(Endoresection). These procedures are technically • Spindle cell subtype B
• Epitheloid type another important prognostic marker—

• Fascicular type and 5 year survival is poor if there is extrascleral
• Mixed type. extension. Anteriorly located tumors
There were inherent limitations in this present much later and therefore has
system and therefore this classification was poorer prognosis. Among medium sized
revised in 1983 by McLean and he classified tumors, posteriorly located tumors have
uveal melanomas as follows: poorer prognosis since it is difficult to
• Spindle cell melanoma (composed of provide brachytherapy to more posterior
spindle B cells) sites. Ciliary body invasion of choroidal
• Epithelioid cell melanoma and tumors indicate a poor prognosis. Tumors
• Mixed types melanoma. recurrent after brachytherapy or external
McLean did not classify Spindle A cells radiotherapy also behave poorly. Tumor
as malignant. They are fusiform cohesive genetic characteristics such as somatic
cells which form choroidal nevus. Spindle mutations in the BAP1 gene are also linked
B cells are also fusiform but are plumper to greater chance of metastasis.
and have poorly defined margins. Liver, bone and lung are the common
Epitheloid cells are round or pleomorphic, sites of metastasis. At presentation only
non-cohesive cells with large nucleus, about 1–2% of patients have detectable
prominent mitotic figures and defined metastases but mortality is up to 50% at
borders. There are also intermediate 10 years.
cells—intermediate between spindle and Q.5. What is a diffuse infiltrating choroidal
epitheloid. These cell types and other melanoma?
histopathological features can only be Ans. It is a variant of choroidal melanoma which
detected on enucleation or block resection does not present as a tumorous mass
specimens. Needle biopsy and tumor but shows lateral growth in the choroid
removed in piece meal are difficult to type. and presents with exudative RD. It has
Choroidal melanomas arise de novo been defined as a tumor <5 mm in height
from the melanocytes in the uveal tissue. and occupying >25% of the uveal tract.
They can also arise from a choroidal It accounts for about 5% of all the choroidal
nevus (in which case histopathology may melanomas. It is difficult to diagnose
show spindle A cells) or a melanocytoma. because of the absence of the tumor and
Immunohistochemical markers of mela presence of an exudative RD. It generally
noma are S-100 and HMB-45. has poor prognosis.
Q.4. What are the prognostic factors of Q.6. What is collaborative ocular melanoma
choroidal melanoma? study?
Ans. Tumor size is the most important pro Ans. Collaborative ocular melanoma study
gnostic factor. 4,5 Several studies have (COMS) is a National Eye Institute
shown that large tumors have the highest sponsored multicentric three armed study
5 year mortality rate—53% while small and started in 1985. The first arm studied
medium-size tumors has a mortality of the natural history of small choroidal
16% and 32% respectively. Tumor histology melanoma. The other two arms were
is another important prognostic factor. randomized control trials evaluating
Epitheloid tumors had a 10-year mortality treatment options of medium and large
of 72–100% while the spindle A tumors sized tumors. Iodine-125 brachytherapy
has a 10 year mortality of 11–19%. Modern was compared against enucleation for
histopathological prognostic markers are medium sized tumors and enucleation
indices such as number of epitheloid cells alone was compared to enucleation
per hpf and inverse standard deviation of preceded by external beam radiotherapy
nucleolar area. Extrascleral extension is in large tumors. The definitions of small,
Retina 299
medium and large tumors are summarized Therefore, the surgery per se is not the cause
in Table 3. of the increased postenucleation mortality
The natural history arm recruited 204 but the treatment of the tumor is.
patients. 6 deaths occurred due to meta Q.8. What are the pros and cons of endo
static melanoma. The 5-year tumor specific resection of tumors, and how are the
mortality rate was 1%. The medium sized results?
tumor trial showed that there was no statis Ans. Endovitreal resection of medium to large
tically significant difference in the mortality sized choroidal melanomas (limited to
rates of patients treated with enucleation the globe) have been reported by multiple
and iodine-125 brachytherapy. The risk of authors. The advantages of this procedure
treatment failure in case of brachytherapy are globe preservation, preservation of
was 10% and was increased in patients some visual function, removal of the tumor
with thicker tumors and in patients with in its entirety, abundant sample for histo
posterior tumors. In the large sized tumor pathology and genetic studies, avoiding
trial, pre-enucleation brachytherapy was radiation complications and photocoagul
not found to significantly affect survival. ation of miscroscopic elements under
Q.7. What is Zimmerman’s hypothesis? direct observation. The disadvantages of
Ans. Zimmerman, McLean, and Foster in 1978 this procedure are the theoretical risk of
hypothesized in their paper that enuclea tumor seeding and metastasis (although
tion may accelerate metastases. This was the MIVS systems with the trocar and
based on their observation of a peak in cannula entry have been shown to be safe),
the mortality rates 2 years postenucleation need for hypotensive anesthesia, demands
which after 6 years stabilized to the pre- more skill, need for multiple surgeries.
enucleation rates. They hypothesized that The reported results are good, comparable
tumor manipulation during surgery caused to other globe salvaging procedures in
dissemination of the tumor causing a spike terms of metastasis. Survival data is as yet
in metastasis and mortality. Based on this limited.7
they also suggested high vigilance during
enucleation for uveal melanoma and pre- REFERENCES
enucleation radiotherapy.6 1. Ryan SJ, Schachat AP, Wilkinson CP, Hinton
Subsequent epidemiological studies DR, Sadda SR, Wiedemann P. Retina. Elsevier
have also shown a post-therapeutic spike Health Sciences; 2012.
(spike after any form of treatment to the 2. Esmaeli B. Ophthalmic Oncology. Springer US;
melanoma—enucleation, brachytherapy or 2010.
proton beam radiotherapy) in the mortality 3. Ossoinig KC. Standardized echography: basic
rates in accordance with the observation principles, clinical applications, and results. Int
made by Zimmerman et al. The current Ophthalmol Clin. 1979;19(4):127-210.
understanding of this phenomenon is that 4. Kaliki S, Shields CL. Uveal melanoma: relatively
it involves host-tumor interaction and host rare but deadly cancer. Eye (Lond). 2016.
immune mechanisms. Also there could be 5. Margo CE. The Collaborative Ocular Melanoma
Study: An Overview. Cancer Control. 2004;
a role played by antiangiogenic mediators
11(5):304-9.
such as angiostatin produced by the
6. Singh AD, Rennie IG, Kivela T, Seregard S,
primary melanoma which may have growth Grossniklaus H. The Zimmerman-McLean-
inhibitory effects on the micrometastases. Foster hypothesis: 25 years later. The British
Removal/treatment of the primary tumor Journal of Ophthalmology. 2004;88(7):962-7.
alters the host immune response and the 7. Venkatesh P, et al. 25 Gauge Endoresection
antiangiogenic signals from the primary for Moderate to Large Choroidal Melanoma.
tumor and causes micrometastases to Indian J Surg Oncol. 2015;7(3):365-7.
progressively grow and cause mortality.
SHORT CASES
CHERRY-RED SPOT
Rajesh Pattebahadur, Brijesh Takkar
Cherry-red spot (CRS) is an important finding Systemic Examination
suggestive of a large group of diseases. Among
Detailed examination may reveal systemic findings
these diseases, diagnosis of metabolic disorders
of the underlying cause, in a case of CRS.
helps in prognosticating the condition, while early zz Cardiovascular system: Patient may be having
diagnosis of conditions like central retinal artery
hypertension along with valvular heart
occlusion (CRAO) help in early management and
disease.
salvaging the permanent loss of vision.1 zz Abdominal system: Hepatosplenomegaly
is important finding suggestive of severe
HISTORY metabolic abnormalities. Along with this
Chief Complaint ascites can also be seen.
zz Neurological system: Delayed response, mental
As stated earlier CRS is not a disease, a patient retardation, myoclonic jerks, hypotonia are
with history of metabolic disorder may show CRS important findings.
on fundus examination. Also a case of sudden zz Respiratory system: Interstitial pneumonia can
diminution of vision (CRAO) or ocular contusion be present.
will have CRS on fundus examination. zz Along with these other features includes
coarse facies, bone changes.
History on Present Illness
The patients age of presentation will have different Ophthalmic Examination
set of complaints and different sets of diagnosis.
In pediatric age group patient may show hepa Following points must be recorded:
tosplenomegaly, bone changes, neurological
zz Uncorrected and best corrected visual acuity
changes, ascites, myoclonic jerk, mental retarda
zz Globes are generally aligned
tion. Whereas in adults, history of trauma to eye zz Eyelids are generally within normal limit
can be present or sudden diminution of vision zz Conjunctiva do not show any specific finding
(CRAO) may be there. but in cases of ocular trauma may show
bleeding or tear in conjunctiva.
Medical History
zz Corneal clouding can be seen, typical of
underlying systemic diseases.
Past medical history of hypertension, dysostosis zz Anterior chamber is usually within normal
multiplex (DM), hypercoagulable states, sickle limit. Angle recession can be seen in cases
cell, atherosclerosis, cardiac valvular disease, of sever ocular trauma. Iridodialysis, lens
sepsis, intravenous drug abuse may be there. subluxation, post-traumatic cataract are few
finding which suggest the ocular trauma as
Family History cause for the CRS.
Family history is important in cases with metabolic zz Distant direct ophthalmoscopy may not reveal
disorders associated with the CRS. any specific finding unless there is cataract.
Retina 301
hypertension, one-fourth have carotid occlusive

disease, diabetes or cardiac valvular disease or
a combination. Bilateral involvement is rare and
suggestive of arteritic disease.1,2
MANAGEMENT
Treatment involves treating the underlying
cause. CRAO, an ophthalmic emergency should
be treated within 24 hours. Medical and surgical
lowering of intraocular pressure, carbon dioxide
rebreathing, steroids (in vasculitis), vasodilator
drugs, hyperbaric oxygen, antifibrinolytic drugs,
barbiturate coma, free radical scavengers and
Fig. 1: Fundus photograph of cherry-red spot in a antioxidants have been tried with variable results.
case of central retinal artery occlusion (CRAO) The CRS resolves in due course with resolution of
edema, however, optic atrophy ensues.1,2
Indirect Ophthalmoscopy
zz Detailed examination should include cup disc VIVA QUESTIONS
ratio, neuroretinal rim, arteriovenous ratio and
foveal reflex. Q.1. What is cherry-red spot (CRS)?
zz The CRS is visualized as a bright to dull red Ans. It is a clinical sign seen in the context of
spot at the center of macula, surrounded and thickening and loss of transparency of
bordered by a grayish white or yellowish halo posterior pole of the retina. The CRS is
(Fig. 1). visualized as a bright to dull red spot at
the center of macula, surrounded and
DIFFERENTIAL DIAGNOSIS accentuated by a grayish white or yellowish
halo. Its color is due to the pigment
As said earlier the underlying cause has to be epithelium and choroid, and therefore may
identified. The common causes are: demonstrate color variability according to
zz Bilateral CRS: Metabolic disorders, quinine the race.1
and other drug toxicity and Leber’s congenital
amaurosis. Q.2. What is differential diagnosis for CRS?
zz Unilateral CRS: CRAO, orbital contusions, Ans. See Table 1.
macular hole with retinal detachment or Q.3. What are causes of CRS—like lesions and
macular hemorrhage. pseudo CRS?
The CRS is more commonly associated with Ans. Certain illnesses are associated with
CRAO in adults. The age, health of patient, history macular lesions resembling a CRS. These
of trauma or vascular disease and unilaterality include:
of lesion may help to distinguish it from the • Adult Niemann-Pick disease (ring of
metabolic disorders. In such instances providing perifoveal crystalloid deposits)
emergency management and treating the • G aucher’s disease (atypical macular
underlying disorders or risk factors constitute vital CRS)
therapeutic interventions.1,2 • Lactosyl ceramidosis (increasing redness
In children with CRAO hemoglobinopathies of macula)
like sickle cell disease, hypercoagulable states • Sea blue histiocyte syndrome (perifoveal
like antiphospholipid antibodies and vasculitis yellowish white scintillating granules in
due to systemic lupus erythematosus are more doughnut shaped pattern)
common causes, whereas in adults two-thirds Conditions like macular hemorrhage or
of all patients with CRAO have associated macular hole with retinal detachment could
Table 1 Differential diagnosis of cherry-red spot prominent. Atrophy of retinal nerve fiber
layer and optic atrophy may also follow.
• Central retinal artery occlusion (CRAO) • I n CRAO the fundoscopy reveals a
• Orbital contusion diffuse retinal arteriole constriction
• Orbital ischemia
often with visible emboli or blood flow
• Tay-Sachs disease
segmentation. The fovea retains its blood
• Sandhoff’s disease
• Sialidosis supply via the choroidal circulation while
• Infantile Niemann-Pick disease type IA the surrounding retina appears milky
• GM1 gangliosidosis white due to infarction, intracellular
• Metachromatic leukodystrophy edema, cellular necrosis and cellular
• Goldberg’s disease debris accumulation.
• Gaucher’s disease (infantile form), Hurler’s • The CRS seen in methanol poisoning is
syndrome due to macular cystoid edema and in
• Mucopolysaccharidosis VII quinine poisoning due to retinal edema.
• Hallervorden Spatz syndrome • In macular hemorrhage, blood which is
• Batten-Mayou; Vogt-Spielmeyer syndrome darker red than the retina contributes to
• Spranger’s disease the CRS appearance.
• Cryoglobulinemia
• Leber’s congenital amaurosis Q.5. Which are predisposing conditions for
• Drugs—Quinine, carbon monoxide, methanol the CRAO?
and dapsone toxicity Ans. Conditions that predispose to CRAO
include retinal emboli due to endogenous or
exogenous source; hypertension, diabetes
mellitus, carotid occlusive disease, cardiac
be considered as pseudo-CRS, because the valvular disease, atheromatous vascular
abnormality is in the foveola rather than disease, arteriosclerotic vascular disease,
parafoveal area. vasculitic syndromes (temporal arteritis,
Q.4. What is pathophysiology of CRS? SLE, etc.), blood dyscrasias (e.g. sickle
Ans. • The retina has around 1 million ganglion hemoglobinopathy), hypercoagulable
cells. states (e.g. antiphospholipid antibodies),
• However, macular region (foveola, in sepsis and DIC, vasospasm, vascular
particular) is almost devoid of these cells. compression, cervical trauma with carotid
• Diseases associated with accumulation artery dissection, intravenous drug use and
of storage material (such as glycolipids migraine.
or sphingolipids) in the retinal cellular Q.6. How to diagnose and manage the CRS?
layers result in swelling and loss of trans Ans. In pediatric age group: Metabolic diseases
parency of the multilayered ganglion constitute the most common cause for CRS.
cells giving it a “white” appearance. The exact metabolic or storage disease can
• The foveola, the thinnest part of the be diagnosed on the basis of age of onset,
retina being devoid of ganglion cells, associated manifestations, inheritance
retains its relative transparency allowing pattern.
the normal choroidal vasculature to be In newborns:
seen through it. • Hepatosplenomegaly with vacuolated
• T hese histological features result in lymphocytes would suggest GM1
the appearance of the central red area gangliosidosis, Niemann-Pick disease
(normal foveola) that is surrounded by type IA or early infantile galactosialidosis.
dull halo resulting from attenuation of • An additional finding of coarse facies,
transparency of the surrounding area. bone changes, edema with ascites
• L ater in the course of the disease, would favor GM1 gangliosidosis or early
ganglion cell death makes the spot less infantile galactosialidosis; Interstitial
Retina 303
pneumonia and neurologic deterioration retardation would suggest juvenile

would suggest Niemann-Pick disease galactosialidosis.
type IA disease. Several inherited metabolic diseases have no
In infancy: definitive treatment. However, early diagnosis
• Exaggerated neuronal response, hepato allows for appropriate counseling and prenatal
splenomegaly, myoclonic jerks, hypo diagnosis (For example, determination of levels
tonia and neuroregression would suggest of hexosaminidase A and B levels in Sandhoff’s
a diagnosis of GM2 Gangliosidosis type 1 disease).
(Tay-Sachs disease) or type 2 (Sandhoff’s
disease). REFERENCES
• Whereas hepatosplenomegaly with bone
1. Suvarna JC, Hajela SA. Cherry-red spot.
changes would favor a diagnosis of late J Postgrad Med. 2008;54:54-7.
infantile galactosialidosis. 2. Chen H, Chan AY, Stone DU, Mandal NA. Beyond
• In late childhood, blindness and pro the cherry-red spot: Ocular manifestations of
gressive myoclonic jerks suggest Sialidosis sphingolipid-mediated neurodegenerative and
(CRS Myoclonus syndrome) whereas inflammatory disorders. Surv Ophthalmol.
bone changes, dysmorphism, angiokera 2014;59(1):64-76.
toma, corneal opacities and psychomotor
CENTRAL SEROUS CHORIORETINOPATHY

Vaishali Ghanshyam Rai, Raghav Ravani
INTRODUCTION zz Unilateral metamorphopsia is the classic

symptom.
Central serous chorioretinopathy (CSCR) is a
maculopathy characterized by idiopathic circum
zz Patients may also present with unilateral
scribed serous retinal detachment, usually con blurred vision, micropsia, impaired dark
fined to the central macula. It is a favorite short adaptation, color desaturation and a relative
case for PG exams. scotoma.
HISTORY Past History

Demography Following points must be noted:
zz History of any emotional stress.
Central serous chorioretinopathy (CSCR) typically
zz History of type A personality is important.
affects individuals in the 20–50 years age range.1,2
Males are much more commonly affected than
zz History long-term steroid use in any form, e.g.
females. The incidence in men was approximately oral, inhaler or topical.
six times higher than in women. Younger patients
zz History of pregnancy in female patients.
usually have unilateral involvement, while zz History of any organ transplant in past should
older patients are more likely to have bilateral be taken.
involvement in CSCR. zz History of similar complaint in past is
important to rule out recurrent CSCR.
Chief Complaint
The patient can present with following: EXAMINATION
Recent unilateral painless diminution of vision
General Examination
zz
is the most common symptom. Often the

patient’s complaint is one of transiently seeing Look for presence of any disease requiring chronic
a dark spot in the center of the visual field in steroid intake. Also, look for signs of steroid
one eye, with or without metamorphopsia. toxicity.
Ocular Examination pigment epithelium rarefaction or small

asymptomatic retinal pigment epithelium
Visual Acuity
detachments. Bilateral involvement
zz Visual acuity ranges from 20/15 (6/5) to 20/200 occurs in approximately 20% of patients.
(6/60) but averages 20/30 (6/9). —— Recurrence of CSCR can be seen in
zz The visual acuity may improve with hyperopic 40–50% of patients in long course. A
correction. patient can have recurrent focal leaks or
progress inexorably to the more visually
Anterior Segment threatening chronic CSCR.
It is usually normal. zz Chronic CSCR
—— It is characterized by a diffuse or multifocal,
Posterior Segment irregular retinal pigment epitheliopathy

that progresses in conjunction with per
zz Acute form of CSCR
sistent or intermittent subretinal fluid. It
—— Oval yellow-gray elevations at parafoveal
is also known as diffuse retinal pigment
or macular area may be seen (Fig. 1).
epitheliopathy (DRPE). Recently, multi
These are generally less than one-fourth of
focal posterior pigment epitheliopathy
a disc diameter in size and are surrounded
(MPPE) has also been described with
by a faint grayish halo.
—— Absence of the normal foveal light reflex.
multiple leaks with large amounts of
—— The subretinal fluid is usually clear, but
SRF. Subretinal fibrosis and choroidal
neovascular membranes (CNVMs) may
granular or fibrinous deposits may be
present in the subretinal space. The ensue as complications.
—— The retinal detachments tend to be
accumulation of granular/fibrinous
material between the RPE and the shallow and more diffuse than in the
neurosensory retina increases with the classic form and with amorphous sub
duration of symptoms. retinal deposits. Subretinal lipid that
—— Abnormalities of the RPE presents as one
typically appears as discrete, hard-edged,
or more yellow spots or a small pigment subretinal accumulations at the borders of
epithelial detachment (PED). a neurosensory detachment can be seen.
—— More often bilateral and may occasionally
—— The fellow eye may show evidence of
either concurrent or previously resolved present with gravitational tracts a term

CSCR, manifested as focal areas of retinal used for oblong, vertical patches of RPE
hypopigmentation that extend inferiorly.
These tracts are produced by subretinal
fluid of high specific gravity sinking
toward the inferior fundus and dissecting
its way through the subretinal space.
zz Rare forms
Bullous form of CSCR:
—— Rarely CSCR can present as a bullous,
inferior nonrhegmatogenous peripheral

retinal detachment. Atrophic tracts of the
RPE extending inferiorly to the serous
retinal detachment can be seen in such
cases.
—— It is often associated with subretinal
fibrinous exudates and shows the pheno

menon of subretinal fluid shifting position
Fig. 1: Fundus photograph of a case of CSR with changes in posture.
Retina 305
—— More common in Japan, and patients with thickening of the choroid on USG. These
history of organ transplant should always be considered for chronic and
Neovascular CSCR: Rarely as a complication of multifocal cases.
chronic CSCR. zz Polypoidal choroidal vasculopathy: Indo
cyanine green angiography of polypoidal
DIFFERENTIAL DIAGNOSIS choroidal vasculopathy demonstrates small-
caliber, polypoidal choroidal vascular lesions
Central serous chorioretinopathy (CSCR) must be and no areas of choroidal hyperpermeability.
differentiated from a neural retinal detachment zz Optic disc pit: The optic nerve pathology is
secondary to: visible on ophthalmoscopy. There are no leaks
zz Subretinal choroidal neovascularization on fluorescein angiography. OCT can show
(CNV): CNV present with thickening at the the tract connecting to optic nerve head along
level of the RPE, notched PEDs and subretinal with splitting of retinal layers, at times with an
or subpigment epithelial blood that are absent impending macular hole.
in CSCR. In addition, there will be coexistent
ocular findings related to the generation of INVESTIGATION
new blood vessel growth in eyes with CNV.
Indocyanine green angiography of subretinal Fundus Fluorescein Angiography
choroidal neovascularization usually reveals zz Fundus fluorescein angiography is not
only one area of hyperfluorescence that required in routinely, but is a good practice for
progressively enlarges during the later frames documenting the baseline disease, in chronic
of the study. forms and when suspecting other disorders.
zz Tumors and infiltrative conditions (leukemia, zz Fundus fluorescein angiography classically
amelanotic melanoma, or metastatic disease) shows, dye from the choroid leaks through a
these lesions generally have a different color focal RPE defect and pools in the subretinal
than the surrounding normal choroid; USG space (Figs 2 and 3). In more than 75% of
would show thickening of the choroid serous patients, this pooling occurs within 1 disc
PEDs are absent. diameter of the fovea, very common in the
zz Inflammatory (posterior scleritis or Harada’s superior nasal area.
disease): There will be signs of intraocular zz Fundus fluorescein angiography findings can
inflammation such as iritis or vitritis, and be described as ‘smokestack leak’ or the more
other sign’s such as patches of yellowish common ‘inkblot leak’ patterns. Multiple leaks
discoloration in the posterior pole, papillitis, may be seen in multifocal disease.
Fig. 2: Fundus photograph of a patient of CSR. Note Fig. 3: Fluorescein angiography (FA) picture of the
the fluid accumulation beneath the fovea and the patient in Figure 1. Large ink blot leak can be seen.
superior-temporal pigment mottling Staining is also visible in the area of pigment mottling
Indocyanine Green Angiography TREATMENT

zz Earlier considered gold standard, choroidal Observation
ischemia and dilatation of larger choroidal
Majority of cases of central serous chorioretino
vessels and hot spots corresponding to leaking
pathy (CSC) resolve spontaneously over period
areas can be seen.
of 3–4 months. Most often, as per conventional
zz Staining appears in the mid phase of the
teaching, the initial treatment of choice is
indocyanine green angiography (ICGA) and
observation. If the patient is using corticosteroids,
fades in the late phase.
these should be discontinued if medically possible.
zz It helps to differentiate CSCR from poly
The patient must be advised to avoid tobacco and
poidal choroidal vasculopathy, choroidal
smoking.
neovascularization.
zz Pick-up CNVMs when doubtful.
Acetazolamide
Optical Coherence Tomography Systemic acetazolamide treatment promotes the
resorption of subretinal fluid and case reports
zz Demonstrate the presence of subretinal fluid suggest that it may reduce subretinal fluid
(Fig. 4). in CSCR. However, there is no evidence that
zz Helps to quantify and follow the amount and treatment promotes healing of the retinal pigment
extent of subretinal fluid and to demonstrate epithelium (RPE) lesion, long-term preservation
thickening of the neurosensory retina. of visual function, or a reduced rate of recurrence.
zz Helps in diagnosis of doubtful cases.
zz Choroidal OCT imaging can reveal pachycho
Laser Photocoagulation
roid epitheliopathy in both eyes, now con
sidered a precursor of CSR. zz Indication
—— Extrafoveal leaks who fail to improve after
zz Posteriorly loculated fluid in choroid can also
be seen on choroidal imaging. 4–6 months
—— Demonstrate permanent changes from
CSCR in the other eye

Optical Coherence Tomography —— Demonstrate multiple recurrences
Angiography —— Occupational: Require improved vision
Role is under investigation. It can pick up CNVMs for work.

and has shown increased choroidal vascularity in zz Laser photocoagulation is applied to the site
both the eyes. of fluorescein leakage. The technique of laser
Fig. 4: OCT image showing subretinal fluid

Retina 307
photocoagulation involves using a green- effective in lowering PAI-1 levels and platelet
wavelength laser to produce a light scar over aggregation.
the focal RPE leak. zz Rifampicin is another drug that has been tried
zz Typically, 6–12 laser burns of 50–200 μm spot for CSR, though evidence level is low.
size at 0.1-second duration and 75–200 mW
are used. Permanent RPE change is induced at
the site of the laser scar. VIVA QUESTIONS
zz Treatment should be avoided if the leak occurs
Q.1. What are the risk factors associated with
within 300 μm of the center of the foveal
CSCR?
avascular zone.
Ans. See Table 1.
Q.2. What is the clinical course and outcome
Photodynamic Therapy
of CSCR?
zz Photodynamic therapy has been used to treat Ans. The visual prognosis is good in majority
chronic CSC (defined as >6 months’ duration of cases of CSCR. The majority of patients
of disease) with diffuse compensation of the suffer no significant permanent visual
RPE and lacking focal FA leak. However, recent loss. Although visual acuity usually
evidence from East Asia supports its role in improves, patients may continue to have
acute uncomplicated CSR as well, especially persistent metamorphopsia probably due
when done at low fluence. to a photoreceptor misalignment causing a
zz Photodynamic therapy is treatment that is Stiles-Crawford effect.
more effective with a lower complication Q.3. Mechanism of action of acetazolamide in
rate for patients with subfoveal or juxtafoveal CSCR.
leaks. Ans. Helps in absorption of the subretinal fluid
by inhibiting carbonic anhydrase pump
Transpupillary Thermotherapy located in the RPE and creating a localized
acidic milieu.
Few studies suggest that this treatment may
accelerate the resolution of CSC, but long- term
safety and efficacy are not known.
Others: Several other modalities have been Table 1 Risk factors and associations with
described.3 central serous chorioretinopathy
zz The beta-adrenergic receptor blocker pro Systemic Type A personality, emotional stress
pranolol and the mixed alpha and beta conditions pregnancy
adrenergic receptor blocker labetalol. Organ transplantation
zz Mifepristone as an antagonist of progesterone Systemic lupus erythematosus
Tobacco and alcohol use
and glucocorticoid receptors.
Membranoproliferative
zz Ketoconazole as an adrenocorticoid antagonist
glomerulonephritis type II
that has been shown to lower endogenous Helicobacter pylori infection
cortisol. Gastroesophageal reflux disease
zz Intravitreal bevacizumab has been investigated Systemic hypertension
for treatment of CSR; theoretically, the Medications Corticosteroids
antipermeability characteristics of this Antihistamines
antibody to vascular endothelial growth Sidenafil citrate
factor (VEGF) may allow for reduced leakage, Psychopharmacologic medications
favoring resorption of the exudative retinal Amphetamine
detachment in CSR. Antacids and anti-reflux medications
zz Aspirin: Plasminogen activator inhibitor-1 Sympathomimetics
(PAI-1) is increased in CSCR and aspirin is Antibiotics
Q.4. Hypothesis for pathogenesis of CSCR. Q.6. What are the indications for treatment of
Ans. Choroidal circulation anomaly of the CSR?
middle choroidal layers leading on to Ans. See chapter for answer.
focal choroidal ischemia, choroidal edema
which sets up a vicious cycle. This leads
REFERENCES
to accumulation of fluid in the choroid,
formation of PEDs which eventually 1. Liew G, Quin G, Gillies M, Fraser-Bell S.
give way to cause leakage and manifest Central serous chorioretinopathy: a review of
as SRF. Choroidal imaging confirms epidemiology and pathophysiology. Clin Exp
this by demonstrating pachychoroid Ophthalmol. 2013;41(2):201-14.
epitheliopathy, localized choroidal fluid 2. Ross A, Ross AH, Mohamed Q. Review and
and increased compensatory vascularity. update of central serous chorioretinopathy.
Curr Opin Ophthalmol. 2011;22(3):166-73.
Q.5. Histopathological classification. 3. Iacono P, Battaglia Parodi M, Falcomatà B,
Ans. Type 1: Only neurosensory detachment Bandello F. Central serous chorioretinopathy
Type 2: Neurosensory with pigment epithe treatments: A mini review. Ophthalmic Res.
lium detachment. 2016;55:76-83.
DIABETIC MACULAR EDEMA

Brijesh Takkar, Dhaval Patel
INTRODUCTION Past History

Macular edema is important cause of decrease In this condition, there should always be a parallel
in vision in cases with diabetes mellitus (DM). focus on the systemic features apart from a
Determination of diabetic macular edema (DME) meticulous ocular workup. Past history of diabetes
is important for ophthalmologists for the early mellitus, hypertension, coronary artery disease,
treatment and visual rehabilitation. DME is very asthma, diabetic nephropathy should be recorded.
frequently kept as short case or a spotter. Conditions such as pregnancy and dyslipidemias
are other risk factors, and should always be carefully
HISTORY recorded. History of use of insulin and the type of
antidiabetic drugs should be recorded as they influ
Chief Complaints ence macular findings and treatment outcomes.
A known case of DM will give history of diminution Importantly, the record of past fundus exami
of vision and other macular symptoms such as nations with grade of diabetic retinopathy recorded
scotoma or distorted vision. The patient may in last visits are essential.1-3 If any treatment, e.g.
also give history of inadequate control of the Laser or intravitreal injections was given in past,
blood sugar levels, or recent fluctuation in blood then detailed record, including the number of
sugar levels or even recent change in diabetic setting of PRP, time since last panretinal laser
medication. Sometimes, DME may be picked photocoagulation (PRP), number of intravitreal
up on routine diabetic screening without injections given, should be noted.
symptoms, especially when noncenter involving.
Also such patients often present after referral Family History
by an endocrinologist with minimal visual Family history of DM should be recorded. It is
symptoms. essential to ask for family history because DM
has familial inheritance. Each relative having
Retina 309
DM should undergo the fundus examination rim, arteriovenous ratio, foveal reflex along with
according to the type and duration since diagnosis. evidence of vitreomacular adhesion (VMA), reti
nal thickening, hard exudates, microaneurysms
EXAMINATION and soft exudates. One should perform a careful
peripheral retinal examination for features of pro
Systemic Examination liferative diabetic retinopathy (PDR) and slitlamp
Since DM is a disease affecting various systems of biomicroscopy examination is a must for classify
the body, detailed examination of cardiovascular, ing the macular edema (Fig. 1).
central nervous system, respiratory system, renal
system, should be done to rule out any comorbidity DIFFERENTIAL DIAGNOSIS
associated with diabetic retinopathy DR.
zz Retina vein occlusion
Ophthalmic Examination zz Ruptured macroaneurysm
Uncorrected visual acuity and Best corrected
zz Irvine-Gass syndrome
visual acuity should be noted. zz Radiation retinopathy
zz Hypertensive retinopathy
Eyeball may not show any specific finding. But it’s zz Subfoveal choroidal neovascularization.
not uncommon for a patient of DM to have cranial
mononeuropathy. If such mononeuropathy is
CLASSIFICATION
present, the patient will have corresponding
squint. zz The diabetic retinopathy study (DRS) first
Eyelids—blepheritis can be seen in patients of gave the term DME. Later, the early treatment
DM, lid edema may indicate nephropathy. diabetic retinopathy study (ETDRS) defined
clinically significant macular edema as
Conjunctiva does not show any specific finding,
presence of:
signs of dryness may be seen as a complication —— Retinal edema located at or within
of neuropathy and chronic conjunctivitis may be
500 μm of the center of the macula.
present. —— Hard exudates at or within 500 μm of the
Cornea—diabetic neuropathy can lead to neuro center if associated with thickening of

trophic keratopathy. So assessment of corneal adjacent retina.
sensation is important. Dryness and epitheliopathy —— A zone of thickening larger than 1 disc
may be noticed on slit-lamp examination. area if located within 1 disc diameter of
Anterior chamber and angle—neovascularization the center of the macula.
of iris (NVI) must always be ruled out.
Lens—status should be noted, since macular
edema can be precipitated or aggravated by
cataract surgery and may cause decrease in visual
acuity. Snowflake cataract is typically seen in
uncontrolled young diabetics.
Vitreous body should be examined for any hemor
rhage, pigments. Asteroid hyalosis is a known
association of DM and can preclude fundus
examination.
IOP measurement is important to rule out
glaucoma, open-angle glaucoma (OAG) is a known
association. Fig. 1: Fundus photograph depicting diabetic macular
edema (DME). Hard exudates can be seen along with
Fundus Examination
retinal thickening within the central area. Other signs
Documentation of details of disc finding should of non-proliferative diabetic retinopathy (NPDR) can
be done, including the cup disc ratio, neuroretinal also be seen
To characterize the severity of macular

edema and for treatment guidelines, the term
clinically significant macular edema (CSME)
is used.
zz The international classification system classi
fies DME as mild, moderate or severe, depend
ing on proximity of retinal thickening to the
foveal center.
zz Based on fundus fluorescein angiography
(FFA), DME may be classified as focal, diffuse
or ischemic.
zz Recently, Diabetic Retinopathy Clinical
Research (DRCR) network has classified it
Fig. 2: Fluorescein angiography (FA) picture of diffuse
as a center involving (within 300 μm) and
diabetic macular edema (DME). Diffuse leaks with few
peripheral.
scattered microaneurysms can be seen. Peripheral
laser marks are also visible
INVESTIGATIONS
Fluorescein Angiogram
The fluorescein angiogram (FA) is used to identify
areas of increased vascular permeability, for
example, leaking microaneurysms or capillary
beds, and to evaluate retinal ischemia. Leakage on
the angiogram does not necessarily indicate retinal
edema since extracellular edema requires that the
rate of fluid ingress into the retina (i.e., as indicated
by leakage on the FA) exceeds the rate of fluid
clearance from the retina (e.g. via the RPE pump).
CSME is further classified into focal or diffuse,
depending on the leakage pattern seen on the
fluorescein angiogram (FA). In focal CSME, Fig. 3: Fluorescein angiography (FA) picture of macular
scattered points of retinal hyperfluorescence ischemia. Distortion of FAZ with increased size is
are present on the FA due to focal leakage. The seen, irregular borders are accompanied by pruned
vasculature
source of this focal leakage are microaneurysms.
It is hypothesized that leaking microaneurysms
are cause of retinal thickening. Commonly, these
collaterals and foveal avascular zone (FAZ)
leaking microaneurysms are surrounded by
changes like irregular shape, increased size with
circinate rings of hard exudates. The exudates are
pruned arterioles.
lipoprotein deposits in the outer retinal layers.
In diffuse DME, areas of diffuse leakage are
noted on the FA due to intraretinal leakage from Optical Coherence Tomography
a dilated retinal capillary bed and/or intraretinal The optical coherence tomography (OCT) has
microvascular abnormalities (IRMA), and/or (in been used for high-resolution imaging of the
severe cases) from arterioles and venules without retina and detection of increased retinal thickness
discrete foci of leaking microaneurysms (Fig. 2). (Fig. 4). There are several studies done for use
There may be associated cystoid macular edema. of OCT in case of CSME. OCT can he used for
Cystoid macular edema results due to fluid accu measuring macular volume, determining central
mulation, primarily in the outer plexiform layer. retinal thickness and determining the type
Ischemic maculopathy (Fig. 3) in presence of of edema. Recently, distortion of inner retinal
large capillary nonperfusion (CNP) areas, macular layers has been described for ischemic DME.
Retina 311
Fig. 4: Optical coherence tomography (OCT) picture of hemi-field DME.

Large cystoids spaces are visible in middle retinal layers
Hyper-reflective foci are also seen in DME. Multifocal electroretinography (MFERG) and
Choroidal thickness is under evaluation for DME microperimetry: These are more of research tools.
with special focus on treatment-induced changes.
Patterns of OCT findings associated with CSME: MANAGEMENT
zz Spongiform
Medical Therapy
zz Cystoid
zz Sensory detachment This includes:
zz VMT zz Strict control of diabetes, hypertension, and
zz TRD and mixed. hypercholesterolemia
Macular thickness map is a specific protocol zz Diet modification
used for analysis of macular thickness on OCT that zz Weight loss
measures in 9 thickness sectors centered on fovea zz Exercise.
in 3 rings of diameter of 1, 3 and 6 mm. Controlling systemic parameters are extremely
Compared to clinical examination, OCT is a important. High-grade evidence in support has
more sensitive and specific method for macular been given by DCCT, UKPDS, ACCORD and
evaluation. It is an excellent tool for follow up FIELD trials. The standard of treatment for CSME
and deciding treatment endpoints, prognosis and has been focal laser photocoagulation in the days
measuring response to therapy. of ETDRS with 50% prevention of moderate visual
loss at the end of 2 years (Table 1). Conventionally,
OCT has several advantages as a retinal imaging
In “focal” CSME, a focal laser pattern is used to
technique:
treat leaking microaneurysms identified on the
zz It is noninvasive (no injection of dye involved)
FA that contribute to the retinal edema. In ‘diffuse’
and well tolerated.
CSME, intraretinal leakage is noted on the FA from
zz It is highly sensitive and specific for retinal
dilated retinal capillary beds or intraretinal micro-
thickness measurement.
vascular abnormalities (IRMA) without isolated,
zz It clearly reveals the presence and extent of
discrete foci of leakage. Modified ETDRS macular
vitreomacular traction.
grid laser was advocated for managing DDME
zz Other investigations like OCTA are useful for
(see Table 1 for details).
visualizing FAZ, microaneurysms, capillary
density and choroidal changes.
Pharmacotherapy
Ultrawide imaging and VR interface enhance
ment with OCT is under investigation for use in Up till anti-VEGF intravitreal treatment came to
DME. These are currently under investigation but the fore, laser was standard of care for managing
are expected to play important roles in deciding CSME. While this still holds true for non-center
treatment protocols soon. involving DME, current evidence states that
Table 1 Different laser procedures for diabetic macular edema

Parameters Focal laser Classic grid laser Modified grid laser
Wavelength Argon Green, Double Frequency Nd:YAG
Duration 100 ms
Spot size 50 µ 50 µ
Power 80 mw (Titrated 50 mw (Titrated according to requirement)
according to
requirement)
Interburn distance – 2 burns
Intensity of burn Grade 2–3 (dirty white)/ Grade 1–2 (Faint white)
Blanching or darkening
of microaneurysms
Area of laser Based on FA findings Laser burns are given at 500 µ Laser burns are given at
and location of from the center of macula to all 500 µ from the center
microaneurysms. Central microaneurysm within 3000 µ of macula in a C-pattern
ring is spared sparing papillomacular
bundle.
Can be done up to
2 DD from the center of
macula
Follow-up 1-week, 1 month, 1 week, 1 month, 4-month (FFA) 1 week, 1 month,
4-month (FFA) 4-month (FFA)
intravitreal anti-VEGF provides more effective another notable study. A newer analyzed drug
treatment for CSME than monotherapy with laser or is steroid implant-dexamethasone (BEVORDEX
steroid in center involving edema. This conclusion study) and flucinolone (FAME study). Basic
has been drawn from long-term results of a advantage of implants is frequency of injections
study from diabetic retinopathy clinical research with noninferior results. Typically, they are useful
(DRCR) network, which found monthly loading in presence of hard exudates and may be for
therapy with ranibizumab followed by as needed macular ischemia.
(also called pro re nata or PRN schedule) main
tenance to have best results in center involving Combined Therapy
DME vis a vis early laser, only laser and steroid
These therapies may be combined, the most fruit
injection. 5-year data has confirmed these
ful example is combination of ranibizumab with
results. Though repeated intravitreal injections
deferred laser.
have the financial constraints and obviously can
lead to untoward ocular complications, the final
visual acuity and macular anatomy is better Surgical Management
than managing with laser monotherapy. As Pars plana vitrectomy (PPV) for removal of
discussed above for ETDRS, laser chiefly halted VMT may be considered. The posterior hyaloid
moderate visual loss, but did not improve acuity. is removed along with any posterior cortical
Triamcinolone injections have poorer results as vitreous strands to the foveal edge and any visually
well as more complications than anti-VEGF. significant epiretinal membrane. 50% of eyes will
Other notable studies on anti-VEGF are READ, have reduction in central subfield thickness to
RISE, RIDE, BOLT, RESTORE and RESOLVE <250 μm. As per the DRCR net study, only
studies. Further, DRCR has analyzed aflibercept 28%–49% of such eyes will have improvement
for DME and found it to be useful in patients of visual acuity, and between 13% and 31%
with worse initial visual acuity; da Vinci study is may have worsening of visual acuity. The
Retina 313
effectiveness of PPV for DME in the absence of VMT is In patients with DME, simultaneous treatment
unclear. ILM peeling has been evaluated as a with intravitreal injections is indicated for pre
treatment for chronic DME as well as primary vention of exacerbation following cataract surgery.
treatment with differing outcomes.
VIVA QUESTIONS
Treating Refractory DME
Q.1. What is the basic pathogenesis of DME?
Though no fixed definition is quotable, commonly Ans. Breakdown of inner and outer blood
used dictum is failure of response to therapy retinal barrier, anatomical disturbance
with multiple anti-VEGF and, at least, one sitting of the VR interface, blood flow changes
of laser. The available options for management like hyperviscosity and other changes like
include checking diagnoses, checking systemic retinal neuropathy.
control and ruling out anatomical causes. Steroids, Q.2. Differentiate between different anti-
higher frequency or dosing of anti-VEGF, change VEGF agents.
in anti-VEGF drug peripheral laser, surgery and Ans. See table of chapter on proliferative diabetic
newer molecules like tyrosine kinase based retinopathy (PDR).
therapies (TIME study), angiopoietin and AGE
Q.3. What are steroid implants used for DME
inhibitors may be tried. management?
Ans. See Table 2.
Managing Macular Ischemia Q.4. What was conclusion of ETDRS regarding
Outcomes remain bad. Often these patients have DME?
poor systemic control. Lasers and anti-VEGF are Ans. ETDRS had defined CSME. It also defined
relative contraindications in presence of ischemia. moderate visual loss as loss of 15 letters
These patients may benefit from peripheral laser or more, or doubling of visual angle,
and steroids/implants. over 2 visits spread 4 months apart.
ETDRS concluded that lasering CSME
Pseudophakic ME and DME prevented moderate visual loss in 50%
of patients till 2 years of follow up. Visual
Differentiation may be difficult, presence of gain was however seen in roughly around
microaneurysms typically indicate DME while 15% of the patients.
late disc stippling indicates Irvine Gass syndrome.
Table 2 Comparison of dexamethasone implant and fluocinolone acetonide

Parameters Dexamethasone implant (Ozurdex) Fluocinolone acetonide (Iluvien)
Formulation Biodegradable implant Non-biodegradable implant
Dose 0.7 mg 0.19 mg
Duration of action (months) ≤6 24–36
Efficacy (15-ETDRS-letter BCVA 18.4–22.2 (% patients) 28.7–27.8% (% patients)
gain at 3 years)
IOP rise 34–36% 37–45%
Cataract 64–67% 81–88%
Incisional glaucoma surgery 0.3–0.6% 4.8–8.1%
Note: The values are from two studies FAME (Fluocinolone acetonide for macular edema) and MEAD
(dexamethasone intravitreal implant in patients with diabetic macular edema)
Lower complication (in the range given in table) were associated with lower dose [i.e Dexamethasone
intravitreal implant 0.35 mg vs 0.7 mg; fluocinolone acetonide low-dose intravitreal implant (0.2 μg/day vs
0.5 μg/day)]4
REFERENCES 3. Heng LZ, Comyn O, Peto T, Tadros C, Ng E,

Sivaprasad S, Hykin PG. Diabet Med. Diabetic
1. American Academy of Ophthalmology retinopathy: pathogenesis, clinical grading,
Retina/Vitreous Panel. Preferred Practice management and future developments.
Pattern® Guidelines. Diabetic Retinopathy. 2013;30(6):640-50. Review.
San Francisco, CA: American Academy of 4. Dugel PU, Bandello F, Loewenstein A.
Ophthalmology; 2016. Available at: www.aao. Dexamethasone intravitreal implant in the
org/ppp. treatment of diabetic macular edema. Clin
2. Nentwich MM, Ulbig MW. Diabetic retinopathy Ophthalmol. 2015;9:1321-35. doi: 10.2147/
—ocular complications of diabetes mellitus. OPTH.S79948. Review.
World J Diabetes. 2015;6(3):489-99.
EPIRETINAL MEMBRANE
Dhaval Patel, Brijesh Takkar
INTRODUCTION zz Vitreous hemorrhage

zz Diabetic retinopathy.
An epiretinal membrane (ERM) is a sheet-like
fibrocellular structure that develops on or above
the surface of the retina. Epiretinal membrane
(ERM) can be given as a short case or spot case for zz Previous history of intraocular surgery may or
ophthalmoscopy examination. may not be present
zz Photocoagulation or cryotherapy can also be
HISTORY the cause for ERM.
Chief Complaints1
EXAMINATION
zz Loss of vision: Ranges from no symptoms
at all to severe visual dysfunction, usually Ocular Examination2
progressive and painless. zz Uncorrected and best corrected visual acuity
zz Metamorphopsia is common; due to distortion (UCVA and BCVA) should be recorded, Amsler
of the sensory retina. grid or M charts may be used to document
zz Early stages hamper monocular function, metamorphopsia.
while late stages cause binocular disturbance zz Anterior segment examination can often be
also. normal. Signs of previous intraocular surgery
zz Patients with idiopathic ERM may also give or trauma or inflammation must be examined
history suggestive of acute posterior vitreous carefully.
detachment (PVD) in recent past preceding zz Vitreous:
the ERM related symptoms. —— Posterior vitreous detachment (PVD)
is present in the most eyes (75 to 90%)

Past History with idiopathic ERMs, although it is not a
A careful past history can often point towards the prerequisite.
probable underlying cause. Following things must —— Signs of degenerative vitreous may be
be recorded carefully: seen.

zz Trauma zz Fundus:
zz Previous retinal tears —— An irregular or glistening light reflex
zz Retinal detachment from the retinal surface may be the only

zz Retinal vascular occlusive diseases sign in asymptomatic patients, similar to
zz Ocular inflammatory diseases cellophane maculopathy (Fig. 1).
Retina 315
Fig. 1: Fundus photograph of early stage ERM with Fig. 2: Thick mature epiretinal membrane (ERM). Gross
minimal distortion of retinal vessels. Absence of signs distortion of retina and pigmentary changes are visible.
of chronicity and retinal distortion indicate good Such membranes ensue poor prognosis
prognoses
—— In more advanced cases, in symptomatic DIFFERENTIAL DIAGNOSIS

patients with actual contraction or zz Vitreomacular traction (VMT): OCT and
shrinkage of the membrane, distinct
careful 90D examination can rule out VMT,
retinal findings can be appreciated, such
though both may be present simultaneously.
as retinal striae radiating from the center zz Cystoid macular edema (CME): Presence of
of the ERM, retinal vessels straightened
cystic spaces and absence of characteristic dis-
toward the membrane center, or tortuosity
tortion of blood vessels helps in differentiating
of retinal vessels and dilated retinal veins
this entity.
(Fig. 2). Wrinkling of the retina may be zz Macular hole or pseudohole: Watzke-Allen test
noted.
and red beam test and in doubtful cases OCT
—— Pigmentary changes indicate chronicity
can differentiate this entity.
of the disease with poorer prognoses
—— A pseudohole can also be there.
INVESTIGATION
—— Peripheral examination should always be
done for retinal breaks. Following investigative modalities are useful in
—— Gass Grading System is useful in ERM.
describing the clinical findings
Grade 0: Cellophane maculopathy— Ocular Coherence Tomography
an irregular translucent sheen is zz By far, the modality of choice.
present in early ERM, best detected zz Ocular coherence tomography (OCT) provides
using green (red-free) light. good visualization of the vitreoretinal juncture
Grade 1: Crinkled cellophane and shows the membrane on the retinal
maculopathy—membrane thickens surface as a hyper-reflective structure (Fig. 3).
and contracts, becomes more obvious zz Ocular coherence tomography (OCT) also may
and causes mild distortion of blood play a role in preoperative and postoperative
vessels evaluation of surgery for ERMs by indicating
Grade 2: Macular pucker—distortion prognostic markers.
of blood vessels, marked retinal zz It also helps in differentiating detaching
wrinkling and striae and obscuration vitreous cortex from ERM, differentiating
of underlying structures. pseudoholes and true lamellar holes.
Fig. 3: Ocular coherence tomography (OCT) picture of vitreomacular traction (VMT) with
fine epiretinal membranes (ERM). An impending full thickness macular hole is visible
zz Disruption of the outer most hyper-reflective zz Secondary associations:

bands, previously termed IS-OS junction, may —— Vascular disease (diabetic retinopathy,
be associated with a worse visual outcome CRVO)

following surgery. However, recent focus for —— Inflammatory disease (posterior uveitis)
prognostication is now shifting towards the —— Trauma
inner retinal layers. —— Retinal surgery (RD surgery, laser
zz Enhancement of the VR interface by vitreous photocoagulation, cryotherapy)

windowing helps in studying it on detail using zz Iatrogenic
OCT. —— Postoperative: Cataract/Retinal detach-
ment/Silicone oil/Retinopexy/Laser or
Fluorescein Angiography cryotherapy.
zz Not required routinely.
Gass clinical grading system: It has been described
zz Anatomic features of retinal distortion may be
in the examination section.
better seen on FA, such as straightened retinal
vessels, retinal vascular tortuosity, foveal
ectopia, and macular dragging. Foos Classification
zz Fluorescein leakage and macular edema zz Simple ERM: Incidental without contraction
secondary to ERM traction-induced vascular features or associated ocular disease.
leakage also may be assessed. zz Intermediate ERM: Thicker than simple ERMs
zz One of the important role of FA is to detect the and contain contraction features and pigment.
presence of other retinal pathology, such as a zz Complex ERM: Present after retinal detach
choroidal neovascular membrane (CNVM).
ment surgery or after trauma and may be
secondary to other ocular conditions. Traction
CLASSIFICATION
retinal detachments may develop as a result of
The different classification systems used for ERM contraction of such membranes.
are as follows. International vitreomacular traction study
(IVTS) Group optical coherence tomography
Standard Classification (OCT)-based anatomic classification system for
zz Idiopathic: diseases of the vitreomacular interface (VMI)
—— Age-related/PVD related has been described in Table 2 of chapter on
—— Up to 20% are bilateral macular hole.
Retina 317
Other classification systems based on OCT VIVA QUESTIONS

and confocal scanning loser ophthalmoscopy
(CSLO) are also notable, but not in common Q.1. How do epiretinal membranes (ERM)
clinical use. develop?
Ans. A dehiscence of the internal limiting
MANAGEMENT membrane allows retinal glial cells
[predominant cellular constituent, probably
The management options for ERM has been derived from the indigenous posterior
described below: hyaloid membrane (PHM) cell population
Observation: It is always advisable to have a short (laminocytes), myofibroblasts and fibro
period of observation before embarking upon blasts] to proliferate along the retinal
surface. Contraction of these membranes
surgery. In early stages with good visual acuity (e.g.
causes cellophane maculopathy or macular
better than 6/12), absence of visually disturbing
pucker. Detachment of the posterior
symptoms and if membrane is nonprogressive
vitreous is present in almost all eyes. PVD
observation is the rule. Spontaneous resolution
may be responsible by inducing micro-
of symptoms can occurs, due to separation of the
retinal breaks.
ERM from the retina as a previously incomplete
PVD completes and also some times in secondary Q.2. What is the chance of recurrence after
cases. surgery?
Ans. Recurrence of the membrane after PPV is
Medical management with enzymatic vitreolysis:
uncommon. Reported recurrence rates are
Use of ocriplasmin (microplasmin) has been
low, with visually significant recurrences
described by MIVI-TRUST trials. In this trial
up to 5%.
27% vitreomacular adhesion (VMA) resolved
as compared to 10% in placebo. But given the Q.3. What is VMA?
expenses and nonavailability is currently not Ans. As per definition, it refers to largely attached
preferred. cortical vitreous with some areas of
detachment not causing retinal structural
Surgical intervention with pars plana vitrectomy changes. In contrast, VMT causes tractional
and epiretinal membrane peeling: Indications for disturbance in the retinal architecture and
surgery:3 in presence of symptoms it termed VMT
zz High visual requirements (occupation, young syndrome. VMA is more likely to have a
age) smooth contour rather than ERM. The
zz VA < 20/60 terminology and classification system for
zz Associated CME or tractional detachment. different diseases of the vitreomacular
Whether internal limiting membrane (ILM) interface (VMI) has been described in
peeling should be combined with ERM removal Table 2 of chapter on macular hole.
or not is controversial, one of school of thought
indicates lesser recurrences while the other is
REFERENCES
based on neuronal damage caused by the ILM peel
itself. Intraoperative OCT is now commercially 1. Brad Bowling. Kanski’s Clinical Ophthalmology:
available and may be seen to have a greater role in A systematic approach, 8th edn. Edinburgh:
Elsevier. 2015.
future (PIONEER study). Long-term results, up to
2. Albert DM, Miller JW, Azar DT. Albert
5 years, have revealed low recurrence rates with
& Jakobiec’s Principles and Practice of
ILM peeling but with associated progressive Ophthalmology; 2008.
thinning of regional macular thickness. See the 3. Ryan SJ, Schachat AP, Wilkinson CP, Hinton
chapter on macular hole for discussion on surgical DR, Sadda SR, Wiedemann P. Retina. 5th edn.
technique and vital dyes. Elsevier Health Sciences, 2012.
FUNDAL COLOBOMA
D Satyasudha, Ruchir Tewari, Atul Kumar
INTRODUCTION Presenting Complaints

Coloboma (plural-colobomata) is derived from The visual loss is typically painless detected on
Greek word “koloboma” meaning mutilated or routine examination in a child or suddenly due to
curtailed. Walther introduced term “coloboma”. its complications. This would vary from cases to
Coloboma of the fundus is a congenital ocular case. In severe cases, the child may present with
malformation, which generally results from failure nystagmus, microphthalmos or even inability
of the fetal or choroidal fissure to close during 5th to follow objects. With concomitant iris defects,
to 7th week of fetal life, at 7–14 mm stage. This is the presentation may be for cosmetic reasons. In
the period between the invagination of the optic peripheral colobomas, the visual acuity is spared
vesicle and the closure of fetal fissure. Closure but other complications may be the cause of
starts at the equator of eye/ciliary body region and presentation.
continues anteriorly and posteriorly. A coloboma
may extend from the iris margin to the optic Past History
disc and involve one or more defects along line This is rather necessary as syndromic association
of fusion. Any ocular structure can be involved is well-defined and actually may even be the
including cornea, iris, ciliary body, zonules, reason for presentation. Typical syndromes have
choroid, retina and optic disc or optic nerve.1-3 been discussed later.
Coloboma can be unilateral or bilateral, the
latter being seen in 60–70% of cases. Bilateral
Family History
colobomas are usually inherited in an autosomal
dominant fashion with variable penetrance. Analysis of pedigree charts is mandatory to identify
Recessive inheritance has also been reported. inheritance patterns. Genetics has been discussed
If fetal fissure fails to close posteriorly, then a later in the chapter.
coloboma affecting the retinal pigment epithe
lium, neurosensory retina or choroid may occur. EXAMINATION
Typical coloboma occurs in the inferonasal
Visual Acuity
quadrant. There can be associated with apparent
lens coloboma due to persistence of mesodermal Varies depending on the type and laterality of
vascular remnants that prevent development of coloboma. Peripheral colobomas sparing macula
zonules in that area leading to flattening of the usually have good visual acuity than those
lens edge. Mutation in PAX6 gene has been involving macula. Similarly, unilateral colobomas
reported in association with syndromic forms of may have accompanying refractive error that may
colobomata. lead to anisometropic amblyopia with consequent
poor visual acuity, even when macula is spared.
HISTORY
Motility and Eyeball
Chief Complaints Usually, not associated with ocular motility defects.
Usual history is of a child or young adult Unilateral cases may have history of long standing
presenting with diminution of vision for distance. squint. Nystagmus/nystagmoid movements may
In some cases, parents may present complaining be noted. Microphthalmos may also be seen.
about small size or different shape of eye.
Sometimes presence may be noted only at time of Lids and Conjunctiva
complications like cataract or retinal detachment Usually, unaffected unless in syndromic associa-
(RD). tion.
Retina 319
Cornea Intraocular Pressures

Usually pear shaped in cases with iris involvement. Usually normal, can be low in cases of RD or thigh
in those associated with glaucoma.
Iris and Pupils
Fundus Examination4-6
“Key hole” pupil due to associated Iris coloboma
(Fig. 1). Iris coloboma may be typical/atypical, Following findings must be noted:
complete/incomplete/partial/total. zz On ophthalmoscopic examination, the white
background of the sclera usually showing a
Lens glistening white sheen replaces normal color
of the fundus (Fig. 3).
There can be nuclear sclerosis of varying degree zz Typically, coloboma is oval.
with associated cortical component, with associ zz Usual location is downwards and inwards.
ated zonular coloboma inferiorly (Fig. 2). True zz Posterior end frequently stops short of the disc
lens colobomas are rare, albeit to surface ecto
(see classification later).
derm origin of the lens vis-a-vis the coloboma. zz Anterior end sometimes reaches forwards
beyond the limits of ophthalmic examination,
due to involvement of the ciliary body region
as well.
zz Sometimes the defect can be relatively small,
round, or transversely oval, or several isolated
defects can be scattered along the line of
fissure.
zz Edges are usually cleanly cut and frequently
pigmented.
zz Floor of the coloboma is usually depressed
below the level of the rest of the fundus.
zz Two types of vessels can be seen, retinal vessels
that dip down into the coloboma as they pass
from the normal fundus and choroidal vessels,
more tortuous and broader lying at a deeper
Fig. 1: “Key hole” pupil due to associated Iris coloboma level (Fig. 4).
Fig. 2: Nuclear sclerosis with associated zonular Fig. 3: Fundal coloboma with sparing of disc and
coloboma inferiorly macula (Type 3 coloboma)
coloboma, though still not advocated for routine

clinical use. VER may have a prognostic value
and be useful in surgical decision making in long
standing cases of RD.
MANAGEMENT
Amblyopia
Uniocular coloboma not involving the macula
can be associated with refractive errors that
need prompt correction to avoid development
of amblyopia. In cases with bilateral coloboma,
severe refractive errors may lead to ametropic
Fig. 4: Choroidal excavation in the area of the amblyopia. Such cases, again, are good candidates
coloboma with overlying retinal vessels for correction of refractive errors.
Family and Genetic Screening

Macular colobomas are atypical coloboma; with
causation different form that of the choroidal Some cases of coloboma may be associated with
coloboma. Peripheral examination must be genetic variations. Family history, including close
conducted for presence of peripheral retinal breaks relatives may be helpful in identifying such cases.
and retinal degenerations. Scleral depression may Genetic screening and genetic counseling in
be necessary for areas not visible to the eye as such cases may help. Other associated anomalies
such. One must look for retinal breaks at the edge arising due to the defective genes may be picked
of the coloboma also. While total RD may be seen, up in unsuspecting family members that may be
sometimes it is present only in the colobomatous amenable to treatment.
area.
Retinal Detachment
DIFFERENTIAL DIAGNOSIS Retinal detachment is a known and frequent
complication of choroidal colobomas. Colobomas
Choroidal colobomas may be confused with may present with different forms of retinal
infective conditions like toxoplasmosis in macular detachments (see Viva questions). Prophylactic
cases and Zika virus infections in predisposed role of laser photocoagulation to coloboma edges
patients. Pathological myopia may also have is proven effective in decreasing chances of retinal
staphylomas with scleral ectasia giving false detachment. Laser photocoagulation may be
appearance of a coloboma. Causes of large chorio- performed at the earliest possible time. Surgery
retinal atrophy areas should also be kept in mind for retinal detachment usually involves pars
(also see viva questions).
plana vitrectomy, endolaser photocoagulation
and silicone oil tamponade. Most of the cases
INVESTIGATIONS have a retinal break in and around the edge of
Systemic investigations should be done for the coloboma. Use of an encircling element is
syndromes as applicable. Genetic tests may controversial, though, may be advocated in cases
also be needed. In hazy media, ultrasonography with significant proliferative vitreoretinopathy.
(USG) is necessary for charting the coloboma These cases usually have poor surgical outcomes
and identifying RD. Although not compulsory, after silicone oil removal with high re-detachment
OCT, with its enhanced penetration, is a handy rates that may necessitate long-term oil tamponade
adjunctive investigation, which is currently and oil exchanges instead of removal to maintain
more of a research tool. It helps in identifying retinal attachment. Buckling surgery has poor
retinal breaks, examining changes at the edge of outcome in cases where the primary break is in
Retina 321
the colobomatous region. It may be used in cases • M argins of the optic cup do not grow
where a peripheral primary break is causative. over inferior part of the lens, showing
a deficiency in this part known as
Cataract choroidal/fetal fissure (usually closes by
6th week, failure to close by 6th to 7th
Earlier onset of cataract is a known finding in
week results in typical coloboma).
colobomas. Most common type of cataract
• Outer layer of the optic cup forms retinal
detected is nuclear sclerosis, being seen in almost pigment epithelium, inner layer forms
half of cases. A distinct type of linear cataract may neurosensory retina.
also be seen in the area of coloboma. As these
eyes may be associated with microphthalmos and Q.2. What are the various types of coloboma?
microcornea, different surgical techniques such Ans. The different types are as follows
Iris coloboma
as scleral tunnel phacoemulsification have been
• Typical/atypical iris coloboma
advocated in such cases. Use of capsular tension
– Typical coloboma is in the inferonasal
rings to stabilize the area of coloboma has also
quadrant as this is the site of closure
been described. Implantation of intraocular lens in
of embryonic fissure
cases with apparent small eye is aided with ultra
– Atypical coloboma: Located anywhere
sound biomicroscopy to measure the sulcus size. other than inferonasal quadrant
• Complete/incomplete iris coloboma
Choriodal Neovascularization – Complete coloboma: Full thickness
Though rare, development of choroidal neovas defect involving both pigment
cularization developing at the edge of the epithelium and iris stroma.
coloboma has been described in literature. Use of – Total: Extending to iris root (keyhole
both photodynamic therapy and anti-VEGF agents pupil)
have been described in such cases. – Partial: Involving pupillary margin
(oval pupil)
• Incomplete coloboma: Partial thickness
VIVA QUESTIONS defect involving either pigment epithe
lium or iris stroma.
Q.1. What is the embryological defect in a case – Wedge shaped
of coloboma? – Demonstrated by transillumination
Ans. Following points must be remembered: • L ens coloboma—misnomer as it is
• Cranial end of differentiating CNS forms actually zonular coloboma that manifests
neural folds as flat lens surface visible through
• Optic pits appear at 21 days of life, on pupillary defect.
each side of neural grove • Posterior segment coloboma—retino-
• Optic grooves (recognizable by 4 weeks) choroidal coloboma
in neural folds • Optic nerve coloboma
• Neural ectoderm evaginates from each Lid colobomas have been discussed
groove towards surface (recognized by in relevant chapter. They should not
25th day) as optic vesicles. be confused with ocular coloboma as
• Optic vesicle is connected to forebrain by involved embryonic layers are different.
optic stalk.
• Surface ectoderm overlying optic vesicle Q.3. What is the pathophysiology of choroidal
thickens forming lens placode – lens pit – coloboma?
lens vesicle. Ans. • In eyes with defective closure of fetal
• Optic vesicle invaginates forming double fissure, inner layer destined to form
layered optic cup; lens vesicle gets neurosensory retina grows faster than
pinched off by 4th week, lies in the optic outer layer destined to form retinal
cup. pigment epithelium, leads to eversion.
• G radual displacement of retinal pigment • asal cell nevus syndrome

B
epithelium, leading to development of • Meckel-Gruber syndrome
double layer of photoreceptors facing • Trisomy 13 (patau), 18 (Edward)
each other. • 13 q deletion syndrome
• Absence of retinal pigment epithelium. • Cat eye syndrome, Rubinstein-Taybi
• Since choroid development is influenced syndrome.
by retinal pigment epithelium, choroid is • Posterior fossa malformations hem
absent in areas of coloboma. angiomas-arterial anomalies-cardiac
defects-eye (PHACE) syndrome.
Q.4. What is the histopathology of coloboma
Q.6. Differential diagnosis of various colobo-
and colobomatous border?
mata.
Ans. Histopathology of coloboma:
Ans. Iris coloboma
• S clera is thinned by loss of its inner
• Iatrogenic—postsurgery
layers, ability for ectasia increases.
• Traumatic—open globe injury
• Absence of choroid and choriocapillaries
• Congenital—sporadic, familial, syndro-
Hermann Schubert, described the histo
mic.
pathology of the colobomatous border:
Choroidal coloboma
• Retina splits into two layers near the
• R etinal scars: Toxoplasma, toxocara
margin of the coloboma. The split in the
• Congenital anomalies: Torpedo maculo
layers of the retina has been identified
pathy, staphyloma
at the level of inner nuclear or outer
plexiform layer or both. Disc coloboma
• The inner layer continues as the inter • E xcavated disc anomalies: Coloboma,
morning glory syndrome, peripapillary
calary membrane onto the coloboma,
staphyloma, optic disc pit.
while the outer layers turn back, become
disorganized, and fuse with the retinal Q.7. What are the various classifications of
pigment epithelium. fundal coloboma?
• The choroid is terminated as a distinct Ans. See Tables 1 and 2.
pigmented layer peripheral to the point Q.8. What is incidence of retinal detachment
of reversal. The junction where this in patients with coloboma?
reversal occurs has been termed “locus Ans. Incidence of rhegmatogenous retinal
minoris resistentiae”. detachment in patients with retinochoroi
• The intercalary membrane progressively dal coloboma is 23–40%.
becomes thinner as it is traced centrally. Q.9. What are the various locations of retinal
Q.5. What are the various systemic associa breaks in coloboma?
tions with coloboma? Ans. Breaks can occur at two locations:7
Ans. There are many syndromes, decreasing the • At the locus minoris resistentiae
specificity of coloboma as an association. • In the intercalary membrane:
For example – 63.8% of breaks are located within
• C HARGE syndrome: Colobomatous 2 disc diameter (DD) of the margin
microphthalmos, heart defects, atresia of coloboma and rest in the central
(choanal), retarded growth, genital portion of the coloboma.
anomalies, and ear anomalies. – 54.1%—breaks at margin
• A ICARDI syndrome: Retinal cystic – 24.7%—within coloboma
dysplasia, occipital encephalocele, – 8.2%—macula
polydactyly, pulmonary hypoplasia. Various clinical situations depending on the
• WARBURG syndrome: Hydrocephalus, combination of the breaks:
agyria, retinal dysplasia. • Break at the locus minoris resistentiae
• Goltz syndrome only—cannot lead to retinal detachment.
Retina 323
Table 1 Ida Mann classification detachment will be seen to extend into

the colobomatous area.
I Above the disc • Breaks in peripheral retina, locus minoris
II Superior border of the disc resistentiae, intercalary membrane–total
III Below the disc (separated from the optic disc retinal detachments.
by normal narrow area of retina) Q.10. Why is it difficult to localize breaks in
IV Isolated disc coloboma (inferior crescent colobomatous area?
below the disc) Ans. It is difficult to localize breaks in coloboma
tous area because of:
V Peripheral with normal retina above and
• Little contrast in colobomatous area due
below (isolated gap in the line of fissure)
to absence of choroid and RPE
VI Pigmentary disturbance • Thinned out retina
VII Extreme peripheral coloboma • Nystagmus
• Ectatic sclera.
Q.11. What is the pattern of retinal breaks and
Table 2 Lingam Gopal classification (1996); detachments in coloboma?
Types of optic disc involvement in Ans. • Type I: Retinal detachment does not
fundus coloboma extend into coloboma.
I Disc outside the fundus coloboma and totally • Type II: Retinal detachment extends
normal (27.8%) into coloboma to a variable extent with
II Disc outside the fundus coloboma and or without retinal detachment outside
abnormal (10.4%) coloboma.
– I I A: Subclinical—Restricted to
III Disc outside the fundus coloboma and
coloboma.
independently colobomatous (8.9%)
– II B: Visible break within coloboma.
IV Disc within the fundus coloboma and normal – II C: Visible break both within and
(5%) outside coloboma.
V Disc within the fundus coloboma and – II D: Break only in peripheral retina.
colobomatous (44.3%) – II E: Break not visible.
VI Disc shape not identified; blood vessels Q.12. What are the causes of low vision in
emanating from superior aspect of the large coloboma?
fundus coloboma Ans. Following can be the causes:
Note: High myopia is more common in types I-III • Refractive error
with better visual acuity. Microphthalmos is more • Subluxated lens
common in types IV–VI. • Optic disc anomalies
• Macular involvement
• B reak in the intercalary membrane only- • Retinal detachment
detachment of intercalary membrane • Cataract
only. • Choroidal neovascular membrane.
• Break in both intercalary membrane and Q.13. What are the various complications asso
the locus minoris resistentiae—leads to ciated with retinochoroidal coloboma?
clinical retinal detachments. Ans. Complications:8
• Breaks in the normal peripheral retina • Retinal detachment
alone—the retinal detachment will be • Cataract/lens subluxation
seen to stop short of the colobomatous • Amblyopia
border. • Anisometropia
• Breaks in the peripheral retina and the • Sensory strabismus
locus minoris resistentiae but no break • Subretinal neovascularization
in the intercalary membrane—the retinal • Secondary glaucoma.
Q.14. What is the management of retinal deta • T

ype 3 (Ida Mann): Margins of the
chments in retinochoroidal coloboma? coloboma is lasered sparing the area
Ans. It includes following: within the temporal vascular arcade and
• P rophylactic laser photocoagulation nasally up to 0.5 mm from the disc.
posteriorly along the edge of the • T ype 5 (Ida Mann): Coloboma is
coloboma for prevention of detachment. surrounded by three rows of laser.
• Proper localization of breaks—if breaks Q.18. Why is buckling difficult in retinocho
are outside the coloboma with an RD not roidal coloboma?
extending into coloboma than buckling Ans. External buckling is difficult in retino
surgery may be indicated. choroidal coloboma because of:
• Vitrectomy—for all retinal detachments • Difficulty in identifying breaks in inter
where breaks are at the margin or inside calary membrane.
colobomatous region. • I mpossibility of creating adhesion
around breaks due to absence of choroid
Q.15. Role of prophylactic laser photocoagula
and retinal pigment epithelium.
tion in fundal coloboma.
• Posterior location of breaks.
Ans. Reduces the incidence of rhegmatogenous
retinal detachment. A recent study 8 Q.19. Why is PPV preferred?
highlighted the importance of prophylactic Ans. Pars plana vitrectomy is preferred because
laser in coloboma for prevention of retinal of:
detachment. They reported prevalence • Ease of identifying breaks in the inter
of retinal detachment to be 2.9% in laser calary membrane with more certainty.
treated eyes compared with 24.1% in • Removal of traction
untreated eyes with a total prevalence of • Closure of breaks or sealing off colobo-
matous regions with laser retinopexy.
17.9%.
Q.16. Which laser is preferred? REFERENCES

Ans. Diode laser is preferred over argon because
1. Duke elder S (Ed). System of ophthalmology, St.
of less damage to retinal nerve fiber layer
Louis: CV Mosby Co, 1963;3:465-9.
owing to deeper penetration. Currently 2. Duke elder S (Ed). System of ophthalmology, St.
frequency doubled Nd:YAG (532 nm) Louis: CV Mosby Co, 1963;3:472-81.
green laser is laser of choice for all retinal 3. Onwochei BC, Simon JW, Bateman JB, et al.
photocoagulation. Ocular colobomata. Surv Ophthalmol.
2000;45(3):175-94. Review.
Q.17. How are various types of coloboma 4. Gopal L, Badrinath SS, Kumar KS, et al. Optic
lasered? disc in fundus colobama. Ophthalmology.
Ans. • Type 1 (Ida Mann): Laser spots are applied 1996;103(12):2120-6; discussion 2126-7.
initially along the superior margin of the 5. Gopal L, Badrinath SS, Sharma T, et al. Surgical
coloboma and then continued along the management of retinal detachments related
whole nasal margin. Temporal margin to coloboma of the choroid. Ophthalmology.
is lasered inferior to the presumed 1998;105(5):804-9.
inferotemporal vascular arcade and 6. Lingam G. Pattern of blood vessels in eyes
with coloboma. Indian J Ophthalmol.
superiorly up to the superotemporal
2013;61(12):743-8.
arcade sparing the macula.
7. Gopal L, Badrinath SS, Sharma T, et al. Pattern
• Type 2 (Ida Mann): Laser spots are of retinal breaks and retinal detachments in
applied starting from nasal to the eyes with choroidal coloboma. Ophthalmology.
optic disc. The nasal margin is lasered. 1995;102(8):1212-7.
Temporally, laser is performed inferior 8. Uhumwangho OM, Jalali S. Chorioretinal
to the presumed inferotemporal vascular coloboma in a paediatric population. Eye.
arcade. 2014;28:728-33.
Retina 325
GIANT RETINAL TEAR

INTRODUCTION intraocular lens (IOL) in an already predisposed

patient.
A giant retinal tear (GRT) is a full-thickness
neurosensory retinal break that extends
circumferentially around the retina for three or
Family History
more clock hours. It accounts for around 1.5% of Family history may be there (Marfans, Stickler,
rhegmatogenous retinal detachments (RRD). Most Ehlers Danlos syndrome).
GRTs are idiopathic (55–66%). The most common
predisposing factors for the development of Past Surgical History
a GRT are trauma (4–31%), hereditary vitreo-
Past history of intraocular surgery may be there.
retinopathies (14.5%), and high myopia (9.7%).1 It
Conventionally, GRTs would be found at the edge
can be given as a short case in postgraduate exams.
of heavy cryo burns.
HISTORY
EXAMINATION
Epidemiology/Demography
Giant retinal tear (GRTs) have a significant male
Giant retinal tear (GRT) may be associated with
preponderance, between 65% and 91%. The mean
Wagner, Stickler, and Marfan syndrome.
age of patients ranges from 30 to 53 years of age.
Right eyes appeared to be more frequently affected,
with most studies reporting a right eye incidence Ocular Examination
of 48% to 67%. GRTs have been estimated to be Visual acuity: Visually, acuity is commonly
the cause of the RD in 0.5–8.3% of cases in adults. very low with inaccurate PR in large GRTs due
In contrast, in the pediatric population (16 years to massive receptor dysfunction, especially in
or younger), the prevalence is higher and is displaced flaps.
between 18% and 31.7%.1 Eyeball: Eyeball may appear large in high myopic
patient.
Chief Complaint
Lid: Lids are generally normal except signs of
The patient usually presents with sudden loss previous trauma such as scar.
of vision in eye, flashes, floaters, and photopsia.
It may be associated with dull ocular pain due to Conjunctiva: Conjunctiva is generally normal.
inflammation. Cornea: It may be large and corneal thinning may
be there (Myopia).
Sclera: Scleral thinning may be there (blue sclera
The sudden loss of vision in eye is commonly in collagen vascular disease). Globe tenderness
associated with floaters; presentation can be can be elicited in presence of inflammation.
unilateral or bilateral. It may be rapidly progressive.
Anterior chamber (AC): AC may be deep in high
History of associated trauma or use of high power
myopic eyes. AC inflammation is frequent in cases
glasses must be recorded in such cases.
of GRT.
History of Past Illness Iris: Iridodialysis or atrophy may be there in cases
of previous trauma.
Past history of trauma, cryo may be there. There
may be history of recent ocular surgery like Pupil: An eccentric pupil may be there in Marfan’s
scleral fixated intraocular lens (SFIOL) or phakic syndrome.
Intraocular pressure (IOP): There is usually zz Extent of tear (in degrees or clock hours),
hypotony in cases of GRT. This is a characteristic location of tear (postoral, equatorial or
feature of GRTs and is observed due to rapid egress posterior)
of fluid via the exposed choroidal circulation. zz Lattice or other degenerations
Gonioscopy: Angle recession or cyclodialysis may
zz Proliferative vitreoretinopathy (PVR)/macular
be there in case of trauma. pucker (Note: PVR is a well-recognized feature
of GRTs. The large surface area of exposed RPE
Lens: Lens may be subluxated or dislocated. increases the propensity for the liberation of
Zonular dialysis may be there. Phacodonesis may RPE cells and subsequent PVR)
be present there in cases of Marfan’s syndrome or zz Retinal detachment; extent of retinal detach
Ehlers-Danlos syndrome or trauma. When the tear ment (total or subtotal), retinal tear flap if
involves retina with patent vasculature, vitreous displaced or mobile.
hemorrhage may develop. Fellow eye must be examined carefully to look
Anterior vitreous: There may be presence of for myopic changes, lattice, vitreous condensation,
vitreous pigments (Shaffer’s sign) or hemorrhage. white without pressure (WWOP) changes, retinal
Shaffer’s sign/tobacco dusting is seen in virtually breaks, giant retinal tear and retinal detachment.
all patients with GRT. Up to 50% of fellow eyes may be predisposed
to RD.
Fundus: To examine retina in cases of displaced
flaps (Fig. 1), patient positioning may be adjusted
accordingly (Note: In virtually all patients with
a GRT, the tear is either partially or completely Giant retinal dialysis: A retinal dialysis is a
inverted. The posterior flap of the tear may invert circumferential retinal disinsertion at the ora
over the optic disc or even the whole macula, serrata, frequently secondary to blunt trauma. The
making it difficult to assess the full extent of the differentiating points are discussed in Table 1.
associated RD. For inverted and mobile GRT,
positioning of the patient appropriately may unfold
Table 1 Difference between GRT and GRD
the tear, facilitating a more accurate examination).
Choroidal detachment and multiple tears are very Giant retinal tear Giant retinal dialysis
common. Following points must be noted: Break may extend The break is located
zz Vitreous syneresis/liquefaction, posterior beyond the posterior anterior to the posterior
vitreous detachment (PVD) limit of the vitreous base limit of the vitreous base
insertion insertion
Vitreous attached to Vitreous attached to
anterior flap posterior flap hence the
tear is prevented from
rolling over or inverting
PVD is usually present PVD is usually absent
Massive preretinal PVR not massive,
vitroproliferation and macular pucker is rare
macular pucker is
present
Radial posterior tear Absence of the radial
extensions can be there posterior tear extensions
Vitreoretinal surgery is Rarely needed; RD
needed surgery is generally
Fig. 1: Intraoperative photograph of a GRT stabilized successful
partially with PFCL. Note the large flap of the retinal
tear that has fallen over the retina. Underlying bare Abbreviations: PVR, proliferative vitreoretinopathy;
PVD, posterior vitreous detachment
choroid is also visible
Retina 327
INVESTIGATIONS complicated cases, surgeons have left PFCL

in situ to attain attachment, and performed
Ultrasonography (USG) is useful in presence silicone oil exchange as a secondary procedure.
of media haze precluding fundus evaluation. There after 3–5 rows of endolaser are placed to
Ultrawide image documentation may be con hold the flap in position. For SRF drainage, PFCL
sidered. Systemic diseases should be screened in silicone oil exchange, or PFCL-air, followed by Air-
cases of GRT. PFCL exchange is done. Particularly, the edge of
the GRT has to be kept dried during this maneuver.
MANAGEMENT
Role of lensectomy: Lensectomy has to be done in
It should be aggressive. One may consider pre- following cases:
operative local steroids if delay is expected. zz Increased risk of development of cataract
Traditionally, primary scleral buckling and buckle within 2 years
pneumatics have been considered for early GRT. zz Subluxation of lens
Vitrectomy is now generally preferred. In the zz Presence of PVR especially in anterior PVR to
pre perfluorocarbon liquids (PFCL) era, prone get access to vitreous base region.
position fluid-air exchange and retinal tacks have
been used. PFCLs have over all revolutionized Surgical Objectives
typically a GRT-RD surgery by acting as the “Third zz Vitrectomy, PVD and restoration of folded flap
hand”. Management of GRT depends on the extent
to original position
and associated PVR changes.2 zz Remove PVR, may remove the anterior retinal
flap and manage edge of GRT
Conventional Protocol zz Retinopexy under PFCL
zz GRT without displacement: Treatment is by zz To drain all SRF
either cryopexy or laser (contiguous and at zz To hold flap in position until retinopexy
edge of tear reaching up to or at edge adhesion occur.
zz GRT with displacement, mobile post flap:
—— Extent <180°—Scleral buckling (DACE) Management of Fellow Eyes
—— Extent ≥ 180°—Vitreoretinal (VR) surgery
Cryotherapy: It is indicated in all retinal holes,
zz GRT with everted/rolled up posterior retinal small dialysis and small retinal tears located
flap with or without star folds/macular pucker: anterior to equator.
—— Treatment—vitreoretinal surgery.
Photocoagulation: All retinal holes, and small

Other Indication of Vitreoretinal Surgery retinal tears located posterior to equator should be
photocoagulated.
zz Opaque media (lenticular opacity, vitreous
hemorrhage) Scleral buckling: Large retinal tears, multiple
zz Retained intraocular foreign body (RIOFB) retinal tears, and tears associated with vitreous
zz Retinal and vitreous incarceration (trauma). hemorrhage.
It should however be remembered that in
today’s era, vitrectomy is generally preferred for Prophylactic Scleral Buckle in High-risk Eye
GRT related RD. The following characteristic puts the patient at
Role of silicone oil: Generally, preferred except in high-risk of having GRT:
early stages. Silicon oil restores and holds flap in zz High myopia >10 diopters.
position until the time retinopexy occurs. It also zz Increasing white without pressure (WWOP).
counters the occurrence of postoperative PVR. zz Increasing vitreous base condensation.
Role of perfluorocarbon liquids (PFCL): PFCL is
a great help by helping in un-displacing the retinal Role of Pneumatic Retinopexy
flap and keeping it in position until retinopexy is Expansible gases with increased longevity are
done. It acts as the third surgical hand. In highly indicated in cases of small GRT and Co-operative
patient with good compliance. Its advantages Q.3. What is risk of developing RD in fellow
are it avoids complication of VR surgery and the eye?
procedure is relatively simple and easy. However, Ans. The incidence of bilateral non-traumatic
the chances of failure are high and chances of GRT at presentation ranges between 0%
slippage of flap are higher than silicon oil. and 21%. The fellow eye is also at risk of
developing a RD unrelated to a GRT. The
Role of Vitrectomy with Scleral Buckle rate of fellow eye RD ranges from 6% to
42%.1
This is a highly debated topic. Scleral buckle would
help by supporting vitreous base and decreasing Q.4. Classification of GRT.
tractional forces. On the other hand, it may result Ans. See Tables 3 and 4.
in flap slipping and fish mouthing. Q.5. Syndromes associated with GRT.
Complications of surgery: Complications of GRT Ans. See Table 5.
surgery are summarized in Table 2. Q.6. Most common complications.
Prognosis: Primary and final anatomical success Ans. See Table 2.
is from 70% to over 90%, but less than 50% of Q.7. Role of PFCL.
patients will achieve 20/40 or better acuity. Poor Ans. See text.
visual prognosis factors3 are macular detachment,
Q.8. Indication of lensectomy in GRT.
hypotony, pseudophakia/aphakia, high grade
Ans. See text.
PVR, GRT>180 degree, poor visual acuity at pre
sentation, and persistent retinal detachment.1 Q.9. Pathogenesis of idiopathic GRT.
Ans. Central vitreous liquefaction followed by
vitreous condensation and formation of
VIVA QUESTIONS
equatorial membranes, transequatorial
Q.1. GRT definition. forces lead to formation of large break
Ans. A giant retinal tear (GRT) is a full-thickness
neurosensory retinal break that extends Table 3 GRT classification (based on extent)
circumferentially around the retina for
three or more clock hours in the presence GRT I <180°
of a posteriorly detached vitreous. GRT II 180–270°
Q.2. Difference between GRT and GRD. GRT III >270–<360°
Ans. See Table 1. GRT IV 360°
Abbreviation: GRT, giant retinal tear
Table 2 Complications of GRT surgery
Intraoperative Slippage (around 3–15%)
Vitreous hemorrhage
Retinal folds Table 4 GRT classification (based on PVR
Rolled edge severity)
Postoperative Recurrent retinal detachment GRT I Without displacement
(40–50%) GRT II With displaced; mobile posterior retinal
Cataract (40–50%) flap
Macular pucker
Hypotony (IOP ≤5 mm) GRT III With everted or rolled up posterior
Corneal decompensation retinal flap
Vitreous hemorrhage GRT IV With everted or rolled up posterior
Hyphema retinal flap and star fold/macular
Phthisis bulbi pucker
Abbreviation: GRT, giant retinal tear Abbreviation: GRT, giant retinal tear
Retina 329
Table 5 Etiology of GRT (4–31%), hereditary vitreoretinopathies

(14.5%) and high myopia (9.7%).1-3
Ocular Systemic
Idiopathic (75–80%) Stickler’s syndrome
Traumatic (10–20%) Marfan’s syndrome REFERENCES
Myopia Ehlers-Danlos syndrome 1. Shunmugam M, Ang GS, Lois N. Giant retinal
Lattice degeneration tears. Survey of Ophthalmology. 2014;59(2):
Previous retinal 192-216.
cryotherapy 2. Ambresin A, Wolfensberger TJ, Bovey EH.
Intravitreal surgery Management of giant retinal tears with
Abbreviation: GRT, giant retinal tear vitrectomy, internal tamponade, and peripheral
360 degrees retinal photocoagulation. Retina.
at posterior edge of vitreous base, as if a 2003;23:622-8.
zipper has opened. 3. Al-Khairi AM, Al-Kahtani E, Kangave D, et al.
Prognostic factors associated with outcomes
Q.10. Most common associations of GRT. after giant retinal tear management using
Ans. The most common predisposing factors perfluorocarbon liquids. Eur J Ophthalmol.
for the development of a GRT is; trauma 2008;18:270-7.
POSTERIOR SEGMENT CYSTICERCOSIS

Harika Regani, Karthikeya R, Yamini Attiku, Atul Kumar

Ocular cysticercosis is most commonly caused by Onset may be sudden or insidious depending on
Cysticercus cellulosae, the larval form of the Taenia the presentation. It could be painful and associated
solium (pork tapeworm), though other species with redness (particularly dying cyst causes severe
may also be involved. It is endemic in tropical areas inflammation and vitritis) or painless (in case
such as Sub Saharan Africa, India, Latin America of retinal detachment or submacular scarring).
and East Asia. The posterior segment is involved The presentation is usually unilateral but can be
in approximately 68% cases.1 In the posterior bilateral in cases of disseminated cysticercosis.
segment, vitreous cavity is the most commonly Most commonly affected are patients from
involved site followed by subretinal space.2 lower socioeconomic strata due to poor hygienic
Posterior segment cysticercus is discussed practices and food habits (contaminated fruits and
here, which can be a short case/spotter in the vegetables with tapeworm ova). Patient may be
vegetarian by diet.2
examination with specific questions.
History of seizures, headache, vomiting,
subcutaneous nodules need to be elicited in
HISTORY patients suspected of cysticercosis. The central
nervous system (CNS) and skin involvement are
Chief Complaint
more common than ocular involvement. It is
The patient is typically a young males and presents rather very common to have CNS involvement
with diminution of vision. An aware patient can in cases of ocular cysts, while solitary ocular
also complain of scotoma due to the presence cysts per se are less common. Over all, CNS
of a cyst, retinal detachment or scarring. Pain manifestations are most frequent, up to 90% in
during ocular movements can occur if optic nerve some descriptions.1
is involved or there is concomitant extraocular Past and family history are usually not
infection. significant in such cases.
EXAMINATION
In general, any organ system may be affected.
zz Thorough work-up of the CNS (motor, sensory
and all cranial nerves) for any signs of focal
neurological deficit.
zz Musculoskeletal system: Palpate major muscle
masses (forearm, arm, thighs and legs) for
evidence of cysts in the muscle belly.
zz Skin examination for subcutaneous nodules.
Ocular Examination
Adnexal and orbital examination and its findings Fig. 1: Intraoperative photo of an intravitreal cyst
are covered elsewhere in the book: Particularly along with its scolex. Presence of scolex is diagnostic
subconjunctival cysts should be looked for, even for the parasite
in absence of findings like proptosis and ocular
movement disorders.
cyst wall, which is movement of the cyst wall due
Vision: Best-corrected visual acuity with projection to a mobile scolex. The size of the cyst may vary
of rays is recorded in both the eyes. Vision can be from 0.5 cm to 3 cm in diameter.
very poor in cases with severe vitritis or retinal
detachment but can also be surprisingly well Posterior segment findings
maintained in a few cases.
zz Subretinal cysts: May vary in size from 3 to 6
DD
IOP: It can be normal or decreased (uveitis) or zz RPE disturbances: Because of presence or
increased (neovascular glaucoma). migration of the cyst through the subretinal
Anterior segment: It can show evidence of active space, which causes mild inflammation and
inflammation (AC cells, flare, keratic precipitates) subsequent RPE changes. Sometimes they
or past inflammation [old keratic precipitates may represent the site of access to subretinal
(KPS), flare, pigments on endothelium, frank space from the choroid.
endothelitis]. Occasionally, a cysticercus can zz Intraretinal hemorrhage.
migrate in to the anterior chamber from the zz Vascular sheathing: Thought to be immune
posterior chamber through zonules. mediated reaction to the antigens of the
cysticercus.
Posterior segment : The cyst can be found zz Retinal detachment.
subretinally (eighty percent of the time being at —— Rhegmatogenous (Rhema being the
the posterior pole, or anywhere in the fundus), site of exit of the cysticercus from the
in the vitreous and rarely on the optic disc. subretinal space)
Sometimes multiple or dumbbell shaped cysts —— Tractional: Due to proliferation of the
(half in vitreous and half subretinal) may also be cells over the retina caused due to
encountered. inflammation.
Gross examination of the cyst: Globular, elongated zz Submacular/subretinal scarring.
or oval, milky white transilluminant cyst with zz Severe vitritis and a picture like endogenous
translucent wall and a white, opaque, dot like endophthalmitis: Due to a dying cyst which
area at one end, which indicates the position of leaks contents into the surrounding vitreous
the scolex (Fig. 1). This is typical for a live cyst. which are strongly antigenic.
On illumination of the cyst with a bright light, zz Calcified cyst/granuloma: A dead cyst can
such as that of the indirect ophthalmoscope, get calcified and may lead to a granuloma
the cyst shows undulating movements of the formation.
Retina 331
DIFFERENTIAL DIAGNOSES should precede neurological management, albeit

without delay.
In presence of mobile cysts with scolex, the Intraocular cysts are best managed by surgical
diagnosis is rather straightforward. Dead cysts removal.2,4 They are the treatment of choice in
may cause difficulty. Else other diagnoses of the modern era. Historically, the cysts used to be
retinal cysts-old RD, hamartomas, retinal mass removed in-toto using large sclerotomies but the
vs calcified cyst, dropped lens as a USG finding in current best practice is to rapidly lyse the cyst
hazy media, ciliary body cyst, etc. in vivo and aspirate the cyst contents with the
vitrector using high suction and high cut rate.
INVESTIGATIONS In toto removal has the advantage of allowing a
histopathological documentation.
Head imaging should always be done, regardless Dead cyst or dying cyst with severe inflam
of symptoms (see above). Following investigations mation is best managed by steroids. Anterior
are carried out: segment cysts may be managed by using visco
zz Complete blood count: To look for eosinophilia expression.
zz Stool examination: To find the eggs or
proglottids of the worm.
zz Ultrasonography: Cyst can be seen as a VIVA QUESTIONS
sonolucent area with well-defined anterior and
posterior margins. An echo dense, curvilinear, Q.1. What is the life cycle of cysticercosis?
highly reflective structure is present within Ans. In the life cycle of Taenia solium, man is the
the cyst corresponding to the scolex. USG is definitive host where sexual reproduction
better than CT for the detection of the scolex. occurs and the eggs are produced. Man
High amplitude spikes correspond to the cyst acquires Taenia infection by eating raw
wall and the scolex. The scolex shows a high or undercooked pork infested with the
amplitude spike due to presence of calcareous cysticerci. These cysticerci exvaginate in
corpuscles. Presence of high reflective scolex the stomach, attach to the intestinal wall
within a clear cyst is diagnostic of cysticercus. through scolex and develop into the adult
zz CT scan: Nonenhanced circular area of low
worms. Adult tapeworms develop, (up to
attenuation with tiny areas of increased
2 to 7 m in length and produce less than
attenuation within the lesion. This confirms
1000 proglottids, each with approximately
the diagnosis and helps to rule out neuro-
50,000 eggs) and reside in the small
cysticercosis.
intestine for years.
zz MRI brain: If neurocysticercosis is suspected.
The eggs/proglottids excreted in the
zz ELISA: Rarely helpful. Positive for anti
excreta are ingested by the intermediate
cysticercal antibodies (cystic lesions without
hosts, pigs, in whom the eggs hatch in
a scolex with positive ELISA for anticysticercal
antibodies is diagnostic). the stomach, pierce the stomach, reach
skeletal muscle through blood and lead
to cysticercus formation. The life cycle is
MANAGEMENT complete when human consumes raw or
In cases of neurocysticercosis, ophthalmic undercooked pork. This cycle usually does
examination should be first done to rule out not lead on to ocular involvement, unless
cysticercosis before starting the patient on autoinfection occurs with the human
cysticidal drugs. Starting cysticidal drugs in the ingesting eggs or retrograde peristalsis.
presence of an intraocular live cysticercus can Typically, cysticercosis of humans occurs
lead to disastrous consequences by leading to when man acts as an accidental inter
severe intense inflammation and eventual loss of mediate host and consumes vegetables
eye. Therefore, in cases with neurocysticercosis (salad) contaminated with eggs of the
and intraocular cysticercosis, ocular management Taenia solium.3
Q.2. How do the cysticerci reach the posterior has faltered immune evading mechanisms,
segment? develops micro leaks in the cyst wall and
Ans. Cysticercosis is caused by the ingestion of leads to severe inflammation. This is the
the eggs of Taenia solium or by reflux of reason why a patient with live intraocular
gravid proglottids in a patient with taeniasis cysticercosis requires treatment for
from lower intestines into stomach and removal of cyst even if he is 6/6 at the time
their subsequent excystment. The embryos of presentation, and not cysticidal therapy.
hatched out of the eggs penetrate the walls of A dead cyst does not incite significant
the stomach, reach bloodstream, and lodge inflammation and may not be removed.
at sites with high blood circulation like the Q.4. What is the prognosis of a case of intra
eye, skeletal muscle, skin, heart and brain. ocular cysticercosis?
It reaches the orbit through the ophthalmic Ans. Prognosis depends on the presentation of
artery and the posterior segment through the condition. An uncomplicated intra
the posterior ciliary arteries. It penetrates vitreal live cysticercosis can be managed
the choriocapillaris and reaches the with pars plana vitrectomy with cyst lysis
subretinal space. Macula has been noted to and aspiration with good outcomes (if no
be the preferred site for the lodgment of the pre-existing macular scar). In cases with
cysticercus possibly due to the rich blood retinal detachment, prognosis is guarded. In
supply. From the macular subretinal space, cases with subretinal cysticercosis, macular
it can enter the vitreous cavity through a scarring, tractional retinal detachment
break in the overlying neurosensory retina. prognosis is guarded.4
When this migration occurs, the defect
in the retina thus formed can give rise to REFERENCES
rhegmatogenous retinal detachment or
1. Duke-Elder S (Ed). Cysticercosis. System of
more commonly, this site heals with a scar
Ophthalmology. CV Mosby: St. Louis; 1978.
due to the inflammation associated with
p. 40.
the cysticercus migration and leads to an 2. Sharma T, Sinha S, Shah N, Gopal L, Shanmugam
area of scarring in the retina. An alternate MP, Bhende P, et al. Intraocular cysticercosis:
route of entry for cysticercus into the clinical characteristics and visual outcome
vitreous cavity has been hypothesized to be after vitreoretinal surgery. Ophthalmology.
directly from the retinal blood vessel or the 2003;110(5):996-1004.
ciliary body.2 3. Junior L, Perera CA. Ocular Cysticercosis. Am J
Ophthalmol. 1949;32(4):523-48.
Q.3. When does cysticercus lead to inflam- 4. Azad S, Takkar B, Roy S, Gangwe AB, Kumar M,
mation? Kumar A. Pars plana vitrectomy with in vivo
Ans. A live cysticercus has mechanisms to evade cyst lysis for intraocular cysticercosis.
the host immune system and only cause Ophthalmic Surg Lasers Imaging Retina.
mild inflammation. A dying cyst, however, 2016;47(7):665-9.
CATARACT IN SILICONE OIL-FILLED EYES

Sagnik Sen, Esha Agarwal, Raghav Ravani, Atul Kumar
INTRODUCTION especially in complicated rhegmatogenous retinal

detachments, old detachments with proliferative
Silicone oil with its biomechanical properties vitreoretinopathy changes, giant retinal tears,
of buoyancy, surface tension and viscosity is a endophthalmitis, etc. But, at the same time
very good agent for endotamponade and has silicone oil implantation has been associated with
been used along with pars plana vitrectomy its own set of changes in the eye when kept for a
Retina 333
long time, namely, oil in the anterior chamber, Scleral and episcleral scarring should be noted
emulsification, cataract in phakic eyes, glaucoma and recorded.
and keratopathy. Management of cataract in Anterior chamber: Oil bubbles inside the anterior
silicone oil filled eyes is different from senile chamber (Hyperoleon sign; Fig. 1) and signs of
cataract not only due to difficulty in getting the emulsification are common.
true biometry in these patients but also due to the
anatomical challenges. Pupil: Pupil dilation, regularity, and neovas
cularization of the iris (NVI) should be noted.
Direct light reflex in the index eye or consensual
HISTORY
reflex of the other eye may be used as good
Chief Complaint prognostic indicators.
Usually, recurrent loss of vision following vitrec Lens: Presence of iridophacodonesis indicating
tomy or in some cases no gain in vision following compromised zonules, lens subluxation, anterior
vitrectomy. capsular plaque, posterior capsular plaque/
defect (Fig. 2). Cataract should be graded as in
History of Presenting Illness any other case. Sometimes emulsified oil bubbles
may be found stuck to the anterior or posterior
The history is generally straightforward; there capsule.
is usually the history of gain in vision following Fundus should be carefully examined
vitreoretinal surgery followed by gradual especially to know the optic nerve status and
diminution of vision. In most of the cases cataract the integrity of macula. One should refer to pre-
develops within a year of vitrectomy, before or operative retinal findings and intraoperative
following its extraction. However, some patients findings for determining prognosis if retina cannot
can present within days or weeks with total be examined due to media haze.
cataract—in such cases iatrogenic damage to the
lens capsule should be suspected. It is important
to take a detailed history of the nature of the
vitreoretinal pathology and the extent of previous Includes other causes of vision loss following
surgery as they have a direct bearing on the vitreoretinal surgery: Secondary glaucoma, band
success or complexity of the phacoemulsification shaped keratopathy, refractive error, retinal
procedure and its overall visual benefit to the complication like RD, epiretinal membrane,
patient. cystoid macular edema, retinal toxicity, optic
neuropathy.
Past History
History of trauma, detailed history of the vitreo-
retinal procedure and pathology for determining
the visual prognosis.
Family History
This is usually not significant to work-up.
EXAMINATION
Eyeball, lids and adnexa should be examined as in
any other case.
Cornea: One should look for band-shaped
keratopathy (BSK), corneal pigments and corneal
opacity, which may have been incurred during the
vitrectomy. Fig. 1: Oil bubbles stuck behind the lens
Determining the length of the anterior

chamber, lens and vitreous cavity separately and
adding these values together can calculate the true
AL.
AL = ACD + LT + VCD (AL = axial length; ACD
= anterior chamber depth; LT = lens thickness;
VCD = vitreous cavity depth).
Theoretically, the velocity of sound in silicone
oil of viscosity 1000 centistokes compared with the
velocity of sound in vitreous humor decreases by a
factor of 0.64 (987 m/s ÷ 1532 m/s). It is therefore
possible to calculate the true depth of the vitreous
cavity (VCDoil × 0.64) and hence the true AL.
The conversion factor of 0.71 multiplied by
Fig. 2: Slit-lamp photograph depicting posterior cap the measured axial length has been reported by
sule plaque behind the IOL after phacoemulsifica
Murray1 to correct for the apparent increase in
tion. Such plaques may be later tackled with laser
axial length induced by silicone oil of viscosity
capsulotomy
1300 cSt.
Other alternative methods include partial
INVESTIGATIONS coherence interferometry (IOL Master, Zeiss),
which is preferred over ultrasonography to
B-scan Ultrasonography calculate the axial length of the silicone oil filled
One should remember that due to the differing eye. It should be remembered that light speed is
sound speed in oil, the eyeball appears to be large not affected to a level significant enough to cause
and enhanced depth mode should be utilized. In falsifications in AL calculation as in USG. Axial
eyes with advanced cataract, fundus evaluation length of the fellow eye can be used in certain cases
with an indirect ophthalmoscopy may not be in which axial length measurement of the oil filled
feasible and an assessment using a B-scan should eye is not possible. Previous records if available
be made. Here the imaging is best carried out in can also be use especially if scleral explants have
sitting position to determine inferior RDs as well. In not been used. Intraoperative retinoscopy has also
addition to that, careful evaluation of the posterior been used for IOL power calculation.
capsule by ultrasound B-scan can be done if direct
visualization is not possible. If the ultrasound
Specular Microscopy
shows an abnormally large lens thickness or an In the post-vitrectomized eyes, the corneal
out-pouching of the posterior lens surface, a defect endothelium is often compromised especially
in the posterior lens capsule should be suspected. in those cases where silicone oil is present in the
anterior chamber, so it is important to perform
Biometry specular microscopy in these patients.
Anterior segment OCT (ASOCT) may also be
Biometry should be performed for IOL power use to analyze PC when appropriate to document
calculation. However, axial length measurement integrity of the PC.
in an oil filled eye is a big challenge. In ultra
sonography, due to change of velocity of sound in
TREATMENT
different viscosity, axial length measurements also
vary as compared to normal vitreous in silicone Cataract surgery, typically using phacoemulsifica
oil filled eyes. Velocity of sound in physiological tion and intraocular lens implantation, is recom
vitreous filled phakic eye is 1532 m/s. Velocity mended for individuals with visually significant
of sound in oil is slower, being 987 m/s in oil of lens opacities. Phacoemulsification with IOL
viscosity 1000 cst, causing measured axial length implantation can be performed safely in post-
to be longer in oil filled eyes. vitrectomized eyes.2 Patients who need both
Retina 335
cataract surgery and silicone oil removal can Q.3. Which is the most common type of
undergo either a combined or two step surgical cataract seen in oil-filled eyes?
approach. Most of the studies show similar visual Ans. Progressive nuclear sclerosis is the most
outcome and complication rates with both the common type followed by posterior
approaches; however, combined surgery offers subcapsular cataract in the young patients.
the advantages of a single surgical event and a
Q.4. What are the various risk factors for
faster visual rehabilitation.3 Silicone IOLs should
development of cataract in post vitrecto
be avoided. In presence of PC defect oil bubbles
mized eyes?
are suddenly seen in AC. Frequent AC wash
Ans. • Older age
may be needed during surgery for removing the
• Degree of preoperative nuclear sclerosis
emulsified oil, and AC tends to collapse often, as
• Intraoperative lens touch
oil tends to rise in supine position. Capsulorhexis
• Diabetic retinopathy
may be difficult to plaques and retroillumination
• Silicone oil injection.
assisted maneuvers are typically difficult due to
poor glow. The capsular opening tends to run out. Q.5. What is the pathomechanism of cataract
Use of viscocohesive should always be considered. formation in oil-filled vitrectomized
eyes?
Ans. However, the exact cause of cataract
VIVA QUESTIONS
formation in oil filled eye is not known
Q.1. What is the incidence of cataract following entirely. However, it has been postulated
vitreoretinal surgery with silicone oil that altered metabolism at the lens-oil
injection in phakic patients? interface and direct oil induced toxicity
Ans. All eyes with silicone oil injection inadver may be responsible, both leading to
tently undergo cataract formation in almost oxygen stress to the lens proteins leading
100% cases. The incidence of development to their oxidation. Also, increased oxygen
of visually significant cataract ranges from exposure to the lens following vitrectomy,
8 to 80% for nuclear sclerotic cataract and lens toxicity from intraocular irrigating
4–34% for posterior subcapsular cataract solution, intraoperative lens touch by
(PSC) in various studies.4,5 Although, early surgical instruments, use of intravitreal
removal of oil has been associated with steroids during vitrectomy, removal of
a decreased risk of cataract formation, barrier function provided by the vitreous,
however, cataracts have been reported even permeability changes in lens capsule,
months after oil removal. uveitis were the various other reported
causes of cataract formation following
Q.2. What are the common morphologies of
vitrectomy.6,7
cataract seen in oil-filled eyes?
Ans. • Posterior subcapsular feathery opacity, Q.6. What are the various refractive changes
seen in early postoperative periods. seen in aphakic and phakic patients
• Development of posterior fibrous pseu following silicone oil injection?
dometaplasia and finally posterior sub Ans. Refractive state in silicone oil filled eyes
capsular cataract and posterior capsular depends on the extent of oil fill inside the
plaque. vitreous cavity and the shape of the anterior
• Formation of lens vacuoles in posterior oil surface. In aphakics, the anterior surface
part of lens is convex; hence acting similar to the
• Early lens opacities leading to nuclear crystalline lens and due to the induced
sclerosis, with or without brunescence. myopia may bring these eyes towards
• Rapid progression of nuclear sclerosis to emmetropia. However, in phakic eyes,
white cataract with hypermaturity, often this anterior surface being concave and
leading to leaking of proteins and uveitic refractive index of oil being higher than that
changes. of the crystalline lens, the oil acts as a minus
lens rendering the eye hypermetropic. Table 1 Surgical difficulties and intraoperative
These myopic and hypermetropic shifts complications
have been on an average close to 6D.
Further changes may occur depending Cornea Peripheral corneal injury
Stripped Descemet’s membrane
on whether or not an encirclage was used
during vitreoretinal surgery. Anterior Fluctuations in AC depth
chamber Infusion deviation syndrome
Q.7. What are the fallacies in measuring axial
length using A-scan ultrasound and Iris Prolapse
Miotic pupil
other methods to measure axial length in
silicone oil-filled eyes? Lens Tears in rhexis margin
Ans. Refer to the chapter above. Marked zonular laxity/dehiscence
Posterior capsular plaque
Q.8. Which type of IOL should be preferred in Unplanned posterior capsulorhexis
silicone oil filled eye? Posterior capsule rupture
Ans. As silicone oil can interact with various Unplanned AC intraocular lens (IOL)
intraocular lens biomaterials with a Posterior Nuclear drop/dropped lens
potential of reducing the optical quality segment fragment
of the lens, the type of IOL used becomes Suprachoroidal hemorrhage
an issue. It has been postulated that it is
Others Conversion from topical to
the hydrophobia of silicone oil, which intracameral anesthesia
influences its interaction with intraocular
lenses. The more hydrophobic a lens
biomaterial is the more the adherence of
silicone oil; the more hydrophilic, the less Table 2 Early and late postoperative
the adherence. Interaction of silicone oil complications
was seen maximal with silicone lenses, so Cornea Moderate to severe corneal edema
they should be best avoided. Acrylic lenses Pseudophakic bullous keratopathy
or polymethyl methacrylate (PMMA) lenses Anterior IOP spike
can be successfully used. As convexo- chamber Wound leak
plano lens with the plane surface facing
Iris Chronic postoperative iritis
posteriorly induces minimal refractive
Irregular pupil
change, they are preferred in silicone oil
Rubeosis iridis
filled eyes.
Lens Incorrect intraocular lens power
Q.9. How to choose an appropriate IOL power Intraocular lens decentration or
in silicone oil-filled eyes? dislocation
Ans. Silicone oil due to its higher index of Capsulorhexis contraction
refraction (1.40) as compared to vitreous Posterior capsular opacification
behaves like an intraocular minus lens Posterior New or persistent macular edema
in pseudophakia. Therefore, without segment Retinal detachment
appropriate power adjustment, significant
hyperopic overcorrection would be
expected. The more curvature or power additional IOL power to be added to the
incorporated in the posterior surface of the calculated IOL power to arrive at the power
lens, the greater is the postoperative error. of IOL to be implanted in a silicone oil filled
The convexo-plano lens with the plane eye:
surface facing posteriorly induces minimal Additional IOL power = {(Ns-Nv)/(AL-
refractive change. ACD)} ×1000
The following formula have been suggested Ns: Refractive index of silicone oil (1.4034)
by Patel (1995) and Meldrum8 to find the Nv: Refractive index of vitreous (1.336)
Retina 337
AL: Axial length in millimeters removal: visual outcome and complications

ACD: Anterior chamber depth in milli- in a combined vs. two step surgical approach.
meters. Retina. 2003;23(5):647-53.
4. Melberg NS, Thomas MA. Nuclear sclerotic
Q.10. What are the various surgical difficulties cataract after vitrectomy in patients less than
and intraoperative and postoperative 50 years of age. Ophthalmology. 1995;102:
complications in silicone oil-filled eyes? 1466-71.
Ans. Surgical difficulties and intraoperative 5. Novak MA, Rice TA, Michels RG, Auer C. The
complications are summarized in Table 1.2 crystalline lens after vitrectomy for diabetic
Early and late postoperative complications retinopathy. Ophthalmology. 1984;91:1480-4.
are summarized in Table 2.2 6. Holekamp NM, Shui YB, Beebe DC. Vitrectomy
surgery increases oxygen exposure to the lens:
a possible mechanism for nuclear cataract
REFERENCES
formation. Am J Ophthalmol. 2005;139:302-10.
1. Murray DC, Durrani OM, et al. Biometry of the 7. Petermeier K, Szurman P, Bartz-Schmidt UK,
silicone oil-filled eye: II. Eye. 2002;16:727-30. Gekele F. Pathophysiology of cataract formation
2. G r us ha YO, Masket S, Mil l er KM . after vitrectomy. Klin Monbl Augenheilkd. 2010;
Phacoemulsification and lens implantation 227:175-80.
after pars plana vitrectomy. Ophthalmology. 8. Shamnas HJ. IOL lens power calculation:
1998;105(2):287-94. Ultrasound measurement of the challenging
3. Krepler K, Mozaffarieh M, Biowski R, Nepp J, eye. Slack Incorporated. Thorofare, NJ, 2004.
Wedrich A. Cataract surgery and silicone oil pp. 113-23.
SILICONE OIL-INDUCED SECONDARY GLAUCOMA

Ashish Markan, Esha Agarwal, Raghav Ravani, Atul Kumar
INTRODUCTION Some patients may develop very high IOPs in

immediate/late postoperative period and may
Silicone oil (polydimethylsiloxane) is a linear present with complaint of nausea, vomiting,
synthetic polymer made of repetitive Si-O units pain, redness and blurred vision. The patient
and is used as an internal tamponade agent in can present with these symptoms within hours
vitreoretinal surgeries. First introduced by Paul or years after surgery. Some cases present with
Cibis in 1960s, it has become an important adjunct history of gradual painless loss of vision following
in vitreoretinal surgery. Secondary glaucoma can vitreoretinal surgery.
occur at any time in the postoperative period, and
may range from mild and transient to severe and Past History
sustained resulting in vision loss.
Detailed history of vitreoretinal pathology for
HISTORY which surgery was done, history of pre-existing
glaucoma, trauma, any history of steroid intake in
Chief Complaint past or present and its mode and duration should
Patients are generally asymptomatic. However, few be taken. Other eye history should also be recorded,
cases may present with acute pain, redness, blurred as that of other risk factors of glaucoma. OT notes
vision and colored halos following vitrectomy. may be reviewed for amount of oil fill if available.
History of Presenting Illness Family History

As most of these patients are asymptomatic most Family history of glaucoma is important, as
of the cases will be detected with glaucoma/ patients with positive family history can be steroid
high intraocular pressure (IOP) on follow-up. responders.
EXAMINATION Lens: Aphakic, phakic or pseudophakic status

as well as presence of any subluxation should be
Eyeball, lids and adnexa should be examined as
documented.
in any other case.
Fundus should be carefully examined
Cornea: Corneal edema and bullous keratopathy especially to know the optic nerve status (vertical
are suggestive of raised IOP. Any band shaped cup disc ratio, neuroretinal rim, bayonetting,
keratopathy, corneal pigments and corneal opacity baring of circumlinear vessels, pallor) and the
should be documented. integrity of macula. A retinal examination should
Scleral and episcleral scarring with special con be performed keeping in mind the original
cern to its site and extent should be noted, it is espe indication of surgery ant OT notes, and findings
cially important if one is planning trabeculectomy. carefully documented.
Anterior chamber: AC depth should be carefully
examined as patients with pupillary block
INVESTIGATIONS
glaucoma or malignant glaucoma will present Pachymetry
with shallow AC. Presence of any AC cells, flare, As variation of central corneal thickness (CCT) in
hyphema, emulsified oil (Fig. 1) or oil globules in normal corneas can lead to falsely higher pressure
anterior chamber should be noted as it helps in readings with thicker corneas and falsely lower
knowing the etiology. with thinner corneas, it is important to measure
Angle: Gonioscopy should be performed to look CCT to know the corrected IOP.
for emulsified oil in angle (superior angle), NVI,
PAS, increase pigmentation, and angle recession. Visual Fields
These patients are prone to surgical failure. Static perimetry (HVF/Octopus) should be
Intraocular pressure measurement: IOP should performed wherever possible as it helps in
be measured using Goldmann applanation diagnosing as well as in detecting progression of
tonometer. glaucoma. If HVF/Octopus is not possible due
Iris: Pupillary ruff atrophy, sphincter tear, NVI, and to poor vision, Goldmann visual field should be
any iridotomy and its patency if present should be performed.
noted.
B-scan Ultrasonography
Pupil: Direct and consensual light reflex should
be checked as it gives the gross idea of optic nerve It should be performed if media is hazy and
status. fundus evaluation is not possible by indirect
ophthalmoscopy. It can also be used to detect
glaucomatous cupping of 0.7 or greater in eyes
in which optic nerve cannot be examined due to
media haze.
RNFL-OCT may be done, scanning laser based
or otherwise. Comparisons with unaffected fellow
eye are helpful to loss of NRR.
MANAGEMENT
Treatment is directed towards treating the etiology
(see viva questions). If planning for surgery,
oil removal should be considered in cases with
emulsification.
Medical Therapy
Fig. 1: Hyperoleon: along with bubbles of silicone oil.
These bubbles induce fibroses in the angle leading zz Corticosteroids and cycloplegics are indi
onto glaucoma cated to reduce the inflammation. Aqueous
Retina 339
suppressants are generally preferred to reduce of trabecular meshwork leading to its collapse
the IOP. Hyperosmotic agents can be used for and sclerosis. Furthermore, SO removal itself
short-term control of IOP. can cause IOP elevation by several mechanism;
zz Success rate of medical therapy in controlling Firstly, due to edema of the trabecular meshwork
high IOP in silicone oil-filled eyes varies from because of postoperative inflammation. Secondly,
30% to 78% in various studies.1,2 the mechanical impact of balanced salt solution
during silicone oil removal may split the silicone
Prophylactic Peripheral Iridectomy oil droplets into much smaller drops, which are
more likely to obstruct the trabecular meshwork.5
Inferior peripheral iridectomy (PI) described by
Therefore, whether oil removal helps or not is still
Ando3 helps to prevent pupillary block glaucoma
a matter of debate.
in aphakic. As silicone oil floats superiorly (specific
gravity 0.97), an iridectomy (ideal size 150–200 μ)
done at 6 o’clock position prevents pupillary block Filtration Surgery
by allowing aqueous passage from the posterior to Conventional filtration surgery has a limited role
the anterior chamber. Superior PI is done in cases and success rate in the management of glaucoma
where heavy silicone oil is used. after pars plana vitrectomy and silicone oil injec
However, postoperative closure of the PI has tion.6 Trabeculectomy in these eyes is also techni
been reported in about one-third of eyes under cally difficult because of conjunctival scarring from
going silicone oil surgery. If the PI is not patent, previous retinal surgeries. Increased postoperative
treatment involves reopening the peripheral inflammation and emulsified silicone oil may lead
iridectomy, either with a YAG laser or surgically. to blockage of internal ostium and trabeculectomy
If the cause is a blockage by fibrin or clot, injection failure. Inferior trabeculectomy is not advisable as
of tissue plasminogen activator (tPA) into anterior it carries high risk of endophthalmitis.
chamber has been reported with success.
Glaucoma Drainage Device
Selective Laser Trabeculoplasty
Glaucoma drainage implants offer a good surgical
Selective laser trabeculoplasty (SLT) may be option and have better surgical outcomes as
considered as a treatment option for the patients compared to trabeculectomy in oil filled eyes.
with open-angle glaucoma (OAG) secondary However, oil migration can occur through the
to emulsified SO which are not at high risk for tube into subconjunctival space inciting an
progressive glaucomatous damage to save time inflammatory reaction and its failure.
before more invasive surgical interventions are
performed.4 It acts by activating the macrophages Cyclodestructive Procedures
loaded with SO and remodeling the extracellular
matrix in the trabecular meshwork by releasing Transscleral photocoagulation can be used to
cytokines, hence increasing trabecular outflow. control IOP in oil filled eyes, but as it carries a risk
Typically, recurrences are seen needing alternative of visual loss, it is generally reserved for cases with
therapy in due course. poor visual outcome.
Silicone Oil Removal VIVA QUESTIONS

The benefit of early SO removal before the
emulsification was demonstrated to be effective Q.1. What is the incidence of secondary glau
for IOP regulation in higher proportion of the eyes. coma following vitreoretinal surgery with
However, the late removal of emulsified SO does not silicone oil injection?
necessarily prevent the development of glaucoma Ans. The true incidence of glaucoma after
as prolonged contact of emulsified silicone oil silicone oil injection is difficult to ascertain
with trabecular meshwork causes organic changes from the literature. First reported by Cibis,
in the endothelium and collagen component the incidence of high IOP intraocular
pressure ranges from 2.2% to 56% in various Q.4. What is the ideal site of peripheral
studies,7,8 depending on the definition of iridectomy in silicone oil-filled eyes?
elevated IOP and the time considered. Ans. It should be inferior at 6 o’clock, peripheral
In the silicone study, 8% of the cases that and not more than 2 mm because larger
underwent SO tamponade experienced more centrally located inferior iridectomy
glaucoma at 36-month follow-up. may allow silicone oil to enter the anterior
chamber, creating a form of reverse pupil
Q.2. What are the various risk factors for lary block with a deep anterior chamber.
development of glaucoma in post-
vitrectomized eyes? Q.5. State whether silicone oil removal helps
Ans. Following are the risk factors for developing in controlling IOP in oil-filled eyes.
silicone oil-induced glaucoma Ans. See discussion in chapter above.
• Pre-existing glaucoma Q.6. What are the causes of failure of
• Diabetes trabeculectomy in silicone oil-filled eyes
• Trauma and ways to prevent it?
• Aphakia Ans. Scleral and episcleral scarring from pre
• Oil in the anterior chamber vious surgery and increased postoperative
• Emulsification of the oil inflammation are the main causes of
• Use of low viscosity silicone oils as trabeculectomy failure in oil-filled eyes.
compared to high viscosity oils Emulsified oil droplets may block the
• Heavy silicone oils ostium/bleb. Use of antimetabolites (MMC
• Duration of oil tamponade. or 5-FU) during trabeculectomy, making
Q.3. What is the pathomechanism of glaucoma large ostium, performing cyclodialysis
combined with trabeculectomy may
in oil-filled vitrectomized eyes?
decrease the chance of failure.
Ans. Several mechanisms have been proposed
for secondary glaucoma following the use Q.7. What are the other causes of glaucoma
of silicone oil in vitreoretinal surgeries: after VR surgery?
Early postoperative rise in IOP Ans. Tight explants, steroid induced, gas overfill,
• Pupillary block improper concentration of gas, NVG,
• Migration of silicone oil into the anterior malignant glaucoma, lens intumescence,
chamber with consequent mechanical inflammatory, choroidal hemorrhage, etc.
impediment to filtration
• Inflammation REFERENCES
• Overfill 1. Honavar SG, Goyal M, Majji AB, et al. Glaucoma
– Absolute after pars plana vitrectomy and silicone oil
– Relative—due to increase choroidal injection for complicated retinal detachments.
thickness Ophthalmology. 1999;106:169-76.
• Pre-existing glaucoma. 2. Al-Jazzaf AM, Netland PA, Charles S. Incidence
Late postoperative rise in IOP and management of elevated intraocular
• Infiltration of the trabecular meshwork pressure after silicone oil injection. J Glaucoma.
2005;14(1):40-6.
by silicone bubbles
3. Ando F. Intraocular hypertension resulting from
• Chronic inflammation
pupillary block by silicone oil. Am J Ophthalmol.
• Synechial angle closure 1985;99(1):87-8.
• Rubeosis iridis 4. Alkin Z, Satana B, Ozkaya A, et al. Selective
• M igration of emulsified and non- laser trabeculoplasty for glaucoma secondary
emulsified silicone oil into the anterior to emulsified silicone oil after pars plana
chamber vitrectomy: a pilot study. Biomed Res Int.
• Idiopathic open-angle glaucoma. 2014;13:469163.
Retina 341
5. Pastor S. Cyclophotocoagulation: A report by 7. Unosson K, Stenkula S, Tornqvist P, Weijdegard

the American Academy of Ophthalmology. L. Acta Ophthalmol (Copenh). 1985;63:
Ophthalmology. 2001;108(11):2130-8. 656-60.
6. Nguyen QH, Llyod MA, Huer DK, et al. 8. de Corral LR, Cohen SB, Peyman GA. Effect of
Incidence and management of glaucoma after intravitreal silicone oil on intraocular pressure.
intravitreal silicone oil injection for complicated Ophthalmic Surg. 1987;18:446-9.
retinal detachments. Ophthalmology. 1992;99:
1520-6.
POSTERIOR DISLOCATED LENS

INTRODUCTION deficiency syndrome, hyperlysinemia, focal

dermal hypoplasia) and coloboma.
Posterior dislocation of lens is one of the worst
complications of cataract surgery. Rarely cases of Past Surgical History
spontaneously dislocated lens can also be seen in
clinical practice. Such cases are commonly given Recent history of cataract surgery may be there
as short cases. These may include lens drop/IOL in case of IOL dislocation or lenticular fragment
drop/lenticular fragment drop/subluxation of dislocation.
lens/decentered intraocular lens (IOLs).
EXAMINATION
HISTORY General Examination/Specific Systemic
Examination
Chief Complaint
A thorough systemic examination is carried
The usual presenting symptoms are loss of vision,
out when ectopia lentis is supposed to be the
pain and redness of the affected eye.
underlying cause.
History of Present Illness Ocular Examination

The patient may present with sudden loss of vision Visual acuity: Uncorrected visual acuity (UCVA),
in eye after trauma or no visual gain after intra best corrected visual acuity (BCVA) both undilated
ocular surgery or gradual visual loss in disorders and dilated (esp. in cases of decentered IOLs/
associated with slow zonular dehiscence. partially subluxated lens) should be checked as
There may be complaints related to recent inflam final management depends on the same.
mation. In the cases where subluxation preceded
Eyeball: Large eyeball is seen in high myopic
dislocation, there may be history of diplopia/edge
patient while small eyeball may be seen in cases of
effect related astigmatism.
coloboma. Nystagmus or squint may be present in
cases of coloboma.
Lid: Lid findings are usually normal.
History of trauma, intraocular surgery, coloboma, Conjunctiva: Scar may be present in case of
pseudoexfoliation syndrome or systemic diseases previous surgery. Ciliary as well as diffuse
associated with ectopia lentis must be ruled out. conjunctival congestion can be there due to
associated inflammation.
Family History Cornea: Cornea may be pear shaped in coloboma.
Family history may be there in cases of ectopia Krukenberg spindle may be present in pseudo-
lentis (Marfans, homocysteinuria, sulfite oxidase exfoliation. Corneal edema or Descemet folds
may be present in cases with history of recent com look for presence of the anterior vitreous in pupil
plicated cataract surgery. This finding is extremely plane/AC or otherwise.
important in deciding the timing of surgery. In Fellow eye: Lens may be subluxated or dislocated
cases of lens drop during phacoemulsification, the in bilateral diseases. Broken zonules or zonular
wound might have been extended in an attempt dialysis may be there. Pseudoexfoliation material
to deliver the nucleus, in such cases careful may be present over anterior lens capsule. A
examination (with fluorescein staining) has to be posterior polar cataract may be present, partially
done to ensure proper wound closure. In addition, explaining the complicated surgery in the other
any vitreous twig extending to corneal wounds eye. Traumatic cases would generally have a
must be ruled out. normal fellow eye. In cases of pseudophakia in the
Sclera: Scleral thinning may be there in cases of other eye, look for evidence of posterior capsular
connective tissue disorders (e.g. blue sclera in rent (PCR) in the other eye; that may suggest a
collagen diseases) and pathological myopia. In posterior polar cataract as the predisposing factor
cases of nucleus drop following small incision for lens drop.1,2
cataract surgery (SICS) careful examination of the Anterior vitreous: Presence of vitreous pigments
wound integrity must be done. also known as tobacco dusting or Shaffer’s sign
Anterior chamber (AC): AC may be deep in high may be there (Note: Shaffer’s sign is one of the
myopic eyes or shallow in pseudoexfoliation. characteristic sign of rhegmatogenous retinal
Anterior chamber cell and flare may be present detachment, however, both trauma and surgery can
(more in case of dislocated crystalline lens). produce this sign).
ACD should be checked as an ACIOL may be Distant direct examination: On distant direct
implanted during rehabilitation. Presence of examination in fellow eye may reveal subluxation
vitreous in AC must be ruled out by careful or dislocation. There will be a poor glow in the
slit-lamp examination. If vitreous is touching aphakic eyes and characteristic crescent reflex is
corneal, endothelium there is a chance of corneal seen in cases of subluxation in the other eye.
decompensation, in such cases the decision to go
Fundus: Indirect ophthalmoscopy is the most
ahead with surgery has to be expedited.
important examination. Dislocated lens (Fig. 1)
Iris: Iridodialysis (in trauma) or atrophy may or IOL (Fig. 2) may be entangled into vitreous/
be there. Transillumination test is positive in vitreous base or may be situated inferiorly. The
pseudoexfoliation syndrome. Iridodonesis is location depends on status of vitreous degenera
common in such cases. tion. One should look for vitreous hemorrhage,
Pupil: Pupillary abnormalities that can be
seen in such cases include eccentric pupil in
Marfan’s syndrome, and a poorly dilating pupil
in pseudoexfoliation syndrome. Size of the pupil
must be evaluated, vis-a-vis. secondary IOL
implantation.
Intraocular pressure (IOP): IOP may be raised in
case of inflammation or pseudoexfoliation.
Gonioscopy: Angle recession or cyclodialysis may
be there in case of trauma. Dense pigmentation of
the angles is seen in pseudoexfoliation.
Lens: IOL may or may not be present, depending
on initial management by the phaco surgeon. It
is necessary to look for presence and status of the Fig. 1: Intraoperative photograph depicting dropped
capsular rim, as the best option is a sulcus IOL sclerotic lens over the posterior pole. Note nuclear
implant for rehabilitation. One should carefully sclerosis
Retina 343
Ultrasonography
Ultrasonography in done in presence of media
haze.
zz Ultrasonography appearance of dropped
nucleus: Seen as a biconvex body, which may
be mobile or fixed. Lens fragment usually
produces vitritis that can be seen as multiple
mild-moderate amplitude spikes.
zz USG appearance of dropped IOL: Appears
similar to a foreign body showing high
reflectivity and shadowing effect behind it.
UBM
Fig. 2: Intraoperative photograph of dropped rigid UBM is useful in cases of angle recession, and for
IOL. The haptics are being freed of vitreous sulcus assessment.
status of PVD, retinal tear, its extent (in degrees or

Specular Count
clock hours), location of tear, retinal detachment, It is done for evaluation of corneal endothelium,
extent of retinal detachment (total or subtotal), and it is necessary for complicated surgeries.
macular edema. In myopia or Marfans disease,
there may be accompanying signs of vitreoretinal OCT
degeneration. Optical coherence tomography (OCT) is done to
In case of lens drop, size of fragment must rule out CME, only if suspected.
be noted as up to 20% sized fragments may
be traditionally left alone. Only cortical drop
MANAGEMENT
can be managed easily with steroid therapy
and inflammation control. Grade of nuclear The management of dropped nucleus begins at
sclerosis must be noted, harder lens would need the time of first surgery itself.
fragmentation while softer lens are amenable to
cutter dissection. In cases of IOL drop, it should Recommendation for Anterior Segment
be noted if the IOL is rigid or foldable, broken or Surgeon
intact. If IOL power is suitable, the IOL may even zz Attempt lens fragment removal if only
be repositioned in the sulcus.
accessible.
Fellow eye: One should look for posteriorly zz Perform anterior vitrectomy to avoid vitreous
dislocated lens, myopic changes, lattice, retinal prolapse in limbal wound.
tear and retinal detachment in the fellow eye. zz Insert PCIOL or ACIOL whenever possible
(unless fragment is very hard and later limbal
DIFFERENTIAL DIAGNOSIS extraction is planned).
zz Close wound in standard fashion and ensure
Usually, the cases are straightforward. In patients
watertight closure.
with media haze, however, Ultrasonography (USG)
zz All vitreous and viscoelastic must be removed
finding can have differentials like endophthalmitis,
completely.
cysticercosis, IOFB, etc.
zz Postoperative medication must include
intensive steroid for inflammation control
INVESTIGATIONS
(both systemic and topical), cycloplegics and
Axial length and keratometry or optical biometry antiglaucoma medication. Include topical
(if possible) is done, if secondary IOL implantation hypertonic saline drops if corneal edema is
is planned. present.
zz Refer to posterior segment surgeon as soon as is rather frequent. PFCL helps in management
possible. of such small pieces as well, while preventing
macular damage.
Conservative Therapy
Prognosis
For smaller nuclear fragments and cortical drop,
conservative management may be planned. Careful case selection and timing of surgery along
Though in the era of safe vitrectomy, usually with postoperative care results in better prognosis.
surgery is recommended. Until the time surgery Around 60–80% patients achieve a visual acuity of
is awaited, delayed, or planned, careful control >20/40 with proper care.3
of IOP, inflammation and corneal edema Other types of posterior surgeries: Torsional frag
should be planned, along with regular posterior mentation, Four-port vitrectomy with chandelier,
examinations. limbal vitrectomy with electrical cutter, PFCL
levitation and removal through limbus for hard
Lens Removal (Phacofragmentation) cataracts, using micro vitreo-retinal knife (MVR)
for lens elevation.
Surgical removal is preferred within 2 weeks after
original cataract surgery.
INTRAOCULAR LENS (IOL) DISLOCATION
Indications of surgery includes following: Inadequate posterior capsular support from
zz Nuclear size >2 mm or >25% capsular/zonular rupture due to trauma is usually
zz Inflammation not responding to treatment by the basis of IOL dislocation. It can be early or late.
1–2 weeks despite optimum therapy. Nucleus
is full of antigens that can incite inflammation. Early Dislocation
zz Persistently raised IOP despite medical
Completely dislocated PCIOL usually occurs in
therapy.
first week. Placing the IOL on anterior hyaloid
zz Retinal detachment, retinal tear, endophthal
through posterior capsule rupture or spontaneous
mitis and other complications.
IOL haptic rotation can cause early dislocation.
Factors affecting the technique of removal of lens
includes: Late Dislocation
zz Size of lens Late dislocation is less common. Trauma or
zz Matter-nuclear or cortical spontaneous loss of zonular support as in
zz Time since surgery pseudoexfoliation syndrome or Laser capsulotomy
zz Presence of inflammation. (After YAG capsulotomy dislocation of IOL,
characteristically foldable IOL dislocate due to
Surgery release tension from fibrosis.) can cause late
Two routes can be used-either limbal or pars dislocation.
plana. The various techniques that can be used
include vitrectomy cutter (for cortical matter); IOL Removal
Ultrasonic fragmentation (for grade 2/3 hardness Three different approaches are followed IOL
of nucleus); mechanical crushing between two removal, IOL removal with IOL exchange or
instruments; limbal extraction of hard nuclear IOL removal with IOL repositioning. For IOL
fragment (advanced grades/brunescent cataract). repositioning capsular rim should be at least, six
However, with modern day vitrectomy machines, clock hours/180 degree in which three clock hours
vitrectomy with fragmentation is the preferred should be intact inferiorly. Best way to judge is
technique. retroillumination.
Role of Perfluorocarbon Liquid (PFCL) Indication for Removal

It floats nucleus anteriorly and decreases Mobile IOLs with attached vitreous cortex
complication. Chattering in the vitreous cavity can typically cause complications and should
Retina 345
be removed. In addition, IOLs stuck over the See recommendations for anterior segment
posterior poles causing visual dysfunction need above in chapter.
removal. Conventionally immobile IOLs away Q.3. What are causes of ectopia lentis?
from posterior pole with detached cortical vitreous Ans. See the chapter on ectopia lentis.
have been left in situ. However, again, in today’s
era of safer vitrectomy, most IOLs are removed. Q.4. Discuss management of dropped lens.
Ans. This has been discussed above.
Other Indications Q.5. What is optimum timing for manage
ment of dropped lens during phacoe-
Substantial intraocular inflam m ation, CME,
mulsification?
retinal detachment, vitreous in wound/attached
Ans. There are two opinions on this. Immediate
to iris.
surgery at the time of the drop has the
Surgery involves vitrectomy, PVD induction,
advantage of a single surgery with less
PFCL injection, freeing the IOL from all vitreous
patient anxiety and quicker rehabilitation.
tags, grasping the IOL at optic-haptic junction,
Later surgery after corneal edema and
careful removal through the limbus while
inflammation control has the advantage
maintaining IOP and protecting endothelium.
of better and easier surgery planned
Secondary IOL may be placed, wound closed
IOL rehabilitation and already loosened
and vitrectomy completed with PFCL removal.
vitreo-retinal attachments. However, most
Complications are similar to lens drop.
surgeons believe if cornea is clear enough
to allow for surgery and vitreoretinal expert
VIVA QUESTIONS is available, it is better to go for immediate
surgery.
Q.1. What are predispositions to complicated Q.6. How to rehabilitate an aphakic patient?
cataract surgery, and what are the signs Ans. Spectacle, contact lens, and secondary
for posterior polar cataract. IOLs (SFIOL/ACIOL/Sulcus IOL) are the
Ans. See chapter on posterior polar cataract. options. Surgery may be deferred if poor
Q.2. Management of PCR during phacoemeul visual function is anticipated. See relevant
sification with prevention of nucleus chapters for discussion.
drop.
Ans. It is important to recognize risk factors for REFERENCES
complicated surgery before beginning the
cataract surgery. See risk factors above. 1. Aasuri MK, Kompella VB, Majji AB. Risk factors
Next the surgeon should recognize for and management of dropped nucleus
during phacoemulsification. J Cataract Refract
presence of PCR early (see chapter on polar
Surg. 2001;27(9):1428-32.
cataract). Further management should
2. Khokhar S, Soni A, Pangtey MS. Risk factors
depend on the size of PCR, size of lenticular for and management of dropped nucleus after
fragments pending for emulsification and phacoemulsification. J Cataract Refract Surg.
surgeons ability. If drop is imminent it 2002;28(8):1310.
would be wiser to enlarge the wound using 3. Ryan SJ, Schachat AP, Wilkinson CP, Hinton
appropriate viscoelastics and deliver out DR, Sadda SR, Wiedemann P. Retina. 5th edn.
the lenticular fragments manually. Elsevier Health Sciences, 2012.
STARGARDT DISEASE
Aswini Kumar Behera, Ruchir Tewari
INTRODUCTION Genetics
Stargardt disease, or fundus flavimaculatus Stgd 1: Most common form of mutation in
(Fundus flavimaculatus is the term designated for Stargardt disease. It is the recessive form caused by
the phenotypic presentation of Stargardt disease mutations in the ABCA4 gene.
in which “flecks” are distributed throughout the
Stgd 3: Seen in dominant form of Stargardt disease
fundus) is an inherited form of juvenile macular
caused by mutations in the ELOVL4 gene.
degeneration.1 It causes progressive vision loss
usually to the point of legal blindness. It is the most Stgd 4: Autosomal dominant transmission.
common childhood inherited macular dystrophy. PROM1 gene-heterozygous mutation.
It is commonly kept as a short case in postgraduate Disease spectrum is determined largely by the
examination. total amount of residual ABCA4 function.2
HISTORY EXAMINATION
Symptoms Systemic Examination
The disease is bilateral and symmetric, though Systemic examination may not reveal findings in
asymmetric presentations may be seen. Main isolated ocular disease and are typically absent.
symptom is loss of visual acuity. Complaints of
diminution of vision may first be recognized as
Ocular Examination
early as 5 years but may even be seen as late as
50 years or more. Very early onset patients usually Visual acuity: Best corrected visual acuity should
have a fairly severe ABCA4 genotype and more be recorded. Refractive errors may be seen. Loss of
sensitive foveal cones. visual acuity, can be as mild as 20/30 or as severe
as 20/200.
History of Presenting Illness Examination of anterior segment, eyeball,
lid adnexa and orbit usually does not show any
Apart from painless, gradual in onset vision loss, findings.
other symptoms includes:
zz Wavy vision/metamorphopsia Posterior segment: While 90 D examination
zz Central scotoma is a must for macular examination, peripheral
zz Blurring examination with 20 D/28 D lens should also be
zz Impaired color vision done. Peripheral lesions like retinal degenerations
zz Difficulty in adapting to dim lighting and pigmentary changes may be associated with
zz Photophobia macular dystrophy. Typical findings includes:
zz Slow dark adaptation.
zz Abnormal fundus appearance that is inciden
Vision is most noticeably impaired when the tally discovered.
macula (center of retina and focus of vision) is
zz Light-colored flecks at the level of the retinal
damaged, leaving peripheral vision more intact. pigment epithelium—more elongated than
Peripheral visual fields also tend to stay stable. round.
zz Pisciform (fish-tail): Two adjacent flecks form
an obtuse angle.
Family History zz Many different fleck configurations.
This is extremely important. Typically autosomal zz Fairly reliable diagnostic sign: Relative sparing
recessive pattern may be traced on pedigree of the peripapillary retinal pigment epithelium
analysis. (RPE).
Retina 347
zz Uniform vermillion or light-brown color to INVESTIGATIONS

the fundus with complete obscuration of the
underlying choroidal details. Autofluorescence
zz Frank RPE atrophy is commonly seen in the Due to lipofuschin deposits, hyper-auto
center of the macula and the bases of these fluorescence may be elicited. However, diagnostic
atrophic lesions have a metallic sheen (Fig. 1). reliability is low as some patients can have
zz “Beaten-bronze” appearance. hypo-autofluorescence also. Amount/density of
zz Choroidal neovascular membranes (CNVMs) autofluorescence may be utilized in follow-up
and subretinal bleed may be seen as also.3
complications.
There is interplay of three factors: Fundus Fluorescein Angiography Finding
1. Severity of ABCA4 genotype (determines rate zz Complete masking of the choroidal circulation.
at which toxic bisretinoids are formed in the zz With angiography, the dye-filled retinal
photoreceptors). vessels lie upon a completely hypofluorescent
2. Relative sensitivity of the foveal cones to the background that results in a finding variously
genotype. known as a dark, silent, or masked choroid.4
3. Relative sensitivity of the retinal pigment
epithelium to the genotype. Optical Coherence Tomography
Optical coherence tomography (OCT) can reveal
DIFFERENTIAL DIAGNOSIS the extent of outer retinal loss and RPE atrophy
Differentials of Bull’s eye maculopathy: and it can also distinguish the anatomic level of
zz Stargardt diseases flecks with accuracy. Choroidal layer changes have
zz Cone and cone-rod dystrophy also been studied, though concrete evidence is
zz Chloroquine retinal toxicity lacking. Choriocapillaris may be lost. Late stages
zz Age-related macular degeneration (ARMD) reveal thinned out macula and may catch CNVMs
zz Chronic macular hole or SRF.
zz Central areolar choroidal dystrophy
zz Olivopontocerebellar atrophy Electrophysiology
zz Ceroid lipofuscinosis As full-field ERG represents a mass response of all
Other macular dystrophies should be kept as photoreceptors, it is typically normal in patients
differential diagnosis. with Stargardt disease. Cone and rod functions may
be affected in severe ABCA4 genotypes.5 Multifocal
electroretinogram (mfERG) is a sensitive tool in
detecting very early involvement in even clinically
normal cases. Significant decrease in ERG wave
form amplitudes is noted in all rings, even the most
peripheral eccentricity group 10°–31°, although
the change from normal goes on decreasing as we
move from central to peripheral eccentricity rings.
Visual Field Testing

Visual field testing in Stargardt patients is often
normal in early disease stages.
Over time, relative central scotomas develop
which progress to absolute central scotomas.
Fig. 1: Macular atrophy and flecks in a case of macular Color vision and contrast sensitivity may be done
dystrophy. Note the pigment mottling and temporal for progressions or as ancillary tests, but do not
disc pallor add to diagnosis.
MANAGEMENT Q.2. What are differential diagnoses of Bull’s

eye maculopathy?
There is currently no proven treatment for this
Ans. See the section on differential diagnosis.
disease. Since a primary defect in ABCA4 associ
ated retinal disease is an accumulation of toxic Q.3. What is Stargardt-like dominant macular
bisretinoids in the RPE and photoreceptors, drugs dystrophy (SLDMD)?
that modulate the visual cycle (e.g. Isotretinoin Ans. Stargardt-like dominant macular dystrophy
and fenretinide), have been investigated for their (SLDMD).
potential to slow the formation of these toxic • Autosomal dominant
products. • Chromosome 6
Gene therapy and cell replacement may be • E lovl4 gene: Elongation of very long
upcoming treatment modality. chain fatty acids-4
• M ost-characteristic features of this
Visual Rehabilitation disease are circular zone of RPE atrophy,
a pigmented spot beneath the fovea, and
Low vision aids (LVA)—such as magnifying glasses a ring of flecks just beyond the margin of
may be help in cases with macular atrophy. the atrophy
• ERG is usually normal.
Patient Counseling
Q.4. What are the fundus findings in Stargardt
zz Stargardt patients should be encouraged to disease?
maintain good sun protection, as exposure to Ans. See above.
bright light can lead to the formation of all-
Q.5. What is the pathophysiology of Stargardt
transretinal in photoreceptors and contribute
disease?
to lipofuscin accumulation.
Ans. See Flow chart 1.
zz Avoidance of cigarette smoking or avoidance
of high-dose vitamin A supplements, including Q.6. What is Fishman classification of
AREDS vitamins, because of their potential to Stargardt disease?
increase the formation of bisretinoids in the Ans. See Table 2.
retina. Q.7. What is fundus flavimaculatus?
zz Sibling screening and genetic counseling is Ans. Fundus flavimaculatus and Stargardt
important. disease are varied clinical presentation
zz These patients should be kept on follow-up of the same disease process and belong
for documenting progression and treating at different ends of the clinical spectrum.
complications. Pure Stargadt disease presents with
macular involvement and pure fundus
VIVA QUESTIONS flavimaculatus presents with multiple mid
peripheral ‘fleck’ lesions with preservation
Q.1. Name few macular dystrophy. of macular region. In most cases, a mixed
Ans. See Table 1. presentation is usually seen. In general,
Table 1 Common examples of macular dystrophy

Macular dystrophy Gene Chromosome Pattern
1 Best macular dystrophy BEST1 11 AD/AR
2 Stargardt disease ABCA4 1 AR
3 Stargardt-like dominant macular dystrophy ELOVL4 6 AD
4 Pattern dystrophy PRPH2 6 AD
5 Sorsby fundus dystrophy TIMP3 22 AD
6 Autosomal dominant radial drusen EFEMP1 2 AD
Retina 349
Flow chart 1: Pathophysiology of Stargardt disease
Table 2 Fishman classification of Stargardt disease

Stage 1 Fundus: Pigmentary change in macula, “beaten-bronze” appearance.
ERG: Normal.
Stage 2 Fundus: Flecks beyond 1 DD from margin of fovea, extending beyond arcade.
ERG: Normal.
Prolonged period of dark adaptation
Stage 3 Fundus: Diffuse flecks and choriocapillary atrophy at macula
ERG: Subnormal cone and rod amplitude
Central field defect as well as peripheral/midperipheral field impairment
Stage 4 Fundus: Diffuse flecks and extensive RPE atrophy throughout the fundus
ERG: Reduced cone and rod amplitude
Peripheral field—Moderate to extensive restriction
patients with extensive extramacular by classic single bilateral macular egg yolk
flecks have poorer long-term visual pro like vitelliform lesions, though, multiple
gnosis than patients with only macular lesions involving the posterior pole may be
involvement. seen. It has an autosomal dominant mode
of inheritance in typical cases, although
Q.8. What are fleck lesions?
autosomal recessive inheritance as well
Ans. These are accumulations of lipofuscin
as adult onset has been described. The
seen at the RPE level. They are usually
classic lesions typically appear in early
more elongated than drusens and may
childhood, though are not usually picked
connect with each other forming a net like
up as visual acuity remains very good till
branching pattern. Different shapes, size,
very late in the disease process. Different
color and location can be seen. They may
stages for the lesions are described but
remain stable in number and location with
the disease may not follow specific staging
preservation of visual acuity or may grow
pattern.
in size leading to widespread atrophy and
Stages:
decline in vision.
• Previtelliform
Q.9. What is Best dystrophy? What are it • Vitelliform
stages? How is it different from Stargardt? • Pseudohypopyon
Ans. Best disease is a juvenile onset vitelliform • Vitelliruptive
macular dystrophy that is characterized • Atrophic
On SD-OCT, the vitelliform lesions amplitudes, a ratio known as the Arden

represent accumulation of hyper-reflective ratio, which represents ratio between the
material in the subretinal space. Full field maximal height of potential in the light
ERG is typically normal. EOG is a specific (light rise) and minimal potential in the dark
investigation for Best disease. An Arden (dark trough) is used to quantify the EOG
ratio of <1.5 is said to be characteristic of values. Most of the response is manifested
Best disease. due to photoreceptor activity and RPE.
Cases with Best disease differ from Stargaadt Electrooculogram is a specific investigation
disease in many ways: for Best disease. A highly abnormal EOG
• Presence of a well-defined yellow lesion with normal ERG is diagnostic of Best
at the macula disease.
• Maintained visual acuity till late stages
• Absence of flecks REFERENCES
• E lectrooculogram (EOG) <1.5 with
normal ERG 1. Hadden OB, Gass JD. Fundus flavimaculatus
• Characteristic SD-OCT with presence of and Stargardt’s disease. Am J Ophthalmol.
1976;82:527-39.
subretinal hyper-reflective material.
2. Cremers FP, van de Pol DJ, van Driel M, et al.
It may become difficult to clinically
Autosomal recessive retinitis pigmentosa
distinguish the two in cases with atrophic and cone-rod dystrophy caused by splice site
Best disease as only well-defined central mutations in the Stargardt’s disease gene ABCR.
atrophy might be present in such cases. Hum Mol Genet. 1998;7:355-62.
EOG may be diagnostic in such cases. 3. Lois N, Halfyard AS, Bird AC, et al. Fundus
Also, autosomal dominant inheritance autofluorescence in Stargardt macular
with presence of lesions in otherwise dystrophy-fundus flavimaculatus. Am J
asymptomatic relatives may help in Ophthalmol. 2004;138:55-63.
diagnosis. 4. Jayasundera T, Rhoades W, Branham K, et al.
Peripapillary dark choroid ring as a helpful
Q.10. What is Ardens ratio? diagnostic sign in advanced Stargardt disease.
Ans. E lectrooculogram is a measure of the Am J Ophthalmol. 2010;149:656-60.e2.
difference in standing potential of the 5. Schindler EI, Nylen EL, Ko AC, et al. Deducing
eye between the cornea that is electrically the pathogenic contribution of recessive
positive and the RPE that is electro- ABCA4 alleles in an outbred population. Hum
negative. As there is much variation in EOG Mol Genet. 2010;19:3693-701.
TRAUMATIC RETINAL DETACHMENT

Priyanka Ramesh, Shreyas Temkar, Dheepak Sundar, Atul Kumar
Traumatic retinal detachment accounts for 12% of Sometimes it is easy to link trauma to RD, whereas
all rhegmatogenous retinal detachment (RD) and sometimes the patient may try to hide the history
is the most common cause of rhegmatogenous RD or may even have forgotten it. The onus is on the
(RRD) in children. RD can occur both following ophthalmologist to identify the precipitating event
open globe and closed globe injury. In closed in the latter case.
globe injury, the detachment is following a retinal
tear or dialysis, whereas in an open globe injury, Chief Complaints
the retinal detachment is due to vitreous traction The patient can present in following ways:
following vitreous prolapse or direct injury related zz Sudden onset of field loss and diminution of
break.1 vision.
Retina 351
zz Incidental RD may also be detected while While pure retinal detachment may cause inac
managing for other manifestations of trauma. curate PR rarely, it can be present in post-traumatic
It is usually seen in young male patients cases, which suggests concomitant optic nerve
with definitive history of trauma either blunt damage and hence poor visual prognosis.
or penetrating. Patients can present either
Eyeball: Presence of squint, ocular movement
immediately following trauma or can present until
restrictions, enophthalmos, discontinuity in the
usually about 2 years following trauma.
orbital rim has to be checked to rule out any orbital
trauma. It is not uncommon to see signs of blow
History of Presenting Illness out fractures.
Detailed history about when the trauma occurred,
Lids, conjunctiva: May show signs of trauma.
the mode of injury has to be taken. In addition,
There may be lid laceration or subconjunctival
following points must be enquired:
hemorrhage. Complex lacerations may need a
zz History of any surgical intervention like
plastic surgical review.
corneal/scleral perforation repair has to be
noted. Sclera: In fresh cases, one should always look at
zz If a patient is presenting late after trauma, then the integrity of the globe and rule out the presence
the previous ocular findings, visual acuity has of any scleral rupture. In doubtful delayed cases, a
to be reviewed. This may help in case wise thorough examination should be done as posterior
prognostication. Children typically present as possible for signs of old repaired scleral wounds.
late after trauma. All these points become very
Cornea: In fresh cases, there may be a corneal
crucial in a medicolegal case.
laceration and in old cases, there might be scars of
zz History of trauma should always be ruled
previously operated corneal laceration or of a self-
out on leading questions in unexplained
sealed corneal wound. Limbal scars are common
ophthalmic cases.
in blunt trauma related open globe injury.
Past History Anterior chamber: In acute injury there might

be hyphema in the anterior chamber. Detailed
History of any surgical procedures done before
examination must be done to check for any signs
the trauma or for its management has to be
of inflammation like cells and flare.
taken. History of recurrent trauma may indicate
patient abuse or even self-mutilation (mentally Iris: There might be associated iridodialysis, which
challenged) or poor functional status of vision. appears as a D shaped pupil and best confirmed
on a distant direct examination. The iris may also
Family History show presence of sphincter tears and traumatic
mydriasis, which causes anisocoria. Sphincter
In doubtful cases, family history of retinal disorders
tears may also be seen.
must be inquired for.
Gonioscopy: It is necessary to do gonioscopy in all
EXAMINATION patients with trauma to rule out angle recession.
Findings must always be compared with the
Systemic Examination other eye. There will be increased width of the
History of loss of consciousness, ENT bleeds and ciliary body band and increased pigmentation.
seizures have to be evaluated in case of multiple Cyclodialysis can also be picked up on gonioscopy.
injuries. In cases of polytrauma, other system Lens: It can be cataractous ranging from total
involvement has to be assessed. These patients cataract to posterior subcapsular variety, typically
would require multidiscipline management. the rosette cataract. There can also be associated
subluxation/dislocation of the lens. These patients
Ocular Examination will have phacodonesis and iridodonesis. One
Visual acuity: Best-corrected visual acuity of both should keenly examine for presence of the Vossius
eyes with projection of rays has to be checked. ring.
Pupillary reactions: Direct and consensual presentation will have chronic RD often with
reflexes must be checked in both eyes and swinging signs of proliferative vitreoretinopathy (PVR)
torch light test must be done for relative afferent (Fig. 1).
pupillary defect (RAPD). In case the affected eye zz Other signs of trauma like commotio retinae,
has traumatic mydriasis or there is obscuration of subretinal bleed, choroidal rupture, macular
the pupil due to hyphema then the consensual in hole should be looked for.4
the other eye becomes a very important predictor zz Optic disc must be evaluated for presence
of an intact optic nerve. of traumatic optic neuropathy or for glau
Intraocular pressure (IOP): In the presence of comatous changes.
retinal detachment, the IOP is usually low, but
zz Other eye examination is necessary for
in the presence of angle recession or increased satisfying Cox’s postulates in doubtful cases.
inflammation, there might be raised IOP. See Table 1 for posterior segment signs of
trauma.
Posterior segment: The idea is two pronged:
Identify signs of trauma and work-up for RD. The
vitreous cavity may show the presence of vitreous
hemorrhage in acute cases. If the posterior See chapter on RD. Specifically in a patient
segment details are obscured due to vitreous with GRT differentials like myopia, iatrogenic
hemorrhage, an ultrasonography (USG) of the
posterior segment is required to rule out retinal
detachment.
If the fundus is visible then the fundus has to be
examined thoroughly to look for:
zz All retinal breaks has to be localized
zz Indentation indirect ophthalmoscopy has to
be done to check for dialysis and vitreous base
avulsion.
zz Retinal dialysis is typical for blunt trauma; SN
is pathognomonic while IT is most common.2,3
zz Other indicative breaks include giant retinal
tear (GRT), ragged margin multiple tears,
etc. As such, any kind of break may be
seen. Work-up should be done as for retinal Fig. 1: PVR in a case of traumatic RD. Note the
detachment. Young patients with delayed encircled peripheral retinal fold
Table 1 Posterior segment signs of trauma

Closed globe injury Open globe injury
Vitreous base avulsion Scleral rupture/penetration
Retinal tears (dialysis, horseshoe tears, operculated holes, tears with Retinal tears
ragged margins) Retinal detachment
Retinal detachment Proliferative vitreoretinopathy
Commotio retinae Retained intraocular foreign body
Macular hole Endophthalmitis
Choroidal rupture
Subretinal bleed
Vitreous hemorrhage
Choroidal hemorrhage
Posteriorly dislocated lens/IOL
Retinitis sclopetaria
Retina 353
and idiopathic, and in patients with retinal Q.2. What are the types of retinal breaks seen
dialysis, inferior temporal retinal dialysis of young following blunt trauma and what is the
should be kept in mind (see discussion). Ragged mechanism of breaks?
margin breaks may also be due to viral retinitis Ans. Blunt trauma (ocular contusion) results
related necrosis. in numerous types of breaks like retinal
dialysis, horseshoe tears, giant retinal tears
INVESTIGATIONS (GRT), operculated holes and macular
hole. These breaks can be due coup injury
USG B scan: Can be used to rule out RD in patients causing breaks at the site of trauma or
where the visualization of the retina is hampered. counter coup, opposite the site of trauma
It can show vitreous incarceration in few cases. and are usually located predominantly in
USG is also useful to rule out presence of retained the vitreous base region. Breaks caused
intraocular foreign body. by retinal necrosis occur slowly and show
X-ray orbit: Used to rule out orbital fractures or ragged uneven edges.
any retained intraocular foreign body. Q.3. What is the definition of retinal dialysis
Optical coherence tomography: May be done for and what is the most common location of
concomitant macular changes due to trauma. dialysis following trauma?
Ans. Retinal dialysis is defined as disinsertion
Visual evoked response (VER): It is useful in cases
of the retina from the nonpigmented
of old RD and those in which traumatic neuropathy
epithelium of the ciliary body at the ora
is suspected.
serrata. It accounts for 8 to 14% of the retinal
detachments. GRT and dialysis account for
MANAGEMENT 69% of all traumatic detachments.
Medicolegal cases need proper documentation Most common location is inferotemporal
while multidepartment approach may be needed. accounting for 66% of cases. This is
Other concomitant manifestations of trauma because the inferotemporal quadrant is
may need management. See chapter of RD for least protected based on orbital anatomy.
detailed management and figures. In patients with Superonasal location is the most patho
blunt trauma, it is necessary to rule out retinal gnomonic of trauma. Weidenthal and
dialysis. PVD is typically absent in these patients. Schepens reported that nasal retina has
Prognostication is of essence as these patients greater susceptibility to traumatic retinal
typically need recurrent surgeries and have poor dialysis secondary to its narrow vitreous
outcomes. Complications like postoperative base. Mean size of post-traumatic dialysis is
glaucoma and cataract are frequent. around 2.4 clock hours.
In patients with open globe injury, it is Q.4. When does retinal detachment occur
necessary to secure the wound in the coats. following trauma?
Ans. Retinal detachment immediately follow
VIVA QUESTIONS ing trauma is rare and in general the
detachment progresses slowly occurring
Q.1. What are the mechanical changes in the weeks to months following trauma. This is
globe following blunt trauma? because of the presence of formed vitreous
Ans. Changes occur in four phases in young patients. Following points must be
1. Compression remembered:
2. Decompression • 12% detachments are identified imme-
3. Overshooting diately.
4. Oscillations • 30% detachments are identified within
Blunt trauma typically causes antero- 1 month.
posterior compression with equatorial • 50% detachments are identified within
expansion of the globe. 8 months.
• 8 0% detachments are identified by Inferotemporal quadrant is most com

24 months. monly involved but superonasal dialysis
GRT following trauma usually shows a is pathognomonic of trauma.
rapid progression. Q.7. What are the posterior segment signs of
Q.5. What is the mechanism of retinal detach trauma.
ment following open globe injury? Ans. See Table 1.
Ans. In the presence of open globe injury, the Q.8. What are the Cox’s postulates for
vitreous is incarcerated in the wound traumatic retinal detachment?
and there is fibrous ingrowth along the Ans. In some cases it is difficult to differentiate
vitreous scaffold. There is also break down between spontaneous retinal detachments
of blood retinal barrier, which initiates from those occurring due to trauma. Cox,
an inflammatory response causing Schepens and Freeman gave certain
proliferation of pigment epithelial cells, postulates, which could point towards
fibroblasts and glial cells. These produce occurrence of retinal detachment from
collagenous extracellular matrix, which trauma. They include:
causes contraction. This causes traction • Unilateral retinal detachment preceded
on the peripheral retina, rolling forward at by ocular contusion.
the vitreous base and junction of the ora. • O bjective signs of contusion in the
Over weeks, the proliferation progresses affected eye.
and causes cyclitic membrane, epiretinal • Absence of visible vitreoretinal degen
membrane and retroretinal membrane. eration of the types known to cause
There can also be abrupt PVD causing retinal breaks in both the affected and
retinal breaks. fellow eyes.
Q.6. What are the seven rings of trauma?
Q.9. What is inferior temporal dialysis of
Ans. Blunt trauma can result in injury to various
young?
tissues of the eye. Seven rings of tissues
Ans. The patient is typically young male and
affected by blunt trauma to the eye are:
has unilateral or bilateral retinal dialysis
1. Tears in Sphincter pupillae—seen as
in the inferior temporal area. Sometimes
disruption of pupillary margin on slit
other changes like WWOP, retinal cystic
lamp.
degeneration and pigmentation/holes
2. Iridodialysis—seen as dehiscence of iris
may be there. The causation is related to
from the sclera with a D shaped pupil.
abnormal development of the inferior
3. A ngle recession—characterized by
temporal pars plana, which normally
separation of circular muscle fibers
develops the last. This is an important
from longitudinal fibers of ciliary body.
differential of a case of traumatic RD with
There is posterior displacement of the
retinal dialysis.
iris and widening of ciliary body band
on gonioscopy. It is always important to Q.10. Tell in brief about BETT classification.
confirm this finding by comparing with Ans. This system utilizes definitions that refer
the fellow eye. to the entire globe, not to a specific tissue.
4. Cyclodialysis: Separation of ciliary body This solves the problem of using confusing
attachment from scleral spur. This can terms with respect to individual tissues.
result in hypotony. Flow chart 1 showing clinical classification
5. Trabecular meshwork tears. of injuries based on BETT system.
6. Zonular dialysis resulting in subluxation BETT system also defines these injuries as
of the crystalline lens. follows:
7. R etinal dialysis—disinsertion of the • Eye wall: Sclera and cornea
retina from nonpigmented epithe • Closed globe injury: No full-thickness
lium of ciliary body at the ora serrata. wound of eye wall
Retina 355
Flow chart 1: Classification of ocular injury
Abbreviation: IOFB, intraocular foreign body
• O pen globe injury: Full-thickness wound • Penetration

of the eye wall • Perforation
• Contusion no wound of the eye wall. The • Retained intraocular foreign body
damage may be due to direct shock wave • Mixed
by the object (e.g. choroidal rupture), or 2. G rade (based on visual acuity at
to changes in the shape of the globe (e.g. presentation):
angle recession) • VA > 20/40
• Lamellar laceration: Partial-thickness • VA 20/50–20/100
wound of the eye wall • VA 19/100–5/200
• R upture: Full-thickness wound of the eye • VA 4/200–light perception
wall, caused by a large blunt object • No light perception.
• Laceration: Full-thickness wound of the
eye wall, caused by a sharp object 3. Pupil:
• Penetrating injury: An entrance wound is • Positive afferent pupillary defect
present • Negative afferent pupillary defect
• IOFB: One or more foreign objects are 4. Zone (based on the extent of the injury):
present • Cornea and limbus
• P erforating injury: Both an entrance and • Up to 5 mm from limbus
an exit wound are present. • > 5 mm from limbus.
Based on the Birmingham Eye Trauma
Terminology, injured eyes are categorized REFERENCES
by four parameters: 1. Campbell DG. In: Shingleton BJ, Hersh OS,
1. Type (based on the mechanism of Kenyon KR (Eds), Eye Trauma. St Louis, Mosby.
trauma): 1991.
Closed globe injury 2. Zion VM, Burton TC. Retinal dialysis. Arch
• Concussion Ophthalmol. 1980;98:1971-4.
• Lamellar laceration 3. Cox MS, Schepens CL, Freeman HM. Retinal
detachment due toocular contusion. Arch
• Superficial foreign body
Ophthalmol. 1966;76:678-85.
• Mixed 4. American Academy of Ophthalmology, Basic
Open globe injury and Clinical Science Course, Section 12: Retina
• Rupture and Vitreous.
CHAPTER
5 Neuro-ophthalmology
and Strabismus
LONG CASES
THIRD CRANIAL NERVE PALSY

Adarsh Shashni, Shipra Singhi
INTRODUCTION acquired form. At times a close observation of

old photographs may be helpful. Age less than
Cranial nerve palsies can be given as long 50 years warrants urgent imaging to rule out
case in exam. The third cranial nerve supplies ICSOL.
majority of extraocular muscles (MR, SR, IR, IO),
levator palpebrae superioris (LPS) and contains
pupillomotor fibers. A complete lesion of the III
Mode of Onset
nerve involving both branches will result in a deficit Acute onset of diplopia or deviation warrants
of elevation, adduction and depression in abduction immediate neuroimaging to rule out life-threaten
leading to the characteristic down and out position ing conditions, hence, the type of onset is extremely
of the eyeball. Most of the cases are acquired. important to note.
Following discussion is primarily on acquired palsy.
Diplopia
HISTORY
It is the presenting symptom in majority of cases.
Chief Complaint Primarily seen as horizontal and vertical binocular
A case of third nerve palsy may present with diplopia. It must be remembered that patients due
following: to ptosis may not volunteer diplopia. It may also be
zz Diplopia absent in patients with long standing disease with
zz Deviation of eyes early age of onset resulting in suppression.
zz Limitation of movements
zz Drooping of eyelid Pain
zz Diminution of vision for near, head posture, Headache, localized pain in the orbit, and peri
face turn, facial asymmetry, protrusion of eye, orbital region can be there suggestive of orbital
chemosis. inflammatory disease like Tolosa-Hunt syndrome.
Aneurysm of posterior communicating artery
History of Present Illness can be associated with severe pain or headache.
Age at onset: It is important to note the age of Ischemic mononeuropathy (e.g. DM) can be
onset to differentiate between congenital and associated with variable degree of pain.
Neuro-ophthalmology and Strabismus 357
Poor Visual Acuity, Abnormal Head Posture, examination must include other cranial nerves
Facial Asymmetry and the peripheral nervous system. In a child, an
otorhinolaryngological review may be sought.
Presence of these suggests congenital nature of the
disease. Ocular Examination
Glare Visual Acuity
Rarely a dilated pupil (due to involvement of It is necessary to lift the ptotic lid to evaluate visual
pupillomotor fibers). acuity. Vision may be reduced due to mydria
sis, particularly for near visual acuity due to loss
of accommodation. It may be reduced due to
Associated Symptoms
amblyopia in congenital or early age onset 3rd
Although rare patient may complain of difficulty CN palsy. Visual acuity must be checked with and
in reading, protrusion of eye, chemosis, headache, without glasses.
pain, vomiting. Patient may present with other
neurological symptoms like contralateral hemi Face
paresis in Weber’s syndrome, contralateral tremors Head posture: Head posture is taken to position
in Benedict syndrome and ipsilateral ataxia in the eye away from direction of action of paralytic
Nothnagel syndrome. muscles so as to minimize the deviation which
can be fused to avoid diplopia. So, for left 3rd CN
History of Past Illness palsy—face turn to right with head tilt to left with
A careful past history is helpful in identifying the chin elevation.
probable cause of third nerve palsy. Important Facial asymmetry—should be noted in con
causes include etiologies for oculomotor palsy: genital cases. This can be assessed by measuring
vasculopathic process (diabetes, hypertension, the lateral canthus to angle of mouth distance and
CAD), trauma, compression (e.g. aneurysm, comparing with that of other side.
ICSOL) and/or infiltrative (e.g. leukemia), inflam Palpebral fissure asymmetry—measurement of
matory, infection, demyelinating disease, toxic palpebral fissure should be done using transparent
(e.g. chemotherapy). ruler in primary gaze, in adduction and abduction
Similar episodes in past must be enquired. and depression to rule out aberrant regeneration.
Recurrent third nerve palsy can occur in DM,
aneurysm, and ophthalmoplegic migraine.
Eyeball
zz The characteristic position of the eyeball is
Past Surgical History “down and out” (Fig. 1) due to unopposed
History of any previous neurosurgical procedure
must be recorded.
Family History and Birth History

In cases of congenital third nerve palsy both these
history are important.
EXAMINATION
Examination
A thorough review of systems should be
conducted, including enquiry about other
neurological symptoms, giant cell arteritis, trauma Fig. 1: Characteristic “down and out” position of
and symptoms of ear disease. Neurological the eyeball in 3rd nerve palsy
Fig. 2: Limited elevation (SR), depression (IR) and adduction (MR) in 3rd nerve palsy
action of the lateral rectus and superior zz In very mild cases, latent deviation or phoria
oblique muscles. can be elicited by Maddox rod or alternate
zz There will be limited elevation (SR), depression cover testing (exo and hypo deviation) to
(IR) and adduction (MR), which may be dissociate the 2 eyes and interrupt fusion).
complete or partial limitations, depending on
the extent of paresis/palsy (Fig. 2). Lid
zz The intact superior oblique muscle also causes Ptosis is usually severe and the upper lid will have to
intorsion of the eye at rest, which increases on be raised by the examiner (or an assistant) in order
attempted down gaze. to perform the ocular motility assessment. If there
zz Check for the presence of IV nerve function is ptosis, one must look for lid fatigue or lid twitch
by asking the patient to attempt to look down (kindly see myasthenia short case), because these
and outwards and observe for the presence of might indicate myasthenia gravis. Lagophthalmos
incyclotorsion during this movement. This can should be checked in all such cases. Aberrant
be checked by observing the movements of the regeneration to LPS from extraocular motor nerve
nasal limbal vessels. fibers should be checked which might improve
zz In case of partial third cranial nerve, palsy ptosis on attempted duction movements of the
the deficit of adduction, supraduction, and respected involved recti muscles (e.g. Pseudo von
infraduction should be apparent even if Graefe sign—lid retraction seen on attempted
partially paralyzed. One can also check for downgaze). Aberrant regeneration is important
saccades which will be floating in direction of to document as it helps in guiding the surgical
action of paretic muscles. management.
Conjunctiva Binocular Function

Conjunctival injection or chemosis can be present Binocular function should be assessed after
in cases of lymphoma and thyroid dysfunction. neutralization of deviation. This is usually absent
especially in congenital and early age onset 3rd
Cornea/Sclera/Iris CN palsy with large angle of deviation. Those with
late onset and in partial 3rd CN palsy binocular
Usually normal, corneal sensation should be
function are usually intact.
checked in all cases of CN palsy, which may be lost
in orbital inflammatory disease. Worth Four-dot Test
This is a dissociation test using red-green glasses,
Pupil
which can be used with both distance and near
3rd CN palsy generally presents with anisocoria fixation and differentiates between BSV, ARC and
more prominent in daylight. This sluggish or suppression. Results can only be interpreted if the
absent reaction to light is there due to involvement presence or absence of a manifest squint is known
of parasympathetic fibers that originate in the at time of testing. If two red and three green lights
Edinger-Westphal subnucleus of the third cranial are seen, diplopia is present.
nerve complex. Aberrant regeneration to pupil
should also be checked. This can be from ciliary Bagolini Striated Glasses
body resulting in light near dissociation (tonic
This is a test for detecting BSV, ARC or suppression.
pupil) or from extraocular motor nerve fibers
Each lens has fine striations that scatter and convert
which can be assessed by checking pupillary
point source of light into line perpendicular to the
constriction on attempted duction (e.g. if from MR
striations, as with the Maddox rod.
than on attempted adduction).
Double Maddox rod test
IOP/Lens/Vitreous zz Red and green Maddox rods, with the cylinders
vertical, are placed one in front of either eye.
Usually normal. zz Each eye will therefore perceive a more or less
horizontal line of light.
Fundus zz In the presence of cyclodeviation, the line
Usually normal. However always rule out papill perceived by the paretic eye will be tilted and
edema (raised ICT), any vasculitis, vascular therefore distinct from that of the other eye.
disease and choroidal fold (orbital mass lesions). zz One Maddox rod is then rotated until fusion
(super-imposition) of the lines is achieved.
zz The amount of rotation can be measured
Special Tests for Squint/3rd Nerve Palsy in degrees and indicates the extent of
Measurement of deviation (kindly see the chapter cyclodeviation.
of 6th nerve palsy and esotropia).
Diplopia Charting
Convergence zz Diplopia charting using red-green glasses: It is
done by using red-green glasses and projecting
This will be absent if the medial rectus muscle is a streak of light on a screen testing the patient
paralyzed. at 1 meter [see 6th nerve palsy]
zz Synoptophore: The synoptophore compensates
Accommodation for the angle of squint and allows stimuli to be
If the underlying cause of the lesion has resulted presented to both eyes simultaneously.
in pupillary dilatation, then fibers to the ciliary It is useful in:
body are also likely to be involved so that zz To investigate the potential for binocular
accommodation will be defective. function in the presence of a manifest squint
and is of particular value in assessing young (dysthyroid orbitopathy, entrapment of ocular

children (from age 3 years), who generally find contents after blowout fracture) exhibit restricted
the test process enjoyable. movements (sometimes termed a positive forced
zz It can also detect suppression and ARC. duction test). Some patients initially have pure
zz It can measure horizontal, vertical and nerve palsy, but contracture of the antagonist
torsional misalignments simultaneously and muscle results in secondary mechanical restriction
is valuable in determining surgical approach of movement. Suction cup devices have been
by assessing the different contributions in the developed for examiners who are wary of using
cardinal positions of gaze. toothed instruments at the limbus; a cotton swab
zz Hess chart: The affected eye will show a may be a sufficient tool in some patients.
markedly constricted field whereas the other
eye demonstrates overaction of its muscles. Active Force Generation Test
Left third nerve palsy—The area enclosed on the Active force generation testing may be used to
left chart is much smaller than that on the right. evaluate the ability of a muscle to move the eye
zz Left exotropia—note that the fixation spots against a resisting force. The forceps is placed at the
in the inner charts of both eyes are deviated limbus of the anesthetized (topical) globe in the
laterally. The deviation is greater on the right meridian of the muscle whose duction is limited
chart (when the left eye is fixating), indicating and the patient requested to rotate the eye in the
that secondary deviation exceeds the primary, direction of the limited duction; the examiner
typical of a paretic squint. judges through the forceps the relative amount of
zz Left chart shows underaction of all muscles force generated. Strain gauges have been devised
except the lateral rectus. that enable quantitation of this force. In case of
zz Right chart shows overaction of all muscles positive AFGT, strengthening surgical procedures
except the medial rectus and inferior rectus, can be done on involved paretic muscle (e.g.
the ‘yokes’ of the spared muscles. resection or plication). If negative the involved
zz In inferior rectus palsy, observing intorsion muscle should be left untouched.
on attempted depression can only assess
the function of the superior oblique muscle. Aberrant Regeneration
Observing a conjunctival landmark using the (Oculomotor Synkinesis)
slit lamp best performs this. Hess charting in
case of left third nerve palsy Change in the actions of muscles supplied by the
third nerve due to regrowth of damaged nerve
fibers following complete or severe third nerve
Forced Duction Test palsy. It is liable to occur when trauma, tumor or
The forced duction test is an attempt by the an aneurysm has caused the breach of endoneural
examiner to move a patient’s eye farther in a given sheath. It may occur from weeks to months
direction than the patient can move it. Topical after the onset of the III nerve paresis. Aberrant
anesthetic is placed on the appropriate limbal regeneration is known to involve pupil, LPS and to
location (generally 180° away from the duction other extraocular muscles.
limitation) with a small cotton swab and the
limbal conjunctiva is grasped firmly with a toothed INVESTIGATION
forceps. The patient is asked to rotate the eye fully
in the direction of the limited duction. An attempt zz Acute onset paralytic squint always warrants
is then made by the examiner to rotate the eye an immediate neuroimaging unless the
beyond the position attained by the patient while physician is certain about the diagnosis.
avoiding globe retraction. Care must be taken not zz Neuroimaging is indicated in following cases
to abrade the cornea. Patients who have pure nerve of acquired 3rd CN palsy—all cases with age
palsy exhibit no restriction to full movement by less than 50 years; age more than 50 years,
the examiner; patients who have pure restriction if patient has headache, nausea, vomiting,
other neurological signs and symptoms, zz For surgical purpose it can be divided into
involvement of pupil, ocular pain, proptosis, three groups
papilledema, loss of corneal sensation, history 1. Complete 3rd CN palsy—supra-maximal
of trauma, nonresolving or worsening cases LR recession, periosteal fixation of
and lastly a willing patient. LR, transposition of LR to MR, Scott
zz Remember, majority (around 60%) of cases procedure, Peter’s procedure (SO to MR).
care idiopathic and of presumed microvascular 2. Partial third CN palsy—MR resection
lesions in older patients. However, a wide- and LR recession (adjustable procedure
range of life-threatening causes (e.g. aneu for co-operative patient), vertical trans
rysm) can be there hence a low threshold position of SR and IR to MR, surgeries
should be adopted for neuroimaging. on SR and IR for correction of vertical
zz CT angiography is usually the preferred deviation.
modality by most physicians. Newer modalities 3. Aberrant regeneration to LPS—to do
such as MRI brain and orbits with venography surgery on the normal eye which takes the
can provide additional information. advantage of fixation duress for correction
zz Vascular risk factor assessment (ESR, CRP, of ptosis, and same side surgery may
CBC, blood sugar, lipid profile, blood pressure, worsen the same
homocysteine levels, etc.) similar to that for zz Scott procedure: Transposition of the insertion
retinal arterial disease should be done to rule of the SO tendon to a point anterior and medial
out microvascular causes. to the insertion of the SR muscle without
zz Supplementary investigation (e.g. lumbar trochleotomy combined with large recessions
puncture) may be required if a rare etiology of the LR muscle and, occasionally, recession-
such as infection (e.g. syphilis, Lyme disease) resection procedures of horizontal rectus
or vasculitis (including giant cell arteritis) is muscles of noninvolved eyes can result in a
suspected. satisfactory cosmetic outcome and alignment
in primary position in some cases.
MANAGEMENT
Observation Botulinum Toxin
zz Appropriate in presumed microvascular cases; Its role in the management of acute or chronic
Majority of case shows signs of recovery at 2 third-nerve paresis has not been adequately
weeks and recover by 3–6 months. investigated. Botulinum toxin injection into the
zz Temporary (e.g. Fresnel stick-on) prisms may uninvolved lateral rectus muscle is sometimes
be useful if the angle of deviation is small, but used to prevent its contracture when recovery time
uniocular occlusion may be necessary to avoid is prolonged.
diplopia if the ptosis component is partial or
recovering. Permanent Prism
zz Young children should be treated with It is used, as an alternative to surgery, to get rid of
alternate patching to prevent amblyopia. troublesome but mild residual deviation.
Surgical Treatment
VIVA QUESTIONS
zz Surgical treatment of the ocular motility
element and ptosis should be contemplated Q.1. What are the causes of isolated third
only after spontaneous improvement has nerve palsy?
ceased, usually not earlier than 6–12 months Ans. • M
icrovascular disease (ischemic
from onset. monon europathy) associated with
zz The aim is to provide binocular fusion in at least hypertension and diabetes is the
primary position and if possible in downgaze most common cause of third nerve
and to correct vision limiting or cosmetically palsy. Marked periorbital pain is often
annoying upper lid ptosis. associated with this.
• A neurysm of the posterior communicating such as syphilis, Lyme disease and

artery at its junction with the internal sarcoidosis, giant cell arteritis and
carotid [remember ischemic mono vasculitis associated with collagen
neuropathy is usually associated with vascular disorders.
pain in the periorbital region, and pupil Q.2. What are the causes of recurrent isolated
is often spared (75% of cases) inspite 3rd nerve palsy?
of significant restriction of motility.
Ans. Episodes of third nerve dysfunction with
In contrast, pain is variably present in
spontaneous recovery over 3–6 months can
aneurysm and pupillary involvement
occur in:
is almost always present inspite of less
• Ophthalmoplegic migraine
severe restriction of motility].
• Diabetes mellitus
• Trauma, both direct and secondary
• Aneurysm
to subdural hematoma with uncal
• Raised ICT.
herniation can produce isolated third
nerve palsy. Q.3. What are the causes and localization of
• M iscellaneous uncommon causes 3rd cranial nerve palsy?
include tumor, inflammatory disease Ans. Summarized in Table 1
Table 1 Causes and localization of third cranial nerve palsy

Level of lesion Etiology Manifestations
Nuclear Infarction • Paralysis of the contralateral SR
portion Hemorrhage • Bilateral ptosis
Neoplasm • Ipsilateral IR, MR, IO palsy
Abscess • Patients with damage to the oculomotor
nuclear complex need not have ipsilateral
pupillary dilation, but when involved, it
indicates dorsal rostral damage.
Fascicular Infarction • Lesions at this level can produce
midbrain Hemorrhage complete or incomplete palsies
portion Neoplasm • Lesion at the superior cerebellar
Abscess peduncle (Nothnagel’s syndrome)
presents ipsilateral 3rd nerve palsy and
cerebellar ataxia.
• Lesions at the red nucleus (Benedikt's
syndrome) are characterized by ipsilateral
third nerve palsy and contralateral
involuntary movement.
• Lesion at the cerebral peduncle (Weber’s
syndrome) produces ipsilateral third
nerve palsy and contralateral hemiplegia.
• Isolated dysfunction of either the
superior and inferior division
Fascicular Aneurysm • CN III palsy with fixed dilated pupil in case
subarachnoid Infectious meningitis—bacterial, fungal/ of surgical lesion
portion parasitic, viral • CN III palsy without pupil involvement in
Meningeal infiltrative case of medical disease
Carcinomatous/lymphomatous/leukemic
infiltration, granulomatous inflammation
(sarcoidosis, lymphomatoid granulomatosis,
Wegener granulomatosis)
Ophthalmoplegic migraine
Contd...
Contd...
Level of lesion Etiology Manifestations
Fascicular Tumor—Pituitary adenoma, meningioma, • Most commonly associated with other
cavernous craniopharyngioma, metastatic carcinoma cranial nerves dysfunctions.
sinus portion Pituitary apoplexy (infarction within existing • It presents as paresis of oculomotor,
pituitary adenoma) trochlear and abducens nerves with
Vascular associated maxillary division of trigeminal
Giant intracavernous aneurysm nerve, producing pain.
Carotid artery—cavernous sinus fistula
Carotid dural branch—cavernous sinus fistula
Cavernous sinus thrombosis
Ischemia from microvascular disease in vasa
nervosa
Inflammatory—Tolosa—Hunt syndrome
(idiopathic or granulomatous inflammation)
Fascicular Inflammatory—Orbital inflammatory • Lesions within the orbit are associated
orbital portion pseudotumor, orbital myositis with visual loss, ophthalmoplegia and
Endocrine (thyroid orbitopathy) proptosis.
Tumor (e.g., hemangioma, lymphangioma, • Third nerve ophthalmoplegia can be
meningioma) associated with trochlear and abducence
nerves palsies.
• It is important to remember that at the
orbit the oculomotor nerve divides into
superior (SR, LPS) and inferior division
(IR, MR, pupillomotor fiber). This can
cause partial oculomotor nerve palsies.
Q.4. What is the importance of pupil sparing sign of third nerve palsy, so mild pupillary
nerve palsy? signs may be clinically significant in such
Ans. • Pupillary involvement usually suggests cases. Lastly, however, pupil sparing in
an underlying ‘Surgical’ lesion such as children (unlike in adults) may not be
aneurysms, trauma and uncal herniation. helpful in differentiating the causes of
These lesions characteristically involve the palsy and third nerve.
the pupil by compressing the pial blood Q.5. What is the course of third cranial nerve?
vessels supplying the superficially loca Ans. Nucleus at the level of the superior colliculi
ted pupillary fibers (parasympathetic ventral to the Sylvian aqueduct.
fibers). ↓
• Pupillary sparing usually suggests an Fasciculus (efferent fibers) that pass from
underlying ‘Medical’ lesion such as the third nerve nucleus through the red
hypertension and diabetes. The micro nucleus and the medial aspect of the
angiopathy involves the vasa nervorum, cerebral peduncle.
causing ischemia of the main trunk of the ↓
nerve, leaving the superficial pupillary Basilar part starts as a series of ‘rootlets’ that
fibers intact. leave the midbrain on the medial aspect of
• However, it must be remembered excep the cerebral peduncle, before coalescing to
tions can be there. Pupillary involvement form the main trunk.
may develop a few days after the onset ↓
of diplopia in case of aneurysm as it Enters the cavernous sinus by piercing the
gradually expands. In few cases such as dura just lateral to the posterior clinoid
basal meningitis and uncal herniation process.
pupillary involvement may be the only Contd...
Contd... • S uperior rectus subnuclei are paired—

↓ each inner vates the respective
Superior orbital fissure contralateral superior rectus. Nuclear
↓ third nerve palsy will therefore spare the
Orbit ipsilateral, and affect the contralateral,
superior rectus.
↓
• M edial rectus, inferior rectus and
Two divisions
inferior oblique subnuclei are paired and
Superior division innervates the levator and
innervate their corresponding ipsilateral
superior rectus muscles.
muscles.
Inferior division innervates the medial
rectus, the inferior rectus and the inferior Q.7. What are the childhood causes of third
oblique muscles. nerve (oculomotor) palsy?
The branch to the inferior oblique also Ans. • Trauma; subdural hematoma
contains preganglionic parasympathetic • Neoplasm
fibers from the Edinger–Westphal sub
• Postoperative cause
nucleus, which innervate the sphincter
• Meningitis/encephalitis/viral or post-
pupillae and the ciliary muscle.
upper respiratory tract infection/
Q.6. Enumerate the subnuclei of third nerve Varicella-zoster virus
nucleus complex. • Aneurysm
Ans. • L
evator subnucleus is unpaired and • Orbital cellulitis
innervates both levator muscles. Lesions • Sinus disease
confined to this area will therefore give • Mesencephalic cyst
rise to bilateral ptosis. • Poison.
SIXTH CRANIAL NERVE PALSY

Shipra Singhi, Swati Phuljhale
The sixth nerve nucleus is located in the pons. The Chief Complaint
sixth nerve contains only somatic efferent fibers.
Runs a long course from the brainstem to the A case of sixth nerve palsy can present in following
lateral rectus muscle, through the superior orbital ways:
fissure and into the orbit through the middle part zz Inward deviation of eyes
of superior orbital fissure and then to lateral rectus. zz Diplopia in acquired cases
The VI cranial nerve supplies the lateral rectus zz Face turn on the same side amblyopia may be
muscle only. A lesion affecting the nerve will result present in congenital cases
in defective abduction of the eye. Sixth nerve has zz Patient may complain of headache, hearing
the longest subarachnoid course of all the cranial problem, depending on the location of
nerve, which makes it vulnerable to injury at many involvement of sixth nerve.
levels. Due to close association between sixth
nerve and seventh nerve (facial) in the brainstem,
there may be involvement of seventh nerve also in
some cases.1,2 Following points must be noted in history:
Age at Onset examine specifically for V, VII and VIII nerve to

locate the site of lesion, particularly, at the level of
Patient has history of deviation of eye inwards cerebellopontine angle. Moreover the fasciculus
and limitation of movement towards outwards. of VII nerve form a bend around the VI nerve
The congenital sixth nerve palsy is present since nucleus before emerging from the pons, and thus
birth. In acquired type the onset can be at any age, in a case of nuclear VI nerve palsy the VII nerve
depending upon the underlying cause. Inspection involvement is frequently seen.
of previous photographs may be useful for the
documentation of strabismus. Deviation may be Ocular Examination
large or small.
Visual Acuity
Mode of Onset This may be reduced if the affected eye fails to
Acute onset of diplopia or deviation warrants fixate properly due to the presence of a marked
immediate neuroimaging to rule out life- deviation. Amblyopia will develop due to presence
threatening conditions, hence, the type of onset is of convergent strabismus.
extremely important to note.
Abnormal Head Posture
Diplopia The face is turned towards the affected side.
Patient usually complains of horizontal diplopia,
which is characteristically worse in the distance Ocular Motility
and/or on lateral gaze and less or absent for near
zz Esotropia in the primary position (As shown in
fixation (Direction of the action of paralyzed
Figure 1, right eye sixth nerve party with right
muscle).
esotropia in primary gaze) due to relatively
unopposed action of the medial rectus and the
Head Posture
deviation (and symptomatic description) is
Face turn on the ipsilateral side is present. Face characteristically worse for distance than near
turn is seen in acquired cases. Face turn in fixation.
congenital cases may prevent amblyopia and zz Limitation of abduction is characteristically
maintain binocular single vision. on the side of the lesion (Fig. 2, showing
limitation of adduction in right eye in right
Past History sixth nerve palsy).
Any history of febrile disease such as viral or bacte Normal adduction of the affected eye is seen
rial, diabetes mellitus, raised intracranial tension (as shown in Figure 3 in case of right sixth nerve
(ICT), trauma or cerebral palsy must be asked palsy).
for. Adult: History of DM, hypertension, thyroid Lid: It is usually normal.
disease, other diseases (duration, treatm ent),
trauma, medication taken must be enquired for.

History of any previous neurosurgical procedure
must be recorded as it gives a clue about diagnosis.
EXAMINATION
The other cranial nerves and the peripheral
nervous system should be examined, if necessary Fig. 1: Esotropia of right eye in primary position in a
by an appropriate specialist. It is important to case of right sixth nerve palsy
cover test should be done with either eye fixing to

differentiate the primary (fixing with the normal
eye) and secondary deviation (fixing with affected
eye). In all paralytic strabismus, the secondary
deviation is always more than the primary
deviation.
Past pointing: Presence of past pointing indicates
recent onset. It can be tested by asking the patient
to point with his finger the object viewed by
the paretic eye (hand–eye coordination) with a
Fig. 2: Limitation of adduction in right eye in septum not allowing him to have a visual feedback
right sixth nerve palsy to correct the coordination due to paresis. In the
presence of paresis extrainnervation is required for
a movement in the direction of field of action of the
paretic muscle which is perceived by the brain as if
the object is located farther than it is, giving extra-
innervation to the hand for pointing. This causes
past pointing. For example, paretic right lateral
rectus palsy requires more innervation to fixate an
object in dextroversion causing past pointing.
Diplopia charting: The subjective deviation is
recorded by asking the subject to quantify the
separation between the double images, which are
dissociated by red-green glasses. This is repeated
Fig. 3: Normal adduction of the right eye in a case of
in all the nine diagnostic positions. In paralytic
right sixth nerve palsy
strabismus, the separation is maximal in the field of
action of the paretic muscle. Using a slit, horizontal
Conjunctiva: Conjunctival injection or chemosis for vertical strabismus and vertical for horizontal
can be present in cases of lymphoma and thyroid strabismus, one can also know the subjective
dysfunction. cyclotropia. Three points to be remembered are:
Cornea/Sclera/Iris: Usually normal. Corneal 1. Maximum separation is in the direction in
sensation may be reduced in acoustic neuroma. which the muscle acts most (field of action)
It is first sign in acoustic neuroma while first 2. The image that appears farthest belongs to the
symptom is hearing loss hearing loss. deviating eye.
3. The image is displaced in the direction of
Pupil: It is usually normal.
action of the paralyzed muscle.
IOP/Lens/Vitreous: Usually normal. In case of lateral rectus, palsy image shifts to
Fundus: Papilledema may be present due to raised outwards.
ICT. Binocular function: This is often retained in
the presence of an abnormal head posture. As
Special Tests the angle of deviation is often smaller for near
fixation, binocular function is usually present on
Cover test: An esodeviation is present that is
near fixation. In cases of head trauma, fusion may
often greater on distance testing. The test should
have been lost. Binocular function can be assessed
be carried out with and without an abnormal
with any of the following tests; Worth four dot
head posture, if one is present. It should be done
test, Bagolini’s striated glasses, Maddox rod and
in all nine gazes, at least in dextroversion and
synoptophore.
levoversion to look presence of incomitance. The
amount of deviation will always be more in the Hess charting: The affected eye will show a
direction of the involved muscle. Similarly, the markedly constricted field whereas the other eye
demonstrates overaction of its muscles. Following by paralysis of the lateral rectus muscle. And if
points must be remembered while interpreting: resistance is encountered it means that the FDT is
zz The smaller chart indicates the eye with the positive, mechanical restrictions do exist medially
paretic muscle. which may be due to contracture of the medial
zz The larger chart indicates the eye with the rectus muscle, conjunctiva, or Tenon’s capsule.
overacting yoke muscle. Important causes for medial rectus contracture
zz The smaller chart will show its greatest include thyroid eye disease, entrapment of
restriction in the direction of action of the medial contents after fracture, myositis, and cys
paretic muscle. ticercosis. Some patients initially have pure nerve
zz The larger chart will show its greatest palsy, but contracture of the antagonist muscle
expansion in the main direction of action of results in secondary mechanical restriction of
the yoke muscle. movement. FDT can be falsely negative if the
zz The degree of disparity between the plotted globe is not lifted out of the orbit while performing
point and the template in any position of gaze the test for recti muscle and if not depressed in
gives an estimate of the angle of deviation side the orbit while performing the test for oblique
(each square = 5°). muscles.
Force duction test (FDT): The FDT is done to Active force generation test (AFGT): AFGT may
look in mechanical restriction due to medial be used to evaluate the ability of a muscle to move
rectus. Under the topical anesthesia, the examiner the eye against a resisting force. After topical
passively moves the patient’s eye in the direction anesthesia, the paralytic muscle is held with the
opposite to that in which mechanical restriction fixation forceps and the patient is asked to look in
is suspected. For example, in a case right lateral the direction of the limited duction; the amount of
rectus limitation, there may be contracture of right force generated by the muscle is felt as a tug by the
medical rectus. After the topical anesthesia, the examiner. The test should be repeated in the other
medial limbal conjunctiva is grasped firmly with eye for the comparison of the forces.
a toothed forceps and the globe is lifted up from
the orbit. The patient is asked to look in abduction DIFFERENTIAL DIAGNOSIS
so that the medial rectus is relaxed. The examiner
then tries to passively move the eye in abduction. See Table 1.
Care must be taken not to abrade the cornea. If
examiner can successfully manage to move the MANAGEMENT
eye until the lateral limbus touches the lateral
canthus that means that there is no mechanical Investigations are tailored depending on
restriction and, the motility defect is clearly caused the suspected etiology. In an elderly patient
Table 1 Differential diagnosis of sixth nerve palsy

Diseases Features
Duane’s retraction syndrome Difficulty on abduction and adduction with eyelid retraction
Grave’s orbitopathy (TED) Proptosis + decreased ability of eye movement + diplopia
Orbital trauma Orbital fracture + Muscle swelling + eye restriction + diplopia
Infantile esotropia Esotropia + limit in abduction (improve after doll’s head maneuver) + IO
overaction + nystagmus + vertical deviation
Spasm of the near reflex Triad of intermittent : esotropia + accommodative spasm +Miosis
Myasthenia Gravis Muscle restriction, diplopia and ptosis
High myopia Can lead to progressive loss of abduction
investigations to rule out diabetes, hypertension, prismatic correction with a large deviation. It may
dyslipidemia, coronary artery disease, should be have to be repeated and it is rarely curative.
done. Surgery should be considered only when
The indications for neuroimaging are: adequate time has been allowed for maximal
zz VI nerve palsy along with involvement of other spontaneous improvement, typically at least 6–12
cranial nerves like V, VII, VIII is suggestive of months from onset.1,2 The aim of the surgery is to
lesion at brainstem and cerebello-pontine correct diplopia and head posture.
angle Partial palsy (paresis), if some force is felt on
zz VI nerve and presence of disc edema (Pseudo AFGT, then adjustable medial rectus recession and
localizing sign) lateral rectus resection in the affected eye can be
zz Isolated VI nerves palsy in patient with <60 yrs done.
of age Complete palsy is treated by transposition of
zz No improvement or if worsening seen within 6 the superior and inferior recti to positions above
weeks of onset in cases of ischemic nerve palsy and below the affected lateral rectus muscle.
zz Development of other neurological signs in This may or may not be coupled with weakening
patients with systemic risk factors. of the ipsilateral medial rectus depending on
FDT. If FDT is positive then one should recesses
Observation the medial rectus to release the globe. In such
cases where medial rectus recession is required,
Since most of the idiopathic and microvascular a partial tendon transposition is done instead of
lesions recover on their own it is advisable not to full tendon. Three rectus muscles should not be
jump to surgery immediately. In meantime the detached from the globe at the same procedure
aim is to avoid diplopia. because of the risk of anterior segment ischemia.
Occlusion: Use of frosted gasses in spectacles, Various types of transposition surgeries are
occluder patches for spectacles, pirate patches, described for the sixth nerve palsy; Hummelsheim
occluder contact lenses, etc. are the various (full tendon transposition of superior and inferior
methods employed for patching depending upon recti to lateral rectus), Jensen (tying of the inferior
the patient’s demands and comfort half the lateral rectus belly with the temporal half
Prismatic (e.g. temporary Fresnel stick-on) for of the inferior rectus belly and tying of the superior
correction of diplopia can be done is deviation is half of the lateral rectus to the temporal half of
small angle. Young children should be treated with superior rectus.
alternate patching to prevent amblyopia.1,2
Botulinum toxin injection into the ipsilateral INVESTIGATION
medial rectus may be used to prevent contracture,
assess residual function and sometimes to facilitate See Table 2 for investigations.
Table 2 Investigations in sixth nerve palsy

Forced duction test (FDT) For diagnosing the presence of mechanical restriction of ocular
motility
Force generation test (FGT) To assess muscle strength of an extraocular muscle
To differentiate paretic from restrictive strabismus
Magnetic resonance imaging (MRI) Provides greater resolution of the orbits, cavernous sinus, posterior
fossa, and cranial nerves
Cerebrospinal fluid (CSF) Help diagnose disease affecting the brain and spinal cord If vasculitis is
suspected clinically
Erythrocyte sedimentation rate and/ Help detect inflammation associated with conditions such
or C-reactive protein as infections, cancers, and autoimmune diseases
Contd...
VIVA QUESTIONS
↓
Intraorbital part enters orbit
Q.1. What is the course of sixth nerve?
↓
Ans. Nucleus at the mid-level of the pons, ventral
Lateral rectus muscle
to the floor of the fourth ventricle
↓ Q.2. What are the causes of sixth nerve palsy?
The fibers (fasciculus) leave the brainstem Ans. Causes of sixth nerve palsy are summarized
ventrally at the pontomedullary junction. in Table 3. The four most common causes
were idiopathic (26%), hypertension
↓
alone (19%), coexistent diabetes and
The basilar part of the nerve enters the
hypertension (12%), and trauma (12%).
prepontine basilar cistern, passes upwards
close to the base of the skull and is crossed Q.3. What is false localizing sign?
by the anterior inferior cerebellar artery. Ans. Raised intracranial pressure may cause
↓ stretching one or both sixth nerves due
Dorello canal (underneath the petroclinoid to their long intracranial course or the
ligament) compression against the petrous tip, in
↓ this situation sixth nerve palsy that may be
Intracavernous part intercavernous sinus bilateral, is a false localizing sign.
↓ Q.4. What is management of diplopia?
Superior orbital fissure Ans. Monocular occlusion or prismatic (e.g.
Contd... temporary Fresnel stick-on) correction of
Table 3 Causes of sixth nerve palsy

Causes Examples
Congenital • Following birth trauma
• Hereditary
• Infection (maternal)
• Failure of lateral rectus development
Acquired Children • Space-occupying lesions
• Infections, bacterial or viral
• Trauma
• Raised intracranial pressure
• Decompensated esophoria
• Infantile esotropia with cross-fixation
• Mobius syndrome
• Duane’s retraction syndrome
Young adults • Trauma
• Space-occupying lesions
• Post-viral inflammation
• Multiple sclerosis
• Diabetes
• High myopia
Older adults • Vascular
• Hypertension
• Diabetes
• Space-occupying lesions
• Senile lateral rectus weakness
Table 4 Total vs partial 6th nerve palsy

Tests Finding
Forced generation test Residual lateral rectus function—feel a tug on forceps
Botulinum toxin injection into a If abduction past midline—partial LR palsy
contracted medial rectus
Saccadic velocity analysis • Mild paresis—varies from 40 degrees per second
• Complete paralysis—slow lateral saccades (160 degrees per second)
Electromyography • Record action potential in LR muscle
• Increased signal in attempt to abduction→ residual LR function
diplopia is appropriate in idiopathic and motor neuron (LMN) facial nerve palsy is
presumed microvascular lesions; up to 90% also common. Isolated sixth nerve palsy is
will recover spontaneously, usually over never nuclear in origin.
weeks to several months. Young children Q.9. What is Foville syndrome?
should be treated with alternate patching Ans. Foville (inferior medial pontine) syndrome
to prevent amblyopia. is most frequently caused by vascular
Q.5. How will you do diplopia charting? disease or tumors involving the dorsal
Ans. See 3rd nerve palsy. pons. It is characterized by sixth nerve
Q.6. How will you do Hess/Lees charting? paresis, horizontal conjugate gaze palsy,
Ans. See 3rd nerve palsy. ipsilateral V, VII, VIII cranial nerve palsy
and ipsilateral Horner’s syndrome.
Q.7. What is relationship of sixth nerve with
other structures in cavernous sinus? Q.10. What is Millard–Gubler (ventral pontine)
Ans. The intracavernous section runs below the syndrome?
third and fourth, and the first division of Ans. Millard–Gubler (ventral pontine) syndrome
the fifth nerves. The sixth nerve is the most involves the fasciculus as it passes through
medially situated and runs through the the pyramidal tract and is most frequently
substance of the sinus in close relation to caused by vascular disease, tumors or
the internal carotid artery. Occasionally, demyelination. In addition to ipsilateral
intracavernous sixth nerve palsy is sixth nerve palsy, there is contralateral
accompanied by a postganglionic Horner hemiplegia and often an ipsilateral LMN
syndrome (Parkinson syndrome) due to facial nerve palsy.
damage to the paracarotid sympathetic Q.11. What is the muscle function test/How to
plexus. differentiate total or partial sixth nerve
Q.8. Can a patient present with isolated palsy?
nuclear sixth nerve palsy? Ans. See Table 4.
Ans. Nuclear sixth nerve presents as horizontal
gaze palsy, where there’s limitation of REFERENCES
abduction due sixth nerve nucleus involve 1. Walsh and Hoyt’s Clinical Neuro-Ophthal
ment as well as adduction limitation on mology. Philadelphia: Lippincott Williams &
contralateral side due to lack of impulse Wilkins, 2005.
to contralateral MLF. Convergence may 2. Azarmina M, Azarmina H. The six syndromes
be preserved in such cases, e.g. Facial of the sixth cranial nerve. J Ophthalmic Vis Res.
(seventh) nerve fibers wrap around the 2013;8(2):160-71.
sixth nerve nucleus, so ipsilateral lower
FOURTH CRANIAL NERVE PALSY

Swati Phuljhale, Shipra Singhi, Patil Mukesh Prakash
INTRODUCTION diplopia is worse in down gaze, it usually associated

with difficulty in climbing stairs or reading.
Fourth cranial nerve (CN) is the thinnest and Post-traumatic superior oblique palsy often
longest CN (75 mm) and the only CN that comes bilateral and these patients may complain of
out from the dorsal aspect of the brainstem. It is the torsional diplopia in downgaze
only CN, which crosses completely to the opposite
side. This originates from the contralateral nucleus. Past History
The IV cranial nerve supplies the contralateral
superior oblique muscle. Its nucleus lies at the Past history should include following:
level of the inferior colliculus. Any lesion affecting
zz Any history of febrile disease (viral or bacte
the nerve may result in difficulties of depression rial), diabetes mellitus, space-occupying
in adduction, incyclotorsion and abduction of lesions.
the eye. The acute onset of vertical diplopia in the
zz Since trauma is the most important cause of
absence of ptosis, combined with a characteristic fourth nerve palsy it should always be ruled
head posture, strongly suggests fourth nerve out, particularly blow to the dorsal aspect of
involvement. Peripheral lesions cause ipsilateral mid brain.
and nuclear lesions contralateral superior oblique
zz In adults history of diabetes mellitus, hyper
weakness. In postgraduate exams, it may come as tension, thyroid disease, and myasthenia
a long case. gravis, other diseases (duration, treatment),
trauma, and medication should be taken.
HISTORY Past Surgical History
Chief Complaint History of any previous neurosurgical procedure
must be recorded.
A case of fourth nerve palsy may present with
following:
zz Sudden hypertropia Birth History
zz Diplopia Birth history, including period of gestation, birth
zz Head posture weight and any problems in utero, with delivery or
zz Although amblyopia may be associated in in the neonatal period should be taken thoroughly.
congenital cases, it is rarely seen in presence
of a head posture. Family History
History of strabismus in the parents or siblings
History of Present Illness may be positive. It shows autosomal dominant
Following points must be recorded. inheritance.
Age at Onset EXAMINATION

It is important to note the age of onset (to differen General Examination/Specific Systemic
tiate between congenital and acquired form). Examination
Inspection of previous photographs may be useful
A thorough review of systems should be
for the documentation of head posture.
conducted, including enquiry about trauma,
diabetes, hypertension, etc. and symptoms viral
Diplopia illness. Neurological examination must include
Vertical diplopia which worse in down gaze is a other cranial nerves and the peripheral nervous
characteristic symptom in acquired cases. Since system.
Ocular Examination Pupil

Visual Acuity It is usually normal. It may be mydriatic in third
nerve palsy.
Vision may be reduced in congenital cases due to
amblyopia. Visual acuity must be checked with
IOP/Lens/Vitreous
and without glasses.
Usually normal.
Facial Asymmetry and Abnormal Head Posture
zz A compensatory head posture avoids diplopia. Special Examination of Squint
The functions of SO muscle include in torsion, Cover Test
depression and abduction. To compensate
for each of these there is a characteristic head All the strabismus examination should be
posture. For example, in a case of right SOP performed after correcting the compensatory head
the held tilt would be towards left shoulder to posture. A latent deviation exists if a compensatory
compensate for in torsion, chin depression is abnormal head posture is adopted. When the test is
present to compensate for depression action repeated with the head straight, the deviation will
and there will be face turn to same side to increase and may become manifest. The affected
compensate for abduction. In bilateral cases eye shows a hyperdeviation and an associated
since in torsion and abduction of both the esodeviation.
eyes is affected there’s no head tilt or face turn, There will be hypertropia of the affected eye
unless the involvement is asymmetrical. Facial which increases on opposite gaze and same side
asymmetry in form of (plagiocephaly) causes head tilt, also known as Park’s three step test.
congenital weakening of superior oblique
muscle. The premature fusion of coronal Park’s Three-step Test
axis on one side causes posterior placement The test is illustrated with the example of left
of trochlea, thus making superior oblique superior oblique paresis (SOP). It should be noted
muscle more parallel to the coronal than the that the test is only for identifying the paralyzed
sagittal axis (de-sagittalization). The posterior cyclovertical muscle.
placement of trochlea causes laxity of the SO Step 1: To assess which eye is hypertropic in the
tendon thus reducing all of its action. primary gaze (Fig. 1A).
zz Bilateral cases demonstrate chin depression In case of left hypertropia, the following
but there will be no face turn or head tilt unless muscles could be involved:
one side is affected more than the other. 1. Depressors of the left eye, i.e. superior oblique
zz Persistent abnormal head posture following and inferior rectus causing hypertropia of left
surgery for IV nerve palsy should be investi eye.
gated to exclude sternocleidomastoid 2. Elevators of the right eye, i.e. the superior
muscle tightness that may have developed in rectus or inferior oblique causing hypotropia
congenital cases. of right eye.
Lid Step 2: To assess which lateral direction has worse
hypertropia (Fig. 1B).
If there is ptosis, one must look for third nerve If the left hypertropia increases on right gaze
involvement. implicates a left superior oblique or right superior
rectus involvement.
Conjunctiva
Increase in the left gaze implicates that either
Conjunctival injection or chemosis can be present the right inferior oblique or left inferior rectus are
in cases of lymphoma and thyroid dysfunction. involved.
In this case, the deviation will be worse in
Cornea/Sclera/Iris opposite gaze, so now from four muscles we have
Usually normal, corneal sensation should be zeroed down to two muscles left SO or right SR
checked in all cases of CN palsy. deviation is Worse On Opposite Gaze (WOOG).
Bilateral involvement should always be

excluded, particularly following head trauma.
zz There is alternate hypertropia in adduction.
Right hypertropia in left gaze and left
hypertropia in right gaze, though orthophoria
may be present in primary position.
A zz Greater than 10° of cyclodeviation (measured
using double Maddox rods or by synoptophore)
and a tendency towards reversal of any hyper-
deviation or diplopic images on lateral gaze.
zz ‘V’ pattern esotropia is often present.
zz Bilaterally positive Bielschowsky head tilt test
The primary underaction of the affected
B
superior oblique muscle results in a number of
sequelae.
zz There is overaction of the contralateral inferior
rectus and of the ipsilateral inferior oblique
muscles.
zz The contralateral superior rectus shows
secondary underaction.
C
The combination of these muscle effects is
Figs 1A to C: Left superior oblique palsy: (A) Left variable and often depends on whether or not the
hypertropia. Four muscles could be involved; Left deviation is long standing.
superior oblique (LSO), left inferior rectus (LIR) or
right superior rectus (RSR), right inferior oblique (RIO);
Hess Charting
(B) Since the hypertropia worsens on right gaze; we
have zeroed down to 2 muscles; RSR or LSO; (C) On The affected eye will show a markedly constricted
head-tilt test, the hypertropia worsens on left tilt due field whereas the other eye demonstrates
to overaction of LSR muscle overaction of its muscles.
For example, recently acquired left fourth
Step 3: Also known as Bielschowsky’s head tilt test nerve palsy would have following abnormalities:
is done to assess, in which head tilt direction is the zz Left chart (chart of left eye with right eye fixing)
hypertropia worse (Fig. 1C) is smaller than the right (chart of right eye with
zz The head tilt test is performed with the patient left eye fixing).
fixating at a straight-ahead target at 3 meters. zz Left chart shows underaction of the superior
zz Increase in left hypertropia on left head oblique and overaction of the inferior oblique.
tilt implies the left superior oblique is zz Left chart shows overaction of the inferior
involved, and increase in right hypertropia rectus and underaction (inhibitional palsy) of
on left head tilt indicates the right superior the superior rectus.
rectus is involved. Since tilt on left side will
cause extorsion in right eye, which needs to Evaluation of Torsion
compensated by an intorsion of the eye to
Unilateral fourth nerve palsy is characterized
balance head movement. In addition, since
by less than 8 degree of cyclodeviation whilst
the SO is paralyzed the SR needs to overact
bilateral cases may have greater than 10 degree of
to bring about the desired intorsion, which
cyclodeviation.
is accompanied by elevation. This increased
elevation is seen as worsening of hypertropia Double Maddox rod test: It includes following steps
on same side head tilt. zz Red and green Maddox rods, with the cylinders
In fourth nerve, palsy the deviation is better on horizontal, are placed one in front of either
opposite tilt (boot). eye.
zz Each eye will therefore perceive vertical line of DIFFERENTIAL DIAGNOSIS

light.
zz In the presence of cyclodeviation, the line A case of fourth CN palsy has to be differentiated
perceived by the paretic eye will be tilted and from following:
therefore distinct from that of the other eye. zz Thyroid eye disease
zz One Maddox rod is then rotated until till both zz Ocular surgery
lines become parallel to each other zz Orbital fracture
zz The amount of rotation can be measured zz Neurosurgery
in degrees and indicates the amount of zz Childhood strabismus
cyclodeviation. zz Skew deviation
zz Third nerve palsy
Fundus—Indirect ophthalmoscopy and fundus
zz Myasthenia gravis
photography: Useful methods for evaluation of
zz Decompensated hyperphoria.
cyclodeviation. Normally the fovea is located
between the two horizontal lines, one passing
through the center of disc and the other cutting INVESTIGATION
the lower pole of the disc tangentially. The usual
location is in the middle of these two horizontal Blood investigations to rule out risk factors
lines, in cases of SOP because of extorsion it is like diabetes, hypertension, and other cause of
displaced downwards. microangiopathy should be done. Neuroimaging
is required only when it is associated with other
Field charting: Vertical displacement of blind spot
neurological signs.
suggests torsion.
Forced duction test (FDT): Exaggerated forced
MANAGEMENT
duction test was described by Guyton is done
to look for laxity on the SO tendon. The globe is Observation
grasped with Pierce Hoskins forceps in supero
temporal and inferonasal quadrant. The globe is Congenital decompensated and presumed micro
retracted inside the orbit and the eye is elevated vascular palsies commonly resolve spontaneously.
and adducted. The globe is rocked in intorsion All patients are seen every 1–2 months whilst being
and extorsion movement. A normal taut SO monitored for stability/recovery. Unilateral cases
tendon will cause globe to pop up during this of vascular origin usually recover within 6 months.
maneuver and a click is felt by the examiner. It is Small-unrecovered deviations with vertical/
always imperative to compare it with other eye to horizontal diplopia can be managed long-term by
differentiate between the normal and lax tendon. incorporating prisms in to spectacles. [Also, see
To test tension in the inferior oblique muscle—the chapter on sixth nerve palsy].
globe is grasped inferotemporal and superonasal
quadrant and is then retracted, depressed and Surgical Management
adducted.
The choice of surgical options is influenced by
Checking fourth cranial nerve function in third various factors:
palsy: Vertical actions cannot be tested, as there zz Laxity of SO tendon
is third CN involvement (adduction). To solve zz Presence of IO overaction
this, note a limbal or conjunctival landmark. Ask zz Amount of deviation in primary position
the patient to look down. The patient will not be zz Gaze of maximum deviation
able to look down as the eye is abducted and not Table 1 shows the algorithm for the manage
adducted. However, the eye should intort as the SO ment of superior oblique palsy according to
works. Check for the conjunctival landmark to see Knapp’s classification. To correct for torsion
if the eye is intorting. Harada-Ito procedure is done. For bilateral
If the conjunctival landmark is moving the eye acquired cases, perform bilateral Harado-Ito
is intorting, thus the fourth CN is intact. procedure is done to correct torsion.
Table 1 Management of IV nerve palsy (Von Noorden modification of Knapp’s classification): for a case
of left superior oblique palsy
Class Maximum deviation in gaze Management
1 Dextroelevation (L/R maximum in this gaze) Left inferior oblique recession
2 Dextrodepression Left superior oblique tuck or Harada-Ito’s procedure +
Right inferior rectus recession
3 All right gazes If hypertropia < 25 PD then left inferior oblique
recession
If hypertropia > 25 then add left superior oblique
tuck
4 All down and right gazes Treatment plan as in 3 with right inferior rectus
recession or left superior rectus recession
5 All down gazes Left superior oblique tuck with right inferior rectus
recession
6 Bilateral with a “V” pattern Bilateral surgery as in class 5
7 All downgazes, primary gaze and Explore trochlea
dextroversion

Trauma frequently causes bilateral fourth
VIVA QUESTIONS
nerve palsy.
Q.1. What is the course of fourth nerve?
Microvascular lesions are relatively
Ans. The nucleus is located at the level of the common.

Aneurysms and tumors are extremely rare.
inferior colliculi ventral to the Sylvian
aqueduct. Q.3. What is relationship of fourth nerve with
↓ other structures in cavernous sinus?
The fasciculus consists of axons that Ans. The intracavernous part runs in the lateral
curve posteriorly around the aqueduct wall of the sinus, inferior to the third nerve
and decussate completely in the anterior and above the first division of the fifth. In
medullary velum. the anterior part of the cavernous sinus,
↓ it rises and passes through the superior
The trunk leaves the brainstem on the orbital fissure above and lateral to the
dorsal surface enters the cavernous sinus. annulus of Zinn.
↓ Q.4. Why fourth nerve is so vulnerable to
Superior orbital fissure trauma?
↓ Ans. It is the thinnest and longest cranial nerve
Intraorbital part enters orbit (75 mm). It is the only CN that comes out
↓ from the dorsal aspect of the brainstem.
The intraorbital part innervates the superior Q.5. How would you differentiate between
oblique muscle. unilateral and bilateral SOP?
Ans. See Table 2.
Q.2. What are the causes of isolated fourth
nerve palsy? Q.6. How would you differentiate between
Ans. Idiopathic lesions are common, and many congenital and acquired SOP?
of these are thought to be congenital Ans. See Table 3.
although symptoms may not develop until Q.7. How would you differentiate between
decompensation occurs in adult life due to congenital vs ocular torticollis SOP?
reduced fusional ability. Ans. See Table 4.
Table 2 Differences between unilateral and bilateral superior oblique palsy

Investigations Unilateral Bilateral
Cover test Hyperdeviation in 1° position Often only slight hyperdeviation in 1°
Reflects extent of palsy position
Ocular motility No reversal of hypertropia and diplopia Reversal of hypertropia and diplopia
on lateral versions on lateral versions
Slight V pattern may be noted Large V pattern
Abnormal head posture Chin depression, head tilt and head turn Chin depression
Torsion Slightly extorsion Extorsion >100
Table 3 Differences between congenital vs acquired SOP

Parameters Congenital Acquired
History Long standing head tilt No long standing head tilt
No history of trauma history of acute trauma
Facial asymmetry Present Absent
Ocular motility No diplopia Diplopia +
Force duction test Lax superior oblique tendon No lax superior oblique tendon
Vertical fusional reserve >40 prism diopters <20 prism diopters
Torsion (Double Maddox rod test) No extorsion Extorsion<150
Table 4 Congenital vs ocular torticollis

Congenital Acquired
Onset at birth Rarely before 18 months
Passive straightening of head difficult Easy passive straightening
Neck muscles firm Palpation negative
No visual disturbances Diplopia is frequent
Tilt not affected by occlusion Generally straightens on occluding the paralytic eye
Q.8. How would you do Parks–Bielschowsky BIBLIOGRAPHY

three-step tests?
Ans. Given in examination. 1. Bagheri A, Fallahi MR, Abrishami M, Salour H,
Aletaha M. Clinical features and outcomes of
Q.9. How do you localize the lesion in case of treatment for fourth nerve palsy. J Ophthalmic
fourth nerve palsy? Vis Res. 2010;5(1):27-31.
Ans. Fourth nerve palsy may be due to lesions 2. Borchert MS. Principles and techniques of the
in the nucleus or fascicular lesions of the examination of ocular motility and alignment.
midbrain. It can be difficult to differentially In: Miller NR, Newman NJ (Eds). Clinical
diagnose nuclear and fascicular lesions Neuro-Ophthalmology, 6th edn. Philadelphia:
as the IV nerves decussate immediately Lippincott Williams & Wilkins; 2005. pp.
after exiting the nuclei and exit the dorsal 887–906.
midbrain after a very short intra-midbrain 3. Von Noorden GK, Campos EC. Binocular Vision
course. The IV nerve is not only susceptible and Ocular Motility: Theory and Management
to damage as it exits the midbrain but also of Strabismus, 6th edn. Mosby: St Louis, MO,
is vulnerable in the cavernous sinus and USA; 2002.
orbital apex.
OPTIC NEURITIS
Ritu Nagpal, Adarsh Shashni
INTRODUCTION zz Flashes of light induced by noise, smell, taste,

touch (photism)
Optic neuritis (ON) is the term used for the zz Diplopia, nystagmus, dysarthria, dysphagia
inflammation of optic nerve. It is also called papillitis zz Electric shock like sensation on neck move
(when the head of the optic nerve is involved) and ment (Uhthoff phenomenon)
retrobulbar neuritis (when the posterior of the zz Sudden worsening of vision on exercise or
nerve is involved). It is caused by many different increase in body temperature (Lhermitte sign)
conditions, and it may lead to complete or partial zz Pulfrich phenomenon, in which anomalous
loss of vision. The most common cause is acute perception of the direction of movement of an
demyelinating optic neuritis often associated with object occurs due to asymmetry of conduction
multiple sclerosis (MS).1-3 velocity in the optic nerves
In exams, ON can be given as both long and zz Weakness and stiffness of lower limbs
short case. zz Numbness and paresthesias
zz Sphincter disturbances.
HISTORY
Chief Complaint History of Present Illness
Usual history is a young adult patient, typically The onset and progression shows a characteristic
less than 45 years of age, (but may be of any age) pattern in a typical case of optic neuritis. Visual loss
presents with unilateral vision loss developing is acute or subacute, varies from mild reduction to
over a period of several hours to few days. no perception of light and progresses over 7–10
days before reaching a nadir. By 2–3 weeks the
This may be associated with: visual acuity starts improving and almost complete
zz Periocular pain (seen in almost 53–92% of recovery occurs by 4–5 weeks of onset.
cases), especially with eye movement that may The pain is typically dull aching type
precede or coincide with visual loss. exacerbated by touching or moving the eye. It
zz Reduced contrast and color vision—loss of reaches maximal severity within 24–36 h and
color and contrast at times out of proportion spontaneously abates within 48–72 h in few cases
to loss of visual acuity strongly suggests optic it may persist for a long time and atypical causes of
nerve pathology. Abnormal color vision by ON must be ruled out in such cases.
Ishihara plates was found in 88% of involved
eyes in the optic neuritis treatment trial Past History
(ONTT). Many a times patient may complain of Similar episodes in past may be there. In the ONTT,
color desaturation refers to a qualitative inter- the recurrence rate was 35% within 10 years.
eye difference in color perception that can Subgroup analysis showed a higher recurrence
be tested by comparing vision of a red object rate (48%) in patients with MS compared to those
with each eye. A patient with monocular “red without MS (24%).1,2
desaturation” may report that the red color
appears “washed out,” pink, or orange when Past Surgical History
viewed with the affected eye. It is important to
remember that problems of color vision can History of prior surgery; such as shunt surgery,
persist even after complete clinical resolution. optic nerve fenestration, cardiac or any spinal
Few symptoms when present strongly points surgery must be recorded carefully.
toward an association with multiple sclerosis such
as: Past Medical History
zz Bright flashes of light with movement of A careful systemic history is required to rule out other
affected eye (phosphenes) causes of optic nerve dysfunction such as NAION.
Hypertension, diabetes mellitus, hyperlipidemia, Posterior Segment

collagen vascular disease, antiphospholipid
(Slit lamp biomicroscopic examination using a
antibody syndrome, erectile dysfunction, hypo
90D/78D lens and indirect ophthalmoscopy)
tension, hyperhomocysteinemia, sleep apnea zz Vitreous—in a typical case of ON vitreous is
syndrome, previously diagnosed multiple sclerosis
usually normal.
and any previous cerebrovascular accident must be zz Optic disc
noted carefully. Prior history of peptic ulceration —— Papillitis with hyperemia and swelling of
and tuberculosis is important especially when IV
the disk (Fig. 1), blurring of disk margins,
steroid therapy is planned.
and distended veins (Fig. 2) is seen in
one-third of patients with optic neuritis.
EXAMINATION —— Two-thirds of these patients have retro
bulbar neuritis with a normal funduscopic

Systemic Examination examination.
—— It must be remembered that the disc
A complete neurological work-up is a must in all
cases of optic neuritis. swelling of demyelinating ON is diffuse
and presence of any segmental changes,
Ocular Examination
Visual Acuity
Uncorrected as well corrected visual acuity (VA)
must be recorded in all cases. VA on presentation
and VA of the other eye often guides the initial treat
ment. Serial VA monitoring helps in differentiating
typical from atypical form of optic neuritis. It must
be remembered VA in optic neuropathies but does
not correlate perfectly with the extent of optic
nerve dysfunction, so it is a somewhat insensitive
measure.
Eyeball
Usually normal. However, nystagmus and ocular Fig. 1: Papillitis with hyperemia and swelling of
motor nerve palsies may be seen in some cases. the disk
Lids, Conjunctiva, Cornea

Usually normal.
Pupils
Presence of relative afferent pupillary defect
(RAPD) is an important sign of optic nerve dys
function. It must be remembered that RAPD is a
nonspecific sign of optic neuropathy. In bilateral
cases, or in cases with a pre-existing optic
neuropathy in the fellow eye, an RAPD may not
be apparent.
Anterior Segment/IOP
Fig. 2: Papillitis with blurring of disk margins, and
Usually normal. distended veins
altitudinal swelling, pallor, arterial —— This test is not specific for diagnosis of
attenuation, and splinter hemorrhages acute optic neuritis.
suggests some alternate diagnosis. —— In ONTT 67% cases had fellow eye
—— Papillitis is more common in children abnormalities.

less than 14 years old and in certain zz Magnetic resonance imaging (MRI)
ethnic populations, including black South —— MRI of the brain and orbits with gado
Africans and Southeast Asians. linium contrast provides confirmation of

—— Peripapillary hemorrhages are rare in the diagnosis of acute demyelinating optic
optic neuritis, but are a common accom neuritis. MRI with FLAIR (fluid attenuated
paniment to papillitis due to anterior inversion recovery) sequencing and
ischemic optic neuropathy Normal or gadolinium infusion and fat suppression
edematous. when available is the preferred modality.
zz Macula: A typical case on ON may not show —— Usual finding shows white matter
any abnormality. Presence edema, exudates or abnormalities characteristic of MS such

star formation suggests atypical nature of the ovoid periventricular (>3 mm) and
disease. corpus callosum plaques with long axis
zz Perivenous sheathing or periphlebitis retinae perpendicular to the ventricular margins
can be seen in about 12% of patients with ON dark on T2 and hyperintense on T1. The
and implies a high risk for MS. longitudinal extent of nerve involvement
zz Examination of fellow eye is important. Disc as seen on MRI correlates with visual
pallor (commonly temporal disc pallor which impairment at presentation and with
may extend beyond the disc margin into visual prognosis.
—— The reported prevalence of white matter
adjacent RNFL) in the fellow eye suggests
previous optic neuritis. abnormalities varies from 23% to 75%.
In the ONTT, almost 40% of patients had
INVESTIGATIONS MRI lesions.
—— It also provides important prognostic
The diagnosis of ON is usually made on clinical information regarding the risk of

grounds. developing MS. Individuals with white
zz Color vision and contrast sensitivity matter abnormalities are at a higher risk
—— Decreased color vision and contrast of developing MS.
sensitivity are highly characteristic of zz Lumbar puncture
optic neuritis. —— Lumbar puncture is not an essential
—— Poor color vision, particularly out of diagnostic test in optic neuritis, but
proportion to loss of acuity, is a very should be considered in atypical cases.
sensitive indicator of optic neuropathy —— Abnormality is seen in >90% cases. It
zz Visual field shows leucocytosis, raised IgG levels,

—— The most common field defect seen in raised IgG/albumin index and oligoclonal
demyelinating optic neuritis is diffuse IgG bands.
depression of sensitivity in the entire zz Optical coherence tomography (OCT): OCT
central 30° followed by altitudinal/ shows RNFL thinning in most (85%) of patients
arcuate defects and focal central/centro with optic neuritis. However, it does not have
cecal scotomas. any diagnostic value since these abnormalities
zz Visual evoked potential (VEP) are also common in patients with MS who do
—— Decrease in amplitude and prolongation not have a clinical history of optic neuritis.
of latency can be seen. A delay in the P100 zz Additional investigations: To be done in the
of the visual evoked response (VER) is presence of atypical features
the electrophysiologic manifestation of —— Complete hemogram with ESR and CRP
slowed conduction in the optic nerve as a —— Antinuclear antibody, anti-ds-DNA
result of axonal demyelination. antibody

—— Mantoux test zz Central retinal vein occlusion (CRVO)

—— IFN-g release assay (Quantiferon TB Gold) —— Sudden onset of visual blurring with
—— Chest X-ray severity depending on the type of CRVO;

—— Gallium scan ischemic or nonischemic
—— S. ACE levels —— Dilated and tortuous vessels with dot/blot
—— Serum and CSF VDRL and FTABs and flame hemorrhages in all 4 quadrants,
—— S. NMO IgG levels most numerous in the periphery
—— Spinal MRI —— Cotton wool spots, optic disc and macular
—— Anti-Lymes antibody titer edema

—— Mitochondrial gene mutation (11778 and —— FFA–delayed A-V transit time, blockage by
14484). hemorrhages
—— Disc collaterals, epiretinal gliosis and
DIFFERENTIAL DIAGNOSIS pigmentary changes at macula in chronic

stage.
The differential diagnoses for unilateral sudden zz Leber’s hereditary optic neuropathy
decrease in vision include following:1-3 —— Acute or subacute painless loss of central
zz Optic neuritis vision

—— Sudden onset vision loss with subsequent —— Sequential involvement of the fellow eye
improvement (visual acuity usually within weeks or months

between 6/18-6/60) —— Disc hyperemia with obscured margins
—— Cellular reaction in the vitreous overlying —— Dilated and tortuous posterior pole
the optic disc and presence of retinal vasculature (telangiectatic microangio-

exudates suggestive of papillitis pathy)
—— Normal appearing optic disc in retro —— Swelling of the peripapillary nerve fiber
bulbar neuritis layer (pseudoedema)

—— FFA—absence of dye leak
—— Central visual loss (central scotoma).
—— Central or centrocecal scotoma
zz Ischemic optic neuropathy
—— Usually poor prognosis.
—— Sudden onset vision loss with no or
incomplete improvement
—— Chalky white edematous disc with CLASSIFICATION OF OPTIC NEURITIS
overlying hemorrhages
—— Sectoral disc edema
Optic neuritis may be classified either on the basis
—— Nerve fiber bundle–type field defects
of ophthalmoscopic findings or on the basis of
underlying etiology.
(originating from and involving the
physiologic blind spot) typically respect
ing the horizontal meridian. Ophthalmoscopic Classification
zz Compressive optic neuropathy zz Retrobulbar neuritis—characterized by
—— Slowly progressive vision loss
normally appearing optic nerve head. This is
—— Chronic disc edema with optociliary
the most common type seen in adults and is
shunt vessels frequently associated with multiple sclerosis.
—— Presence of pseudodrusen zz Papillitis—characterized by hyperemia and
—— Central visual loss (central scotoma)
edema of the optic disc with or without
zz Papillophlebitis peripapillary flame-shaped hemorrhages and
—— Mild vision loss (6/12 or better) cells in the posterior vitreous. This is the most
—— Slow progression compared to optic frequently encountered in children.
neuritis or NAION zz Neuroretinitis—characterized by papillitis
—— Mild disc swelling associated with inflammation of the nerve
—— Disc surface telangiectasia fiber layer and a macular star development.
—— Spontaneously resolves. This is the least common type of optic neuritis.
Table 1 Difference between typical and atypical optic neuritis

Feature Typical optic neuritis Atypical optic neuritis
Onset Acute Chronic
Age Young patient (mean age in ONTT was 32 years) Older patient
Sex Caucasian female Male
Laterality Typically unilateral Bilateral simultaneous or rapidly
sequential
Pain > 90% have pain with eye movements. This helps Lack of pain or protracted pain
to differentiate optic neuritis from NAION
Optic disc Appears normal in 2/3rd cases Significant papillitis
Hemorrhage Usually not seen Marked hemorrhages
Exudates Uncommon Macular star may be seen
Uveitis Rare Pars planitis, choroiditis, phlebitis
Course Vision worsens over several days to 2 weeks and Lack of improvement or progression
then begins to improve. About 75% patients recover
visual acuity of 6/9 or better
Etiological Classification zz Patients with severe bilateral visual loss

zz Poor vision in the fellow eye.
zz Demyelinating—the most common type
Regimen: Intravenous methyl prednisolone
zz Parainfectious—usually occurs following a
1g daily for 3 days followed by oral prednisolone
viral infection or immunization
(1 mg/kg/day) for 11 days and then tapered over
zz Infectious—occurs due to sinus infections or
3 days. Oral corticosteroids were associated with
in association with cat scratch fever, syphilis,
an increased risk of recurrence of optic neuritis
lyme disease, cryptococcal meningitis in in ONTT hence this form of therapy should be
patients with AIDS and herpes zoster avoided.
zz Noninfectious—occurs in association with
systemic causes such as sarcoidosis, systemic
lupus erythematosus, polyarteritis nodosa and
VIVA QUESTIONS
other forms of vasculitis. Q.1. What are the signs of optic nerve
dysfunction?
Depending upon Course of the Disease Ans. Signs of optic nerve dysfunction include:
• Reduced visual acuity for distance and near
Typical and atypical (see Table 1). • Afferent pupillary defect
• Dyschromatopsia mainly affecting red and
TREATMENT green
• Diminished light brightness sensitivity
Acute Therapeutic Options for Optic Neuritis • Diminished contrast sensitivity
• V isual field defects depending on the
Routine treatment of typical demyelinating ON
underlying pathology.
with corticosteroids is not advised due to lack
of long-term benefit and the potential for side- Q.2. How do you differentiate typical optic
effects.1-3 There are specific situations where neuritis from atypical optic neuritis?
corticosteroids may be offered to shorten the Ans. See Table 1.
period of functional impairment. Corticosteroids, Q.3. What were the results of optic neuritis
therefore, are considered for following patients: treatment trial (ONTT)?
zz Who require faster recovery, such as occupa Ans. • T he objectives of the study were to
tional requirements evaluate the efficacy of corticosteroid for
the treatment of acute optic neuritis and established MS optic neuritis occurs in 50%
to investigate the relationship between cases at some point of time. The overall
optic neuritis and multiple sclerosis. 10 years risk of developing MS following an
• Patients were randomized into three acute episode of optic neuritis with one or
arms: placebo, oral (low-dose) predni more characteristic brain lesions, normal
sone (1 mg/kg/day for 14 days) and high- brain MRI is 56% whereas it is 22% if the
dose intravenous methyl prednisolone MRI is normal. At 15 years the risk increases
(250 mg 4 times daily for 3 days), followed to 25% in patients with normal baseline
by oral prednisone (1 mg/kg/day for MRI and 70% in patients with abnormal
11 days). baseline MRI.1-3
• At 6 months, color vision and contrast Q.5. What is the role of brain MRI?
sensitivity and visual fields were found Ans. The likelihood of progression of optic
to be significantly better in the methyl neuritis to MS is best predicted by brain
prednisolone arm; however, after 1 MRI done at the time of diagnosis. In the
year, there was no significant difference ONTT baseline, brain MRI was found to be
between treated and untreated patients the most powerful predictor of likelihood
in any of the functional outcome. of clinically definite multiple sclerosis
• Intravenous methyl prednisolone was (CDMS).
found to accelerate the rate of visual
recovery over the first 15 days. By day 30 Q.6. What are the low risk factors for MS?
nearly complete recovery occurred in all Ans. Lack of pain, severe disc edema, peri
patients. papillary hemorrhage, retinal exudates and
• No significant difference was found mild visual acuity loss.
between oral prednisolone and placebo Q.7. What are the diagnostic criteria for
in any of the parameter. At 12 months, diagnosis of MS?
all three groups were similar in terms of Ans. Mc Donald criteria: Clinical history and
visual functions. presentation in the presence of neuro
• In a subsequent analysis, patients rando imaging abnormalities with or without CSF
mized to receive treatment with high- abnormalities or abnormal VEP response.
dose intravenous methylprednisolone Recurrent optic neuritis in the absence of
in conjunction with 11-day low-dose other clinical or laboratory manifestations
oral prednisone taper exhibited a is not sufficient for diagnosis.
significantly reduced risk of developing Q.8. What is neuromyelitis optica?
clinically definite MS (defined by Ans. Neuromyelitis optica or Devic’s disease, is
the development of new neurologic characterized by necrotizing demyelinating
symptoms attributable to demyelination lesions of bilateral optic nerves and spinal
other than optic neuritis in either eye cord. The spinal lesions extend contiguously
occurring at least 4 weeks after the over three of more vertebral segments.
study entry and lasting more than 24 Serum antibody, NMO-IgG, which targets
hours with abnormality documented the autoantigen aquaporin-4, is a useful
on neurological examination) over the marker for diagnosis. Treatment with
subsequent 2 years. Beyond 2 years no rituximab has been tried to be beneficial.
significant disease-modifying effects of
steroids was seen. Q.9. What are the treatment modalities avail
able for acute optic neuritis apart from
Q.4. What is the overall risk of developing corticosteroids?
multiple sclerosis in a patient presenting Ans. Treatment modalities available for acute
with optic neuritis? optic neuritis are described below:4,5
Ans. Approximately 15–20% of MS patients • Short-acting agents (for acute exacer
present with optic neuritis. In patients with bations): High dose intravenous steroids.
• Longer-acting agents (delay the develop thickness of peripapillary retinal

ment of CDMS): nerve fiber layer.
– Disease modifying drugs: Interferon • Teriflunomide
β-1a and 1b, glatiramer acetate • Adrenocorticotropic hormone
– Immunosuppressives: Mitoxantrone, • Dimethyl fumarate
Natalizumab, Fingolimod • Antibody against LINGO1 (anti-LINGO),
• Newer treatment options a CNS protein that acts as a negative regu
– Intravenous immunoglobulins (IVIg): lator of oligodendrocyte precursor differ
have been tried for acute optic entiation to promote CNS remyelination
neuritis but with no long-term effects • Phenytoin.
on visual function or on the latency of Q.10. What are the newer investigative modali
VEP responses after AON. ties available for the management of
– Plasma exchange (PLEX): PLEX acute optic neuritis?
has demonstrated efficacy in the Ans. Various investigative modalities are as
treatment of refractory AON and in follows:
AON associated with neuromyelitis
• O CT and SLP: To demonstrate RNFL
optica (NMO). The addition of PLEX
edema which is evident in approximately
to intravenous methylprednisolone
80% of the affected optic nerves. On an
in the acute treatment of NMO-
average, patients with AON lose 22 μm
associated AON has showed signi
more RNFL in their affected eye than in
ficant improvement in high-contrast
their unaffected eye at 3–6 months after
acuity, visual fields and temporal
the inception of visual symptoms.
retinal nerve fiber layer (RNFL)
• Diffusion tensor imaging (DTI): Provides
thickness, but not low-contrast letter
a sensitive modality to complement
scores or color vision. The early, first-
RNFL structural injury in the evaluation
line use of PLEX in the treatment of
of acute ON injury.
AON is yet to be evaluated. PLEX is
• Electrophysiology: Prolongation of the
presumed to mediate a therapeutic
VEP P100 latency is used as a measure
effect, at least in part, through the
of conduction delay through the optic
removal of pathogenic humoral and
nerve and is a sensitive marker of
plasma factors.
demyelination. Reduction in the ampli
– Erythropoietin: Systemic infusion
tude of VEP serves as a measure of axonal
of erythropoietin with and without
injury. The sensitivity of mfVEP is further
methylprednisolone has demons
trated beneficial effects on retinal enhanced by using low-contrast pattern-
ganglion cell (RGC) function and reversal stimuli allowing detection of
survival in a rat model of experimental mild residual injury or occult damage
autoimmune encep halomyelitis. in the so-called ‘unaffected’ eye. The
Er ythrop oietin administration traditional ERG with optical nerve
increases protein levels of various head component (ONHC) waveform—
antia poptotic factors such as The ONHC waveform represents the
phospho-Akt, phospho-MAPK 1 and transformation of slow membrane
2 and Bcl-2 which helps to limit the conduction to fast salutatory conduction,
apoptosis of retinal ganglion cells after as axons traverse the lamina cribrosa
AON. Erythropoietin administration and become myelinated. After AON, the
has shown partial recovery of pattern- ONHC waveforms are abolished and
reversal VEPs and improvement later recover, representing the transient
in flash electroretinograms and effects of conduction block due to
significant improvement in the reversible demyelination. Eyes with
previous optic neuritis in patients with REFERENCES

MS exhibit changes or loss in the ONHC
1. Bowling B. Kanski’s Clinical ophthalmology:
waveform that correlate with reduction
in low-contrast letter acuity, retinal Elsevier; 2015.
nerve fiber layer (RNFL) thickness, visual 2. Albert DM, Miller JW, Azar DT. Albert
field depression and amplitude loss and and Jakobiec’s principles and practice of
latency delay on mfVEP. ophthalmology; 2008.
• Biomarkers: Serum and plasma neuro 3. Toosy AT, Mason DF, Miller DH. Optic neuritis.
filament levels, heavy (NfH) and light Lancet Neurol. 2014;13(1):83-99.
(NfL), are elevated in patients with 4. Bennett JL, Nickerson M, Costello F, et al.
Re-evaluating the treatment of acute optic
ON, independent of the inflammatory
neuritis. J Neurol Neurosurg Psychiatry. 2015;
mechanism. The levels of NfH and NfL
86(7):799-808.
have been observed to correlate with 5. Petzold A, Wattjes MP, Costello F, et al. The
the extent of vision loss and the loss of investigation of acute optic neuritis: a review
retinal nerve fiber thickness following and proposed protocol. Nat Rev Neurol. 2014;
ON. 10(8):447-58.
ESODEVIATION
Shipra Singhi, Ritika Mukhija, Adarsh Shashni
INTRODUCTION zz The earlier the onset, the more likely the need
for surgical correction. The later the onset, the
Esodeviations are the most common type of greater is the likelihood of an accommodative
ocular misalignment. It represents 50% of ocular component (mostly arising between 18 and 36
deviations in pediatric age group. In exams, it is months).
given as long case. zz The longer the duration of squint in early
childhood the greater the risk of amblyopia,
HISTORY unless fixation is freely alternating.
Chief Complaint zz Inspection of previous photographs may be
useful for the documentation of strabismus.
zz Deviation of eyes zz History of head posture, chin position or face
zz Diminution of vision turn may be there.
zz Head posture.
Past History
Previous ocular history including refractive
zz Following points must be recorded in history:
prescription and compliance with spectacles or
—— Age at onset
occlusion, previous surgery or prisms is important
—— Constant or intermittent
to decide upon the treatment options and
—— Unilateral or bilateral
prognosis.
—— Progression
—— Abnormal movement of the eyes
—— Preceding history of febrile illness,

Birth History
meningitis, or any neurological events Including period of gestation, birth weight and
—— Exacerbating and ameliorating factors any problems in utero, type of delivery (normal or
zz Patient has history of deviation of eyes that can forceps) is important. Following factors increases
be present since birth or after some time and the risk of ET in a child:
can be associated with diminution of vision. zz Maternal smoking during pregnancy
zz Maternal age (>30 years) zz Placement of the corneal light reflexes is

zz LBW (low birth weight) baby observed (more or less centered in the pupil
zz Retinopathy of prematurity and Down’s of the fixating eye, but will be decentered in a
syndrome. squinting eye, in the direction opposite to that
of the deviation).
Family History zz The distance of the corneal light reflection
from the center of the pupil is noted. Each
Note down any history of strabismus in the parents
millimeter of light displacement across the
or siblings. There is 73% concordance rate of
cornea is equivalent to 7° of decentration or
esotropia in monozygotic twins, as compared to
14Δ.
35% in dizygotic twins.
zz Alight reflection at the pupillary border
signifies a 15° or 30Δ deviation, at the mid-iris
EXAMINATION a deviation of 30° or 60Δ, and at the limbus a
General Examination deviation of 45° or 90Δ.
zz The Hirschberg method relies on a pupil size
A careful examination especially focusing on of 4 mm and performing in a case with dilated
development of the child and the neurological
pupil is not reliable.
system must be carried out. zz It provides rough estimate of the angle of
strabismus in cases where the patient may not
Local Examination allow prisms to be placed in front of the eyes.
Visual Acuity Krimsky test
zz Visual acuity chart (for elderly children) zz Preferred over the Hirschberg test as it allows a
zz VER (< 6 months). more exact estimate of the alignment.
zz However, it requires a cooperative patient.
Fixation preference tests (for young kids) zz Corneal reflex assessment is combined with
zz Central, steady and maintained (CSM)
prisms to measure the angle in a manifest
zz Fixation preference: Alternation
deviation.
zz Maintenance of fixation: 15 secs zz A neutralizing prism is placed (A prism bar
zz 10 pd BD/BU prism test
starting with a relatively low prism power) over
zz 25 pd BI alternation test
either eye to position the corneal light reflex in
zz Cross fixation: Tracking past midline.
its normal position.
zz Generally, the prism is placed over the fixing
Measurement of Alignment eye to improve visualization of the light reflex
Light reflex tests in the deviating eye. Few authors call it prism
zz Generally used to assess deviations in small reflection test when the prism is placed in front
children and in patients who cannot fixate of the deviating eye until the corneal light
with either or both eyes. reflections are symmetrical.
zz Require minimal cooperation from the patient
Bruckner test
zz Drawback: Assess only tropias and less accu zz Done with the help of a direct ophthalmoscope
rate than cover testing. and watching the red reflex.
zz In cases where the goal of surgery is improved zz If the red reflex coming from one eye is different
appearance and an angle kappa is present, from the other, strabismus is suspected. The
light reflex tests (especially the Krimsky test) deviating eye gives a brighter reflex.
are essential as they are likely to be the best zz Easy to perform, but it does require some
choice for determining surgical dosages. expertise.
Hirschberg test zz Generally used for screening purposes only. It
zz A pen torch is shone into the eyes from arm’s is an excellent test to screen a preverbal child
length and the patient asked to fixate the light. for strabismus, anisometropia or amblyopia.
Cover-uncover test: The gold standard for evaluat Prism Bar cover test
ing strabismus is the cover-uncover test. It can zz The prism cover test measures the angle of
diagnose both the tropia and phoria components. deviation on near or distance fixation and in
When performed with prisms, it can be quantitative any gaze position.
also. It has to be done for both near and distance and zz The procedure is similar to the alternate cover
with and without refractive correction. The fixation test, but prisms of increasing power are placed
distance should be 33 cm for near and 6 meters for in front of one of the eyes until the eyes stop
distance. For distance fixation target is given of a shifting.
figure or letter size of 6/9 of Snellen’s chart. zz The apex of the prism is towards the direction
of the deviation. Thus, the position of prism is
Prerequisites
base-out for esotropia, base-in for exotropia,
zz Both eyes must be able to fixate the target.
and base-down for a hypertropia.
zz Both eyes must have central fixation. zz The amplitude of the refixation movement
zz No severe motility defect. should gradually decrease as the strength of
Cover test prism approaches the extent of deviation.
zz To start with, cover the apparently fixating eye. zz The end-point is approached when no
zz Observe if the apparently deviating eye moves movement is seen. To ensure the maximum
to take up fixation. angle is found, the prism strength can be
zz If the uncovered eye moves, tropia exists. increased further until a movement is observed
in the opposite direction (the point of reversal)
Uncover test
and then reduced again to find the neutral
zz It is done to unmask the phoria.
value; the angle of deviation is then taken from
zz After covering the eye fusion breaks and if
the strength of the prism.
there is any heterophoria the eye behind the zz In cases of incomitant strabismus, both
cover deviates. This can be observed with primary and secondary deviations should be
the use of translucent occluder (Spielmann’s determined [primary deviation—deviation of
occluder). the paretic eye when normal eye is fixating;
zz After removing the cover if it remains deviated, secondary deviation—deviation of the
it confirms a latent squint with poor fusion normal eye when paretic eye is fixating]. The
(poor recovery). If it recovers, the examiner secondary deviation is always larger than the
observes the speed of recovery, which primary deviation.
shows strength of fusion and it, is important
prognostic sign. Accommodative Convergence to
Alternate cover test Accommodation Ratio
zz The alternate cover test induces dissociation It characterizes the difference in alignment
to reveal the total deviation when fusion is observed between distance and near fixation. A
disrupted. It should be performed only after normal accommodative convergence to accom
the cover–uncover test. modation ratio (AC/A Ratio) allows the eyes to
zz One eye is covered for several seconds. The remain aligned and in focus as a target moves
occluder is quickly shifted to the opposite closer. Preoperative determination of AC/A ratio
eye for 2 seconds, then back and forth several may be helpful in predicting the extent to which a
times. patient may respond to plus lenses when a surgical
zz After the cover is removed, the examiner notes overcorrection is obtained.
the speed and smoothness of recovery as the AC/A ratio can be determined by following
eyes return to their predissociated state. methods:
zz A patient with a well-compensated hetero zz Gradient method: IPD is not needed as
phoria will have straight eyes before and after vergence is measured at the same distance.
the test has been performed whereas a patient At 33 cm with patient wearing his proper
with poor control may decompensate to a refractive correction, deviation is measured
manifest deviation. with and without +3.0DS lens (if assessed using
distance target -3.0SD Lens is used) placed in Maddox Rod

front of both the eyes. zz The Maddox rod consists of a series of fused
Prolonged mono-ocular occlusion used to cylindrical red glass rods that convert the
break vergence after effect before test. appearance of a white spot of light into a red
AC/A = Deviation without lens – deviation with streak. The optical properties of the rods cause
lens the streak of light to be at an angle of 90° with
Power of the lens in diopters. the long axis of the rods; when the glass rods
zz Heterophoria method: Deviation measured are held horizontally, the streak will be vertical
at distant vision with full optical correction and vice versa.
(no accommodation exerted), and at a near zz The rod is placed in front of the right eye.
distance (e.g. 33 cm). This dissociates the two eyes: the red streak
AC/A = IPD + ND-DD/AN [IPD = inter pupillary seen by the right eye cannot be fused with the
distance (cm); ND = near deviation (Diopters); unaltered white spot of light seen by the left
DD = Distance deviation (Diopters); AN = eye.
Accommodation for near (1/3 = 3 Diopters)] zz The amount of dissociation is measured by
[Note: Esotropia + sign is used and for Exotropia the superimposition of the two images using
– sign is used for deviation while calculating prisms. The base of the prism is placed in
AC/A ratio]. the position opposite to the direction of the
zz Graphic method, fixation disparity method deviation.
and haploscopic method are other methods zz Both vertical and horizontal deviations can be
for AC/A ratio: measured.
Routine AC/A ratio is normally not done
clinically; instead, the difference in measure Fusional Amplitudes
ments at distance and near is assessed. Any
Fusional amplitudes measure the efficacy of
difference of >10 PD suggests an abnormal
vergence movements. It can be tested with prisms
AC/A relationship and should be considered
bars or synoptophore. A progressively increasingly
into the treatment plan for the patient.
strong prism is placed in front of one eye, which
will then abduct (base-in prism) or adduct (base-
Maddox Wing out prism) to maintain bifoveal fixation. Thus for
zz The Maddox wing dissociates the eyes for near fusional amplitude of convergence base-out prism
fixation (1/3 m) and measures heterophoria. is used and for divergence base in prism is used.
zz The instrument is constructed in such a way When the power of prism exceeds the fusional
that the right eye sees only a white vertical amplitude, diplopia is reported or one eye drifts
arrow and a red horizontal arrow, whereas the in the opposite direction, indicating the limit of
left eye sees only horizontal and vertical rows vergence ability. This is known as break point and
of numbers. gradually then decreases the strength of the prism
zz Horizontal deviation is measured by asking till patient realign his eyes this is the recovery
the patient to which number the white arrow point. This should be checked for both distance
points. and near fixation.
zz Vertical deviation is measured by asking the
patient which number intersects with the red Near Point of Convergence
arrow. It is the nearest point on which the eyes can
zz The amount of cyclophoria is determined by maintain binocular fixation. It can be measured
asking the patient to move the red arrow so with the RAF ruler that rests on the patient’s cheeks.
that it is parallel with the horizontal row of A target is slowly moved along the rule towards
numbers. the patient’s eyes until one eye loses fixation and
drifts laterally (objective NPC). The subjective near —— When all four lights are seen in the
point of convergence (NPC) is the point at which presence of a manifest deviation, harmo
the patient reports diplopia. Normally, the NPC nious ARC is present
should be nearer than 10 cm without undue effort. —— Two red lights are seen if left eye
suppression is present
Near Point of Accommodation —— Three green lights are seen if right
suppression is present
It is the nearest point at which the eyes can —— If two red and three green lights are seen,
maintain clear focus. It is also measured with the diplopia is present.
RAF ruler. The patient fixates a line of print, which —— If the green and red lights alternate,
is then slowly moved towards the patient until it alternating suppression is present.
becomes blurred. The distance at which this is
first reported is the near point of accommodation Bagolini Striated Glasses
(NPA). The NPA continuously recedes with age.
At the age of 20 years, the NPA is 8 cm and by the
zz Test for detecting BSV, ARC or suppression.
age of 50 years approximately 46 cm.
zz Each lens has fine striations that convert a
point source of light into a line similar to the
Maddox rod.
Inter-pupillary Distance (IPD) zz Procedure: The two lenses are placed at 45° and
It is measured with the help of two scales or 135° in front of each eye and the patient fixates
synoptophore. on a focal light source. Each eye perceives an
oblique line of light, which is perpendicular to
that perceived by the fellow eye.
Diplopia Charting zz When the two streaks intersect at their
Test requires red green glasses. Diplopia charting centers in the form of a cross (X), the patient
is done in all 9 gazes with the help of a linear light has BSV with NRC if the eyes are straight, or
source. harmonious ARC in the presence of manifest
strabismus.
Tests for Binocular Vision and Sensory zz When the two lines are seen but they do not
Anomalies form a cross, diplopia is present.
zz When only one streak is seen, there is no
Worth Four-dot Test simultaneous perception and suppression is
zz A dissociation test used with both distance and present.
near fixation. zz If a small gap is seen in one of the streaks, a
zz Differentiates between BSV (binocular central suppression scotoma is present (e.g.
single vision), ARC (anomalous retinal microtropia). However, it is often difficult
correspondence) and suppression. to appreciate by the patient and the patient
zz Presence or absence of a manifest squint must describes a cross. This scotoma can be
be known at time of testing. confirmed clinically with the 4 Δ prism test.
zz Procedure: The patient wears a red lens in front
of the right eye (which filters out all colors Four Δ Prism Test
except red) and a green lens in front of the zz It distinguishes bifoveal fixation (normal
left eye (filter out all colors except green). The BSV) from foveal suppression (also known
patient then views a box with four lights–one as a central suppression scotoma (CSS) in
red, two green and one white. microtropia.
zz Results zz With bifoveal fixation: The prism is placed
—— BSV is present when all four lights are base-out (microtropia is commonly esotropic
seen not exotropic) in front of the right eye with
deviation of the image away from the fovea zz Randot test: It uses Julsez’s random dot
temporally, followed by corrective movement background to mask the monocular clues. It
of both eyes to the left [kindly read it once, the requires Polaroid glasses. Near randot is done
message is not clear]. at 40 cm and distance randot is done at 3 m.
zz The left eye then converges to fuse the images zz TNO test: The TNO random dot test consists
zz In left microtropia: The patient fixates a of seven plates of randomly distributed paired
distance target with both eyes open and a 4 Δ red and green dots viewed with red–green
prism is placed base-out in front of the eye with spectacles, and measures from 480 down to 15
suspected CSS. The image is moved temporally seconds of arc at 40 cm.
in the left eye but falls within the CSS and no zz Frisby Davis distance test: this is a real depth
movement of either eye is observed. test. Measured at 6 m, 3 m or 1 m measure
zz The prism is then moved to the right eye stereoacuity from 4 sec to 200 sec of arc.
which, adducts to maintain fixation; the left zz Lang’s pencil gross streopsis test: Pencils held
eye similarly moves to the left consistent with horizontally to avoid patient seeing end on
the Hering law of equal innervation, but the view. We ask the patient to touch the pencil
second image falls within the CSS of the left eye tip held by him to that of the examiner. Simple
and so no subsequent re-fixation movement is bedside test works well to demonstrate gross
seen. stereopsis (3000–5000 sec of arc).
zz Synoptophore test: The synoptophore compen
Base-out Prism sates for the angle of squint and allows stimuli
This is a simple method used for detecting fusion to be presented to both eyes simultaneously.
in children. The test is performed by placing a 20 Δ zz It can thus be used to investigate the potential
base-out prism in front of one eye of the patient. for binocular function in the presence of a
The prism displaces the retinal image temporally manifest squint and is of particular value in
resulting in diplopia. Most children with good assessing young children (from age 3 years),
BSV can overcome a 20 Δ prism from the age of 6 who generally find the test process enjoyable.
months; if not, weaker prisms (16 Δ or 12 Δ) may It can also detect suppression and ARC.
be tried, but the response is then more difficult to zz The instrument consists of two cylindrical
identify. tubes with a mirrored right-angled bend and
a + 6.50 D lens in each eyepiece. This optically
After Image Testing (with Synaptophore) sets the testing distance as equivalent to about
6 m.
It is a dissociative test to know suppression, ARC or zz The synoptophore can measure horizontal,
central scotoma. vertical and torsional misalignments
simultaneously and is valuable in determining
Stereopsis surgical approach by assessing the different
Various tests, using differing principles, are contributions in the cardinal positions of gaze.
employed to assess the stereoacuity. Random dot zz Hess/Lees testing: A Hess chart is plotted to aid in
tests (e.g. TNO, Frisby) provide the most definitive the diagnosis and monitoring of a patient with
evidence of high grade BSV. Where this is weak incomitant strabismus, such as an extraocular
and/or based on ARC, contour-based tests (e.g. muscle palsy (e.g. third, fourth or sixth nerve
Titmus) may provide more reliable information. paresis) or a mechanical or myopathic
zz Titmus test: The Titmus test consists of a three- limitation (e.g. thyroid ophthalmopathy, blow-
dimensional polarized vectograph comprising out fracture or myasthenia gravis). The chart
two plates in the form of a booklet viewed is commonly prepared using either the Lees
through polarized spectacles. On the right is or Hess screen, which facilitate plotting of
a large fly, and on the left is a series of circles the dissociated ocular position as a measure
and animals. The test should be performed at a of extraocular muscle action. Information
distance of 40 cm. provided by the Hess chart should be regarded
in the context of other investigations such as zz The larger chart indicates the eye with the
the field of binocular single vision. overacting yoke muscle (left eye).
zz The smaller chart will show its greatest
Hess Screen restriction in the main direction of action of
The Hess screen contains a tangent pattern the paretic muscle (right lateral rectus).
displayed on a dark gray background. Red lights zz The larger chart will show its greatest
that can be individually illuminated by a control expansion in the main direction of action of
panel indicate the cardinal positions of gaze within the yoke muscle (left medial rectus).
a central field (15° from primary position) and a zz The degree of disparity between the plotted
peripheral field (30°); each square represents 5° point and the template in any position of gaze
of ocular rotation. The eyes are dissociated by the gives an estimate of the angle of deviation
use of reversible goggles incorporating a red and a (each square = 5°).
green lens, the red lens in front of the fixating eye
and the green lens the nonfixating eye. Red points Forced Duction Test
of lights are illuminated at selected positions
on the screen. The patient holds a green pointer, The forced duction test (FDT) is an attempt by the
and is asked to superimpose a green light over examiner to move a patient’s eye farther in a given
each red light in turn. In orthophoria the two direction than the patient can move it. Topical
lights should be more or less superimposed in all anesthetic is placed on the appropriate limbal
positions of gaze. The goggles are then reversed location (generally 180° away from the duction
and the procedure repeated. Software is available limitation) with a small cotton swab and the
that facilitates the plotting of a Hess chart using a limbal conjunctiva is grasped firmly with a toothed
standard desktop computer screen. forceps. The patient is asked to rotate the eye fully
in the direction of the limited duction. An attempt
Lees Screen is then made by the examiner to rotate the eye
beyond the position attained by the patient while
This apparatus consists of two opalescent glass avoiding globe retraction. Care must be taken not
screens at right-angles to each other, bisected to abrade the cornea.
by a two-sided plane mirror that dissociates the Patients who have pure nerve palsy exhibit
eyes; each of the eyes can see only one of the two no restriction to full movement by the examiner;
screens. Each screen has a tangent pattern (two- patients who have pure restriction (dysthyroid
dimensional projection of a spherical surface) that orbitopathy, entrapment of ocular contents after
is revealed only when the screen is illuminated. blowout fracture) exhibit restricted movements
The patient is positioned facing the nonilluminated (sometimes termed a positive forced duction test).
screen with his or her chin stabilized on a rest. Some patients initially have pure nerve palsy, but
Using a pointer, the examiner indicates a target contracture of the antagonist muscle results in
point on the illuminated tangent pattern and the secondary mechanical restriction of movement.
patient positions a pointer on the nonilluminated Suction cup devices have been developed
screen, at a position perceived to be superimposed for examiners who are wary of using toothed
on the dot indicated by the examiner. The non- instruments at the limbus; a cotton swab may be
illuminated screen is briefly illuminated by the a sufficient tool in some patients. Forced duction
examiner using a footswitch to facilitate recording testing of oblique muscles may be performed, but
of the dot indicated by the patient. When the two forceps are used and the globe is depressed
procedure has been completed for one eye, the forcibly into the orbit.
patient is rotated through 90° to face the previously
illuminated screen and the procedure repeated.
Active Forced Generation Test
Interpretation Active force generation testing may be used to
zz The smaller chart indicates the eye with the evaluate the ability of a muscle to move the eye
paretic muscle (right eye). against a resisting force. The forceps is placed
at the limbus of the anesthetized globe in the Table 1 Types of comitant esotropia
meridian of the muscle whose duction is limited
and the patient requested to rotate the eye in the Accommodative Nonaccommodative
direction of the limited duction; the examiner • Refractive • Infantile esotropia
judges through the forceps the relative amount of accommodative
force generated. Strain gauges have been devised • Nonrefractive • Acute onset
that enable quantitation of this force. This is done accommodative
in patients with paralytic squint (example- in
• Partially • Acquired or late onset
esotropia due to 6th CN palsy).
accommodative
• Hypoaccommodative • Microtropia
CLASSIFICATION
• Cyclic esotropia
zz Esophoria: Latent deviation that is controlled
• Nystagmus blockage
by fusion under binocular conditions
syndrome
zz Intermittent esotropia: Deviation that is
controlled by fusion sometimes, but manifests
in exertion/illness Table 2 Incomitant esotropia types
zz Esotropia: Deviation is constantly manifested Paralytic 6th CN palsy, divergence palsy,
Right, left or alternating myasthenia
Comitant (Table 1) vs incomitant (Table 2) Restrictive Eso DRS, tumor, postoperative,
Primary/secondary/consecutive strabismus fixus, endocrine myopathy
zz Primary: No other cause. Most of the esotropias
are primary Spastic
zz Secondary: Consequence of loss or impairment
of vision It depends upon the type of esotropia:
zz Consecutive: Overcorrection of an initial zz For accommodative esotropia—all patient
exotropia. requires is full cycloplegic refraction with or
without near add depending upon AC/A ratio
for correction of their deviation fully (Table 3).
MANAGEMENT
zz For partially accommodative esotropia—the
In all patients of esotropia should undergo full accommodative element requires correction
cycloplegic refraction before measurement of with glasses and for the nonaccommodative
deviation. element surgery is needed.
Occlusion therapy should be given to those zz For nonaccommodative esotropia surgery
with amblyopia. is needed. For convergence excess (near
Table 3 Classification of accommodative esotropia

Refractive normo- Refractive hyper- Nonrefractive hyper- Nonrefractive hypo-
accommodative accommodative accommodative accommodative
Refractive error Hyperopia Hyperopia Not significant Not significant
AC/A ratio Normal Increased Increased Normal
no convergence excess
NPA – – – Remote
NPC – – – Remote
N-D deviation <15 >15 >15 >15
(prism Diopter) No significant
esotropia for distance
>distance by 10PD) bimedial recession or – Accommodative component is

medial recti recession with posterior fixation present—greater deviation at near
is done. For divergence insufficiency (distance – Accommodation is not linked to
> nearby 10 PD) bilateral LR resection is refractive error; there is synkinesis
preferred. For basic type (near distance with accommodative convergence
disparity less than 10 PD) either monocular – Costenbader: Small refractive error,
MR recession and LR resection or bimedial remote near point of accommodation,
recession is done. small distance but large near
deviation
VIVA QUESTIONS – Excessive accommodative effort and
a large persistent deviation with the
Q.1. What is accommodative esotropia? refractive error fully corrected
Ans. See Table 1 for types Management: Treat with bifocals and
• R efractive accommodative: (Normal miotics
AC/A ratio) esotropia restored to ortho – Miotics—echothiophate iodide 0.06%
tropia at all fixation distances and in all or 0.125%/BE OD for 6 weeks—dose
positions of gaze by optical correction of may be reduced subsequently
underlying refractive error. – Complications—RD, iris cysts, etc.
– Onset between 6 months—5 years of – Most patients improve with therapy
age – Surgery: Advocated if nonaccommo
– Associated hyperopia dative component develops
– Insufficient fusional divergence • Partially accommodative esotropia
– Often hereditary – Esotropia having both accommodative
– I ntermittent at onset– becoming and non-accommodative elements
constant are considered as partially accommo
– Deviation: 20–40 Δ dative esotropias.
– Precipitation—trauma, illness – May be: Decompensated accommo
• Treatment of refractive accommodative dative esotropia
esotropia: – Esotropia with subsequent develop
– Cycloplegic refraction ment of accommodative component.
– Full cycloplegic correction of hyper Treatment:
metropic refractive error – Eventually amblyopia therapy with or
– Initial atropinization to relax accom- without surgery is needed.
modation – Cycloplegic refraction with bifocals.
– 60–70% of these patients respond – Surgical correction of residual
well to treatment with glasses; the deviation (nonaccommodative part)
remaining require surgical treatment – Explaining to parents that children
(Dyer et al ) would still require glasses after
– It is important to treat the entire surgery.
accommodation (latent + manifest) – Alignment of the eyes with glasses or
– Orthoptic treatment to overcome surgery alone usually does not make
sup p ression and build fusional the eyes work together. Vision therapy
divergence. does.
• Nonrefractive accommodative: (High – Occasionally the deviation may
AC/A ratio) increase after patient gains stereopsis
– Esotropia N > D for near; one should repeat refraction
– Abnormally high AC/A ratio at this point.
– Normal NPA – Supportive orthoptic treatment to
– Age: 2–3 years promote fusional divergence.
– Usually has hypermetropia (2.25 D) • Hypoaccommodative:
but may have myopia – Esotropia N > D
– Unrelated to refractive error • R efractive errors—Most commonly asso

– Weakness in accommodation (primary ciated with mild hyperopia, followed by
or secondary) → excess accommo moderate and high hyperopia, and lastly
dative effort → excess convergence myopia associated with hypermetropia
– The NPA is receded (> 85 %) rare in myopia (6–8 %).
Q.2. Describe clinical features and manage Ciancia syndrome
ment of essential infantile esotropia. • Essential infantile esotropia
Ans. Essential infantile esotropia: • Latent nystagmus
– Manifest esodeviation • Head turn toward adducting eye
– Onset between birth and 6 months of • Apparently limited abduction in both
age eyes
– Neurologically normal infant prevalence: Lang syndrome
– 0.1% of newborns • Esotropia
– Even with this reduced prevalence it is • DVD
the most common form of strabismus • Excycloduction of the nonfixating eye
Clinical characteristics • Abnormal head posture
• Onset from birth to 6 months Differential diagnosis
• Large angle (≥30∆) • Congenital
• Stable angle – Bilateral abducens paralysis
• Initial alternation with cross fixation – Duane syndrome type 1
• No clinically apparent CNS involvement – Mobius syndrome
• Asymmetrical optokinetic nystagmus— • Acquired
OKN asymmetry is present in all infants – Sensory esotropia
but becomes symmetrical by 6 months in – Refractive accommodative esotropia
the normal. Patients with congenital ET – Nystagmus blockage syndrome
retain OKN asymmetry – Esotropia in association with CNS
• Temporal to nasal (T/N) OKN—smooth, problems
following and rapid refixation. Management
• Nasal to temporal (N/T) OKN—jerky Goals of treatment
inaccurate movements with halting – Restoration of single binocular vision
refixation. – Normal visual acuity
• Incidence: 1–2% – Normal stereoacuity
Variable findings1,2 – Normal retinal correspondence
• Amblyopia – Stable sensory and motor fusion
• Apparently defective abduction Nonsurgical treatment
• Apparently excessive adduction – C orrect all hypermetropias (full
• Up or down shoot on adduction cycloplegic refraction)
• A or V pattern Amblyopia treatment
• Inferior oblique over action (68%) – Conventional, full time occlusion.
• DVD/DHD (50%) – End-point being free alternation of the
• Manifest latent nystagmus (33%) two eyes which is equally maintained.
• Manifest nystagmus (rare) – It should be treated before surgery
• Anomalous head posture because…
• Heredity: Transmission may be irregular - The earlier the treatment betters the
autosomal dominant, or recessive. results.
Waardenburg reported concordance in - The diagnosis of amblyopia and
monozygous twins to be 81%, compared monitoring of fixation preference
with 9% in dizygotic difficult, once eyes aligned.
• History of strabismus in the parents or - Patient neglect their follow-up
siblings—positive appointment for amblyopia.
- The outcome of surgery is less – Smaller turns may only require vision
favorable. therapy.
- The only situation when surgery Botulinum toxin: It is an effective treatment
is indicated in the face of residual modality for the management of infantile
amblyopia is a tight medial rectus esotropia in infants.
muscle that causes one eye to be Q.3. What are microtropias? Describe its
buried in the medial canthus even clinical features and management.
when the good eye is patched. Ans. • These are ultra-small angle esodeviations
Surgical management which may be missed by ordinary
• Timing of surgery methods of examination.
– Early surgery provides better chances • Can be primary or secondary, latter are
of functional improvement. residual deviation postsurgery.
– A secondary change that occurs in • P ark monofixation syndrome has
extraocular muscles, the conjunctiva, macular scotoma with good peripheral
and Tenon’s capsules makes a fusion and fusional amplitude with gross
surgical correction at later stages stereopsis.
more difficult and less predictable. • Lang’s microtropia—small angle hetero
– In some cases of infantile esotropia, tropia (<5°) these have harmonious
persisting deviation warrants early ARC with mild amblyopia and partial
surgery (3–4 months ) stereopsis. These are of 3 types—type 1
– Treatment of infantile esotropia must has central fixation; type 2 has eccent
be started at an early age frequently at ric fixation without identity; type 3 has
6 months. eccentric fixation with identity (this
– Esotropia: Surgery done within a means that angle of anomaly is same as
year of misalignment yields better the eccentricity of fixation)
stereopsis. • Clinical features
• Surgical approaches: – Amblyopia
– Bilateral medial rectus (MR) recession – ARC
– MR recession with lateral rectus (LR) – Relative scotoma at fixation spot
resection – Near normal fusional amplitude
– Safe limits lesser in infants MR: – Defective stereopsis
5.5 mm, LR: 6.5 mm for recession. – Size of deviation (5°–8°)
– 3 or 4 muscle surgery has also been – Foveal or eccentric fixation
advocated – Presence or absence of anisometropia
– Inferior oblique overaction: Muscle • Test used for diagnosis
weakening procedures – Bagolini’s glasses—detects central
– Alignment within 7–8 Δ of orthopho scotoma
ria: Acceptable. However, ortho – 4 prism diopter test—already
tropia/small—esotropia— better in explained
comparison • Management: Primarily involves treat
– One must carefully look at the fixation ment of amblyopia.
pattern to make sure that there is no
amblyopia. REFERENCES
– Treatment after 2 years reduces prog
1. Costenbader FD. Essential infantile esotropia.
nosis for re-establishment of bin Trans Am Ophthalmol Soc; 1961.
ocular vision. 2. Gunter K, von Noorden, Emilio C Campos.
– Older children with infantile isotropic Binocular Vision and Ocular Motility: Theory
need both surgical intervention if the and Management of Strabismus, 6th edn,
turn is large and vision therapy. St Louis, MO, Mosby; 2002.
EXODEVIATION
INTRODUCTION zz Refractive error: Patient may have uncor

rected myopia, high degree of uncorrected
Exotropia (XT) is outward deviation of the eyes. It hypermetropia, anisomyopia, anisoastig
is less more frequently seen than esotropia (ET). matism can also be there.
The approximate ratio of XT to ET is 1:3. According
to some studies XT is more prevalent in the Middle History of past illness: Any history of febrile
East, the Orient, and Africa. Most studies report a disease or cerebral palsy must be noted. Previous
normal distribution of refractive errors with XT. ocular history including refractive prescription
In exams, it can be given as a long case. and compliance with spectacles or occlusion,
previous surgery or prisms is important to future
treatment options and prognosis.
HISTORY
Chief Complaint Birth History
zz Deviation of eyes zz Children with craniofacial anomalies more
zz Headaches, eyestrain, blurring of vision (older likely to exhibit XT.
children and adults) zz Maternal smoking during pregnancy increases
zz Difficulty with prolonged period of reading the risk of XT [maternal smoking is more
zz Diplopia, photophobia, image is perceived as commonly associated with ET and for XT it is
becoming smaller and coming closer controversial].
zz Low birth weight (LBW) baby increase the risk.
History of Present Illness zz Retinopathy of prematurity and Down’s
syndrome increases the risk of XT.
Patient (parents in case of small children) may
Family history: Note down any history of
complaint of headaches, eyestrain, blurring of
strabismus in the parents or siblings. There is
vision (older children and adults), and difficulty 17-fold increased risk in monozygotic twin.
with prolonged period of reading.
zz Deviation of eyes: Deviation of eyes towards
EXAMINATION AND SPECIAL TESTS
outside which can be intermittent or constant.
Deviation may be present since birth or shortly For examination please refer to chapter of esotropia.
after birth. Classification: Based on difference in measurement
zz Diplopia: Seen in adults with a mature visual of deviation for distance and near, intermittent
system, which can be intermittent. Children exotropia can be divided into following types:
with intermittent or constant exotropia are zz Divergence excess pattern-distance deviation
less symptomatic as suppression eliminates exceeds near deviation by 10 prism diopters
diplopia. zz Convergence insufficiency pattern—near
zz Photophobia: Bright light dazzles the retina deviation exceed distance deviation by 10
so that fusional vergence is disrupted, prism diopter
causing manifest deviation. Child closes one zz Basic exodeviation—when distance and near
eye to avoid confusion and diplopia [this deviation is same or does not exceed 10 prism
phenomenon is called Diplopia- Phobia] diopter.
zz Micropsia: Rare, patient uses accommodative zz Simulated or pseudodivergence excess—
convergence to maintain BSV. Image is these are basic deviation but appear as diver
perceived as becoming smaller and coming gence excess exotropia due to either tenacious
closer. proximal fusional convergence (TPFC) or due
to accommodation as in patients with high zz 1/2 to 1/3rd of deviation is corrected in inter

AC/A ratio. Following test differentiates the mittent exotropia.
true and pseudodivergence excess.
Occlusion test of Scobee–Burian: Differentiate true Orthoptics
and simulated divergence excess due to TPFC. zz Anti-suppression exercises—appreciation of
zz Distance and near deviation is measured diplopia.
zz U/L occlusion of one eye for 24 hour (Scobee)/ zz Convergence exercise is done with the help of
30–45 min (Burian) a convergence trainer. These do not affect the
zz Ensure that patient does not use both eye basic deviation but decrease the manifestation
simultaneously even momentarily of tropia to phoria.
zz Measurement of deviation for near fixation is zz Should be done only after antisuppression
repeated. exercises in cases of suppression.
In simulated divergence excess after patching zz Should not be done in intermittent exotropia
there is increase in near deviation, so that it equals for distance only in whom surgery is planned
or exceeds that at distant fixation. as can cause postoperative over convergence.
True divergence excess is not influenced by zz Exercises on synoptophore should always be
occlusion. supplemented by home exercises.
zz Aim is to obtain normal near point of
+ 3.0DS lens test: Differentiate true and simulated
convergence.
divergence excess due to accommodative
convergence.
Occlusion Therapy
zz A +3.0DS lens is used and deviation for near
fixation is repeated. zz Useful in small angle exotropia.
zz A large increase of near deviation indicates a zz Occlusion of the preferred eye for 3–5 hours
high AC/A ratio. per day decreases the angle of deviation—
zz These patients are good candidate for over evaluate 4 monthly.
minus therapy, i.e using more minus for
myopic and using less plus for hypermetropic Surgery
to stimulate accommodation that aids in Indications: The indications for surgery in—
decreasing or controlling the amount of zz Intermittent exotropia include following:
exodeviation. —— Exotropia > 50% of waking hrs
zz Preoperative determination of AC/A ratio —— Newcastle scoring >3 (Table 1)
may also be helpful in predicting the extent —— Deviation > 20 D
to which a patient may respond to plus lenses —— Asthenopic symptoms
when a surgical overcorrection is obtained. —— Increasing basic deviation
—— Secondary convergence insufficiency
TREATMENT with asthenopic symptoms

—— Development of suppression
Optical —— Decreasing stereopsis
zz Full cycloplegic refraction to be done. zz In constant exotropia: Surgery is almost always

zz Over minus therapy as described above can indicated with preoperative and postoperative
be prescribed specially in patients with high orthoptic treatment.
AC/A ratio. Timing of surgery: Knapp advocated early surgery
to prevent sensory changes especially in patients
Prisms with infantile exotropia.
zz Indicated in overcorrected/under corrected The best approach is to assess the timing in
exotropia. each case individually.
zz Base out prisms to stimulate fusional Factors to consider before surgery:
convergence. zz Age
Table 1 N
ewcastle scoring for intermittent of exotropic drift in postoperative period
exotropia especially in patients with poor vision.
zz No suppression.
Home control Score zz Stereopsis should be salvaged by performing
X(T) or mono-ocular eye closure- never seen 0 early surgery especially in very young children
<50% times for distance fixation 1 with constant deviation.
<50% times for near fixation 2
Types of Surgery
>50% times for both distance and near 3
fixation
zz True divergence excess type: Bilateral recession
of lateral recti.
Clinic control zz Basic exotropia and stimulated divergence
Distance excess: Unilateral rectus recession and medial
Immediate realignment after cover test 0 rectus resection.
zz Convergence insufficiency: Bilateral resection
Realignment after blink or refixation 1
of medial recti.
No realignment/manifest spontaneously 2 zz Lateral gaze inhibition: Bilateral lateral recti
Near recession should be avoided.
Immediate realignment after cover test 0
Realignme nt after blink or refixation 1 VIVA QUESTIONS
No realignment/ manifest spontaneously 2
Q.1. How will you perform occlusion test of
Scobee–Burian?
Ans. Given in examination section.
zz Type and size Q.2. Differentiate between true and simulated
zz Comparative deviation at 33 cm, 6 m, and in divergence and name the tests used for
the far distance (20 m). that.
zz The size of the AC/A ratio and determining Ans. Given in examination section.
whether the patient has a true or simulated
Q.3. What is epidemiology of exotropia?
divergence excess.
Ans. Incidence
zz If there is a change of deviation on lateral
Exotropia appears less frequently than
versions.
esotropia (ET). The approximate ratio of XT
zz Lateral gaze inhibition (i.e. decrease in
to ET is 1:3
deviation on lateral gaze) If there is a V or
In Costenbader’s series of 472 patients with
A phenomenon with or without associated
IDS, deviation was present
inferior oblique or superior oblique overaction.
• At birth – 204
These should not be missed especially A
• 6 months – 16
pattern as this is a risk factor for postoperative
• 6–12 months – 72
residual exotropia.
• After 5 years – 24
• Sex – women (approximately 60–70%)
Aims Other associations
zz Phoria for distance and near in primary • Facial symmetry associated with
position. exodeviations
zz In children aim of surgery is to either give • Children with craniofacial anomalies
optimal correction or slightly under correct (up more likely to exhibit exotropia
to 8 PD is desirable) as consecutive esotropia • Maternal smoking during pregnancy
can result in amblyopia. • LBW
zz In adult surgery can be aimed at slight • 17-fold increased risk in monozygotic
overcorrection (up to 8PD) as there is risk twin.
Table 2 Difference between alternating and Table 3 Classification intermittent exotropia

unilateral constant exotropia Burian classification Kushner classification
Alternating Unilateral Divergence excess High AC/A ratio
Visual acuity is almost Fixation preference Strong proximal convergence
equal in both eyes Simulated Tenacious proximal fusion
Angle of deviation is large, Deviation is large divergence excess
nearly equal for distance
Basic pattern Basic pattern
and near
Associated commonly Vertical deviations are Convergence Low AC/A ratio
with secondary vertical more common insufficiency Fusional convergence
deviation, deviating insufficiency
abducted eye is elevated Pseudoconvergence
insufficiency
If there is NRC and bifoveal Marked suppression
fusion-suppression of in the deviating eye
deviating eye • Secondary
– Sensory exotropia
Amblyopia is less
– Consecutive exotropia
severe than esotropia
Incomitant
Treatment is almost • Paralytic
always surgical
• Restrictive
• Musculofascial anomalies
Q.4. What is constant (early onset) exotropia? • Dissociated horizontal deviation
Ans. Constant (early-onset) exotropia has B. On the basis of underlying fusional
following features: reserve
Presentation is often at birth. Primary • Exophoria XP
constant infantile exotropia is rare • Intermittent exotropia X(T)
Signs • Manifest exotropia XT
• Normal refraction C. Burian’s classification of intermittent
• Large and constant angle exotropia
• DVD may be present • B asic pattern: Distance and near
• Neurological anomalies are frequently deviation is almost equal
present, in contrast with infantile • C onvergence insufficiency: Near
esotropia. deviation is larger than distance
• Treatment is mainly surgical and consists deviation by >15 pd
of lateral rectus recession and medial • D ivergence excess: Distance deviation
rectus resection. exceeds near deviation by >15 pd
Differential diagnosis is secondary exo • S imulated divergence excess: Initial
tropia, which may conceal serious ocular distance deviation exceeded the near
pathology. deviation by > 15 pd, but after 1 hr
Difference between alternating and uni of monocular occlusion 2 measure-
lateral constant exotropia is summarized in ments showed difference of < 15 pd.
Table 2. Burian classification did not specify the
Q.5. Classify exodeviation. type of convergence that was insufficient
Ans. A. Classification (accommodative or fusional) which
Concomitant/incomitant was included in Kushner classification
Comitant (Table 3).
• Primary D. Duane’s classification:
– Infantile exotropia • Divergence excess pattern
– Intermittent exotropia • Convergence insufficiency pattern
Q.6. What are Calhounz’s phases of exodevia-

tion.
Ans. • Intermittent exotropia at distance, ortho
phoria at near, asymptomatic
• E xotropia (Fig. 1) at distance, ortho
phoria/exophoria at near, symptomatic
for distance, no suppression scotoma
• E xotropia at distance, exotropia or
intermittent exo at near, binocular vision
for near, suppression scotoma develops
for distance
• Exotropia for distance and near. Lack of
binocularity.
Fig. 1: Left exotropia Q.7. What are the causes of pseudoexotropia?
Ans. Pseudoexotropia:
• Hypertelorism
• Basic exodeviation • Positive angle kappa
• S imulated or pseudodivergence • Large interpupillary distance
excess • Broad nasal bridge
True divergence excess • Narrowing of lateral canthi
• E xophoria—exotropia at distance,
normal near point of convergence, Q.8. What are the causes of pseudoesotropia?
adequate prism convergence, Ans. • Negative angle kappa
intermittency, equal vision, good ste- • Small interpupillary distance
reopsis, and ARC when exodeviated • Epicanthus.
• C ostenbader—definition of diver- Q.9. What are rules of thumb for prescribing
gence excess
spectacles for young children refractive
• Near: Distance > 15 pd
error without strabismus?
• Associated with a high AC/A ratio in
Ans. • Hyperopia greater than 5 D  
60% Kushner
• Myopia greater than 3 D  
Simulated divergence excess:
• A stigmatism greater than 2 D if not
• Exodeviation distance—near > 15pd
• After breaking fusion, distance—near oblique, greater than 1 D if  oblique  
< 10 pd • A nisometropia greater than 2 D for
• Due to vergence after effect (tonic myopic, greater than 1 D if  hyperopic,
and accommodative). Dissipated by greater than 2 D if astigmatic Refractive
prolonged mono-ocular occlusion error with strabismus; treat:  
• Over 80% of divergence excess type • Hyperopia or hyperopic astigmatism
patients greater than astigmatism  greater than
Convergence insufficiency 1.25 D (esotropia)  
• Near deviation—distance >15pd • Myopia greater than 1 D (exotropia) 
• Patients have either a low AC/A child’s age and symptoms as well as other
ratio or a fusional convergence factors must be taken into account.
insufficiency
• Patient usually in teens BIBLIOGRAPHY
• Asthenopic symptoms with intermit- 1. Binocular Vision and Ocular Motility: Theory
tent diplopia at near and Management of Strabismus, 6th edn. In:
• No X or X(T) initially at distance or Gunter K von Noorden, Emilio C Campos (Eds).
near. Seen as disease progresses. St Louis, MO, Mosby, 2002.
SHORT CASES
DUANE RETRACTION SYNDROME

Shipra Singhi, Saranya Devi K
INTRODUCTION aperture (co-contraction of muscles) can be there.

Diminution of vision may be there (Anisometropia/
Duane retraction syndrome (DRS) forms a part amblyopia).
of a group of conditions called congenital cranial History of past illness—history of fever/illness
dysinnervation disorders (CCDD), which occur Family history—may be hereditary.
due to the developmental errors in the innervation
of ocular and facial muscles. In Duane retraction EXAMINATION
syndrome (DRS) there is failure of innervation
of the lateral rectus by a hypoplastic sixth nerve General Examination/Specific Systemic
nucleus, with anomalous innervation of the lateral Examination
rectus by the third nerve. The condition is often Preauricular tag, hemivertebra, low hair line,
bilateral. Up to half of the patients have associated torticollis, malformation of the jaw, cheek and
systemic defects such as deafness, external ear ear, usually on one side of the face, radial ray
abnormalities, speech disorder and skeletal defect, Klippel-Feil anomaly, deafness, congenital
abnormalities. Associated mutations in several paresis of facial and abducens cranial nerves,
genes have been found. Approximately 10% of abnormalities of the upper limbs and heart may
cases are familial muscles.1 be present.
HISTORY Ocular Examination

Chief Complaints Head posture: Abnormal head posture is adopted
to achieve alignment of the two eyes in order
Patients present with deviation of either eye,
to obtain binocularity. Longstanding torticollis
limitation of extraocular movements, abnormal
(since birth) leads to craniofacial asymmetry.
movement of eye (upshoot and downshoot),
shortening of eye (leash phenomenon), bigger size Face turn: A face turn is typical, conferring BSV
of eye (retraction of globe), diminution of vision, in the primary position and avoiding amblyopia;
abnormal head and face posture. usually a face-turn to the affected side in types 1
and 3 and to the opposite side in type 2.
History of Present Illness Eyeball: Retraction of the globe on adduction
Patients may complaint of inward or outward because of co-contraction of the medial and lateral
deviation of either eye. Complete or less recti with resultant narrowing of the palpebral
often partial, absence of outward movement fissure (Figs 1A to C).
(abduction) of the affected eye or deficiency of
inward movement (adduction) of the affected Extraocular Movement (EOM)
eye. Abnormal movement of eye (upshoot and zz Complete or less often partial, absence of
downshoot) can be there while doing inward outward movement (abduction) of the affected
movement. Abnormal head posture is adopted eye
in order to obtain better vision (binocularity). zz Partial, or rarely complete, deficiency of inward
A face turn is typical, conferring binocular movement (adduction) of the affected eye
single vision (BSV) in the primary position and zz Partial closure of the eyelids (pseudoptosis) of
avoiding amblyopia. Narrowing of the palpebral the affected eye when it is adducted.
Amblyopia (anisometropia) occurs in about

10% of individuals and will respond to standard
therapy if detected early.
Special examination for squint: See section on
esotropia long case.
Lid: Partial closure of the eyelids (pseudoptosis) of
the affected eye when it is adducted.
Conjunctiva—previous scar of surgery may be
there.
A
Fundus—Disc anomaly may be there.
(Morning glory syndrome).
It includes:
zz Congenital sixth nerve palsy
zz Infantile esotropia
zz Mobius syndrome.
MANAGEMENT
B
Nonsurgical
zz Spectacles or contact lenses for refractive error
zz Prism glasses to improve the compensatory
head position
zz Treat amblyopia with standard therapy
zz Botulinum toxin: Botulinum toxin decreases
the amount of deviation and leash pheno
menon (upshoot or downshoot of globe with
adduction).
C
Surgical
Figs 1A to C: Left Eso-DRS with left eye limitation of
abduction with globe retraction The aims of surgery are:
zz To correct a manifest strabismus
zz To centralize the field of binocular single
vision,
zz An upshoot or downshoot in adduction may zz To overcome or reduce the need for a large
be present; in some cases, this is produced
compensatory head posture.
by a tight lateral rectus muscle slipping over
or under the globe to produce an anomalous Surgery is indicated for the following reasons:
vertical movement. zz Decomposition, giving rise to manifest
zz Strabismus: 76% of individuals have frank strabismus
strabismus in primary gaze. zz Abnormal head posture
zz Poor binocular vision. zz Severe globe retraction with or without
zz Deficiency of convergence in which the upshoot and downshoot
affected eye remains fixed in the primary zz Avoid lateral rectus resection.
position while the unaffected eye is converging. Different scenarios
Reviews of DRS patients have shown hyper zz For types 1 and 3 with head turn: Recession
metropia of greater than +1.50 in 71% of the of medial rectus muscle or horizontal
patients. transposition of vertical rectus muscles
zz For types 1 and 3 with leash phenomenon • 70% of cases are isolated
and/or severe globe retraction: Recession of • 3 0% of cases are associated with other
both medial and lateral rectus muscles with congenital anomalies.
possible Y-splitting of the lateral rectus muscle Isolated cases: 90% occur sporadically and
zz For type 2 with head turn and fixation with are commonly unilateral. Remaining 10%
uninvolved eye: Recession of ipsilateral lateral are inherited and these are commonly
rectus muscle bilateral.
zz For type 2 with head turn and fixation with Syndromic forms:
involved eye: Recession of contralateral lateral • O kihiro’s syndrome: Duane syndrome
rectus muscle and radial ray defects
zz For type 2 with leash phenomenon: Recession of • Goldenhar syndrome: Malformation of
lateral rectus muscle with possible Y-splitting the jaw, cheek and ear, usually on one
zz Associated V phenomenon with inferior oblique side of the face
overaction with upshoot: Horizontal recti plus • Wildervanck syndrome: Duane synd
inferior oblique recession. rome, Klippel-Feil anomaly, and
zz Associated a phenomenon with superior deafness
oblique overaction with downshoot: Ipsilateral • Moebius syndrome: Congenital paresis of
horizontal recti recession plus superior facial and abducens cranial nerves
oblique weakening. • Holt-Oram syndrome: Abnormalities of
zz Resection of the horizontal recti of the same the upper limbs and heart
eye should not be attempted even to correct • Morning glory syndrome: Abnormalities
the ocular deviation. of the optic disc.
zz Recession and retroequatorial myopexy Q.2. Name the systemic syndromes associated
(Faden) of the contralateral synergist can also with DRS.
be done to correct face turn and may improve Ans. See the discussion part.
the limitation of abduction of the involved eye.
Q.3. What is pathogenesis of DRS?
zz Y splitting of lateral recti can be done in
Ans. Various theories are as follows:
upshoot and downshoot.
• Myogenic theory: This theory, suggested
Complications of surgery: by earlier studies, indicates there is
zz Undercorrection of primary position esotropia fibrosis or inelasticity of the lateral
and the compensatory head turn rectus muscles and that the medial
zz Overcorrection leading to secondary exotropia rectus muscle inserts abnormally far
zz New vertical deviations can occur after vertical posteriorly.
rectus transposition procedures. • Neurogenic theory: There is a disturbance
in embryologic development between
weeks 4–8 which results in an absent
VIVA QUESTIONS abducens nerve with anomalous
innerv ations of the lateral rectus
Q.1. What is epidemiology of DRS? muscle by a branch of the oculomotor
Ans. Epidemiology: nerve. Simultaneous activation of the
• Prevalence of about 1/1000 in general medial and lateral rectus muscles, as
population demonstrated by EMG studies, may be
• Females (60%) > Males (40%) the cause of global retraction.
• Accounts for up to 4% of all strabismus
cases Q.4. Describe surgical management in DRS—
• M ost common type of congenital indications and procedures.
aberrant ocular innervation Ans. See management.
• 80% of cases occur unilaterally, with LE Q.5. Classify DRS.
predominance Ans. See Tables 1 and 2.
Table 1 Classification of DRS

Type 1 Type 2 Type 3 Type 4
Poor abduction, good Poor adduction, good Poor adduction, Paradoxical abduction on
adduction abduction poor abduction attempt adduction
Agenesis of 6th nerve, 6th nerve intact, 6th nerve agenesis, 6th nerve agenesis,
3rd nerve split innervate 3rd nerve split innervate 3rd nerve split 3rd nerve split innervate
LR, MR, LR, MR, innervate LR, MR, LR, MR,
Adduction intact as most of Poor adduction as LR The split is equal Most of the nerve innervates
the nerve goes to MR contract against MR LR
Abbreviations: LR, lateral rectus; MR, medial rectus; ADD, adduction; ABD, abduction
Table 2 Classification (Huber) of DRS

Type I Type Ii Type Iii
Most common, Least common, Characterized by
characterized by— characterized by: • Limited adduction and abduction.
• Limited or absent abduction. • Limited adduction • In the primary position, straight or
• Normal or mildly limited • Normal or mildly limited slight esotropia
adduction abduction • In some cases phenotypic variants
• In the primary position, straight • In primary position, have been allied to differing
or slight esotropia straight or slight exotropia genotypes
REFERENCE
1. Binocular Vision and Ocular Motility: Theory and Management of Strabismus. 6th edn, by Gunter K. von
Noorden, MD, and Emilio C. Campos, MD, St Louis, Mo, Mosby, 2002.
OCULAR MYASTHENIA GRAVIS

Myasthenia gravis (MG) is an autoimmune Chief complaint: Drooping of eyelid, double
disease in which antibodies mediate damage vision, limitation of eye movements, abnormal
and destruction of postsynaptic acetylcholine eye movement, difficulty in eye closure, light
receptors in striated muscle. The resultant sensitivity, fatigue of eye muscles, difficulty in
impair m ent of neuromuscular conduction swallowing, speech, breathing, muscular weak
causes weakness and fatigability of skeletal ness, lack of facial expression.
musculature, but not of cardiac and involuntary
Past history: Previous history of similar complaints
muscles. This resulting in progressive muscle
or any history of thymoma, thyroid dysfunction,
weakness with use of the muscle and recovery
intracranial mass, lung carcinoma.
of strength after a period of rest. Weakness is
experienced once number of receptors is 30% Pastsurgical history: Any history of surgery of
or less.1 extraocular muscles or ptosis or thymoma, thyroid
dysfunction, intracranial mass, lung carcinoma.
Systemic Myasthenia Table 1 Ocular signs of myasthenia gravis

zz Symptoms are typically of onset in the third Muscle involved Clinical sign
decade and may include painless fatigue,
Levator Ptosis
often brought on by exercise, commonly in
palpebrae Cogan’s lid twitch
conjunction with ptosis and diplopia.
superioris Lid hopping
Fatigability affects the musculature of the Enhanced ptosis
limbs, facial expression, ocular movements,
chewing and speech. Bulbar symptoms Extraocular Cranial nerve III, IV, or VI
movements weakness
include dysphagia and dysarthria; difficulty
(EOMs) Gaze palsies
with breathing is rare.
Pseudo-internuclear
zz Signs: The most important feature is peripheral ophthalmoplegia
weakness, particularly of the arms and (unilateral or bilateral)
proximal leg muscles, with wasting in long- Complete ophthalmoplegia
standing cases. There is characteristically a Intrasaccadic fatigue
lack of facial expression (myopathic facies). End gaze nystagmus
There may be several reasons why eye muscles Orbicularis oculi Weakness of forced closure
are more frequently involved. However, this is not Peek sign
completely understood.
One hypothesis is that patients may simply
notice eye weakness more often than mild zz Cogan twitch sign is a brief upshoot of the
weakness in other muscle groups in the body. eyelid as the eyes saccade from depression to
Another hypothesis is that the eye and eyelid the primary position.
muscles are structurally different from muscles in zz If one eyelid is elevated manually as the
the trunk and limbs. For example, they have fewer patient looks up, the fellow eyelid may show
acetylcholine receptors, which is where the defect fine oscillatory movements.
occurs in autoimmune MG. Eye muscles contract Diplopia: Diplopia is frequently vertical, although
much more rapidly than other muscles and may be any or all of the extraocular muscles may be
more likely to fatigue. affected. This most often occurs when looking up
Perhaps the most important difference or to the side. To compensate for the weakness,
between eye and eyelid muscles compared with the patient may tilt his/her head or turn their face
other muscles of the body is that eye muscles to allow the stronger eye to work. For example, if
respond differently to immune attack. The the muscle which allows the eye to look upward is
differences in the response of eye muscles to weak, the patient could tilt their head back to look
immune attack may explain why eye muscles are up.
also targeted in other autoimmune conditions,
Nystagmoid movements: Nystagmoid movements
such as autoimmune thyroid disease.
resemble nystagmus, but the initial pathological
defoveating movement is a saccadic intrusion.
Ocular Myasthenia
Ocular flutter and opsoclonus: These entities
Ocular involvement (Table 1) occurs in 90% of consist of saccadic oscillations with no inter
cases and is the presenting feature in 60%. Two- saccadic interval; in ocular flutter oscillations
thirds of patients have both ptosis and diplopia. are purely horizontal, and in opsoclonus they are
Less than 10% of patients have ptosis alone and multiplanar. Causes include viral encephalitis,
less than 30% have diplopia alone.1 myoclonic encephalopathy in infants (‘dancing
eyes and dancing feet’), as a transient idiopathic
Ptosis: Ptosis is insidious, bilateral and frequently
occurrence in healthy neonates, or may be
asymmetrical.
drug-induced.
zz Typically worse at the end of the day.
zz Worse on prolonged (60 second) upgaze due Ocular bobbing: Ocular bobbing manifests
to fatigue. with rapid downward conjugate eye movements
with a subsequent slow drift up to the primary Eaton syndrome is suspected, or an intra-
position. Causes include pontine lesions (usually cranial mass for ocular myasthenia.
hemorrhage), cerebellar lesions compressing the Ice pack test: This test for an improvement after
pons, and metabolic encephalopathy. an ice pack is placed on the ptotic eyelid (or
Peek sign: A test to check for fatigue and weakness other affected muscle) for 2 minutes, as cold
of the eye muscle which may be done by the inhibits the breakdown of acetylcholine by
examining doctor includes attempting to open acetylcholinesterase. It is around 75% sensitive but
the eyes while the patient tries to hold them shut, highly specific.
sometimes called a “peek sign”. This may result in Sleep test: The “sleep test”, which is based on the
one or both eyes opening, and the patient appears tendency for MG symptoms to improve following
to “peek” at the examiner. rest, may be used in small children and patients who
Forced duction test: It differentiates from paralytic have allergies or sensitivity to anticholinesterase
strabismus. drugs such as Tensilon. The patient is placed in a
quiet, darkened room and instructed to close their
DIAGNOSIS eyes for 30 minutes. The patient is photographed
and eye movement is measured before and after
The edrophonium (Tensilon) test is a first line test the test. The test is considered positive if there is
for diagnosis of MG. The Tensilon test consists improvement in the ptosis and/or eye movement
of injecting a small amount of medication (motility) following the 30 minutes rest period.
edrophonium intravenously. If the patient has The morning/evening comparison test is
MG the ocular muscle weakness, the ptosis, and similar in concept to the sleep test. The patient is
general muscle weakness and/or nystagmus will photographed, and the ptosis and ocular motility
improve dramatically for a short period of time. are compared at different times during the day.
In recent years an ice test is being more widely Old photos are very helpful to determine how
used. This is when ice is applied to the eyes, long the patient has had drooping of the upper
after a short period of time; the eyes will have eyelid.
an improvement of ocular symptoms. Usually, a Fatigue test: Another simple test for ptosis is the
blood test called acetylcholine receptor antibody “fatigue” test. This consists of having the patient
titer (AChR Ab) is ordered as well. Additional look at an object held up by the examiner in front
blood work may include other antibody studies, of the patient. After a short period of time the
thyroid profile, and a sedimentation rate. eyelid (s) will droop in the person with ocular MG.
Investigations Acetylcholine receptor (AChR) antibodies: Anti
body testing supports a diagnosis of MG and
Blood predicts the likelihood of thymoma. Testing is
zz AchR-Ab—Positive in 80% with generalized confounded by recent (within 48 hours) general
MG. Positive in only 50% with ocular involve anesthesia with muscle relaxants. Present in
ment only also present in 90% of patients with around 90% of systemic cases but only 50–70%
penicillamine-induced MG of ocular myasthenics. Rarely present in
zz Antistriated muscle Ab Lambert–Eaton.
zz Anti-muscle specific kinase Ab (Anti-MuSK Ab MuSK protein antibodies are positive in 50%
– positive in patients with AChR Ab –ve). of those negative for AChR antibodies; positive
patients are less likely to have ocular features and
Imaging thymoma.
zz CXR (chest x-ray)—thymus (anterior media Striational antibodies: Antibodies against several
stinal mass), aspiration pneumonia contractile elements of skeletal muscle (e.g. titin)
zz Thoracic imaging (MR, CT, CT/PET) to detect may be present; they are found in 80–90% of those
thymoma, present in 10%. Imaging may also with thymoma and one-third of those without and
be used to rule out a lung tumor if Lambert– can be a marker of more severe MG.
Voltage-gated calcium channel antibodies are GRADING

characteristic of Lambert–Eaton syndrome.
Myasthenia Gravis Foundation of America
Edrophonium (Tensilon) test: Edrophonium is
a short-acting anticholinesterase that confers a zz Grade 1—affects the ocular muscles only
transient improvement of weakness in MG. The zz Grade 2—mild weakness affecting muscles
estimated sensitivity is 85% in ocular and 95% in other than ocular muscles
systemic MG. Diagnostic and distinguishes from zz 2A—affects the limb and axial muscles
cholinergic crisis. zz 2B—affects the respiratory and bulbar muscles
zz An intravenous test dose of 0.2 mL (2 mg) zz Grade 3—moderate weakness (3A and 3B)
edrophonium hydrochloride is given. If zz Grade 4—severe weakness (4A and 4B)
definite symptomatic improvement (or adverse
zz Grade 5—intubation required.
reaction) is noted, the test is terminated.
zz The remaining 0.8 mL (8 mg) is given after Osserman’s Grading
60 seconds if necessary. Pre- and post- zz I: Ocular
procedure measurements of ptosis and/or zz II A: Mild generalized with slow progression
motility (Hess chart) are compared; the effect zz II B: Moderate generalized
lasts only 5 minutes. zz III: Acute fulminant MG
zz Cardiac monitoring for bradycardia and zz IV: late severe MG (takes 2 years to progress
asystole (Rx with atropine). from I to II).
zz 1 mg test dose and up to 10 mg.
zz In cholinergic crisis, will get increased DIFFERENTIAL DIAGNOSIS
salivation, etc.
zz Potential but uncommon complications zz Fourth nerve palsy
include bradycardia and death; resuscitation zz Third nerve palsy
facilities and appropriate expertise must be zz Progressive external ophthalmoplegia.
readily available on site in case of emergency,
and its use may be limited to cases in which TREATMENT
less invasive tests have given equivocal Emergencies in crisis (ABC)—treat exacerbating
results. factors, stop medications that can exacerbate, treat
zz Atropine 0.3 mg is given intravenously to fever with antipyretics, treat infections.
minimize muscarinic side effects. zz Oral pyridostigmine, neostigmine, steroids,
Electromyography shows characteristic azathioprine, cyclosporine
features. zz Plasmapheresis
Muscle biopsy reveals neuromuscular junction zz Intravenous immunoglobulin (IVIG).
antibodies and characteristic electron microscopy
features, but is not commonly performed. Ocular Myasthenia Gravis
Thyroid function testing should be performed
as autoimmune thyroid disease can be associated People with ocular MG and their caregivers
with MG. should balance the severity of the symptoms with
the risks and benefits of treatment. People who
have primarily cosmetic problems due to ptosis
Electrodiagnostic Studies or diplopia may consider no pharmacological
Repetitive nerve stimulation test—shows a treatment, such as:
decrease in the compound muscle action potential zz Wearing dark glasses in bright light, which
by 10% in the 4th or 5th response to a train of nerve some patients find helpful.
stimuli. zz Using eyelid tape (a special type of tape used
Single fiber nerve electromyography— to hold the eyelids open without injuring
evidence of neuromuscular blockade with the eyelids). This can be used for ptosis and
increased jitter. may be preferable to drug therapy that alters
the immune system using agents such as muscle tension while the eyes and face are
glucocorticoids (prednisone or similar agents), straight and may be performed to address
zz Azathioprine (Imuran), cyclosporine or a compensatory head posture. Recession of
mycophenolate mofetil (CellCept). all horizontal recti has been successful in
zz Applying a patch to one eye. This permits reducing the amplitude of nystagmus in some
patients with double vision to see one image. If patients without a significant null point.
the same eye is consistently patched, vision in zz When ptosis does not respond to medication,
that eye will decrease. Therefore, it is important or conservative treatment, surgery may be
to alternate the patch from one eye to the suggested to lift the eyelid or lids. Surgery is
other to avoid permanent vision loss. Another only done in very rare cases.
method is wearing one contact lens which is zz When ocular symptoms are severe or disabling,
opaque (cannot be seen through). This may treatment with immune system
also be changed periodically from one eye to zz Modulating therapy may be considered.
the other. Special prism glasses may also help zz Agents that improve neuromuscular transmis
to correct double vision in some cases. The use
sion, such as Mestinon may be helpful for
of non-allergenic tape such as paper tape is
ptosis, but are generally not very useful for
suggested.
diplopia.
zz Using eyelid crutches (clever devices attached
to glasses to hold the eyelids open) for ptosis
and eyeglass prisms for diplopia. These are Thymectomy
rarely used, older methods of treatment for
Thymectomy is usually not considered for people
ocular MG. Once again, alternating taping of
with ocular MG unless these treatments offer
eyelids is recommended to prevent eye strain.
only a temporary improvement and repeated
Another way to hold one or both eyelids open is
to have ptosis bars or eyelid crutches attached treatments are necessary to sustain the effect.
to the eyeglasses. These are thin, flexible wires While thymectomy (removal of the thymus
which attach at the bridge of the nose and are gland) is often recommended for patients with
free-floating at the other end or attached to the generalized MG, it is rarely used in purely ocular
frame near the hinges. The wires rest against MG unless a thymoma is suspected. The clinical
the eye socket and hold the eyelid open like a observations which distinguish ocular myasthenia
brace or crutch. It is important to remove the from generalized MG include: highly restrictive
glasses frequently to allow the eyes to close ocular symptoms such as diplopia, ptosis, and
so that they can be moistened. Artificial tear weak eye closure. The good news for patients
drops and forced blinking may also help. with ocular MG is that if they continue to have
zz Weakness of the muscles that control eye only ocular symptoms for three years, there is a
closure (orbicularis oculi) may result in the very good chance their symptoms will not increase.
patient getting soap in their eyes when bathing, The use of the above strategies can make it easier
and in excessive tearing due to incomplete to live with ocular MG.
blinking. The use of non-irritating shampoo,
such as baby shampoo, may be of help.
Swimming goggles may also be worn when VIVA QUESTIONS
bathing or swimming to prevent eye irritation.
zz Medication such as baclofen and gabapentin Q.1. How common is the thymus involved?
may be helpful. Ans. • 75% of cases of which 15% are thymomas
zz Botulinum toxin injection into the extraocular and 85% are thymic hyperplasia
muscles has had some success but can be • Myasthenia gravis may be ocular, bulbar
unpredictable and long-term treatment is (affecting the cranial nerves arising from
required. the lower brainstem) or generalized.
zz Surgery for nystagmus with a null point is Congenital and juvenile forms are rare.
aimed at moving muscles in order to mimic A similar clinical picture is found in the
Lambert–Eaton myasthenic syndrome the extra ocular muscle may cause diplopia
mediated by antibodies against pre and visual disturbances to occur. These
synaptic voltage gated calcium symptoms occur due to weakness of the
channels; in 60% this is a paraneoplastic muscle that control eyeball and eyelid
phenomenon associated with a lung movement. Light sensitivity due to sluggish
tumor. Patients positive for anti-MuSK pupils may occur in some patients.
(muscle-specific kinase) antibody may Q.4. What are the complications?
have a distinct form of MG. A range of Ans. • Myasthenic crisis
drugs can exacerbate MG, and should • Severe exacerbation of MG
be avoided if possible; those with • 10% require intubation
ophthalmic relevance include many • Treatment complications
antibiotics, and beta-blockers. • Cholinergic crisis.
Q.2. How common is the thyroid involved?
Q.5. What can exacerbate MG or precipitate
Ans. Up to 30% of patients with MG have
crisis?
antithyroid antibodies.
Ans. • Noncompliance to medications
Q.3. What are the common presentations? • Infection
Ans. • Age- 2 peaks. • Emotions
• 20 to 30 years old with female predomi • Drugs
nance overall, the ratio of affected • Antibiotics: aminoglycosides, tetracyc
females to males in generalized MG is lines, macrolides and fluoroquinolones
3:2 or higher. • C VS: Beta blockers, calcium channel
• >50 years old with male predominance. blockers (verapamil)
• Ptosis, diplopia • Others: Chloroquine, quinidine, pro
• D ysarthria, difficulty swallowing (iso caina m ide, Li, Mg, prednisolone,
lated bulbar muscles involvement occurs quinine, penicillamine. Symptoms are
in 20%). frequently influenced by environmental,
• G eneralized weakness or reduced emotional, and physical factors. Some
exercise tolerance. of these factors include bright sunlight,
• Respiratory failure in 1%. extreme temperature, emotional stress,
• Tends to occur extraocular muscles first, illness, surgery, menstruation, and
then to facial to bulbar and to limbs and pregnancy, among others. Symptoms
truncal. tend to be worse at the end of the day.
Over two-thirds of all patients with myas
thenia gravis (MG) begin with symptoms Q.6. Ocular involvement is seen in how many
relating to their vision. Overall, the ratio of patients of myasthenia gravis? What is
affected females to males in generalized MG the most common presenting ocular
is 3:2 or higher. In ocular myasthenia, men feature in it?
are more frequently affected, especially Ans. Ocular involvement occurs in 90% of
after the age of 40. In addition, the average cases and is the presenting feature in 60%.
age of onset for generalized myasthenia is Two-thirds of patients have both ptosis
33 years, while that of ocular MG is 38 years. and diplopia. Less than 10% of patients
The ocular motor system may be especially have ptosis alone and less than 30% have
vulnerable to MG since it cannot adapt diplopia alone.
rapidly to variable weakness. The most
common symptoms seen in patients with REFERENCE
ocular MG are diplopia (double vision), 1. Nair AG, Patil-Chhablani P, Venkatramani
ptosis (droopy eyelids), and incomplete DV, Gandhi RA. Ocular myasthenia gravis:
eye closure. Compared to other involved A review. Indian Journal of Ophthalmology.
skeletal muscles, only slight weakness of 2014;62(10):985-91.
CHAPTER
6
Lens
LONG CASES
ZONULAR CATARACT
Manpreet Kaur, Ashutosh Kumar Gupta, Jeewan S Titiyal
INTRODUCTION in the heat-shock transcription factor-4 gene

(HSF4) located at 16q21-q22.1.
Zonular cataract is the most common visually An environmental form of zonular cataract
significant variant of pediatric cataract. It may be may occur and is associated with vitamin D
congenital or occur at a later stage of development, deficiency. Occasionally, maternal rubella
and is characterized by an opacity which occupies infection contracted between 7th and 8th week
a discrete zone in the lens. It is usually bilateral, and of gestation may also cause zonular (lamellar)
has an autosomal dominant inheritance pattern. cataract.
It may be given as a long or short case in
Age at presentation: Patients with zonular cataract
postgraduate/DNB/Diploma examination.
usually present in early childhood, though
occasionally, they may present soon after birth.
HISTORY
Presenting features: Bilateral involvement is
Chief Complaints characteristic of zonular cataracts. Since the
The informants are usually the parents who notice patients are often in the pre-school age group, a
few or all of the following symptoms: definitive history of diminution of vision may be
difficult to elicit. The parents usually notice that the
zz Child does not recognize objects, toys or
child is unable to see toys placed nearby, stumbles
parents (diminution of vision)
often and does not recognize faces of familiar
zz White pupillary reflex (leukocoria)
persons. The parents may notice a white reflex or
zz Involuntary continuous rhythmic movements
leukocoria. In cases where there is an asymmetric
of the eye (nystagmus)
involvement of both eyes, strabismus may be the
zz Deviation of eye (Strabismus).
presenting complaint. Congenital cases may have
associated involuntary ocular movements due to
History of Present Illness impaired development of fixation.
Etiology and genetics: It is usually hereditary with Antenatal and perinatal history: A history of fever
an autosomal dominant genetic pattern. This type associated with rash may be present in the mother
of congenital cataract may be caused by mutations in the antenatal period, which may point towards
rubella as the causative etiology. It is essential to Eyeball: Strabismus may be present, usually
rule out maternal malnutrition and history of drug convergent squint. Nystagmus is present in cases
or toxin intake in the antenatal period. Recurrent with congenital onset of cataract
neonatal infections and malnutrition should be Eyelids, conjunctiva, cornea, sclera, iris, and
ruled out. pupil: Examination is usually unremarkable.
Family history: A positive family history of Microcornea may be present in cases with
congenital or developmental cataract is present. underlying systemic syndrome.
Intraocular pressure is usually normal.
EXAMINATION Lens: Typically, zonular cataract occurs in the
zone of fetal nucleus surrounding the embryonic
nucleus. The main mass of the lens internal and
Examination
external to the zone of cataract is clear, except
Zonular cataract is familial and usually not for small linear opacities like spokes of a wheel
associated with any underlying systemic disorder. (riders), which may be seen towards the equator
However, a detailed examination is essential (Figs 1 and 2).
to rule out systemic disorders that are commonly
Vitreous and fundus: Examination is usually
associated with bilateral congenital cataracts,
normal in bilateral zonular cataract. Salt and
such as Toxoplasmosis, Other Agents, Rubella,
Cytomegalovirus, and Herpes simplex (TORCH)
syndrome, galactosemia and various mutational
syndromes. The presence of microcephaly,
d e a f n e s s, c a rd i a c a b n o r m a l i t i e s a n d
developmental delay point towards an underlying
systemic etiology and warrants a need for further
investigations.
Ocular Examination
Visual acuity: Visual acuity may be difficult to
establish in very young children. Indirect evidence
of diminution of vision include:
zz Child does not follow objects or light
zz Inability to maintain central steady fixation Fig. 1: Zonular cataract with clear periphery and riders
zz Child resists occlusion of the good eye
zz Strabismus (usually convergent squint)
zz Nystagmus
Objective assessment of visual acuity may be
made by the following tests:
zz Infants: Preferential looking tests, Teller
acuity cards, Catford drum test, Optokinetic
nystagmus and visual-evoked responses
zz 1–2 years: Worth’s ivory ball test, Sheridan’s
ball test, Boek’s Candy test
zz 2–5 years: Dot acuity test, Miniature toy test,
Sheridan-Gardiner test (HOTV) test, Tumbling
E test, Allen picture cards, Beale-Collins
picture chart tests, Kay picture test, Landolt C
test, Snellen’s chart Fig. 2: Zonular cataract retroillumination
Lens 411
pepper retinopathy may be present in congenital zz Biometry: Axial length and keratometry
rubella syndrome. Persistent hyperplastic primary measurements for IOL power calculation
vitreous may be associated, especially in unilateral zz A-scan ultrasonography to give an estimate
cases. of axial length in infants and children
uncooperative for biometry
DIFFERENTIAL DIAGNOSIS zz B-scan ultrasonography: To rule out any
posterior segment pathology.
zz Persistent hyperplastic primary vitreous
zz Retinoblastoma
MANAGEMENT
zz Endophthalmitis
zz Retinal detachment Conservative Management
zz Toxocariasis
Partial cataracts, cataracts with less than 3 mm
zz Coat’s disease
diameter and pericentral cataracts may not
zz Retinopathy of prematurity
immediately require surgery and can be observed.
zz Astrocytic hamartoma
Pupillary dilatation with 2.5% phenylephrine and
zz Vitreous hemorrhage.
part time occlusion of good eye may be tried in
unilateral partial cataracts.
INVESTIGATIONS
Systemic Investigations Surgical Management
Zonular cataract with positive family history and Indications for Treatment
established hereditary basis for the cataract does
zz Cataract >3 mm diameter
not warrant any further investigation.
zz Dense nuclear cataract obstructing view of
In other cases with bilateral cataract, the
fundus
investigations should include the following:
zz Associated strabismus/nystagmus
zz Serology for intrauterine infections: TORCH
zz VA < 20/80.
titres (TORCH = toxoplasmosis, rubella,
cytomegalovirus, herpes simplex virus, other
viruses such as varicella), VDRL titres for Time of Surgical Intervention
syphilis. zz Bilateral dense cataracts require early
zz Urine examination: Urinalysis for the presence surgery within 6–8 weeks of age to prevent
of reducing sugars (galactosemia); urine the development of stimulus deprivation
chromatography for amino acids (Lowe’s amblyopia. In asymmetrical cataract, the eye
syndrome). with the denser cataract should be addressed
zz Serum electrolytes (serum calcium and first.
phosphorus) zz Unilateral dense cataract should be operated
zz Fasting blood glucose as soon as possible, ideally before 4–6
zz Serum galactokinase weeks of age. Results are often poor due to
zz Thyroid function tests dense amblyopia and non-compliance with
zz Referral to a pediatrician may be warranted occlusion therapy.
for dysmorphic features or suspicion of zz Bilateral partial cataracts may not require
other systemic diseases. Genetic testing and surgery until later, if at all. In case of doubt, it
chromosome analysis may be useful in this may be prudent to defer surgery. Monitor lens
context. opacities and visual function and intervene
later, if vision deteriorates.
zz Partial unilateral cataract can usually be
Ocular Investigations
observed or treated non-surgically with
zz Visual evoked responses (VER): To assess visual mydriasis, and possibly part-time contralateral
acuity and estimate visual potential occlusion to prevent amblyopia.
Biometry and IOL Power Calculation to minimize the risk of visual axis opacification
(VAO) and subsequent additional surgical
Accurate measurements of axial length and
procedures. In children older than 7 years, lens
keratometry may be difficult in the preoperative
aspiration with IOL implantation is performed.
period because of poor patient cooperation
Primary IOL implantation is preferred in all
and poor fixation. Usually, examination under
unilateral cataract cases as well as bilateral cases
anesthesia has to be performed to determine axial
when possible. In-the-bag implantation of single-
length and keratometry. Immersion biometry is
piece hydrophobic IOL is preferred. Multipiece
more predictable than the contact method for IOL-
IOL in the sulcus may be implanted when in-the-
power calculation.
bag IOL implantation is not possible.
An under-correction of the IOL power is
Wound closure by stromal hydration alone may
usually recommended in cases of pediatric
be inadequate because of low scleral rigidity, and
cataract to account for the myopic shift following
sutures are often required to effectively seal the
IOL implantation. Dahan et al. suggest an under-
corneal incisions.
correction of 10% in children between 2 to 8 years
and under-correction of 20% in children less than
2 years of age. They also suggested IOL power Postoperative Visual Rehabilitation
selection based on axial length alone (Table 1). Postoperative occlusion therapy and visual
In cases with unilateral cataract, emmetropia rehabilitation is essential to achieve optimal
may be aimed for to minimize the risk of amblyopia. outcomes, as postoperative amblyopia may limit
Piggyback IOL or IOL exchange may be needed at the visual potential in even a well-done cataract
a later date in such cases. surgery. Optical correction in an aphakic child
depends upon the patient age and laterality of
Surgical Procedure aphakia.
The primary surgical management consists of lens zz Aphakic glasses are effective for visual
aspiration or lensectomy. Coaxial or bimanual lens rehabilitation in older children with
aspiration via limbal route is preferred. Pars plana bilateral aphakia. However, they result in
lensectomy may be undertaken in cases where IOL unacceptable anisometropia and aniseikonia
implantation is not planned. in unilateral aphakia. Aphakic glasses are
Anterior capsulorhexis may pose a challenge heavy, cosmetically unattractive and result in
due to the elastic pediatric anterior capsule, which spherical as well as prismatic aberrations.
has a propensity to extend. Cohesive OVD such as zz Contact lenses provide a superior optical
Healon GV facilitates anterior capsulorhexis as it solution for both unilateral and bilateral
maintains anterior chamber stability and offsets aphakia. Tolerance is usually reasonable
the vitreous upthrust. until the age of about 2 years; problems with
Posterior continuous curvilinear capsulorhexis compliance may start after this period as the
(PCCC) with limited anterior vitrectomy is child becomes more active and independent.
recommended for children less than 7 years of age Contact lens may become dislodged or lost,
leading to periods of visual deprivation with
the risk of amblyopia. Maintenance of hygiene
Table 1 IOL power based on axial length may be problematic in young children, leading
(Dahan’s formula) to a risk of microbial keratitis. The maintenance
Axial length (mm) IOL power (D) issues and financial cost limits the widespread
17 mm 28 D usage of contact lenses.
zz IOL implantation is increasingly being
18 mm 27 D performed in young children and even
19 mm 26 D infants, and appears to be effective and safe in
20 mm 24 D selected cases. Awareness of the rate of myopic
shift, which occurs in the developing eye,
21 mm 22 D
combined with accurate biometry, allows the
Lens 413
calculation of an IOL power targeted at initial VIVA QUESTIONS

hypermetropia (correctable with spectacles),
which will ideally regress towards emmetropia Q.1. What is the etiology of congenital
later in life. However, final refraction is cataracts?
variable and emmetropia in adulthood cannot Ans. Etiology of bilateral cataracts:
be guaranteed. • Idiopathic
zz Occlusion therapy to prevent and treat • Familial (hereditary); usually autosomal
amblyopia is vital in order to achieve dominant
optimal outcomes, especially in cases that • Chromosomal abnormality—Trisomy-21
have undergone unilateral cataract surgery. ( D ow n ) , T r i s o my - 1 8 ( E d w a rd ) ,
Atropine penalization may also be considered. Trisomy-13 (Patau). Other translocations,
deletions, and duplications
Complications • Craniofacial syndromes: Hallermann-
Streiff, Rubinstein-Taybi, Smith-
zz Visual axis opacification (VAO) is nearly Lemli-Opitz.
universal, if the posterior capsule is retained • Musculoskeletal—Conradi, Albright,
in a child under the age of 6 years. An intact myotonic dystrophy
anterior hyaloid phase provides a scaffold for • Renal—Lowe, Alport
proliferation of lens epithelial cells and may • M etabolic—Galactosemia, Fabr y,
result in VAO despite posterior capsulorhexis. Wilson, mannosidosis, diabetes mellitus
The incidence of opacification is reduced • Maternal infection (TORCH diseases)—
when posterior capsulorhexis is combined Rubella, cytomegalovirus, varicella,
with vitrectomy. Surgical membranectomy syphilis, toxoplasmosis
via limbal or pars plana route is required for • O cular anomalies—Aniridia, Anterior
management. Nd: YAG capsulotomy may be segment dysgenesis syndrome
tried in cooperative children. • Iatrogenic—Corticosteroids, Radiation
zz Secondary membranes may form across the (may also be unilateral)
pupil, particularly in microphthalmic eyes Etiology of unilateral cataracts:
or those with associated chronic uveitis. • Idiopathic
A fibrinous postoperative uveitis in an • O cular anomalies—Persistent fetal
otherwise normal eye, unless vigorously vasculature (PFV), anterior segment
treated, may also result in membrane dysgenesis, retinal detachment
formation. Thin membranes can be treated • Traumatic (rule out child abuse)
with laser capsulotomy, thick membranes may
require surgical excision. Q.2. What are the contraindications for IOL
zz Secondary glaucoma may develop in 3–32% implantation in pediatric cataract?
of eyes undergoing pediatric cataract surgery. Ans. Contraindications of IOL implantation are:
Pupillary block glaucoma may occur in the • Microphthalmos
immediate postoperative period, especially • Rubella cataract
in microphthalmic eyes. Secondary open- • Aniridia
angle glaucoma may also develop years after • Uveitis
the initial surgery. It is therefore important to Q.3. What are the surgical challenges faced in
monitor the intraocular pressure regularly for pediatric cataracts?
many years. Ans. The intraoperative challenges faced in
zz Retinal detachment is an uncommon and pediatric cataracts are:
late complication after cataract surgery. The • Difficulty in capsulorhexis formation
incidence of retinal detachment following • Positive intravitreal pressure
cataract surgery has been reported between • Intraoperative miosis
1% and 1.5%. • Wound leak
Table 2 Morphological variants of pediatric cataract associated with systemic diseases

Systemic disease Cataract morphology Associated findings
Fabry syndrome Spoke-like Corneal whorls
Mannosidosis Spoke-like Hepatosplenomegaly
Diabetes Vacuoles ↑ Blood glucose level
Hypoparathyroidism Multicolor flecks ↓serum calcium
Myotonic dystrophy Multicolor flecks Characteristic facial features, tonic “grip”
Wilson disease Green “sunflower” Kayser-Fleischer corneal ring
Lowe syndrome Thin disciform Hypotonia, glaucoma
Q.4. What are characteristic morphological 2. Kugelberg M, Zetterström C. Pediatric cataract

variants of pediatric cataracts associated surgery with or without anterior vitrectomy.
with systemic diseases? J Cataract Refract Surg. 2002;28(10):1770-3.
Ans. Refer Table 2. 3. Lambert SR, Drack AV. Infantile cataracts. Surv
Ophthalmol. 1996;40:427-58.
Q.5. Explain IOL power calculations in 4. Medsinge A, Nischal KK. Pediatric cataract:
pediatric patients. challenges and future directions. Clinical
Ans. Refer text. Ophthalmology (Auckland, NZ). 2015;9:77-90.
5. Ram J, Brar GS, Kaushik S, Gupta A. Role
BIBLIOGRAPHY of posterior capsulotomy with vitrectomy
1. Bradford GM, Keech RV, Scott WE. Factors and intraocular lens design and material in
affecting visual outcome after surgery for reducing posterior capsule opacification after
bilateral congenital cataracts. Am J Ophthalmol. pediatric cataract surgery. J Cataract Refract
1994;117:58-64. Surg. 2003;29:1579-84.
ECTOPIA LENTIS
Ruchita Falera, Manpreet Kaur, Prafulla Kumar Maharana
INTRODUCTION Marfan’s syndrome is the most common heritable

cause of ectopia lentis. Disruption or dysfunction
Ectopia Lentis (subluxation of lens) is of the zonular fibers of the lens is the underlying
characterized by partial displacement of the pathophysiology of ectopia lentis, regardless of
lens from the patellar fossa. The first case of lens cause (trauma or heritable condition). The degree
dislocation was reported by Berryat in 1749 and of zonular impairment determines the degree of
the term Ectopia Lentis was coined by Stellwag in lens displacement.
1856.1 The terms ectopia lentis and subluxated lens It is given as a long or short case in post
have been used interchangeably in literature, it is graduate/DNB/Diploma examination.
however, preferable to use ectopia lentis in cases of
subluxation secondary to inheritable causes. Lens HISTORY
subluxation may be heritable without associated
systemic syndrome, heritable with associated Epidemiology
systemic syndrome, in association with ocular Ectopia lentis can occur at any age. It may be
comorbidities or because of trauma (Table 1). present at birth, or it may manifest late in life.
The most common cause of lens subluxation is A male preponderance is reported, as males
trauma, which accounts for nearly 50% of all cases. appear more prone to ocular trauma than
Lens 415
Table 1 Etiology of subluxated lens of Marfan’s syndrome include aortic and

pulmonary artery dilatation, mitral and
• Traumatic subluxation of lens tricuspid valve prolapse with or without
• Hereditary causes without systemic associations regurgitation. Dilatation of the sinus of
– Isolated ectopia lentis
Valsalva is found in 60–80% of adult and
– Ectopia lentis et pupillae
mitral valve prolapse (MVP) is present in 80%
• Hereditary causes with systemic associations
– Marfan’s syndrome patients.1,2
– Homocystinuria zz Homocystinuria is a recessively inherited
– Weill-Marchesani Syndrome disorder caused by deficiency of cystathionine
– Hyperlysinemia synthase leading to accumulation of
– Sulfite oxide deficiency homocysteine and methionine. Affected
– Ehlers-Danlos syndrome persons have tall, thin habitus similar to
– Crouzon’s syndrome Marfan’s syndrome patients but infrequent
– Oxycephaly arachnodactyly. Any history of developmental
• Associated with other ocular diseases delay or mental retardation should be
– Mature or hypermature cataract elicited as homocystinuria is associated with
– Buphthalmos subnormal intelligence.
– High myopia zz History of recurrent fractures, hip dislocation
– Megalocornea
– Pseudoexfoliation
or any musculoskeletal abnormalities
– Retinitis pigmentosa should be asked to rule out presence of any
– Eales disease connective tissue diseases such as Ehlers-
– Retinal detachment Danlos syndrome.
Family History
females. Isolated ectopia lentis and Marfan’s
syndrome have an autosomal dominant mode of A three generation pedigree chart must be
inheritance. prepared. History of similar complaints in siblings
and parents should be specifically asked.
Chief complaints: The patients may present with
the following symptoms:
EXAMINATION
zz Decreased or fluctuating vision
zz Photophobia Systemic Examination
zz Glare
zz Monocular diplopia A careful systemic examination must be carried
zz Suboptimal correction with spectacles out and the findings suggestive of underlying
systemic disorders must be noted.
History of present illness: Heritable ectopia lentis Marfan’s syndrome may present with the
is bilateral, symmetric and stable from early following systemic signs:
childhood. Time of onset of disease, its progression zz Long thin limbs
and its effect on visual function should be noted. zz Arachnodactyly-long spider like fingers
There is frequent change in glasses with a progressive zz Arm span: Height ratio > 1.05
increase in the power of glasses. Subluxation of lens zz Upper segment of body (Head to pubic bone):
associated with trauma can present with sudden Lower segment ratio <0.86
diminution of vision or other symptoms like zz Scoliosis
photophobia and monocular diplopia. zz Chest wall deformities (Pectus excavatum/
Past medical history: Ectopia lentis may be Pectus carinatum)
associated with underlying systemic disorders and zz Thumb sign (The thumb sign is positive when
a careful history must be elicited regarding the the entire distal phalanx of the adducted thumb
following: extends beyond the ulnar border of the palm)
zz Any history of previous cardiac illness must zz Wrist sign (The wrist sign is positive when the
be taken. Cardiovascular manifestations tip of the thumb covers the entire fingernail
of the fifth finger when wrapped around the Sclera: Thinning of sclera may be present, giving it
contralateral wrist.) a bluish hue in Marfan’s Disease as well as in cases
zz High-arched palate of connective tissue disorders such as osteogenesis
Musculoskeletal anomalies are also present in Imperfecta and Ehler-Danlos syndrome.
homocystinuria, Weill-Marchesani syndrome and Anterior chamber and angle:
connective tissue disorders. zz Ectopia lentis is usually associated with a deep
Cardiovascular examination should be anterior chamber, however, it may be irregular
performed to rule out underlying valvular heart zz Gonioscopy must be done in all cases to
defects in Marfan’s syndrome rule out angle recession (in post-traumatic
Homocystinuria may have associated mental subluxation of lens)
retardation and may necessitate IQ testing and
evaluation of higher mental functions. Pupil:
zz The pupil may be eccentrically placed as in
cases of ectopia lentis et pupillae where the
Ocular Examination
pupil is displaced opposite to the direction of
Visual acuity: Visual acuity should be carefully subluxation.
assessed considering the following points: zz The pupil may be poorly dilating on account of
zz Uncorrected visual acuity and BCVA must be hypoplastic dilator muscles
assessed in all cases zz Relation of the lens with respect to the
zz Near vision should be documented undilated pupil should be noted.
zz Refraction should be carried out through Lens:
phakic and aphakic zones (Fig. 1). zz Any evidence of phacodonesis should be
Eyeballs: Strabismus may be associated with documented
Marfan’s syndrome, and if uncorrected in children zz Evidence of cataract, if any should be
can result into amblyopia. It may be a presenting documented
sign of the disorder. Delayed and inadequate zz Extent of subluxation should be noted in clock
correction of refractive errors as well as deficient hours.
fibrillin in extraocular muscle pulleys causing zz Direction of subluxation (Fig. 2) must be
their instability may explain the high incidence of noted.
strabismus in Marfan’s patients. zz Zonules: Condition of the zonules should be
documented as they may be stretched in cases
Conjunctiva is usually normal.
of Marfan’s disease (Fig. 3) or broken in case of
Cornea: Patients with Marfan’s syndrome can homocystinuria
present with flat cornea (cornea plana) or steep The subluxation of the lens is generally
cornea. Associated keratoconus may be present. superotemporal in cases of Marfan’s syndrome.
Fig. 1: Phakic and aphakic zones in ectopia lentis Fig. 2: Superotemporal displacement
Lens 417
Preferential focusing of ultraviolet B light on Fundus examination:

the inferonasal quadrant of the crystalline lens zz Distant direct ophthalmoscopy examination:
is hypothesized to explain the predominantly A crescent-shaped reflex seen on distant
supero-temporal dislocation (Fig. 2) of the lens direct ophthalmoscope is pathognomic of
in Marfan syndrome, while it is inferonasal in subluxated lens.
cases of homocystinuria. However, the direction of zz Detailed fundus examination is mandatory
subluxation is not pathognomic, and it may occur in all cases. Retinal detachment may occur in
in any direction. Premature cataracts and other 5–11% of patients with Marfan’s syndrome,
lens and capsule opacities are commonly found in and its incidence increases to 8–38% in
Marfan’s syndrome with presentation at a younger the presence of ectopia lentis. 1 Unstable
age (30–50s) compared to the general population. subluxated or dislocated lens capsule exerts
In some cases, the patients may be aphakic traction on the vitreous base, leading to
with posterior dislocation of the lens into the small tears or holes in the retinal periphery.
vitreous cavity. Axial myopia presents in Marfan’s syndrome
Intraocular pressure: Intraocular pressure should predisposes to early vitreous liquefaction
be documented in all cases. Primary open-angle and posterior vitreous detachment, retinal
glaucoma is most common, but glaucoma can be thinning, lattice degeneration, and peripheral
secondary to anterior lens dislocation or anterior breaks.
chamber angle abnormalities.
Ectopia lentis is diagnosed clinically on the basis
of slit-lamp examination. Various etiologies
responsible for ectopia lentis are tabulated in
Table 1. Marfan’s syndrome and homocystinuria
are common heritable systemic disorders
associated with ectopia lentis and their
differentiating features are summarized in Table 2.
INVESTIGATIONS
Systemic Investigations
A case of ectopia lentis must be managed with
a multidisciplinary approach along with a
Fig. 3: Stretched zonules in cases of Marfan’s disease pediatrician, cardiologist and orthopedician.
Table 2 Differentiating features of Marfan’s disease and homocystinuria

Clinical features Marfan’s disease Homocystinuria
Etiology Fibrillin-1 (FBN1) gene Deficiency of cystathionine synthase leading to
mutation on chromosome 15 accumulation of homocysteine and methionine
Mode of inheritance Autosomal dominant Autosomal recessive
Mental retardation Absent Present
Direction of subluxation Superotemporal subluxation Inferonasal subluxation of lens
of lens
Condition of zonules Primarily present with Absent and broken zonules
stretched zonules
It should be evaluated for underlying systemic motion phacoemulsification with PCIOL

abnormalities. implantation in the bag can be attempted.
zz X-ray Chest/ECG/2D Echo should be done in Slow motion phacoemulsification includes
all cases to rule out cardiac abnormalities in phacoemulsification at low flow rate, low
Marfan’s syndrome. vacuum and low infusion bottle height in
zz Sodium nitroprusside test (in urine) must be order to minimize stress on the zonules
done to rule out homocystinuria. zz Subluxation of 3–5 clock hours: In case of lens
zz X-ray of spine and extremities are undertaken subluxation of 3–5 clock hours, slow motion
to evaluate the skeletal deformities phacoemulsification can be done. Insertion of
capsular tension ring (CTR) or capsule tension
Ocular Investigations segments (CTS) may be needed to support
the bag. With the use of CTR, any force that is
zz Biometry should be done to calculate IOL
transmitted to the capsule does not directly
power
impact the adjacent zonules, but is rather
zz B-scan ultrasonography: In cases of mature
distributed to the entire zonular apparatus.
cataract where the fundal view is obscured, a zz Subluxation of 5–7 clock hours: Slow motion
USG must be done to rule out retinal detachment
phacoemulsification with the use of Cionni ring
or any posterior segment pathology.
with PCIOL implantation can be attempted
in cases of severe or progressive zonular
MANAGEMENT weakness. Cionni ring has a hook, which
needs to be kept opposite to the direction of
Conservative Management decentration. A transcleral suture applied to
zz A complete refraction considering the it allows it to be pulled peripherally thereby
undilated central pupillary position, size of the counteracting the capsular bag decentration.
phakic and aphakic zones and preferred visual zz Subluxation of > 7 clock hours: Intracapsular
axis needs to be done. cataract extraction (ICCE) with sclera fixated
zz Appropriate spectacle correction, aphakic IOL or ACIOL implantation can be done in
glasses or contact lens can be given. cases with extensively subluxated cataractous
zz Other methods such as miotics to minimize lens.
diplopia or mydriatics to enlarge the aphakic zz In cases with significant posterior subluxation
zone are rarely used these days. of lens, pars plana lensectomy (PPL) with pars
plana vitrectomy (PPV) can be done, followed
Surgical Management by IOL implantation in the anterior chamber
or sulcus-fixated IOL.
Indications for Surgery The use of capsular support devices is not
zz Subluxated lens bisects the pupil leading to preferred in cases of progressive zonular weakness.
a phakic and aphakic zone in an undilated These are of particular importance in secondary
pupillary axis causes of subluxation such as post-traumatic
zz Cataractous lens subluxation of lens and pseudoexfoliation where
zz Associated complications like glaucoma or the basic zonular anatomy is not compromised.
pupillary block In heritable ectopia lentis with or without
zz Presence of lenticular astigmatism underlying systemic disorders, intralenticular
zz Anteriorly or posteriorly dislocated lens lens aspiration (Figs 4A to D) is the preferred
Surgical management of subluxated lens technique, which is described as follows:
depends on the degree of subluxation. The zz In this technique, two openings are made
following are the modalities of management are in the lens capsule with a microvitreoretinal
mainly for secondary causes of lens subluxation. (MVR) blade and the lens matter is aspirated
zz Subluxation < 3 clock hours: In cases of lens using a bimanual irrigation and aspiration
subluxation of less than 3 clock hours, slow system.
Lens 419
A B
C D
Figs 4A to D: Intralenticular lens aspiration
zz The capsular bag is then cut and aspirated rim fibroses, thus avoiding the complications
using a vitrectomy probe. and difficulties associated with anterior
This technique has several advantages such as: chamber and sulcus-fixated IOLs.
zz The lens can be stabilized with the irrigation
cannula, the area to be aspirated can be
brought into focus, and a complete lens VIVA QUESTIONS
aspiration can be easily performed.
zz Additionally, the irrigation cannula hydrates Q.1. What is the etiopathogenesis of Marfan’s
the cortical matter, enabling complete disease?
aspiration. Ans. Marfan’s syndrome is an autosomal
zz Furthermore, creating two small capsular dominant disorder with near complete
openings in the midperiphery of the lens penetrance and variable expression.
that are directly visible often eliminates the There is a mutation in the Fibrillin locus
problem of poor visibility. (FBN1), which lies on the long arm of
zz There is less chance of vitreous becoming chromosome 15 (15q21). This results in
hydrated and lens matter falling into the abnormal biosynthesis of fibrillin, a 350
vitreous cavity, as aspiration is intralenticular. kd cysteine rich glycoprotein which is a
zz Another added advantage is that the major constituent of microfibrils present in
capsular rim is left intact. This may allow IOL connective tissue of suspensory ligaments
implantation in the sulcus once the capsular of crystalline lens.
Q.2. What are the differences between • H ind foot deformity–2 (plain pes
Homocystinuria and Marfan’s disease? planus –1)
Ans. Refer Table 2. • Pneumothorax –2
• Dural ectasia –2
Q.3. Type of astigmatism seen in subluxation
• Protrusio acetabuli –2
of lens.
• R educed US/LS and increased arm/
Ans. Irregular/compound myopic astigmatism
height and no severe scoliosis –1
is seen due to following mechanism:
• Scoliosis or thoracolumbar kyphosis –1
• Weak zonules lead to relaxation of lens
• Reduced elbow extension –1
capsule (which is normally in a state of
• Facial features (3/5) –1 (dolichocephaly,
stretch by zonules) making the lens more
enophthalmos, down-slanting palpebral
spherical along the axis with increased lens
fissures, malar hyoplasia, retrognathia)
power and consequent myopia.
• Skin striae –1
• Area where zonules are still intact, the lens
• Myopia > 3 diopters –1
remains as such or may slightly bulge.
• Mitral valve prolapse (all types) –1
• Therefore, there is more myopia in one axis
Maximum total: 20 points; score ≥ 7
while less myopia in other axis.
indicates systemic involvement.
Q.4. What are the diagnostic criteria for
Marfan’s syndrome?
BIBLIOGRAPHY
Ans. Marfan’s Disease is diagnosed according to
Modified Ghent’s Criteria 1. Anteby I, Isaac M, BenEzra D. Hereditary
In absence of family history: subluxated lenses: Visual performances
• Aortic root dilation (Z score >2) and and long-term follow-up after surgery.
Ectopia lentis Ophthalmology. 2003;110:1344-8.
• Aortic root dilation and FBN1 mutation 2. Loeys BL, et al. Revised Ghent criteria for the
• Aortic root dilation and systemic score >7 diagnosis of Marfan syndrome (MFS) and
points related conditions. J Med Genet. 2010;47:476-85.
• Ectopia lentis and FBN1 with known 3. Nelson LB, Maumenee IH. Ectopia lentis. Surv
aortic root dilation Ophthalmol. 1982;27:143-60.
In presence of family history: 4. Nemet AY, Assia EI, Apple DJ, Barequet IS. Current
• E ctopia lentis and family history: concepts of ocular manifestations in Marfan
Marfan’s syndrome syndrome. Surv Ophthalmol. 2006;51:561-75.
• Systemic score (>7 points) and family 5. Rubin SE, Nelson LB. Ocular manifestations
history of Marfan’s syndrome of autosomal dominant systemic conditions.
• Aortic dilation and family history of Duane’s Clinical Ophthalmology on CD-ROM.
Marfan’s syndrome Vol. 3. Ch. 58. Philadelphia: Lippincott Williams
Systemic score: & Wilkins, 2006.
• Wrist and thumb sign –3 (Wrist or thumb 6. Sinha R, Sharma N, Vajpayee RB. Intralenticular
sign –1) bimanual irrigation: aspiration for subluxated
• Pectus carinatum deformity –2 (pectus lens in Marfan’s syndrome. J Cataract Refract
excavatum or chest asymmetry –1) Surg. 2005;31(7):1283-6.
Lens 421
SHORT CASES
LENTICONUS
Manpreet Kaur, Prafulla Kumar Maharana, Jeewan S Titiyal
INTRODUCTION Presenting features: Patients with anterior

lenticonus present with gradual progressive
Lenticonus is characterized by a localized conical diminution of vision in both eyes. It is not amenable
protrusion of the anterior or posterior lens to correction by either spectacles or contact lenses.
capsule and the underlying cortex. The anomaly Posterior lenticonus is unilateral and
is usually restricted to the axial area and can amblyopia is the most significant visual problem
reach a diameter of 2–7 mm. Posterior lenticonus associated with it. Amblyopia may be a result
is more common than anterior lenticonus and is of the optical distortion induced by the conical
usually unilateral and axial in location. Anterior protrusion of the lens surface, anisometropia or by
lenticonus is bilateral and usually associated with visual deprivation due to cataract.
Alport syndrome.
It is given as short case in postgraduate/DNB/ Associated ocular features: Anterior lenticonus
Diploma examination. may be associated with the following ocular
anomalies:
zz Dot-and-fleck retinopathy
HISTORY
zz Posterior polymorphous corneal dystrophy
Chief Complaints zz Temporal macular thinning
zz Subcapsular and cortical cataract.
zz Decrease in visual acuity
zz Minimal improvement with spectacle or Posterior lenticonus may be associated with
contact lenses. the following ocular manifestations:
zz Amblyopia
zz Subcapsular and cortical cataract
zz Strabismus
Epidemiology: Anterior lenticonus occurs zz Glaucoma (in Lowe syndrome).
bilaterally in patients with Alport syndrome, which Family history: A positive family history of
is a hereditary systemic disease with a prevalence hematuria, early onset deafness, and renal
of 1/5000 in a normal population. Alport syndrome insufficiency may be present, especially in male
is X-linked in 85%, autosomal recessive in 10% and patients with anterior lenticonus.
autosomal dominant in only a small fraction of the
patients.
Posterior lenticonus is a unilateral congenital EXAMINATION
defect that usually occurs in a sporadic manner,
with a prevalence of 1–4 per 100,000 children. Systemic Examination
It has no predilection for either sex. It may Alport syndrome is characterized by progressive
also occur in association with Lowe syndrome renal failure and sensorineural deafness
(oculocerebrorenal syndrome). in addition to anterior lenticonus. Gross or
Age at presentation: Anterior lenticonus manifests microscopic hematuria is the most common
before the age of 30 years, most commonly in the and earliest manifestation of Alport syndrome;
second decade. Posterior lenticonus is a congenital microscopic hematuria is observed in all males
anomaly and the age at diagnosis lies between and in 95% of females. Proteinuria develops in
3 and 7 years. males with X-linked Alport syndrome and in
males and females with autosomal recessive

Alport syndrome. It progresses with age and can
occur in the nephrotic range in as many as 30%
of patients. Hypertension is usually present in
males with X-linked Alport syndrome and in males
and females with autosomal recessive Alport
syndrome. Incidence and severity increases with
age and degree of renal failure.
Bilateral, high-frequency sensorineural
hearing loss begins by late childhood and is
present in approximately 50% of male patients
with X-linked disease by the age of 25 years, and
about 90% are deaf by the age of 40 years.
Posterior lenticonus may be associated with
Lowe syndrome (oculocerebrorenal syndrome). It Fig. 1: Transparent, localized, axial, sharply demarcated
is a rare X-linked recessive disorder characterized conical projection of the lens capsule and cortex in
anterior lenticonus. There is an increase in lens thickness
by congenital cataracts, hypotonia and areflexia,
intellectual disability, proximal tubular acidosis,
aminoaciduria, phosphaturia, and low-molecular-
weight proteinuria.
Ocular Examination
Eyeball: Strabismus may be present in cases
with posterior lenticonus. Eyeball shape and
movements are normal.
Lid: Eyelids are normal.
Conjunctiva: Conjunctival examination is normal.
Cornea: Posterior polymorphous corneal
dystrophy may be observed in cases with anterior Fig. 2: Oil-droplet reflex observed on retro-
lenticonus. Posterior lenticonus may be associated illumination in a case of anterior lenticonus
with microcornea. Fundus: A dot-and-fleck retinopathy may be
Sclera: Scleral examination is normal observed in cases with anterior lenticonus.
Iris: Iris examination is normal Temporal macular thinning may also be observed
in cases with anterior lenticonus.
Pupil: Pupils are normal
Retinoscopy: Scissoring reflexes are observed on
Intraocular pressure: Glaucoma may be present retinoscopy.
in 50% of cases with Lowe syndrome.
Lens: Transparent, localized, sharply demarcated DIFFERENTIAL DIAGNOSIS
conical projection of the lens capsule and cortex The diagnosis of lenticonus is clinical in nature and
is observed, usually axial in localization (Fig. 1). is easily confirmed on a slit-lamp examination.
There is an increase in lens thickness. The differences between anterior and posterior
‘Oil-droplet’ reflex is observed on retro- lenticonus are highlighted in Table 1.
illumination (Fig. 2). Anterior lenticonus is rare and bilateral, and
Associated subcapsular and cortical opacities may be associated with the following syndromes:
appear in advanced stages of lenticonus. zz Alport syndrome (most common association
Vitreous: Posterior lenticonus may be associated with anterior lenticonus)
with persistent hyperplastic primary vitreous. zz Waardenburg syndrome (rare)
Lens 423
Table 1 Differentiating features of anterior and posterior lenticonus

Clinical features Anterior lenticonus Posterior lenticonus
Laterality Bilateral Unilateral
Age at presentation Second decade 3–7 years
Etiology Associated with Alport • Usually sporadic
syndrome • May be associated with Lowe syndrome
Sex M>F M=F
Associated ocular • Posterior polymorphous • Amblyopia
features corneal dystrophy • Strabismus
• Cataract • Cataract
• Dot-and-Fleck retinopathy • Glaucoma (in Lowe syndrome)
• Temporal macular thinning
Associated systemic • Renal dysfunction • Usually none
features • Sensorineural hearing loss • Renal and cerebral manifestations in Lowe
syndrome
Management Lens aspiration with IOL • Lens aspiration with IOL implantation
implantation • Amblyopia therapy
Posterior lenticonus is more common and MANAGEMENT

unilateral. It may be associated with the following
conditions: zz Lens aspiration with in-the-bag intraocular
zz Sporadic (most common) lens implantation is the treatment of choice.
zz Associated with persistent hyperplastic zz Amblyopia management with occlusion
primary vitreous therapy is mandatory in cases with unilateral
zz Associated with hyaloid artery remnant posterior lenticonus to achieve optimal visual
zz Familial posterior lenticonus and microcornea outcomes.
zz Lowe syndrome (oculocerebrorenal syndrome)
zz Trauma
VIVA QUESTIONS
INVESTIGATIONS
Q.1. Describe the pathogenesis of anterior
Systemic investigations should be undertaken to lenticonus associated with Alport
evaluate for associated syndromes. syndrome.
zz Renal function tests Ans. Alport syndrome is caused by mutations
zz Urine examination: Hematuria and proteinuria affecting the gene that encodes for type
zz Audiometry: Evaluate sensorineural hearing IV collagen. Type IV collagen is present
loss in the basement membranes of the
glomerulus, cochlea, lens capsule and
Ocular Investigations cornea. Histopathologic examination of
zz A-scan ultrasonography may reveal an the anterior lens capsule shows thinning
increased lens thickness and vertical dehiscences. Initially, an
zz B-scan ultrasonography may show herniated increase of the lens thickness is observed
lenticular material, suggestive of a lenticonus with thin anterior capsule in the central
zz Aberrometry reveals lenticular astigmatism area, followed by progressive central
and aberrations protrusion of the capsule-cortex complex
zz Biometry to calculate intraocular lens power. and development of anterior lenticonus.
Q.2. Describe the pathogenesis of posterior In bilateral cases, a genetically determined

lenticonus. congenital weakness of the posterior lens
Ans. The pathogenesis of posterior lenticonus capsule may be the causative factor.
is unclear; traction on the posterior lens
capsule by remnants of the hyaloid artery
system as well as a disturbance in the tunica BIBLIOGRAPHY
vasculosa have been suggested as possible 1. Colville DJ, Savige J. Alport syndrome: a review
mechanisms. Vitritis or an overgrowth of of the ocular manifestations. Ophthalmic
posterior lens fibers that produce a phakoma Genetics. 1997;18:161-73.
of the lens have also been suggested as 2. Jacobs K, Meire FM. Lenticonus. Bull Soc Belge
possible pathophysiologic mechanisms. Ophtalmol. 2000;(277):65-70.
POSTERIOR POLAR CATARACT

Manpreet Kaur, Devika S Joshi, Jeewan S Titiyal, Sandeep Gupta
INTRODUCTION acuity is forward light scattering (light scattering

toward the retina). The diminution of vision is
Posterior polar cataract is a rare form of congenital progressive in nature.
cataract with incidence ranging from 3 to 5 in Patient profession must be noted carefully,
1000. It is bilateral in 65–80% of the cases. It poses since it may influence decision making before
a surgical challenge due to its fragile posterior cataract surgery.
capsule and an increased risk of posterior capsule
rupture with vitreous loss.
EXAMINATION
Diploma examination. General Examination/Specific Systemic
Examination
HISTORY
Posterior polar cataract may be associated with
Chief complaints: The patient presents with the number of systemic disease such as:
following complaints: zz Psychosomatic disorders
zz Diminution of vision for distance and near zz Ectodermal dysplasia
zz Glare, especially during night driving zz Rothmund disease
zz Intolerance to bright light. zz Scleroderma
zz Incontinentia pigmenti
History of Present Illness zz Congenital dyskeratosis
zz Congenital ichthyosis
Epidemiology and genetics: Posterior polar cataract
zz Congenital atrophy of the skin.
has an autosomal dominant inheritance pattern,
although it may occasionally be sporadic. Positive
family history may be present in 40–55% of the Ocular Examination
patients. There is no gender predilection and the zz Eyeballs: Microphthalmia may be an associated
disease is usually bilateral in nature. feature
Presenting features: Early cases may present zz Eyelids are usually normal.
with complaints of glare and reduced vision in zz Conjunctiva is usually normal
bright light. There is a difficulty in near work and zz Cornea: Microcornea may be present
intolerance to bright light. The cause of glare, zz Sclera, iris and pupil are usually normal
reduced contrast sensitivity and decreased visual zz Intraocular pressure is normal.
Lens 425
Lens: Dense, circular plaque is present in the central zz Posterior lenticonus: There is a conical
posterior part of the lens giving rise to the classic protrusion of the posterior capsule and
Bull’s-eye appearance. Concentric rings of opacity underlying cortex, which may or may not be
are present around the central opacity (onion associated with cataract.
peeling) (Fig. 1). It can be surrounded by vacuoles
and smaller areas of degenerated lens material. CLASSIFICATION
Co-existent nuclear sclerosis may be present
Three different classification systems have been
in advanced cases.
described as:
In cases where the other eye has already
undergone cataract surgery, it is important to
Duke Elder Classification
examine the posterior capsule status of the fellow
eye carefully. This is especially important where the zz Stationary form (most common—accounts
cataract is in an advanced stage and it is difficult to for 65% of cases): Well-circumscribed circular
rule out posterior polar cataract. In presence of any opacity, localized on the central posterior
sign of posterior capsule rent in the operated eye capsule. The concentric thickened rings
(such as posterior capsular rupture, decentered around the central plaque opacity give an
IOL, vitreous strands, pupillary peaking, IOL in appearance of a Bull’s eye. The opacity may
sulcus, etc.), the cataract in the other eye must be camouflaged by nuclear sclerosis or
be suspected as posterior polar cataract and all smaller satellite rosette lesions may be present
precautions during surgery must be taken. adjacent to the central opacity.
zz Progressive form: Whitish opacification
Vitreous: Examination of the anterior vitreous may
changes take place in the posterior cortex in
reveal oil-like droplets or particles. The presence
the form of a radiating rider opacity. It has
of such finding should raise the possibility of pre-
feathery and scalloped edges but they do not
existing capsular opening (Fig. 2). This is also
involve the nucleus, and does not extend as far
known as ‘fish-tailing’.
anteriorly as the original opacity.
Fundus: Examination is usually normal; signs of
incontinentia pigmenti may be present, if associated. Singh Classification
zz Type 1: Posterior polar opacity is associated
DIFFERENTIAL DIAGNOSIS with posterior subcapsular cataract.
zz Posterior subcapsular cataract : There is zz Type 2: Sharply defined round or oval opacity
generally a clear space between the posterior with ringed appearance such as an onion with
subcapsular cataract and the posterior capsule or without grayish spots at the edge.
Fig. 1: Posterior polar cataract with concentric rings of Fig. 2: Pre-existing posterior capsular defect in a case
opacity (onion-peeling or Bull’s eye appearance) of posterior polar cataract with whitish particles in
anterior vitreous
recovery. In addition, the possibility of leaving

the patient aphakic should be explained. Also, the
need for Nd:YAG capsulotomy for residual plaque
should be discussed.
Surgery in posterior polar cataract: Posterior
polar cataract poses a unique surgical challenge,
with a high incidence of posterior capsular
rupture ranging from 8% to 36%. Various
techniques have been described to avoid
intraoperative complications in posterior polar
cataract, such as inside-out phacoemulsification,
slow motion phacoemulsification, bimanual
microphacoemulsification, viscodissection,
Fig. 3: Disc-like opacity in the posterior capsule on pre-surround division technique and layer-
retroillumination by-layer phacoemulsification, etc. The basic
surgical principle governing these techniques is
avoidance of hydrodissection, and attempting to
create a cushion of cortical matter with careful
zz Type 3: Sharply defined round or oval white hydrodelineation. The aim is to avoid stress on the
opacity with dense white spots at the edge compromised posterior capsule during surgery.
often associated with thin or absent posterior
Capsulorhexis: Aim is to keep an adequate sulcus
capsule. These dense white spots are a
(approximately around 5 mm) so that the IOL can
diagnostic sign (Daljit Singh sign) of posterior
be placed in sulcus in case of PCR.
capsule leakage with or without repair and
Hydrodissection should be avoided as posterior
extreme fragility.
polar opacities adhere firmly to the posterior
zz Type 4: Combination of the above 3 types with
capsule around the opacity. Hydrodissection may
nuclear sclerosis.
result in rupture of the thinned posterior capsule
or widen any congenital capsular opening. If at all
Schroeder Classification it is done, it should be performed gently in multiple
Based on the effect of opacity on pupillary quadrants with minimal fluid. A fluid wave should
obstruction in the red reflex testing not be allowed to pass across the posterior capsule
zz Grade 1: Small opacity without any effect on in the center where the posterior capsule is weak.
the optical quality of the clear part of the lens Hydrodelineation is also performed with
(Fig. 3). minimal fluid. Careful hydrodelineation is done
zz Grade 2: Two-thirds obstruction without other to achieve multiple planes of separation (seen as
effects. multiple golden rings in co-axial illumination) so
zz Grade 3: Disc-like opacity in the posterior that layer-by-layer nuclear aspiration can be done.
capsule is surrounded by an area of further Not more than 0.2 cc of irrigating fluid should be
optical distortion. Only the dilated pupil shows used for hydrodissection and hydrodelineation.
a clear red reflex surrounding this zone. Phacoemulsification parameters: Slow motion
zz Grade 4: Opacity is totally occlusive; no phacoemulsification with low parameters should
sufficient red reflex is obtained by dilation of be used in cases with posterior polar cataract. The
the pupil. power should be 60%, bottle height 55–70 cm,
aspiration rate 15–25 mL/min, vacuum 30–100 mm
MANAGEMENT Hg. The low vacuum and aspiration rates help to
Preoperative counseling: It is essential to inform maintain a very stable chamber and the reduced
the patient of the possibility of a PC rupture, a infusion drives less fluid around the lens.
relatively long-operative time, secondary posterior Nucleus emulsification: A fine chopper should be
segment intervention, and a delayed visual used to incise the endonucleus in perpendicular
Lens 427
meridians, dividing the nucleus into small VIVA QUESTIONS

quadrants. This should be done without counter-
traction and the quadrants are then emulsified. Q.1. Describe the pathogenesis of posterior
Epinucleus removal: Epinucleus can be effectively polar cataract.
removed using viscodissection. Ophthalmic Ans. The developing lens requires nutrition that
viscoelastic device (OVD) is injected under is obtained through the tunica vasculosa
the anterior capsular margin in one quadrant lentis (TVL), which is a vascular network,
to elevate the epinucleus, which can then be supplied posteriorly by the hyaloid artery,
aspirated using irrigation/aspiration (I/A) a branch of the primary dorsal ophthalmic
handpiece. The peripheral cortex is aspirated artery, and anteriorly from an anastomosis
first using I/A handpiece. To avoid fluctuations with vessels in the pupillary membrane.
in anterior chamber depth, an attempt should be It has been suggested that posterior polar
made to keep the I/A tip always occluded. The cataracts are caused by persistence of the
central posterior portion of cortex is elevated with hyaloid artery or invasion of the lens by
viscoelastic and aspirated. mesoblastic tissue. The genetic mutation
is expressed as an abnormality in lens
Adequate anterior chamber stability: It is provided
development, specifically in the lens fibers
by the low-infusion and low-vacuum system.
that fail to develop normally and form an
Biaxial microincisional phacoemulsification may
opacity close to and sometimes adherent
be used to enhance safety and reduce risk of
to the posterior. Posterior polar cataract
complications. Anterior chamber should not be
forms during embryonic life or early in
allowed to collapse at any step during surgery.
infancy and usually becomes symptomatic
Residual plaque: Sometimes, the opacity may 30–50 years later.
come off spontaneously due to infusion pressure
Q.2. What is the inheritance pattern of
during removal of the peripheral cortex. However,
posterior polar cataract?
in some cases, part of the opacity may be firmly
Ans. Refer text.
adherent to the posterior capsule and may not be
separated. In such cases, residual plaque can be left Q.3. Classify posterior polar cataract.
in situ and later removed by Nd:YAG capsulotomy. Ans. Refer text.
Management of posterior capsular defect (pre-
existing/iatrogenic): If a defect is present in BIBLIOGRAPHY
the posterior capsule, a dispersive OVD such as
Viscoat should be injected over the area of defect 1. Duke-Elder S. Congenital deformities. Part 2.
Normal and Abnormal Development. System
before withdrawing the phaco or I/A probe from
of Ophthalmology; Vol. III. St. Louis: CV Mosby;
the eye. Convert the posterior capsular defect
1964.
into posterior capsulorhexis followed by anterior 2. Kalantan H. Posterior polar cataract: A review.
vitrectomy, if necessary. IOL can be implanted in Saudi Journal of Ophthalmology. 2012;26(1):41-
the bag in cases with a small PCR. If in-the-bag IOL 9. doi:10.1016/j.sjopt. 2011.05. 001.
implantation is not possible, sulcus implantation 3. Lee MW, Lee YC. Phacoemulsification of
of a multipiece IOL may be done followed by optic posterior polar cataracts: a surgical challenge.
capture in the bag, if needed. Br J Ophthalmol. 2003;87:1426-7.
MICROSPHEROPHAKIA
Manpreet Kaur, Devika S Joshi, Prafulla Kumar Maharana
INTRODUCTION acute onset diminution of vision associated with

pain and circumciliary congestion.
Microspherophakia is a developmental abnormal
ity and is characterized by a crystalline lens,
which has a small diameter and spherical shape.
EXAMINATION
The entire lens diameter can be characteristically General Examination/Specific Systemic
visualized during slit-lamp examination with a
Examination
fully dilated pupil.
It is given as short case in postgraduate/DNB/ Systemic examination should be undertaken to
Diploma examination. evaluate the manifestations of the associated
syndromes.
HISTORY Features of Weill-Marchesani syndrome
include short fingers (i.e. brachydactyly) and
Chief complaint : The patient with micro muscular hypertrophy. Marfan’s syndrome is
spherophakia presents with the following features associated with arachnodactyly, tall stature, high-
zz Diminution of vision arched palate and cardiac valvular anomalies.
zz Acute painful red eye with diminution of vision Homocystinuria, mandibulofacial dysostosis,
(acute angle-closure episode) Alport’s syndrome and Klinefelter’s syndrome may
also rarely be associated with microspherophakia.
Genetics: Isolated spherophakia is an autosomal Ocular Examination
recessive disorder resulting from homozygous The eyeballs and eyelids are usually normal.
mutations in LTBP2 (13q24.1-q32.12). Parental
Conjunctiva: Circumciliary congestion may be
consanguinity was present in reported families.
present in cases presenting with acute angle-
Microspherophakia is a clinically and
closure episode
genetically heterogeneous disorder and usually
found in association with Weill-Marchesani Cornea: is usually normal. Corneal opacity is
syndrome. Other syndromes that may be usually present in Peter’s anomaly
associated include Marfan’s syndrome, Peter’s Sclera: Scleral thinning and posterior staphyloma
anomaly, Alport syndrome, Lowe syndrome, is present in Marfan’s syndrome
homocystinuria, mandibulofacial dysostosis, and
Anterior chamber is shallow
Klinefelter’s syndrome.
Iris and pupil: Sphincter dysplasia may be present
Etiopathogenesis: Microspherophakia is a result
and pupils may be ectopic
of the faulty development of the secondary lens
Intraocular pressure is raised in cases of
fibers during embryogenesis. The underdeveloped
pupillary block.
zonules of Zinn are unable to exert enough force
on the lens to make it form the usual oval shape. Lens: There is a decrease in the equatorial lens
diameter and the whole lens is visible with full
Presenting features: The disease is bilateral in
mydriasis (Fig. 1). The anteroposterior lens
nature. The patient presents in childhood with
diameter is increased and the lens assumes a
diminution of vision and the use of high minus
relatively spherical shape. The lens may move with
lenses as a result of the lenticular myopia. The lens
changes in posture, and may dislocate or subluxate
may subluxate into the vitreous cavity in advanced
into the anterior chamber or vitreous.
cases. Secondary angle-closure glaucoma may
occur because of pupillary block induced by the Vitreous: Posteriorly dislocated lens may be
spherical lens. The pupillary block manifests as an visible in the vitreous cavity in advanced cases.
Lens 429
MANAGEMENT
Management of Lens
Clear lens extraction is the treatment of choice for
myopia and glaucoma in microspherophakia.
Indications for Lens Extraction

zz Cataract
zz Corneolenticular touch
zz High myopia
zz Intermittent pupillary block
zz Secondary glaucoma.
Fig. 1: Whole lens is visible with full mydriasis in a case Surgical Approach
of microspherophakia
zz Lens aspiration via limbal route
Fundus: Glaucomatous optic nerve head cupping zz Pars-plana lensectomy.
may be visible in cases with co-existent secondary
glaucoma. Posterior staphyloma, myopic crescent Surgical Challenges
and retinal detachment may be present.
Capsulorhexis: Iris hooks may be needed to
Refraction: High myopia is present without an stabilize the lens.
increase in axial length.
Intraocular Lens Implantation
DIFFERENTIAL DIAGNOSIS zz Successful in-the-bag implantation of acrylic
The lens findings on slit-lamp examination are single piece hydrophobic lens has been
pathognomic and characteristic of microsphero- described; however, phacodonesis persists in
phakia. The differential diagnoses for the etiology the postoperative period.
of microspherophakia are described in Table 1. zz Capsular support using a modified capsular
tension ring (M-CTR) and capsular tension
INVESTIGATIONS segment (CTS) sutured to the sclera along with
implantation of a foldable intraocular lens
Systemic Investigations inside the bag may be tried.
Systemic investigations are directed towards the
zz Scleral fixated IOL in the same sitting or a
underlying syndrome, and may include X-rays of second sitting can be done in cases without
spine and extremities, cardiac ECHO and genetic capsular bag support.
testing as needed.
zz Aphakic glasses or contact lens may be
prescribed in cases where IOL is not implanted.
Ocular Investigations
Management of Glaucoma
Specular microscopy: For endothelial cell count,
especially in cases with co-existent glaucoma. The pupillary-block glaucoma associated with
microspherophakia is also known as inverse
Perimetry: To evaluate visual fields in cases with glaucoma as miotics aggravate the condition by
secondary glaucoma. stimulating ciliary muscle contraction, thereby
Biometry: Axial length and keratometry for IOL loosening the zonules and further increasing
power calculations. anterior lens displacement. Mydriatics tighten the
zonules and are the preferred treatment.
B-scan ultrasonography: To evaluate cases with
posterior staphyloma or posteriorly dislocated Nd: YAG laser peripheral iridotomy is useful
lens. in relieving angle-closure glaucoma and
430
Table 1 Differential diagnoses for the etiology of microspherophakia

Familial Weill-Marchesani Marfan’s Klinefelter’s Alport’s Lowe
Features microspherophakia syndrome syndrome Peter’s anomaly syndrome syndrome syndrome
Inheritance Autosomal recessive Autosomal Autosomal Autosomal X-Linked X-linked X-linked
recessive, rare dominant dominant, (Aneuploidy) recessive
autosomal autosomal
dominant recessive
Other ocular fea- Ectopia lentis, Ectopia lentis Ectopia lentis, Anterior Microphthalmia, Lenticonus, Congenital
Ophthalmology Clinics for Postgraduates
tures (in addition lenticular (displaces glaucoma, segment colobomas of posterior cataract,
to microsphero- myopia, posterior inferiorly), axial myopia, dysgenesis the iris, choroid polymorphous glaucoma
phakia) staphyloma, posterior retinal (corneal and optic nerve, corneal
ectopic pupil, synechiae, detachment, opacity, strabismus dystrophy,
retinal detachment, glaucoma blue sclera, iris glaucoma, dot-and-fleck
glaucoma hypoplasia sclerocornea, retinopathy,
corectopia, iris temporal macular
hypoplasia, thinning
cataract)
Systemic features None Short stubby Cardiac and Developmental Tall stature, Renal Central
fingers musculo- delay, gynecomastia, dysfunction, nervous
(brachydactyly), skeletal dysmorphic small testes, and sensorineural system
short stature, anomalies facial features, infertility hearing loss and renal
broad hands, cardiac, anomalies,
joint stiffness genitourinary, hypotonia
and central
nervous system
malformation
Lens 431
should be done prophylactically in all cases of tension in the rudimentary zonular fibers
microspherophakia. of the lens, which arrests development so
Raised IOP may be managed with topical and that the lens remains spherical.
oral anti-glaucoma medications; trabeculectomy Q.2. What is inverse glaucoma?
may be required in chronic cases refractory to Ans. Refer text.
conservative medical therapy.
Q.3. What are the syndromes associated with
microspherophakia
VIVA QUESTIONS Ans. Refer Table 1.
Q.1. Describe the pathogenesis of micro- BIBLIOGRAPHY

spherophakia.
Ans. An arrest of development of the 1. Bhattacharjee H, Bhattacharjee K, Medhi
secondary lens fibers or the insertion of J, DasGupta S. Clear lens extraction and
intraocular lens implantation in a case
abnormally thin secondary fibers are said
of microspherophakia with secondary
to be responsible for the development
angle-closure glaucoma. Indian Journal
of microspherophakia. Both may be of Ophthalmology. 2010;58(1):67-70. doi:
secondary to a nutritional deficiency from 10.4103/0301-4738.58477.
defects in the tunica vasculosa lentis and 2. Chan RT, Collin HB. Microspherophakia. Clin
occur at the 5–6 months of embryonic life Exp Optom. 2002;85(5):294-9.
when the lens is normally spherical. 3. Willoughby CE, Wishart PK. Lensectomy in the
An alternative theory suggests that management of glaucoma in spherophakia.
microspherophakia is caused by a lack of J Cataract Refract Surg. 2002;28:1061-4.
POSTERIOR CAPSULAR OPACIFICATION

Prafulla Kumar Maharana, Manpreet Kaur
INTRODUCTION The interval between surgery and PCO ranges from

three months to four years after the surgery. There
Posterior capsular opacification (PCO) or after- is an inverse correlation with age and young age is
cataract is the most common complication of a significant risk factor for PCO. There is a history
cataract surgery with an incidence of 2–63% three of good gain in visual acuity following cataract
years after phacoemulsification. It is a major surgery, following which there is a gradual,
cause of diminution of vision in the postoperative painless, progressive diminution of vision. In early
period after an uneventful extracapsular cataract cases, the best corrected visual acuity may be
surgery. optimal but the patient may complain of glare.
It may be given as a short case in postgraduate/
DNB/Diploma examination.
EXAMINATION
HISTORY General Examination/Specific Systemic
Chief complaint: The patient presents with the
Examination
following complaints: Systemic examination is usually unremarkable.
zz Diminution of vision
zz Glare. Ocular Examination
Visual acuity: There is a decrease in the uncorrected
History of Present Illness as well as best spectacle corrected visual acuity
The patient presents few months to few years after (BSCVA), both for distance and near. Refraction
undergoing an extracapsular cataract extraction. must be done in all cases to find out the BSCVA.
The eyeballs and eyelids are usually normal.

Conjunctiva is usually normal. Conjunctival scars
may be present in cases that have undergone
ECCE or SICS.
Cornea: Scars of previous cataract surgery
may be present. Sutures or suture marks may be
present.
Sclera: Scleral incision may be visible in cases that
have undergone SICS.
Iris: Usually normal; however, in complicated
cases iris atrophy, chafing, peripheral anterior
synechiae or capsuloiridic adhesion must be
noted carefully Fig. 1: Posterior capsular opacification fibrotic type
Pupil: Usually normal; however, in complicated
cases abnormality in shape and size may be there.
In cases with dense PCO, pupillary reflexes must
be noted carefully to rule out relative afferent
pupillary defect (RAPD) to determine the visual
prognosis.
Intraocular pressure is usually normal. Following
Nd: YAG laser capsulotomy, a transient rise in IOP
often occurs. So any raised IOP or glaucoma must
be controlled with medication before proceeding
for capsulotomy.
Lens: Intraocular lens is present in the bag, or rarely
in the sulcus. Any IOL decentration, adhesion
between anterior capsular margin and posterior
Fig. 2: Soemmering’s ring
capsule or between iris and posterior capsule must
be noted carefully as these clinical conditions
increases the risk of early PCO formation. DIFFERENTIAL DIAGNOSIS
Posterior capsular opacification is visible
behind the lens. Different morphological variants Localized Endophthalmitis
of PCO may be observed, such as fibrotic type Propionibacterium acnes may induce a chronic
(Fig. 1), Elschnig pearls and Soemmering’s ring endophthalmitis with low-grade inflammation that
(Fig. 2). may mimic a PCO. The bacteria are sequestered
Vitreous: Usually normal; may not be clearly within the capsular bag and form whitish
visible as a result of media haze induced by precipitates and plaques. Laser capsulotomy is
PCO. contraindicated in such cases to avoid dispersion
of the infective material into the vitreous cavity.
Fundus: A thorough fundus examination is
essential to rule out any retinal tear/hole/traction,
since Nd: YAG laser capsulotomy increases the risk Cell Precipitates and Membranes
of retinal detachment. Cystoid macular edema Breakdown of the blood-aqueous barrier as a
must be ruled out as the inflammation induced result of cataract surgery may lead to inflammation
by laser capsulotomy predisposes towards and the aqueous dispersion of erythrocytes,
the development of macular edema. Indirect chronic inflammatory cells such as macrophages
ophthalmoscopy must be done in all cases. and giant cells as well as protein, pigments and
Lens 433
fibrin. They may form thin, translucent diffuse or Table 1 Sellman and Lindstrom posterior
punctate opacities that may mimic PCO. Increased capsule opacification (PCO) grades
frequency of topical steroid instillation may help
resolve such inflammatory membranes. Grade Definition
1 No or slight PCO without reduced red
Capsular Bag Distention Syndrome reflex, also no pearls at all or pearls not to
the IOL edge
Capsular bag distention syndrome (CBDS)
is a complication of continuous curvilinear 2 Mild PCO reducing the red reflex, Elschnig
pearls to the IOL edge
capsulorhexis done in phacoemulsification and
in the bag IOL implantation. It usually presents 3 Moderate fibrosis or Elschnig pearls inside
in the immediate postoperative period, with IOL edge but with a clear visual axis
shallowing of the anterior chamber, unexpected 4 Severe fibrosis or Elschnig pearls covering
myopic refraction and accumulation of liquefied the visual axis and severely reducing the
substance between the implanted lens and red reflex
posterior capsule. It may rarely present many years
after surgery with reduced vision but no significant 2. Fibrotic PCO: In fibrotic PCO, lens epithe
refractive change. The management consists of Nd: lial cells of the anterior capsule undergo
YAG capsulotomy or capsular bag lavage. transformation to myofibroblasts, causing
fibrosis and contraction of the capsule
INVESTIGATIONS bag. This can lead to decentration of
the IOL and hinder visualization of the
zz B-scan ultrasonography: To rule out any peripheral retina.
posterior segment complications. Presence zz Sellman and Lindstrom has described four
of PVD is a good sign before proceeding for grades of posterior capsule opacification
laser capsulotomy since risk of traction and (Table 1).
subsequent retinal detachment is less. It also
helps to rule out low-grade endophthalmitis,
especially P. acnes endophthalmitis.
MANAGEMENT
zz Pentacam: Not done routinely. Scheimpflug Conservative Management
imaging may be useful to document a distended Cases with mild PCO that achieve optimal visual
capsular bag with fluid accumulation behind acuity with refraction may be observed and kept
the lens in CBDS. on regular follow-up. Refraction with prescription
of glasses may be adequate in such cases.
CLASSIFICATION
Clinically, PCO can be divided into two types:
zz
Nd: YAG Laser Capsulotomy
1. Regeneratory PCO: Regeneratory PCO is a zz Indications
result of migration of lens epithelial cells —— Interference with daily activities
along the posterior capsule, behind the —— Decreased vision
IOL. These cells (also known as bladder —— Increased glare
cells due to their appearance) proliferate —— Difficulty visualizing the fundus
to form layers of lens material and zz Preoperative preparation: Before beginning

Elschnig pearls, leading to opacification. the capsulotomy, informed consent
When arranged in a form of ring (between should be obtained. One hour before the
anterior capsular rim and posterior laser capsulotomy, a drop of a pressure-
capsule), it is called Soemmerring’s ring lowering drug such as apraclonidine may
(Fig. 2). It is more common and is one be administered along with a mydriatic to
of the important cause of a decrease in dilate the pupil. Topical anesthesia drops are
visual function after cataract surgery. administered.
zz Procedure/laser parameters: An Abraham YAG Q.2. Describe the pathogenesis of PCO.

capsulotomy lens is used in conjunction with Ans. The development of PCO is a very
a coupling agent, such as 2% hydroxypropyl dynamic process, and involves three basic
methylcellulose. Laser spots are applied in a phenomena: proliferation, migration, and
cruciate, circular, inverted U or ‘Christmas-tree’ differentiation of residual lens epithelial
pattern. Cruciate pattern is most commonly cells (LECs). LECs left behind in the
used. The capsulotomy should be centred on capsular bag after cataract surgery convert
the visual axis with diameter slightly larger from epithelial to mesenchymal cells,
than the mesopic pupil size. Laser energy is set deposit collagen and generate lens fibers,
at 1-3 mJ and posterior offset of laser beam is leading to PCO development.
set at 125–150 microns to avoid hitting the lens.
Q.3. What do you mean by ‘A’ and ‘E’ cells?
zz Post-laser medication: After the procedure,
Ans. The anterior-central zone (corresponding
topical anti-glaucoma medications may be
to the zone of the anterior lens capsule)
prescribed for a week along with topical
consists of a monolayer of flat cuboidal,
steroids to reduce inflammation.
epithelial cells with minimal mitotic
activity. In response to a variety of stimuli,
Membranectomy
these anterior epithelial cells (‘A’ cells)
Surgical membranectomy may be indicated in the proliferate and undergo fibrous metaplasia.
following situations: Continuation of anterior lens cells around
zz Pediatric patients the equator form the equatorial lens bow
zz Uncooperative or mentally challenged patients (‘E’ cells). Unlike within the A-cell layer,
zz Dense fibrotic PCO. cell mitoses, division and multiplication
are quite active in this region, and new lens
Surgical Approach fibres are continuously produced in this
zz Limbal route—with anterior vitrectomy cutter zone throughout life.
zz Pars-plana route: Especially, in dense fibrotic Q.4. Describe the Soemmering’s ring and
PCO with in-the-bag IOL, where it may be Elschnig pearls.
difficult to access the posterior capsule via Ans. Elschnig pearls consist of clusters of
limbal route. swollen, opacified epithelial “pearls” or
clusters of posteriorly migrated equatorial
epithelial (E) cells (Bladder or Wedl cells)
VIVA QUESTIONS The Soemmering’s ring (Fig. 3) is
a dumb-bell or donut-shaped lesion
Q.1. Describe the prevention of PCO.
that often forms following any type of
Ans. Following factors may help to minimize the
incidence of PCO:
Surgical techniques to prevent PCO
formation
• C ortical cleaving hydrodissection and
cortical clean-up
• In-the-bag IOL fixation
• O ptimally sized continuous curvilinear
capsulorhexis with 360° IOL coverage
IOL-related factors (“Ideal” IOL)
• Biocompatible IOL material to reduce
stimulation of cellular proliferation
• Maximal IOL optic—posterior capsule
contact, angulated haptic, ‘adhesive’
biomaterial to create a ‘shrink wrap’
• IOL optic geometry—square, truncated edge Fig. 3: Soemmering’s ring with bladder cells
Lens 435
ECCE (manual or phacoemulsification). been developed to precisely deliver the

Equatorial cells (E-cells) are responsible pharmacological agents within the capsular
for formation of a Soemmering’s ring. The bag, while minimizing the potential for
pathogenetic basis of a Soemmering’s ring collateral ocular damage. Implantation
is rupture of the anterior lens capsule with of intracapsular ring may prevent central
extrusion of nuclear and some central lens PCO after cataract surgery by mechanically
material. Soemmering’s ring is a direct blocking migration of lens epithelial cells
precursor to PCO. towards the central visual axis.
Q.5. What are the drugs/agents used intra BIBLIOGRAPHY

operatively to reduce PCO? 1. Pandey SK, Apple DJ, Werner L, Maloof AJ,
Ans. Intraocular application of pharmacologic Milverton EJ. Posterior capsule opacification: a
agents has been investigated to prevent review of the aetiopathogenesis, experimental
PCO. The basic principle is to selectively and clinical studies and factors for prevention.
destroy the LECs and avoid toxic side effects Indian J Ophthalmol. 2004;52:99-112.
2. Raj SM, Vasavada AR, Johar SRK, Vasavada VA.
on other intraocular tissues such as corneal
Postoperative capsular opacification: A review.
endothelium. Pharmacologic agents being
IJBS. 2007;3(4):237-50.
investigated include antimetabolites (such 3. Tami R, Sellman TR, Lindstrom RL. Effect
as methotrexate, mitomycin, daunomycin, of a plano-convex posterior chamber lens
5-FU, colchicine, and daunorubicin), anti- on capsular opacification from Elschnig
inflammatory substances, hypo-osmolar pearl formation. J Cataract Refract Surg.
drugs, and immunological agents. Sealed 1988;14(1):68-72.
capsular irrigation (SCI) device has
TRAUMATIC CATARACT
Deepali Singhal, Ruchita Falera, Manpreet Kaur
INTRODUCTION History of present illness: Onset, duration and

mode of trauma should be recorded.
Cataract formation is a well-known complication
zz Onset and duration of diminution of vision
of blunt and penetrating trauma. It results from
and whitish opacity should also be noted.
direct lens trauma or concussion effect on the lens
zz Any history of deviation of eye or limitation of
and is often associated with trauma to cornea, iris,
motility should be noted
angle and posterior segment.
zz It is important to note, if there is any medico-
legal case associated with trauma.
Diploma examination.
Past history: Any history of ocular surgery or
HISTORY glaucoma should be enquired.
Chief complaint: Presenting complaints depend
on the type of injury. EXAMINATION
Blunt trauma Systemic Examination
zz Progressive diminution of vision A complete systemic examination should be
zz Whitish opacity done including cranial nerves examination,
zz Monocular diplopia, if associated with especially in cases associated with acute onset
subluxation diplopia. It is important to remember the ABC
Penetrating trauma (airway, breathing, circulation) in a case of
zz Sudden diminution of vision multisystemic trauma presenting with acute
zz Whitish opacity trauma.
Ocular Examination zz Intumescent or normal thickness lens

zz Associated intact or ruptured anterior capsule
Visual acuity: Uncorrected and best-corrected
zz Status of the zonules.
visual acuity (BCVA) helps in planning the
In majority of young patients, the opacity
treatment, especially in early cataract. It is
is localized and stationary, which starts in
important to note projection of rays, since it
subcapsular zone and eventually lies deeply due to
can suggest presence of posterior segment
the formation of new lens fibers.
complications of blunt trauma such as retinal
Whereas, older age group is associated
detachment (RD).
with more diffuse and progressive cataract due
Eyeball: Ocular deviation and both uniocular and to activation of degenerative process of senile
binocular movements should be tested cataract.
Eyelid: Laceration or scarring may be seen. Morphological classification of traumatic cataract
Conjunctiva: Subconjunctival hemorrhage, due to blunt trauma is as follows.
chemosis or scar may be present.
Cornea: On slit-lamp biomicroscopy, following Vossius Ring
signs must be noted. Vossius ring is the epicapsular deposition of iris
zz Corneal clouding/edema pigment. It is a reddish-brown ring corresponding
zz Corneal perforation to the pupillary aperture, about 1 mm in breadth
zz Scar may be seen formed due to extreme miosis at the time of trauma.
zz Sutures may be seen in repaired perforations It is usually segmented due to constrictions on the
zz Intrastromal foreign body may be present. posterior surface of iris. At times, a double ring can
Sclera: Repaired scleral perforation or scar should be seen due to immediate pupillary constriction
be looked for. followed by dilatation.
Anterior chamber: Anterior Chamber (AC) cells,

flare, hyphema, vitreous or lens matter must be
Localized Subcapsular Opacities
looked for. zz Disseminated subepithelial opacity: Small,
discrete/flake like anterior subcapsular
Iris : Iridodonesis/iridodialysis/posterior
opacities, which are commonly stationary. This
synechiae/iris atrophy may be present.
can also present as a large, round, discrete-
Pupil: Sphincter tear, eccentric pupil/traumatic layered opacity called as Cataracta Nodiformis1
mydriasis (Fig. 2), oval/peaking pupil/irregular/ zz Cobweb opacity: It presents as a subcapsular
vitreous entangling pupillary area should be ruled and a more diffuse filmy structure supporting
out. Direct and consensual light reactions of both fine dust-like opacities commonly in young
eyes should be recorded. Presence of relative patients. This can be seen in both blunt and
afferent pathway defect (RAPD) suggests posterior penetrating trauma. It is permanent, occurs
segment complications such as RD or traumatic in the absence of capsular rupture and may be
optic neuropathy. due to mechanical damage to the epithelium.1
IOP: IOP may be raised due to angle recession/ zz Zonular (Lamellar) opacity: It is the result of
subluxation or trabecular damage. disseminated opacities occurring extensively
IOP may also be low in case of globe perforation over the lens or rosette opacity. This is a rare
or vitreous loss. In acute cases of trauma, IOP is presentation as a unilateral zonular cataract,
low due to ciliary shock. seen in young patients. The density may vary
while the outline is irregular and typical riders
Gonioscopy: Angle recession, pigmentation, PAS, may be evident.
cyclodialysis, zonular dialysis and trabecular zz Rosette cataract: Early/late.
meshwork splitting may be seen.
Early rosette cataract
Lens: Lens examination should include: —— It is recognized shortly after trauma, few
zz Determination of type and extent of cataract hours to few weeks.

Lens 437
be flatter without folds, which are present in

penetrating trauma.
zz Presenile and senile changes: These can be
observed in the form of coronary cataract,
water clefts, punctate cortical opacities and
sclerotic nuclear opacities. There is rapid and
premature progression of such changes as
compared to senile cataract.
Diffuse concussion cataract: It is rare, and is
usually associated with capsular tear. It is due to
rapid imbibition of aqueous by the lens matter
leading to opacification. In case of a large tear,
the swollen lens fibers may herniate into anterior
Fig. 1: Vossius ring chamber and vitreous, and later become granular
and necrotic. In young patients, slow and total
—— It may be seen in anterior subcapsular absorption of cataract may occur; whereas, in the
area in concussive injuries or in posterior old, iritis and secondary glaucoma may occur.
subcapsular area in penetrating injuries.
—— Initially seen as fine-fluid droplets formed
Vitreous: Vitreous hemorrhage may be present
or vitreous base avulsion with a bucket handle
between the radiating lens fibers, which
appearance may be seen.
then form feathery parallel rays radiating
from the dark suture lines (Fig. 1). Fundus: Retinal dialysis, giant retinal tear with
—— In mild injuries, these are translucent and retinal detachment and macular hole can occur
may disappear within a few days. due to trauma. Indirect ophthalmoscopy with
—— More commonly, they are permanent, indentation of periphery is a must in all cases of
stationary, and cause vision impairment. trauma. Other features include commotio retinae,
It is gradually buried deeply in cortex due traumatic optic neuropathy or disc avulsion.
to formation of new lens fibers.
Late rosette cataract DIFFERENTIAL DIAGNOSIS
—— Seen few years after trauma
zz Uveitic cataract
—— It is located deep in the cortex or nucleus
zz Developmental cataract (unilateral)
and is due to minimal degree of damage zz Glaucomafleckens.
to subcapsular fibers.
—— In early rosette, the sutures run upto the
center of petals and the rays run from INVESTIGATIONS

them as a midriff whereas, in late rosette, zz Biometry: Axial length and keratometry
the sutures run between the petals which zz Visual potential assessment: Laser Inter
are formed by the outcrop of rays from ferometry or VER
two neighboring sutures. It can also zz Ultrasonography: For posterior segment
extend much further in the periphery and evaluation
may turn backwards to form a second zz NCCT head and orbit/X-ray orbit: To look for
posterior rosette. IOFB and orbital injuries
zz Post-traumatic atrophy of the lens: It may be zz UBM: To identify occult zonular damage and
seen few years after a severe blunt trauma posterior capsular rupture.
where lens capsule is intact. It is characterized
with thinning of the lens substance and
MANAGEMENT
associated with shrinkage of the nuclear and
cortical matter, which can be symmetrical Standard recommendation in acute-onset
or asymmetrical leading to deformation of traumatic cataract with penetrating/perforating
whole architecture. Anterior capsule seems to injuries is primary globe closure followed by a
secondary lens aspiration with IOL placement.

This is because the degree and visual significance
of cataract may not be apparent in the acute
setting and a small opacity may become
visually insignificant later. Moreover, IOL power
calculation and decision about the type of lens and
positioning may be compromised in acute setting
and surgery may be difficult due to hazy media.1,2
Indications of Surgery
zz Anterior capsular rupture with swollen lens
or lens matter in AC require primary lens
aspiration
zz Lens-induced glaucoma or inflammation Fig. 2: Early rosette cataract
zz Visually significant cataract causing
diminution of vision
zz Poor visualization of posterior segment, which
impedes management of posterior segment
injuries.
Primary cataract removal helps control
inflammation and allows early direct visualization
of posterior segment. In children, cataract surgery
should be performed within one year of ocular
trauma to reduce the risk of amblyopia. In the
acute setting, accurate IOL calculations may be
difficult to obtain so data from the other eye can
also be taken.
Surgical Management
The surgical approach depends upon the capsular Fig. 3: Late rosette cataract
and zonular status and degree of lens injury
(Fig. 2). There can be four different scenarios such
as: Anterior Capsular Rupture with Cataract
1. Nondislocated cataract with intact capsule
Primary lens aspiration should be performed
2. Anterior capsular rupture with cataract
in these cases to decrease the chances of
3. Posterior capsular rupture with cataract
inflammation and secondary glaucoma. After the
4. Subluxated lens with cataract (Fig. 3).
initial incision, viscoelastic should be injected
and the extent of anterior capsular rupture along
Nondislocated Cataract with Intact Capsule with the presence of vitreous prolapse should be
Standard phacoemulsification technique can determined. Vitrectomy cutter should be used in
be used with associated glaucoma and iris cut/IA mode to remove the vitreous and cortical
injuries taken into consideration. Adequate size matter from AC. Then, the anterior capsulorhexis
capsulorhexis should be made with capsular can be completed and lens aspiration is done.
staining which will help in better visibility. Anterior In cases of thick fibrotic capsule, vannas scissors
capsular staining can be performed with trypan can be used to complete the capsulorhexis.
blue 0.06%. Generous hydrodissection should be Femtosecond laser assisted anterior capsulorhexis
done with low flow phacoemusification to avoid can also be performed to minimize zonular
stress on the zonules.2 stress.1,2
Lens 439
Posterior Capsular Rupture with Cataract the direct trauma to the lens epithelium and
capsule leading to imbibition of aqueous
The management depends upon the type and the
and cataract formation. Countercoup
size of tear. In type I tears the margins are thickened
refers to damage occurring at a distal site
and fibrosed, so there are less chances of extension
due to shock waves, which can disrupt the
with irrigation. Type 2 tears are present in early
capsule and the lens fibers. Blunt trauma
surgeries and they have thin, transparent margins,
causes anteroposterior compression and
which may rapidly enlarge during irrigation. Tears
equatorial expansion of the globe, which
larger than 6 mm are unable to support in the bag
results in transmission of shock waves. This
IOL. When using an anterior approach, further
may result in damage to capsule causing
hydration of vitreous and extension of tear should
necrosis and increased permeability,
be avoided by using dry aspiration techniques
or damage to lens fibers, or zonular
and meticulous control of infusion and anterior
dehiscence. Damage to lens fibers can also
vitrectomy. Phacoemulsification with low-flow
result from the impact of aqueous and iris
settings should be used. Posterior pars plana
or lens rebound mechanism.
approach is used in cases of posterior dislocation,
and if there is associated retinal pathology.2 Q.2. Discuss the seven rings of trauma.
Ans. See section of traumatic RD.
VIVA QUESTIONS REFERENCES

1. MacFaul PA, Duke-Elder SS. Concussion
Q.1. Describe the pathophysiology of cataract
and contusion. In: System of Ophthalmology
in trauma.
by Duke-Elder, vol. XIV, part I, 1972. Henry
Ans. Several mechanisms have been described Kimpton.
for cataract formation after ocular trauma, 2. Mian SI, Azar DT, Colby K. Management of
which includes coup injury, countercoup, traumatic cataracts. Int Ophthalmol Clin.
and equatorial expansion. Coup injury is 2002;42(3):23-31.
CHAPTER
7
Instruments
OPHTHALMIC INSTRUMENTS
Pranita Sahay, Devesh Kumawat
The ophthalmic instruments can be classified into zz Removal of conjunctival and corneal foreign
the following. body
zz Examination of children and patients with
LID SPECULUM severe blepharospasm.
It is used to keep the lids open during any ocular

surgery. The two commonly used lid speculums
are described below.
Universal Metallic Eye Speculum

As the name suggests, the same speculum can
be used for either eye (right or left) but the
disadvantages are that it is heavy in weight and
cannot keep the eyelashes away from the operating
field (Fig. 1).
Self-retaining Barraquer Eye Speculum Fig. 1: Universal metallic eye speculum

This lid speculum has a screw that helps in giving
desired exposure of the surgical site which can be
changed as per the surgery or surgeon’s choice.
The disadvantage is that the screw increases
pressure over the eyeball and thereby increases the
intraocular pressure. Hence, it is not advisable in
perforated cases (Fig. 2).
Uses
zz All intraocular surgeries like cataract surgery,
glaucoma surgery, keratoplasty, buckling and
other vitreoretinal surgery Fig. 2: Self-retaining Barraquer eye speculum
Instruments 441
FORCEPS Lim Forceps

Forceps of different design are available for It is also a toothed forceps for holding the cornea
different purposes. or sclera and rarely the iris (Fig. 7).
Plain Forceps Iris Forceps

It is a blunt forceps without any tooth. These are toothed forceps especially designed for
Its tip has serrations (either horizontal/ holding the iris for the purpose of iridectomy.
vertical) for holding tissue (Fig. 3).
Uses
zz For holding conjunctiva, scleral flap or skin
zz For holding the sutures while tying.
Globe Fixation Forceps

The tip of this forceps is toothed for better grip
while holding the tissue.
It is used to hold the conjunctiva and episcleral
tissue near the limbus (Fig. 4).
Uses Fig. 4: Globe fixation forceps

zz For fixing the eyeball during surgery
zz For holding the eyeball during forced duction
test.
Superior Rectus-holding Forceps

It is a toothed forceps with ‘S’ shaped curve
specially designed to fit into the orbit while trying
to grasp the muscle belly (Fig. 5).
Colibri Forceps
It is a fine-toothed forceps for holding flaps of
cornea or sclera and rarely the iris (Fig. 6). Fig. 5: Superior rectus-holding forceps
Fig. 3: Plain forceps Fig. 6: Colibri forceps

A
Fig. 7: Lim forceps Figs 9A and B: Artery (hemostatic) forceps:
(A) Plain; (B) Curved (Inset)
Fig. 8: Epilation forceps Fig. 10: Utrata capsulorhexis forceps
Epilation Forceps Utrata Capsulorhexis Forceps

It is a small stout forceps with blunt and flat ends This forceps has a fine titanium tip with a sharp point
(Fig. 8). that enables to initiate the capsular tear then securely
grasp the capsule to perform the capsulorhexis. It
Uses has an “iris stop platform” at 8.5 mm from the tip to
zz Epilation of cilia in trichiasis and stye stop the shaft of the forceps from completely closing
zz Removal of cilia after electrolysis and cryolysis. when tips are closed (Fig. 10).
Artery (Hemostatic) Forceps McPherson Forceps

It is a blunt-tipped forceps with multiple serrations It is a fine sharp tipped non-toothed forcep with
near the tip and a locking mechanism near the angulation (Fig. 11).
other end. It is available in various sizes small,
medium and large. The small-sized forceps Uses
are called mosquito forceps and are the most zz Holding the intraocular lens (IOL) while
commonly used variety in ophthalmic surgeries implanting it
(Figs 9A and B). zz Holding the suture while tying the knot
zz Suture removal.
Uses
zz Holding the bleeders during surgery
Pierce-Hoskin Forceps
zz To crush the muscle before cutting in squint
surgery. It is a fine toothed tissue holding forceps (Fig. 12).
Instruments 443
Fig. 11: McPherson’s forceps Fig. 13: Lens hook/Von Graefe retractor
Fig. 12: Pierce-Hoskin forceps Fig. 14: Green hook
Uses Green Hook

zz Holding the corneal tissue firmly It has a straight shaft with flat-ended hooked tip.
zz For suture tying It is used for hooking the muscle during squint or
zz For forced duction test. enucleation surgery (Fig. 14).
HOOKS AND RETRACTORS Jameson Hook

It has a straight shaft with flat hooked end and
Lens Hook/Von Graefe Retractor paddle shaped tip. It is used for retrieving the
It has a flat metal handle which is curved at its end rectus muscles at their insertion site during squint
and has a knob (Fig. 13). or enucleation surgery (Fig. 15).
Uses Desmarre Retractor

It is a saddle-shaped instrument available in two
zz For applying pressure on the limbus at
sizes—small and large (Fig. 16).
6 o’ clock position during delivery of lens in
intracapsular cataract extraction as well as
extracapsular cataract extraction Uses
zz It can be used as an alternative to muscle hook zz For double eversion of the eyelid and
in squint surgery. evaluation of superior fornix
Fig. 15: Jameson hook Fig. 17: Barraquer needle holder
Fig. 16: Desmarre retractor Fig. 18: Castroviejo needle holder
zz For retraction of the upper eyelid while NEEDLE HOLDERS

removing corneal sutures or foreign body
zz For retraction of the conjunctiva after peritomy Barraquer Needle Holder
in buckling surgery. It is available in two designs with or without
The only disadvantage with this instrument is locking system.
that it is not self retaining. It has fine serrations at its jaw for better grip
while passing the suture through conjunctiva,
Cat’s Paw Lacrimal Wound Retractor cornea, sclera and extraocular muscles (Fig. 17).
It is used to retract the soft tissue from the operative
field during lacrimal sac and lid surgery. It has an Castroviejo Needle Holder
added advantage of having a hemostatic effect. It is a needle holder with S-shaped locking system.
The uses are same as that of Barraquer needle
Müller’s Self-retaining Adjustable holder (Fig. 18).
Hemostatic Retractor
It has two limbs with three pins in each. As the name Arruga Needle Holder
suggests, it has the advantage of being hemostatic It is a large needle holder with one end being flat
and self-retaining along with retracting the skin for placement of the surgeon’s thumb and the
from the surgical site in dacryocystorhinostomy other end having serrations for better grip of the
(DCR) surgery.
Instruments 445
suture. It is available in two designs—with and KNIVES

without a locking system (Fig. 19).
Von Graefe Knife
Uses It is a long, narrow, thin blade with a sharp tip with
cutting edge on one side. It was used for making
zz In eyelid surgery
the corneoscleral entry in cataract surgery.
zz For passing superior rectus bridle suture.
Keratome
CASTROVIEJO CALIPER
It is a thin blade with a diamond-shaped apex and
It is a divider-like instrument with a graduated cutting edge on both sides. It is available in both
scale at one end (marking in millimeters) and the straight and curved design as well as in various sizes
other arm moves by a screw over the scale (Fig. 20). (2.8 mm, 3 mm, 3.5 mm and 5.5 mm). It is used for
making self-sealing corneal incisions in cataract
Uses surgery, iridectomy and keratoplasty (Fig. 21).
zz Measuring the diameters of host or dono-r
cornea MVR or V-Lance Blade
zz Marking the site for muscle insertion in It is a fine straight instrument with triangular knife
recession surgery at its distal end having cutting edge on both the
zz Marking the site for pars plana entry for either sides. It is used for making the side port entry at
surgery or intravitreal injection. the limbus (Fig. 22).
Fig. 19: Arruga needle holder Fig. 21: Keratomes
Fig. 20: Castroviejo caliper Fig. 22: MVR or V-Lance blade

Crescent Knife zz For blunt dissection of the soft tissue in squint

and oculoplasty procedures.
It is blunt-tipped instrument with bevel side of the
knife up and having cut-splitting action on both
the sides (Fig. 23).
Castroviejo Corneoscleral Scissors
It is a fine-curved scissors that works on spring
Uses action (Fig. 25).
zz For dissection in pterygium surgery
zz For dissection of the scleral flap in trabeculec
Uses
tomy/small incision cataract surgery (SICS)/ zz To enlarge the corneal or corneoscleral incision
Descemet’s stripping automated endothelial for intracapsular cataract extraction (ICCE)/
keratoplasty (DSAEK) extracapsular cataract extraction (ECCE)
zz For corneal dissection in anterior lamellar zz To enlarge corneal incision in keratoplasty
keratoplasty. zz To cut the scleral tissue flap.
SCISSORS DeWecker Scissors

It is a fine scissor with small blades at right angle to
Steven Tenotomy Scissors
the arm that works on spring action (Fig. 26).
It is a plain scissors with blunt end. It comes in two
design—straight and curved (Fig. 24). Uses
zz For performing iridectomy
Uses zz For cutting the vitreous strands prolapsing
zz For cutting the muscle from the wound.
Fig. 23: Crescent knife Fig. 25: Castroviejo corneoscleral scissors
Fig. 24: Steven tenotomy scissors Fig. 26: DeWecker scissors

Instruments 447
Fig. 27: Westcott scissors Fig. 29: Enucleation scissors
Fig. 28: Vannas scissors Fig. 30: Lens spatula
Westcott’s Scissors OTHER INSTRUMENTS

It is a stout scissor with straight or curved blades Cataract Surgery
that work on spring action.
It is used for conjunctival dissection (Fig. 27). Lens Spatula
It has a metallic handle with spoon-shaped end
Vannas Scissors which is used to apply pressure at 12 o’clock position
It is a fine scissor that works on spring action. It has for expression of nucleus in Smith’s technique in
two wings to operate—one sharp and one blunt extracapsular cataract extraction (Fig. 30).
(Fig. 28).
Wire Vectis
Uses It is a wireloop attached to metallic handle
zz For cutting sutures (Fig. 31).
zz For anterior capsulotomy in ECCE
zz For cutting the vitreous strand while Uses
performing anterior vitrectomy. It is used to remove subluxated lens in ICCE as well
as nucleus in ECCE.
Enucleation Scissors
It is a thick-curved scissor with blunt tip used for Irrigating Wire Vectis
cutting the optic nerve in enucleation surgery It is a modification of the wire vectis. It has a hollow
(Fig. 29). rim with a 0.3 mm opening at the anterior end
Fig. 31: Wire vectis Fig. 34: Dastoor iris repositor
Simcoe Irrigation and Aspiration Cannula

It is available in the classical and reverse design
with both right-handed and left-handed models
available in each design. It has an irrigation system
through the main port and aspiration system
through the port on the side which is attached to a
syringe through a silicon tube (Fig. 33).
Uses
zz For irrigation and aspiration of cortical matter
in ECCE and open sky cataract surgery in
Fig. 32: Irrigating wire vectis
keratoplasty
zz For aspiration of hyphema.
Dastoor Iris Repositor

It is a flat and straight/bent blade with blunt edges
(Fig. 34).
Uses
zz To reposit the iris in the anterior chamber
zz To tuck the donor cornea underneath the host
cornea in keratoplasty surgery.
Cystotome Needle
Fig. 33: Simcoe irrigation and aspiration cannula
It is prepared with a 26-gauge needle by bending
the needle tip down while holding the bevel up.
and a hollow handle at the posterior end which is Then, while maintaining this needle orientation,
attached to a hub similar to that of a hypodermic bend the needle up near the hub (Fig. 35).
needle through which fluid can be injected Uses
(Fig. 32). zz It is used to make the anterior capsulotomy
in ECCE as well as capsulorhexis in
Uses phacoemulsification
It is used for hydro/viscoexpression of the nucleus zz Posterior capsulorhexis in pediatric cataract
in ECCE and SICS. surgery.
Instruments 449
Fig. 35: Cystotome needle Fig. 37: IOL-holding forceps
Fig. 36: Arruga intracapsular (capsule holding) Fig. 38: Sinskey hook or IOL dialer
forceps
Arruga Intracapsular (Capsule Holding) Sinskey Hook or IOL Dialer

Forceps It is a fine instrument with a bent tip. The tip can
This forcep has a cup at inner side of the tip of engage the dialing holes of the IOL (Fig. 38).
each limb. The edges of the cup are smooth and
atraumatic to the lens capsule (Fig. 36). Uses
Uses zz Dialing of the nonfoldable PMMA IOL for
proper positioning in the capsular bag or
zz For removal of the lens during forceps method
sulcus
of intracapsular cataract extraction zz For nucleus manipulation in phacoemulsi-
zz For removal of the lens capsule remnant after
fication.
accidental extracapsular cataract extraction.
IOL-holding Forceps Chopper

It is a spring action forceps with short, blunt It is similar in appearance to the Sinskey hook
and curved blades smooth edges and tips. but the difference lies in the tip which is sharp
It is used to hold the optic of nonfoldable with cutting edges in a chopper. It is used to split
polymethylmethacrybate (PMMA) intraocular or chop the nucleus into smaller pieces during
(IOL) during implantation (Fig. 37). phacoemulsification (Fig. 39).
Fig. 39: Chopper Fig. 41: Kelley’s punch
Fig. 40: Phaco needle tip Fig. 42: Flieringa ring
Phaco Needle Tip This helps to enhance the emulsification effect and
reduce the postocclusion surge.
It is made of titanium with a distal opening 0.9 mm
The Cobra tip is a bell-shaped tip, which
in diameter with a silicon sleeve which has two
increases the surface producing the ultrasound to
openings on the side 180° apart, through which the
reduce the level of energy required.
irrigation fluid flows. The phaco needle threads
directly onto the phaco handpiece (Fig. 40).
Glaucoma Surgery
The tip can have bevel with 0°, 15°, 30°, 45° or
60°. The greater is the angulation of the bevel tip, Kelley’s Punch
better is the sculpting effect and visibility of the tip It is used to perform bone punch in dacryocysto
but leads to poor occlusion. The 30° bevel offers rhinostomy (DCR) surgery but can also be used
the best compromise and leads to better sculpting, to perform punch sclerectomy in trabeculectomy
visibility as well as occlusion. The silicon sleeve surgery (Fig. 41).
acts as an insulator and the fluid flowing through
the sleeve keeps the tip cool and prevents wound Keratoplasty
burn.
The Kelman tip has a 22° angulation of the Globe Fixation Rings
shaft 3.5 mm from the tip. This enhances the Flieringa Ring
emulsification as well as allows the surgeon to use It is made of stainless steel and is useful for
the phaco tip for manipulation of nucleus during maintaining the architecture of the globe once
surgery. the host corneal button has been removed. They
The flared tip has an outer diameter greater are available in 11 sizes from 12 mm to 22 mm
at the distal end of the tip than 1–2 mm behind it. (Fig. 42).
Instruments 451
Fig. 43: RK marker Fig. 44: Disposable handheld trephine
Uses: Keratoplasty in aphakic eyes especially where

vitrectomy has been performed. Keratoplasty
in pseudophakic eyes and pediatric eyes as the
eyeball has a tendency to collapse in these cases
after trephination.
Disadvantage is that it can distort the shape
of the eyeball and cause an oval cut during
trephination and subsequent high astigmatism.
McNeill-Goldman Ring
It provides support at four strategically placed
sutures. The ring has medial and temporal
openings for greater access to the surgical field Fig. 45: Handheld trephine with obturator
and two lid retractors to prevent eyelid closure by
the patient. It is available in three sizes—small, result in inaccurate centration during
medium and large. trephination of the recipient’s cornea
The examples of hand-held trephines
Corneal Marking Instruments
RK marker, Vajpayee corneal marker (20 radial with obturator are the Castroviejo
arms) and Anis corneal marker (8 radial arms) are trephine and the Grieshaber-
the various instruments that guide the optimal Franceschetti trephine.
placement of sutures in keratoplasty (Fig. 43).
—— Mechanized: The disadvantages associ
ated with motor driven trephines include
corkscrew edge effect on the corneal
Corneal Trephines
stroma.
Types of Trephines —— Suction-fixation type
zz Conventional circular cutting trephines It is devised to obtain a perpendicular
—— Handheld cut in the recipient cornea. These

Ranging from 3 mm to 17 mm in trephine systems essentially consist
diameter. In some trephines, there of an outer corneal suction ring for
is a central obturator, which can be fixation and an inner circular cutting
adjusted to select the depth of the blade.
corneal cut and hence an inadvertent Hessburg-Barron trephine has a cross
entry into the anterior chamber (Figs hair device for improved centration
44 and 45). and an outer ring of corneal marks
However, the obturator obscures the at equal intervals to assist in suture
view of central cornea which may placement. It is available in diameters.
Fig. 46: Hessburg-Barron trephine Fig. 47: Paton spatula
6.0–9.0 in 0.5 mm increments as well

as diameter of 7.75 mm. For each
spoke (90 degrees) turned, the blade
is lowered or raised approximately
0.06 mm (Fig. 46).
The barron vacuum punch features
a solid stainless steel blade which
is permanently mounted in a nylon
housing. Four steel guide posts align
with four corresponding holes in the
cutting block base, automatically
centering the blade over the donor Fig. 48: Teflon block
cornea.
Special-Purpose Type zz Noncontact trephines (Lasers): Laser non

zz The olson calibrated cornea trephine system contact trephination eliminates corneal topo
used to trephinate both the donor and graphy distortion, provides the visualization of
recipient corneas. The system consists of the the entire cornea and enhances centration.
anterior chamber maintainer, the reusable
blade holder (with micrometer setting), Graft Holder (Paton Spatula)
and the suction ring. One revolution of the
micrometer is equivalent to 500 microns. Graft is placed over viscoelastic and is kept covered
zz Skin biopsy punches: The skin biopsy punches, till the recipient dissection is complete (Fig. 47).
which have been used in dermatological
practice, are especially useful in harvesting of Cutting Block
small patch grafts used for tectonic purposes The various cutting blocks available for corneal
in cases of impending/frank perforation. grafting are paraffin block, Teflon block and
zz Single point cutting trephines: The single point polycarbonate and nylon blocks (Fig. 48).
cutter trephines were designed to decrease The Kaufmann corneal cutting block is the
corneal torsion, e.g. Leiberman single point simplest design which consists of a Teflon block
cutter. with a metal cover.
zz Combination trephines: Hanna trephine The Brightbill polytef cutting block is
system has got a circular razor-cutting blade the modern, compound curved block which
and incorporates many of the salient features approximates the central, midperipheral and
of single point cutting trephines. peripheral curvature of the donor tissue.
Instruments 453
Fig. 49: Blade breaker Fig. 50: King’s clamp
Corneal Endothelial Punches donor tissue and maintain adequate pressure

while lamellar dissection or full thickness
To cut a donor button from endothelial side corneal
trephination is being performed. It is designed
punches are also available which use disposable
in a bright blue color to provide a high contrast
trephine blades. The advantage of corneal punch is
background for visualizing the cornea and
that they yield sharp vertical cuts without beveling,
aiding in the lamellar dissection of the cornea.
e.g. Cottingham corneal punch, Troutman corneal
zz King’s clamp (Fig. 50).
punch, IOWA PK press corneal punch, Lieberman
gravity-action punch, Rothman-Gilbard corneal
Lamellar Dissectors
punch.
Tooke knife: The pocket for the initiation of the
Cutting Instruments lamellar dissection may be performed with a Tooke’s
Blade Breaker knife. It has a smooth blade at one end, which can
A disposable razor blade is broken and mounted be inserted intralamellarly to create a pocket.
on the tip of a metallic pencil handle. It is used Paufique knife: It has a double-edged sharp angled
for a controlled entry into the anterior chamber blade that helps in outlining the graft, making the
(Fig. 49). pocket and in dissecting the lamellar plane.
Diamond Knife Desmarre lamellar dissector: It is used in the open
This is the sharpest cutting instrument and is type of dissection, which has a curve in its vertical
available in various sizes and shapes. It is the most meridian and it is used to sweep across the fibers in a
durable and useful instrument for stab incisions as cutting and teasing motion. A duckbill shape lamellar
well as to complete the trephine cuts. dissector is used for closed type of dissection, which
Forceps with special functions is curved in the horizontal dimension.
zz Double corneal forceps, colibri style: It has two Gill’s lamellar dissector: It has a 3 mm wide blade
2.75 mm long tips separated 1 mm with 0.4 which can be either straight or curved.
mm pierse tips. It is 72 mm long and has a Guarded diamond knife: It is a micrometer
serrated handle. adjusted guarded diamond knife, useful for
zz Colibri-style Polack double Corneal forceps: It is obtaining irregular shaped lamellar grafts.
used for the first corneal suture. The cut edge
of the graft is gently grasped at the junction of Crescent knife: This is another useful instrument
the epithelium and stroma with fine toothed for the lamellar dissection. It has a 2.0 mm blade.
forceps.
Instruments for donor cornea dissection in
Automated Lamellar Therapeutic Keratoplasty
lamellar keratoplasty Machine
zz Barron’s artificial anterior chamber and The Moria automated lamellar therapeutic
clamp: The chamber is used to mount the keratectomy (ALTK) microkeratome system
Fig. 51: Moria automated lamellar therapeutic Fig. 53: DSAEK spatula (Stripper)
keratectomy microkeratome system
Fig. 52: Moria artificial chamber maintainer Fig. 54: Busin glide
utilizes the CBm microkeratome and an artificial and removal of Descemet’s membrane without
chamber which is manually driven by the inadvertent damage to the stroma (Fig. 53).
surgeon. Multiple microkeratome heads may be
used to achieve dissection of various thicknesses Busin Glide
ranging from 130 to 350 (130, 150, 250, 300, 350 It allows insertion of the taco by pull through
micrometer). The Moria ALTK artificial anterior technique through 3.2 mm incision. It facilitates
chamber requires a donor scleral rim that is the unfolding of the graft and simplifies centration
symmetrically greater than 16 mm (max 19 mm) of the donor button in the anterior chamber. It
in diameter to provide proper vacuum during the helps to minimize intraoperative manipulation
microkeratome pass. The surgical time is greatly of the graft and the possibility of endothelial loss
reduced as compared to manual dissection (Fig. 54).
technique (Figs 51 and 52).
DSAEK Busin Forceps
DSAEK Spatula (Stripper) It is a microincision forceps with 20G diameter and
It is designed to strip the recipient’s Descemet’s distal action. It is designed to position the graft
membrane during the DSAEK procedure. The in the glide and to pull it from the glide into the
DSAEK strippers, made of surgical steel, are anterior chamber. Its tips have been specifically
available in 45 and 90 degrees angled models, designed to contact the periphery of the graft
in both irrigating and nonirrigating versions. such that the endothelial and the stromal surfaces
The angled tips facilitate the efficient dissection remain untouched in the optical zone.
Instruments 455
Fig. 55: Chalazion clamp Fig. 57: Jaeger’s lid spatula
Fig. 56: Chalazion scoop Fig. 58: Lid clamp or Snellen’s entropion clamp
Lid Surgery as well as protect the globe during lid surgeries

such as entropion, ectropion, ptosis, etc. (Fig. 57).
Chalazion Clamp
It consists of a circular disc attached a circular rim Lid Clamp or Snellen’s Entropion Clamp
attached to each other with the help of a handle
It has a D-shaped plate attached to a U-shaped
that can be tightened with a screw. The disc side
rim which when tightened with the help of a screw
is placed toward the skin while the rim is placed
clamps the tissue and provides hemostatis. There
towards the conjunctiva. It is available in various
are separate clamps for the right and left eye. The
sizes from 10 mm to 21 mm. It is used to stabilize
plate is placed on the conjunctival side while the
the lid and chalazion, and also provides hemostasis
rim is placed on the skin side and the handle is
while performing incision and curettage of the
always directed temporally (Fig. 58).
chalazion (Fig. 55).
It offers the advantage of being self-sustaining
and providing hemostasis. The disadvantage is that
Chalazion Scoop
it reduces the surgical field and can cause pressure
It has a small cup with sharp margins attached to a necrosis of the tissue, if applied too tightly. It
narrow handle. It is used to scoop out the contents is used in lid surgeries such as ectropion and
of chalazion during incision and curettage entropion to stabilize the lid, protect the eyeball
(Fig. 56). and provide hemostasis.
Jaeger Lid Spatula Berke’s Ptosis Clamp

It is a simple metal plate having a slightly convex It is a clamp with J-shaped end with internal
surface on both ends. It is used to support the lid, serrations. It has a locking mechanism as well.
Fig. 59: Berke’s ptosis clamp Fig. 61: Bowman lacrimal probe
Fig. 60: Nettleship’s punctum dilator Fig. 62: Freer periosteal elevator
It is used to hold the levator palpebral superioris Uses

muscle during ptosis surgery (Fig. 59). zz Probing the lacrimal canaliculi and naso
lacrimal duct to find the location of block
Crawford’s Fascia Lata Stripper zz DCR surgery
zz Therapeutic probing in children.
It is used in ptosis surgery for harvesting the fascia Freer Periosteal Elevator
lata. It has a proximal slot for holding the fascia It has a concavoconvex end which is used to lift
lata and has the advantage of harvesting the tissue the periosteum and lacrimal sac from underlying
through a small incision. fossa (Figs 62 and 63).
Thudichum Nasal Speculum
Lacrimal Sac Surgery It is used for anterior rhinoscopy (Fig. 64).
Nettleship Punctum Dilator Kerrison Bone Punch
It is available in various sizes ranging from size
It has a conical pointed tip and is used to dilate the 0=1 mm to size 4=5 mm. It has a cutting end up or
puncta prior to probing or syringing. It is available down design. It is predominantly used to create an
in multiple sizes corresponding to the probe sizes ostium in DCR surgeries (Fig. 65).
(Fig. 60).
Enucleation and Evisceration Surgery
Bowman Lacrimal Probe Wells Enucleation Spoon
It is available in sizes ranging from 0000 to 4 with It is a spoon-shaped instrument with a central
size 0=1 mm diameter (Fig. 61). cleavage to engage the optic nerve during
Instruments 457
Fig. 63: Lacrimal sac dissector and curette Fig. 66: Wells enucleation spoon
Fig. 64: Thudicam nasal speculum Fig. 67: Mule evisceration spatula
uveal tissue from sclera in evisceration surgery

(Fig. 67).
Evisceration Curette
It consists of a round cup with blunt margins
attached to a handle. It is used to curette out the
intraocular contents during evisceration surgery.
Vitreoretinal Surgery
Trocar and Cannula
Fig. 65: Kerrison bone punch
Trocars are used to make pars plana sclerotomy
entries. Trocar needle can be 20G/23G/25G/27G.
enucleation procedure so that it can be easily cut Microcannulas made up of polyimide, are
with an enucleation scissor (Fig. 66). preloaded on the needle trocars. Microcannula can
be valved or nonvalved. Valved cannulas eliminate
the need for plug placement while exchanging
Mule Evisceration Spatula
instruments or removing it.
It consists of a handle with small but stout Combined components of the trocar needle,
rectangular blade with convex surface and blunt microcannula, and trocar handle are referred
edges at its distal end. It is used to separate the to as the trocar-cannula assembly. This system
Fig. 68: Trocar Fig. 70: Vitrectomy cutter
Fig. 69: Infusion cannula Fig. 71: End-grasping forceps
maintains the alignment between the entry holes 2. Cutter using oscillating mechanism/Peyman
in conjunctiva and sclera, as well as provides type: This type of cutter is considered to be
unobstructed instrument access (Fig. 68). superior to the first one as it has less shearing
effect.
Infusion Cannula 3. The Guillotine type cutters: It has an outer tube
which is fixed and has an opening through
Self-retaining infusion cannula of different sizes
which vitreous is aspirated. The inner tube
according to microcannula (20G/23G/25G/27G)
slides across the port thus cutting the vitreous
are used to introduce irrigating solution into the
(Fig. 70).
vitreous cavity (Fig. 69).
Vitrectomy Cutter End-grasping Forceps

Vitreous cutters utilize suction and inclusive These forceps have jaws at the tip to hold tissues
shearing force to cut vitreous. at the edge only. The tips are fine and allow
visualization of the tissue while grasping. These are
These can be of two broad types:
used for epiretinal membrane peeling (Fig. 71).
1. Electrodynamic cutters: It is heavy, becomes
hot causing fatigue and exacerbates tremors.
2. Pneumatic cutters: It is light, so cause fewer ILM Forceps
tremors and are cheap. These have fine tips with smaller jaws which help
Vitrectomy cutters are of three types based on the in picking up of delicate tissues like ILM. These are
cutting mechanism: used for internal limiting membrane peeling in
1. Cutters using rotating mechanism: There is risk macular hole surgery (Fig. 72).
of vitreous spooling and traction on retina.
Instruments 459
Fig. 72: ILM forceps Fig. 74: Charles flute needle
Fig. 73: Foreign body forceps Fig. 75: Back flush
Serrated Forceps Egress of fluid occurs when cannula tip is in fluid

and exit port is open, driven by infusion pressure
These have large flat grasping blades without jaws,
which is above the atmospheric pressure. The blunt
which help to attaining strong grip over tissues
tip can be replaced with a soft silicone tip needle
while managing proliferative vitreoretinopathy.
as well which decreases the risk of iatrogenic
These are used in tough epiretinal membrane
retinal damage (Fig. 74).
peeling and retinal pucker release.
Backflush is a modified flute handle with large
silicone reservoir. Pressure on this reservoir leads
Foreign Body Forceps to retrograde flushing of the fluid or accidently
These are large gauge forceps with serrated or aspirated/incarcerated tissues. It can also be
diamond dusted tips for removal of intraocular used to disperse sedimented preretinal bleed. It
foreign bodies. These have stout jaws which help can be used with either blunt or soft tip needle
in firm holding of the foreign bodies (Fig. 73). (Fig. 75).
Extrusion Instruments Cannula

Charles flute needle: It consists of a blunt needle Cannula tips can be of several types:
attached to a detachable handle. It is used for zz Silicone brush tip cannula: The soft silicone
controlled passive extrusion of fluid during brush tip allows gentle brushing and
internal drainage of subretinal fluid, removal of manipulation of the retina. These are excellent
preretinal blood, and fluid-air exchange. Internal for manipulation and removal of blood from
channel leads to an exit port on the side of handle. the retina surface.
Fig. 76: Soft silicone tip cannula Fig. 77: Diamond-dusted membrane scraper
zz Diamond dusted soft silicone tip cannula:

These are used for triamcinolone removal.
zz Charles flute cannula: This helps to aspirate
blood and debris from the posterior segment.
Smooth, finished tip provides atraumatic entry
and reduces risk of trauma to surrounding
tissue.
zz Soft silicone tip cannula: The soft flexible tip on
the cannula provides atraumatic entry through
retinal or macular tears or holes. These are
used for fluid-fluid or fluid-air exchange in
vitrectomy surgery (Fig. 76). Fig. 78: Vitreoretinal scissors
zz Dual bore cannula: Simultaneous infusion
of heavy liquids and aspiration of intraocular
fluids with dual bore cannula helps to control
constant intraocular pressure during injection. Their blades can have a gentle curve or can be
Cannula can be connected to flute handle or straight, with angle of 30 or 45° to the shaft.
backflush handle or active extrusion handle. Vertical scissors have vertical blades with
pointed tips that move along the axis of shaft.
Diamond-dusted Membrane Scraper Proximal blade moves down toward the fixed
distal blade to cut the tissue vertically. These are
Tano diamond dusted membrane scraper (DDMS)
used for epiretinal membrane segmentation
helps to find the edge of the epiretinal membranes.
(Fig. 78).
It is made of tongue shaped soft silicone with
inert diamond dust. Perfectly suited for both
internal limiting membrane (ILM) and epiretinal Gass Retinal Detachment Hook
membrane (ERM) removal, the diamond dusted, Used for localization of retinal breaks onto the
soft silicone tip helps in finding and grasping the sclera in retinal detachment surgery (Fig. 79).
edge of the membrane quickly and easily (Fig. 77).
Magnets
Vitreoretinal Scissors These are used to remove magnetic intraocular
Horizontal scissors are used for delamination foreign bodies. Electromagnets are more powerful
during epiretinal membrane removal. Their cutting than rare earth magnets (REM) and their magnetic
edge moves conformal to the retinal surface. force can be varied but they are used only as
Instruments 461
Fig. 79: Gass retinal detachment hook Fig. 81: Schocket scleral depressor
external magnets. Rare earth magnets are available

for both intra-ocular and extraocular use (Fig. 80).
Schocket Scleral Depressor

It has a rounded end designed for depressing the
sclera, and a curved marking end for reaching
behind the globe (Fig. 81).
Fig. 80: Magnets

Index
Page numbers followed by f refer to figure and t refer to table
A Amelanotic melanoma 305 Anti-glaucoma medications,

Aminoaciduria 422 timing of 174
Aberrant regeneration 360
Amniotic membrane Anti-inflammatory drugs 194
Acanthamoeba 90, 91, 93, 93f
graft 104, 152 Antioxidant supplementation 281
cysts of 99
transplantation 150 Anti-thyrotropin receptor
growth of 99
Amorphous corneal dystrophy, antibodies 43
keratitis 90, 95
posterior 123 Antiviral agents 77
species, cysts of 99 Apex of orbit 39
Amphotericin 102
trophozoites 99 Aphakic keratopathy 130
Amsler grid testing 226
Accommodative esotropia, Aponeurotic 22
Amsler-Krumeich
classification of 391t ptosis 23
classification 110t, 116
Acetazolamide 306 Aqueous misdirection 184
Acetylcholine receptor Amyloidosis 211
primary 205 Aquired keratoglobus 144
antibody 405 Areflexia 422
test 22 Anemia 237, 240
Aneurysm 357, 375 Arruga intracapsular
Acute angle closure attack, forceps 449, 449f
management of 182 Angioid streaks 277
Angiomatous proliferation 276 Arruga needle holder 445f
Acute spastic entropion, Arsenic exposure 10
management of 63 Angioneurotic edema 107
Arteriovenous malformations 85
Adenocarcinoma, primary 47 Angiotensin-converting
Artery forceps 442
Adenoid cystic carcinoma 4, 44, 45f, enzyme 149
Aspiration cannula 448f
47, 50, 50t, 51 Angle anatomy, distortion of 198
Aspiration of hyphema 448
Adnexa 338 Angle closure glaucoma
Aspirin 307
Adnexal diseases 18 classification of 182t, 184
role of 242
Adult vitelliform macular management of 182t
Assorted retinal diseases 195
dystrophy 276 primary 178, 181, 182
Asteroid hyalosis 211
Afferent papillary defect 246 signs of 209
Astigmatism, type of 420
Age-related eye disease 281, 282 Angle closure, mechanisms of 183 Astrocytic hamartoma 411
study 281 Angle recession glaucoma, Asymmetric orbital size 9
Age-related macular degeneration treatment of 198 Asymmetric palpebral fissure 9
182, 208, 214, 272, 275, Anisometropic amblyopia 164 Atopy-bronchial asthma 107
279, 282, 347 Ankyrin repeat proteins 283 Atresia of external auditory
classification of 281t Anophthalmia 18 meatus 86
clinical classification of 280t Anophthalmos 164 Atrophy of
Aicardi syndrome 322 Anterior chamber fundus 247
Alcohol intake 230 paracentesis 97, 100 orbits 245
Allergic conjunctivitis, signs of 107 Anterior segment optical Attack of hydrops 145
Allergic reactions 52 coherence tomography Atypical mycobacteria 90
Allergy 75 131, 137, 165, 182, 334 Atypical optic neuritis 381, 381t
Allograft rejection 138 Anti VEGF injections, role of 235 Autoantibody 226
episodes of 137 Antiangiogenic drugs 194 Autofluorescence 347
Alport’s syndrome 423, 428 Antibiotics 408 Autosomal dominant
Amblyopia 52, 82, 88, 164, 166, Antibody against lingo1 383 transmission 346
320, 421 Antifungal agents 101 Avellino corneal dystrophy,
management of 67, 166 Antifungal drugs, penetration of 103 signs of 119
treatment 393 Antiglaucoma drugs 178, 194 Axenfeld-Rieger syndrome 158
B Blindness, family history of 241 Canaloplasty 176

Blink reflex, loss of 54 Cancer-associated retinopathy 247
Baclofen 407
Blood 405 Capillary hemangioma 82, 83f, 84
Bacterial corneal ulcers,
glucose 102 course of 83
treatment of 101
investigations 73 Capillary nonperfusion 240, 310
Bacterial keratitis,
removal of 459 Capsular bag distention
management of 100
sugar 73, 211 syndrome 433
Bacterial ulcer 91
transfusions 224 Capsular tension ring 418, 429
Bagolini striated glasses 359, 388
vessels, lack of 79 Capsule on retroillumination,
Band shaped keratopathy 125
Blood-retina barrier 242 posterior 426f
Bandage contact lens 103, 132, 152
Blowout fracture 39 Capsule tension segments 418
Band-shaped keratopathy 148, 333
etiopathogenesis of 34 Capsulorhexis 426
Bardet-Biedl syndrome 247
of orbit 30 anterior 412
Bared circumciliary vessels 177
Bluish colored swelling 63, 86 Capsulotomy, anterior 447
Barkan’s membrane 189
Bluish discoloration of sclera 188 Cardiac disease 160
Barraquer needle holder 444, 444f
Blunt trauma 210, 353, 435 Cardiovascular abnormalities 86
Barron’s artificial anterior
chamber 453 manifestations of 198 Cardiovascular disease 163,
Basal cell carcinoma 9, 12, 13, 69 Blurred vision, symptoms of 157 172, 300
Basal hole diameter 254 Blurring 141, 346 Cardiovascular system 230
Basal laminar deposits 284 Bone mineral density, Carotid angiography 8
Bassen-Kornzweig syndrome 247 measurement of 65 Carotid cavernous fistula 9
Beam radiation therapy, external 50 Bony orbital walls 8 Carotid Doppler scan 219
Beaten-bronze 347 Bony spicules 250 Carotid-cavernous fistula 39, 173
Bell’s palsy, treatment for 77 Boston ocular surface Carpal tunnel syndrome 118
Bell’s phenomenon 4, 18, 61, 73 prosthesis 147 Castroviejo caliper 445, 445f
loss of 37 Botulinum toxin 361, 394, 401, 407 Castroviejo corneoscleral
Berke’s criteria 54t role of 63 scissors 446, 446f
Berke’s method 17 Bowman lacrimal probe 456, 456f Castroviejo needle holder 444, 444f
Berke’s ptosis clamp 455, 456f Bowman layer dystrophies 123 Cat’s paw lacrimal wound
Bevacizumab 283 Bowman’s layer 145 retractor 444
Bielschowsky’s head tilt 373 loss of 162 Catabolism of bloodlike 210
Bifoveal fixation 388 Brainstem auditory evoked Cataract 12, 162, 290, 321, 429, 438
Binocular single vision 400 response 74 cause of 335
Biocompatible 30 Bruch’s membrane 292 detection of 130
Biogenic implants 30 Bruckner test 385 in silicone oil-filled eyes 332
Biointegrated implants 30 Bulbar conjunctiva 11 in trauma,
Bionic eye 250, 284 Bull’s eye pathophysiology of 439
Biopsy 45 appearance 425f presence of 130, 160
Bladder cells 434f maculopathy 347 surgery 206t, 440, 447
Bleeding diagnoses of 348 effects of 237
disorder 209 Bullae, rupture of 129 history of 130, 192
from abnormal vessels 214 Buphthalmos 18, 188, 415 small incision 342, 446
risk of 220 bilateral 188f type of 238, 335
Blepharitis 69 diagnosis of 189 Cataracta nodiformis 436
Blepharochalasis 54 reversible 190 Cat-scratch diseases 290
Blepharoconjunctivitis 69 Burian’s classification 398 Causative organism, type of 90
Blepharokymographic analysis 74 Busin glide 454 Causes, variety of 74
Blepharophimosis 63, 66f Byron smith classification 27 Cavernous hemangioma 83, 84, 84t
epicanthus inversus management of 83
syndrome 64f Cavernous sinus 8
syndrome 18, 54, 55
C Cell precipitates 432
management of 67 Calcified cyst 330 Central areolar choroidal
Blepharophimosis-ptosis- Canal of petit 210 dystrophy 347
epicanthus inversus Canalicular stenosis 12 Central cloudy dystrophy of
syndrome 66 Canaliculi 29 francois 123
Index 465
Central corneal thickness Choroideremia 247 Congenital atrophy of skin 424

increased 136, 159 Chromosomal abnormalities 190 Congenital cataracts, etiology of 413
variation of 338 Chronic blepharoconjunctivitis 68 Congenital corneal
Central Eales disease 211f Chronic infection 29 opacity 161t
Central nervous system 185, 329 Chronic macular hole 347 causes of 161t
anomalies 160 Chronic sun exposure, signs of 151 Congenital cranial dysinnervation
Central retinal Ciancia syndrome 393 disorders 400
artery occlusion 193, 195, 300, Cicatricial ectropion 57, 58f, 61, 62 Congenital dyskeratosis 424
301f, 302 causes of 62 Congenital ectropion 57, 59
vein occlusion 193, 195, 216, 380 management of 58 uvea 190
types of 217t Cicatricial skin changes 57 Congenital entropion 61, 62, 62t
Central scotoma 346 Cigarette smoking, avoidance of 348 Congenital epiblepharon 62, 62t
Central serous chorioretinopathy Cilia Congenital eyelid colobomas, family
254, 303, 305, 307t epilation of 442 history of 64, 86
Central suppression scotoma 388 removal of 442 Congenital glaucoma 143, 154, 161,
Central vein occlusion 222 Ciliary body 198f, 285 187, 187t, 188f, 190t
Cerebrospinal fluid 368 Clear hexagonal margins, classification of 190
Cerebrovascular accident 233 loss of 159 diagnosis of 189
Ceroid lipofuscinosis 347 Climatic droplet keratopathy 150 loops of blood vessels 189
Chalazion 69 Coagulase negative management of 190
clamp 455, 455f staphylococcus 90 Congenital hereditary
scoop 455, 455f Coat’s disease 195, 214, 240, 411 endothelial dystrophy 123, 130,
Chandler syndrome 157, 158 Cobweb opacity 436 131, 153 155, 156, 161
Charge syndrome 322 Cochet-Bonnet esthesiometer 94 stromal dystrophy 155
Charles flute Cogan’s twitch sign 22 Congenital Horner syndrome 23
cannula 460 Cogan-Reese syndrome 157-159 Congenital ichthyosis 424
needle 459, 459f Cold intolerance 12 Congenital keratoglobus 144
Charleaux sign 109 Colibri forceps 441, 441f Congenital myogenic ptosis 23
Chemical injury 29 Colibri-style polack double corneal Congenital neurogenic ptosis 23
Chemotherapy 12, 357 forceps 453 Congenital ptosis 18, 19t, 21, 52
Cherry-red spot 300, 301 Collaborative ocular melanoma causes of 54
diagnosis of 302t study 298 family history of 52
Chest wall deformities 415 Collagen vascular disorders 225 pathology in 55
Childhood onset retinal dystrophy, Collection of corneal material 98 severe 16f, 52f
severe 248 Coloboma 86, 88, 318, 321, 322, 341 treatment of 20
Chloroquine retinal toxicity 347 detachments in 323 type of 21
Choriodal neovascularization 321 histopathology of 322 Congenital rubella 154, 247
Chorioretinal venous incomplete 321 Congenital simple ptosis 65
anastomosis 221 lasered, types of 324 Congenital stromal corneal
Chorioretinopathy 306 of eyelid 86 dystrophy 123
Choriostoma 81 types of 321 Congenital torticollis 376t
Choroid 247 Colobomata, diagnosis of 322 Congestive heart failure 233
Choroidal coloboma 322 Color vision 248 Conjuctival dermoid cyst 79
pathophysiology of 321 Combination trephines 452 Conjunctiva 56, 65, 76, 90, 107, 309,
Choroidal hemorrhage 269, 352 Comitant esotropia, types of 391t 318, 325, 359, 366, 372,
Choroidal melanoma 291, 293t, Commotio retinae 352 378, 410, 416, 422, 424,
294, 297, 298 Complete blood count 7, 88, 428, 436
Choroidal neovascular 166, 331 edema of 40
membrane 214, 215, 254, Complete coloboma 321 retraction of 444
304, 316, 347 Complete palsy 368 Conjunctival cicatrizing diseases 29
Choroidal neovascularization 255, Compressive neuropathy 82 Conjunctival dermoid cyst 79, 80
273, 275, 281, 282 Compressive optic neuropathy 380 Conjunctival dissection 447
Choroidal nevus 294 Cone-rod dystrophy 107, 248, 347 Conjunctival flaps 103
Choroidal rupture 352 Cones, types of 108 Conjunctival fornix 12
Choroidal vessels 249 Congenital abnormality 39 Conjunctival keratinization 12
Conjunctival lymphoma 72 Corneal foreign body 440 Corynebacterium diphtheria 91, 92

Conjunctival swab 97 Corneal graft 104 Cosmetic deformity 163, 166
Conjunctival, removal of 440 rejection, acute 137t, 139 Cosmetically unacceptable
Connective tissue disorder 91, 92, Corneal granular dystrophy 107 enophthalmos 35
200, 342 Corneal guttae, causes of 133 Costenbader 399
Consciousness, loss of 351 Corneal haze 100 Cox’s postulates 354
Constant exotropia 396 Corneal hydrops 109f Cranial nerve 237, 371
Contact lens 91, 111, 144 Corneal hypoesthesia 238 Crawford’s fascia lata stripper 456
types of 144 Corneal inflammation 94 Crescent knife 446f
wear 15, 106 Corneal nerves, increased Crescentic lamellar
Contracted socket 25 visibility of 108 keratoplasty 147
causes of 28 Corneal nodules 152f Crouzon’s syndrome 415
classification of 27t Corneal opacity 87, 118, 162 Cryosurgery 12
Contralateral enophthalmos 9 treatment of 162 contraindications of 12
Contrast sensitivity 248 Corneal pachymetry 131 Cryotherapy 268, 316, 327
Control cardiovascular risk in Corneal perforation 94 application of 12
diabetes 237 risk of 101 Cryptophthalmos 86, 89
Cornea 56, 65, 90, 92, 94, 309, 319, Corneal protrusion 145 Cuff of subretinal fluid 253
325, 359, 366, 372, 378, Corneal scar 97, 108, 108f, 164, 167 Cutaneous lesion, site of 186
410, 416, 422, 424, 428, infection 164 Cuticular drusen 277
432, 436 presence of 132 Cycloablative procedures 189
anterior 119 trauma 164 Cyclodestructive
appearance of 154 Corneal sensations 121 procedure 195, 339
center of 92, 106 Corneal sensitivity, loss of 54 Cyclophotocoagulation 186
central 111 Corneal stromal Cycloplegic refraction 164
flaps of 441 dystrophy 117, 122t Cyclosporin A 138
in peters’ anomaly 162 edema, stage of 129 Cyst
normal 116 Corneal surface, irregularity of 130 evidence of 330
palpebral adhesions 87 Corneal thickness 111, 121 recurrent 82
perforation of 103 central 107, 110, 121, 173 rupture of 78, 82
slices of 111 increased 154f Cysticercosis, life cycle of 331
transparency of 115 measurement of 111 Cysticercus cellulosae 329
Corneal biopsy 100, 104 Corneal thinning 94, 145 Cystoid 311
Corneal choristoma classification development of 94 macular edema 245, 246, 285,
Mann’s scheme 167 Corneal tissue firmly 443 286, 288, 289f, 290, 315
Stargardt scheme 167 Corneal topography 143, 146 retinal spaces 280f
Corneal clouding 153 Corneal transplantation 112 Cystotome needle 448, 449f
Corneal decompensation 184, 205 Corneal trauma 151 Cytokines, release of 106
Corneal diameter, normal 142f Corneal trephines 451 Cytomegalovirus 410, 411
Corneal dystrophy Corneal ulcer 90, 91, 91t, 94, 94f, 95,
classification of 122 96, 97t, 103, 104
signs of 94 central 92f
D
Corneal edema 94, 132, 157f, 159 diagnosis of 97, 104 Dace procedure 269
blepharophimosis, grading of 96, 97t, 104 Dacryocystorhinostomy 450
presence of 153 perforation of 91 surgery 444
Corneal endothelial size of 93 Dahan’s formula 412t
punches 453 trial 101 Dalrymple’s sign 37
reflex 108 Corneal vascularization 121 Dark adaptation, loss of 233
Corneal endothelium 343 Corneolenticular touch 429 Dastoor iris repositor 448, 448f
Corneal epithelial Corona sign 276 Deafness 160
defect, presence of 129 Correct osmolarity 256 Deep anterior lamellar keratoplasty
edema, stage of 129 Cortical cataract 421 112, 118, 124
Corneal erosion, recurrent 117, 124, Corticosteroid 42, 172, 200 Deep dermoid cyst 81
230, 238 intake, history of 174 Deep seated ocular pain 197
Corneal exposure 74 use of 101 Deep stromal lesions 124
Index 467
Deeply pigmented skin 12 Diamond-knife assisted Duke elder classification 425

Deficient amounts of skin 64 DALK 114, 127 Dumb-bell 82
Degeneration 125 Dietary factors 273 dermoids 82
Delineate fovea 241 Diffuse concussion cataract 437 Dysostosis multiplex 300
Dellen formation 164, 166 Diffuse diabetic macular Dysplastic eyelids 65
Dense premacular hemorrhage 213 edema 310f structure of 65
Deoxyribonucleic acid 233 Diffuse retinal pigment Dystrophic calcification 274
Dermatochalasis 18 epitheliopathy 304 Dystrophy 125
Dermoid Diffusion tensor imaging 383 with keratoconus 127
cyst 4, 77, 79 Dilated blood vessels 10
anterior 81 Dilated retinal vessels 276
E
diagnosis of 164 Diode laser
epidemiology of 80, 167 cyclophotocoagulation Eale’s disease 195, 211, 213, 415
grading of 167 195 Ear
recurrent 164, 167 Diplopia 356, 365, 371, 395, 404 abnormalities 64
Dermolipoma 164 charting 32, 359, 366, 370, 388 alterations of 89
Descemet stripping management of 369 anomalies 169f
automated endothelial treat persistent 35 Earliest sign of keratoconus 107
keratoplasty 156f Disc coloboma 322 Early age-related macular
Descemet’s folds 145 Disc diameter 256 degeneration 280
Descemet’s membrane 115, 122, Disciform scar 280f Early rosette cataract 436, 438f
123, 133, 454 Disease discrete, early stages of 120 Early surgery, reasons for 215
compression of 108 Disease progresses 109, 128
Echinocandins 101
endothelial keratoplasty 132 Disease, early stage of 211
Echocardiography 166
Descemet’s stripping Disorganized epithelium 162
Ectatic protrusion of cornea 108f
automated endothelial Disproportionate proptosis 37f
Ectodermal dysplasia 424
keratoplasty 132, Disseminated subepithelial
Ectopia lentis 341, 414
155, 446 opacity 436
endothelial keratoplasty 132 causes of 345
Distant direct ophthalmoscopy
Descemetocele 93f examination 417 et pupillae 415
Desmarre lamellar dissector 453 Distended veins 378 Ectopic lacrimal gland 164
Desmarre retractor 443, 444f Docosahexaenoic acid 249 Ectropion 12, 56
Developmental disorders 181 Dot acuity test 410 basic cause of 58
Dewecker scissors 446, 446f Double corneal forceps 453 grade of 58
Diabetes 208, 414 Double eversion of eyelid 443 of upper lip 88
control 237 Double-freeze thaw technique 290 uveae, presence of 192
mellitus, diagnosis of 236 Down syndrome 107 Eculizumab 283
duration of 236 Doyne honeycomb retinal Edema, type of 227
family history of 209 dystrophy 276 Edrophonium
mellitus 16, 36, 200, 229, Droopy eyelid, compression of 52 chloride test 22
236, 252, 308 Dropped lens, management of 345 test 405, 406
Diabetic macular edema 230, Drusen 273, 275, 284 Ehler-Danlos syndrome 415, 416
233, 308, 309f Dry eye 54, 86, 163 Electrodiagnostic testing 74
laser procedures for 312t syndrome 18 Electrodynamic cutters 458
Diabetic papillopathy 240, 243 Dry socket, treatment of 28 Electromyography 406
Diabetic retinopathy 195, 219, Drying of cornea 151 Electroneurography 74
236, 238t, 252, 309, DSAEK busin forceps 454 Electronic magnifiers 285
310, 312, 314, 316 DSAEK spatula 454 Electrooculogram 350
study, early treatment 309, Dual bore cannula 460 Electrophysiology 347, 383
233, 237 Duane retraction syndrome 24, 400 Electroretinogram 220, 241, 247
vitrectomy study 234 Duane’s classification 398 Elschnig’s operation 59
Diabetic subjects 234 Duane’s syndrome, types of 17 Elschnig’s pearls 433, 434
Diamond knife 453 Duane’s retraction Encephalocele 78
Diamond-dusted membrane syndrome 86, 164 End-grasping forceps 458f
scraper 460, 460f Dubowitz syndrome 65 Endocrine 7
Endogeneous Ethmoidal mucocele 1 coloboma 89

disease 174 Evisceration curette 457 surgical 87f
release of corticosteroids 172 Exacerbations, acute 382 treatment of 88
Endophthalmitis 101, 257, 269, Excision biopsy 8, 11 crutches 407
352, 411 Exodeviation 395 defects 13
Endoscopic approach 33 Exophoria 399 drooping of 52, 63
Endothelial cells 160 Exophthalmometer, type of 6 edema of 40
Endothelial dysfunction, first Exophthalmometry 6 fullness of 40
indicator of 136 Exotropia 395 margin, distortion of 10
Endothelial dystrophies 123 alternating constant 398t mechanical ptosis,
Endothelial keratoplasty 132, epidemiology of 397 right upper 19f
140, 189 unilateral constant 398t notching 12
Endothelial rejection 134, 137 Exposure keratitis 88 repair, scar of 197
Enophthalmos 18 Exposure keratopathy, signs of 7 structures 88
Entropion 12, 60 Extensive disease 12 swab 97
causing keratopathy 60f Extracapsular cataract Eylea 283
types of 61 extraction 446
Enucleation scissors 447, 447f Extrafoveal 281 F
Enucleation socket 63 Extraocular diseases 195
Enzymatic vitreolysis 317 Fabry’s syndrome 414
Extraocular movement 400
Enzyme-linked immunosorbent Face 357
Extraocular muscle 7
assay 211 turn 400
abnormalities 9 Facial angiomatosis, bilateral 185f
Epibulbar dermoid 164 Extravasated blood, presence of 208
Epicanthus inversus 63, 65 Facial asymmetry 64, 163, 357, 372
Extreme ectropion 58 presence of 6
syndrome 63, 66f
Exudative retinal detachment 260 Facial nerve 76
Epidermal dermoid cyst 77, 79
Exudative vasculopathy 249 anatomy of 76
Epikeratophakia 114
Eye lesion 39
Epilation forceps 442, 442f
buphthalmos, right 188f Facial palsy, signs of 57
Epinucleus removal 427
complete coverage of 88 Facial symmetry 16
Epiphora 63
deviation of 52, 395 Familial exudative vitreoretino-
triad of 188
disorders, allergic 91 pathy 210, 211
Epiretinal membrance 227, 245,
lid margins 10 Familial vitreoretinal disorders 262
246, 259, 288, 290, 314,
movement Fasanella Servat operation 20
317, 460
limitation of 45 Fasting blood glucose 411
Episcleral osteoma 164
Episcleral plaque restriction of 10 Fatigability 22
brachytherapy 297 rubbing 106 Feeling pulsation 2
Epithelial defect 167 Eyeball 1, 3, 309, 318, 325, 338, 357, Fellow eye 211
size of 93 378, 400, 410, 416, 422, examination of 121
Epithelial dystrophy 122 424, 428, 436 role of 215
Epithelial erosion 148 absence of 63, 86 Femtosecond laser 114, 127
Epithelial recurrent erosion during surgery 441 Fibrinoid syndrome 213
dystrophy 122 size of 153 Fibrovascular disciform scar 276
Epithelial scraping 124 small size of 63, 86 Fibrovascular membrane 196, 268
Epithelioid cell melanoma 298 Eyebrow hair, loss of 86 Fibrovascular tissue,
Equatorial cells 435 Eyelash 173, 179 proliferation of 192
Erythrocyte sedimentation loss of 10, 12, 68 Filtration surgery 189, 195, 199, 339
rate 7, 211, 219, 288 rubbing cornea 61f trabeculectomy 176
Erythropoietin 383 Eyelid 56, 69, 88, 309, 410, Fine toothed tissue holding
Escherichia coli 99 424, 436, 428 forceps 442
Esodeviation 384 abnormal 63 Fine-needle aspiration biopsy 8
Esophoria 391 adnexal structures of 9 Fine-needle aspiration cytology 11
Esotropia 391, 395, 397 anatomy of 76 Fish
of right eye 365f architecture, disruption of 68 mouthing 269
Essential infantile esotropia 393 carcinoma of 11 strike sign 256
Index 469
Fishman classification 348, 349t Fundus 32, 65, 78, 314, 326, 359, diagnostic tools of 177
Fixed hypopyon 96f 366, 374, 410, 422, 425, drainage device 176, 186, 339
Fleck corneal dystrophy 123 429, 432, 437 surgery 195
Fleck lesions 349f autofluorescence 280, 295 family history of 337
Flieringa ring 450f coloboma 323t in angle recession 199
Floaters, history of 230, 236 examination 109, 181, 263, 319, increased risk of 172
Floppy eyelid syndrome 73, 107 417 management of 429
Fluid occurs, egress of 459 flavimaculatus 348 mechanism of 250
Fluorescein angiogram 248, fluorescein medications 287
288, 310 angiograms 275 primary open angle 171,
Fluorescein angiography 226, 255, angiography 217, 219, 240, 200, 205
305, 310, 347 progression of 172
277, 295, 310f, 316
ophthalmoscopy 374 refractory 162
Fluorescein staining 61
photography 174, 240, 374 risk of 202
Fluorinated pyrimidines 101
tessellated appearance 249 stage, angle-closure 196
Fluoroquinolones 101
Fungal corneal ulcer 93 surgery 440, 450
Focal cataract 184
Fungal keratitis 103 treatment of 162
Force duction test 32, 367 Glaucomatocyclytic crisis 200
management of 101
Force generation test 32, 368 Glaucomatous cyclitis 268
Fungal ulcer 93
Force injury 190 Glaucomatous optic
Furrow degeneration 145
Forced duction test 360, 368, damage, severity of 177
Fusarium species 91
374, 390, 405, 443 Fusional amplitudes 387 neuropathy, severity of 195
Forceps injury 154, 155 Glaukomflecken 180f
Forehead wrinkles, loss of 57 Glioma of optic nerve 3
G
Foreign body 93 Globe fixation
forceps 459 Gabapentin 407 forceps 441, 441f
granuloma 164 Ganglion cell layer 257 rings 450
irritation 163 Gas permeable contact lenses 111 Globe injury
sensation 60, 72, 117, 148, 163 Gass clinical grading system 316 closed 355
Fornices, depth of 26 Gass retinal detachment hook 460 presence of open 354
Four Δ prism test 388 Gastrointestinal ulcer disease 43 Globe perforation 12
Fourth cranial nerve Gaucher’s disease 301, 302 Globular corneal
function in third palsy 374 Gelatinous drop-like corneal protrusion 141f, 142f
palsy 371 dystrophy 123 Globus type of cone 108
Gelatinous thickening of limbus 107 Glucose tolerance test 240
Fourth nerve
Genetics 428 Gold weight implantation 75
course of 375
of stromal dystrophies 127 Goldenhar syndrome 87, 89, 168,
palsy 376
Genital anomalies 89 168t, 169f, 402
Foveal avascular zone 226, 241, 310
Genitourinary malformations 64 Gonioscopy 161, 164, 189, 326, 436
Foveal drusen 254
Geographic atrophy 274 of both eyes 180
Foville syndrome 370
Ghost cell glaucoma 213 Goniotomy 186, 189
Fraser syndrome 87, 89 Giant retinal Gopal Krishna classification 26
Freer periosteal elevator 456 dialysis 326 Graft edema
Frequent change of glasses 106, 172 tear 265, 325, 326, 328, 329, 353 development of 134
Frequent eye infections 60 Gill’s lamellar dissector 453 presence of severe 137
Frisby Davis distance test 389 Glaucoma 104, 171, 421, 174, 176, Graft host junction 139
Frontalis overaction 16 177, 179, 187, 192, 431 Graft rejection 134, 140
Fuch’s endothelial corneal angle closure 250 acute 134, 135, 136f, 137
dystrophy 131 angle recession 174, 197, 198 episode of 137
Fuch’s heterochromic cause of 186 Granular corneal
iridocyclitis 290 closure 179 dystrophy 117, 123
Fuchs’ endothelial damage, severity of 177 Granulation tissue, mass of 72
dystrophy 158 development of 339 Granuloma 330
corneal 123, 128 diagnosis of 173, 174 Graves’ disease 1
Fundal coloboma 318, 319f, 322 unilateral 198 signs of 3
Graves’ orbitopathy 41 Hertel exophthalmometer 6, 32 Idiopathic juxtafoveal

Grayson-Wilbrandt corneal Hessburg-Barron trephine 451, 452f telangiectasis 240
dystrophy 123 Hexagonal cells, Idiopathic lesions 375
Greater visual field loss 174 percentage of 131, 230 Idiopathic polypoidal
Gross examination of cyst 330 High myopia 200, 209, 267, 415, 429 choroidal vasculopathy 213, 215
Gross mass of tumor plus 11 Hirschberg test 385 choroidopathy 275, 276
Group of non-inflammatory 117 Histochemical stains, types of 125 Idiopathic retinal vasculitis 213
Guillain-Barré syndrome 24, 73 Histopathological classification 308 Idiopathic senile flaccidity 39
Guillotine type cutters 458 Holt-Oram syndrome 402 Immunohistochemistry 47
Gunderson’s flap 103 Homocystinuria 415, 428, 417t Immunologic disorders 95
Gyrate atrophy of retina 247 disease 420 Immunosuppressive treatment,
Hormone replacement therapy 67 side effects of 43
H Horner syndrome 21, 53, 54 Impending macular holes 253
Hoskin’s anatomic 191 Implantable miniature
Haab’s striae injury 190 Human papilloma virus 10 telescope 284
Hallmark of Human T-lymphotropic virus In vivo confocal microscopy 100
graft rejection 135 type 1 290 Incision biopsy 8, 11
keratoconus 110 Hundred-day glaucoma 196 Incomitant esotropia types 391t
Harada’s disease 305 Hyalitis 285 Indocyanine green 275
Harboyan syndrome 156 Hyaloid membrane, posterior 317 angiography 275
Hard exudates 275 Hybrid contact lenses 112 Infection
Hassall-Henle bodies 130 Hydraulic theory 34 plus, signs of 100
Head posture, abnormal 63, Hydrodelamination 114, 127 recurrent 64
357, 365, 372 Hydrodelineation 426 Infectious keratitis, diagnosis of 97
Headache 329 Hydrodissection 426 Inferior dystopia 37f
Healing keratitis 94f Hydrogel junction 112 Inferior lid retractor laxity 56
sign of 93 Hydrops, acute 141, 144 Inferior rectus muscle 39
Healing, signs of 104 Hyperglycemia, severity of 236 Infiltration, resolution of 101
Hearing loss 64, 163 Hyperlipidemia 237 Inflammation
Heavy silicone oils 340 Hyperlysinemia 415 degree of 195
Hemangioma 84 Hypermature cataract 415 iris, presence of severe 96
triad of 84 Hyperopic correction 304 presence of severe 92
Hemangiopericytoma 84 Hyperostosis 7 signs of 7
Hematological disorder, Hypertension 25, 208, 237 Inflammatory disease 316
discover signs of 209 absence of 226 Inflammatory orbitopathy 39
Hematuria 423 family history of 209 Infliximab 283
Hemodynamic crisis 172 Hypertensive retinopathy 240 Infusion cannula 458, 458f
Hemoglobin A1C 240 Hyperthyroidism, signs of 36 Inkblot leak 305
Hemoglobinopathies 240 Hypertrophic scar 12 Inner retina, dimpling of 257
Hemorrhage Hypertropia 18 Inoperable disease 12
resolution of 220 Hyphema 184, 192, 218 Insulin-dependent
types of 210 Hypoparathyroidism 414 diabetes mellitus 234
Hemosiderin pigments 210 Hypoplastic disc 164 diabetic 241
Hemostatic forceps 442 Hypopyon 96f Intact red blood cells 210
Hereditary systemic disorders 262 height of 95 Intellectual disability 422
Herpes keratopathy 151 role of 104 Intercalary membrane 322
Herpes simplex 410 size of 95 Intermediate uveitis 285, 288, 290
virus 411 Hypotonia 422 causes of 286, 290
Herpetic disciform keratitis, Hypotony 269 Intermittent esotropia 391, 398
central 130 classification 398t
Herpetic eye disease 105t Intermittent proteinuria 118
I Intermittent pupillary block 429
Herpetic footprints 130
Herpetic keratitis 94 Iatrogenic break 269 Internal limiting membrane 234,
Herpetic keratouveitis, Ice cell 159 256, 257, 317, 460
recurrent 184 Ice pack test 22, 405 peeling 256
Index 471
International Thyroid Eye Intravitreal injection 445 Juvenile xanthogranuloma 164

Disease Society 41 Intravitreal pharmacotherapy 220 Juxtafoveal 281
International vitreomacular Intravitreal ranibizumab,
traction study 316 efficacy of 227 K
Interpalpebral calcification 149f Intravitreal steroid 200
Kaposi’s sarcoma 71
Interpupillary distance 64, 388 Intravitreal triamcinolone
Kasabach-Merritt syndrome 84
Intracameral injection of acetonide 200
Kawasaki disease. 290
bevacizumab 194 Intrinsic lesions of optic nerve 1
Kearns-Sayre syndrome 15, 54, 247
Intracapsular cataract Inverse Bell’s phenomenon 18
Kelley’s punch 450, 450f
extraction 446 Inverse glaucoma 429
Keratic precipitates 130, 135, 287
Intracorneal ring segment IOL-holding forceps 449f
Keratin, result of 79
insert 112, 114 Ionizing radiation 10 Keratitis 12, 18, 104, 115
Intracranial tension 365 Iridectomy 441 active infectious 103
Intraepithelial spread of tumor 13 Irido fundal coloboma 164 specific 95
Intralenticular lens Iridocorneal endothelial 157 Keratoacanthoma 70
aspiration 418, 419f dystrophy 131, 155 Keratoconus 106, 107, 111, 115,
Intralesional corticosteroids 72 syndrome 154, 157, 157t, 147, 249
Intraocular cysticercosis 332 181, 194 cause of 106
Intraocular foreign body 355 Iridophacodonesis 333 complications of 115
removal of 262, 459 Iridoschisis 198 development of 248
Intraocular lens 442 Iris 32, 65, 96, 359, 366, 372, 410, diagnosis of 110t
dislocation 344 422, 424, 428, 432, 436 moderate cases of 114
basis of 344 atrophy 157, 158f posterior 115
implantation 429 progressive 158 progression 106
primary 412 segmental 180f severe variants of 144
power calculation 412 bleeding 184 signs of 116
removal coloboma 322 treatment of 111
holding forceps 449 diaphragm 199 Keratoglobus
removal 344 forceps 441 diagnosis of 143
Intraocular malignancy 45 melanosis 159 treatment of 144
Intraocular pressure 7, 96, 136, 171, nevus syndrome 159 Keratome 445, 445f
182, 184, 192, 193, 202, pigment, disruption of 203 Keratometry, disadvantages of 110
209, 263, 265-267, 287, root of 194 Keratopathy 61f
309, 319, 326, 342, 359, retrodisplacement of 198f causes of exposure 37
366, 372, 410, 417, 422, sphincter tears 198 exposure 87
424, 428, 436 stop platform 442 Keratoplasty 440, 450
measurement 338 vessels result of 104
range of 176 normal 194, 195 Kerrison bone punch 456, 457f
reduction of 132 sizes of 194 Kimura’s spatula 97
symptoms of raised 197 Irregular astigmatism 166 King’s clamp 453, 453f
Intraocular surgery 78, 163, 262, 341 Irrigating wire vectis 448f Klinefelter’s syndrome 428
Ischemic maculopathy 240 Knapp’s procedure 55
Intraocular tumor 7
Ischemic mononeuropathy 361 Koloboma 318
Intraoperative choroidal 186
Ischemic optic neuropathy 380 Krause-Kivlin syndrome 162
Intraoperative complications 336t
anterior 240 Krimsky test 385
Intraretinal edema 253
Isolated coloboma 87
Intraretinal hemorrhage 330
Intraretinal microvascular
Isolated ectopia lentis 415 L
abnormalities 242, Lacrimal fossa
310, 311 J mass, diagnosis of 47, 51
Intrauterine infections, Jaeger lid spatula 455, 455f tumors 4
serology for 411 Jaw-winking Lacrimal gland 45f
Intravascular papillary endothelial phenomenon 15 enlargement 40
hyperplasia 72 ptosis 54 mass 78
Intravenous immunoglobulin 383, Jones criteria 100, 104 diagnosis of 47t
406 Juvenile rheumatoid arthritis 290 tumor 3, 6, 44, 46, 48t
Lacrimal sac 29 Lens 65, 96, 115, 309, 319, 326, 409, reconstruction 13, 14t
surgery 456 410, 416, 422, 425, 428, resection procedures,
tumors 3 432, 436 disadvantages of 59
Lacrimal system 87 absorptive 285 retraction 37f
patency assessment 61 aspiration 412 signs in proptosis 38t
Lactosyl ceramidosis 301 coloboma 321 speculum 440
Lagophthalmos drop 343 surgery 455
causes of 76t epithelial cells 434 tumor 9, 10
degree of 73 hook 443f Life-threatening injuries 31
etiology of 76 posterior Light reflex tests 385
various, severe 75 dislocated 341 Limbal dermoid 87, 163, 163f,
Lamella reconstruction displacement of 199 164f, 169f
posteriorly dislocated 352 treatment of 166
anterior 13
size of 344 Limbic keratoconjunctivitis,
posterior 14
spatula 447, 447f superior 69
Lamellar keratectomy, anterior 103
technique of removal of 344 Limbus-to-limbus corneal
Lamellar keratoplasty 124, 143,
type of 112 thinning 141, 142f
147, 152, 166
Lensectomy 412 Limited anterior vitrectomy 412
advantage of 126
role of 327 Lingam gopal classification 323t
anterior 150 Lipodermoid 78
Lenticonus 421
Lamellar laceration 355 Lisch epithelial corneal
anterior 423t
Lamellar macular hole 254, 257 dystrophy 123
posterior 422, 423t, 425
Lamellar therapeutic keratoplasty, Locus minoris resistentiae 322
Lenticular refractive surgery 112
anterior 152 Loss of vision, sudden painless 216
Leopard skin retinopathy 247
Lang’s pencil gross streopsis Low potency steroid 202
Lesion
test 389 Low vision
anterior 81
Larynx, alterations of 89 aids 285, 348
diameter of 12
Laser capsulotomy 262 of anterior ethmoidal sinus 3 cause of 226
Laser epithelial keratomileusis 115 Less severe occlusion 216 in coloboma, causes of 323
Laser peripheral iridotomy 182, 183 Leukemia 209, 214, 240, 305, 357 Lowe’s syndrome 190, 414, 421
complications of 184 Leukemic vitreous infiltration 211 Lower eyelid
Laser photocoagulation 213, Leukocoria 409 coloboma 87, 89, 164
306, 316 Levator action 53 retractor reinsertion 62
Laser trabeculoplasty 176, 198, 201 Levator function 53 tightening 75
selective 339 measurement of 53 Lower lid 39
Lash loss 12 Levator palpebrae superioris 15, coloboma 89t
Late rosette cataract 437, 438f 23, 356 retraction 39
Lateral anterior dermoid 78 Luedde’s exophthalmometer 6
function 17
Lateral canthal Lumbar puncture 379
Level of Bowman’s membrane 148
laxity test 61 Lyme’s disease 74, 290
Lhermitte sign 377
tendon laxity 57 Lymph node
Lid 318, 325, 338, 358, 365, 372,
Lateral tarsal strip procedure 58f enlarged 69
378, 401, 422
involvement 11
Lattice corneal dystrophy 123, clamp 455, 455f
Lymphatic malformation 84
125, 151 coloboma 88
Lymphoma 3, 164, 211
Lattice dystrophy 127 acquired 87
Laxity of facial skin 118 drooping of upper 15
Leber’s congenital examination of 4
M
amaurosis 248, 251 excursion 17 Macroaneurysm 213
hereditary optic neuropathy 107 fold, presence of 53 Macula 379
Leber’s hereditary optic laxity 58-60 center of 254
neuropathy 380 amount of 56 Macular atrophy 254
Left exotropia 399f lower, components of 58 Macular colobomas 320
Left inferior rectus 373 malignancy, signs of 13 Macular corneal dystrophy 117, 123
Left lower eyelid 10f margins, ulceration of 10 Macular degeneration,
Left upper eyelid 10f opening, normal 72f signs of age related 152
Index 473
Macular dystrophy 117, 127, Mechanical debridement 149 Mohs’ micrographic

347f, 348, 348t Mechanical ectropion 58 surgery 11
Macular edema 211f, 218f, 219, management of 59 technique 69
220, 239, 243, 310 Mechanical ptosis 24 Moist socket, treatment of 28
causes of 240 Media haze, causes of 290 Moll gland of eyelid 67
development, Media opacity, causes of 210, 211 Monocular diplopia 415
pathophysiology of 242 Medial anterior dermoid 78 Monster vessels 189
first line of treatment for 229 Medial canthal Mooren’s ulcer 93, 145
management of 229 laxity test 61 Moraxella 90
Macular hemorrhage 254, 302 tendon laxity 56, 59 Morning glory syndrome 402
Macular hole 252, 257, 352 Medial orbital Motility 318
formation 260 lymphangioma 3f deficits 81
index 255 wall, fracture of 1 Mucocele 78
management of 256t Medial rectus, bilateral 394 Mucopolysaccharidoses 154, 161
surgery for 255 Meesmann corneal dystrophy 123 Muir-Torre syndrome 70
Macular ischemia 219, 228, 310f Megalocornea 142, 415 Müller’s muscles 75
Macular swelling, causes of 240 Membrane, type of 263 Multicenter uveitis steroid
Maddox rod 387 Membranectomy 434 treatment 289
Maddox wing 387 Membranoproliferative Multifocal electroretinography 311
Male graves’ disease 36 glomerulonephritis 277 Multiple follicular cysts 70
Malignant contracted socket 27 Membranous conjunctivitis, Munson’s sign 115
Malignant melanoma, severe 62 Muscle
manifestation of 14 Menstrual history 64 biopsy 406
Malignant mixed tumor 49, 50 Mental retardation 417 of retraction 76
Malignant tumor 46, 47 Mestinon 407 tone, reduced 64
Malpositioning of eyelid 52 Metabolic disorders 190 Musculoskeletal system 330
Managing macular ischemia 313 Metallic eye speculum 440f Musk protein antibodies 405
Mandibulofacial dysostosis 428 Metastasis 70 Mustard’s technique 88
Mannosidosis 414 Metastatic disease 305 Myasthenia gravis 39, 55, 403, 408
Map biopsy 13, 69 Metastatic tumor 3, 50 Myasthenia, tests to confirm 22
Marcus Gunn jaw Microaneurysm development, Mycobacteria species 105
grading of 21 pathophysiology of 241 Mycobacterium tuberculosis 92
winking 19, 21 Microphthalmia 18 Mycophenolate mofetil 138
syndrome 23, 54 unilateral 29f Myoepithelioma 47
Marcus Gunn ptosis 55 Microphthalmos 164 Myogenic ptosis 23
Marcus Gunn syndrome 15 Micropsia 395 acquired 23
Marden-Walker syndrome 65 Micropulse 243 Myogenic theory 402
Marfan’s disease 417f, 417t, 420 Microspherophakia 428 Myopia, signs of 267
etiopathogenesis of 419 etiology of 430t Myopic crescent 429
Marfan’s syndrome 107, 415-417, Micro-spherophakia, Myopic glasses, use of 252
420, 428 pathogenesis of 431 Myotonic dystrophy 54, 414
Margin crease distance 17 Microvascular disease 361
Margin limbal distance 17 Microvascular lesions 375
Margin-reflex distance 17 Mild congenital ectropion 59
N
Margins of ulcer 93 Mild contracted socket 27 Naffziger’s method 3
Marked ectropion 58 Mild ptosis 53 Nasolacrimal duct stenosis 12
Mass lesion, reducibility of 4 Millard-Gubler syndrome 370 Nasopharyngeal tumor 3
Mass, reducibility of 4 Miniature toy test 410 Naugle exophthalmometer 6
Mature cataract 415 Misdirected third nerve Nausea, complaint of 337
Maxillary sinus ptosis 19, 55 Necrotic material 103
carcinoma of 3 Mitomycin C 69, 183, 194, 195 Neovascualar glaucoma 181, 192,
tumor 3 use of 166 198, 216, 217, 219, 222,
Mc Donald criteria 382 Mitral valve prolapse 107, 415 230, 238
McNeill-Goldman ring 451 Mixed types melanoma 298 causes of 195
McPherson’s forceps 442, 443f Moebius syndrome 402 stages of 193t
Neovascularisation iris 181, 192, Occlusion therapy 166, 396 Oil tamponade, duration of 340
193, 193f, 209, 230, Ocular Oil-filled eyes 335, 340
238, 263, 309 adnexa 225 Okihiro’s syndrome 402
Nephropathy 237 associations 107 Old retinal detachment 247
Nerve fiber layer 173 balance 46 Olivopontocerebellar atrophy 347
Nettleship’s punctum dilator 456f bobbing 404 Open-angle glaucoma 171, 309
Neurocysticercosis 331 coherence tomography 315, 316f Ophthalmic artery occlusion 251
Neurogenic ptosis 23 cysts 329 Ophthalmic division of
acquired 24 dermoids 168 trigeminal nerve 4
Neurologic disorders 39 developmental disorders 190 Ophthalmic examination 36,
Neuromyelitis optica 382 disease 190 300, 309
Neuroretinal rim 174 severe ischemic 25 Ophthalmic instruments 440
Neurotrophic keratitis 230, 238 epibulbar dermoids 168 Ophthalmic viscoelastic device 427
Neurotrophic ulcer 93 examination 3, 188, 273, 286, Ophthalmology,
New iris vessels 195 292, 325, 351, 357, 365, epidemiology of 184
Night blindness 248 372, 378, 400, 428, 431 Ophthalmopathy, treatment of 42
N-methyl d-aspartate 176 flutter 404 Ophthalmoplegia, external 65
Nocardia species 90, 105 function 12 Ophthalmoscopic classification 380
Nonantigenic 29 hypertension 174 Ophthalmoscopy, indirect 301
Noncontact trephines 452 hypotony 269 Optic atrophy 219
Nonhealing corneal ulcer 104 inflammation, Optic canal 8
Non-healing of epithelial defect 101 postoperative 205 Optic disc 173, 378
Non-insulin-dependent inflammatory diseases 163, 195 cup, enlargement of 173, 173f
diabetes mellitus 234 injury, classification of 355 hemorrhage 177
Nonpenetrating deep investigation 288 pit 305
sclerectomy 176 ischemic syndrome 219, 240 types of 323t
Nonpenetrating glaucoma lymphangiectasia 72 Optic foramina, enlargement of 7
surgery 176 manifestations of Bell’s palsy 76t Optic nerve
Nonproliferative diabetic motility 31, 365 damage, extent of 192
retinopathy 229, 236 abnormal 53 dysfunction, signs of 381
Nonrefractive accommodative 392 limitation of 31 glioma 3f
Nonresolving vitreous movements 267 head
hemorrhage 221 myasthenia 404 changes 174
Nonspecific orbital gravis 403, 406 examination of 188
inflammatory disease 39 neoplasms 195 hypoplasia 193
Noonan syndrome 65 neovascularization 220 Optic neuritis 377, 380
Norrie’s disease 195 pemphigus 62 classification of 380
Nose, alterations of 89 perforation 167 options for 381
Nuclear sclerotic cataracts 257 predisposition 209 treatment trial 377, 381
Nucleus response analyzer 111 Optical coherence
drop 342 side effects 291 tomography 279, 282
emulsification 426 signs of myasthenia gravis 404t uses of 240
expression of 447 surface Oral
Nyctalopia 233 diseases 134 hypoglycemic agents 237
Nystagmoid movements 404 irregularity 60 pentoxifylline, role of 221
Nystagmus 52 surgery 64, 86, 107, 134, 172, 195 prednisone, short course of 32
surgery for 407 symptoms and signs of Bell’s Orbicularis muscle weakness 57
palsy 75 Orbicularis muscular tone 61
torticollis 376t Orbicularis oculi muscle 76
O Orbit excision of tumor 50
trauma 30, 60, 172, 174
Oblique muscles directly 4 Oculocerebrorenal syndrome 422 Orbit syndrome, expanded 34
Oblique palsy Oculodigital sign 248 Orbital apex, region of 8
bilateral superior 376t Oculomotor abnormalities 18, 55 Orbital cavity, enlargement of 7
unilateral superior 376t Oculoplasty 1 Orbital cellulitis 78, 82
Occasional intermediate uveitis 249 procedures 446 Orbital contents, entrapment of 31
Index 475
Orbital decompression 43 Parinaud’s syndrome 39 Phaco needle tip 450f

Orbital extension 12 Parks-Bielschowsky three-step Phacoemulsification 258
Orbital fascial connective tissue, tests 376 Phacovitrectomy 258
restriction of 4 Pars plana lensectomy 312, 418 Phenylephrine 231
Orbital fat 76 Pars plana vitrectomy 256, 418 test 21
Orbital floor Pars planitis 290 Phosphaturia 422
boundaries of 35 Partial palsy 368 Photocoagulation, adequacy of 212
fracture of 30, 31f Past ocular disorders 224 Photodynamic therapy 12, 283, 307
repair 34t Patch graft, types of 104 Photophobia 72, 91, 148, 153, 188,
Orbital fracture, type of 30 Patchy iris stromal atrophy 180f 346, 395, 415
Orbital hemangioma 82 Pathological myopia 267 Photopsia 261
classify 84 Paton spatula 452f Photorefractive keratectomy 115
Orbital hemorrhage 1 Pattern-scanning retinal laser 235 Phototherapeutic keratectomy 115,
recurrent 2 Pediatric cataracts 413 124, 149, 152
Orbital implants, types of 30 Peek sign 405 complications of 150
Orbital mass 8 Pegaptanib 283 Phototherapeutic kerectomy 118
diagnosis of 8 Pellucid marginal Pierce-Hoskin forceps 442, 443f
Orbital myositis 39 corneal degeneration, Piggyback systems 112
Orbital pseudotumor 78 diagnosis of 146t Pigment dispersion syndrome 207
Orbital rim, fracture of 30 Pigment epithelial
degeneration 110, 144, 146t
Orbital septum 76 abnormalities 273
Penetrating keratoplasty 112, 118,
Orbital tumors 39 detachment 275, 279, 304
132, 143, 147, 152, 189
Orbital venography 8 Pigment epithelial-derived growth
Penetrating trauma 435
Orbital wall fractures 52 factor 283
Pentacam 111, 433
Orbito-lid apposition, grade of 58 Pigment, loss of 246
Perfluorocarbon liquid 327
Orbitotomy, anterior 49 Pigmentary glaucoma 174, 205
role of 327, 344
Orthophoria 394 Pigmentation 93
Perforated corneal ulcer 94f, 100
Ovarian failure, management of 67 Pigmented basal cell carcinoma 10f
Ovarian function, early loss of 65 Peribulbar dermoid, triad of 86
Pigmented choroidal melanoma
Ovarian insufficiency, primary 65 Perifoveal yellowish 301
294f
Oversized prosthesis, Periocular tumors 2
Pinch test 56, 60
avoidance of 29 Periodic orbital edema 2
Placement of bandage contact
Periorbital inflammation 4
lens 150
P Periorbital tumor 54
Placental growth factor 233, 283
Peripapillary atrophy 173f
Pagetoid spread, evidence of 69 Plain forceps 441f
Peripheral anterior synechia 157, Plasma
Pain proptosis, presence of 2
158, 193, 198 exchange 383
Pain, symptoms of 129
Peripheral blood smear 211 homocysteine level 219, 226
Palpebral aperture 17
Peripheral iridectomy 172, 182, 339 Plateau iris syndrome 181
Palpebral conjunctiva 11
Peripheral retinal Platelet rich plasma,
Palpebral fissure distance 53
breaks 257 complications of 233
Palpebral preauricular
fold 352f Pleomorphic adenocarcinoma 49
lymph nodes 10
Panretinal photocoagulation 194, Peripheral ulcerative keratitis 218 Pleomorphic adenoma 45-47, 49,
220, 232 Peripheral vision, loss of 250 50, 50t, 51
Papillae, presence of 107 Peritomy, closure of 268 Plexiform neurofibroma 78
Papilledema 219 Permanent loss of vision 72 Plural-colobomata 318
Papillophlebitis 380 Permanent prism 361 Pneumatic cutters 458
Paracentral scotomata 236 Permanent tarsorrhaphy 75 Pneumatic retinopexy 269
Paracentral Staphlococcus Persistent epithelial defect 230 role of 327
aureus 92 Persistent fetal vasculature 413 Pneumococcus species 90
Paralytic ectropion 57f Persistent hyperplastic primary Pneumoretinopexy,
management of 59 vitreous 190, 195, 411 principles of 269
Parapapillary atrophy 177 Peters’ anomaly 154, 155, 160, 162 Polar cataract
Parathyroid hormone 149 Peters’ plus syndrome 162 classify posterior 427
Paresis 368 Phaces syndrome 84 posterior 424, 425f, 427
Polymerase chain reaction 100 Prophylactic peripheral Pulfrich phenomenon 377

Polymorphous corneal dystrophy, iridectomy 339 Pulsatile proptosis 8
posterior 123, 130, Prophylactic treatment 194 causes of 9
131, 155 Prophylaxis, methods of 272 Pulse steroid therapy 32
Polymorphous dystrophy, Propionibacterium acnes 290 Punctal malposition 59
posterior 158 Proptosis 1, 3, 8, 18, 36, 82, 83 Pupil 73, 96, 319, 333, 359, 366, 372,
Polypoidal choroidal bilateral 9 378, 410, 416, 422, 424,
vasculopathy 305 cause of 2t, 9 428, 432, 436
Poor bell’s phenomena 54 bilateral 2, 2t examination of 7
Poor cosmetic appearance 25 consequence of 37 reaction 263
Poor dilation of pupil 205 direction of 3 sparing nerve palsy 363
Poor functional status of vision 351 early cases of 3 Pupillary margin 204f
Poor orbicularis muscle function 54 severe bilateral 37f Pupillary reaction 16, 352
Poor tumor differentiation 12 subacute course of 45 Purified protein derivative
Poor vision 31 unilateral 2, 9 skin test 288
management of 284 Proteinuria 423 Putterman’s method 17
Poor visual acuity 357 Protrusion of eye 3 PXF syndrome 203
Port-wine stain 187 Provision of low vision aids 282 Pyogenic granuloma 71, 71f
Position of lacrimal puncta 57 Proximal tubular acidosis 422
Posner-Schlossman syndrome 290 Pseudoepithelial hyperplasia 12 R
Post-enucleation socket Pseudoesotropia, causes of 399
Radial keratotomy 107
syndrome 30 Pseudoexfoliation 415
Radial optic neurotomy 221
Posterior capsular defect, glaucoma 174, 198, 203
Radiation retinopathy 219, 240
syndrome 203
management of 427 Radiation therapy 42
Pseudohypopyon 349
Post-laser medication 434 Radiotherapy, complications of 12
Pseudomacular holes 254
Postpenetrating keratoplasty Raised intraocular pressure 176
Pseudomonas 91, 92, 94 Randot test 389
herpetic keratitis 105
aeruginosa 90 Ranibizumab 283
Postperforating injury 101
Pseudophakic bullous keratopathy Rare syndromes 87
Postsurgical ptosis 22
130 Real vital dye 256
Post-traumatic atrophy of lens 437
Pseudoproptosis 8 Recalcitrant cases,
Post-traumatic impairment 39
Pseudopterygium, management of 28
Post-vitrectomized eyes 335, 340
formation of 166, 167 Recalcitrant macular edema 229
Preauricular lymph nodes 4
Pseudoptosis 55 Rectus muscles 7
Pre-Descemet’s corneal causes of 18 Rectus-holding forceps,
dystrophy 123 Pseudotumor of orbit 55
Prednisolone, dose of 43 superior 441, 441f
Psoralen plus ultraviolet A 10 Red eyes 60
Preferential hyperacuity Psychosomatic disorders 424 Red-free imaging 241
perimeter 279 Pterygium Refraction 429
Pre-glaucoma stage 196 atypical 164 Refractive accommodative 392
Preretinal blood, removal of 459 surgery 446 Refractive error 393, 395
Pre-retinal hemorrhage 233 Ptosis 15, 20, 53, 55, 63, 65, 66f, 404 Refractive index 336
Previtelliform 349 acquired 19, 19t, 21 of vitreous 336
Prisms 396 amount of 15 Refractive lens exchange 115
Progressive external classification of 19t, 21 Refractive surgery 107, 115
ophthalmoplegia, evaluation of 18 Refsum disease 247
chronic 15, 39 measurement of 17 Regional lymph nodes 4
Proliferative diabetic retinopathy mechanical causes of 16 Regular astigmatism 166
193, 209, 213, 229, 236, progression of 15 Rehabilitate aphakic patient 345
313 recurrent episodes of 15 Reis-Bücklers corneal
Proliferative vitreoretinopathy 261, repair 20, 54 dystrophy 123
265, 270, 352 section of 52 Relative afferent
signs of 352 severe 52 pathway defect 192, 436
Prominent corneal nerves 108 severity of 53, 64 pupillary defect 32, 246, 263,
Prominent scleral spur 198 to jaw movement, amount of 52 352, 378, 432
Index 477
Renal function Retinal surgery 316 Salmon patch 46

measurements 149 Retinal tears 314, 352 Salzmann nodular
test 88, 166, 224, 240, 423 Retinal vascular degeneration 125
Repetitive nerve anomalies 214 Sandhoff’s disease 303
stimulation test 406 occlusive diseases 195, 314 Scanning slit combined 110
Residual tumor, locations of 12 Retinal vasculitis 210 Schirmer’s test 18, 46, 56, 61, 73
Resolution of ulcer, early sign of 94 Retinal vein occlusion 216, 240, 252 Schnyder corneal dystrophy 123
Respiratory system 300 branch 195, 223 Schnyder’s crystalline
Retained intraocular foreign Retinal vessel dystrophy 117
body 327, 352, 355 distortion of 315f Schocket scleral depressor 461, 461f
Reticular pseudodrusen 272 rupture of normal 215 Schroeder classification 426
Retina 208 Retinitis pigmentosa 244, 247, 415 Schwartz-Jampel syndrome 65
attached 212 inversus 247 Schwartz-Matsuo syndrome 266
thinning of 257 severe 251 Scissoring of red reflex 107
Retinal aplasia 251 sine pigmento 247 Sclera 65, 95, 359, 366, 372, 410,
Retinal artery occlusion, branch 195 stages of 244 416, 422, 424, 428, 432,
Retinal break 265 Retinitis punctata albescens 247 436, 441
types of 353 Retinitis sclopetaria 352 Scleral buckling 268, 327
Retinal capillary nonperfusion 241 Retinoblastoma 265, 411 surgery, steps in 268
Retinal change in diabetes, Retinochoroidal coloboma 323, 324 Scleral involvement 101
sign of 242 Retinopathy 242 Scleral rupture 352
Retinal cryopexy, anterior 194 decreases, progression of 237 Scleral wounds,
Retinal cryotherapy, anterior 213 in diabetes 234 signs of old repaired 351
Retinal cyst 263 of prematurity 190, 195, 210, Scleritis 263
Retinal detachment 195, 198, 260, 262, 411 posterior 305
262, 262t, 270, 286, 290, severity of 237 Scleroderma 424
314, 318, 320, 330, 352, to anemia 219 Sealed capsular irrigation 435
354, 411, 413, 415, 429, Retinoschisis 263 Sebaceous adenomas 70
436 Retinoscopy 422 Sebaceous carcinoma 69, 70
diagnosing 263 Retinovascular disease 240 in systemic disease 71
in myopes 267 Retroillumination, presence of 184 Sebaceous cell carcinoma 11, 68, 69
management of 324 Retropulsion test 4 Sebaceous epitheliomas 70
presence of 210 Retropulsion theory 34 Sebaceous gland carcinoma 9, 10f,
surgery for 265t Rhegmatogenous retinal 13, 67, 68f
types of 261t detachment 257, 260, Secondary glaucoma 429
Retinal dialysis 198, 353 265, 325 Secondary tumor 3
Retinal disorders 262 sign of 342 Segment coloboma, posterior 321
family history of 351 Ribonucleic acid 233 Segment cysticercosis,
Retinal folds 269 Rigid gas permeable 107, 111 posterior 329
lenses 111, 144
Retinal ganglion cell 383 Self-retaining barraquer eye
Ring sctoma, presence of 248
Retinal hemorrhages 239f speculum 440
Ring shaped ulcer 93
Retinal hypoxia 196 Semi-integrated implants 30
Rizzuti phenomena 109
Retinal incarceration 269 Senile cataract occurs 238
Rizzuti’s sign 115
Retinal ischemia Senile ectropion 57f, 62
Roof of orbit, development of 9
cause of 192 Sensory detachment 311
Rosette cataract 436
evidence of 193 Sentinel lymph node 13
Rothmund disease 424
Retinal laser 237 biopsy 13
Rubella 410, 411
Retinal mass 263 Serum
Rubeosis iridis 196
Retinal necrosis, acute 290 angiotensin converting
presence of 194
Retinal nerve enzyme 219
Rupture 355
fiber layer 174, 177, 383, 384 calcium 149
thickness 174 galactokinase 411
Retinal pigment
S lipids, management of 237
epithelial tear 276 Sabouraud’s dextrose agar 98 phosphorus 149
epithelium 260, 272, 294, 306 Saddle-shaped instrument 443 uric acid 149
Seven rings of trauma, discuss 439 Spastic entropion Subcutaneous emphysema 31

Severe complicated ptosis, acute 61 Subcutaneous tissue 76
right-sided 24f causes of 63 Subepithelial dystrophy 122
Severe contracted socket, Spectacle 144 Subepithelial fibrillary lines 109
management 28 Specular biomicroscopy 205 Subepithelial mucinous corneal
Severe visual loss, harbinger of 229 Spheroidal degeneration 150, dystrophy 123
Shaffer’s sign 210 151f, 153 Subhyaloid hemorrhage 184
Shallow anterior chamber 179f Spindle cell melanoma 298 Subluxated lens 414
Sheridan-Gardiner test 410 Spongiform 311 etiology of 415t
Sickle cell disease 209, 214 Squamous cell carcinoma 9, 11, Subluxation of lens 87
Silicone brush tip cannula 459 12, 69, 70 Subretinal bleed 269, 352
Silicone oil 336 of eyelid 10f Subretinal choroidal
disadvantages of 270 Squamous layer of epidermis 11 neovascularization 305
filled eyes 336, 340 Squamous papilloma 72 Subretinal cysts 330
induced secondary Squint 351 Subretinal exudates 263
glaucoma 337 examination for 372, 401 Subretinal fluid 254, 306f
removal 339 tests for 359 internal drainage of 459
role of 258, 327 Staphylococcus aureus 90-92 Subretinal hemorrhages 198
Singh classification 425 Staphyloma, posterior 429 Sulcus defect, superior 18
Single-fiber Stargardt disease 346-348, 349t Sulfite oxide deficiency 415
electromyogram 22 pathophysiology of 348, 349 Sunglasses 42
nerve electromyography 406 Stereopsis 389 preferred, type of 251
Sinusotomy 176 lack of 263 Superficial foreign body 355
Steroid 36, 101, 137 Superficial keratectomy 124, 149
Sixth cranial nerve palsy 364
implants, discuss 291 Superior rectus, right 373
Sixth nerve palsy 368t
induced glaucoma 200-202 Superotemporal displacement 416f
causes of 369t
prevention of 201 Surgery
diagnosis of 367t
side effects of 291 complications of 167, 328, 402
right 365f, 366f
therapy, discontinuation of 200 for ectropion, selection of 58
Sixth nerve, course of 369
type of 202 performed, type of 25
Sjögren syndrome 46
Steroid-sparing immunosuppressive type of 195
Skeletal anomalies 89
drugs 42 Swimming goggles 407
Skewed radial axes 116
Steven tenotomy scissors 446, 446f Swiss cheese 46, 51
Skin 76
Steven-Johnson syndrome 29, 60, Sympathomimetic drugs 39
atrophy 12 62, 134 Syndromic retinitis pigmentosa
biopsy punches 452 Stiles-Crawford effect 307 245t
Sleep test 405 Still’s syndrome 150 Syndromic variants 87
Sling surgery, complications of 55 Stimulation deprivation Synechiae
Slit-lamp amblyopia 164 anterior 135
biomicroscopy 164 Stool examination 331 posterior 184
examination 61 Strabismic amblyopia 164 Synkinetic ptosis 19
Small graft 139 Strabismus 16, 82, 88, 164, 166, Synoptophore 359
Smear examination, result of 97 356, 401, 409, 421 test 389
Smith-Lemli-Opitz syndrome 65 Striational antibodies 405 Systemic antibiotic 101
Smokestack leak 305 Stromal dystrophy 123 Systemic anticoagulants, role of 221
Smoking 230, 237 types of 118 Systemic antimicrobial agents,
history of 36 Stromal scars 145 use of 95
Snailtrack degeneration 266 Sturge-Weber syndrome 173, 185, Systemic chemotherapy 297
Snap-back test 56, 60 187t, 190 Systemic disease 91
Snellen’s entropion clamp 455, 455f Subcapsular cataract, posterior 246, Systemic disorders 190
Soemmering’s ring 432f, 433, 249, 335, 425 Systemic inflammatory disease 2
434, 434f Subcapsular opacities 436 Systemic medications 172, 179
Soft silicone tip cannula 460, 460f Subconjunctival Systemic myasthenia 404
Soft tissue, herniation of 32 hemorrhage 32, 197 Systemic steroid 137
Solar retinopathy 254 Subconjunctival mass 7 therapy 79
Index 479
T dust 263, 267 influence, depth of 90

smoking 43 progressive 93
Taenia solium 329, 331
Tonometry 78, 164 Umbilical hernia 89
Tarsoconjunctiva 58 Universal metallic eye
Topical mitomycin C application 12
combined, excision of 58 speculum 440
Topical steroids 137, 182
scarring of 58 Upper eyelid
Torsion, evaluation of 373
Tarsorrhaphy, limitation of 75 coloboma 89, 164
Total detachment of retina 251
Tay-Sachs disease 303 fornix 87
Toxocariasis 411
Tears, artificial 42 retraction 18, 43, 75, 444
Toxoplasmosis 410, 411
Tectonic patch graft 104 Upper lid
Trabecular meshwork 171
Teflon block 452 coloboma 88, 89t
Trabeculectomy 186, 195, 201
Telangiectasia’s, small 68 retraction 39
Trabeculotomy 186, 189
Tenon’s patch graft 104 Urine examination 411, 423
Trachoma 62
Tensilon test 22, 405, 406 Usher syndrome 247
Traction on retina 458
Tension glaucoma, normal 200 Utrata capsulorhexis
Tractional retinal detachment 211,
Teratoma 78, 81 forceps 442, 442f
219, 230, 231, 260, 262
Terrien’s marginal Uveal inflammation 96
Transient ischemic attacks 233
degeneration 146 Uveitis 212
Transmitted cerebral pulsations 2
Terson’s syndrome 210, 212, 214 anterior 184
Transpupillary
Therapeutic keratectomy micro chronic 247
thermotherapy 283, 307
keratome system 454f posterior 263, 316
Trans-scleral
Therapeutic penetrating signs of 205, 225
cyclophotocoagulation 195
keratoplasty 104
panretinal cryotherapy 194
Thermal injury 29
Trauma V
Thiazide diuretics 68
effects of 32 van Herick’s method 183
Thick hyaloid 263
history of 52, 91, 208, 333, 341, Vannas scissors 447f
Thick mature epiretinal
351 Variable intraocular pressure 37
membrane 315f
seven rings of 354 Vascular disease 316
Thiel-Behnke corneal dystrophy 123
signs of 197, 198, 254, 263, 351 Vascular endothelial growth factor
Third cranial nerve
Traumatic hyphema 199 233, 234, 307
course of 363
Traumatic ptosis 23 Vascular occlusion 209
palsy 53, 356, 362
Traumatic retinal type of 216
Third nerve palsy, causes of 364
detachment 350, 354 Vascular sclerosis, gross signs of 219
Thudichum nasal speculum 456,
Treacher-Collins syndrome 89 Vascularization of host cornea 139
457f
Trephines, types of 451 Vasculitis 240, 301
Thymectomy 407
Treponemal serology 219 Vaso-inhibitory factors 197
Thyroid Vasospastic diseases 172
Triamcinolone acetonide 256
associated ophthalmopathy 35 Vein occlusion, branch 227
Trocar and cannula 457
disorder 2 Venous beading 239f
Tube surgery 189
eye disease 37f Tumor 208 Venous malformations 85
function test 7, 39, 73, 406, 411 diameter 12 Venous stasis retinopathy 219
gland dysfunction, treatment of extent of 50 Vernal keratoconjunctivitis 106
42 recurrent 12 Viral keratitis 103t, 104
involved 408 type of 4 management of 102
ophthalmopathy 7 Tunica vasculosa lentis 427 Virectomy, indications of 213
treatment of 42 Tunnel vision 249 Viscocanalostomy 176
related ophthalmopathy 3, 35 diagnosis of 250 Viscosity silicone oils,
Thyroxine 44 Typical optic neuritis 381t use of low 340
Tight medial rectus muscle 394 Vision 350
Tissue achieved, quality of 114
adhesives 103
U
acute loss of 230
diagnosis 296 Uhthoff phenomenon 377 better quality of 112
Titmus test 389 Ulcer blurring of 73, 77, 86, 117, 118,
Tobacco base of 93 144, 148, 153, 163, 233,
chewing 230 edge of 98 285
deterioration of 10 Vitamin Vitritis 211

diminution of 15, 63, 77, 86, 90, A Vogt Kayanagi Harada’s disease 195
163, 329, 346 palmitate 249 Vogt’s striae 108
dimness of 192 supplements, avoidance of Volkmann’s ischemic
gradual loss of 230 high-dose 348 contracture 31
gradual painless loss of 337 C 282 Voltage-gated calcium channel
in eye, gradual painless E 282 antibodies 406
loss of 272 Vitelliform 349 Vomiting 329
loss 1, 91, 314, 341 Vitelliruptive 349 von Graefe knife 445
causes of 290, 333, 234 Vitrectomy 268, 324 von Graefe retractor 443, 443f
progression of 172 benefits of early 234
cutter 458, 458f
preceding pain,
in diabetic patients 234 W
dimness of 192
role of 221 Waardenburg syndrome 422
progressive loss of 118
sequential 258 Warburg syndrome 322
sudden diminution of 300
with scleral buckle, role of 328 Warthin tumor 47
sudden loss of 144, 272
year of 333 Watzke-Allen test 254
Visual acuity 3, 16, 52, 253, 286, Vitreomacular adhesion 225, 309
304, 318, 325, 357, 365, Wavy vision 346
Vitreomacular tension, Weill-Marchesani
372, 378, 385, 410, 416, symptoms of 252
431, 436 syndrome 415t, 428
Vitreomacular traction 240, 315 Westcott scissors 447, 447f
best corrected 106, 135, 230, syndrome 254 White blood cells 95
237, 341, 436 Vitreoretinal pathology 337 White eyed blowout fracture 34
best spectacle corrected 431 Vitreoretinal procedure 333 Wildervanck syndrome 402
decreased 91 Vitreoretinal scissors 460, 460f
Wilson disease 414
gain of 228 Vitreoretinal surgery 333, 440, 457
Wire vectis 448f
loss of 346 Vitreoretinal traction 268
Worm’s eye 3
significant loss of 91 Vitreous 314, 378, 410, 422, 425, 428,
Worsen diabetic retinopathy 234
uncorrected 230, 341 432, 437
Worsening of ptosis 15
Visual axis 151, 166 anterior 210, 285, 326
Wound closure 412
opacification 413 base 264
Visual complaints, avulsion 352
progress of 223 body 309 X
Visual correction, type of 111, 144 degeneration 211
Xanthelasma 237
Visual evoked detachment, posterior 210, 212,
potential 379 214, 231, 261, 263, 314,
response 353, 379, 411 326 Y
Visual field 220 gel, syneresis of 210
haze 290 Yellow-gold spherules 151f
defect 257, 261
hemorrhage 198, 208, 210, 219,
loss of 233
loss, advanced 172
230, 263, 290, 314, 352, Z
411
normal 174 Zeaxanthin 282
causes of 215t
testing 21, 347 Zellweger’s syndrome 190
resorption of 233
Visual function 251 Zimmerman’s hypothesis 299
incarceration 327
Visual loss 118, 172, 179, 261 loss 205, 262 Zonular cataract 409, 410f
significant 124 membranes 263 retroillumination 410f
Visual potential 195 pigments 210, 342 Zonular coloboma
assessment 437 prolapse 269 inferiorly 319f
Visual rehabilitation 249, 348 severe 233 Zonular dialysis 205
postoperative 412 spooling 458 Zonular opacity 436
Visual requirements 317 substitute 270t Zonular weakness,
Visual symptoms 184 function of 258 cause of 206
Visualization of gadolinium- role of 258 Zonules 416
enhanced lesions 8 syneresis 263 Zygomatic complex fracture 31

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OPHTHALMOLOGY CLINICS

Prafulla Kumar Maharana MD DNB

Atul Kumar MD FAMS

The Health Sciences Publisher

Overseas Offices Jaypee-Highlights Medical Publishers Inc.

My parents, Dr Ramesh C Sharma and Mrs Maitreyi Pushpa

My late parents, Mr Sanat Kumar and Mrs Swarna Kumar

Atul Kumar MD FAMS Bijnya Birajita Panda MS FICO (UK)

Brijesh Takkar MD FICO FAICO

Amar Pujari MD Devesh Kumawat MD DNB

Ashish Markan MBBS Devika S Joshi MS

Dewang Angmo MD DNB MNAMS FRCS

Aswini Kumar Behera MD

Dheepak Sundar MD Jyotiranjan Mallick MS (Ophthal)

Divya Agarwal MBBS Karthikeya R MD DNB

D Satyasudha MBBS Mandeep S Bajaj MD

Esha Agarwal MD Manpreet Kaur MD

Neelima Aron MD DNB FICO

Patil Mukesh Prakash MBBS MD FICO

Prafulla Kumar Maharana MD DNB

Prakhar Goyal MBBS DNB Rajesh Sinha MD

Prateek Kakkar MD DNB FICO FAICO Ruchita Falera MD DNB FICO

Priyanka Ramesh MBBS Ruchir Tewari MD

Pulak Agrawal MD Sagnik Sen MBBS

Raghav Ravani MD Sandeep Gupta MS DNB MNAMS

Rajesh Pattebahadur MD Sapna Raghuwanshi MS

Saranya Devi K MD DNB FICO Talvir Sidhu MD DNB

Shipra Singhi MD Tushar Agarwal MD

Shorya Vardhan Azad MS

Shreyas Temkar MD DNB FICO FAICO Varsha Varshney MBBS MS

Swati Phuljhale MD Yamini Attiku MBBS

“The whole art of medicine is in observation”.

Prafulla Kumar Maharana

2. CORNEA AND CONJUNCTIVA 90

• Central Retinal Vein Occlusion 216

• Branch Retinal Vein Occlusion 223

• Proliferative Diabetic Retinopathy 229

• Nonproliferative Diabetic Retinopathy 236

• Age-related Macular Degeneration 272

5. NEURO-OPHTHALMOLOGY AND STRABISMUS 356

• Posterior Capsular Opacification 431

INTRODUCTION hemangioma and Graves’ disease] or induced

inflammatory and infective process,

complaints: suggests tumors, lymphoma—prolifera­

of optic nerve such as meningiomas or optic however, gradual progression with

Table 1 Causes of proptosis based on the age of onset

—— Rapid progression: It indicates infection/ Table 2 Common causes of bilateral proptosis

can be there in some malignant tumors History of Past Illness

sinus, lacrimal sac tumors, Lym­

Glioma of optic nerve (Fig. 1), Optic

zz Ocular motility: Ocular motility disturbance separated

—— Involvement of the rectus or oblique only

been described in Table 3. facial vein), medial upper lid, central

Table 3 Lid signs in proptosis

Causes are multiple and include: • Orbital varies

INTRODUCTION The common periocular malignancies are basal

HISTORY zz Ulceration of the lid margins

plane. The defect is then reconstructed by the —— Ectropion

2. CORNEA AND CONJUNCTIVA 90

5. NEURO-OPHTHALMOLOGY AND STRABISMUS 356

complaints: suggests tumors, lymphoma—prolifera

sinus, lacrimal sac tumors, Lym

Pupil: Pupillary examination is important in With oculomotor abnormalities

the evaluation of ptosis. Pupil abnormalities With blepharophimosis syndrome

Synkinetic ptosis zz The difference from normal in bilateral cases

• A large pupil might be a sign of third- • S

—— Limitation: It is subjective (depends on Vision: 1 point