

PROPTOSIS


Prepared by: Dr Ahmad Fayaz ‘Rajabzada’

 Proptosis

 Orbit has rigid walls;
any space occupying
lesion of the orbit causes
eyeball to protrude
forward or also displace
in some other direction,
i.e. medial, lateral, up or
down depending on
location of the pathology
in the orbit.
Pseudoproptosis

 Apparently proptosed eyeball
though it is normal in position
 This happens with
enophthalmos of the
contralateral eye due to
previous forgotten trauma
such as orbital blowout
fractures.
 Lid retraction and high
myopia can also make the
eyeball look proptosed
 Proptosis can be measured by
exophthalmometer.
AETIOLOGY

 shows the various conditions
causing proptosis and can be
remembered by the acronym
of VEIN.

Vascular:
 Venous varix, cavernous
haemangioma, carotid-
cavernous fistula
a Patient : Arteriovenous malformation of the
left orbital and cavernous sinus,
demonstrating proptosis of the left eye with
corkscrew episcleral blood vessels.
b Patient 12. A vascular malformation of the
right orbit. Axial CT image demonstrating a
lesion in the right orbit that later was
diagnosed as an AVM.
c Patient 18. Compound capillary
hemangioma.
Endocrinal:

Graves’ disease
which may cause
bilateral or
sometimes even
unilateral
proptosis
 Inflammations and infections:

 Idiopathic orbital
inflammation
(pseudotumour of orbit),
orbital cellulitis or abscess,
mucormycosis or
aspergillosis of sinuses,
Wegener’s granulomatosis,
inflammations of lacrimal
gland.
Neoplastic:

 Tumours (both benign and
malignant) or tumour-like
conditions arising from the orbital
contents or its adjoining
structures.
 Orbit contains eyeball, optic nerve,
muscles, nerves, blood vessels and
lacrimal gland and tumour and
tumour-like conditions can arise
from them. They can also arise
from paranasal sinuses and cranial
cavity and invade the orbit.
 
 (a) Primary tumours of orbit, its walls or adnexa (lid, lacrimal gland and
conjunctiva). Dermoid cyst, cavernous or capillary haemangioma,
 schwannoma, glioma, retinoblastoma, fibrous dysplasia, osteoma,
histiocytosis X, orbital meningioma, pleomorphic adenoma of
 lacrimal gland. Malignant tumours include rhabdomyosarcoma,
lymphoma, leukaemic deposits, malignant tumours of lacrimal gland
 and melanoma of choroid.
 (b) Tumours of paranasal sinuses. Mucocele of frontal or ethmoidal
sinuses, inverted papilloma, angiofibroma, malignant tumours of
sinuses.
 Tumours from cranial cavity. Meningioma of the sphenoid ridge.
 (c) Metastatic tumours. Carcinoma breast (most common), lung, prostate,
kidney, thyroid, gastrointestinal trac
 1. Idiopathic orbital inflammation.

 Cause is uncertain. 
 It may be diffuse or localized to specific structures in the orbit, e.G.
Muscles (myositis), lacrimal gland, sclera (scleritis) or optic nerve
(perineuritis).
 Patient complains of dull orbital pain especially on eye movements.
 Proptosis is seen in 70-80% of patients.
 Ct scan with enhancement shows enlargement of the affected
structures.
 An important feature is involvement of muscle and its tendon
attached to the globe
 Differentiates it from thyroid-related disease where only muscle
belly is involved but not its tendon.
 Biopsy shows nonspecific inflammation without evidence of
vasculitis.
 Treatment is oral steroids. In some cases, immunosuppression with
cyclophosphamide, cyclosporine or radiotherapy may be required.
 2. Graves ophthalmopathy:

 This is the most common cause of bilateral and
sometimes unilateral proptosis.
 Patient is hyperthyroid but sometimes he is
euthyroid or even hypothyroid.
 Lid retraction and lid lag may be present with
chemosis and lid oedema.
 CT scan is useful to differentiate it from idiopathic
orbital inflammation (vide supra).
 Visual loss can occur.
 Extreme proptosis causes corneal ulceration and
may require orbital decompression which nowadays
can be done endoscopically through the nose.
 3. Haemangioma of orbit.

 It can be cavernous or capillary.
 Cavernous haemangioma is the most common benign tumour in
adult.
 It is more common in females in the age group of 18-67 years.
 It manifests as painless, progressive, unilateral proptosis.
 CT/MRI reveals a round or oval mass without associated
inflammation or infiltration around it.
 It is an encapsulated mass.
 Intraconal in location and enhances on i.v. contrast.
 Treatment is complete excision with its capsule by lateral
orbitotomy.
 4. Capillary Haemangioma.

 Most common tumour of orbit in infants and children.
 May be isolated or associated with a lesion on the
upper lid or elsewhere on the skin.
 Most of them involute by age 7.
 CT/MRI with contrast is diagnostic.
 Tumour is nonencapsulated and infiltrates the
surrounding structures.
 Local or systemic steroids help to involute the mass.
 Total excision is not possible.
5. Venous varix of the orbit

 . It presents with positional proptosis and
congestion.
 Proptosis can also be induced by Valsalva
manoeuvre.
 A carotid-cavernous fistula is either spontaneous or
traumatic;
 it presents with pulsatile proptosis, bruit, visual loss,
dilated and arterialized blood vessels in the
conjunctiva or limbus.
6. Lymphoma:

 It is the most common malignant tumour of adults.
 It may be isolated or associated with systemic disease.
 Most of the patients are between 50 and 70 years with female
preponderance.
 It presents as painless progressive exophthalmos. Usually lesions are
located anteriorly and can be palpated or seen under the conjunctiva.
 Most of them are extraconal.
 CT shows a homogenous tumour without bone involvement.
 Biopsy is necessary to differentiate it from the benign lymphoid or
other tumours.
 Isolated lymphoma can be treated by radiation alone while systemic
ones require chemotherapy in addition to orbital radiation.
 7. Rhabdomyosarcoma.

 It is the most common primary malignant tumour
of orbit in children and is usually seen at 6-7
years of age.
 It can occur even in the newborn.
 It presents as painless but progressive proptosis
and can spread to the adjoining paranasal
sinuses.
 It may be intraconal or extraconal.
 CT is helpful in diagnosis.
 Biopsy should be taken.
 Treatment is radiation and chemotherapy.
 Five-year survival of 90% can be achieved in
localized disease.
8. Dermoid cyst.

 It is the most common benign tumour of
orbit in children.
 It is due to the trapped ectoderm that occurs
at suture lines during development.
 Deep dermoids of orbit arise from the
sphenoethmoid or sphenozygomatic sutures.
 They may remain asymptomatic till adult
age.
 They present with painless, progressive
proptosis with globe displacement.
 CT orbit may show a cyst with pressure
effects .
 Large cysts may communicate with temporal
fossa, paranasal sinuses or the cranial cavity.
 Treatment is surgical excision.
 9. Tumours of optic nerve.

 Glioma of optic nerve
is usually seen in
children and may be
associated with
neurofibromatosis.
 It causes progressive
proptosis and visual
loss.
EVALUATION OF PROPTOSIS


 A case of proptosis requires a detailed history including
onset, duration and progression of the disease.
 Associated illnesses (thyroid disease, tumours of nose
or paranasal sinuses,
 systemic disorders such as leukaemia, lymphoma,
 Wegener’s granulomatosis) should be looked for.
 Pain is a feature of inflammation or infection.
 Visual loss may be present and should be
documented.
Physical examination

 Type of proptosis (straight forward or of globe
displacement in upward,
 downward, lateral or medial direction),
 condition of the conjunctiva (swelling and chemosis),
scleral appearance, ocular movements and vision.
 Note should also be made if the proptosis is
pulsatile or associated with change in position of the
head or appears on performing Valsalva (venous
varix).
Diagnostic Exams

 CT and MRI are important and give clue to the type of
tumour (intraconal/extraconal), smooth or infiltrative,
location in the orbit and its extent, any changes in the
adjoining bone or extension to sinuses or cranial cavity.
 They can help to differentiate thyroid orbitopathy from
the idiopathic orbital inflammation.
 Ultrasonography may be required to find abscess or
cystic lesions.
 FNAC or biopsy of the lesion may be required for
histologic diagnosis.
 
Systemic diseases causing orbital lesions such as
lymphoma, Grave’s disease and leukaemia should be
investigated as mandated by history and clinical
examination and relevant investigation.
MANAGEMENT

 Imaging techniques help to make the diagnosis.
Biopsy of the lesion can be taken depending on its
location in the orbit.
 Anteriorly located lesions can be approached by lid
or conjunctival incision.
 Excisional biopsy is useful in encapsulated and well-
circumscribed lesions such as dermoid, cavernous
haemangioma and pleomorphic adenoma of the
lacrimal gland.
 All cases causing proptosis do not require surgery.
Medical treatment

 Includes antibiotics in orbital cellulitis, steroids in pseudotumour,
chemotherapy for lymphoma, radiation for malignancies and
sometimes pseudotumour.
Surgery of orbit includes debulking of lymphangioma or
plexiform neurofibroma to relieve pressure on the optic nerve
 Orbital exenteration for mucormycosis and malignancies.
Endoscopic orbital decompression may be required in Graves
ophthalmopathy.
 Lateral orbitotomy is required for lesions of lacrimal gland or
those situated intraconally.
 Transcranial approach is used for lesions at the orbital apex or
those invading intracranially from the orbit or vice versa.
Remember:

 In children, dermoid cyst of the orbit is the most
common benign tumour and rhabdomyosarcoma the
malignant one.
 In adults, cavernous haemangioma is the most
common benign tumour of orbit and lymphoma the
malignant one.
