Strabismus PDF

You might also like

Download as pdf or txt
Download as pdf or txt
You are on page 1of 170

STRABISMUS

STRABISMUS

Himadri Datta MS
Associate Professor
Regional Institute of
Ophthalmology
Kolkata

JAYPEE BROTHERS
MEDICAL PUBLISHERS (P) LTD
New Delhi
Published by

Jitendar P Vij
Jaypee Brothers Medical Publishers (P) Ltd
EMCA House, 23/23B Ansari Road, Daryaganj
New Delhi 110 002, India
Phones: 23272143, 23272703, 23282021, 23245672, 23245683
Fax: 011-23276490
e-mail: jpmedpub@del2.vsnl.net.in
Visit our web site: http://www.jpbros.20m.com

Branches
• 202 Batavia Chambers, 8 Kumara Kruppa Road
Kumara Park East, Bangalore 560 001, Phones: 2285971, 2382956
Tele Fax : 2281761
e-mail: jaypeebc@bgl.vsnl.net.in
• 282 IIIrd Floor, Khaleel Shirazi Estate, Fountain Plaza
Pantheon Road, Chennai 600 008, Phone: 28262665 Fax: 28262331
e-mail: jpmedpub@md3.vsnl.net.in
• 4-2-1067/1-3, Ist Floor, Balaji Building, Street No.6, Ramkote Cross Road,
Hyderabad 500 095, Phones: 55610020, 24758498 Fax: 24758499
e-mail: jpmedpub@rediffmail.com
• 1A Indian Mirror Street, Wellington Square
Kolkata 700 013, Phone: 22451926 Fax: 22456075
e-mail: jpbcal@cal.vsnl.net.in
• 106 Amit Industrial Estate, 61 Dr SS Rao Road
Near MGM Hospital, Parel, Mumbai 400 012
Phones: 24124863, 24104532 Fax: 24160828
e-mail: jpmedpub@bom7.vsnl.net.in

Strabismus
© 2004, Himadri Datta
All rights reserved. No part of this publication should be reproduced, stored in a
retrieval system, or transmitted in any form or by any means: electronic, mechanical,
photocopying, recording, or otherwise, without the prior written permission of the
author and the publisher.
This book has been published in good faith that the material provided by author
is original. Every effort is made to ensure accuracy of material, but the publisher,
printer and author will not be held responsible for any inadvertent error(s). In
case of any dispute, all legal matters to be settled under Delhi jurisdiction only.
First Edition : 2004
ISBN 81-8061-246-5
Typeset at JPBMP typesetting unit
Printed at Gopsons Papers Ltd, A-14, Sector 60, Noida 201 301, India
The book is dedicated to
my teachers
my family members
my patients
Preface

Many textbooks on strabismus by great strabismologists are available. However,


the subject is still considered difficult to understand. The present attempt is a humble
one with the goal to present the subject in a simplified way.

Himadri Datta
Contents

1. Anatomy of Extraocular Muscles and Their Fascia 1


2. Development of Vision-Binocular Vision, Suppression 15
3. Amblyopia 24
4. Classification of Strabismus and Approach to a Patient
with Strabismus—Basic Guidelines 31
5. Heterophoria, Accommodation and Convergence Insufficiency 56
6. Exotropia 68
7. Esotropia 74
8. A, V, Y, X Pattern Strabismus 90
9. Paralytic Squint 95
10. Vertical Strabismus 104
11. Mechanical Restrictions and Syndromes 115
12. Non-Surgical Management of Strabismus 125
13. Surgical Management of Strabismus 133
14. Nystagmus 154
Index 159
1
Anatomy of Extraocular
Muscles and Their Fascia

There are six extraocular muscles of the eyeball: the four recti muscles and the
two oblique muscles. The extraocular muscles develop from three different masses
of primordial cells. In the presomite embryo, cells proliferate laterally from a cen-
tral mass located at the anterior end of notochord. In the 25 day-old embryo a pair
of premandibular condensations can be seen, which give rise to the group of eye
muscles (superior, inferior and medial recti and inferior oblique) later innervated by
the oculomotor nerve. The lateral rectus and superior oblique muscles arise from
its own adjacent tissue mass in the maxillo-mandibular mesoderm. At 1 month the
ocular motor nerves reach their respective muscles. The muscle striations appear
early in the 2nd month. The trochlea forms at 6 weeks.
The lateral rectus muscle is supplied by the abducens nerve (6th nerve), the
superior oblique muscle is supplied by the trochlear nerve (4th nerve) and the
remaining muscles are supplied by the oculomotor nerve (3rd nerve). The oculomotor
nerve has two divisions: the upper division supplies the superior rectus muscle (and
also the levator palpebrae superioris); the lower division supplies the medial rectus,
the inferior rectus and the inferior oblique muscles. The parasympathetic nerve
supply to the sphincter pupillae muscle and the ciliary muscle traverses with the
branch of the lower division of the oculomotor nerve that supplies the inferior
oblique muscle.
When the eye is directed straight ahead with the head also being straight, the
position of the eye is defined as it’s primary position. The primary action of a
muscle is considered as the major effect on the position of the eye when the
muscle contracts while the eye is in primary position. The secondary and tertiary
actions of a muscle are the additional effects on the position of the eye in the
primary position. The eyeball can be moved 50° in each direction from the primary
position.
Horizontal rectus muscles: The horizontal rectus muscles are the medial rectus
and the lateral rectus muscles. They arise from the annulus of Zinn, courses ante-
riorly along the medial and lateral orbital walls, and insert 5.5mm and 6.9mm from
the limbus, respectively. The medial and lateral recti have only horizontal actions,
with the medial rectus being an adductor and the lateral rectus being an abductor.
Vertical rectus muscles: The vertical rectus muscles are the superior rectus and
inferior rectus muscles. The superior rectus muscle originates from the annulus of
2 Strabismus

Zinn, courses anteriorly, upwards over the eyeball and laterally forming an angle of
1 23° with the visual axis of the eye in the primary position. It inserts 7.7mm from the
limbus. The inferior rectus muscle arises from the annulus of Zinn, courses anteri-
orly, downwards, and laterally along the floor of orbit, forming an angle of 23° with
the visual axis of the eye in the primary position. It inserts 6.5mm from the limbus.
The superior rectus muscle’s primary action is elevation; secondary actions are
adduction and incycloduction or intortion. The inferior rectus muscle’s primary action
is depression; secondary actions are adduction and excycloduction or extortion.
Oblique muscles: The superior oblique muscle originates from the annulus of Zinn
and passes anteriorly and upwards along the superomedial wall of the orbit, be-
coming tendinous before passing through the trochlea located on the nasal side of
the superior orbital rim. The tendon is then reflected inferiorly, posteriorly and
laterally, forming an angle of 51° with the visual axis of the eye in the primary
position. It inserts in the posterosuperior quadrant of the eyeball, almost or entirely
lateral to the mid-vertical plane or center of rotation, passing inferior to the supe-
rior rectus muscle. The primary action of the superior oblique muscle is incycloduction
or intorsion and the secondary actions are depression and abduction.
The inferior oblique muscle originates from the periosteum of the maxillary
bone, just posterior to the orbital rim and lateral to the orifice of the lacrimal fossa.
It passes laterally, superiorly and posteriorly, going inferior to the inferior rectus
muscle and under the lateral rectus muscle to insert in the posterolateral portion of
the eyeball, below the horizontal meridian. As it passes, it forms an angle of 51°
with the visual axis of the eye in the primary position. The primary action of the
inferior oblique muscle is excycloduction or extortion; secondary actions are
elevation and abduction.
Blood supply of the extraocular muscles: The most important blood supply is
from the medial and lateral branches of the ophthalmic artery. The lateral muscu-
lar branch supplies the lateral rectus, superior rectus and superior oblique muscles;
the medial muscular branch (the larger one) supplies the inferior rectus, medial
rectus and inferior oblique muscles.
The lacrimal artery partially supplies the lateral rectus muscle; the infraorbital
artery partially supplies the inferior oblique and inferior recti muscles.
The muscular branches give rise to seven anterior ciliary arteries accompany-
ing the four recti muscle; each rectus muscle has two anterior ciliary arteries,
except the lateral rectus muscle which has only one. These pass to the episclera,
and then supply blood to the sclera, limbus and conjunctiva. The venous system
parallels the arterial system, emptying into the superior and inferior orbital.
The Venous system parallels the arterial system, emptying into the superior and
inferior orbital veins.
Anatomy of Extraocular Muscles and Their Fascia 3

Table 1.1: Showing origin, insertion, action and nerve supply of the extra ocular muscles

Muscle Length of
active mus-
Origin Insertion Direction Tendon Arc of Action
of pull length contact
Innervation
(cranial
1
cle(mm) (mm) (mm) nerves)
Medial 40 Annulus of 5.5 mm 90° 4.5 7 Adduction 3rd nerve
rectus Zinn from (lower
limbus division)
Lateral 40 Annulus of 6.9-7 mm 90° 7 12 Adduction 6th nerve
rectus Zinn from
limbus
Superior 40 Annulus of 7.7-8 mm 23° 6 6.5 Elevation 3rd nerve
rectus Zinn limbus Intorsion (upper
Adduction division)
Inferior 40 Annulus of 6.5 mm 23° 7 6.5 Depression 3rd nerve
rectus Zinn from Extorsion (lower
limbus Adduction division)
Superior 32 Orbital apex Posterior 51° 26 7.8 Intorsion 4th nerve
oblique above to equator Depression
Annulus of in supero- Abduction
Zinn(Funct- temporal
inal origin at quadrant
the trochlea)
Inferior 37 Behind Posterior 51° 1 15 Extortion 3rd nerve
oblique lacrimal to equator Elevation (lower
fossa in infero- Abduction division)
temporal
quadrant

Fig. 1.1: Insertions of the Recti muscles Fig. 1.2: Origins of the Recti muscles and
the superior oblique muscle
MUSCLE ACTIONS
• All the RECTI are ADDUCTORS except LATERAL RECTUS.
• Both the OBLIQUES are ABDUCTORS.
• Both the SUPERIOR MUSCLES are INTORTERS.
• Both the INFERIOR MUSCLES are EXTORTERS.
4 Strabismus

Fig. 1.3: Origin, course and insertion Fig. 1.4: Origin, course and insertion of
of the superior oblique muscle the inferior oblique muscle

Fig. 1.5: Actions of the extraocular muscles

PRIMARY, SECONDARY AND TERTIARY ACTIONS


Horizontal recti are purely horizontal movers around the vertical axis and they
have a primary action only.
Vertical recti have a direction of pull that is mostly vertical as their primary
action, but the angle of pull from origin to insertion is 23 degrees inclined to the
visual axis, giving rise to torsion (i.e., any rotation to vertical corneal meridian).
Intorsion (incycloduction) is the secondary action for superior rectus and extorsion
(excycloduction) is the secondary action for the inferior rectus. Adduction is the
tertiary action for both the muscles.
Anatomy of Extraocular Muscles and Their Fascia 5

Fig. 1.6A: Relative positions of orbital Fig. 1.6B: Relative positions of the optical axis and
and optical axes the axis of muscle action in vertical recti and
vertical oblique muscles.

As oblique muscles are inclined at 51 degrees to the visual axis, torsion is their
primary action. Vertical rotation is their secondary action and horizontal rotation is
their tertiary action.
Spiral of Tillaux: The rectus muscle tendons insert progressively further from the
limbus in the order of medial rectus, inferior rectus, lateral rectus and superior
rectus. By drawing a continuous curve which passes through these insertions, a
spiral is formed. This is known as the Spiral of Tillaux.
Fine Structure of the Extraocular Muscles
Extraocular muscle is a specialized form of skeletal muscle.They have certain
differences from typical skeletal muscles. For example, not all fiber types propa-
gate action potentials and highly fatigue-restricted fiber types also may have fast
twitch characteristics. Thus, the traditional fiber type classifications can not be
applied to them. The rectus and oblique muscles exhibit two distinct regions, each
with different fiber content:
a. an outer orbital layer adjacent to the periorbita and orbital bone and
b. an inner global layer adjacent to the eyeball and optic nerve.
While the global layer extends the full muscle length, inserting via a well defined
tendon, the orbital layer ends before the muscle becomes tendinous.
The extraocular muscle fiber classification system resolves six fiber types:
a. Orbital singly-innervated fiber: This is the predominant fiber type (80%) in
the orbital layer of rectus and oblique muscles. Neuromuscular contacts are at
a single site, but nerve terminals spiral around the fiber. On the basis of oxida-
tive capacity, this fiber type is likely the most fatigue-resistant skeletal muscle
fiber type. Normal eye muscle tension levels never drop below 8-12 grams; this
fiber type most likely is a major contributor to the sustained force levels.
b. Orbital multiply-innervated fiber: This fiber accounts for the remaining 20
per cent of fibers. This fiber exhibits multiple nerve terminals distributed along
its length. This fiber type has twitch capability near its center and is non-twitch
proximal and distal to the end plate band.
6 Strabismus

c. Global red singly-innervated fiber: This fiber constitutes about one-third of


1 the fibers in the global layer. This fiber type closely resembles that of the orbital
singly-innervated fiber. Global red singly-innervated fibers are fast-twitch and
highly fatigue resistant.
d. Global intermediate singly-innervated fiber: This type constitutes about one-
fourth of the fibers in the global layer. This is a fast-twitch fiber type with an
intermediate level of fatigue resistance.
e. Global pale singly-innervated fiber: This type comprises one-third of the
global layer. This is a fast-twitch fiber type that is used only sporadically because
of low fatigue resistance.
f. Global multiply-innervated fiber: This fiber constitutes the remaining 10 per
cent of the global layer. They exhibits a slow graded, non-propagated response
following either neural or pharmacologic activation.
Table 1.2: Difference between extraocular and skeletal muscles
Characteristics Extraocular muscle Other Skeletal muscle
1. Embryonic origin Precordial/somitomere mesoderm Somite mesoderm
2. Fiber types Atypical, six types that are Three traditional types
special to this muscle group
3. Innervation pattern Singly and multiply innervated Singly innervated
4. Mode of contraction Twitch and non-twitch fibers Twitch fibers only
5. Fatigue resistance Highest of any skeletal muscle Variable
6. Stretch reflex Absent Present
7. Functional diversity Individual muscles have a wide Individual muscles serve either
dynamic range postural, gross movement
or fine control functions
8. Response to axotomy Limited atrophy, type grouping Severe atrophy, completely
not seen reversible
9. Botulinum toxin denervation Mild atrophy, lasting fiber type- Severe atrophy, completely
specific effect reversible
10. Local anesthetic toxicity Mild fiber type-specific effect Degeneration of all fiber types.

Orbital and Fascial Relationships


Tenon’s capsule: This is composed of elastic connective tissue and is attached to
the optic nerve posteriorly and becomes fused with the intermuscular membrane
3mm from the limbus to which it is attached anteriorly. Posterior to the equator, it
is a fibrous condensation that separates the orbital fat inside the muscle cone from
the sclera, thereby keeping that compartment free from fat. Anterior to the equa-
tor, it extends forwards over the muscles and separates them from the orbital fat
and structures lying outside the muscle cone. All the extraocular muscles pen-
etrate the Tenon’s capsule.
Muscle cone: This lies posterior to the equator. It is composed of the extraocular
muscle, the extraocular muscle sheaths, and the intermuscular membrane. The
muscle cone extends posteriorly up to the annulus of Zinn.
Muscle sheath: The rectus muscles have a surrounding fascial sheath that extends
with the muscle from its origin to its insertion.
Anatomy of Extraocular Muscles and Their Fascia 7

Intermuscular septum: Each of the rectus muscles sends an extension to the adja-
cent rectus muscle sheath. This is a thin elastic tissue. This is called the intermuscular
membrane and it joins the rectus muscles at all times in the muscle cone except
1
where they penetrate Tenon’s capsule. It becomes fused with Teton’s capsule
3mm from the limbus.
Check ligaments: They extend from the muscle sheath, anterior to the equator,
pass through the Tenon’s capsule and insert on the corresponding orbital walls.
They function as support structures for the eyeball and surrounding tissue. They
limit eye movements only when they are scarred. They are slightly more deve-
loped for the horizontal rectus muscles.
Ligament of lockwood: The muscle sheath of the inferior oblique muscle is con-
nected to muscle sheath of the inferior rectus muscle. This fusion is called the
Ligament of Lockwood.
Adipose tissue: The eye is supported and cushioned by a large amount of fatty
tissue in the orbit. External to the muscle cone, the fatty tissue comes forward with
the rectus muscles, stopping about 10 mm from the limbus. The fatty tissue is also
found inside the muscle cone, kept away from the sclera by the Tenon’s capsule. If
Tenon’s capsule is torn or cut, fat can prolapse inside Tenon’s capsule and may
form firm adhesion to sclera, muscle, intermuscular membrane, and/or conjunctiva.
Some Important Anatomical Implications
1. The nerves to the rectus muscles and superior oblique muscle enter the muscle
about one third of the distance from the origin to the insertion (trochlea in case
of superior oblique muscle). These nerves are rarely damaged during anterior
surgeries though a thrust more than 26mm posterior to the rectus muscle’s
insertion may damage it. The nerve to the inferior oblique muscle enters the
lateral part of it where it crosses the inferior rectus muscle. It can be damaged
by surgery in this area. As the parasympathetic innervation to the sphincter
pupillae and the ciliary muscle accompanies this nerve, they may be damaged at
the same time.
2. As the inferior rectus muscle is bound to the lower lid by the fascial extension
from its sheath, any alteration of the inferior rectus may be associated with lid
fissure changes. Recession of the inferior rectus muscle tends to widen the lid
fissure and resection of it tends to narrow the lid fissure.
3. The superior rectus muscle is loosely attached to the levator palpebrae superioris
muscle. This causes the eyeball to roll up 30° when the lid closes completely
(Bell’s phenomenon). Following resection of the superior rectus muscle, the
upper eye lid may be pushed forward, causing narrowing of the lid fissure.
4. Simultaneous surgery on three rectus muscles may induce anterior segment
ischemia. The reason behind it is that the blood supply to the extraocular muscles
provides almost all of the temporal half of the anterior segment circulation and
the majority of the nasal half of the anterior segment circulation, which also
receives some blood from the long posterior ciliary artery.
8 Strabismus

5. If the integrity of the Tenon’s capsule 10mm posterior to the limbus is lost, fatty
1 tissue may prolapse through the Tenon’s capsule and may form a restrictive
adhesion and limit ocular motility.
6. Accidental severing of vortex vein may occur in procedures like inferior rectus
and superior rectus muscle recession or resection, inferior oblique weakening
and exposure of superior oblique muscle tendon.
7. As the sclera is thinnest just posterior to the insertions of the four recti muscles,
there is a risk of scleral perforation during the eye muscle surgery which may
be minimized during the eye muscle surgery by using spatulated needles with
swedged sutures, working in a clean, blood-free surgical field and using good
illumination.

MOTOR PHYSIOLOGY
Axes of Fick and Listing’s plane:
Axes of Fick-
The axes of Fick are designated as x, y and z.
X-axis – It is a transverse axis passing through the center of the eye at the equa-
tor. Voluntary vertical rotations of the eye occur about this axis.
Y-axis – It is a sagittal axis passing through the pupil. Involuntary torsional rota-
tions occur about this axis.
Z-axis – It is a vertical axis. Voluntary horizontal rotations occur about this axis.
Listing’s Planes–
a. Horizontal plane - Horizontal eye movements around Z-axis.
b. Vertical plane - Vertical eye movements around X-axis.
c. Torsional - Torsional eye movements around Y-axis, which is antero-posterior.
Positions of Gaze
A.Primary position : The primary position is the position of the eyes when
fixing an object at infinity (20 feet or 6 meters) straight
ahead. The head should be straight.
B. Secondary position : They are the straight up, straight down, right gaze
and left gaze positions.
C. Tertiary position : They are the four oblique positions-up and right, up
and left down and right, and down and left.
D.Cardinal positions : They are up and right, up and left, right, left, down and
right and down and left positions. They are those six
positions of gaze in which the prime mover is one
muscle of each eye, together called the ‘yoke’ muscles.
E. Midline positions : They are the straight up and straight down from the
primary position.
F. Diagnostic positions : All the nine gaze positions- the six cardinal positions,
mid line positions and primary position.
Anatomy of Extraocular Muscles and Their Fascia 9

Position of Rest
The position of each eye in the orbit without any innervation to the extraocular 1
muscles is the position of rest. The position of each eye is slightly divergent in
normal positions.
Motor Unit
An individual motor nerve fiber and its several muscle fibers is a motor unit.
Recruitment
It is the process by which more and more motor units of a particular muscle are
activated and brought into play by the brain to help pull the eye in the direction of
action of that muscle. As the eye fixates further in the field of action of that
muscle, the frequency of activity of each motor unit increases until it reaches a
peak.
Saccadic Movement
Saccadic movements need a sudden, strong pulse of force from the muscle in
order to move the eye rapidly against the viscosity produced by the fatty tissue and
fascia in which the globe lies. The velocity of the saccadic movement and the high
forces that must be produced are affected by muscle paresis, and study of sac-
cadic velocity help in determining paresis of muscles and abnormal innervation.
Ductions
Ductions are monocular rotations of the eye.
Adduction Movement of the eye nasally
Abduction Movement of the eye temporally
Elevation (Supraduction/Sursumduction) Upward movement of the eye
Depression (Infraduction/Deorsumduction) Downward movement of the eye
Intorsion (Incycloduction) Nasal rotation of the superior portion of the verti-
cal corneal meridian
Extorsion (Excycloduction) Temporal rotation of the superior portion of the
vertical corneal meridian

Muscles used in monocular eye movement:


Agonist The primary muscle moving the eye in a given
direction
Synergist The muscle in the same eye as the agonist that acts
with the agonist to produce a given movement (In-
ferior oblique is a synergist with the agonist superior
rectus for elevation of the eye).
Antagonist The muscle in the same eye as the agonist that acts
in the direction opposite to that of the agonist
(medial rectus and lateral rectus muscles are antago-
nist).
10 Strabismus

Sherrington’s Law of Reciprocal Innervation


1 It states that increased innervation and contraction of a given muscle are accom-
panied by a reciprocal decrease in innervation and contraction of its antagonist.
Example: As the left eye adducts, the left medial rectus muscle receives increased
innervation, while the left lateral rectus receives decreased innervation.
Versions
When the binocular eye movements are conjugate and the eyes move in the same
direction, they are called versions.
Dextroversion (right gaze) Movement of both eyes to the patient’s right.
Levoversion (left gaze) Movement of both eyes to the patient’s left.
Sursumversion (Elevation/up gaze) Upward rotation of both eyes
Deorsumversion (Depression/down gaze) Downward rotation of both eyes
Dextrocycloversion Both eyes rotate so that the superior portion of the
vertical corneal meridian moves to the patient’s
right.
Levocycloversion Both eyes rotate so that the superior portion of the
vertical corneal meridian moves to the patient’s
left.

‘Yoke’ Muscles (Contralateral Synergists)


A pair of muscles (one in each eye) that are the prime movers of their respective
eyes in the cardinal positions of gaze.
Cardinal positions Yoke muscles
Eyes up and right(Dextrosursumversion) Right superior oblique and left inferior oblique
Eyes up and left(Levosursumversion) Left superior rectus and right inferior oblique
Eyes right(Dextroversion) Right lateral rectus and left medial rectus
Eyes left(Levoversion) Left lateral rectus and right medial rectus
Eyes down and right (Dextrodeorsumversion) Right inferior rectus and left superior oblique
Eyes down and left(Levodeorsumversion) Left inferior rectus and right superior oblique

Hering’s Law of Motor Correspondence


It states that equal and simultaneous innervation flows to synergistic muscles con-
cerned with the desired direction of gaze.
Since the amount of innervation to both eyes is always determined by the fixat-
ing eye, the angle of deviation will vary depending on which eye is fixating. When
the normal eye is fixating, the amount of deviation is called the primary deviation.
When the paretic eye is fixating, the amount of deviation is called the secondary
deviation. The secondary deviation is often greater than the primary deviation.
Vergences
When the eye movements are disjugate and the eyes move in the opposite direc-
tions, such movements are called vergences.
Anatomy of Extraocular Muscles and Their Fascia 11

Convergence

Divergence
Movement of both eyes nasally relative to a given
position
Movement of both eyes temporally relative to a
1
given position
Incyclovergence Rotation of both eyes so that the superior portion
of each vertical corneal meridian rotates toward the
median plane.
Excyclovergence Rotation of both eyes so that the superior portion
of each vertical corneal meridian rotates away from
the median plane.
Vertical vergence One eye moves upward and the other downward.

Tonic Convergence
The constant innervational tone to the extraocular muscles when a person is awake
and alert. The convergence tone is necessary in the awake state to maintain straight
eyes.
Accommodative Convergence
The accommodative convergence of the visual axes occurs as part of the synkinetic
near reflex. A fairly consistent increase of accommodative convergence(AC)
occurs for each diopter of accommodation(A). The ratio between AC and A i.e.,
AC/A ratio is important. With an abnormally high AC/A ratio, the excess
convergence tends to produce esotropia during accommodation on near targets.
An abnormally low AC/A ratio will tend to make the eyes exotropic when the
person looks at near targets.
Fusional Vergences
a. Fusional convergence: An optomotor reflex to converge and position the eyes
so that similar images project on corresponding retinal areas. It is prompted by
bitemporal retinal image disparity.
b. Fusional divergence: An optomotor reflex to diverge and align the eyes so that
similar images project on corresponding retinal areas. It is prompted by binasal
retinal image disparity.
Supranuclear Control Systems for Eye Movements
There are five supranuclear eye movement systems.
Saccadic system It generates all fast movements Saccades are initiated by burst cells
or eye movements or eye move- within the paramedical pontine
ments of refixation. It functions reticular formation(PPRF). Their
to place an object of interest on activation requires suppression of
the fovea or to move the eyes pause cell activity. Pause cells are
from one object to another. inhibited by corticobulbar pro-
Velocity- 400 to 500 degrees/sec jections from the frontal lobe.
Smooth pursuit system It generates all following, or pur- The pathway starts with the
suit eye movements. Pursuit striate cortex, which receives
Contd...

http://ophthalmologyebooks.com
12 Strabismus

Contd...
1 latency is shorter than that for
saccades.
input from lateral geniculate bodies.
Extastriate visual areas then rece-
Velocity-30 to 60 degrees/sec ive input and project ipsilaterally
to the dorsolateral pontine nuclei.
Finally, the vestibular nuclei receive
the input and transmit it to the ocu-
lar motor nuclei of cranial nerves
III, IV and VI.
Vergence system Controls disjugate eye move- Supranuclear control mechanism is
ments (convergence and not yet fully understood.
divergnce).
Position maintainance Maintains a specific gaze osition, The site of this system is not
system allowing an object of interest to known.
remain on the fovea.
Nonoptic reflex system- 1. Labyrinthine reflex system
Integrate eye movements involving the semicircular canals
and body movements. of inner ears.
2. Systems involving utricle and
saccule of inner ears.
3. Cervical or neck recepts also
provide input.

AXIAL ANGLES OF THE EYE


Geometric Axis
This is a line joining the anterior and posterior poles of the eye.
Optic Axis
This is a line on which the main optical components (Cornea and lens) are cen-
tered. It passes through the nodal point of the eye. It coincides more or less with
the geometric axis.
Nodal Point
This is the optical center of the eye. It lies on the optical axis slightly in front of the
center of the eyeball and is adjacent to the posterior surface of the lens.
Center of Rotation
This is the point around which the eye rotates. It is situated slightly behind the
centers of the eye on the optical axis line. It lies behind the nodal point.
Visual Axis
This is a line passing from the object of fixation to the fovea. It passes through the
nodal point.
Central Pupillary Line
This line is perpendicular to the corneas and passes through the center of the pupil
to reach the nodal point of the eye. It may be regarded as equivalent to the optical axis.
Anatomy of Extraocular Muscles and Their Fascia 13

Fixation Axis
This is a line passing from the object of fixation to the center of rotation of the eye. 1

Fig. 1.7: Showing the axial angles of the eye

Angle Alpha
It is angle formed between the optical axis and the visual axis at the nodal point.
The optical axis sometimes coincides with the visual axis and hence angle alpha is
zero. Usually, the visual axis passes on the nasal side of the optical axis and then
the term used is a positive angle alpha. Rarely, the visual axis passes on the tempo-
ral side of the optical axis and then the term used is a negative angle alpha.
A positive angle alpha causes a slightly divergent appearance of the eyes and a
negative angle alpha causes a slightly convergent appearance of the eyes.
Angle Gamma
It is the angle formed between the optical axis and the fixation axis at the center of
rotation of the eye. This angle has similar positive and negative values like angle
alpha.
Angle Kappa
It is the angle formed by the pupillary axis and the visual axis at the center of the
pupil. An angle kappa is caused by the failure of the optical axis of the eye and the
visual axis to coincide.
Measurement
a. Clinically angle kappa may be diagnosed by simply observing the corneal reflec-
tion. If it is not in the center, and if the eye does not move on cover test, then it
is either eccentric fixation or a large angle kappa. If the vision is 6/6, then it must
be angle kappa and if the vision is poor then it must be eccentric fixation.
b. Synoptophore: To measure the angle kappa with the synoptophore a special
slide is used.
4 3 2 1 0 A B C D
14 Strabismus

This slide contains a horizontal row of letters and numbers, separated by inter-
1 vals of 1 or 2 degrees. This slide is introduced in one of the slots and the other slot
is kept empty. The patient is then asked to fixate the zero and the position of the
corneal light reflection is noted. The patient shifts his fixation to each of the letters
or numbers in turn until the examiner finds the light reflection to be centered on the
cornea. This gives the angle kappa in degrees, which is positive for the right eye if
the fixation has to be shifted to the numbers and negative if it has to be shifted to
the letters and vice versa for the left eye.
Types
a. Positive angle kappa: The optical axis touches the posterior pole of the globe
slightly nasal and inferior to the fovea. When the eye fixates a light, the reflec-
tion from the cornea will not be centered but will be slightly nasal to the center.
This is called a positive angle kappa. If the angle is large enough, this will appear
as an exodeviation. Thus, a positive angle kappa will produce a nasal deflection
and will produce a nasal deflection and will lead to a pseudodivergence. A
positive angle kappa is more common.
b. Negative angle kappa: If the position of the fovea is nasal to the angle at
which the optical axis cuts the posterior part of the globe, the corneal reflection
of a light fixated by that eye will appear to lie on the temporal side of the
pupillary center. This is called a negative angle kappa. This may simulate an
esodeviation again producing a pseudostrabismus.
Vertical Angle Kappa
It may be present thus simulating a hyperdeviation. It is caused by superior or
inferior displacement of the macula from scar tissue.
Etiology a. Physiological- commonest
b. Pathological-It is due to ectopia of the macula which can occur in
retrolental fibroplasias, chorioretinitis, congenital retinal folds, diabetic
retinopathy etc.
2
Development of Vision-
Binocular Vision, Suppression
Visual acuity is about 6/36 at 6 months and 6/6 at 24 months. Stable alignment of
the visual axes is present at about 4 months of age if fusion and stereopsis have
developed normally. Although good vision, stereopsis and fusion develops by 4
months of age, they become firmly established by the age of 8 years. Visual acuity
deterioration and suppression usually do not occur in patients who develop a strabis-
mus after the age of 5 years and are rare after the age of 8 years. Hence patients
over the age of 8 years are called visual adults. Only in cases of organic damage to
the visual system, amblyopia may occur in these patients.
Development of Monocular Vision
For normal monocular vision to develop, the fovea should receive a clearly focused
image throughout the period of visual immaturity (up to 8 years of age). Normal
monocular visual acuity is then firmly established throughout the life. If these con-
ditions are not fulfilled, amblyopia may result.
Development of Normal Binocular Vision
Normal binocular vision usually implies some level of co-ordination between the
eyes and ultimate bifoveal fusion with 40 seconds of arc of stereopsis. The eye
alignment stabilizes within the first 3 months of life. This is achieved by the use of
the two eyes together, so that separate and slightly dissimilar images formed in
each eye are appreciated as a single image by the process of fusion.
Grades of Binocular Vision
A. Simultaneous macular perception,
B. Fusion,
C. Stereopsis
It is important to determine the binocular status in order to establish a prognosis
and plan the management in case of a manifest squint. The state of retinal corres-
pondance must be assessed and the presence or absence of fusion be determined.
Cases of manifest strabismus may be considered as:
a. Already functional: Abnormal retinal correspondence (ARC) with anomalous
fusion.
b. Potentially functional: Normal retinal correspondence(NRC) with demonstrable
fusion.
c. Cosmetic: Lack of retinal correspondence and absence of fusion.
16 Strabismus

Normal Binocular Fusion


2 It is the cortical unification of two visual objects into a single percept made possible
by simultaneous stimulation of corresponding areas of retina. The images must be
similar in size and shape.
It occurs when relay from the ganglion cells of the corresponding points of
each retina reaches a shared cortical cell in the brain. It is highly vulnerable and is
readily disrupted in the visually immature years by any of the amblyogenic factors.
Equal input from corresponding retinal points should reach the same cortical cell.
If there is any significant difference in the input from each eye, it causes competi-
tion between the two and the clearer image prevails whereas the other image is
inhibited. The visual fields of the two eyes overlap in an area 60 degrees to either
side of fixation to provide stereopsis and central and peripheral fusions. Normal
binocular vision is characterized by stereoacuity of 60 seconds or better with the
Titmus test.
Normal range of fusional amplitude for near (25 cm): The normal ranges are
16 prism dioptres of divergence, 38 prism dioptres of convergence and 2.6 prism
dioptres of vertical amplitude.
Normal range of fusional amplitude for distance (6 m): The normal ranges are
6 prism dioptres of divergence, 14 prism dioptres of convergence and 2.5 prism
dioptres of vertical amplitude.
Three factors are required for its development:
• Reasonably clear vision in both eyes.
• Ability of the visual areas in the brain to cause fusion of two slightly dissimilar
images.
• The accurate co-ordination of the two eyes in all directions of gaze. There must
exist a precise physiological relationship between the two retinae (retinal corre-
spondence) for achieving fusion. If retinal areas of two eyes share a common
subjective visual direction, i.e., if their simultaneous stimulation results in the
subjective sensation that the stimulating targets come from the same direction in
space, these retinal areas are said to be corresponding. If the simultaneous
stimulation of retinal areas in the two eyes results in the sensation of two sepa-
rate visual directions, or diplopia, these areas are said to be noncorresponding or
disparate.
Assessment of Fusion
A.NRC: The angle of deviation must be corrected either with
a. Prisms: A prism fusion range may then be measured. Bagolini glasses
may be used to check the responses.
or
b. Synoptophore: A range of fusion may be measured using fusion
slides with controls.
B. Harmonious ARC: The angle of deviatiaon does not need correction.
C. Unharmonious ARC: Fusion could be best assessed by the synoptophore.

http://ophthalmologyebooks.com
Development of Vision-Binocular Vision, Suppression 17

Fig. 2.1: Slides used with synoptophore for assessment of:


a. Simultaneous macular perception: an ability to interpret two dissimilar images
which do not require fusion but which do not antagonize each other
b. Fusion: a process by which visual cortex combines two almost similar objects into one
c. Stereopsis: a process by which a slight but perceptible difference in two retinal images
is interpreted at higher center as depth perception.
18 Strabismus

Stereopsis
2 Stereopsis is a binocular sensation of relative depth caused by horizontal retinal
image disparity. Stereopsis and depth perception are not synonymous. Monocular
clues like object overlap, relative object size, highlight and shadows, motion paral-
lax, and perspective all contribute to depth perception
Retinal Rivalry
The rapid alteration in the perceived image of two dissimilar objects presented to
the foveal areas of each eye is called retinal rivalry. It occurs because of the
physiologic inability of the two foveas to simultaneously perceive dissimilar objects.
Magnocellular (M-cells) and Parvocellular (P-cells)—
Nuclei in Lateral Geniculate Body
Ganglion cell stimulation from a retinal image results in simultaneous parallel pro-
cessing through two different pathway. Nuclei in the lateral geniculate body can be
divided into P-cells and M-cells. P-cell neurons are more sensitive to color, high
spatial frequencies, two point discrimination and fine stereopsis. They project to
areas of the central visual field and fovea. M-cell neurons are sensitive to direction,
motion, speed ,flicker ,gross binocular disparities and gross stereopsis. They project
to parafoveal and more peripheral retina. Even though the two pathways are dif-
ferent , they overlap and both systems act together to process visual information.
In the striate cortex parvo- and magno- recipient lamellae are segregated and
there are interconnecting pathways .From the striate cortex information from M-
cells goes to parieto-occipital areas, and information from P-cells goes to temporo-
occipital areas. Abnormal P-cell development may be associated with pattern
deprivation amblyopia and abnormal M-cell development may be associated with
strabismus and may lead to latent nystagmus and asymmetrical horizontal smooth
pursuit.
Vieth-Muller circle, Horopter and Panum’s area:

Fig. 2.2: Panum’s area, Horopter and Vieth-Muller circle


Development of Vision-Binocular Vision, Suppression 19

If the two eyes have normal retinal correspondence and each fovea fixates an
identical point, this point will be seen singly. Points on either side of the fixation
point will fall on the corresponding retinal areas and will also be seen singly, as long
2
as they lie on a horizontal plane, the Vieth-Muller circle. The Vieth-Muller circle
passes through the optical centers of each eye and the point of fixation. When
attempts are made to duplicate this circle experimentally, the locus of all points
seen singly falls on the circle but on a curved surface called empirical horopter,
which exists not only on two dimensions but is actually a three-dimensional space
obtained by rotating the horizontal horopter around an axis connecting the centers
of rotation of the two eyes. All points lying on the horopter curve will stimulate
corresponding retinal elements and thus be seen singly and all points not lying on
the horopter will fall on disparate retinal element and is expected to cause double
vision. However, double vision does not occur within a limited area surrounding the
horopter curve, because the visual system fuses the two disparate retinal images,
resulting in binocular single vision with stereopsis. The slightly dissimilar images
caused by the three-dimensional object stimulates stereoscopic perception.
Panum’s area is the space where objects that fall on disparate retinal areas are
physiologically seen singly. Objects outside this area fall on widely disparate retinal
areas and are seen as coming from two different visual directions, causing diplopia.
Abnormalities of Binocular Vision:
A.Diplopia
Diplopia usually results from an acquired misalignment of the visual axes that
causes an image to fall simultaneously on the fovea of one eye and on a nonfoveal
point in the other eye. The nonfoveal point must be outside Panum’s area to be
seen double.
B. Confusion
Usually, adult patients with acquired ocular misalignment see two of the same
image. Rarely, patients will describe the simultaneous perception of two different
images superimposed on each other. As the eyes are misaligned, dissimilar
images fall on corresponding retinal areas, causing visual confusion.
C. Suppression
Suppression is a cortical adaptive phenomenon occurring in strabismus, which
eliminates the problem of visual confusion and strabismic diplopia.
Physiological suppression is the mechanism to prevent physiological diplopia
from reaching consciousness.
Pathological suppression is an adaptation of a visually immature brain to avoid
diplopia resulting from strabismic misalignment of the visual axes.
Central and Peripheral Suppression:
Central suppression is the mechanism that keeps the foveal image of the deviat-
ing eye from reaching consciousness, thus preventing confusion. As the two
foveas can not perceive the dissimilar images simultaneously, this central
scotoma of the deviating fovea is a physiological suppression.
20 Strabismus

Peripheral suppression is the mechanism that eliminates diplopia by preventing


2 awareness of the image that fall on peripheral retina in the deviating eye and
resembles the image falling on the fovea of the fixating eye. This is a pathologi-
cal suppression and develops as a cortical adaptation only within an immature
visual system. Adults can not develop peripheral suppression.
Monocular and Alternating Suppression:
If the suppression always causes the image from the dominant eye to predomi-
nate over the image from the deviating eye, the suppression is monocular. This
may result in strabismic amblyopia.
If the process switches over a period of time between the images the images of
the two eyes, the suppression is alternating.
Facultative and Obligatory Suppression:
Facultative suppression is the condition which is present only when the eyes are
in a deviating condition and absent in other states, e.g. in intermittent exotropia,
suppression is present when the eyes are divergent but stereopsis is present
when the eyes are straight.
Obligatory suppression is present at all the times.
Tests for Suppression:
a. Worth four dot test: A red glass filters out all colours except red and a
green light is not visible through a red filter. Similarly every colour except
green will become visible when viewed through a green filter. Looking through
a pair of diplopia goggles (with red before the right eye and green before the
left eye) the patient views a box with 4 lights (one red, two greens and one
white) at 6 metres and at 1/3rd metre.The patient’s responses are inter-
preted accordingly.
Responses:
If the patient sees all 4 lights: It indicates fusion or ARC
(The white light is reported to undergo colour rivalry)
If the patient sees only 2 reds or 3 greens: It indicates suppression
If the patient sees 5 lights It indicates diplopia
b. Red lens test: A red lens should be placed in front of one eye as the patient
fixates a light. If only one light is noted, suppression is present.
c. Bagolini lenses may also be used.
Management of suppression:
1. Refraction
2. Occlusion to permit equal and alternate use of each eye and to treat amblyo-
pia, if present.
3. Alignment of the visual axes to permit simultaneous stimulation of corre-
sponding retinal elements by the same subject.
D.Abnormal retinal correspondence: In young children with long-standing stra-
bismus, a sensory shift in retinal correspondence may develop so that retinal
areas that receive the same image may develop a common visual direction. This
Development of Vision-Binocular Vision, Suppression 21

adaptation is to enable the development of a crude type of binocularity in the


presence of strabismus. 2
Types of Retinal Correspondence
1. Normal Retinal Correspondence (NRC): Both fovea share a common visual
direction. Nasal retina of one eye corresponding to temporal retina of the other
eye. Anatomically corresponding points from each eye localize to the same
point in space. Fibres from the corresponding points from each eye comes to
the same lateral geniculate body though to different layers there. In the cortex
these fibres share a cell. Once NRC has been firmly established and maintained
until visual adulthood, it is not possible to change it to ARC.
2. Harmonious Abnormal Retinal Correspondence: The fovea of the fixing eye
corresponds to a point other than the fovea of the deviating eye. The pairing of
all other retinal points is similarly changed.
3. Unharmonious Abnormal Retinal Correspondence: Sensory re-orientation
between NRC and ARC is not fully developed. In these cases, either diplopia
(incongruous) or suppression results.
4. Lack of Retinal Correspondence: The two eyes do not relate to each other.
Diagnosis of Retinal Correspondence
1. Bagolini glasses: They are optically plano lenses with imperceptible striations
that blur the environment hardly at all but produce a luminous stripe when a
person is looking at a light through it. The stripe forms an angle of 135° in right
eye and 45° in left eye. The test is performed at 1/3rd meter and 6 meters.
a. NRC with manifest squint: Diplopia or suppression occurs. If the angles are
corrected there is X response.
b. Harmonious ARC with manifest squint: X response with manifest devia-
tion occurs. Possibly there is a central suppression. Paradoxical diplopia is
possible.
c. Unharmonious ARC with manifest squint: Suppression or incongruous
diplopia occurs. X response occurs with angle partially corrected.
d. Lack of retinal correspondence in manifest squint: Suppression or diplo-
pia occurs. X response is not obtained with prisms.
2. Worth’s four dot test: This is a gross test. Even when dense central suppression
scotoma is present, the results may indicate fusion. This test provides information
only about the status of peripheral binocular co-operation.
a. NRC: Suppression or diplopia occurs. 4 lights are seen with angle corrected.
b. Harmonious ARC: 4 lights are seen with manifest deviation. Paradoxical
diplopia is possible.
c. Unharmonious ARC: 4 lights are seen with angle partially corrected. Diplo-
pia or suppression may be seen.
d. Lack of retinal correspondence: 4 lights are never seen.

http://ophthalmologyebooks.com
22 Strabismus

3. Stereotests:
2 A. Titmus fly test- It consists of a 3 dimensional Polaroid vectograph consisting
of two plates in the form of a booklet. On the right there is a large fly and on
the left there are a series of circles and animals. The plates are viewed with
Polaroid glasses.
• Fly: This is a test of gross stereopsis. The fly should appear in a ‘solid’ 3
dimensions. In the absence of stereopsis the fly will appear as a flat
photograph.
• Circles: This is a graded series which tests fine depth perception.
B. TNO: It consists of 7 plates each of which contains various shapes (squares,
dots, crosses) created by random dots in complementary colours which are
viewed through red and green spectacles.
Results
a. NRC: Binocular single vision (BSV) is demonstrable with angle corrected.
b. Harmonious ARC: BSV is demonstrable with a manifest deviation.
c. Unharmonious ARC: BSV is demonstrable with angle partially corrected.
d. Lack of retinal correspondence: No BSV is present.
4. Vertical prism test: This test is indicated when a suppression response is obtained.
A vertical prism is used to lift the image out of the suppression scotoma. The
horizontal image separation is assessed with horizontal prisms. Red and green
glasses may be used in this test.
a. NRC: The subjective separation is equal to the objective separation.
b. Unharmonious ARC: The subjective separation is less than the objective
measurement.
c. Lack of retinal correspondence: The images can not be vertically aligned.
5. Synoptophore: Retinal correspondence is determined by comparing the objec-
tive and subjective angles of deviation. By rotating the arms of the synopto-
phore, the patient attempts to place the lion in the cage. The amount the arms
are moved is noted from the scale of the synoptophore. This represents the
subjective angle. The objective angle is measured by moving the arms of the
synoptophore and alternately turning the illumination in the tubes off and on
from one eye to another until there is no refixation movement. This point repre-
sents the objective angle and it can be recorded directly from the scale on the
instrument.
a. NRC: The objective and the subjective angles are equal.
b. Harmonious ARC: The subjective angle is zero. There is an angle of anomaly
equal to the angle of deviation.
c. Unharmonious ARC: The subjective angle is less than the objective angle.
There is an angle of anomaly less than the angle of deviation.
d. Lack of retinal correspondence: Superimposition of images can not be
obtained.
6. Binocular convergence: This test is useful in cases of esotropia to differenti-
ate between NRC and Harmonious ARC.
Development of Vision-Binocular Vision, Suppression 23

a. NRC: The angle of deviation reduces with convergence. Binocularity is


present at the intersection of the visual axes.
b. Harmonious ARC: The person converges with the angle of deviation
2
remaining constant.
7. After image test: Each eye fixates the central black mark of a glowing filament,
first presented horizontally to the eye with better visual acuity and then verti-
cally to the eye with less visual acuity for 20 seconds in a dark room while the
fellow eye is occluded. The patient indicates the relative position of the two
gaps in the center of the after-image. The gaps correspond to the visual direc-
tion of each fovea if central fixation is present.
a. NRC: Cross response is present. Both the fovea project to the same point in
space.
b. Harmonious ARC: A displaced cross is noted. The fovea project to the
different points in space.

ASSESSMENT OF FUSION
A.NRC: The angle of deviation must be corrected either with
a. Prisms: A prism fusion range may then be measured. Bagolini glasses may
be used to check the responses.
or
b. Synoptophore: A range of fusion may be measured using fusion slides with
controls.
B. Harmonious ARC: The angle of deviation does not need correction.
C. Unharmonious ARC: Fusion could be best assessed by the synoptophore.
3
Amblyopia

Amblyopia means dull vision. It may be defined as a failure of visual acuity


consolidation resulting either from lack of stimuli or from the presence of inadequate
stimuli during a critical period of development. It is a condition of diminished visual
form sense which is not alleviated by the elimination of any defect which constitutes
a dioptric obstacle to the formation of a retinal image, nor is it the result of any
structural anomaly of the visual pathways. There is no detectable ocular pathology.
It is a leading cause of diminished monocular vision in adults. The prevalence of
amblyopia is between 2 to 4 per cent.
Its etiology, pathogenesis and treatment are not yet clearly established and a
consensus is lacking in this field. It is caused by inadequate stimulation of the visual
system during the critical period of visual development during early childhood. It
may be uniocular or binocular.
There are few established findings which contribute to our understanding the
pathophysiology of amblyopia. It is ascertained that in the lateral geniculate nucleus
(LGN) and the visual cortex neuro-physiological and morphological changes take
place which are common to the various types of amblyopia. The primary anomaly
is localizable in the visual cortex. This has been shown in humans with the demon-
stration of an altered glucose metabolism.
Another important aspect concerns the sensitive period of the visual system to
amblyogenic factors. The visual system appears to be sensitive to conditions which
can alter its function within a given period (Sensitive period) from birth to stages of
infancy. The therapy for amblyopia is effective if started during the sensitive period;
the earlier the treatment is started, the results are better and length of treatment is
shorter. Its duration is not exactly defined in humans, but it continues until about
8-9 years of age. Improvements of visual acuity have been seen after treatment,
even in older patients. However there are few fallacies about this achievment. The
patient continues to use the better eye for fixation; visual acuity of the amblyopic
eye will invariably show a deterioration and treatment can not be continued for
indefinite period. In the case of functional loss of the good eye, a spontaneous
improvement in the visual acuity of the amblyopic eye has been seen.
Amblyopic anomalies, which involve visual acuity and other functions, are gen-
erally but not always reversible if timely appropriate treatment is done. Certain
types of amblyopia, such as that found in anisometropia with microstrabismus
respond poorly.
Amblyopia can be explained in terms of lack of retinal image formation (depri-
vation) or defocused image (ametropic or anisometropic amblyopia) and is found
Amblyopia 25

most commonly in association with strabismus. Secondary functional changes have


been shown to occur in amblyopia at lower levels, also involving the retina. 3
CLASSIFICATION OF AMBLYOPIA
Causes of Amblyopia are
1. Strabismus
2. Anisometropia
a. Anisohypermetropia
b. Anisomyopia
3. Isoametropia
4. Form vision deprivation (amblyopia ex anopsia)-unilateral/bilateral
a. Complete ptosis, media opacities (corneal, lental), unilateral occlusion and
prolonged atropinisation
b. Uncorrected bilateral high hypermetropia
c. Astigmatism (meridional amblyopia)
d. Nystagmus.
The most common causes are anisometropia and muscle imbalance i.e., an
incomitant deviation which explains the lack of free alteration of fixation.
The amblyopia caused by anisomyopia is usually less severe than that caused
by anisohypermetropia. Uniocular myopia is very often accompanied sooner or
later by organic alterations of the macula, thus modifying the initially better func-
tional prognosis.
High bilateral hypermetropia causes a bilateral deprivation amblyopia. They
rarely exhibit full visual acuity, even if perfectly corrected optically. In congenital
nystagmus constant movement of the object causes poor image formation on the
retina causing amblyopia.
Strabismic Amblyopia
This is the commonest form of amblyopia which develops in the consistently
deviating eye of a child with ocular misalignment. Constant nonalterating tropias
(commonly esotropias) are most likely to cause significant amblyopia. They most
probably result from competitive or inhibitory interaction between neurons carrying
the nonfusible inputs from the two eyes, which leads to domination of cortical
vision centers by the fixing eye and chronically reduced responsiveness to the non-
fixing eye’s input.
It has some special features. Grating acuity (used in Teller card), the ability to
detect patterns composed of uniformly spaced stripes, which normally corresponds
closely to the Snellen’s chart, is often reduced considerably less than Snellen’s
acuity in strabismic amblyopia. When the illumination is reduced, the acuity of an
eye with strabismic amblyopia tends to decline less sharply than that of a normal or
diseased eye (Neutral density filter effect). Minor degrees of eccentric fixation,
detectable with special tests like visuoscopy and Haidinger brushes are seen in
many patients with strabismic amblyopia.
26 Strabismus

Anisometric Amblyopia
3 This is the second commonest form of amblyopia which develops when unequal
refractive error in two eyes causes the image on one retina to be chronically
defocused. It results partly from the direct effect of image blur on the development
of visual acuity in the affected eye and partly from interocular competition or
inhibition. Relatively mild degrees of hypermetropia or astigmatic anisometropia
(1-2 D) can induce mild amblyopia. Mild myopic anisometropia (< –3D) usually
does not cause amblyopia. Unilateral high myopia (–6D) may cause severe am-
blyopia. Detection and treatment are often delayed until school age and sometimes
even more, when the recovery of vision is sometimes unsatisfactory.
Isoametropic Amblyopia
A bilateral reduction of visual acuity, which is usually mild, results from large,
approximately equal, uncorrected refractive errors in both eyes of a young child. It
is caused by the effect of blurred retinal images alone. Hypermetropia > +5D and
myopia > –10D may cause bilateral amblyopia. Uncorrected bilateral astigmatism
in early childhood may cause loss of resolving ability limited to the chronically
blurred meridians (meridian amblyopia).
Deprivation Amblyopia (Amblyopia Ex anopsia)
This is usually caused by congenital or early acquired media opacities. This type of
amblyopia is the least common, most damaging and most difficult to treat. Dense
congenital cataracts occupying the papillary area (central 3-4mm) cause severe
amblyopia. Cataracts of same type acquired between 8-10 years are equally harmful.
Occlusion amblyopia caused by excessive therapeutic patching is a form of depri-
vation amblyopia.

DIAGNOSIS
1. Visual Acuity Measurement
By convention a difference in visual acuity between the two eyes of at least two
lines of the chart is indicative of amblyopia. It is always necessary to test the best
visual acuity of the good eye, which may be better than that found with the small-
est symbols of the chart.
Visual acuity should be measured both by using a normal chart (line acuity) and
by presenting one symbol at a time(letter acuity). In amblyopia there is a discrep-
ancy between the results of these two types of measurement which is absent in
other organic pathologies. Line acuity is worse than letter acuity, probably because
of an alteration of lateral inhibition, typical of amblyopia.
Line acuity should be measured with charts in which the distance between
symbols equals the square in which the symbol is inscribed. Snellen E’s are used as
symbols. Alphabet letters are of differential difficulty and standardization is difficult.

http://ophthalmologyebooks.com
Amblyopia 27

Near visual acuity can be measured also with optotypes made out of differently
oriented Snellen E’s. It is appropriate to then evaluate near visual acuity by having
the patient read a text.
3
Finally, it is important to note the speed with which the patient reads the chart.
A reduced speed of reading is a sign of amblyopia.
2. Pattern of Fixation
Two aspects should be noted:
a. the capability of an eye to take up and maintain fixation and
b. the pattern of fixation found.
i. Fixation capability: An eye with dense unilateral amblyopia can not fix and the
better eye is used for fixation.If the good eye is occluded to perform a cover
test a baby resents, because its only seeing eye is closed.
The absence of free alteration of fixation in a subject younger than 4-5 years
is the indirect proof of amblyopia of the deviated eye. Even if a patient is able to
fixate by occluding the good eye, the fixation returns to the good eye immedi-
ately after removal of the occluder. Absence of free alteration is not a sign of
amblyopia in patients older than 4-5 years. In these patients a monofixation (i.e.,
a lack of alteration) is found in spite of isoacuity, when amblyopia was not
treated before that age. During amblyopia treatment in very young children
(preverbal), the fixation capability changes as the patient gradually improves his
capability of maintaining fixation.
ii. Fixation pattern: It is useful to find out whether fixation is central and steady
or eccentric and/or wandering. This test can be performed in children older than
2-3 years of age. The evaluation of fixation is useful in order to understand the
reasons for an otherwise inexplicable low visual acuity. Treatment with direct
occlusion should be attempted, provided that the patient is still in the plastic age
of the visual system. Fixation is noted with a direct ophthalmoscope with a
fixation pattern. Asking the patient to look into the target first with the good eye
(in order to become familiar with the test) and then with the amblyopic eye.
Following Features are Noted
a. Whether the patient uses the central fovea for fixation and
b. Whether fixation is stable or wandering.
A central wandering fixation has a better prognosis than a stable eccen-
tric fixation.
Fixation can be grossly evaluated, even in babies using an indirect Oph-
thalmoscope with a +20 D lens as to detect whether the patient is using the
central fovea for fixation.
iii. The presence of normal binocularity is the indicator for good treatment results
of monocular amblyopia. “Normal binocularity” indicates normal sensory and
motor fusion which can be measured by the Bagolini glasses and a bar of prisms,
respectively, and by presence of random dots stereopsis. Normal binocularity
maintains a continuous use of both eyes.
28 Strabismus

TREATMENT MODALITIES
3 The refractive error should be corrected first. Optimal prescription for amblyopic
eye should correct the full refractive error as determined by cycloplegia. Cataracts
capable of producing amblyopia must be operated at the earliest. Refractive cor-
rection for aphakia following cataract surgery in childhood must be given promptly.
i. Classical full-time occlusion is the best method even in the presence of
nystagmus. A patch applied in the skin is far preferable to a patch applied on the
glass as the child can easily take off the glasses or look outside the occluded glass.
Regarding choice of the occluder it must be noted that some cause skin irritation.
Occlusion of the fixating eye removes inhibitory stimuli to the amblyopic eye. It
may be either total or part-time. To prevent occlusion amblyopia of the sound eye
alternate occlusion should be done. Visual acuity of both the eyes should be moni-
tored at regular intervals of 4 weeks.
Occlusion Regime
Age of the child Amblyopic eye: Better eye
1 Year 1:1
2 Years 1:2
3 Years 1:3
4 Years 1:4
5 Years 1:5
6 Years and above 1:6
Overtreatment may lead to ‘occlusion amblyopia’ in the originally better eye.
Close monitoring is needed particularly in a young child. Recent studies have shown
part-time occlusion is as effective as total occlusion, particularly as maintenance
therapy. A one-hour occlusion is very effective in order to maintain and refine the
results achieved. Occlusion should be used with care at early ages because of the
risks of inducing a deprivation amblyopia in the better eye. At later ages, prolonged
occlusion, even if altering, can cause diplopia, because of its weakening effect on
suppression. This diplopia will disappear by discontinuing the treatment, if the prob-
lem is noted early. Otherwise, diplopia may become untreatable.
Unilateral amblyopia with normal binocularity can be effectively treated up to
7-8 years of age. This condition is usually due to anisometropia and often due to the
presence of astigmatism in one eye. Part-time occlusion with full retinoscopic cor-
rection can solve this problem within a short period.
Amblyopia in strabismic patients is more difficult to treat. An occlusion should
be attempted by 6-7 years even if vision is low (as 6/60) and fixation is eccentric If
no improvement is noted in one month then it is not worthy to continue the treat-
ment. The same applies if diplopia starts. If small improvements are noted then the
continuation of the treatment is worthy up to 9-10 years of age though a full recov-
ery is not expected. In cases with eccentric fixation differentiation must be made
between “true eccentricity”, which remained unchanged with all the treatments,
and “eccentric viewing”, which becomes centric with one month of full occlusion.
Amblyopia 29

In order to achieve good results parental co-operation should be good.


ii. Alternative treatment modalities: Other treatment modalities include
pleoptics, penalization, sectors and CAM stimulator.
3
Other unsuccessful methods include red filters and prisms for eccentric
fixation.
a. Pleoptics: Pleoptics consists of stimulating an amblyopic eye with prolonged
sessions of dazzling light flashes in order to improve visual acuity.This method
was introduced by Bangerter (1940s) and used by Cuppers later on. In the
1950s pleoptics was used in school children, even after 8-9 years of age. Flash
stimulations improve the visual acuity of an amblyopic eye only temporarily and
there is invariably a deterioration over time and the improvement is usually lost
as early as one year after its completion. Moreover, pleoptics is difficult to
administer to children younger than 6-7 years of age. It may also can cause
permanent monocular diplopia by stimulating the central fovea in the presence
of eccentric fixation. For all these reasons pleoptics has no current role in the
management of amblyopia.
Though amblyopia with eccentric fixation was considered to have a poor
prognosis, normalization may be obtained if it is diagnosed in early childhood and
treated properly. Favourable cases respond to conventional occlusion. If no posi-
tive response is obtained with conventional occlusion they are considered to be
lost cases.
b. Penalization: The principle of this treatment is selective fogging of one eye by
means of glasses and/or cycloplegic drugs. Thus a penalization for amblyopic
eye for distance will force using the good eye only for near vision and the
amblyopic eye for distance. In case of near- penalization the opposite phenom-
enon occurs. A total penalization fogs one eye both for near and distance. The
amount of plus or minus correction to be added in order to have a shift of
fixation must be determined empirically for each patient.Often, fixed amounts
of over correction are prescribed which is risky as it may produce deprivation
amblyopia in the good eye.
Penalization can be prescribed in selected patients who do not accept occlu-
sion. However, efficacy of penalization has been found to be similar to that of
occlusion though the patient can easily take of the glasses. A prolonged use of
atropine to fog the eye is often ineffective for obtaining a shift in fixation. More-
over, systemic effects of atropine have been described in some cases.
c. Sectors: This method involves masking portions of the glasses. Sectors are cut
and glued on to the lenses. This method is not very popular.
d. CAM stimulator: This is a rotating disc with black and white stripes down its
surface. The rationale behind this treatment is the presentation of spatial fre-
quencies to the visual cortex connected with the amblyopic eye, in order to
improve its function.The results are not very encouraging.
30 Strabismus

CRITERIA FOR VISUAL IMPROVEMENT


3 1. Isoacuity between the good and the previously amblyopic eye.
2. Equal speed of reading for both the eyes. If there is any difference between
them maintenance treatment should be continued.
3. Free alteration of fixation ensures that a cure has been obtained. If a cure is
achieved without alteration, there is risk of a relapse. If the patient reaches
isoacuity after 6th year of life free alteration is difficult to achieve and close
observation is advisable until 10-12 years of age.
4. Isoaccommodation is achieved when a cure is obtained and free alternation of
fixation is reached.
Recurrence may occur in about 50 per cent of patients when amblyopia treat-
ment is discontinued after fully or partially successful completion of occlusion
therapy. However, this can nearly always be reversed with renewed therapeutic
effort. Repeated backsliding can be prevented by an maintenance regimen and
regular followup thereafter.
Eccentric fixation: It usually respond to conventional management but sometimes
Inverse occlusion of the squinting eye is done for a period of up to 3 months before
starting conventional occlusion. This is said to disrupt the use of the eccentric
fixation. Red filter treatment is also sometimes used. The fixing eye is occluded
and a red filter is used in front of the squinting eye. This is said to stimulate foveal
fixation.
4
Classification of Strabismus and
Approach to a Patient with
Strabismus—Basic Guidelines

CLSSIFICATION OF STRABISMUS
According to the fusional status:
i. Phoria: A latent deviation in which fusional control is always present
ii. Intermittent tropia: Fusional control is present part of time
iii. Tropia: A manifest deviation in which fusional control is not present
According to the variation of deviation of the deviation with gaze position:
i. Comitant (concomitant): The deviation does not vary with direction of gaze or
fixing eye.
ii. Incomitant (noncomitant): The deviation varies with direction of gaze or
fixing eye. They may be paralytic or restrictive.
According to the type of fixation:
i. Alternating: Spontaneous alteration of fixation occurs from one eye to another.
ii. Monocular: There is a definite preference for fixation with one eye.
According to the age of onset:
i. Congenital: A deviation noted in early infancy, presumably related to a defect
present at birth .
ii. Acquired: A deviation with later onset, after a period of apparently normal
visual development.
According to the type of deviation:
i. Horizontal:
a. Esodeviation: The eye is rotated so that the cornea is deviated nasally
and the fovea is rotated temporally.
b. Exodeviation: The eye is rotated so that the cornea is deviated temporally
and the fovea is rotated nasally .
ii. Vertical:
a. Hyperdeviation: The eye is rotated so that the cornea is deviated inferi-
orly and the fovea is rotated superiorly .
b. Hypodeviation: The eye is rotated so that the cornea is deviated superi-
orly and the fovea is rotated inferiorly.
iii. Torsional:
a. Incyclodeviation: The eye is rotated so that the superior portion of the
vertical meridian is torted nasally and the inferior portion of the vertical
meridian is torted temporally.

http://ophthalmologyebooks.com
32 Strabismus

b. Excyclodeviation: The eye is rotated so that the inferior portion of the


4 vertical meridian is torted nasally and the superior portion of the vertical
meridian is torted temporally.
Pseudosquint
There are some factors which can lead to the squint where, in fact, no squint is
present and constant binocular single vision is being maintained. They can be
differentiated by the demonstration of a conclusive negative cover test and by
demonstration of binocular single vision.
Examples: i. Facial asymmetry.
ii. Pseudo-convergence due to epicanthus, negative angle alpha,
gamma and kappa.
The goals of a strabismus evaluation are:
1. To find the reason behind and type of the strabismus
2. To assess the binocular status
3. To measure the amount of deviation
4. To diagnose amblyopia, if any.
So, a strabismus patient must be examined in the following order:
• History taking
• Visual acuity testing
• Cycloplegic refraction
• Fundus examination
• Sensory tests
• Measurement of deviation
• Ductions and version
• Special tests
History Taking
i. Age of onset and duration of strabismus
ii. Nature of onset: Sudden/gradual/intermittent
iii. Any apparent precipitating factors like trauma, febrile episode or cerebrovas-
cular accident should be asked for
iv. Past history patching, intermittent diplopia/blurring, spectacle wear, trauma,
previous surgery (strabismus, cataract, retinal detachment, blow out fracture,
glaucoma implant, sinus surgery or neurosurgery)
v. Family history
Visual Acuity Testing
Visual acuity of a new born is 6/240 which increases to 6/90 by the 1st month. By
4-6 months it varies between 6/18 to 6/6.
A. Pre verbal children
a. Fixation pattern: Fixation should be central and stable. Unsteady or eccentric
fixation usually indicates poor visual acuity. In a strabismus patient, fixation
preference for a particular eye also indicates poor visual acuity in the other eye.
Classification of Strabismus and Approach to a Patient with Strabismus 33

The fixation is diagnosed with the visuoscope, which is a modified ophthal-


moscope that projects the fixation target on the fundus. The eye not to be tested
should be occluded. The examiner should project the fixation target to the macula
4
and the patient should be asked to look at the black star shaped aperture. The
position of the star on the patient’s fundus should be noted. The fixation behaviour
is designated as central, parafoveal, parafoveolar or peripheral (Fig. 4.1 and
4.2).

Fig. 4.1: Visuoscope

Fig. 4.2: Fixation patterns


34 Strabismus

Fixation preference can also be tested by using a vertical prism of 15 prism


4 dioptres base up or base down in front of one eye, which induces a vertical
strabismus and refixational movement should be looked for. If the prism is placed
in front of one eye and refixational movement is noticed in that eye then it
indicates that this eye is fixing,but if there is no movement in either eye it indi-
cates that the other eye is fixing.
b. Optokinetic nystagmus (OKN): It can be used to assess the visual acuity in
very young children. The highest spatial frequency which produces a response
can be quantified to give visual acuity. The target must be presented in a rigidly
standardized condition.
c. Preferential looking tests (Teller cards): Here, the child is offered a choice
between black and white grating and plain area of the same size and equal
luminance. It is presumed that the child will prefer to look at grating than plain
area. Visual acuity can be quantified by spatial frequency of grating presented.
Response of the child is observed by the observer through a peep hole. Spatial
frequency of grating presented gives an estimate of visual acuity.
d. Visually evoked potential.
B. Preschool children: Here we use matching tests in which the child is asked to
match the letter or symbol by a matching card. The tests should be first demon-
strated at near distance so that the child is able to comprehend the test. These tests
include Sheridian Gardinar test, Tumbling E test, Allen cards, Stycar letters, Cam-
bridge crowding cards, Kay’s pictures, Cardiff acuity cards etc (Fig. 4.3).
C. Older children: Visual acuity can be tested by using Snellen’s chart or near
vision chart.
D. Visual acuity in nystagmus: Monocular vision recording in these patients is
difficult as when an occluder is placed over one eye it often worsens the nystag-
mus and visual acuity is reduced. This problem can be eliminated by placing an
occluder at some distance in front of one eye or high plus lens can be used to fog
the other eye. Neutral density filters can also be used.
Cycloplegic Refraction
Cycloplegic refraction is absolutely essential for a case of strabismus. One per
cent Atropine sulphate is preferred in young children. Adverse reactions to Atro-
pine like allergic reaction, flushing, dryness and fever should be explained to the
parents. In older children Cyclopentolate and Homatropine can be used.
The patients with phorias must be given appropriate refractive corrections.
The vertical and tortional phorias are almost always accompanied with horizontal
phorias. In exophorias, myopic corrections should be given though hypermetropic
corrections less than +2 DSph is not recommended. In esophorias, hypermetropic
corrections must be given, particularly if it is more than +1.25 DSph, but myopic
corrections must be avoided.
Classification of Strabismus and Approach to a Patient with Strabismus 35

INSTRUCTION
PRESCHOOL VISION TEST, HENRY F. ALLEN, M.D.
4
Explanation—THIS TEST IS A VALID INDEX OF VISUAL ACUITY RECORDED IN TERMS OF A
30-FOOT DENOMINATOR. IT IS INTENDED FOR PRESCHOOL CHILDREN AND HAS GIVEN
RELIABLE RESULTS FROM THE AGE OF TWO YEARS AND UP. IT IS ALSO USEFUL FOR
RETARDED OLDER CHILDREN AND FOR ILLITERATE ADULTS. IT CAN BE USED FOR MASS
SCREENING OR FOR INDIVIDUAL TESTING. NO PRETRAINING OF YOUNGER CHILDREN IS
NECESSARY.
METHOD OF TESTING—THE PICTURES ARE SHOWN TO THE SEATED CHILD AT CLOSE
RANGE WITH BOTH EYES OPEN AND THE CHILD IS ASKED TO GIVE A NAME TO EACH
PICTURE. THE PICTURES MOST EAGERLY RECEIVEDARE MOST LIKELY TO BE USEFUL. ONE
EYE IS THEN COVERED AND THE EXAMINER PRESENTS THE PICTURES IN SEQUENCE WHILE
BACKING AWAY FROM THE CHILD. THE GREATEST DISTANCE AT WHICH THREE OF THE
PICTURES ARE CONSISTENTLY RECOGNIZED BY EACH EYE IS THEN RECORDED AS THE
NUMERATOR OF A 30-FOOT DENOMINATOR, FOR EXAMPLE:
RIGHT EYE MAXIMUM DISTANCE = 15 FEET: VISION = 15/30
LEFT EYE MAXIMUM DISTANCE = 10 FEET: VISION = 10/30
NOT ALL THE PICTURES NEED BE USED; THE SAME PICTURES SHOULD BE SHOWN TO EACH
OF THE CHILD’S TWO EYES IN DIFFERENT SEQUENCE.
INTERPRETATION—COMPARISON OF THE VISUAL ACUITY OF A CHILD’S TWO EYES IS
MORE IMPORTANT THAN ABSOLUTE VALUES OBTAINED. NORMAL CHILDREN BETWEEN 2
AND 3 CAN USUALLY IDENTIFY THE PICTURES AT 12 TO 15 FEET. CHILDREN BETWEEN 3 AND
4 CAN USUALLY IDENTIFY THEM AT 15 TO 20 FEET. ADULTS WITH EXCELLENT VISUAL
ACUITY CAN RECOGNIZE THEM AT DISTANCES GREATER THAN 30 FEET IN A GOOD LIGHT. A
DIFFERENCE OF 5 FEET BETWEEN A CHILD’S TWO EYES IS PROBABLE CAUSE FOR REFERRAL.

PRESCHOOL VISION TEST PUBLISHED BY OPHTHALMIX


HENRY F. ALLEN, M.D. LA GRANGE, ILL.

Fig. 4.3: Preschool vision test


36 Strabismus

Proper cycloplegic refraction is also necessary for management of refractive


4 accommodative esotropias.
Fundus Examination
Many cases of sensory esotropia and exotropia are associated with disorders of
optic nerve and retina.
Sensory Tests
It is done with the patient wearing full refractive correction.
A. Tests for Stereopsis
Each eye is presented with a separate field of view and each of them contains
elements imaged on corresponding retinal areas. Stereopsis must be noted for both
near and distance.
Two pencil test for stereopsis: The examiner should hold a pencil vertically in
front of the patient. The patient should be asked to touch the tip of the examiner’s
pencil from the above with a second pencil held by the patient. If the patient fails to
touch with one eye closed and touches with both eyes open, it indicates that gross
stereopsis is present (Fig. 4.4).

Fig. 4.4: Two pencil test from stereopsis

http://ophthalmologyebooks.com
Classification of Strabismus and Approach to a Patient with Strabismus 37

a. Near Stereoacuity: It is assessed by the following methods:


i. Titmus stereo test:
This consists of a three dimensional polaroid vectograph consisting of two
4
plates in the form of a booklet. On the right is a large fly and on the left are
a series of circles and animals. The plates are viewed with Polaroid glasses.
The ‘fly’ is used for testing gross stereopsis where the ‘fly’ should appear in
solid three dimensions. The ‘circles’ and the ‘animals’ are used for fine
depth perception (Fig. 4.5).

Fig. 4.5: Titmus stereo test


ii. Randot stereo test:
It is a Polaroid test similar to Titmus stereotest but utilizing a randot-dot type
of target instead of contour targets. It is polarized in the same way and
viewed with the same kind of glasses. This test is better than the Titmus test
in term of giving fewer monocular clues, but does not appear to be better
than the TNO test, except in so far as the red-green glasses of the TNO test
tend to produce more dissociation in patients with a poorly controlled phoria.
Another version of the test consists of a random-dot letter-‘E’. It should be
performed at 50 cm and the child is required to identify which of the two
plates contain the ‘E’ (Fig. 4.6).

Fig 4.6: Randot stereo test

iii. TNO Random-dot test:


It consists of seven plates each of which contains various shapes (squares,
dots, crosses) created by random dots in complementary colours which are
38 Strabismus

viewed with red-green spectacles. The plates contain both visible features
4 which can be seen with or without the red-green goggles and hidden shapes
which are visible only with red-green goggles.The first three plates enable
the examiner to find out the presence of stereopsis and the other plates are
used to determine its level. As there are no monocular clues, this test pro-
vides a true measurement of streopsis (Fig. 4.7 and 4.8).

Fig. 4.7: TNO Random dot test

Fig. 4.8: TNO random-dot test


iv. Frisby stereo test
It can be used to assess stereopsis for near. It does not require special
spectacle. It is a stereotest based on actual depth of the target. It consists of
three perspex sheets of different thickness and the patient is asked to iden-
tify which figure of four on each plate is the one that is either closer or
farther away , the fixation target having been printed on one or the other side
of the perspex sheet. The disparities are between 600 and 15 seconds of arc
and it is easy to use in the relatively small children (Fig. 4.9).
v. Lang stereo test
It is useful in children who refuse to wear red-green or Polaroid spectacles.
It is based on pantographic presentation of random dot pattern. Eyes are
dissociated through the cylindrical elements imprinted on surface lamination
of the card. These are stout plastic cards with a ribbed surface, one set of
Classification of Strabismus and Approach to a Patient with Strabismus 39

Fig. 4.9: Frisby stereo test Fig. 4.10: Lang stereo test

images being printed on one slope and another on the opposite slope. If they
are viewed at an appropriate working distance one eye will tend to see the
left hand side of the picture and the other eye the right. If a random-dot
image is printed onto the postcard then the patient will see something stand-
ing out from the background. This is a fairly gross test and detects stereo-
disparities between 1200 and 200 seconds of arc. On card I all the targets
have stereodisparity and consist of a cat ,a car and a star. On card II there
is a crescent moon, a car and an elephant, but in addition there is one target
that does not have stereodisparity in which the child can identify. This is in
order to encourage the child to pick at least one of the targets and thereby
does not feel frustrated by its failure (Fig. 4.10).
b. Distance stereoacuity: It can be measured by American Optical vectogram
which used polarized glasses. Mentor B vat system can assess stereopsis for
distance. It is a computerized system in which the liquid crystal binocular glasses
are provided which are connected to a microprocessor. Each eye is presented
with disparate images at a high frequency with disparate images at a high
frequencyo.
B. Tests for Retinal Correspondence
Anatomically corresponding points from each eye localize to the same point in
space. This is accomplished by the crossing of optic nerve fibres in the optic chi-
asma. This brings fibres from the corresponding points from each eye to the same
lateral geniculate body but to different layers there. In the cortex these fibres
share a cell. Normal correspondence (NRC) is seen in straight eyes or when the
subjective and objective angles are the same. In anomalous retinal correspondence
(ARC), fovea of deviating eye loses common visual direction with the fovea of
fixing eye, and fovea of fixing eye shares a common visual direction with a periph-
eral retinal element of the deviating eye. Anomalous retinal correspondence can
be harmonious or unharmonious. Retinal correspondence can be assessed by using:
a. Bagolini’s striated glasses: The Bagolini glasses are optically plano lenses
with imperceptible striations that do not blur the environment but produce a
luminous stripe when a person is looking at a point light. The test is performed at
1/3rd metre and 6 metres. The glasses should be placed before the patient’s
40 Strabismus

eyes in such a manner that the axis of striation is oriented at 135 degrees before
4 the right eye and at 45 degrees in front of the left eye. It is advisable to eliminate
all other bright light sources in the examination room during the test.
i. In NRC, there will diplopia or suppression and there will be X response with
angle corrected.
ii. In harmonious ARC, there will be X response with manifest deviation and
there may be possible central suppression or possible paradoxical diplopia.
iii. In unharmonious ARC, there will be X response with angle partially cor-
rected and there will be suppression or diplopia.
iv. In absent retinal correspondence, there will be suppression or diplopia and X
response will not be obtained with prisms (Fig. 4.11).

Fig. 4.11: Bagolini striated glass test


b. After image test: This test can also be used to assess ARC. In this test each
fovea is stimulated separately and labels each eye with a linear after image.
High intensity 12 V illumination is required to create the after image. Slides for
after image consist of a white vertical slit with black background and with a
central red fixation dot before one eye and a white horizontal slit with a similar
fixation dot before the other. These slides are inserted into the carriers with the
matt surface out of the optical pathway by rotating the control levers. This
allows more light to pass through the slide and thus a strong after image is
produced. The selector switch is rotated clockwise from the ‘normal’ to the
first position marked ‘right eye’. The patient is instructed to fix on the red spot
for 10 seconds with the right eye. The selector switch is turned clockwise to the
next position ‘change’ which is the ‘off’ position. Right slide is removed and the
diffusing screen raised again. The selector switch is then moved to ‘off’ position,
Classification of Strabismus and Approach to a Patient with Strabismus 41

the left slide is removed and the swivel screen is raised. Automatic flashing is
then employed to enhance the after change. The selector switch is rotated to
‘both eyes’ and the autoflash switch is switched on. When the retinal
4
correspondence is normal the patient will observe the after image as + with a
central gap. If it is abnormal the after image will be seen as in the figure. In
esotropia with ARC the after images are crossed and in exotropia with ARC,
after images are uncrossed (Fig. 4.12).

Fig. 4.12: After image key chart

c. Synoptophore: It can also be used to assess retinal correspondence. If the


subjective and the objective angles are same, it indicates NRC.
d. Worth 4 dot test: It can also be used to assess ARC. If the patient sees 4 lights
in presence of manifest deviation, it indicates ARC.
C. Tests for Suppression
Suppression is alteration of visual sensation resulting from inhibition of one eye’s
images from reaching consciousness. It is a sensory adaptation to avoid diplopia. It
is a purely binocular phenomenon. Suppression can be central or peripheral, mono-
cular or alternating, facultative or obligatory.
a. Worth 4 dot test: A red glass filters out all colours except red and a green light
is not visible through a red filter. Similarly every colour except green will be-
come visible when viewed through a green filter. Looking through a pair of
diplopia goggles (with red before the right eye and green before the left eye) the
patient views a box with 4 lights (one red, two greens and one white) at 6
metres and at 1/3rd metre. The patient’s responses are interpreted accordingly
(Fig. 4.13, Plate 1).

http://ophthalmologyebooks.com
42 Strabismus

Responses
4 1. If the patient sees all 4 lights It indicates fusion or ARC
2. If the patient sees only 2 reds or 3 greens: It indicates suppression
3. If the patient sees 5 lights: It indicates diplopia
b. 4 prism base out test: Small central scotomas (1 to 2 degrees) are difficult to
detect in cases of small angles of esotropia/microstrabismus. This test demon-
strates small foveal suppression scotoma associated with microtropias. In a
person with bifoveal fixation if a 4 prism dioptre is placed in front of right eye,
the right eye will move nasally and left eye temporally followed by a refixational
movement of the left eye. If a suppression scotoma is present in the right eye
the placement of the prism will not elicit any movement in either eye. If we
place the prism in front of the left eye, the right eye will show initial conjugate
saccade towards the apex of the prism but there will be no refixational move-
ment (Fig. 4.14).

Fig. 4.14: 4Δ base-out prism test


Classification of Strabismus and Approach to a Patient with Strabismus 43

c. Bagolini’s glasses: It can also detect suppression. The patient will perceive
only 1 line. 4
MOTOR TESTING
A. Head posture: Comitant heterotropias usually have a normal head posture.
Abnormal head postures are seen in incomitant squints, A/V patterns, nystagmus ,
in Duane’s retraction syndrome and in Brown’s syndrome. Abnormal head posture
is adopted either to avoid diplopia or achieve binocularity. Abnormal head posture
can take form of face turn or head tilt. There can be chin elevation or depression
(Fig. 4.15 and 4.16).

Fig. 4.15: Abnormal head postures

CHIN

Raised Depressed
RSR, RIO, LSR, LIO RIR, RSO, LIR, LSO

Face turn: To the right To the left To the right To the left

RSR, LIO LSR, RIO RIR, LSO RSO, LIR

To the To the To the To the To the To the To the To the


right left right left right left right left
Head tilt RSR LIO RIO LSR LSO RIR LIR RSO

Fig. 4.16: The chart showing abnormal head posture


44 Strabismus

B. Lid position: Position of lids and palpebral fissure should be examined. Any
4 ptosis or pseudoptosis should be noted.
C. Binocular motor functions:
i. Ocular movements: There are 3 types of ocular movements. Ductions are mo-
nocular pursuit movements. Versions are binocular pursuit movements and
vergences are binocular movements where eyes move in opposite directions.
To check for ocular movements
, patient fixates at a small fixation
target and it is moved in nine
different positions of gaze.
Ductions are first tested and then
the versions. Any overaction or
under-action of the muscles should
be noted. Any A or V pattern
should also be noted. For A and V
patterns, prism cover test should be
performed at near fixation on an
accommodative target with the
patient wearing his full correction
and the eyes in 25 degrees eleva-
tion, primary position and 35
degrees depression. The deviations
in each positions are recorded
(Fig. 4.17 and 4.18).

Fig. 4.17: Ductions (right eye) Fig. 4.18: Versions


Classification of Strabismus and Approach to a Patient with Strabismus 45

To measure the motility Kastenbaum’s limbus test of motility can be used.


This test is done by holding a transparent ruler in front of the eye and in order to
measure abduction, position of nasal limbus is marked on ruler in primary position
4
and on maximum abduction. The difference is noted in the millimeter scale. In
the similar way adduction, elevation and depression can be measured. Normal
values for abduction, adduction and depression are 10 millimeter and for elevation
it is 5-7 millimeter. In normal adduction an imaginary vertical line through lower
lacrimal punctum should coincide with inner 1/3rd and outer 2/3rd of cornea. If
more cornea is hidden, there is excessive adduction and if sclera remains visible
it is defective. In normal abduction corneal limbus should touch outer canthus. If
the limbus passes that point abduction is excessive and if it fails to reach it the
abduction is defective.
Fusional vergence amplitudes should be noted using prism bar or the
synoptophore. Near point of convergence and AC/A ratio are measured in
selected cases.
ii. Corneal reflex tests
a. Hirschberg’s test: The fixation light should be held at 1/3 meter from the
patient. This test is based on the calculation that 1 mm shift in light reflex
from the center is equal to 7 degrees of deviation of visual axis. Therefore
a light reflex at pupillary margin which is 2 mm away from center corresponds
to 14 degrees of deviation. In mid-iris region it is equal to 30 degrees and at
limbus it corresponds to 42-45 degrees of deviation (Fig. 4.19).

Fig. 4.19: Hirschberg’s test


b. Modified Krimsky’s test (Prism bar reflex test): This test utilizes light reflex
from both the eyes. The patient fixates a pen light with his better eye and
prisms are added till light reflex is centred in the deviated eye. The amount
46 Strabismus

of prism needed gives the amount of deviation. In cases of esodeviation


4 base-out prisms and in cases of exodeviations base-in prisms are used
(Fig. 4.20).
c. Bruckner test: It offers an assess-
ment of eye alignment in young
patints whose eye alignment may
be impossible to assess using the
subjective methods and whose
limited co-operation and fixation
prevent an accurate assessment
by cover test. Here, a direct
ophthalmoscope is used to elicit a
red reflex from both eyes simul-
taneously. In patients with
strabismus, this test shows
asymmetric reflexes with a
brighter reflex coming from the
deviated eye. The child should be
placed in a parent’s lap watching
the examiner positioned about 1
meter from him. The room
illumination should be dim to help
focus the child’s attention on the
examiner’s light and to aid the
Fig. 4.20: Prism bar reflex test
examiner’s observation. As the
test is influenced by asymmetrical refractive errors, the habitual refraction
should be worn. Testing should be done with natural pupils before dilatation
or cycloplegia. The examiner should focus the ophthalmo-scope so that the
pupil and red reflexes are in sharp focus for optimal observation. The
advantage of Bruckner’s test is its ability to detect deviations smaller than
the lower limit of Hirshberg’s test (about 5 to 10 prism dioptres).
Bruckner suggested that a deviation of 3 prism dioptres induced an
observable change in the red reflex of the deviating eye.
iii. Cover tests
a. Cover test: The apparently fixing eye is covered and the movement of the
other eye is noted. If the other eye moves to take up fixation it indicates
presence of manifest squint. If there is no movement in the fellow eye then
that eye is covered and the other eye is observed. Cover test is performed
for both near and distance fixation. It not only establishes the presence of a
manifest squint but also diagnoses latent nystagmus, which becomes mani-
fest when one eye is covered. It also gives an idea about the degree of
alteration of strabismus (Fig. 4.21, Plate 1).

http://ophthalmologyebooks.com
Classification of Strabismus and Approach to a Patient with Strabismus 47

b. Cover/uncover test: It is a test to detect latent strabismus. Here, as one and


then the other eye is covered while patient fixes at a target, movement of the
eye under the cover is noted as the cover is removed. If the eye moves in to
4
take fixation it indicates exophoria and if it moves out it indicates esophoria.
The phoria may be also of vertical or tortional type. This test should be done
both with or without glasses and for both near (1/3 meter) and for distance
(6 meters) (Fig. 4.22, Plate 2).
c. Alternate cover test: Here, the total deviation is measured. It does not dif-
ferentiate between latent and manifest strabismus. It can be combined with
prisms to measure the angle of deviation (Prism bar cover test). To perform
this test we alternately cover each eye while the patient maintains fixation.
Prisms of increasing strength are placed in one eye with the apex oriented
towards direction of squint (base out for esotropia and base in for exotropia)
till no redressal movement is elicited. The amount of prism needed to achieve
is the angle of deviation. This test is done for both near and distance. If the
patient has a refractive error the angle should be measured both with and
without glasses for near and distance (Fig. 4.23).

Fig. 4.23: Prism bar cover test


48 Strabismus

iv. Dissimilar image tests: Diplopia is produced by presenting two dissimilar images.
4 Distance between the images is the measurement of deviation. The images
may be crossed as in exotropia or uncrossed as in esotropia.
a. Red filter test: A red filter is placed over the fixating eye. The patient fixates
over a small light and reports whether red image is crossed or uncrossed, up
or down. Red glass should be dark enough to differentiate as well as disso-
ciate the eyes. Sometimes a vertical prism is added to the red filter to appre-
ciate the diplopia better.
b. Maddox rod test: It is a lens which consists of a series of parallel cylinders
which convert a point source of light to a streak which is oriented perpendicular
to that in the Maddox rod. Maddox rod also dissociates the eyes. The test
should be performed both for near (1/3 meter) and distance (6 meters).
During the test the patient must wear his spectacle corrections and the vision
must be sufficient to see the light with one eye and the line with the other.
One eye perceives a point of light while the other sees a red vertical line, if
the Maddox rod is placed horizontally.Maddox rod is traditionally placed
over the right eye. If the line bisects the light there is orthophoria, if it is on
the right side of the light there is esodeviation and if it is on the left side of the
light there is exodeviation. This test can not differentiate between heterophoria
and heterotropia. Maddox rod can be used to detect horizontal as well as
vertical deviations. To measure vertical deviation the cylinders are placed
vertically.When the line passes below the light there is left hyperphoria and
when it passes above the light there is right hyperphoria. To measure the
deviations, prisms are placed with apex in the direction of deviation till the
line crosses the light. It can also be used to measure the tortion. It is placed
in vertical direction. Patient is asked to rotate the lens till one becomes
horizontally straight. Degree of torsion can be read off the trial frame (Fig.
4.24) (Fig. 4.25 and 4.26, Plate 2).

Fig. 4.24: The maddox rod: This high powered cylindrical lens is used
clinically to form a line image from a point source of light.
Classification of Strabismus and Approach to a Patient with Strabismus 49

c. Double maddox rod test: This is


used to diagnose and measure cyclo-
deviations. A red Maddox rod is
4
placed in front of right eye while a
white one is placed on left eye with
cylinders aligned vertically so that the
patient sees two horizontal lines. The
patient is allowed to rotate axis of
one of the cylinders till the two lines
are aligned. Deviation can be
measured in degrees directly from
the trial frame (Fig. 4.27, Plate 3, and
4.28).
d. Maddox wing test: It is a device
which records the degree of
heterophoria (for near) at a distance
of 1/3 meter from the eyes. The
patient looks through the two slits in
the eyepiece and the fields of each
eye are separated by a plate. The
right eye sees a white arrow pointing
vertically upwards and a red arrow
pointing horizontally to the left and Fig. 4.28: Maddox double prism test
the left eye sees a horizontal row of digits in white and a vertical row of
digits in red. These are calibrated to read in degrees of deviation (Fig. 4.29,
Plate 3):
i. The horizontal phorias are measured by noting the digit which is pointed
by the white arrow. If the arrow points the digits on the left hand side of
‘0’ there is exophoria and if it points to the right hand side of ‘0’ there is
esophoria.
ii. The vertical phorias are measured by noting the digit which is pointed by
the red arrow. If the red arrow points any digit above ‘0’ there is left
hyperphoria and if it points to any digit below ‘0’ there is right hyperphoria.
iii. Tortional phorias are diagnosed if the white line and the red arrow are
not parallel. The degree of rotation required to make the red arrow par-
allel to the white line is the measurement of cyclophoria.
v. Dissimilar target tests: These tests are based on the patient’s response to dis-
similar images created by each eye seeing different targets. The prerequisite
for this test is the presence of NRC. The deviation is measured with the first
eye fixing and then the second eye.
a. Hess’s screen test/ Lee’s screen test: This test utilizes the red-green goggles
and a screen that has a red light each in 8 inner and 16 outer positions and a
green slit projection. Each point on the inner field represents fixation 15
50 Strabismus

degrees from the primary position, and on the outer field at 30 degrees.
4 Conventionally, the green filter is placed before the eye to be tested. The
patient sits at 50 cm. and is asked to put green slit over each of the red dots.
The red-green glass is then reversed to record the secondary deviation. A
polarized version of Hess screen is also available (Fig. 4.30 and 4.31, Plate
3).
Interpretation
• Size of the fields: A difference in size shows incomitance. The smaller field
indicates the primarily affected eye. Equal size shows concomitance, sug-
gesting a long-standing deviation or a non paretic etiology.
• Sloping sides of the fields: This shows an ‘A’ or ‘V’ pattern in which the
horizontal deviation becomes relatively more convergent on elevation or
depression respectively. This usually implies an underlying bilateral defect of
ocular motility.
• Examination of the smaller field:1. The type of deviation in primary posi-
tion indicates primary deviation. 2. The position of the greatest restriction of
the field indicates the position of the main limitation of movement. 3. The
overactions are indicated by enlargement of the field.
• Examination of the larger field: 1. The type of deviation in primary posi-
tion indicates the secondary deviation. 2. The position of the greatest en-
largement of the field shows the main overaction.
• Examination of the outer fields: The outer field must always be plotted
and may show a defect when the central fields appear normal, particularly
where a mechanical defect is present, or in cases of slight paresis (Fig.
4.32).

Fig. 4.32: Diagnosis of right inferior oblique paralysis


Classification of Strabismus and Approach to a Patient with Strabismus 51

b. Lancaster red/green test: This test also has a screen with squares. The
patient wears a red-green goggles and sits at 2 metres. The examiner projects
red slit on screen while patient tries to coincide with green slit in his hand.
4
The goggles is then reversed to record secondary deviation.
c. Diplopia test: The patient should wear a red and green goggles. This test
should be combined with ocular movements. The patient should look at a
light in the nine cardinal positions of gaze and should be asked to indicate the
relationship of the diplopia images to each other, i.e., whether separations
are horizontal or vertical or both. The patient should be asked to indicate
where the separation of the images is greatest and also whether ,at any
point, he sees single. He may also complain of tortional (tilted image) diplo-
pia. This is likely to occur with a palsy of an oblique muscle. In such a case
a bar light should be used and the patient must describe which way one or
both of the lines are tilted, indicating an intorted or extorted image. Diplopia
can be recorded either on a chart or by verbal description (Fig. 4.33, Plate 3)
and (Fig. 4.34, Plate 4)).
d. Synoptophore: It projects dissimilar targets which the patient is asked to
superimpose. The deviation can be read directly from the scale.
Adjusting the Synoptophore
Adjustment of the synoptophore should be made first.
i. The patient’s interpupillary distance should be measured and the pointer should
be adjusted on the scale accordingly.
ii. The height of the chinrest should be adjusted by means of control.
iii. The projection of the chinrest should be adjusted by sliding it and the patient’s
eyes should be adjusted as close to the eyepiece as possible.
iv. The projection of the forehead rest should be adjusted.
v. All the pointers should be set at zero
• Pointers on horizontal deviation scales
• Pointers on vertical deviation scales
• Pointers on tortional scales
• Pointers on elevation depression scales (Fig. 4.35)

Fig. 4.35: The synoptophore

http://ophthalmologyebooks.com
52 Strabismus

Uses
4 i. Assessment of Binocular Vision
When the corneal reflexes are at the centers and when there are no movements
of the eyeballs on alternate illumination of the eyes by the synoptophore lights
the binocularity of the eyes are determined by the simultaneous macular perception
(SMP)/simultaneous paramacular perception(SPMP) slides. If the person can
recognize both the slides at the same time then SMP/SPMP is present. Fusion
slides and stereopsis slides are used to determine the presence of fusion and
stereopsis.
ii. Measurement of the Angle of Deviation
The objective angle of deviation is measured with each eye fixing in turn to a
pair of SMP slides. One of the lights is extinguished by depressing one of the
two flashing switches and the patient is asked to concentrate on the illuminated
picture. After ensuring that the fixation is accurate, the light for that side is
extinguished while simultaneously switching on the other. The nonfixing eye is
then observed as it moves to take up position and the movement is compensated
by adjusting the horizontal and vertical controls until there is no movement of the
nonfixing eye when it takes up position. The angle thus measured is the objective
angle and if the fixation is central the corneal reflections are observed to be in
the centers of the pupils.
The subjective angle is measured by asking the patient to move the handles
and adjust the vertical control until the two pictures are superimposed. If the
objective and the subjective angles are equal then the retinal correspondence is
normal. If the two angles differ, then the retinal correspondence is abnormal
and the difference between these two is the angle of anomaly.
The elevation/depression controls are used for measuring the angle of deviation
in different vertical directions of gaze. The tubes can be rotated upwards or
downwards to the extent of 30 degrees although beyond 15 degrees elevation or
depression is rarely required.
iii. Measurement of Vergences (Range of Fusion)
Adduction and abduction can be measured by setting the tubes at the angle of
deviation and by asking the patient to fix at a pair of fusion slides. The tubes are
locked and the knobs for vergence control are rotated up to a point when ‘fusion’
breaks. Vertical vergences can be measured by rotating the elevation and
depression controls.
iv. After Images
v. Haidinger Brushes
This phenomenon is caused by polarized light falling on the macula. Since the
center of the brush coincides with the fovea this effect is employed in cases of
Classification of Strabismus and Approach to a Patient with Strabismus 53

eccentric fixation and abnormal retinal correspondence. The motorized units of


the Haidinger brush are fitted into the slots adjacent to the slide carrier. HB
lights are switched ‘on’ and the motor switch when lowered, rotates the Polaroid
4
discs. In the eye pieces blue filters are incorporated in the grooves.
vi. Prism Dissociation Test
A 6 prism base down should be held before one eye and a rotary prism should
be held before the other. In the presence of a horizontal heterophoria the patient
will have vertical and horizontal diplopia. By the means of the rotary prism the
images are aligned horizontally until one is seen on the top of the other (Fig.
4.36).

Fig. 4.36: Prism dissociation test

vii. Bielchowsky Head Tilt Test


This is a three step test.
First step: This step is to find out whether there is right over left or left over
right. If there is right over left then the affected muscle is either the right eye
depressors (RSO or RIR) or the left eye elevators (LIO or LSR). If there is left
over right then the affected muscle is either, the right elevators (RSR or RIO) or
the left eye depressors (LIR or LSO).
Second step: This step is to find out whether the hypertropia is larger on right or
left gaze. If hypertropia is more on right gaze then it indicates that the right gaze
vertical muscles are weak (RSR, RIR work vertically in abduction and LIO,
LSO work vertically in adduction). If the hypertropia is more on left gaze then
the left gaze vertically acting muscles are weak (LSR, LIR and RIO, RSO).
After second step only two muscles are left as possible offenders. There is
always one from each eye, one is an oblique and the other is a rectus muscle,
but both are either intorters (superior muscles) or extorters (inferior muscles).
Third step: This step is to find out whether the hypertropia is larger when
measured during head tilt to the right or left? Proper measurement requires
holding the base of the prism parallel to the floor of the orbit. The right head tilt
54 Strabismus

torters are the RSO, RSR, LIO and LIR. The left head tilt torters are the RIO,
4 RIR, LSO and LSR. These two muscle pairs would be circled leaving only one
muscle with the three circles around it when the circles are superimposed. This
is the palsied muscle (Fig. 4.37 and 4.38).

Fig. 4.37: Bielchowsky head tilt test

Fig. 4.38: Diagnosis of paretic muscle in patients with right hypertropia


Classification of Strabismus and Approach to a Patient with Strabismus 55

Special Tests
A.Forced duction test: This is done to differentiate between restrictive and para- 4
lytic strabismus. Indications are incomitant squints, thyroid ophthalmopathy, blow
out fracture, Duane’s and Brown’s syndromes. To perform this test topical
anesthetic drops are applied. The eye is grasped near the limbus with forceps
and moved passively in the direction of the limitation of movements. Passive
movement is possible in neurogenic pathology while it is not possible in restric-
tive pathology. The test should be performed before, during and at the comple-
tion of the squint surgery (Fig. 4.39).

Fig. 4.39: Forced duction test

B. Active force generation test: It assesses the relative strength of a muscle. This
is done in paralytic squint. The patient is asked to move the eye in a given
direction while the examiner fixes the eye with an instrument. If the muscle
tested is paretic, the examiner will feel less than normal tension.
C. Saccadic velocity: This test is done to differentiate between neurogenic and
restrictive pathologies. It provides a graphic record of the speed and direction of
eye movements.
D.Field of binocular vision: This test is done on Goldman perimeter or on tan-
gent screen. This tests the limit of version movements. Normal field of binocular
fixation measures around 45 to 50 degrees from the fixation point.
5
Heterophoria, Accommodation
and Convergence Insufficiency

A heterophoria is a deviation from the orthoposition that is present only when


fusion is interrupted. During binocular vision the fusion keeps the eyes in the ortho-
position. When fusion is interrupted,e.g., by occluding one eye, the eye behind the
occluder will deviate from its position and as the occluder is removed the fusion
reflex will turn the eye back into the orthoposition.
Types of Heterophoria
1. Esophoria: Here, the eyes become convergent on dissociation.
Types
a. Convergence excess type: Here, the esophoria is greater on near fixation than
on distant fixation.
b. Divergence weakness type: Here, the esophoria is greater on distant fixation
than on near fixation.
c. Non-specific type: Here, the esophoria does not vary significantly in degree for
any distance.
2. Exophoria: Here, the eyes become divergent on dissociation.
Types
a. Convergence weakness type: Here, the exophoria is greater on near fixation
than on distant fixation.
b. Divergence excess type: Here, the exophoria is greater on distance than on
near fixation.
c. Non-specific type: Here, the exophoria does not vary significantly in degree for
any distance.
3. Hyperphoria/Hypophoria: A vertical deviation occurring on dissociation in
which one eye rotates upwards and the other downwards depending on fixation.
4. Cyclophoria: Here, either eye wheel rotates on dissociation so that the upper
end of the vertical axis is nasal (incyclophoria) or temporal (excyclophoria).
Esophoria: It is a convergent strabismus kept latent by the presence of bifoveal
fusion. This occurs less frequently than exophoria.
Characteristics
1. A latent convergent strabismus becomes manifest when:
i. fusion is disrupted, or
ii. when the ocular muscle become tired (fatigue) or undermined by disease,
drug or alcohol.

http://ophthalmologyebooks.com
Heterophoria, Accommodation and Convergence Insufficiency 57

Diplopia occurs immediately when the esodeviation occurs.


2. Esophoria is prevented from becoming an esotropia by divergence fusional
amplitudes that keep the eyes straight. Normally, divergence fusional ampli-
5
tudes are rarely more than 10 prism dioptres. So, if there is an esophoria of 10
prism dioptres, the patient may experience intermittent diplopia when the muscles
become tired or undermined by the factors mentioned.
3. Esophoria is more common in hypermetropes.
Evaluation: Patients with esophoria must have a refraction and wear their full
optical correction before evaluation. Myopia increases esophoria. Following tests
should be done:
a. Sensory tests: Diplopia tests, worth four dot test, stereopsis, fusional amplitude.
b. Motor tests: Cover-uncover test, prism cover test.
Diplopia test: The eyes should be dissociated with an occluder and the patient
should be asked to stare when the cover is removed. The patient will experience
diplopia while the esodeviation is manifest. The speed of recovery to single vision
is an indicator of the person’s ability to control the esophoria.
Worth four dot test: An uncrossed diplopia response (five dots) or a fusion res-
ponse (four dots) should be expected.
Stereopsis: A normal response should be expected.
Fusional amplitude: All patients with a heterophoria have fusion. Their fusional
amplitudes may differ. The assessment should be done by using a synoptophore or
prisma or both. Some patients do not have a sufficient fusional amplitude to allow
them to keep their eyes straight without symptoms.
Cover test: This test will reveal a latent deviation.
Prism cover test: The deviation must be measured with prisms and cover test in all
positions of gaze for distance and for near .
Measurement of the Amount of Esophoria
a. Maddox rod test: This test should be used to measure the amounts of hetero-
phoria both for near and for distance
b. Maddox wing test: This test should be used to measure the amount of hetero-
phoria for near.
Management
1. Orthoptic treatment: The aim of this treatment is to improve the fusional
divergence (relative negative convergence). This can be accomplished by the
following:
a. Divergence exercises with prism at any fixation distance
b. Divergence exercises on the synoptophore
c. Home exercises using the stereograms in the crossed position
d. Bar reading in convergence excess types.
58 Strabismus

Treatment should be carried out weekly in clinic and should be supported by


5 homework exercises. It is more difficult to improve relative negative convergence
than relative positive convergence. Therefore, esophorias respond less well to
orthoptic treatment alone.
Stereograms: A stereogram is a card bearing two pictures which when fused
make a complete picture, sometimes with stereopsis. Physiological diplopia is used
to obtain diplopia of the card and by adjusting the convergence to fuse the two
central pictures. Either homonymous or heteronymous physiological diplopia is used
so that fixation is either in front of the card or beyond it. Accommodation should be
fixed for the distance of the card by ensuring that the central picture is clear.
a. Exercise for positive relative convergence: The patient holds the card at arm’s
length and holds a pen midway between his eyes and the card. He looks at the
pen and notices diplopia of the card. He must adjust the position of the pen until
the two central pictures are fused. He must then try and see this central picture
clearly. He is exercising convergence in excess of accommodation since he is
fixing on the pen but accommodating for the card.
b. Exercise for negative relative convergence: The patient holds the card at
about half metre from his eyes and looks beyond it into the distance. He appre-
ciates diplopia of the card and by adjusting the position of the card he fuses the
two central pictures. He must try and see this central picture clearly. He is
exercising less convergence than accommodation because he is fixing in the
distance but accommodating for a distance of half metre.
2. Miotics: Usually Phospholine Iodide (0.06% or 0.125% once daily initially) is
used. It may be used as an aid to orthoptic treatment in convergence excess
types of decompensating esophorias if orthoptic treatment alone is insufficient
to relieve the symptoms.
3. Prisms: It may be effective in eliminating symptoms and are more acceptable to
the patients wearing glasses. As these patients need base-out prisms, the glasses
look thicker.
Larger amount of prism may be used in the Fresnel form for temporary use.
It may be used temporarily as an aid to orthoptic treatment or while waiting for
surgery, and should only be used permanently when all the other measures fail.
4. Surgery: If the deviation is too large to be controlled comfortably despite a good
range of fusional divergence then surgery is indicated. Usually it is needed in
cases of divergence weakness type as the range of fusional divergence in the
distance is small.
a. If the esophoria for distance is greater than that for near, a bilateral lateral
rectus resection is preferred. The aim of surgery should be to correct fully or
slightly overcorrect the distance measurement with full optical correction.
b. If esophoria for distance and near are equal, a recession of the medial rectus
and resection of the lateral rectus of the same eye is chosen. Adjustable
suture technique is preferred, the aim being to overcorrect the deviation by 5
to 10 prism dioptres in the immediate postoperative period.
Heterophoria, Accommodation and Convergence Insufficiency 59

Exophoria: It is a divergent strabismus kept latent by the presence of bifoveal


fusion. This is the commonest form of heterophoria. 5
Characteristics
1. A latent exodeviation becomes manifest when:
a. the bifoveal fusion is disrupted or
b. ocular muscles which keep the eyes straight become tired (fatigue) or are
undermined by drugs/alcohol/disease. Diplopia is always present when the
devia-tion is manifest.
2. As the patient’s convergence fusional amplitude controls the exophoria and
often the amplitude exceeds 50 prism dioptres, some young patients with exo-
phoria manage to control large exophorias.
3. In most patients the deviation measures the same for at infinity, 6 metres and at
near.
4. Some patients complain of double vision when they are tired or sick and some
complain of discomfort and headache as extra muscular effort is needed to
keep the eyes aligned.
5. Exophoria is differentiated from intermittent exotropia by the absence of sup-
pression and the presence of diplopia. Patients with exophoria are able to correct
the diplopia as it is rarely manifested. Whereas patients with intermittent exotropia
are not warned by diplopia due to immediate suppression and it is manifested.
Exophoria usually measures less than 20 prism dioptres, whereas in intermittent
exotropia the deviation is usually more than 20 prism dioptres.
Evaluation: The patient must have a refraction checkup and wear their full optical
correction before evaluation. Hypermetropia increases exophoria.
a. Sensory tests: Diplopia test, worth four dot test, stereopsis, fusional amplitude.
b. Motor tests: Cover-uncover test, prism cover test.
Diplopia test: The eyes should be dissociated with an occluder and the patient
should be asked to stare when the cover is removed. The patient must not blink
when the cover is removed. The patient will experience diplopia while the
exodeviation is manifest. The speed of recovery to single vision is an indicator of
the person’s ability to control the exophoria.
Worth four dot test: An crossed diplopia response (five dots) or a fusion response
(four dots) should be expected.
Stereopsis: A normal response should be expected.
Fusional amplitude: The assessment should be done using a synoptophore or
rotary prisms/prism bar.
Cover test: This test will reveal a latent deviation. There will be no manifest devia-
tion.
Prism cover test: The deviation must be measured with prisms and cover test in all
positions of gaze for distance and for near.
60 Strabismus

Measurement of the Amount of Exophoria


5 a. Maddox rod test: This test should be used to measure the amounts of hetero-
phoria both for near and for distance.
b. Maddox wing test: This test should be used to measure the amount of hetero-
phoria for near.
Treatment
Treatment is only indicated in adults if the patient is suffering from symptoms. In
children, treatment should be done if the exophoria is showing a tendency to dec-
ompensate into a manifest deviation irrespective of symptoms.
1. Orthoptic treatment: The aim of this treatment is to improve the fusional con-
vergence (relative positive convergence). The methods are:
a. Convergence exercises with prisms: Base-out prisms should be used.
b. Convergence exercises on the synoptophore.
c. Using stereograms in the uncrossed position. This exercise should be done
for 2 to 3 minutes, 5 to 6 times a day.
d. If the binocular convergence is weak, exercises should be done to improve
the near point of convergence also.
Treatment should be carried out weekly in the clinic and should be supported by
home exercises. At the end of each session the patient should look into the
distance to relax his eyes.
2. Prisms: These may be used temporarily as an aid to orthoptic treatment, and
should only be used as a permanent measure when all other measures have
failed to relieve the symptoms. The smallest amount of prism that gives
comfortable control of the deviation should be given. The prisms should be
base-in, the thick part therefore being adjacent to the nose, which is cosmetically
acceptable.
3. Surgery: This may be necessary, if the deviation is too large to be controlled
despite good fusional convergence. It is more often needed in cases of exophoria
of the divergence excess type or non-specific type as the range of relative posi-
tive convergence is limited for distant fixation. The aim of the surgery should be
to correct fully but not overcorrect the deviation, since the patient’s divergence
fusional amplitude are often poor (6 prism dioptres or less). Wherever possible,
an adjustable suture technique should be preferred.
Hyperphoria/Hypophoria: It is a vertical deviation occurring on dissociation in
which one eye moves upwards or downwards. The incidence is about 30 per cent
and it is often associated with horizontal phorias. Since vertical fusional amplitudes
are small asthenopic symptoms are marked.
Treatment
1. Prisms: Prisms are used in small deviations. The smallest prism which is com-
fortable in use.
Heterophoria, Accommodation and Convergence Insufficiency 61

2. Surgery: Surgery is indicated in situations where the deviation is too large to be


controlled by prisms especially those of paretic origin. 5
Cyclophoria: It is the tendency of either eye to rotate around the anteroposterior
axis on dissociation. When the 12 O’clock position of cornea rotates nasally it is
called incyclophoria and when it rotates temporally it is called excyclophoria.
Excyclophoria is more common.
Treatment
1. Correction of any associated phoria: As cyclophorias are usually associated
with vertical phorias, the primary treatment should be directed towards the asso-
ciated vertical phorias.
2. Surgery.
Convergence Insufficiency
This is the inability to obtain and/or maintain adequate binocular convergence with-
out undue effort.
Characteristics
1. There is weakness in converging their eyes or maintaining convergence to the
near point of convergence. Patients with a true convergence insufficiency rarely
have a near point of convergence that is more remote than 20 to 25 cm.
2. Symptoms present will be general asthenopic symptoms, as caused by decom-
pensating phorias, while performing visual tasks at the reading distance. There
may be headache, blurred vision and sometimes diplopia when convergence
fails.
3. There may be orthophoria or a small exophoria or esophoria (of 2 to 4 prism
dioptres) for distance and there is usually exophoria for near.
4. Defect of accommodation may cause an apparent convergence insufficiency.
5. It should be differentiated from:
a. accommodative problems e.g., presbyopia, accommodative insufficiency for
the age of the patient, accommodative paresis and
b. paresis of convergence.
Tests
1. Near point of convergence (NPC): A near point rule, such as RAF near point
rule, should be shown to the patient and brought gradually closer in a straight
line between the eyes. The end point of this test should be noted objectively
when one of the patient’s eyes is seen to diverge or subjectively by the patient
reporting when the fixation object appears double.
2. Near point of accommodation (NPA): In this test, a detailed target,such as a
small print, is used to assess uniocular and binocular near points of accommoda-
tion. This should be done by using a RAF near point rule. The patient is asked to
indicate when the letters first appear to be blurred. The result of this test must

http://ophthalmologyebooks.com
62 Strabismus

be interpreted with regard to the age of the patient and must be carried out with
5 the patient wearing the distance correction of any refractive error. The patient
with an abnormal near point of accommodation, in addition to convergence in-
sufficiency, rarely responds to orthoptic treatment.
Treatment
Treatment is indicated in:
a. symptom producing adults and
b. in children.
Aims of the treatment: At the end of the treatment the patient should:
i. be symptomfree
ii. recognize diplopia when convergence fails
iii. be able to maintain convergence for several seconds when a target is slowly
brought in slowly from about 50 cm and then held at the tip of the nose
iv. have a prism vergence amplitude of 40 prism dioptres base-out and 4 to 6
prism dioptres base-in for distance fixation
v. have a convergence fusional amplitude of 80 prism dioptres and divergence of
6 to 8 prism dioptres in a synoptophore
vi. be able to converge voluntarily.
Treatment Objectives
a. To overcome suppression and recognition of pathological diplopia when conver-
gence fails.
b. To improve binocular convergence.
c. Recognition of physiological diplopia.
d. To improve relative positive convergence.
e. To be able to perform voluntary convergence.
Treatment
1. Refraction and fundus examination and ordering the correct glass.
2. Orthoptic exercises: Treatment consists of 6 half-hourly visits per week with
10 to 15 minutes of daily homeworks between the visits. Symptoms, particularly
head and eye aches, often increase during the first 2 or 3 weeks of treatment
and patients should be warned of this in advance.
3. Basic convergence exercises:
a. Gradual convergence: The patient’s attention must be kept by using an
interesting test object. A reduced Snellen test type is an useful target and the
patient should be asked to try to read the chart as it moves closer. While
practising convergence exercises, the patient should be asked to always try
to fuse the diplopic images when convergence fails and not immediately
move the target away in order to get single vision again. If fusion cannot be
regained, the target should be moved slightly backwards until fusion is
regained; then the target should be moved toward the patient once more.
The aim of the exercise should be of trying to establish a near point of about
6 cm.
Heterophoria, Accommodation and Convergence Insufficiency 63

b. Jump convergence: The patient should be taught to change fixation from a


near to a distance fixation target, and vice versa , with ease. The near target
should be anywhere within arm’s length from the patient, even placed at the
5
tip of the patient’s nose. Single vision of the fixation target at any distance
should be rapidly obtained and held for about 10 seconds before attention is
diverted to the other target.
c. Voluntary convergence: Once the patient has a sustainable near point of 6
cm on both gradual and jump convergence, voluntary convergence should
be the target. The patient should be able to maintain convergence once the
fixation target is removed. The patient should be instructed to imagine that
the fixation target is still in the same spot even though it has been removed.
Relative negative convergence should be used as a relaxation exercise
after convergence exercises.
4. Surgery: It is only indicated when there is a combination of exophoria and
convergence weakness. The amount of surgery is calculated to correct fully the
distance deviation. Resection of both medial rectus muscles should be done.
5. In patients with convergence weakness with associated weakness or
absence of accommodation and a paresis of convergence: This should be
suspected if orthoptic exercises are not helpful in a co-operative patient with
convergence weakness.In these cases reading glasses incorporating both plus
lenses and appropriate base-in prisms for the distance required are the only
solution. Surgery is only indicated if there is an exophoria for the distance .Surgery
will not relieve the problem at near.
Convergence Paralysis
Congenital convergence paralysis is rarely seen. The asthenopic symptoms are
usually absent as these patients show a complete inability to converge the eyes,
voluntarily or involuntarily, in the presence of full adduction of each eye. Patients
usually suppress when looking at objects at the reading distance. No surgical treat-
ment is helpful. Plus reading glasses with base-in prisms provide limited help in
cases with diplopia. A paralysis of convergence may also be acquired.
Congenital convergence paralysis: They are orthophoric in the distance, becom-
ing in the distance, becoming increasingly exotropic at near fixation measuring
about 25 prism dioptres at 33 cm. Accommodation is also usually involved but can
be spared. Partial congenital paralysis of convergence and accommodation may
be present and be mistaken for functional convergence weakness or insufficiency
which responds to orthoptic treatment. Partial congenital paralysis of convergence
and accommodation is not improved by orthoptic exercises. Surgery is not helpful.
Bifocal glasses with appropriate base-in prisms in the reading segments or sepa-
rate reading glasses with prisms incorporated may give some relief.
Acquired convergence paralysis: The commonest cause is head trauma. Acquired
lesions affecting the midbrain area can cause convergence palsy. Multiple sclerosis,
encephalitis, tabes and mushroom poisoning can also cause convergence palsy. A
64 Strabismus

thorough neurologic assessment is needed in these cases. Surgery is of no help.


5 Appropriate bifocal glasses with base-in prisms in the reading portion or separate
reading glasses with prisms incorporated may give some relief.

ANOMALIES OF ACCOMMODATION
Anomalies of accommodation may be of 2 types.
a. Overaction of the accommodative process and
b. Underaction of the accommodative process.
Overaction of the Accommodative Process
A.Excessive accommodation: A certain degree of sustained accommodation is
often found in young uncorrected hypermetropes as a physiological adaptation.
This may also occur sometimes in myopes and in patients with astigmatism
particularly young people doing a lot of close work. It usually occurs in associa-
tion with excessive convergence. Sometimes it is seen in middle age at the onset
of presbyopia when the accommodation is being strained by close work.
Etiology
1. Large amount of close work, particularly in deficient or excessive illumination.
2. Refractive error or ill-fitting/incorrect glass.
3. General debility or ill-health.
4. Frequently unstable or neurotic temperament.
Symptoms
1. Pseudomyopia
2. Asthenopic symptoms
Treatment
1. Refraction under cycloplegia and order correct glasses.
2. Close work should be stopped for a period and then slowly increased again.
3. In more severe cases 1 per cent atropine sulfate may be prescribed for 1 to 2
weeks.
B. Spasm of accommodation: This is a spasm of the ciliary muscle which is be-
yond the control of the patient. It may reach 10 dioptres or more causing blurred
vision for the distance (pseudomyopia).
Etiology
1. Very often a psychiatric cause is present and often occurs in school children
and young adults (mostly females).
2. May have an organic cause.
Symptoms and Signs
1. Pseudomyopia: A high degree of subjective myopia is frequently seen.
2. Convergence spasm: Present with esophoria/esotropia particularly for the dis-
tance.
Heterophoria, Accommodation and Convergence Insufficiency 65

3. Poor range of accommodation.


4. Macropsia: Where objects appear larger as a result of a delusion of distance
induced by the disturbance of accommodation.
5
Treatment
1. Refraction under cycloplegia and order correct glasses.
2. Prescribe 1 per cent atropine sulfate for 4 weeks or more.
3. Stop close work for a period.
Underaction of the Accommodative Process
A.Insufficiency of accommodation: The power of accommodation is constantly
below the lower limit of the normal range for the patient’s age.
Factors Responsible
1. Lenticular: Arising from undue sclerosis of the lens and therefore a premature
presbyopia. This is a stable condition and gives rise to no symptom except for
those of presbyopia which starts at an earlier age than usual.
2. Weakness of the ciliary muscle: As this condition involves the physiological
accommodation, it is usually labile, varying within wide limits from time to time.
Etiology
1. Excessive close work in poor conditions
2. General debility and fatigue
3. Organic causes, e.g., prodromal stages of chronic simple glaucoma.
Signs and Symptoms
1. Asthenopic symptoms: Headaches, eye-strain
2. Blurred near vision
3. Usually reduced convergence
4. Exophoria.
Management
1. Refraction and prescription of correct glasses.
2. Extra convex lenses may be needed for near work.
3. Base-in prisms may be needed for treatment of exophoria and convergence
deficiency.
4. Treatment for convergence deficiency may be given by using accommodative
target for convergence to near point.
5. Miotics can sometimes be effective in these conditions.
B. Ill-sustained accommodation/accommodative fatigue: This is essentially the
same as accommodative insufficiency but is less accentuated. The range of
accommodation is initially normal but any attempt to use the eyes for close work
for a prolonged period causes the accommodative power to weaken and the
66 Strabismus

near point gradually recedes causing blurred vision for near. This is often
5 accompanied by convergence failure. Frequently this is the initial stage for a
true accommodative insufficiency. The causes are the same for these two
conditions and the managements are also the same.
C. Inertia of accommodation: In this rare condition, the patient has difficulty in
altering the range of his accommodation. It takes some time and involves some
effort for him to focus on a near object after looking into the distance.
Etiology
The causes are the same as other causes of underaction of accommodation e.g.,
excess near work, uncorrected refractive error.
Management
1. Refraction and prescription of correct glasses.
2. Any associated convergence deficiency, if present, should be treated.
3. Exercises in changing focus should be given.
D.Paralysis of accommodation: This may be artificially produced by drugs such
as atropine or may be the result of some disease process. It is frequently asso-
ciated with paralysis of convergence.
Etiology
It is due to paralysis of the ciliary muscle or the oculomotor nerve and is usually
accompanied by pupillary dilatation.Its origin may be nervous, toxic or traumatic. It
may occur in diseases of the central nervous system e.g., in encephalitis, meningi-
tis etc. It has been noted in herpes zoster.
Symptoms
1. Blurred near vision
2. Dilated pupil causing photobia
3. Exophoria/exotropia for near
4. Micropsia due to a delusion of distance induced by accommodative anomaly.
Management
1. Treatment of the causative factor.
2. Refraction and prescription of the correct glasses.
3. If the recovery is delayed a presbyopic correction should be ordered to allow
the patient to read in comfort.
4. Base-in prisms may be incorporated in glasses, if required.
Royal Air force (RAF) rule: It may be used for the Measurement of accommoda-
tion and convergence:
A. Measurement of Accommodation
Accommodation is measured in dioptres. One dioptre has a focal distance of
1 metre and therefore accommodation is calculated in terms of the reciprocal of

http://ophthalmologyebooks.com
Heterophoria, Accommodation and Convergence Insufficiency 67

the fixation distance. The near point of accommodation is assessed by using the
RAF rule. The rule cheek pads are placed on the patient’s cheeks and held in a
slightly depressed reading position. The patient wears the distance correction, if
5
any. Using the N series target, the patient observes the smallest line seen with
either eye. With both eyes open, the carrier is moved towards the patient from the
end of the rule and the patient is asked to state when the print becomes blurred.
The monocular near point of accommodation is also assessed to find out any
difference between the monocular and binocular accommodative states. The results
are recorded in centimetres and repeated three times, both binocularly and
uniocularly, to assess the effect of fatigue. The examiner is able to assess if the
level of accommodation is adequate for the patient’s age from the age scale, while
the amount of accommo-dation exerted at a given distance can be determined
from the dioptre scale. The patient’s blur point is an indicator of the amplitude of
accommodation.
B. Measurement of Convergence
The near point of convergence is assessed objectively by using the fixation target

Fig. 5.1: Measurement of convergence

on the RAF rule. Normal convergence is 6 centimetres. The fixation target of the
RAF rule is gradually moved from at least one-third of a metre from the patient ,
slowly and steadily towards the patient’s eyes. The patient is instructed to maintain
fixation on the target and state when the target blurs, jumps or becomes double.
The examiner notes the point at which this occurs by observing the break of con-
vergence in the patient’s eyes. Pupil dilatation may be seen at this stage which is
an additional indicator of convergence failure.
6
Exotropia

CLASSIFICATION
I. Congenital exotropia
II. Intermittent exotropia
III. Consecutive constant exotropia
IV. Secondary exotropia

I. CONGENITAL EXOTROPIA
It is much less common than congenital esotropia. It has the same general features
as the congenital esotropia syndrome except that the apparent limitation of abduction
and cross-fixation that occurs in some cases of congenital esotropia are absent.
General Features
1. A large constant exotropia of 30 to 50 prism dioptres is present.
2. The patient’s fixation usually alternates (alternating divergent squint) and thus
strabismic amblyopia is rare.
3. In some cases, dissociated vertical divergent (DVD) strabismus and microny-
stagmus occur.
4. In some cases, other neurological syndromes and defects occur.
Difference between Congenital and Intermittent Exotropia
Congenital exotropia Intermittent exotropia
Present in all the distances. Eyes are never Eyes are aligned some of the time, usually
correctly aligned. at near fixation.
In large intermittent exotropia with poor
control, the eyes are intermittently aligned
for near fixation.

Treatment
Goal: To strengthen the eyes to within 10 prism dioptres before the age of 2 years.
If this is achieved some peripheral fusion may possibly be obtained.
1. Glass prescription: Glasses are rarely indicated before surgery. After surgical
alignment, if there is more than 1 dioptre of anisometropia or astigmatism, glasses
may be indicated.
However, hypermetropia more than 3D Sph or myopia more than 2D Sph
requires glass correction before surgery.
2. Occlusion: It should be done to achieve equal visual acuity unless fusion is
absent, otherwise symptomatic alternation may occur in adult life.
Exotropia 69

3. Surgery: It should be done to align the eyes to less than 10 prism dioptres of
esotropia or exotropia.
4. Management of micronystagmus and DVD are the same as in congenital esotro-
6
pia syndrome.

II. INTERMITTENT EXOTROPIA


Intermittent exotropia usually starts before 18 months of age. Here, one eye
becomes intermittently divergent leading to suppression of vision. Due
to suppression, the patient cannot appreciate diplopia. Typically, it is reported that
one of the patient’s eyes move out, particularly when the patient is tired, sick,
absent-minded or under emotional stress. Prolonged occlusion test (prolonged cover
test) may help in detecting this disease.
There is often a family history and the tendency for an exodeviation is probably
present at birth.Intermittent exotropia constitutes between 70 and 90 per cent of all
exotropia. It is usually preceded by a stage of exophoria.
Clinically, there are two distinct groups of patients.
a. Patients with a deviation less than 20 prism dioptres, who can control the devia-
tion, usually and grow up with exophoria, excellent control and diplopia in case
of divergence of the eyes,
b. Patients with a deviation more than 20 prism dioptres, who are unable to control
the deviation all the time and there is frequent divergence. As this phenomenon
starts from an early age, suppression is common.
If there is diplopia when the eye is divergent, it is a case with decompensating
exophoria without suppression, but if there is no diplopia with manifest divergence,
it is a case with intermittent exotropia and suppression.
In intermittent exotropia, suppression occurs when the eye is divergent and if
the deviation is made esotropic by prisms or surgery, the patient will notice diplopia.
Exotropic patients have a larger binocular peripheral visual field than normal
patients because of the exotropic deviation of the eyes.
Symptoms
1. Occasionally, when one eye diverges, their eyes feel uncomfortable or they
close one eye particularly in bright light.
2. Usually headache, asthenopic symptoms or reading problems are absent unless
there is also a convergence weakness.
Types of Intermittent Exotropia
1. Divergence excess type: This is the commonest type. The deviation with full
optical correction in place is greater at distance than at near fixation. The devia-
tion may increase further at infinity with fixation on a far distant object.
2. Convergence weakness type: Here, the exotropia is greater at near fixation
with full optical correction in place due to weak convergence.
70 Strabismus

3. Basic type: Here, the deviations are equal in the distance and near with full
6 optical correction in place.
4. Simulated divergence excess type (with +3D Sph lenses at near): These are
the cases with greater exotropia in the distance in whom, the exotropia at near
approximates that in the distance when measured with the patient looks through
the +3D Sph lenses.
Investigations
a. Assessment of vision, fusion and stereopsis
b. Test for suppression (to differentiate between exophoria and intermittent exo-
tropia)
1. The patient fixes a small light at distance. Many cases becomes manifest
spontaneously. A cover test can be done, if necessary.
2. The patient is asked to find out how many lights are seen while the eyes are
divergent. If one light is seen, suppression is confirmed and intermittent
exotropia is diagnosed (An exophoria rarely manifest under these circum-
stances and divergence may occur after prolonged dissociating cover test.
The patient with an exophoria will see two lights when eyes diverge due to
absence of suppression).
c. Deviations for distance (at 20 feet) and at near fixation are measured, though it
is often difficult to get detailed measurements in patients at the age of 2 years
when surgery may be indicated.
d. Patients with a typical history of intermittent exotropia, which could not be detec-
ted by these tests can have an eye patched for half an hour or even as long as
one day and then be examined for detection of divergence. These patients often
do not need any treatment and reviewed at an interval of few months.
e. In an older child, stereopsis should be assessed. Patients should have perfect
stereopsis (40 seconds of arc using Titmus test). If there is imperfect stereopsis
and a constant flick exotropia with the cover-uncover test at near, monofixation
intermittent exotropia syndrome should be suspected.
Management of Intermittent Exotropia
Aim of the treatment is to eradicate the suppression and reduce/eliminate the
exotropia. To eliminate suppression the best method is to operate when the child is
young, preferably under 4 years of age. An exotropia of 5 prism dioptres is sufficient
to cause suppression. The ideal approach would be to produce a small esophoria
so that when the patient is tired or relaxes fusion, the eyes instead of becoming
divergent, becomes slightly convergent. As reliable repeated measurements are
rarely possible before 2 years of age, surgery should be postponded until then.
Patients operated on before 4 years of age have a better prognosis than those who
are operated on at a later age when the suppression is more entrenched.
When intermittent exotropia is first diagnosed under the age of 2 years, it should
be reviewed around the age of 2 years. The patients with poor control of deviation
Exotropia 71

(manifest deviation as 30-40 times a day or almost always when the child looks at
a distance) require surgery earlier than the patients with good control most of the
time, in whom surgery should be postponed and check-up after 6 months should be
6
done.
Treatment Options
A.No treatment: If no treatment is done, the deviation is most likely to remain
constant throughout the patient’s life. These patients never develop strabismic
amblyopia and always retain stereopsis at near fixation.
B. Optical: Rarely in exotropia with less than 20 prism dioptres at distance fixation,
base-in prisms (to neutralize the distance deviation) may be prescribed along
with-1D Sph lenses to stimulate accommodative convergence. Myopic patients
respond well to this treatment as they need glasses all the times. Emmetropic
patients do not respond to this treatment).
C. Orthoptics: The aim is to increase the patient’s fusional amplitude and improve
the near point of convergence. Orthoptic treatment may be helpful in some
patients:
a. where deviation is intermittent and fusion is demonstrable,
b. where deviation is less than 20 prism dioptres
c. where the patients are mature (more than 8years of age), motivated and in a
position to attend prolonged treatment sessions lasting half an hour, once a
week for 6 weeks initially.
D.Surgery: The aim is to achieve a small esotropia. If the angle is less than 20
prism dioptres and is cosmetically reasonable, surgery is usually contraindicated
and orthoptics is adviced.
1. In patients under 3 years of age:
i. Divergence excess type: Bilateral lateral rectus recession to fully elimi-
nate the deviation in distance. This produces an overcorrection at near
and diplopia which disappears within a few days of surgery. Esotropia
should not persist in these cases for more than 7 days and in those cases
base-out prisms should be given to eliminate the deviation and when
esotropia disappears the prisms should be withdrawn. If exotropia reap-
pears, base-in prisms are used to prevent the return of suppression.
ii. Basic exotropia: Surgery should be limited to the deviating eye (reces-
sion of lateral rectus, resection of medial rectus).
2. In patients over 3 years of age: The aim is to produce an overcorrection of
10 prism dioptres of esotropia for distance, which usually disappears within
10 days.
Treatment of Persistent Postoperative Esotropia
A.Constant esotropia: If a constant esotropia is present for more than 1 week
after surgery, alternate occlusion (one eye one day) for one week should be
considered. If esotropia (both for near and distance) is still present prisms should

http://ophthalmologyebooks.com
72 Strabismus

be given in patients under the age of 3 years to prevent possible amblyopia. If


6 the patient’s eye is straight in either for near or for distance no treatment is
advocated. An older patient may be reviewed weekly, for assessing the vision.
Base-out prisms are used to manage the diplopia and the patient may be watched
for another 2 months for the esotropia to subside. If the patient is fusing with the
prisms and uses the glasses regularly, no further treatment is needed.
B. Blind spot nightmare: Rarely, an postoperative esotropia of 10 to 15 prism
dioptres may increase to as much 30 prism dioptres in one week due the fact
that the patient is using the blind spot by increasing the esotropia to avoid diplopia.
They should undergo cycloplegic refraction for detection of hypermetropia,if
any. Glasses should be prescribed to incorporate any refractive error and 15
prism dioptre base-out Fresnel prism over each eye. Usually, the patient with
the help of these prisms get rid of this problem within 2 months. When the
patient becomes able to keep their eyes straight without the prisms, the prisms
can be removed from one lens and after another 2 months they can be removed
from the other. Patients who refuse to wear glass/prisms and who do not respond
to this treatment need surgery.
C. Surgery for persistent esotropia that does not respond to prisms: Surgery
should be planned according to measurements found and not on the previous
surgery performed.
D.Botulinum toxin for persistent consecutive esotropia present after 1 month:
Persistent consecutive esotropia in a patient with good fusional potential and
normal ductions may be treated with an injection of botulinum toxin into one
medial rectus muscle.
Treatment for Recurrence of Intermittent Exotropia
If the exotropia has recurred, base-in prisms with minus lenses can be used to
maintain fusion as far as possible. Base-in prisms that correct the distant deviation
fully along with -1D Sph lenses to stimulate some accommodative convergence
are to be used. In myopic patients an addition of –1D Sph is given. If the angle of
deviation is less than 20 prism dioptres, this treatment may be continued for years.
This method is curative for some patients. More surgery should be considered
after a few months if the patient is under the age of 8 years, if this treatment fails.
In older patients surgery is done only for cosmetic purpose. In case of divergence
excess, recession of the lateral recti or if needed resection medial recti are in-
cluded. If deviations are the same for both near and distance, resection of both the
medial recti are done.
Criteria for Cure of Intermittent Exotropia
These criteria should be maintained for at least 1 year after treatment.
1. Absence of manifest tropia in any position of gaze or at any distance.
2. Absence of winking or closing of one eye in bright light.
3. Stereopsis of 60 seconds of arc or better using the Titmus test at near fixation.
4. Must have convergent or divergent fusional amlitude and and experience diplo-
pia when these are exceeded.
Exotropia 73

5. Near point of convergence better than 8cm.


6. Central fusion using a synoptophore and subjective a visual angle less than 5
degrees.
6
Convergence Weakness and Convergerce Weakness
Convergence weakness may cause intermittent exotropia where the deviation at
near is greater than the deviation for the distance. In convergence paralysis there
is an exotropia of 25 to 30 prism dioptres at near with straight eyes in the distance.

III. CONSECUTIVE CONSTANT EXOTROPIA


(FOLLOWING AN ESOTROPIA)
Treatment
1. If there is good fusion potential, an esotropia is treated by surgically overcor-
recting the deviation up to 10 prism dioptres. This exotropia is usually corrected
if adduction is normal. Sometimes it may take a month or so to recover. It is
better to wait for 2 months in a patient older than 4 years before a decision for
surgery is taken. During this period, active duction and convergence exercises
are advised.
2. If there is good fusion potential in a patient under the age of 2 years and con-
stant deviation of more than 10 prism dioptres, surgery should be done to realign
the eyes to within 10 prism dioptres of deviation to allow the patient to develop
peripheral fusion.
3. In patients under 6 years of age without any fusion potential, particularly in
patients with congenital esotropia with postoperative correction, cosmetic sur-
gery may be done after at least 2 months following the previous surgery.
4. In visually adult patients without any fusion potential, who develop consecutive
exotropia over the age of 8 years and experience diplopia, surgery may be done
to restore esotropia and to eliminate the diplopia.
5. If an unexpectedly large angle (70 prism dioptres) exotropia follows the surgery
for esotropia, there is the most likely possibility of a slipped muscle (medial
rectus).

IV. SECONDARY EXOTROPIA


This is usually defined as an exotropia secondary to loss of fixation in an eye from
an organic cause e.g., in cataract, organic amblyopia from a retinal lesion like
retinoblastoma.
Treatment
The fundus should be examined to detect treatable or organic causes of loss of
fixation. Ductions, versions and full motility testing should be done along with the
sensory evaluation. If there is no foveal pathology or retinoblastoma, the angle
kappa of the fixing eye should be assessed. Surgery should be aimed to make the
patient small angle esotropic without diplopia. An adjustable suture technique should
be used in an older patient.
7
Esotropia

Esotropia may be of two types—Congenital esotropia syndrome and acquired


esotropia.

I. CONGENITAL (OR INFANTILE) ESOTROPIA SYNDROME


The prevalence is 0.1 per cent of newborns.
This strabismus syndrome is not present at birth but usually develops at about
2 to 3 months of age. It never develops later than 6 months of age. As it is likely
that the defect that results in the esotropia at 2 to 3 months of age is present at
birth, this condition is referred as congenital esotropia. However, it may be
cosmetically unnoticeable up to 18 months of age when in response to more
accommodation the esotropia becomes more prominent. Because of its early onset
that compromises the binocular vision, which may be irreversible if not timely
treated, this entity is of special importance.
Etiology
It appears to be multifactorial, some of the factors may be heritable and some
anomaly in the developmental process in the first 4 months are possibly respon-
sible. Following factors have been considered:
I. Innervational disturbance in the form of an imbalance between tonic conver-
gence and divergence.
II. Role of accommodation: An accommodative element may be responsible par-
tially or wholly in some cases and in some cases an accommodative element
may be super-added over the basic deviation at the age of 2 to 3 years.
General Features
1. Large angle esotropia (both for near and distance): Usually more than 35
prism dioptres. The angle of deviation is usually stable except in few cases
having an accommodative element in the etiology.
2. Deficient abduction: Many of these patients cross-fixate and only change fixa-
tion and never use abduction. It is sufficient if the patient can be shown to
abduct each eye just passing midline. It is very rare for someone to be born with
a bilateral 6th nerve palsy and unilateral 6th nerve palsy due to birth trauma or
any other cause recovers before 6 months of age.
3. Any or all of the following
a. Cross-fixation: These patients, if alternate, generally mature with 6/6 vision
in each eye, alternating suppression and no fusion; they may be amblyopic if
Esotropia 75

they do not alternate—this amblyopia is usually in the 6/9 to 6/18 range and

b.
usually respond well to occlusion.
Dissociated vertical deviation (DVD): Most cases are bilateral. It may not
7
become obvious before 18 months of age. It is characterized by an eye
floating up and excyclorotation under cover. When the cover is removed, the
eye comes down and rotates inwards, returning to the same position as
before dissociation. It does not cause a “V” syndrome as seen with overaction
of inferior oblique muscles.
c. Inferior oblique overaction with “V” pattern: It should be diagnosed only
in presence of a “V” pattern. It is characterized by a decrease of esotropia
in upgaze by at least 10 prism dioptres. The nonfixing eye will be hypotropic
and will come up to fix when the fixing eye is covered (Treatment: Myec-
tomy of both inferior obliques).
d. Unilateral superior oblique paresis: It may be associated with overaction
of direct antagonist inferior oblique. There is hypertropia of the affected eye
when fixing with the unaffected eye and a hypotropia of the unaffected eye
when fixing with paretic superior oblique muscle. The Bielschowsky test is
(+)ve.
e. Nystagmus: Present in 50 per cent cases (micronystagmus). It is elicited
when one eye is occluded (latent feature) and mostly when the preferred
eye is occluded forcing the non-preferred eye to fix. Patient finds a null point
for better visual acuity and this explains the head tilt or turn in patient with
congenital esotropia syndrome.
f. Convergence blocked nystagmus (CBN): Both the eyes may be turned
in(esotropic) if this feature is present. Here, convergence is used to dampen
nystagmus. It simulates bilateral 6th nerve palsy. The esotropia is present
even if one eye is occluded. Consequently, the patient adopts a face turn so
that one esotropic eye can see straight ahead. Nystagmus is visible when
the patient is made to straighten or abduct the eye.
g. Asymmetrical optokinetic nystagmus (AOKN): It is present because there
is no fusion. Tracking of objects temporal to nasal is smooth while tracking
nasal to temporal is cogwheel.
h. Rarely, refractive error more than + 2.00D Sph is present. Glasses are sel-
dom indicated before surgery. More than 50 per cent of the patients require
glasses at some stages of treatment.
Common Variants
a. Ciancia syndrome: It is characterized by the presence of early onset esotropia
along with bilateral limitation of abduction with manifest-latent jerk nystagmus
(fast phase in the direction of the fixing eye), increasing in abduction and
decreasing in adduction. This causes a face turn towards the side of the fixing
eye, that is the fixing eye remains in adduction (as the null point is in adduction).
76 Strabismus

b. Lang’s syndrome: It is characterized by the presence of early onset esotropia


7 with dissociated vertical deviation (DVD), nystagmus and excyclodeviation of
the non-fixing eye. This is associated with torticollis.
Natural Course of Untreated Congenital Esotropia
The eyes turn in constantly about 2 months afterbirth and both the eyes may be
esotropic and abduction is limited in each eye. Cross-fixation is typical and fixation
is alternated only on side gaze. By the age of 2 years, the cross fixating feature
changes so that the child becomes a spontaneous alternator with one eye straight
and the other eye turned in. Abduction of each eye improves and finally becomes
normal. Nystagmus and DVD may become obvious by 2 to 4 years of age. Fusion
never develops. The overall field of vision is smaller than if the eyes were straight-
ened. Abnormal head tilt or face turn to improve the visual acuity is present with
nystagmus.
Differential Diagnosis
a. Broad epicanthal fold: Esotropia on near fixation is found particularly after
cycloplegia with 1 per cent cyclopentolate.
b. Unilateral 6th nerve palsy: It may occur at birth due to birth trauma.Recovery
is usually complete by 3 months of age.
c. Accommodative esotropia: It may occur within the first 6 months and is asso-
ciated with high hypermetropia. Eye straighten completely when refractive error
is corrected.
d. High AC/A ratio: Here the eyes are straight for distance and marked esotropia
for near when accommodating.
e. Duane’s retraction syndrome type I: It is differentiated by retraction, changes
in palpebral aperture and associated upshoots or downshoots on adduction.
f. Down’s syndrome
g. Mobius syndrome
h. Cerebral palsy
i. Albinism
j. Nystagmus blockage syndrome
Diagnostic Tests
a. Bruckner Test: The red reflexes in each eye are compared.
b. Hirschberg Test
c. Krimsky Test (or prism and cross-cover test): The angle of deviation is mea-
sured for distance and near fixation and n up and down gaze.
d. Bielschowsky head tilt test and Park’s three step test: This test is done if a
vertical strabismus is present together with the esotropia.
e. Fixation pattern
f. Cross-fixation: It should be seen whether the patient can hold fixation with
each eye.

http://ophthalmologyebooks.com
Esotropia 77

g. Eye movements
h. Refraction
i. Fundus examination: Indirect ophthalmology should be done using +20 dioptre
7
lens. Direct ophthalmoscope is used if disc disease (optic nerve dysplasia or
cupping) is present.
j. Occlusion of one eye should be done to elicit nystagmus in abnormal head posi-
tion.
Treatment of Congenital Esotropia
The aim is to obtain a monofixation syndrome characterized by suppression of one
fovea and the surrounding macula and fusion using the paramacular peripheral
areas. Some gross stereopsis may also be present. Stereoacuity is usually within
100 seconds of arc with the Titmus test at near and the fusional amplitudes are
between a few prism dioptres to over 30. In order to obtain this monofixation
syndrome, the eyes should be straightened to within 10 prism dioptres before the
age of 2 years and maintain this alignment during visual immaturity. About 50 per
cent of the patients with this treatment attain monofixation syndrome and the rest
end up without fusion or stereopsis. If peripheral fusion is obtained then there is
more stable alignment and cosmesis. Gross stereopsis may be of some help in
judging depth. If peripheral fusion is present the patients have a near-normal bin-
ocular visual field, particularly in cases with a small angle esotropia.
1. Glasses: In congenital esotropia of 50 prism dioptres or more unless the refrac-
tive error under cycloplegia (with 1 per cent atropine eye ointment 3 times/day
for 3 days) is more than 3D Sph, glasses are not prescribed. Postoperatively if
there is some recurrence of esotropia any refractive error should be corrected.
50 per cent of the patients with congenital esotropia will need glasses for treat-
ment before 8 years of age.
2. Occlusion: In congenital esotropia without any possibility of fusion, occlusion
should be attempted with the target to have a dominant eye with better vision so
that the patient does not become a true alternator to avoid spontaneously chang-
ing fixations and noticing the environment to change positions. Further, if DVD
is present, it will only become manifest in the non-dominant eye, which will be of
help during treatment.
3. Surgery: Children with congenital esotropia should be operated on between 6
and 18 months of age. Surgery is not done before 6 months of age as occasion-
ally some patients improve (presumably in bilateral 6th nerve paresis, which
have recovered).
Surgery is done between 6 to 12 months of age except in cases where there is
a. a variable angle of deviation,
b. a refractive error more than +3D Sph, and
c. any condition which may make a general anesthesia risky at a young age. In
these patients surgery should be done between 18 to 24 months of age. Fusion
is less likely to develop if surgery is delayed beyond this period. Fusion rarely
develops if treatment is delayed up to 4 years of age.
78 Strabismus

Surgery should aim to reduce the deviation to less than 10 prism dioptres. A
7 recession up to 7mm of both the medial rectus muscles is preferred than a reces-
sion of medial rectus and resection of lateral rectus because of frequent presence
of nystagmus block features. However, if more correction is needed, resection of
one or both lateral rectus muscles may be done and maximum correction should be
attempted on one muscle before proceeding to another.
Treatment of Special Features
a. Nystagmus block syndrome: As these patients usually have an esotropia of 80
to 90 prism dioptres, a large amount of surgery, on three muscles, should be
undertaken. A 7 mm recession of both the medial recti and a 10 mm resection
of one lateral rectus is required. This amount of surgery will correct 4 prism
dioptres of correction per millimetre. If a residual esotropia more than 15 prism
dioptres persists for more than 2 months postoperatively, further surgery on the
unoperated lateral rectus is needed. If still more effect is needed, a repeat
resection of the previously resected lateral rectus should be done.
b. Inferior oblique overaction: A myectomy of both inferior oblique muscles
should be done in cases of bilateral inferior oblique overaction. In cases of
unilateral superior oblique palsy with a positive Bielschowsky test and overact-
ing ipsilateral inferior oblique, surgery should be done to that inferior oblique
muscle alone. If a “V” pattern is absent, weakening of inferior oblique muscles
should not be done as the deviation is almost certainly due to DVD.
c. DVD: Treatment of DVD should be deferred, if cosmesis is acceptable, as this
condition may slightly improve as the patient grows.
Patients with a dominant eye ,who do not alternate, only present with DVD of
the non-fixing eye.A recession of the superior rectus or a resection of the inferior
rectus of the non-fixing eye should be done in these cases. Recession of superior
rectus muscle should not exceed 8 mm because the resultant weakness may cause
overaction of the contralateral yoke inferior oblique muscle.
Patients with alternate fixation have equal visual acuity and alternate fixation at
times. These patients usually complain of DVD in one eye only and if this eye is
operated on and the deviation is fully corrected,it usually precipitates a same amount
of deviation in the other eye.For this reason, patients with alternate fixation and
unilateral DVD should not be fully corrected and only three quarters of the devia-
tion should be corrected ,which leaves the patient with a slight residual DVD on
that side and will not provoke DVD in the other eye. Inferior rectus resection of
appropriate amount brings the lid up to help to disguise the remaining uncorrected
DVD.
Follow up: The patient should be checked at 3 months intervals or more frequently
if occlusion is included. At the age of 4 years, tests for fusion and stereopsis can be
performed. The glass correction should be given at this stage, if required. Occlusion
should be tried to provide near equal vision in both the eyes. However, in patients
who do not have fusion, occlusion should not be given as it may encourage alteration,
Esotropia 79

which is a disadvantage in treating DVD. Further surgery is done in patients with


more than 15 prism dioptres deviation with full optical correction and in whom
amblyopic treatment has resulted in equal vision.
7
Strabismus in patients with cerebral palsy: About 50 per cent children with
cerebral palsy have the congenital strabismus syndrome, particularly in patients
having hypertonicity and spasticity ,due to variable tone in eye muscles. Any physi-
cal or emotional stress makes the condition worse. Bizarre variability in the stra-
bismus pattern may be noted. These patients should be treated conservatively and
surgery may be done only for cosmetic reasons after 5 or 6 years of age.
Role of botulinum injections: Botulinum injections as an alternative to surgery
have been tried. In infants the weakening effect is supposed to be prolonged due to
the immaturity of the infant’s myofibrils. A paresis of the medial rectus triggers a
spread of comitance favourably aligning the eyes.

II. ACQUIRED ESOTROPIA


Classification of Acquired Esotropia
Comitant
Primary
A. Constant
a. Accommodative
b. Non accommodative
B. Intermittent
a. Accommodative
1. Fully Accommodative
2. Convergence excess
b. Non accommodative
1. Relating to distance
i. Near esotropia
ii. Distant esotropia
iii. Non-specific
2. Relating to time (cyclic)
i. Regular
ii. Irregular
C. Secondary
D. Consecutive
Incomitant or Paralytic
1. Neurogenic (Nerve paralysis)
2. Myogenic (Myaesthenia)
Children who are born with straight eye and develop an esotropia (usually
between 18 months to 3 years of age) most of the times have a hypermetropic
error and a family history.As the child is still visually immature, active inhibition of
the deviating eye at the cortical level, will occur resulting in a deterioration in visual
acuity, fusion and stereopsis.
80 Strabismus

If the child is below 2 years of age and the deviation is present for more than
7 2 months without any treatment, normal central bifoveal fusion is very unlikely to
be regained. If immediate treatment is undertaken within a few days of the onset
of the esotropia, full care with perfect bifoveal fusion is likely. If the treatment is
delayed for several months, a monofixation syndrome with one fovea suppressed
is likely to occur and if it is neglected for years, eccentric fixation with gross
deterioration of vision may occur.
In the children below 3 years of age, the interval between the onset of constant
esotropia and its treatment will determine the visual and fusional outcome. Devel-
opment of amblyopia should be prevented by patching and loss of fusion should be
prevented by glass, surgery or both. If glasses fail to correct the deviation within a
short period(few days), an immediate return to occlusion until surgery can be done,
if essential.Surgery should be done to overcorrect by 10 prism dioptres, the resi-
dual distance deviation present after wearing the glasses for 2 days.
Suppression is only present when both eyes are open and suppression replaces
normal fusion and becomes entrenched in visually immature patients. For this rea-
son, occlusion of one eye must be maintained at all times. If the patient complains
of diplopia, then no suppression is present there. Total occlusion of one eye or the
other must be continued until glasses or surgery eliminates the deviation. Occlusion
amblyopia is prevented in this regimen by changing the occlusion from one eye to
the other as necessary. When the glasses alone do not completely correct the
deviation, persisting with glasses without occlusion results in entrenchment of sup-
pression.
Etiology of Esotropia
A.Refractive errors
1. Hypermetropia: It may be the sole cause of esotropia (Fully accommoda-
tive esotropia) or it may add accommodative elements to the pre-existing
deviation (Partially accommodative esotropia).
2. Congenital myopia: In moderately high myopia, the patient cannot see for
distance and never relaxes the visual axes leading to esotropia.
3. Anisometropia: It acts as a dissociating factor where one eye receives less
clear image. It may cause microtropia.
B. Anatomical factors
1. Defect in extraocular muscles, fascia and check ligaments
2. Narrow interpupillary distance, enophthalmic eyes.
C. Abnormalities of fusion mechanism: The underlying defect(poor/low fusion
range) may result in manifest squint following precipitating factors e.g., fever,
emotional stress, mental retardation, occlusion of one eye etc.
D.AC/A ratio: Abnormally high AC/A ratio causes convergence excess type of
esotropia.
E. Heredity
F. Age factor: Young patient have predisposition to esodeviation up to adolescence
due to strong accommodation and convergence mechanism.
Esotropia 81

Fully Accommodative Esotropia


In a fully accommodative esotropia, wearing the full optical correction , neutralizes 7
the hypermetropia and straighten the eyes completely.
Etiology
The reason of this condition is overconvergence associated with the extra accom-
modation necessary to overcome a hypermetropic refractive error. The condition
appears when the child actively starts accommodation, usually between 1 and 3
years of age. However, this condition may occur within the first 6 months of life or
as late as 5 to 6 years of age.
Characteristics
1. It is associated with moderate uncorrected hypermetropia, which forces the
child to exert excessive accommodation to clear the retinal image, thus evoking
excessive convergence. This in itself does not cause strabismus, if the ampli-
tude of fusional divergence is sufficient to cope with the increased convergence
tonus. When the motor fusion is inadequate, the exodeviation becomes manifest.
2. Binocular single vision is present both for near and distance, when hypermetro-
pia is fully corrected.
3. Ocular deviation is usually variable and larger at near than at distance fixation.
The evolution is a gradual one and a phase of intermittent strabismus is present.
4. Amblyopia (if present) will be slight.
5. Onset is usually between 2 to 3 years of age.
Management
1. Atropine cycloplegic refraction and ordering of full correction should be done.
The patient should be followed every week, until it is certain that the deviation is
controlled by glasses. The constant wearing of glasses at all times is an essen-
tial part of treatment. When hypermetropia is less than 3.5D Sph in children
below 3 years of age, there is a possibility of being able to give up glasses at a
later age. When the hypermetropia exceeds 4D Sph or if there is marked astig-
matism, glasses will be necessary for clear vision and also for keeping the eyes
correctly aligned.
2. Correction of amblyopia to equalize the visual acuity, if necessary. Monitor the
fixation and vision to see that the amblyopia does not return.
3. Measurement of deviation for distance and near with glasses on, using an
accommodative target in cases there is a high AC/A ratio.
4. Orthoptic treatment: Patients over 8 years of age with refractive error less
than + 3.5D Sph and an esotropia which is fully controlled with glasses need
orthoptic exercise.
Aim: To restore binocularity without glasses. The patients fall into two groups
depending on the degree of hypermetropia

http://ophthalmologyebooks.com
82 Strabismus

a. If the error is less than +3.50D Sph with 1Dcyl of astigmatism, ultimate discard
7 of the spectacle may be possible.
b. If the error is more than +3.50D Sph with 1Dcyl of astigmatism, the patient will
never be able to achieve adequate visual acuity without glasses but will appre-
ciate cosmetic advantage of “control” of deviation.
Method
a. Diplopia recognition without glasses when deviation manifest
b. Control of esotropia by fusion of diplopic images
c. Improvement of binocular visual acuity
d. Orthoptic treatment may be combined with gradual reduction of hypermetropic
correction.
Partially Accommodative Esotropia
Here, the esotropia is reduced when the glasses are worn but is not completely
eliminated at either distance or near.
It is unlikely that a complete cure with bifoveal fusion will be achieved in these
patients unless
a. they are diagnosed and treated soonafter the onset i.e., within a few weeks,
b. they are patched until they are realigned so that both suppression and am-
blyopia do not develop and
c. the onset is at a later age i.e., over 3 years so that fusion and stereopsis have
developed and are strongly established.
Features
i.
Esotropia for all distances with and without hypermetropic correction.
ii.
It is usually unilateral particularly in anisometropia.
iii.
Amblyopia is common.
iv.Normal binocular function is found in only a minority of cases. Abnormal reti-
nal correspondence may develop or there may be suppression and absence of
binocular single vision (BSV).
v. Commonly unilateral or sometimes bilateral inferior oblique overactions may
be present.
Management
i. Refraction and examination of the fundus should be done.
ii. Amblyopia should be treated first. Much occlusion is often required to improve
and maintain the visual acuity. Glass should be prescribed then. The patient
should then return for re-examination. In a partially accommodative esotropia
there will be a residual angle.
iii. The deviation should then be measured with the glasses on for distance and
near fixation as well as side and up gaze.
iv. Surgery
Esotropia 83

Cases may be
a. Functional: Surgery is aimed to restore BSV with glasses. The patient be-
comes fully accommodative. The common procedure is unilateral medial rectus
7
recession with lateral rectus resection of the squinting eye.
b. Cosmetic: Surgery is aimed for cosmetic purposes.
Choice of surgery: Attempt should be made to avoid consecutive exotropia.
Principle
i. In high hypermetropia (+4D Sph or more): There is greater risk of consecu-
tive exotropia. The aim should be to undercorrect particularly in presence of
intractable amblyopia.
ii. In small and moderate hypermetropia, where discard of glass is possible:
The size of deviation without the glass should be considered.
iii. In moderate hypermetropia where glass will be needed: The deviation which
persists with glasses should be considered.
iv. Vertical element may require surgery.
v. Possibility of postoperative diplopia must be considered in older children or
adults.
Occlusion should be resumed to prevent the eyes from being open together
until after operation, when,it is expected the eyes will be straight with glasses on. If
monofixation syndrome is the end result, it should be preserved instead of making
an attempt to convert it into a bifoveal fixation by surgery or prisms.
Nonaccommodative Esotropia
These patients do not have any accommodative factor and the esotropia is not
reduced by wearing the glasses.
Treatment
1. Occlusion 2. Surgery.
Esotropia with a High AC/A Ratio (Convergence Excess)
This condition is characterized by an overconvergence associated with accommo-
dation. The esotropia at near is more than 20 prism dioptres greater than the distance
measurement when the full optical correction is worn and the patient is fixing an
accommodative target. The onset may be from a very early age even before 1
year.
Features
1. BSV is present for distance fixation but esotropia is present for near fixation on
accommodation.
2. This is associated with a high AC/A ratio.
AC/A ratio: This is the relationship between accommodative convergence measured
in prism dioptres and accommodation measured in dioptre spheres. For each dioptre
of accommodation exerted there is an accompanying amount of accommodative
84 Strabismus

convergence. The normal AC/A ratio varies between 3 to 5:1. When the AC/A
7 ratio is low less accommodative convergence is exerted resulting in near
exodeviation or distance esodeviation. When the AC/A ratio is high more
accommodative convergence is exerted resulting in convergence excess eso-
deviation or distance simulated exodeviation.
Measurement of AC/A Ratio
1. Heterophoria method: Phoria should be determined by prism and alternate
cover test at optical infinity and one-third metre distances. Accommodation
should be controlled acuity should be corrected to 6/9 with least plus lens.
AC/A = Interpupillary distance (cm) + (Distance phoria – Near phoria)/
Near fixation distance in dioptres of vergence.
2. Gradient method: Phoria should be determined by prism and alternative cover
test at a fixed distance, generally one-third metre. Accommodation should be
controlled and acuity should be corrected to 6/9 with least plus lens. Lens power
held before eyes should be varied and alignment remeasured.
AC/A = (Deviation with added lens minus deviation without the added lens)/
Power of lenses in dioptres.
3. This may occur with emmetropia or hypermetropia: The etiology is closely
related with an abnormal synkinesis between accommodation and accommo-
dative convergence—the efforts to accommodate elicits an abnormally high
accommodative convergence response. If motor fusion can cope with increased
convergence tonus at near fixation, an esotropia results and if it is insufficient,
non-refractive accommodative esotropia will manifest.
Management
1. Atropine refraction: Full correction of hypermetropia, where present.
2. Equalise visual acuity by treating amblyopia.
3. Measurement of deviation for distance with the full optical correction
4. General plan of management:
a. In patients with fusion potential:
i. If esotropia is less than 10 prism dioptres for distance with full optical
correction: These patients has a chance of developing fusion. They should
be treated with a bifocal add of equal strength in front of each eye. If this
proves difficult, try 1 per cent atropine eye ointment to encourage use of
bifocals.
ii. If esotropia is more than 10 prism dioptres for distance with full
optical correction: These patients virtually has no chance of developing
any fusion unless the deviation is reduced to below 10 prism dioptres. If
the patient is visually immature, surgical correction should be done to
reduce the deviation to within 10 prism dioptres. Medial rectus recession
is preferred as this tends to decrease the high AC/A ratio. Following this,
bifocals may be needed.
Esotropia 85

b. In patients without fusion potential:


Where esotropia for the distance is present from an early age—bifocal glasses
are not helpful.
7
5. Following methods may be tried:
a. Miotics
b. Orthoptic exercises.
Bifocals
The patients having a high AC/A ratio will have a better alignment for at near with
the bifocals and this will improve the overall sensory and motor outcome when the
child becomes a visual adult.
The bifocals should be +3.00D Sph executive flat topped type fitted very high so
that the bifocal segments bisect the pupils or are even slightly higher than this.
Bifocal therapy should be continued at least upto the age of 8 years. After 8
years, the addition should be gradually reduced. If it is not done, the patient may
experience double vision at near without the bifocals and still have a significantly
high AC/A ratio. If the patient do not experience diplopia when the eyes
overconverge, the bifocals can be removed at 10 years of age.
The suitable cases are:
a. small distance deviation,
b. residual deviation persisting after surgery,
c. unwilling to surgery and
d. older patients i.e., over 4 years of age.
The disadvantages are:
a. spectacles must be well fitted to ensure correct use of the reading addition
and
b. some authors suggest that this may lead to problems of weak accommodation
in young patients.
If a child do not wear bifocal glasses, 1 per cent Atropine eye drop to both the
eyes 3 times a week can be used so that the bifocal segments provide some visual
advantage for near. Once this has encouraged the habit of wearing the bifocals,
the drops can be withdrawn.
Miotics
Miotics have been used to reduce the high AC/A ratio. As the treatment is needed
for at least 6 to 8 years, use of miotics is not very popular. Miotics act from peripheral
level.
Drugs
a. Phospholine iodide (0.06% to 0.125%) is used initially at higher strength once
daily in each eye at night. Patient is reviewed at fortnightly interval and checked
for side effects. Trial is given for one month. If there is no improvement it is
discontinued. If there is improvement (BSV for near), treatment is continued
for 6 months and then gradually discontinued reducing the strength and fre-
quency of instillation.
86 Strabismus

b. Pilocarpine (4%) is less effective. Suitable cases are:


7 i. small deviation,
ii. respond better if aware of diplopia when manifest deviation occurs before
treatment commences,
iii. may be used for residual deviations postoperatively and
iv. can be used in younger age group instead of bifocals.
The disadvantages are:
a. Side effects- headache, stomach cramp, hallucination- rare,
b. Anaesthetic risk,
c. Pain on instillation of drops, risk of cyst formation and
d. Hypermetropes may experience reduced distance visual acuity and wish
to remove glasses.
Orthoptic Exercises
This requires excellent co-operation and is suitable only for older children with
small deviations.
The advantages are:
a. awarwness of diplopia when deviation manifest,
b. control of esotropia by fusion of diplopic images involving relaxation of accom-
modation and
c. improvement of near visual acuity.
The disadvantage is that it rarely achieve result alone and is best combined with
other methods.
Surgery
It may be indicated in many cases. Bilateral medial rectus recessions are generally
performed. Occassionally it is combined with bilateral FADEN on medial recti. A
5 mm recession of both medial recti is indicated in patients where there is no
deviation in the distance and 20 prism dioptres or more esotropia for near. Surgery
should only be considered if the patient has good central fusion or good peripheral
fusion.
Esotropia without Fusional Potential
If the child is teased, surgical correction is advised at an early age for cosmetic
reasons. Otherwise, waiting until mid-to-late adolescence is advisable when ad-
justable suture surgery could be performed for better results. The aim of surgery
should be to leave an esotropia of 5 to 10 prism dioptres as there is a tendency for
the eyes to diverge as the patients grow older. If a positive angle kappa gives a
divergent appearance, the eyes can be left with an esotropia of 10 prism dioptres
or more.
Other Esotropias
1. Emotional esotropia: A child under emotional stress may suddenly exhibit an
increase in the esotropia and even give rise to diplopia. The effects of the emotions

http://ophthalmologyebooks.com
Esotropia 87

is particular likely to be exhibited in patients with a high AC/A ratio, but all types
of esotropia may be increased by emotions.
2. Voluntary produced esotropia: A visual adult can see asymmetrical conver-
7
gence to create a similar to acquired strabismus of 30 prism dioptres. This type
of esotropia is diagnosed by-
i. miosis of the pupil (with convergence),
ii. measuring the deviations in side gaze (it is difficult to maintain esotropia in
side gaze voluntarily), and
iii. dynamic retinoscopy will show an accommodative myopic change in refrac-
tion.
3. Esotropia following recovered 6th nerve palsy: Following 6th nerve palsy,
there will be marked esotropia of the ipsilateral eye. Occlusion of the unaffected
eye should be done to prevent contracture of the medial rectus of the paretic
eye. Even, if the lateral rectus may restore full movement of the involved eye, a
residual esotropia of 30 to 40 prism dioptres frequently remains. This residual
esotropia may be concomitant (same amount of deviation in all the positions). In
a visual adult, good fusion potential may be best achieved by adjustable suture
surgery. Botulinum toxin injection to the medial rectus of the affected eye may
be helpful in preventing this condition.
4. Acute esotropia: Sudden appearance of diplopia and strabismus (30 to 60 prism
dioptres), which remains the same in all directions of gaze (Hess chart showing
exactly comparable squares) and with fusion (when the angle of deviation is
eliminated with prisms or synoptophores). If this condition could not be
explained on the basis of decompensated esophoria, if no significant
hypermetropia is present and there is no positive family history, a complete
neurological examination and CT scan is necessary, particularly if the patient is
over 4 years of age, as this type of strabismus may be caused by a supranuclear
acquired lesion, particularly neoplasms.
5. Cyclic esotropia: Esotropia (of about 30 to 40 prism dioptres) which is present
at regular or irregular intervals, BSV being present at times, is often associated
with psychogenic disturbances. Cyclic strabismus may be of any type. The com-
monest type is cyclic esotropia. The cycles may vary in length but are usually of
24 hours.If left untreated, it tends to become constant within 6 months.
Management: i. Though the patients are usually emmetropic, full correction of
the refractive error should be done,whenever necessary, and
ii. If the deviation becomes constant surgical correction is necessary (the amount
of surgery is calculated on the esotropia present during the strabismic cycle).
6. Occlusion esotropia: Here, esotropia may be precipitated following the intrruption
of binocular vision by occlusion of one eye. This is more likely if there is uncor-
rected hypermetropia and positive family history. This may occur during the
treatment of anisometropic amblyopia, unilateral corneal ulcer, postoperative
ptosis or occlusion after lid surgery or a swollen lid from trauma.
Management: Correction of refractive error. Surgery may be necessary.
88 Strabismus

7. Secondary esotropia: It may occur following loss of fixation due to an organic


7 lesion at the fovea. Fovea should be carefully examined to exclude lesions such
as retinoblastoma, toxoplasmosis or foveal damage from trauma.
8. Esotropia associated with divergence paralysis: This rare condition is char-
acterized by an esotropia of 10 to 20 prism dioptres on distance fixation, fusion
at about 1 metre with orthophoria and normal ductions and versions. The esotro-
pia for distance remains the same for all the positions of gaze at distance fixa-
tion. The patient has fusion for near with convergence fusional amplitude but no
fusional divergence. This condition may be congenital or acquired. There is a
possibility that ‘divergence cells’ at midbrain, just rostral to the 3rd nerve nucleus,
may be affected by a small lesion. In addition to head trauma, the condition like
influenza, increased intracranial tension, encephalitis, multiple sclerosis, vascu-
lar lesions or neoplasms may be the causative factor. Some patients get better
spontaneously when the condition is likely to be due to divergence insufficiency.
9. Consecutive esotropia: It may occur following overcorrection of primary
exotropia. It may be deliberate to guard against re-divergence.
Where consecutive esotropia is undesirable following measures may be
considered:
a. possible change in glasses e.g., temporary discard of minus lenses, or increase
in any plus correction,
b. base-out prisms,
c. if deviation occurring at near, miotics may help and
d. may require further surgery if diplopia is troublesome or cosmesis is poor.

MICROTROPIA
These are the cases of small angle squint with BSV where manifest deviation does
not exceed 10 prism dioptres. They are unilateral, cover test is often negative and
more common in esotropia but can occur in exotropia. Most of the cases have
anisometropia leading to amblyopia, abnormal retinal correspondence, a relative
scotoma on fovea or the fixation spot of the deviating eye, eccentric fixation point
on the border of the scotoma, normal or near normal peripheral fusion with ampli-
tudes and defective stereo-acuity.
They may be classified according to the cover/uncover test:
1. Microtropia detectable on cover test: Very small manifest strabismus,
2. Microtropia not detectable on cover test: Explained by parafoveal fixation (It
may be differentiated from amblyopia by examination with visuoscope to estab-
lish whether the fixation is foveal or parafoveal).
Significance
a. Microtropia will persist after any treatment for associated deviation,
b. Treatment of any amblyopia does not recover equal visual acuity and a differ-
ence of one line will persist, and
Esotropia 89

c. Its presence may mean a slightly less response to treatment for any associated
condition. 7
Therapy
1. In older child or adult: No therapy is required. Such patients have comfort-
able and nearly normal BSV with good peripheral fusional amplitudes, and
2. In young children up to the age of 5 years: Attempts should be made to
correct amblyopia. Occlusion of fixating eye may change fixation of the amblyopic
eye from parafoveal to central and steady behaviour, vision may become nor-
mal, ARC may become NRC and stereo-acuity may improve.
8
A, V, Y, X Pattern Strabismus

The letters A,V,Y and X are used to describe a changing pattern of deviations
noted in horizontal strabismus when looking up and looking down.

TYPES
1. A-Pattern
This is a condition in which there is relative convergence on up gaze and relative
divergence on down gaze. There must be a minimum of 10 prism dioptres differ-
ence between up gaze and down gaze.
There may be A-Exotropia or A-Esotropia.
A-Exotropia:
a. Medial rectus underaction results in less adduction on depression.
b. High lateral rectus insertions result in more abduction on depression.
c. Inferior rectus underaction results in less adduction on depression. The superior
rectus will overact, resulting in more adduction on elevation.
d. Superior oblique sagittalisation results in superior oblique overaction.
e. Mongoloid facial features.
A-Esotropia:
a. Lateral rectus underaction results in less abduction on elevation.
b. Low medial rectus insertions result in more adduction on elevation due to differ-
ent muscle position and increased muscle tension.
c. Inferior oblique underaction results in less abduction on elevation. The superior
oblique will overact, resulting in more abduction on depression.
d. Superior oblique sagittalisation results in superior oblique overaction.
e. Mongoloid facial features.
2. V-Pattern
This is a condition in which there is relative divergence on up gaze and relative
convergence on down gaze. There must be a minimum of 15 prism dioptres differ-
ence between up gaze and down gaze.
There may be V-Exotropia or V-Esotropia.
V-Exotropia:
a. Medial rectus overaction results in more adduction on depression.
b. Low lateral rectus insertions result in more abduction on elevation.
A, V, Y, X Pattern Strabismus 91

c. Superior rectus underaction results in less adduction on elevation. The inferior


rectus will overact, resulting in more adduction on depression.
d. Inferior oblique sagittalisation results in inferior oblique overaction.
8
e. Antimongoloid facial features.
V-Esotropia:
a. Lateral rectus oveeraction results in more abduction on elevation.
b. High medial rectus insertions result in more adduction on depression.
c. Superior oblique underaction results in less abduction on depression. The infe-
rior oblique will overact, resulting in more abduction on elevation.
d. Inferior oblique sagittalisation results in inferior oblique overaction.
e. Antimongoloid facial features.

Fig. 8.1: A: A-esotropia; B: A-exotropia; C: V-esotropia; D: V-exotropia

http://ophthalmologyebooks.com
92 Strabismus

3. X-Pattern
8 There is relative divergence on both up and down gaze.
4. Y-Pattern
Eyes will go out in up gaze but maintain approximately straight alignment in the
primary and down gaze positions. This is a variation of the V-pattern.
5. Inverted Y–Pattern
There is relative divergence on down gaze with no significant difference between
the primary position and up gaze.
6. Diamond Pattern
There is relative convergence on both up and down gaze.
There are various theories relating to the etiology of A and V patterns. There
may be imbalance of vertically acting extraocular muscles, sagittalisation or struc-
tural anomalies. Sagittalisation occurs where there is a small angle between the
inferior oblique, superior oblique or both, and the sagittal plane. The muscle is
closer to the sagittal axis. This results in decreased torsional power and the over-
acts to compensate.
Diagnostic Features
1. Head posture (chin up or chin down): This may indicate the presence of
nystagmus or an A,V or X pattern. In order to fuse the patient may adopt the
preferred head position.Chin elevation is noted in A-eso and V-exo and chin
depression in A-exo and V-eso.
2. Deviation must be measured with full optical corrections and in the primary
position at 6 metres, chin down with the patient looking up as far as possible and
then with chin up looking down below the horizontal meridian at 6 metres to
simulate the reading position. The fixation object remains stationary at 6 metres
and the patient either tilts the chin about 30 degrees and 20 degrees down. The
measurements with fixation object at 6 metres are important to avoid accom-
modative influences on the deviation at the reading distance.
3. The measurements should be taken both with and without glasses.
4. If a V-pattern is present, the versions should be checked for over action of both
the inferior obliques (due to weakness of both superior oblique muscles).
5. If an A-pattern is present, the versions must be checked for over action of both
superior oblique muscles (associated with weakness of both inferior oblique
muscles).
6. In intermittent exotropia, the appearance of an A or V pattern may be due to the
fact that the patient controls the deviation more easily in up gaze or down gaze.
Indications for Treatment
1. To improve head posture (chin up or chin down) e.g., in bilateral superior oblique
palsy.
A, V, Y, X Pattern Strabismus 93

2. To achieve fusion.
3. Cosmetic improvement in V-exotropia (with overaction of both inferior obliques).
Myectomy of both inferior obliques is indicated.
8
4. To prevent recurrence of A-exotropia without fusion. If there is an long standing
exotropia of 35 prism dioptres or more in the primary position, some contracture
of superior oblique occurs with resultant over action and an A-pattern. If an
overaction is present and the deviation increases by more than 20 prism dioptres
in down gaze, both superior obliques should be weakened by a tenotomy in
addition to the horizontal muscle surgery needed to improve the exotropia in the
primary position. If this A-pattern is not treated, recurrence of exotropia in the
primary position may occur. Horizontal rectus muscle surgery should be planned
to allow for the additional 10 prism dioptres esotropic effect in the primary
position and 20 to 30 prism dioptres esotropic effect in down gaze from the
tenotomy of both superior obliques.
Surgery for A and V Patterns

A. The V-pattern with Bilateral Inferior Oblique Overaction


Myectomy of both inferior oblique muscles is indicated a small section of the infe-
rior oblique is excised between the inferior rectus and the lateral rectus muscles.
The effects of bilateral inferior oblique myectomies on the horizontal alignment are:
a. 20 prism dioptres of esotropia in up gaze, thereby reducing an exotropia in up
gaze by 20 prism dioptres
b. No effect on the deviation in primary or down gaze positions
c. Occasionally, a small vertical strabismus may result immediately after this op-
eration and rarely persists for more than 1 or 2 weeks.
B. A-pattern with Bilateral Superior Oblique Overaction
1. Tenotomy of both superior oblique muscles: The tendon should be isolated
between the trochlea and the medial border of the superior rectus. The tendon
and the sheath should then be cut through a cautery. This produces the desired
weakening effect and does not result in a paralysis of the superior rectus muscle
because of the many fascial connections of superior oblique tendon in an around
this region. The effects of bilateral superior oblique tenotomies are:
a. The effect on horizontal alignment is to produce 0 to 10 prism dioptres of
esotropia in the primary position and 20 to 40 prism dioptres of esotropia in
down gaze relative to the amount of overaction present. This reduces an
exotropia in down gaze by 20 to 40 prism dioptres.
b. There is no effect on the horizontal alignment in up gaze.
c. Sometimes a significant vertical strabismus may result postoperatively which
disturbs a patient with fusion ability and may result in compensatory head
posture. For this reason this operation is not indicated in patients with good
fusion and horizontal muscle transposition is preferred as it is less likely to
produce problems.
94 Strabismus

Fig. 8.2: Transposition of the medical rectus muscles—up in A and down in V patterns

Fig. 8.3: Transposition of the lateral rectus muscles—down in A and up in V patterns

2. Horizontal rectus muscle transposition in absence of oblique dysfunction


Principle
a. Medial rectus muscles should be moved vertically half a tendon width in the
direction of greatest esotropia (or least exotropia) i.e., up in A and down in V
patterns.
b. The lateral rectus muscle should be moved vertically half a tendon width in the
direction of greatest exotropia (or least esotropia), i.e., up in V and down in A
patterns.
The approximate effect on pattern is reduction of pattern by about 10 to 15
prism dioptres.
c. This effect can be increased by recessing the upper or lower margin of the
appropriate transposed horizontal muscle insertion 2 mm. more in the direction
that more weakening is required, e.g., V esotropia in bilateral superior oblique
palsy.
X-Pattern
It is seen in patients having both superior and inferior oblique overaction resulting
in combination of A and V patterns. No treatment is needed as the patients are
usually fusing in the primary position. Sometimes tight lateral rectus muscles from
previous surgery or a congenital anomaly may produce an X-pattern. No treat-
ment is needed as most of these patients fuse in the primary position.
9
Paralytic Squint
If an extraocular muscle is paralyzed, a deviation will result from the unopposed
pull of the direct antagonist muscle. The deviation is greatest when the patient
looks in the direction in which the affected muscle works. The patient may turn the
face or head in that direction to preserve the binocular vision (Compensatory head
posture). Incomplete paralysis is called paresis.
CLASSIFICATION OF EXTRAOCULAR MUSCLE PALSY
1. Congenital Extraocular Muscle Palsy
a. Cases in which normal binocular single vision has never developed: A
constant manifest deviation is present since childhood.
b. Cases in which binocular single vision has developed in a limited field
with or without the aid of compensatory head posture:
i. Remaining fully compensated in adult life,
ii. Becoming decompensated in adult life (symptom—producing intermit-
tent manifest deviation),
iii. Becoming decompensated during developmental period for binocular single
vision (A constant manifest deviation is present with sensory sequelae i.e.,
suppression, amblyopia/eccentric fixation, normal retinal correspondence/
absence of normal retinal correspondence/abnormal retinal corres-
pondence).
2. Acquirred Extraocular Muscle Palsy
a. Of recent onset
b. Long-standing
i. Onset in childhood
ii. Onset in adult life
Congenital and old paralysis Recent paralysis
Diplopia Rare but may occur suddenly Always present but may be
with decompensation limited to the paretic field
Amblyopia May be present Absent
Comitance Spread of comitance may Characteristically incomitant
obscure original paresis
Abnormal head posture May persist on covering paretic Disappears on covering paretic
eye because of secondary scoliosis eye. Old photographs do not
and contracture of neck muscles. show anomalous posture.
Old photographs may show
anomalous posture.
Facial asymmetry Frequent with torticollis of Absent
long-standing.
Contracture of antagonist with Present Absent
positive forced ductions
96 Strabismus

Alternatively it may be classified as:


9 A.Neurogenic
i. Supranuclear
ii. Nuclear
iii. Internuclear
iv. Infranuclear-fascicular (nerve or subdivision)
B. Myogenic
i. Nerve-muscle junction lesion (Myasthenia)
ii. Muscle
a. Congenital-absence, hypoplasia, malinsertion or musculofascial anoma-
lies
b. Traumatic-laceration, disinsertion
c. Inflammatory (myositis)
d. Myopathy (dysthyroid)
e. Dystrophy
Investigations for Paralytic Squint
1. History: In an acquired muscle palsy of recent onset, the patient will complain
of diplopia, which may be horizontal, vertical or torsional, or a combination of
these. Any variation of diplopia in different positions of gaze and distance from
the patient will give some indications as to which muscle is affected. In long-
standing muscle palsies, the patient may have diplopia but is unlikely to be much
troubled by it. He is more likely to complain of blurred vision, headache and eye-
strain. Any previous treatment should be noted, particularly if the patient had a
squint since childhood suggesting the status of binocular function.
2. Abnormal head posture: The patient may be able to adopt an compensatory
abnormal head posture in order to see single in the primary position. This is
usually done in acquired cases only if the change in the head if the change in
head position is not too great and uncomfortable to adopt. In congenital cases
the abnormal head position may be more marked because of the strong desire
for binocular single vision, marked abnormal head position can be adopted with-
out much discomfort. In these cases the patient is unaware of the abnormal
head position, whereas in recently acquired cases any change in head position is
very obvious to the patient.
3. Visual acuity: The vision should be normal unless there is some refractive error
or some condition which may be related to the muscle palsy e.g. damage to eye
caused by blow to head.
Amblyopia: Strabismic amblyopia may occur in strabismus of any type, if
acquired in the visually immature years. The younger the patient, the greater is
the risk.
4. Cover test (with and without head posture): This is performed at one-third
metre and at 6 metres with and without glasses, if worn. If the patient has

http://ophthalmologyebooks.com
Paralytic Squint 97

Compensatory Head Posture

Position of the Chin


9
Chin Raised Chin Depressed
(RSR, RIO, LSR, LIO) (RIR, RSO, LIR, LSO)

Face Turn Face Turn

To the Right To the Left To the Right To the Left


(RSR, LIO) (LSR, RIO) (RIR, LSO) (RSO, LIR)

Head Tilt Head Tilt Head Tilt Head Tilt

To the To the To the To the To the To the To the To the


Right Left Right Left Right Left Right Left
(RSR) (LIO) (RIO) (LSR) (LSO) (RIR) (LIR) (RSO)

adopted an abnormal head posture, the cover test must be performed with and
without this. It is expected that there will be binocularity with the abnormal head
position and a manifest deviation with diplopia in the primary position without it.
In a recently acquired palsy the deviation will be greater, while fixing with the
affected eye(secondary deviation).
5. Ocular movements: Ocular movements combined with cover test are examined
in nine cardinal positions of gaze. The patient should fix a light or other target.
Any underactions and overactions, updrifts and downdrifts, should be noted,
looking for the maximum limitation of movement and, therefore, the affected
muscle or nerve. The amount of other underactions and overactions will depend
on how much the muscle sequelae has developed.

MUSCLE SEQUELAE
With the onset of a muscle palsy, changes occur in the actions of other ocular
muscles—both in the affected and in the non-affected eye. Later on (after months
or years) the deviation becomes more concomitant.
Following Changes Occur
1. Contracture (overaction) of the ipsilateral antagonist: It occurs due to the
normal tone and tension now unopposed by the underacting muscle. This re-
sults in the primary deviation when the unaffected eye is fixing.
98 Strabismus

2. Contracture of the contralateral synergist: It occurs due to Herring’s law of


9 equal innervation. Overaction of the contralateral synergist (yoke muscle) is
demonstrated while fixing with the affected eye. This results in the secondary
deviation.
3. Secondary inhibitional palsy of the contralateral antagonist: It occurs
due to Sherrington’s law of reciprocal innervation. This is the final stage in the
development of concomitance.
Primary palsy Contraction of Contracture of Secondary inhibitional
ipsilateral antagonist contralateral synergist palsy of contralateral
antagonist
Superior rectus Inferior rectus Inferior oblique Superior oblique
palsy overaction overaction underaction
Inferior rectus Superior rectus Superior oblique Inferior oblique
palsy overaction overaction underaction
Lateral rectus Medial rectus Medial rectus Lateral rectus
palsy overaction overaction underaction
Medial rectus Lateral rectus Lateral rectus Medial rectus
palsy overaction overaction underaction
Superior oblique Inferior oblique Inferior rectus Superior rectus
palsy overaction overaction underaction
Inferior oblique Superior oblique Superior rectus Inferior rectus
palsy overaction overaction underaction

There can be more than one affected muscle which will be evident while
assessing ocular movements e.g., oculomotor nerve paralysis and bilateral
superior oblique muscle paralysis.
The cover test should also be performed in direct elevation and depression
to determine the presence of ‘A’ or ‘V’ pattern.
6. Diplopia test: The patient may wear a red and green goggles. The test should
be combined with ocular movements. The patient should look at a light in nine
cardinal positions of gaze and should be asked to describe the relationship of
the diplopic images to each other (whether there is horizontal or vertical sepa-
ration or both). The patient should be asked to indicate where the separation of
the images is maximum and also whether there is any position with single
vision. The patient may also complain of torsional diplopia. In these cases a
bar light should be used and the patient should be asked to describe which way
one or both of the lines of light are tilted. Diplopia can be recorded either on a
chart or by verbal description.
7. Hess/Lees screen: A Hess chart is plotted using a Hess or Lees screen. This
is a very useful aid to diagnosis and should be plotted at every visit to record
any change in the state of palsy and thus gives a valuable permanent pictorial
record. The test is performed fixing either eye and it shows the deviation in the
primary position and the stage in the development of the muscle sequelae. The
Paralytic Squint 99

more recent the onset , the more incomitant the deviation will be, as the muscle
sequelae has not yet developed. If the paralysis is long-standing, the muscle
sequelae will be more advanced and it may become very difficult to diagnose
9
the primarily affected muscle.
8. Measurement of deviation: The deviation should be measured in the primary
position, fixing either eye by the prism cover test. If there is a vertical and
horizontal deviation, two prism bars are placed over one eye, thus maintaining
a fixing eye. The prism bar cover test is performed at one-third metre and
6 metres and in elevation and depression to measure ‘A’ and ‘V’ patterns, if
indicated. The deviations may also be measured in nine cardinal positions of
gaze by synoptophore. This test may be repeated under the same condition.
This should be done fixing with either eye and in a recently acquired palsy, the
maximum deviation will be on fixing with the affected eye and looking into the
field of action of the affected muscle. Torsion can be measured by using special
slides, a cross and a segmented circle. The patient should be instructed to
place the cross in the circle and adjustments are made to correct any tilting of
either slide. This should be performed in the nine positions of gaze, fixing either
eye. Torsion can also be measured by placing two Maddox rods, one red and
one white , into the trial frames. If there is any torsion, the patient can rotate
the rods in the frame until the streaks of light are parallel and horizontal. The
amount of torsion can be measured from the degree scale on the trial frames.
The amount of deviation measured while fixing with the non-paralytic eye is
known as the primary deviation and the amount of deviation measured while
fixing with the paralytic eye is known as the secondary deviation. In a patient
with paralytic squint, the secondary deviation is greater than the primary
deviation.
9. Assessment of binocular function: Binocular function must be assessed in all
cases. Ideally prism fusion test, stereo tests etc. should be performed. If the
deviation is constant then it should be first corrected by using a synoptophore
and then binocular function is assessed (the angle of deviation is corrected and
then fusion and stereopsis are assessed).
10. Subjective Park’s test for cyclovertical muscle imbalance: A red glass should
be placed in front of the right eye and a green glass in front of the left eye. The
patient should be asked to fix a light at 6 metres and to identify if the red or the
green light is higher. Then the patient should be asked to tilt his head to the left
shoulder, then to the right shoulder, and identify in which position the images
are displaced the widest. Finally, the patient should be asked to identify if the
images are wider when the eyes are turned to the left with the face turned to
the right or the eyes turned to the right with the face turned to the left. This test
can also be performed at one-third of a metre. The conclusion as to which
cyclovertical muscle is paretic is obtained by the following table:
100 Strabismus

9 Step I (which eye is


higher?)
Right eye
Step II (Vertical separation
greater tiling right or left)
Tilt left
Step III (Vertical separation
greater gazing right or left)
Right gaze
Paretic muscle

RIR
Left gaze LSR
Tilt right Right gaze LIO
Left gaze RSO
Left eye Tilt left Right gaze LSO
Left gaze RIO
Tilt right Right gaze RSR
Left gaze LIR

11. Forced duction test: This is done to differentiate between restrictive and
paralytic strabismus. Indications are incomitant squints, thyroid ophthalmopa-
thy, blow out fracture, Duane’s and Brown’s syndromes. To perform this test
topical anesthetic drops are applied. The eye is passively moved with forceps
in the direction the limitation of movements. Passive movement is possible in
neurogenic pathology while it is not possible in restrictive pathology.The forced
duction test is only reliable if it is markedly positive and a mechanical restric-
tion is obvious.
12. Active force generation test: This is done in paralytic squint. The patient is
asked to move the eye in a given direction while the examiner fixes the eye.
The test is most useful in testing the actions of the rectus muscles. This test is
performed to see if the rectus muscle being assessed is capable of working
properly or is completely paralyzed.
13. Saccadic velocity: This test is done to differentiate between neurogenic and
restrictive pathologies. It provides a graphic record of the speed and direction
of eye movements. The patient is asked to change fixation horizontally between
two fixation targets 20 degrees to 30 degrees apart in the appropriate gaze
position to evaluate the suspected weak muscle. The eye with a weak muscle
may show slowing of the saccade when compared with the normal side.
14. Neurological investigations: The patient should be referred to a neurologist
for complete evaluation to exclude conditions like myasthenia gravis and multiple
sclerosis.
Treatment
Non-Surgical Treatments
1. Prisms are not commonly useful in the treatment of paralytic strabismus unless
it has become concomitant.
2. Occlusion therapy to prevent amblyopia should be started immediately in a young
patient whose visual acuity could not be tested and who has not adopted a
compensatory head posture to preserve fusion.
In 6th nerve palsy, occlusion of the normal eye followed by botulinum toxin
injection into the medial rectus is indicated if there is no recovery within 1 month.
Surgical correction should be considered if there is no recovery within 3 months.
Paralytic Squint 101

An acquired cyclovertical muscle palsy(such as superior oblique) is not usually


associated with contracture and surgery may be delayed for 6 months or more.
Active duction and version exercises may help to recover the paralytic muscle.
9
The patient should cover the unaffected eye and try to move the paralytic eye
as far as possible into the field of action of the paralytic muscle. This should be
repeated for a few minutes several times a day.
Surgical Treatments
General Principle
1. Weakening either the antagonist or contralateral synergist of the involved paretic
muscle results in best improvement when all fields of gaze are taken into con-
sideration.
2. Attempted strengthening of the totally paralyzed muscle is usually ineffective.
3. When the deviation becomes concomitant, the above rules are not valid. In
horizontal deviations that have become concomitant, surgery may be confined
to the affected eye.

ACQUIRED SIXTH NERVE PALSY


Non-Surgical Treatments
a. Occlusion of the unaffected eye: It may be done for two purposes.
i. To prevent amblyopia in the visually formative years
ii. To prevent contractures of the antagonist muscles from developing.If the
good eye is covered, the patient is able to bring the other eye straight by
complete inhibition of the medial rectus muscle, which prevents contracture
of the medial rectus muscle.
b. Botulinum A exotoxin injection: Botulinum toxin injection may be indicated in
adults who show slow or no improvement after 1 month. This treatment is
useful in the management of acquired 6th nerve paralysis in visually immature
children. In a visually mature child, if occlusion fails to control the development
of contracture of the medial rectus after 1 month, botulinum toxin should be
injected into the antagonist medial rectus muscle. The effects of the exotoxin
wear off in about 2 months, and by that time the 6th nerve palsy usually recov-
ers.
Surgical Treatment
A.If some action of the lateral rectus muscle can be shown by the active force
generation test, some abduction movement past the straight ahead midline posi-
tion is elicited or some abduction is noted after the injection of botulinum toxin
into the medial rectus- an adjustable recession of the medial rectus and resec-
tion of the lateral rectus should be done to correct the deviation in the primary
position.

http://ophthalmologyebooks.com
102 Strabismus

B. If there is absolutely no action in the lateral rectus


9 1. Jensen’s procedure:
i. Nonabsorbable 5 to 0 sutures should be used to tie, permanently, the
loops from the vertical recti and the lateral rectus 6 mm from the inser-
tion. The sutures should be placed 6 mm from the insertion in the outer
half of the vertical rectus muscle and the adjacent half of the paralytic
rectus muscle so they are joined up 6 mm from the insertion. This must
create an exotropia of about 25 to 30 prism dioptres.Postoperatively the
patient will assume fusion with a face turn opposite to the preoperative
face turn, which will be corrected within 2 months.
As there is a possibility of anterior rectus ischemia in patients over 50
years of age, the vertical recti should be divided in half, making sure that
one intact artery remains in the half of the vertical rectus muscle not
involved in the operation.
ii. The ipsilateral medial rectus should be recessed by 6 mm. The anterior
ciliary arteries should be preserved if possible when recessing the medial
rectus.
Results: The affected eye will be within 10° of being straight and the
patient will have some fusion with amplitude. Some pseudo-abduction up
to 20° may be present due to the elastic band effect of the transposed
muscle.
2. Surgery combined with botulinum exotoxin:
i. Injection of botulinum toxin to the ipsilateral medial rectus 7 days prior to
the surgery will allow the paralysis of the medial rectus (which usually
occurs within 5 days) followed by Jensen’s procedure (except the medial
rectus recession).
Or
ii. Injection of botulinum toxin to the ipsilateral medial rectus 7 days prior to
the surgery follwed by transposition of both the vertical recti to the inser-
tion of the paralytic lateral rectus. Finally, if there is some abduction of
the eye following the temporary paralysis of the medial rectus, a large
adjustable recession of the medial rectus and resection of the lateral
rectus in the involved eye should be combined with an adjustable reces-
sion of the contralateral medial rectus when the effects of the botulinum
have worn off.

THIRD NERVE PALSY


It is very difficult to cosmetically correct a complete congenital or acquired third
nerve palsy. The eye is down 15 to 20 prism dioptres and exotropic about 70 prism
dioptres. Complete ptosis is always present unless some aberrant regeneration has
occurred.
To find out whether there is an associated 4th nerve palsy, the patient should be
asked to look down while observing the horizontal conjunctival or other markings
Paralytic Squint 103

to note whether the attempt to look down produces incyclotorsion of this eye,
which would indicate that the superior oblique muscle is working. 9
Treatment
1. Non-surgical: Occlusion should be done to prevent amblyopia in a child in the
amblyogenic age group.
2. Surgery: Following steps should be combined:
a. A maximum recession (12 mm) of the ipsilateral lateral rectus should be
done by scleral sutures. Neither an adjustable suture nor a hang-back method
should be employed as there is a risk that the muscle may creep forward and
reattach itself to the sclera closer to the limbus.
b. If the superior oblique is functioning, transposition of the tendon of the supe-
rior oblique can be done. If there is an hypotropia of more than 20 prism
dioptres, the superior oblique tendon should be cut between the trochlea and
the medial border of the superior oblique muscle and the end of the superior
oblique tendon is straightened out so that it comes to lie along the belly of the
paralytic medial rectus muscle. The tendon comes straight from the trochlea
to the insertion of the medial rectus muscle. The tendon should be resected
in such a manner that it should be tight enough to hold the eye in 20 prism
dioptre adduction at the end of the surgery. The overcorrected position soon
disappears and after 2 months an exotropia of about 15 prism dioptres
remains, which is a marked cosmetic improvement.
If the hypotropia is less than 15 prism dioptres, the trochlea should be
dislocated from the frontal bone with mosquito clamps. Then, the tendon of
the superior oblique should be cut between the trochlea and the medial bor-
der of the superior rectus muscle and brought up tightly beside the medial
rectus muscle. It will now lie parallel to the medial rectus muscle. The trans-
posed tendon should be resected in such manner that an esotropia of 20
prism dioptres with a positive forced duction test is created at the end of the
surgery. Within 2 months, cosmetic improvement occurs and a small exotro-
pia remains in primary position.
c. Management of ptosis: If complete ptosis is present, a frontalis sling opera-
tion should be performed.

FOURTH NERVE PALSY


Described in the VERTICAL STRABISMUS chapter.
10
Vertical Strabismus
A vertical deviation is a vertical misalignment of the visual axes. It may be comitant
but is usually incomitant. They can occur alone or with a horizontal component.
The commonest cause of vertical strabismus is superior oblique palsy. A superior
oblique palsy is usually congenital. The second most common cause is trauma—
particularly head injury. The other causes may be intracranial neoplasm, central
nervous system vascular problems or diabetes.

SUPERIOR OBLIQUE PALSY (4TH CRANIAL NERVE PALSY)


It is the commonest isolated cyclovertical muscle paralysis. It may be unilateral or
bilateral. Markedly asymmetric bilateral palsies that initially present as unilateral
are termed ‘masked bilateral’.
Unilateral Superior Oblique Palsy
Clinical Features
1. There is head tilt to the opposite shoulder.
2. The affected eye is hypertropic when the eye is straight.
3. There is overaction of the inferior oblique in the affected eye, which results in
an increase of the deviation in gaze to the same side as the head tilt, i.e., away
from the affected side.
4. There is a positive Bielschowsky head tilt test with resultant increase of
hypertropia in tilting the head to the affected side.
5. Unusual feature: A hypotropia of the unaffected eye instead of the expected
hypertropia of the affected eye and the patient fixing with the affected eye.
Assessment
a. A complete measurement of strabismus in all positions of gaze should be done.
b. If there is head tilt:
i. If one eye is occluded, the patient’s head should straighten in a child and
might not straighten if the patient is an adult.
ii. Fusion is possible with the preferred head position and absent when the head
is straight.
iii. The cover test usually shows no vertical deviation (sometimes present in
patients having only peripheral fusion) with the head in the preferred position
but present when the head is straight.
iv. Diplopia test (using a red filter in front of one eye and a light as a fixation
target) should be done in the nine positions of gaze to find out where the
vertical separation of the muscle is maximal.
Vertical Strabismus 105

c. Tests for torsion: Torsion becomes apparent when the fusion target is torted
somewhat in excess of 10 degrees. If torsion exceeds 15 degrees, it may be a
barrier to fusion.
10
i. Double Maddox Rod Test: Two Maddox rods, preferably a red and a white
should be used. The red one should be placed in front of one eye and the
white one in front of the other in such a way that the cylinders should be
vertical in front of each eye and the patient sees two lines horizontally through
the light. The patient should then be asked to make the two lines parallel by
rotating one Maddox rod in its frame, which is marked in degrees. The
patient will see the horizontal line tilted inward with the margin of the line
tilted down toward the nose, if there is excyclotorsion. In order to make the
lines parallel, the patient will have to rotate the Maddox rod downward on
the temporal side. The direction in which the patient has to rotate the Maddox
rod is noted. If this in outward, there is excyclotorsion and the measurement
can be read in degrees from the dial.
ii. Bagolini striated glasses in a trial frame can be used in the same way.
iii. Synoptophore: Specially designed dissimilar slides should be used and the
patient should be asked to superimpose them. The synoptophore arm in front
of the deviating eye should be adjusted horizontally, vertically and torsionally
until both the images are superimposed and upright. The angle and type of
deviation can be read off a scale for each type of deviation. The synopto-
phore can be used to measure the deviation of gaze with either eye fixing,
particularly in primary position and on 25 degrees of down gaze where the
torsion effects are usually the greatest.
iv. Hess chart: This test cannot calculate the amount of torsion.
Treatment
A.Prisms: It may be used sometimes to reduce the symptoms.
B. Surgery: In long-standing cases with fusional amplitudes, the vertical deviation
should be undercorrected. In large vertical deviations one muscle should prefer-
ably be operated at a time and assessment should be done after 2 months before
considering a further procedure. Inferior rectus muscle should better be avoided
and only performed if definitely indicated. An inferior oblique myectomy or
superior rectus recession should be done.
a. Inferior rectus weakening procedures:
i. Myectomy of inferior oblique muscle: An alternative surgery is reces-
sion of inferior oblique.
Indications: Overaction of the antagonist inferior oblique muscle as well
as a hypertropia of 5 prism dioptres or more in the primary position with
the head straight.
Procedure: 5 mm of muscle between the inferior rectus and lateral rec-
tus should be cut out. The conjunctiva is incised in the inferior fornix,
between the lateral and inferior rectus muscles, 6 mm from the limbus.
106 Strabismus

Strabismus hooks are used around the inferior and lateral rectus muscles
10 and the eye is pulled up. When the inferior oblique is clearly visualized
another strabismus hook is passed around it. Then the inferior and lateral
rectus muscles are released. Another strabismus hook is placed under
the inferior oblique muscle. Both the hooks should pass around the whole
belly of the inferior oblique muscle. If a small snip of the muscle is missed,
the muscle will join up again, making the procedure a failure. The two
strabismus hooks around the inferior oblique are separated by about 5
mm and clamps placed across the muscle adjacent to each hook. The
hooks are removed and the muscle between the clamps is excised. The
ends are cauterized and allowed to retract into the lower fornix. The
maximum effect usually does not exceed correction of 20 prism dioptres
of hypertropia in the primary position with the head straight. If the devia-
tion is more than 20 prism dioptres,revaluation should be done after 2
months. If the residual deviation can be controlled by the patient, no
further treatment is indicated. The same procedure is performed for ver-
tical deviations of 5 to 20 prism dioptres. Inferior oblique action usually
returns within 3 weeks after surgery.
ii. Recession of the contralateral inferior rectus muscle: If the inferior
oblique muscle is not overacting, recession of the contralateral inferior
rectus muscle should be considered. The hypertropia should be greater in
downgaze and particularly in the field of action of the paretic superior
oblique muscle. The surgery must be done aiming to undercorrect the
deviation in primary and down gaze. If more than 3 mm. of recession or
resection of this muscle is done, the lower lid height will be affected. An
adjustable suture on the inferior rectus should preferably be used. A
correction of 3 prism dioptres per millimeter in the primary position and 5
prism dioptres per millimeter in downgaze should be calculated.
iii. Recession of the ipsilateral superior rectus muscle: Some cases of
long-standing superior rectus palsy may show same amount of hypertropia
in side gaze to the left and right as well as head tilting. If there is a
minimum increase of hypertropia in down gaze, a recession of ipsilateral
superior rectus muscle up to 6 mm should be done. If a larger recession
is done, subsequent overaction of the yoke inferior oblique muscle may
result. An adjustable suture technique is preferable. If there is any over-
correction in the field of action of the muscle it will be in up gaze (in
contralateral inferior rectus muscle surgery, it will affect the down gaze
making reading, going up and down stairs etc. very difficult).
iv. Tuck of the ipsilateral superior oblique tendon (the paretic muscle)
for torsion: It is indicated where torsion exceeds 10 degrees and inter-
fering with fusion.
v. Tuck of the ipsilateral superior oblique tendon (the paretic muscle)
for hypertropia: It is sometimes indicated in patients with a hypertropia

http://ophthalmologyebooks.com
Vertical Strabismus 107

of 10 to 15 prism dioptres in the primary position remaining after a


recession of the yoke inferior rectus muscle and the hypertropia increasing
to double this amount in down gaze and particularly in the field of action
10
of the paretic superior oblique muscle. For an unilateral superior oblique
palsy, the contralateral inferior rectus muscle should not be recessed
more than 6 mm. Even if the inferior rectus has been recessed for 4
mm., a tuck of ipsilateral superior oblique should be preferred as the
inferior orbit is prone to scarring. However, a tuck of superior oblique
tendon is difficult to titrate and may cause iatrogenic Brown syndrome.
The tendon is tucked for about 8 mm (4 mm on either side of the clamp
or hook). The tuck should be done as temporally as possible, pulling the
superior oblique tendon under the belly of superior rectus muscle to the
temporal side to minimize the possibility of inducing a Brown syndrome.
Bilateral Superior Oblique Palsies
Clinical Features
1. It may occur as a congenital anomaly though commonly this is an acquired
condition with severe head trauma.
2. There is chin down head position for fusion on up gaze.
3. There is a small vertical deviation in the primary position.
4. There is a left hypertropia on right gaze and a right hypertropia on left gaze.
5. There is an esotropia on down gaze exceeding the measurements in the primary
position by at least 10 prism dioptres (V pattern).
6. There is an excyclotorsion, often bilateral, exceeding 10 degrees in the primary
position with a marked increase of excyclotorsion on down gaze to as much as
30 degrees. Unilateral superior oblique palsies usually have an excyclotorsion
less than 10 degrees, whereas bilateral superior oblique palsies have an
excyclotorsion more than 10 degrees.
7. There is a left hypertropia on left head tilt and a right hypertropia on head tilt
(bilateral positive Bielschowsky head tilt test).
8. Diplopia is present in all positions except in up gaze.
Management
The goal of management is to obtain single binocular vision while looking straight
ahead and looking down.
If the torsion is preventing the fusion, then surgery to alleviate the torsion should
be done first.
A.If excyclotorsion exceeds 15 degrees in down gaze, torsion may prevent fusion.
If neutralization of the horizontal and vertical deviations are done with prisms or
by using a synoptophore, fusion is absent and only possible when torsion has
been corrected by synoptophore. Here, bilateral superior oblique tuck (usually 8
mm) is needed as the primary procedure. If there is a significant hypertropia,
108 Strabismus

tucking should be done more on the side of the hypertropia. Evaluation should be
10 done 1 month after the surgery. Bilateral tucks may produce some correction of
esotropia in down gaze and an acceptable correction of excyclotorsion.
B. If one or both inferior oblique muscles are overacting and there is more than 10
prism dioptres of deviation in primary position associated with this overaction, a
weaking procedure like inferior oblique myectomy or recession of inferior ob-
lique should be done. When examined after 2 month, if marked overaction of
the other inferior oblique is noted, an inferior oblique myectomy or recession
should be done on the other inferior oblique. If, both the inferior obliques are
overacting and there is a vertical squint of less than 6 prism dioptres, a myec-
tomy of both inferior obliques should be done first.
C. If there is esotropia in down gaze, superior oblique surgery, particularly tucking,
may help.
D.Bilateral medial rectus infraplacement and angulation for V-esotropia: Both
the medial rectus muscles should be recessed and infraplaced one-half the width
of the tendon insertion downwards. The upper border should be recessed to
produce an exophoria of about 6 prism dioptres in the primary position. The
lower border should be recessed an additional 2 mm from a straight line drawn
down from the normal insertion. This will slant the insertion and together with
the infraplacement will neutralize the V-esotropia in down gaze, which usually
exceeds the primary deviation by at least 10 prism dioptres.

OTHER CAUSES OF VERTICAL SQUINT


I. DISSOCIATED VERTICAL DIVERGENCE
When the vision of either eye is embarrassed the eye deviates progressively
upwards, but reverts to its original position when the embarrassment ceases. It
may sometimes be so asymmetrical as to be virtually unilateral and may be associ-
ated with binocular single vision (BSV) or a manifest deviation.
The etiology of dissociated vertical divergence (DVD) is obscure. There are
many theories in this regard. It appears to be associated with early disruption of
binocular development.
Clinical Characteristics
1. It is a bilateral condition, but may be asymmetrical.
2. Its presence is often not detected before the age of 2 years. In some cases
there is a manifest nystagmus which precedes DVD. The nystagmus becomes
latent with time and following this the diagnosis of DVD is eventually confirmed.
In other cases DVD may be noted for the first time following surgery to correct
a congenital esotropia.
3. There is progressive upward deviation of each eye, when occluded. Occasion-
ally, there may be a spontaneous upturning of either eye when fatigued or day-
dreaming.
Vertical Strabismus 109

4. Elevation under cover is slow and tends to increase on prolonged dissociation. It


is often more marked on distance fixation. 10
5. When the occluder is removed the eye slowly drifts down.
6. More commonly, DVD is associated with a manifest squint. There is a reported
high incidence of DVD in cases of congenital esotropia. DVD may sometimes
be associated with BSV.
7. Latent nystagmus and extorsion (the eye intorts as it depresses) are frequently
associated.
8. Measurement of DVD may be done by using a base down prism in front of the
deviating eye under an occluder. The occluder should then be switched to the
fixating eye. Base down prism should then be added to measure the amount.
Bielschowsky Phenomenon: DVD and alternating hyperphoria are the conditions
where the eyes elevate under cover. Bielchowsky phenomenon differentiates these
two conditions.
a. One eye is covered and it will elevate under cover.
b. A darkening glass wedge (Sbiza bar) is placed before the fixing eye and slowly
moved from the least dense filter to the most dense filter.
c. The examiner observes the eye behind the occluder which will make a down-
ward movement and may pass below the midline in DVD.
d. The elevated position of the occluded eye is unchanged by placing increasingly
dense filters before the fixing eye in alternating hyperphoria.
Treatment for DVD is indicated if the vertical deviation occurs spontaneously,
is frequent, and is cosmetically significant. Patching or optical means, which changes
the fixation pattern, is most effective in unilateral or highly asymmetric bilateral
DVD. Surgery often improves the condition but rarely eliminates its:
a. Recession of superior rectus (7 to 9 mm) – ideal for bilateral DVD
b. Faden operation of superior rectus (retroequatorial myopexy) may be effective
particularly if combined with (a)
c. Resection of inferior rectus: if (a) and (b) fails
d. Total anteropositioning of inferior obliques—thus the inferior obliques are con-
verted into depressors.

II. INFERIOR OBLIQUE OVERACTION


This may be a result of mechanical or innervational causes or a combination of the
two. Primary inferior oblique muscle overaction has been found to develop be-
tween 1 to 6 years in two-third of patients with congenital esotropia. Rarely it is
seen in association with acquired esotropia or exotropia. It usually tends not to
cause a primary gaze hypertropia. Secondary overaction is caused by a paresis of
its antagonist superior oblique muscle. It is characterized by overelevation of the
eye in adduction.
110 Strabismus

Clinical features include overelevation of the eye in adduction.


10 DVD Inferior oblique overaction
Hypertropia present in all gaze positions Present in adduction only
Does not obey the Hering’s law Obeys the Hering’s law
Superior oblique may overact Superior oblique usually underact
No pseudoparesis of contralateral superior rectus Pseudoparesis of contralateral superior rectus
Incycloduction on refixation No incycloduction on refixation
Slow floating abduction, elevation, excyclotortion Rapid elevation of adducted eye while abducting
movement eye fixing.
Not associated with A or V pattern Often associated with V pattern
Proportional to ambient illumination in fixing eye Not proportional to illumination in fixing eye
Frequent presence of latent nystagmus Latent nystagmus may be associated
Bielschowsky phenomenon is present. Bielschowsky phenomenon is absent.

When overelevation of the adducted eye is clinically significant, a weakening


procedure of the inferior oblique (inferior oblique recession or myectomy) is indi-
cated.

III. SUPERIOR OBLIQUE OVERACTION


Almost all bilateral superior oblique muscle overactions can be considered primary.
A vertical deviation often occurs in primary position with unilateral or asymmetric
bilateral overaction of the superior oblique muscles. The hypotropia is in the side of
the unilaterally overacting superior oblique muscle. An associated horizontal
deviation, usually exotropia, may be present. The overacting superior oblique muscle
also causes depression with resulting hypotropia on adduction.
In presence with A-pattern or significant ocular deviation due to bilateral supe-
rior oblique overaction, a bilateral superior oblique weakening procedure is indi-
cated. The procedure should be either a superior oblique tenotomy or a superior
oblique lengthening by insertion of a silicone expander or a non-absorbable suture.

IV. INFERIOR RECTUS MUSCLE PARESIS


This condition is noted as a result of trauma to the nerve or to the inferior rectus
muscle and can occur either at the time of injury or at the time of repair of an
orbital floor fracture. If inferior rectus muscle paralysis is noted without entrap-
ment, a hypertropia is usually seen in the primary position. If paresis is present with
entrapment, the patient may have very little deviation or even a slight hypotropia,
which decreases on down gaze, is noted.
If recovery does not occur within 6 months of the injury, surgery is indicated.
A.For partial inferior rectus muscle paresis—a resection of the affected muscle
and a recession of the ipsilateral superior rectus muscle is indicated. Alterna-
tively, a recession of the contralateral inferior rectus muscle with or without
posterior fixation can be done.
B. For complete inferior rectus muscle paralysis—transposition of the ipsilateral
medial and lateral rectus muscles to the inferior rectus muscle (Inverse Knapp’s
procedure) may be performed.
Vertical Strabismus 111

V. SKEW DEVIATION: CONCOMITANT ACQUIRED


VERTICAL STRABISMUS 10
This is generally an acquired lesion in older people and usually due to a possible
vascular insult in the midbrain area. It is a supranuclear lesion and is usually asso-
ciated with normal ductions. The deviation may be the same in up, down, side gaze
and head tilting.
Treatment
If the strabismus is concomitant and within 8 prism dioptres or the patient wears
glasses, prisms may be tried. Otherwise superior rectus recession in the hypertropic
eye (3 prism dioptres/mm up to a maximum of 8 mm) should be done.

VI. BROWN SYNDROME (SUPERIOR OBLIQUE TENDON


SHEATH SYNDROME) AND INFERIOR OBLIQUE PALSY
Brown’s syndrome or inferior oblique palsy should be looked for when the unaf-
fected eye shoots upward.
Brown syndrome occurs in congenital, acquired, constant, and intermittent
forms.It is bilateral in 10 per cent of cases. Restriction of free passage of the
superior oblique tendon through the trochlear pulley is the commonest form even
when related to trauma or rheumatological conditions. Acquired Brown syndrome
is more likely to be intermittent and to spontaneously resolve than congenital Brown
syndrome. Congenital Brown syndrome may spontaneously resolve over many
years, sometimes becoming intermittent before resolving completely.
Clinical features include a deficiency of passive elevation in adduction. With
rotation of the eye out of the field of vertical action of the superior oblique muscle,
elevation improves so that restriction is reduced in midline and no elevation or
minimal elevation is noted in abduction. Divergence is usually seen with midline
elevation. During adduction the palpebral fissure widens and a downshoot of the
eye is often noted. There is minimal or no ipsilateral superior oblique muscle
overaction. This finding and a positive forced duction test demonstrating restricted
passive elevation in adduction differentiates it from inferior oblique palsy.
Brown syndrome can be graded as mild, moderate severe:
Grade Features
Mild No hypotropia is present in primary position and no down
shoot of the eye occurs in adduction
Moderate No hypotropia is present in primary position but down
shoot of the eye occurs in adduction
Severe Hypotropia is present in primary position and down shoot
of the eye occurs in adduction. Often accompanied by
abnormal head posturing. A chin up head posture is most
commonly seen, although a face turn away from the affected
eye may be present.

http://ophthalmologyebooks.com
112 Strabismus

Mild and moderate forms make up about two-third of all Brown syndrome cases
10 and as they do not have strabismus in primary position, they are most often left
untreated.
Treatment
In Brown’s syndrome where there is a hypotropia of the affected eye of 10 to 15
prism dioptres with the head straight and some fusion ability, treatment is indicated.
Observation alone remains the most common management. Range-of-motion eye
exercises, oral corticosteroids, and corticosteroids injected near the trochlea have
each produced improvement in selected cases. If there is associated adult rheuma-
toid arthritis, juvenile rheumatoid arthritis or other systemic inflammatory diseases,
resolution may occur with systemic treatment. As sinusitis has also led to Brown
syndrome, CT scans of the orbits and paranasal sinuses should be done in all
acute-onset cases.
Surgery is indicated in cases where a hypotropia in primary position and abnor-
mal head posture are present and spontaneous resolution is unlikely. Ipsilateral
modified superior oblique tenotomy, preserving intermuscular septum during teno-
tomy, can improve elevation in adduction.
Inferior Oblique Muscle Palsy
This is a rare condition. The exact cause is not known. Damage to the inferior
division of oculomotor nerve and particularly to the branch that supplies the inferior
oblique may be the causative factor.
There is deficiency of elevation in adducted position. An a pattern is usually present
and the superior oblique muscle usually overacts. Forced duction test is negative.
Clinical Features
Inferior oblique muscle palsy Forced duction test is negative.
A pattern strabismus.
Superior oblique overaction is usually present.
Brown’s syndrome Forced duction test is positive.
V pattern strabismus.
Superior oblique overaction is usually minimal or absent.

Treatment is indicated in presence of abnormal head posture, vertical deviation


in primary position and diplopia. Management is usually superior oblique tenotomy.
Contralateral superior rectus muscle recession is done when ipsilateral superior
oblique muscle overaction is not present.

VII. MECHANICAL RESTRICTION/ORBITAL FLOOR FRACTURES


Usually the inferior part of the orbit is affected. Mechanical restriction in the infe-
rior part of the orbit usually results in a noticeable vertical strabismus in up-gaze.
This may be associated with the restriction of the inferior rectus muscle, thyroid
ophthalmopathy, blow-out fracture or congenital abnormalities. The patient com-
plain of one eye shooting upward on up-gaze.
Vertical Strabismus 113

Blow-out Fracture
Blunt facial trauma is the usual cause of orbital floor fractures. 10
Features
i. Ecchymosis of the involved eye
ii. Diplopia in some or all positions of gaze immediately following injury. It may
persist in up-gaze or down-gaze.
iii. Paresthesia or hypoesthesia of the infraorbital area, secondary to the dam-
age of the infraorbital nerve.
iv. Enophthalmos, either early or late.
v. Entrapment of the inferior rectus muscle, inferior oblique muscle, or sur-
rounding tissue.
vi. Hypotropia in the primary position that increases with up-gaze and may
decrease or show hypertropia in down-gaze. This suggests combined
mechanical restriction to elevation and inferior rectus paresis or pseudoparesis
to depression.
vii. Medial orbital wall fractures, resulting in medial rectus muscle entrapment.
Forced duction test is positive. CT scans and MRIs are helpful in exact localiza-
tion of the fracture.
Surgical management is controversial. There is difference of opinion regarding
the proper timing of surgical intervention and also regarding the method. Residual
strabismus can generally be corrected by using standard techniques (recession or
resection) without the necessity of exploring or repairing the orbital floor.

VIII. CONGENITAL DOUBLE ELEVATOR PALSY


The term ‘Double elevator palsy’ implies a paresis of both the inferior oblique and
superior rectus muscles. Patient complains of one eye shooting upwards. This is
the normal eye and the other eye is the affected eye. A patient with fusion may
adopt the chin up head position with a hypotropia of 10 to 20 prism dioptres of the
affected eye. Pseudoptosis is usually present and can be distinguished from true
ptosis by getting the patient to fix with the eye under the drooping eyelid. The upper
eyelid remains at the same level in true ptosis but elevates to the normal position in
pseudoptosis provided the eye comes up almost at the primary position, as in this
condition.
Diagnostic Criteria
1. Duction usually show complete inability to raise the eye above the primary
horizontal straight ahead position.
2. The forced duction test is normal, excluding mechanical restriction in the infe-
rior orbit.
3. Bell’s phenomenon is normal as double elevator palsy is a supranuclear lesion.
This differentiates it from an infranuclear palsy of superior rectus muscle.
114 Strabismus

Treatment
10 Transposition of the horizontal recti to the superior rectus in the affected eye
(Knapp’s operation) improves the alignment in the primary position but rarely results
in any upward movement.

IX. DOUBLE DEPRESSOR PALSY


This is a rare congenital condition.
Diagnostic Criteria
1. The patient has a complete inability to depress the eye below the horizontal
primary position.
2. A forced duction test should be done to exclude mechanical restriction in the
upper orbit.
Treatment
The horizontal recti in the involved eye should be transferred to the insertion of the
inferior rectus muscle.

X. SUPERIOR OBLIQUE MYOKYMIA


The patient experiences torsional and slight vertical diplopia in the form of oscillopsia,
usually lasting for 1 to 2 minutes (Frequency of the attacks may vary from once or
twice a week to several times a day). During attack, the intorsional fine nystagmus
may be seen. The aetiology is unknown.
Medical treatment (Cabamazine) has been tried with variable results. Surgical
treatment constitutes of tenotomy of the involved superior oblique combined with a
myectomy of the antagonist inferior oblique.

PSEUDOVERTICAL SQUINT
Where there is an appearance of vertical deviation though the visual axes are
actually aligned and bifoveal vision is present. This phenomenon is seen in-orbital
asymmetry, displacement of globe by an orbital mass, facio-maxillary fractures,
vertical angle-kappa, displaced macula, asymmetric eyelid retraction/ptosis.
11
Mechanical Restrictions and Syndromes
Duane’s Retraction Syndrome (DRS)
In 1905, Duane published a series of 54 cases of this ocular disorder. The inci-
dence is 1-4 per cent. Females are more susceptible (58%). In unilateral cases, the
left eye is more commonly involved (72%).
Alfred Huber (1974) Classified DRS in the Following Types
Type I
• It is the commonest type (78%).
• There is marked limitation of abduction with minimally defective or normal
adduction.
• There is retraction of globe and narrowing of the palpebral fissure in adduction.
• There is widening of the fissure on abduction.
• Simultaneous EMG recording of the lateral and medial recti showed paradoxi-
cal innervation of lateral rectus, with peak impulses on adduction and defective
impulses on attempted abduction. There was normal electrical behavior of the
medial rectus.
Type II
• It is the rarest type (7%).
• There is marked limitation of adduction with exotropia of the affected eye.
• The Abduction is normal or slightly limited.
• There is retraction of the globe and narrowing of the fissure on attempted ad-
duction.
• On EMG, the lateral rectus showed peak impulses on abduction and a second
paradoxical peak on attempted adduction. There was normal behavior of the
medial rectus.
Type III
• The incidence is about 15 per cent.
• There is combined limitation or absence of both abduction and adduction.
• There is retraction of the globe and narrowing of the palpebral fissure on attempted
adduction.
• The EMG findings showed intense innervation of both the lateral and medial
recti muscles, whether in primary gaze, abduction or adduction.
Ahluwalia (1988) modified Huber’s classification. He expanded on Huber’s
system by including three subgroups in each of the three types based on the devia-
tion noted in the primary position of gaze. All of the other features found in Huber’s
classification remained the same. Thus, Type IA, IB and IC DRS would be an
116 Strabismus

abduction deficiency with the deviation in primary gaze being an esotropia, exotro-
11 pia and orthophoria respectively. Type IA (25%) and type IIIC (21%) were found
to be the most frequent subgroups.
Pathogenesis
Mechanical, innervational and CNS abnormalities have all been implicated in DRS.
Surgical findings, cadaver studies and electrophysiological studies all seem to prove
that DRS encompasses aspects of all three theories of pathogenesis-mechanical,
anatomical and innervational. Lateral rectus muscle has been described by many
as fibrotic, inelastic and unyielding though some have found the muscle to be
completely normal. Co-contraction of the medial and lateral rectus muscle has
been considered as one of the major factors though sometimes EMG findings fail
to confirm it. Both the co-contraction theory and the lateral rectus fibrosis theory
can explain the retraction of the globe. The fibrotic changes which may occur in
the lateral rectus muscle are possibly due to the longstanding innervational anomalies.
Upshoots and downshoots of the affected eye and the enophthalmos are both
possibly due to co-contraction. Apparently a ‘bridle’ or ‘tethering’ effect in the
horizontal muscles and some amount of slippage is responsible. Elevation or
depression outside the primary position causes a change in the horizontal rectus in
reference to the center of rotation of the globe, causing these muscles to become
elevators and depressors in addition to their horizontal actions. Some consider that
vertical displacement of the insertion of the horizontal rectus muscles to be a
responsible factor.
Embryogenesis

DRS has been found to be frequently associated with congenital malformations.


According to Cross and Pfaffenbach, the associated anomalies could be divided
into four categories: skeletal, auricular, ocular and neural. Skeletal anomalies usually
involve the palate and vertebral column. Auricular abnormalities involve the external
ear, external auditory meatus and semicircular canals. Ocular defects involve the
extraocular muscles and the eyelids and include ocular dermoids. Neural
abnormalities involve the nuclei of the 3rd, 4th and 6th cranial nerves. A disturbance
between the 4th to 10th week of embryogenesis could explain the various nonocular
abnormalities reported with DRS.
Features
Most patients with DRS have strabismus in primary position. Patient may adopt
small relatively unnoticed head positions or face turn. Esotropia is the commonest
presentation. Hypermetropia more than +1.50DSph is seen in 71 per cent of patients.
Strabismic amblyopia is found in 14 per cent of patients. Upshoots and downshoots
(over-elevation and depression) may be seen in adducted position. Various types of
A, V and X patterns (V pattern is more common) may also be seen. In typical

http://ophthalmologyebooks.com
Mechanical Restrictions and Syndromes 117

DRS, the involved medial rectus is usually found to be tight as seen on lateral
rotation during forced ductions. Saccadic velocity testing (By using EOG) reveals
reduced abduction speeds in DRS types I and III due to paradoxical innervation of
11
lateral rectus muscle on attempted adduction. Of the other ocular findings nystagmus
is the commonest, followed by epibulbar dermoid, anisocoria and ptosis. Many of
the epibulbar dermoids are seen with Goldenhar’s syndrome. Sometimes, optic
nerve coloboma and fundus coloboma are also found. Other findings include Marcus-
Gunn jaw-wink, congenital cataract, heterocromia and optic nerve hypoplasia.
Presence of paradoxical-gustatory-lacrimal reflex (crocodile tears) has also been
reported.
Associated Non-Ocular Findings
1. Wildervanck syndrome (cervico-oculo-acoustic syndrome): It consists of DRS,
Klippel-Fiel anomaly and congenital sensorineural deafness, Klippel-Fiel anomaly
consists of a malformation of cervical vertebrae with possible webbed neck,
torticollis and facial asymmetry.
2. Okihiro syndrome: It consists of DRS with congenital thenar hypoplasia.
3. Goldenhar’s syndrome: It consists of vertebral anomalies, epibulbar dermoids,
facial hypoplasia and preauricular skin tags. It has been associated with DRS in
many instances.
4. Holt-Oram syndrome: It consists of congenital cardiac anomalies and musculo-
skeletal anomalies of the upper limb, in particular, hypoplasia of the thumb. A
familial occurrence of DRS and the Holt-Oram syndrome has been reported.
Variants of DRS
a. Vertical retraction syndrome: There is some degree of limitation of the affected
eye on elevation or depression associated with globe retraction and narrowing
of the palpebral fissure.
b. Combined abduction deficit with synergistic divergence: It presents as
unilateral adduction deficit and simultaneous abduction of the eyes on attempted
lateral gaze into the field of action of the apparently paretic medial rectus muscle.
There may be large exotropias, horizontal face turn away from the affected
eye, simultaneous abduction nystagmus on attempted adduction (inverse
nystagmus) and narrowing of the palpebral fissure in he affected eye on
adduction.
c. Pseudo-Duane’s retraction syndrome (Acquired retraction syndrome):
Usually there is a history of orbital trauma with subsequent medial wall blow-
out fracture and entrapment of the medial rectus. The entrapment may also be
due to orbital metastasis (Breast and prostate). Diplopia is present. There is
retraction of globe (reverse retraction) on attempted abduction.
Differential Diagnosis
1. Abducens nerve palsy: They have normal adduction and preservation of
convergence. A definite onset is diagnosed by a new esotropia with diplopia.
Esotropia is not associated with globe retraction, vertical upshots or downshoots.
118 Strabismus

2. Moebius syndrome (Congenital diplegia): It is characterized by 6th and 7th


11 cranial nerve palsies. It may present with a unilateral/bilateral limitation of
abduction, adduction and convergence. The mask-like facies and associated
abnormalities of the limb, chest and tongue are the differentiating features. There
is no globe retraction and vertical upshots/downshoots. Differentiation from
cases with bilateral DRS is difficult.
3. Congenital oculomotor apraxia: There is impairment of voluntary horizontal
gaze. This is characterized by jerky horizontal head thrusts.
4. Congenital or infantile esotropia: These patients may cross-fixate and appear
to have a clinical abduction deficit. With patching, the child with congenital
esotropia will abduct fully in time. They usually have much larger deviation than
seen in patients with DRS.
Management
Absolute Indications
a. A noticeable horizontal ocular deviation.
b. Abnormal head position.
Relative Indications
a. Marked retraction of globe on attempted adduction.
b. Cosmetically unacceptable downshoot and/or upshot of the eye in adduction.
Goal
a. Reducing or eliminating the face turn and tropia.
b. To center and enlarge the binocular diplopia-free field.
c. To increase the rotational movement of the abduction in the affected eye.
Surgical Procedures
A.Horizontal muscle surgery: Various surgical approaches have been advocated.
a. Unilateral and bilateral medial rectus muscle recessions in DRS with esotro-
pia.
b. Recession of lateral rectus muscle and Faden posterior fixation suture in
DRS with exotropia.
c. Vertical rectus muscle transposition alone and combined with a medial rectus
recession.
Recession operations do not normalize the motility of the eye but it normalizes
head position, reduces globe retraction and relieves upshoots without risking reduced
vertical tropias.
In cases of esotropias, if the deviation is over 20 prism dioptres, a bilateral
medial rectus recession should be done. Large medial rectus muscle recessions up
to 6 or 7 mm. on the affected eyes have also been recommended in these cases.
If the esotropia is between 20 to 30 prism dioptre range and a marked retraction
is present, bilateral medial rectus and ipsilateral lateral rectus recession should be
Mechanical Restrictions and Syndromes 119

done. Recession operations create less risk of enophthalmos and is less traumatic
than resections, thus decreasing the risk of adhesions.
In patients with exotropia, a recession of the ipsilateral lateral rectus should be
11
done and it should relieve exotropia, globe retraction and abnormal head posture. If
exotropia is more than 25 prism dioptres, recession of contralateral lateral rectus
muscle should be considered.
Resection of either medial or lateral usually worsen its features and should be
avoided.
B. Transposition procedures: Transposition of superior and inferior rectus muscles
towards the lateral rectus has been tried in esotropia. This procedure will in-
crease abduction, increase the tendency for upshots and worsen co-contrac-
tion. It causes vertical tropia in 10 per cent of the patients producing diplopia.
There is also increased risk of anterior segment ischemia when three or four
muscles are operated in one eye. The success rate of this operation is between
50 to 75 per cent. 10 to 15 degrees of abduction may be achieved.
C. Posterior fixation suture (Faden) procedure: Posterior fixation suture on lat-
eral rectus muscle produces satisfactory results with elimination of the upshot.
Posterior fixation suture on vertical rectus muscle did not change the abnormal
eye movement.
D.Approach to Upshoots, Downshoots, A and V pattern: The mechanical cause
for upshoots and downshoots has been attributed to the side-slip of the tight
lateral rectus muscle as the adducted globe moves above or below the horizon-
tal plane (the ‘bridle effect’). The paradoxical innervation of the lateral rectus
on adduction produces a taut lateral rectus muscle when it should be relaxing.
As the contracture increases, the lateral rectus can slip over the globe and
produce upshoots and downshoots on adduction. Three different overshoots
can occur:
a. upshot with V-pattern,
b. downshoots with A-pattern and
c. Combination of the two, resulting in a X-pattern.
The upshoots and downshoots caused by side slip of the lateral rectus can
be improved by:
i. Recession of the lateral rectus muscle. Recessing a very stiff, fibrotic
muscle by 7 to 8 mm can significantly reduce the overshoots. A non-
fibrotic lateral rectus with mildly positive forced duction should be recessed
by 10 to 12 mm to get this result.
ii. Posterior fixation (Faden) sutures on the lateral rectus muscle, with or
without similar sutures on the ipsilateral medial rectus muscle, alongwith
appropriate recession of the muscle. Posterior fixation suture on vertical
muscles do not change abnormal overshoots.
iii. A weakening of a vertical rectus muscle can relieve an upshoot if there
is a primary innervational cause.
iv. Lowering the insertion of the lateral rectus muscle may reduce upshots.
120 Strabismus

v. Splitting of the ends of the lateral rectus into a Y-configuration along


11 with a moderate recession of medial rectus was advocated by Jampolsky.
The lateral rectus muscle should be split into two halves to a point 10mm
posterior to the insertion. The two halves of the lateral rectus muscle
should be reattached to the sclera forming the Y-pattern.
E. Adjustable suture technique: Lateral rectus recession with an adjustable su-
ture technique did not produce favourable results.
F. Botulinum toxin injection: Botulinum toxin injection on ipsilateral medial rec-
tus muscle were ineffective.
Complications
a. Anterior segment ischemia: Vertical rectus muscle transposition with a reces-
sion of medial rectus may produce anterior segment ischemia. If necessary, a
medial rectus recession should be performed as a secondary procedure after 4
months following the transposition.
b. Decrease in adduction and postoperative exotropia: There is a risk of severely
compromising adduction resulting in exotropia after large medial medial rectus
recessions in patients with DRS. If adduction saccadic velocities are very slow
and limitation of adduction is present, recession of medial rectus of the affected
eye should be limited within 5 mm and recession of the opposite medial rectus
should be done to fully correct the esotropia.

BROWN’S SYNDROME (SUPERIOR


OBLIQUE TENDON SYNDROME)
A. Congenital Brown’s Syndrome
This is caused by a short tight superior oblique tendon or sheath that prevents the
eye from moving up in the adducted position. Nodule on superior oblique tendon ,
which prevents its free movements through the tendon, may be another possible
causative factor. It can only be differentiated from an Inferior Oblique palsy by a
forced duction test.
Characteristics
The patients are normally orthophoric in primary position, on down gaze and for
reading. Only when they look up, the affected eye fails to go up properly. In the
adducted position, it does not go up at all and usually goes down on adduction. The
eye can be elevated in the abducted position. There may be widening of the palpe-
bral fissure on adduction. There may be abnormal head posture .In 10 per cent of
cases it may be bilateral. Spontaneous improvement begins around 7 years of age
and most cases show virtually full improvement by 12 to 15 years.
Treatment
If the eyes are orthophoric in the primary position and on down gaze, no treatment
is needed. Some patients have a hypotropia of the affected eye in the primary
Mechanical Restrictions and Syndromes 121

position and preserve their fusion and vision by tilting their up. If there is a hypotropia
of about 10 to 15 prism dioptres in the primary position with the head straight, a
tenotomy of the involved superior oblique tendon and sheath should be performed
11
between the trochlea and the superior rectus muscle. This usually improves the
vertical deviation and the secondary chin elevation. It may also help the inferior
oblique muscle elevate the eye in the adducted position.
Tenotomy of the Superior Oblique Muscle
At first the conjunctiva should be incised 8mm from the limbus between the medial
and superior recti. Then a strabismus hook should be placed under the superior
rectus and the eye should be turned down. Adequate exposure should be achieved
to visualize the superior oblique tendon in its sheath. Two strabismus hooks should
be placed under the superior oblique tendon being careful to include all of the
tendon. The tendon and its sheath are then cut by scissors or cautery with mini-
mum dissection of the sheath or surrounding fascia and check ligaments. This
seems to allow the recovery of superior oblique function a few weeks after the
tenotomy.
B. Acquired Brown’s (Superior Oblique Tendon) Syndrome
This may be due to the presence of an abnormal nodule on the superior oblique
tendon sheath, preventing it from gliding smoothly through the trochlea. This may
result from trauma or an inflammatory disease and may be associated with some
collagen disease. A tight superior oblique tendon from a surgical tuck may also
cause the syndrome.
Treatment
1. Conservative treatment with anti-inflammatory medication should be tried.
2. Injection of steroid in the trochlear region should be tried.
3. Surgical exploration with attempted removal of any nodule on the sheath could
be done.

INFERIOR OBLIQUE PALSY


Inferior oblique palsy presents with the same clinical features as Brown’s syn-
drome except for a normal forced duction test up and in, in the affected eye. If a
negative forced duction test in the field of action of the inferior oblique is seen in
the presence of a hypotropia of 10 to 15 prism dioptres, it is diagnostic of infrior
oblique palsy.
Treatment
Tenotomy of the ipsilateral oblique tendon is indicated.

ORBITAL BLOW-OUT FRACTURE


Blunt trauma over one eye or directly over the maxilla can cause orbital floor or
medial wall fracture with intact orbital rim. There is often trapping of the inferior

http://ophthalmologyebooks.com
122 Strabismus

rectus muscle, inferior oblique muscle or surrounding tissue. Less frequently,


11 paralysis to the nerve to inferior rectus muscle may occur.
Symptoms
1. Vertical diplopia – once reduction in swelling allows opening of the eyes.
2. Altered sensation in the cheek, upper lip and upper teeth on the side of trauma.
Signs
1. Restriction of eye movement, related to the nature of the lesion. Forced duction
test should be done in older patients, which may show mechanical restriction.
2. Confirmation by plain, tilted X-rays. If needed CT scan may be performed.
3. Ocular contusion injury should be looked for.
4. Orthoptic recording of
a. Field of binocular fixation and
b. Hess charting should be done.
Management
Once the oedema and swelling has subsided, if there is more than 3mm. of
enophhalmos,surgery should be done to reduce the herniated orbital tissue.If
enophthal-mos is 2mm. or less ,recording of he ocular movements and the field of
binocular fixation should be done and it should be repeated at 3 to 4 day intervals.
The patient should be instructed not to blow his nose in the meantime. If there is a
history of recurrent sinusitis, a course of antibiotic should be given. If repeated
orthoptic measurements show improving movement the decision on surgery may
be deferred until 14 days from the injury.
Indications for surgery:
a. If there is severe retraction of the eye on attempted elevation or a pressure rise
of more than 6 mm of Hg on attempted elevation
b. Diplopia showing no improvement within 14 days
c. Enophthalmos of 3mm or more and
d. Floor fracture with large herneation of orbital contents into the antrum.
The fractured floor may need to be replaced by a supramid sheet.
Once healing has started, attempts to replace the floor of the orbit and free the
inferior muscle from incarceration are difficult and it is safer to tackle the motility
problems by moving the appropriate vertical rectus muscle involved.

DYSTHYROID EYE DISEASE


Eye signs include proptosis, chemosis, dilated conjunctival vessels, SLK and verti-
cal strabismus.
Limited ocular rotations are due to fibrosis of the muscles. Any muscle may be
affected in one or both eyes, but the inferior and the medial recti are most frequently
involved. Limited abduction occurs in medial rectus tethering, limited elevation
occurs in inferior rectus tethering whereas limited depression occurs in superior
Mechanical Restrictions and Syndromes 123

rectus tethering. CT scan may reveal thickening of the affected muscle, particularly
in the posterior half of the muscle, therefore seen behind the posterior margin of
the globe.
11
Management
1. Fresnel prism may be used to correct the diplopia.
2. Surgery should not be done in the active phase of thyroid disease. Once the
Hess chart and fields of BSV have been stable for at least 6 months, then
muscle surgery of recession of the affected muscle may be carried out. Adjust-
able strabismus surgery is the procedure of choice.

MECHANICAL RESTRICTION FOLLOWING


RETINAL DETACHMENT SURGERY
As the retinal detachment surgery involves an explant with an encircling band
passing under the insertion of the rectus muscles, it may result in squint with or
without mechanical restriction.

GENERAL FIBROSIS SYNDROME


This is a dominantly inherited condition characterized by a general fibrosis of the
muscles of the eye and orbit resulting in ptosis and various strabismic abnormali-
ties. The eyes are pulled down by severe involvement of the inferior recti and
sometimes inward, with an inability to elevate the eye. The patient adopts a marked
chin up position.
Treatment
Recession of both inferior rectus muscles on an adjustable suture gives the patient
maximum area of binocular single vision. Recurrence of hypotropia and esotropia
is common.

PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA


This rare condition usually involves the eye and orbit, resulting in bilateral ptosis
and strabismus associated with a weak muscle.They usually have a poor or absent
Bell’s phenomenon due to the inability of the eyes to move upward properly. Pig-
mentary and other forms of retinal degeneration may be present. Some of these
patients have cardiac conduction abnormalities.
Treatment
Many of these patients can be improved with ptosis and strabismus surgery.

MOBIUS’ SYNDROME
This syndrome is characterized by combined congenital 6th and 7th nerve palsies
in both the eyes. It may also sometimes affect the 3rd nerve. Aplasia of the involved
124 Strabismus

brainstem nuclei has been suggested as the etiology. The patient has restricted
11 horizontal eye movements but better vertical movements.
Treatment
Recession of the medial recti muscles may give some improved cosmesis.

STRABISMUS FIXUS
This rare and presumably congenital condition usually involves fibrous contracture
of both medial rectus muscles, often with fibrotic bands and check ligaments me-
chanically anchoring the eyes in extreme esotropia.
Treatment
Surgical release of the involved muscle and fibrotic bands should be performed
until the forced duction test is normal. The affected muscle should then be recessed
using the adjustable suture hang-back technique. This must be combined with a 10
mm resection of the antagonist lateral rectus muscle in esotropic strabismus fixus.

SUPERIOR OBLIQUE MYOKYMIA


The patient gives a history of torsional and slightly vertical diplopia in the form of
oscillopsia, usually lasting for 1 to 2 minutes. Attacks may vary from once or twice
a week to several times a day. During an attack, the intorsional fine nystagmus
may be seen. The etiology is unknown.
Treatment
a. Carbamazepine has been tried reportedly with some success.
b. Tenotomy of the involved superior oblique combined with myectomy of the
antagonist inferior oblique is reported to be successful.

MYASTHENIA GRAVIS
Intermittent acquired ptosis, diplopia or strabismus are the presenting symptoms.
Unexplained acquired strabismus like isolated medial, superior or inferior rectus
weakness or paralysis may be due to myasthenia gravis. Myasthenia gravis may
also occur in patients with thyroid ophthalmopathy.
12
Non-Surgical
Management of Strabismus

Non-surgical management of the strabismus comprises:


1. Optical treatment.
2. Orthoptic exercises.
3. Pharmacological management.

OPTICAL TREATMENT
Optical management comprises:
1. Use of lenses.
2. Use of prisms.
Use of Lenses
In addition to correction of refractive errors, the spherical lenses can be used to aid
in the management of strabismus.
Spherical lenses induce relaxation of accommodation and thereby influence the
amount of convergence. It also can supplement defective accommodation in
accommodative insufficiency.
a. Convex lenses up to + 3D can be used to control the near deviation in accom-
modative esotropia of the convergence excess type.
b. Concave lenses up to –3D can be used to stimulate accommodation and con-
vergence to control the exotropia for distance fixation in intermittent distance
exotropia.
c. Low-power convex lenses (up to + 2D) can be used for 2 to 3 weeks to assess
whether the patients with esodeviations can obtain binocular single vision with
the lenses.
d. To assess if binocular single vision with a satisfactory near binocular visual
acuity can be achieved in accommodative esotropia with convergence excess
type.
e. In residual esotropia after surgery for a larger esotropia convex lenses can be
added to the patient’s hypermetropic correction after surgery.
f. In consecutive esotropia (< 10-15 prism dioptres)after surgery for an intermit-
tent exotropia, the patient should be observed for 2-3 weeks to allow time for
spontaneous relaxation. If esotropia persists convex lenses can be used.
126 Strabismus

Use of Prisms
12 Types of Prisms
a. Relieving prisms: The objective is to deviate the image of the fixation object
onto or close to the fovea, so that the patient can fuse diplopia and control the
residual deviation. These prisms are commonly used.
b. Exercising prisms: The objective is to deviate the image of the fixation object
away from the fovea, so that the patient has to exert motor fusion to maintain
binocular single vision. They are rarely prescribed, but the principle is used for
exercises to increase the fusional amplitude.
Prisms are Used in the Form of
a. Fresnel prisms: They are applied for temporary use. Though up to 30 prism
dioptres can be applied to either eye, usually more than 20 prism dioptres can
not be worn comfortably.
Application of Fresnel prisms:
i. Prism should be cut in such a way so that it is marginally smaller than the
spectacle lens.
ii. The prism must be placed either vertically or horizontally.
iii. Normally the prism is fitted to the back surface of the spectacle lens.
iv. Care must be taken to ensure that the spectacle lens is clean.
v. Prism strength should be divided between the two eyes unless it is 6 prism
dioptres or less.
b. Clip-on prism: They are used only when the patient can not tolerate Fresnel
prisms. Up to 10 to 20 prism dioptres may be used in each eye.
c. In spectacle frames: If there is high refractive error the spectacle lenses can
be decentred to give the desired prismatic effect. This method is used when
permanent or long-term use is indicated.
Relieving prisms can be used diagnostically or therapeutically.
Diagnostic uses: Prisms are worn for a period of days or weeks in order to assess
the following factors:
1. To assess the state of normal and anomalous binocular function, particularly the
presence or absence of fusion.
2. To assess if the symptoms are due to heterophoria, particularly if associated
with small degrees of esophoria.
3. To determine the type and extent of muscle surgery.
Therapeutic uses: Prisms may be used as a temporary/permanent measure for
treatment of:
1. Diplopia: To enable the patient to fuse the images
2. Symptoms caused by the effort to maintain binocular single vision. Permanent
use of relieving prisms may be indicated in:
i. When a small vertical deviation causes symptoms
ii. When patient refuses or is unfit for surgery
iii. When the deviation is unstable and may change in the future.

http://ophthalmologyebooks.com
Non-Surgical Management of Strabismus 127

ORTHOPTIC EXERCISE
Orthoptic exercises are indicated: 12
1. to compensate a latent strabismus
2. to improve control of an intermittent strabismus
3. as a pre- or post-operative measure in constant strabismus.
Exercises are only given to patients who have or have had normal binocular
single vision. Those with lack of fusion, abnormal retinal correspondence or intrac-
table amblyopia are not suitable candidates. Suitable candidates must fulfill the
following pre-conditions-
a. The deviation must have been acquired after two years of age.
b. The normal sensory and motor fusion must be present.
c. The corrected visual acuity must be equal or near equal.
d. The patient must be in a position to attend the clinic regularly.
e. The patient must be sufficiently cooperative to understand and carry out the
exercises.
Types of Orthoptic Exercises
A.Antisuppression exercises
This treatment is now rarely given as it has debatable value. Suppression is the
patient’s defence against diplopia. In fact, this adaptive mechanism (suppression)
may be helpful in patients when restoration of binocular function remains
unattainable. Antisuppression therapy is aimed at bringing the image formed on
the suppressed retinal area back into consciousness. Several therapeutic
approaches are used. One consists of forcing the suppressed area to be used
concurrently with the corresponding area of the dominant eye by means of
differential stimulation. This can be achieved by placing a red filter over the
dominant eye and having the patient trace with a red pencil or select red beads
for a necklace. It is important that the color of the filter and the object match so
that perception of the red objects with the eye on which the filter is worn is
totally extinguished.
B. Control of manifest deviation
Exercises to aid control of the deviation is indicated in some types of intermit-
tent strabismus. Essentially, the patient must appreciate when the strabismus is
manifest by recognizing diplopia.
C. Extension of fusional amplitude
a. Prism bar
b. Synoptophore
Positive fusion amplitude (convergence):
This is increased by
i. Increasing the near point of amplitude to 8-10 cm.
ii. Using a prism bar base-out, gradually increasing the prism strength while the
patient fuses the images until he can fuse the images through a 40-45
128 Strabismus

prismdioptres base-out prism at 33 cm and a 20-25 prismdioptres base-out


12 prism at 6 m. Improvement of convergence is indicated in exodeviations.
Negative fusional amplitude (divergence):
The prism bar is used base-in. The patients with esophoria of the divergence
weakness type often have very poor divergence at 6m. They may be benifited
by using low-powered loose prisms to use at home.
D.Improvement of relative (fusional) convergence
Positive relative convergence: Here, convergence is exerted in excess of ac-
commodation. This component should be improved in exodeviations so that fu-
sional convergence can be used to control the strabismus but sufficient accom-
modation can be exerted to give clear vision at all distances.
Negative relative convergence: Here, convergence is relaxed in relation to the
amount of accommodation exerted. It should be improved in esodeviations so
that sufficient accommodation can be used for clear vision at all distances with-
out associated convergence.
Methods
a. Stereogram cards:
The cards are printed with two figures. By looking in front of the card un-
crossed diplopia is obtained, resulting in four figures. The distance of the
near fixation target can be adjusted until the innermost figures fuse to give a
central figure common to both eyes with two peripheral figures, each seen
by either the right or left eye. The same effect is obtained by fixating on a
distant target beyond the card, obtaining crossed physiological diplopia of the
figures.

Fig. 12.1: Stereogram cards

b. Dot card
A series of small circles is spaced out along a line drawn the length of a card
about 30 cm long. The card is used to improve convergence and positive
relative convergence. The patient first fixates the farthest dot and should
obtain crossed physiological diplopia of the line and the closer dots. He then
fixates each dot in turn, fusing the images. The more remote dots and line
then appear as uncrossed diplopia.
Non-Surgical Management of Strabismus 129

12

Fig. 12.2: Dot card

c. Diploscope
This apparatus consists of a shaft of 27 cm length, having a nose rest at the
proximal and a card at the distal end. A septum with four holes is placed 7
cm in front of the card. The card is printed with the letters DOG. D and O
are visible to the right eye and G and O are visible to the left eye. Red and
green control marks are seen by the right and the left eyes respectively. The
letters on the card can be seen in several combinations through the central
holes of the septum. The control marks are seen through the top and bottom
holes. This apparatus can be used in four positions to exercise positive or
negative relative convergence. When physiological diplopia of the card is
obtained, the unwanted letters are obscured by the septum. The patient holds
the instrument by a handle on the shaft.

Fig. 12.3: Diploscope


130 Strabismus

d. Bar reading
12 Bar-reading is based on crossed physiological diplopia. A subject with bin-
ocular single vision can see the print behind the bar on the left side of the
page with his left eye and the print on the right with his right eye. He should
be able to read without the bar obscuring the print. The patient’s binocular
visual acuity should be tested first at 33 cm. A print which is a size larger
than the binocular visual acuity should be selected and the bar, usually the
‘short thumb’ type, should be held by the examiner in the center of the page
while the patient reads the print. Binocular single vision is present if the
patient reads as well with the bar in position as he does without it. Suppres-
sion or a manifest strabismus is present if the bar covers the print.
The purposes of bar-reading in treatment are:
i. To exercise binocular single vision when reading: The patient wears
his refractive correction and reads the print using the bar as a control.
Provided that two bars are seen and the print is not obscured, he is
reading correctly.
ii. To improve relative convergence: Negative relative convergence-Con-
cave lenses are used to increase the amount of accommodation exerted.
Large prints are used initially, the print- size is then gradually reduced as
the binocular visual acuity improves.
Positive relative convergence: Additional convex lenses are used to
relax accommodation. Convergence remains static for the distance of
the page.

Fig. 12.4: Bar reading

e. Remy separator
This instrument is based on foveal projection rather than physiological diplopia.
It consists of a septum which rests on the nose. Transparencies are placed
in the holder at the opposite end of the apparatus. The transparencies present
either dissimilar simultaneous perception targets or fusion targets. The patient
looks through the transparencies and relaxes his convergence, causing the
images to fall on the foveae, where they should appear superimposed or
fused. If they are clearly seen, negative convergence is exercised.
Non-Surgical Management of Strabismus 131

12

Fig. 12.5: Remy separator

PHARMACOLOGICAL MANAGEMENT
The drugs used in the management of strabismus are miotics, cycloplegics and
botulinum toxin (described in the chapter Surgical management of Strabismus).
Miotics
Miotics cause constriction of the pupil and act on the ciliary muscle, either by
facilitating neuromuscular transmission in the case of cholinesterase inhibitors, or
by causing an accommodative spasm in the case of parasympathomimetics.
Therapeutic effect of of miotics is based on the facilitation of accommodation; the
accommodative effort is reduced , and less accommodative convergence occurs.
Longer-acting cholinesterase inhibitors (DFP, phospholine iodide) are preferred
than short- acting drugs (physostigmine, pilocarpine). Miotics may change esotropia
to esophoria at near fixation, thereby helping development of fusional amplitudes.
Miotics may be used in esotropic infants to differentiate between nonaccommo-
dative and refractive and nonrefractive accommodative esotropia. Failure to
respond to miotics is a consistent feature of nonaccommodative esotropia.
In the therapy of accommodative esotropia, with high AC/A ratio, miotics are
occasionally used in children who do not tolerate glasses and refuse to use it.
Postoperative miotic therapy is used to reduce a residual esodeviation in patients
with acquired esotropia who have a good potential for restoration of normal
binocular function.
For the treatment of amblyopia, Knapp advocated miotics in the amblyopic eye
and atropine in the sound eye.
Side effects: Anticholinesterase drugs reduce cholinesterase in red blood cells.
Cholinesterase is required for hydrolysis of succinylcholine, a drug used by some
anesthesiologists to facilitate intubation. In cases with reduced cholinesterase level,

http://ophthalmologyebooks.com
132 Strabismus

prolonged respiratory paralysis may occur. So, succinylcholine should be avoided


12 in patients treated with anticholinesterase drugs within 6 weeks before surgery.
Other systemic side effects include perspiration, nausea, vomiting, excessive
salivation, frequent micturition, diarrhoea and abdominal cramping.
Ocular side effects include transient blurring of vision and development of iris
cysts. 2.5 per cent Phenylephrine ,if used in combination with DFP or echothiophate
iodide prevents formation of iris cysts. Iris cysts regress spontaneously after therapy
is discontinued.
Cycloplegics
One per cent Atropine is recommended in combination of overcorrection of a
hypermetropic refractive error for treatment of accommodative esotropia.
Atropine is also used in the treatment of amblyopia (Penalization).
13
Surgical Management of Strabismus

AIMS AND OBJECTIVES OF SURGERY


To straighten the eyes for functional and/or cosmetic reasons and to relieve symp-
toms
a. Accurate alignment is indicated in a concomitant strabismus with normal sen-
sory and motor fusions or an acquired paralytic strabismus
b. Undercorrection is indicated when operating on esotropia for cosmetic reasons,
in many congenital cranial nerve palsies and in dysthyroid eye disease.
c. Overcorrection is indicated in intermittent exotropia
Operations are of Three Main Types
1. Weakening
a. Recession: The muscle is disinserted and reattached to the globe at a posi-
tion posterior to its original insertion.
b. Myotomy: The muscle is incised or completely transected (cut across).
c. Myectomy: A portion of the muscle is excised.
d. Tenectomy: A portion of the tendon is excised as might be done with supe-
rior oblique.
e. Tenotomy: A tendon is completely transected as might be done with supe-
rior oblique.
f. Marginal Myotomy: The muscle is weakened by reducing the number of
contractile elements without changing its arc of contact with the globe.
g. Post Fixation Suture: The muscle is sutured behind the equator to the sclera,
which serves to selectively weaken the muscle in its primary field of action
2. Strengthening
a. Resection: The muscle is disinserted and then a portion of it is excised and
reattached at the site of the original insertion.
b. Tucking: The muscle is shortened by double-breasting the tendinous/muscu-
lar part.
c. Advancement: The muscle is disinserted and then re-inserted closer to the
limbus, thus stretching the muscle and making it more taut and effective.
3. Transposition operations

GENERAL PRINCIPLES REGARDING


RESECTION-RECESSION SURGERY
1. First, we must consider that how many muscles need surgery. The angle of
deviation determines the number of muscles to resect or recess. Whereas a
134 Strabismus

small-angle strabismus (<20PD) may be connected by operating on one muscle


13 2.
only, a large deviation may need surgery on three or four muscles.
When doing a recession-resection procedure, the recession is performed first.
In a resection the muscle is shortened and then brought forwards to the insertion.
This procedure causes tension on the resected muscle, making it difficult to
bring the resected muscle to the insertion site. If recession is done first it decreases
the tension pulling the globe away from the resected muscles and makes it
easier to bring the resected muscle to the insertion site and to tie the sutures on
the resected muscle.
3. When performing surgeries on an oblique muscle and a rectus muscle of the
same eye simultaneously it is preferable to operate on the oblique muscles first.
The oblique muscles are more difficult to identify and isolate by the muscle hook
than the recti. Strabismus surgery causes swelling of Tenon’s capsule and bleeding
may obscure the view and may make oblique muscle identification difficult.
4. More than two recti muscles are not operated in one eye at the same time to
avoid anterior segment ischemia.
5. A recession-resection operation on the same eye tends to correct the same
amount of deviation at distance and near. Symmetrical surgery on medial recti
tends to produce a greater effect at near than distance. Symmetrical surgery on
the lateral recti tends to correct more in the distance compared with the near
measurements.
6. Surgery of a muscle, that has had several previous operations, gives more effect
compared with surgery on previously unoperated muscles.
7. Recession of the tight muscle associated with a positive forced duction test
gives much more effect than surgery on a muscle without associated mechani-
cal restriction.
8. In cases of oblique muscle overaction, the appropriate oblique muscle should be
weakened. Weakening of the inferior obliques correct a V pattern, whereas
weakening of the superior obliques correct an A pattern.
9. In cases without oblique muscle dysfunction, the horizontal recti are “ supraplaced’
or “infraplaced”. The medial recti are displaced toward the point of the A or V,
whereas the lateral recti are moved in the opposite direction.
Preoperative Assessment
1. The history and a detailed motility evaluation, often repeated several times to
corroborate the earlier findings, and a complete ocular examination should be
done. Measurement of the angle of deviation for near and distance and in other
directions of gaze should be taken when indicated. Accurate measurement of
torsion in paralytic strabismus is important. The evaluation should include sen-
sory testing, forced duction testing, active force generation testing, saccadic
velocities and diplopia visual fields.
2. Estimation of binocular function.
Surgical Management of Strabismus 135

3. Whether postoperative diplopia is likely to occur in patients undergoing cos-


metic surgery. 13
Anesthesia for Extraocular Muscle Surgery
General anesthesia is essential for the children and is commonly preferred for
adults.It also allows a fundus examination and cycloplegic retinoscopy. Local
anesthesia may be used in adult patients if general anesthesia is contraindicated.
Injections of local anesthetic (lignocaine hydrochloride 2% with adrenaline) into
the muscle sheaths and retrobulbar spaces induce a more profound anesthesia.
Suture Material
6-0 Polyglactin 910 (Vicryl) or polyglycolic acid (Dexon) sutures are routinely
used. They takes months to get absorbed completely and rarely cause significant
tissue reaction. If allergic reactions occur, 5-0 plain or 6-0 chromic catgut can be
substituted. If allergy to catgut sutures occur, 6-0 white silk or 5-0 white Dacron
may be used.
Needles
A spatula needle is the safest needle to use for surgery.

GUIDELINES FOR SURGICAL CORRECTION


The results of strabismus surgery are not precisely predictable in terms of prism
dioptres correction per millimeter of recession or resection. The dose-response
tables derived from retrospective analysis of surgical outcomes and described by
different authors though give the impression of accurate predictability of surgical
effect are rather useless because it is often seen that an identical operation, per-
formed by the same surgeon on several patients with apparently similar conditions,
may give different results in each patient. These tables may serve as a general
guideline. The reason for variability of surgical results depends on numerous and
only partially known factors. The sensory state of the patient, the operative tech-
nique, the manner in which the muscle is exposed, how thoroughly it is freed, its
stiffness, whether the check ligaments are severed, the placement of sutures, the
occurrence of intra- and postoperative bleeding, the tendency to form adhesions
and scarring, the state of conjunctival elasticity , and the anatomical variations of
the muscle insertions are some of the variables that may influence the effect of the
operation.
Following nomograms are given only as a general guideline.
Deviation Magnitude

Esotropia > 20PD


Exotropia > 25PD
Vertical Tropia > 10PD
136 Strabismus

Deviation smaller than these may require surgery if the patient’s motor fusion
13 ability remains limited in spite of therapy, if annoying symptoms persist or if there is
continued regression from heterophoria to strabismus.

THE EFFECT OF SURGERY (RECESSION OR RESECTION)


ON PREVIOUSLY UNOPERATED HORIZONTAL MUSCLES
This should act as a guideline for the effect of surgery on the previously unoperated
extraocular muscle.
Amount of surgery (m.m.) Amount of correction
1-5 2.5 PD/m.m.
6-7 3PD/ m.m.
8-9 4PD/ m.m.
10 5PD/m.m.

THE EFFECT OF SURGERY ON PREVIOUSLY


UNOPERATED VERTICAL MUSCLES
Amount of surgery (m.m.) Probable correction of deviation
1-8 3PD/m.m.
>8 No further effect

Following Points Must be Noted


A.The effect on the horizontal muscles increases with the amount of surgery
done, once it exceeds 5mm.
B. A recession or resection operation on the same eye tends to correct the same
amount of deviation at distance or near measurements. Symmetrical surgery on
the medial recti tends to produce a greater effect at near than distance
measurements. Symmetrical surgery on the lateral recti tends to produce more
effect in the distance than near measurements.
C. Single muscle surgery may be used for small deviations. Recessions of one
muscle larger than 7mm may give rise to incomitance in the field of action of the
muscle. Resections do not produce this effect.
D.Advancement, recession or resection of a muscle, that has had several previous
operations, gives more effect compared to surgery on previously unoperated
muscles.
E. Recession of the tight muscle associated with a positive forced duction test
gives more effect than surgery on a muscle without associated mechanical
restriction.
F. Recession or resection of the previously unoperated vertical rectus muscle is 3
prism dioptres per millimeter upto 8mm. A larger recession than 8mm does not
produce further effect.
G. There is usually more weakening effect in the field of action of the recessed
vertical muscle if it is recessed more than 5mm, particularly in case of inferior

http://ophthalmologyebooks.com
Surgical Management of Strabismus 137

rectus. The expected result from recession of inferior rectus muscle should be
calculated on 3 prism dioptres/millimeter in the primary position and 5 prism
dioptres/millimeter in the down gaze position.
13
H.The sclera is thinnest (0.3 mm) at the insertion site of recti. So, at least, 0.5 mm.
of muscle stump should be left for resection resuturing.
I. When grossly different results are noted the possibility of some abnormal muscle
tone (e.g., cerebral palsy) should be considered.
For esodeviations either symmetrical or asymmetrical surgeries may be per-
formed.
A.Symmetrical surgeries: Either bilateral medial rectus recession or bilateral lateral
rectus resection should be performed.
Table 13.1: Esodeviation: Symmetrical surgery
(Bilateral MR recession or LR resection)

Angle of deviation MR recession (mm) LR resection (mm)


15PD 3.0 4.0
20PD 3.5 5.0
25PD 4.0 6.0
30PD 4.5 7.0
35PD 5.0 8.0
40PD 5.5 8.0
50PD 6.0 9.0

B. Recession and resection surgeries: Recession and resection operations should


be performed in one eye only.
Table 13.2: Esodeviation: Monocular recess-resect procedures

Angle of deviation MR recession (mm) LR resection (mm)


15PD 3.0 4.0
20PD 3.5 5.0
25PD 4.0 6.0
30PD 4.5 7.0
35PD 5.0 8.0
40PD 5.5 9.0
50PD 6.0 9.0

For exodeviations either symmetrical or asymmetrical surgeries may be per-


formed.
A.Symmetrical surgeries: Either bilateral lateral rectus recession or bilateral medial
rectus resection should be performed.
Table 13.3: Exodeviation symmetrical surgery

Angle of deviation Bilateral recession (mm) LR Bilateral resection (mm) MR


15PD 4.0 3.0
20PD 5.0 4.0
25PD 6.0 5.0
30PD 7.0 6.0
40PD 8.0 6.0
138 Strabismus

B. Monocular recession and resection surgeries: Recession and resection


13 operations should be performed in one eye only.
Table 13.4: Exodeviation: Monocular recess+resect procedures

Angle of deviation LR recession (mm) MR resection (mm)


15PD 4.0 3.0
20PD 5.0 4.0
25PD 6.0 5.0
30PD 7.0 6.0
40PD 8.0 6.0
50PD 9.0 7.0
60PD 10.0 8.0
70PD 10.0 9.0
80PD 10.0 10.0

Weakening Operations
1. Recession Operations
The aim of recession operation is to weaken the muscle without causing limitation
of movement. The insertion of the muscle is moved closer to its point of origin in
the same plane, producing a slackening of muscle fibers and weakening of the
action of the muscle. There is a linear relationship between the weakening effect
and the amount of recession until the new insertion coincides with the tangential
point of the globe when the eye moves into the field of action of the recessed
muscle. Recession beyond this point causes mechanical restriction of movement.
When movement is limited by mechanical contraction, recession of the contracted
muscle will reduce the limitation of movement by a progressive amount.
Steps: Sutures should be placed close to the tendinous insertion of the exposed
muscle.
The muscle should be detached by transecting the tendon as close to its inser-
tion as possible (Fig. 13.1a). This minimizes bleeding, makes the measurements
more accurate and avoids an ugly muscle stump.The desired amount of recession
should be measured out using the callipers (Fig. 13.1b) directed posteriorly from
both ends of the original insertion and the sclera should be marked at the intended
re-insertion points. The rectus tendon should be re-inserted parallel to its original
attachment. The two needles of a double-ended interwoven suture should pass
transversely through the sclera to emerge adjacent to each other along the line of
intended reattachment and tied (Fig. 13.1c).
Large Amounts of Recession have been Proposed
in the Following Conditions
a. Lateral rectus paralysis: recession of the contracted ipsilateral medial rectus
or contralateral medial rectus,
b. Duane’s syndrome: recession of contralateral medial rectus,
c. Esotropia associated with a blind microtropic eye.
Surgical Management of Strabismus 139

13

a b

c
Fig. 13.1a, b, c: Recession
d. Congenital nystagmus with a marked face turn: recession of medial rectus in
one eye and lateral rectus in the other,
e. Infantile esotropia: large recession of both medial recti,
f. Ciancia’s syndrome: recession of medial rectus on the side to which the face is
turned; both medial recti may be recessed if the face is turned to either side
g. DVD: recession of the superior recti to prevent the up-drift.
Hang-Back Technique
The insertion of the muscle to be recessed should be exposed and check ligaments
are cut. A 6-0 Vicryl suture with a spatula needle at each end should then be
threaded through the muscle and locked at each border. The muscle should then be
cut from the sclera. The two needles should then be inserted about 4mm apart in
the central portion of the original insertion stump. Using a suture forceps, the muscle
should be pulled forward until the cut border flush with the insertion. A second
suture forceps should then be used to grip both the suture ends at the desired
position measured by calipers, which equals the amount of recession needed. Sev-
eral knots should then be tied at this point. The muscle should then be allowed to
140 Strabismus

slide back and will come to lie against the sclera with the muscle recessed the
13 desired amount. The muscle will grow onto the sclera at this position. In recessions
of more than 8mm., the muscle may creep forward reducing the amount of reces-
sion by 1 to 2mm. In recessions greater than 8 mm., the muscle should be stitched
directly to sclera directly at the measured amount of retroplacement. Insertion of
the sutures, by this technique, minimizes the danger of scleral perforation.
The average effect on vertical recti is only 20 prism dioptres of vertical correc-
tion, that on lateral rectus is about 35 prism dioptres of exotropic correction and
that on the medium rectus is about 140+ prism dioptres which produces a complete
inability to adduct the eye past the midline position.

Fig. 13.2: Hang-back technique

2. Z-Myotomy
This is another weakening operation in which the muscle is lengthened by means
of transverse incisions and thereby weakened, but continues to act from its original
insertion point. The area of the muscle should be first crushed with straight artery
forceps. Three transverse incisions should be made through two-thirds of the muscle
width at 5 mm intervals behind the muscle insertion , two being placed on one side
and the other between them on the opposite side. It is usually used when a muscle
has already been maximally recessed or when reoperation is required. It is of
limited value.

Fig. 13.3: Myotomy


Surgical Management of Strabismus 141

3. Myectomy
This operation is usually performed on Inferior Oblique. The aim is to produce a 13
predictable weakening of the Inferior oblique without altering its line of action. The
muscle should be approached through a circumferential bulbar conjunctival inci-
sion in the lateral part of the lower temporal quadrant (Fig. 13.4a). Tenon’s capsule
should be opened separately Fig. 13.4b). The eye should be retracted upwards and
medially using the muscle hooks inserted under the inferior and lateral rectus inser-
tions. The anterior margin should be identified in the lower temporal quadrant
under direct vision. Dissection should be carried backwards until the posterior
edge of the muscle has been identified. The muscle belly should be hooked for-
wards and separated from its sheath and Tenon’s capsule for a distance of 6 mm.
midway between the inferior border of the lateral rectus and the lateral border of
the inferior rectus (Fig. 13.4c). The muscle should be crushed with artery forceps
and a portion of 4 to 5 mm is excised using hot cautery (Fig. 13.4d).The incision in
Tenon’s capsule and conjunctiva are then closed. Great care must be taken not to
damage the inferotemporal vortex vein while dissecting out and hooking forward
the inferior oblique. As the muscle belly is very vascular, thorough crushing and
cauterization is essential before complete division in order to avoid a postoperative
hematoma, which may result in the development of adhesions and mechanical
tethering.
Effect of Myectomy of one Inferior Oblique
If 4 to 5mm of muscle belly is excised midway between the inferior rectus and
lateral rectus, 5 to 20 prism dioptres of correction can be expected in the primary
position. The muscle grows on to the sclera at just the righ position to reduce the
overaction in adduction and the hypertropia in the primary position to within the 5
to 20 prism dioptres range. Near-normal action of the inferior oblique is usually
restored within one month after surgery.

Fig. 13.4a: Inferior oblique myectomy

http://ophthalmologyebooks.com
142 Strabismus

13

b c

upper

lower
d

Fig. 13.4b, c, d: Inferior oblique myectomy

4. Tenotomy or Tenectomy
This operation is usually performed on Superior Oblique muscle. Division of the
tendon can be carried out nasal or temporal to the superior rectus. Division in the
upper nasal quadrant is preferable as the lateral approach has a uncertain due to
the fact that the cut tendon can be reattached to the superior rectus sheath or to
the globe anterior to the equator. The tendon should be identified just medial to the
superior rectus. The tendon within its sheath should be brought forward on a muscle
hook. The intermuscular septum should be incised longitudinally and the exposed
tendon should be completely divided within the septal sheath. The cut ends usually
retract 2 to 4mm from each other from each other without change alignment. The
effect of the surgery is more profound if the surrounding tendon sheath is also
divided.
The indications of the operation are—Brown’s- syndrome, A-exotropia (bilat-
eral superior oblique tenotomy), complete 3rd nerve palsy.
Surgical Management of Strabismus 143

13

a b
Fig. 13.5a, b: Superior oblique tenotomy

5. Faden Operation (Posterior Fixation Suture)


The aim of this operation is to produce progressive weakening as the eye moves
into the field of action of the operated rectus muscle. This operation involves binding
the muscle belly to the sclera as far back as is surgically possible. This procedure
retroplaces the effective insertion, thereby decreasing the muscle power in the
field of action of the muscle without affecting the position of the eye in the primary
position. It can be combined with a recession of required amount if some correction
is needed in the primary position. Possible hazards include inadequate exposure,
resulting in tearing a vortex vein or perforating the eye ball.
Indications
i. High AC/A ratio (sutures on both medial recti),
ii. DVD (suture on superior rectus),
iii. Vertical strabismus present only in down gaze (suture on inferior rectus of the
hypotropic eye),
iv. Incomitant strabismus (suture on the appropriate rectus muscle for the desired
weakening effect).

Fig. 13.6: Faden operation


144 Strabismus

Strengthening Operation
13 1. Resection
The muscle is shortened and therefore strengthened and maintains its action through
the point of original insertion. The sutures should be woven and locked transversely
through the muscle belly at the required distance behind the insertion. The portion
of the muscle infront of the sutures should be crushed and the shortened muscle
should be reattached to the original insertion.

a b

c d

Fig. 13.7a, b, c, d: Resection

2. Advancement
Advancement operation is now only rarely carried as the muscle becomes visible
through the conjunctiva after surgery.
Surgical Management of Strabismus 145

13

a
b

Fig. 13.8a, b: Advancement

3. Tucking (Plication)
Tucking strengthens the muscle’s action by folding the terminal portion of the muscle
and tendon, thereby decreasing the muscle length. The muscle is not disinserted.
Mattress sutures should be passed through the muscle belly at the required point
and locked before being passed through the tendinous insertion, tightened and tied.

a b

Fig. 13.9a, b, c, d: Tucking


146 Strabismus

The tendon then forms a tuck superficial to the plane of the muscle. The muscle
13 should not be shortened by more than 3 to 4 mm, otherwise an ugly raised fold of
muscle forms under the conjunctiva. The use of permanent sutures causes a risk
of granula formation and of long-term suture migration and erosion through the
conjunctiva. Plication of the rectus muscles is of very limited value and is rarely
performed. Advancement and resection operations of superior technically difficult
and tendon plication is widely used in this case.
Transposition Operations
A. Whole Muscle Transposition
Either the horizontal or the vertical recti can be transposed. Muscles can be trans-
posed singly , in ipsilateral pairs or in contralateral pairs. Ipsilateral pairs are trans-
posed in order to rotate the eye in the direction of transposition. Contralateral pairs
are transposed symmetrically in order to alter V and A patterns. The tendon is
disinserted and re-attached in the indicated position. To change an V or A pattern,
a half to full rectus muscle’s width of transposition may be needed. To assist the
action of an adjacent muscle, a minimum of a whole rectus muscle width transpo-
sition is needed. In cases of severe limitation of ocular movement the transposed
muscle should be reinserted alongside the adjacent rectus insertion. Care should
be taken to transpose pairs of muscles an equal amount, otherwise, muscle imbalance
is created, which can be an obstacle to binocularity. This surgery is indicated in
i. V and A patterns,
ii. Double elevator palsy (medial and lateral recti are reinserted alongside the
medial and lateral borders of the superior rectus insertion),
iii. Lateral rectus palsy (superior and inferior recti are transposed to the upper
and lower poles of lateral rectus insertion),
iv. Duane’s syndrome – Type A (same as lateral rectus palsy) and
v. Lost muscle (two adjacent rectus muscles) can be transposed to the insertion
of the severed muscle.
B. Part-Muscle Transposition
Here, only half the muscle is mobilized and transposed, reducing the risk of ante-
rior segment ischemia. Usually two muscles are transposed to the third.
Types
a. Hummelsheim’s operation
This procedure is used in lateral rectus paralysis. The lateral halves of the supe-
rior and inferior recti are disinserted and the medial and lateral fibers are sepa-
rated for a distance of about 15 mm behind their insertions. The lateral halves of
each muscle is reinserted alongside the upper and lower poles of the lateral
rectus. The contracted ipsilateral medial rctus is also recessed. This operation
may also be used in double elevator palsy (upper halves of horizontal recti are
transposed to the borders of superior rectus).

http://ophthalmologyebooks.com
Surgical Management of Strabismus 147

b. Jensen’s operation
Here, transposition is done without disinserting the tendons of the muscles
involved, thus maintaining the anterior segment blood supply. Three adjacent
13
recti are split in half along the line of their muscle fibers from just behind the
insertion to a point 15 mm posterior to it. If this procedure is used in lateral
rectus palsy, the lateral halves of the superior and inferior recti are tied fairly
loosely to the upper and lower halves of the split lateral rectus, using strong non-
absorbable sutures. The ties are made 12 mm behind the limbus. The sutures
should be tied loosely in this operation to avoid crushing the muscle fibers and
causing ischemia, but should be tight enough to prevent the the suture loops
migrating gradually from their original positions.

b
a

c
Fig. 13.10: Jensen’s operation

Superior Oblique Muscle Transposition (Harada-Ito Procedure)


The anterior half of the superior oblique tendon is disinserted and split from the
posterior portion along the line of its fibers for 10 mm. The mobilized portion is then
reattached 8 mm posterior to the lateral rectus insertion and just above that muscle’s
upper margin. Bilateral reinsertion at the lateral rectus upper border corrects 15 to
20 degrees of excyclodeviation, while bilateral reattachment 4 mm above the border
148 Strabismus

corrects 68 degrees of excyclodeviation. This is the operation of choice in patients


13 with unilateral or bilateral superior oblique palsies whose symptoms are mainly due
to excyclodeviation.
Inferior Oblique Muscle Transposition (Desagittalization)
The extorting action of the inferior oblique can be enhanced and the elevating and
abducting actions decreased if the muscle is disinserted and reattached in a more
transverse direction relative to the anteroposterior axis of the globe. It can be
achieved by total disinsertion and reattachment of the anterolateral pole of the
muscle insertion at the equator of the globe midway between the lateral and infe-
rior recti.
Vertical Strabismus Surgery
A. Horizontal Muscle Surgery for Vertical Strabismus
Surgically both medial and lateral rectus tendons in the same eye raises that eye
raises that eye relative to the other eye slightly and lowering them has the opposite
effect. 1 prism dioptre of vertical correction occur for each millimeter both ten-
dons are raised or lowered to a maximum of about 6 prism dioptres.
B. Surgical Correction of Vertical Strabismus Remaining in Down Gaze
After one or more operations have been performed for a vertical strabismus, if
further conventional cyclovertical muscle surgery would not reduce the problem,
these procedures may be considered:
a. A posterior fixation suture placed as far back as possible on the inferior rectus
muscle on the eye hypotropic in down gaze. This will weaken the inferior rectus
in down gaze, thereby reducing the hypotropia in down gaze upto 10 prism
dioptres. Injury to vortex veins should be avoided.
b. Adjustable recession of both vertical recti in the hypotropic gaze: The
remaining muscle imbalance in position other than down gaze, including head
tilting, should be concomitant within 5 prism dioptres. The inferior rectus is
recessed on an adjustable suture calculating an effect of 3 prism dioptres per
millimeter in the primary position and 5 prism dioptres per millimeter in down
gaze. Then the superior rectus is recessed on an adjustable suture to neutralize
the resultant hypertropia in the primary position.
Conjunctival Closure
The conjunctival incisions are best closed by 6-0 or 8-0 synthetic absorbable sutures.
Care must be taken not to trap any Tenon’s capsule in the incision line which may
result in granuloma formation. Bulky knots at the limbus may interfere with corneal
tear film and cause ‘dellen’ formation.
Adjustable Sutures
The ability to modify the position of a newly operated muscle by the use of an
adjustable suture gives more control over the final outcome of the surgery. Stable
Surgical Management of Strabismus 149

postoperative adjustment is mainly limited to recession operations. The sutures


allow adjustment of the amount of recession when the effects of the anesthetic
have worn off but before the healing process has started
13
Colored sutures are preferred as they are easier to identify. The conjunctiva
should be opened with a limbal incision. A 6-0 Vicryl suture with a spatula needle
at each end should then be threaded through the muscle and locked at each border.
The muscle should then be cut from the sclera. The two needles should then be
inserted about 4mm. apart in the central portion of the original insertion stump. The
muscle should be allowed to hang back the desired amount and should be secured
temporarily in this position by a double throw knot and a single bow. The conjunc-
tival flap should be left sufficiently open to be sutured under topical anesthesia
after tying off the adjustable suture in the final desired position at the time of final
posterative adjustment.
In adjustable surgery of the superior rectus, it should be ensured that the knot
and bow temporarily securing the muscle are within 3mm. of the superior limbus to
facilitate the final adjustment.At the time of adjustment, attention should be paid to
which eye is fixing. I. Patients without fusion, fixation with the dominant eye must
be ensured. If the optical correction makes a difference to the deviation, the patient
should wear them at the time of adjustment.
Patients may have their sutures adjusted anytime from immediately on awaken-
ing from the anesthetic to 24 hours later. Preferably it should be done a few hours
post-operatively.
Complications of Strabismus Surgery
1. Ocular alignment problems:
Some patients are under- or overcorrected. Using the adjustable suture technique,
the outcome of the surgery is much better than the fixed suture technique.
Although the immediate postoperative alignment is satisfactory, if no fusion is
present the eye may deviate again after just a few weeks.
2. Diplopia:
Postoperative diplopia may occur in some cases.
3. Conjunctival complications:
i. Prolapse of the Tenon’s capsule: A small piece of Tenon’s capsule may
prolapse through the conjunctival wound. It appears much more white in
contrast to the conjunctiva and is usually cosmetically obvious. Careful clean-
ing of Tenon’s capsule from the operative site with good wound closure of
the conjunctiva is necessary. If it is noted post-operatively, it is wise to wait
for a few days as Tenon’s capsule may shrink back inside the conjunctiva.
Otherwise, it should be excised.
ii. Suture granuloma: Sometimes, an inflammatory, slightly tender lump over
the suture site may develop within one or two weeks of surgery. It is a
foreign body reaction either to the suture itself or some other foreign material
like cotton wool fibres. Initially corticosteroid drops should be instilled. If it
150 Strabismus

fails to bring improvement, removing the suture and excising the small lump
13 should be done if it is ten days after the surgery when the muscle should be
firmly adherent to the sclera.
iii. Suture abscess: It is an abscess localized to the suture knot may occur
within a few days of surgery. The ends of any protruding suture should be
trimmed. A smear and culture should be taken before starting topical and
parental antibiotics. If the abscess is not resolved in two days, the suture
removal should be considered except in the case of medial rectus, where
drainage should be done and the suture may be removed after tenth post-
oprerative day. Topical corticosteroid should be started. Resolution usually
occurs within ten days.
iv. Allergic reaction to sutures: It may occasionally occur with polyglycolic
acid sutures.
v. Cysts: Epithelial inclusion cysts may arise at the site of the conjunctival
incision. A large cyst may be excised.
vi. Mobile conjunctiva: If the conjunctiva fail to permanently adhere to the
underlying tissues and move around with the eye movements a small ellipse
may be excised and the conjunctiva may be sutured to the underlying sclera.
vii. Conjunctival scarring and the plica: Though a permanent scar remains
following conjunctival incision, it is usually symptomless and remains
unnoticeable. Sometimes, particularly following multiple procedures, increased
conjunctival vascularization may result in obvious scarring. Sometimes,
conjunctival scarring involving the region between the limbus and the plica
draws out the medial canthal area toward the limbus, forming a very obvious
reddish lump. The lump should be excised and the area should be covered
with conjunctiva brought down from the adjacent areas.
viii. Prolapse of the fat pad may occur subconjunctivally causing subconjunctival
lumps and conjunctival congestion and inflammation, if the Tenon’s layer is
broken. The conjunctiva should be opened along the previous scar and the
subconjunctival fat should be excised.
4. Dellen:
Dry spots on the cornea, near the limbus, may be formed by conjunctival folds
which prevents adequate distribution of tear film with blinking in the post-opera-
tive period. Artificial tears may be used temporarily and surgery may be needed
to remove conjunctival folds if Dellen persists.
5. Mechanical restriction:
i. Multiple Surgery: The more operations that are performed on the same eye
muscles, there is more risk of fibrosis and mechanical restriction, particu-
larly if the fat pad has been disturbed.
ii. Restriction of up gaze following inferior oblique muscle surgery
(Adherence syndrome): It rarely occurs after inferior oblique myectomy
surgery. If the fat has been grossly disturbed and much bleeding has occurred,
postoperative scarring may occur resulting in mechanical restriction of the
Surgical Management of Strabismus 151

eye moving upward within few days after surgery. The treatment should be
a recession of inferior rectus muscle in the affected eyes calculating 3 prism
dioptres per millimeter of effect for the hypotropia in the primary position.
13
6. Lost muscle:
Medial rectus is the only muscle that creates a major problem when it slips back
into the orbit either at the time of surgery or postoperatively. The other muscles
are easy to recover if they slip due to the fascial connections between the
inferior rectus and inferior oblique, and the superior rectus, levator, and the
superior oblique.
The only cure is to find out the lost muscle. The following measures are
helpful to find out a lost muscle-
i. A CT scan can locate the muscle.
ii. A large incision just on the limbal side of plica should be given. The muscle
should be searched by taking sufficient time. The anesthetist should be asked
to avoid atropine preoperatively so that vasovagal oculocardiac reflex may
be observed if a structure that might be the muscle is pulled. If the heart
slows considerably, it is highly probable, that the muscle is being pulled.
Once the medial rectus muscle has been secured, it should be sewn back
onto the sclera with the eye with the eye held in full abduction so that the
muscle is fully stretched to prevent a mechanical limitation to abduction.
7. Postoperative infection:
i. Conjunctivitis: Postoperative infection of conjunctiva may occur. To pre-
vent this topical antibiotic should be given after surgery as a prophylaxis.
ii. Orbital Cellulitis: If there is postoperative conjunctivitis, infection may spread
to the orbit along the conjunctival wounds.
iii. Endophthalmitis: It may occur very rarely following perforation of the globe.
8. Perforation of the globe
The sclera may be perforated alone by the passage of the needle or all layers
may be perforated. If perforation of the globe occurs with leakage of vitreous,
cryotherapy should be done surrounding the perforation. The effect should be
monitored through the dilated pupil by indirect ophthalmoscopy. A culture should
be taken from the lashes and the conjunctiva and appropriate broad spectrum
antibiotic should be lavaged around the perforation site.
9. Damage to a vortex vein:
If a vortex vein is torn, it will bleed profusely, if it can not be cauterized success-
fully it should be tied off using a fine absorbable suture.
10. Anterior segment ischemia:
The blood supply of the anterior segment of the eye is done by the major arterial
circle of iris, formed by the seven penetrating anterior ciliary arteries and two
posterior ciliary arteries. The lateral rectus insertion contains ciliary vessel and
each of the other three recti contain two, those of the vertical recti being situ-
ated in the medial halves of the muscles. Disinsertion of these recti divides
these anterior ciliary arteries, and the loss of blood supply risks inducing anterior

http://ophthalmologyebooks.com
152 Strabismus

segment ischemia.Operating on three rectus muscles at a time may result in


13 Anterior segment ischemia. It is more likely to occur in persons over 60 years of
age and is very rare in children. It is more likely to occur in patients with vascu-
lar problems and arteriosclerosis and those patients who are getting operated
for strabismus following retinal detachment surgery with an encircling band. If
more than two muscles need to be operated on in one eye, it should be done at
two separate operations, doing two muscles initially and the other muscle or
muscles after at least a 4 month interval. Anterior ciliary arteries should be
dissected out and spared, if possible. The patient presents within 24 hours of
surgery with pain and blurred vision. The eyelids, conjunctiva and deep layers of
cornea all becomes oedematous. The pupil is dilated with a diminished light
reflex and is often irregular in shape. The anterior chamber is deep with a heavy
flare and circulating pigment. The vitrous is hazy and the intraocular pressure is
low. Corticosteroids should be used topically and systemically.
11.Malignant hyperthermia:
It is a rare hereditary disorder characterized by a fulminant hypermetabolic
state of skeletal muscle. It may be precipitated by general anesthesia and, per-
haps, by stress or exercise. Halothane and succinylcholine are known triggering
agents. The diagnosis is confirmed by a positive halothane-caffeine contracture
test on biopsied skeletal muscle. Otherwise unexplained tachycardia, tachyp-
nea, muscle rigidity, cardiac arrhythmias and pyrexia are observed while under
anesthesia and a coincident metabolic and respiratory acidosis is identified. The
treatment includes immediate elimination of all triggering agents and hyperven-
tilation of the patient with 100 per cent oxygen. Intravenous dantrolene sodium
should be given. The metabolic acidosis should be corrected and active cooling
measures begun.Cardiac arrhythmias, if persistent, should be treated by intra-
venous procainamide.
12.Oculocardiac reflex:
The oculocardiac reflex is a slowing of heart rate that is caused by traction on
the extraocular muscles. In its most severe form it can produce asystole. If
heart rate is dropped excessively during surgery, tension on the muscle should
be released immediately. Intravenous Atropine can protect against oculocardiac
reflex.
Chemodenervation Treatment of Strabismus using Botulinum Toxin
Purified Botolinum toxin A (Botox), a protein drug produced from Clostridium
botulinum, when injected directly into the selected extraocular muscles localized
with a portable electromyographic device, is bound and internalized in 24 to 48
hours within local motor nerve terminals, where it remains for many weeks and
interfere with the release of acetylcholine. Paralysis of the injected muscle begins
within 2 to 4 days after injection and lasts clinically for at least 5 to 8 weeks in the
extraocular muscles. An extraocular muscle lengthens while it is paralyzed by
Surgical Management of Strabismus 153

botulinum toxin, and its antagonist contracts. These changes may produce long-
term improvement in the alignment of the eyes. Multiple injections are frequently
required for maximal benefit. Best results occur when there is fusion to stabilize
13
the alignment.
Botulinum is most effective in
a. Small to moderate angle esotropia and exotropia (less than 40 prism dioptres)
b. Postoperative residual strabismus
c. Weakening of an antagonist muscle in acute paralytic strabismus (particularly
the 6th cranial nerve) to block its contracture while the agonist recovers
d. Cyclic esotropia
e. Active thyroid ophthalmopathy or inflamed or periphthisical eyes when surgery
is contraindicated.
The dose of Botulinum toxin for treatment of strabismus is from 2.5 to 5.0 units
in the adult. In infants who weigh less than 12.5 Kg, the dose is reduced. Because
placement of needle tip for delivery of Botulinum toxin under electromyographic
guidance requires nerve activity, the injection is accomplished with the patient
awake or, with ketamine anesthesia which preserves electrical activity. Topical
anesthesia in the conjunctiva should be used in all the cases.
14
Nystagmus

Nystagmus is an involuntary, regular, rhythmic, repetitive to-and-fro oscillation of


the eyes. Nystagmus can be physiological, congenital or it may indicate disease of
the visual system, central nervous system or generalized abnormalities. It is mostly
binocular. Rarely it is uniocular as in internuclear ophthalmoplegia. It may be asym-
metric in spasmus nutans.

NYSTAGMUS ARE CHARACTERIZED BY


a. Direction of the fast phase.
b. Type- Jerky (a movement of unequal speed i.e., it has a fast and a slow compo-
nent)/ pendular (equal velocity in each direction).
Alexander’s law: The amplitude of jerk nystagmus is largest in the gaze of
direction of the fast component.
c. Amplitude: Coarse/ fine: Large- > 15 degrees; moderate- 5 to 15 degrees; fine-
< 5 degrees.
d. Velocity: Rapid/slow (expressed in cycles/second or Hertz): Slow- 1 to 2 Hz;
medium- 3 to 4 Hz; fast- > or = 5 Hz.
e. Plane of oscillation: Horizontal/ vertical/ rotatory/retractory.
f. Conjugate/dissociated: Conjugate: symmetric in direction, amplitude and rate.
Dissociate: differs in one or more parameters.

DEGREES OF NYSTAGMUS
First degree: Nystagmus only in the gaze in the direction of the fast component
Second degree: Nystagmus also in the primary position of gaze
Third degree: Nystagmus in addition to the above two gazes is also present in the
direction of the slow component.

CLASSIFICATION
A.Physiologic:
1. End-point nystagmus
2. Vestibular nystagmus (caloric or rotational)
3. Optokinetic nystagmus
4. Voluntary nystagmus
B. Pathologic:
1. Nystagmus associated with poor vision
a. Retinal diseases: Retinoblastoma, retinopathy of prematurity, persistent
hypoplastic primary vitreous, retinal dysplasia, Leber’s amaurosis, tape-
toretinal degeneration, coloboma, retinoschisis, congenital stationary night
Nystagmus 155

blindness, achromatopsia, albinism, intrauterine infection like rubella, toxo-


plasma, etc.
b. Optic nerve hypoplasia
14
c. Anterior segment pathology: cataract, aniridia, mesodermal dysgenesis.
2. Nystagmus associated with neurologic diseases
a. Gaze paretic nystagmus
b. Convergence retraction nystagmus
c. Vestibular
i. Central: Brainstem nuclei
ii. Peripheral: Labyrinthine, vestibulocochlear nerve
d. Downbeat: Cervicomedullary junction
e. Upbeat: Cerebellum(vermis), medulla
f. Seesaw: Parasellar regio
g. Periodic alternating – acquired.
3. Nystagmus associated with strabismus
a. Latent nystagmus
b. Manifest nystagmus
c. Nystagmus blockage syndrome
4. Miscellaneous
a. Spasmus nutans
b. Congenital motor nystagmus
c. Periodic alternating nystagmus

CONGENITAL NYSTAGMUS
Congenital nystagmus can be sensory or motor.
A.Sensory Nystagmus: The cause of sensory nystagmus is loss of foveal fixation
due to an organic abnormality of the media, macula, retina, or optic nerve.
Etiologies include foveal hypoplasia associated with albinism, congenital cataracts,
aniridia, macular defects, Leber’s congenital amaurosis, congenital stationary
blindness, or achromatopsia. Patients with sensory nystagmus tend to have poor
vision from organic causes in addition to limited foveation time resulting from
nystagmus.
B. Motor Nystagmus: This term is used when there is no sensory cause. As the
fovea is unaffected, the vision tends to be better. Some of them may even have
6/6 vision.
Characteristics: It is present from early age. It may be pendular initially and jerky
later. Oscillopsia is unusual. It may have a “null point(s)”. The ‘null point/zone’ is a
position of gaze in which the nystagmus is dampened and the patient sees better.
Commonly, the patient adopts a compensatory head posture in order to bring the
eyes into the null zone. All types of nystagmus are reduced by convergence and
increased by attempted fixation. It is abolished during sleep. It is often associated
with squint. It may have added latent nystagmus component. The characteristic
156 Strabismus

abnormalities are inverse fast phase and damping of nystagmus. It may be associ-
14 ated with head nodding (spasmus nutans). Accelerating slow phase is noted on
electronystagmography. The amplitude and frequency of congenital motor nystag-
mus increase with stress, particularly emotional stress.
Spasmus nutans: This syndrome consists of patients with congenital motor nys-
tagmus, who have a nodding of head in addition to an abnormal head posture. The
nodding may be an attempt to increase foveation. It varies and is increased when
the patient is under stress, particularly emotional stress. It may persist into adult-
hood. Occasionally, the nystagmus is more prominent in one eye and may be called
dissociate nystagmus. There is an occasional case of head nodding that is caused
by intracranial neoplasm, such as optic glioma. This is associated with nystagmus
acquired after 4 months of age. A CT scan should be done.
Motor nystagmus without strabismus: The nystagmus is usually present from
birth. There may be a family history of nystagmus and the hereditary pattern may
vary. There may be a good fusion and stereopsis.
Motor nystagmus associated with the congenital nystagmus syndrome: About
50 per cent of the patients with the congenital esotropia syndrome have nystagmus.
It may manifest itself as the nystagmus block syndrome in congenital esotropia.

LATENT NYSTAGMUS
Characteristics: It presents with bilateral congenital jerk nystagmus. The nystagmus
is more obvious on occluding one eye. It typically increases with abduction of
uncovered/fixing eye. It may have head turn towards the fixing eye. Tiny oscillations
are present even when viewing binocularly. It is often associated with infantile
(congenital) esotropia. On electronystagmography, there is reducing amplitude during
the slow phase. It is difficult to check the monocular vision as nystagmus increases
with it- to overcome this problem neutral density filter should be used in front of
one eye. For the treatment of amblyopia conventional occlusion or penalisation
may be used.
Manifest latent nystagmus: It is the condition where latent nystagmus is made
manifest by reduced vision in one eye as in strabismic amblyopia.

NYSTAGMUS BLOCK SYNDROME


In some cases of infantile esotropia, there is purposive excessive convergence to
damp down congenital nystagmus. The nystagmus is revealed when convergence
has been treated surgically.

MANAGEMENT OF NYSTAGMUS
Indications
1. To improve vision
2. For correction of abnormal head posture
3. For management of associated squint.

http://ophthalmologyebooks.com
Nystagmus 157

Refractive Errors and Amblyopia from Nystagmus


a. In young patients in whom a visual acuity can not be obtained, refractive errors 14
should be corrected to improve the foveal visual acuity and stabilize the nystag-
mus. In older patients, glasses should only be prescribed if the patient is more
comfortable with the refractive error corrected.
b. Contact lenses are often an advantage over glasses for patients who assume a
null zone that causes them to look away from the optical centers of their glasses.
c. The near vision is usually sufficient particularly when the matter is held close to
the eyes. This is done by dampening the nystagmus by using convergence and
also magnifying the retinal image. Visual aids for near are usually not required in
congenital nystagmus unless an organic lesion of the fovea is present.
d. Monocular telescopic aids for distance are useful in nystagmus.
Prisms: Indications
a. To predict the effects of surgery both bases in same direction as head turn.
b. As supplement to surgery.
c. To stimulate convergence.
Surgery: For head turn greater than 15-20 degrees.
Principle: Optimal use of the null point
i. By rotating the eyes to move the null point to a more useful central position.
ii. Induce a gaze palsy to limit eccentric movement of the eyes.
iii. Generous amounts of surgery are recommended as most cases lose about half
of the effect with time.
Kestenbaum procedure: Surgery is not advised for the null zone position unless
there is an improvement of distance visual acuity of several lines with the face
turn. Surgery should be delayed until the patient is over 8 years of age.
Indications for surgery in patients with Nystagmus and Face Turn
a. Improved corrected visual acuity of at least two Snellen lines with head turn.
b. Adopted face position is causing significant problems.
c. Patient always turns the face to the same side.
d. Patient understands and accepts the permanent side effects of markedly re-
stricted gaze on one side after surgery.
Method: The surgical technique involves turning the eyes in the same direction as
the patient turns the face to reach the null zone. A 10mm. recession and a 10mm.
resection on all four horizontal rectus muscles should be done to move the eyes
maximally in the direction of the preferred face turn. One year after surgery, some
recurrence may occur. Surgery is contraindicated in patients using two null zones.
When nystagmus co-exists strabismus without fusion, the fixing eye is turned maxi-
mally with a 10mm recession and 10mm resection operation on the horizontal recti.
Supramaximal recession or faden operation of all four horizontal recti (10
mm each): It is indicated in cases which do not have any definite null position.
Pendular nystagmus with relatively good visual potential are suitable candidates.
158 Strabismus

NEUROLOGICAL NYSTAGMUS
14 A.Localising nystagmus
1. Dissociated nystagmus: Usually indicates posterior fossa disease e.g.,
a. Internuclear ophthalmoplegia:
Nystagmus is seen in the abducting eye. Slowed abducting saccades are
seen. Most likely causes are multiple sclerosis in the young or vascular
abnormality in the elderly.
b. In anterior visual path gliomas in childhood:
Unilateral vertical nystagmus may be seen.
2. Downbeat nystagmus: Usually seen in cervicomedullary junction pathology
(at foramen magnum) e.g., in Arnold-Chiari malformation, Brainstem stroke,
Multiple sclerosis, Spinocerebellar degeneration, Platybasia.
There is characteristic fast phase down.
3. Upbeat nystagmus: Usually indicates brainstem disease. It occurs with cer-
ebellar vermis lesions.
4. See-saw nystagmus: Usually present with suprasellar lesions and bitempo-
ral hemianopia. It can also be congenital.
It is characterized by- pendular; conjugate- rotatory, disconjugate-vertical;
one eye elevates and intorts and the other eye depresses and extorts.
5. Convergence-retraction nystagmus: Here co-contraction of extraocular
muscles causes jerk convergence retraction especially on attempted upgaze
or convergence. It can be elicited by vertical OKN. It is seen in Parinaud’s
dorsal midbrain syndrome ,which is characterized by- defective vertical gaze,
especially upgaze, light near dissociation, lid retraction, convergence-retrac-
tion nystagmus and skew deviation.
6. Vestibular (central/peripheral)
B. Non-localising nystagmus.
Increases in the direction of gaze.
1. Drug induced: anti-epileptics, sedatives
2. Posterior fossa disorders- bilateral brainstem infarcts, cerebellar disease.
Nystagmoid movements: Movements which are not regular and rhythmic are called
nystagmoid movements.
Nystagmoid conditions: Oculopalatal myoclonus, opsoclonus, ocular bobbing,
Heimann-Bielschowsky’s unilateral vertical bobbing.
Index

A Combined abduction deficit 117 E


Abducens nerve palsy 117 Comitant (concomitant) 31
Complications of strabismus surgery Eccentric fixation 30
Adjustable sutures 148 Esodeviation 31
After image test 23 149
Confusion 19 Esophoria
Amblyopia 24, 96 characteristics 56
classification 25 Congenital myopia 80
management 57
anisometric 26 Congenital oculomotor apraxia 118
measurement 57
deprivation 26 Conjunctival closure 148
Esotropia 74
isoametropic 26 Conjunctival scarring and the plica
acquired
occlusion 28 150
comitant 79
strabismic 25 Convergence 11
incomitant or paralytic 79
diagnosis 26 Convergence insufficiency
congenital (or infantile) 74
pattern of fixation 27 characteristics 61
common variants 75
visual acuity measurement 26 tests 61
differential diagnosis 76
criteria for visual treatment 62
etiology 74
improvement 30 Convergence paralysis 63
general features 74
treatment modalities 28 Cyclophoria 61
natural course 76
Angle of deviation 52 Cycloplegic refraction 34
treatment 77
Anisometropia 80 treatment of special features
Anomalies of accommodation 64 D
78
overaction Dellen 150 esotropia with a high AC/A ratio
excessive 64 Desagittalization 148 83
spasm 64 Development of vision management 84
underaction Monocular 15 esotropia without fusional poten-
ill-sustained 65 Normal binocular 15 tial 86
inertia 66 abnormalities 19 etiology 80
insufficiency 65 grades 15 fully accommodative 81
paralysis 66 Diplopia 19, 149 management 82
Anterior segment ischemia 120, 151 Diplopia test 57, 98 principle 83
Axial angles of the eye 12 Diploscope 129 nonaccommodative 83
Dot card 128 other types 86
B Double Maddox rod test 105 acute 87
Bagolini glasses 21 Duane’s retraction syndrome 115 associated with divergence
Bar reading 130 associated non-ocular findings paralysis 88
Bell’s phenomenon 7 117 consecutive 88
Bifocals 85 complications 120 cyclic 87
Blind spot nightmare 72 differential diagnosis 117 emotional 86
Blow-out fracture 113 embryogenesis 116 following recovered 6th
Botulinum injections 79 features 116 nerve palsy 87
Botulinum toxin 152, 153 management 118 occlusion 87
Brown’s syndrome 111, 120 pathogenesis 116 secondary 88
acquired 121 surgical procedures 118 voluntary produced 87
congenital 120 types 115 partially accommodative 82
Bruckner test 46 variants 117 Excyclodeviation 32
Duane’s retraction syndrome 76 Exodeviation 31
C Duane’s syndrome 138 Exophoria 56, 59
CAM stimulator 29 Ductions 9, 44 characteristics 59
Ciancia syndrome 75, 139 Dysthyroid eye disease 122 measurement 60
Classification of strabismus 31 management 123 treatment 60
160 Strabismus

Exotropia 68 Holt-Oram syndrome 117 Hess’s screen test/Lee’s screen


congenital Horizontal muscle surgery 118 test 49
general features 68 Horopter 18 Lancaster red/green test 51
treatment 68 Hummelsheim’s operation 146 synoptophore 51
consecutive constant 73 Hyperdeviation 31 head posture 43
intermittent 69 Hypermetropia 80 lid position 44
investigations 70 Hyperphoria/Hypophoria 56, 60 prism dissociation test 53
management 70 red filter test 48
synoptophore 51
symptoms 69 I
Motor unit 9
treatment of persistent post-
Incomitant (noncomitant) 31 Muscle sequelae 97
operative 71
Incyclodeviation 31 Myaesthenia 79
treatment for recurrence 72 Myasthenia gravis 124
types 69 Infantile esotropia 118
Inferior oblique muscle palsy 112, Myectomy 141
secondary 73
Extraocular muscle palsy 121
N
acquirred 95 Inferior oblique overaction 78
Non-surgical management of strabis-
congenital 95
J mus 125
Extraocular muscles 1 optical
actions 3 Jensen’s operation 147 use of lenses 125
primary, secondary and use of prisms 126
tertiary 4 orthoptic exercise 127
K
anatomical implications 7 pharmacological 131
blood supply 2 Knapp’s operation 114 cycioplegics 132
fiber classification 5 Krimsky’s test (Prism bar reflex test) miotics 131
fine structure 5 45 Normal binocular fusion 16
kinds assessment 16, 23
horizontal rectus 1 L Nystagmus 75
lateral rectus 1 characteristics 154
oblique muscles 2 Lang’s syndrome 76 classification 154
vertical rectus 1 Lateral rectus paralysis 138 congenital 155
oribital and fascial relationships latent 156
6 M neurological 158
degrees 154
Maddox rod test 48
management 156
F Malignant hyperthermia 152 refractive errors and amblyopia
Measurement of convergence 67 157
Faden operation 143
Micerotropia 88 Nystagmus block syndrome 78, 156
Field of binocular vision 55
Miotics 85
Fixation axis 13
Mobius’ syndrome 123 O
Fixation patterns 33 Moebius syndrome congenital diple
Forced duction test 55 Oculocardiac reflex 152
gia 118 Okihiro syndrome 117
Fundus examination 36 Motor testing 43 Optokinetic nystagmus 34
Bielchowsky head tilt test 53 Orbital blow-out fracture 121
G binocular motor functions 44 management 122
General fibrosis syndrome 123 ocular movements 44 Orthoptic exercises 86
Goldenhar’s syndrome 117 corneal reflex tests 45
cover tests
P
alternate cover test 47
H
cover/uncover test 47 Panum’s area 19
Haidinger brushes 52 dissimilar image tests 48 Paralytic squint 95
Hang-back technique 139 double Maddox 49 acquired sixth nerve palsy 101
Hering’s law 10 Maddox wing test 49 fourth nerve palsy 103
Hess/Less screen 98 red filter test 48 investigations 96
Heterophoria 56 dissimilar target tests 49 third nerve palsy 102
Hirschberg’s test 45 diplopia test 51 treatment 100
Index 161

Paresis 95 tests for retinal correspondence Tropia 31


Pattern strabismus 90 39 Tucking (plication) 145
A-pattern after image test 40 Types of prisms 126
A-esotropia 90 Bagolini’s striated glasses 39
A-exotropia 90 synoptophore 41
diagnostic features 92 Worth 4 dot test 41 V
diamond pattern 92 tests for stereopsis 36
Frisby stereo test 38 Vergences 10
indications for treatment 92 fusional 11
inverted Y-pattern 92 Lang stereo test 38
Randot stereo test 37 Vergences (Range of Fusion) 52
surgery for A and V patterns 93
titmus stereo test 37 Versions 10, 44
V-pattern 90
TNO random-dot test 37 Vertical retraction syndrome 117
V-esotropia 91
two pencil test 36 Vertical strabismus 104
V-exotropia 90
tests for suppression 41 bilateral superior oblique palsies
X-pattern 92, 94
4 prism base out test 42 clinical features 107
Y-pattern 92 Bagolini’s glasses 43
Penalization 29 management 107
Sherrington’s law 10
Perforation of the globe 151 Stereoacuity 37, 39 Brown syndrome 111
Phoria 31 Stereograms 58 congenital double elevator palsy
Pleoptics 29 Stereopsis 18 113
Preschool vision test 35 Stereotests 22 dissociated vertical divergence
Prisms 16 Sterogram cards 128 clinical characteristics 108
Progressive external ophthalmople- Strabismus fixus 124 double depressor palsy 114
gia 123 Superior oblique tendon syndrome inferior rectus muscle paresis 110
Prolapse of the tendon’s capsule 149 120 mechanical restriction 112
Pseudo-Duane’s retraction syndrome Superior opblique myokymia 124
pseudovertical 114
117 Suppression
central and peripheral 19 skew deviation 111
Pseudosquint 32
facultative and obligatory 20 superior oblique myokymia 114
monocular and alternating 20 superior oblique overaction 110
R
tests 20 unilateral superior oblique palsy
Recession operations 138 Supranuclear eye movement systems assessement 104
Remy separator 130 11 clinical features 104
Resection 144 Suture abscess 150 treatment 105
Resection recession surgery 133, 138 Suture granuloma 149 Vertical strabismus surgery 148
anesthesia for extraocular Symmetrical surgeries 137
Vieth-Muller circle 18
muscle surgery 135 Synoptophore 13, 16, 22, 23, 51,
105 Visual acuity testing 32
effect of surgery 136
guidelines for surgical correction
135 T W
preoperative assessment 134 Wildervanck syndrome 117
Teller cards 34
Retinal correspondence 20 Worth’s four dot test 21
Tenon’s capsule 6
diagnosis 21 Tenotomy 121
types 21 Tenotomy or tenectomy 142 Y
Retinal rivalry 18 Tests for torsion 105
Royal Air force (RAF) rule 66 Transposition operations 146 ‘Yoke’ muscles 10
inferior oblique muscle 148
S
part-muscle 146 Z
Saccadic velocity 55 superior oblique muscle 147
Sensory tests 36 whole muscle 146 Z-myotomy 140

http://ophthalmologyebooks.com

You might also like