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Strabismus PDF
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Strabismus PDF
STRABISMUS
Himadri Datta MS
Associate Professor
Regional Institute of
Ophthalmology
Kolkata
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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
Himadri Datta
Contents
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. 1.6A: 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
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
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...
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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.
Fixation Axis
This is a line passing from the object of fixation to the center of rotation of the eye. 1
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
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Development of Vision-Binocular Vision, Suppression 17
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:
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
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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
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
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.
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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
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.
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32 Strabismus
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.
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Classification of Strabismus and Approach to a Patient with Strabismus 37
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. 4.9: 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).
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).
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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).
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).
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
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).
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Classification of Strabismus and Approach to a Patient with Strabismus 47
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
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).
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)
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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
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).
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).
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
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Heterophoria, Accommodation and Convergence Insufficiency 57
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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
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
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
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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
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.
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
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72 Strabismus
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
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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
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
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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.
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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
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
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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
Fig. 8.2: Transposition of the medical rectus muscles—up in A and down in V patterns
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Paralytic Squint 97
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
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
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.
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102 Strabismus
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.
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
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Vertical Strabismus 107
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.
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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.
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.
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.
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
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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
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
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.
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122 Strabismus
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.
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.
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
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.
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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
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
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.
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.
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
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,
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132 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.
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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)
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.
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.
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.
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142 Strabismus
13
b c
upper
lower
d
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
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
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
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
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).
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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
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
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152 Strabismus
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
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
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.
MANAGEMENT OF NYSTAGMUS
Indications
1. To improve vision
2. For correction of abnormal head posture
3. For management of associated squint.
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Nystagmus 157
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
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