Squint

BACKGROUND
Squint, or strabismus, refers to the misalignment of the visual axes of the eyes
and is thought of as a disorder of eye movement. Normal eye movement is
dependent on correct functioning at three levels:
1. 6 Extraocular muscles (EOM): work to provide a full range of eye
movement. Muscles: superior, inferior, lateral and medial rectus; superior
and inferior oblique.
2. 3 Cranial nerves: provide motor input to the EOMs, coordinating
movement of both eyes. Nerves: Abducens (supplies lateral rectus),
trochlear (supplies superior oblique) and oculomotor (supplies remaining 4
muscles).
3. Higher cortical centres and other brainstem nuclei: provide input to
the cranial nerves, to carry out tasks such as controlling the speed of eye
movement and maintaining focus on a moving object or while the body
itself is moving e.g. vestibular nuclei; gaze cortex.
Normal range of movement and various terms used to describe them are as
follows (click on table1/figure1 to enlarge):

Coordinated eye movement and alignment allow for both eyes to be directed
towards the same object. An image of said object is created on corresponding
parts of each retina, and fed back to processing centres in the brain. The two
retinal images are then fused and interpreted by the higher cortical
centres as a single image. Because each eye has a slightly different angle and
field of vision, the images formed on each retina are slightly different to each
other. The brain processes these disparities to construct a 3D image of the object
being viewed. This gives rise to two important concepts:
Binocular single vision: the perception of one single image,
created from two separate retinal images. This is important in that

two eyes working together increases the total field of vision and eliminates
the natural blindspot (the blindspot of one eye falls in the visual field of the
other).
Stereopsis: the perception of a three-dimensional image, created
from two seperate 2D retinal images. This is particularly important for
depth perception and visual judgement.

Pathological changes in squint

Abnormalities of eye movement and misalignment of the eye, as in squint,
interferes with these two functions. Because both eyes are focused on different
fields of view, the retinal images are too disparate to fuse together into a single
3D image. This can manifest in two common ways:
Diplopia: this tends to be the case in patients with new squint occuring
after the visual pathways in the brain have fully developed. Patients
describe seeing double of the same object, due to poor fusion of the
two seperate retinal images created. This is often associated with eye
strain, headaches and poor visual judgement.
Amblyopia: this tends to occur in young patients, with persistent squint,
particularly under the age of 5. At this stage, visual pathways are still
malleable - the brain attempts to resolve the two disparate brain images
by suppressing one of them. If the image from the same eye is persistently
suppressed over a period of time, the visual acuity in said eye reduces.
The eye is said to be amblyopic, and the patient is effectively left with
monocular vision, but no diplopia.
DEFINITION AND TERMINOLOGY
Squint, or strabismus, is a misalignment of the visual axes of the eyes. A squint
can be described in a number of ways - the common terminology used is
discussed in table 2.

Another important system of classifying squints, based on aetiology and

pathogenesis is as follows:
Non-paralytic squint (a.k.a concomitant squint): when the eye
movements are full and equal in both eyes, but only one eye is directed
towards the object being viewed. The angle of deviation between the two
eyes is constant, regardless of the direction of gaze. This is the type of
squint commonly associated with children.
Paralytic squint (a.k.a incomitant squint): when the affected/squinting
eye has restricted or limited range of movement due to underaction of one
or more EOMs. The angle of deviation between the two eyes is dependent
on the direction of the gaze (it increases in the direction that the affected
muscle normally works). Paralytic squints may occur due to (1) cranial
nerve palsy; (2) EOM disease; (3) tethering of the globe in the orbit
These two terms are illustrated in figure 2:

NON-PARALYTIC SQUINT
Epidemiology:
Neonatal squint: common, affecting about 73% of one-month old babies. In
the vast majority of cases, the misalignment of the eyes corrects itself by 4
months.
Pathological squints tend to occur/persist when the child is older than 6
months.
Most common squint = esotropia
Exotropias, when they occur, are more likely to be intermittent than
constant.
Aetiology:
Non-paralytic squints may be idiopathic, developing in a child with otherwise
normal eyes, or may arise secondary to another eye problem. Idiopathic cases
are postulated to be related to disordered central control of eye movement),
while secondary squints are related to disorders that distort retinal images
produced by each eye, asymmetrically. The brain, unable to process two different
images, attempts to compensate by altering eye movement and positioning.
Common causes of secondary squint include:
Anisometropia: dissimilar refractive errors in both eyes causes the relative
blurring of retinal images.
Opacification of the cornea or lens (cataract): also contributes to relative
blurring of images
Abnormalities of retina: causes incorrect transmission of images
Other risk factors for developing squint include family history, prematurity,
neonatal jaundice, neurological illness (meningitis, encephalitis, cerebral palsy)
and craniofacial abnormalities (including syndromes associated with
dysmorphism such as Down's).
Presentation
Visible squint - noticed by parents/teachers
Functional problems - e.g. difficulty with motor skills

Detection at preschool/school screening clinics

Evaluation
Good history taking is important, particularly in young babies, where it is
essential to differentiate pathological squint from the self-remitting squint of new
borns. Key features of the latter include:
Intermittent squint - if pathological, may be constant or intermittent
Decreasing degree of deviation over time - if pathological, should
worsen/stay the same
Gone by 4 months - if pathological, will persist beyond this time
Examination is aimed at
(1) Ruling out pseudostrabismus. This refers to certain facial features that cause
a person with normally aligned eyes to look like they have a squint. On
examination look for features known to be associated with pseudostrabismus:
Prominent epicanthal folds (skin folds on either side of the nose, covering
the medial canthus)
Broad nasal bridges
Facial asymmetry
(2) Confirming Squint: the following tests help identify presence of a squint

(3) Identifying the underlying problem. This includes testing eye movements,
stereopsis, refraction and examination of the cornea, lens and retina
Management:
Management of a non-paralytic squint involves:
1. Treatment of the cause, if identified: typically this involves glasses to
correct refractive error
2. Correction of amblyopia, if the child is under 8 years old (visual pathways
still malleable): Amblyopia is suspected when visual acuity is not improved

by glasses. Its correction involves patching the child's good eye, to allow
the amblyopic eye adequate visual stimulation, thereby encouraging it to
recover visual acuity. Occasionally, cycloplegic drops may also be used in
the good eye.
3. Correction of squint: this has both functional and cosmetic benefits and is
done by prisms in spectacle lenses, eye exercises (sufficient for mild
exotropias) or by surgical realignment. The latter involves manipulation of
the EOMs to realign the eyes:
Recession of an EOM (moving its point of insertion backwards on the globe)
weakens its action
Resection of an EOM (removing a segment of the muscle at its point of
insertion) strengthens its action
Squint surgery is required when there is no identifiable/treatable cause, or in
large squints, that have not responded to correction. When indicated, it is
recommended that surgery be done as early as possible, to reduce the risk of
amblyopia. The surgery is variable in its results as under- or over-correction may
occur. These patients may require further procedures to get satisfactory
realignment. Occasionally adjustable sutures may be used, allowing for minor
correction in alignment to be made in the awake patient, without having to return
to theatre. A few patients go on to develop a second squint, despite initially good
results after surgery.
Prognosis
Early intervention with glasses, patching and/or surgical realignment can greatly
improve vision in the squinting eye and prevent amblyopia. Surgical realignment,
where successful, also reduces the psychoscoial ramifications of squint. However,
while these children will develop good binocular single vision, they very rarely
develop good stereopsis.
PARALYTIC SQUINT
Paralytic squint is caused by decreased function of one or more EOMs, due to:
Palsy of cranial nerve III, IV and/or VI: palsies may be isolated or multiple;
unilateral or bilateral.
EOM disease/ocular myopathies: tend to be bilateral, and are generally
associated with painful eye movement
Tethering of the globe: e.g. after an orbital fracture, orbital neoplasm.
Presentation
Diplopia = main presenting complaint of patients with paralytic squint. It
may be horizontal/vertical/oblique doubling of vision.
Other symptoms associated with underlying pathological process
Diplopia in paralytic squint is binocular - i.e. double vision is corrected when one
eye is occluded. It should be differentiated from monocular diplopia (i.e. patient
still sees double when one eye is occluded), which is usually due to refractive
error or cataract.
Notably, paralytic squint is not typically associated with amblyopia as the
affected patient is usually older and has surpassed the critical period of visual
development. Even in cases of congenital paralytic squint, amblyopia does not

develop as there is usualy binocular vision in most directions of gaze, including

primary position - squint is only manifest in one or two cardinal positions.
Cranial nerve palsies
The presentation of cranial nerve palsies along with common causes are
discussed in the table below. Diplopia associated with cranial nerve palsies is
usually constant:

EOM Disease
A few of the important EOM pathology associated with double vision and squint
are described in the table below:

Management of non-paralytic squint:

1. Identify and treat the underlying cause: variable (e.g. diabetes/thyroid
control, aneurysm coiling, anti-inflammatories, etc).
2. Correction of residual squint/double vision: usually with prisms in
glasses. Surgical correction is rarely indicated/useful in this group.
PSYCHOSOCIAL IMPACT OF SQUINT
Although the mains aims of managing squint are the reduction of double vision
and the prevention of amblyopia, it is important to be respectful and considerate
about the holistic impact that squint can have on the individual. Patients may feel
extremely self-conscious or develop negative body image. They may feel the
stigma of having a squint in other aspects of their life as well, including peerinteraction, job opportunities and relationships. Studies have shown that both
children and adults find squints disturbing to look at, irrespective of their degree
of personal experience with them.
Additionally, new onset diplopia has ramifications for driving. Patients are
required to inform the DVLA and cease driving, until their diplopia has been
adequately corrected by glasses/wearing a patch while driving.