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Investigation and
Management of Strabismus Examination
Assessment of Vision
The visual acuity (VA) assessment should
C-19096 O/D be carried out monocularly with the best
refractive correction in place unless the
Saurabh Jain MB, BS, MS, FRCOphth
patient is not cooperative, in which case
Strabismus is a pathological misalignment of the visual axes that can
the best binocular VA is noted. Even in
present in various ways depending on the aetiology and age of onset. 47
the latter case, fixation preference and
In the UK, strabismus affects 2.1% of the childhood population.1,2 They resistance to occlusion may indicate
usually arise before the age of five years in children but it is possible to that one eye sees better than the other.
present with a completely new onset in adulthood too. Timely, adequate The method of VA assessment depends
detection and treatment of strabismus is essential in childhood to prevent on the age of the patient (Table 3). It
the development of amblyopia and loss of stereopsis, whilst in adults it can is important to implement the use of
cause profound psychosocial effects.3-7 This article describes the optometric crowded tests as soon as possible in
children, as they are more sensitive to

01/06/12 CET
investigation and management of strabismus in children and adults.
the detection of amblyopia. If the child
is too young or uncooperative with any
of these tests, simple observation of the
Classification attributed to being a “bad habit” by the
child’s fixation can give an indication
There are various ways of classifying parent. It is also important to enquire
of their level of vision. Normally,
strabismus, as summarised in Table 1. about any birth problems such as
fixation should be central on a small
It is also possible to classify strabismus prematurity,9-11 motor milestones12,13
pen torch or a letter target and steady
according to the aetiology, as summarised and family history of strabismus
when the target is moved and followed.
in Table 2. Each eye is housed in the or amblyopia.14 Previous history of
orbit and has six extraocular muscles occlusion therapy and strabismus
that are supplied by three cranial surgery is also relevant in guiding Ocular alignment
nerves which arise from the brain stem. treatment for the present episode. Assessment of ocular alignment is
The coordination of eye movements Strabismus acquired in adulthood performed by viewing the Hirschberg
is controlled by higher centres of the usually presents acutely with diplopia, corneal reflexes when shining a penlight
brain (supranuclear and cortical fusion whereas patients with a congenital or onto both eyes from a distance of 33cm
centres). It is quite helpful to describe the childhood strabismus have usually and observing the position of the corneal
strabismus in terms of the site involved developed sensory adaptations such reflexes. If an eye is deviated outwards,
as it has a bearing on the management. as suppression, and therefore do the reflex falls nasally to the pupil and
not experience diplopia. In some conversely it is temporal in the case of
History Taking cases a longstanding strabismus a convergent strabismus. The amount
It is important to obtain a detailed may decompensate following, for of deviation can be approximately
history that includes the age of onset of example, illness or trauma, leading judged by the position of the reflex
symptoms and/or signs, details about to diplopia. A sudden presentation (Table 4). A variation to this is the
the characteristics of the strabismus of diplopia with strabismus should, prism reflex test, whereby the angle of
(including which eye, direction of however, be treated as an urgent deviation is measured by neutralising
deviation, and whether it is intermittent case unless otherwise confirmed. In the displacement of the light reflex
or constant) and associated visual particular in adults it is important to using prisms. The prism is oriented with
symptoms. For younger children, parents rule out vascular causes of strabismus its apex towards the direction of the
may fail to volunteer information that that include underlying diabetes, deviation, i.e. base out for an esotropia
they think is unrelated to the problem. hypertension, hypercholesterolemia, and base in for an exotropia. It is better
For example, intermittent divergent coagulopathies and previous to perform this by holding the prism in
strabismus can decompensate in sunlight cerebrovascular accidents (CVA), and front of the fixating eye and increasing the
causing the child to shut one eye when so a detailed history of systemic health strength of the prisms until the light reflex
outdoors, which could mistakenly be problems should also be recorded. falls on the pupil of the deviated eye. The

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Classification Sub-Groups Description

According to:
Age of onset Congenital/Infantile The deviation is noticed before six months of age

Acquired The deviation is noticed later in childhood or as an adult. Family albums can be very
useful in determining the age of onset of a squint

48 Fusional status Manifest (heterotropia) The deviation is constant or present all the time

Latent (heterophoria) The deviation is controlled by the fusional mechanisms under normal binocular viewing
conditions and becomes apparent only on dissociation

Fixation/laterality Constant The deviation is limited to one eye, which usually has associated reduced vision
(amblyopia)
Alternating The deviation tends to drift between the two eyes. An alternating strabismus is usually
associated with equal vision in both eyes
01/06/12 CET

Angle of deviation in Comitant (Concomitant) The size of the deviation is similar in all directions of gaze and indicates that the
various gazes strabismus is congenital or longstanding

Incomitant The deviation varies in size in different directions of gaze and/or the fixating eye, and
may indicate an acute onset and/or an underlying restrictive pathology. Paralytic
strabismus tends to be worse in the direction of action of the affected muscle

Direction of Horizontal Convergent deviations (esotropia or ESOT) occur when the eye(s) point inwards towards
misalignment the nose.
Divergent deviations (exotropia or EXOT) occur when the eye(s) point outwards.
ESOT deviations are more common (60% than EXOT deviations (40%) in the UK8

Accommodative effort
Vertical The deviating eye can be higher than the fixating eye (hypertropia or HYPERT) or lower
(hypotropia or HYPOT)
Rotational A torsional deviation of the eye can be outwards (away from the nose – excyclotropia) or
inwards (towards the nose – incyclotropia)
Accommodative The deviation is linked to accommodative effort thus leading to a larger deviation
for near; it is relieved by relaxing accommodation using convex lenses. It is useful to
measure the accommodative convergence/ Accommodation ratio (AC/A) in these
patients
Non-accommodative or The deviation does not vary with accommodative effort (non-accommodative) or is not
partially accommodative completely relieved with convex lenses (partially accommodative)

Table 1
Classification of strabismus
amount of deviation can be described
in degrees or prism dioptres (which
is the no of degrees multiplied by 2).
Cover-Uncover Test
The cover-uncover test should
performed by asking the patient to
focus on an accommodative target. On
covering the fixating eye, the deviated
eye moves to take up fixation. The cover
is then removed and the behaviour of
the eye under the cover studied; if the
patient can maintain fixation with the
previously deviated eye through to the
next blink, the strabismus can be said to
alternate. If however, they immediately
be switch fixation back to the original
configuration, it indicates a constant
strabismus with a fixation preference for
the undeviated eye. In such situations
there is likely to be a difference in VA.
If no strabismus is apparent on the
Hirschberg test or the cover-uncover test,
it is possible that the patient has a latent
strabismus or heterophoria rather than a
heterotropia. In this case, it is helpful to
carry out an alternating cover test where
the cover is moved from one eye to
another to disrupt fusion and uncover the
strabismus. An alternative method is to
patch one eye for an hour before carrying
out the cover test to help detect the full
amount of the strabismus. The cover and

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alternate cover tests should be carried
Disorders of Disorders of the Disorders of the Disorders of the
out for both near and distance using the extraocular orbit cranial nerves brain stem/cerebral
appropriate accommodative fixation muscles cortex
targets rather than a diffuse flashlight. Thyroid eye Blow out Vascular e.g. diabetes Vascular e.g. strokes,
If the patient has an alternating disease fractures mellitus, hypertension diabetes mellitus,
hypertension,
strabismus, a prism cover test (PCT)
intracranial
is useful to determine the size of the haemorrhage
deviation. A cover test is performed
49
Myasthenia Trochlear Inflammation e.g. post Demyelination e.g.
with increasing amounts of prism Gravis inflammation or viral nerve palsies multiple sclerosis
placed in front of the deviating eye trauma

until there is no movement elicited Congenital Space occupying Mechanical e.g. Mechanical e.g.
on cover-uncover test. This is a more fibrosis syndrome lesions of the orbit e.g. aneurysms, tumours, tumours, idiopathic
dermoids, tumours, intracranial hypertension intracranial
accurate method of measuring the varices hypertension
angle of deviation but relies on good
Myositis Orbital apex syndrome Trauma

vision in both eyes to take up fixation.
Ocular motility
Ocular motility should be examined
in nine positions of gaze using a pen
torch. The function of the extraocular
muscles can be difficult to judge as they
have primary, secondary and tertiary
actions. As such, primary over- or under-
actions of each muscle can be judged by
excursion of the eyes into the plane of
action of the muscle, as shown in Figure 1.
In restrictive forms of strabismus, eg,
thyroid eye disease, both ductions
(single eye movements) and versions
(binocular eye movements) are affected.
In muscular pareses (palsies), versions
are affected more than ductions, which
may in fact be almost normal with
effort. It is thus important to test ocular
motility in both monocular and binocular
conditions when an abnormality is
detected. A Lees screen may be used
to document the ocular motility and
helps distinguish between restrictive
and paralytic causes of strabismus.
Other myopathies Tolosa Hunt syndrome
Following retinal
detachment surgery
secondary to buckling
Table 2
Classification of strabismus according to aetiology
as shown in Table 5. A synoptophore
can be used to assess all three grades
of binocularity. Measuring binocular
function is an integral part of assessing
strabismus. Patients who have a congenital
strabismus usually have no binocular
function and employ suppression of the
deviating eye to prevent diplopia. Adults
or those with late onset strabismus
retain some degree of binocular function
that can be measured using the tests
01/06/12 CET
described in Table 5. The normal level
of stereopsis is 60” or better in adults.
Calculating the AC/A ratio
Patients who have a significant increase
in esotropia at near may have an increased
convergence response to accommodation
and should have their accommodative
convergence to accommodation (AC/A)
ratio measured. There are different
methods that can be used, with one

Tests for Binocularity

Binocularity is the ability to view objects
in three dimensions, which relies on the
disparity of images obtained from the
two eyes. There are three progressively Figure 1
increasing grades of binocularity, which Primary actions of the extraocular muscles; IO = inferior oblique; SO = superior oblique; SR = superior
can be tested using various methods, rectus; LR = lateral rectus; MR = medial rectus; IR = inferior rectus
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too if thyroid eye disease is suspected.

Age of child Recommended Vision Test

6 months to 1 year Forced choice preferential looking tests (Keeler’s

gratings) Management of Strabismus
The aims of treatment of strabismus are
12 months to 18 months Cardiff Acuity Cards (also a preferential looking to restore ocular alignment and binocular
test)
vision, to prevent loss of vision and/
18 months to 2 years Kay Pictures
or stereopsis in children, to eliminate
50
2 years to 3 years Keeler Crowded Cards or reduce diplopia and to improve
abnormal head position. The modalities
Crowded Kay Pictures
of treatment are described below.
Keeler Crowded Cards Following retinal detachment surgery
secondary to buckling
Conservative
Above 3 years LogMAR tests The majority of cases of strabismus
seen in newborns are intermittent and
Table 3
01/06/12 CET

temporary in nature. Normal binocular

Recommended tests for measuring visual acuity in children viewing should be established by
three months of age and any persistent
Reflex at the edge of the pupil 30 Prism Dioptres
strabismus following that is significant.16
Reflex midway between pupil and limbus 45 Prism Dioptres In adults, acute onset strabismus can
resolve partially or completely with time
Reflex at the limbus 60 Prism Dioptres
e.g. diabetes mellitus, hypertension,
Reflex on the sclera >60 Prism Dioptres (up to 100 Prism Dioptres) CVA, trauma, and myasthenia gravis.
However, acute onset strabismus with
Table 4 diplopia should always be referred
Estimation of strabismus size according to the position of the Hirschberg corneal reflexes urgently and investigated medically
to ascertain the cause of the deviation.
Grade of Binocularity Appropriate Test(s)

I = Simultaneous Macular Perception Bagolini’s lenses Refractive correction

II = Fusion
III = Stereopsis
Table 5
Grades of binocularity and appropriate tests to use for their assessments
being the gradient method. This involves
measuring the change in the size of
deviation (by prism cover test) with
accommodative change. The size of the
deviation at near is measured with full
refractive correction and then again with
an additional +3.00DS lens in place. The
change in size of the deviation is then
divided by the lens power to obtain the
AC/A ratio. For example, in a patient
with 24Δ base out with full refractive
correction and 12Δ base out with +3.00DS
lenses, the AC/A ratio is (24-12)/3 =
4. The normal AC/A ratio is 3-5:1.
Worth 4 Dot test
Frisby, Lang, TNO, Randot, Wirt’s, Titmus fly
patients with asthenopic symptoms,
convergence may be measured by using
a RAF rule (near point of convergence).
Ocular Examination
It is important to examine the pupils and
fundus in the presence of strabismus, to
rule out cranial nerve damage (e.g. IIIrd
nerve palsy) or ocular pathology in cases
of reduced vision due to, for example,
papilloedema or other optic
anomalies. It is also important to measure
intraocular pressure, assess for proptosis,
15
In assess visual fields and colour vision
An accurate refraction is essential in all
patients who present with strabismus. In
children under the age of 12 years, this
is best performed under full cycloplegia
especially in the presence of esotropia.
Adequate cycloplegia can be obtained
by the use of 1% cyclopentolate drops,
30 minutes prior to examination (0.5%
in children less than one year of age).
Retinoscopy is carried out in a
darkened room using hand held lenses
for children, though a trial frame can
be used for older children and adults. It
is essential to refract on axis especially
in the deviated eye, which may be
facilitated by occlusion or blurring
nerve of the other eye during refraction. A
post cycloplegic subjective test may be
carried out in children over 8 years old.
Hypermetropia and anisometropia

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greatly increase the risk of developing Orthoptic Excercises it causes temporary paralysis, lasting
amblyopia and strabismus.17-19 Young Patients who have a small angle around three months. It can be used
children with moderate hypermetropic decompensating exophoria with to prevent over-action and subsequent
errors (up to 4.00DS) do not need asthenopic symptoms may
benefit contracture of the antagonist of the
spectacles as long as they have from ‘pen to nose’ exercises designed paralysed muscle (e.g. ipsilateral medial
age-appropriate VA and binocular to increase the near fusion range. rectus in sixth nerve palsy). A recent
functions, normal ocular alignment These are more helpful in small angle application of this toxin is to restore
deviations and in younger patients. Dot ocular alignment in young infants with
51
and no significant anisometropia or
astigmatism. Astigmatism of more cards, stereograms and synoptophore esotropia with the hope that the cortical
than 1.00D needs to be monitored exercises may also be used in the office fusional centres will help maintain
closely and spectacles prescribed setting to supplement home therapy. the new, reduced angle of deviation
if there is any suspicion of reduced even when the toxin has worn off.20
vision. Myopes with a refractive Prismatic Correction
error of more than 2.00D also need Prisms can be used to neutralise the angle Surgical Treatment
of deviation and correct the associated All the extraocular muscles arise from the

01/06/12 CET
to be prescribed with spectacles.
Children with convergent strabismus diplopia. Fresnel prisms are “stick on” bony orbit and insert on the sclera. They
should be prescribed their full plastic prisms that can be placed on the can be approached via the conjunctiva and
hypermetropic correction if possible. patients own spectacles and are useful manipulated to correct the deviation of
In some cases, this may need to be when the angle of the deviation is still the eyes. All strabismus surgery is carried
done in a stepwise manner building variable e.g. early stages of a vascular out under general anaesthetic although
up to the full prescription. Bifocal cranial nerve palsy, myasthenia gravis it may be possible to adjust the resulting
lenses with a near add are used for etc. When the deviation is stable the deviation under topical anaesthesia.
treatment of convergence excess prisms can be incorporated into the Recession or weakening of the muscle
esotropia with a high AC/A ratio. patients own spectacle with is performed by disinserting the muscle
lenses,
Divergent strabismus responds well the prism split between the two eyes. from the globe and reattaching it further
to a myopic prescription as concave back. Resection or plication consists of
lenses stimulate accommodation Botulinum Toxin shortening the muscle to increase its effect
and induce convergence. Some Botulinum toxin is a very safe and effective (Figure 2). Disinsertion of the muscle
patients may need over-minused lenses way of treating strabismus. It is injected without reattachment may be performed
(-1.00DS to -2.50DS) to help control using electromyography (EMG) guidance to completely neutralise its effect. Muscles
decompensating divergent strabismus. into the chosen extraocular muscles where can be transposed to take up action of a
non-working muscle, e.g. superior and
inferior recti moved to the lateral rectus
in sixth nerve palsy. Absorbable vicryl
sutures are used for the operation and it
is the authors practice to use adjustable
sutures for all adults. The conjunctival
incision can be placed in the fornix so
there are no visible scars after surgery.

Conclusion
Strabismus can present in various ages and
Figure 2 with a variety of symptoms. It is important
(a) Recession of the rectus muscle, whereby the muscle is inserted further back on the globe and hence to obtain a thorough, focussed history and
weakening the overall effect it exerts. (b) Resection of the rectus muscle, whereby a segment of the carry out relevant ocular examinations
muscle is excised and the newly exposed end reattached to the globe at the original site of insertion. to reach an appropriate diagnosis and
This has the overall effect of strengthening the muscle through increasing the tension within it. Courtesy plan management. In children, it is
of Dr Rohit Jolly. important to exclude amblyopia, which if

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present can be treated with appropriate Ophthalmic Surgeon at the Royal Free which includes clinical work, training
refractive correction and occlusion, London NHS Foundation Trust. He junior surgeons and allied clinical
or penalisation therapy. In adults has special expertise in all aspects of professionals, research and educational
and older children, the deviation can paediatric ophthalmology, squints, activities. www.saurabhjain.co.uk
be treated with prisms, botulinum general ophthalmology and cataract
toxin and extraocular muscle surgery. surgery. He is involved in running References
a busy full time clinical service at See www.optometry.co.uk/
52 About the author the Royal Free, Whittington, Barnet clinical. Click on the article title and
Saurabh Jain is a Consultant General and Edgware hospitals, then on ‘references’ to download.

Module questions Course code: C-19096 O/D

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1. What is the AC/A ratio for a 5-year-old child with 30Δ BO esotropia 4. A corneal reflex test is performed and the reflex is found to be
at near, which decreases to 20Δ BO esotropia with a +2.00DS lens? just temporal to the pupil edge in the primary position. The angle of
a) 25:1 which is normal for their age deviation is likely to measure:
b) 20:1 which is abnormal for their age a) 45Δ esotropia
c) 10:1 which is normal for their age b) 45Δ exotropia
d) 5:1 which is normal for their age c) 30Δ esotropia
d) 30Δ exotropia
2. What is the likely diagnosis for a patient who presents with
constant double vision, convergent strabismus in the primary 5. What is the MOST appropriate Fresnel “stick-on” lens to use to
position that increases in left gaze, and with restriction of lateral gaze correct a 30Δ right esotropia?
in the left eye? a) 15Δ prism base pointing outwards on both the right eye and left eye
a) Left sixth nerve palsy leading to an incomitant strabismus b) 15Δ prism base pointing inwards on both the right eye and left eye
b) Left sixth nerve palsy leading to a concomitant strabismus c) 30Δ prism base pointing outwards on the right eye
c) Right third nerve palsy leading to an incomitant strabismus d) 30Δ prism base pointing inwards on the left eye
d) Right third nerve palsy leading to a concomitant strabismus
6. Children with early onset strabismus are less likely to experience
3. The alternating cover test is used to detect a: double vision due to:
a) Manifest strabismus a) Depression
b) Latent strabismus b) Reversal
c) Both a manifest and a latent strabismus c) Suppression
d) Ptosis d) Confusion

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