

Strabismus is a frequent disease in children (3-5% incidence).
Normal eye position is called orthophoria
or orthotropia. Strabismus is such a severe lack of eye alignment
that makes binocular vision impossible.
It can be:
horizontal
- convergent or esotropia
- divergent or exotropia.
vertical
hypertropia (Fig. 1)
hypotropia.
Fig.1
Hypertropia
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Convergent strabismus counts 80% of eye deviations with 65%
of unilateral deviations, which always effect the same eye, and
35% of alternating deviations, which affect both one eye and the
contralateral
one.
Strabismus is defined concomitant when the deviation is identical
in all eye positions and incomitant when the deviation is greater
in a certain eye position, as occurs in paralytic strabismus.
Esotropia can be distinguished in 3 groups:
1) early onset,
2) late onset,
3) microstrabismus.
Exotropia or divergent strabismus can be either constant or intermittent.
The intermittent form has the best prognosis because stereoscopic
vision is preserved. Exodeviations, which appear at a very early
stage and with a high angle, are frequently found in children
with collicerebral lesions in infancy.
Generally strabismus’ early onset - especially if it is
unilateral - can be a sign of eye disease (cataract, retinoblastoma,
chorioretinal scars or other congenital diseases). Strabismus
associates motor signs with important sensory signs and symptoms.
The most relevant of these are: suppression, amblyopia and abnormal
retinal correspondence.
Amblyopia is a visual deficit, whose severity can vary. This sign
is the most important one to be recognised and treated when associated
with strabismus.
Diagnostic evaluation
The cross-eyed patient’s examination involves:
-
inspection and observation of the position of the head
- study of eye motility in the nine eye positions corneal
reflexes
- cover test to define the presence of deviation
- measurement of the deviation visual field examination
- refraction in cycloplegia
- examination of the anterior segment and of the fundus
oculi |
Corneal Reflexes
They exploit the cornea’s mirroring features. The penlight
stimulus pointed before the eyes is placed symmetrically in the
centre of the pupil in orthophoric patients. A shifted reflection
with reference to the centre and its asymmetry are a sign of deviation.(
fig.2)
Fig.2
Corneal reflex in esotropia
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Cover test
The apparently staring eye is covered to evaluate the behaviour
both of the same eye and of the contralateral one when the screen
is removed. No movement is appreciated in conditions of orthotropia.
In cases of unilateral strabismic deviation, the deviated eye straightens
when the apparently staring eye is covered, but it returns to the
strabismic position when the screen is removed.
In cases of alternating deviation, when the apparently staring eye
is covered the contralateral one straightens and maintains the position
after the screen is removed.
4 Dioptre Vision Test
A 4 dioptre prism placed before the eye of normal individuals enables
to observe the behaviour of the contralateral eye, which first has
a rapid movement towards the apex of the prism and a second slow
returning movement. In cases of microstrabismus no movement is observed
when a prism is placed before the eye that is not staring. (
fig.10)
Fig.10
Instruments for orthoptic examination |
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Refraction
The refraction test must always be performed after cycloplegia through
the administration of colliria such as cyclopentolate, which is
currently the most frequently used one. Some authors prefer to use
1% tropicamide.
Atropine is currently reserved to certain cases. The examination,
which evaluates the presence of refractive defects is called sciascopy.
In collaborative patients sciascopy, which remains the main test,
is currently combined with autorefractometry, which is a computerised
detection method of refraction.
Sight defects are hypermetropia, myopia and astigmatism.
Either associated with astigmatism or not, hypermetropia is the
most common defect that has been found in convergent strabismus.
Myopia is more frequently associated with divergent strabismus.
The correction of the visual defect when strabismus is present must
be complete and consider the values found in cycloplegia.
Examination of the anterior segment and of the fundus oculi The
evaluation of the anterior segment by means of a slit lamp and the
examination of the fundus oculi complete the eye examination and
are necessary to rule out eye diseases as a cause of strabismus.
Visual Acuity Test
The type of test required to detect visual acuity depends on the
patient’s age. Along with the well known methods that make
use of optotypes with figures, E tests and letters, we wish to mention
tests that can be used in a pre-oral age with Teller’s Cards.
These cards enable to measure visual acuity in children under 12-15
months. It is a psychophysical test based on the child’s ability
to recognise structured stimuli (black and white stripes) against
an even grey background. The small patient is presented gradually
decreasing stimuli and the evaluation is based on the screen with
the smallest black and white stripes that can evoke a visual response.
(
Fig. 5 - Fig. 6 – Fig. 7)
A difference in the response to the test between the two eyes is
a sign of amblyopia. Another simple test, which can be used in early
infancy, is the occlusion test, which evaluates the child’s
reactions when the healthy eye is closed.
Fig.10
Teller's Card for visual acuity in babies 6 month/1 years
age
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Treatment principal
Medical Treatment
The first treatment envisages complete optical correction of the
defect in refraction with permanent lenses. When amblyopia is present
the choice treatment is occlusion of a variable duration depending
on the patient’s age. (
fig. 3)
Fig.3
Occlusion therapy
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The occlusion must not be passive but active (games or work for
near sight), both to achieve better patient compliance and to obtain
satisfactory results in a shorter time. The maintenance treatment
after occlusion makes use of penalising methods such as the use
of filters applied to lenses. The use of adequately built bifocal
lenses is recommended in the forms characterised by excessive convergence
(high AC/A ratio). (
Fig. 11)
Fig.11
Bifocal lenses for incomitance far/near
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Surgical Treatment
The end of surgical treatment is both functional and aesthetical.
Surgical treatment is the final action in the diagnostic-rehabilitative
course of strabismic patients who require a repeated series of tests
and examinations. Surgery must possibly be recommended when there
is alternating strabismus and equal vision in both eyes. Surgical
treatment aims at removing the visual axes’ relative deviations.
Surgery is performed on eye muscles to influence the very muscles’
activity and subsequently eye alignment. Operations are hence distinguished
either in surgery focused either on weakening muscle action (withdrawal)
or on strengthening it (resection).
The choice of the type and extent of surgery depends on the assessment
of eye motility, on the characteristics of the deviation and on
the measurement of the same. Let us now study some special cases
of strabismus.
Essential infantile esotropia
Strabismus with an early onset (essential infantile esotropia) sets
in during the first 4-6 months of life: parents often notice the
deviation at birth. This deviation is characterised by:
- large deviation angle (often more than 50 dioptres)
- crossed fixation
- abduction deficit (due to the lateral rectal muscle’s failure
to contract) latent nystagmus
- ocular stiff neck (not always present)
- mild hypermetropia (rarely high)
- hyperfunction of the mall oblique muscles (it can be noticed after
the child’s 1st year) (
Fig. 4)
- dissociated vertical deviation (to be distinguished from the hyperfunction
of the small oblique muscles).
Fig.4
Esohypertropia
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Diagnosis
When there is strabismus an eye examination must be performed as
soon as possible to highlight the presence of sensory anomalies,
the most important of which is amblyopia (sight deficit due to lack
of use). When amblyopia is present early treatment is essential
to obtain good results and the total recovery of sight.
Treatment
correction of the refractive defect occlusion traditional surgery
(withdrawal of the median rectal muscles either associated or not
with the withdrawal of the small oblique muscles).
Botulinum toxin treatment has been recently used as an alternative
to traditional surgery.
The injection is administered to the median rectal muscles according
to a well coded method within the child’s 7th – 8th
month of life. Literature has reported satisfactory results in terms
of
eye alignment (60-70%). (
Fig. 8 and
Fig.
9)
Fig.8
Esotropia before tossina
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Fig.9
Esotropia after tossina
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Non refractive accomodative esotropia
High AC/A ratio
It is another form of accommodative esotropia, which cannot be
corrected with lenses used for hypermetropia. In this case the
patient is orthophoric at distant sight and esotropic at near
sight when the correction is applied. These patients respond very
well to bifocal lenses, which must be adequately produced to ensure
that the section of the lens corresponds to the centre of the
pupil.
Microstrabismus
It is strabismus with a small strabismic deviation, which parents
find hard to detect. Hence the diagnosis can be delayed. Microstrabismus
must be suspected when there is a small unilateral visus.
It is characterised by:
-
anisopia
- amblyopia
- eccentric fixation
- abnormal retinal correspondence
- lack of stereoscopic perception. |
Diagnosis
4 prism dioptre test (lack of the refixation reflex when the prism
is placed before the deviated eye).
Treatment
Correction of a refractive defect.
Occlusion.
Exotropia
This term defines divergent strabismus.
Exodeviations are less frequent than esodeviations with a 1:4
ratio.
Classification:
basic exotropia
excessive divergence
convergence deficit.
Forms resulting from excessive divergence have a greater deviation
with distance sight rather than near sight. Inadequate convergence
has a greater deviation for near sight rather than distant sight.
Exotropia can either be intermittent or constant
Intermittent exotropia
Intermittent exotropia is a condition which alternates periods
of deviation control with periods of decompensation. A typical
feature of deviation is the closing of one eye when the patient
is outdoors and in brightly lit environments.
Treatment of Exotropia
Spherical negative lenses can improve deviation control especially
in exophoria/ tropia and in intermittent forms. Orthoptic exercises
improve the visual field but they cannot substitute surgery. The
recommendation to perform surgery generally considers the type
of exotropia: inadequate convergence with high deviation angles
requires resection of the median rectal muscles.
Alphabetic attitudes
Alphabetic syndromes are conditions in which horizontal deviation
changes when one shifts from a downward stare to an upward stare.
It is called syndrome A when maximum convergence occurs upwards,
while it is called syndrome V when the maximum convergence is
downwards. An esotropia in A will have a maximum horizontal deviation
with the upward stare and a minimum horizontal deviation with
the downward stare. An esotropia in V will have the maximum deviation
with the downward stare and the minimum horizontal deviation with
the upward stare.
The opposite occurs with exotropia.
These attitudes can be associated with an abnormal position of
the head (i.e. chin turned upwards or downwards to maintain the
position in which the eyes are most aligned).
In case of esotropia in V associated with an oblique muscle dysfunction,
the procedure envisages withdrawal of the same.
In cases which present a slight alphabetic attitude, vertical
movements of the horizontal rectal muscles can be implemented
along with withdrawal of the median rectal muscles.
The rule is to move the median rectal muscles towards the apex
of A or V and vice versa the lateral rectal muscles.
Conclusions
Strabismus is an important disease, which requires special dedication
and commitment both on the part of the specialist and the patient.
Since the disease has a higher incidence in children, the treatment
requires frequent and careful control of the visual function to
enable the appropriate and symmetrical development of the two
eyes’ visual acuity. Early recognition also enabled by growing
collaboration with the paediatrician is doubtless a good starting
point to achieve the best results.
Surgical treatment must be considered the final action in this
diagnostic-therapeutic course designed to achieve an eye alignment
that is closest to orthophoria, keeping in mind that surgical
correction’s primary goal is not the aesthetical aspect,
but the functional one of reestablishing binocular cooperation.
Dr.ssa E. Piozzi, Dr.ssa A. Del Longo
Paediatric Ophthalmology, Niguarda
Ca' Granda Hospital, Milan