Strabismus - Fundamentals of Clinical Ophthalmology.pdf

CHILDHOOD ONSET OF STRABISMUS
(a)
(b)
Figure 4.3 Bilteral inferior oblique overaction in a
young man (a, b) with an enlarged inferior oblique
muscle found at surgery (c). Confirmation of the
diagnosis is based upon presence of a V pattern and
enlarged inferior oblique muscle on exploration of the
muscle. DVD may also be misdiagnosed and is often
not associated with a V pattern although may be
present
(c)
nystagmus with a null point, with the difference
that the null point is in a position of convergence.
Ciancia syndrome has all the appearance of
infantile esotropia, with the exception that, when
the eye abducts, a type of nystagmus occurs in
the infant. There is a face turn usually marked
with the fixing eye in adduction. These children
may cross-fixate and therefore make large head
turns to the left or to the right, depending on
which eye they prefer to use at the time.
Management of nystagmus blockage syndrome
is similar to that of infantile esotropia. However,
Ciancia syndrome generally requires a larger
amount of strabismus surgery and the effect of
this may be to eliminate the abduction nystagmus
altogether. 9 The abnormal head posture is often
only obvious when detailed material is being
studied. In children there may be difficulty in
the classroom seeing the blackboard. The child
should sit on the side of the classroom to which
the face turn is directed.
majority of cases of congenital esotropia
syndrome will have latent nystagmus, that is,
nystagmus observed under cover and in the fixing
eye. This may be manifest as a small amplitude
nystagmus that may be associated with a head
turn so that the fixing eye is adducted. If the child
has no preference with either eye in congenital
esotropia syndrome the head posture may
alternate with the face turned toward the side
of the eye that is fixing and convergent.
Ciancia syndrome is the association of
congenital esotropia with head turn to compensate
for nystagmus. In this syndrome asymmetrical
OKN will be present. Induced OKN reveals a
normal response to the target moving from the
temporal to nasal field but an abnormal response
to the target moving from the nasal to temporal
field. The persistence of this primitive response
usually indicates a poor prognosis for binocular
vision.
This syndrome, also referred to as nystagmus
blockage syndrome, is another example of
Differential diagnosis of congenital
esotropia syndrome
It is important to consider the differential
diagnoses in this syndrome. There is a need to
exclude a monocular sensory deficit, as may
occur with structural damage to the macula and
the retina, for example in retinoblastoma.
Duane syndrome and Moebius syndrome are
important oculomotor disorders to recognise. In
Duane syndrome, there is non-comitancy of the
strabismus pattern with an abduction deficit and
enophthalmos on abduction. Moebius syndrome
may also present as an esotropia but with
obvious abduction deficits and bilateral facial
nerve paresis.
Parents need to be informed that the
management of infantile esotropia does not
end with ocular alignment and that treatment
with occlusion remains an important part of
management until the child’s visual system is
mature. The large angle with cross-fixation and
with no amblyopia, once converted to a very
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