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However the characteristic presentation with widened palpebral fissures and
gross bilateral adduction deficits left little doubt about hypoplasia of medial
recti. Smooth pursuit asymmetry points towards infantile deviation and in
the absence of any opportunity for binocular interaction, it was retained.
However absence of latent nystagmus, the visuo- vestibular component in the
yaw plane espouses a different mechanism for it’s evolution. 7
The presence of primary inferior oblique overaction and dissociated vertical
deviations, the other 2 fellow travelers of infantile deviations in pitch and roll
plane underscore the pre-requisite of normal development of horizontal gaze
pathways for the bubbling up of these visuo- vestibular abnormalities. 7
MRI is helpful but may not reveal the aplasia as some tenuous attenuated
tissue is invariably found. Surgical exploration is confirmatory, however no
anterior ciliary arteries were visualized here, as is often the case with inferior
rectus aplasias, the reasons for the same are difficult to fathom and underscore
the primacy of anterior ciliary circulation for vertical recti.
Surgical management in the form of large lateral rectus recessions followed
by horizontal transposition of vertical recti with lateral augmentation with
Foster suture produced entirely satisfactory results. Three mm resection
of inferior rectus was carried out to ameliorate DVD but it did not seem to
work well as DVD still persisted Hypoplasia/ aplasia of medial recti can be
associated with subtle craniofacial features and should be suspected in the
presence of large angle exotropia with grossly limited adduction, widened
palpebral fissures, slowing of adduction saccades and presence of smooth
pursuit asymmetry. Primary inferior oblique overaction, DVD, a V rather A
pattern may be cognated as fellow travelers for infantile deviations along-with
bilateral extorsion. Abnormalities linked to migration of neural crest cells may
also be associated. An MRI is helpful in the diagnosis.
Surgical outcomes are fair as far as horizontal alignment is concerned
but functional outcomes are more difficult to achieve as there has been no
opportunity for binocular interaction, especially if surgical intervention is
delayed. Surgical planning should also take cognizance of coexisting DVD,
inferior oblique overaction, V pattern and abnormal extorsion. An early
diagnosis with high index of suspicion and prompt surgical intervention are
suggested as they may help normalization of smooth pursuit asymmetry
and negate emergence of other visuo vestibular movements inimical to
development of good binocularity, in the form of latent nystagmus, inferior
oblique overaction and dissociated deviations, as normal development of
binocularity is almost completely pre-empted. 7 The cognated link between
medial rectus aplasia and inferior oblique overaction, DVD, V pattern and
extorsion is stressed and is being reported for the first time.
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