Squint Free Papers - aioseducation

Squint Free Papers
Clinical Features and Surgical Management
of Bilateral Medial Rectus Agenesis: A Rare
Presentation
Dr. Neha Rathi, Dr. Pramod Kumar Pandey, Dr. Abhishek Sharma,
Dr. Anupam Singh, Dr. Shagun Sood
Absence of one or more extra-ocular muscles is a rare event and is usually
seen in association with syndromic craniofacial disorders. 1 In such
situations superior rectus and superior oblique are affected most frequently.
Bilateral hypoplasias and aplasias have been described, with inferior recti2 and superior oblique tendons being affected most frequently. Isolated bilateral
absence of medial recti has been described recently in a 10 month old boy and
a diagnosis of congenital cranial dysinnervation disorder invoked with out
any tangible evidence of dys- innervation. 3
We describe the clinical presentation, systemic nonsyndromic craniofacial
associations along with successful surgical management of bilateral absence
of medial recti in a 14 year old girl and educe the underpinnings of infantile
exotropia in the esoteric presentation.
RESULTS
A 14 year old girl from non-consanguineous marriage presented with inability
to move either eye medially and a large angle divergent squint since early
childhood. Birth history was unremarkable. Family history of a divergent
strabismus was positive with her younger sister also sporting one. Her
unaided visual acuity was 20/20 with a retinoscopy of + 2.5 diopter sphere
OU. She adopted a slight face turn to opposite side of fixating eye.
There was no syndromic craniofacial disorder but mandibular hypoplasia,
high arched (Byzantine) palate, microdontia, polydontia and arachnodactyly
were noted. No telecanthus was suggested. Palpebral fissures measured
15 mm vertically and 32 mm horizontally and bare sclera was visible both
superiorly and inferiorly suggesting lid retractions. She sported a large angle
exotropia of >70 prism diopter with gross limitation of adduction bilaterally
with reduced saccadic velocity and alternate fixation. Convergence was
absent, accommodation was unaffected. Smooth pursuit asymmetry was
noted in both eyes along with other markers of infantile deviation in the form
of primary inferior oblique overaction and dissociated vertical deviations
OS>OD.
Latent nystagmus was conspicuous by its absence. No globe retraction was
noted on attempted adduction. Fundi were normal, abnormal extorsion
OS>OD was present with V pattern. Forced ductions were mildly positive for
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