Hypoplastic inferior rectus muscle in association with accessory extraocular muscle and globe retraction

Ocular motility problems related to congenital abnormalities of extraocular muscles are rare conditions. Such congenital abnormalities may present in a spectrum from accessory additional rectus muscles to absence of extraocular muscles. Absence of one or more extraocular muscles is a rare condition usually seen in association with craniofacial syndromes and only rarely in isolation. None of the cases with the absence of an extraocular muscle were reported to be in association with an accessory extraocular muscle or paradoxical contraction of an extraocular muscle. We present a case with bilateral hypoplasia of the inferior rectus muscle with an accessory extraocular muscle in association with globe retraction and lambda-pattern exodeviation.