Bilateral inferior rectus muscle aplasia, severe hypertropia, deprivation amblyopia

  

An infant was referred to BPEI for strabismus/oculoplastic evaluation. They were born prematurely at 32 weeks of gestation with a previous surgical history notable for superior rectus tenotomy. The patient presented with severe ptosis OU and an ocular position in extreme up gaze bilaterally. There was a severe limitation to infraduction (not reaching the midline). Their visual acuity was grimace to light OU. An MRI was performed revealing inferior rectus muscle aplasia OU. Surgery was recommended to improve ocular alignment in order to prevent severe amblyopia and maintain functional vision. Anterior transposition of inferior oblique muscle in both eyes was performed and helped depress the globe . At postoperative week 2, the patient had marked improvement of their hypertropia with chin down positioning when attempting to focus. Patient will undergo frontalis sling procedure for ptosis management.

Presentation Date: 09/05/2024

Issue Date: 09/23/2024