Surgical management of hypotropia in congenital fibrosis of extraocular muscles (CFEOM) presented by pseudoptosis

Abstract

PurposeTo describe the demographics, characteristics, management pitfalls, and outcomes of pseudoptosis associated with congenital fibrosis of the extraocular muscles (CFEOM).MethodsA retrospective review was performed of eight patients presenting with ptosis and hypotropia to oculoplastic service. All patients underwent full ocular evaluation and magnetic resonance imaging of brain and orbit. Five of these patients underwent stepwise correction of hypotropia by single-stage adjustable strabismus surgery (SSASS), followed by a frontalis sling if needed.ResultsEight patients had congenital strabismus with severe ptosis and a positive forced duction test. There was a highly significant improvement from preoperative mean hypotropia angle of 30 prism diopters (PD) to 9 PD mean postoperative angle (P = 0.006). Surgery for ptosis was not needed in 80% of eyes.ConclusionCFEOM involving both ptosis and hypotropia could be properly managed with the correct sequence of surgical steps. Proper vertical alignment by correction of hypotropia utilizing SSASS may alleviate the need for ptosis surgery.