Surgical correction of entropion and excess upper eyelid skin in congenital cutis laxa: a case report

Abstract

Congenital cutis laxa is a rare generalized inherited elastosis, characterized by the appearance of premature aging and skin laxity with mild to severe systemic anomalies. Ocular manifestations include excess skin in the eyelids, ptosis and lower lid ectropion. Of the hyperelasticity syndromes – Ehlers Danlos, Pseudoxanthoma elasticum and cutis laxa – only cutis laxa has normal skin wound healing. The diagnosis must therefore be established before surgical options for treatment are considered. We report an unusual case of a 5-month-old male child with cutis laxa who presented with upper lid entropion secondary to severe redundant upper eyelid skin. An anterior lamellar repositioning procedure successfully corrected the lid margin malposition with complete relief of symptoms.