Retinal detachments in incontinentia pigmenti

Abstract

We report on retinal detachments in six eyes of four patients with incontinentia pigmenti. These nonrhegmatogenous traction retinal detachments are characterized by extensive preretinal and vitreous fibrous organization that pull the retina anteriorly behind the lens. The clinical course and fundus appearance of the retinal detachments, beginning with the avascular peripheral retina and leading to traction retinal detachment, are similar to those of cicatricial retinopathy of prematurity. Vitreous surgery was performed on three eyes (two patients), with partial reattachment of a total retinal detachment in one eye and complete reattachment of a partial retinal detachment in a second eye. These cases represent the first successful surgical interventions reported for retinal detachment in incontinentia pigmenti.