Histopathologic and Trypsin Digestion Studies of the Retina in Incontinentia Pigmenti

Objective To report the ocular histopathologic features of a 55-year-old patient with incontinentia pigmenti retinopathy. Design Observational case report. Participant A 55-year-old patient with incontinentia pigmenti retinopathy. Methods Examination of eyes by light microscopy and retinal trypsin digestion. Main Outcome Measures Clinical and histopathological findings. Results Histopathologic examination disclosed inner retinal ischemic atrophy, capillary beading, arteriolar–venous anastomoses, preretinal neovascularization, vasculopathy located at the junction of central vascular and peripheral avascular retina, retinal tears, and tractional retinoschisis. Conclusions Patients with retinal manifestations of incontinentia pigmenti may progress to proliferative vitreoretinopathy or retinal detachment and should be observed closely over the course of their lifetime.