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.

Methods: Observational case report. Methods: A 55-year-old patient with incontinentia pigmenti retinopathy. Methods: Examination of eyes by light microscopy and retinal trypsin digestion. Methods: 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.