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Histopathologic and trypsin digestion studies of the retina in incontinentia pigmenti.

Bell WR, Green WR, Goldberg MF

Eye Pathology Laboratory, Department of Pathology, Wilmer Institute, Johns Hopkins Medical Institutions, Baltimore, Maryland, USA.

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.

Published 2 May 2008 in Ophthalmology, 115(5): 893-7.
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