Elsevier

Ophthalmology

Volume 87, Issue 12, December 1980, Pages 1189-1200
Ophthalmology

Retinal Pigment Epithelial Abnormalities in Fundus Flavimmaculatus: A Light and Electron Microscopic Study

https://doi.org/10.1016/S0161-6420(80)35106-3Get rights and content

Abstract

Light, fluorescent, and scanning and transmission electron microscopic examinations of two postmortem eyes from a 24-year-old man with well-documented fundus flavimaculatus with atrophic macular degeneration revealed striking abnormalities in the retinal pigment epithelium (RPE). Beginning near the equator, scanning electron microscopy demonstrated a progressively marked heterogeneity in the size of the RPE cells. Surrounded by a fairly regular mosaic of relatively normal appearing cells, enormously enlarged hypomelanotic cells measuring up to 80 microns in diameter occurred in irregular aggregates that became more prevalent posteriorly. Diffusely and intensely PAS-positive, the RPE was packed with a granular substance with ultrastructural, autofluorescent, and histochemical properties consistent with an abnormal form of lipofuscin. The greatest concentration of lipopigment was noted posteriorly. Stains for acid mucopolysaccharide were only mildly positive. The clinical and fluorescein angiographic manifestations of fundus flavimaculatus are consistent with accumulation of a lipofuscin-like substance in the RPE. The massive amounts of lipopigment encountered in this young individual suggest that disordered lipopigment metabolism may play a major role in the pathogenesis of this retinal pigment epithelial disorder.

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  • Cited by (0)

    Presented in part at the Association for Research in Vision and Ophthalmology Meeting, Orlando, May 1980, and at the Wills Eye-.Hospital Clinical Conference, April 1980.

    Supported in part by the Gretel and Eugene Ormandy Teaching and Research Fund, Scheie Eye Institute.

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