Abstract
The authors report the first clinicopathologic study of autosomaldominant fundus flavimaculatus with late-onset atrophic macular degeneration in a 62-year-old man. Results of histopathologic examination disclosed the retinal pigment epithelium (RPE) to be distended by a periodic acid-Schiff (PAS)-positive, acid mucopolysaccharide-negative material. Transmission electron microscopy showed marked accumulation of lipofuscin and melanolipofuscin granules within the RPE. The different modes of genetic transmission and ultrastructural heterogeneity suggest that fundus flavimaculatus is a clinical syndrome representing several genetically and mechanistically distinct disorders whose common endstage is a topographically similar accumulation of lipofuscin.
Original language | English (US) |
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Pages (from-to) | 798-809 |
Number of pages | 12 |
Journal | Ophthalmology |
Volume | 97 |
Issue number | 6 |
DOIs | |
State | Published - Jan 1 1990 |
ASJC Scopus subject areas
- Ophthalmology