Adult-onset foveomacular pigment epithelial dystrophy: clinicopathologic correlation of three cases

• Published in: Retina (Philadelphia, Pa.) Vol. 20; no. 6; pp. 638 - 649
• Main Authors: Dubovy, S R, Hairston, R J, Schatz, H, Schachat, A P, Bressler, N M, Finkelstein, D, Green, W R
• Format: Journal Article
• Language: English
• Published: United States 2000
• Subjects: Age of Onset; Aged; Aged, 80 and over; Female; Fluorescein Angiography; Fovea Centralis - metabolism; Fovea Centralis - ultrastructure; Humans; Lipofuscin - metabolism; Macular Degeneration - etiology; Macular Degeneration - metabolism; Macular Degeneration - pathology; Male; Ophthalmoscopy; Photoreceptor Cells, Vertebrate - metabolism; Photoreceptor Cells, Vertebrate - ultrastructure; Pigment Epithelium of Eye - metabolism; Pigment Epithelium of Eye - ultrastructure; Visual Acuity
• ISSN: 0275-004X
• DOI: 10.1097/00006982-200006000-00010

The authors describe the clinicopathologic features of three patients with adult onset foveomacular pigment epithelial dystrophy (AOFPED).
The eyes of three patients were studied ophthalmoscopically and by fluorescein angiography, and obtained postmortem and studied by light and electron microscopy.
Histopathologic study of the three patient's eyes disclosed central loss of the retinal pigment epithelium and photoreceptor cell layer with a moderate number of pigment-containing macrophages present in the subretinal space and outer retina. To either side, the retinal pigment epithelium was distended with much lipofuscin. Basal laminar and basal linear deposits were present throughout the central area. No discontinuities of Bruch membrane were present.
The findings in the eyes of three patients with AOFPED included marked aging changes that are similar to those seen in age-related macular degeneration. Pigmented cells with lipofuscin in the subretinal space account for the vitelliform appearance.