Faculty Collaboration Database

Histopathologic study of autosomal dominant vitreoretinochoroidopathy in a 26-year-old woman. Arch Ophthalmol 1995 Dec;113(12):1561-6

Date

12/01/1995

Pubmed ID

7487628

DOI

10.1001/archopht.1995.01100120093017

Scopus ID

2-s2.0-0028877425 (requires institutional sign-in at Scopus site)   11 Citations

Abstract

The clinicopathologic findings were obtained from enucleated eyes, obtained post mortem, of a 26-year-old woman with autosomal dominant vitreoretinochoroidopathy. Light microscopy demonstrated atrophic, disorganized peripheral retina with retinal blood vessels obscured by pigmented cells surrounding periodic acid-Schiff-positive deposits. Peripheral retinal pigment epithelial cells showed multilayering and pigmentation, with a thickened basal lamina. By electron microscopy, the peripheral retinal vessel endothelium was replaced by an arrangement of morphologically polarized pigmented cells of presumed retinal pigment epithelial origin oriented with their basal surfaces toward a fibrillar matrix occupying the vessel lumen. The similarity of the findings in this young patient to those of an aged patient described previously suggest that autosomal dominant vitreoretinochoroidopathy is an early-onset dystrophy of the peripheral retina with minimal subsequent progression, characterized by a retinal pigment epithelial response that includes marked intraretinal migration and extracellular matrix deposition.

Author List

Han DP, Burke JM, Blair JR, Simons KB

MESH terms used to index this publication - Major topics in bold

Adult
Choroid Diseases
Electroretinography
Female
Fluorescein Angiography
Fundus Oculi
Humans
Male
Pigment Epithelium of Eye
Retina
Retinal Diseases
Vitreous Body