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Histopathology of the Eye in Cockayne's Syndrome
Peter S. Levin;
W. Richard Green, MD;
David I. Victor, MD;
Angus L. MacLean, MD
Arch Ophthalmol. 1983;101(7):1093-1097.
Abstract
• The eyes of a 44-month-old boy with Cockayne's syndrome had retinal pigmentary abnormalities that included variable pigmentation and excessive lipofuscin deposition in the retinal pigment epithelium and unusual pigmented cells in the retina and subretinal space. There was optic nerve atrophy with loss of nerve fibers and myelin sheaths and also atrophy of the retinal nerve fiber and ganglion cell layers consistent with the histologic features of a demyelinating disease. Widespread pigment dispersion was found in the anterior segment. There was no evidence of vascular disorder, ocular calcification, neuronal storage disorder, or dystrophic corneal changes.
Author Affiliations
From the Eye Pathology Laboratory, The Wilmer Ophthalmological Institute, and the Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore. Dr Victor is now at the Leahey Eye Clinic, Lowell, Mass.
Footnotes
Accepted for publication July 13, 1982.
Reprint requests to Eye Pathology Laboratory, The Johns Hopkins Hospital, 600 N Wolfe St, Baltimore, MD 21205 (Dr Green).
This study was supported in part by research grant 01684 from the National Eye Institute (Dr Green).
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