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King W. To, MD; Michael Adamian; Frederick A. Jakobiec, MD; Eliot L. Berson, MD

Arch Ophthalmol. 1996;114(8):950-955.

Abstract
Objective
To describe the clinical and histopathologic features of clumped pigmentary retinal degeneration (CPRD).

Design
Retrospective case series.

Setting
Tertiary referral center.

Patients
Twenty-four patients, aged 7 to 83 years, were identified from the medical record files of the Berman-Gund Laboratory, Boston, Mass, as having the clinical features of CPRD. The autopsy eye from a 56-year-old man with CPRD was studied with light and electron microscopy.

Main Outcome Measures
Visual acuities, visual fields, dark-adaptation thresholds, and results of electroretinograms; histopathologic study of an autopsy eye.

Results
The functional deficit of patients with CPRD seems to be similar to that of patients with typical retinitis pigmentosa. Different degrees of severity were observed among patients of similar age. The histopathologic data showed that the clinically distinct areas of clumped pigment are due to excessive accumulation of melanin granules in retinal pigment epithelial cells.

Conclusion
Based on the distinct clinical and histopathologic appearance, CPRD should be considered as a separate form of retinal degeneration.

Author Affiliations
From the Berman-Gund Laboratory for the Study of Retinal Degenerations and the Ophthalmic Pathology Service, Department of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston.

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