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Article
September 1976

"Atypical Angiomatosis Retinae"-Reply

Author Affiliations

Louisville

Arch Ophthalmol. 1976;94(9):1629-1630. doi:10.1001/archopht.1976.03910040459026
Abstract

In Reply.  —Angiomatosis retinae was reported as a separate ocular entity by von Hippel in 1911 (von Hippel disease). Typically, the disease consists of a peripheral capillary angiomatous hamartoma connected to the disc by dilated afferent and efferent vessels. In only 20% of cases is the disease transmitted as an autosomal dominant trait with incomplete penetrance and variable expressivity. In 1926, Lindau noted involvement of the central nervous system and other organs in approximately 25% of patients with ocular tumors (von Hippel-Lindau disease). Angioma near the disc or within the optic nerve is an uncommon but well recognized variation, the manifestation of which is an absence of enlarged draining and feeder vessels. These lesions may have surface features suggesting other specific or general diagnostic categories. Vascular decompensation within angiomas in any location may lead to subretinal exudation.Juxtapapillary angioma was first described by Carr and Stallard1 and more recently

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