Angiomatosis Retinae

Von Hippel-Lindau disease is an autosomal dominantly inherited multi-system disorder characterised by hae-mangioblastic lesions of the central nervous system and visceral organs [126]. Angiomatosis retinae (retinal hae-mangioblastoma) is often the first observable manifestation of von Hippel-Lindau disease. Histology shows a proliferation of capillary endothelial cells and vacu-olated stromal cells.

In some patients with von Hippel-Lindau disease or in the close relatives of such patients, unusual retinal vascular hamartomas other than retinal angiomas can be detected.

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