Retinal Capillary Hemangiomas and von Hippel-Lindau Disease

Abstract

von Hippel-Lindau disease is a multisystem familial cancer syndrome that commonly manifests in the eye as retinal capillary hemangiomas. As the earliest manifestation of the disease, these benign hamartomas can lead to secondary visual loss. Their typical clinical characteristics can lead to accurate diagnosis and appropriate treatment with either laser photocoagulation or cryotherapy enhancing one's probability of preserving vision before becoming symptomatic. Accurate diagnosis can also lead to surveillance of the various other organ systems involved in the disease such as the CNS, kidneys, adrenal glands and pancreas. This will also enable the clinician to provide genetic counseling for patients regarding the lifelong manifestations of this disease.

KEYWORDS:

• von Hippel-Lindau disease
• retinal capillary hemangiomas
• hamartomas