von Hippel-Lindau disease (angiomatosis of the retina and cerebellum).

von Hippel-Lindau disease is a multi-system disorder characterized by hemangioblastic lesions of the central nervous system and visceral organs. Angiomatosis retinae is often the first observable manifestation of von Hippel-Lindau disease. Optometry may be the initial point of entry into the health care system for the von Hippel-Lindau patient. As such, the eye care practitioner should be aware of the diffuse nature of the disease and initiate the search for silent, possibly lethal, lesions associated with the disease. Management may involve consultation with a neurosurgeon, internist, and retinal specialist. The presentation and clinical course of a young man with von Hippel-Lindau disease is presented.