von Hippel-Lindau disease: angiomatosis of the retina and central nervous system.

Daniel Albert // Publications // Dec 01 1981

PubMed ID: 7322482

Author(s): Wing GL, Weiter JJ, Kelly PJ, Albert DM, Gonder JR. von Hippel-Lindau disease: angiomatosis of the retina and central nervous system. Ophthalmology. 1981 Dec;88(12):1311-4. PMID 7322482

Journal: Ophthalmology, Volume 88, Issue 12, Dec 1981

von Hippel-Lindau disease is a phakomatosis that is characterized by multiple angiomatous hamartomas located in the retina, central nervous system (CNS),and visceral organs. Retinal angiomas causing blindness and CNS angiomas causing death are familiar consequences of this disease. In certain cases, early detection and treatment of these lesions may prevent their disastrous effects. Since this disease is considered to have an autosomal dominant inheritance pattern, the physician should initiate a systematic examination of all the patient’s family members.