Biomaterial Database

Retinal capillary hemangioma: von Hippel (-Lindau) disease. 1997

I Verhaeghe, and B A Lafaut, and A Brandt, and J J De Laey

Department of Ophthalmology, University Hospital Gent.

The clinical manifestations of von Hippel and von Hippel-Lindau's disease are illustrated. Four cases are presented with special emphasis on the angiographic characteristics, the evolution and the complications of the retinal capillary hemangiomas. Special attention is given to fundoscopy, fluorescein and indocyanine green angiography and to the systemic manifestations. We summarize new molecular genetic insights. The importance of systemic and familial evaluation is stressed. Retinal angiomatosis is a clinical diagnosis but the angiography has an adjuvant value.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D005260	Female		Females
D006623	von Hippel-Lindau Disease	An autosomal dominant disorder caused by mutations in a tumor suppressor gene. This syndrome is characterized by abnormal growth of small blood vessels leading to a host of neoplasms. They include HEMANGIOBLASTOMA in the RETINA; CEREBELLUM; and SPINAL CORD; PHEOCHROMOCYTOMA; pancreatic tumors; and renal cell carcinoma (see CARCINOMA, RENAL CELL). Common clinical signs include HYPERTENSION and neurological dysfunctions.	Cerebelloretinal Angiomatosis, Familial,Lindau Disease,Angiomatosis Retinae,Familial Cerebello-Retinal Angiomatosis,Hippel-Lindau Disease,Lindau's Disease,VHL Syndrome,von Hippel-Lindau Syndrome,Angiomatoses, Familial Cerebello-Retinal,Angiomatoses, Familial Cerebelloretinal,Angiomatosis, Familial Cerebello-Retinal,Angiomatosis, Familial Cerebelloretinal,Cerebello-Retinal Angiomatoses, Familial,Cerebello-Retinal Angiomatosis, Familial,Cerebelloretinal Angiomatoses, Familial,Familial Cerebello Retinal Angiomatosis,Familial Cerebello-Retinal Angiomatoses,Familial Cerebelloretinal Angiomatoses,Familial Cerebelloretinal Angiomatosis,Hippel Lindau Disease,Lindau's Diseases,Lindaus Disease,VHL Syndromes,von Hippel Lindau Disease,von Hippel Lindau Syndrome
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000792	Angiography	Radiography of blood vessels after injection of a contrast medium.	Arteriography,Angiogram,Angiograms,Angiographies,Arteriographies
D018450	Disease Progression	The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.	Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease