Renal arteriography in von Hippel-Lindau Diease

Abstract

Since 1960, 17 patients with von Hippel-Lindau disease have had cerebellar hemangioblastomas removed at the Victoria General Hospital, Halifax, Nova Scotia. The 4 most recent patients have had renal arteriography as a part of their investigation. Though 3 of these had negative urography, all 4 had positive findings on arteriography. The first patient had bilateral renal cell carcinomas and cysts and failed to respond to 1 year of 'Laetrile' therapy; the second had small cysts in each kidney; the third had bilateral renal cysts, multiple left renal cell carcinomas and a clinically unsuspected right pheochromocytoma; the fourth had a small renal cell carcinoma. It is recommended that all patients with von Hippel-Lindau disease and siblings with any manifestations of the disease have renal arteriography as part of their early investigation.