Retinal angiomatosis and pancreatic cysts in von Hippel-Lindau disease: use of computed tomography scanning for diagnosis and surveillance

Abstract

Reports of the retinal and pancreatic manifestations of von Hippel-Lindau disease have been previously published. We present a case of von Hippel-Lindau disease in which computed tomography scanning was used in a new manner for diagnosis of retinal disease and for diagnosis and surveillance of pancreatic disease. We emphasize the importance of these new findings and recommend the use of yearly CT scanning of the abdomen. This replaces the previous recommendation of exploratory laparotomy in patients with pancreatic lesions. We further advocate that CT surveillance of the offspring of von Hippel-Lindau disease patients begin in the second decade, with the anticipation of curing potentially serious, blinding, or life-threatening lesions.