‘Case series of retinal capillary hemangioma in patients with and without von hippel lindau disease’.

Abstract

The Von Hippel-Lindau (VHL) Disease is an autosomal dominant disorder that implies a genetic alteration resulting in the loss of the tumor suppressor function of the VHL gene (1) located in chromosome 3 (3p25.3). The main manifestations of VHL are hemangioblastomas of the central nervous system and retina, renal carcinomas and cysts, bilateral pheochromocytomas, cystic and solid tumors of the pancreas, cystadenomas of the epididymis, and endolymphatic sac tumors. Retinal capillary hemangiomas (RCH) are a common feature of VHL disease and are often the first manifestation of the disease, appear in 49–62% of cases (2). We report six cases of RCH seen at Hospital Selayang in which three patients are with VHL and another three are without VHL disease to observe the presenting age, clinical features, treatment modalities and visual outcome of each patients .