Von Hipple Lindau disease – management of two cases with mini review

Abstract

Von Hipple Lindau syndrome (VHL) is genetically determined benign tumors of various organs with some tendency to malignant transformation. Two young male and female patients of 25 years and 18 years presented with bilateral retinal and central nervous system hemangioblastoma. One patient underwent panretinal photocoagulation and resection of cerebellar tumor and other received transpupillary thermotherapy (TTT). Proper genetic counseling and screening of the patients and close relatives were done. Though VHL is not curable, timely and proper treatment can save life and sight. The Ophthalmologist can play a role in reducing morbidity and mortality of patients and their family members by screening and proper referral.