Von Hippel Lindau disease (VHL) is a rare hereditary disease characterized by the development of highly vascularised tumors in various organs. Some of these benign tumors are referred to as angiomas or hemangioblastomas. There is often ophthalmological damage, with angiomas (multiple in many cases) located in the retina.
The diagnosis is based on examination of the fundus and fluorescein angiography. In the absence of treatment, retinal angiomas develop, increase in size and may be responsible for severe retinal complications that can lead to the total loss of the eye.
Treatment is designed to destroy the angiomas. It is based on laser photocoagulation and cryo-application. Most patients develop new angiomas after the initial lesion. Regular ophthalmological screening is therefore required for the rest of the patient’s life.