Early identification of retinal angiomas in a large kindred von Hippel-Lindau disease

We studied prospectively a large kindred afflicted with von Hippel-Lindau disease. Of 111 members examined ophthalmoscopically, 14 definite retinal angiomas were found and three lesions were thought to be angiomas. The lesions found could be divided into the following three groups: (1) classic angiomas, (2) atypical angiomas, and (3) lesions indicative of early angiomas. A correlation between increasing age, systemic evidence of disease, and size and appearance of angiomas was found.