Angiomatosis retinae is one of a group of congenital, hereditary, and familial diseases known as phacomatoses (φακός, meaning birthmark). Angiomatosis of the retina has been known to ophthalmologists for a long time and has been reported in one form or another since 1879 (Panas and Rémy1). Von Hippel2 established it as an ocular clinical entity in 1904. It was not until 1926 that Lindau3 recognized its relationship to cerebellar cysts. Since then it has been frequently designated as von Hippel-Lindau disease, but such designation is valid only if a cerebellar cyst can be demonstrated.
Angiomatosis retinae is a rare disease. About 150 cases have been reported in the literature. The lesion occurs in 36% of the cases bilaterally. In the unilateral cases the left eye is more frequently involved than the right. Males are more frequently affected. The disease has a definite hereditary trend.
The disease is
KRONENBERG B. Diathermic Surgery for a Case of Angiomatosis Retinae. AMA Arch Ophthalmol. 1956;55(1):25-27. doi:10.1001/archopht.1956.00930030027006