Retinal hemangioblastoma

Retinal hemangioblastomas tend to be located peripherally, and may hemorrhage and cause retinal detachment. Erythrocytosis may be due to erythropoietin liberated by the tumor.

a) Retinal hemangioblastomas occur in > 50% of Von Hippel-Lindau disease patients.Mean age of presentation:25 years

b) frequently bilateral,multifocal,and recurrent

c) often asymptomatic. Visual symptoms occur with progressive growth, edema, retinal detachments, and hard exudates

d) typically located in the periphery and near or on the optic disc

e) microangiomas measuring a few hundred microns without dilated feeding vessels maybe located in the periphery

f) retrobulbar HGB are rare (5.3% in NIH cohort)

g) severity of optic disease correlates with CNS and renal involvement

h) early diagnosis and treatment with laser photocoagulation, and cryotherapy can prevent visual loss. Low dose external XRT may be an option for refractory cases