Von Hippel-Lindau disease is an autosomal dominant disorder that consists of some combination of retinal angioma, cerebellar or spinal cord hemangioblastoma, renal cell carcinoma, pheochromocytoma, and visceral cysts. About 25% of patients with retinal angiomas will have at least one non-ocular manifestation. About 60% of patients with non-ocular manifestations will have retinal angiomas.
Thus any patient who has Von Hippel-Lindau disease must undergo periodic ophthalmologic examination. Conversely, any patient found to have a retinal angioma should be evaluated for non-ocular manifestations.
The yellow mound fed by a large arteriole and drained by an even larger vein is a capillary hemangioma. It is located in the retinal periphery, beyond the view of the direct ophthalmoscope.
Patients often have many such lesions. If untreated, they will gradually grow, bleed, and leak serous fluid that will finally detach the retina.
Fortunately, laser photocoagulation destroys small lesions. Larger lesions are harder to extinguish; they are treated with a freezing probe placed on the outside of the globe.
