Von Hippel-Lindau: Retinal Angioma
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, , and leak serous fluid that will finally detach the retina.
Fortunately, destroys small lesions. Larger lesions are harder to extinguish; they are treated with a freezing probe placed on the outside of the globe.
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.
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