A Case of von Hippel-Lindau's Disease

ABSTRACT

The authors presented a case of von Hippel-Lindau's disease. The patient was a 33-year-old man who visited to our hospital with visual disturbance on the right eye for a month and blind in the left eye for 10 years on the 4th May, 1973. On the eye examination, the left eye revealed ocular hypertension, deep chamber, and vitreous opacity which interfered with the visuality of the fundus. The right eye was normotensive and 0.1 incorrectable in visual acuity. The fundus revealed two angioma associated with large feeding vessels, scattered hemorrhages, and massive exudates along the engorged and tortuous vessels at the temporal retina. On general physical examination, no neurological signs were found, but multiple hard corn sized masses were palpated on the both sides of epididymal head. The masses were confirmed as epididymal cyst on microscopic examination. The x-ray findings were pulmonary tuberculosis far advanced on chest, an extrinsic pressure indentation in the floor of bladder on I.V.P., and abnormal intracranial vascularity, on right carotid angiogram. The blood chemistry showed an abnormal increase of amylase and lipase titers.