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KMID : 0602519950010010147
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Dankook Medical Journal
1995 Volume.1 No. 1 p.147 ~ p.152
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A Case of von Hippel-Lindau disease
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Abstract
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We have experienced a case of von Hippel-Lindau disease with bilateral retinal angiomas and cerebellar hemangioblastoma. The patient was a 19-year-old Korean male who visited our hospital because of the visual disturbance in the left eye for 3
years and
recently developed headache. On the ocular examination, visual acuity was 1.5 in the right eye and no light perception in the left eye. Left eye showed fixed dilated pupil with marked rubeosis iridis and intraocular pressure was 62 mmHg by
applanation
tonometry. Fundus examination of the right eye showed one small angioma measuring 0.8 disc diameter located at superonasal retina with papilledema. In the left eye, one large angioma measuring 2.5 disc diameter located at inferotemporal retina
accompanying exudative total retinal detachment. MRI scan of the brain revealed hemangioblastoma in the right cerebellar hemisphere near fourth ventricle extending to posterior portion of medulla.
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