Von Hippel tumors in siblings with retinitis pigmentosa.
The conditions of three siblings with concurrent retinitis pigmentosa and von Hippel tumors were evaluated. Their eyes showed the characteristic arteriolar attenuation expected in retinitis pigmentosa, except in the retinal sectors containing von Hippel tumors, where large feeder vessels supplied each tumor. Two of the siblings suffered further loss of vision as a result of large exudative retinal detachments associated with the von Hippel tumors. Both patients had satisfactory resolution of the retinal detachments following cryotherapy and laser therapy of their retinal tumors. The third sibling has a small retinal detachment that should te amenable to therapy. Visually handicapped patients require routine follow-up examinations to detect new diseases that may result in low of the remaining vision.
D000328:Adult; D000798:Angiomatosis / Q000150:complications / Q000235:genetics*; D005134:Eye Neoplasms / Q000150:complications / Q000235:genetics* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D010375:Pedigree; D011247:Pregnancy; D012163:Retinal Detachment / Q000150:complications / Q000235:genetics / Q000601:surgery; D012164:Retinal Diseases / Q000150:complications / Q000235:genetics*; D012174:Retinitis Pigmentosa / Q000150:complications / Q000235:genetics*