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Year : 2009  |  Volume : 2  |  Issue : 2  |  Page : 82-85

A case of von Hippel-Lindau disease with exudative maculopathy

Vitreoretinal Division, Department of Ophthalmology, Al-Hussein Hospital, King Hussein Medical Center, Amman, Jordan

Correspondence Address:
Basel T Ba'arah
Post Box: 2327, Amman - 11181
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-620X.53038

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Von Hippel-Lindau (VHL) disease is a rare multisystem familial tumor syndrome of autosomal dominant inheritance. Hallmark lesions include retinal, cerebellum and spinal cord hemangioblastomas, renal cell carcinomas, adrenal pheochromocytomas, angiomatous or cystic lesions of the kidneys, pancreas, and epididymis. We report a case of VHL disease in a 26-year-old patient who presented with exudative macular edema. Ocular and systemic studies revealed the presence of retinal and central nervous system hemangioblastomas, adrenal pheochromocytoma, multiple pancreatic, and kidney cysts. The retinal angiomas were successfully treated with argon laser photocoagulation and cryotherapy.

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