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Year : 2013  |  Volume : 5  |  Issue : 2  |  Page : 90-92

Von Hippel Lindau disease with exudative retinal detachment and retinal hemangioma

Department of Ophthalmology, Dr. D. Y. Patil Medical College & Hospital, Pune, Maharashtra, India

Correspondence Address:
Akash P Shah
Dr. D. Y. Patil Medical College & Hospital, Pune, Maharashtra
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DOI: 10.4103/1858-540X.124849

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Von Hippel Lindau (VHL) disease is a rare multisystem autosomal dominant disease characterized by retinal, cerebellum and spinal cord hemangioblastomas, renal cell carcinomas, adrenal pheochromocytomas, angiomatous or cystic lesions of the kidneys and pancreas. Early detection and treatment of VHL disease may prevent loss of vision and loss of life. We report a case of VHL disease in a 34-year-old male who presented to us with a massive exudative retinal detachment with multiple hemangiomas in right eye and peripheral angioma with regressed papillomacular bundle angioma in left eye. Vision in the right eye was already lost. He had mass in head region of pancreas, cholelithiasis and right renal cortical cyst. Patient had undergone bilateral adrenalectomy for pheochromocytoma, craniotomy for cerebellar hemangioma and photodynamic therapy in left eye for papillomacular bundle angioma.

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