Abstract

A RARE CASE OF VON HIPPEL LINDAU SYNDROME

Dr. Vijayalakshmi Senthil, Dr. Arunkumar S. Bilodi*, Dr. MuthuRam Lingam, Dr. Sathish Kumar and Dr. Mariappan

Abstract

Von Hippel Lindau syndrome is a rare familial syndrome involving multisystem of the body characterized by abnormal growth of blood vessels. The lesions that includes retinal, cerebellum, spinal cord haemangiomas, renal cell carcinoma, adrenal pheochromocytoma, angiomatous cystic lesions of the kidneys, pancreas & epididymis. Here we reporting a case of unilateral lesion due to exaudative retinal detachment as initial manifestation of Von Hippel Lindau disease associated with renal cell carcinoma and bilateral renal cyst (Fig 1) and multiple pancreatic cysts, secondaries in both the lungs in a male aged 57 years since ten days. He was thoroughly investigated, diagnosed as Von Hippel Lindau syndrome which is very rare hereditary disease that runs in the family. Hence studied & reported.

Keywords: Retinal haemangioma, exudative retinal detachment, pancreatic cyst, renal cyst, renal cell carcinoma.