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A RARE CASE OF VON HIPPEL LINDAU DISEASE

FEBIN K M

Abstract


A 33 yr old male referred from Neurosurgery  department as a case of cerebellar hemangioblastoma with complaints of gradual progressive loss of vision left eye  associated with headache. Similar history in father. On Ocular examination, left eye RAPD present, otherwise both eyes anterior segment was normal. Visual acuity was 660 in right eye hand movements in left eye. Fundus examination   revealed multiple tumour masses in left eye with dilatation and tortousity of supplying artery and draining vein extending from optic disc, suggesting retinal  capillary  hemangioblastoma along with tractional retinal detachment. Fundus Examination of Right eye was normal. FFA of left eye showed early hyperfluorescence, late leakage with rapid filling and exit of dye. B scan revealed vitreous hemorrhage,  vitreous and retinal detachment. MRI brain showed focal hyperintensity in cerebellum on T1 image suggesting  cerebellar hemangioblastoma. CT abdomen showed  pancreatic renal cyst, pheochromocytoma hyperintense lesion in spinal cord (spinal hemangioblastoma). The patient was advised vitreoretinal surgery with endolaser photocoagulation. This case is presented for its rarity

 


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