DR. JEOTSNA RAJPUT

Dr. smriti gaur, Dr.shivani shrivastava, DR. TANMAY SRIVASTAV

Abstract

Purpose: Von Hippel-Lindau disease is characterized by spectrum of multiorgan benign and malignant tumors.Aim of study was to demonstrate clinical characteristics in young male.

Method:He presented with painless diminution in vision bilaterally.He had positive family history of demise of his two elder siblings due to systemic illness.The patient underwent detailed ophthalmic examination including slit lamp and fundus examination and radiological investigations.

Results:On complete ocular examination he had simple myopia of -0.50 dioptres.On Fundus examination well-defined red lesion was seen arising from retinal capillary in right eye suggestive of retinal capillary haemangioma.On radiological examination,multiple cysts were found in kidney and pancreas.

Conclusion:On basis of examination he was diagnosed with Von Hippel-Lindau syndrome.Early detection of condition and regular follow-up is required for reduce more vision loss and detection of complications.