ENUCLEATION IN A PATIENT WITH NEUROFIBROMATOSIS TYPE 1 WITH BUPHTHALMOS AND OCULAR DEFORMITY

Abstract

To report a case of buphthalmos with neurofibromatosis (NF-1) who suffered with blindness suffered enucleation. Physical examination & histopathology were performed. Physical examination revealed Buphthalmos & vascularized cornea with lower lid ectropion and orbital deformity. Histopathologic findings shows plexiform neurofibroma in orbital tissue with scleral wall involvement. And hamartomatous proliferation in choroid & cilliary bodies with melanocytic elements. Few NF-1 cases with buphthalmos have been reported. But cases with complicationg buphthalmos (retinal Detachment & optic nerve Atrophy) suffering enucleation are very rare.

Keywords

• Neurofibromatosis-1
• plexiform neurofibroma
• buphthalmos
• enucleation