Author/Authors :
Asadi-Amoli, F Department of Pathology - Farabi Eye Hospital - School of Medicine - Medical Sciences/ University of Tehran - Tehran, Iran , Ariapad, A Department of Pathology - Farabi Eye Hospital - School of Medicine - Medical Sciences/ University of Tehran - Tehran, Iran , Tabatabaei, S.Z Department of Ophthalmology - Farabi Eye Hospital - School of Medicine - Medical Sciences/ University of Tehran - Tehran, Iran
Abstract :
In infants with neurofibromatosis type 1, plexiform neurofibroma with altering anterior
segment outflow system may result in raised intraocular pressure. Here we report a case of buphthalmos
with neurofibromatosis (NF-1) who suffered with blindness suffered enucleation. Physical examination
and histopathology were performed. Physical examination revealed Buphthalmos and vascularized
cornea with lower lid ectropion and orbital deformity. Histopathologic findings showed plexiform
neurofibroma in orbital tissue with scleral wall involvement. and hamartomatous proliferation in
choroid and cilliary bodies with melanocytic elements. Few NF-1 cases with buphthalmos have been
reported. But cases with complicationg buphthalmos (retinal detachment and optic nerve atrophy)
suffering enucleation are very rare
