Paper Information

Journal:   ACTA MEDICA IRANICA   NOVEMBER-DECEMBER 2007 , Volume 45 , Number 6; Page(s) 515 To 520.
 
Paper: 

ENUCLEATION IN NEUROFIBROMATOSIS-1 WITH BUPHTHALMOS AND OCULAR DEFORMITY

 
Author(s):  ASADI AMOLI F.*, ARIAPAD A., TABATABAII S.Z.A.D.
 
* DEPARTMENT OF PATHOLOGY, 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.

 
Keyword(s): NEUROFIBROMATOSIS-1, PLEXIFORM NEUROFIBROMA, BUPHTHALMOS, ENUCLEATION
 
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