enucleation in a patient with neurofibromatosis type 1 with buphthalmos and ocular deformity
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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.
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Journal title:
acta medica iranicaجلد ۴۵، شماره ۶، صفحات ۵۱۵-۵۲۰
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