Surgical treatment of Cranio-facial Neurofibromatosis

ABSTRACT

Craniofacial neurofibromatosis may cause severe soft tissue deformity together with pulsating exophthalmos. The orbital cavity is often enlarged, and the greater wing of the sphenoid bone is usually absent, the eye is proptotic and blind. In these situations, enucleation of the blind eye with soft tissue excision and orbital reconstruction, followed by replacement with a suitable prosthesis, are recommended. Eight patients with localized craniofacial neurofibromatosis were treated surgically, the results were satisfactory only in two patients, acceptable in four and unsatisfactory in two. This reflects the difficulty in treating the lesion and the need for multiple operative sessions