Le neurofibrome plexiforme orbito-temporal : à propos de 6 cas

Abstract

Orbito-temporal plexiform neurofibroma is characteristic of Von Recklinghausen neurofibromatosis. We report 6 cases of orbito-temporal plexiform neurofibromas and review different aspects of diagnosis, course and treatment. Six patients, 4 males and 2 females, with orbito-temporal plexiform neurofibromas were treated between 1986 and 2003. Patient age varied between 10 and 29 years (age average=19). Computed tomography and magnetic resonance imaging were performed in all 6 patients. Surgical resection of the neurofibroma was performed in all patients. The tumor was located on the left in 5 cases and on the right in one. Spheno-orbital dysplasia was found in all patients and was associated with fronto-temporal meningoencephalocele and pulsate exophthalmia. Surgical resection enabled reduction of the neurofibroma. Exenteration was performed in all patients because of a nonfunctional eye. Two patients underwent cure of fronto-temporal meningoencephalocelus with reconstruction of the large wing of the sphenoid using an iliac bone graft and a titanium plate. Surgery is essential for orbito-temporal plexiform neurofibroma but is usually difficult, particularly when the tumor has developed increasing the risk of hemorrhage. Bony reconstruction is difficult considering the risk of progressive osseous dysplasia. The cosmetic results achieved in this patient were considered good.