NEUROFIBROMATOSIS OF THE ORBIT AND SKULL BASE

Von Recklinghausen's disease is characterized by multiple neurofibroma s, pigmentations, and pachydermatoceles of the skin depending on the d isorder of the neural crest derivative. We report on a 25-year-old pat ient suffering from neurofibromatosis localized in the cranio-orbital region, whose main problem was a pulsating right exophthalmos caused b y herniation of the frontotemporal lobe through a defect of the greate r wing of the sphenoid. The lesion was approached through a combined r oute (i.e., transfrontally and transfacially [upper eyelid incision]). Surgical strategy and associated problems are discussed.