Neurofibromatosis of the orbit and skull base

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