Clauser L, Carinci F, Galie M
Department of Cranio-Maxillo-Facial Surgery, St. Anna Hospital, Ferrara, Italy.
J Craniofac Surg. 1998 May;9(3):280-4. doi: 10.1097/00001665-199805000-00020.
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.
冯·雷克林霍增氏病的特征是存在多个神经纤维瘤、色素沉着以及因神经嵴衍生物紊乱导致的皮肤厚皮膨出。我们报告了一名25岁患有局限于颅眶区域神经纤维瘤病的患者，其主要问题是额颞叶通过蝶骨大翼缺损疝出导致右侧搏动性眼球突出。通过联合入路（即经额部和经面部[上睑切口]）处理该病变。讨论了手术策略及相关问题。
