Snyder B J, Hanieh A, Trott J A, David D J
Australian Craniofacial Unit, Women and Children's Hospital, Adelaide, South Australia.
Plast Reconstr Surg. 1998 Sep;102(3):633-42. doi: 10.1097/00006534-199809030-00005.
Neurofibromatosis is a systemic disease that often produces striking disfigurement. Orbital manifestations are common and include sphenoid dysplasia with or without infiltration of the periorbital soft tissues. The resultant deficiency of the posterolateral orbital wall may lead to protrusion of the temporal lobe into the orbit, displacement of the globe, and pulsatile exophthalmos. Treatment at our unit has consisted of transcranial orbital reconstruction with bone grafts and periorbital soft-tissue correction. Observation of complete bone graft resorption in one patient prompted an assessment of the Australian Craniofacial Unit's experience with particular attention paid to the stability of operative correction. Of 36 patients with head and neck neurofibromatosis treated during the period from 1981 to 1995, 14 patients underwent transcranial correction of orbital deformities secondary to sphenoid dysplasia. The treatment and outcomes of this transcranial group are reviewed. The most notable finding was that of recurrent globe pulsation in four patients following initial resolution. Computed tomography scans have documented partial to complete bone graft resorption in three of these patients. Titanium mesh is now being utilized to provide a more durable reconstruction.
神经纤维瘤病是一种常导致明显容貌毁损的全身性疾病。眼眶表现很常见，包括伴有或不伴有眶周软组织浸润的蝶骨发育异常。由此导致的眶后外侧壁缺损可能会引起颞叶突入眼眶、眼球移位和搏动性眼球突出。我们科室的治疗方法包括采用骨移植进行经颅眼眶重建和眶周软组织矫正。一名患者骨移植完全吸收的情况促使我们评估澳大利亚颅面外科中心的经验，并特别关注手术矫正的稳定性。在1981年至1995年期间治疗的36例头颈部神经纤维瘤病患者中，14例患者接受了经颅矫正继发于蝶骨发育异常的眼眶畸形。本文回顾了该经颅治疗组的治疗情况及结果。最显著的发现是，4例患者在最初症状缓解后出现了眼球搏动复发。计算机断层扫描显示其中3例患者存在部分至完全的骨移植吸收。目前正在使用钛网进行更持久的重建。
