Janes Lindsay E, Sabino Jennifer, Matthews Jamil A, Papadimitriou John C, Strome Scott E, Singh Devinder P
Division of Plastic Surgery, University of Maryland Medical Center, Baltimore, Maryland, USA.
J Craniofac Surg. 2013 Jul;24(4):1273-7. doi: 10.1097/SCS.0b013e318285d337.
Neurofibromatosis type 1 is a rare, autosomal dominant disorder than can present with varying degrees of disfigurement depending on the associated tumor extent and location. Surgical resection is considered the most effective management of these typically benign tumors, indicated when symptoms include pain, extreme deformity, or interference with normal physical function. Giant tumors of the craniofacial region present particular difficulty due to the size of the post-resection wound deficit and the high risk surgery poses to function such as vision and facial animation in this region. Strategies of management are discussed.
1型神经纤维瘤病是一种罕见的常染色体显性疾病,根据相关肿瘤的范围和位置,可能会出现不同程度的毁容。手术切除被认为是治疗这些通常为良性肿瘤的最有效方法,当出现疼痛、极度畸形或影响正常身体功能等症状时可考虑进行手术。由于切除后伤口缺损的大小以及手术对该区域视力和面部活动等功能造成的高风险,颅面部巨大肿瘤的治疗存在特殊困难。本文讨论了治疗策略。