丛状神经纤维瘤的手术原则及策略  

Principles and strategies of surgical treatment of plexiform neurofibromas

作　　者：朱倍瑶 顾熠辉 王薇 魏澄江 顾斌[1,2] 李青峰[1,2] 王智超 Zhu Beiyao;Gu Yihui;Wang Wei;Wei Chengjiang;Gu bin;Li Qingfeng;Wang Zhichao(Department of Plastic and Reconstructive Surgery,Shanghai Ninth People’s Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200011,China;Neurofibromatosis Type 1 Center and Laboratory for Neurofibromatosis Type 1 Research,Shanghai Ninth People’s Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200011,China)

机构地区：[1]上海交通大学医学院附属第九人民医院整复外科,上海200011 [2]上海交通大学医学院附属第九人民医院I型神经纤维瘤病诊疗中心及Ⅰ型神经纤维瘤病实验室,上海200011

出　　处：《中华整形外科杂志》2023年第11期1244-1250,共7页Chinese Journal of Plastic Surgery

基　　金：国家自然科学基金项目(82102344,82172228)。

摘　　要：丛状神经纤维瘤(pNF)是Ⅰ型神经纤维瘤病的重要特征,对患者的生活质量和日常功能有很大的负面影响,手术是主要治疗方式。但由于其血运丰富、神经缠结、边界不清,手术切除难度高,同时存在复发和并发症的风险。该文通过广泛的文献检索和综合分析,总结了目前pNF手术治疗的各种方案和辅助技术。术前的影像学检查、血管栓塞、术中的神经监测和荧光素导航等辅助技术能有效地降低手术风险,手术时机须参考适应证和禁忌证,手术方案则根据患者实际情况决定,必要时进行多学科管理。总体来说,pNF的手术治疗需要全面地评估患者情况和采用综合性的辅助措施,以在最大程度上切除瘤体的同时,降低复发和并发症的风险。Plexiform neurofibromas(pNF)is significant hallmark of typeⅠneurofibromatosis,posing substantial negative impacts on patients’quality of life and daily functionality.Surgical intervention remains the primary modality of treatment.However,the surgery is fraught with challenges due to the rich vascularization,intricate neural involvement,and ill-defined borders of the tumors.The risk of recurrence and complications further complicates the surgical approach.Through extensive literature review and integrative analysis,this article summarizes the current surgical treatment options and adjunctive techniques available for pNF management.Preoperative radiological assessments,vascular embolization,intraoperative neural monitoring,and fluorescence navigation are among the ancillary techniques that can effectively mitigate surgical risks.The timing of surgery should be guided by specific indications and contraindications,while the surgical plan must be individualized based on the patient’s unique presentation,sometimes necessitating a multidisciplinary approach.Overall,a comprehensive assessment of the patient’s condition and the implementation of multifaceted adjunctive measures are imperative for maximizing tumor resection while minimizing the risks of recurrence and complications.

关 键 词：神经纤维瘤病Ⅰ型 外科手术 磁共振成像 血管造影术 栓塞 术中神经生理学监测 复发 

分 类 号：R596[医药卫生—内科学]