神经纤维瘤病-Ⅱ型显微手术治疗策略

Strategy for the microsurgical treatment of neurofibromatosis Type Ⅱ

目的探讨神经纤维瘤病-Ⅱ型(NF-2)显微手术切除的治疗策略。方法回顾性分析2000年1月至2015年12月期间16例NF2经显微手术切除的临床资料。其中双侧听神经瘤11例,另5例为Segmental型(不完全型) NF-2。均采用乙状窦后入路切除听神经瘤,术中采用面神经、三叉神经和听性脑干反应(ABR)监测。根据肿瘤大小、听力损伤程度选择手术切除时机和侧别。结果分期分侧切除双侧听神经瘤9例,单侧听神经瘤切除7例,共切除听神经瘤25侧。肿瘤全切除22侧(22/25,88%),近全切除3侧(为唯一存在听力耳)。椎管内神经鞘瘤切除3例,颅内脑膜瘤切除1例,均获得肿瘤全切除。大部分切除的3侧肿瘤和尚未行肿瘤切除的2侧听神经瘤(尚存有效听力),以及颅内1例多发性神经鞘瘤和多发性脑膜瘤1例,术后3个月内行伽玛刀治疗。术前有残余听力的11侧耳,术后5侧耳保留不同程度听力,听力保留率为45. 5%(5/11)。23侧肿瘤术中均解剖保留面神经(23/25,92%),术后半年复查面神经功能采用House-Brackmann(H-B)评分I～Ⅲ23侧,H-BⅣ级2侧。随访11～78个月,平均随访38. 4个月,肿瘤均得到有效控制,未见肿瘤复发或增大。结论 NF-2应根据肿瘤的大小、听力水平、解除脑干压迫的必要性、肿瘤的生长速度,制定恰当的手术方案。对于伴发的脑膜瘤或脊膜瘤、椎管内和颅内其它神经鞘瘤,根据是否引起临床症状来决定是否需要切除肿瘤。

Objective The strategies for microsurgical treatment and outcomes of the patients with neurofibromatosis TypeⅡ(NF-2)were explored.Methods Retrospective analysis included16consecutively neurofibromatosis2patients who underwent microsurgery for vestibular schwannoma removal with a follow-up11to78months from January2000to December2015.Eleven cases had bilateral acoustic neuromas,another5cases were Segmental(incomplete type)type NF2.The clinical informations,imaging and operative reports,follow-up neurological,audiometric examinations,and neuroradiological findings of neurofibromatosis Type2patients were analyzed.Suboccipital retrosigmoid approach was taken for25tumors of VS resection and intraoperative monitoring of the facial and trigeminal nerves and auditory brainstem responses(ABR)was used in all cases.According to tumor size and the extent of hearing disability,all the patients were treated by selective resectional timing and side of surgical resection.Results Staging side resection of bilateral acoustic neuroma was achieved in9cases,unilateral acoustic neuroma resection in 7 cases,and total resection25sides of acoustic neuroma.Total tumor removal was achieved in 22 sides(22/25,88%)and subtotal resection in 3 sides(effective hearing ear only).Three cases of intraspinal schwannoma and1case of intracranial meningioma achieved total resection.For3sides of subtotal removal and 2 sides without resection(surviving effective listening),1case of multiple intracranial schwannomas and1case of multiple meningioma,gamma knife was performed at3months after operation.For11ears with residual hearing before operation,5ears retained different degrees of hearing after operation,with a hearing preservation rate of 45.5%(5/11).Twenty-three sides of the tumor were anatomically preserved facial nerve(23/25,92%).Facial nerve function was evaluated by House-Brackmann(H-B)and the results showed I to Ⅲ on 23 sides at6months after operation and IV on 2 sides.All patients were followed up for11to78months,with an average follow-up of 38.4months.All tumors were effectively controlled and no tumor recurrence or enlargement was observed.Conclusion For NF2,tumor size,hearing level,relieve the necessity of brain stem compression,tumor growth rate,are needed to consider for the choice of appropriate surgical strategy.For combining with meningioma or spinal meningioma,spinal and intracranial other schwannomas,tumor resection should be chosen according to the clinical symptoms.