Chen Li-Hua, Zhang Hong-Tian, Xu Ru-Xiang, Zhang Li, Li Wen-De, Sun Kai
Department of Neurosurgery, The Affiliated Bayi Brain Hospital, The PLA Army General Hospital, Beijing, China.
Medicine (Baltimore). 2018 Apr;97(17):e0270. doi: 10.1097/MD.0000000000010270.
Most patients diagnosed with neurofibromatosis type 2 (NF2) have bilateral vestibular schwannomas (VS). Through reviewing surgical method and clinical outcomes, we tried to find out a strategy for treatments in NF2 patients with VS.We retrospectively reviewed patients diagnosed pathological NF2 and have had microsurgery (MS) for VS in the PLA Army General Hospital. Seventeen patients were included from January 2000 to December 2016. Fifteen patients had progressive hearing impairment, and 7 ears were totally deaf. Computed tomography and magnetic resonance imaging were used for preoperative and postoperative evaluation. House-Brackmann (H-B) classification was used to evaluate facial function, and the hearing outcome was classified according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification system. The outcomes included functional hearing, facial function, and complications.In the 17 patients, 9 were men, and the mean age was 27.2 years old. The mean duration of disease was 38.4 months. Twenty-six VS were excised. Nine patients with bilateral VS and unilateral surgery had repeated surgery for the contralateral tumor after 3 to 12 months. The hearing preservation rate was 41.6%. In the 26 excisions for VS, 24 had intact facial nerve. In the other 2 tumor excision, damaged facial nerves had head-to-head adhesion using biological fibrin glue. The rate of facial nerve function preservation was 60%. No mortality or major complication was reported. The follow-up time ranged from 11 to 78 months with a mean value of 39 months.MS is an effective treatment for NF2 patients with VS. The operation for bilateral VS should be staged according to tumor size and bilateral hearing function. However, methods on how to preserve functional hearing and facial function remain the issue. Further randomized controlled studies are needed to find out a better treatment for NF2 patients with VS according to the overall condition.
大多数被诊断为2型神经纤维瘤病（NF2）的患者患有双侧前庭神经鞘瘤（VS）。通过回顾手术方法和临床结果，我们试图找出NF2合并VS患者的治疗策略。我们回顾性分析了在中国人民解放军总医院被病理诊断为NF2且接受了VS显微手术（MS）的患者。纳入了2000年1月至2016年12月期间的17例患者。15例患者有进行性听力减退，7耳完全失聪。术前和术后均采用计算机断层扫描和磁共振成像进行评估。采用House-Brackmann（H-B）分级评估面神经功能，听力结果根据美国耳鼻咽喉头颈外科学会（AAO-HNS）听力分级系统进行分类。结果包括功能性听力、面神经功能和并发症。17例患者中，男性9例，平均年龄27.2岁。平均病程38.4个月。共切除26个VS。9例双侧VS且接受单侧手术的患者在3至12个月后对侧肿瘤进行了再次手术。听力保留率为41.6%。在26次VS切除术中，24例面神经完整。在另外2例肿瘤切除术中，受损面神经使用生物纤维蛋白胶进行了端端粘连。面神经功能保留率为60%。未报告死亡或严重并发症。随访时间为11至78个月，平均39个月。MS是治疗NF2合并VS患者的有效方法。双侧VS的手术应根据肿瘤大小和双侧听力功能分期进行。然而，如何保留功能性听力和面神经功能仍是问题。需要进一步的随机对照研究，以便根据整体情况为NF2合并VS患者找到更好的治疗方法。
