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目的 探讨延髓血管母细胞瘤的显微外科手术疗效.方法 2011年6月至2016年6月首都医科大学附属北京天坛医院神经外科在神经电生理监测下采用显微外科手术治疗88例延髓血管母细胞瘤患者,回顾性分析其临床资料和手术疗效.结果 88例中,肿瘤为囊实性38例,纯实性50例;单发者70例,多发者18例.合并Von Hippel-Lindau病者18例(20.5％).延髓病变均得到全切除;术后围手术期因肺部并发症死亡3例(3.4％);术后发生暂时性肺栓塞1例(1.1％)、应激性消化道出血3例(3.4％)、肺炎24例(27.3％)、术区出血5例(5.7％)、深静脉血栓形成7例(8.O％)、脑脊液漏4例(4.5％)、中枢神经系统感染6例(6.8％).81例患者获3 ～ 60个月随访,失访4例.术后6个月内,56例术前有头晕、头痛、恶心、呕吐症状者均有不同程度的好转;17例术前有肢体肌力障碍者,11例恢复正常,6例无变化;15例术前有后组脑神经障碍者,9例好转,6例无明显变化.术后肿瘤新发15例(17.0％),其中2例延髓病变全切除后4年和10年于原位新发,均行二次手术;13例于非延髓部位新发,其中10例行二次手术治疗.随访期死亡3例(3.4％).结论 在神经电生理监测下,显微外科手术治疗延髓血管母细胞瘤的疗效良好.

Objective To explore the microsurgical outcome of medulla hemangioblastornas.Methods A total of 88 cases of medulla hemangioblastoma underwent microsurgical treatment assisted with intraoperative neurophysiological monitoring at Department of Neurosurgery,Beijing Tiantan Hospital,Capital Medical University between June 2011 and June 2016,whose clinical data and surgical outcomes were retrospectively analyzed.Results Among the 88 patients of medulla hemangioblastoma,38 cases were cyst and solid and the remaining ones were purely solid.Moreover,there were 70 cases with a single lesion and 18 cases with multiple lesions.The Von Hippel-Lindau syndrome was present in 18 (20.5％) patients.All dorsal medullary tumors underwent gross total resection (GTR).Three (3.4％) deaths occurred postoperatively due to pulmonary infection.Temporary pulmonary embolism occurred in one patient.Other complications were also documented including stress-related gastrointestinal bleeding in 3 cases,pneumonia in 24 (27.3％) cases,hematoma in 5 cases,deep venous thrombosis (DVT) in 7 cases,cerebrospinal fluid (CSF) leakage in 4 cases and infection of central nervous system in 6 cases.Eighty-one patients were followed up for about 3-60 months.Dizziness,headache,nausea and vomiting were reported in 56 instances,all of which were improved to varying degrees within 6 months.Out of 17 cases with decreased muscle strength prior to operations,11 recovered and the others remained the same.Among 15 cases who presented with the lower cranial nerve-related symptoms,9 were improved but the other 6 were not.Tumor recurrence developed in 15(17.0％) patients postoperatively including 2 cases with local recurrence that occurred 4 years and 10 years post GTR,respectively,and underwent the second operation.There were 13 cases developing tumor recurrence in other regions and 10 out of them underwent the second operation.Three (3.4％) patients died during the follow-up.Conclusion Satisfactory clinical outcomes could be achieved through microsurgery assisted with intraoperative neurophysiological monitoring.