显微神经外科手术联合血管内栓塞治疗延髓背侧实质性多血性血管母细胞瘤

目的 探讨应用显微神经外科手术联合血管内栓塞治疗延髓背侧实质性多血性血管母细胞瘤的方法.方法 回顾性地分析11例延髓背侧实质性血管母细胞瘤患者采用显微神经外科手术联合血管内栓塞治疗方法,临床效果通过KPS评分进行评价.所有的患者均进行了术前的神经系统影像学检查,包括核磁共振成像（MRI）和全脑血管造影（DSA）.首先在全麻插管下对异常血管团进行血管内栓塞治疗,在凝血指标正常后,即一般在介入治疗后2周内再进行显微神经外科手术切除实质性血管母细胞瘤.结果 11例患者栓塞过程顺利,栓塞后即刻造影显示肿瘤血管和肿瘤染色大部分消失,未出现并发症.肿瘤均经显微神经外科手术全切除,术中均未输血,无手术死亡.术后神经系统的功能障碍改善和恢复者10例,1例因术后急性心肌梗死死亡.术后的随访显示除死亡病例外,肿瘤全切者无肿瘤复发,且KPS评分均有所增加.手术并发症主要有：交通性脑积水,切口感染,肺炎,切口脑脊液漏等.而且无论血管栓塞还是手术治疗,术中及术后均无正常灌注压突破综合征（NPPB）的发生.结论 对延髓背侧实质性血管母细胞瘤患者进行术前栓塞,对于提高手术的安全性,减少手术并发症具有一定的意义,而且术前对于供瘤动脉的栓塞范围应个体化,以不损伤重要脑组织供血为原则.

Combined microneurosurgical treatment and intravascular embolotherapy for solid and sanguineous hemangioblastoma of dorsal medulla oblongata

Objective To investigate the application of combined microneurosurgical treatment and intravascular embolotherapy for solid and sanguineous hemangioblastoma of the dorsal medulla oblongata.Methods The clinical data of 11 solid hemangioblastomas of the dorsal medulla oblongata underwent combined microneurosurgical treatment and intravascular embolotherapy were retrospectively analyzed.Clinical outcomes were evaluated by Karnofsky Performance Scale （KPS） and all patients received preoperative magnetic resonance image （MRI） and digital subtraction angiography （DSA）.Abnormal vessels were intravascularly embolized at first,when the index of blood clotting was normal after 2 weeks,solid hemangioblastomas were resected microneurosurgically.Results 11 hemangioblastomas were successfully embolized,and the post-embolotherapy DSA showed the tumor vessels and tumor stains were almost vanished,and no complications occurred.Total tumor removal was achieved in 11 patients.None required intraoperative blood transfusion,and no death occurred.Neurologic functional disturbances in 10 patients improved or recovered,and 1 patient died of acute myocardial infarction after the operation.Follow-up showed no tumor recurrence,and postoperative increase of KPS scores were recorded in all patients.The complications included communicating hydrocephalus,incision infection,pneumonia and cerebrospinal fluid leakage.Most importantly,there was no normal perfusion pressure breakthrough （NPPB） during or after the intravascular embolotherapy and operation.Conclusions Preoperative intravascular embolotherapy is a safe and effective adjunctive therapy for solid hemangioblastoma of the dorsal medulla oblongata,which can reduce the operating bleeding,shorten the operating time,and be helpful to totally remove the tumors,relieve the injury of brain tissue and decrease the postoperative complications.