脑-硬脑膜-颞肌血管融合术治疗幼儿烟雾病

Encephaloduromyosynangiosis treating moyamoya disease in young children

目的 探讨脑-硬脑膜-颞肌血管融合术（EDMS）对幼儿烟雾病的治疗效果.方法 2011年1月至2013年9月复旦大学附属儿科医院神经外科采用EDMS治疗≤6岁的烟雾病患儿27例,其中出血型1例,缺血型26例.共40侧次手术,其中左侧8例,右侧6例,双侧13例.平均手术时间（136±22） min,术中失血量（21 ±3）ml.结果 所有患儿均得到6～38个月随访,平均15.8个月.术后3个月磁共振血管成像（MRA）和（或）血管造影（DSA）显示,40侧血管吻合侧脑膜中动脉、颞深动脉及颞浅动脉与皮质动脉之间形成广泛的血管融合,超过骨窗范围覆盖整个大脑中动脉和部分大脑前动脉、大脑后动脉的供血区域,血管融合成功率为100％;单光子发射计算机断层成像（SPECT）显示,27例脑血流灌注均得到改善;31侧次手术（77.5％）使神经功能缺损症状得到改善,8侧次手术（20.0％）使进展性症状稳定.术后1个月内共4例发生脑梗死,3例为手术对侧半球的新发脑梗死,1例为手术侧的脑梗死.所有患儿未出现手术侧出血、颞肌占位及头皮坏死等并发症.结论 EDMS治疗幼儿烟雾病安全、有效.颅外血管能够多层次大范围地与皮质血管融合.

Objectives To evaluate the therapeutic effect of encephaloduromyosynangiosis （EDMS） in young children for moyamoya disease（MMD）.Methods Twenty-seven MMD chilidren confirmed by digital subtraction angiography （DSA） or magnetic resonance angiography （MRA） in our center were admitted between January 2011 and September 2013.The clinical manifestation was described as below：1 with cerebral hemorrhage history,26 with Ischemic history.Eight left brain hemispheres,6 right hemispheres and 13 bilateral hemispheres were operated.All the procedures were performed within （1 36 ± 22） min with （21 ± 3） ml blood loss.Results The mean period of follow-up after surgery was 15.8 months,with a range of 6-38 months.（1）The cerebral blood flow in the effected regions was increased 3 months after the operations,all cases got vascularization.The spontaneous revascularization from the deep temporal artery,superficial temporal artery and the middle meningeal artery was found in DSA or MRA.（2）Twenty-seven SPECT reviewed cases had better perfusion after the operation.（3）The ischemic symptoms were relieved in 31 （77.5%） hemispheres,while stabilized in 8 （20.0%） hemispheres.There were 4 cerebral infarctions （10%） within one month after the operation,1 located in the operated hemisphere,while 3 in the contralateral sites.Conclusions EDMS was a safe,effective and secluded treatment for MMD in young children.The extracranial vessels could be revascularized in a multi-layered extensively manner,which significantly improved patients＆#39; neurological function.