烟雾病的治疗需要结合每位小朋友的病情做到个体化治疗。一般来说，烟雾病小朋友至少需要接受颞肌贴附术暨间接血管融合术。双侧累计的孩子，需要分期完成两次间接血管融合术。10%左右的小朋友在之后的随访过程中出现后循环血管的狭窄甚至闭塞，如果造成视野视力受损的话，需要再次接受枕部血管融合手术。 随着诊治的儿童烟雾病位居全国首位，疑难病例数逐渐增多，很多小朋友起病非常急，往往直接脑梗造成肢体功能受损。对于这样的小朋友，我个人的体会是，先通过高压氧仓结合康复治疗稳定脑梗病情。在血供缓解的基础上还需要接受搭桥手术，暨联合搭桥术（间接血管融合术+血管搭桥）。手术方案包涵血管搭桥后，才能在术后第一时间提高受累大脑皮层的血供和改善神经功能。单纯的间接血管融合往往需要一到两月的时间血管代偿新生，可能来不及拯救缺血的神经元。当然儿童血管搭桥技术难度非常高，需要有足够经验并且具备血管吻合资质的医生完成。 

Enlarged encephalo-duro-myo-synangiosis treatment for moyamoya disease in young children---Wenjnun Shen

Abstract  

OBJECTIVE: To retrospectively evaluate the mid-term therapeutic effect of enlarged encephalo-duro-myo-synangiosis (EDMS) for moyamoya disease (MMD) in young children. 

METHODS: Seventy-seven children confirmed by digital subtraction angiography (DSA) or magnetic resonance angiography (MRA) were admitted between January 2011 and December 2014 in our center. Their clinical features, imagines and operation reports were studied. 

RESULTS: 4 cases were presented as cerebral hemorrhage while 73 with ischemic history.11 left brain hemispheres, 9 right hemispheres, and 57 bilateral hemispheres were operated in 143±24 min with 28±9 mL blood loss. The mean period of follow-up was 28.43±15.31 months. Cerebral blood flow increased 3 months after the operations in the previously affected regions. Spontaneous revascularization from the deep temporal artery, superficial temporal artery, and the middle meningeal artery was found by DSA or MRA. Single photon emissions computed tomography-reviewed cases had better perfusion after the operations. The ischemic symptoms were relieved in 118 (88.06%) hemispheres, and stabilized in five (8.21%) hemispheres. There were 12 cerebral infarctions (8.96%) within one month post-operatively.  

CONCLUSIONS: Enlarged EDMS is safe and effective for MMD in young children. Extensive and multi-layered revascularization could significantly preserve neurological function. Long-term effect in posterior circulation disease development need further follow-up. 全文发表于《World Neurosurgery》

2017年10月106期第9-16页。 脑-硬脑膜-颞肌血管融合术治疗幼儿烟雾病 ----沈文俊

【摘要】

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。左侧手术的8例，右侧6例，双侧13例（总共40侧次）。平均手术时间（136 ± 22）min，术中失血量(21 ± 3)ml。

结果　所有患者均得到6 ～ 38个月随访, 平均15.8个月。（1）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%。（2）术后单光子发射计算机断层成像显示，27例患者脑血流灌注均得到改善。（3）31侧次手术（77.5%）改善了神经功能,8侧次手术（20.0%）稳定了症状。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，改善术后神经功能。 

【关键词】烟雾病；幼儿；血管重建　 全文发表于《中华神经外科杂志》2015年3月31卷3期。