Moyamoya disease and Moyamoya syndrome: experience in Europe; choice of revascularisation procedures
Moyamoya disease and Moyamoya syndrome: experience in Europe; choice of revascularisation procedures
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DOI：
10.1007/s00701-003-0148-5
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发表时间：
2003-12-01
影响因子：
2.4
通讯作者：
Yonekawa, Y
中科院分区：
文献类型：
--
作者：
Khan, N;Schuknecht, B;Yonekawa, Y
目标.介绍我们在第一组23例欧洲烟雾病血管病（疾病和综合征）患者中疾病表现和血运重建术选择方面的临床经验。自1997年至2001年，我科共收治23例患者。所有患者均接受术前血管造影、彩色多普勒检查、头颅MRI和/或CT扫描、HMPAO-SPECT或（H2O）-O-15 PET检查19名患者表现为儿童-青少年烟雾病血管病，平均发病年龄为8岁。4例成人Moyamoya，平均发病年龄34岁（23-40岁）。除一名患者外，所有患者在诊断时均存在双侧病变。所有患者均接受了直接和/或额外的间接血运重建手术。21例患者接受了双侧血运重建术。2例患者行单侧手术，所有患者均行STA-MCA直接搭桥术（颞浅动脉分支-大脑中动脉分支吻合术）。10例患者接受了额外的直接STA-ACA旁路（STA -大脑前动脉分支吻合）。3例患者在大脑后动脉（PCA）区域使用枕动脉进行间接动脉联合。另2例采用STA额分支行额部动脉联合。14例行间接额部硬脑膜血管成形术。根据血管造影结果以及（H2O）-O-15 PET上观察到的灌注储备减少部位确定多次血运重建手术的数量和位置。7例患者行一期血运重建术。14例患者接受了二期手术。除1例成人患者因非手术侧大脑中动脉大面积梗死术后死亡外，所有患者术后均未发生并发症。由于区域性血流动力学障碍似乎是烟雾病和/或综合征的特征，根据我们系列中欧洲患者的经验和迄今为止的几份报告，认为多次血运重建术治疗是合理的。
Objectives. To present our clinical experience in terms of disease presentation and choice of revascularisation procedure in our first group of 23 European Moyamoya angiopathy (disease and syndrome) patients.Method and patient selection. Twenty three patients were diagnosed and treated from 1997-2001 in our neurosurgical department. All patients underwent preoperative angiography, colour Doppler examination, cranial MRI and/or CT scans, HMPAO-SPECT or (H2O)-O-15 PET (baseline and Diamox challenge) scans.Nineteen patients presented with child-juvenile Moyamoya angiopathy with an average age at presentation of 8 years (range 1-17 years), in 4 patients adult Moyamoya was diagnosed with a mean age at presentation of 34 years (range 23-40 years).Results. In all but one patient bilateral affection was present at the time of diagnosis. All patients underwent direct and/or additional indirect revascularisation procedures. Twenty-one patients underwent bilateral revascularisation procedures. In two patients a unilateral procedure was performed.A direct STA-MCA bypass (superficial temporal artery branch - middle cerebral artery branch anastomosis) was performed in all patients. Additional direct STA-ACA bypass (STA - anterior cerebral artery branch anastomosis) was performed in 10 patients. Indirect arteriosynangiosis using the occipital artery was performed in the posterior cerebral artery (PCA) territory in 3 patients. The frontal branch of the STA was used for arteriosynangiosis in the frontal region in another 2 patients. Indirect frontal durasynangiosis was performed in 14 patients. The number and location of multiple revascularisation procedures was determined according to the angiographic findings as well as the site of decreased perfusion reserves seen on (H2O)-O-15 PET. A one stage revascularisation procedure was performed in 7 patients. Fourteen patients underwent two stage procedures. Following operation no complications were encountered in all but one adult patient who died postoperatively due to a massive middle cerebral artery infarct on the nonoperated side.Conclusions. As territorial hemodynamic disorder seems to be a characteristic in Moyamoya disease and/or syndrome, judging from our experience with European patients in our series, and several reports hitherto, treatment with multiple revascularisation procedures is considered to be justified.