Starke Robert M, Komotar Ricardo J, Hickman Zachary L, Paz Yehuda E, Pugliese Angela G, Otten Marc L, Garrett Matthew C, Elkind Mitchell S V, Marshall Randolph S, Festa Joanne R, Meyers Philip M, Connolly E Sander
Department of Neurological Surgery, Columbia University, New York, New York 10032, USA.
J Neurosurg. 2009 Nov;111(5):936-42. doi: 10.3171/2009.3.JNS08837.
The object of this study was to report the clinical features, surgical treatment, and long-term outcomes in adults with moyamoya phenomenon treated at a single institution in the US.
Forty-three adult patients with moyamoya disease (mean age 40 +/- 11 years [SD], range 18-69 years) were treated with encephaloduroarteriosynangiosis (EDAS). Neurologists examined patients pre- and postoperatively. Follow-up was obtained in person or by structured telephone interview (median 41 months, range 4-126 months). The following outcomes were collected: transient ischemic attack (TIA), infarction, graft collateralization, change in cerebral perfusion, and functional level according to the modified Rankin scale (mRS). Kaplan-Meier estimates of infarction risk were calculated for comparison of surgically treated and contralateral hemispheres.
The majority of patients were women (65%), were Caucasian (65%), presented with ischemic symptoms (98%), and had bilateral disease (86%). Nineteen patients underwent unilateral and 24 patients bilateral EDAS (67 treated hemispheres). Collateral vessels developed in 50 (98%) of 52 hemispheres for which imaging was available and there was evidence of increased perfusion on SPECT scans in 41 (82%) of the 50 hemispheres evaluated. Periprocedural infarction (< 48 hours) occurred in 3% of the hemispheres treated. In the follow-up period patients experienced 10 TIAs, 6 infarctions, and 1 intracranial hemorrhage. Although the hemisphere selected for surgery was based upon patients' symptoms and severity of pathology, the 5-year infarction-free survival rate was 94% in the surgically treated hemispheres versus < 36% in the untreated hemispheres (p = 0.007). After controlling for age and sex, infarction was 89% less likely to occur in the surgically treated hemispheres than in the contralateral hemispheres (hazard ratio 0.11, 95% CI 0.02-0.56). Thirty-eight (88%) of 43 patients had preserved or improved mRS scores, relative to baseline status.
In this mixed-race population of North American patients, indirect bypass promoted adequate pial collateral development and increased perfusion in the majority of adult patients with moyamoya disease. Patients had low rates of postoperative TIAs, infarction, and hemorrhage, and the majority of patients had preserved or improved functional status.
本研究的目的是报告在美国一家机构接受治疗的患有烟雾病现象的成年人的临床特征、手术治疗及长期预后。
43例烟雾病成年患者（平均年龄40±11岁[标准差]，范围18 - 69岁）接受了脑硬脑膜动脉血管融通术（EDAS）。神经科医生在术前和术后对患者进行检查。通过亲自随访或结构化电话访谈获得随访结果（中位时间41个月，范围4 - 126个月）。收集以下结果：短暂性脑缺血发作（TIA）、梗死、移植血管侧支形成、脑灌注变化以及根据改良Rankin量表（mRS）评估的功能水平。计算梗死风险的Kaplan - Meier估计值，以比较手术治疗侧和对侧半球。
大多数患者为女性（65%），是白种人（65%），表现为缺血症状（98%），且患有双侧疾病（86%）。19例患者接受单侧EDAS，24例患者接受双侧EDAS（共治疗67个半球）。在可获得影像的52个半球中，50个（98%）形成了侧支血管，在评估的50个半球中有41个（82%）在SPECT扫描中有灌注增加的证据。围手术期梗死（<48小时）发生在3%的治疗半球中。在随访期间，患者经历了10次TIA、6次梗死和1次颅内出血。尽管选择手术的半球是基于患者的症状和病理严重程度，但手术治疗半球的5年无梗死生存率为94%，而未治疗半球<36%（p = 0.007）。在控制年龄和性别后，手术治疗半球发生梗死的可能性比未治疗半球低89%（风险比0.11，95%可信区间0.02 - 0.56）。43例患者中有38例（88%）相对于基线状态mRS评分保持或改善。
在这个北美患者的混合种族群体中，间接搭桥促进了大多数成年烟雾病患者软脑膜侧支的充分发育并增加了灌注。患者术后TIA、梗死和出血发生率低，且大多数患者功能状态保持或改善。
