Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease. Clinical article.
Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease. Clinical article.
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DOI：
10.3171/2009.3.jns08837
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发表时间：
2009-11
影响因子：
4.1
通讯作者：
Connolly ES Jr
中科院分区：
文献类型：
--
作者：
Starke RM;Komotar RJ;Hickman ZL;Paz YE;Pugliese AG;Otten ML;Garrett MC;Elkind MS;Marshall RS;Festa JR;Meyers PM;Connolly ES Jr
报告在美国一家机构接受治疗的成人烟雾病患者的临床特征、手术治疗和长期结局。43例成人烟雾病患者（平均年龄40+/-11岁;范围18 - 69岁）接受了脑硬脑膜动脉合管病（EDAS）治疗。神经科医生在术前和术后对患者进行了检查。通过面对面或结构化电话访谈获得随访（中位数41个月;范围4至126）。收集以下结局：短暂性脑缺血发作（TIA）、梗死、移植物侧支化、脑灌注变化和根据改良兰金量表（mRS）的功能水平。计算手术侧和对侧半球之间的Kaplan-Meier梗死风险。大多数患者为女性（65%）、高加索人（65%）、出现缺血症状（98%）和双侧疾病（86%）。19例患者接受了单侧EDAS，24例患者接受了双侧EDAS（n=67）。52例患者中有50例（98%）出现侧支血管，SPECT扫描中有41例（82%）灌注增加。围手术期半球梗死（<48小时）的发生率为3%。在随访期间，患者发生了10例TIA、6例梗死和1例颅内出血。尽管选择手术的半球是基于患者症状和病理严重程度，但手术半球的5年无梗死生存率为94%，而非手术半球的5年无梗死生存率低于36%（p=0.007）。在控制年龄和性别后，手术半球发生梗死的可能性比对侧半球低89%（风险比：0.11; 95% CI 0.02-0.56）。43例患者中有38例（88%）的mRS相对于基线状态保持或改善。在这个北美患者的混血人群中，间接旁路促进了大多数烟雾病成人患者充分的软脑膜侧支循环发育和灌注增加。患者术后TIA、梗死和出血的发生率较低，大多数患者的功能状态保持或改善。
To report the clinical features, surgical treatment, and long-term outcomes of adults with moyamoya phenomenon treated at a single institution in the United States. Forty-three adult patients with moyamoya disease (mean age of 40+/−11 years; range 18 to 69) were treated with encephaloduroarteriosynangiosis (EDAS). Neurologists examined patients pre- and post-operatively. Follow-up was obtained in-person or by structured telephone interview (median 41 months; range 4 to 126). 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 infarction risk was calculated between operated and contralateral hemispheres. The majority of patients were women (65%), Caucasian (65%), presented with ischemic symptoms (98%), and had bilateral disease (86%). Nineteen patients underwent unilateral and 24 patients bilateral EDAS (n=67). Fifty of 52 (98%) patients with available imaging developed collateral vessels, and 41 of 50 (82%) had increased perfusion on SPECT scan. The incidence of peri-procedural hemisphere infarction (<48 hours) was 3%. In the follow-up period patients experienced 10 TIAs, 6 infarcts, and 1 intracranial hemorrhage. Although the hemisphere selected for surgery was based upon patient symptoms and severity of pathology, the five year infarction free survival rate was 94% in operated hemispheres versus less than 36% in non-operated hemispheres (p=0.007). After controlling for age and sex, operative hemispheres were 89% less likely to experience infarction than contralateral hemispheres (hazard ratio: 0.11; 95% CI 0.02–0.56). Thirty-eight of 43 patients (88%) had preserved or improvement in mRS over 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.