Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center
Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center
复制标题
脑硬动脉连接术治疗小儿烟雾病：单中心100例长期随访
DOI：
10.3171/2018.2.peds17591
复制
发表时间：
2018-08-01
影响因子：
1.9
通讯作者：
Wang, Qian-Nan
中科院分区：
文献类型：
--
作者：
Zhang, Yong;Bao, Xiang-Yang;Wang, Qian-Nan
目的总结脑硬脑膜动脉联合血管成形术（EDAS）治疗儿童烟雾病（MMD）的远期疗效，探讨影响EDAS疗效的因素。分析了2002年至2007年在作者所在机构接受EDAS治疗的18岁以下MMD患者的临床结局采用Kaplan-Meier方法估计EDAS后的卒中风险。神经系统预后的预测因素进行了评估。症状发作时的平均年龄为7.3 ± 40岁。家族性MMD发病率为11.3%，男女之比为1：1.16。共施行EDAS手术232例，术后并发症发生率为3%。54%的患者进行了术后数字减影血管造影，约80%的半球显示良好或极佳的结果。新生血管与延迟时间（从症状发作到首次手术）、Suzuki分期和术前卒中显著相关（均p < 0.05）。对100例患者进行了临床随访，平均随访时间为124.4 ± 10.5个月。术后10年累积生存率为96.5%，卒中风险为0.33%/人-年。92%的患者报告独立生活，无明显残疾。良好的预后与Suzuki分期低相关（p = 0.001）年龄较大的儿童和术前无卒中的儿童的临床预后更好（p < 0.05）。结论根据长期随访数据，作者得出结论，EDAS是一种安全有效的治疗儿童MMD的方法，可以降低后续神经系统事件的风险，并可以改善生活质量。缺血相关并发症的风险在年轻患者中更高，年龄较大的儿童显示出更好的结果。脑缺血越严重，代偿越大。长期临床结果在很大程度上取决于术前卒中的存在和程度。
OBJECTIVE The object of this study was to summarize the long-term effect of encephaloduroarteriosynangiosis (EDAS) for the treatment of pediatric moyamoya disease (MMD) and to investigate factors influencing the clinical outcomes of EDAS.METHODS Clinical features, angiographic findings, and clinical outcomes were analyzed among MMD patients younger than 18 years who had been treated with EDAS between 2002 and 2007 at the authors' institution The Kaplan-Meier method was used to estimate stroke risk after EDAS. Predictors of neurological outcome were assessed.RESULTS One hundred fifteen patients were identified. The mean age at symptom onset was 7.3 +/- 40 years. The incidence of familial MMD was 11.3% The female/male ratio was 1:1.16. A total of 232 EDAS procedures were performed, and the incidence of postoperative complications was 3%. Postoperative digital subtraction angiography was performed in 54% of the patients, and about 80% of the hemispheres showed good or excellent results. Neovascularization showed significant correlations with delay time (from symptom onset to first operation), Suzuki stage, and preoperative stroke (all p < 0.05). Clinical follow-up was available in 100 patients with a mean follow-up of 124.4 +/- 10.5 months. Ten-year cumulative survival was 96.5% after surgery, and the risk of stroke was 0.33%/person-year. An independent life with no significant disability was reported by 92% of the patients. A good outcome correlated with a low Suzuki stage (p = 0.001) Older children and those without preoperative stroke had better clinical outcomes (p < 0.05).CONCLUSIONS On the basis of long-term follow-up data, the authors concluded that EDAS is a safe and effective treatment for pediatric MMD, can reduce the risk of subsequent neurological events, and can improve quality of life. The risk of ischemia-related complications was higher in younger patients, and older children showed better outcomes. Compensation was greater with more prominent cerebral ischemia. The long-term clinical outcome largely depended on the presence and extent of preoperative stroke.