Long-term follow-up of surgically treated juvenile patients with Moyamoya disease.
Long-term follow-up of surgically treated juvenile patients with Moyamoya disease.
复制标题
DOI：
10.3171/2012.8.peds11539
复制
发表时间：
2012-11
期刊：
影响因子：
--
通讯作者：
M. Mukawa;T. Nariai;Y. Matsushima;Yoji Tanaka;M. Inaji;T. Maehara;M. Aoyagi;K. Ohno
中科院分区：
文献类型：
--
作者：
M. Mukawa;T. Nariai;Y. Matsushima;Yoji Tanaka;M. Inaji;T. Maehara;M. Aoyagi;K. Ohno
目的：手术血运重建术被认为是治疗青少年烟雾病（MMD）的有效方法。然而，手术治疗患者的长期结果仍然需要澄清。自作者所在科室开始使用间接吻合技术集中治疗儿童烟雾病患者以来，已经过去了30多年。在这项研究中，作者调查了这些患者的现状。方法采用改良Rankin量表（mRS）对患者进行日常生活活动（ADLs）调查和临床现状评估。手术治疗后脑血管事件也被记录。结果自1979年以来，对208例年龄小于19岁的烟雾病患者进行了手术治疗，随访3年。172例患者（83%）的数据可用，平均随访14.3年（范围3-32年）。日常生活活动结果：mRS评分0 ~ 2的患者138例（80.2%），评分为3的患者29例（16.9%），评分为4的患者1例（0.6%），评分为5的患者1例（0.6%），评分为6的患者3例（1.7%）。术后8年及以上发生脑血管事件6例（3.4%），即出血6例，梗死3例。手术后10年、20年和30年迟发性卒中的累积风险分别为0.8%、6.3%和10.0%。结论：这项长期调查表明，大多数手术治疗的儿童烟雾病患者保持良好的ADL预后。然而，大量新的脑血管事件发生在首次手术后10年以上。额外的随访将有助于确定哪些事件可能发生在作为儿童治疗的患者的成年期。
OBJECT Surgical revascularization is considered an effective treatment for juvenile patients with moyamoya disease (MMD). Yet the long-term outcome in surgically treated patients still needs to be clarified. More than 30 years have passed since the authors' department started intensively treating pediatric patients with MMD using indirect anastomosis techniques. In this study the authors surveyed the current status of these patients. METHODS Activities of daily living (ADLs) were surveyed and present clinical status was assessed based on the modified Rankin scale (mRS). Cerebrovascular events subsequent to surgical treatment were also recorded. RESULTS Since 1979, 208 patients younger than 19 years of age with MMD were surgically treated and followed up for > 3 years. Data were available on 172 patients (83%), who had been followed up for a mean of 14.3 years (range 3-32 years). Activity of daily living outcomes were as follows: 138 patients (80.2%) had mRS scores of 0-2, 29 (16.9%) a score of 3, 1 (0.6%) a score of 4, 1 (0.6%) a score of 5, and 3 (1.7%) a score of 6. Cerebrovascular events occurred 8 or more years after surgery in 6 patients (3.4%), that is, 6 hemorrhages and 3 infarctions. The cumulative risk of late-onset stroke at 10, 20, and 30 years after surgical intervention was 0.8%, 6.3%, and 10.0%, respectively. CONCLUSIONS This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good ADL outcomes. However, a significant number of new cerebrovascular events occurred more than 10 years after the initial surgery. Additional follow-up will help to identify which events may occur during the adult years of patients treated as children.