Department of Neurological Surgery, University of California, San Francisco, CA, USA.
Department of Radiology, University of California, San Francisco, CA, USA.
J Clin Neurosci. 2020 Sep;79:137-143. doi: 10.1016/j.jocn.2020.07.016. Epub 2020 Aug 19.
Moyamoya is a progressive cerebrovascular arteriopathy that affects children of any age. The goal of this study was to determine imaging and clinical outcomes as well as complication rates in a pediatric cohort undergoing either a combined direct/indirect or indirect-only revascularization approach. Patients with moyamoya disease or syndrome ≤ 18 years of age at the time of initial surgery were identified, and clinical data were collected retrospectively. Over a 12-year period, 26 patients underwent revascularization procedures on 49 hemispheres with a median follow-up of 2.6 years from surgery. Median age at surgery was 7.3 years (range 1.4-18.0 years). Thirty-three hemispheres (67.3%) underwent combined revascularization with a direct bypass and encephalomyosynangiosis, and sixteen hemispheres (32.7%) underwent indirect-only revascularization. The rate of 30-day perioperative complication was 10.2%, and the rate of postoperative clinical stroke by end of follow-up was 10.2% by hemisphere. There was a 5.7% rate of intraoperative bypass failure requiring conversion to an indirect revascularization approach. On follow-up imaging, 96.9% of direct bypasses remained patent. On multivariate analysis, higher preoperative Pediatric Stroke Outcome Measure (PSOM) scores were associated with lower rates of good clinical outcome on follow-up (unit OR 0.03; p = 0.03). Patients with age < 5.4 years had lower rates of good clinical outcome on follow-up. In this North American cohort, both combined direct/indirect and indirect only revascularization techniques were feasible. However, younger children < 5.4 years of age have worse outcomes than older children, similar to east Asian cohorts.
烟雾病是一种影响任何年龄段儿童的进行性脑血管病。本研究的目的是确定接受直接/间接联合或单纯间接血运重建的儿科患者的影像学和临床结果以及并发症发生率。回顾性收集了初始手术时年龄≤18 岁的烟雾病或烟雾综合征患者的临床资料。在 12 年期间，26 名患者在 49 个半脑上进行了血运重建手术，手术至随访的中位数时间为 2.6 年。手术时的中位年龄为 7.3 岁（范围 1.4-18.0 岁）。33 个半脑（67.3%）行直接旁路联合脑肌血管融合术的联合血运重建，16 个半脑（32.7%）行单纯间接血运重建。30 天围手术期并发症发生率为 10.2%，随访期末以半脑为单位的术后临床卒中发生率为 10.2%。有 5.7%的术中旁路失败率，需要转换为间接血运重建方法。随访影像学检查显示，96.9%的直接旁路仍然通畅。多因素分析显示，术前小儿脑卒中结局量表（PSOM）评分较高与随访时良好的临床结局相关（单位 OR 0.03；p=0.03）。年龄<5.4 岁的患者随访时良好临床结局的发生率较低。在这个北美队列中，直接/间接联合和单纯间接血运重建技术都是可行的。然而，5.4 岁以下的儿童比年龄较大的儿童预后更差，这与东亚队列相似。
