Winstead Michael, Sun Peter P, Martin Kenneth, Earl Janice, Neumayr Lynne, Hoppe Carolyn, Vichinsky Elliott
a Department of Hematology-Oncology , Children's Hospital and Research Center Oakland , Oakland , California , USA.
b Blood and Marrow Transplantation and Cellular Therapies Division , Children's Hospital of Pittsburgh of UPMC , Pittsburgh , Pennsylvania , USA.
Pediatr Hematol Oncol. 2017 Mar;34(2):100-106. doi: 10.1080/08880018.2017.1313917. Epub 2017 May 22.
Moyamoya syndrome occurs in sickle cell disease (SCD) as a secondary complication of large-artery stenosis. Moyamoya increases the risk of stroke, but its optimal management in SCD is not established. Encephaloduroarteriosynangiosis (EDAS) is a neurosurgical revascularization procedure for moyamoya whose use has been reported in SCD patients. We report the outcomes of 11 patients with SCD systematically evaluated for EDAS by a multidisciplinary team and compare the rate of stroke in patients who received EDAS to those who did not. Moyamoya syndrome was diagnosed by flow abnormalities on magnetic resonance angiography at median age of 8.2 years. Four patients deferred surgery. Seven patients underwent EDAS at median age of 19 years. There were no intraoperative complications, perioperative strokes, or deaths. Transient postoperative complications occurred in six cases (86%). On follow-up, three patients (43%) had no evidence of flow in their EDAS grafts, and one later developed a hemorrhagic stroke. Five EDAS patients (71%) had radiographically stable vasculopathy. Compared to the four patients who deferred surgery, the incidence of stroke in EDAS group was no different. The optimal use of EDAS in patients with SCD-associated moyamoya syndrome requires further investigation by a prospective, controlled clinical trial.
烟雾病综合征在镰状细胞病（SCD）中作为大动脉狭窄的继发性并发症出现。烟雾病会增加中风风险，但在SCD中的最佳治疗方法尚未确定。脑-硬脑膜-动脉血管融通术（EDAS）是一种针对烟雾病的神经外科血管重建手术，已有在SCD患者中使用的报道。我们报告了一个多学科团队对11例接受EDAS系统评估的SCD患者的治疗结果，并比较了接受EDAS治疗的患者与未接受治疗的患者的中风发生率。烟雾病综合征通过磁共振血管造影上的血流异常在中位年龄8.2岁时被诊断出来。4例患者推迟手术。7例患者在中位年龄19岁时接受了EDAS手术。术中无并发症、围手术期中风或死亡发生。6例（86%）出现短暂的术后并发症。在随访中，3例患者（43%）的EDAS移植物中没有血流迹象，其中1例后来发生了出血性中风。5例接受EDAS治疗的患者（71%）血管病变在影像学上稳定。与4例推迟手术的患者相比，EDAS组的中风发生率没有差异。对于SCD相关烟雾病综合征患者，EDAS的最佳使用需要通过前瞻性对照临床试验进一步研究。
