Riordan Coleman P, Storey Armide, Cote David J, Smith Edward R, Scott R Michael
J Neurosurg Pediatr. 2019 Mar 1;23(5):586-592. doi: 10.3171/2019.1.PEDS18457. Print 2019 May 1.
There are limited data on the long-term outcomes for children undergoing surgical revascularization for moyamoya disease (MMD) in North America. The authors present a series of pediatric MMD patients who underwent a standard revascularization procedure, pial synangiosis, more than 20 years previously at a single institution by a single surgical team.
This study is a retrospective review of all patients aged 21 years or younger treated for MMD at Boston Children's Hospital who were operated on more than 20 years previously by the senior author (R.M.S.). Radiographic and operative reports, outpatient clinical records, and communications with patients and families were reviewed to document current clinical status, ability to perform daily activities, and concurrent or new medical conditions.
A total of 59 patients (38 female [64.4%], 21 male [35.6%]; median age at surgery 6.2 years [IQR 0.5-21 years]) were identified who were diagnosed with MMD and underwent surgical revascularization procedures more than 20 years previously. Clinically, all but 2 patients (96.6%) presented with the following symptoms alone or in combination: 43 (73%) presented with stroke, 22 (37%) with transient ischemic attack, 12 (20%) with seizures, 7 (12%) with headache, 3 (5%) with choreiform movements, and 2 (3%) with hemorrhage; MMD was incidentally detected in 2 patients (3%). Five patients had unilateral MMD at presentation, but 3 of these ultimately progressed to develop bilateral MMD after an average of 16 months; therefore, pial synangiosis was ultimately performed in a total of 116 hemispheres during the study period. Clinical follow-up was available at a median interval of 20.6 years (IQR 16.1-23.2 years). Modified Rankin Scale scores were stable or improved in 43 of 50 patients with evaluable data; 45 of 55 are currently independent. There were 6 patient deaths (10.2%; 3 due to intracranial hemorrhage, 2 due to tumor-related complications, and 1 due to pulmonary artery stenosis), 4 of whom had a history of previous cranial radiation. One patient (1.7%) experienced a late stroke. Synangiosis vessels remained patent on all available late MRI and MRA studies. Four patients reported uneventful pregnancies and vaginal deliveries years following their revascularization procedures.
Revascularization for MMD by pial synangiosis appears to confer protection from stroke for pediatric patients over long-term follow-up. A history of cranial radiation was present in 4 of the 6 patients who died and in the lone patient with late stroke. Most patients can expect productive, independent lives following revascularization surgery in the absence of significant preoperative neurological deficits and comorbidities.
在北美，关于接受烟雾病（MMD）外科血管重建术的儿童的长期预后的数据有限。作者介绍了一系列20多年前在单一机构由单一手术团队进行标准血管重建手术——软脑膜血管吻合术的儿科MMD患者。
本研究是对波士顿儿童医院所有21岁及以下接受MMD治疗的患者进行的回顾性研究，这些患者在20多年前由资深作者（R.M.S.）进行手术。回顾了影像学和手术报告、门诊临床记录以及与患者及其家属的沟通，以记录当前的临床状况、进行日常活动的能力以及并发或新出现的医疗状况。
共确定了59例患者（38例女性[64.4%]，21例男性[35.6%]；手术时的中位年龄为6.2岁[四分位间距0.5 - 21岁]），他们被诊断为MMD并在20多年前接受了外科血管重建手术。临床上，除2例患者（96.6%）外，所有患者均单独或合并出现以下症状：43例（73%）出现中风，22例（37%）出现短暂性脑缺血发作，12例（20%）出现癫痫发作，7例（12%）出现头痛，3例（5%）出现舞蹈样动作，2例（3%）出现出血；2例患者（3%）MMD为偶然发现。5例患者初诊时为单侧MMD，但其中3例最终在平均16个月后进展为双侧MMD；因此，在研究期间共对116个半球进行了软脑膜血管吻合术。临床随访的中位间隔时间为20.6年（四分位间距16.1 - 23.2年）。在50例有可评估数据的患者中，43例改良Rankin量表评分稳定或改善；55例中有45例目前能够独立生活。有6例患者死亡（10.2%；3例死于颅内出血，2例死于肿瘤相关并发症，1例死于肺动脉狭窄），其中4例有既往颅脑放疗史。1例患者（1.7%）发生晚期中风。在所有可用的晚期MRI和MRA研究中，血管吻合血管均保持通畅。4例患者报告在血管重建手术后数年妊娠及阴道分娩过程顺利。
通过软脑膜血管吻合术对MMD进行血管重建似乎能在长期随访中为儿科患者预防中风。6例死亡患者中有4例以及唯一发生晚期中风的患者有颅脑放疗史。在没有明显术前神经功能缺损和合并症的情况下，大多数患者在血管重建手术后有望过上有意义的独立生活。
