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首页> 外文期刊>Neurosurgical focus >Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases
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Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases

机译:镰状细胞性贫血患儿moyamoya综合征的心房滑膜血管合成:报道病例的全面回顾

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Object Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature. Methods The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes. Results The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003). Conclusions Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.
机译:对象镰状细胞性贫血(SCA)的儿科患者具有发生烟雾病综合征(MMS)和脑缺血的重大风险。作者试图通过对文献中所有先前报道的病例进行全面的综述,以回顾性评估脊髓性血管新生在治疗SCA患儿MMS中的安全性和有效性。方法作者回顾性回顾了纽约摩根士丹利儿童医院(MSCHONY)治疗的17例小儿SCA患者的临床和影像学记录,这些患者在1996年至2012年间获得了MMS的放射学证据并经历了小脑突触形成。在文献中报道了该人群的小脑突触血管形成病例,以对临床和影像学结果进行综合分析。结果合并数据包括对30例患者进行的48例心室滑膜增生手术。其中27例(90%)表现为癫痫发作,中风或短暂性脑缺血发作,而3例(10%)经颅多普勒筛查后转诊。手术时,中位年龄为12岁。 13名患者(43%)在接受长期输血治疗时发生了缺血性中风。 39个(81%)接受治疗的半球术后平均25个月可以进行长期随访成像(MR血管造影或导管血管造影)。在这些半球中的34个(87%)中,有明显的侧支血管成像。在1590个月的累积随访中有4次神经系统事件,或每33个患者年1次事件。在可获得完整数据的患者中(MSCHONY系列,n = 17),术后卒中发生率比术前下降了六倍以上(p = 0.0003)。结论伴有SCA,MMS和脑缺血的患者发生颈椎滑膜增生是一种安全有效的治疗方法。建议对无症状的SCA患者行经颅多普勒和/或MRI检查以诊断MMS。

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