目的:这项研究的主要目的是开发和实施一种新型的半球手术评分,以指导患有烟雾病(MMD)的儿科患者的治疗。此外,我们旨在描述一个全面的评估流程图,治疗,并对这些患者进行随访,并分享我们在转诊儿科医院对大型儿科队列进行跨学科管理的经验。
方法:我们使用儿科医院诊断为MMD的患者的病历进行了一项回顾性观察性研究。JuanP.Garrahan博士在布宜诺斯艾利斯,阿根廷,2013年7月至2023年7月。从2016年7月起,在实施半球手术评分和流程图后,对数据进行前瞻性分析.评估包括临床,MRI,和血管造影标准,患者由跨学科团队管理。人口统计,临床,收集和分析神经影像学数据。
结果:分析了40例患者的80个半球,对37例患者的72个半球进行了脑血管重建术。半球手术评分和流程图标准化治疗决策,并减少了对血管造影等侵入性研究进行随访的需求。大多数患者(79.1%)有良好的结果,在35.8个月的中位随访期间,疾病进展完全停止,影像学和临床评分均无恶化。
结论:半球手术评分和综合流程图通过规范治疗和减少不必要的侵入性操作,改善了儿科患者MMD的管理。这种跨学科的方法导致了更好的患者结果,强调需要在更大规模的研究中进一步验证,并通过随机临床试验比较不同的血运重建技术.
OBJECTIVE: The primary purpose of this study was to develop and implement a novel Hemispheric Surgical Score to guide the treatment of pediatric patients with Moyamoya disease (MMD). Additionally, we aimed to describe a comprehensive flowchart for the evaluation, treatment, and follow-up of these patients and to share our experience with the interdisciplinary management of a large pediatric cohort at a referral pediatric hospital.
METHODS: We conducted a retrospective observational study using medical records of patients diagnosed with MMD at the Pediatric Hospital \"Prof. Dr. Juan P. Garrahan\" in Buenos Aires, Argentina, from July 2013 to July 2023. From July 2016 onward, data were analyzed prospectively following the implementation of the Hemispheric Surgical Score and the flowchart. Evaluations included clinical, MRI, and angiographic criteria, and patients were managed by an interdisciplinary team. Demographic, clinical, and neuroimaging data were collected and analyzed.
RESULTS: Eighty hemispheres from 40 patients were analyzed, with cerebral revascularization performed on 72 hemispheres from 37 patients. The Hemispheric Surgical Score and flowchart standardized treatment decisions, and reduced the need for invasive studies like angiographies for follow-up. The majority of patients (79.1%) had favorable outcomes, with complete disease progression arrest and no worsening of imaging nor clinical scores during a median follow-up of 35.8 months.
CONCLUSIONS: The Hemispheric Surgical Score and the comprehensive flowchart have improved the management of MMD in pediatric patients by standardizing treatment and reducing unnecessary invasive procedures. This interdisciplinary approach has led to better patient outcomes, highlighting the need for further validation in larger studies and comparisons of different revascularization techniques through randomized clinical trials.