Pediatric Neurosurgery Department, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.
Pediatric Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Childs Nerv Syst. 2021 Jun;37(6):2011-2023. doi: 10.1007/s00381-021-05074-2. Epub 2021 Mar 10.
Moyamoya angiopathy (MM) is a chronic, progressive steno-occlusive arteriopathy of the distal internal carotid artery and its proximal branches. MM is recognized as a shared end-pathway common to a broad range of inciting pathologies, suggesting that tailored management is important. Pediatric MM differs from MM in adults. Currently, there are many uncertainties and controversies regarding the diagnosis and management of children with MM. Hence, we conducted an international survey to identify the contemporary management trends followed worldwide.
A survey relating to lifestyle modifications, medical management, diagnosis, surgical management, and follow-up for pediatric MM was circulated across web-based platforms, through various international pediatric neurological and neurosurgical societies. Data collected included geographic region of practice, experience, responses to questions, and comments.
One hundred twenty-seven responses were evaluated (104 neurosurgeons and 23 neurologists, from 32 countries, across 6 continents). We found wide variations in the recommendations for management and lifestyle modification, with significant differences between regions of practice. Eighty percent recommend restrictions on physical activity, particularly for symptomatic and non-operated patients. Eighty-four percent prescribe aspirin. Sixty-five percent perform indirect revascularization. Seventy-eight percent recommend performing a staged surgery for bilateral MM. Only 26% perform acetazolamide challenge SPECT to evaluate brain perfusion. Only 15% of responders were from highly experienced centers.
This survey reflects the contemporary trends in management of pediatric MM, while highlighting the heterogeneity in the management approach of these patients. There is a need for multicenter, international studies to evaluate the safety, efficacy, and long-term outcome of various aspects of treatment of these patients.
烟雾病（MM）是一种慢性、进行性的颈内动脉远端和其近端分支的狭窄性闭塞性血管病。MM 被认为是广泛激发性病变的共同终末途径，提示个体化治疗很重要。儿科 MM 与成人 MM 不同。目前，关于 MM 患儿的诊断和治疗存在许多不确定性和争议。因此，我们进行了一项国际调查，以确定全球范围内的当代治疗趋势。
通过各种国际儿科神经学和神经外科学会，在网络平台上发布了一项关于儿科 MM 的生活方式改变、医学管理、诊断、手术管理和随访的调查。收集的数据包括实践的地理区域、经验、对问题的回答和意见。
共评估了 127 份回复（来自 6 大洲 32 个国家的 104 名神经外科医生和 23 名神经科医生）。我们发现，在管理和生活方式改变的建议方面存在广泛差异，实践地区之间存在显著差异。80%的人建议限制体力活动，特别是对有症状和未手术的患者。84%的人开阿司匹林。65%的人进行间接血运重建。78%的人建议对双侧 MM 进行分期手术。只有 26%的人进行乙酰唑胺 SPECT 挑战以评估脑灌注。只有 15%的回复者来自经验丰富的中心。
这项调查反映了儿科 MM 治疗的当代趋势，同时突出了这些患者管理方法的异质性。需要进行多中心、国际研究，以评估这些患者治疗各个方面的安全性、有效性和长期结果。
