Pediatric Neurosurgery, Fondazione Policlinico Universitario A. Gemelli IRCCS, Largo A. Gemelli, 8, 00168, Rome, Italy.
Pediatric Neurosurgery, AOU Citta' della Salute e della Scienza di Torino, Torino, Italy.
Neurol Sci. 2022 Feb;43(2):1311-1326. doi: 10.1007/s10072-021-05317-9. Epub 2021 Jun 7.
Chiari malformation type 1 (CM1) is a rare condition where agreed classification and treatment are still missing. The goal of this study is to achieve a consensus on the diagnosis and treatment of CM1 in children.
A multidisciplinary panel formulated 57 provisional statements based on a review of the literature. Thirty-four international experts (IE) participated in a Delphi study by independently rating each statement on a 4-point Likert scale ("strongly disagree," "disagree," "agree," "strongly agree"). Statements that were endorsed ("agree" or "strongly agree") by < 75% of raters were re-formulated, or new statements were added, and another Delphi round followed (up to a maximum of three).
Thirty-five IE were contacted and 34 agreed to participate. A consensus was reached on 30/57 statements (52.6%) after round 1. Three statements were added, and one removed. After round 2, agreement was reached on 56/59 statements (94.9%). Finally, after round 3, which took place during the 2019 Chiari Consensus Conference (Milan, Italy), agreement was reached on 58/59 statements (98.3%) about four main sections (Definition and Classification, Planning, Surgery, Isolated Syringomyelia). Only one statement did not gain a consensus, which is the "definition of radiological failure 24 month post-surgery."
The consensus document consists of 58 statements (24 on diagnosis, 34 on treatment), serving clinicians and researchers following children with CM1. There is a clear need for establishing an international network and registry and to promote collaborative studies to increase the evidence base and optimize the long-term care of this patient population.
Chiari 畸形 1 型（CM1）是一种罕见的疾病，目前仍缺乏共识的分类和治疗方法。本研究旨在就儿童 Chiari 畸形 1 型的诊断和治疗达成共识。
一个多学科小组根据文献回顾制定了 57 项临时陈述。34 名国际专家（IE）通过对每个陈述进行 4 分李克特量表（“非常不同意”、“不同意”、“同意”、“非常同意”）的独立评分，参与了一项德尔菲研究。未被<75%的评分者认可的陈述被重新制定，或添加新的陈述，然后进行下一轮德尔菲研究（最多三轮）。
联系了 35 名 IE，其中 34 名同意参与。第一轮达成了 30/57 项声明（52.6%）的共识。增加了三项陈述，删除了一项陈述。第二轮达成了 56/59 项声明（94.9%）的共识。最后，在 2019 年 Chiari 共识会议（意大利米兰）期间进行的第三轮中，就四个主要部分（定义和分类、计划、手术、孤立性脊髓空洞症）达成了 58/59 项声明（98.3%）的共识。只有一项声明未达成共识，即“术后 24 个月影像学失败的定义”。
共识文件由 58 项声明（24 项诊断，34 项治疗）组成，为治疗 Chiari 畸形 1 型儿童的临床医生和研究人员提供参考。显然需要建立一个国际网络和登记处，并促进合作研究，以增加该患者群体的证据基础并优化长期护理。
