背景:Chiari畸形1型(CM1)是一种罕见的疾病,其中仍缺少商定的分类和治疗方法。这项研究的目的是就儿童CM1的诊断和治疗达成共识。
方法:一个多学科小组根据文献综述制定了57项临时声明。34名国际专家(IE)通过对4分Likert量表的每个陈述进行独立评分,参与了Delphi研究(“强烈反对,\"\"不同意,\"\"同意,\"\"强烈同意\")。<75%的评估者认可的陈述(“同意”或“强烈同意”)被重新表述,或者添加了新的语句,接下来是另一轮Delphi(最多三轮)。
结果:联系了35个IE,34个同意参加。在第一轮之后,就30/57声明(52.6%)达成了共识。增加了三个声明,一个被删除。在第2轮之后,就56/59声明达成了协议(94.9%)。最后,在2019年Chiari共识会议期间举行的第3轮(米兰,意大利),就关于四个主要部分(定义和分类,规划,手术,孤立性脊髓空洞症)。只有一个声明没有达成共识,这是手术后24个月放射失败的定义。\"
结论:共识文件包括58项声明(24项关于诊断,34关于治疗),为CM1儿童的临床医生和研究人员服务。显然需要建立一个国际网络和登记册,并促进合作研究,以增加证据基础并优化该患者人群的长期护理。
BACKGROUND: 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.
METHODS: 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).
RESULTS: 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.\"
CONCLUSIONS: 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.