1Department of Neurosurgery, Angers University Hospital, Angers.
2Department of Pediatric Neurosurgery, Necker-Enfants Malades Hospital, Assistance Publique-Hôpitaux de Paris.
J Neurosurg Pediatr. 2022 Jun 3;30(2):210-216. doi: 10.3171/2022.4.PEDS21410. Print 2022 Aug 1.
Chiari malformation type I (CM-I) is frequent in children and remains a surgical challenge. Several techniques have been described for posterior fossa decompression. No decision algorithm has been validated, and strategies are highly variable between institutions. The goal of this study was to define therapeutic guidelines that take into consideration patient specificities.
The authors retrospectively collected data from patients who were < 18 years of age, were diagnosed with CM-I, and were treated surgically between 2008 and 2018 in 8 French pediatric neurosurgical centers. Data on clinical features, morphological parameters, and surgical techniques were collected. Clinical outcomes at 3 and 12 months after surgery were assessed by the Chicago Chiari Outcome Scale. The authors used a hierarchical clustering method to define clusters of patients by considering their anatomical similarities, and then compared outcomes between surgical strategies in each of these clusters.
Data from 255 patients were collected. The mean age at surgery was 9.6 ± 5.0 years, syringomyelia was reported in 60.2% of patients, the dura mater was opened in 65.0% of patients, and 17.3% of patients underwent a redo surgery for additional treatment. The mean Chicago Chiari Outcome Scale score was 14.4 ± 1.5 at 3 months (n = 211) and 14.6 ± 1.9 at 12 months (n = 157). The hierarchical clustering method identified three subgroups with potentially distinct mechanisms underlying tonsillar herniation: bony compression, basilar invagination, and foramen magnum obstruction. Each cluster matched with specific outcomes.
This French multicenter retrospective cohort study enabled the identification of three subgroups among pediatric patients who underwent surgery for CM-I, each of which was associated with specific outcomes. This morphological classification of patients might help in understanding the underlying mechanisms and providing personalized treatment.
Chiari 畸形 I 型（CM-I）在儿童中很常见，仍然是一个手术挑战。已经描述了几种用于后颅窝减压的技术。尚未验证任何决策算法，并且机构之间的策略差异很大。本研究的目的是定义考虑患者特异性的治疗指南。
作者回顾性收集了 2008 年至 2018 年间在法国 8 个儿科神经外科中心接受手术治疗的年龄<18 岁、诊断为 CM-I 的患者的数据。收集了临床特征、形态参数和手术技术的数据。术后 3 个月和 12 个月通过芝加哥 Chiari 结局量表评估临床结局。作者使用层次聚类方法，通过考虑患者的解剖相似性来定义患者聚类，然后在每个聚类中比较手术策略之间的结果。
共收集了 255 名患者的数据。手术时的平均年龄为 9.6±5.0 岁，60.2%的患者存在脊髓空洞症，65.0%的患者硬脑膜开放，17.3%的患者因额外治疗而进行再次手术。211 名患者术后 3 个月的平均芝加哥 Chiari 结局量表评分为 14.4±1.5，157 名患者术后 12 个月的平均评分为 14.6±1.9。层次聚类方法确定了三个潜在具有不同扁桃体疝机制的亚组：骨压迫、基底凹陷和枕骨大孔阻塞。每个亚组都与特定的结果相关。
这项法国多中心回顾性队列研究能够识别出接受 CM-I 手术的儿科患者中的三个亚组，每个亚组都与特定的结果相关。这种患者的形态分类可能有助于理解潜在机制并提供个性化治疗。
