1Division of Pediatric Neurosurgery, Department of Neurosurgery; and.
Departments of2Internal Medicine.
J Neurosurg Pediatr. 2021 Apr 23;27(6):620-628. doi: 10.3171/2020.9.PEDS20347. Print 2021 Jun 1.
In Chiari malformation type I (CM-I), a variety of imaging findings have been purported to be important; however, results have been inconclusive, inconsistent, or not replicated in independent studies. The purpose of this study was to report imaging characteristics for a large cohort of patients with CM-I and identify the imaging findings associated with surgical decompression.
Patients were identified using ICD-9 codes for CM-I for the period from 1996 to 2017. After review of the medical records, patients were excluded if they 1) did not have a diagnosis of CM-I, 2) were not evaluated by a neurosurgeon, or 3) did not have available preoperative MRI. Retrospective chart review was performed to collect demographic and clinical data. Imaging parameters were measured according to the Chiari I Malformation Common Data Elements.
A total of 731 patients were included for analysis, having a mean follow-up duration of 25.5 months. The mean age at presentation was 8.5 years. The mean tonsil position was 11.4 mm below the foramen magnum, and 62.8% of patients had a pegged tonsil shape. Two hundred patients (27.4%) underwent surgery for life-dominating tussive headache, lower cranial nerve dysfunction, syrinx, and/or brainstem dysfunction. Surgical treatment was associated with a syrinx (OR 20.4, 95% CI 12.3-33.3, p < 0.0001), CM-1.5 (OR 1.797, 95% CI 1.08-2.98, p = 0.023), lower tonsil position (OR 1.130, 95% CI 1.08-1.18, p < 0.0001), and congenital fusion of cervical vertebrae (OR 5.473, 95% CI 1.08-27.8, p = 0.040). Among patients with benign CM-I, tonsil position was statistically significantly associated with future surgery.
Comprehensive imaging characteristics for a large cohort of patients with CM-I are reported. Analysis showed that a lower tonsillar position, a syrinx, and CM-1.5 were associated with undergoing posterior fossa decompression. This study demonstrates the importance of considering imaging findings in the context of patient symptomatology.
在 Chiari 畸形 I 型（CM-I）中，多种影像学表现被认为具有重要意义；然而，这些结果在独立研究中并不明确、不一致或无法复制。本研究的目的是报告大量 CM-I 患者的影像学特征，并确定与手术减压相关的影像学发现。
使用 1996 年至 2017 年的 CM-I 的 ICD-9 编码识别患者。在审查病历后，如果患者 1）没有 CM-I 的诊断，2）未由神经外科医生评估，或 3）没有术前 MRI，则将其排除在外。对病历进行回顾性分析，以收集人口统计学和临床数据。根据 Chiari I 畸形通用数据元素测量影像学参数。
共纳入 731 例患者进行分析，平均随访时间为 25.5 个月。发病时的平均年龄为 8.5 岁。平均扁桃体位置低于枕骨大孔 11.4mm，62.8%的患者扁桃体呈钉状。200 例（27.4%）患者因咳嗽性头痛、颅神经功能障碍、脊髓空洞症和/或脑干功能障碍而接受手术治疗。手术治疗与脊髓空洞症（OR 20.4，95%CI 12.3-33.3，p < 0.0001）、CM-1.5（OR 1.797，95%CI 1.08-2.98，p = 0.023）、低位扁桃体位置（OR 1.130，95%CI 1.08-1.18，p < 0.0001）和颈椎先天性融合（OR 5.473，95%CI 1.08-27.8，p = 0.040）相关。在良性 CM-I 患者中，扁桃体位置与未来手术显著相关。
报告了大量 CM-I 患者的综合影像学特征。分析表明，低位扁桃体、脊髓空洞症和 CM-1.5 与后颅窝减压有关。本研究表明，在考虑患者症状的情况下，影像学发现非常重要。
