Department of Neurological Surgery - UT Southwestern Medical Center, Dallas, TX, USA.
Children's Medical Center, Dallas, TX, USA.
Childs Nerv Syst. 2024 Aug;40(8):2527-2534. doi: 10.1007/s00381-024-06403-x. Epub 2024 May 22.
Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement.
The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis.
Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05-4.48); p = 0.036), scoliosis (OR 5.33 (2.34-10.86); p = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01-1.30); p = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16-0.47); p = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26-0.92); p = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p = 0.049), better CCOS scores (15.4 vs 14.2; p = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p = 0.002) and reoperation (0.5% vs 35.7%; p = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04-0.95; p = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00-1.25; p = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08-5.50; p = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p = 0.028).
Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.
小儿 Chiari I 畸形（CM-I）可导致严重的神经和功能障碍。此外，脊髓空洞的存在通常预示着临床失代偿，但很少关注与脊髓空洞形成相关的特征以及脊髓空洞缩小对临床症状改善的影响。因此，本研究旨在确定与术前脊髓空洞存在和术后脊髓空洞缩小相关的临床和影像学变量，并确定术后脊髓空洞缩小与临床症状改善之间的关系。
作者对 2001 年至 2021 年在一家三级儿科医疗中心接受 CM-I 手术治疗的 435 例连续小儿患者进行了回顾性分析。所有患者均行术前和术后 MRI，记录并进行推断分析临床和影像学变量。
脊髓空洞在发病时与脊髓功能障碍的症状（比值比 2.17（95%置信区间 1.05-4.48）；p=0.036）、脊柱侧凸（比值比 5.33（2.34-10.86）；p=0.001）和更大的 pB-C2（后颅底至 C2 距离）测量长度（比值比 1.14（95%置信区间 1.01-1.30）；p=0.040）独立相关。脊髓空洞在发病时与发作性头痛（比值比 0.27（95%置信区间 0.16-0.47）；p=0.001）和发病时颅神经缺陷（比值比 0.49（95%置信区间 0.26-0.92）；p=0.025）呈负相关。术后，影像学显示脊髓空洞改善的患者症状改善率更高（93.1%比 82.1%；p=0.049），CCOS 评分更好（15.4 比 14.2；p=0.001），再入院率更低（6.0%比 25.0%；p=0.002）和再手术率（0.5%比 35.7%；p=0.001）。脊髓空洞缩小的差异虽相似但无统计学意义（10.3%比 16.7%；p=0.251）。AO 关节异常（比值比 0.20，95%置信区间 0.04-0.95；p=0.026）和枕骨大孔直径（比值比 1.12，95%置信区间 1.00-1.25；p=0.049）是脊髓空洞改善的唯一独立预测因素，手术技术是脊髓空洞缩小的唯一预测因素（比值比 2.44，95%置信区间 1.08-5.50；p=0.031）。脊髓空洞改善的患者枕骨大孔直径比脊髓空洞未改善的患者更宽（34.3 比 31.7；p=0.028）。
影像学上脊髓空洞的改善与症状改善率的提高、再入院率和再手术率的降低有关。AO 关节异常和更窄的枕骨大孔是脊髓空洞改善不良的独立危险因素。这些新的见解将有助于指导小儿 CM-I 治疗的术前患者咨询、术前和术中手术决策以及术后临床预后。
