Department of Medical Imaging, CHEO, University of Ottawa, Ottawa, ON, Canada.
University of Ottawa, Ottawa, ON, Canada.
Childs Nerv Syst. 2024 May;40(5):1443-1448. doi: 10.1007/s00381-023-06273-9. Epub 2024 Jan 12.
Given that syrinx is often considered an indication of surgery in children with Chiari I malformation (CM1), understanding of the natural history of these patients is very challenging. In this study, we investigate the natural history of children with CM1 that have syrinx and/or prominence of the central canal on presentation.
All pediatric Chiari I patients who had syrinx and/or prominence of the central canal who underwent MR imaging of the head and spine from 2007 to 2020 were reviewed. Patients were divided into 3 groups (early surgery, delayed surgery, and conservative management). We focused on those patients who did not initially undergo surgery and had at least 1 year of clinical follow-up. We assessed if there were any radiological features that would correlate with need for delayed surgical intervention.
Thirty-seven patients met the inclusion criteria. Twenty-one patients were female and 16 were male. The mean age at presentation was 8.7 (5.8 SD). Fourteen (38%) patients had early surgical intervention, with a mean of 2.5 months after initial presentation, 8 (16%) had delayed surgery due to new or progressive neurological symptoms and 46% of patients did not require intervention during follow-up. The length of tonsillar herniation and the position of the obex were associated with the need of surgery in patients who were initially treated conservatively.
In pediatric patients with CM1 with syringomyelia and prominence of the central canal, conservative treatment is initially appropriate when symptoms are absent or mild. Close follow-up of patients with CM1 and dilatation of the central canal who have an obex position below the foramen magnum and greater tonsillar herniation is suggested, as these patients show a trend towards clinical deterioration over time and may require earlier surgical intervention.
鉴于脊髓空洞通常被认为是 Chiari I 畸形（CM1）儿童手术的指征，因此了解这些患者的自然病史极具挑战性。在这项研究中，我们研究了在出现时存在脊髓空洞和/或中央管膨出的 CM1 患儿的自然病史。
回顾了 2007 年至 2020 年间所有因存在脊髓空洞和/或中央管膨出而行头颅和脊柱磁共振成像（MRI）检查的小儿 CM1 患者。患者被分为 3 组（早期手术、延迟手术和保守治疗）。我们重点关注那些最初未行手术且至少有 1 年临床随访的患者。我们评估了是否存在任何影像学特征与延迟手术干预相关。
符合纳入标准的患者有 37 名。21 名女性，16 名男性。就诊时的平均年龄为 8.7 岁（5.8 标准差）。14 名（38%）患者行早期手术干预，平均在初次就诊后 2.5 个月，8 名（16%）因新出现或进行性神经症状而行延迟手术，46%的患者在随访期间无需干预。初诊时行保守治疗的患者中，扁桃体疝出的长度和后颅窝凹陷最低点（obex）的位置与手术需求相关。
在存在脊髓空洞和中央管膨出的 CM1 儿科患者中，当症状不存在或轻微时，最初采用保守治疗是合适的。建议对存在中央管扩张且 obex 位置低于枕骨大孔和扁桃体疝出较大的 CM1 患者进行密切随访，因为这些患者随着时间的推移有临床恶化的趋势，可能需要更早的手术干预。
