Division of Neurosurgery, Arkansas Children's Hospital, 1 Children's Way, Slot 838, Little Rock, AR, 72202, USA.
Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Childs Nerv Syst. 2021 Apr;37(4):1185-1190. doi: 10.1007/s00381-020-04913-y. Epub 2020 Oct 2.
While there are increasing numbers of studies published regarding Chiari I malformation (CM1) in children, most of these focus on surgical indications, technique, and outcomes. Few studies examine the natural history of CM1 once the decision is made to treat a patient conservatively. In this study, we seek to determine the percentage of pediatric patients who undergo surgery for CM1, both after initial consultation and in a delayed fashion, the natural history of CM1 after a decision to pursue non-operative management, and attempt to identify patient factors that may predict development of new or worsening CM1 symptoms.
From our database of 465 pediatric patients with CM1, we identified those who were seen for initial consultation from July 1, 2011, to June 30, 2016. We examined rates of surgical intervention, types of surgical intervention, age, gender, and presence or absence of headache and syrinx, and looked carefully at the patients who had new or worsening symptoms prompting delayed surgical intervention.
We identified 226 patients meeting inclusion criteria. Overall, 15% of patients had surgery, the majority being Chiari decompression. Just over half of these patients had surgery within 6 months of initial consultation. Of those with delayed surgery, only 4 patients had new symptoms/syrinx and 1 patient had symptom progression. The other patients had various reasons for surgical delay not related to symptom development or progression. There were no obvious commonalities among these 5 patients that could predict progression prospectively. All patients who had surgery did so within 2 years of initial consultation.
Overall, the natural history of asymptomatic CM1 is benign. Patients treated non-operatively are unlikely to progress. If they do progress, this is likely to occur within 2 years of initial consultation. There were no factors identified in this study that predicted new or worsening symptoms over time.
虽然越来越多的研究涉及儿童 Chiari I 畸形（CM1），但大多数研究都集中在手术适应证、技术和结果上。很少有研究探讨在决定保守治疗患者后 CM1 的自然史。在这项研究中，我们旨在确定接受 CM1 手术的儿科患者比例，包括初次就诊时和延迟就诊时；决定采用非手术治疗后 CM1 的自然史；并尝试确定可能预测新的或恶化的 CM1 症状发展的患者因素。
从我们的 465 例 CM1 儿科患者数据库中，我们确定了 2011 年 7 月 1 日至 2016 年 6 月 30 日初次就诊的患者。我们检查了手术干预率、手术类型、年龄、性别、头痛和脊髓空洞症的存在或缺失，并仔细观察了因新的或恶化的症状而导致延迟手术干预的患者。
我们确定了符合纳入标准的 226 例患者。总体而言，15%的患者接受了手术，大多数是 Chiari 减压术。其中一半以上的患者在初次就诊后 6 个月内进行了手术。在延迟手术的患者中，只有 4 例出现新症状/脊髓空洞症，1 例出现症状进展。其他患者手术延迟的原因与症状发展或进展无关。这 5 例患者之间没有明显的共性可以预测未来的进展。所有接受手术的患者都在初次就诊后 2 年内进行了手术。
总体而言，无症状 CM1 的自然史是良性的。非手术治疗的患者不太可能进展。如果进展，很可能在初次就诊后 2 年内发生。本研究未发现可预测随时间出现新症状或症状恶化的因素。
