Purpose

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.

Methods

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.

Results

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.

Conclusion

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.

中文翻译：

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儿童Chiari I畸形的自然史：回顾性分析

目的

尽管有关儿童Chiari I畸形（CM1）的研究越来越多，但大多数研究都集中在手术适应症，技术和结局上。一旦做出保守治疗的决定，很少有研究检查CM1的自然病史。在这项研究中，我们寻求确定接受初始手术后并以延迟方式接受CM1手术的儿科患者的百分比，决定采取非手术治疗的CM1的自然病史，并尝试确定患者因素可能预示着新的或恶化的CM1症状的发展。

方法

从我们的465例CM1儿科患者的数据库中，我们确定了从2011年7月1日至2016年6月30日接受初诊的患者。我们研究了手术干预率，手术干预类型，年龄，性别以及没有头痛和syrinx症状，并仔细检查了出现新症状或病情恶化，促使延迟手术干预的患者。

结果

我们确定了226名符合纳入标准的患者。总体而言，有15％的患者接受了手术，其中大部分为Chiari减压。这些患者中，刚过一半的患者在初次咨询后的6个月内接受了手术。在那些延迟手术的患者中，只有4例有新症状/症状，而1例有症状进展。其他患者有各种原因导致的手术延迟，与症状的发展或进展无关。在这5名患者中，没有明显的共同点可以预测未来的进展。所有接受外科手术的患者均在初诊后2年内这样做。

结论

总体而言，无症状CM1的自然病史是良性的。非手术治疗的患者不太可能进展。如果他们确实取得了进展，这很可能会在初诊的两年内发生。在这项研究中没有发现可以预测随着时间推移出现新的或恶化的症状的因素。