Benglis David, Covington Derek, Bhatia Ritwik, Bhatia Sanjiv, Elhammady Mohamed Samy, Ragheb John, Morrison Glenn, Sandberg David I
Department of Neurosurgery, University of Miami/Miller School of Medicine and Miami Children's Hospital, Miami, Florida 33155, USA.
J Neurosurg Pediatr. 2011 Apr;7(4):375-9. doi: 10.3171/2011.1.PEDS10341.
The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention.
The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up.
The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5-22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up.
The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.
未经治疗的Ⅰ型Chiari畸形（CM-Ⅰ）的自然病史尚不明确。本研究的目的是调查未接受手术干预而接受随访的小儿CM-Ⅰ患者的预后情况。
作者回顾性分析了1999年7月至2008年7月期间收治的124例CM-Ⅰ患者，这些患者均未接受手术治疗且接受了随访。患者年龄在0.9至19.8岁之间（平均7岁）。随访时间为1.0至8.6年（平均2.83年）。记录患者就诊时以及随访期间的影像学表现、症状和神经系统检查结果。
就诊时扁桃体下疝的平均程度为8.35毫米（范围为5至22毫米）。7例患者就诊时存在脊髓空洞症。在随访影像学检查中，这些患者的脊髓空洞症大小未发生变化。在接受后续影像学检查的其余患者中未出现新的脊髓空洞症。出现症状的患者总数为81例。在这81例患者中，67例表现出的症状并非CM-Ⅰ的典型症状。在14例归因于CM-Ⅰ的症状患者中，9例症状不严重或不频繁，不足以进行手术，其余5例患者建议进行手术。43例无症状患者因各种原因进行了影像学检查，也被诊断为Ⅰ型Chiari畸形。在随访期间，任何患者均未出现新的神经功能缺损。
大多数未接受手术治疗而接受随访的CM-Ⅰ患者在临床或影像学上均无进展。需要对该队列进行更长时间的随访，以确定多年后是否会出现症状或新的神经学发现。
