Pediatric Chiari I malformations
Pediatric Chiari I malformations
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小儿 Chiari I 畸形
DOI：
10.1212/wnl.53.6.1271
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发表时间：
1999
期刊：
影响因子：
9.9
通讯作者：
D. Ferriero
中科院分区：
文献类型：
--
作者：
Yvonne W Wu;C. Chin;K. Chan;A. Barkovich;D. Ferriero
背景：虽然Chiari I畸形在儿童中的认识越来越多，但对其在这个年龄段的临床表现知之甚少。目的：探讨儿童Chiari I畸形的临床与MRI表现之间的关系。方法：对49例Chiari I畸形患儿进行病历回顾和MRI分析。扁桃体异位的程度与发病年龄、脊髓空洞症的存在和神经系统严重程度评分进行比较：无症状=0，有症状的神经系统检查正常=1，有症状的异常检查=2。结果：出现症状的年龄为10个月至14岁。57%的患者没有症状。头痛和颈部疼痛是最常见的主诉。14%的患者出现脊髓空洞症，50%的患者出现颅底异常。扁桃体异位的大小(5-23 mm)与严重程度评分相关(p=0.04)，但与其他临床指标无关。结论：儿童Chiari I畸形的临床症状与成人几乎相同。MRI上扁桃体异位程度较大的儿童更有可能出现症状。
Background: Although Chiari I malformation is increasingly recognized in children, little is known about its clinical presentation in this age group. Objective: To evaluate the relationship between clinical and MRI features of pediatric Chiari I malformations. Methods: We performed a chart review and MRI analysis of 49 children with Chiari I malformation. The degree of tonsillar ectopia was compared with age at onset, presence of syringomyelia, and a neurologic severity score as follows: asymptomatic = 0, symptomatic with normal neurologic examination = 1, and symptomatic with abnormal examination = 2. Results: Age at onset of symptoms ranged from 10 months to 14 years. Fifty-seven percent of patients were asymptomatic. Headache and neck pain were the most frequent complaints. Syringomyelia was detected in 14% of patients and skull base abnormalities in 50%. The magnitude of tonsillar ectopia (5 to 23 mm) correlated with severity score (p = 0.04) but not with other clinical measures. Conclusions: The clinical symptoms of Chiari I malformations in children are nearly identical to those seen in adults. Children with greater amounts of tonsillar ectopia on MRI are more likely to be symptomatic.