Chiari I malformation in the very young child: The spectrum of presentations and experience in 31 children under age 6 years
Chiari I malformation in the very young child: The spectrum of presentations and experience in 31 children under age 6 years
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DOI：
10.1542/peds.110.6.1212
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发表时间：
2002-12-01
期刊：
影响因子：
8
通讯作者：
Menezes, AH
中科院分区：
文献类型：
--
作者：
Greenlee, JDW;Donovan, KA;Menezes, AH
导言。在非常小的儿童中，没有骨髓发育不良的后脑突出的实体一直被很少地描述。方法对6岁前确诊为Chiari I畸形(CM I)的患儿进行回顾性分析。自1985年以来，爱荷华大学医院和诊所已诊断出31名1岁(0.3-5.8岁)的CM儿童。对他们的记录进行回顾，包括症状、体征、放射学表现、治疗、并发症和结果。平均确诊年龄为3.3岁。16名患者年龄在3岁以下。主要症状包括口咽功能受损(35%)、脊柱侧弯(23%)、头痛或颈部疼痛(23%)、感觉障碍(6%)、虚弱(3%)和其他(10%)。在3岁以下的儿童中，69%的儿童口咽功能异常。3名3岁以下的患者(19%)在确诊CM I之前接受过胃底折叠术和/或胃造口术。常见的体征包括肌腱反射异常(68%)、脊柱侧弯(26%)、呕吐反射异常(13%)和正常检查(13%)。声带功能障碍(26%，均在3岁以下)和脊髓空洞症(52%)也可见。25例患者在我们医院接受手术治疗，包括后颅窝减压术、硬脑膜成形术和小脑扁桃体萎缩。三名患者失去了随访机会。91%的患者报告在最后一次随访时症状有所改善(平均3.9年)。有3名患者因症状复发需要再次手术。所有患者的脊髓空洞症均有改善。8例患者中有2例脊柱侧凸缓解，5例改善，1例稳定。手术无永久性并发症。我们发现Chiari I型畸形的儿童在3岁以下极有可能出现口咽功能障碍，3-5岁的儿童则可能出现脊柱侧弯或头痛或颈部疼痛加重的Valsalva。这些症状很可能在Chiari减压后得到改善，这是一种发病率较低的手术方式。出现口咽功能障碍、Valsalva加重的疼痛或脊柱侧弯的幼儿应促使临床医生考虑将CM I作为可能的原因。
Introduction. The entity of hindbrain herniation without myelodysplasia in the very young child has been poorly described. A retrospective analysis of children diagnosed with Chiari I malformation (CM I) before their sixth birthday is presented.Methods. Since 1985,31 children with CM 1 (0.3-5.8) years of age have been diagnosed at University of Iowa Hospitals and Clinics. Their records were reviewed for presenting symptoms, signs, radiographic findings, treatment, complications, and outcome.Results. The average age at diagnosis was 3.3 years. Sixteen patients were under age 3. Chief presenting complaints included impaired oropharyngeal function (35%), scoliosis (23%), headache or neck pain (23%), sensory disturbance (6%), weakness (3%), and other (10%). Sixty-nine percent of children under age 3 had abnormal oropharyngeal function. Three patients under age 3 (19%) had undergone fundoplication and/or gastrostomy before diagnosis of CM I.Common physical findings included abnormal tendon reflexes (68%), scoliosis (26%), abnormal gag reflex (13%), and normal examination (13%). Vocal cord dysfunction (26%, all under age 3) and syringohydromyelia (52%) were also seen.Twenty-five patients were treated surgically at our institution with posterior fossa decompression, duraplasty, and cerebellar tonsillar shrinkage. Three patients were lost to follow-up. Ninety-one percent of patients reported improved symptomatology at last follow-up (mean: 3.9 years). Three patients required reoperation for recurrence of symptoms. Syringomyelia improved in all patients. Scoliosis resolved in 2 of 8 patients, improved in 5, and stabilized in 1. There was no permanent morbidity from surgery.Discussion. We show that children with Chiari I abnormality are very likely to present with oropharyngeal dysfunction if under age 3, and either scoliosis or headache or neck pain worsened by valsalva if age 3 to 5. These symptoms are very likely to improve after Chiari decompression, which can be done with low morbidity.Conclusions. Very young children presenting with oropharyngeal dysfunction, pain worsened by valsalva, or scoliosis should prompt the clinician to consider CM I as a possible cause.