Division of Respiratory Disease, Department of Pediatric Medicine, Boston Children's Hospital, Boston, Massachusetts, USA.
Pediatr Neurol. 2013 Apr;48(4):299-307. doi: 10.1016/j.pediatrneurol.2012.12.009.
Chiari I malformation is characterized by downward herniation of the cerebellar tonsils through the foramen magnum. Scant data are available on the clinical course, relationship to the extent of herniation on magnetic resonance imaging in Chiari I malformation and the presence of sleep-disordered breathing on polysomnography. Retrospective analysis was performed looking at polysomnographic findings of children diagnosed with Chiari I malformation. Details on how Chiari I malformation was diagnosed, brainstem magnetic resonance imaging findings, and indications for obtaining the polysomnogram in these patients were reviewed. We also reviewed available data on children who had decompression surgery followed by postoperative polysomnography findings. Twenty-two children were identified in our study (11 males, median age 10 years, range 1 to 18). Three had central sleep apnea, five had obstructive sleep apnea, and one had both obstructive and central sleep apnea. Children with sleep-disordered breathing had excessive crowding of the brainstem structures at the foramen magnum and were more likely to have a greater length of herniation compared with those children without sleep-disordered breathing (P = 0.046). Patients with central sleep apneas received surgical decompression, and their conditions were significantly improved on follow-up polysomnography. These data suggest that imaging parameters may correlate with the presence of sleep-disordered breathing in children with Chiari I malformation.
Chiari I 畸形的特征是小脑扁桃体通过枕骨大孔向下疝出。关于 Chiari I 畸形的临床病程、与磁共振成像上疝出程度的关系以及睡眠呼吸障碍多导睡眠图的存在，数据很少。对诊断为 Chiari I 畸形的儿童的多导睡眠图检查结果进行了回顾性分析。回顾了 Chiari I 畸形的诊断方式、脑干磁共振成像结果以及这些患者获得多导睡眠图的指征的详细信息。我们还回顾了接受减压手术后进行术后多导睡眠图检查结果的儿童的现有数据。在我们的研究中，共确定了 22 名儿童（男 11 例，中位年龄 10 岁，范围 1 至 18 岁）。3 例有中枢性睡眠呼吸暂停，5 例有阻塞性睡眠呼吸暂停，1 例有阻塞性和中枢性睡眠呼吸暂停。有睡眠呼吸障碍的儿童在枕骨大孔处的脑干结构过度拥挤，与没有睡眠呼吸障碍的儿童相比，更有可能出现更长的疝出（P=0.046）。有中枢性睡眠呼吸暂停的患者接受了手术减压，他们的病情在随访多导睡眠图检查时明显改善。这些数据表明，影像学参数可能与 Chiari I 畸形儿童睡眠呼吸障碍的存在相关。
