Kirjavainen Turkka, Miraftabi Päriä, Martelius Laura, Karppinen Atte
Department of Pediatrics, New Children's Hospital, Helsinki, Finland; Department of Clinical Neurophysiology and Neurological Sciences, New Children's Hospital, Helsinki, Finland.
HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Sleep Med. 2024 Apr;116:32-40. doi: 10.1016/j.sleep.2024.02.019. Epub 2024 Feb 16.
Chiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep.
This is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008-2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging.
We included 104 children with CM1 with a median age of 7 (interquartile range (IQR) 5-13) years. The median extent of tonsillar descent (TD) was 13 (IQR 10-18) mm. Syringomyelia was present in 19 children (18%). Of all children, 53 (51%) had normal PSG, 35 (34%) showed periodic breathing or central apnea and hypopnea index ≥5 h, and 16 (15%) displayed features of compensated central hypoventilation and end-tidal or transcutaneous carbon dioxide 99 percentile level above 50 mmHg. TD had the best predictive value for central breathing disorders. In a linear model, both age (61%) and TD (39%) predicted median breathing frequency (R = 0.33, p < 0.001).
Although severe CSA is a rare complication of brainstem compression in pediatric patients with CM1, short arousal-triggered episodes of periodic breathing and mild compensated central hypoventilation are common. TD shows the best but still poor prediction of the presence of a central breathing disorder. This highlights the use of PSG in patient evaluation. Posterior fossa decompression surgery effectively treats central breathing disorders.
Chiari 1型畸形（CM1）偶尔会导致中枢性睡眠呼吸暂停（CSA）。我们在一个大型儿科CM1患者临床队列中，研究了CM1对睡眠期间呼吸的影响。
这是一项回顾性单中心儿科肺病学研究，对2008年至2020年间接受多导睡眠图（PSG）检查的18岁以下儿科CM1患者进行系统评估。排除患有综合征的儿童。所有患者均接受了头部和脊柱磁共振成像检查。
我们纳入了104例CM1患儿，中位年龄为7岁（四分位间距（IQR）5 - 13岁）。扁桃体下疝（TD）的中位程度为13mm（IQR 10 - 18mm）。19名儿童（18%）存在脊髓空洞症。在所有儿童中，53例（51%）PSG正常，35例（34%）表现为周期性呼吸或中枢性呼吸暂停及低通气指数≥5次/小时，16例（15%）表现为代偿性中枢性通气不足特征且潮气末或经皮二氧化碳分压第99百分位数水平高于50mmHg。TD对中枢性呼吸障碍具有最佳预测价值。在一个线性模型中，年龄（61%）和TD（39%）均能预测中位呼吸频率（R = 0.33，p < 0.001）。
虽然严重CSA是儿科CM1患者脑干受压的罕见并发症，但由觉醒触发的短暂周期性呼吸发作和轻度代偿性中枢性通气不足很常见。TD对中枢性呼吸障碍的存在显示出最佳但仍较差的预测能力。这凸显了PSG在患者评估中的应用。后颅窝减压手术可有效治疗中枢性呼吸障碍。
