Department of Plastic, Reconstructive, and Hand Surgery, Dutch Craniofacial Centre, Erasmus Medical Centre--Sophia Children's Hospital, Rotterdam, The Netherlands.
Arch Dis Child. 2013 Jul;98(7):538-43. doi: 10.1136/archdischild-2012-302745. Epub 2013 May 23.
To describe the course of obstructive sleep apnoea syndrome (OSAS) in children with syndromic craniosynostosis.
Prospective cohort study.
Dutch Craniofacial Centre from January 2007 to January 2012.
A total of 97 children with syndromic craniosynostosis underwent level III sleep study. Patients generally undergo cranial vault remodelling during their first year of life, but OSAS treatment only on indication.
Obstructive apnoea-hypopnoea index, the central apnoea index and haemoglobin oxygenation-desaturation index derived from consecutive sleep studies.
The overall prevalence of OSAS in syndromic craniosynostosis was 68% as defined by level III sleep study. Twenty-three patients were treated for OSAS. Longitudinal profiles were computed for 80 untreated patients using 241 sleep studies. A mixed effects model showed higher values for the patients with midface hypoplasia as compared to those without midface hypoplasia (Omnibus likelihood ratio test=7.9). In paired measurements, the obstructive apnoea-hypopnoea index (Z=-3.4) significantly decreased over time, especially in the first years of life (Z=-3.3), but not in patients with midface hypoplasia (Z=-1.5). No patient developed severe OSAS during follow-up if it was not yet diagnosed during the first sleep study.
OSAS is highly prevalent in syndromic craniosynostosis. There is some natural improvement, mainly during the first 3 years of life and least in children with Apert or Crouzon/Pfeiffer syndrome. In the absence of other co-morbid risk factors, it is highly unlikely that if severe OSAS is not present early in life it will develop during childhood. Ongoing clinical surveillance is of great importance and continuous monitoring for the development of other co-morbid risk factors for OSAS should be warranted.
描述综合征型颅缝早闭患儿阻塞性睡眠呼吸暂停综合征（OSAS）的病程。
前瞻性队列研究。
荷兰颅面中心，2007 年 1 月至 2012 年 1 月。
共有 97 例综合征型颅缝早闭患儿进行了 III 级睡眠研究。患者通常在生命的第一年接受颅骨重塑，但仅根据需要进行 OSAS 治疗。
连续睡眠研究得出的阻塞性呼吸暂停低通气指数、中枢性呼吸暂停指数和血红蛋白氧减饱和度指数。
III 级睡眠研究定义的综合征型颅缝早闭患儿中，OSAS 的总体患病率为 68%。23 例患者因 OSAS 接受治疗。对 80 例未治疗患者的 241 项睡眠研究进行了纵向分析。混合效应模型显示，与无面中部发育不良的患者相比，面中部发育不良的患者的这些值更高（整体似然比检验=7.9）。在配对测量中，阻塞性呼吸暂停低通气指数（Z=-3.4）随时间显著降低，尤其是在生命的最初几年（Z=-3.3），但面中部发育不良的患者（Z=-1.5）没有显著降低。如果在第一次睡眠研究中未诊断出严重 OSAS，则在随访期间无患者发生严重 OSAS。
综合征型颅缝早闭患儿 OSAS 患病率很高。存在一定的自然改善，主要发生在生命的前 3 年，在 Apert 或 Crouzon/Pfeiffer 综合征患儿中最少。在没有其他合并症危险因素的情况下，如果在生命早期没有出现严重 OSAS，则极不可能在儿童时期出现 OSAS。持续的临床监测非常重要，应持续监测 OSAS 其他合并症危险因素的发展。
