Hui S, Wing Y K, Kew J, Chan Y L, Abdullah V, Fok T F
Department of Paediatrics, Chinese University of Hong Kong, Shatin, Hong Kong.
Sleep. 1998 May 1;21(3):298-303.
Craniofacial anomalies are recognized causes of obstructive sleep apnea syndrome (OSAS) in children. Current literature is limited due to rarity of cases. Furthermore, the mechanism of upper airway obstruction is not clearly understood. We would like to report a family (father and 2 sons) who are suffering from Crouzon's syndrome. The two brothers (ages 1 and 3) were found to have significant obstructive sleep apnea syndrome (OSAS) with failure to thrive. Nasal continuous positive airway pressure (CPAP) markedly improved their OSAS and resulted in accelerated weight gain. The nasoendoscopy and magnetic resonance imaging (MRI) scan taken during natural sleep showed that choanal stenosis, maxillary hypoplasia, posteriorly displaced tongue, lengthened soft palate and adenoid tissues were important in the pathogenesis of upper airways obstruction in Crouzon's syndrome. Nasal CPAP improved airway obstruction by opening a narrow slit as demonstrated by MRI. Our results suggest that OSAS occurred in children with Crouzon's syndrome and that nasal CPAP was a useful treatment modality.
颅面畸形是儿童阻塞性睡眠呼吸暂停综合征（OSAS）的公认病因。由于病例罕见，目前的文献有限。此外，上气道阻塞的机制尚不清楚。我们想报告一个患有克鲁宗综合征的家庭（父亲和两个儿子）。这两个兄弟（分别为1岁和3岁）被发现患有严重的阻塞性睡眠呼吸暂停综合征（OSAS）且发育不良。鼻持续气道正压通气（CPAP）显著改善了他们的OSAS，并导致体重加速增加。自然睡眠期间进行的鼻内镜检查和磁共振成像（MRI）扫描显示，后鼻孔狭窄、上颌骨发育不全、舌后移、软腭延长和腺样体组织在克鲁宗综合征上气道阻塞的发病机制中起重要作用。如MRI所示，鼻CPAP通过打开一条狭窄缝隙改善了气道阻塞。我们的结果表明，克鲁宗综合征患儿会发生OSAS，且鼻CPAP是一种有效的治疗方式。
