Lim C T, Koh M T
Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Australas Radiol. 1992 May;36(2):158-9. doi: 10.1111/j.1440-1673.1992.tb03108.x.
Cerebro-costo-mandibular syndrome (CCM) is a very rare entity with oro-facial features closely resembling Pierre-Robin anomaly (1,2). We describe a patient with typical features: severe micrognathia, glossoptosis, central cleft soft palate and multiple posterior rib-gap defects seen on chest radiograph. Respiratory distress which was evident soon after birth was relieved by an oro-pharyngeal airway. He suffered from a cardiorespiratory arrest and succumbed, presumably secondary to aspiration of milk feeds, before a tracheostomy could be performed.
脑-肋-下颌综合征(CCM)是一种非常罕见的病症,其口腔面部特征与皮埃尔-罗宾畸形极为相似(1,2)。我们描述了一名具有典型特征的患者:严重小颌畸形、舌后坠、中央性腭裂以及胸部X线片上可见的多处后肋间隙缺损。出生后不久即出现的呼吸窘迫通过口咽气道得以缓解。在能够进行气管切开术之前,他发生了心肺骤停并最终死亡,推测原因是喂奶时误吸。