Cerebro-costo-mandibular syndrome.
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.
Lim
C T
CT
Department of Paediatrics, Faculty of Medicine, University of Malaya, Kuala Lumpur, Malaysia.
Koh
M T
MT
D006801:Humans; D007231:Infant, Newborn; D008296:Malaysia / Q000453:epidemiology; D008297:Male; D010855:Pierre Robin Syndrome / Q000000981:diagnostic imaging / Q000453:epidemiology*; D011859:Radiography; D012272:Ribs / Q000002:abnormalities*