Aim Children with Cornelia De Lange Syndrome (CDLS) frequently present to paediatric services with hearing problems. Airway problems have not previously been reported. We wish to describe our experience of the management of these children.

Methods Retrospective case note review of children diagnosed with CDLS presenting to our department between 2005 and 2013.

Results Five patients were seen. Airway problems consisted of laryngeal overspill with severe gastrooesophageal dysmotility and reflux despite structurally normal airway (1 case), laryngomalacia requiring supraglottoplasty (2 cases), reflux laryngitis with secondary laryngomalacia and coincidental tracheal diverticulum (1 case) and obstructive sleep apnoea (1 case). Supraglottoplasty produced a dramatic improvement in feeding and breathing in both children who underwent the procedure. Two children had a submucous hcleft palate and one underwent cochlear implantation for a profound sensorineural hearing loss.

Conclusion Children with CDLS have multifaceted clinical problems. Airway pathology has not previously been described in CDLS but has been common in our experience. We wish to highlight that laryngomalacia in Cornelia de Lange Syndrome responds well to supraglottoplasty.