Management of congenital cartilaginous sleeve trachea in children

AimsChildren with congenital tracheal cartilaginous sleeve may present to otolaryngology services with airway problems. We wish to describe our overall management in a series of four children with this very rare anomaly.MethodsRetrospective case note review of children diagnosed with congenital tracheal cartilaginous sleeve presenting to our department between 2006 and 2014.ResultsFour patients were seen. One had Opitz G syndrome, two had Pfeiffers syndrome and one had no associated anomalies. Two children were successfully managed with laryngeal reconstruction using an anterior costal cartilage graft, while the third and fourth required a short period of tracheostomy only. All four are well and currently asymptomatic from an airway point of view.ConclusionCongenital tracheal cartilaginous sleeve is a very rare and potentially challenging problem. Otolaryngologists should be aware that it can occur in children with syndromes other than craniosynostosis (and indeed, those with no syndrome) and that it can be successfully treated using established airway management techniques.Ethical approvalRegistered with Clinical Governance Committee.