Jane Hamilton1, W Andrew Clement2, Haytham Kubba3. 1. University of Glasgow, Scotland, United Kingdom. 2. Department of Otolaryngology - Head and Neck Surgery, Royal Hospital for Sick Children, Glasgow G3 8SJ, Scotland, United Kingdom. 3. Department of Otolaryngology - Head and Neck Surgery, Royal Hospital for Sick Children, Glasgow G3 8SJ, Scotland, United Kingdom. Electronic address: hkubba@nhs.net.
Abstract
AIMS: Children 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. METHODS: Retrospective case note review of children diagnosed with congenital tracheal cartilaginous sleeve presenting to our department between 2006 and 2014. RESULTS: Four 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. CONCLUSION: Congenital 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 APPROVAL: Registered with Clinical Governance Committee.
AIMS: Children 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. METHODS: Retrospective case note review of children diagnosed with congenital tracheal cartilaginous sleeve presenting to our department between 2006 and 2014. RESULTS: Four 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. CONCLUSION:Congenital 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 APPROVAL: Registered with Clinical Governance Committee.
Authors: Tara L Wenger; John Dahl; Elizabeth J Bhoj; Anna Rosen; Donna McDonald-McGinn; Elaine Zackai; Ian Jacobs; Carrie L Heike; Anne Hing; Avni Santani; Andrew F Inglis; Kathleen C Y Sie; Michael Cunningham; Jonathan Perkins Journal: Genet Med Date: 2016-05-26 Impact factor: 8.822