Tracheal cartilaginous sleeve contributing to obstructive sleep apnea in a child with Crouzon syndrome

SummaryTracheal cartilaginous sleeve is characterized by the fusion of tracheal rings. Patients with tracheal cartilaginous sleeve may develop frequent respiratory tract infections, coughs, increased mucus production, sporadic cyanosis episodes, and failure to thrive due to alterations in the lower airway. Tracheal cartilaginous sleeve is seen exclusively in craniosynostosis. Craniosynostosis is described as the early fusion of skull sutures and causes abnormal development of the head. Crouzon syndrome is the most common syndromic craniosynostosis. Otolaryngologists may be asked to evaluate and manage breathing difficulties, sleep apnea, and hearing difficulties associated with Crouzon syndrome. Upper airway abnormalities such as hypertrophy of the tonsils and adenoids, midnasal stenosis, and choanal atresia have been reported. Lower airway abnormalities in the presence of obstructive airway symptoms have not been widely studied in Crouzon syndrome. We describe the diagnosis and management of multilevel airway problems in a child with Crouzon syndrome who presented with obstructive sleep apnea.