Tracheal Cartilaginous Sleeve in Syndromic Craniosynostosis: An Underrecognized Source of Significant Morbidity and Mortality.

Abstract

Tracheal cartilaginous sleeve (TCS) is a rare and previously unrecognized source of morbidity and mortality in patients with certain craniosynostosis syndromes. There is a paucity of reporting on this airway anomaly, and the true incidence of TCS is largely unknown. The purpose of this study was to investigate the incidence of TCS among patients with syndromic craniosynostosis at our institution. Patients with syndromic craniosynostosis who underwent direct bronchoscopy and laryngoscopy were evaluated retrospectively by pediatric otolaryngologists for the presence of TCS and associated anomalies. Among patients with a diagnosis of syndromic craniosynostosis in our craniofacial database, 10 (37%) were found to have previous direct bronchoscopy and laryngoscopy reports. Of these 10 patients, 2 had Crouzon syndrome, 3 had Pfeiffer syndrome, 3 had Apert syndrome, 1 had Muenke syndrome, and 1 had Antley-Bixler syndrome. Eighty percent (8/10) of these patients were found to have some evidence of TCS. The most commonly observed associated findings included the following: tracheostomy dependency (7/10; 70%), hearing loss (6/10; 60%), obstructive sleep apnea (5/10; 50%), cervical spine anomalies (5/10; 50%), developmental delay (5/10; 50%), and enlarged cerebral ventricles (4/10; 40%). Larger multicenter studies are required to further characterize this airway anomaly and its impact on this patient population. Our results confirm the importance of thorough airway evaluation at initial presentation and the need for validated screening protocols.