Tracheal cartilaginous sleeve in craniosynostosis

Abstract

Tracheal cartilaginous sleeve (TCS) is a congenital malformation involving fusion of the tracheal arches that may be isolated to a few tracheal arches, include the entire trachea, or extend beyond the carina into the bronchi. Tracheotomy was required in 9 of 23 craniosynostosis cases undergoing gradual distraction at Osaka City General Hospital from March 2002 to April 2006. TCS was diagnosed in 5 of 9 cases-four Pfeiffer patients and one Crouzon patient. Diagnosis was made intraoperatively during tracheotomy or at autopsy. 3D-CT was not useful in diagnosing TCS. Aggressive management of respiratory infection and pulmonary secretion, selection of appropriate tracheostomy tubes, and endoscopic evaluation are very important to care in managing TCS patients.