Tracheal cartilaginous sleeve in Crouzon syndrome

Tracheal cartilaginous sleeve (TCS) is a congenital malformation characterized by 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. TCS has been reported only in children with craniosynostosis. Seven cases of TCS and Crouzon syndrome (CS) are mentioned in the literature. In addition to our case study, a review of the literature on TCS in CS, a classification of TCS and treatment options will be provided. Tracheotomy and frequent airway endoscopy coupled with a keen understanding of the shifting sites of obstruction will permit longterm survival in patients with TCS and CS. (C) 2002 Elsevier Science Ireland Ltd. All rights reserved.