Congenital tracheal cartilaginous sleeve

The congenital tracheal cartilaginous sleeve (TCS) results from a vertical fusion of the tracheal cartilages. This rare malformation is usually associated with one of the craniosynostosis syndromes, such as Crouzon's disease, Pfeiffer's syndrome, or Goldenhar's syndrome. Three new cases of TCS are reported, two with autopsy findings including the histopathology of horizontal tracheal sections. Salient features of the clinical presentation, diagnostic evaluation, endoscopic findings, histopathology, treatment, and prognosis for TCS are summarized from the perspective of the otolaryngologist. In addition, the literature is reviewed, and previously reported cases are discussed. Although infants with TCS often have multiple abnormalities, this tracheal malformation is not incompatible with life. Since multiple lesions of the larynx and trachea may be present, endoscopic evaluation is recommended for infants with TCS who experience airway distress. A smooth trachea lacking the normal ridges of tracheal arches suggests the diagnosis of TCS. With early recognition and appropriate management (including tracheotomy, if necessary, and aggressive management of pulmonary hygiene), patients may survive into childhood.