CONGENITAL TRACHEAL CARTILAGINOUS SLEEVE

The congenital tracheal cartilaginous sleeve (TCS) results from a vert ical fusion of the tracheal cartilages. This rare malformation is usua lly associated with one of the craniosynostosis syndromes, such as Cro uzon's disease, Pfeiffer's syndrome, or Goldenhar's syndrome. Three ne w cases of TCS are reported, two with autopsy findings including the h istopathology of horizontal tracheal sections. Salient features of the clinical presentation, diagnostic evaluation, endoscopic findings, hi stopathology, treatment, and prognosis for TCS are summarized from the perspective of the otolaryngologist. In addition, the literature is r eviewed, and previously reported cases are discussed. Although infants with TCS often have multiple abnormalities, this tracheal malformatio n is not incompatible with life. Since multiple lesions of the larynx and trachea may be present, endoscopic evaluation is recommended for i nfants with TCS who experience airway distress. A smooth trachea lacki ng the normal ridges of tracheal arches suggests the diagnosis of TCS. With early recognition and appropriate management (including tracheot omy, if necessary, and aggressive management of pulmonary hygiene), pa tients may survive into childhood.