Congenital tracheal anomalies in the craniosynostosis syndromes

The authors present the case of a 12-year-old girl with Pfeiffer's syndrome who underwent successful resection of a tracheal cartilaginous sleeve (TCS ) for treatment of sleep apnea. There is growing recognition of the inclusi on of TCS in the spectrum of congenital cartilage malformations seen in pat ients with craniosynostosis (CS) syndromes, This case demonstrates the diff icult therapeutic challenge created by the combination of hypopharyngeal an d intrinsic airway abnormalities present in CS patients. The early recognit ion of TCS in these patients may provide the opportunity for improved outco me in this severely affected subgroup of CS patients with otherwise high mo rtality. J Pediatr Surg 34:1036-1039. Copyright (C) 1999 by W.B. Saunders C ompany.