Tracheal stenosis and congenital heart disease in patients with Down syndrome: diagnostic approach and surgical options

Upper airway obstruction is a prevalent feature in patients with Down syndr ome. However, these patients may be completely asymptomatic in the early mo nths of life. The recognition of a problem within the airway quite often oc curs when these children present for cardiac surgery, diagnostic radiograph y with sedation, or during the induction and intubation for anesthesia. Tra cheal stenosis is rare in the general population, but is seen somewhat more frequently in patients with Down syndrome. The incidence of tracheal steno sis in children with congenital heart disease, which is seen in 40% of pati ents with Down syndrome, has been reported to be 1.2%. Patients with Down s yndrome also tend to have other upper airway obstructive pathology such as nasopharyngeal, oropharyngeal, and subglottic compromise. These entities, c ombined with the high incidence of cardiac disease, put these children at r isk for acute and chronic cardiopulmonary compromise. We present two patien ts with Down syndrome and congenital heart disease who were found to have s ignificant tracheal stenosis at the time of their cardiac surgery. The peri operative management of their airway defects including diagnostic evaluatio n and treatment modalities are discussed. (C) 2000 Elsevier Science Ireland Ltd. All rights reserved.