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

Abstract

Upper airway obstruction is a prevalent feature in patients with Down syndrome. However, these patients may be completely asymptomatic in the early months of life. The recognition of a problem within the airway quite often occurs when these children present for cardiac surgery, diagnostic radiography with sedation, or during the induction and intubation for anesthesia. Tracheal stenosis is rare in the general population, but is seen somewhat more frequently in patients with Down syndrome. The incidence of tracheal stenosis in children with congenital heart disease, which is seen in 40% of patients with Down syndrome, has been reported to be 1.2%. Patients with Down syndrome also tend to have other upper airway obstructive pathology such as nasopharyngeal, oropharyngeal, and subglottic compromise. These entities, combined with the high incidence of cardiac disease, put these children at risk for acute and chronic cardiopulmonary compromise. We present two patients with Down syndrome and congenital heart disease who were found to have significant tracheal stenosis at the time of their cardiac surgery. The perioperative management of their airway defects including diagnostic evaluation and treatment modalities are discussed.