Upper airway obstruction in children with Down syndrome.

OBJECTIVES: To analyze the complex nature of upper airway obstruction (UAO) and the response to surgery in children with Down syndrome.

DESIGN: Retrospective medical chart review of all patients with Down syndrome who had UAO during a 5-year period.

SETTING: Academic tertiary care children's hospital.

PATIENTS: Seventy-one pediatric patients with Down syndrome who had significant UAO. Thirty-four patients had pulmonary artery hypertension; 44 patients had multiple sites of airway obstruction. The obstructive problems included lymphoid hyperplasia, macroglossia, narrow nasopharynx, laryngomalacia, congenital subglottic stenosis, tracheobronchomalacia, and tracheal stenosis.

INTERVENTIONS: Children with Down syndrome and UAO underwent surgical procedures including tonsillectomy, adenoidectomy, tonsillar pillar plication, uvulopalatopharyngoplasty, anterior tongue reduction, tonguehyoid suspension, laryngotracheoplasty, and tracheotomy.

MAIN OUTCOME MEASURES: Postoperative symptoms found on medical chart review, or parental telephone survey, or both, and results of postoperative diagnostic tests. Patients were grouped as "improved" or having "significant residual symptoms."

RESULTS: Twenty-seven of the 55 surgical patients had mild obstructive symptoms, and most improved after tonsil or adenoid surgery, or both. The remaining patients were younger and had more severe symptoms, multiple sites of obstruction, and a high incidence of cardiac disease. Eleven (39%) of the 28 patients in this group had significant residual symptoms after surgery. Four children are tracheotomy-dependent. Five deaths occurred; 3 were attributable to the upper airway.

CONCLUSIONS: Upper airway obstruction in children with Down syndrome often is a complex process with multifocal causes. Residual symptoms of airway obstruction are common after surgery. A comprehensive and individualized approach is important in the management of UAO in Down syndrome.