UPPER AIRWAY-OBSTRUCTION IN CHILDREN WITH DOWN-SYNDROME

Objective: To analyze the complex nature of upper airway obstruction ( UAO) and the response to surgery in children with Down syndrome. Desig n: Retrospective medical chart review of all patients with Down syndro me who had UAO during a 5-year period. Setting: Academic tertiary care children's hospital. Patients: Seventy-one pediatric patients with Do wn syndrome who had significant UAO. Thirty-four patients had pulmonar y artery hypertension; 44 patients had multiple sites of airway obstru ction. The obstructive problems included lymphoid hyperplasia, macrogl ossia, narrow nasopharynx, laryngomalacia, congenital subglottic steno sis, tracheobronchomalacia, and tracheal stenosis. Interventions: Chil dren with Down syndrome and UAO underwent surgical procedures `includi ng tonsillectomy, adenoidectomy, tonsillar pillar plication, uvulopala topharyngoplasty, anterior tongue reduction, tonguehyoid suspension, l aryngotracheoplasty, and tracheotomy. Main Outcome Measures: Postopera tive symptoms found on medical chart review, or parental telephone sur vey, 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 obstruc tive symptoms, and most improved after tonsil or adenoid surgery, or b oth. The remaining patients were younger and had more severe symptoms, multiple sites of obstruction, and a high incidence of cardiac diseas e. Eleven (39%) of the 28 patients in this group had significant resid ual symptoms after surgery. Four children are tracheotomy-dependent. F ive deaths occurred; 3 were attributable to the upper airway. Conclusi ons: 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 individualiz ed approach is important in the management of UAO in Down syndrome.