Upper airway obstruction caused by vascular anomalies in children.

Vascular ring or sling is one of the important etiologies for congenital upper airway obstruction in children. Between July 1988 and July 1995, a total of 92 children with stridor were initially evaluated by an esophagogram and two-dimensional echocardiogram. Seven children (7.7%) with variable types of vascular anomalies were diagnosed including pulmonary artery sling (2 cases), double aortic arch (1 case), right arch with left aberrant subclavian artery and ligamentum ductus (1 case), double aortic arch with one atretic arch (2 cases), and innominate artery compression of the trachea (1 case). The age at diagnosis ranged from 18 days to 3 years old. Only one patient was associated with an intracardiac anomaly. Five of the 7 patients were diagnosed as having laryngomalacia previously. Six patients had esophageal indentation on their esophagograms. Besides external airway compression, three patients also had intrinsic tracheal stenosis. Six patients received surgery because of severe respiratory distress. The patients' symptoms improved after surgery, but a premature baby died after a second operation for intrinsic tracheal stenosis at the age of 8 months.