Successful management of congenital tracheal stenosis in infancy.

Seven infants with congenital tracheal stenosis were evaluated and treated to assess the efficacy of current techniques of management. All had multiple congenital anomalies in addition to tracheal stenosis. Notably, three infants had imperforate anus and three had vascular rings. Each infant presented early with respiratory distress. While bronchoscopy and/or bronchography were performed in some, the diagnosis could be made from high contrast x-rays of the chest in each instance. The complexity of the tracheal lesion(s) determined the operative approach. Complicating factors included tracheomalacia, multiple stenoses, pulmonary hypoplasia, and carinal involvement. The trachea was approached through an anterolateral thoracotomy in five cases and through the neck in one. Cardiopulmonary bypass was never required. Simple resection was possible only once. Four patients received costal cartilage grafts, and one graft was created from dura. Grafts varied from 2 1/2 to 6 cm in length and extended onto the bronchi in two cases. All patients with grafts were treated with postoperative endotracheal stents and ventilatory assistance and all were extubated successfully. One patient had trisomy 16p+ detected after successful extubation and died later. One patient was treated expectantly and died. While each case must be individualized, repair of complex tracheal stenosis often results in dramatic immediate improvement and long-term success.