Pulmonary agenesis (aplasia), esophageal atresia, and tracheoesophageal fistula: a different treatment strategy.

This is a report of a patient with esophageal atresia, distal tracheoesophageal fistula, and right pulmonary agenesis who survived for 10 months. Death was due to progressive respiratory failure following a complicated clinical course. Several lessons were learned. First, the remaining lung is at high risk. Since the number of respiratory units in these infants is already decreased, the loss of more units has a more profound effect than in an otherwise healthy child. Second, while the establishment of esophageal continuity is important, all attempts to perform an esophagoesophagostomy should be abandoned if doing so further jeopardizes the solitary lung. Third, since all of these children have some degree of tracheomalacia, early tracheostomy should be considered. The tracheostomy will not only act as a tracheal stent but will also avoid progressive laryngeal pathology, which would result from long-term endotracheal intubation.