Congenital Malformations of the Esophagus

Congenital malformations of the esophagus consist of congenital esophageal stenosis, esophageal atresia and tracheoesophageal fistula, and laryngotracheoesophageal cleft. Congenital esophageal stenosis presents in three variants, most often treated with dilation but occasionally requiring resection. Esophageal atresia and tracheoesophageal fistula present in five variants with esophageal atresia with a distal tracheoesophageal fistula being by far the most common. All are treated with an operation, either open or thoracoscopic. There are many special situations with these babies, the most difficult being the problem of a long gap between the esophageal ends commonly found in pure esophageal atresia. The long-term outcome for these patients is generally good, with dysphagia and reflux being the most common issues extending into adulthood. Laryngotracheoesophageal clefts present in four varieties, with the most extensive being a connection between the trachea and the esophagus extending down to the area of the carina. Most but not all of these anomalies require surgical repair.