Practice Essentials
Esophageal atresia refers to a congenitally interrupted esophagus.
One or more fistulae may be present between the malformed esophagus and the trachea. The lack of esophageal patency prevents swallowing. In addition to preventing normal feeding, this problem may cause infants to aspirate and literally drown in their own saliva, which quickly overflows the upper pouch of the obstructed esophagus. If a tracheoesophageal fistula (TEF) is present, fluid (either saliva from above or gastric secretions from below) may flow directly into the tracheobronchial tree.
The condition was first described anecdotally in the 17th century. In 1670, Durston described the first case of esophageal atresia in one conjoined twin. In 1696, Gibson provided the first description of esophageal atresia with a distal TEF. In 1862, Hirschsprung (a famous pediatrician from Copenhagen) described 14 cases of esophageal atresia. In 1898, Hoffman attempted primary repair of the defect but was not successful and resorted to the placement of a gastrostomy.
At the start of the 20th century, surgeons were theorizing about how the lesion could be repaired. In 1939 and 1940, Ladd of Boston and Lever of Minnesota first achieved surgical success in stages; success meant that the affected children survived and skin-lined pharyngogastric conduits were eventually constructed. In 1941, Haight of Michigan successfully repaired esophageal atresia in a 12-day-old baby using a primary single-stage left-side extrapleural approach. Subsequent to that child’s survival and with advances in surgical and anesthetic techniques, esophageal atresia is now regarded as an eminently correctable congenital lesion.
The treatment plan for each baby must be individualized. (See Treatment.) Prognostic classifications (eg, the Waterston, Spitz, and Poenaru prognostic classification systems) can provide guidance in patients with multiple problems and help determine the indications for and timing of surgical repair, but early and decisive identification of the most life-threatening anomaly is essential. Surgical approaches to treatment vary according to surgeons’ preferences and variations in pathologic anatomy.
For patient education resources, see the Esophagus, Stomach, and Intestine Center and Procedures Center, as well as Choking and Bronchoscopy.