Esophageal Atresia

The esophagus is a muscular tube that transports a food bolus from the pharynx to the stomach. The esophagus is derived from the endoderm germ layer which forms the pharynx, esophagus, stomach, and the epithelial lines of the aerodigestive tract. The trachea and esophagus arise from the separation of a common foregut tube during early fetal development. Failure of separation or complete development of this common foregut tube can lead to tracheoesophageal fistula (TEF) and esophageal atresia (EA). Prenatally, patients with EA may present with polyhydramnios, mostly in the third trimester, which may be a diagnostic clue to EA.  Additionally, approximately 50% of patients with TEF/EA will have associated congenital anomalies including VACTERL (vertebral defects, anal atresia, cardiac defects, TEF, renal anomalies, and limb abnormalities) or CHARGE (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities) syndrome. Once the neonate is born, the most common symptoms of EA include excessive drooling, choking, and failure to pass a nasogastric tube. Furthermore, if there is an associated TEF, there will be gaseous distension of the stomach as air travels from the trachea through the distal esophageal fistula then into the stomach.  Patients with this constellation of symptoms should have an expedited workup for EA and TEF, and prompt referral to a higher level of care for pediatric surgeon evaluation.