Esophageal atresia and tracheoesophageal Fistula

1. Esophageal atresia/tracheoesophageal fistula (EA/TEF) is an interruption of the normal formation of the esophagus. The most common variant seen is an esophageal atresia with a tracheoesophageal fistula to the distal esophagus. 2. All children diagnosed with EA/TEF require a VACTERL (vertebral, anorectal, cardiac, tracheoesophageal, renal, limb) workup for associated anomalies to include a physical examination, echocardiogram, renal ultrasound, and plain radiographs of the spine. The echocardiogram should be performed prior to operative intervention. 3. Surgical correction can be performed through an open thoracotomy or thoracoscopically based upon the surgeon's experience and comfort. 4. Many children with EA/TEF will develop gastroesophageal reflux disease, tracheomalacia, or an esophageal stricture at the anastomosis which may prompt further operative treatments. 5. Long-gap esophageal atresia is best treated by a delayed operative intervention to allow for growth of the esophageal pouch. Multiple options are available for reconstruction or replacement of the native esophagus and depends upon institutional experience.