Esophageal atresia is a sporadic congenital disease of varied anatomic presentation defined by atresia and/or fistulization of the esophagus to the trachea. It is the most common congenital abnormality of the esophagus but presents only in 1: 2500 to 1: 4500 births. The type of esophageal atresia is classified on the basis of specific anatomic patterns and the frequency of occurrence. The etiology is unclear, with several theories proposed, generally involving abnormal formation of a tracheal diverticulum during embryogenesis.
Genetic abnormalities have also been identified but none that are uniformly present in infants with this syndrome. Diagnosis can be made with prenatal ultrasound approximately half the time, but the disorder is typically suspected in an infant with copious secretions, suffocation and cyanosis (particularly with breast feeding), and inability to pass an orogastric tube. Prompt recognition is essential with diagnostic testing, including chest radiography and bronchoscopy. The surgical procedure is dictated by the type of atresia and/or fistulization present. Prognosis is dependent on preoperative birth weight, respiratory status, and the rates of morbidity and mortality that may accompany associated anomalies, particularly cardiac disorders such as ventricular septal defects and tetralogy of Fallot. Notably, 50% of infants with esophageal atresia have other congenital abnormalities. An acronym used clinically to describe a subset of these infants is VACTERL (vertebral, anal, cardiac, tracheal, esophageal, renal, and limb). The timing of surgery depends on the type of atresia and the respiratory status of the infant. Infants with esophagotracheal fistulization and/or aspiration need urgent surgery, whereas infants without respiratory compromise may be followed for several months with gastrostomy tube feeding until the esophagus matures and grows and is more amenable to an anastomosis between the proximal and distal ends. One of the great challenges in this surgery is having enough remnant esophagus to accomplish this. Indeed, substitutes may be needed, including stomach or intestinal interposition. Short and longterm postoperative complications include anastomotic lea and/or stricture and severe gastroesophageal reflux.