atresiaTEF

Esophageal atresia with tracheoesophageal fistula (EA/TEF) is a congenital anomaly characterized by an esophagus that ends in a blind pouch and, in most cases, an abnormal connection between the distal esophagus and the trachea. The condition occurs in about 1 in 4,000 live births and is frequently associated with other congenital anomalies, notably within the VACTERL spectrum. The most common form is distal TEF with EA (Gross Type C), where a fistula connects the lower esophageal segment to the trachea.

Embryology and classification underlying EA/TEF involve abnormal foregut development, including failure of esophageal recanalization and incomplete separation of the trachea and esophagus. The Gross classification describes several forms: Type A (isolated EA with no TEF), Type B (proximal TEF), Type C (distal TEF, most common), Type D (proximal and distal TEFs), and Type E (TEF without EA, H-type). These variations influence surgical planning and prognosis.

Clinically, affected newborns present with excessive drooling, choking, coughing or cyanosis during feeding, and respiratory distress. Prenatal polyhydramnios may be detected. Postnatal diagnosis often begins with inability to pass a nasogastric tube into the stomach; radiographs typically show a blind proximal esophageal pouch, and if a distal TEF is present, gas may be seen in the stomach and intestines. A workup for associated anomalies is routinely performed.

Management involves immediate stabilization: airway protection, suctioning, keeping the infant NPO, IV fluids, and gastric decompression. Broad-spectrum antibiotics may be used if pneumonia is suspected. Definitive surgical repair is usually performed within the first days of life. The procedure typically includes ligation of the fistula and connection (anastomosis) of the esophageal segments; approaches vary by anatomy and patient stability, with primary repair for short-gap EA and staged repairs or lengthening/replacements for long-gap cases. Postoperative care focuses on nutrition, monitoring for anastomotic leak or strictures, and managing reflux.

Prognosis has improved with neonatal specialty care; survival in developed centers often exceeds 80-90%, depending on associated anomalies. Long-term issues can include esophageal strictures and gastroesophageal reflux, requiring ongoing multidisciplinary follow-up.