tefs

TEFs, short for tracheoesophageal fistulas, are congenital abnormal connections between the trachea and esophagus. They often occur with esophageal atresia, a condition in which the esophagus ends in a blind pouch, preventing normal passage of food. The most common presentation is esophageal atresia with a distal tracheoesophageal fistula (often referred to as type C in classic classifications). A separate but related variant is the H-type TEF, where a fistula connects the trachea and esophagus without esophageal atresia.

Epidemiology and causes

Tracheoesophageal fistulas occur in about 1 in 3,000 to 4,500 live births. The condition is usually sporadic, with no known single cause, though associated congenital anomalies can occur.

Clinical presentation

Newborns typically exhibit coughing, choking, cyanosis, or tachypnea during feeds, excessive secretions, or aspiration events. Prenatal signs may include polyhydramnios. A nasogastric tube often cannot be passed beyond a proximal esophageal pouch, and chest radiographs may show a coiled tube in the neck area; in cases with a distal fistula, air may be present in the stomach.

Diagnosis and management

Diagnosis is based on clinical presentation and imaging, with X-ray verification of anatomy. Initial management focuses on stabilization: maintain airway, decompress the stomach with a nasogastric tube, keep the infant NPO, and provide IV fluids and broad-spectrum antibiotics if needed. Definitive treatment is surgical repair, usually performed within the first several days of life. Short-gap EA with distal TEF is typically repaired with primary anastomosis and fistula closure; long-gap EA may require staged repair, including possible gastric or intestinal lengthening or interposition techniques. H-type TEF is treated by ligation and division of the fistula.

Prognosis and complications

Survival has improved with modern neonatal care. Complications can include anastomotic stricture, gastroesophageal reflux, recurrent TEF, and airway issues such as tracheomalacia. Long-term follow-up focuses on growth, feeding, and respiratory health.