Esophageal Atresia & Tracheoesophageal Fistula (TEF)

• • 85% esophageal atresia with distal TEF, 5-7% pure esophageal atresia, 2-6% TEF alone, other configurations more rare

  • Risk factors:

  • -First pregnancy

    -Advanced maternal age

    -Affected parent

    -Affected siblings

Epidemiology

• • 1/2500 to 1/20,000 births affected

Symptoms and Signs

• • Asymptomatic first few hours of life

  • Excessive drooling

  • Choking, coughing

  • Regurgitation

  • Respiratory distress

  • Cyanosis

  • Scaffoid abdomen if pure esophageal atresia

Imaging Findings

• • Prenatal US: Polyhydramnios

  • Chest and abdominal x-ray

  • -Blind esophageal pouch when NG inserted

    -Gasless abdomen means no associated TEF

    -Gas filled means associated TEF

Rule Out

• • Other associated abnormalities (present in 50% of patients)

  • -Vertebral

    -Anorectal

    -Cardiac

    -Renal

    -Radial limb

• • History and physical exam

  • Chest and abdominal films with presence of NG tube

  • Occasionally esophageal contrast study to verify presence of fistula

  • Occasionally bronchoscopy or esophagoscopy to verify presence of fistula

Surgery

• • Decompressive gastrostomy if TEF causing gastric distention and subsequent difficulty breathing secondary to compression of diaphragm

  • Resection of fistula with primary repair of esophageal atresia

Indications

• • Resolution or stabilization of pulmonary or cardiac abnormalities

Medications

• • Sump suction to esophageal pouch

Complications

• • Anastomotic leak, 10-20%

  • Stricture, 40%

  • Recurrent TEF, 10%

  • Gastroesophageal reflux disease, all

  • Esophageal dysmotility, all

  • Tracheomalacia

Prognosis

• • 85-90% survival

References