TE Fistula

• Incidence 1/4000

• Caused by abnormal budding of the trachea from the primitive foregut

• Multiple types of malformations. Majority have esophageal atresia

• May present with polyhydramnios

• Most commonly present with feeding intolerance shortly after birth; Cannot pass feeding tube to stomach

• Proximal esophageal pouch with distal fistula most common (85%) and usually presents early with drooling, choking, cyanosis with feeds

• H-type may present later in life with persistent pneumonia or cough

• 50% of patients have other anomalies

  • Cardiac (VSD, PDA, ToF)

  • GI (duodenal atresia, imperforate anus)

  • GU (renal agenesis, horseshoe kidney)

  • VATER/VACTERL

  • Down syndrome

• Rx: NPO and keep in prone position to minimize reflux until surgery; keep esophageal pouch decompressed

• Surgical correction required but medical issues persist after surgery

  • Esophageal dysmotility

  • Esophageal strictures at site of anastomosis

  • Recurrence of fistula

  • GERD, recurrent aspiration

  • RAD

Diagram below:

1. esophageal atresia with distal TEF 87%

2. isolated esophageal atresia 8%

3. isolated TEF 4%

4. esophageal atresia with proximal TEF 1%

5. esophageal atresia with proximal and distal TEF 1%