Tracheoesophageal fistula and esophageal atresia repair

Definition

Tracheoesophageal fistula and esophageal atresia repair is surgery to repair two birth defects in the esophagus and trachea. The defects usually occur together. 

Alternative Names

TEF repair; Esophageal atresia repair

Description

The esophagus is the tube that carries food from the mouth to the stomach. The trachea (windpipe) is the tube that carries air into and out of the lungs.

The defects usually occur together. They may occur along with other problems as part of a syndrome (group of problems):

This surgery is almost always done soon after birth. Both defects can often be repaired at the same time. Briefly, the surgery takes place this way:

Often the two parts of the esophagus are too far apart to sew together right away. In this case:

Sometimes the surgeon will wait 2 to 4 months before doing the surgery. Waiting allows your baby to grow or have other problems treated. If your child's surgery is delayed:

Why the Procedure Is Performed

Tracheoesophageal fistula and esophageal atresia are life-threatening problems. They need to be treated right away. If these problems are not treated:

Risks

Risks of anesthesia and surgery in general include:

Risks of this surgery include:

Before the Procedure

Your baby will be admitted to the neonatal intensive care unit (NICU) as soon as the doctors diagnose either of these problems.

Your baby will receive nutrition by vein (intravenous, or IV) and may also be on a breathing machine (ventilator). The care team may use suction to keep fluids from going into the lungs.

Some infants who are premature, have a low birth weight, or have other birth defects beside TEF and/or EA may not be able to have surgery until they grow larger or until other problems have been treated or have gone away.

After the Procedure

After surgery, your child will be cared for in the hospital's NICU.

Additional treatments after surgery usually include:

If both the TEF and EA are repaired:

If only the TEF is repaired, a G-tube is used for feedings until the atresia can be repaired. Your baby may also need continuous or frequent suction to clear secretions from the upper esophagus.

While your baby is in the hospital, the care team will show you how to use and replace the G-tube. You may also be sent home with an extra G-tube. The hospital staff will inform a home health supply company of your equipment needs.

How long your infant stays in the hospital depends on the type of defect your child has and whether there are other problems in addition to the TEF and EA. You will be able to bring your baby home once they are taking feedings by mouth or gastrostomy tube, is gaining weight, and safely breathing on their own.

Outlook (Prognosis)

Surgery can usually repair a TEF and EA. Once healing from the surgery is complete, your child may have these problems:

During infancy and early childhood, many children will have problems with breathing, growth, and feeding, and will need to continue seeing both their primary care provider and specialists.

Babies with TEF and EA who also have defects of other organs, most commonly the heart, may have long-term health problems.

References

Dingledein M. Selected gastrointestinal anomalies in the neonate. In: Martin RJ, Fanaroff AA, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine: Diseases of the Fetus and Infant. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 88.

Madanick R, Kaila V. Anatomy, histology, embryology, and developmental anomalies of the esophagus. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 43.

Rothenberg SS. Esophageal atresia and tracheoesophageal fistula malformations. In: Holcomb GW, Murphy JP, St. Peter SD, eds. Holcomb and Ashcraft's Pediatric Surgery. 7th ed. Philadelphia, PA: Elsevier; 2020:chap 27.



Review Date: 12/31/2023
Reviewed By: Mary J. Terrell, MD, IBCLC, Neonatologist, Cape Fear Valley Medical Center, Fayetteville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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