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Congenital Esophageal Artesia and Tracheoesophageal Fistula

What are the esophagus and trachea?

  • Esophagus: tube that connects the mouth to the stomach
  • Trachea: "windpipe"
  • Atresia: absence of a normal opening
  • Congenital: found at birth
  • Fistula: abnormal passage from a body organ to the body surface or between two internal body organs.

What is congenital esophageal atresia (EA) and Tracheoesophageal fistula (TEF)?

Congenital esophageal atresia (EA) represents a failure of the esophagus to develop as a continuous passage. Instead, it ends as a blind pouch. Tracheoesophageal fistula (TEF) represents an abnormal opening between the trachea and esophagus. EA and TEF can occur separately or together. EA and TEF are diagnosed in the ICU at birth and treated immediately.

How is Congenital Esophageal Atresia and Tracheoesophageal Fistula diagnosed?

The presence of EA is suspected in an infant with excessive salivation (drooling) and in a newborn with drooling that is frequently accompanied by choking, coughing and sneezing. When fed, these infants swallow normally but begin to cough and struggle as the fluid returns through the nose and mouth. The infant may become cyanotic (turn bluish due to lack of oxygen) and may stop breathing as the overflow of fluid from the blind pouch is aspirated (sucked into) the trachea. The cyanosis is a result of laryngospasm (a protective mechanism that the body has to prevent aspiration into the trachea). Over time respiratory distress will develop.

If any of the above signs/symptoms are noticed, a catheter is gently passed into the esophagus to check for resistance. If resistance is noted, other studies will be done to confirm the diagnosis. A catheter can be inserted and will show up as white on a regular x-ray film to demonstrate the blind pouch ending. Sometimes a small amount of barium (chalk-like liquid) is placed through the mouth to diagnose the problems.

How is Congenital Esophageal Atresia and Tracheoesophageal Fistula Treated?

Treatment of EA and TEF is surgery to repair the defect. If EA or TEF is suspected, all oral feedings are stopped and intravenous fluids are started. The infant will be positioned to help drain secretions and decrease the likelihood of aspiration. Babies with EA may sometimes have other problems. Studies will be done to look at the heart and spine. Sometimes studies are done to look at the kidneys.

Surgery to fix EA is rarely an emergency. Once the baby is in condition for surgery, an incision is made on the side of the chest. The esophagus can usually be sewn together. Following surgery, the baby may be hospitalized for a variable length of time. Care for each infant is individualized. If you have any questions do not hesitate to ask a member of the pediatric surgery team.

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Contacts

The University of Texas Health Science Center Professional Building
6410 Fannin, Suite 370
Houston, TX 77030
Phone: (713) 486-5000

Office Hours: 8 a.m. to 5 p.m. (Monday-Friday except major holidays)

This information, although based on a thorough knowledge and careful review of current medical literature, is the opinion of doctors at The University of Texas Medical School and is presented to inform you about surgical conditions. It is not meant to contradict any information you may receive from your personal physician and should not be used to make decisions about surgical treatment. If you have any questions about the information above or your child's care, please contact our doctors.