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Tracheoesophageal Fistula and Esophageal Atresia

What Are Tracheoesophageal (TE) Fistula and Esophageal Atresia?

A tracheoesophageal fistula and esophageal atresia happen when two throat tubes don't develop properly before birth. The tubes are:

  • the trachea (windpipe), which carries oxygen into the lungs
  • the esophagus, which carries food to the stomach

Most babies with a tracheoesophageal fistula (TE fistula) also have esophageal atresia. These can cause breathing and feeding problems at birth, so they need treatment right away.

Surgery done soon after birth can fix both problems. But babies with esophageal atresia may need more surgeries.

What Happens in a Tracheoesophageal Fistula?

A tracheoesophageal fistula (tray-kee-oh-eh-sof-uh-JEEL FISS-chuh-luh) is an unusual connection that goes between the trachea and esophagus. Normally, these two tubes are side-by-side and closed off from each other. But when a baby has a TE fistula, these tubes share a pathway.

Side view of baby with TE fistula and esophageal atresia. Instead of the esophagus being one long tube, it is split into two segments. The first segment is short and closed off at one end. The second segment is connected to the trachea.

So, when a baby drinks breast milk or formula, it goes down the throat and can get into the lungs. When a baby breathes, air can fill the stomach. Stomach acids can back up into the lungs. This can cause:

A baby with a small TE fistula and no esophageal atresia may not have symptoms at birth. But when that child is older, he or she could have:

  • feeding problems
  • poor weight gain
  • frequent lung infections
  • wheezing

What Happens in Esophageal Atresia?

Esophageal atresia (ih-sof-uh-JEEL ah-TREE-zah) happens when the upper part of the esophagus does not connect to the lower part. This causes a blockage, so saliva (spit) and food can't go down into the stomach.

Babies with esophageal atresia have:

  • drooling
  • coughing
  • breathing problems
  • feeding problems

What Causes a Tracheoesophageal Fistula and Esophageal Atresia?

Doctors don't know exactly what causes these problems. It's likely a combination of:

  • genes
  • something in the environment during a woman's pregnancy that changes the way the trachea and esophagus form

About half of babies born with a TE fistula and esophageal atresia have no other problems. Some babies born with a TE fistula will have other health issues, like heart or kidney problems. A TE fistula and esophageal atresia are more common in babies with a genetic condition like Down syndrome (trisomy 21).

How Are Tracheoesophageal Fistula and Esophageal Atresia Diagnosed?

These problems usually are diagnosed before a baby is born on a prenatal ultrasound. If not, doctors will suspect a problem at birth if a baby:

  • drools a lot
  • has frothy mucus in the mouth and nose

The baby also might cough, choke, throw up, or turn blue, especially when feeding.

To diagnose esophageal atresia, doctors will try to pass a tube through the baby's nose or mouth into the stomach. If the tube cannot reach the stomach, esophageal atresia is likely.

Doctors also do chest X-rays, computed tomography (CT) scans, and other tests to check for both esophageal atresia and a TE fistula.

Because these babies are more likely to have other health conditions, doctors also check for problems with a baby's bones, digestive tract, heart, and other organs.

How Are Tracheoesophageal Fistula and Esophageal Atresia Treated?

Both conditions are treated with surgery:

  • If a baby is born with a TE fistula, surgeons will remove the fistula and close the opening between the trachea and esophagus soon after birth.
  • If a baby also has esophageal atresia, doctors usually fix it during this surgery as well. If the two ends of the esophagus are close, surgeons sew them together. If the ends are farther apart, doctors may wait to repair the esophagus. The ends often grow closer together as a baby gets older. Some babies may need procedures to make the esophagus longer.

Sometimes babies with esophageal atresia get a gastrostomy tube (G-tube) until surgeons can repair the esophagus. A G-tube goes in through the belly to deliver breast milk or formula right to the stomach.

A medical team cares for babies with TE fistula and esophageal atresia. Sometimes called an aerodigestive team, it includes specialists who:

  • do surgery
  • treat digestive tract disorders (gastroenterologists)
  • treat ear, nose, and throat problems (otolaryngologists)
  • treat lung problems (pulmonologists)
  • offer nutrition counseling (dietitians)
  • treat feeding and swallowing disorders (speech-language therapists)

The team works together to help the babies eat, breathe, and swallow.

What Else Should I Know?

Gastroesophageal reflux (GER) is common in children who had a TE fistula and esophageal atresia fixed. These kids also are more likely to:

  • have swallowing problems
  • cough, wheeze, or have asthma
  • get a lot of respiratory infections

If your child had a TE fistula or esophageal atresia, be sure to go to all follow-up doctor visits. Work with the care team to treat any related health problems.

Reviewed by: Mary L. Gavin, MD
Date Reviewed: 25-04-2019

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