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What to Know About Tracheoesophageal Fistula and Esophageal Atresia

Medically Reviewed by Dan Brennan, MD on August 06, 2022

Tracheoesophageal fistula and esophageal atresia are birth defects that happen within the trachea and esophagus, which are tubes in the throat. Although the two are different defects, they often occur together.

What Is Tracheoesophageal Fistula?

Inside your throat are two muscular tubes: the esophagus, the tube that leads from your mouth to your stomach, and the trachea, the tube that leads to your lungs. When babies are developing in the womb, these tubes start to develop as one and then separate later on. 

A tracheoesophageal fistula happens when the esophagus and the trachea don’t separate in the way they’re supposed to. When this defect occurs, the two tubes are open to each other, leading to potential issues with eating and breathing. This can also lead to pneumonia. Babies born with tracheoesophageal fistula often have esophageal atresia as well.

Tracheoesophageal Fistula Symptoms

For a baby born with a tracheoesophageal fistula but not esophageal atresia, their symptoms may not appear immediately after birth. The most common tracheoesophageal fistula symptoms include coughing while feeding and lung infections.

Unfortunately, tracheoesophageal fistula doesn’t show up on prenatal scans, so diagnosis isn’t possible until after birth, usually after symptoms become noticeable. To diagnose a tracheoesophageal fistula, your baby’s doctor may recommend options such as:

  • X-rays to look at the baby's chest and abdomen
  • An endoscopy, which involves sliding a thin tube with a camera on the end down the esophagus to diagnose issues in the upper digestive system
  • A bronchoscopy, a procedure similar to an endoscopy except the tube goes through the trachea into the lungs

Tracheoesophageal Fistula Treatment

Tracheoesophageal fistula is treated through surgery. The exact surgical methods will depend on how many tracheoesophageal fistulas — or individual connections between the esophagus and trachea — are present, their severity, their locations, and whether any other conditions, like esophageal atresia, are present as well. Treatment may also depend on your baby’s health and the opinions of the surgeons and other doctors.

Surgery typically involves making a small incision in the neck or back, closing the connection between the esophagus and trachea, and making any necessary repairs.

What Is Esophageal Atresia?

Esophageal atresia is a condition where the esophagus doesn’t form correctly or doesn’t form all the way. It often occurs with tracheoesophageal fistula. There are four different types of esophageal atresia:

  • Type A: In Type A esophageal atresia, the upper and lower parts of the esophagus do not connect, and each part has a closed end.
  • Type B: Type B is a rare type that occurs along with a tracheoesophageal fistula. In Type B, the upper part of the esophagus is connected to the trachea, but the lower end is not connected and has a closed end.
  • Type C: This is the most common type of esophageal atresia. It also occurs with a tracheoesophageal fistula. Type C happens when the top of the esophagus is closed off and the bottom end is attached to the trachea.
  • Type D: Type D is the most severe but also the rarest type of esophageal atresia. In Type D, the upper and lower parts of the esophagus are each connected separately to the trachea instead of to each other.

Approximately 1 in 4,100 babies are born with esophageal atresia in the US. Researchers still don’t know the exact cause of esophageal atresia, but it often shows up with other birth defects, such as:

  • Trisomy 13, trisomy 18, or trisomy 21
  • Tethered spinal cord
  • Other digestive tract problems
  • Kidney and urinary tract problems
  • Heart problems
  • Muscular or skeletal problems

Esophageal Atresia Symptoms

The exact symptoms of esophageal atresia may vary depending on which type of the condition your baby has. Common esophageal atresia symptoms include:

  • Coughing and choking when feeding
  • Difficulty breathing
  • Frothy white bubbles in the baby’s mouth
  • Bluish skin

Esophageal atresia can sometimes be identified before birth. Otherwise, the symptoms of esophageal atresia appear much earlier than the symptoms of tracheoesophageal fistula, usually shortly after birth when the baby tries to feed. Diagnosis is usually confirmed via X-ray.

Esophageal Atresia Treatment

There are several options available to treat esophageal atresia, and some are more invasive than others. Current options for treatment include:

  • The Foker process: A revolutionary new technique that works by stimulating the ends of the esophagus, causing them to grow, and attaching the two together once they’ve grown enough to do so.
  • Internal traction: Both static internal traction and dynamic internal traction are additional methods of growing the esophagus.
  • Jejunal interposition: This technique uses a segment of the small intestine to replace the missing length of the esophagus.
  • Primary anastomosis: This procedure closes the esophageal gaps in children with smaller gaps.

Prognosis for Tracheoesophageal Fistulas and Esophageal Atresia

When tracheoesophageal fistulas and esophageal atresia are caught early in infants, and when treatment isn't complicated by other factors, recovery can be quick. Your baby will need to stay in the hospital and be fed through an IV or stomach tube until they can swallow properly. After treatment, many infants may also have tracheomalacia, which is a weak and floppy trachea.

Long-term effects of tracheoesophageal fistulas and esophageal atresia may include gastroesophageal reflux disease, also called GERD, as well as scar tissue that may need future surgery.

Tracheoesophageal Fistula vs. Esophageal Atresia

Tracheoesophageal fistulas and esophageal atresia are issues that can affect the trachea and esophagus, and they often appear together. To summarize:

  • Tracheoesophageal fistula is an opening between the trachea and esophagus, whereas esophageal atresia is when the two ends of the esophagus don’t meet, sometimes resulting in a tracheoesophageal fistula.
  • Tracheoesophageal fistula symptoms may not appear right away after delivery, but esophageal atresia symptoms are usually obvious the first time the baby tries to feed. Both may be diagnosed via X-ray. Tracheoesophageal fistulas may also require an endoscopy or bronchoscopy for diagnosis. Esophageal atresia may be diagnosable during pregnancy.
  • Treatment for tracheoesophageal fistula intends to repair any holes between the two tubes, whereas treatment for esophageal atresia involves joining the two ends of the esophagus together and repairing any holes caused by tracheoesophageal fistula.

Show Sources

SOURCES:

Boston Children’s Hospital: “Esophageal Atresia,” “Tracheoesophageal Fistula.”

Centers for Disease Control: “Facts About Esophageal Atresia.”

Children’s National: “Pediatric Tracheoesophageal Fistula and Esophageal Atresia.”

Family Doctor: “Esophageal Atresia and Tracheoesophageal Fistula.”

Mayo Clinic: “Bronchoscopy,” “Upper endoscopy.”

Stanford Medicine Children’s Health: “Tracheoesophageal Fistula and Esophageal Atresia.”

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