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Esophageal Atresia and Tracheoesophageal Fistula

Newborn baby with esophageal atresia or tracheoesophageal fistula

What is esophageal atresia?

Esophageal atresia (EA) is a type of birth defect that affects a baby’s esophagus. It forms while the baby is developing in their mother’s uterus.

The esophagus is a tubular structure that connects the throat to the stomach. In a baby with EA, the esophagus is interrupted during fetal development and does not develop into the shape of a tube.

Instead, it forms into two separate parts. One part connects to the throat and the other connects to the stomach, but these parts do not connect to one another.

Babies with EA are not able to digest food or liquids. Because their esophagus is split into two segments, they can consume foods and liquids, but they cannot digest it.

One of the first signs that a baby might have EA is their inability to swallow their own saliva right after birth. If this occurs, the doctor may order an X-ray to confirm the diagnosis. Other times, a prenatal ultrasound can reveal the presence of EA before the baby is born.

What is tracheoesophageal fistula?

In a baby with tracheoesophageal fistula (TEF), there is an abnormal connection in at least one location between the esophagus and the trachea.

The trachea is a tube that travels from the throat to the baby’s windpipe and lungs. A critical part of their airway system, it carries oxygen-rich air to their lungs and carbon dioxide out of them.

Under normal conditions, the esophagus and trachea are two separate tubes that exist and operate independently of one another. They do not connect.

With TEF, a fistula forms that connects the two tubes together. When they swallow formula, milk, or saliva, those liquids can travel across that connection, passing through the esophagus and into the trachea. There, they can be inhaled into the baby’s lungs.

This can cause the baby to cough and choke during feeding. It can also lead to respiratory issues as they get older, as well as frequent lung infections or pneumonia.

There are five main tracheoesophageal fistula types, categorized from Type A to Type E. The most common is Type C. This occurs when the upper portion of the baby’s esophagus ends in a blind pouch and the lower portion connects to the trachea by a fistula. About 85% of babies born with TEF have Type C.

Are esophageal atresia and tracheoesophageal fistula related?

Both EA and TEF can occur on their own. However, it is most common for these two birth defects to occur simultaneously. Babies born with EA typically also have TEF.

What causes esophageal atresia and tracheoesophageal fistula?

As babies grow in their mother’s uterus before birth, their organ systems are also maturing. In all babies, the esophagus and trachea first develop as one single tube.

However, during normal development, a wall will form between the fetus’ esophagus and trachea around four to eight weeks after conception. This wall separates the organs into two distinct tubes.

In babies with EA and/or TEF, this wall does not form properly or completely. When this happens, either birth defect can occur.

What are the symptoms of tracheoesophageal fistula and esophageal atresia?

Most of the time, doctors will notice the signs of TEF or EA almost immediately after a baby is born. While each child’s experience is unique, these are the most common symptoms:

  • Coughing or choking during feeding
  • White, frothy bubbles in the mouth
  • Blue tinge to the skin, especially during feeding
  • Vomiting
  • Breathing difficulties
  • Round, full abdomen

What tests are usually done to diagnose the problem?

Doctors can perform a physical examination to evaluate whether a baby has TE and/or EA. To confirm a diagnosis, they may conduct an imaging study to get a closer look at the area of concern.

How will this affect my baby?

We understand the concern that new parents have when they discover that their baby has been diagnosed with TEF and/or EA. Our skilled surgeons can work quickly to reverse the condition, helping restore your child’s health and well-being.

Even with treatment, some children born with EA will develop long-term problems related to swallowing foods or liquids. These can develop due to issues, such as:

  • Foods and liquids cannot move normally down the esophagus (peristalsis)
  • Wounds heal after surgery and form scars, partially blocking the passage of food

If food and liquids are getting stuck after surgery, doctors can perform a procedure to widen your baby’s esophagus. This can usually be done while they’re still under general anesthesia from the initial surgery.

Children who undergo EA repair may also have issues with gastrointestinal reflux disease, or GERD. This condition affects about half of all individuals who receive treatment for their condition.

What other conditions are associated with tracheoesophageal fistula or esophageal atresia?

While these two problems are not thought to be inherited, they often occur in babies who have other preexisting conditions. Up to half of all babies with TEF or EA are also born with another type of birth defect. These may include:

  • Trisomy 13, 18, or 21
  • Digestive tract problems
  • Heart problems
  • Muscular or skeletal problems
  • Kidney and urinary tract problems
  • VATERL syndrome (vertebral, anal, cardiac, TE fistula, renal, and limb abnormalities)

Doctors can perform X-rays and scans to ensure that your baby does not also have any of these co-occurring conditions.

Treatment for esophageal atresia

If your baby has EA, the surgeon will try to merge the two ends of their esophagus together through a small opening on the side of their chest. This can successfully connect their throat and stomach, allowing them to eat and drink comfortably.

In some babies, the ends of the esophagus are too far apart from each other to be combined. If this is the case, the surgeon can place a feeding tube called a gastrostomy into the baby’s stomach so they can eat and drink. Sometimes, this procedure can require more than one surgery.

While a gastrostomy can work when your baby is small, it is not usually designed as a permanent solution. The surgeon will monitor their condition as they grow and can fit them with a new esophagus when they’re bigger.

Treatment for tracheoesophageal fistula

If your baby has TEF, the surgical procedure follows a different course. With this technique, the surgeon will remove the abnormal passage, or fistula, from between your baby’s esophagus and trachea to close that unnatural connection.

Treatment for EA and TEF

If your baby has both EA and TEF, the surgeon will typically conduct the TEF surgery first. Once they’ve successfully removed the fistula, they will conduct the EA surgery and attempt to bring the two ends of the esophagus together.

What can I expect after surgery?

After surgery, they may need a little help breathing on their own. In the meantime, they’ll be connected to a mechanical ventilator that will support their healthy respiratory function and help them build back their strength. Our doctors will also perform frequent suctioning to remove their saliva until they can swallow it independently.

Most babies with EA and/or TEF will need to stay in the hospital for several weeks or months to complete the full extent of monitoring required. Your surgical care team will create a custom timeline and treatment plan based on your unique needs.