A tracheoesophageal fistula (TEF) is an abnormal connection between the trachea (windpipe) and the esophagus (the tube that carries food to the stomach). These two tubes run side by side in the chest but are normally completely separate. When a fistula forms between them, food, saliva, or stomach contents can pass into the airway, and air can leak into the digestive tract. Most cases are congenital, meaning babies are born with them, but adults can develop one too after injury, prolonged medical procedures, or cancer.
How a TEF Affects the Body
The trachea and esophagus share a wall along part of their length. A fistula creates an opening in that shared wall, essentially turning two sealed systems into one leaky one. Liquid or food that should travel straight to the stomach can spill into the lungs instead, causing choking, coughing, and repeated infections. Air meant for the lungs can also enter the stomach and intestines.
The size and location of the opening determine how severe the symptoms are. A tiny fistula might go unnoticed for months or even years, while a large one causes life-threatening breathing problems within hours of birth.
Congenital TEF in Newborns
Most tracheoesophageal fistulas are present at birth. In the majority of cases, the fistula occurs alongside esophageal atresia, a condition where the esophagus doesn’t form as one continuous tube. Instead, it ends in a blind pouch, with the lower portion connecting to the trachea through the fistula. This is the most common pattern, accounting for roughly 85% of cases.
Less commonly, a baby can be born with a fistula but a fully intact esophagus. This is sometimes called an H-type fistula because of its shape. Because food can still reach the stomach normally, these babies often don’t show obvious symptoms right away. Over time, though, they tend to develop frequent lung infections and coughing during feedings.
Babies born with esophageal atresia and a fistula together typically show symptoms immediately after delivery: coughing, choking when trying to swallow, and difficulty breathing. These signs are usually noticed in the delivery room or nursery.
Causes in Adults
Adults can develop a TEF for several reasons, though it’s far less common than the congenital form. The most frequent causes include cancer of the esophagus or trachea eroding through the shared wall, prolonged use of a breathing tube pressing against the esophageal wall, and traumatic injury to the chest. In rare cases, someone born with a small congenital fistula that was never detected may not be diagnosed until adulthood.
Regardless of the cause, an acquired TEF carries serious risks. Complications include recurrent pneumonia, lung abscesses, poor nutrition from difficulty eating, and in severe cases, respiratory failure.
The VACTERL Connection
About half of babies born with a TEF also have other birth defects. Doctors look for a pattern called VACTERL association, an acronym for a cluster of anomalies that frequently occur together: vertebral (spine) defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal (kidney) anomalies, and limb abnormalities.
Among children identified with the VACTERL pattern, 50 to 80 percent have a tracheoesophageal fistula. Heart defects appear in 40 to 80 percent, kidney problems in 50 to 80 percent, and limb differences in 40 to 50 percent. Because these conditions cluster together, a baby diagnosed with a TEF will typically be checked for the others as well.
How TEF Is Diagnosed
The most useful imaging tool is a contrast swallow study, where a water-soluble dye is swallowed (or delivered through a tube into the esophagus) while X-ray video captures its path in real time. The dye highlights whether liquid leaks through the wall into the trachea. This method detects the fistula in up to 80% of cases, though smaller or unusually positioned openings can be missed on the first attempt.
When the contrast study is inconclusive, doctors may turn to bronchoscopy, inserting a small camera into the airway while a blue dye is placed in the esophagus. If the dye appears in the airway, the fistula is confirmed. A similar approach using a camera in the esophagus (esophagoscopy) can locate the opening from the digestive side. For recurrent fistulas that reopen after prior surgery, a specialized pull-back technique with the contrast tube improves detection rates significantly.
Surgical Repair in Newborns
Surgery is the primary treatment for congenital TEF. The surgeon makes an incision between the ribs on the side of the chest, closes the abnormal connection between the trachea and esophagus, and sews the two ends of the esophagus together so food can travel normally to the stomach.
Sometimes the gap between the upper and lower portions of the esophagus is too wide to reconnect in one operation. When that happens, the first surgery focuses on closing the fistula to protect the lungs, and a feeding tube is placed directly into the stomach through the skin to provide nutrition. A second surgery to reconstruct the esophagus is scheduled once the baby has grown enough for the repair.
Managing TEF Caused by Cancer
When a fistula develops because a tumor has eaten through the wall between the esophagus and airway, surgery to repair the opening is often not an option. In these cases, the goal shifts to sealing the fistula and keeping both the airway and digestive passage open. Doctors typically place stents, small expandable tubes, inside the esophagus, the airway, or both.
A study of 30 patients with large malignant fistulas found that placing stents in both the airway and esophagus (a “double stent” approach) resulted in significantly longer survival, averaging 110 days, compared to 24 days with an airway stent alone. The double stent seals the fistula from both sides while keeping food and air flowing through their proper channels.
Long-Term Recovery and Complications
Survival rates for congenital TEF repair have improved dramatically, and most children go on to live normal lives. But the repair site can cause ongoing issues. Scar tissue where the esophagus was sewn together sometimes narrows the passage, making swallowing difficult. These strictures may need to be stretched with a balloon or dilator, sometimes more than once.
Gastroesophageal reflux is another common long-term issue after repair. Acid from the stomach flows back into the esophagus more easily, which can worsen any narrowing at the surgical site.
Tracheomalacia, a softening of the tracheal cartilage near the fistula site, affects some children after surgery. The weakened section of the airway can partially collapse during breathing, causing a barking cough or noisy breathing. This is usually mild and resolves on its own by 18 to 24 months of age as the cartilage strengthens. In more severe cases, a stent or other airway support may be needed.