Tracheo-Esophageal Fistula

A tracheo-esophageal fistula (TEF) is an abnormal pathway between the trachea, or windpipe, and the esophagus, which connects the throat with the stomach. There are two forms of TEF:

  • Primary TEF - this form is present from birth (congenital) and almost exclusively diagnosed in infants and children.
  • Secondary TEF - this is a result of another medical condition, such as cancer and is most often diagnosed in adults.

Young patients born with TEF, frequently have an esophagus that is not completely formed, a condition known as esophageal atresia.

Patients with cancer of the esophagus may develop a tracheo-esophageal fistula if the tumor grows from the esophagus into the trachea.

The disorder is serious because things that are swallowed enter the lungs and cause pneumonia.



Symptoms of a tracheo-esophageal fistula can vary depending on the age of the patient, the size of the opening, and whether associated medical conditions are present. The most common symptom of TEF is repeated lung infections such as pneumonia.

Symptoms of TEF in infants are generally worse while they are feeding and may include:

  • Frothy white bubbles in the mouth
  • Coughing or choking during feeding
  • Vomiting
  • Blue color of the skin, especially during feeding
  • Trouble breathing
  • Very round, full stomach

Symptoms of TEF in adult patients may include:

  • Chest pain
  • Shortness of breath
  • Labored breathing
  • Coughing or choking when eating or drinking
  • Drooling or excess mucus in the mouth
  • Enlarged abdomen
  • Swallowed food or liquids are coughed out or suctioned from the airways
  • Wheezing or bubbly sounds following each breath

Causes and Risk Factors

A tracheo-esophageal fistula is most frequently diagnosed in infants or young children and occurs in one out of every 3,000 to 5,000 births. One third of patients born with the condition are born prematurely and TEF is often associated with other birth defects such as:

  • Trisomy 13, 18 or 21
  • Other digestive tract problems, such as diaphragmatic hernia, duodenal atresia or imperforate anus
  • Heart problems, such as ventricular septal defect, tetralogy of Fallot, or patent ductus arteriosus
  • Kidney and urinary tract problems, such as horseshoe or polycystic kidney, absent kidney, or hypospadias
  • Muscular or skeletal problems
  • VACTERL association, which involves vertebral, anal, cardiac, TE fistula, renal, and limb abnormalities
  • Adult patients with lung cancer are also at an increased risk of developing the condition

Adult patients with lung cancer are also at an increased risk of developing the condition.



For some patients, diagnosis of a tracheo-esophageal fistula can occur during a routine prenatal ultrasound while the baby is still developing in the womb. In infants and children, diagnosis of TEF usually begins with a physical exam and a review of the patient’s medical history and symptoms. In adult patients, the physician will determine if the patient’s symptoms indicate the presence of esophageal cancer, which can cause TEF.

For newborn patients, the main diagnostic tool uses a nasogastric tube (NG tube) to determine if the esophagus is blocked. This tube is passed through the baby’s nose in an attempt to guide it through the esophagus and into the stomach. If a patient has TEF, the tube is stopped by a blockage, usually around the mid-chest. The medical team will then look at the blockage on chest x-rays. This is normally all that is needed to diagnose primary TEF.

For adults with secondary TEF, additional diagnostic tools may be used to determine if the patient has esophageal cancer. This may include CT scans to see inside the chest and lungs, or a biopsy to sample the affected tissue. During a biopsy, a small sample of tissue is removed using a needle and then examined under a microscope.



The physicians at the Women's Guild Lung Institute are experts at treating tracheo-esophageal fistulas. The treatment almost always involves surgery to address the condition. In infants this involves closing the hole between the esophagus and the trachea, while in adult patients surgery may focus on managing the side effects of esophageal cancer.

For young patients born with the condition, surgery to close the hole between the esophagus and trachea will be the main focus of treatment. If the patient was also born with esophageal atresia, surgery to correct this defect may be performed at the same time.

Some infants will need to become stronger before surgery is performed. For these patients, gastrostomy may be needed because the child may not be able to eat food by mouth. A gastrostomy involves a surgeon cutting a hole in the baby’s abdomen and inserting a tube into their stomach. The baby can then receive the nutrients needed directly through the tube.

In adult patients, stenting may be performed to close the hole between the esophagus and trachea. Esophageal stents are a silicone tube that is surgically inserted through the tumor. As the stents expand, they block off the fistula and open up the tumor. This allows food to pass beyond the tumor to the stomach without going into the lungs.

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