Tracheoesophageal Fistula

What is a tracheoesophageal fistula?

Tracheoesophageal (tra-ke-o-e-sof-ah-JE-al) fistula (FIS-tu-lah), or TEF, is an abnormal connection between the trachea and the esophagus. The trachea (windpipe) is the passageway through which the air we breathe travels to the lungs. The esophagus (food pipe) is a tube that carries food from the mouth to the stomach. Normally, the trachea and esophagus are not connected. In TEF, air can pass from the trachea into the stomach or food can pass from the esophagus into the lungs. This may lead to breathing or swallowing problems, which can be serious or life-threatening.

What causes a tracheoesophageal fistula?

TEF may be congenital (something that happened before birth) or it may occur anytime during life. The following conditions may cause TEF:

  • Congenital: Problems during the growth of the fetus (baby) may cause defects in body parts. TEF may develop along with other birth defects that involve the esophagus, such as esophageal atresia.

  • Acquired: Injury to the neck or chest is the usual cause of TEF. This is often due to mechanical ventilation, such as a ventilator, or having a tracheostomy tube put in. A tracheostomy tube is put into the windpipe (trachea) to help you breathe. The injury may cause inflammation (swelling) or perforation (tear). The damaged tissues may stick together, forming scars and an abnormal connection between the trachea and esophagus. TEF may also be due to tumors, placement of nasogastric (NG) tubes, or infections, such as tuberculosis.

What are the signs and symptoms of a tracheoesophageal fistula?

The signs and symptoms of TEF depend largely on the size of the fistula. The signs may also be different if you are on a ventilator. You may have one or more of the following:

  • Chest pain, shortness of breath, or labored breathing.

  • Coughing or choking when eating or drinking in those who are not on a ventilator.

  • Drooling or having lots of mucus (secretions) in the mouth.

  • Enlarged abdomen (stomach).

  • Swallowed food or liquids may be coughed out or suctioned from the airways.

  • Wheezing or bubbly sounds following every breath.

How is a tracheoesophageal fistula diagnosed?

You may have one or more of the following tests:

  • Barium swallow: This test is an x-ray of your throat and esophagus, the tube connecting your throat to your stomach. This test may also be called a barium esophagram. You will drink a thick liquid called barium. Barium helps your esophagus and stomach show up better on x-rays. Follow the instructions of your caregiver before and after the test.

  • Bronchoscopy: This is a procedure to look inside your airway and learn the cause of your airway or lung condition. A bronchoscope (thin tube with a light) is inserted into your mouth and moved down your throat to your airway. You may be given medicine to numb your throat and help you relax during the procedure. Tissue and fluid may be collected from your airway or lungs to be tested.

  • Chest x-ray: Caregivers use it to see how your trachea, esophagus, lungs, and heart look like and are doing. This may also be used to look for signs of infection, like pneumonia, or collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.

  • Computerized tomography scan: This test is also called a CT or CAT scan. A special x-ray machine uses a computer to take pictures of your neck and chest. Before taking the pictures, you may be given a dye through an IV. The dye helps the esophagus, trachea, lungs, heart, and blood vessels show up better in the pictures. People who are allergic to shellfish (lobster, crab, or shrimp) may be allergic to this dye. Tell your caregiver if you are allergic to any of these.

  • Endoscopy: This test uses a scope to see the inside of your digestive tract. A scope is a long, bendable tube with a light on the end of it. A camera may be hooked to the scope to take pictures. During an endoscopy, caregivers may find problems with how your digestive tract is working. Samples may be taken from your digestive tract and sent to a lab for tests. Small tumors may be removed, and bleeding may be treated during an endoscopy.

How is a tracheoesophageal fistula treated?

Treatment of TFE includes treating its underlying cause. This may include treating a cancer or removing an NG tube for a while. You may also need one or more of the following:

  • Medicines: You may be given medicines to relieve your symptoms. Antibiotics may also be given to fight germs and prevent infections.

  • Respiratory support: You may need extra oxygen to help you breathe easier.

    • Oxygen: You may need extra oxygen if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils. Ask your caregiver before you take off the mask or oxygen tubing.

    • Endotracheal (ET) tube: An endotracheal tube may be put into your mouth or nose. It goes down into your windpipe to help keep your airway open and help you breathe. It may be hooked to a ventilator (breathing machine), and you may get extra oxygen through your ET tube. You will not be able to talk while the ET tube is in place.

  • Stent: A stent (tube) may be placed in your esophagus or trachea to keep them open.

  • Surgery: Surgery may be needed to close or remove the part with the fistula. The esophagus may be reconnected to make it a continuous tube that is separate from the trachea. In some cases, a piece of tissue from the large bowel is used to join the parts. Ask your caregiver for more information about these surgeries to treat your TEF.
With treatment, such as medicine and surgery, TEF may be cured and your quality of life may be improved.

Where can I get more information?

Having a tracheoesophageal fistula is a life-changing condition for you and your family. Accepting that you have TEF is hard. You and those close to you may feel angry, sad, or frightened. These feelings are normal. Talk to your caregivers, family, or friends about your feelings. Contact the following for more information about TEF:

  • American Lung Association
    1301 Pennsylvania Ave. NW
    Washington , DC 20004
    Phone: 1- 202 - 785-3355
    Phone: 1- 800 - 548-8252
    Web Address: www.lung.org
  • National Heart, Lung and Blood Institute
    Health Information Center
    P.O. Box 30105
    Bethesda , MD 20824-0105
    Phone: 1- 301 - 592-8573
    Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
  • National Digestive Diseases Information Clearinghouse (NDDIC)
    2 Information Way
    Bethesda , MD 20892-3570
    Phone: 1- 800 - 891-5389
    Web Address: www.digestive.niddk.nih.gov

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2014 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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