Tracheoesophageal fistula (TEF) is a disorder of the esophagus characterized by formation of connection between esophagus (pipe connecting the throat and stomach) and the trachea (pipe connecting the throat to lungs and wind pipe). This usually develops in infants with esophageal atresia characterized by inappropriately formed esophagus. About 17-70% of the children with developmental anomalies such as down’s syndrome, cardiovascular defects, gastrointestinal anomalies, genitourinary anomalies and musculoskeletal anomalies are at risk of developing TEF. Presence of chromosomal abnormalities also increases the risk for TEF.
Types of tracheoesophageal fistula
Basing on when the fistula develops TEF can be classified in to two types.
- Congenial tracheoesophageal fistula: These develop by birth due to abnormalities in the development of fetus. In majority of the cases, these are diagnosed immediately after birth or within first year after birth. Rarely, they are expressed in adulthood.
- Acquired tracheoesophageal fistula: These develop after birth secondary to an infection, malignant diseases, trauma or ruptured diverticula. Very rarely TEF develops due to use of mechanical ventilation using an endotracheal tube or tracheostomy tube for a very long time.
Basing on the site where the connection develops between trachea and esophagus there are three types of TEF.
- Type II: Upper part of esophagus gets connected to the trachea.
- Type III or H-type: Esophagus and trachea will be separate but a connection forms in between them. This type of abnormality is very difficult to diagnose as the baby will be able to carry out breathing and eating normally.
How tracheoesophageal fistula develops?
During the development of fetus in the womb of the mother, esophagus and trachea form as single tube initially. Later, a wall forms between esophagus and trachea separating them in to two different tubes. Wall formation occurs in the first trimester of pregnancy. If the wall formation does not happen properly then tracheoesophageal fistula develops. About 1 in 4000 babies born alive suffers with this problem.
Symptoms of tracheoesophageal fistula
- Distended abdomen due to passage of air in to the stomach.
- Pneumonia and other respiratory tract infections due to passage of fluids ingested by the baby in to the lungs through the connection between esophagus and trachea.
- Increased salivation that is generally observed as white bubbles.
- Bluish skin that can be observed clearly while feeding the baby.
- Difficulty in feeding due to choking.
- Frequent coughing while feeding.
- Difficulty to breath.
Diagnosis and Treatment of tracheoesophageal fistula
Physical examination and history of symptoms often reveals the presence of tracheoesophageal fistula. X-ray of the chest and abdomen and other imaging studies are conducted to confirm the presence of abnormality.
Treating tracheoesophageal fistula
Surgery is the only way to correct TEF. During surgery the connection is closed to leave esophagus and trachea as two separate tubes. TEF being a life threatening situation, surgery is done soon after birth by giving general anesthesia.
In the absence of congenital abnormalities there is 100% survival rate of infants with TEF after performing surgery to correct the faulty connection. However, the survival rate is very low in patients who have acquired TEF later in life.
Issues with surgery
As with any surgery, surgery to correct TEF is also associated with certain risk factors.
- Collapse of lungs.
- Leaking of food from the site of surgery.
- Fall in body temperature.
- Re-opening of the closed connection.
- Thinning of the repaired organs.
Post surgery care
- Antibiotics are given to prevent the risk of infections.
- Baby is kept on ventilator.
- Chest tube is placed in the chest wall to remove the fluids before they reach the lungs.
- Intravenous fluids are administered to provide required nutrition.
- Pain medications are given to reduce pain.