Normally, a person’s esophagus and trachea are separate. However, there is a condition that can cause the two to be connected, called a tracheoesophageal fistula.
This is one of the birth defects that can occur in a fetus that is developing in the womb. If not treated properly, the baby can experience various other conditions including pneumonia .
Definition of Tracheoesophageal Fistula

According to the Cleveland Clinic , tracheoesophageal fistula (TEF) is an abnormal connection between the esophagus and the trachea. This condition is often congenital, meaning it occurs while the fetus is developing in the womb.
However, it is possible that TEF can also be obtained in adulthood. The trigger is because someone has cancer , infection or trauma. Surgery is needed to close the fistula.
When a baby with a tracheoesophageal fistula swallows, fluid can pass through the abnormal connection between the esophagus and the trachea. If this happens, fluid can enter the baby’s lungs and can cause pneumonia and other problems.
Tracheoesophageal fistula usually occurs with a related condition called esophageal atresia (EA). With esophageal atresia, the esophagus does not form properly as the fetus develops before birth, resulting in two segments. One that connects to the throat, and another that connects to the stomach. However, these two segments do not connect to each other.
Symptoms of Tracheoesophageal Fistula
Symptoms of tracheoesophageal fistula in babies depend on whether or not they also have esophageal atresia. Babies who are born with TEF and not EA usually do not show symptoms at birth. However, over time, babies may show the following signs:
- Frequent lung infections
- Coughing while breastfeeding
Babies who have esophageal atresia with tracheoesophageal fistula usually show symptoms shortly after birth. The most common symptoms include:
- Cough
- Choking while trying to swallow
- Respiratory problems
- Foamy, white bubbles appear in the mouth
- Vomit
- Blue skin color (cyanosis), especially when the baby is breastfeeding
- Difficulty breathing
- The stomach is very round and full
Symptoms of tracheoesophageal fistula or esophageal atresia may resemble other conditions or medical problems. It is important to talk to your child’s medical team to receive a proper diagnosis.
Causes of Tracheoesophageal Fistula
As the fetus grows and develops in the mother’s womb, the trachea and esophagus begin to develop as a single tube. At about four to eight weeks after conception, a wall forms between the fetus’s esophagus and trachea to separate them into two distinct tubes.
The formation of this wall is the crucial point that causes this anomalous condition. If the wall is not formed perfectly, various abnormal conditions can occur in babies, including tracheoesophageal fistula.
Even if primary TEF has been repaired, there is a chance that TEF will come back (recurrent TEF). Sometimes the cause is an infection during surgery that damages the trachea.
After repeated TEFs, increased esophageal pressure forces fluid into the airway during swallowing. Eventually the TEF becomes coated with mucosa and becomes permanent.
Incident Number
This incident can be considered quite rare. Quoted from Children’s National , tracheoesophageal fistula is a birth defect, which occurs in 1 in 5,000 births. This occurs when the fetus is formed in the mother’s womb.
Risk Factors

Neither TEF nor EA are believed to be hereditary. However, there are several factors that increase a baby’s risk of developing it. Usually when they also have 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 kidneys, absent kidneys, or hypospadias )
- Muscle or bone problems
- VACTERL syndrome (involving Vertebral, Anal, Cardiac, TE, Renal, and Limb abnormalities)
About half of all babies with tracheoesophageal fistula or esophageal atresia also have other birth defects.
Diagnosis
In rare cases, your healthcare provider may suspect a tracheoesophageal fistula before your baby is born. This is assessed by abnormalities seen during an ultrasound.
However, in most cases, your healthcare team will not suspect TEF until a few hours after your baby is born. For example, if your baby has excessive mucus, is having trouble breathing, or is unable to swallow. To confirm a diagnosis of TEF or EA, your healthcare provider will likely perform an X-ray of your baby’s chest and abdomen. Your healthcare provider may also recommend tests such as:
- Imaging studies, such as x-rays
- Endoscopy or bronchoscopy, which is a technique for viewing the inside of a child’s airways using a thin tube equipped with a small light and camera.
If your baby is diagnosed with TEF or EA, your doctor may order additional tests to identify or rule out other congenital conditions.
TEF may come and go, making it difficult to diagnose. The procedures listed above will usually be the first step in getting a correct diagnosis.
Related Article: Low amniotic fluid during pregnancy, be aware of babies experiencing birth defects
Handling and Care
When your baby has been diagnosed with tracheoesophageal fistula or esophageal atresia, your doctor may then recommend surgery to treat it. The type of surgery that will be performed depends on the following:
- Types of abnormalities
- Baby’s overall health and medical history
- The opinions of surgeons and other health care providers involved in the baby’s care
- Expectations regarding the actions taken
- Parents’ opinions and preferences
When TEF is repaired, the connection between the esophagus and trachea is surgically closed. Repair of esophageal atresia depends on how close the two parts of the esophagus are to each other.
Sometimes esophageal atresia requires more than one surgery. Your baby’s surgeon will then decide when is the best time to perform surgery based on your baby’s condition and the type of problem.
Repair of esophageal atresia or tracheoesophageal fistula with esophageal atresia can be performed with an open surgical approach (thoracotomy) or an invasive approach. Depending on the length of the gap between the upper and lower parts of the severed esophagus and the experience of the pediatric surgeon, the esophagus can be reconnected using a minimally invasive approach in most cases.
In some cases, one procedure is not enough. Several procedures are needed to connect the upper and lower esophageal segments.
Possible Complications

After your child has undergone surgery, parents may worry about complications as they grow up. In fact, some children born with esophageal atresia have the potential to experience long-term problems.
Difficulty Swallowing
Some children will have difficulty swallowing food and liquids. This may be due to problems with the normal movement of food and liquids down the esophagus (peristalsis).
Pneumonia
If your baby’s tracheoesophageal fistula is not treated immediately, complications can occur in his body. When he swallows, fluid can pass through the abnormal connection between the esophagus and trachea. This can cause fluid to enter the baby’s lungs and cause pneumonia.
Post-Surgical Scarring
Scar tissue may develop in the esophagus after surgery. This can block the passage of food. Sometimes, the narrowed esophagus can be widened with a special procedure done while the child is under general anesthesia. In other cases, another surgery may be needed to open the esophagus so that food can pass properly into the stomach.
GERD
About half of children who have had esophageal atresia repaired will have problems with GERD , or gastrointestinal reflux disease. GERD causes acid to back up into the esophagus from the stomach.
When acid moves from the stomach into the esophagus, it causes a burning or painful feeling known as heartburn. GERD can usually be treated with medications or with a minimally invasive surgical antireflux procedure known as fundoplication.
Prevention
Tracheoesophageal fistula is a congenital condition that occurs since the fetus is developing in the womb. Therefore, there is no way to prevent this from happening.
Even in adults, TEF is caused by cancer and infection. So just like in babies, there is nothing you can do to reduce your or your baby’s risk of the condition.
Tracheoesophageal fistula can happen to anyone, especially newborns. Maintain a healthy lifestyle so that you and your little one are always healthy.
Hopefully this article is useful.
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Republished with permission from theAsianparent Indonesia