What are tracheoesophageal fistulas?
Tracheoesophageal fistulas are congenital or acquired passages between the esophagus and the trachea. Normally there are no connections between these two tubes. The word “tracheoesophageal” is made up of two terms: “trachea” is the windpipe and “esophagus” means food pipe. Both have different functions: Food enters the stomach through the esophagus, while vital air flows into the lungs via the trachea. In English, the disease is called “tracheoesophageal fistula” or simply TEF.
Tracheoesophageal fistulas can have a variety of causes. Sometimes they are congenital and babies are born with the “wrong” connection. However, some people acquire the “short circuit” between the trachea and oesophagus in the course of their lives. Fistulas can occur after medical procedures, for example, but also as a result of certain diseases, inflammations or injuries to the trachea and oesophagus.
The symptoms of tracheoesophageal fistulas are triggered by the aspiration of saliva or food. They can vary greatly in severity and range from coughing attacks to pneumonia. The treatment depends on the cause. Doctors can close tracheoesophageal fistulas by surgery or with the help of a stent (vascular support).
Tracheoesophageal fistulas – frequency and age
Tracheoesophageal fistulas are rare diseases. Their frequency cannot be quantified with certainty because they often occur in connection with various diseases or medical interventions. Therefore, no preferred age of onset can be determined.
Acquired fistulas are most commonly found in people suffering from esophageal cancer. The tumor penetrates through the wall of the esophagus and trachea and leads to the formation of a connection.
Congenital tracheoesophageal fistulas, on the other hand, are not that rare. Approximately 1 in 2,000 to 4,000 newborns is born with a fistula.
Tracheoesophageal fistulas: There are many causes
The causes of tracheoesophageal fistulas can be very different. A distinction is usually first made as to whether the fistulas are congenital or acquired in the course of life. The following reasons may be responsible for the unnatural passages between the trachea and esophagus:
- Congenital fistulas between the trachea and esophagus: The causes lie in an abnormal development in the womb. Babies are born with the malformation. In addition, affected babies often have another problem – esophageal atresia (there are different forms). The esophagus does not form properly. It then consists of two parts that are not connected to each other. As a result, ingested food and liquids such as milk do not pass through the esophagus into the stomach as they should. Other anomalies can also occur, such as Down’s syndrome (trisomy 21) or heart defects.
- Medical interventions: Tracheoesophageal fistulas can also rarely occur as a complication after esophageal or laryngeal surgery. “Iatrogenic” is the technical term for when the medical effect causes illness.
- Radiotherapy, which is often used for cancer such as esophageal or lung cancer, can also cause tracheoesophageal fistulas to form.
- Infections, for example tuberculosis (Mycobacterium tuberculosis) or severe inflammation that causes the tissue to shrink and die (necrosis).
- Cancer, for example cancer of the esophagus, lungs or lymph glands(lymphomas)
- Mechanical causes: Long-term ventilation, foreign bodies or injuries to the trachea and esophagus
Symptoms: Tracheoesophageal fistulas cause various symptoms
The symptoms of tracheoesophageal fistulas can vary greatly depending on the cause. When swallowing, liquid or food can pass through the fistula from the esophagus into the trachea – and thus also into the lungs. Various problems then develop there – often pneumonia.
- Congenital tracheoesophageal fistulas and esophageal atresia: Typical symptoms are white foam bubbles in front of the mouth, coughing or choking during feeding, vomiting, blue coloration of the skin (cyanosis) – especially during feeding – and shortness of breath; symptoms appear immediately after birth.
- Coughing attacks after eating – sometimes affected people cough up the food again.
- Fever and other symptoms of pneumonia, such as shallow, rapid breathing, chest pain and shortness of breath
- With long-term ventilation: The amount of lung secretions increases massively, solid or liquid substances penetrate the lungs and pneumonia can occur. The abdomen may also be bloated because air accumulates. In addition, contents from the gastrointestinal tract can enter the respiratory tract.
Tracheoesophageal fistulas: Diagnosis with us
The diagnosis of tracheoesophageal fistulas always begins (if possible) with a discussion of the patient’s medical history. We always ask the parents of affected children. The following questions, among others, are of interest to us:
- What symptoms do you have and how long have you had them?
- How pronounced are your complaints?
- Do the symptoms intensify in certain situations, for example after eating?
- Do you have any known illnesses, for example cancer or infections?
- Have you recently undergone a medical procedure, for example surgery on the esophagus or larynx?
- Have you received radiotherapy in the past?
Their answers provide us with initial clues as to the cause of the symptoms. This is usually followed by a physical examination, in which we palpate the neck and chest, for example. We then generally use imaging techniques to detect possible tracheoesophageal fistulas. The most important examination methods are
- Esophageal passage with contrast medium: You drink a contrast medium and the radiologist takes an X-ray. This allows the esophagus and the transition from the esophagus to the stomach to be visualized. We can detect possible fistulas or leaks after a surgical procedure. The examination method is also known as esophageal broth swallowing.
- Esophagoscopy and tracheobronchoscopy (endoscopy of the esophagus and lungs): Fistulas can be diagnosed very reliably by endoscopic examination of the esophagus and airways. We can also take tissue samples as part of these examinations. A pathologist then analyzes the cells under a microscope – benign cells can thus be distinguished from cancer cells. Any germs present can also be detected in the samples taken.
- Computed tomography (CT): This X-ray examination produces detailed slice images and shows the body in “slices”. Fistulas are less visible on CT, but the examination is helpful if a tumor is suspected. The CT images also show how far the tumor has spread and what stage it is at.
Tracheoesophageal fistulas: Prevention, early detection, prognosis
There are no special measures for the prevention and early detection of tracheoesophageal fistulas. As a general rule, always seek medical advice if you notice any unusual symptoms.
Sometimes infections with bacteria (e.g. tuberculosis) are the cause of tracheoesophageal fistulas. In general, you can prevent infections by ensuring adequate hygiene and thus reducing the risk of infection. For example, wash your hands regularly and thoroughly.
A strong immune system also protects against infectious diseases. You can make your defenses more effective if you:
- give up smoking
- drink alcohol only moderately
- Eat a healthy diet (lots of fruit and vegetables)
- provide plenty of exercise
- Reduce stress in everyday life and at work
These measures can also protect against cancer to a certain extent, for example against esophageal or lung cancer. Alcohol and smoking play a significant role in both types of cancer.
Course and prognosis of tracheoesophageal fistulas
The course and prognosis of tracheoesophageal fistulas cannot be generally predicted. Both always depend on the cause and the treatment. However, the quality of life of patients with fistulas between the trachea and oesophagus is often considerably restricted. Without adequate treatment, life expectancy also decreases.
For fistulas that are not related to cancer, treatment is often successful if we diagnose them early enough. In the case of fistulas caused by cancer, however, the survival time is often only a few weeks to months.
Babies with congenital tracheoesophageal fistulas have a very good prognosis after surgery.
Tracheoesophageal fistulas: Treatment depends on the cause
The treatment of tracheoesophageal fistulas always depends on the cause.