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Tracheoesophageal Fistula Explained: Types, Symptoms & Care
Written by Medicover Team and Medically Reviewed by Dr Seshadri LN , Pediatric Surgeon
A tracheoesophageal fistula (TEF) is an abnormal connection between the esophagus and the trachea. Normally, these two tubes are separate, one carries food to the stomach, and the other carries air to the lungs. When a fistula forms, food and liquids can accidentally enter the lungs, causing serious problems. TEF most often occurs in babies at birth, but can also develop later in life due to illness or injury. Surgery is the main treatment to fix this problem.
What is Tracheoesophageal Fistula?
Your esophagus is the tube that moves food and liquids from your mouth to your stomach. Your trachea, or windpipe, carries air to your lungs. In people with tracheoesophageal fistula, there is an abnormal opening between the two tubes. This opening lets food and drink go into the lungs, which can lead to coughing, choking, and lung infections.
TEF usually happens during fetal development, meaning babies are born with it. Sometimes, adults develop TEF after lung or esophageal cancer, infections, or injuries.
What Are the Different Types of Tracheoesophageal Fistula?
There are several types of tracheoesophageal fistula, classified based on the location and nature of the abnormal connection:
- Type A (Pure Esophageal Atresia): In this form, there is no actual fistula. Both ends of the esophagus end in blind pouches, meaning food cannot reach the stomach. This type accounts for approximately 8% of cases.
- Type B: A rare form in which the upper part of the esophagus connects to the trachea while the lower portion ends blindly. This form makes up about 2% of cases.
- Type C: The most common type of TEF, where the upper esophagus ends in a blind pouch and the lower esophagus connects to the trachea. This configuration is responsible for about 85% of all TEF cases.
- Type D: This is the rarest form, where both the upper and lower parts of the esophagus connect to the trachea. It accounts for less than 1% of TEF cases.
- Type E (H-type Fistula): In this variant, the esophagus is intact and connects normally to the stomach, but there is a fistula connecting the esophagus and trachea. It accounts for around 4% of cases and may be harder to detect initially.
What Are the Common Causes of Tracheoesophageal Fistula?
Congenital TEF happens because of a developmental problem early in pregnancy. Normally, the trachea and esophagus develop from a single tube that later divides. When this division is incomplete or incorrect, a fistula can form. The exact cause is often unknown but can be linked to genetic syndromes like VACTERL or chromosome abnormalities.
Acquired TEF is usually caused by:
- Advanced esophageal or lung cancer, which can erode the walls between the esophagus and trachea
- Chronic infections like tuberculosis or fungal infections
- Prolonged intubation or tracheostomy, especially in ICU patients
- Radiation therapy to the chest
- Surgical trauma or injury from medical procedures
- Ingestion of caustic substances (e.g., strong acids or alkalis)
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Get Second OpinionWhat are the Tracheoesophageal Fistula Symptoms and Warning Signs?
Symptoms can vary depending on whether the patient has only TEF or both TEF and esophageal atresia. In newborns, symptoms are typically apparent shortly after birth. Common signs include:
- Coughing and Choking: Infants may experience frequent coughing and choking, especially during feeding.
- Difficulty Breathing: Respiratory distress and difficulty breathing are common due to the abnormal connection between the trachea and esophagus.
- Excessive Drooling: The inability to swallow saliva effectively can lead to excessive drooling.
- Cyanosis: A bluish discoloration of the skin, indicating inadequate oxygenation.
- Recurrent Pneumonia: Aspiration of food and fluids into the lungs can lead to recurrent respiratory infections and pneumonia.
In babies with both TEF and esophageal atresia, these symptoms are more immediate and severe. Babies with H-type fistulas may seem fine at birth but develop symptoms like recurring lung infections or feeding problems over time.
In adults with acquired TEF, symptoms often include:
- Persistent coughing or choking when eating or drinking
- Recurring episodes of pneumonia
- Difficulty swallowing (dysphagia)
- Unexplained weight loss
- Voice changes or hoarseness
How is Tracheoesophageal Fistula Diagnosed?
In some cases, TEF may be suspected prenatally if an ultrasound shows signs such as excess amniotic fluid (polyhydramnios) or absence of a stomach bubble. However, most diagnoses occur shortly after birth when symptoms become evident.
Diagnostic steps may include:
- Attempting to pass a feeding tube into the stomach. If unsuccessful, it may indicate esophageal atresia.
- X-rays of the chest and abdomen with a feeding tube in place to visualize the esophageal structure
- Contrast esophagogram to show abnormal connections
- Endoscopy or bronchoscopy to directly visualize the fistula
- CT or MRI scans in complex or adult cases for detailed imaging
What are the Tracheoesophageal Fistula Treatment Options?
Surgery is the main treatment for TEF. The aim is to close the abnormal connection and, if esophageal atresia is present, reconnect the esophagus to the stomach.
Surgical methods vary depending on the patient and TEF type. Surgeons may use traditional open surgery or minimally invasive techniques where possible.
In newborns, surgery usually happens within the first few days of life. Adults with acquired TEF often require surgery and treatment of the underlying cause, such as cancer.
After surgery, careful care includes:
Nursing Care Plan for Tracheoesophageal Fistula
Effective nursing care is vital in managing patients with tracheoesophageal fistula. A complete nursing care plan should address the following aspects:
Respiratory Management
- Airway Patency: Ensuring a clear and patent airway through suctioning and appropriate positioning.
- Oxygen Therapy: Administering supplemental oxygen to maintain adequate oxygenation.
- Monitoring: Continuous monitoring of respiratory status, including oxygen saturation and respiratory rate.
Nutritional Support
- Feeding Techniques: Implementing specialized feeding techniques, such as using a feeding tube or positioning the infant upright during feeding.
- Nutritional Assessment: Regular assessment of nutritional status and growth parameters.
- Parenteral Nutrition: Providing parenteral nutrition if oral feeding is not feasible.
Infection Prevention
- Hand Hygiene: Strict adherence to hand hygiene protocols to prevent infections.
- Antibiotic Therapy: Administering prophylactic antibiotics as prescribed.
- Monitoring for Infections: Vigilant monitoring for signs of infection, such as fever and increased white blood cell count.
The treatment of tracheoesophageal fistula typically involves a combination of surgical intervention and supportive care.
When to See a Doctor?
Tracheoesophageal fistula (TEF) is a condition where there is an abnormal connection between the windpipe (trachea) and the food pipe (esophagus). This can cause serious problems with feeding and breathing, especially in newborns and infants.
See a doctor immediately if you notice in a baby or child:
- Coughing, choking, or turning blue while feeding
- Trouble swallowing or feeding
- Frequent drooling or vomiting
- Breathing difficulties or noisy breathing
- Repeated lung infections or pneumonia
Early diagnosis is crucial because TEF needs prompt treatment to prevent serious lung and nutritional problems.
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Who Does Tracheoesophageal Fistula Affect?
TEF is mostly a condition found in newborns, with most cases diagnosed soon after birth. It affects babies because their esophagus and trachea did not separate properly before they were born.
Adults rarely develop TEF, but when they do, it is usually due to cancer, infections like tuberculosis, or damage from medical procedures.
Complications of Tracheoesophageal Fistula
Despite advances in surgical techniques and postoperative care, tracheoesophageal fistula can be associated with several complications:
- Anastomotic Leak: Leakage at the surgery site, which can cause infection and slow healing
- Stricture Formation: Narrowing of the esophagus at the repair site, causing swallowing problems
- Recurrent Fistula: The fistula reopening or forming again
- Gastroesophageal Reflux: Increased acid reflux, leading to esophagitis and lung problems
- Respiratory Issues: Ongoing pneumonia and chronic lung disease from food or fluids entering the lungs
Prevention of Tracheoesophageal Fistula
There's no known way to prevent congenital TEF since it happens during early pregnancy. Families with a history of birth defects may benefit from genetic counseling.
To prevent acquired TEF, efforts focus on:
- Proper treatment of cancers affecting the esophagus or lungs
- Avoiding long-term use of breathing tubes when possible
- Quickly treating chronic infections
- Preventing swallowing of harmful substances
- Careful surgery to avoid injury
Our Experience Treating Tracheoesophageal Fistula
At Medicover, we provide compassionate care for patients with TEF, from newborns to adults. Our team includes surgeons, lung specialists, and nutrition experts who work together to help patients heal.
We offer:
- Advanced diagnosis tools like imaging and endoscopy are used to find the problem clearly
- Surgery using the latest minimally invasive techniques
- Care to help with feeding and breathing before and after surgery
- Follow-up support to ensure healthy growth and lung health
At Medicover, we're committed to giving gentle, expert care to help children and adults with TEF live healthier, fuller lives.
