Tracheoesophageal Fistula: Type, Sign, Symptoms, Diagnosis, and Treatment Explained

Tracheoesophageal fistula (TEF) is a medical condition where an abnormal connection forms between the trachea and the esophagus. The trachea is the tube that carries air to the lungs, while the esophagus is the tube that carries food and liquids to the stomach. Normally, these two structures are separate, but in individuals with tracheoesophageal fistula, they become connected.^(1,2)

Tracheoesophageal fistula is mostly observed in infants, and is less common in adults, though it can happen in cases were individuals have cancer, injuries, or infections.

Tracheoesophageal fistula typically occurs during fetal development when the trachea and esophagus fail to separate properly. This results in the formation of a passageway or fistula between them. As a consequence, substances can pass between the trachea and the esophagus, leading to various complications and challenges.

The most common type of tracheoesophageal fistula is known as esophageal atresia with distal tracheoesophageal fistula. In this form, the upper part of the esophagus ends in a blind pouch, while the lower part connects to the trachea. This abnormal connection can cause problems with swallowing, breathing, and digestion.⁽³⁾

Tracheoesophageal fistula requires medical attention and is typically diagnosed shortly after birth. Treatment usually involves surgical intervention to repair the abnormal connection and restore the normal separation between the trachea and the esophagus. With early detection and appropriate treatment, many individuals with tracheoesophageal fistula can lead healthy lives. However, ongoing monitoring and follow-up care are often necessary to manage any potential long-term complications. It is estimated that most cases of Tracheoesophageal fistula in infants occur along with esophageal atresia (EA). Both EA and TEF occur only in around 1 out of every 4000 births in the United States.⁽⁴⁾

Tracheoesophageal fistulas are typically classified into five main types – type A, B, C, D, and E. Let us look at each of these five types.

• Type A – Esophageal Atresia with Distal TEF: This is the most common type of TEF. In this case, the upper part of the esophagus ends in a blind pouch and does not connect to the stomach, while the lower part of the esophagus is connected to the trachea. This results in the inability to pass food from the mouth to the stomach and causes the swallowed substances to enter the lungs.⁽⁵⁾
• Type B – Isolated TEF: In isolated Tracheoesophageal fistula, there is an abnormal connection between the trachea and the esophagus without the presence of esophageal atresia.
• Type C – Esophageal Atresia without Fistula: This type involves a complete separation of the upper and lower segments of the esophagus, with no connection to the trachea. The upper part of the esophagus forms a blind pouch, while the lower part terminates in the stomach. In this case, there is no fistula, but the esophagus is not continuous, leading to difficulties in feeding.⁽⁶⁾
• Type D – H-Type Fistula: H-Type fistula is characterized by a small connection or fistula between the trachea and the esophagus, usually lower down in the esophagus. It is called “H-Type” due to the shape of the connection.⁽⁷⁾
• Type E – Recurrent Fistula: Type E TEF refers to the recurrence of a fistula after surgical repair. It can occur due to complications or failure of the initial repair. Recurrent fistulas require further surgical intervention to correct the abnormal connection.⁽⁸⁾

Each type of tracheoesophageal fistula presents its own set of challenges and requires appropriate medical intervention, typically through surgical repair, to restore the normal separation between the trachea and the esophagus and ensure proper functioning of both the respiratory and digestive systems.

Tracheoesophageal fistula can present with a variety of signs and symptoms, which can vary depending on the specific type of Tracheoesophageal fistula. Here are some common signs and symptoms associated with TEF:

• Excessive Salivation or Drooling: Infants with Tracheoesophageal fistula may exhibit excessive drooling or salivation due to the abnormal connection between the esophagus and the trachea. This occurs because saliva can pass through the fistula into the trachea instead of flowing into the stomach.
• Choking or Cyanosis during Feeding: TEF can cause feeding difficulties and episodes of choking or cyanosis (bluish discoloration of the skin) during feeding. The abnormal connection allows food or liquid to enter the respiratory system, leading to respiratory distress.
• Coughing, Choking, or Gagging with Respiratory Symptoms: Tracheoesophageal fistula can result in recurrent episodes of coughing, choking, or gagging, particularly when the infant attempts to swallow. This occurs due to the entry of saliva, food, or liquid into the trachea, triggering a reflexive response to clear the airway.
• Abdominal Distension or Vomiting: Infants with TEF may experience abdominal distension (swelling) due to air accumulating in the stomach. The presence of the fistula can prevent the normal passage of air from the stomach into the intestines, leading to gastric distension and subsequent vomiting.⁽⁹⁾
• Respiratory Distress: TEF can cause respiratory distress in infants, characterized by rapid or labored breathing, wheezing, and cyanosis. The abnormal passage between the trachea and the esophagus can allow fluid or food particles to enter the lungs, leading to respiratory complications.

It is important to note that the severity and combination of symptoms can vary, depending on the specific type of Tracheoesophageal fistula and any associated complications.

Diagnosis and treatment of tracheoesophageal fistula involve several steps to properly evaluate and manage the condition. Here is an overview of the diagnostic process and treatment options for Tracheoesophageal fistula:

• Physical Examination: The healthcare provider will perform a thorough physical examination of the infant, looking for signs and symptoms of TEF, such as excessive drooling, respiratory distress, and abdominal distension.
• Imaging Studies: Imaging techniques like X-rays, contrast studies (such as an upper gastrointestinal series or contrast esophagography), or more advanced imaging modalities like fluoroscopy or computed tomography (CT) scan may be used to visualize the abnormal connection between the trachea and the esophagus.
• Esophagoscopy or Bronchoscopy: A flexible tube with a camera (endoscope) may be inserted into the esophagus or trachea to directly visualize the presence and location of the fistula.

• Surgery: Surgical intervention is the primary treatment for Tracheoesophageal fistula. The goal of surgery is to repair the abnormal connection between the trachea and the esophagus and restore the normal separation. The specific surgical technique used depends on the type and location of the fistula.⁽¹⁰⁾
• Preoperative Stabilization: In some cases, especially when the infant is born prematurely or has other medical complications, stabilization and supportive care may be provided before the surgical repair. This includes ensuring proper nutrition, respiratory support, and addressing any associated conditions.
• Postoperative Care: Following surgery, infants will require postoperative care, including monitoring for complications, wound healing, and adequate nutrition.
• Long-term Follow-up: Infants who undergo surgery for TEF may require long-term follow-up to monitor their growth, development, and any potential complications that may arise.

Tracheoesophageal fistula (TEF) is a complex condition characterized by an abnormal connection between the trachea and the esophagus. Prompt diagnosis and appropriate treatment are crucial for infants with TEF to ensure their well-being and long-term outcomes. Long-term follow-up is necessary to assess the infant’s growth, development, and potential complications that may arise.

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