eMedicine Specialties > Gastroenterology > Esophagus

Tracheoesophageal Fistula

Author: Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital
Coauthor(s): Donald Duerksen, MD, Assistant Professor, Department of Medicine, Section of Gastroenterology, University of Manitoba, Canada
Contributor Information and Disclosures

Updated: Jun 16, 2006

Introduction

Background

A tracheoesophageal fistula (TEF) is a congenital or acquired communication between the trachea and esophagus. TEFs often lead to severe and fatal pulmonary complications.

The Table below describes the 5 main categories of congenital TEFs.Classification of Congenital Tracheoesophageal Fistulas and Esophageal Atresia

Open table in new window

Most patients with TEFs are diagnosed immediately following birth or during infancy. TEFs are often associated with life-threatening complications, so they are usually diagnosed in the neonatal period. In rare cases, patients with a congenital TEF may present in adulthood.

Acquired TEFs occur secondary to malignant disease, infection, ruptured diverticula, and trauma. Postintubation TEFs uncommonly occur following prolonged mechanical ventilation with an endotracheal or tracheostomy tube.

Historical perspectives

The credit for the very first description of TEFs goes to Thomas Gibson, who, in 1697, reported a case of an infant with esophageal atresia and a TEF. In 1839, Thomas Hill recounted the symptoms of another infant with a TEF and an associated imperforate anus. In 1888, Charles Steels, a London surgeon, became the first surgeon to operate on esophageal atresia. In the 19th century, innovative work by many surgeons ultimately led to Cameron Haight's successful primary repair in 1941. Pioneering of surgical techniques in the last several decades has produced survival rates of almost 100% for infants with this once hopeless congenital anomaly.

Pathophysiology

Approximately 17-70% of children with TEFs have associated developmental anomalies. These anomalies include Down syndrome, duodenal atresia, and cardiovascular defects. The following congenital anomalies have been reported with variable frequency:

• Cardiac anomalies include ventricular septal defect, patent ductus arteriosus, tetralogy of Fallot, atrial septal defect, and right-sided aortic arch.
• Genitourinary anomalies include renal agenesis or dysphagia, horseshoe kidney, polycystic kidney, ureteral and urethral malformations, and hypospadias.
• Gastrointestinal anomalies include imperforate anus, duodenal atresia, malrotation, intestinal malformation, Meckel diverticulum, and annular pancreas.
• Musculoskeletal anomalies include hemivertebrae, radial dysphagia or amelia, polydactyly, syndactyly, rib malformation, scoliosis, and lower limb defect.

Embryology

Knowledge of embryology is essential to understand the pathogenesis of congenital TEFs.

The esophagus and trachea both develop from the primitive foregut. In a 4- to 6-week-old embryo, the caudal part of the foregut forms a ventral diverticulum that evolves into the trachea. The longitudinal tracheoesophageal fold fuses to form a septum that divides the foregut into a ventral laryngotracheal tube and a dorsal esophagus. The posterior deviation of the tracheoesophageal septum causes incomplete separation of the esophagus from the laryngotracheal tube and results in a TEF.

Incomplete formation of the esophagus is known as esophageal atresia, which may be associated with TEFs. Many anatomic variations of esophageal atresia with or without a TEF may occur. The most common anomaly consists of a blind esophageal pouch and a distal TEF. Pure esophageal atresia without a TEF is the second most common form. The third most common anomaly is the H-type fistula, which consists of a TEF without esophageal atresia.

Acquired nonmalignant TEFs

Traumatic TEFs occur secondary to either blunt trauma or open avulsion injury to the neck and thorax. In blunt traumatic injuries, the TEF is intrathoracic and is usually located at the carina level. The TEF appears several days later as a result of tracheal wall necrosis. TEFs caused by endotracheal tube intubation depend on several factors, including prolonged intubation, an irritating or abrasive tube, and pressure exerted by the cuff. Pressures exceeding 30 mm Hg can significantly reduce mucosal capillary circulation and result in tracheal necrosis. Cuff pressure is particularly risky when exerted posteriorly against a rigid nasogastric tube in the esophagus. Poor nutrition, infection, and steroid use cause tissue alteration, which predisposes patients to development of TEFs.

TEFs occur uncommonly at the time of tracheostomy or secondary to improper positioning of the tracheal tube because of improper tracheal incision. The malpositioned tracheostomy tube exerts posterior pressure against the esophagus, resulting in tissue damage and a TEF.

Acquired malignant TEFs

This devastating complication results in contamination of the respiratory tract, leading to pulmonary infections and death from sepsis within a few weeks of development. Although the most common tumor site is the esophagus, tumors at other sites, including the lungs, trachea, and metastatic lymph nodes in the larynx, may also result in TEFs. The anatomic site of a TEF is the trachea in more than 50% of cases; approximately 40% occur in the left and right mainstem bronchi, and a smaller number (6%) occur in lung parenchyma. Despite aggressive management, the prognosis is generally poor in these patients.

Frequency

United States

TEFs are a common congenital anomaly with an incidence of 1 case in 2000-4000 live births. Acquired TEFs are quite rare, and incidence rates have not been well documented.

Acquired nonmalignant TEFs occur in approximately 0.5% of patients undergoing tracheostomy (Harley, 1972). Incidence of malignant TEFs was reported at 4.5% for primary malignant esophageal tumors, and 0.3% for primary malignant lung tumors (Burt, 1991). Other investigators have reported the incidence of TEFs secondary to esophageal carcinoma to be 4.3-8.1%.

Mortality/Morbidity

• Surgical and perioperative management of congenital TEFs have improved significantly. Survival rates of 100% can be achieved in infants who do not have severe associated congenital anomalies.
• Patients may develop morbidities following TEF repair, including tracheomalacia, esophageal dysmotility, gastroesophageal reflux, and dysphagia. Additionally, patients may develop pulmonary problems from recurrent aspiration.
• Patients with acquired TEFs have high mortality and morbidity rates because of critical illnesses and comorbidities.

Race

No racial predilection is apparent.

Age

• Congenital TEFs are primarily observed in neonates and during the first year of life.
• Adults rarely present with congenital TEFs that were undiagnosed during their early years of life.
• Acquired TEFs may occur in individuals of any age, and elderly individuals are at increased risk if they become ventilator dependent because of respiratory failure.

Clinical

History

• Esophageal atresia in the fetus should be considered as a cause of maternal polyhydramnios. Absence of stomach gas on prenatal ultrasound is another indication of esophageal atresia.
• Neonates with esophageal atresia usually develop copious, fine white frothy bubbles of mucus in the mouth and nose. Secretions recur despite suctioning.
• Infants may develop rattling respiration and episodes of coughing and choking in association with cyanosis.
• Symptoms worsen during feeding in the presence of a TEF.
• The symptoms induced by malignant TEFs are cough, aspiration, and fever. The average duration of symptoms from onset to diagnosis is approximately 12 days.

Physical

• Perform a careful physical examination to document/exclude other associated developmental anomalies.
• In the presence of a TEF, abdominal distention may occur secondary to collection of air in the stomach.

Causes

• Although no definite cause exists for congenital TEFs, an association with trisomies 18, 21, and 13 has been reported.
• Causes of acquired TEFs include iatrogenic injury, blunt chest or neck trauma, prolonged mechanical ventilation via endotracheal or tracheostomy tube, and excessive tube cuff pressure in patients ventilated for lung disease.

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