Tracheoesophageal Fistula

Updated: Jul 10, 2017
  • Author: Sat Sharma, MD, FRCPC; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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Overview

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.

See the images below.

Tracheoesophageal fistula. H-type of tracheoesopha Tracheoesophageal fistula. H-type of tracheoesophageal fistula.
Tracheoesophageal fistula. Esophageal atresia with Tracheoesophageal fistula. Esophageal atresia with distal tracheoesophageal fistula.

The table below describes the 5 main categories of congenital TEFs.

Table. Classification of Congenital Tracheoesophageal Fistulas and Esophageal Atresia (Open Table in a new window)

Anatomic Characteristics Percentage



of Cases



Esophageal atresia with distal TEF 87
Isolated esophageal atresia without TEF* 8
Isolated TEF 4
Esophageal atresia with proximal TEF 1
Esophageal atresia with proximal and distal TEF 1
*Classified as TEF although there is no fistula.

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 (especially tuberculosis), 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.

For patient education resources, see Bronchoscopy.

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Pathophysiology

Approximately 17-70% of children with tracheoesophageal fistulas (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.

In areas and populations where tuberculosis (TB) is still common, involvement of the esophagus and mediastinal lymph nodes may result in an acquired tubercular TEF. Diagnosis is established at endoscopy and biopsy; treatment is with antitubercular therapy—rarely, surgical repair may be required. [1]

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.

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Etiology

Although no definite cause exists for congenital tracheoesophageal fistulas (TEFs), an association with trisomies 18, 21, and 13 has been reported. In addition, the use of decongestants that contain imidazoline derivatives by women during the first trimester of pregnancy has been linked to an increased risk for congenital TEFs. [2]

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. There has even been a case report of an impacted denture causing TEF. [3]

Spigel et al investigated the development of TEFs in patients with small-cell and non-small-cell lung cancer. They reported their findings from 2 small, independent phase II clinical trials in which patients were administered bevacizumab combined with chemotherapy and radiation. [4] Both trials were closed early for safety reasons. However, the data suggested an association between the use of bevacizumab and chemoradiotherapy and a relatively high incidence of TEFs in the settings of small-cell and non-small-cell lung cancer. [4]

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Epidemiology

United States data

Tracheoesophageal fistulas (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. [5] The incidence of malignant TEFs was reported at 4.5% for primary malignant esophageal tumors, and 0.3% for primary malignant lung tumors. [6] Other investigators have reported the incidence of TEFs secondary to esophageal carcinoma to be 4.3-8.1%.

Race- and age-related demographics

No racial predilection is apparent.

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.

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Prognosis

The survival rate in healthy infants who undergo surgical repair for a congenital tracheoesophageal fistula (TEF) may be 100%. In groups of infants who have comorbidities or who are not fit enough for early repair, the survival rate is 80-95%. In a series of 118 patients, overall survival was more than 90%. [7]

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.

In infants with proximal esophageal atresia and distal TEF, morbidity after primary repair appears to remain high. In a retrospective study of data from 292 infants who underwent primary repair, there was an overall 6% mortality, mainly related to the presence of congenital heart disease, and a 62% postoperative complication rate. [8] Moreover, the use of empiric postoperative antibiotics for longer than 24 hours showed no difference in rates of infection, shock, or death relative to their use for a shorter period, and empiric acid suppression did not necessarily prevent complications.

Surgical outcomes of primary repair in extremely and very low-birth-weight infants with type C esophageal atresia and distal TEF (most common type; upper esophageal segment ends in a blind pouch while the lower esophageal segment attaches to the trachea via a TEF) appear to be similar to those of neonates weighing 1500 g or more. [9] Staged repair may be restricted to unstable neonates.

Overall survival in patients with H-type TEF (see the image below) without esophageal atresia (ie, congenital isolated TEF) appears to be good, with a multicenter retrospective reporting a 97% overall survival and a 16% in-hospital complication rate (excluded vocal cord issues; the main complication was postoperative leak [8%]) in 102 patients. [10] However, there was a high postrepair rate of recurrent laryngeal nerve injury (22%), which the investigators indicated probably not only warrants early vocal cord evaluation in any infants with postoperative respiratory difficulty but also routine evaluation of postrepair vocal cord function in this type of TEF. [10]

Tracheoesophageal fistula. H-type of tracheoesopha Tracheoesophageal fistula. H-type of tracheoesophageal fistula.

Patients may develop morbidities following TEF repair, including tracheomalacia, esophageal dysmotility, gastroesophageal reflux, and dysphagia. Use of a transanastomotic tube has been associated with higher rates of stricture, and interposition of prosthetic material has associated with higher leak rates. [8]  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.

Complications

Congenital and acquired TEFs are associated with multiple complications, including recurrent pneumonia, acute lung injury, acute respiratory distress syndrome, lung abscess, poor nutrition, bronchiectasis from recurrent aspiration, respiratory failure, and death.

In patients with esophageal atresia and a TEF, abnormal esophageal motility is always present because of abnormal development and innervation of esophagus. Long-term follow-up studies have reported complications of esophagitis, Barrett esophagus, and hiatal hernia.

The major postoperative complications are tracheal stenosis and recurrent fistula. Tracheal stenosis occurs in patients who have extensive injury to the posterior tracheal wall. Surgical repair of tracheal stenosis may be performed at a later date. Recurrent fistulas develop in patients who require continued postoperative intubation. This generally occurs from breakdown of the repair, and the risk of infection spreading into the soft tissue planes, neck, and mediastinum is high. Recurrent TEF in adults may also be a late complication of childhood surgical repair. [11]

Gastroesophageal reflux disease may later occur in half of patients who had repair for esophageal atresia and TEFs during the neonatal period. Treatment for reflux is antisecretory therapy. Rare complications of reflux are Barrett esophagus and esophageal carcinoma.

In children operated for esophageal atresia (EA) and/or TEFs, follow-up deglutitive and respiratory symptoms may occur and should be evaluated with videofluoroscopy. [12]

Vocal cord paresis/paralysis may occur more often in patients treated for esophageal atresia (EA) with and without fistula with thoracoscopic repair compared with open repair. [13] This may be due to thoracoscopic dissection of the esophagus high into the thoracic inlet. [13]

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