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Tracheoesophageal Fistula

  • Author: Sat Sharma, MD, FRCPC; Chief Editor: Julian Katz, MD  more...
 
Updated: Jan 03, 2016
 

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 Percent 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

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.

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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.

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Epidemiology

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.[1] Incidence of malignant TEFs was reported at 4.5% for primary malignant esophageal tumors, and 0.3% for primary malignant lung tumors.[2] Other investigators have reported the incidence of TEFs secondary to esophageal carcinoma to be 4.3-8.1%.

Mortality/Morbidity

See the list below:

  • 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

See the list below:

  • 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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Contributor Information and Disclosures
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

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, World Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Donald Duerksen, MD Assistant Professor, Department of Medicine, Section of Gastroenterology, University of Manitoba, Canada

Donald Duerksen, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, American Society for Parenteral and Enteral Nutrition

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Noel Williams, MD, FRCPC FACP, MACG, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Additional Contributors

Marco G Patti, MD Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine

Marco G Patti, MD is a member of the following medical societies: American Association for the Advancement of Science, American Surgical Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, Association for Academic Surgery, Pan-Pacific Surgical Association, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Southwestern Surgical Congress, Western Surgical Association

Disclosure: Nothing to disclose.

References
  1. Harley HR. Ulcerative tracheo-oesophageal fistula during treatment by tracheostomy and intermittent positive pressure ventilation. Thorax. 1972 May. 27(3):338-52. [Medline].

  2. Burt M, Diehl W, Martini N et al. Malignant esophagorespiratory fistula: management options and survival. Ann Thorac Surg. 1991 Dec. 52(6):1222-8; discussion 1228-9. [Medline].

  3. Yau WP, Mitchell AA, Lin KJ, Werler MM, Hernández-Díaz S. Use of decongestants during pregnancy and the risk of birth defects. Am J Epidemiol. 2013 Jul 15. 178(2):198-208. [Medline]. [Full Text].

  4. Spigel DR, Hainsworth JD, Yardley DA, et al. Tracheoesophageal fistula formation in patients with lung cancer treated with chemoradiation and bevacizumab. J Clin Oncol. 2010 Jan 1. 28(1):43-8. [Medline].

  5. Zhu H, Shen C, Xiao X, Dong K, Zheng S. Reoperation for anastomotic complications of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 2015 Dec. 50 (12):2012-5. [Medline].

  6. Smith N. Oesophageal atresia and tracheo-oesophageal fistula. Early Hum Dev. 2014 Dec. 90 (12):947-50. [Medline].

  7. Wang B, Tashiro J, Allan BJ, et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res. 2014 Aug. 190 (2):604-12. [Medline].

  8. Zani A, Wolinska J, Cobellis G, Chiu PP, Pierro A. Outcome of esophageal atresia/tracheoesophageal fistula in extremely low birth weight neonates (<1000 grams). Pediatr Surg Int. 2015 Oct 30. [Medline].

  9. Yalcin S, Demir N, Serel S, Soyer T, Tanyel FC. The evaluation of deglutition with videofluoroscopy after repair of esophageal atresia and/or tracheoesophageal fistula. J Pediatr Surg. 2015 Nov. 50 (11):1823-7. [Medline].

  10. Woo S, Lau S, Yoo E, Shaul D, Sydorak R. Thoracoscopic versus open repair of tracheoesophageal fistulas and rates of vocal cord paresis. J Pediatr Surg. 2015 Dec. 50 (12):2016-8. [Medline].

  11. Holder TM, Ashcraft KW, Sharp RJ, Amoury RA. Care of infants with esophageal atresia, tracheoesophageal fistula, and associated anomalies. J Thorac Cardiovasc Surg. 1987 Dec. 94(6):828-35. [Medline].

  12. Ghali S, Chang EI, Rice DC, Walsh GL, Yu P. Microsurgical reconstruction of combined tracheal and total esophageal defects. J Thorac Cardiovasc Surg. 2015 Nov. 150 (5):1261-6. [Medline].

 
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Tracheoesophageal fistula. During development of respiratory and digestive systems, a single primitive tube develops lung bud and tracheoesophageal septum forms by 4-6 weeks of gestational age. The septum separates the foregut and tracheobronchial tree by 6 weeks of gestational age.
Tracheoesophageal fistula. The cuff of endobronchial causes circumferential ischemia and injury to the trachea; the erosion leads to formation of tracheoesophageal fistula.
Tracheoesophageal fistula. H-type of tracheoesophageal fistula.
Tracheoesophageal fistula. Esophageal atresia with distal tracheoesophageal fistula.
Tracheoesophageal fistula. Isolated esophageal atresia without tracheoesophageal fistula.
Table. Classification of Congenital Tracheoesophageal Fistulas and Esophageal Atresia
Anatomic Characteristics Percent 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
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