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Tracheoesophageal Fistula Treatment & Management

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

Medical Care

Surgical repair is required following confirmation of a diagnosis of TEF.

  • In healthy infants without pulmonary complications, primary repair is performed within the first few days of life. Repair is delayed in patients with low birth weight, pneumonia, or other major anomalies. Initially, treat patients conservatively with parenteral nutrition, gastrostomy, and upper pouch suction until they are considered to be low risk.
  • Preoperatively, a cuffed endotracheal tube is placed distal to the fistula site in order to prevent reflux of gastric contents into the lungs. The ongoing mechanical ventilation following tracheal reconstruction is associated with recurrence of TEFs or restenosis. A conservative approach is therefore used until the patient is weaned from the mechanical ventilator. A tracheostomy tube is placed distally to the TEF if possible. The head of the bed is elevated, and oral secretions are frequently suctioned. A gastrostomy tube is placed to minimize gastroesophageal reflux, and a jejunostomy feeding tube is placed for nutritional purposes. If soilage of the respiratory tract continues, esophageal diversion procedures may be required.
  • Since acquired TEFs do not close spontaneously, surgical repair is planned if the patient is stable enough. Critically ill patients are managed conservatively until stable enough for a major surgical procedure.
  • Treatment of malignant TEFs must be individualized, and the treatment should be instituted promptly. The therapy is generally palliative. Palliation consists of relief of obstruction and diversion of contamination from the respiratory tract. The procedures offered include esophageal exclusion or bypass, resection, direct closure, or endoprosthesis. Supportive therapy is recommended for patients who present late in the course of the fistula and already have pulmonary sepsis. The supportive measures include nasogastric drainage, tracheostomy, gastrostomy, and intravenous hydration and antibiotics.
  • Burt reported that bypass therapy and radiation therapy were the only treatments that significantly prolonged survival compared to supportive care. With radiation therapy, TEFs initially heal but usually recur, leading to respiratory tract contamination. Esophageal bypass with gastric, colonic, or jejunal interposition would have significantly improved survival rates but has a high risk of immediate mortality.
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Surgical Care

Congenital TEFs

In the preoperative phase, risk of aspiration should be reduced. Continuous suctioning of the blind esophageal pouch with an 8F catheter may decrease the risk of aspiration. The infant's head should be elevated, and he or she should be hydrated and provided energy intake (caloric intake) via intravenous dextrose solution.

If the patient develops acute respiratory failure, endotracheal intubation and mechanical ventilation are performed. Administer broad-spectrum antibiotics for patients who may have developed lower respiratory tract infection. For patients known to have pneumonia or other pulmonary problems, a gastrostomy for gastric decompression may be required to prevent further reflux of gastric contents into the trachea. The use of proton pump inhibitors may be helpful.

Operative repair

  • Timing of operation and choice of surgical approach in congenital TEFs are crucial. Make decisions based on the size and condition of the infant. Most infants are recommended to undergo primary care; however, a staged repair several weeks following birth is recommended for infants who are premature and have severe respiratory distress syndrome. The presence of other severe comorbidities, such as aspiration pneumonia, congenital cardiac disease, or other life-threatening conditions, should also delay the primary repair. Tracheostomy is required only if planning a staged repair. Infants who have severe respiratory distress syndrome may require the use of a Fogarty balloon catheter to obliterate the TEF while awaiting surgery.
  • The repair is performed via right thoracotomy in the left lateral decubitus position, and the head of table is elevated to avoid gastric reflux. A posterolateral thoracotomy incision is made through the fourth intercostal space, and a retropleural exposure is obtained. During the dissection, the azygos vein is divided and the vagus nerve is identified. The distal esophagus is identified and dissected distal to the TEF. The fistula is divided and closure is performed with stay sutures. Dissection is carefully performed to avoid interruption of blood supply or the branches coming off the vagus nerve. Tracheal suture line may be covered with a flap of mediastinal pleura. Prior to esophageal anastomosis, the proximal pouch of the trachea is mobilized.
  • If a fistula lies between the esophageal pouch and trachea, it is divided and closed. The esophageal anastomosis is performed in 1-2 layers and is covered with mediastinal pleura. A nasogastric feeding tube is placed through the esophagus into the stomach prior to the chest closure, and a chest tube is placed in the retropleural space.
  • Postoperatively, the infant is ventilated as needed, nasogastric or gastrostomy feedings are resumed, and a contrast swallow radiographic examination is performed on the seventh postoperative day. If no leak is detected, oral feedings are resumed. Approximately 3 weeks later, the esophagus is dilated up to a 24F size in order to prevent future esophageal stenosis.
  • The most common complications of surgery are pneumonia and atelectasis leading to respiratory failure in postoperative period. A leak at the anastomotic site and pneumothorax are other complications. Most patients who develop an anastomotic leak also develop strictures, which may be dilated later. [5] Rarely, a recurrent TEF may develop. The management of recurrent TEFs usually requires repeat surgical repair. Some patients develop periodic apneic spells that are likely secondary to gastroesophageal reflux and associated laryngospasm.
  • Esophageal atresia with tracheo-esophageal fistula is a relatively common congenital anomaly. Research with rodent models is contributing to the scientific understanding of the condition. Advances in surgical care and neonatal management have improved survival to approximately 90%. Long-gap and isolated esophageal atresia present significant management challenges. Post-operative and long-term complications including esophageal stricture, gastro-esophageal reflux, and respiratory compromise remain relatively common and continue to present a challenge for ongoing patient management. [6]
  • Repair of esophageal atresia and tracheoesophageal fistula has traditionally been performed via thoracotomy. Recent attempts at thoracoscopic repair have shown that such repair is feasible but technically challenging. However, more data are needed for further evaluation of this approach, particularly in long-gap defects that require more extensive dissection and difficult anastomosis.
  • Wang and colleagues examined national outcomes in newborn patients with esophageal atresia and tracheoesophageal fistula (EA/TEF) in the United Sates, using the Kids' Inpatient Database (KID). Investigators analyzed inpatient admissions for pediatric patients with EA/TEF. They identified 4168 cases with a diagnosis of EA/TEF. The overall in-hospital mortality was 9%. Univariate analysis revealed lower survival in patients with associated acute respiratory distress syndrome, ventricular septal defect (VSD), birth weight (BW) < 1500 g, gestational age (GA), time of operation within 24 h of admission, coexisting renal anomaly, imperforate anus, African American race, and lowest economic status. Multivariate logistic regression identified BW < 1500 g, operation within 24 h, GA <28 wk, and presence of VSD as independent predictors of in-hospital mortality. Investigators found that children's general hospitals and children's units in a general hospital achieved a lower mortality rate compared with facilities not identified as a children's hospital. [7]
  • In a different study of 268 infants with EA/TEF, 8 (3%) were extremely low birth weight (ELBW, <1000 g) and had high morbidity and mortality mainly associated with complications not related to EA/TEF repair. [8]

Acquired nonmalignant TEFs

Gastric decompression is achieved via a nasogastric tube. Patients who are not fit enough to withstand early surgery are treated conservatively with decompressing gastrostomy and feeding jejunostomy. If a patient is critically ill and reparative surgery cannot be undertaken in a timely manner, consider esophageal ligation, creation of a high salivary fistula, and feeding gastrostomy.

Operative repair and patient management

  • Before the actual procedure, a clinical decision is made regarding whether the fistula can be simply resected and closed or whether tracheal resection and reconstruction is required. A low collar incision is used for the repair of most fistulas; however, a right lateral thoracotomy is used for fistulas around the carina. A small fistula and normal trachea does not require tracheal resection. The fistula is identified and divided, and the esophageal defect is closed in layers. During fistula repair, the esophagus and trachea are closed primarily. The strap muscle pedicle flaps are positioned between the trachea and esophagus to reinforce the closure. The muscles used for pedicle interposition are sternohyoid or sternothyroid muscles. In the lower thorax, following closure of the esophagus, reinforcement with a flap comprised of pleura, intercostal muscle, and rib periosteum is commonly performed.
  • A large defect with tracheal damage often requires tracheal resection and reconstruction. Following resection of the trachea, the esophageal defect is closed longitudinally in 2 layers, tracheal reconstruction is carefully performed, and anastomotic tension is avoided. The strap muscle is used to cover the esophageal suture line and to separate it from tracheal suture line in order to prevent recurrence.
  • Postoperative management is determined by the patient's general health status. If the patient requires prolonged intubation, care is taken to avoid positioning the cuff at the suture line. The patient should not have a simultaneous nasogastric tube in order to prevent a risk of recurrence.

Acquired malignant TEFs

More than 75% of the patients with TEFs secondary to tracheal intubation require tracheal resection because of circumferential damage to the trachea. Mechanical ventilation following tracheal reconstruction is contraindicated because of excessive risk of tracheal dehiscence.

In patients who present within 2-3 days after onset and are in good general condition, offer gastric bypass with esophageal exclusion. Esophageal exclusion may be performed by cervical esophagostomy and gastrostomy, with closure of the esophagus above and below the fistula. This procedure may still not prevent pulmonary sepsis and death in many individuals.

Esophageal endoprosthesis

  • Placement of an esophageal endoprosthesis has been used to palliate patients with malignant TEFs. A variety of stents are currently available; these include plastic stents (eg, Medoc, Atkinson, Celestin) and self-expanding metallic stents. Some authors have proposed the esophageal stents as the first line of therapy for malignant strictures associated with a TEF.
  • The placement of an endoprosthesis may be complicated by an inability to see the endoprosthesis well, enlargement of the TEF, and ongoing contamination of the respiratory tract. Other complications associated with esophageal stents include migration, obstruction, ulceration, esophageal necrosis, and delayed perforation. Insertion of plastic stents requires aggressive dilatation.
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Consultations

Patients should be seen by a gastroenterologist or pulmonologist for diagnostic workup.

Consultation with a thoracic surgeon is required for definitive surgical repair.

Any patient who is ill must be cared for in the intensive care unit so that the staff can monitor the patient's respiratory system and ensure adequate nutrition.

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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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