Tracheoesophageal Fistula Treatment & Management
- Author: Sat Sharma, MD, FRCPC; Chief Editor: Julian Katz, MD more...
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.
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.
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.  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. 
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. 
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. 
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.
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.
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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|Anatomic Characteristics||Percent of Cases|
|Esophageal atresia with distal TEF||87|
|Isolated esophageal atresia without TEF||8|
|Esophageal atresia with proximal TEF||1|
|Esophageal atresia with proximal and distal TEF||1|