Tracheoesophageal Fistula

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 tracheoesophageal fistula.

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)

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.

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.

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]

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.

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 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]

History

Esophageal atresia in the fetus should be considered as a cause of maternal polyhydramnios. Absence of stomach gas on prenatal ultrasonography 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 tracheoesophageal fistula (TEF).

The symptoms induced by malignant TEFs are cough, aspiration (especially on swallowing liquids), and fever. The average duration of symptoms from onset to diagnosis is approximately 12 days.

Physical examination

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.

Prenatal diagnosis of congenital tracheoesophageal fistulas (TEFs)

Prenatal ultrasonography may reveal polyhydramnios, absence of fluid-filled stomach, small abdomen, lower-than-expected fetal weight, and a distended esophageal pouch.

Postnatal diagnosis of congenital TEFs

Note the following:

• Plain chest radiographs may reveal tracheal compression and deviation. Absence of a gastric bubble indicates esophageal atresia without a TEF or esophageal atresia with a proximal TEF. Chest radiography leads to the diagnosis of TEF in most cases of congenital TEF, and other investigations are rarely required.

• Aspiration pneumonia in the posterior segments of the upper lobes may occur secondary to aspiration of the contents from the esophageal pouch or stomach. Recurrent or massive aspiration may lead to acute lung injury in some patients. (Infiltrates occur diffusely in these patients.)

• Insertion of a nasogastric tube may show coiling in the mediastinum of patients who have concomitant esophageal atresia. This finding is diagnostic of TEFs associated with esophageal atresia.

• Contrast studies are seldom required to confirm the diagnosis. These studies have the risk of aspiration pneumonitis and pulmonary injury, and they add minimal information to the diagnostic workup. If the contrast study is performed, 1-2 mL of barium is instilled through an 8F catheter placed into the esophagus. Chest radiographs are taken in the lateral decubitus position as well as the anteroposterior position to detect spilling of the contrast into the trachea.

• Use of multidetector-row CT scans have made 3-dimensional (3D) displays of many organs and structures a popular clinical examination tool, as the quality of images has markedly improved. Presence of TEF was correctly diagnosed with multidetector-row CT esophagography. Furthermore, the images provided crucial information for planning surgery, and, without contrast medium, it is a less invasive examination.

Diagnosis of acquired TEFs

Acquired TEFs can be diagnosed by instillation of contrast media into the esophagus or during direct visualization by flexible esophagoscopy or bronchoscopy. Either method can be useful, depending on the individual center's expertise and experience.

Some clinicians prefer to visualize the fistula and assess its exact location prior to surgery. The diagnosis of a TEF secondary to malignancy is confirmed by contrast radiography, esophagoscopy, bronchoscopy, and clinical testing (methylene blue).

Flexible esophagoscopy or flexible bronchoscopy may be useful in the diagnosis of acquired tracheoesophageal fistulas (TEFs). Either or both of these procedures may be required to evaluate the anatomy of these structures and to exclude an unsuspecting mucosal lesion. The role of endoscopic procedures is especially important in localizing the acquired nonmalignant or malignant TEF.

Transfer infants and children with tracheoesophageal fistula (TEF) to a pediatric center experienced with surgical repair of TEF. The center should be experienced with providing support of critically ill pediatric patients.

Adults who develop acquired TEF must be transferred to a facility with thoracic surgery/therapeutic endoscopy support and other adequate support services.

Surgical repair is required following confirmation of a diagnosis of tracheoesophageal fistula (TEF). Note the following:

• 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.

• Because 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 endoprosthesis (covered self-expandable metal stent [SEMS]), esophageal exclusion or bypass, resection, or direct closure. 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 et al reported that bypass therapy and radiation therapy were the only treatments that significantly prolonged survival compared to supportive care.[6] 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.

Congenital tracheoesophageal fistulas (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

Note the following:

• Timing of the operation and the 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.[14] 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 tracheoesophageal 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.[15]

• 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.[16] 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.[17]

• 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.[18]

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

Note the following:

• 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

Note the following:

• 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 covered self-expanding metallic stents (SEMS).[19] 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.

• Airway stents are also being placed to prevent contamination of the respiratory tract.

Patients should be seen by a gastroenterologist/therapeutic endoscopist 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.

Patients on prolonged ventilatory support are at risk of developing a tracheoesophageal fistula (TEF). The incidence of TEFs has decreased markedly following the introduction of endotracheal tube cuffs of high volume and low pressure. In critically ill patients, cuff pressure of 30-40 mm Hg may decrease capillary perfusion and result in tissue ischemia. Cuff pressures should be maintained below 25 mm Hg, even at the expense of a small leak. Optimal nutrition and use of flexible, small-caliber nasogastric feeding tubes may be of further help.