Congenital Anomalies of Esophagus Treatment & Management

Updated: Jun 15, 2021
  • Author: Mark V Mazziotti, MD, MEd; Chief Editor: Eugene S Kim, MD, FACS, FAAP  more...
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Approach Considerations

All children with esophageal atresia (EA) and many with congenital stenosis require surgical intervention. The diagnosis of EA or tracheoesophageal fistula (TEF) can be made antenatally and after birth by clinical signs and supportive findings on imaging studies.

Antenatally, a finding of a small or absent stomach bubble on ultrasonography (US) suggests EA with 42% sensitivity. Antenatal magnetic resonance imaging (MRI) was used to evaluate the esophagus of fetuses with a small or absent stomach bubble on US evaluation. [9] Positive findings on antenatal MRI had a sensitivity of 100% and specificity of 80%. After birth, failure of passage of a rigid radiopaque 10-French catheter from the mouth to the stomach suggests EA. The diagnosis is typically confirmed with plain radiography.

The presence of air in the stomach and intestines indicates EA with a distal fistula; the absence of abdominal gas suggests pure atresia, EA with a proximal fistula, or, on rare occasions, EA with an occluded distal fistula. A small upper esophageal pouch is suggestive of a proximal fistula, and the presence of a proximal TEF can be confirmed with fluorography, endoscopy, bronchoscopy, or upper esophageal contrast studies.

An isolated TEF may be detected by barium esophagography, cinefluoroscopy, bronchoscopy, or esophagoscopy. Because of the risk of aspiration, the use of contrast for visualization of congenital esophageal anomalies must be approached with extreme care and performed only by an experienced radiologist.

Although a diagnosis of EA or TEF is no longer considered a surgical emergency, because of improvements in neonatal intensive care, respiratory problems may still develop and rapidly progress. A period of 24-48 hours between diagnosis and surgical repair allows for a thorough assessment of the neonate and treatment of any pulmonary complications. In general, vigorous infants weighing more than 1300 g and without pulmonary insufficiency or major associated anomalies should be considered for repair.

Historically, prognostic risk classification was based on birth weight, the presence and severity of pneumonia, and congenital anomalies for infants with EA. Subsequently, ventilator dependence and severe anomalies, not birth weight, have been linked to mortality. [10] Regardless of the classification used, stronger infants with fewer concomitant disorders have lower risks associated with the surgical repair.

Intervention for esophageal stenosis, webs, and tracheobronchial remnants is indicated according to the diagnosis and the presence of symptoms.

The selection and timing of surgical treatment for congenital esophageal anomalies depend on the type of lesion, the presence and severity of associated anomalies, the vigor of the infant, pulmonary status, and the presence of infection. Prematurity, life-threatening congenital anomalies, sepsis, and respiratory compromise may necessitate delay of surgical treatment. Lung infiltrates, particularly those involving the left lung, usually require treatment before the operation.

In general, the overall health of the infant should be considered in preparing for surgery, and the most serious abnormalities should be corrected first.


Medical Therapy

To prevent accumulation of mucus, aspiration, and respiratory deterioration continuous or intermittent low-pressure suction of the upper esophageal pouch should be initiated with a double-lumen Replogle catheter. In small infants, intermittent suction may be better.

The infant should be positioned to minimize gastric fluid reflux. The infant is typically positioned in a 45º sitting position. In addition, infant handling should be minimized because excess disturbance may lead to further respiratory complications, increased oxygen consumption, cold stress, and increased regurgitation of gastric contents.

Oxygen therapy should be administered as needed to maintain oxygen saturation. Endotracheal intubation is not performed routinely, but it may be required on the basis of the infant's respiratory status. Bag-mask ventilation should be avoided because it may cause gastric distention leading to increased reflux.

Intravenous (IV) fluid therapy consisting of 10% dextrose and hypotonic sodium chloride solution is used to maintain fluid, electrolyte, and glucose balance. Broad-spectrum antibiotics should be administered at the time of diagnosis or after cultures are obtained. A vitamin K analogue should also be administered before surgery.

Under no conditions should the infant be orally fed. If surgical treatment is delayed more than a few days, total parenteral nutrition (TPN) is used. In addition, the infant should be transferred to a tertiary care pediatric institution with a neonatal intensive care unit (NICU) and a pediatric surgery team.


Surgical Therapy

The choice of operative procedure for infants with congenital esophageal abnormalities depends on the specific type of anomaly present, the condition of the infant, and the presence of other congenital anomalies. [11, 12]


The staged approach for patients with pure EA and in some infants with EA and TEF includes initial placement of a Stamm gastrostomy, followed later by fistula division and later esophageal reconstruction. Gastrostomies may cause problems in infants with EA and TEF, especially in premature infants with severe respiratory distress syndrome requiring positive-pressure ventilation.

Because of the TEF, the infant's respiratory and upper gastrointestinal (GI) tracts function as a single unit. Therefore, the sudden decrease in intragastric pressure may result in preferential airflow through the fistula. Fistula ligation, occlusion of the fistula with a Fogarty catheter, and an underwater seal for the gastrostomy tube are methods used to maintain ventilatory pressure in these cases.

Most surgeons perform a gastrostomy in the first 24 hours of life in an infant with pure EA. This allows enteral feedings while the child grows and the esophageal gap shortens. A gastrostomy may be used in premature or unstable infants with EA and TEF.

Esophageal atresia with tracheoesophageal fistula

Fistula division with primary anastomosis is the surgical treatment for EA with TEF. A posterolateral thoracotomy on the side opposite the aortic arch is used. The patient is typically positioned on the left side with a small axillary roll. The right arm is extended above the head with the neck slightly flexed. Typically, manual ventilation control is used until the fistula is ligated.

For infants in whom ventilation is difficult because of the passage of air through the fistula into the stomach, insertion of a Foley catheter through the fistula into the lower esophagus may be helpful. This can be done through a bronchoscope or performed retrograde through the stomach accessed with a laparotomy incision. A fourth intercostal extrapleural approach is employed through a transverse incision along the inferior angle of the scapula, from the anterior axillary line posteriorly to the paravertebral region.

Although some surgeons prefer the transpleural technique for its speed, the extrapleural approach provides added protection against empyema should an anastomotic leak occur. The latissimus dorsi is divided, and the serratus anterior can be divided or reflected to protect its innervation. Muscle-sparing of both the latisumus dorsi and the serratus anterior can also be performed.

The thorax is entered through the fourth intercostal space (ICS) by dividing the intercostal muscles. The extrapleural dissection begins posteriorly and proceeds superiorly, inferiorly, and finally anteriorly, where the risk of a pleural tear is highest. Wet cotton-tipped applicators, gauze swabs, moist peanut dissectors, or gentle finger dissection facilitates the pleural dissection from the chest wall.

The azygos vein is identified, ligated and divided, or retracted (see the image below). The lung and pleura are medially retracted. The distal esophageal segment is identified by following the right vagus nerve inferiorly, and the connection of the esophagus to the trachea is located. The lower esophagus is dissected circumferentially near the fistula, with care taken not to damage the vagal fibers or vascularization.

Intraoperative photograph showing azygos vein. Rig Intraoperative photograph showing azygos vein. Right thoracotomy and retropleural dissection have been performed.

Extensive mobilization of the distal pouch is not recommended, because of its segmental blood supply. However, distal pouch mobilization may be necessary to achieve a primary anastomosis. The fistula is divided close to the trachea. A 1- to 2-mm esophageal cuff should be left on the trachea to minimize the risk of postoperative tracheal stricture. Leaving too large a cuff may lead to a tracheal diverticulum. Interrupted 5-0 or 6-0 polydioxanone, silk, or polypropylene sutures are used to close the fistula.

The air-tightness of the tracheal closure should be assessed by filling the chest with saline and looking for any bubbles when positive pressure is applied by the anesthesiologist. Atraumatic handling of the distal esophagus is important. Two fine stay sutures in the distal esophagus allow for mobilization and gentle traction without the use of forceps.

The proximal segment is identified by gently advancing the Replogle tube, and traction sutures may be placed. The upper pouch can and should be mobilized all the way to the thoracic inlet. Circumferential mobilization to the thoracic inlet aids in identifying proximal fistulas. Care must be taken during the dissection of the proximal esophagus to avoid injury to the trachea or recurrent laryngeal nerve.

A primary anastomosis should be performed whenever possible. If the esophageal segments cannot be joined without undue tension after mobilization of both ends, a circular myotomy may be performed on the proximal segment. A primary anastomosis is accomplished by opening the proximal pouch at the lowermost point. The opening created in the upper pouch should approximate that of the lower pouch. The distal segment is incised only if it is clearly devascularized or narrow and fibrous.

Interrupted absorbable sutures are used to perform the anastomosis. Lateral stay sutures are placed. Ensuring mucosal apposition during the anastomosis is important. Usually, five or six sutures are needed to complete the posterior row. After all posterior sutures have been placed, the knots are tied on the inside of the esophageal lumen to prevent subsequent twisting of the esophagus. As the sutures are tied, tension gradually is distributed to the tied sutures. The lateral stay sutures are not tied.

A small feeding tube can be passed across the anastomosis into the stomach to ensure luminal patency and protect the posterior wall of the anastomosis while the anterior wall is sutured. Some surgeons leave the tube in postoperatively to decompress the stomach and provide a postoperative feeding route (see the image below).

Chest radiograph immediately following repair of e Chest radiograph immediately following repair of esophageal atresia and distal tracheoesophageal fistula (TEF). Note chest tube (CT) in retropleural space and feeding tube passing through anastomosis (A) into stomach.

The anterior suture layer of the anastomosis is then completed over the tube, and the knots are tied on the outside (see the image below). A muscle or pleural tissue flap may also be placed between the anastomosis and the repaired trachea to decrease the risk of fistula recurrence.

Primary anastomosis (A) between proximal and dista Primary anastomosis (A) between proximal and distal esophagus.

Most surgeons place a retropleural chest tube with the tip positioned near, but not touching, the anastomosis (see the image below). The tube is placed to water seal drainage to avoid an extrapleural pneumothorax. Suction applied to the tube may disrupt the anastomosis. Intubation should be maintained as is clinically required. Premature extubation that results in aggressive bag-mask ventilation and reintubation can be disastrous to the repair.

Chest radiograph following repair of esophageal at Chest radiograph following repair of esophageal atresia. Chest tube is placed in retropleural space (CT). No feeding tube is passing across anastomosis.

Premature and medically unstable children can be maintained on TPN with a tube in the proximal pouch until surgery. Emergency thoracotomy and fistula ligation are used for infants with respiratory distress when nonoperative management is unsuccessful.

Thoracoscopic approaches

In some cases, thoracoscopic techniques have been used to repair tracheoesophageal atresia and distal fistula. [13]  The use of minimally invasive techniques has been gaining traction as a consequence of the evidence for the long-term morbidity of thoracotomy in these patients. Even when muscle-sparing techniques are employed, thoracotomy can result in thoracic scoliosis, winged scapula, and shoulder weakness. [14] In addition, a multicenter study showed that the outcomes and complication rates of thoracoscopy were similar to those of thoracotomy. [15]

Given confirmation that the aortic arch is on the left side, the approach is through the right chest. The patient is positioned in the left lateral decubitus position, but leaning slightly toward the prone position; this affords better access to the posterior mediastinum. An incision is made near the tip of the scapula, a 5-mm trocar is placed, and the chest is insufflated to a pressure of 5 mm Hg; this renders lung isolation unnecessary. Additional working ports are placed near the axilla and the posterior axillary line near the seventh rib. 

The azygos vein (see the first image below) is taken with a sealing device or is ligated or clipped and divided, exposing the tracheoesophageal fistula. The fistula is clipped or ligated and divided near its tracheal junction (see the second image below). As in the open repair, the proximal pouch is extensively mobilized up into the neck to gain length. This dissection can be aided by the anesthesiologist pushing on a tube in the proximal esophagus. Extensive dissection of the distal esophagus is avoided because of its segmental blood supply.

Azygos vein prior to division. Distal fistula lies Azygos vein prior to division. Distal fistula lies beneath this vein.
Distal tracheoesophageal fistula is clipped and di Distal tracheoesophageal fistula is clipped and divided.

The end of the proximal pouch is opened with endoscopic shears. The anastomosis is then performed with interrupted 5-0 absorbable sutures. To bring the ends together, the first suture may be tied extracorporeally. The remainder of the sutures are then tied intracorporeally with the knots on the inside for the back wall, which usually requires about five sutures. (See the image below.) A tube is then passed from the proximal pouch to the distal esophagus. This allows the anterior wall to be closed with minimal chance of catching the back wall of the esophagus with any of these sutures.

Posterior wall of anastomosis is completed. Interr Posterior wall of anastomosis is completed. Interrupted absorbable sutures on anterior wall will complete anastomosis.

A chest tube is placed at the conclusion of the procedure.

Esophageal atresia with proximal fistula

The repair for a proximal fistula is the same as that described above. If a proximal fistula is present with no distal fistula, a cervical approach may be used instead.

Pure esophageal atresia

Most children with pure EA undergo gastrostomy placement followed by delayed repair. After a period of growth, repair of EA without fistula consists of a thoracotomy with retropleural dissection, mobilization of the two esophageal segments, and a primary anastomosis (see the images below). Esophageal continuity can usually be achieved without an esophageal replacement.

Repair of esophageal atresia. Head is to left. Azy Repair of esophageal atresia. Head is to left. Azygos vein has been divided. Proximal (P) and distal (D) esophageal pouches can be easily observed.
Primary anastomosis (A) between proximal and dista Primary anastomosis (A) between proximal and distal esophagus.

Several techniques are used for lengthening the esophageal ends to achieve a primary anastomosis. Bouginage (bougienage) is the most common mechanical lengthening procedure. Upper pouch bouginage is performed by passing a weighted bougie through the mouth into the upper pouch and applying forward pressure once or twice daily. This procedure is performed for 6-12 weeks and is followed by a delayed primary repair. Both internal and external traction sutures have been used by some surgeons.

During surgery, lengthening techniques may also be used. [16, 17] Myotomy is a common method that provides a 1-cm increase in length. The muscular layers are divided to create a plane between the muscularis propria and submucosa. Circular myotomies involve a circumferential division, whereas spiral myotomies preserve the muscular continuity of the proximal pouch and maintain closed submucosal layers. As many as three myotomies can be performed. The most proximal myotomy should be performed first.

This procedure can be performed on both esophageal segments, but myotomies performed on the distal stump may increase the incidence of gastroesophageal reflux (GER).

When additional length is necessary, a portion of the stomach may be brought up through the diaphragmatic hiatus. This procedure has been successfully used even in infants with low birth weight. A Collis lengthening procedure may also be used.

A cervical esophagostomy is used when an anastomosis is impossible and in cases of failed surgery. For this procedure, a left transverse incision is performed 1 cm above and parallel to the medial third of the clavicle. The incision is deepened through the platysma. The sternal head of the sternocleidomastoid is divided, the sternothyroid is divided or reflected, and the carotid sheath is retracted. The esophagus is mobilized circumferentially and dissected distally. The esophageal end is brought out to the lateral end of the skin incision and sutured with interrupted absorbable sutures.

This procedure allows the child to swallow normally. Sham feeds are administered to stimulate lengthening by a natural bouginage effect and to avoid oral aversion. The infant may be discharged home on gastrostomy feedings until esophageal repair or replacement can be performed.

A variant of this method is the multistaged extrathoracic elongation, which has also been used in long-gap treatment. In this procedure, the upper esophagus is initially mobilized and brought out as an end cervical esophagostomy. Over a period of weeks to months, the esophagus and stoma are progressively translocated down the anterior chest until adequate length is achieved to permit an end-to-end anastomosis. Again, the child is able to swallow and can be discharged home during the intervening period. 

External or internal traction sutures may be used, in a technique originally described by Foker. [18] Once the two ends of the esophagus are identified, seromuscular sutures are placed on both sides of each end in such a way as to include bovine pericardium pledgets, thus keeping the sutures from pulling through the esophageal tissue.

The proximal traction sutures can be brought out through the chest wall near the eighth rib to provide inferior traction, while the distal esophageal sutures are brought out through the chest wall at the second rib to provide superior traction. The ends are marked with clips so that progress can be monitored with plain radiographs. When the ends are approximately two vertebral bodies apart, a primary anastomosis can be performed.

Esophageal replacement

Esophageal substitutions are used to restore esophageal continuity when the patient's native esophagus is not an option. No esophageal replacement is ideal, and a poorly functioning esophagus may even be preferable to any esophageal substitute. Because of advances in the surgical techniques to treat EA, the need for esophageal replacement has diminished; however, conditions that necessitate an esophageal substitution are noted.

Many types of conduits have been used, including colon, stomach, gastric tubes, and jejunum. The choice of substitute to be used is influenced by the length and segment of esophagus to be replaced, the presence of any associated anomalies, and the vascular adequacy of the proposed replacement. Most often, the surgeon's preference and experience contributes to the selection.


Segments of the right, left, or transverse colon may be used (see the image below). The colon interposition may be placed in a posterior mediastinal or retrosternal position within the thorax. Colonic interpositions act as passive conduits, allowing isoperistaltic and antiperistaltic positions within the chest. Reports of spasm, regurgitation, and discomfort with the antiperistaltic colonic conduits indicate some retention of motor function. The isoperistaltic method is often favored.

Contrast study demonstrating colonic interposition Contrast study demonstrating colonic interposition. Colon is sutured to cervical esophagus proximally and to stomach distally. No leak or stricture is noted.

Advantages of colonic replacement of the esophagus include the following:

  • Adequate length available
  • Acts as a conduit antiperistaltically or isoperistaltically
  • Good vascular supply via marginal artery
  • Conserves native stomach
  • Can be placed in esophageal bed of posterior mediastinum
  • Has memory (but variable) for propelling solid bolus
  • Has mucous shield, which protects against reflux
  • Responds to acid with a peristaltic rush for clearance
  • May allow preservation of entire native esophagus
  • Minimizes/eliminates tension on the upper and lower esophageal segments
  • May improve esophageal motility and minimize reflux

Disadvantages of colonic replacement of the esophagus include the following:

  • Requires three anastomoses
  • Empties more slowly than the esophagus
  • Requires preoperative bowel preparation
  • Long surgical procedure with extensive mobilization
  • Dilates and becomes redundant over time
  • Slows food transit
  • Anastomotic leakage and stricture
  • Graft necrosis
  • Long-term growth retardation

Gastric tube

Esophageal replacement can be achieved by fashioning a tube from the stomach. The two most common tubes are the reversed (antiperistaltic) tube and the nonreversed (isoperistaltic) tube. Delaying the procedure allows stomach enlargement, and a long tube can then be constructed. Gastrostomy tubes should be placed close to the lesser curvature of the stomach in these infants.

The reversed gastric tube is constructed most commonly. It is proximally based and supplied by the left gastroepiploic vessels. This tube can be used to replace the entire esophagus but has a more limited blood supply than the isoperistaltic tube, and care must be taken during the procedure not to injure the spleen. The nonreversed, or isoperistaltic, tube is based distally and supplied by the right gastroepiploic vessels.

A one-stage or two-stage procedure may be performed. A staged procedure involves constructing the gastric tube and externalizing it in the neck. A cervical anastomosis is completed 4-6 weeks later. The gastric tube and gastric remnant should be studied radiographically before reanastomosis.

Advantages of a gastric tube include the following:

  • Thick-walled straight conduit
  • Does not become dilated, tortuous, or redundant
  • Less risk of ischemia because of robust blood supply
  • Simplified construction through stapler use
  • Has a favorable anatomic location in the upper abdomen
  • Possible construction variations
  • Has adequate length
  • Requires fewer anastomoses
  • Has comparable diameter and occupies less space in the thorax and neck
  • Has only one suture line
  • Requires no bowel preparation and is a faster procedure
  • Rapidly transits

Disadvantages of a gastric tube include the following:

  • Leaks and strictures
  • Extensive GER, which may lead to peptic ulceration, nocturnal coughing, and Barrett epithelium
  • Leaves a small gastric reservoir
  • Creates a long suture line
  • May result in gastric outlet obstruction
  • May be unable to reach high in neck
  • Difficult to place in posterior mediastinum
  • Occasional perforation

Gastric transposition

In a gastric transposition, the entire stomach assumes an intrathoracic position and serves as a passive conduit (see the image below). The fundus of the stomach is joined to the upper esophageal pouch, and the lower esophageal segment is not used. 

Contrast swallow (oblique view) demonstrating gast Contrast swallow (oblique view) demonstrating gastric pull-up used as esophageal replacement in child with pure esophageal atresia. Note stomach in chest.

A gastric transposition can be performed in infants and children of all ages but is typically performed around the first year of life. Although some prefer this method of esophageal replacement, others advise against its use in infants and children because of the effects of the gastric capacity crowding the lungs and trachea and the excessive gastroesophageal reflux. An initially placed gastrostomy is not a contraindication.

Advantages of gastric transposition include the following:

  • Readily available and easily mobilized stomach
  • Involves single anastomosis
  • Adequate length available
  • Can use numerous surgical approaches
  • Has excellent blood supply
  • Involves a technically easy procedure
  • Has a low incidence of leaks and strictures

Disadvantages of gastric transposition include the following:

  • Large bulk possibly causing space problems intrathoracically
  • Reflux
  • Barrett epithelium development possible in proximal esophagus
  • Possible stricture or aspiration due to lack of gastroesophageal valve
  • Poor gastric emptying
  • May affect pulmonary function
  • Results in depleted iron stores causing anemia
  • May affect growth
  • Microvasculature easily disturbed with rough handling
  • May not reach as high in neck as other methods because of blood supply
  • Difficult to initiate oral feedings
  • May result in dumping as a common postoperative symptom


Jejunal esophageal substitution is rarely used. The jejunal replacement is unique because it retains peristaltic activity and, therefore, must always be positioned isoperistaltically. However, peristalsis is not synchronous with swallowing and may slow food transit.

Proximal jejunal segments are preferred for graft construction because of their larger and fewer vascular arcades, which can be located and dissected more easily. Jejunal segments may range from 2 to 12 cm in length, but the tight radii of the superior mesenteric artery branches prevent construction of straight and longer segments. The jejunum is typically used only for replacements of short lower esophageal lengths.

Advantages of jejunal replacement of the esophagus include the following:

  • A remarkably disease-free organ
  • Readily available
  • Jejunal caliber similar to that of normal esophagus
  • Functions as reliable food transporter
  • Results in low incidence of leaks and strictures
  • Functions as an effective gastroesophageal barrier
  • Does not require a bowel preparation
  • Jejunal wall at correct thickness for comfortable suture placement

Disadvantages of jejunal replacement of the esophagus include the following:

  • Length of conduit limited by blood supply
  • Infarction commonly resulting from passage through chest
  • Procedure more technically difficult
  • Requires three anastomoses
  • Has high peptic ulcer susceptibility
  • Blood supply lacking marginal artery
  • Difficult to obtain a straight conduit with sufficient vascularization
  • Has high failure rate (free graft)

Isolated tracheoesophageal fistula

H-fistulas or N-fistulas are typically located at the T1-3 level, coursing downward from the trachea to the esophagus. Most can be repaired through a cervical approach. An undiagnosed H-type fistula may also be identified while the proximal esophagus is being mobilized during surgery for EA (see the image below).

Intraoperative photograph demonstrating proximal H Intraoperative photograph demonstrating proximal H-type fistula (F) between proximal esophagus (P) and trachea (T). Blue vessel loop is around fistula. H-type fistula was not diagnosed preoperatively but was identified during mobilization of proximal esophagus in child with congenital esophageal stenosis.

A supraclavicular incision is made 1-1.5 cm above and parallel to the right clavicle to minimize the risk of injury to the thoracic duct. The sternocleidomastoid is retracted posteriorly, and the sternal head is divided if necessary. The inferior thyroid artery and the middle thyroid vein are divided if needed to expose the plane between the trachea and the esophagus.

Care should be taken to clearly identify and preserve the recurrent laryngeal nerves, vagal nerve fibers, and posterior trachea. The fistula may be located higher than might be expected. The esophagus is encircled with rubber vessel loops to facilitate mobilization. The fistula is also encircled when it is identified (see the image above). The fistula is divided close to the esophagus, leaving a 2-mm esophageal cuff on the trachea. Interrupted sutures are used to close the esophagus and trachea.

A muscle flap may be interposed between the two suture lines to decrease the likelihood of a recurrent fistula. Fistula ligation without division should not be performed. Wound drainage is typically not necessary for an H-fistula repair, and the endotracheal tube should be left in because tracheal swelling is a frequent postoperative occurrence.

Congenital esophageal stenosis, webs, and tracheobronchial remnants

Treatment should relieve the obstructive symptoms and maintain the antireflux mechanism of the esophagogastric junction. Bouginage (bougienage) or balloon dilation may successfully treat fibromuscular hypertrophy. Membranous webs, complete occlusion, and tracheobronchial remnants usually require surgical excision (see the image below).

Intraoperative photograph of infant thought to hav Intraoperative photograph of infant thought to have esophageal atresia. Proximal (P) and distal portions of esophagus were connected by fibrotic segment of congenital esophageal stenosis (CES) beginning at azygos vein (A). No distal tracheoesophageal fistula was present.

A right thoracotomy is usually used. Lesions in the abdominal esophagus can be approached through the abdomen. A segmental resection and primary anastomosis can be achieved in most instances (see the image below). The phrenic and vagus nerves should be identified and preserved. Esophageal replacement may be needed for long segments of fibromuscular hypertrophy.

Intraoperative photograph following resection of a Intraoperative photograph following resection of atretic segment of esophagus and primary anastomosis (A) of proximal (P) and distal esophagus. Azygos vein (AV) was preserved.

Postoperative Care

Postoperative care of an infant with EA involves a team approach, typically in the NICU. A chest radiograph is immediately obtained following surgery (see the images below).

Chest radiograph immediately following repair of e Chest radiograph immediately following repair of esophageal atresia and distal tracheoesophageal fistula (TEF). Note chest tube (CT) in retropleural space and feeding tube passing through anastomosis (A) into stomach.
Chest radiograph following repair of esophageal at Chest radiograph following repair of esophageal atresia. Chest tube is placed in retropleural space (CT). No feeding tube is passing across anastomosis.

The infant should be kept in a semiupright position, with the head supported to prevent neck extension, which may disrupt the anastomosis. Intravenous fluid replacement should be maintained, and prophylactic antibiotic treatment should be continued. The pharynx should be frequently suctioned to prevent respiratory infection, but deep suctioning should be avoided. Appropriate temperature, humidity, and oxygen atmospheric control are essential.

If the anastomosis was performed under extensive tension, some surgeons recommend elective paralysis and mechanical ventilation for several days postoperatively. In addition to paralysis, surgeons may choose to keep the neck flexed, including suturing the chin to the anterior chest, in order to reduce the tension to the anastomosis. Otherwise, the patient is weaned from the ventilator as soon as possible.

Contrast esophagography is performed postoperatively to assess for esophageal leak, stricture, motility, and GER (see the image below). The swallowing reflex and positions of the duodenum and ligament of Treitz should also be examined.

Esophagogram 1 week following primary anastomosis Esophagogram 1 week following primary anastomosis for pure esophageal atresia. No leak or stricture is observed.

The timing of the initiation of feeding varies. Some advocate starting gastrostomy or nasogastric feedings on postoperative day 1 or 2 in uncomplicated cases. Other surgeons advise against gastrostomy or nasogastric tube feedings because of the potential for acid reflux and injury to the anastomosis. TPN should be used when enteral feedings are not started. If no leaks are observed on the postoperative contrast study, feedings are initiated and the chest tube is removed. The child may be discharged when feedings are tolerated and appropriate weight gain is observed.



The severity of complications following esophageal surgery is often dictated by the extent of the repair. Anastomotic tension is involved in 79% of complications, and the most common complications include anastomotic leakage, recurrent fistula, stricture, and GER.

Anastomotic leakage occurs in anywhere from 14% to 21% of children who have undergone a surgical EA repair (see the image below). Leaks result from the small friable lower segment, ischemia of the esophageal ends, excess anastomotic tension, sepsis, poor suturing techniques, and inaccurate mucosal apposition.

Anastomosis disruption following repair of esophag Anastomosis disruption following repair of esophageal atresia with tracheoesophageal fistula. Contrast was injected through gastrostomy and refluxed through distal esophagus. Leak (L) is draining into retropleural chest tube.

Early extubation with reintubation also puts infants at increased risk for anastomotic leakage. Most leaks are small, occur late after the first 48 hours, and require only conservative management. Chest tube drainage, antibiotics, and time allow most to heal. Spontaneous healing occurs in 95% of leaks when a mediastinal drain is present. A repeat esophagogram is obtained each week until the leak has resolved.

More significant leaks occur early, within the first few days. The large output of bubbly saliva in the chest tube in conjunction with a leak seen on esophagography is often an indication for reexploration. Some of these significant leaks may be managed medically with the use of glycopyrrolate, an anticholinergic agent that decreases copious salivary secretion and may promote spontaneous closure of the leak. [19] Major anastomotic disruptions account for only 3-5% of leaks. Large leaks can be fatal or may lead to fistula recurrence.

Fistula recurrence is observed in 3-14% of patients treated for EA-TEF or isolated TEF. Fistulas usually recur within a few months but may be found as late as 2 years postoperatively. Fistula recurrence is caused by anastomotic leakage with local inflammation and erosion at the previous repair site, ischemia, and surgical dissection too near the trachea.

Recurrent TEF should be suspected when choking episodes occur during feeding and/or recurrent pneumonia is observed. Esophagography under video fluoroscopy with the patient in the prone position or bronchoscopy provide the best methods of diagnosis. Routine contrast swallows do not reveal 50% of recurrent TEFs. Fistulas do not spontaneously close and require surgical division and suturing.

Attempts have been made to close recurrent fistulas with fibrin glue administered into the fistula. During surgical repair, a tissue flap should be interposed between the suture lines of the trachea and esophagus. Recurrence rates remain in the 10-20% range when repeat surgery is needed.

Esophageal strictures are also common after esophageal surgery. Anastomotic strictures occur in as many as 40% of children with an EA repair (see the images below). Strictures can result from the natural healing process, the different sizes of the two anastomosed segments, tension, GER, and leaks. Asymptomatic narrowing observed on initial esophagography can improve over time without the need for intervention. Strictures are clinically suspected, and the diagnosis can be confirmed via contrast esophagography and esophagoscopy.

Esophagogram 1 week after repair of esophageal atr Esophagogram 1 week after repair of esophageal atresia with distal tracheoesophageal fistula. Note narrowing at anastomosis (A). Child was asymptomatic and required no dilatations. Image 1 of 3 in series.
Follow-up esophagram demonstrating spontaneous imp Follow-up esophagram demonstrating spontaneous improvement of narrowing at anastomosis (A). Image 2 of 3 in series.
Esophagogram. Note spontaneous resolution of narro Esophagogram. Note spontaneous resolution of narrowing at anastomosis (A). No intervention was required in this child. Image 3 of 3 in series.
Esophagogram 7 days following repair of esophageal Esophagogram 7 days following repair of esophageal atresia. Note change in caliber from proximal esophagus to distal esophagus at anastomosis (A). Retropleural chest tube can be observed (CT). This patient had gastroesophageal reflux and developed progressive stricture. Image 1 of 3 in series.
Esophagogram obtained 3 weeks later demonstrating Esophagogram obtained 3 weeks later demonstrating stricture (A) and gastroesophageal reflux (R and green arrow). Medical therapy was initiated. Image 2 of 3 in series.
Esophagogram performed 2 weeks later demonstrating Esophagogram performed 2 weeks later demonstrating worsened anastomotic stricture (A). Patient responded well to Bouginage dilatation and medical management of reflux. Image 3 of 3 in series.

Most strictures can be managed with serial dilatations. Both bougienage dilators and balloon dilators have been used. Multiple dilatations over several months are needed in many cases. For recalcitrant strictures that do not improve with dilatations alone, the addition of a steroid injection (eg, triamcinolone) into the strictured area may be helpful. Also, there have been reports of the successful use of topical mitomycin c, an antineoplastic agent, for intractable esophageal strictures following TEF repair. [20]

Strictures unresponsive to dilation treatment may require surgical revision.

GER is a common complication of esophageal surgery, occurring in 40-70% of patients undergoing EA repair. Symptoms of GER include coughing, apnea, recurrent pneumonia, failure to thrive, and stricture formation (see the images below). Reflux is thought to be related to tension, dysmotility of the lower esophagus, and an altered angle of His from distal esophageal mobilization.

Esophagogram obtained 3 weeks later demonstrating Esophagogram obtained 3 weeks later demonstrating stricture (A) and gastroesophageal reflux (R and green arrow). Medical therapy was initiated. Image 2 of 3 in series.
Esophagogram performed 2 weeks later demonstrating Esophagogram performed 2 weeks later demonstrating worsened anastomotic stricture (A). Patient responded well to Bouginage dilatation and medical management of reflux. Image 3 of 3 in series.

GER is clinically diagnosed and may be confirmed with an upper GI series or pH probe. Reflux is initially medically managed. Initial steps include keeping the patient in a prone head-up position after feeding, thickening feeds, and giving smaller more frequent meals. Acid reduction agents (eg, histamine H2 receptor blockers or proton pump inhibitors [PPIs]) and prokinetic agents may be needed. If symptomatic reflux persists, a fundoplication is needed. Fundoplications are required in up to one half of patients with GER after an EA repair. [21]

Tracheomalacia is a condition in which weakness of the trachea results in compression of the anterior and posterior walls between the aorta and dilated esophagus during expiration or coughing. This complication occurs more frequently in the presence of a fistula and is present in 10-20% of infants after an EA-TEF repair.

The region of compression is typically located at or just above the level of the original fistula but may involve the entire trachea. The marked tracheal anterior-posterior collapse is observed easily during bronchoscopy performed while the patient is awake. Tracheomalacia usually improves slowly with time. In some cases, tracheomalacia may prevent extubation, and intervention with aortopexy, tracheostomy, or tracheal stenting is needed.

Esophageal dysmotility is frequent after surgery for congenital esophageal lesions. Food impaction can occur at the level of the anastomosis, especially if a stricture is present (see the image below). Altered esophageal peristalsis has been documented with manometric and radionuclide studies, video fluoroscopy, scintigraphy, and cine esophagraphy after EA repair. Discontinuity of peristaltic function is observed above and below the surgical anastomosis. Dysmotility appears to persist and has been reported in 32-year follow-up studies. Children learn to compensate for the dysmotility by eating in an upright position and drinking frequently during eating.

Esophagogram demonstrating impacted food in 5-year Esophagogram demonstrating impacted food in 5-year-old child with history of esophageal atresia repair.

Esophageal diverticula may develop at the anastomosis or a site where a circular myotomy was performed. The myotomy site may balloon progressively over time and cause ventilatory obstruction and dysphagia.


Long-Term Monitoring

Frequent follow-up visits are necessary during the first year after repair. If the child is doing well, visits can be decreased to once or twice per year until school age. Because of scarring at the anastomosis, the child may tolerate only pureed food up to age 12-18 months and then minced food until age 5 years. At age 5 years, the child has typically learned to chew well before swallowing and has developed sufficient teeth to aid in this task. The child's parents should be informed about the signs of GER, recurrent fistula, tracheomalacia, and other complications.