This most common subtype accounts for about 85% of EA anomalies. The very dilated proximal esophagus has a thickened wall and descends blindly into the superior mediastinum, usually to a point between the second to third or fourth thoracic vertebrae. The distal esophagus is slender and has a thin wall. It enters the trachea posteriorly either at the level of the carina or 1–2 cm higher. The distance between the esophageal ends is variable. Very rarely, the distal fistula may be occluded, leading to the misdiagnosis of EA without distal fistula.

Pure EA has an incidence of about 7%. The proximal and distal esophagus end blindly in the posterior mediastinum. The proximal end is dilated, has a thickened wall, and typically ends at the level of the azygos vein. The distal esophagus is short and often suspended by a fibrotic band. The distance between the two segments is considerable, frequently precluding immediate anastomosis.

H-type TEF without atresia occurs with an incidence of about 4%. The fistula starts from the membranous trachea and runs caudad to enter the esophagus. Normally the fistula is short, although the diameter may be variable. The fistula is usually situated at the thoracic aperture or higher in the neck.

The incidence of a proximal fistula with EA is thought to be about 2% but may be higher than is generally appreciated. In a series of 13 children with EA but without a distal fistula, a proximal fistula was found in 7. An upper esophageal fistula is usually not found at the end of the pouch. This fistula is similar to the H-type, which starts proximally on the trachea and ends distally in the dilated proximal esophagus. Usually, there is only one proximal fistula, but two or three have been described. The fistula is usually located at the thoracic aperture or higher in the neck. Although limited in length, fistula diameter varies. If not diagnosed preoperatively, it may be suspected during operative repair when bubbles are seen on opening the proximal esophagus.

The incidence of EA with proximal and distal fistulas is thought to be <1%. EA with one distal fistula and two proximal fistulas has also been described. Also reported is a near-complete membranous obstruction of the esophagus in conjunction with a single TEF at the level of the membrane, communicating with both parts of the esophagus.

Current prenatal ultrasound (US) technology does not allow for the certain diagnosis of EA/TEF. The positive predictive value of the typical prenatal ultrasonography findings is estimated to be only 44%–56%. A recent study performed at 11 pediatric surgery institutions in the United States found that only 13% of 396 infants with EA anomalies were diagnosed in the prenatal period. The prenatal diagnosis of EA/TEF relies in principle on two nonspecific signs: polyhydramnios and an absent or small stomach bubble. However, polyhydramnios is associated with a wide range of fetal abnormalities and is nonspecific. Similarly, the absence of a stomach bubble on US may point to a variety of fetal anomalies. The combination of a small stomach together with a dilated cervical esophagus (the pouch sign) has been confirmed to be diagnostic for pure EA in a number of patients. Three-dimensional power Doppler imaging has been used both antenatally and postnatally. For example, aortic arch anomalies have been diagnosed using this modality. ^, Magnetic resonance imaging (MRI) has been used to identify other fetal thoracic lesions and may be beneficial in patients deemed to be at risk of EA on prenatal US. Sensitivity in various studies is between 60% and 100%, and the diagnosis is made by the lack of visualization of the thoracic esophagus.

Postnatal passage of a tube or catheter into the stomach should be performed to assess for esophageal patency whenever a pregnancy was complicated by polyhydramnios. The same holds true when the child presents with anomalies that fit the VACTERL association (e.g., radial aplasia). As EA prevents the passage of saliva down the esophagus, saliva accumulates in the proximal esophagus and mouth, and feeding should be withheld until esophageal continuity is confirmed. This is best done with a stiff 10 French catheter gently inserted either through the nose or mouth. Note that smaller-caliber tubes are prone to curling in the upper esophagus, giving a false sense of esophageal continuity. A chest film should be obtained with a small amount of downward pressure on the tube. With EA, the tip of the tube is found to be slightly curled in the blind upper pouch around T2–T4 (Fig. 25.2 ). This technique not only identifies the atresia but gives some clue about the length of the upper pouch. Often, the dilated upper esophageal pouch is also visualized by the air contained within it. Air in the stomach and more distally signifies the presence of a distal TEF. If the tip of the catheter passes beyond the level of the carina, then the diagnosis of EA should be questioned. Esophageal stenosis, tracheal rings, and iatrogenic perforation of the esophagus can be confused with EA. ^, If there is any question about the diagnosis, a small amount of isotonic absorbable contrast (1–2 mL) can be dripped into the upper pouch under fluoroscopy, but this should be done with caution under the direction of a surgeon to minimize the risk of contrast aspiration.

Plain radiograph depicting the classic features seen in an infant with EA and TEF. An oroesophageal tube is seen in the upper pouch and has kinked at the end of the pouch. There is air in the stomach and bowel, which signifies the presence of a distal TEF.

Once the diagnosis of EA has been established, the baby should be transferred to a Level I pediatric surgical center if not already there. The child is positioned head-up, and a 10 French Replogle tube is placed in the upper esophagus and set to continuous suction. Intravenous access is obtained. The operative treatment of EA/TEF is not usually an emergency procedure, and there is time to confirm the diagnosis and assess for associated anomalies. Because EA may be part of a syndrome, consultation by a geneticist is also recommended.

Radiographs may reveal associated anomalies such as vertebral and rib anomalies or other problems such as duodenal atresia (Fig. 25.3 ). The absence of air in the stomach points to EA without distal fistula (see Fig. 25.3A). Mediastinal US has been described as a helpful adjunct in the diagnosis of pure EA. Echocardiography should be performed prior to operation, as it may reveal cardiac and/or aortic arch anomalies. A right descending aorta, which occurs in about 5% of patients, may make a left-sided thoracic approach preferable, although it is still technically feasible to approach via either chest. ^, US and spine radiographs should be obtained as well. Advanced imaging modalities—computed tomography (CT) or MRI—are not typically necessary but can aid in diagnosing cardiac and aortic arch anomalies.

Radiographs depicting less common presentations of EA. (A) Isolated EA. The oroesophageal tube ( arrow ) is seen in the proximal pouch. There is no air in the gastrointestinal tract. (B) EA with distal TEF and duodenal atresia. An oroesophageal catheter ( arrow ) sits in the upper esophageal pouch. An endotracheal tube is also seen. The stomach and bulbous duodenum are distended with air, but no air is seen distal to the duodenum.

A long gap between the two esophageal segments may not be amenable to an initial primary repair. For example, in one report operative management was linked to the measured gap length: fewer than two vertebrae, then primary anastomosis; two to six vertebrae, then delayed primary anastomosis; more than six vertebrae, then esophageal replacement. However, the length of the esophageal gap is usually not known preoperatively. In addition, there are varying definitions of a long gap based upon either the number of vertebral bodies (typically 2–5), the measured gap length (typically 2–5 cm), or simply a surgeon’s intraoperative assessment. Even in long-gap EA (atresia without distal fistula) as defined by the International Network of Esophageal Atresia (INoEA), the gap length can vary.

In newborns with isolated EA, the first procedure is generally a gastrostomy, which allows for both enteral feeding and assessment of the length and location of the lower pouch. To measure the distal esophageal remnant, a guidewire, endoscope, or metal probe can be inserted retrograde through the gastrostomy and advanced into the distal pouch under fluoroscopy. If possible, endoscopic intubation of the distal pouch is preferred because a blindly placed probe can indent the diaphragm in a similar manner to a short distal pouch. The gap length is definitively determined by measuring the distance between the two esophageal ends with the use of a radiopaque ruler. This assessment can be done at the time of the initial gastrostomy, or more routinely, later when the gastrostomy tract has matured. If bougies or an endoscope are introduced into the distal esophagus, the amount of pressure on these instruments will affect the measurement of the gap between the two esophageal segments and may under- or overestimate the gap length.

When these measurements are not possible (e.g., in the absence of gastrostomy), the gap length may not be fully appreciated until the time of exploration. However, it is often necessary to ligate the fistula in the early postnatal period regardless, and the gap length can be assessed at that time. With concern for a long gap, a thoracoscopic approach may be beneficial in that it allows for minimal morbidity if the decision is to ligate the fistula only without esophageal repair. If the lower pouch is identified and found to be of adequate length, a primary repair can be attempted. If not, then a gastrostomy can be placed and delayed repair planned.

Recently, van der Zee and Patkowski have advocated for early thoracoscopic exploration without gastrostomy and the use of an internal traction suture technique to achieve an anastomosis in the first week or two of life in cases of long-gap EA. Although this approach appears promising, other centers have reported significant challenges and complications with the technique, and more study is needed to validate this aggressive early approach.

While not the norm, some infants do require urgent postnatal intervention. If the child is in respiratory distress, endotracheal intubation and ventilation may be needed. Forceful ventilation can overdistend the stomach when a distal fistula is present, potentially causing diaphragmatic splinting and even gastric rupture. ^, Gentle low-pressure ventilation is therefore essential. High-frequency ventilation may also be advantageous. However, on occasion, emergency ligation of the fistula may be needed as a lifesaving maneuver. ^, After ligation of the fistula, a delayed primary repair can be performed when the infant is more stable. Some pediatric surgeons recommend not postponing the second operation longer than 7–14 days because recanalization of the fistula can occur. ^{, ,} Furthermore, to avoid recanalization, some pediatric surgeons advocate for dividing the fistula in all such cases. To prevent retraction of the divided distal esophagus and resultant increase in esophageal gap length, the distal esophagus can be tacked to the prevertebral tissues.

If the infant is extremely unstable, an emergency gastrostomy to decompress the stomach may be the best option. However, gastrostomy can result in a significant loss of tidal volume in the presence of a patent TEF. Placing the gastrostomy to a water seal device to increase the resistance to flow of ventilation across the fistula is a helpful adjunct. Placement of a Fogarty catheter into the fistula via a bronchoscope is another option. Finally, occlusion of the distal esophagus at the gastroesophageal junction is another rescue maneuver available in cases of neonatal distress from inadequate ventilation and/or gastric distention secondary to tidal volume passage through a TEF.

The value of routine preoperative rigid bronchoscopy is much debated because the incidence of a simultaneous proximal and distal fistula is less than 5%. However, a proximal fistula has been found with a much higher incidence in cases of otherwise-presumed pure EA. In cases with a distal TEF, some pediatric surgeons advocate for bronchoscopy to identify the site of the distal fistula to help plan the repair, as the distance to the carina provides a clue as to the length of the gap between the esophageal segments: the closer the fistula is to the carina, the longer the expected gap between esophageal ends. The entrance of the distal fistula is usually well seen (Fig. 25.4 ). Others have advocated for bronchoscopy to place a “blocker” such as a Fogarty catheter to occlude the fistula until operative control is obtained. With the availability of small-diameter flexible fiberscopes, tracheobronchoscopy can now be performed after intubation through the endotracheal tube. ^, However, forceful ventilation must be avoided, not only to avoid lung damage, but also to prevent gastric distention and gastric perforation as described above. Bronchoscopy may also reveal abnormalities such as a laryngotracheoesophageal cleft, tracheal stenosis, or a tracheal bronchus to the right upper lobe. However, an indication of the severity of tracheomalacia is possible only when the child is breathing spontaneously.

Photographs depicting the distal TEF entering the trachea at different levels. (A) The fistula ( asterisk ) enters in the midtrachea. (B) The fistula ( asterisk ) enters at the carina.

The downside of bronchoscopy is that it may prolong the procedure, and the infant can decompensate prior to ligation of the fistula. The surgeon and the anesthesiologist should discuss the advantages/disadvantages of bronchoscopy in each individual case. Following intubation, it may also be helpful to position the endotracheal tube distal to the fistula, provided that the fistula is not at the carina.

The operation is performed with the patient under general anesthesia and with adequate venous access. An arterial line may be beneficial depending on the baby’s clinical status. Generally, a pulse oximeter and end-tidal CO ₂ monitor are adequate. The operation can be performed through a thoracotomy or using the thoracoscopic approach. The side of entrance into the chest has traditionally been opposite the turn of the aortic arch when known preoperatively: right for a left descending aorta, left for a right descending aorta. If a right-sided aortic arch is not detected until the operation has begun, conversion to the left side is appropriate if the thoracoscopic approach was initially chosen. If a thoracotomy has already been performed, an anastomosis from the right chest may be more appropriate, although perhaps with a higher risk of stricture. ^,

In a baby with a left descending aorta, the child is placed in a left lateral decubitus position. A small axillary role is placed under the chest to enlarge the right-sided intercostal spaces. The surgeon stands to the right of the patient (the infant’s back) with the assistant opposite. With a right descending aorta, the baby is placed in a right lateral decubitus position close to the left edge of the table. The surgeon then stands on the left side of the table with the assistant on the right. The patient’s arm is positioned over the head (Fig. 25.5 ). Suction is removed from the Replogle tube, but the tube is left in place so that it can be advanced during the operation to aid in identifying the proximal pouch.

Positioning for a right thoracotomy. The ipsilateral arm is positioned over the head of the patient. A 4–5 cm incision is made 1 cm below the tip of the scapula. (B) A peanut or sterile cotton swab being used to gently push the pleura away from the chest wall.

A slightly curved 4–5 cm long incision is made 1 cm below the inferior tip of the scapula. With the use of a muscle-sparing approach, the auscultatory triangle is opened and the muscles are retracted (i.e., the latissimus dorsi posteriorly and the serratus anterior anteriorly). ^, If the serratus muscle needs to be transected, this should be done as low as possible to preserve the long thoracic nerve. The fourth or fifth intercostal space is then entered.

An extrapleural approach has been suggested to protect the pleural space in case of an anastomotic leak, but there is no evidence that it is better than a transpleural one. ^, However, an extrapleural approach does aid in exposure as it is easier to retract the lung when it is encased within the pleura. With the extrapleural approach, the pleura is gently pushed away from the endothoracic fascia, first in the middle of the incision so that an infant rib spreader can be inserted and opened (see Fig. 25.5B). With the rib spreader opened even farther, the pleura is carefully pushed away even more posteriorly until the posterior mediastinum is exposed.

The azygos vein is an important landmark because the distal TEF is frequently located nearby. The distal fistula may start from the trachea directly underneath the azygos vein, in which case the azygos vein is transected between 3–0 or 4–0 ligatures or simply cauterized and divided (Fig. 25.6 ). If the distal fistula originates more cephalad on the trachea, the vein can be left intact. A relationship between division of the azygos vein and the development of an anastomotic leak has been suggested. ^,

(A) Ligation of the azygos vein. (B) The distal fistula is mobilized from its insertion in the trachea.

The distal esophagus is identified as it distends with each inspiration. The vagus nerve is intimately attached. Once identified, the distal segment should be followed proximally to locate where the fistula enters the trachea. The fistula should be dissected and mobilized close to the trachea, which will spare as many vagal nerve branches as possible (see Fig. 25.6B). The fistula can be encircled with a vessel loop or suture to aid in exposure. There are several ways to ligate the fistula on the tracheal side. In cases in which repair will be deferred, ligation without division can result in a higher recanalization rate as discussed previously. ^, Prior to dividing the fistula sharply, it is beneficial to place traction sutures on each end. A series of 4–0 or 5–0 sutures are used to close the tracheal side, taking care not to compromise the tracheal lumen (Fig. 25.7 ). Another option is to apply a single 5-mm clip across the fistula where it connects to the membranous trachea, which is simple and efficient and may result in a smaller pouch remnant on the posterior tracheal wall. However, there are anecdotal reports of clip migration and recanalization of the fistula using this technique. The intactness of the tracheal closure can be verified by instilling warm saline into the chest and applying a higher ventilation pressure to assess for an air leak.

The distal esophagus is identified, looped, and carefully dissected up to its junction with the trachea, meticulously sparing the segmental vessels from the aorta. (A) Traction sutures may be placed for gentle handling of the segments. The fistula is divided close to the trachea without narrowing its lumen. (B) The tracheal end of the tracheoesophageal fistula is closed with continuous or interrupted sutures. Adjacent tissue, if available, is tacked over the closure.

Reprinted with permission from Spitz, L., & Coran, A. (Eds.), 2013. Esophageal atresia with and without tracheoesophageal fistula. In Operative Pediatric Surgery (7th ed., p. 134). CRC Press

Attention is then turned to the proximal esophagus, which can be identified by asking the anesthesiologist to push on the indwelling Replogle tube. A traction suture is sometimes helpful, taking a stout bite of the top of the muscular proximal esophageal pouch. Using this traction suture, the proximal pouch can be freed posteriorly and laterally by blunt dissection. Anteriorly, however, the pouch is adherent to the membranous trachea. Usually, it can be dissected sharply, staying on the esophageal side to avoid entrance into the membranous trachea. Extensive dissection is not warranted, unless there is a long gap and added length is needed. The dissection can damage the tracheal or esophageal walls and may interfere with innervation to the upper esophagus or even cause injury to the recurrent laryngeal nerve. ^, Extensive dissection of the proximal pouch just to search for a proximal fistula should not be performed routinely because, as previously mentioned, the incidence of a proximal fistula in combination with a distal one is very low. If a proximal fistula were missed at birth and diagnosed after repair of the EA, it can usually be repaired through a neck incision or thoracoscopically at a later date.

After mobilization of the proximal pouch, the tip is amputated so that the lumen and mucosa become visible. An end-to-end anastomosis is performed with 4–0 or 5–0 sutures starting in the middle of the back wall of each esophageal segment (Fig. 25.8 ). It is important to include both the mucosa and the muscular wall with each suture. The sutures in the back wall of the anastomosis are tied intraluminally. Then the front part of the anastomosis is performed with sutures tied extraluminally.

Esophageal anastomosis. (A) The back wall of the anastomosis has been sutured from the inside, and a small orogastric tube has been passed through the anastomosis. The front part of the anastomosis is being sutured with knots on the outside. (B) Completed anastomosis.

Before finishing the front part of the anastomosis, an 8 or 10 French tube can be passed into the stomach. This helps protect the lumen from inadvertent closure and allows for gastric decompression. There has been debate regarding the risks and benefits of leaving this transanastomotic tube in place postoperatively. Advocates of leaving the tube suggest that this gastric access allows for the provision of earlier enteral feeds, as well as a stent across a potential stricture should dilations eventually be necessary. However, a transanastomotic tube has also been associated with an increased risk of anastomotic stricture in multiple studies. A consortium of 11 institutions performed a retrospective review of all EA/TEF cases, open and thoracoscopic, from 2009 to 2014 and found that placement of a transanastomotic tube was associated with an over two-fold increase in the development of esophageal stricture. Similarly, a large single-institution study found a three-fold increase in strictures with use of transanastomotic tubes (56% vs. 17%). A meta-analysis of four retrospective studies (455 patients) confirmed these findings, showing that the use of a transanastomotic tube was associated with a significantly increased risk of stricture (relative risk, 1.83, P < .0005).

The chest incision is then closed in layers. The ribs should be approximated with absorbable sutures. These sutures should be tied gently so that the intercostal space is not obliterated. If a muscle-sparing approach was used, the muscles are allowed to fall back into their normal position, and the skin is closed with absorbable sutures. The use of a chest drain is typical but optional. ^, We usually place a drain and leave it until an esophagram is obtained on postoperative day 5. Obtaining an esophagram on postoperative day 5, instead of waiting until postoperative day 7, does not result in delayed diagnosis of or missed anastomotic leaks. A routine esophagram may not even be necessary in asymptomatic patients with no evidence of an anastomotic leak. ^, Apart from standard perioperative antibiotic prophylaxis, extended postoperative antibiotics are not necessary. Empiric postoperative antibiotic courses after 24 hours were not associated with any lower risk of infection, shock, or death.

The first successful repair was reported in 2000 and the first series reported in 2002. ^, Since then, there have been a number of retrospective reports describing experience with the thoracoscopic approach. Visualization can be challenging given the small working space. Initially attempts were made to obtain single-lung ventilation by intubating the left mainstem bronchus. However, this was often time consuming and unsuccessful, so the endotracheal tube is now more frequently placed in the trachea just above the carina, and right lung collapse is achieved with CO ₂ insufflation alone. Several studies have found that elevated intrathoracic pressure during thoracoscopy in neonates can lead to hypercarbia, acidosis, and decreased regional cerebral oxygen saturation. ^, Some have described using the oscillating ventilator to both effect lung collapse and negate the adverse effects of prolonged hypercarbia.

Positioning

Once the endotracheal tube is secure, the patient is placed in a modified prone position with the right side elevated approximately 30 degrees (Fig. 25.9 ). If there is a right-sided arch, then a left-sided approach is generally used. The baby is positioned near the edge of the table so that the handles of the instruments do not collide with the table. This positioning gives the surgeon access to the area between the anterior and posterior axillary lines for port placement while allowing gravity to retract the lung away from the posterior mediastinum. This arrangement allows excellent exposure of the fistula and esophageal segments without the need for an extra instrument or lung retractor. The assistant should not be situated on the opposite side of the table, as this will place him/her at a complete paradox with the telescope. The scrub nurse can be on either side of the baby depending on the room layout. Because of the fine manipulation necessary, the surgeon and the assistant should position themselves so that they are in the most ergonomic and comfortable positions.

Positioning more prone than lateral for right thoracoscopic repair. The surgeon (S) stands on the left side of the operating table when the aortic arch turns to the left. The surgeon, operative field, and screen are in-line. The assistant and camera holder (SA/C) are situated to the left of the surgeon when the surgeon is right-handed. The scrub nurse (SN) stands to the right of the operating table. M, monitor; A, anesthesiologist.

From Holcomb GW, Rothenberg SS, Georgeson KE. Atlas of Pediatric Laparoscopy and Thoracoscopy . Elsevier; 2009.

Surgical Technique

Port placement is extremely important because of the small chest cavity and the intricate nature of the dissection and reconstruction. Usually three ports are satisfactory, but a fourth one can be used if needed (Fig. 25.10 ). The initial port (3 or 4 mm) is placed in the fifth intercostal space behind the tip of the scapula. This is the camera port and allows for excellent visualization of the posterior mediastinum. An angled telescope (30 degrees or 45 degrees) is essential. The two instrument ports are then introduced. The first is in the midaxillary line one or two interspaces above the telescope port in the axilla. This cephalad port is 5 mm (if a clip applier will be used) or 3 mm and serves as the access site for the clip applier (if used) and the needle driver/suture. The lower port is 3 mm and is located two interspaces below the camera port and almost directly below it or even slightly posterior to it. Ideally, these ports are positioned so that the instrument tips will approximate a 90-degree angle at the level of the TEF, which will facilitate performing the anastomosis.

Optimal port positions for a thoracoscopic repair. The ports are inserted in triangulation to allow the working instruments to meet at 90 degrees. The camera port is inserted just below and posterior to the tip of the scapula, which is also the site for insufflation. One working port (step, in photograph) is inserted in the midaxillary line in the axilla. The second working port ( arrow ) is positioned just below and slightly posterior to the camera port.

Once the lung is collapsed, the surgeon should first identify the fistula. In most cases, the fistula is attached to the membranous portion of the trachea just above the carina, which is usually near the azygos vein. After the azygos is identified, it is mobilized for a short segment and then cauterized and divided with a small hook cautery or the 3-mm vessel sealer. As mentioned earlier, some advocate leaving the azygos intact if possible.

With the vein divided or retracted, the lower esophageal segment is identified and followed proximally to the fistula. Because of the magnification afforded by the thoracoscopic approach, it is easier to visualize exactly where the distal fistula enters the back wall of the trachea (Fig. 25.11 ). A 5-mm endoscopic clip can then be applied safely. Care should be taken to avoid the vagus nerve. A single clip is usually sufficient. The fistula can then be divided with scissors. On the other hand, the distal segment can retract, making it difficult to visualize, so it may be preferable to wait until the upper pouch is mobilized before completely dividing the fistula. Alternatively, the fistula can also be suture ligated or cut and oversewn with interrupted sutures as has been done traditionally in the open repair. However, this requires delicate suturing at a time when an air leak from the opened fistula may be causing respiratory compromise.

(A) After ligation and division of the azygos vein ( arrow ), the tracheoesophageal fistula has been mobilized and is encircled with an angled dissecting instrument. The upper esophageal pouch ( asterisk ) was mobilized before ligation and division of the fistula. (B) The tracheoesophageal fistula is being ligated with a 5-mm endoscopic clip applier. Two clips are usually applied, and the fistula is divided distal to the second clip. The upper esophageal pouch is marked with an asterisk.

Attention is then turned to the thoracic inlet, and the anesthesiologist places pressure on the Replogle tube to help identify the upper pouch. The pleura overlying the pouch is incised sharply, and the pouch is mobilized using blunt and sharp dissection. The plane between the esophagus and trachea is easily seen, and the two are separated. Mobilization of the upper pouch can be carried out well into the thoracic inlet.

Once adequate mobilization has been achieved, the distal tip of the pouch is resected, exposing the mucosa. With the two esophageal segments mobilized, the anastomosis is performed using 4–0 or 5–0 sutures on a small, tapered needle. The sutures are placed one at a time in an interrupted fashion, back wall first and then the front wall after the nasogastric tube is advanced, as described for the open operation (Fig. 25.12 ). Once the anastomosis is completed, the transanastomotic tube is removed, a chest drain is typically introduced through the lower port site, and its tip is positioned near the anastomosis. The other ports are removed, and the sites are closed with absorbable sutures.

Esophageal anastomosis. (A) The posterior suture line has been completed with interrupted sutures. A small silastic tube was guided through the anastomosis and into the stomach. (B) The completed anastomosis is seen.

From Holcomb GW III, Rothenberg SS, Georgeson KE. Atlas of Pediatric Laparoscopy and Thoracoscopy . Elsevier; 2009.

The timing of the esophageal reconstruction is debatable and often depends on the gap length. A period of a few weeks to several months has been advocated. ^, During this waiting period, the proximal esophagus is emptied by continuous suction through a 10 French Replogle tube. If the child has persistent respiratory problems, a proximal fistula should again be considered. Cervical esophagostomy should be avoided at all costs, as this will likely jeopardize the ability to perform a primary esophagoesophagostomy in the future. Home care during the waiting period has been described, but most of the reported patients have instead required a prolonged inpatient stay. ^,

The gap between the esophageal segments should be periodically measured and is usually expressed in terms of the number of vertebral bodies, thus taking the child’s length into account. Measurement can be done with a simultaneous contrast study of the upper and lower esophagus or by insertion of a metal dilator transorally into the upper esophagus and through the gastrostomy into the lower esophagus. Others have advocated for inserting a gastroscope through the gastrostomy site and directing it into the distal pouch to verify it is correctly identified. Timing of the repair is often determined by these “gap studies,” and each surgeon has her/his own criteria for attempting the repair. In general, a period of 4–8 weeks is reasonable. It seems significant added “growth” after this is unlikely to occur, although some do choose to wait longer.

At the time of definitive repair, several techniques to lengthen the native esophagus have been described, including esophageal myotomy or extensive mobilization of the proximal and distal esophagus (Box 25.1 ). There is little doubt that all these maneuvers damage the esophagus and that the long-term results may be less than optimal. If a delayed primary anastomosis with or without lengthening is simply not feasible, an alternative procedure should be considered, such as a gastric transposition/pull-up or a jejunal, ileal, or colonic interposition. However, the need for these procedures is rare. In several series, all long gaps were successfully closed without esophageal replacement. ^{, , ,} In one study examining 258 consecutive patients who were treated with EA from 1980 to 2013, 16% required reoperations for a variety of complications, but more than 90% of these patients survived with a functioning native or reconstructed esophagus.

There is much debate on the use of mechanical traction (Foker process), either external or internal, and its benefit in bringing the two esophageal segments together, thereby reducing the incidence of anastomotic complications. The mean traction time in one study was 3 weeks, and the mean number of thoracotomies required was 2.1. In a study from Boston Children’s Hospital, 52 patients underwent the external Foker technique. The Foker process was used in 27 patients primarily and 25 patients secondarily. The median time to anastomosis was 14 days for the primary group and 35 days for secondary cases. This process was advantageous for the primary group but less so for those requiring revisional procedures. In a meta-analysis examining mechanical traction, the authors found 71 infants who underwent the Foker process and compared them to 450 children from 44 studies in whom delayed primary anastomosis was performed. The Foker process was associated with a significantly lower risk of complications (leak, stricture, reflux) and a significantly shorter time to definitive anastomosis. In this review, the use of mechanical traction was, at the very least, as effective as delayed primary anastomosis for the management of long-gap EA. Proponents of the Foker technique have evolved their technique over time to include both open and minimally invasive approaches, external or internal traction, and eventual right- or left-sided repairs.

Thoracoscopy may be an ideal starting point to further evaluate the gap length to be bridged and determine next steps (Fig. 25.13 ). The ends can be dissected and mobilized, and an anastomosis can be performed thoracoscopically when feasible. If an anastomosis is not feasible, the procedure can be aborted to allow for potential additional growth. Alternatively, a thoracoscopic Foker procedure with internal traction sutures can be performed at that time. ^,

Thoracoscopic view of esophageal atresia without fistula in a 3-month-old neonate. (A) The short distal esophageal segment ( asterisk ) is seen in the inferior portion of the mediastinum. (B) The proximal pouch ( asterisk ) reaches to the azygos vein, which has been coagulated and transected.

Finally, magnetic compression anastomosis has recently been introduced as a nonsurgical or at least minimally invasive treatment for EA. This technique uses attraction between magnets placed in the proximal and distal esophagus to lengthen the esophageal ends and then through pressure necrosis create an anastomosis after apposition of the magnets. Esophageal anastomosis using magnets has been described for cases of pure EA (and less commonly for EA with distal fistula after ligation and division of the fistula). In some cases, thoracoscopic visualization and traction suture placement have aided in creation of the magnetic anastomosis. Nearly all patients develop an anastomotic stenosis requiring multiple dilations after utilization of magnetic anastomosis devices. However, most patients have been able to maintain their native esophagus and have eventually converted to an oral diet. Two magnetic devices are now available, the newer of which may have a lower risk of anastomotic stenosis. ^, This new approach appears to be a safe and feasible alternative to EA repair in properly selected cases.

An overall leak rate of 3.5%–23% has been reported. ^{, , ,} In the largest thoracoscopic report, the leak rate was 7.6%. Published reports often include all types of EA and do not always mention whether the leak was detected at routine esophagography or based on clinical presentation. Major leaks that require active intervention occur less frequently—3.5% in one study and 4.5% in another one —which confirms the concept that most leaks will close spontaneously. The anticholinergic medication glycopyrrolate has shown promise in facilitating the resolution of anastomotic leaks after EA repair. ^, For persistent leaks that fail conservative management, the use of endoluminal negative pressure vacuum-assisted closure (VAC) devices to facilitate resolution of the anastomotic leak has gained popularity.

As with anastomotic leaks, no uniform definition has been used for an anastomotic stricture. It has been defined as a narrowing of >50% of the lumen, as a narrowing detected on a contrast study, or at esophagoscopy in combination with symptoms. Reported incidences range from 17% to 60%. ^{, , ,} In a large thoracoscopic series, 3.8% of patients developed a stricture, which was defined based on the initial esophagogram. Anastomotic tension, anastomotic leakage, placement of a transanastomotic tube, and gastroesophageal reflux (GER) have been implicated as risk factors. In a study whose goal was to identify risk factors for predicting the development of anastomotic stricture following EA repair, 35 patients operated on over an 8-year period were evaluated with routine endoscopy performed 1 month after repair. The authors found that patients with GER and anastomotic tension, as well as long-gap EA, had an increased risk of developing an anastomotic stricture.

Routine postoperative dilation is not indicated because many patients never require dilatation. Symptomatic strictures usually respond well to dilation, steroid or mitomycin injection, and/or advanced endoscopic techniques such as needle knife. Resection of the stricture is rarely required. Balloon dilatation seems superior to bouginage dilation, but clear evidence is lacking.

The reported incidence of a recurrent TEF varies between 3% and 15%. A 2014 review of 1-year outcomes from the French National Esophageal Atresia registry reported a recurrent TEF rate of 4%. In one study, a 10% incidence of recurrent TEF in the period 1986–1995 dropped to 5% in the period 1996–2005. Recently, the Midwest Pediatric Surgery Consortium similarly found a rate of approximately 5% for recurrent TEF. ^, In a large multiinstitutional study of thoracoscopic repairs, the incidence was 1.9%. The etiology of a recurrent fistula is almost certainly related to an anastomotic leak.

A recurrent fistula is suspected when the child starts to cough during feeding, has apneic or cyanotic episodes, or has repeat respiratory infections. The diagnosis can sometimes be made by esophagram using a water-soluble contrast medium (Fig. 25.14 ). However, bronchoscopy is necessary to definitively exclude a recurrent fistula and should be performed despite a negative esophagram in patients with consistent symptoms. Treatment of a recurrent TEF can be difficult. Thus, attempts at prevention have been primarily described. Interposition of a biosynthetic patch between the tracheal closure and esophageal anastomosis has been described for this purpose (Fig. 25.15 ). However, one multiinstitutional study found that placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased risk of leak and no difference in the risk of recurrent fistula. Native tissue such as pleura, pericardium, or intercostal muscle are preferable.

On this water-soluble contrast study, a recurrent TEF ( arrow ) is seen.

Surgisis interposition between the tracheal closure and esophageal anastomosis as a means to prevent a recurrent TEF after thoracoscopic repair of esophageal atresia and distal fistula.

From St. Peter SD, Calkins CM, Holcomb GW III. The use of biosynthetic mesh to separate the anastomoses during the thoracoscopic repair of esophageal atresia and tracheoesophageal fistula. J Laparoendosc Adv Surg Tech 2007;17:380–382. Reprinted with permission.

When a recurrent TEF develops, attempts at endoscopic management with cautery, fibrin glue, chemocauterization, and small intestine submucosa plugs have been described (Fig. 25.16 ). The results are mixed in these small series with success rates reported between 20% and 80%. One report described the application of 50% trichloroacetic acid to 12 recurrent TEFs. All closed with a mean number of applications of 1.8 over a median follow-up of 41 months. Surgical repair of recurrent TEF has often utilized rotational pexy of the trachea and/or esophagus to separate the suture lines at the site of repair.

Bronchoscopic view showing surgisis ( asterisk ) placed into a recurrent TEF to occlude the fistula.

Recurrent laryngeal nerve paralysis or paresis is quite common after EA repair, with recent studies showing a 3%–11% risk of vocal cord dysfunction. ^{, , ,} Although the exact etiology of the paralysis/paresis is difficult to assess, this complication is generally thought to occur from retraction against or stretching of the recurrent laryngeal nerves during EA repair. Although long-term paralysis is possible from direct injury to the nerves, most cases are instead consistent with a nerve paresis with expected long-term resolution of the vocal cord dysfunction. The risk of vocal cord dysfunction is especially high in H-type TEF, occurring in up to 50% of cases. ^, Preoperative laryngoscopy or bronchoscopy to identify infants with congenital vocal fold paralysis prior to operative repair may be beneficial, especially in patients requiring revision surgery. Intraoperative nerve monitoring has also been described to decrease the risk of recurrent laryngeal nerve injury.

Thoracotomy, especially in the newborn, can lead to significant morbidity, such as winged scapula, elevation or fixation of the shoulder, asymmetry of the chest wall, rib fusion, scoliosis, and breast and pectoral muscle maldevelopment. These negative consequences of a thoracotomy can be alleviated by using the thoracoscopic approach or by using a muscle-sparing approach to the thoracotomy.

Tracheomalacia is a generalized or localized weakness of the trachea that allows the anterior and posterior walls to come together during expiration or coughing. The area of collapse is usually in the region of the fistula. The cartilage of the rings is softened, and the length of the transverse muscle is increased. As a result, the airway collapses during expiration, which produces expiratory stridor varying from a hoarse barking cough to acute life-threatening episodes of cyanosis or apnea. In EA without fistula, tracheomalacia does not commonly occur.

Treatment is required in symptomatic infants with severe tracheomalacia. The therapy of choice is aortopexy. The principle behind this operation is that if the ascending aorta and arch are suspended against the posterior surface of the sternum via aortopexy sutures, the anterior wall of the trachea, which is loosely attached to the aorta, is suspended as well. This anterior aortic suspension opens the tracheal lumen. Aortopexy is classically performed through a left anterolateral thoracotomy or median sternotomy but can also be performed through a low cervical incision and partial sternal split, or thoracoscopically either from the left ^, or from the right. It seems best to check the effect of the suspension with simultaneous tracheoscopy.

Disturbed motility of the esophagus is a significant long-term sequela in EA patients. Symptoms include dysphagia, episodes of foreign body impaction, heartburn, and vomiting. The oral phase of swallowing is normal, but the pharyngeal and esophageal phases are abnormal in all patients, both on videofluoroscopy ^, and with manometry.138 ^, Whether the cause of this disturbed motility is congenital or acquired has been a long-standing debate. Whatever the cause, it brings with it several problems, not the least of which is GER.

Although GER tends to improve during the first year of life in most infants, this may not be the case in infants with EA. The incidence of significant reflux in patients with EA has been stated to be up to 50%, and prophylactic acid suppression has traditionally been recommended for at least the first year of life in infants with EA. While GER is thought to contribute to the development and persistence of anastomotic strictures, recent studies have found that the use and duration of postoperative acid suppression were not associated with the development of strictures. ^, About half of those with GER historically required antireflux surgery. ^{, , ,} However, antireflux operations in patients with repaired EA have a higher failure rate than in non-EA patients. While some surgeons have advocated for partial wraps because of concerns over dysmotility, there is little evidence to support this practice. A review of 3479 patients with EA/TEF using the Pediatric Health Information System (PHIS) database found that 12% underwent fundoplication within 2 years of discharge.

A considerable number of patients with EA have complaints in adult life pointing toward GER as the etiology. Early normal pH values in the esophagus do not exclude significant reflux on follow-up. Antireflux medication, including gastric acid suppression, is successful in only about half of cases. The long-term sequelae of acid exposure in patients with prior esophageal atresia is significant, with a four-fold higher risk of Barrett’s esophagus and an over 100-fold increase in the risk of esophageal squamous cell carcinoma. ^, In one study of 51 patients followed over an 18-year period with regular pH probes and endoscopic investigations, esophageal metaplasia was found in 15% of patients, and the lag time for developing metaplasia from the time of the initial operation was found to be about 10 years. For these reasons, current guidelines recommend lifelong endoscopic surveillance among those who underwent EA repair as a child, including throughout childhood (after stopping acid suppression, before the age of 10 years, and at transition to adulthood). ^,

Patients with persistent symptoms of dysphagia, reflux, food impaction, and stricturing despite standard treatment of GER should be endoscopically evaluated for eosinophilic esophagitis. Recent studies have shown that eosinophilic esophagitis develops frequently in children with EA/TEF, likely related to underlying esophageal dysmotility and poor esophageal clearance. Eosinophilic esophagitis has been found in 12%–17% of patients with prior EA/TEF, which is significantly higher than the general population. ^, Treatment of eosinophilic esophagitis in children with EA/TEF is similar to treatment in the general population, including dietary modifications (elimination diets or elemental diets), oral/topical steroids, and proton pump inhibitors.