An understanding of the anatomy involved with each case of EA and TEF is important when devising a treatment strategy. There have been several classification systems, but a description of each type is the easiest and most practical way to classify the five different types of EA and TEF as shown in Fig. 11.1. The most common configuration is EA with a distal TEF. This configuration occurs in 86 % of cases [6]. The proximal esophagus ends blindly in the upper mediastinum. The distal esophagus is connected to the tracheobronchial tree usually just above or at the carina. The second most common type is the isolated EA without a TEF. This configuration occurs in 8 % of cases [6]. The proximal esophagus ends blindly in the upper mediastinum, and the distal esophagus is also blind ending and protrudes a varying distance above the diaphragm. The distance between the two ends is often too far to bring together shortly after birth. The third most common configuration, occurring in 4 % of cases [6], is a TEF without EA. The esophagus extends in continuity to the stomach, but there is a fistula between the esophagus and the trachea. The fistula is usually located in the upper mediastinum running from a proximal orifice in the trachea to a more distal orifice in the esophagus. This is also known as an “H”-type or “N”-type TEF. Two more forms of EA and TEF exist, both of which occur about 1 % of cases [6]. These are EA with both proximal and distal TEF and EA with a proximal TEF. These two forms correspond to the first two forms described with the addition of a proximal fistula between the upper pouch and the trachea. A proximal fistula is often difficult to diagnose preoperatively even when bronchoscopy is performed, resulting in the real incidence being higher than previously reported [7]. Again the EA with proximal TEF, similar to its counterpart without the proximal fistula, will have a long gap between the two ends of the esophagus, making it difficult to repair shortly after birth.

As noted earlier, prematurity and associated cardiac and chromosomal anomalies often determine the outcome of a baby with EA and TEF. Babies with EA and TEF are often born prematurely due in part to the polyhydramnios resulting from fetal esophageal obstruction [8]. Recent series revealed a mean gestation age of 36.5 weeks with 3.5 % being born between 24 and 29 weeks estimated gestation age (EGA), and 38.3 % between 30 and 36 weeks EGA [9], and a birth weight of 2500 g [10]. Up to two thirds of EA and TEF babies have one or more associated anomalies that are usually chromosomal or related to the VACTERL association [9, 11]. The VACTERL association includes abnormalities in the following areas: vertebral, anorectal, cardiac, tracheal, esophageal, renal, and limb. A breakdown of the individual incidences of the anomalies in babies with EA and TEF is presented in Table 11.3 [9]. The VACTERL syndrome requires three or more of these abnormalities and occurs in up to 33 % of babies with EA and TEF [9]. Chromosomal abnormalities occur in 5 % and include trisomies 13, 18, and 21 [12]. Other syndromes associated with EA and TEF include the Feingold syndrome, CHARGE syndrome, anophthalmia-esophageal-genital (AEG) syndrome, Pallister-Hall syndrome, Opitz syndrome, and Fanconi’s anemia [13]. Infants with EA and TEF also have a higher incidence of pyloric stenosis compared to the normal population [14].

Prenatal diagnosis of EA remains difficult with only 16–32 % of babies born with EA and TEF having the diagnosis before delivery [15, 16]. The use of fetal ultrasound to screen for polyhydramnios and a small or absent stomach followed by MRI looking for a dilated upper esophageal pouch for confirmation can lead to a correct diagnosis of EA and TEF in 66.7 % of those suspected [17]. The majority of babies diagnosed prior to delivery will have pure EA as it is more difficult to diagnose EA with a distal TEF [16, 18]. The vast majority of cases of EA and TEF are initially diagnosed shortly after birth with inability to handle saliva and episodes of coughing, choking, and cyanosis, especially with the first attempt to feed. This usually leads to the placement of a tube in the esophagus, which meets resistance. Plain films of the chest and abdomen will show the tube coiled in the upper mediastinum. This confirms the presence of esophageal atresia. If there is gas in the distal bowel, a distal TEF is present, while a pure EA will present with a gasless abdomen. The remainder of the preoperative evaluation targets the associated anomalies and looks to determine the presence of a proximal fistula between the trachea and the esophagus. The VACTERL anomalies can be identified using physical exam (limb and anorectal), plain films (tracheal, esophageal, vertebral, and limb), abdominal ultrasound (renal and a tethered spinal cord), and an echocardiogram (cardiac). The position of the aortic arch needs to be identified during the echocardiogram. If a right-sided arch is present, which occurs in 2–6 % of series [19], evaluation with a CT scan or an MR angiogram will find a complete vascular ring 37 % of the time [20]. A chromosome analysis should also be considered. A proximal pouch fistula can be interrogated in two ways. A pouchogram, or contrast evaluation of the proximal esophageal pouch, will often reveal a proximal fistula if present. An experienced radiologist should perform this exam with 1 or 2 ml of contrast material to decrease the risk of aspiration. Rigid bronchoscopy looking for a proximal fistula just prior to surgical repair is often used in conjunction with the pouchogram. During the dissection of the proximal pouch, the surgeon should always look carefully for a proximal fistula. If the proximal pouch is not thick walled and dilated, there may be a proximal fistula that has relieved the usual distending pressure in the proximal pouch. A TEF without EA (H-type fistula) may not present in the initial neonatal period and is more difficult to diagnose. The tube will go into the stomach when originally passed, but persistent coughing and choking with feeds by mouth should prompt a search for an isolated fistula. A prone pullback esophagram and bronchoscopy with esophagoscopy are used to identify the isolated fistula.

After the diagnosis is confirmed, plans for operative repair should be made. In healthy newborns, the operation can take place within the first 24 h of life to minimize the risk of aspiration and resulting pneumonitis. Before the operation, the baby should be kept supine with the head elevated 30–45°. A tube should be in the proximal pouch to constantly suction saliva and prevent aspiration. Intravenous access should be established and fluids instilled along with perioperative antibiotics and vitamin K.

The goal of operative therapy for EA and TEF is to establish continuity of the native esophagus and repair the fistula in one setting. Most of the time, primary repair can be achieved. There are special situations where this may not be possible or advisable. These situations will be described later. In the usual scenario, the baby, who is stable both hemodynamically and from a pulmonary standpoint, is brought to the operating room and placed under a general anesthetic. Rigid bronchoscopy may be performed to locate the distal fistula, usually at or near the carina, look for a proximal fistula or cleft, and assess for tracheomalacia. The baby is then placed in the left lateral decubitus position in preparation for a right posterolateral thoracotomy. If the preoperative echocardiogram reveals a right-sided aortic arch, which occurs in 2–6 % of cases, the repair should be approached from the left chest [19, 21]. Attempting to bring the ends of the esophagus together over a right-sided aortic arch results in a high anastomotic leak rate in the range of 40 % due to increased tension [22]. The preoperative echocardiogram identifies a right-sided aortic arch correctly in 20–62 % of the cases [20, 22]. A right-sided arch discovered intraoperatively should prompt an attempt at repair of the esophagus through the right chest. If this cannot be completed due to tension, divide the fistula, close the right chest, and complete the anastomosis through a left-sided thoracotomy.

In the typical case, a right-sided posterolateral thoracotomy using a muscle-sparing, retropleural approach gives access to the mediastinal structures. An extrapleural axillary approach provides another option to gain exposure. The azygos vein is divided, revealing the tracheoesophageal connection. The distal esophagus is divided, and the tracheal connection is closed with 5-0 monofilament suture. Manipulation of the distal esophagus is minimized to protect the segmental blood supply to this portion of the esophagus. The proximal esophagus has a rich blood supply coming from the thyrocervical trunk and may be extensively dissected as depicted in Fig. 11.2. The dissection of the upper esophageal pouch proceeds on the thickened wall of the esophagus to prevent tracheal injury. Dissection is carried as high as possible to gain length for a tension-free anastomosis and to look for a proximal fistula, which occurs rarely. A single-layered end-to-end anastomosis is performed as depicted in Fig. 11.3. A tube placed through the anastomosis into the stomach allows decompression of the stomach and eventual enteral feeding. A chest tube placed in the retropleural space next to the anastomosis controls any subsequent leak. Some surgeons prefer not to use a chest tube if the pleura remains intact. The advantage of a retropleural approach is that if the anastomosis leaks, the baby will not soil the entire hemithorax and develop an empyema. A leak into the retropleural space will result in a controlled esophagocutaneous fistula that will almost always close spontaneously.

Since the first thoracoscopic repair of EA by Lobe in 1999, this approach has become increasingly popular [23]. Visualization of the posterior mediastinum depends on patient position and creation of a pneumothorax. The baby is placed in a semi-prone position elevating the right chest about 30°. The camera trocar avoids competing with the working ports by being placed just posterior to the scapula tip in the fifth intercostal space. The two working ports are then placed two interspaces above the midaxillary line and one to two interspaces below and slightly posterior to the camera port. This allows the instruments to meet at a 90° angle at the site of the anastomosis [24]. Insufflation of CO₂ gas at a flow of 1 L/min and a pressure of 4 mmHg provides an adequate working space. The improved visualization allows for a precise division of the fistula at the membranous portion of the trachea and an extensive mobilization of the proximal fistula up into the neck while preserving the recurrent laryngeal nerves. Suture placement proves to be the biggest challenge especially when the ends of the esophagus are brought together under tension. Advantages of the thoracoscopic approach include better cosmetic results, improved musculoskeletal function of the thorax, and improved visualization. Disadvantages include a steep learning curve even for those with excellent thoracoscopic skills, difficulty in small (<2,000 g) infants or in neonates with significant cardiac or pulmonary disease, and the potential impact of elevated pCO₂, acidosis, and cerebral hypoperfusion from prolonged carbon dioxide pneumothorax [25]. A meta-analysis comparing thoracoscopic and open repair of EA with or without TEF found no difference in leakage rate or anastomotic strictures [26].

Postoperatively, the baby is returned to the intensive care unit and continued on intravenous nutrition and antibiotics. Special care should be directed toward preventing aspiration with frequent oropharyngeal suctioning and elevation of the head of the bed 30–45°. Feedings may be started through the transanastomotic tube into the stomach 2–3 days after the operation. Acid-suppressive therapy should be instituted to prevent acid irritation of the anastomosis and subsequent stricture. On postoperative day 5–7, an esophagram is obtained to check the integrity of the anastomosis. Feeds are initiated orally, and if there is no leak clinically or radiographically, the chest tube is removed. If a leak is present, it is treated conservatively with intravenous antibiotics, nutrition, and chest tube drainage. Another esophagram is ordered in a week. These leaks will invariably close without further operative intervention [27]. Only a complete disruption of the anastomosis requires further operative procedures. In that case the proximal esophagus should be brought out of the left neck as a cervical esophagostomy, the distal esophagus should be tied off, and the mediastinum and chest should be adequately drained.

Three unique situations require different tactics: babies with EA and TEF with concomitant respiratory insufficiency where the fistula contributes to the ventilator compromise, long-gap EA, and H-type TEFs. Babies with respiratory insufficiency and a TEF are usually premature neonates with lung immaturity requiring significant ventilatory support. The connection between the trachea and the distal esophagus may be the preferred path for air provided by the ventilator. The stiff lungs have a higher resistance than the fistulous tract, allowing a significant portion of each inspiratory volume to go into the distal esophagus and then the stomach, resulting in abdominal distention and elevation of the hemidiaphragms, further impeding ventilation. Various strategies have been developed to deal with this situation. A change to high-frequency ventilation decreases the portion of tidal volume lost to the fistula [28]. A number of techniques designed to prevent ventilation through the fistula have been proposed: advancing the endotracheal tube past the fistula opening [29], bronchoscopically placing and inflating Fogarty catheters for temporary occlusion [30], and temporarily occluding the esophagus at the gastroesophageal junction [31]. If a gastrostomy tube is present, the tube can be placed to underwater seal to increase the resistance of the tract and reduce airflow through the fistula [6]. However, to prevent further respiratory decompensation, and to ameliorate the risk of gastric perforation, these babies often require an urgent thoracotomy and control of the TEF. If the baby stabilizes, the remainder of the repair can proceed at that time, which is the usual case [32]. However, if the baby remains unstable, the esophagus is secured to the prevertebral fascia, the chest is closed, a gastrostomy tube is placed, and the definitive repair is completed when the baby is stabilized. In very low birth weight infants, less than 1500 g, a staged repair should be considered. Compared to a primary repair in these very low birth weight infants, staged repair resulted in fewer anastomotic leaks and strictures [33].

The second special situation occurs when there is a long gap between the two ends of the esophagus. This often occurs with pure EA or EA with a proximal TEF. On occasion, a baby with EA and distal TEF may fit into this special group. If the baby presents with a gasless abdomen, a long gap should be suspected. The baby is brought to the operating room for a gastrostomy tube placement to allow enteral feedings while waiting for the two ends of the esophagus to grow spontaneously, so a primary anastomosis can be attempted. The stomach is quite small in these babies because it was unused during fetal life and has not yet stretched to its full capacity. Care must be taken to avoid injury to the small stomach and its blood supply while placing the gastrostomy tube. Careful placement will not compromise the use of the stomach for an esophageal replacement if necessary. During gastrostomy tube placement, an estimate of the distance between the two ends of the esophagus is made using a neonatal endoscope in the distal esophagus and fluoroscopy. If the two ends of the esophagus are more than three vertebral bodies apart, they will not be easily connected. The baby is then nursed with a tube in the proximal pouch to remove the saliva and is fed via the gastrostomy tube. During the first several months of life, the gap between the two ends of the esophagus shortens because of differential growth of the atretic esophagus [34]. The upper pouch may undergo serial dilation attempting to stretch the pouch [35]. The gap distance is measured every 2–4 weeks, and, if the two ends are within two to three vertebral bodies, a thoracotomy and attempt at anastomosis are performed. Waiting longer than 4 months rarely provides extra growth of the esophageal ends. The gap will close to within two to three vertebral bodies in close to 70 % of these babies [36]. Intraoperatively, several techniques help gain length on the esophageal ends if needed. These include complete dissection of the upper pouch to the thoracic inlet. A circular myotomy of livaditis performed on the upper pouch produces about 1 cm of length for each myotomy as depicted in Fig. 11.4 [37]. A tubularized graft of the upper pouch can be created and connected to the distal esophagus [38]. If these techniques do not allow an adequate anastomosis, the distal esophagus is mobilized, despite its segmental blood supply, to gain length [6]. If these maneuvers do not allow an adequate anastomosis, then one of three options must be chosen. The first option is a two-stage procedure in which a cervical esophagostomy is initially created in the left neck followed by an esophageal substitution at a later time. The esophagostomy will allow the baby to take sham feeds to prevent oral aversion without the risk of aspiration while awaiting esophageal replacement. The replacement operation usually takes place between 9 and 12 months of age. The second option is a one-stage esophageal-substitution procedure using a gastric transposition, a gastric tube, or a colon interposition to replace the native esophagus. Currently, a gastric transposition is our preferred approach and has been shown to be a reliable and reproducible procedure at other centers worldwide [39]. A third option for esophageal reconstruction in long-gap esophageal atresia involves the placement of traction sutures on both ends of the esophagus and either attaching them under tension to the prevertebral fascia if the gap is moderate length or bringing them out through the back and increase the tension on them sequentially over the ensuing 2 weeks (Foker technique). A delayed primary anastomosis is carried out after the two ends of the esophagus are in close proximity [40]. Although the Foker technique allows for a primary repair, it requires multiple thoracotomy incisions, is associated with a very high stricture rate, and invariably requires a gastric fundoplication procedure to control gastroesophageal reflux [41]. Thoracoscopic techniques have been introduced for both the gastric transposition [42] and the Foker technique [43].

The third special situation is the H-type tracheoesophageal fistula without esophageal atresia. An H-type fistula will often escape the discovery in the neonatal period but will be found later during evaluation of coughing and choking episodes with feeds. Often the fistula is identified by contrast studies, usually a prone pullback esophagram as shown in Fig. 11.5. However, it is not unusual to also require bronchoscopy and esophagoscopy to make the diagnosis. To repair this fistula, rigid bronchoscopy and esophagoscopy are used to find the fistula, place a glidewire through it, and bring the two ends out of the mouth to aid in its identification during the exploration. The right neck is then explored through an incision just above the clavicle. The fistula is identified and divided. If possible, the muscle or other available vascularized tissue is placed between the two suture lines to help prevent a recurrence.