Congenital Anomalies of Esophagus 

Updated: Mar 23, 2018
Author: Robert K Minkes, MD, PhD; Chief Editor: Eugene S Kim, MD, FACS, FAAP 

Overview

Background

Congenital anomalies of the esophagus occur in as many as 1 per 3000-5000 births, with esophageal atresia (EA) and tracheoesophageal fistula (TEF) being the most common types (and, therefore, the types that receive more emphasis). Congenital stenosis or obstruction is also encountered. Congenital muscular hypertrophy, webs, cysts, and tracheobronchial remnants are observed.[1]  Other lesions, such as congenital esophageal stenosis, duplications, and cysts, occur less frequently.

The prognosis and treatment course for infants with EA and/or TEF and other congenital lesions have improved over the past 70 years. Advances in perinatal and neonatal care have been paramount in reducing the morbidity and mortality associated with these conditions. Currently, associated congenital anomalies and pulmonary complications contribute most significantly to adverse outcomes.

Infants with very low birth weight or serious cardiac abnormalities are at increased risk for poor outcome.[2] Improvements in the prevention and management of these high-risk infants would improve outcome and survival rates. In addition, enhanced prenatal detection of EA and/or TEF and other congenital anomalies allows for better prenatal counseling and preparation for the delivery at a tertiary medical center.

Esophageal defects are currently repaired with thoracoscopy; robotic-assisted surgery may be used in the future.[3]

Tissue engineering for esophageal replacement, in utero intervention, and minimally invasive techniques such as thoracoscopy and robotic assistance may be used in years to come to further improve treatment of these infants.

For patient education resources, see the Digestive Disorders Center, as well as Choking.

Anatomy

The esophagus can be divided into several segments on the basis of its blood supply. The cervical portion of the esophagus is well vascularized and thought to have good intramural vascular communications. The cervical esophagus is supplied by the inferior thyroid artery and accessory vessels derived from the common carotid, subclavian, vertebral, ascending pharyngeal, superficial cervical, and costocervical arteries. Mobilization of the upper esophagus is generally well tolerated.

The thoracic portion of the esophagus has a segmental blood supply. The connections in this region are the most tenuous, and care should be taken during mobilization of this segment to reduce the risk of ischemia. The bronchial arteries provide the main vascular supply at this level, and one to three bronchial branches enter the esophagus at the level of the tracheal bifurcation. Variable branches originating directly from the aorta may also be present in this region.

The lower thoracic esophagus is supplied by three unpaired esophageal branches arising directly from the aorta. These branches may anastomose with branches from the intercostal and bronchial arteries. Branches from the internal mammary and carotid arteries may also be present here. The abdominal esophagus is supplied by the ascending branch of the left gastric artery and branches of the left inferior phrenic artery.

Before surgery, the position of the aortic arch should be confirmed, preferably by echocardiography.[4] The surgical approach should be performed on the side opposite of the aortic arch. A right-side aortic arch occurs in 5% of infants with EA.

Although the venous drainage of the esophagus is not described here, the azygos vein serves as a good landmark during surgery. The esophageal ends, particularly the distal segment in a TEF, are often readily visualized once the azygos vein has been divided or reflected.

The esophagus is largely innervated by the autonomic nervous system. Sympathetic innervation plays a minor role and arises from the pharyngeal plexus in the upper esophagus and the stellate ganglia in the lower cervical and upper thoracic portions. The aortic plexus, sympathetic chain, and splanchnic nerves supply the remainder of the thoracic esophagus.

In the abdominal segment, fibers from the celiac ganglion pass around the left gastric and inferior phrenic arteries to innervate the esophagus. Parasympathetic innervation to the esophagus is provided by the vagus. Parasympathetic function includes stimulation of smooth muscle and secretory activity. The vagus also aids the sphincteric function of the lower esophagus. The recurrent laryngeal nerves pass cranially in a groove between the esophagus and trachea, supplying the cervical and upper one third of the thoracic esophagus.

The vagus nerves descend caudally, arborize to form the esophageal plexus, and then coalesce into the left and right vagal trunks, which overlie the anterior and posterior lower esophagus, respectively. Because of its course along the esophagus, the vagus is another helpful landmark during operative esophageal procedures. Disruption or injury to the vagus nerves during surgical manipulation has been proposed as a mechanism of dysmotility following esophageal repair.

Pathophysiology

Esophageal atresia and tracheoesophageal fistula

EA is a condition in which the proximal and distal portions of the esophagus do not communicate. The upper segment of the esophagus is a dilated blind-ending pouch with a hypertrophied muscular wall. The pouch typically extends to the level of the second to fourth thoracic vertebra. In contrast, the distal esophageal portion is an atretic pouch with a small diameter and a thin muscular wall; it usually extends 1-2 cm above the diaphragm.

TEF is an abnormal communication between the trachea and esophagus. When associated with EA, the fistula commonly enters the trachea posteriorly just above the carina. However, isolated TEF, or an H-fistula, can occur at any level from the cricoid cartilage to the carina.

Because the esophagus is discontinuous, an infant with EA cannot swallow and appropriately handle secretions. Infants exhibit persistent drooling and aspiration or regurgitation of food after attempted feedings. Patients who have EA with distal TEF are at risk for additional complications related to the tracheoesophageal communication. When infants with this anomaly strain, cough, or cry, air enters the stomach through the fistula. As a result, the stomach and small intestine become dilated, elevating the diaphragm and making respiration more difficult.

Gastric secretions may also reflux retrograde through the fistula into the tracheobronchial tree, contributing to pneumonia and atelectasis. Abnormal esophageal motility is common in children with congenital anomalies of the esophagus.

Several different types of EA and TEF have been described. The frequencies calculated from a summary of six long-term studies are provided for each type. The most common abnormality (84%) is EA with a distal TEF. Isolated atresia with no fistula is the next most common finding (8%), followed by isolated TEF with no atresia (4%). EA with proximal and distal fistulas (3%) and EA with a proximal fistula (1%) are less common.

Esophageal stenosis, web, and muscular hypertrophy

Congenital esophageal stenosis is a narrowing of a region of the esophagus. A web, or diaphragm, consists of a thin squamous epithelial membrane in the esophageal lumen. It typically causes a partial obstruction in the middle to lower esophagus. Congenital muscular hypertrophy is characterized by submucosal proliferation of smooth muscle and fibrous connective tissue beneath a normal squamous epithelium. Individuals with congenital muscular hypertrophy may be asymptomatic.[5, 6]

Esophageal duplications, rests, and cysts

An esophageal duplication may be open at both ends (double esophagus), open at one end (diverticulum), or closed (elongated cyst). Rests are areas where embryonic tracheal or esophageal cells reside in mesodermal tissues. These areas may form cysts in the muscular tissues. A choristoma is a distinct cartilaginous cyst that partially or completely encircles a region typically in the lower third of the esophagus.

Columnar epithelium–lined lower esophagus

The congenital form of this condition is associated with gastroesophageal reflux (GER). Whether this lesion, also called Barrett esophagus, is congenital or acquired is unclear.

Laryngotracheoesophageal cleft

Laryngotracheoesophageal cleft (LTEC) is defined as a midline communication among the larynx, trachea, and esophagus.

Etiology

The etiology of EA is unknown; however, many theories have been proposed.

The esophagus and trachea both are derivations of the primitive foregut. The larynx and trachea outpouch from the foregut at 22 days' gestation, and the lung buds are typically formed by day 26. Lateral mesodermal ridges form in the proximal esophagus during the fourth week of gestation, and the fusion of these grooves in the midline separates the esophagus from the trachea at approximately 26 days' gestation. The esophageal lumen forms following a process of mucosal proliferation and subsequent vacuole formation. Esophageal anomalies result from failure of these processes.

Numerous theories have been postulated concerning the embryogenesis of EA, including asymmetric growth of the esophageal mesenchyme and the epithelial lining, an increased cell proliferation rate in the trachea, a lack of tracheoesophageal separation, notochord abnormalities, delayed or absent apoptosis, and neural crest abnormalities.

Several theories have also been suggested for TEF. Failure of lateral ridge fusion or incomplete septation, abnormal epithelial connections that develop between the separated trachea and esophagus, and vascular deficiencies have been proposed to explain EA and TEF. Intestinal atresias have been experimentally produced by interrupting the blood supply to the intestine, but convincing support for this theory has not been demonstrated for EA.

The fetal heart begins beating during 4 weeks' gestation, and a vascular accident causing EA would have to occur before the sixth week of development. However, a 9-mm embryo with an established EA-TEF has been reported, indicating that the defect may occur before the vascular tree is fully developed.

Many children with EA and TEF have been reported to have an insufficient esophageal blood supply. Furthermore, several reports have shown an association between EA and a single umbilical artery or other abnormalities that may result in vascular compromise.

Aberrant vessels and an enlarged heart have also been cited as potential causative agents for tracheoesophageal malformations. These may cause excessive pressure on nearby organs, such as the esophagus and trachea. Other reports suggest that structures of the developing embryo are not rigid enough to be injured by this mechanism.

Although genetics has not been found to play a definitive role in the origination of EA-TEF, several chromosomal defects and gene associations have been suggested. Genetic involvement has also been described for conditions in which EA is one of many anomalies, such as oculodigitoesophageoduodenal (ODED) syndrome (ie, Feingold syndrome), trisomy 18, and Down syndrome.

Other etiologic factors (eg, vitamin deficiencies; drug exposures; viral, chemical, and physical external events) have been reported to cause tracheoesophageal malformations in experimental models and in humans.

Other congenital anomalies

One proposition is that esophageal webs result from a failure of esophageal vacuoles to coalesce at 25-31 days' gestation, which normally leads to complete luminal patency.

True esophageal duplications may develop from persistent esophageal vacuoles, whereas cysts result from remnants of the dorsal notochord, abnormal tracheobronchial tree branching, or primitive foregut diverticula. Cysts may be formed when groups of cells that are capable of forming a portion of esophagus, stomach, or pulmonary tree are pinched off from the developing foregut.

Congenital rings are thought to result from incomplete separation of respiratory tissue from the esophagus during fetal life.

Stenosis results from abnormal rests of respiratory tissue in the esophageal wall or fibromuscular hypertrophy.

LTECs may result from faulty growth of the foregut folds, resulting in failure of the posterior larynx to close and allowing persistence of the primitive tracheoesophageal space.

Epidemiology

Esophageal atresia has an incidence of 1 in 3000 in the United States. Internationally, EA occurs in 1 per 3000-5000 live births. Males have a slightly increased risk for EA compared with females, and one study in California reported a higher incidence of EA in white populations (1 per 10,000 births) compared to nonwhite populations (0.55 per 10,000 births).

True congenital stenosis of the esophagus is rare. It occurs in 1 in 25,000-50,000 births. Incidence is higher in Japan.

Prognosis

Survival rates for patients with EA and/or TEF have immensely improved since Haight's first successful repair in 1941. Early diagnosis and advancements in neonatal anesthesia, surgical technique, treatment of associated anomalies, and intensive care management have improved the prognosis. Most children treated for EA have a normal lifespan. Despite an increased number of patients with severe congenital anomalies, survival rates have been reported as high as 95%. In uncomplicated cases, survival rates are virtually 100%.

Traditionally, prognosis for children with EA-TEF was based on birth weight and the presence of pneumonia and associated congenital anomalies. Because of advancements in neonatal care, birth weight does not affect survival rate unless it is severely low, and pneumonia may be treated successfully. Currently, cardiac and chromosomal abnormalities are the most significant causes of death. Infants with a birth weight less than 1500 g, major congenital cardiac abnormalities, severe associated anomalies, preoperative ventilator dependence, and/or long gap are at increased risk.

Dysphagia, frequent night coughs, dyspepsia, and recurrent respiratory infections are frequent results of the less distensible esophagus and gastroesophageal reflux (GER). GER occurs in as many as one half of these patients and many require antireflux operations.

Feeding difficulties also are common, particularly during the first several years after repair. Choking, vomiting, and food impaction occur. These symptoms, like many following EA repair, diminish over time, and 70-80% of adolescents report no or only occasional swallowing impairment. Most patients who have undergone EA repair have abnormal peristalsis with decreased contractile activity and inefficient clearance capacities.

In one series, after an average of 8.8 years of follow-up care, all patients were reported to eat excellently or satisfactorily, with more than 90% eating no differently than their siblings. Normal respiratory function is observed in half of patients 3 months postoperatively. Tracheomalacia, vascular rings, and decreased lung volumes account for the abnormal respiratory function in the other children. Tracheomalacia occurs in 10% of patients with TEF. Most outgrow this problem; however, some children require more aggressive therapy.

Growth retardation has been observed in some children who have had EA repair, but this observation varies. Patients treated for EA-TEF are at higher risk for developing esophagitis and Barrett epithelium. Reports of esophageal carcinoma decades after EA-TEF repair are becoming more frequent as the first generation of survivors progresses through adulthood. Surveillance esophagoscopy has been proposed to provide early detection for esophageal abnormalities.[7]

Despite the complications, the results of EA-TEF repair have dramatically improved. Many symptoms are alleviated over time, and most children and adults enjoy normal lifestyles and have no complaints concerning their quality of life or eating habits. Even by school age, children who had many complications in infancy reported few restrictions at school or in participation in sports with little or no effect on school attendance and social activities. The outcome for these children and children treated for other congenital lesions is generally good.[8]

 

Presentation

History and Physical Examination

The earliest clinical sign of an infant with esophageal atresia (EA) is polyhydramnios resulting from the infant's inability to swallow and absorb amniotic fluid through the gut. The infant may have a small or absent stomach on ultrasonographic study. Note that polyhydramnios is observed in infants with many diagnoses. Only 1 in 12 infants with polyhydramnios has EA.

Polyhydramnios is observed in 95% of infants with EA and no fistula and in 35% of patients who have EA with a distal fistula. Increased pressure because of the amniotic fluid accumulation results in a higher number of premature births and newborns with low birth weight. One third of infants with EA weigh less than 2250 g.

Postnatally, infants with pure EA become symptomatic within the first few hours of life. Children with an isolated tracheoesophageal fistula (TEF) have more subtle symptoms that may not be initially recognized. Excess salivation and fine frothy bubbles in the mouth and sometimes nose result from an inability to swallow. Any attempts at feeding result in choking, coughing, cyanotic episodes, and food regurgitation.

The presence of a fistula increases the risk of aspiration of gastric secretions into the trachea and lungs. Pneumonitis and atelectasis develop quickly in these neonates, and rattles heard during respirations are common. Fistulas also allow air to enter into the stomach and intestines, which can lead to abdominal distension. Gastric perforations occur, especially in the presence of imperforate anus. In the presence of atresia alone, the abdomen appears scaphoid.

Many anomalies are associated with EA, and 50-70% of children with EA have some other defect. The VACTERL association describes the following more commonly associated combination of defects: vertebral, anorectal, cardiac, tracheal, esophageal, renal, and limb. Cardiac abnormalities are the most common, especially ventricular septal defects and tetralogy of Fallot. Imperforate anus and skeletal malformations may also be found upon examination. In the absence of such associated anomalies, the physical examination findings of infants with EA are fairly unremarkable.

Symptoms of congenital esophageal stenosis related to membranous webs, diaphragm muscular hypertrophy, or tracheobronchial remnants occur in infancy with progressive dysphagia and vomiting. Most patients present after semisolid or solid foods are introduced. More rarely, patients with congenital stenosis present with regurgitation and aspiration as newborns. A foreign body in the esophagus may be the first symptom. Cysts may be identified on chest radiography or computed tomography (CT) obtained for recurrent pneumonia or unrelated reasons.

 

Workup

Laboratory Studies

Prenatal laboratory abnormalities have been reported with this anomaly. In cases of unexplained polyhydramnios, amniocentesis may be performed. An elevated alpha-fetoprotein (AFP) level and a positive acetylcholinesterase test result may be observed. Chromosomal analysis is also performed on the amniotic fluid.

Routine neonatal and preoperative laboratory tests should be obtained.

Imaging Studies

Antenatal imaging

Ultrasonography

Antenatal detection of esophageal atresia (EA) relies on the ultrasonographic finding of a small or absent fetal stomach bubble associated with maternal polyhydramnios. The positive predictive value of this combination is 56%, and the sensitivity of antenatal ultrasonography in the diagnosis of EA is 42%. The diagnostic accuracy is increased if an anechoic area is also present in the middle fetal neck. Polyhydramnios alone is a poor predictor of EA. Only one in 12 patients with polyhydramnios is found to have EA.

Magnetic resonance imaging

In one study, fetuses with ultrasonographic evidence of EA underwent single-shot rapid-acquisition magnetic resonance imaging (MRI).[9] Findings were negative if the entire esophagus could be visualized and positive if the esophagus was absent in the midchest (see the images below). MRI had a sensitivity of 100% and a specificity of 80%.

Fetal MRI showing normal esophagus. This study is Fetal MRI showing normal esophagus. This study is negative for esophageal atresia. The hypopharynx and proximal esophagus are normal caliber (arrows), and a normal thoracic esophagus (E) is observed.
Fetal MRI that reveals esophageal atresia. The pro Fetal MRI that reveals esophageal atresia. The proximal esophagus and hypopharynx are dilated (arrow). The thoracic esophagus is not discernible.

Plain radiography

The inability to pass a rigid nasogastric tube from the mouth to the stomach is diagnostic of EA. The position of the tip of the tube in the upper pouch is confirmed with a chest radiograph. A small amount of air may be injected into the tube to insufflate the upper pouch.

Air in the stomach and intestine suggests the presence of a distal tracheoesophageal fistula (TEF); see the first image below). A gasless abdomen suggests isolated EA (see the second image below). In cases of EA and distal TEF, the gap length can be estimated by measuring the distance between the tube in the upper pouch and the carina (see the video below). A gap of three or more vertebral bodies or 5 cm or longer is considered long. The gap distance for pure EA is difficult to determine without a gastrostomy in place.

Plain radiograph in a newborn suspected of having Plain radiograph in a newborn suspected of having esophageal atresia. Air in the stomach and intestine suggest a distal tracheoesophageal fistula.
Pure esophageal atresia. Newborn with tube in prox Pure esophageal atresia. Newborn with tube in proximal pouch. Note that no air is in the stomach or intestine (a gasless abdomen).
Animation illustrating method to estimate distance for esophageal atresia (EA) with distal tracheoesophageal fistula (TEF). A small amount of air has been injected into the upper pouch. The upper pouch and bifurcation of the trachea are marked. The distal fistula is usually posterior to the carina.

Plain chest radiography also provides information about the presence of associated congenital anomalies. Pneumonitis; atelectasis; cardiac, vertebral, and rib anomalies; and aortic arch location may be discerned from chest radiographs. A right-side aortic arch is indicated roentgenographically by a denser shadow on the right side of the mediastinum, a right-side tracheal indentation, a dilated inlet to the left subclavian artery, and a normal heart configuration.

Contrast studies

Contrast studies are rarely needed but may be necessary to identify or locate a proximal fistula (see the image below). To reduce the complications associated with contrast aspiration, a water-soluble agent should be used. Contrast studies should be performed under fluoroscopic control, and only 0.5-1.0 mL of contrast should be injected and later removed via aspiration.

Contrast study of the upper pouch in an infant wit Contrast study of the upper pouch in an infant with esophageal atresia and distal tracheoesophageal fistula.

An isolated, or H-type, fistula presents a different radiographic picture, and establishing the diagnosis is often more difficult. Recurrent pneumonia, particularly of the right upper lobe is suggestive of this condition. Contrast studies specifically looking for the fistula are needed. A tube is placed in the esophagus, and dilute barium is instilled into the esophagus, beginning just above the lower esophageal sphincter. As contrast is injected, the tube is slowly withdrawn in a proximal direction.

This method typically demonstrates only tracheal filling. The fistula is difficult to visualize. A roentgenographic study using videotape or cinefluoroscopy may show an H-fistula not visible on barium swallows, but bronchoscopy or esophagoscopy are often required to confirm the diagnosis. An amotile or hypomotile esophageal segment in a child with recent pneumonia should also alert a fluoroscopist to the possibility of an H-fistula.

Computed tomography and magnetic resonance imaging

Computed tomography (CT) and MRI examinations are rarely needed in the evaluation of EA. Both tests are sensitive and specific for visualization of most great vessel anomalies, but the high costs and need for transport limit their use. They are more useful when evaluating a mass, such as a foregut duplication or cyst.

Studies to detect duplications

Esophageal duplications are rare. Most are segmental and occur in the posterior mediastinum. Plain chest radiography reveals a round or oval posterior mediastinal mass close to the esophagus, sometimes causing esophageal deviation or compression. The cystic nature of this mass is revealed on CT.

Studies to detect rings

Cartilaginous rings are found in the distal third of the esophagus, whereas nonspecific congenital rings are located in the distal two thirds of the esophagus. Tracheobronchial remnants may coexist with EA (see the image below). Esophageal rings are typically not apparent on radiography unless the distal esophagus is well distended. Barium may fill linear clefts, ducts, or cystic spaces extending perpendicular to the ring. Fluoroscopic examination reveals thickened annular narrowing at the ring site. Note that rings may spontaneously change in caliber and configuration during fluoroscopy.

Tracheobronchial remnant in distal esophagus of an Tracheobronchial remnant in distal esophagus of an infant with pure esophageal atresia.

Studies to detect webs

Esophageal webs are typically located in the proximal esophagus within a few centimeters of the cricopharyngeus. Incomplete webs are placed anteriorly and can be observed only in the lateral projection. Complete webs create a characteristic jet effect distally upon fluoroscopic examination. Just as with esophageal rings, the distal esophagus must be adequately distended in order to visualize the abnormality.

Screening for associated congenital anomalies

The presence of other congenital anomalies affects the choice and timing of the repair. Patients with cardiac, pulmonary, chromosomal, and renal anomalies have higher mortality rates. Echocardiography and renal ultrasonography should be used routinely in cases of esophageal anomalies. Echocardiography is used to determine the position of the aortic arch. Chromosomal analyses may also be indicated.

Diagnostic Procedures

The diagnosis of EA or TEF is suggested by several tests, in addition to the clinical signs previously discussed. After birth, inability to pass a rigid radiopaque 10-French catheter from the mouth to the stomach suggests EA, and diagnosis is confirmed upon identification of the tube, arrested 9-12 cm from the alveolar ridge, in the upper pouch. Presence of air in the stomach and intestines indicates EA with a distal fistula, whereas absence of abdominal gas suggests pure atresia, EA with a proximal fistula, or, rarely, 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 esophagraphy, cinefluoroscopy, bronchoscopy, or esophagoscopy. Because of the risk of aspiration, use of contrast for visualization of congenital esophageal anomalies must be approached with extreme care and performed only by an experienced radiologist.

Esophagoscopy may be used to identify strictures, webs, or fistulas within the upper pouch. A TEF may be better visualized from the tracheal side during bronchoscopy. Retrograde endoscopy through a gastrotomy allows a 3- or 4-French catheter to be placed into the distal esophagus.

Bronchoscopy findings confirm the presence of a fistula and are useful in detecting laryngotracheal clefts. Bronchoscopy provides knowledge of the precise fistula location and can be used to identify proximal TEFs or unusual lesions, such as triple TEF. A small Fogarty catheter can be passed through the fistula and used for occlusion in infants too unstable to undergo fistula ligation. The catheter can also serve as a guide during repair.

Estimating gap length is important in the preoperative period. Gap length may be determined using various techniques (see the image below). High upper pouch, vascular ring, vertebral and rib anomalies, and isolated EA or EA with a proximal fistula are more common if a long gap is present.

Animation illustrating method to estimate distance for esophageal atresia (EA) with distal tracheoesophageal fistula (TEF). A small amount of air has been injected into the upper pouch. The upper pouch and bifurcation of the trachea are marked. The distal fistula is usually posterior to the carina.

In children with pure EA who are undergoing staged repair, gap length is monitored by serial gapograms (see the images below).

Gapogram estimating distance between the proximal Gapogram estimating distance between the proximal pouch (P) and the distal pouch (D). The proximal pouch has mercury-weighted dilator, and the distal pouch is filled with contrast injected through a gastrostomy (G). The distance is more than 5 cm and 5 vertebral bodies.
Gapogram demonstrating 3.4-cm gap. The distal pouc Gapogram demonstrating 3.4-cm gap. The distal pouch is being stretched with radiopaque dilator.
Gapogram demonstrating pure esophageal atresia bef Gapogram demonstrating pure esophageal atresia before surgical repair. Green lines show gap distance less than 2 cm between mercury-weighted dilator in upper pouch and contrast in distal pouch.

Contrast is placed through the gastrostomy tube and refluxed into the distal esophagus. The proximal pouch is stretched and visualized with a tube or mercury-weighted dilator (see the images below).

Gapogram estimating distance between the proximal Gapogram estimating distance between the proximal pouch (P) and the distal pouch (D). The proximal pouch has mercury-weighted dilator, and the distal pouch is filled with contrast injected through a gastrostomy (G). The distance is more than 5 cm and 5 vertebral bodies.
Gapogram demonstrating 3.4-cm gap. The distal pouc Gapogram demonstrating 3.4-cm gap. The distal pouch is being stretched with radiopaque dilator.
Gapogram demonstrating pure esophageal atresia bef Gapogram demonstrating pure esophageal atresia before surgical repair. Green lines show gap distance less than 2 cm between mercury-weighted dilator in upper pouch and contrast in distal pouch.

Similarly, a dilator can be placed through a gastrostomy into the distal pouch (see the image below).

Gapogram demonstrating 3.4-cm gap. The distal pouc Gapogram demonstrating 3.4-cm gap. The distal pouch is being stretched with radiopaque dilator.

Histologic Findings

During development, the esophageal epithelium undergoes several transitions, from pseudostratified columnar to ciliated columnar. A stratified squamous epithelium appears in the middle third and migrates cranially and caudally until it completely lines the esophagus of the fetus.

Variances in the esophageal epithelium are observed in histologic EA examinations. Tracheobronchial remnants (eg, ciliated pseudostratified columnar epithelium, seromucous glands, cartilage) are often observed. Irregular smooth muscle fibers are also observed in the distal esophagus of infants with EA and TEF. Fistula tracts may be lined with ciliated respiratory epithelium that extends variable distances from the fistula origin. In addition, the muscular coat of the fistula tract may be absent at the origin. Tracheobronchial remnants as the histologic cause of congenital esophageal stenosis, an associated anomaly of EA, have also been described.

Other congenital anomalies may be noted as well. Histologic investigation of esophageal rings has shown the upper surface to be lined by squamous epithelium, while a columnar epithelium lies below. The ring core consists of a lamina propria with no muscularis mucosa. Inflammation and fibrosis are also absent. Rings may contain tracheobronchial cartilage and respiratory epithelial remnants. The ducts and cystic spaces are typically lined with a respiratory epithelium.

Histologic examinations of esophageal webs have shown plications of normal squamous mucosa with inflammation or patches of heterotopic gastric mucosa. The histologic appearances of foregut cysts differ by type; bronchogenic cysts are lined with respiratory epithelium, gastroenteric cysts are lined by gastrointestinal (GI) epithelium, and neurenteric cysts are lined with GI epithelium, in addition to neural elements.

Congenital stenoses may consist of rings of muscle or tracheobronchial elements.

 

Treatment

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, an ultrasonographic finding of a small or absent stomach bubble 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 ultrasonographic 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 based on the infant's respiratory status. Bag-mask ventilation should be avoided because it may cause gastric distension 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]

Gastrostomy

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 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. A muscle-sparing approach can be followed.

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 illustrating azygous vei Intraoperative photograph illustrating azygous vein. A right thoracotomy and retropleural dissection has 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. Interrupted 5-0 or 6-0 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 the chest tube (CT) in the retropleural space and the feeding tube passing through the anastomosis (A) into the 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 tissue flap may also be placed between the anastomosis and the repaired trachea to decrease the risk of fistula recurrence.

Intraoperative photograph showing primary anastomo Intraoperative photograph showing primary anastomosis (A) between the 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. The chest tube is placed in the retropleural space (CT). No feeding tube is passing across the 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.

In some cases, thoracoscopic techniques have been used to repair tracheoesophageal atresia and distal fistula.[13]

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.

Intraoperative photograph during repair of esophag Intraoperative photograph during repair of esophageal atresia. The head is to the left. The azygous vein has been divided. The proximal (P) and distal (D) esophageal pouches can be easily observed.
Intraoperative photograph showing primary anastomo Intraoperative photograph showing primary anastomosis (A) between the proximal and distal esophagus.

Several techniques are used for lengthening the esophageal ends to achieve a primary anastomosis. Bougienage is the most common mechanical lengthening procedure. Upper pouch bougienage 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.[14, 15] 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 bougienage 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. The use of short-term traction sutures to narrow the esophageal gap has been described.

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 advancements 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.

Colon

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. The colon is sutured to the cervical esophagus proximally and the 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 a ga Contrast swallow (oblique view) demonstrating a gastric pull-up used as esophageal replacement in a 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

Jejunum

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 mobilizing the proximal esophagus during surgery for EA (see the image below). 

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

A right-sided supraclavicular incision 1-1.5 cm above and parallel to the right clavicle is made to minimize 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 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 (see the image below).

Intraoperative photograph demonstrating proximal H Intraoperative photograph demonstrating proximal H-type fistula (F) between the proximal esophagus (P) and trachea (T). The blue vessel loop is around the fistula. The H-type fistula was not diagnosed preoperatively; it was identified while mobilizing the proximal esophagus in a child with congenital esophageal stenosis.

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 gastroesophageal junction. 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 an infant thought to Intraoperative photograph of an infant thought to have esophageal atresia. The proximal (P) and distal esophagus was connected by a fibrotic segment of congenital esophageal stenosis (CES) beginning at the azygous 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 an atretic segment of esophagus and primary anastomosis (A) of the proximal (P) and distal esophagus. The azygous 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 the chest tube (CT) in the retropleural space and the feeding tube passing through the anastomosis (A) into the stomach.
Chest radiograph following repair of esophageal at Chest radiograph following repair of esophageal atresia. The chest tube is placed in the retropleural space (CT). No feeding tube is passing across the 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. 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 one week following primary anastomosi Esophagogram one 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.

Complications

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 a tracheoesophageal fistula. Contrast was injected through a gastrostomy and refluxed through the distal esophagus. The leak (L) is draining into the 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.[16] 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 a distal tracheoesophageal fistula (TEF). Note narrowing at anastomosis (A). The 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 the anastomosis (A). Image 2 of 3 in series.
Esophagogram. Note spontaneous resolution of narro Esophagogram. Note spontaneous resolution of narrowing at the 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 the change in caliber from the proximal esophagus to the distal esophagus at the anastomosis (A). The retropleural chest tube can be observed (CT). This patient had gastroesophageal reflux and developed a 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 a series.
Esophagogram performed 2 weeks later demonstrating Esophagogram performed 2 weeks later demonstrating worsened anastomotic stricture (A). The patient responded well to Bouginage dilatation and medical management of the reflux. Image 3 of 3 in a 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.[17]

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 a series.
Esophagogram performed 2 weeks later demonstrating Esophagogram performed 2 weeks later demonstrating worsened anastomotic stricture (A). The patient responded well to Bouginage dilatation and medical management of the reflux. Image 3 of 3 in a 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.[18]

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 a 5-ye Esophagogram demonstrating impacted food in a 5-year-old child with a 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 gastroesophageal reflux, recurrent fistula, tracheomalacia, and other complications.