Esophageal Atresia With or Without Tracheoesophageal Fistula 

Updated: Jul 18, 2019
Author: Amulya K Saxena, MD, PhD, DSc, FRCS(Glasg); Chief Editor: Eugene S Kim, MD, FACS, FAAP 

Overview

Background

Esophageal atresia refers to a congenitally interrupted esophagus.[1] One or more fistulae may be present between the malformed esophagus and the trachea. The lack of esophageal patency prevents swallowing. In addition to preventing normal feeding, this problem may cause infants to aspirate and literally drown in their own saliva, which quickly overflows the upper pouch of the obstructed esophagus. If a tracheoesophageal fistula (TEF) is present, fluid (either saliva from above or gastric secretions from below) may flow directly into the tracheobronchial tree.

The condition was first described anecdotally in the 17th century. In 1670, Durston described the first case of esophageal atresia in one conjoined twin. In 1696, Gibson provided the first description of esophageal atresia with a distal TEF. In 1862, Hirschsprung (a famous pediatrician from Copenhagen) described 14 cases of esophageal atresia. In 1898, Hoffman attempted primary repair of the defect but was not successful and resorted to the placement of a gastrostomy.

At the start of the 20th century, surgeons were theorizing about how the lesion could be repaired. In 1939 and 1940, Ladd of Boston and Lever of Minnesota first achieved surgical success in stages; success meant that the affected children survived and skin-lined pharyngogastric conduits were eventually constructed. In 1941, Haight of Michigan successfully repaired esophageal atresia in a 12-day-old baby using a primary single-stage left-side extrapleural approach. Subsequent to that child's survival and with advances in surgical and anesthetic techniques, esophageal atresia is now regarded as an eminently correctable congenital lesion.

For patient education resources, see the Esophagus, Stomach, and Intestine Center and Procedures Center, as well as Choking and Bronchoscopy.

Pathophysiology

The variants of esophageal atresia have been described using many anatomic classification systems. To avoid ambiguity, the clinician should use a narrative description. Nevertheless, Gross of Boston described the classification system that is most often cited (see the image below).[2]

Esophageal atresia classification according to Gro Esophageal atresia classification according to Gross.

According to the system formulated by Gross, the types of esophageal atresia and their approximate incidence in all infants born with esophageal anomalies are as follows:

  • Type A - Esophageal atresia without fistula or so-called pure esophageal atresia (10%)
  • Type B - Esophageal atresia with proximal TEF (< 1%)
  • Type C - Esophageal atresia with distal TEF (85%)
  • Type D - Esophageal atresia with proximal and distal TEFs (< 1%)
  • Type E - TEF without esophageal atresia or so-called H-type fistula (4%)
  • Type F - Congenital esophageal stenosis (< 1%) (not discussed in this article)

A fetus with esophageal atresia cannot effectively swallow amniotic fluid, especially when TEF is absent.[3] In a fetus with esophageal atresia and a distal TEF, some amniotic fluid presumably flows through the trachea and down the fistula to the gut. Polyhydramnios may be the result of this change in the recycling of amniotic fluid through the fetus. Polyhydramnios, in turn, may lead to premature labor. The fetus also appears to derive some nutritional benefit from the ingestion of amniotic fluid; thus, fetuses with esophageal atresia may be small for their gestational age.

The neonate with esophageal atresia cannot swallow and drools copious amounts of saliva. Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an aspiration pneumonitis. In a baby with esophageal atresia and a distal TEF, the lungs may be exposed to gastric secretions. Also, air from the trachea can pass down the distal fistula when the baby cries, strains, or receives ventilation.[4] This condition can lead to an acute gastric perforation, which is often lethal.

Prerepair esophageal manometric studies have revealed that the distal esophagus in esophageal atresia is essentially dysmotile, with poor or absent propagating peristaltic waves. This condition results in variable degrees of dysphagia after the repair and contributes to gastroesophageal reflux (GER).

The trachea is also affected by the disordered embryogenesis in esophageal atresia. The membranous part of the trachea, the pars membranacea, is often wide and imparts a cross-sectional D shape to the trachea, as opposed to the usual C shape. These changes cause secondary anteroposterior structural weakening of the trachea, or tracheomalacia.

This weakening can result in a sonorous cough as the intrathoracic trachea resonates and partially collapses with forceful expiration. Secretions can be difficult to clear and may lead to frequent pneumonias. Also, the trachea can partially collapse during feeding, after repair, or with episodes of GER; this partial collapse can lead to ineffective respiration; hypoxia; and, somewhat inexplicably, apnea.

Etiology

No human teratogens that cause esophageal atresia are known. Esophageal atresia that occurs in families has been reported. A 2% risk of recurrence is present when a sibling is affected. The occasional association of esophageal atresia with trisomies 21, 13, and 18 further suggests genetic causation. Also, twinning occurs about six times more frequently in patients with esophageal atresia than in those without the condition.

Most authorities believe that the development of esophageal atresia has a nongenetic basis.[5] Debate about the embryopathologic process of this condition continues, and little about it is known. The old His theory that lateral infoldings divide the foregut into the esophagus and trachea is attractively simple, but findings from human embryology studies do not support this theory.

In 1984, O'Rahilly proposed that a fixed cephalad point of tracheoesophageal separation is present, with the tracheobronchial and esophageal elements elongating in a caudal direction from this point.[6] This theory does not easily account for esophageal atresia but explains TEF as a deficiency or breakdown of esophageal mucosa, which occurs as the linear growth of the organ exceeds the cellular division of the esophageal epithelium.

In a 1987 report, Kluth eschewed the concept that tracheoesophageal septation has a key role in the development of esophageal atresia.[7] Instead, he based the embryopathologic process on the faulty development of the early, but already differentiated, trachea and esophagus, in which a dorsal fold comes to lie too far ventrally; thus, the early tracheoesophagus remains undivided. He also suggested that esophageal vascular events, ischemic events, or both may be causes in cases of esophageal atresia without fistula.

In 2001, Orford et al postulated that the ectopic, ventrally displaced location of the notochord in an embryo at 21 days' gestation can lead to a disruption of the gene locus, sonic hedgehog-signaled apoptosis in the developing foregut, and variants of esophageal atresia.[8]  This situation may be due to various early gestation teratogenic influences such as twinning, toxin exposure, or possible abortion.

In 2003, Spilde et al reported esophageal atresia-TEF formations in the embryos of rat models of doxorubicin-induced teratogenesis.[9] Specific absences of certain fibroblast growth factor (FGF) elements have been reported, specifically FGF1 and the IIIb splice variant of the FGF2R receptor.[10] These specific FGF-signaling absences are postulated to allow the nonbranching development of the fistulous tract from the foregut, which then establishes continuity with the developing stomach.

Epidemiology

The incidence of esophageal atresia is 1 case in 3000-4500 births. This frequency may be decreasing for unknown reasons.[11]  Internationally, the highest incidence of this disorder is reported in Finland, where it is 1 case in 2500 births.

Prognosis

Statistics regarding mortality in esophageal atresia are constantly changing and improving.[12, 13, 14, 15] One must consider the classification system used in reporting such statistics.

Mortality relative to the Montreal classification is as follows[16] :

  • Class I - Mortality of 7.3%
  • Class II - Mortality of 69.2%

Mortality relative to the Spitz grouping is as follows[17] :

  • Group I - Mortality of 3%
  • Group II - Mortality of 41%
  • Group III - Mortality of 78%

Mortality relative to the Waterston categorization is as follows[18] :

  • Category A - Mortality of 0%
  • Category B - Mortality of 4%
  • Category C - Mortality of 11%

Fetuses with antenatal diagnoses of esophageal atresia seem to have a worse prognosis.[19] The cohort of babies in whom esophageal atresia is detected antenatally has a 75% mortality, whereas the cohort of babies in whom esophageal atresia is not detected antenatally has a 21% mortality. Babies who survive have varied morbidities related to any of the associated anomalies and complications. However, most children who undergo a successful repair of esophageal atresia are relatively healthy.

 

Presentation

History and Physical Examination

A mother who is carrying a fetus with esophageal atresia may have polyhydramnios, which occurs in approximately 33% of mothers with fetuses with esophageal atresia and distal tracheoesophageal fistula (TEF) and in virtually 100% of mothers with fetuses with esophageal atresia without TEF.

Characteristically, the neonate born with esophageal atresia drools and has substantial mucus, with excessive oral secretions. If suckling at the breast or bottle is allowed, the baby appears to choke and may have difficulty maintaining an airway. Significant respiratory distress may result. In the delivery room, the affected infant may have the sonorous seal-bark cough that indicates concomitant tracheomalacia. If an oral tube is placed to suction the stomach, as it is in some delivery rooms, it characteristically becomes blocked 10-11 cm from the lips.

The acronym VACTERL (vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, and limb deformities) refers a set of associated anomalies that should be readily apparent upon physical examination.[20] If any of these anomalies are present, the presence of the others must be assessed. The VACTERL syndrome exists when three or more of the associated anomalies are present. This syndrome occurs in approximately 25% of all patients with esophageal atresia.

VACTERL anomalies include the following:

  • Vertebral defects - Multiple or single hemivertebrae, scoliosis, rib deformities
  • Anorectal malformations - Imperforate anus of all varieties, cloacal deformities
  • Cardiovascular defects -  Ventricular septal defect (most common), tetralogy of Fallot, patent ductus arteriosus, atrial septal defects, atrioventricular canal defects, aortic coarctation, right-sided aortic arch, single umbilical artery
  • Tracheoesophageal defects - Esophageal atresia
  • Renal anomalies - Renal agenesis including Potter syndrome, bilateral renal agenesis or dysplasia, horseshoe kidney, polycystic kidneys, urethral atresia, ureteral malformations
  • Limb deformities - Radial dysplasia, absent radius, radial-ray deformities, syndactyly, polydactyly, lower-limb tibial deformities

Other associated conditions include the CHARGE (coloboma, heart defects, atresia choanae, developmental retardation, genital hypoplasia, and ear deformities) anomalies.

The following anomalies also occur with increased frequency in esophageal atresia:

Also, trisomies 13, 21, or 18 and Fanconi syndrome may be present. The overall incidence of associated anomalies is approximately 50%. Cardiovascular anomalies occur in 35% of cases, genitourinary (GU) anomalies occur in 20% of cases, and associated GI anomalies occur in approximately 20% of cases. A tethered cord is usually detectable with ultrasonography (US) in the newborn period or later in life with magnetic resonance imaging (MRI)—or, less desirably, computed tomography (CT)—if findings are equivocal.

 

Workup

Laboratory Studies

In babies with esophageal atresia, samples should be obtained to determine baseline values for the following:

  • Complete blood count (CBC)
  • Electrolyte levels
  • Venous gas concentrations
  • Blood urea nitrogen (BUN) and serum creatinine levels
  • Blood glucose level
  • Serum calcium level
  • Arterial blood gas (ABG) concentrations, as necessary

Ultrasonography

Antenatal ultrasonography (US) may reveal the size of the gastric bubble, polyhydramnios, and VACTERL (vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, and limb deformities) anomalies, all of which may indicate esophageal atresia in the fetus. The sensitivity of antenatal US is approximately 40%. An antenatal diagnosis of esophageal atresia may be associated with a worse prognosis.

In infants and neonates, early renal US is mandatory and is performed to evaluate associated kidney anomalies, ureteral anomalies, or both.

Echocardiography is indicated early in the care of infants with esophageal atresia who have clinical signs of cardiovascular disease.[21] However, a 1-day-old neonate with significant congenital heart disease may have normal findings on physical examination. Therefore, some argue that echocardiography should be performed in all infants with esophageal atresia. This examination also provides the surgeon with information regarding the side of the aortic arch. A right-side aortic arch is not uncommon in cases of esophageal atresia, and the surgeon should be aware of this finding.

Spinal US is a simple test that takes advantage of the neonate's relatively transparent lumber lamina in the assessment of an associated tethered cord. This examination may be performed when the baby is younger than 1 month, though it is not critically important in the early care of the infant.

Radiography

Chest radiography (see the images below) is mandatory and should be performed as soon as possible if esophageal atresia is suspected.

This chest radiograph reveals esophageal atresia a This chest radiograph reveals esophageal atresia and distal tracheoesophageal fistula. Note Replogle tube in upper pouch and GI air below diaphragm.
This chest radiograph reveals esophageal atresia w This chest radiograph reveals esophageal atresia without tracheoesophageal fistula. Note absence of gas below diaphragm.

The value of chest radiography is enhanced if a Replogle tube is in place and if 5-10 mL of air is injected to distend the upper pouch. Great caution should be exercised if liquid contrast material is injected into the proximal pouch. First, to prevent spillage into the airway, only about 1 mL of isotonic water-soluble contrast should be used; a catheter with an end-hole should be employed. Second, if an upper-pouch fistula is present, the contrast material flows directly into the airway. Usually, a contrast-enhanced study is unnecessary.

The heart shadow and size should be assessed. Vertebral and rib anomalies should be assessed. The lung fields should be assessed for possible aspiration pneumonitis and for the rarely associated diaphragmatic hernia or congenital lung lesion.

The presence or absence of gastrointestinal (GI) air below the diaphragm is an important finding. Complete absence of gas in the GI tract denotes the absence of a distal tracheoesophageal fistula (TEF); however, distal fistulae simply occluded by mucous plugs have been rarely reported. In cases of esophageal atresia without fistula, it may be assumed that the distance between the ends of the atretic esophagus is too long for early single-stage primary repair. These infants require a delayed repair (see below).

Limb radiography (see the image below) is indicated if the limbs appear abnormal. The possibility of associated radial-ray deformities should be investigated.

This radiograph reveals radius without radial ray This radiograph reveals radius without radial ray deformity.

In cases where the distance between the two atretic ends of the esophagus is suspected to be too long for a primary repair, a "gapogram" (see the image below) is useful in assessing that distance.

Contrast material has been administered, and probe Contrast material has been administered, and probe has been placed through gastrostomy in this child with pure esophageal atresia. Air-filled upper pouch can be observed superiorly, with Replogle tube within it. This gapogram reveals very wide gap (>5 vertebral bodies), which requires esophageal replacement. This study is dynamic investigation, one in which surgeon and radiologist should be present to view real-time fluoroscopic images.

A gastrostomy is created, and the upper pouch is intubated with a 10-French Replogle tube with radiopaque markings. A small-diameter Bakes dilator is introduced into the gastrostomy and directed superiorly under fluoroscopic guidance into the distal esophageal segment. With gentle but definite force on both the Bakes dilator and the Replogle tube, the two ends are pushed toward each other under fluoroscopic control.

At the point of least separation, an image is obtained, and the distance between the two ends is determined in terms of number of vertebral bodies, which provide an inherent reference for measurement. Generally, a separation distance of two (some say three) vertebral bodies or fewer is usually small enough to permit an anastomosis. If greater distances separate the ends, a delay of weeks to months may be required for the ends to grow closer together, for reassessment with gapograms every 4-6 weeks, or for esophageal replacement or lengthening surgery.

 

Treatment

Approach Considerations

The treatment plan for each baby must be individualized. The prognostic classifications (see Indications for and Timing of Surgical Intervention below) can provide guidance in patients with multiple problems, but early and decisive identification of the most life-threatening anomaly is essential.

Management plans for a delayed repair of the esophageal atresia may include placing a 10-French Replogle double-lumen tube through the mouth or nose well into the upper pouch to provide continuous suction of pooled secretions from the proximal portion of the atretic esophagus. The baby may be positioned in the 45° sitting position. Prophylactic broad-spectrum antibiotics (eg, ampicillin and gentamicin) may be used. General supportive care and total parenteral nutrition (TPN) are needed.

With careful bedside attendance, these measures may permit a delay of days to perhaps weeks. Some have described cases in which the baby was discharged home with a Replogle tube in situ while waiting for staged repair of an esophageal atresia. However, deaths have been reported in infants in whom the tube did not maintain an empty upper pouch. A gastrostomy, distal tracheoesophageal fistula (TEF) ligation, or cervical esophagostomy may permit longer delays in the esophageal atresia repair. However, each intrusion carries a price.

If no distal TEF is present, a gastrostomy may be created. In such cases, the stomach is small, and laparotomy is required. In all cases of esophageal atresia in which a gastrostomy is created, care should be taken to place it near the lesser curvature to avoid damaging the greater curvature, which can be used in the formation of an esophageal substitute. When a baby is ventilated with high pressures, the gastrostomy may offer a route of decreased resistance, causing the ventilation gases to flow through the distal fistula and out the gastrostomy site. This condition may complicate the use of ventilation.

In cases such as those above or in cases in which a distal fistula continues to cause lung soiling, distal TEF ligation should be considered. This ligation is performed by means of a right-side thoracotomy, ideally via an extrapleural approach. The fistula may be clipped or simply ligated. If it is ligated and divided, subsequent staged repair of the esophageal atresia may be difficult because the distal esophageal segment tends to retract inferiorly to a substantial degree when it is detached from its tracheal mooring. However, simple fistula ligation may allow subsequent reopening of the fistula. Division of the fistula and attempts to anchor it at the midchest with sutures are usually unsuccessful.

A cervical esophagostomy or spit fistula may be constructed in the right or left side of the neck, depending on the choice for subsequent esophageal substitution. It allows drainage of the upper pouch and precludes aspiration from the upper pouch. Sham feeding may be commenced in cases in which a long delay to repair is anticipated. This feeding may prevent subsequent oral aversion, which is a real problem in babies who have not been fed by mouth in their early weeks to months of life. However, cervical esophagostomy usually dooms the child to some form of esophageal substitution.

In May 2017, the US Food and Drug Administration approved the Flourish Pediatric Esophageal Atresia Anastomosis (Cook Medical) for management of esophageal atresia in infants up to 1 year old who do not have teeth and do not have a TEF (or have had a TEF repaired).[22] The device closes the gap in the esophagus by using magnets to pull together the upper and lower portions of the esophagus. It is not indicated for use in patients in whom the distance between the esophageal segments is 4 cm or greater.

Future and controversies

In the future, more accurate antenatal diagnosis and antenatal treatment may be possible. Minimally invasive techniques for repair with thoracoscopic surgery are now used in some centers, with good results.[23] A better understanding of the pathoembryologic processes of this condition may reveal its causative agents or genetic factors. This knowledge, in turn, may lead to specific antenatal treatments or preventive techniques. In recent years, the incidence of this disorder has decreased, perhaps because of increased usage of antenatal folic acid supplements.

Debates continue about the best operative technique (eg, right-side or left-side thoracotomy) for patients with right-side aortic arches, suture type and technique, esophageal lengthening strategies, and procedures for mobilizing the distal esophagus.[24] Other discussions include when to use cervical esophagostomy and the choice of esophageal replacement.[25] The advent of esophageal atresia repairs that combine both minimally invasive and radiologic interventional techniques may be near.

The management of gastroesophageal reflux (GER) in esophageal atresia is particularly challenging; some advocate aggressive fundoplication, and others prefer more conservative medical treatment. In addition, the true incidence and treatment of tracheomalacia continues to be the subject of debate. Finally, the proper evidence-based guidelines for long-term follow-up remain to be established.

Tissue engineering of the esophagus may offer solutions for replacement of lost esophageal tissue.[26, 27, 28] Experimental studies have shown promising results in the culture of esophageal epithelial cells and esophageal smooth-muscle cells. Viability of these cell on biodegradable scaffolds in vitro may provide the necessary replacement esophageal tissue in future.[29] Studies in large animal models have shown promising results in the generation of rudimentary esophageal tissue using tissue engineering and regenerative medicine technology.[30]

Indications for and Timing of Surgical Intervention

The indications for and timing of surgical repair may be determined by using the Waterston, Spitz, or Poenaru prognostic classification system.

In 1962, Waterston developed a prognostic classification system for esophageal atresia that is still used today.[31] Category A includes patients who weigh more than 5.5 lb (2.5 kg) at birth and who are otherwise well; category B includes patients who weigh 4-5.5 lb (1.8-2.5 kg) and are well or who have higher birth weights, moderate pneumonia, and congenital anomalies; and category C includes patients who weigh less than 4 lb (1.8 kg) or have higher birth weights, severe pneumonia, and severe congenital anomalies. Management strategies are as follows:

  • Category A - Immediate primary repair
  • Category B - Delayed repair
  • Category C - Staged repair

In 1994, after analyzing findings in 387 patients, Spitz et al recognized that the presence or absence of cardiac disease is a proven major prognostic factor.[17] Accordingly, they suggested the following groups, which were analogous to those in the Waterston classification system:

  • Group I - Birth weight >1.5 kg and no major cardiac disease
  • Group II - Birth weight < 1.5 kg or major cardiac disease
  • Group III - Birth weight < 1.5 kg and major cardiac disease

In 1993, Poenaru proposed a simple two-group classification system based on logistic regression analysis findings in 95 patients.[16] Class I included patients who are low-risk and do not meet criteria in class II, and class II included patients who are high-risk and ventilator-dependent or who have life-threatening anomalies, regardless of pulmonary status. Birth weight was not included as a factor.

In 1989, Randolph et al refined the Waterston classification and reported a clinically helpful system that used a patient's physiologic status to determine surgical management (ie, immediate repair, delayed primary repair, or staged repair).[32] Weight, gestational age, and pulmonary condition were not considered. If the patient's physiologic parameters were good, they were managed with immediate repair. Staged repairs were used for infants who were severe compromised infants, especially those with severe cardiac anomalies. In this group, the survival rate was 77%, and the overall survival was 90%.

The aforementioned prognostic groupings can allow for the stratification of high-risk patients with esophageal atresia in planning for delayed repair, staged repair, or both; low-risk babies can usually undergo early (ie, within the first 24-48 hours) primary single-stage repair. For instance, a 2-kg baby with esophageal atresia and distal TEF who also has tetralogy of Fallot is in Waterston category C, Spitz group II, and Poenaru class II; for this patient, delayed or staged repair may be best.

These classification systems help physicians to compare results in an organized and meaningful way. When the three prognostic classification systems are compared, the Spitz classification appears to have the most applicability in current practice.[33] Ductal-dependent cardiac lesions still seem to have a significant effect on the survival of children born with esophageal atresia.

Potter syndrome is bilateral renal agenesis and carries a 100% mortality; therefore, repair of esophageal atresia is contraindicated.

Options for Surgery

This section provides some details about surgical approaches for the repair of the most common type of esophageal atresia (ie, esophageal atresia with distal TEF) in low-risk patients. The video below presents a thoracoscopic repair of esophageal atresia with TEF.

Thoracoscopic repair of esophageal atresia with tracheoesophageal fistula. Procedure performed by Keith Kuenzler, MD, ColumbiaDoctors, New York, NY. Video courtesy of ColumbiaDoctors (https://www.columbiadoctors.org/).

Surgical techniques vary according to surgeons' preferences and variations in pathologic anatomy.[34] Modifications for special anatomic challenges are briefly discussed. In particular, infants born with esophageal atresia without fistula represent a specific and challenging subgroup. These babies should undergo an early gastrostomy procedure in the newborn period. A gapogram (see Workup) should be performed to assess the prospects for anastomotic repair. (See the image below.)

Contrast material has been administered, and probe Contrast material has been administered, and probe has been placed through gastrostomy in this child with pure esophageal atresia. Air-filled upper pouch can be observed superiorly, with Replogle tube within it. This gapogram reveals very wide gap (>5 vertebral bodies), which requires esophageal replacement. This study is dynamic investigation, one in which surgeon and radiologist should be present to view real-time fluoroscopic images.

In infants with atresia without fistula, surgical decisions must be made regarding the following[35] :

  • Length of time to wait for the ends to grow closer
  • Whether to perform one of numerous esophageal lengthening procedures, such as the Kimura, Livaditis, Scharli, or Foker procedures
  • Whether to perform an esophageal substitution procedure, with or without the formation of a cervical esophagostomy
  • Whether to use a gastric tube (reversed and proximally based or antegrade and distally based)

The use of colonic (left chest or substernal), gastric pull-up, or jejunal vascularized[36] graft segments is difficult and should be based on the condition of the infant, the pathologic anatomy, associated defects (eg, gastric pull-up is usually contraindicated in significant cardiac disease, and colonic esophageal replacement is usually contraindicated with concomitant imperforate anus), and the surgeon's experience.

As a rule, a child's own esophagus is better than any substitution. Favorable reports of the Foker technique used for serial dynamic lengthening in cases of long gaps suggest that advantage.[37, 38, 39] The Foker technique involves two thoracotomies. First, anchoring sutures are placed securely at the two ends of the atretic esophagus and brought out diagonally to the chest wall. Over a period of days to weeks, the two ends are brought closer together through a series of daily lengthenings achieved by traction on the exposed sutures. The closure of the gap is monitored radiologically with radiopaque markers at the atretic ends. A second thoracotomy is then performed to effect a tension-free anastomosis.

Surgical Therapy

Preparation for surgery

The preparation of a 1-day-old neonate for surgery includes the following measures:

  • Intravenous (IV) fluid containing an adequate glucose concentration (ie, 10% glucose) is administered at a rate appropriate for the neonate's gestational age and weight
  • Prophylactic broad-spectrum IV antibiotics (eg, ampicillin, gentamicin) are administered
  • The neonate is kept warm by using an incubator or overhead warmer and is positioned supine in the Fowler position, with the head elevated by approximately 45°
  • A 10-French Replogle tube is placed nasally or orally well into the upper pouch and is connected to a continuous suction device; every 30 minutes, the tube is checked for patency first by suctioning with an empty syringe and then by gently injecting 5 mL of air (never water); in small infants, an 8-French double-lumen tube may be used instead
  • The parents should be fully briefed about the nature of the congenital anomaly; a diagram is invaluable for explaining not only the pathologic anatomy and intended repair but also the possible complications; parents' consent for treatment should be obtained, and the discussion with them should be documented in appropriate detail on the baby's medical record

Bronchoscopy

Bronchoscopy performed just before repair of the esophageal atresia may enable the following:

  • Detection of an upper-pouch fistula
  • Localization of the distal fistula, which usually lies at a level just above that of the carina
  • Detection of an aberrant right-upper-lobe bronchus emanating from the trachea, which is not uncommon in cases of esophageal atresia
  • Early assessment of the cross-sectional shape of the trachea, which may help in determining the risk of significant postoperative tracheomalacia
  • Assessment of specific vascular anomalies (eg, right-side aortic arch, aberrant right subclavian artery [for which one looks for the pattern of pulsation on the tracheal wall])

Identification of laryngotracheoesophageal cleft

The infant is endotracheally intubated without paralysis. The anesthesiologist must be mindful of the distal fistula. With skill, the long end of the distal endotracheal tube bevel may be positioned over the fistula to decrease the passage of gases into the stomach. This maneuver helps prevent gastric distention, maximizes ventilation, and minimizes the chances of a gastric perforation.

As much as possible, the baby should be allowed to breathe spontaneously until the fistula is occluded. In reality, and especially because the chest is open and the lung is retracted, the anesthesiologist manually assists with the baby's ventilation. However, mechanical ventilation should be avoided until the fistula is controlled. This procedure requires great skill, experience, and focus on the part of an anesthesiologist in caring for these babies in the operating room.

Managing infants with premature lungs

In positioning the baby in full right thoracotomy position, the surgeon must ensure that the anesthesiologist has full and easy access to the infant's nose and to the Replogle tube, which is not taped so that it can move in or out. If a right-side aortic arch is detected preoperatively, controversy exists about whether a left thoracotomy provides easier access. A left-side approach has its merits, but in this instance, the esophagus is still a right-side structure, and access from the right is best.

Finally, the baby is covered with antiseptic solution, and drapes are placed so as to expose the areas from the nipple to the midback and from the axilla to the 10th rib.

Operative details

The surgeon should wear magnification loupes. The assistants and nurses should be briefed about their duties and about special points of care regarding the delicate nature of the procedure and the baby's tissues.

A transverse right thoracotomy incision is made from the anterior axillary line to approximately one fingerbreadth posterior to the posterior axillary line at a level 1 cm inferior to the palpable tip of the scapula. The latissimus dorsi is divided with the coagulating current of the electrocautery device. The fascia lying just posterior to the posterior margin of the serratus is divided with the electrocautery, and the serratus is retracted anteriorly. Usually, an incision in the serratus is not needed.

The scapula is then lifted away from the chest wall, and the ribs are counted from the first to the fourth. Ideally, the chest is entered through the fourth interspace. With careful use of forceps and the electrocautery device, the outer and innermost intercostal muscles are divided in this interspace down to the parietal pleura.

By using either moist sponges or peanut gauze on the forceps, the parietal pleura is dissected away from the chest wall; dissection proceeds posteriorly but also somewhat superiorly and inferiorly as well. A small mechanical Finochietto-type rib retractor is placed in the open thoracotomy site, and the pleural dissection proceeds to a point medial to the azygos vein.

If the preoperative echocardiogram demonstrates normal venous return from the inferior vena cava, then the azygos vein is ligated and divided with fine silk. If there is evidence of an interrupted inferior vena cava, then the azygos vein may represent the patient's primary venous return from the lower body, and test occlusion of the azygos vein should precede ligation.

The extrapleural dissection then allows retropleural repair of the esophagus. If an anastomotic leak occurs, it tends to be more contained as compared with the empyema that results if the repair is performed transpleurally.

At this point in the dissection, the anatomy is defined first by having the anesthesiologist push on the indwelling Replogle tube; this action usually reveals the upper pouch that rhythmically bulges out in the apex of the right chest cavity. The distal fistula is usually located near the level of the carina and lies just beneath the divided azygos vein. It expands slightly with each inspiration. One must take great care not to mistake the aorta for the fistula. Mistaken ligation of the aorta is possible; in case of doubt, a 25-gauge needle can be passed into the structure to check.

Gaining control of the fistula now relieves the anesthesiologist. A silicone rubber vessel loop can be passed around the fistula at a convenient level near the trachea. Gentle retraction on this occludes the fistula. Most advise division of the fistula with suturing of its tracheal aspect. This division can be accomplished by cutting into the fistula as it enters the back wall of the trachea in short snips and by oversewing the tracheal aspect as it opens in stages. Usually, about four interrupted sutures suffice. Most advocate the use of an absorbable synthetic suture material such as polyglactin. This sutured fistula site may be covered with an azygos or pleural patch for extra security.

The fistula closure should be checked by covering the closure in saline and manually ventilating the patient for a Valsalva test. If bubbles appear, the closure is leaking and must be resutured. Turning his or her attention to the upper pouch, the anesthesiologist can again push on the Replogle tube to facilitate placement of a traction suture into the distal end of the upper pouch.

The upper pouch is then circumferentially dissected cephalad to increase its length. Blood supply to the upper portion is linearly arrayed from the cervical and subclavian vessels; ischemia is not a concern. This dissection must be carefully performed between the pouch and the trachea while the presence of an upper pouch fistula that emanates from the side (not the end) of the pouch is determined. Also, the back wall of the trachea may be inadvertently entered. This condition is repairable with absorbable sutures.

Extensive dissection of the distal end must be avoided; its blood supply is segmental from the aorta, and it can easily become ischemic. A gap between the ends may seem to be present. If it is very lengthy, the muscular covering of the upper pouch may be cut without entry into the lumen to obtain 1 cm or so of additional length. Distal dissection may be performed; the risk of ischemia should be recognized. If absolutely necessary, the two ends may be simply bridged using two stout silk sutures in the hopes that they form a fistula and that they can be dilated to form a functional esophagus. More commonly, the two ends are reasonably close, and an anastomosis is possible.

The distal portion of the upper pouch is cut off, and the proximal portion of the distal segment is trimmed. The mucosal and muscularis layers of the esophagus should be carefully sutured together in a single layer to form an anastomosis with simple interrupted stitches. Once again, most advocate the use of an absorbable synthetic suture with a caliber of approximately 5-0 (eg, braided polyglactin). The back wall is sutured, and the upper pouch tube is passed through the half-completed anastomosis into the stomach to help rule out a distal stricture and to empty the stomach of accumulated gas.

This tube is left in place as the anterior wall of the anastomosis is completed. The tube is then gently withdrawn from the body. Some advocate leaving the transanastomotic tube to act as a stent, though this tube may be partially moved, potentially injuring the anastomosis. A small-caliber 10-French chest tube may be left in place as an extrapleural chest drain. The ribs are reapproximated by encircling them with two 3-0 absorbable sutures and by restoring their normal anatomic position. The muscles and skin are closed in layers with absorbable sutures.

In a number of pediatric surgical centers, surgeons repair esophageal atresia by means of a minimally invasive thoracoscopic approach.[40, 41, 42, 43, 44] This approach should be undertaken only by those who have extensive experience in pediatric thoracoscopic surgery.

Postoperative Care

The intubated patient is transported to the neonatal intensive care unit (NICU). Antibiotics are continued until the chest drain is removed. A large retrospective series suggested that continuing antibiotics for longer than 24 hours after primary repair of proximal esophageal atresia with distal TEF is unnecessary.[45]

The endotracheal tube is suctioned as necessary. Oral suctioning to a depth of no more than 7 cm from the lips is performed every half hour for the first day, then every hour or more frequently as necessary on day 2 and as needed thereafter. Suctioning is required to handle the sometimes copious oral secretions that can build up in the first day or so after surgery. As the swelling of the esophagus settles, the secretions taper.

The chest draining tube is placed in 2 cm of water only to seal it; it is not connected to a suction device, which could encourage an anastomotic leak. Morphine is infused as necessary for the patient's comfort, and peripheral parenteral nutrition should be commenced. The endotracheal tube should remain until weaning from ventilation is ensured, usually after 1-2 days. Premature extubation and subsequent intubation in the setting of a freshly closed tracheal fistula invites reopening of the fistula.

The surgeon should be alert for any saliva exiting out the chest drain; this is a signal of anastomotic leakage. Often, it is accompanied by visible distress. Signs of sepsis may or may not be present. A chest radiograph should be obtained. Provided that the baby is stable, a contrast-enhanced study of the esophagus with a water-soluble isotonic medium may be performed on day 5, 6, or 7 to assess for leaks and to view the caliber of the repair (see the image below).

This postoperative contrast-enhanced radiograph re This postoperative contrast-enhanced radiograph reveals esophageal gastric tube replacement. Anastomosis to upper pouch is in chest. Linear staple line of tube can be observed.

If the esophagus is patent and reasonably sized, the baby may be orally fed; starting with expressed breast milk is ideal. Then, the chest tube is removed. As soon as the baby is feeding well, the IV line is discontinued, and the baby can be discharged.

Oral ranitidine or a proton pump inhibitor (PPI) is often prescribed because of the propensity for GER in this group of patients and because of the risk of stricture as a secondary effect. It has been suggested that empiric acid suppression may not effectively prevent stricture, leak, or pneumonia.[45]  It is likely that esophagitis in esophageal atresia patients is multifactorial, with peptic esophagitis being only one of several possible causes of esophageal inflammation in this setting.[46]  

Complications

Early

Early complications may include anastomotic leakage,[47] recurrent TEF, and anastomotic stricture.

Anastomotic leak

An anastomotic leak tends to occur 3-4 days after surgery. This leak has been reported in approximately 15% of cases. Pain and distress are often evident. Signs of sepsis may be present. The chest tube drains saliva. Treatment is supportive; appropriate antibiotics should be used, and the child should be given nothing by mouth. Surgery is not indicated, even with huge leaks. If the leak persists, esophagography may be performed with water-soluble contrast material to assess its magnitude.

The usual protocol is to wait and let the leak close. If an extrapleural approach was used, the child is usually less ill than with other approaches, and the resultant esophagocutaneous fistula closes within days. If a transpleural approach was used, the child is usually more ill and has an empyema that may require further treatment and drainage. No absolute evidence indicates that postoperative leaks lead to anastomotic stenoses.  For high-volume postoperative leaks, some studies reported successful use of the anticholinergic agent glycopyrrolate, which markedly reduced salivary production and facilitated leak closure.[48]

Recurrent tracheoesophageal fistula

Recurrent TEF may occur within days; most often, it occurs weeks later. Its incidence has been reported to be in the range of 3-14%. The first manifestation may be pneumonia, though the child may cough and have respiratory distress with feeding. The diagnosis is made by means of esophagography performed with water-soluble contrast material under fluoroscopic guidance with the child prone. The contrast material is slowly injected through a catheter in the esophagus as the tube is slowly withdrawn, and lateral views are obtained by means of videofluoroscopy.

The recurrent fistula is observed as a wisp of contrast material that suddenly crosses over to the trachea. This so-called pullback esophagography is the most accurate method for diagnosing a recurrent fistula. Bronchoscopy and esophagoscopy may provide supplementary information. One endoscopic technique is to inject 0.5 mL of methylene blue into the endotracheal tube and through the esophagoscope while watching for it to come through the fistula.

Historically, these fistulae were believed to require surgical repair by means of repeat right-side thoracotomy; however, the authors have been successful in a minority of cases of fistulae by allowing them to close spontaneously while maintaining the nothing-by-mouth restriction and while administering antibiotics for 1 week. Endoscopic cautery and fibrin glue have also been reported to be occasionally successful.

Anastomotic stricture

Anastomotic stricture has been reported in as many as 50% of cases, but the rate partially depends on the definition of stricture. Essentially, 100% of babies have a waist at the anastomotic site, but this may not be functionally significant. In cases in which the stricture appears to be functionally significant on oral contrast-enhanced studies, esophageal dilation is best and is most safely performed by means of a Grüntzig balloon technique under fluoroscopic control (in the authors' opinion).

This procedure should be performed by an experienced radiologist who can monitor the balloon pressure, position, and inflation diameter. In newborns, this technique of dilatation would best be deferred until the child is aged at least 6 weeks and for at least 4 weeks after the anastomosis. Alternatively, the balloon dilation technique can be performed under endoscopic and fluoroscopic guidance by the surgeon, a gastroenterologist, or both. Direct endoscopic visualization and passage of a guide wire past the area of the stricture help faciliate proper balloon placement and dilation.

Other methods involve the passage of tapered dilators of various sorts (eg, Tucker and Maloney dilators). Certainly, these methods can be effective, but they are performed in essentially a blind manner unless done under fluoroscopic control. They also involve longitudinal and radial force vectors, as opposed to the pure radial force vectors of the Grüntzig technique. Repeat dilations are often necessary. Histamine 2 (H2)-receptor blockade should be started because acid reflux can be both an aggravating and a causative factor in stricture formation.

Other factors to consider include the following:

  • Surgical technique
  • Type of suture used
  • Length of the atretic gap
  • Ischemia of the distal portion
  • Whether an anastomotic leak may have occurred

Strictures resistant to a few dilations require more aggressive treatment, which may include an antireflux operation, stricture resection, or both; rarely, they necessitate esophageal replacement.

There have been anecdotal reports concerning injection of various agents (eg, triamcinolone and mitomycin C) to treat the dilated stricture medically and prevent recurrence. In one report, topical application of the antineoplastic agent mitomycin C was found to be effective in preventing stricture in refractory cases.[49]

Stents have been used but are still investigational. Surprisingly, parents can be taught to perform regular Maloney dilations at home in selected cases.

Late

Late complications may include GER, esophageal dysmotility, and tracheomalacia. Some of these complications may appear early.

Gastroesophageal reflux

GER is particularly problematic in patients with esophageal atresia because of congenital distal dysmotility of the esophagus, dysfunction of the physiologic antireflux barrier, possible partial vagotomy during surgery, or essential vagal dysfunction that can lead to delayed gastric emptying. Essentially all babies with esophageal atresia have detectable GER. Patients who require treatment must be carefully identified.

All babies with esophageal atresia should be prophylactically treated with ranitidine or a PPI (eg, omeprazole). Failure to thrive, coughing, choking spells, wheezing and asthma, recurrent pneumonias, vomiting, cyanosis, dying spells, excessive drooling, and apparent dysphagia are all indications to investigate the degree of GER. Oral contrast material should be administered, and endoscopy should be performed. Strictures should be dilated. A pH probe study may help if the probe is placed below any present stricture. A gastric emptying scan should be obtained. All factors should be carefully considered.

Surgical approaches to helping the child may include an antireflux operation. A partial-wrap fundoplication is usually preferred because of the dysmotility of the repaired esophagus. Dysphagia after even a very loose wrap is not uncommon. If the stomach has delayed emptying, balloon pyloroplasty or surgical pyloroplasty may be considered to speed emptying. The authors have used a surgically conservative approach in children with this condition; they prefer to treat the reflux medically with H2-receptor blockade or a PPI when possible. It is certain, however, that some patients require a surgical approach for later complications.

Esophageal dysmotility

Esophageal dysmotility is an ongoing problem.[50] It has various dysphagic manifestations. The children eventually learn that they must masticate thoroughly and drink fluids when eating. Food bolus obstructions, even without a significant stricture, are not uncommon in toddlers. Parents must be mindful of this possibility and choose their child's foods accordingly. The use of motility agents such as domperidone may help.

Tracheomalacia

Tracheomalacia is a manifestation of disordered embryogenesis. In its severe form (occurring in ~10% of patients), dramatic signs include an inability to wean the patient from a ventilator and the classic dying spells in which the patient becomes pale and limp and, usually, apneic and cyanotic for a short time. Children with this condition require examination and treatment. Milder cases of tracheomalacia may cause recurrent pneumonias or asthma attacks, and in general respiratory ailments are common in these children.

Bronchoscopy performed while the patient is spontaneously breathing reveals a trachea that significantly collapses, flattens, or closes upon expiration. Treatment consists of aortopexy,[51] which suspends the aortic arch to the underside of the sternum and thereby secondarily suspends the anterior tracheal wall anteriorly, preventing its collapse. If this is unsuccessful, stent placement may help, but this option is controversial. Tracheostomy is the final management option. Fortunately, tracheomalacia tends to improve with time, growth, and maturation.

Long-Term Monitoring

If all is well with the patient and if the parents have been briefed on what to look for, a reasonable follow-up regimen may include the following steps:

  • The surgeon makes contact with the community physician who is responsible for the general medical care of the child and ensures that he or she is briefed on the baby's history, condition, and expected outcome
  • The nurse on the surgical team follows up by telephone in 1 week
  • The surgeon follows up in 1 month to interview the parents and to make a general assessment of the child's condition, growth, and healing at the surgical site
  • The patient returns at 3 months for a similar assessment
  • At a 1-year follow-up and general assessment, swallowing function, respiratory issues, and other factors are addressed; signs of an undetected stricture may manifest with the conversion to table food

Radiologic assessment of the esophagus is required only if there is a significant history of choking, cyanosis, regurgitation, dysphagia, growth failure, coughing, or wheezing. Subsequent endoscopic evaluation can be performed as indicated.

Follow-up care when the child is older can be performed as needed. Specific reassessment with esophageal endoscopy and biopsy when the patient is aged approximately 12 years has been advised by some who also advise follow-up with periodic endoscopy every few years until the patient is an adult. Although Barrett esophagus and subsequent malignant change have been described in this condition, presumably because of GER, it remains unclear whether endoscopic surveillance is necessary in patients with repaired esophageal atresia.[52, 53]