eMedicine Specialties > Pediatrics: Surgery > General Surgery

Congenital Anomalies of the Esophagus

Author: Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy
Coauthor(s): Alison Snyder-Warwick, MD, Research Fellow, Department of Developmental Biology and Department of Surgery, Division of Plastic and Reconstructive Surgery, Washington University School of Medicine; Mark V Mazziotti, MD, Assistant Professor of Pediatric Surgery, Department of Surgery, Baylor College of Medicine, Texas Children's Hospital; Jacob C Langer, MD, Professor, Department of Surgery, University of Toronto Faculty of Medicine
Contributor Information and Disclosures

Updated: Oct 31, 2008

Introduction

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. Other lesions, such as congenital esophageal stenosis, duplications, and cysts, occur less frequently.

For excellent patient education resources, visit eMedicine's Esophagus, Stomach, and Intestine Center. Also, see eMedicine's patient education article, Choking.

History of the Procedure

The recorded history of EA dates back as early as 1670 when Durston described the presence of a blind-ending upper esophageal pouch in a conjoined twin; however, surgical therapy for EA was not suggested until 1869. Steele made the first attempt at surgical correction for EA in 1888. He performed a gastrostomy in a patient with pure EA, hoping to perforate what he suspected to be an esophageal membrane. In 1913, Richter proposed fistula ligation with anastomosis of the 2 esophageal ends for EA with TEF. Although he considered primary repair to be the best option, he also acknowledged the impracticality of this procedure at the time. Instead, he ligated the fistula intrathoracically. His patient did not survive long enough to attempt an esophageal anastomosis.

The first patient to survive a congenital esophageal anomaly was born in 1931 with a TEF and no atresia. The fistula was repaired with a transtracheal incision in 1935, the same year that the first survivor of EA was born. The infant with EA was treated with gastrostomy feedings and a jejunal interposition. Both of these children had an isolated defect (atresia or fistula), and treatment was successful without a thoracotomy. The treatment for EA with TEF proved to be more difficult. Pneumonia, mediastinitis, poor airway control, and fluid management problems were frequent complications.

In 1936, Lanman was the first to perform a repair with an extrapleural approach. The first patient to undergo the technique survived only 3 hours. In 1938, Shaw performed the first fistula ligation and primary anastomosis of the esophagus for EA-TEF. This patient died 12 days postoperatively from a transfusion reaction.

In 1939, the first 2 successful treatments of patients with EA-TEF occurred independently, one day apart, by Leven and Ladd. They performed staged repairs involving gastrostomy placement followed by fistula ligation 5 weeks and 4 months later, respectively. Cervical esophagostomies, the use of jejunal interposition, and an antethoracic skin tube for esophageal reconstruction were use in the years to follow.

Haight completed the first successful primary repair in 1941. The procedure involved a left extrapleural approach, fistula ligation, and a single-layer esophageal anastomosis. Haight later switched to a right extrapleural approach and modified his technique to a 2-layer telescoping anastomosis in an attempt to diminish leak risk.

By 1944, one third of the children with EA-TEF survived primary repair. Advances in preoperative preparation, antibiotic treatment, and intraoperative and postoperative management contributed to more favorable survival rates. Despite the increased success, leaks, strictures, and lower esophageal segment dysmotility were common postoperative problems.

Problem

EA and TEF are the most common anomalies and, therefore, receive more emphasis. Congenital stenosis or obstruction is also encountered. Congenital muscular hypertrophy, webs, cysts, and tracheobronchial remnants are observed.

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.

Several different types of EA and TEF have been described. The frequencies calculated from a summary of 6 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.

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. 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.

Frequency

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.

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 the 26th day. 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 need 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.

Pathophysiology

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.

Presentation

The earliest clinical sign of an infant with 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 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 CT scanning obtained for recurrent pneumonia or unrelated reasons.

Indications

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 prenatally and after birth by clinical signs and supportive findings on imaging studies. Prenatally, an ultrasonographic finding of a small or absent stomach bubble suggests EA with 42% sensitivity. Prenatal MRI was used to evaluate the esophagus of fetuses with a small or absent stomach bubble on ultrasonographic evaluation.1 Positive findings on prenatal MRI had a sensitivity of 100% and specificity of 80%. After birth, failure of passage of a rigid radiopaque 10F 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 esophagraphy, 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. More recently, ventilator dependence and severe anomalies, not birth weight, have been linked to mortality.2 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 based on diagnosis and the presence of symptoms.

Relevant Anatomy

The esophagus can be divided into several segments based on 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 1-3 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 3 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.3 The surgical approach should be performed on the side opposite of the aortic arch. A right-sided aortic arch occurs in 5% of infants with esophageal atresia (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 tracheoesophageal fistula (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.

Contraindications

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 when preparing for surgery, and the most serious abnormalities should be corrected first.

More on Congenital Anomalies of the Esophagus

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Workup: Congenital Anomalies of the Esophagus
Treatment: Congenital Anomalies of the Esophagus
Follow-up: Congenital Anomalies of the Esophagus
Multimedia: Congenital Anomalies of the Esophagus
References
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Further Reading

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Keywords

congenital anomalies of the esophagus, esophageal atresia, EA, tracheoesophageal fistula, TEF, esophageal stenosis, esophageal cyst, tracheobronchial remnant, esophageal atresia and tracheoesophageal fistula, EA-TEF, esophageal web, esophageal muscular hypertrophy, esophageal duplications, esophageal rests, columnar epithelium–lined lower esophagus, Barrett esophagus, Barrett's esophagus, laryngotracheoesophageal cleft, LTEC, gastroesophageal reflux, GERD, oculodigitoesophageoduodenal, ODED, Feingold syndrome, trisomy 18, Down syndrome, pneumonia, atelectasis, polyhydramnios, pneumonitis, tetralogy of Fallot, imperforate anus

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Contributor Information and Disclosures

Author

Robert K Minkes, MD, PhD, Professor of Surgery, University of Texas Southwestern; Chief of Surgical Services, Children's Medical Center of Dallas-Legacy
Robert K Minkes, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Alison Snyder-Warwick, MD, Research Fellow, Department of Developmental Biology and Department of Surgery, Division of Plastic and Reconstructive Surgery, Washington University School of Medicine
Alison Snyder-Warwick, MD is a member of the following medical societies: Alpha Omega Alpha, Phi Beta Kappa, and Sigma Xi
Disclosure: Nothing to disclose.

Mark V Mazziotti, MD, Assistant Professor of Pediatric Surgery, Department of Surgery, Baylor College of Medicine, Texas Children's Hospital
Mark V Mazziotti, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Jacob C Langer, MD, Professor, Department of Surgery, University of Toronto Faculty of Medicine
Jacob C Langer, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Society for Surgery of the Alimentary Tract, and Society of University Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Robert Kelly, MD, Chairman, Department of Surgery, Departments of Surgery and Pediatrics, Children's Hospital of the King's Daughters; Associate Professor, Eastern Virginia Medical School
Robert Kelly, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society of Abdominal Surgeons, Medical Society of Virginia, Norfolk Academy of Medicine, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Deborah F Billmire, MD, Associate Professor, Department of Surgery, Indiana University Medical Center
Deborah F Billmire, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Phi Beta Kappa, and Society of Critical Care Medicine
Disclosure: Nothing to disclose.

CME Editor

H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
Disclosure: Nothing to disclose.

Chief Editor

Marleta Reynolds, MD, Professor of Surgery, Feinberg School of Medicine, Northwestern University; Interim Head, Division of Pediatric Surgery, Department of Surgery, Children's Memorial Hospital of Chicago
Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association
Disclosure: Nothing to disclose.

 
 
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