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. On ultrasonography (US), the infant may have a small or absent stomach. Note that polyhydramnios is observed in infants with many diagnoses. Only one 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 the 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 distention. 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.
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Fetal MRI showing normal esophagus. This study is negative for esophageal atresia. Hypopharynx and proximal esophagus are of normal caliber (arrows), and normal thoracic esophagus (E) is observed.
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Fetal MRI revealing esophageal atresia. Proximal esophagus and hypopharynx are dilated (arrow). Thoracic esophagus is not discernible.
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Plain radiograph in newborn suspected of having esophageal atresia. Air in stomach and intestine suggest distal tracheoesophageal fistula.
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Pure esophageal atresia. Newborn with tube in proximal pouch. Note that no air is in stomach or intestine (gasless abdomen).
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Animation illustrating method of estimating distance for esophageal atresia (EA) with distal tracheoesophageal fistula (TEF). Small amount of air has been injected into upper pouch. Upper pouch and bifurcation of trachea are marked. Distal fistula is usually posterior to carina.
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Contrast study of upper pouch in infant with esophageal atresia and distal tracheoesophageal fistula.
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Tracheobronchial remnant in distal esophagus of infant with pure esophageal atresia.
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Gapogram estimating distance between proximal pouch (P) and distal pouch (D). Proximal pouch has mercury-weighted dilator, and distal pouch is filled with contrast injected through gastrostomy (G). Distance is more than 5 cm and five vertebral bodies.
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Gapogram demonstrating 3.4-cm gap. Distal pouch is being stretched with radiopaque dilator.
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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.
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Intraoperative photograph showing azygos vein. Right thoracotomy and retropleural dissection have been performed.
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Repair of esophageal atresia. Head is to left. Azygos vein has been divided. Proximal (P) and distal (D) esophageal pouches can be easily observed.
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Primary anastomosis (A) between proximal and distal esophagus.
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Contrast study demonstrating colonic interposition. Colon is sutured to cervical esophagus proximally and to stomach distally. No leak or stricture is noted.
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Contrast swallow (oblique view) demonstrating gastric pull-up used as esophageal replacement in child with pure esophageal atresia. Note stomach in chest.
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Intraoperative photograph demonstrating proximal H-type fistula (F) between proximal esophagus (P) and trachea (T). Blue vessel loop is around fistula. H-type fistula was not diagnosed preoperatively but was identified during mobilization of proximal esophagus in child with congenital esophageal stenosis.
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Intraoperative photograph following division and repair of H-type fistula. Note sutures in esophagus (E) and trachea (T).
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Intraoperative photograph of infant thought to have esophageal atresia. Proximal (P) and distal portions of esophagus were connected by fibrotic segment of congenital esophageal stenosis (CES) beginning at azygos vein (A). No distal tracheoesophageal fistula was present.
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Intraoperative photograph following resection of atretic segment of esophagus and primary anastomosis (A) of proximal (P) and distal esophagus. Azygos vein (AV) was preserved.
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Chest radiograph immediately following repair of esophageal atresia and distal tracheoesophageal fistula (TEF). Note chest tube (CT) in retropleural space and feeding tube passing through anastomosis (A) into stomach.
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Chest radiograph following repair of esophageal atresia. Chest tube is placed in retropleural space (CT). No feeding tube is passing across anastomosis.
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Esophagogram 1 week following primary anastomosis for pure esophageal atresia. No leak or stricture is observed.
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Anastomosis disruption following repair of esophageal atresia with tracheoesophageal fistula. Contrast was injected through gastrostomy and refluxed through distal esophagus. Leak (L) is draining into retropleural chest tube.
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Esophagogram 1 week after repair of esophageal atresia with distal tracheoesophageal fistula. Note narrowing at anastomosis (A). Child was asymptomatic and required no dilatations. Image 1 of 3 in series.
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Follow-up esophagram demonstrating spontaneous improvement of narrowing at anastomosis (A). Image 2 of 3 in series.
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Esophagogram. Note spontaneous resolution of narrowing at anastomosis (A). No intervention was required in this child. Image 3 of 3 in series.
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Esophagogram 7 days following repair of esophageal atresia. Note change in caliber from proximal esophagus to distal esophagus at anastomosis (A). Retropleural chest tube can be observed (CT). This patient had gastroesophageal reflux and developed progressive stricture. Image 1 of 3 in series.
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Esophagogram obtained 3 weeks later demonstrating stricture (A) and gastroesophageal reflux (R and green arrow). Medical therapy was initiated. Image 2 of 3 in series.
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Esophagogram performed 2 weeks later demonstrating worsened anastomotic stricture (A). Patient responded well to Bouginage dilatation and medical management of reflux. Image 3 of 3 in series.
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Esophagogram demonstrating impacted food in 5-year-old child with history of esophageal atresia repair.
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Azygos vein prior to division. Distal fistula lies beneath this vein.
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Distal tracheoesophageal fistula is clipped and divided.
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Posterior wall of anastomosis is completed. Interrupted absorbable sutures on anterior wall will complete anastomosis.
