Esophageal atresia

Changed by Roland Warner, 26 Jun 2019

Updates to Article Attributes

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Oesophageal atresia refers to an absence in the continuity of the oesophagus due to an inappropriate division of the primitive foregut into the trachea and oesophagus. This is the most common congenital anomaly of the oesophagus. 

Epidemiology

It is thought to occur in ~1:3000-4500 live births 3.

Clinical presentation

Oesophageal atresia may be suspected on antenatal ultrasound (see below) or in the neonate due to inability to swallow saliva or milk, aspiration during early feedings, or failure to pass a catheternasogastric tube into the stomach successfully.

Pathology

It results from a failure of the primitive foregut to divide into the trachea anteriorly and the oesophagus posteriorly. This usually occurs at ~ the 4th week of gestation.

General pathogenesis includes teratogenic effects caused by early pregnancy use of antithyroid drugs 7

Genetics

Most cases have a sporadic occurrence 6.

Subtypes

It is frequently associated with a tracheo-oesophageal fistula. As such, the types of oesophageal atresia / tracheo-oesophageal fistula can be divided into 4:

  • proximal atresia with distal fistula: 85%
  • isolated oesophageal atresia: 8-9%
  • isolated fistula (H-type): 4-6%
  • double fistula with intervening atresia: 1-2%
  • proximal fistula with distal atresia: 1%

See main article:oesophageal atresia classification.

Associations

Oesophageal atresias are frequently associated with various other anomalies (50-75% of cases). They include

Radiographic features

Plain radiograph

Chest x-ray:

  • may show a dilated pharyngeal pouch
  • the presence of air in the stomach and bowel in the setting of oesophageal atresia implies that there is a distal fistula
  • if an oesophagogastric (feeding) tube insertion has been attempted this may show the tube blind looping and turning back at the upper thoracic part of the oesophagus or heading into the trachea and/or bronchial tree
Fluoroscopy

Contrast swallow may show contrast blindly ending and pooling in an oesophageal stump and/or may show evidence of the tracheo-oesophageal fistula. Fluoroscopy is particularly useful in demonstrating H-type fistula. A withdrawal esophagogram is performed for a H-type fistula. The patient is positioned prone and a horizontal beam is used, contrast material is injected through the nasogastric tube into the distal esophagus while the tube is slowly withdrawn under fluoroscopy 8. If the imaging study fails to locate the fistula while there is high suspicion then combined bronchoscopy and esophagoscopy is used 8. The gap between esophageal pouches is determined by inserting a Higer dilator through gastronomy into the distal esophagus and Repogle tube into proximal pouch; both tubes are radio-opaque 8

Antenatal ultrasound

  • non-specific and but may not show the expected shape and position of the stomach
  • should be suspected if other VACTERL anomalies are seen
  • polyhydramnios may be present particularly in cases where there is no concurrent fistulation
  • the fetus may appear growth restricted
  • the subtypes without a tracheo-oesophageal fistula may be suspected in the context of persistent non-visualisation of the fetal stomach
CT
  • has been trialled for 3D detailed visualisation of anatomy but not part of routine investigation 3

Treatment and prognosis

Surgical intervention with a re-anastomosis can be attempted post delivery. The prognosis is variable dependent on other associated anomalies. 

Oesophageal strictures secondary to surgical correction of oesophagal atresia is a known complication of the procedure and usually requires further surgical correction.

  • -<p><strong>Oesophageal</strong><strong> atresia</strong> refers to an absence in the continuity of the oesophagus due to an inappropriate division of the primitive foregut into the <a href="/articles/trachea">trachea</a> and <a href="/articles/oesophagus">oesophagus</a>. This is the most common congenital anomaly of the oesophagus. </p><h4>Epidemiology</h4><p>It is thought to occur in ~1:3000-4500 live births <sup>3</sup>.</p><h4>Clinical presentation</h4><p>Oesophageal atresia may be suspected on antenatal ultrasound (see below) or in the neonate due to inability to swallow saliva or milk, aspiration during early feedings, or failure to pass a catheter into the stomach successfully.</p><h4>Pathology</h4><p>It results from a failure of the primitive foregut to divide into the trachea anteriorly and the oesophagus posteriorly. This usually occurs at ~ the 4<sup>th</sup> week of gestation.</p><p>General pathogenesis includes teratogenic effects caused by <a href="/articles/birth-defects-linked-to-antithyroid-drug-treatment-in-pregnancy">early pregnancy use of antithyroid drugs</a> <sup>7</sup></p><h5>Genetics</h5><p>Most cases have a sporadic occurrence <sup>6</sup>.</p><h5>Subtypes</h5><p>It is frequently associated with a <a href="/articles/congenital-tracheo-oesophageal-fistula">tracheo-oesophageal fistula</a>. As such, the types of oesophageal atresia / tracheo-oesophageal fistula can be divided into <sup>4</sup>:</p><ul>
  • +<p><strong>Oesophageal</strong><strong> atresia</strong> refers to an absence in the continuity of the oesophagus due to an inappropriate division of the primitive foregut into the <a href="/articles/trachea">trachea</a> and <a href="/articles/oesophagus">oesophagus</a>. This is the most common congenital anomaly of the oesophagus. </p><h4>Epidemiology</h4><p>It is thought to occur in ~1:3000-4500 live births <sup>3</sup>.</p><h4>Clinical presentation</h4><p>Oesophageal atresia may be suspected on antenatal ultrasound (see below) or in the neonate due to inability to swallow saliva or milk, aspiration during early feedings, or failure to pass a nasogastric tube into the stomach successfully.</p><h4>Pathology</h4><p>It results from a failure of the primitive foregut to divide into the trachea anteriorly and the oesophagus posteriorly. This usually occurs at ~ the 4<sup>th</sup> week of gestation.</p><p>General pathogenesis includes teratogenic effects caused by <a href="/articles/birth-defects-linked-to-antithyroid-drug-treatment-in-pregnancy">early pregnancy use of antithyroid drugs</a> <sup>7</sup></p><h5>Genetics</h5><p>Most cases have a sporadic occurrence <sup>6</sup>.</p><h5>Subtypes</h5><p>It is frequently associated with a <a href="/articles/congenital-tracheo-oesophageal-fistula">tracheo-oesophageal fistula</a>. As such, the types of oesophageal atresia / tracheo-oesophageal fistula can be divided into <sup>4</sup>:</p><ul>
  • -<li>non-specific and but may not show the expected shape and position of the stomach</li>
  • +<li>non-specific but may not show the expected shape and position of the stomach</li>

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