Esophageal atresia
Updates to Article Attributes
An oesophageal atresia refers to an absence in contiguity 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 1.
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 successfully pass a catheter into the stomach.
Pathology
It results from a failure of the primitive foregut to divide in to 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.
Sub types
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
- other intestinal atresias
- annular pancreas
- pyloric stenosis
- VACTERL association inclusive of congenital cardiac anomalies
- CHARGE syndrome
- increased incidence of chromosomal anomalies such as
Radiographic features
Chest radiograph
- 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 oesophago-gastric (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.
Contrast swallow/fluoroscopy
- 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 fistulae.
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 sub types 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.
Esophageal strictures secondary to surgical correction of esophageal atresia is a known complication to the procedure and usually requires further surgical correction.
-<li><a href="/articles/edward-syndrome">trisomy 18</a></li>- +<li><a href="/articles/edwards-syndrome-1">trisomy 18</a></li>
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