An esophageal atresia refers to an absence in the contiguity of the esophagus due to an inappropriate division of the primitive foregut into the trachea and esophagus. This is the most common congenital anomaly of the esophagus.
It is thought to occur in ~1:3000-4500 live births 3.
Esophageal 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.
It results from a failure of the primitive foregut to divide into the trachea anteriorly and the esophagus posteriorly. This usually occurs at ~ the 4th week of gestation.
General pathogenesis includes teratogenic effects caused by early pregnancy use of antithyroid drugs 7
Most cases have a sporadic occurrence 6.
It is frequently associated with a tracheo-esophageal fistula. As such, the types of esophageal atresia / tracheo-esophageal fistula can be divided into 4:
- proximal atresia with distal fistula: 85%
- isolated esophageal 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: esophageal atresia classification.
Esophageal 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
- may show a dilated pharyngeal pouch
- the presence of air in the stomach and bowel in the setting of esophageal atresia implies that there is a distal fistula
- if an esophagogastric (feeding) tube insertion has been attempted this may show the tube blind looping and turning back at the upper thoracic part of the esophagus or heading into the trachea and/or bronchial tree
Contrast swallow may show contrast blindly ending and pooling in an esophageal stump and/or may show evidence of the tracheo-esophageal fistula. Fluoroscopy is particularly useful in demonstrating H-type fistula.
- 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-esophageal fistula may be suspected in the context of persistent non-visualization of the fetal stomach
- has been trialled for 3D detailed visualization 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 oesophagal atresia is a known complication of the procedure and usually requires further surgical correction.
- 1. Berrocal T, Madrid C, Novo S et-al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24 (1): e17. doi:10.1148/rg.e17 - Pubmed citation
- 2. Mccook TA, Felman AH. Esophageal atresia, duodenal atresia, and gastric distention: report of two cases. AJR Am J Roentgenol. 1978;131 (1): 167-8. AJR Am J Roentgenol (citation) - Pubmed citation
- 3. Fitoz S, Atasoy C, Yagmurlu A et-al. Three-dimensional CT of congenital esophageal atresia and distal tracheoesophageal fistula in neonates: preliminary results. AJR Am J Roentgenol. 2000;175 (5): 1403-7. AJR Am J Roentgenol (full text) - Pubmed citation
- 4. Bluestone CD. Pediatric otolaryngology. Saunders. (2003) ISBN:0721691978. Read it at Google Books - Find it at Amazon
- 5. Lam WW, Tam PK, Chan FL et-al. Esophageal atresia and tracheal stenosis: use of three-dimensional CT and virtual bronchoscopy in neonates, infants, and children. AJR Am J Roentgenol. 2000;174 (4): 1009-12. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Entezami M, Albig M, Knoll U et-al. Ultrasound Diagnosis of Fetal Anomalies. Thieme. (2003) ISBN:1588902129. Read it at Google Books - Find it at Amazon
- 7. Andersen SL, Olsen J, Wu CS et-al. Birth defects after early pregnancy use of antithyroid drugs: a Danish nationwide study. J. Clin. Endocrinol. Metab. 2013;98 (11): 4373-81. doi:10.1210/jc.2013-2831 - Pubmed citation
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