congenital atresia of the oesophagus

Last edited 08/2022 and last reviewed 08/2022

Oesophageal atresia is a congenital abnormality occurring with an incidence of about 1 in 2500 live births.

• in the United States, the prevalence is estimated to be 2.3 per 10,000 live births (1)

The oesophagus develops as a derivative of the foregut, from the floor of where the larynx and trachea become separated by the laryngo-tracheal groove.

• failure of separation or complete development of this foregut tube can lead to tracheo - oesophageal fistula (TOF) and oesophageal atresia (OA)
  • oesophageal atresia presents in neonates as excessive drooling, choking, and failure to pass a nasogastric tube
  • infants with TOF classically present with respiratory distress, feeding difficulties, choking, and risk for aspiration (2)

It is often associated with a tracheo-oesophageal fistula.

There different types of oesophageal atresia:

• Type A oesophageal atresia
  • isolated oesophageal atresia without associated tracheo-oesophageal fistula and has a prevalence of 8%
• Type B oesophageal atresia
  • oesophageal atresia with a proximal tracheo-oesophageal fistula
  • the rarest with a prevalence of 1%
• Type C oesophageal atresia
  • is the most prevalent at 84%
  • a proximal oesophageal atresia with distal tracheo oesophageal fistula
• Type D oesophageal atresia
  • is oesophageal atresia with both a proximal and distal tracheo - oesophageal atresia
  • is rare at 3% of cases
• Type E esophageal atresia (H-type fistula) (2)
  • an isolated tracheoesophageal fistula without associated oesophageal atresia
  • prevalence around 4%

Oeosophageal atresia has a high incidence in mothers with polyhydramnios - as high as 85% - and should always be excluded in the baby of mothers with this condition.

Notes:

• approximately 50% of patients with TOF/EO will have associated congenital anomalies including:
  • VACTERL syndrome (vertebral defects, anal atresia, cardiac defects, TEF, renal anomalies, and limb abnormalities) or
  • CHARGE syndrome (coloboma, heart defects, atresia choanae, growth retardation, genital abnormalities, and ear abnormalities)

Reference:

• Baldwin D, Yadav D. Esophageal Atresia. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK560848/
• Salik I, Paul M. Tracheoesophageal Fistula. In: StatPearls. StatPearls Publishing, Treasure Island (FL); 2021. PMID: 30570997.