Oesophageal atresia

Oesophageal atresia is a congenital abnormality occurring with a worldwide prevalence of 2.4 to 3.2 per 100,00 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 oesophageal atresia (H-type fistula) (2)
  • an isolated tracheoesophageal fistula without associated oesophageal atresia
  • prevalence around 4%

Oesophageal 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:

1. Traini I, Menzies J, Hughes J, et al; Oesophageal atresia: The growth gap. World J Gastroenterol. 2020 Mar 28;26(12):1262-1272.
2. Salik I, Paul M. Tracheoesophageal Fistula. In: StatPearls. StatPearls Publishing, Treasure Island (FL); 2021. PMID: 30570997.