A tracheoesophageal fistula is a connection between the trachea and oesophagus, usually as a result of a failure of normal development.
Tracheoesophageal fistula is generally associated with oesophageal atresia
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.