V. ESOPHAGEAL ATRESIA (EA) WITH OR WITHOUT TRACHEOESOPHAGEAL
FISTULA (TEF)
Esophageal atresia with or without tracheoesophageal fistula occurs in about 1 in
4500 births. EA/TEF can occur in associated with other anomalies, therefore in a child
with EA/TEF these anomalies must be sought out. The cluster of anomalies often
found with EA is termed VACTERL (acronym for the systems affected. In a baby with
EA/TEF, Vertebral anomalies (baby gram), Anus, Imperforate (physical exam), Cardiac
(ECHO), Tracheo-Esophageal, Renal anomalies (ultrasound), Limb anomalies (radial
dysplasia) can be found. In addition, up to 7% of babies can have a tethered cord. No
genetic association has been found.EA/TEF is usually occurs sporadically, although familial cases have been reportedBabies with EA/TEF may have antenatal history of maternal polyhydramnios..
After birth, there are copious secretions, often w/coughing and choking. Intermittent
cyanosis can be seen, as the baby may aspirate their oral secretions. If the baby was
bagged during delivery, abdominal distention may be seen if a distal fistula is present.
A definitive bedside test is the inability to pass an orogastric tube in the stomach.
Several anatomic types of EA and TEF occur. The most common is EA with
distal TEF (85-87%). Isolated EA (aka “long gap” EA) occurs in 7% of cases. A fistula
can occur connecting an intact trachea and esophagus (“H-type fistula”) occurs 4% of