This complex group of anomalies, with an incidence of 1 in 2440-4500 live births,4'5 results from failure of correct division of the tracheal primordium from the esophagus during early embryonic development. The precise etiology is unknown with a number of embryological theories proposed to explain the different variants of this anomaly. There is a high incidence of co-existing abnormalities including the VACTERL syndrome, CHARGE association and isolated cardiovascular anomalies.6
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