Tracheal agenesis in infants with VATER association

Four newborns had tracheal agenesis and numerous features of the VATER (vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) association. Forty-two additional reported cases of tracheal agenesis have been reviewed to establish whether this defect usually occurs in association with other VATER abnormalities. Since tracheal agenesis usually is accompanied by other VATER-associated defects and since similar abnormalities in embryological mechanisms and timing are implicated, according to current theories of development, we propose that tracheal agenesis should be considered a component of the VATER association.