High proximal pouch esophageal atresia with vertebral, rib, and sternal anomalies: an additional component to the VATER association.

The base of the proximal esophageal pouch in babies born with esophageal atresia varies in location from the low cervical region to the upper third of the mediastinum, but the relevance of this finding to the VATER association is not known. The initial radiographs and hospital records of 42 newborns with esophageal atresia and a distal tracheoesophageal fistula treated in the Newborn Special Care Unit of the Yale-New Haven Hospital between 1977 and 1986 were examined to (1) determine the location of the base of the upper esophageal pouch on its superimposed vertebral body; (2) identify coexisting VATER associated anomalies; and (3) detect the presence of vertebral, rib, or sternal anomalies. Ten of 11 babies with the upper esophageal pouch at the first thoracic vertebra or above had additional VATER defects including vertebral, rib, or sternal anomalies. These patients also required staged repair or circular myotomy for wide gap esophageal atresia and were at greater risk of anastomotic leak, stricture, or gastroesophageal reflux requiring fundoplication. High proximal pouch esophageal atresia accompanied by vertebral, rib, and sternal anomalies constitutes a heretofore unrecognized high risk group with the VATER association.