Azygos vein anomaly: the best predictor of a long gap in esophageal atresia and tracheoesophageal fistula.

The gap between the pouches has a direct bearing on the tension at the anastomosis that ultimately determines the anastomotic leak in esophageal atresia with or without tracheoesophageal fistula (EA-TEF). Long-gap EA has been reported to be associated with aortic-arch anomalies and 13 pairs of ribs. Our observation that EA-TEF with an azygos-vein anomaly (AVA) invariably had a long gap led us to retrospectively analyze our data. The mean gaps (+/- SD) in the groups with 13 pairs of ribs (n = 6), right-sided aortic arch (n = 16), and AVA (n = 9) were 1.25 +/- 0.27, 2.18 +/- 0.98, and 3.16 +/- 0.16 cm, respectively. There was no statistically significant difference in the gap in patients who had 13 pairs of ribs compared with controls who had 12 pairs of ribs. The gap was highest in the AVA group and was statistically significant (P < 0.001) compared with patients with a normal azygos vein. In addition, the AVA group had a significant increase in mortality (P < 0.05) compared to the groups with a normal azygos vein; although there was no statistically significant difference in other factors: birth weight, time between delivery and surgery, cardiac anomalies, anorectal malformations, renal malformations, and chest infection in the AVA group and controls. An extra pair of ribs is not associated with a long gap, and an AVA per se is an independent predictor of a long gap and mortality in EA-TEF.