Outcome of primary repair in extremely and very low-birth-weight infants with esophageal atresia/distal tracheoesophageal fistula

Purpose The optimal surgical management of extremely (ELBW) and very low-birth-weight (VLBW) neonates with esophageal atresia and distal tracheoesophageal fistula (EA/TEF) (Gross type C) is still debated. The aim of this study was to evaluate the surgical outcome of primary repair in these patients and compare it to ≥1500g neonates. Methods Medical records of neonates with repaired EA from 2002 to 2016 were reviewed. Results 4 ELBW, 7 VLBW, and 24 ≥1500g infants had type C EA/TEF and underwent primary repair. Anastomotic leakage occurred in 0% ELBW, 0% VLBW and 8.3% ≥1500g patients and anastomotic stricture in 25% ELBW, 28.5% VLBW and 37.5% ≥1500g patients. 50% ELBW, 14.2% VLBW and 20.8% ≥1500g patients underwent secondary fundoplication. One patient of the VLBW group and one patient of the ≥1500g group died postoperatively of causes not related to EA/TEF. Conclusions In extremely and very low-birth-weight neonates with type C EA/TEF surgical outcome after primary repair is comparable to the outcome in ≥1500g neonates. Primary repair can be performed in most of these patients and staged repair can be restricted to unstable patients. Level of evidence Treatment study level III.