Surgical Management and Outcomes in 100 Infants with Long-Gap Esophageal Atresia: A Nationwide Analysis of Children’s Hospitals
Annalise B. Penikis1, Pooja S. Salvi2, Shelby R. Sferra1, Abigail Engwall-Gill1, Daniel Rhee1, Daniel Solomon2, Shaun Kunisaki1
1Pediatric Surgery, Johns Hopkins School of Medicine, Baltimore, Maryland, United States, 2Pediatric Surgery, Yale University School of Medicine, New Haven, Connecticut, United States
Objective. Infants with long-gap esophageal atresia (LGEA) represent a rare but important subgroup of esophageal atresia patients with respect to operative strategy and postoperative complications. The purpose of this study was to evaluate the contemporary surgical management of LGEA infants using a national database. Design. Retrospective multicenter cohort study Setting. Participating children's hospitals in the Pediatric Health Information System database Patients. Infants with esophageal atresia who underwent neonatal gastrostomy with subsequent delayed primary esophageal repair after 28 days of life were identified between 1/1/2014 and 12/31/2021. Those with a birthweight less than 1.5 kg and those who received cardiac surgery in the first month of life were excluded. Interventions. Esophageal repair, endoscopic dilation, fundoplication Main Outcome Measures. Type and timing of esophageal repair, hospital length of stay (LOS), number of postoperative procedures Results. Of 1,035 infants who underwent operative management of esophageal atresia identified from 47 hospitals, 100 (9.7%) had LGEA. Cardiac anomalies were seen in 43.0%. The median age at esophageal repair was 87 days [IQR (62-133)]. Of the 86.0% of patients undergoing primary repair who did not require reoperation, median age of repair of 85.5 days [IQR (61.3-133)]. Ten percent underwent planned or unplanned early reoperation (defined as <=30d after index procedure) while 4.0% required reoperation >30 days after their index surgery. Among those requiring reoperation, median time to first reoperation was 9 days [IQR (7-59.8)]. Mortality during index admission was 5.0% and median postoperative LOS was 143 days [IQR (99-193)]. Fifty two percent required at least one postoperative dilation for anastomotic stricture. Median time to first dilation after index repair was 69.5 days [IQR (42.3-172.5)]. Eight percent required a subsequent fundoplication for reflux with a median time to surgery from index repair of 295 days [IQR (99.3-327)]. Conclusion. In LGEA, this large multicenter study suggests a high rate of successful delayed primary repair without the need for subsequent reoperations. Nevertheless, postoperative length of stays are prolonged, and nearly half of infants require endoscopic dilation. These data highlight the current challenges of LGEA and are useful for counseling discussions with families in the early postnatal period.
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