Esophageal morbidity in patients following repair of esophageal atresia: A systematic review

Background: Esophageal atresia (EA) is a life-threatening congenital condition, affecting one in 2600 newborns. Morbidity remains high, with many patients experiencing complications, including anastomotic leak/stricture, and gastro-esophageal reflux disease (GERD). Increased understanding of esophageal motility patterns may help explain the etiology of these complications. Aims: We aimed to review knowledge regarding esophageal motility and related complications in children with EA, evaluate patients' symptomatology and relate this to esophageal motility. Methods: We performed a systematic review (PROSPERO: CRD42018092277), according to the PRISMA protocol. Two investigators independently conducted search strategies (OvidMEDLINE, PubMed, Cochrane Review, BMJ BestPractice), identifying complications in patients following EA repair. Rates of esophageal dysmotility, GERD, dysphagia, anastomotic leak, anastomotic stricture, recurrent fistula formation, and esophagitis were sought. Results: A total of 65 publications met selection criteria (n = 4882). Rates of morbidity were high: esophageal dysmotility (78%), GERD (43%), dysphagia (44%), anastomotic leak (19%), anastomotic stricture (26%), recurrent fistula formation (7%), and esophagitis (47%). No correlation appeared to exist with severity of symptoms. Conclusions: This systematic review identified high rates of complications in children with EA, with esophageal dysmotility present in the majority of patients. Increasing survival, with resultant longer timeframes to develop morbidities, makes standardized follow-up regimens crucial. Type of study: Prognosis study. Level of evidence: Level 3.