Narrative ReviewManagement of Adults With Esophageal Atresia
Section snippets
Description of the Disease
EA is the most common congenital anomaly of the esophagus, occurring in approximately 1 in 3000 live births3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13 (Figure 1). Type C type distal TEF occurring in 85% of cases is the most common type of EA. The embryologic origin of EA is thought to be an incomplete evagination of the tracheal diverticulum to form the trachea, failure to form septal tissue dividing the esophagus and trachea, or regression of initially successful esophageal tube formation. Several
Gastroesophageal Reflux Disease
The main complications that follow EA surgery persisting into adulthood are gastroesophageal reflux disease (GERD) and dysphagia. GERD is reported to occur in 27% to 75% of adult patients with EA depending on GERD definition.27, 21, 22, 28, 29, 30 In one study, erosive esophagitis found in 19% of patients.31 When GERD is defined by symptoms, there is a marked variation in the reported prevalence of GERD symptoms in the literature ranging between 17% and 75%.28,32,33 This is potentially
Barrett’s Esophagus and Esophageal Cancer
One of the concerns in adult patients with EA is the development of Barrett’s metaplasia owing to chronic gastroesophageal reflux.27,29,30,69 Patients as young as 20 years old with Barrett’s have been described.70,71 An interesting finding from multiple studies is that grossly visible metaplastic epithelium seen on endoscopy does not consistently indicate the presence of intestinal metaplasia. For example, in one study of 60 patients with EA followed for 30 years, Barrett’s esophagus (BE) was
Conclusion
Previously a congenital anomaly that uniformly led to death in infancy, patients with EA can expect to live long healthy lives. Nevertheless, the surgical magnitude of initial therapy, the chronic complications that may ensue from this disorder and its correction, and the potential for health-impairing additional congenital anomalies mandate a lifetime of medical care and supervision. This supervision needs to attend to multiple potential issues including dysphagia from anastomotic strictures
CRediT Authorship Contributions
Julie Khlevner (Conceptualization; Writing Review and Editing; Supervision)
Daniela Jodorkovsky (Conceptualization; Writing Review and Editing; Supervision)
Dominique D. Bailey (Conceptualization; Writing Review and Editing; Supervision)
William Middlesworth (Conceptualization; Writing Review and Editing; Supervision)
Amrita Sethi (Conceptualization; Writing Review and Editing; Supervision)
Julian Abrams (Conceptualization; Writing Review and Editing; Supervision)
David A. Katzka (Conceptualization;
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Cited by (0)
Conflicts of interest These authors disclose the following: Julie Khlevner reports speaker’s bureau for Abbott Pediatric Nutrition. Amrita Sethi reports consultant for Boston Scientific, Olympus, Fujifilm, and Medtronic. Julian Abrams reports consulting for Exact Sciences and Castle Biosciences; and research support from Pentax. David A. Katzka reports consulting for Regeneron and Celgene. The remaining authors disclose no conflicts.
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Authors share co-first authorship.