Screening and Surveillance in Esophageal Atresia Patients: Current Knowledge and Future Perspectives

Esophageal atresia (EA) is a rare congenital anomaly. Enhanced operative techniques and intensive care treatment have improved survival among children with repaired EA (range, 93-95%). Many (up to 67%) suffer from gastroesophageal reflux (GER). The high incidence of GER and improved survival among EA patients raises concerns about an increased risk of developing Barrett esophagus (BE) and esophageal cancer. This review provides an overview of the prevalence of esophagitis, BE, and esophageal cancer in EA patients and outlines suggestions for future research. A literature search indeed revealed a higher prevalence of BE in EA patients than in the generalized population and that this condition occurs at a much younger age. It should be noted that in some studies gastric metaplasia without intestinal metaplasia is defined as BE. Gastric-type mucosa in columnar-lined esophagus is probably of less clinical importance in terms of the likelihood of malignant transformation. Its inclusion therefore confounds the risk of esophageal adenocarcinoma. A total of eight cases of esophageal carcinoma at a young age, either squamous cell carcinoma or adenocarcinoma, have been reported. These observations bear important implications prompting for early onset lifelong BE/esophageal cancer surveillance to facilitate the diagnosis of (pre)neoplastic changes and early treatment.