Cluster of 4 cases of esophageal squamous cell cancer developing in adults with surgically corrected esophageal atresia--time for screening to start

BackgroundCurrently, no recommendations exist for the endoscopic screening of patients in adulthood, with surgically corrected esophageal atresia (EA), for the development of esophageal cancer. A small number of individual case reports in the literature have raised concern that these cancers pose an increased risk (2 adenocarcinoma and 3 squamous cell carcinoma).MethodsSt Vincent's hospital has set up an EA clinic to review adult patients previously operated on for correction of EA. These patients underwent clinical review and were offered endoscopic evaluation if they had symptoms of dysphagia or gastroesophageal reflux. Among those patients, 3 have developed esophageal squamous cell carcinoma (SCC). A retrospective review of the EA database from the Royal Children's Hospital (798 patients [309 patients older than 40 years]) was then performed to identify any other cases of esophageal cancer developing in this cohort. One further patient was identified.ResultsTo date, 4 of 309 patients have developed esophageal SCC over the age of 40 years. The cumulative incidence of esophageal SCC in this age group was 50 times that expected in the general population.Conclusions(1) This cluster provides strong evidence that there is a substantial risk of SCC in these adults with surgically repaired EA. (2) We believe that long-term surveillance endoscopy enhanced by advanced imaging techniques is indicated in all adults from the age of 20 years who have had surgical repair of EA.