Adenocarcinoma of the esophagus complicating Cornelia de Lange syndrome

Complicated gastroesophageal reflux disease may be seen in a variety of congenital developmental syndromes, but progression of Barrett's metaplasia to adenocarcinoma is rare. We report the first case of esophageal adenocarcinoma in a young adult with Cornelia de Lange syndrome who had long-standing gastroesophageal reflux disease. We review the literature concerning Barrett's metaplasia in children and make recommendations for surveillance.