Familial Barrettʼs Esophagus

Abstract

Purpose: Barrett's Esophagus is seen in 3–12% of patients with chronic reflux symptoms. This is a study of three family members (father, son, and daughter) all of whom had long-segment Barrett's esophagus that rapidly progressed from low grade dysplasia (LGD) to high grade dysplasia (HGD) within a year. This study demonstrates that there is a genetic component to this disease, and that, in familial cases, there can be rapid histological progression suggesting that the biological behavior is distinct from that seen in sporadic cases of Barrett's esophagus. Methods: A 77 year old male with chronic acid reflux was found to have long-segment Barrett's esophagus with LGD that progressed to HGD within a year. A laparoscopic esophagectomy was performed with the finding of HGD. The pstient's son and daughter were both subsequently diagnosed with long-segment Barrett's esophagus that rapidly progressed from LGD to HGD within a year. Both son and daughter had laparoscopic esophagectomy with finding of HGD. The patient's grandson was found to have short segment Barrett's esophagus without dysplasia. The patient's wife has erosive esophagitis as does a granddaughter. Results: A 77 year old male, his daughter and son all were found to have long-segment Barrett's esophagus that rapidly progressed from LGD to HGD within a year. The patient's grandson was found to have short segment Barrett's esophagus without dysplasia and is under close surveillance. Conclusion: Barrett's esophagus is felt to be an acquired condition resulting from chronic acid reflux. This report suggests that there is a genetic component to this disease, and, where there are familial clusters, the biological behavior can be more aggressive with rapid progression to HGD emphasizing the need to consider some modification of endoscopic screening strategies for this group of patients. Early intervention with ablation of Barrett's tissue might be considered in patients with a strong family history of Barrett's esophagus.