Pancreaticobiliary maljunction in Turkish patients: a multicenter case series

Abstract

Background and aims Pancreaticobiliary maljunction (PBM) is a malformation in which the pancreatic and bile ducts join outside the duodenal wall. It is associated with various biliary and pancreatic diseases. In addition, patients with PBM carry a substantial lifetime risk of developing biliary or gallbladder carcinoma. We aimed to present a multicenter case series of PBM from Turkey. Methods This study was conducted in adult and pediatric PBM patients who were referred to three tertiary reference centers of Turkey for endoscopic retrograde cholangiopancreatography (ERCP) between July 2007 and May 2020. The clinical presentations, types of PBM, ERCP findings, surgical histories, and the postoperative courses, including the development of biliary malignancies, were retrospectively reviewed. Results The study group included 47 (31 adult and 16 children) patients. Type D PBM was more frequent (13/41: 27.7%) than that reported in Eastern studies. Type A PBM was more common in the adults (51.6% vs. 12.5%, p < 0.05), whereas type C was more common in pediatric patients (31.3% vs. 13.2%, p < 0.05). Although fusiform anatomy was predominant in both of the groups, cystic dilatation was more common (25.8% vs. 12.5%) in adults and the common bile duct diameter was greater [22 mm (range 11-58) vs. 12 mm (range 5-33)] in adult patients compared to pediatric patients. Resective surgeries were more frequently done in pediatric patients (73.3% vs. 53.6%), whereas cholecystectomy was more frequently performed in adult patients (21.4% vs. 6.7%). Conclusion Although our findings were compatible with Eastern studies, type D PBM (associated with pancreas divisum) was more frequent in our study population.