Most Recent Analysis of a Japanese Nationwide Survey of Pancreaticobiliary Maljunction over a Quarter of a Century

Abstract

We herein present the most recent data on pancreaticobiliary maljunction (PBM) from a nationwide survey, which 3,303 individuals with PBM were registered at over 100 medical institutions in Japan for over 25 years. In this analysis, clinical features of eligible patients (n = 3,184) were compared according to age and presence of biliary dilatation (BD), and key points of this analysis were the coexistence of associated cancers and the surgical procedure including postoperative complication. Of the adults with CBD, 7.0 and 13.5% had gallbladder or bile duct cancers, whereas 4.0 and 37.2% of those with PBM without BD had these cancers. Three children with congenital biliary dilatation (CBD) had bile duct cancers (0.24%). As to individuals without asociated cancers, cholecystectomy combined with extrahepatic bile duct resection had been performed on 94.3% of children with CBD and on 90.7% of those with PBM with BD. In contrast, this procedure had been performed on 29.8% of adults with PBM without BD but on 87.1% of those with CBD. Postoperative complication rates of this procedure ranged from 10 to 20%. Thus, this largest and most recent series could be widely used to assist in making decisions about treatment strategies and understanding the pathophysiology of PBM.