Japanese clinical practice guidelines for pancreaticobiliary maljunction

Abstract

There have been no clinical guidelines for the management of pancreaticobiliary maljunction (PBM). The Japanese Study Group on Pancreaticobiliary Maljunction (JSPBM) has proposed to establish clinical practice guidelines on how to deal with PBM, with the support of the Japan Biliary Association (JBA). Because the body of evidence-based literature is relatively small, we decided to create guidelines based on the consensus of experts, using the medical literature for reference. A total of 46 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs covered distinct aspects of PBM: (1) Concepts and Pathophysiology (10 CQs); (2) Diagnosis (10 CQs); (3) Pancreatobiliary complications (9 CQs); and (4) Treatments and prognosis (17 CQs). Statements and comments for each CQ were prepared by the guidelines committee members and collaborating partners. The CQs were completed after review by members of the editorial committee, meetings of this committee, public comments on the homepages of the JSPBM and the JBA, public hearings, and assessment and approval by the guidelines evaluation board. PBM includes cases where the bile duct is dilated (PBM with biliary dilatation) and those in which it is not (PBM without biliary dilatation). In these guidelines, PBM with biliary dilatation is defined as being identical to congenital biliary dilatation of Todani type I (except for type Ib) and type IV-A, both of which are accompanied by PBM in almost all cases. These guidelines are created to provide assistance in the clinical practice of PBM management; their contents focus on clinical utility, and they include general information on PBM to make this disease more widely recognized.

Clinical practice guidelines for the management of biliary tract cancers 2015: the 2nd English edition

“…(Grade 1, evidence level C) Statement: PBM is a congenital anomaly consisting of a union of the pancreatic and bile ducts located outside the duodenal wall [17]. Due to the lack of sphincter action at the union of the pancreatic and bile ducts, bile and pancreatic juice are mixed, resulting in the activation of cell toxic factors.…”

“…These factors are considered to induce chronic cholangitis, which may result in carcinogenesis [18,19]. PBM is divided into two groups by the existence of bile duct dilatation (PBM with or without bile duct dilatation) [17].…”

Pancreaticobiliary maljunction and biliary cancer

“…In cases with a common channel B9 mm on MRCP, direct cholangiography is needed to confirm PBM [12]. PBM can be diagnosed if junction outside the wall can be depicted by high-resolution images with multiplanar reconstruction (MPR) provided by multidetector row computed tomography (MD-CT), and endoscopic ultrasonography (EUS) [3,13,14].…”

“…According to a nationwide survey in Fig. 1 The pathophysiology of pancreaticobiliary maljunction [3] Japan (n = 2561) [2], biliary cancer was detected in 21.6 % of adult patients with congenital biliary dilatation and in 42.4 % of PBM patients without biliary dilatation. In patients with biliary cancers in association with PBM, the location ratio of cancers in the bile duct and gallbladder were 32.1 % and 62.3 % in congenital biliary dilatation, and 7.3 % and 88.1 % in PBM patients without biliary dilatation, respectively.…”

“…Japanese clinical practice guidelines on how to deal with PBM were created in 2012, as the first in the world [3]. Diagnostic criteria for PBM were revised in 2013, taking recent advances in diagnostic imaging techniques into consideration [4].…”