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Pancreaticobiliary Maljunction
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Long common channel
            • Definitions

              • Pancreaticobiliary maljunction (PBM): Congenital malformation where pancreatic duct & common bile duct (CBD) join outside of duodenal wall
                • Long common channel allows pancreatobiliary & biliopancreatic reflux
                • Chronic pancreatobiliary reflux leads to high risk of gallbladder & biliary cancer
                • Biliopancreatic reflux causes acute pancreatitis
              • 2 types of PBM
                • PBM with congenital biliary dilation
                • PBM without congenital biliary dilation
              • Congenital biliary dilation
                • Previously referred to as type I or IVa choledochal cyst
                • Requires enlarged diameter of CBD + PBM
                • Definition for enlarged CBD depends on age
                  • Age 0 years: > 3 mm
                  • Age 5 years: > 3.9 mm
                  • Age 10 years: > 4.5 mm
                  • Age 15 years: > 5 mm
                  • Age 20 years: > 5.9 mm

            IMAGING

            • General Features

              • CT Findings

                • Ultrasonographic Findings

                  • MR Findings

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • Staging, Grading, & Classification

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      Selected References

                                      1. Ono A et al: Imaging of pancreaticobiliary maljunction. Radiographics. 40(2):378-92, 2020
                                      2. Kamisawa T et al: Pancreaticobiliary maljunction: markedly high risk for biliary cancer. Digestion. 99(2):123-5, 2019
                                      3. Kamisawa T et al: Pancreaticobiliary maljunction and congenital biliary dilatation. Lancet Gastroenterol Hepatol. 2(8):610-8, 2017
                                      4. Hamada Y et al: Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci. 23(6):342-6, 2016
                                      5. Kamisawa T et al: Diagnostic criteria for pancreaticobiliary maljunction 2013. J Hepatobiliary Pancreat Sci. 21(3):159-61, 2014
                                      6. Wang CL et al: Magnetic resonance cholangiopancreatography study of pancreaticobiliary maljunction and pancreaticobiliary diseases. World J Gastroenterol. 20(22):7005-10, 2014
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                TERMINOLOGY

                                                • Synonyms

                                                  • Long common channel
                                                • Definitions

                                                  • Pancreaticobiliary maljunction (PBM): Congenital malformation where pancreatic duct & common bile duct (CBD) join outside of duodenal wall
                                                    • Long common channel allows pancreatobiliary & biliopancreatic reflux
                                                    • Chronic pancreatobiliary reflux leads to high risk of gallbladder & biliary cancer
                                                    • Biliopancreatic reflux causes acute pancreatitis
                                                  • 2 types of PBM
                                                    • PBM with congenital biliary dilation
                                                    • PBM without congenital biliary dilation
                                                  • Congenital biliary dilation
                                                    • Previously referred to as type I or IVa choledochal cyst
                                                    • Requires enlarged diameter of CBD + PBM
                                                    • Definition for enlarged CBD depends on age
                                                      • Age 0 years: > 3 mm
                                                      • Age 5 years: > 3.9 mm
                                                      • Age 10 years: > 4.5 mm
                                                      • Age 15 years: > 5 mm
                                                      • Age 20 years: > 5.9 mm

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • Ultrasonographic Findings

                                                      • MR Findings

                                                        • Other Modality Findings

                                                          • Imaging Recommendations

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • Staging, Grading, & Classification

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          Selected References

                                                                          1. Ono A et al: Imaging of pancreaticobiliary maljunction. Radiographics. 40(2):378-92, 2020
                                                                          2. Kamisawa T et al: Pancreaticobiliary maljunction: markedly high risk for biliary cancer. Digestion. 99(2):123-5, 2019
                                                                          3. Kamisawa T et al: Pancreaticobiliary maljunction and congenital biliary dilatation. Lancet Gastroenterol Hepatol. 2(8):610-8, 2017
                                                                          4. Hamada Y et al: Diagnostic criteria for congenital biliary dilatation 2015. J Hepatobiliary Pancreat Sci. 23(6):342-6, 2016
                                                                          5. Kamisawa T et al: Diagnostic criteria for pancreaticobiliary maljunction 2013. J Hepatobiliary Pancreat Sci. 21(3):159-61, 2014
                                                                          6. Wang CL et al: Magnetic resonance cholangiopancreatography study of pancreaticobiliary maljunction and pancreaticobiliary diseases. World J Gastroenterol. 20(22):7005-10, 2014