{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T12:09:46Z","timestamp":1790078986107,"version":"4.0.1"},"reference-count":92,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T00:00:00Z","timestamp":1775088000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T00:00:00Z","timestamp":1775088000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Hepato Biliary Pancreat"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Pancreaticobiliary maljunction (PBM) is an anomaly in which the bile and pancreatic ducts join outside the area of action of the sphincter. PBM is typically accompanied by choledochal cysts (dilated PBM); however, cases of PBM without biliary dilatation (nondilated PBM) have also been reported. PBM manifests throughout life. The presentation remains unclear due to age\u2010dependent differences in its pathophysiology. PBM symptoms may be attributed to biliary obstruction caused by substances that differ with age: calcium bilirubinate debris in neonates, protein plugs in children, and gallstones in adults. Each substance is associated with PBM types. Debris occurs in dilated PBM with a narrow segment. Plugs are produced by pancreatobiliary reflux in dilated PBM but rarely in nondilated PBM. Bile duct gallstones have been implicated in dilated PBM with a narrow segment, while gallbladder gallstones may be coincident. Pancreatobiliary reflux appears to induce carcinogenesis silently and cause symptoms in the elderly. A large number of adults with PBM are reported to be asymptomatic. In summary, PBM on its own appears to cause no symptoms. However, associated biliary stricture and pancreatobiliary regurgitation produce obstructive substances or cancers, which may lead to symptoms. The substances may vary with the patient's age and PBM types.<\/jats:p>","DOI":"10.1002\/jhbp.70110","type":"journal-article","created":{"date-parts":[[2026,4,2]],"date-time":"2026-04-02T09:24:53Z","timestamp":1775121893000},"page":"661-669","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Pathophysiology Underlying Manifestation of Pancreaticobiliary Maljunction Throughout Life"],"prefix":"10.1002","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7767-1027","authenticated-orcid":false,"given":"Kenitiro","family":"Kaneko","sequence":"first","affiliation":[{"name":"Department of Gastroenterological Surgery Aichi Medical University  Nagakute Aichi Japan"},{"name":"Chairperson of the Scientific Committee Japanese Study Group on Pancreaticobiliary Maljunction (JSGPBM)  Tokushima Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5273-5362","authenticated-orcid":false,"given":"Yasuyuki","family":"Fukami","sequence":"additional","affiliation":[{"name":"Department of Gastroenterological Surgery Aichi Medical University  Nagakute Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4645-5556","authenticated-orcid":false,"given":"Shoko","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of Gastroenterological Surgery Aichi Medical University  Nagakute Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6145-8993","authenticated-orcid":false,"given":"Kentaro","family":"Shinohara","sequence":"additional","affiliation":[{"name":"Department of Gastroenterological Surgery Aichi Medical University  Nagakute Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9605-4745","authenticated-orcid":false,"given":"Takaaki","family":"Osawa","sequence":"additional","affiliation":[{"name":"Department of Gastroenterological Surgery Aichi Medical University  Nagakute Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5085-6120","authenticated-orcid":false,"given":"Tsuyoshi","family":"Sano","sequence":"additional","affiliation":[{"name":"Department of Gastroenterological Surgery Aichi Medical University  Nagakute Aichi Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,4,2]]},"reference":[{"key":"e_1_2_13_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2468-1253(17)30002-X"},{"key":"e_1_2_13_3_1","article-title":"Scientific Committee of the Japanese Study Group on Pancreaticobiliary Maljunction","author":"Kaneko K.","year":"2022","journal-title":"Atlas of Pancreaticobiliary Maljunction & Congenital Biliary Dilatation"},{"key":"e_1_2_13_4_1","first-page":"20","article-title":"Anomalous Junction of Pancreaticobiliary Duct Without Congenital Choledochal Cyst: A Possible Risk Factor for Gallbladder Cancer","volume":"82","author":"Yamauchi S.","year":"1987","journal-title":"American Journal of Gastroenterology"},{"key":"e_1_2_13_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/bjs.1800800527"},{"key":"e_1_2_13_6_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2168.1998.00744.x"},{"key":"e_1_2_13_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00534-013-0606-2"},{"key":"e_1_2_13_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.8"},{"key":"e_1_2_13_9_1","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.2011.243"},{"key":"e_1_2_13_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamasurg.2015.0226"},{"key":"e_1_2_13_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hpb.2016.04.005"},{"key":"e_1_2_13_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13304-025-02138-9"},{"key":"e_1_2_13_13_1","first-page":"1413","article-title":"A Case of Cystic Dilatation of the Common Bile Duct and Etiology of the Disease (In Japanese)","volume":"30","author":"Kozumi K.","year":"1916","journal-title":"Tokyo Medical Journal"},{"key":"e_1_2_13_14_1","first-page":"231","article-title":"Congenital Choledochal Cysts: New Etiological Concept Based on Anomalous Relationships of the Common Bile Duct and Pancreatic Bulb","volume":"12","author":"Babbitt D. P.","year":"1969","journal-title":"Ann Radiol (Paris)"},{"key":"e_1_2_13_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9610(77)90359-2"},{"key":"e_1_2_13_16_1","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.139.8.855"},{"key":"e_1_2_13_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02489025"},{"key":"e_1_2_13_18_1","doi-asserted-by":"publisher","DOI":"10.1308\/003588409X391947"},{"key":"e_1_2_13_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00535-012-0702-0"},{"key":"e_1_2_13_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/MPG.0b013e3181623291"},{"key":"e_1_2_13_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(85)80239-6"},{"key":"e_1_2_13_22_1","doi-asserted-by":"publisher","DOI":"10.1089\/lap.2021.0634"},{"key":"e_1_2_13_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0016-5107(99)70223-3"},{"key":"e_1_2_13_24_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.12187"},{"key":"e_1_2_13_25_1","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.1969.01340100089011"},{"key":"e_1_2_13_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3468(71)90303-4"},{"key":"e_1_2_13_27_1","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.1978.01370140049010"},{"key":"e_1_2_13_28_1","first-page":"26","article-title":"Choledochal Cysts. A Review of the Cystic Anomalies of the Biliary Tree","volume":"56","author":"Olbourne N. A.","year":"1975","journal-title":"Annals of the Royal College of Surgeons of England"},{"key":"e_1_2_13_29_1","doi-asserted-by":"publisher","DOI":"10.1259\/0007-1285-53-633-906"},{"key":"e_1_2_13_30_1","doi-asserted-by":"publisher","DOI":"10.1053\/jpsu.2002.36186"},{"key":"e_1_2_13_31_1","doi-asserted-by":"publisher","DOI":"10.3109\/00365521.2014.946084"},{"key":"e_1_2_13_32_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00268-017-4064-x"},{"key":"e_1_2_13_33_1","doi-asserted-by":"publisher","DOI":"10.1006\/jsre.1996.0019"},{"key":"e_1_2_13_34_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.485"},{"key":"e_1_2_13_35_1","first-page":"40","article-title":"Congenital Dilatation of the Common Bile Duct in Children. The Etiologic Significance of the Narrow Segment Distal to the Dilated Common Bile Duct","volume":"39","author":"Ito T.","year":"1984","journal-title":"Zeitschrift f\u00fcr Kinderchirurgie"},{"key":"e_1_2_13_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/bjs.7588"},{"key":"e_1_2_13_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2010.12.022"},{"key":"e_1_2_13_38_1","first-page":"601","article-title":"Contributions to the Aetiology and Pathology of Idiopathic Cystic Dilatation of the Common Bile\u2010Duct With Report of Three Cases; a New Aetiological Theory Based on Supposed Unequal Epithelial Proliferation at the Stage of the Physiological Epithelial Occlusion of the Primitive Choledochus","volume":"30","author":"Yotuyanagi S.","year":"1936","journal-title":"Gann (Japanese Journal of Cancer Research)"},{"key":"e_1_2_13_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(98)90190-7"},{"key":"e_1_2_13_40_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.590"},{"key":"e_1_2_13_41_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2011.09.056"},{"key":"e_1_2_13_42_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40792-021-01175-x"},{"key":"e_1_2_13_43_1","first-page":"733","article-title":"Choledochal Cysts in Infancy and Childhood","volume":"121","author":"Fonkalsrud E. W.","year":"1965","journal-title":"Surgery, Gynecology & Obstetrics"},{"key":"e_1_2_13_44_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.1158"},{"key":"e_1_2_13_45_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12887-024-05043-z"},{"key":"e_1_2_13_46_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10620-004-9599-7"},{"key":"e_1_2_13_47_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10620-006-9398-4"},{"key":"e_1_2_13_48_1","first-page":"1018","article-title":"Protein Plugs Cause Symptoms in Patients With Choledochal Cysts","volume":"92","author":"Kaneko K.","year":"1997","journal-title":"American Journal of Gastroenterology"},{"key":"e_1_2_13_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(82)80488-0"},{"key":"e_1_2_13_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3468(90)90072-H"},{"key":"e_1_2_13_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(85)80392-4"},{"key":"e_1_2_13_52_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2008-1066188"},{"key":"e_1_2_13_53_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00534-012-0519-5"},{"key":"e_1_2_13_54_1","first-page":"125","article-title":"Spontaneous Perforation of Choledochal Cyst","volume":"181","author":"Ando H.","year":"1995","journal-title":"Journal of the American College of Surgeons"},{"key":"e_1_2_13_55_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2022.775378"},{"key":"e_1_2_13_56_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.12069"},{"key":"e_1_2_13_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2007.10.071"},{"key":"e_1_2_13_58_1","doi-asserted-by":"publisher","DOI":"10.18632\/oncotarget.16228"},{"key":"e_1_2_13_59_1","doi-asserted-by":"publisher","DOI":"10.4174\/astr.2021.101.5.306"},{"key":"e_1_2_13_60_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2007.11.004"},{"key":"e_1_2_13_61_1","doi-asserted-by":"publisher","DOI":"10.1111\/ans.15674"},{"key":"e_1_2_13_62_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00595-021-02322-5"},{"key":"e_1_2_13_63_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00268-001-0171-8"},{"key":"e_1_2_13_64_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00464-021-08490-2"},{"key":"e_1_2_13_65_1","doi-asserted-by":"publisher","DOI":"10.1002\/jhbp.691"},{"key":"e_1_2_13_66_1","first-page":"1121","article-title":"Abnormal Connection Between the Choledochus and the Pancreatic Duct in Case of Congenital Choledochal Cyst Examined by Endoscopic Pancreatocholangiography (In Japanese With English Abstract)","volume":"9","author":"Oi I.","year":"1977","journal-title":"Shonigeka (Pediatric Surgery)"},{"key":"e_1_2_13_67_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(05)80102-2"},{"key":"e_1_2_13_68_1","doi-asserted-by":"publisher","DOI":"10.4240\/wjgs.v15.i8.1784"},{"key":"e_1_2_13_69_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2004-835963"},{"key":"e_1_2_13_70_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2018-224891"},{"key":"e_1_2_13_71_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-0528.1993.tb15119.x"},{"key":"e_1_2_13_72_1","doi-asserted-by":"publisher","DOI":"10.1272\/jnms.JNMS.2024_91-401"},{"key":"e_1_2_13_73_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajog.2009.01.026"},{"key":"e_1_2_13_74_1","article-title":"Choledochal Cysts in Adults: A Case Report and Literature Review","volume":"16","author":"Villarino Zapata P.","year":"2024","journal-title":"Cureus"},{"key":"e_1_2_13_75_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.20534"},{"key":"e_1_2_13_76_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198002143020702"},{"key":"e_1_2_13_77_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9610(79)90055-2"},{"key":"e_1_2_13_78_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2362.2000.00703.x"},{"key":"e_1_2_13_79_1","doi-asserted-by":"publisher","DOI":"10.1053\/gast.1997.v112.pm9178697"},{"key":"e_1_2_13_80_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12944-021-01527-4"},{"key":"e_1_2_13_81_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9610(77)90403-2"},{"key":"e_1_2_13_82_1","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.1983.01390080088022"},{"key":"e_1_2_13_83_1","first-page":"816","article-title":"Biliary Carcinoma Risk in Patients With Pancreaticobiliary Maljunction and the Degree of Extrahepatic Bile Duct Dilatation","volume":"53","author":"Kamisawa T.","year":"2006","journal-title":"Hepato\u2010Gastroenterology"},{"key":"e_1_2_13_84_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-023-05582-z"},{"key":"e_1_2_13_85_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000658-197511000-00017"},{"key":"e_1_2_13_86_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gassur.2023.11.007"},{"key":"e_1_2_13_87_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13863"},{"key":"e_1_2_13_88_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0040-1715610"},{"key":"e_1_2_13_89_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12885-021-09000-2"},{"key":"e_1_2_13_90_1","first-page":"1238","article-title":"Prospective Study of the Detection of Anomalous Connections of Pancreatobiliary Ducts During Routine Medical Examinations","volume":"43","author":"Yamao K.","year":"1996","journal-title":"Hepato\u2010Gastroenterology"},{"key":"e_1_2_13_91_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0000000000004482"},{"key":"e_1_2_13_92_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10620-011-1964-8"},{"key":"e_1_2_13_93_1","doi-asserted-by":"publisher","DOI":"10.1177\/1457496918822617"}],"container-title":["Journal of Hepato-Biliary-Pancreatic Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jhbp.70110","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/jhbp.70110","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jhbp.70110","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T11:30:11Z","timestamp":1790076611000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jhbp.70110"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,2]]},"references-count":92,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2026,9]]}},"alternative-id":["10.1002\/jhbp.70110"],"URL":"https:\/\/doi.org\/10.1002\/jhbp.70110","archive":["Portico"],"relation":{},"ISSN":["1868-6974","1868-6982"],"issn-type":[{"value":"1868-6974","type":"print"},{"value":"1868-6982","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,2]]},"assertion":[{"value":"2025-10-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-18","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-04-02","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}