Pancreaticobiliary maljunction: pathophysiological and clinical aspects and the impact on biliary carcinogenesis

Matsumoto, Yoshirô; Fujii, Hideki; Itakura, Jun; Matsuda, Masanori; Yang, Yang; Nobukawa, Bunsei; Suda, K

doi:10.1007/s00423-003-0370-x

Cited by 60 publications

(18 citation statements)

References 29 publications

(40 reference statements)

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“…Most patients with types I and IV lesions also have an anomalous pancreaticobiliary junction, in which the pancreatic duct joins the bile duct 1 to 2 cm proximal to the sphincter of Oddi. 15,17,18 This results in a common channel of 15 mm or more, which has been thought to allow reflux of pancreatic enzymes into the bile duct, increasing ductal pressure, and causing it to dilate. 19 Experimental diversion of pancreatic juice into the bile duct produces progressive dilation in dogs.…”

Section: Resultsmentioning

confidence: 99%

Congenital Choledochal Cysts in Adults

Visser¹

2004

Arch Surg

156

148

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Hypothesis: Excision of the extrahepatic portion of congenital choledochal cysts (CCs) avoids the risk of cancer. The standard classification scheme is out of date. Design: Retrospective case series and literature review. Setting: Tertiary care university hospital. Patients: Thirty-eight adult patients diagnosed as having CC from 1990 to 2004. Main Outcome Measures: Clinical and radiographic imaging findings, operative treatment, pathologic features, and clinical outcome. Results: Thirty-nine adult patients were treated for CCs (mean [SD] age at diagnosis, 31 [17] years, and mean [SD] age at surgery 37 [14] years). The primary report was abdominal pain (36 of 39 patients). Eight patients had cholangitis, 5 had jaundice, and 6 had pancreatitis. Radiographic imaging studies and operative findings showed that the abnormality predominantly involved the extrahepatic bile duct in 30 patients, the intrahepatic and extrahepatic bile ducts in 7 patients; and 2 were diverticula attached to the common bile duct. Surgical treatment in 29 (90%) of 31 patients with benign cysts (regardless of intrahepatic changes) consisted of resection of the enlarged extrahepatic bile duct and gallbladder and Roux-en-Y hepaticojejunostomy. Eight patients (21%) were initially seen with associated cancer (cholangiocarcinoma of the extrahepatic duct in 6; gallbladder cancer in 2). Seven of 8 patients had a prior diagnosis of CC but had undergone a drainage operation (3 patients), expectant treatment (3 patients), or incomplete excision (1patient). In none of the patients with cancer was surgery not curative. Nine patients had previously undergone a cystoduodenostomy and/or cystojejunostomy as children. Four of them had cancer on presentation as adults. There were no postoperative deaths. Cancer subsequently developed in no patient whose benign extrahepatic cyst was excised, regardless of the extent of enlargement of the intrahepatic bile duct. Conclusions: Congenital CCs consist principally of congenital dilation of the extrahepatic bile duct with a variable amount of intrahepatic involvement. We believe that the standard classification scheme is confusing, unsupported by evidence, misleading, and serves no purpose. The distinction between type I and type IV CCs has to be arbitrary, for the intrahepatic ducts were never completely normal. Although Caroli disease may resemble CCs morphologically, with respect to cause and clinical course, the 2 are unrelated. The other rare anomalies (gallbladderlike diverticula; choledochocele) are also unrelated to CC. Therefore, the term "congential choledochal cyst" should be exclusively reserved for congenital dilation of the extrahepatic and intrahepatic bile ducts apart from Caroli disease, and the other conditions should be referred to by their names, for example, choledochocele, and should no longer be thought of as subtypes of CC. Our data demonstrate once again a persistent tendency to recommend expectant treatment in patients without symptoms and the extreme risk of nonexcisional treatment. The entir...

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Section: Resultsmentioning

confidence: 99%

Congenital Choledochal Cysts in Adults

Visser¹

2004

Arch Surg

156

148

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“…They are observed in 24.1% of adults and 9.0% of pediatric patients, indicating that this is a frequent complication in adults [52]. It is reported that in CBD, the ratio of occurrence is: gallbladder stones 12.7%, common bile duct stones 65.8%, and intrahepatic stones 21.5% [53]. According to another report, among the PBM cases, 100% of biliary stones occurring with cystic dilatation are bile duct stones [54].…”

Section: Commentsmentioning

confidence: 99%

Japanese clinical practice guidelines for congenital biliary dilatation

Ishibashi

Shimada

Kamisawa

et al. 2017

J Hepatobiliary Pancreat Sci

Self Cite

112

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Until now, there have been no practical clinical guidelines for congenital biliary dilatation (CBD). In this review article, the Japanese Study Group on Congenital Biliary Dilatation (JSCBD) propose to establish clinical practice guidelines for CBD. Because the 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 20 clinical questions (CQs) were considered by the members of the editorial committee responsible for the guidelines. The CQs included the distinct aspects of CBD: (1) Concepts and Pathology (three CQs); (2) Diagnosis (six CQs); (3) Pancreaticobiliary Complications (three CQs); Treatments and Prognosis (eight CQs). Each statements and comments for CQs were made by the guidelines committee members. CQs were finally approved after review by members of the editorial committee and the guidelines evaluation board of CBD. These guidelines were created to provide assistance in the clinical practice of CBD management; their contents focus on clinical utility, and they include general information on CBD to make this disease more widely recognized.

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“…Matsumoto et al . postulated the theory that APBDJ arises as a result of a disturbance in the embryonic connections of the pancreatic and biliary duct system 19 . The authors have observed that only one‐third of patients with adult type cysts (enlargement involving the whole extrahepatic bile duct and/or main branches of the intrahepatic bile duct) had the long common duct.…”

Section: Discussionmentioning

confidence: 99%