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Pancreaticobiliary maljunction
Terumi Kamisawa1, Kensuke Takuma, Hajime Anjiki
1Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, Bunkyo-ku, Tokyo, Japan. kamisawa@cick.jp
Insights
Pancreaticobiliary maljunction (PBM) is a congenital anomaly causing reflux and increasing biliary cancer risk. Early diagnosis and treatment, like prophylactic surgery, are crucial for managing PBM and preventing premalignant changes.
Area of Science:
- Gastroenterology
- Surgical Oncology
- Congenital Anomalies
Background:
- Pancreaticobiliary maljunction (PBM) is a congenital anomaly characterized by an abnormal junction of pancreatic and bile ducts outside the duodenal wall.
- This anatomical variation creates a long common channel, facilitating reflux between the pancreaticobiliary and biliopancreatic tracts.
- Elevated pancreatic enzyme levels in bile due to reflux are linked to a high incidence of biliary cancers.
Purpose of the Study:
- To investigate the relationship between pancreaticobiliary maljunction and the development of biliary and gallbladder cancers.
- To differentiate the risks and management strategies for PBM with and without biliary dilatation (congenital choledochal cyst).
- To assess the premalignant potential of gallbladder mucosa in PBM patients.
Main Methods:
- Retrospective analysis of patients with PBM, categorized by the presence or absence of biliary dilatation (congenital choledochal cyst).
- Evaluation of cancer incidence (bile duct and gallbladder) in relation to PBM subtypes.
- Histopathological assessment of gallbladder mucosa for premalignant changes, including K-ras mutations.
Main Results:
- In 42 patients with congenital choledochal cyst, 7 developed bile duct cancer and 8 developed gallbladder cancer.
- In 49 patients with PBM without biliary dilatation, 33 were diagnosed with gallbladder cancer.
- Gallbladder mucosa in PBM patients exhibited increased cell proliferation and K-ras mutations, indicating premalignant potential.
Conclusions:
- Pancreaticobiliary maljunction, particularly with a long common channel, predisposes patients to pancreatobiliary reflux and premalignant changes in the gallbladder.
- Congenital choledochal cyst associated with PBM carries a significant risk of both bile duct and gallbladder cancer.
- Prophylactic flow-diversion surgery is recommended for PBM with biliary dilatation, while prophylactic cholecystectomy is typically performed for PBM without biliary dilatation.
Abstract:
Pancreaticobiliary maljunction (PBM) is a congenital anomaly defined as a junction of the pancreatic and bile ducts located outside the duodenal wall, usually forming a markedly long common channel. In PBM patients, this anomaly allows regurgitation between the pancreatobiliary and biliopancreatic tract. Since hydrostatic pressure within the pancreatic duct is usually higher than that in the common bile duct, pancreatic juice frequently refluxes into the bile duct. As a result, pancreatic enzyme levels are generally very high in the bile and there is a related high incidence of biliary cancer. PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst [CCC]) and PBM without biliary dilatation (maximal diameter of the bile duct
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