choledochal cyst
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Definition: Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extrahepatic bile ducts. Their exact pathophysiology remains unclear.
Pancreaticobiliary ductal dysplasia
A choledochal cyst is not just an aneurysmal dilatation of the common bile duct, but it is the most obvious manifestation of a more widespread pancreaticobiliary ductal dysplasia.
Most patients have abnormalities of the pancreatic ducts and, frequently, multiple areas of dilatation involving the intrahepatic bile ducts.
Complications
Extensive complications exist with untreated cysts including biliary stasis and inflammation, with resultant stricture and stone formation, cholangitis, pancreatitis, and malignant degeneration.
Most commonly reported malignancies include cholangiocarcinoma, adenocarcinoma, and gallbladder cancer.
Complications
biliary epithelial dysplasia
bile duct tumors
- cholangiocarcinoma
- adenocarcinoma
- squamous cell carcinoma
- gallbladder cancer
- Pleomorphic rhabdomyosarcoma (#16967793#)
chronic calcific pancreatitis (+/- papillary stenosis)
See also
biliary malformations
- choledochal malformations
- gallbladder malformations