Caroli’s disease: a diagnostic challenge

Padmini Yadav, Shailesh Adhikari, Narendra Pandit, Lalijen Awale, Keerthi Vasan, Sarada Khadka


Caroli’s disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. It predisposes to biliary stasis and consequent lithiasis, cholangitis, abscesses, and septicemia. Sometimes it is difficult to diagnose and differentiate it from other similar disease conditions. 60-year-old female presented with features of recurrent cholangitis with hepatolithiasis and multiple cyst in liver, cholelithiasis was planned for cholecystectomy and drainage procedure. Patient underwent open cholecystectomy with common bile duct (CBD) exploration with T-tube placement due to intraoperative instability. Post-operative T tube cholangiogram was done. Post-operative T tube cholangiogram showed bilobar major duct cystic dilatation with predominant left lobe involvement, with few cysts containing calculi. Patient was planned for left hepatectomy with bile duct excision but patient refused to undergo aforementioned surgery. Therefore ERCP and sphincterotomy was done in view of poor performance status.Caroli’s disease being a rare disease is sometimes difficult to diagnose and treat in an old age patient with bilobar involvement and poor general conditions.


Caroli’s disease, Diagnostic challenge, Old age, Right upper abdominal pain

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