Caroli's disease history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

History and Symptoms

  • Patients often present in young adulthood
  • 80% present before 30 years of age.
  • Patients often present with recurrent episodes of fever/chills and abdominal pain due to cholangitis.
  • Biliary lithiasis
  • Occurs in about a third.
  • Occasionally patients will present later in life with sequelae of liver disease and portal HTN.
  • Ductal rupture
  • Has been described with intrahepatic as well as extrahepatic bile duct cysts. This can result in peritonitis.
  • Liver abscess
  • Can occur in association with the intrahepatic cysts, abnormal biliary flow, cholangitis, and/or stone disease.
  • Malignancy
  • Is more common in the affected tissues, including cancer of the gall bladder, biliary tree, and liver.
  • This often occurs in patients ~50 years of age, about 2 decades before the mean age of carcinoma in the general population, and at a much higher rate (~7%) than the general population.
  • The reason for this increase in carcinoma is not clear, but may occur in association with pancreatic/biliary regurgitation.
  • Removal of cysts does not necessarily reduce the risk.
  • Once malignancy develops, prognosis is dismal.
  • More common in these patients.

References