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.
- Portal HTN and Varices
- 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.
- Renal tubular ectasia or renal cystic disease
- More common in these patients.