Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Caroli's disease history and symptoms
Introduction
History and Symptoms
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.
- 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.
- 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.
References
References
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]