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 pathophysiology
Introduction
Pathophysiology
Pathophysiology
- Biliary cysts have been described throughout the biliary system, and are classified according to their location and anatomy.
- Many biliary cysts are congenitally acquired, perhaps as a consequence of unequal proliferation of epithelial cells during embryonic biliary duct development. Some biliary cysts are acquired, and some may develop in association with anatomic variations that lead to abnormally high ductal pressures in association with other predisposing factors.
- Type I (75-85%)
- Choledochal cysts – cystic dilatation of the common bile duct (CBD)
- Often presents during infancy with significant liver disease.
- Type II (2-3%)
- Diverticulae anywhere in the extrahepatic ducts.
- Presenting symptoms’s may reflect cystic compression of nearby structures.
- Type III (1-5%)
- Choledochocele cysts
- Often present with pain and obstructive jaundice; many have pancreatitis.
- Type IV (18-20%)
- IVA – Multiple cysts in the Intra- and Extra-hepatic ducts
- IVB – Multiple cysts in the Extra-hepatic ducts only
- Type V – Caroli’s Disease – rare
- Single or Multiple Intrahepatic bile duct cysts
References
References
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]