Fact-sheet: Caroli disease
Updated on 10/06/2016 at 9:18 AM
View all RADEOS cases associated with this fact-sheet
Definition
Caroli disease (CD) = non-obstructive congenital dilatation of the intrahepatic bile ducts.
Caroli syndrome (CS) = Caroli disease + congenital hepatic fibrosis
Clinical features
PSC: recurrent cholangitis
PSC with cirrhosis: cholangitis + portal hypertension, renal failure
Cholangiocarcinoma: Caroli disease and PSC (approximately 10%)
Ultrasound
Pseudocystic dilation of the bile ducts
Dot Sign: portal pedicle centered within the dilated bile duct (visible in all cases of major bile duct dilation)
Macro-calculi of the bile ducts
Signs of portal hypertension if PSC
CT
Diffuse pseudocystic dilation of the intrahepatic bile ducts
Pigmented biliary calculi
MRI
MRCP ++:
confirms the dilation
Signs of portal hypertension, calculi
Signs of cholangitis Cholangiocarcinoma
Management
Symptomatic treatment
Liver transplantation, or combined liver-kidney transplantation.
Differential diagnosis
Multiple cystic lesions:
- Hamartomatosis - Von Meyenburg complexes: MRCP or delayed-phase sequence after injection of hepatospecific contrast agent