Caroli Disease &

Colangiosarcoma
Table of Contents
Caroli Disease
Pathogenesisi
Clinical manifestations
Differential diagnosis
Cholangiosarcoma
Caroli Disease &
Syndrome
Congenital disorder
characterized by
multifocal segmental
dilation of large
intrahepatic bile ducts
Caroli disease
bile ductular extasia
without other apparent
hepatic abnormalities
Segmental dilation of large
intrahepatic biliary ducts
Caroli syndrome
Segmental dilation of small
intra-hepatic biliary ducts &
congenital hepatic fibrosis
ETIOLOGY
ARPKD gene for fibrocystin
linked to caroli syndrome
Pathophysiology
Biliary tree develops from Ductal plate
Caroli Disease
Abnormalities of bile duct at large intrahepatic
ducts- dilation and ectasia
Biliary stasis can lead to cholelithiasis, cholangitis,
sepsis, and increased risk of cholangiocarcinoma
Caroli Syndrome
Ductal plate malformation at smallet portal racts-
varying degrees of portal fibrosis
Caroli Disease &
Syndrome
Generally children (as it is congenital)
Very rare: disease is 1:100,000 births, Syndrome
1: 20,000
F>M
Present with history of intermittent abdominal
pain, RUQ pain, fever or family history of ARPKD
Differential Diagnosis:
Congenital hepatic fibrosis
Pediatric gallstones
Primary sclerosing cholangitis
Increased risk of developing cholangiocarcinoma
Cholangiocarcinoma
Malignancies of
biliary duct
system
Origginate in liver
and extrahepatic
bile ducts
Intrahepatic
Extrahepatic
(perihilar)
Distal
extrahepatic
Cholangiocarcinoma
2500 new cases
Grow slowly and each year
infiltrate walls of 2-6:100,000 in
ducts western
Local extension: countries
liver, porta 5.5:1000,000
hepatitis, Japan,
regional lymph 7.3:100,000
nodes (celiac and Israel
pancreaticoduod Most commonly
enal change) women older
than 60 years
Risk Factors & Diagnosis:
Cholangiocarcinoma
ParasiticLiver Diseases Diagnosis
Opisthorchis viverrini
Clonorchis sinensis Bippsy
Vrial Liver Disease Blood tests: CA
Cirrhosis
19-9
HIV infection
Congenital Liver
Abdominal
abnormalities Imaging (CT and
Hepatolithiasis ultrasound
Thorotrast exposure Endoscopic
IBD
Retrograde
Alpha1-antitrypsin
deficiency
Cholangiopancrea
obesity tiography
Cholangiocarcinoma
History
Jaundice 90% of patients
Clay colored are not eligibale
stools for curative
Bilirubinuria resection
Pruritis
Survival is
Weight loss
approximately 6
Abdominal pain
months
+Courvoisier
sign
Morphology