Caroli’s Disease

Question 1

what is carolis disease

Answer 1

Rare congenital abnormality most likely inherited in an autosomal recessive fashion.

Question 2

what occurs in carolis disease

Answer 2

Communicating cavernous ectasia of the intrahepatic ducts characterized by congenital segmental saccular cystic dilation of major intrahepatic bile ducts.

Question 3

who is carolis disease found in

Answer 3

Found in the young adult or pediatric population; may be associated with renal disease or congenital hepatic fibrosis

Question 4

Symptoms

Answer 4

Patient symptoms include recurrent cramplike upper abdominal pain, secondary to biliary stasis, ductal stones, cholangitis, and hepatic fibrosis.

Question 5

what is associated with carolis disease

Answer 5

Cystic disease of the kidney (medullary sponge kidney) is strongly associated with Caroli’s disease

Question 6

what are the types of carolis disease

Answer 6

Two types of Caroli’s disease
Simple classic form
More common form associated with periportal hepatic fibrosis

Question 7

Carolis disease sonographic appearance

Answer 7

Multiple cystic structures in the area of the ductal system converge toward the porta hepatis.
Masses seen as localized or diffusely scattered cysts communicate with the bile ducts.
Differential diagnosis includes polycystic liver disease.
Ducts may show a beaded appearance as they extend into the periphery of the liver.
Ectasia of the extrahepatic and common bile ducts may be present.
Sludge or calculi may reside in the dilated ducts.