Carolidisease

Ahmed Abdulghany

In 1958, Jacques Caroli first described a rare

congenital condition in which there was a non-

obstructive saccular dilatation of large intrahepatic

bile ducts.

Caroli Syndrome: more common variable in which

bile duct dilatation is associated with congenital

hepatic fibrosis

Genetics

Incompletely understood however gene for

autosomal recessive polycystic kidney disease has

been linked to caroli disease coding for Fibrocystin.

Clinical manifestations

There are several modes of presentations

depending on the age of onset and predominance of

hepatic or renal involvement.

Biliary dilation predispose to biliary stagnation and

stone formation.

Bacterial cholangitis occur frequently and may be

complicated by septicemia.

Diagnosis

Established by imaging studies (US & MRCP)

showing irregular, cystic dilation of proximal

intrahepatic bile ducts with normal common bile

duct.

Liver functions show mild cholestatic pattern

Liver biopsy is rarely required for diagnosis

Differential diagnosis

1ry biliary cirrhosis

1ry sclerosing

cholangitis

Drug induced

cholestasis

Intrahepatic

cholestasis of

pregnancy

Sarcoidosis

Amyloidosis

Infiltrating neoplasms

Treatment

Supportive

Complications as cholangitis and portal

hypertension

Liver transplantation for recurrent cholangitis

Prognosis

Variable depending upon severity of the disease

and presence of coexisting renal dysfunction.

Although risk of cholangiocarcinoma is up to 7%,

surveillance is unclear. Suspect if there is

unexplained clinical deterioration or development

of new biliary strictures.

Thank you