Multiple intrahepatic bile duct dilation with bile lakes and concurrent sepsis is suggestive of

Caroli's disease Clinical features : Symptoms include cholangitis (64%), poal hypeension (22%) and abdominal pain(18%) More common in males Septa containing poal veins protrude into the lumen of the ecstatic bile ducts (central dot sign) The main and often the only symptom of bacterial cholangitis secondary to caroli's disease is fever without abdominal pain and jaundice Frequent episodes of cholangitis indicates poor prognosis Most stones are pigmented in caroli's disease Ref: Sabiston 20th edition Pgno :1511