URSODEOXYCHOLIC ACID TREATMENT OF PRIMARY HEPATOLITHIASIS IN CAROLIS SYNDROME

Congenital cystic dilation of the intrahepatic bile ducts (Caroli's sy ndrome) is a rare cause of chronic cholestasis and hepatolithiasis in young adults. Long-term prognosis is poor even with surgical drainage. We treated twelve patients who had Caroli's syndrome and intrahepatic stones with ursodeoxycholic acid (UDCA), 10-20 mg/kg daily. The duode nal bile of these patients contained cholesterol crystals, which sugge sts that the stones were cholesterol rich. UDCA led to sustained clini cal remission, return to normal liver function, and dissolution of int rahepatic stones on ultrasound in all patients (nine partial, three co mplete) after 48 (range 12-114) months' follow-up. Litholytic therapy is indicated for intrahepatic stones in Caroli's syndrome.