All are true regarding caroli's disease except

CONGENITAL DILATATION OF THE INTRAHEPATIC DUCTS (CAROLI'S DISEASE) This rare congenital condition is characterised by multiple irregular saccular dilatations of the intrahepatic ducts, sepa- rated by segments of normal or stenotic ducts, with a normal extrahepatic biliary system. In Caroli's syndrome, the biliary dilatation is associated with congenital hepatic fibrosis. The presentation is varied, with many patients presenting with abdominal pain, cholangitis or end-stage liver disease. The majority of patients present before the age of 30. Sex distri- bution is equal. Management is multidisciplinary: cholangitis or jaundice are treated with appropriate antibiotic therapy and endoscopic or interventional stenting. Malignancy is a complication of long-standing disease. Hepatic resection is indicated for patients with limited disease. Patients with dif- fuse disease and concomitant hepatic fibrosis are candidates for liver transplantation. Recurrence is common, paicularly after resection, and long-term surveillance is required. Ref: Bailey and love 27th edition Pg no :1200