All are true regarding caroli’s disease except
Correct Answer: Commonly presents with ascites
Description: 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
Category:
Anatomy
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