A Case Report of Caroli’s Disease in an Infant With Review of Literature.

Abstract

The authors report an infant with bilobar Caroli’s disease, a rare congenital disorder ‘with multiple cystic dilatations of the intrahepatic bile ducts. This disease entity presents a diagnostic as well as therapeutic dilemma. Various investigations used for diagnosis include ultrasound scan, hepatobiliary scan and percutaneous transhepatic cholangiography. Treatment of this rare disorder poses a challenging problem because various conservative surgical procedures are bound to fail. Hepatic resection in case of unilobar disease, and liver transplantation for biolobar disease are the only definitive curative procedures for this problem which is other-wise associated with a high morbidity and mortality.