Caroli's disease – A rare case report

Abstract

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Learning Objectives: 1. To illustrate a rare case report of Caroli' disease with review of literature.

Abstract Body: A 11 year old boy with complaints of abdominal pain and fever of 15 days duration was referred for hepatobiliary scintigraphy, with a suspicion of Caroli’s disease, based on ultrasonography (USG) findings. His renal parameters were within normal limits. Hepatobiliary scintigraphy revealed a typical pattern of intrahepatic biliary duct dilatation, with delayed drainage of tracer activity into the gut – a pattern consistent with that of Caroli’s disease. Caroli’s disease is a rare congenital disorder, in which there is non-obstructive saccular dilatation of intrahepatic biliary ducts. Generally seen in young children, with a female preponderance. It is believed to occur due to an in-utero event that arrests ductal plate remodeling at the level of larger intrahepatic bile ducts, leading to dilatation of the ducts. Symptoms include abdominal pain and fever. Laboratory investigations would reveal abnormal liver function tests, especially elevation of serum alkaline phosphatase. Imaging studies include Ultrasonography, Scintigraphy, CT, MRI, ERCP and PTc, each having their own limitations. Morbidity is common due to complications, which include cholangitis, sepsis, progression to secondary biliary cirrhosis, etc…Malignant transformation occurs in 7% of patients. Treatment is aimed at any diagnosed complication. Liver transplantation is the definite form of therapy.