Focal Caroli’s Disease Presenting as Fusiform Dilatation of Intrahepatic Biliary Radicles

Background Caroli's disease is a rare congenital disorder characterized by segmental, non obstructive, cystic dilatation of intra hepatic bile ducts. It belongs to the spectrum of fibropolycystic liver disease which results from in utero malformation of ductal plate. Two forms of this disorder have been described, the less common 'Pure' form involves only the large intrahepatic bile ducts and the more 'complex' form is associated with congenital hepatic fibrosis (CHF), and is known as Caroli's Syndrome. Case A young female presented with features of cholangitis and in addition to all the routine investigations, non invasive imaging in the form of ultrasonography, computed tomography and magnetic resonance imaging was done. Diagnosis Final diagnosis of focal Caroli's disease with cholangitis was made on clinical and imaging findings. Treatment Conservative treatment was given and patient referred to gastro surgery department for partial hepatectomy. Conclusion An early recognition of Caroli's disease with non invasive imaging can bring down the morbidity.