The aim of our educational poster is to: Illustrate the distinguishing features of caroli disease. Differentiate between caroli disease and caroli syndrome. Highlight some of the rarely seen associated disorders and their imaging pictures. List main differential diagnosis of cystic liver disease.

Caroli disease is a congenital disorder characterized by segmental non-obstructive saccular dilatation of the intrahepatic bile ducts. The term Caroli disease is applied if the disease is limited to ectasia of large intrahepatic ducts and it is less common than Caroli syndrome, in which smaller interlobular bile ducts are affected resulting in congenital hepatic fibrosis. In this poster, we will illustrate the characteristic radiological features of caroli disease and represent number of cases of the rarely concomitant disorders.

Caroli disease is a rare congenital disorder with autosomal recessive inheritance pattern [1,2]. The disease belongs to a spectrum of fibropolycystic liver disease which result from in utero ductal plate malformation [3]. Caroli disease versus caroli syndrome: Two form of Caroli disease have been described [4], [5-8]: Simple/Isolated type: Large bile ducts are affected and it is characterized by cystic dilatation of intrahepatic bile ducts that is often limited to one lobe (usually left lobe). However extra hepatic bile ducts involvement may exist. Complex type...

Caroli disease belongs to the family of congenital fibropolycystic liver disease originating from ductal plate malformation. Consequently various hepatic and biliary malformations usually co-exist. Hence, it is of a clinical significance to screen for the concomitant disorders in a patient diagnosed with caroli disease for early detection and appropriate management.

Eiman Rashid Musa, BS, MD, CABMS-RAD, clinical fellow at the Clinical Imaging Department, Body Section, Hamad General Hospital, Hamad Medical Corporation, Doha/Qatar. E.mail: [email protected]. A. Sayedin, MBBCH, CABMS-RAD. E.mail: [email protected]

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