Biliary Fibrosis and Portal Hypertension as a Rare Cause of Cholangiocarcinoma

Excerpt

Caroli’s syndrome (type 2) is a rare, complex form of Caroli’s disease (type 1) with an estimated incidence of 1/1,000,000. The syndrome manifests as saccular dilatations of the intra-hepatic biliary ducts and is associated with fibrosis and portal hypertension, distinguishing it from the disease form [1, 2]. Importantly, it predisposes patients to regionalized malignancy. Generally this presents before the age of 40. Intriguing however in our case was the late presentation of the disease. The aim of this case report is to educate clinicians as to an obscure presentation of this rare disease in an attempt to help prevent cirrhosis as well as the subsequent early detection of cholangiocarcinoma that is precipitated by this syndrome. …