Congenital hepatic fibrosis is part of the spectrum of hepatic cystic diseases, and is characterized by aberrant bile duct proliferation and periductal fi-brosis. More details on this disease are given in Chapter 4, "Imaging of Benign Focal Liver Lesions", section 4.1.5,"Cysts".
In typical congenital hepatic fibrosis, cysts are not visible due to their very small size. Hepatic involvement in patients with polycystic kidney disease occurs in approximately 30-50% of cases. Clinically, the majority of patients present in childhood, when congenital hepatic fibrosis predominates with bleeding, varices and other manifestations of portal hypertension. In patients with predominating polycystic liver disease, the lesions are usually identified incidentally. Approximately 70% of patients with polycystic liver disease also have adult polycystic kidney disease. Congenital hepatic fibrosis is also related to Caroli's disease.
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