A case of congenital hepatic fibrosis: first report from Bangladesh

Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture, often occurring in association with cystic lesions of the kidney. It is inherited as autosomal recessive trait. The underlying abnormality lies in the development of biliary ductules, classically described as “Ductal Plate Malformation” [1, 2]. It commonly presents in childhood with features of portal hypertension nevertheless incidental diagnosis in adulthood or even autopsy diagnosis has been reported [3]. Though frequently confused with liver cirrhosis [2], congenital hepatic fibrosis do not present with feature of hepatocellular insufficiency [1]. Absence of signs of liver failure may rather indicate possibility of congenital hepatic fibrosis in those who are mistakenly considered to have liver cirrhosis. Here we report a case of congenital hepatic fibrosis that was misdiagnosed and treated as a case of liver cirrhosis for 13 years.