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Congenital hepatic fibrosis: Aspects of diagnosis and surgical management

Five cases of congenital hepatic fibrosis are described. In all the patients the diagnosis was delayed as a result of either failure to perform liver biopsy or misinterpretation of the histological features. Four patients had multiple operations for the control of haematemesis or the relief of portal hypertension. It is stressed that a high index of suspicion, the use of endoscopy and a liver biopsy are essential to avoid delay in diagnosis. The patients have a good prognosis provided that the diagnosis is established at an early stage and portacaval anastomosis performed.