CONGENITAL HEPATIC FIBROSIS, A CASE REPORT

Congenital hepatic fibrosis can present in young adults with predominant clinical and imaging findings of portal hypertension. The imaging finding of enlargement of the entire quadrate lobe with associated right lobe atrophy and intra hepatic ductal plate malformation makes the diagnosis highly suggestive. The disease can be confirmed with a liver biopsy which is highly specific. Caroli's syndrome combines the features of Caroli's disease with congenital hepatic fibrosis.