Congenital hepatic fibrosis causing portal hypertension in children

1. 1. A new case of congenital hepatic fibrosis causing portal hypertension and hemorrhage is reported. 2. 2. A description of the clinical and pathological features of this condition is presented. 3. 3. The difference in the factors determining the timing and form of therapy of congenital hepatic fibrosis, intrahepatic obstruction due to cirrhosis, and extrahepatic obstruction are emphasized. 4. 4. End-to-side portacaval shunt performed electively at 5 years of age or earlier is suggested as the treatment of this condition. 5. 5. An effective portacaval shunt appears to offer a permanent cure.