Extrahepatic portal hypertension in children

In children the principal cause of extrahepatic portal hypertension is a “cavernomatous transformation” of the portal vein. In 54 children seen over a 20-yr period, hemorrhage was by far the most common presenting symptom, occurring in 30 patients. Occasionally these children presented with a symptomless splenomegaly. Liver function studies were always normal. The patients were classified into four groups, depending on the particular primary surgical treatment given. One-third of patients received no surgical treatment and all are well. Of the patients who have had a surgical procedure, postoperative bleeding has been frequent and further surgery has often been necessary. There have been seven deaths. Operation is not recommended in the child under 4 yr or during acute bleeding episodes. For recurrent life-threatening hemorrhage, a porto-systemic shunt is indicated.