Extrahepatic Portal Vein Obstruction in Childhood

In nine children with upper gastrointestinal bleeding and splenomegaly, who were suspected to have the prehepatic protal hypertension on the clinical ground, angiographic diagnosis was tried to confirm the extrahepatic portal hypertension with portal vein obstruction. Selective celiac and superior mesenteric arteriography in six children and splerioportography in three children clearly showed hepatofugal and hepatopetal collaterals in addition to the obstruction of the portal venous system. Barium swallow examination demonstrated esophageal varix in all these patients. Ascites and jaundice were not found in these children. Abnorrnalities of liver function test including transaminase and abnormal bleeding tendency were not observed in these children. Initial clinical presentation was hematemesis in five children, melena without hematemesis in one child and asymptomatic splenomegaly in three children. Age at onset of the initial clinical presentation was ranged from 3 months to 8 years of age. The mean interval between initial presentation and diagnosis was about 4 years and 6 months and, ranged from 5 months to 10 years and 8 months delay being usually due to failure to perform the selective arteriography or splenoportography. The cause of the portal vein thrombosis in these children was not known. However, omphalitis and neonatal sepsis were found as the predisposing condition in two of these children with extrahepatic protal hypertension