Extrahepatic portal hypertension due to congenital obstruction of the portal vein and associated gross hepatic lobulation.

A 10-233k old girl presented with splenomegaly and recurrent hematemesis from esophageal varices. Splenoportography revealed a dilated extrahepatic portion of the portal vein with nonvisualization of its intrahepatic tributaries. The child died following an episode of hematemesis and was found to have a dilated portal vein which ended blindly. In addition, there was abnormal lobulation of the inferior surface of the liver which was not cirrhotic. The portal vascular anomaly, which presumably was responsible for the portal hypertension, was probably due to failure of communication between the embryonic vitelline veins or to atresia of the portal vein secondary to pressure from the abnormal hepatic lobulation in utero. It would appear that congenital factors may be significant in the etiology and pathogenesis of some cases of extrahepatic portal hypertension in early life and recognition of such developmental anomalies is of importantance in management.