Extrahepatic portal vein obstruction in adults detected by ultrasound with frequent lack of portal hypertension signs

Extrahepatic portal vein obstruction (EHPO) was seen in 54 adult patients at the Chiba University Hospital and affiliated hospitals from 1978 to 1991. They were classified according to the background disease (Group A, unknown aetiology; Group B, benign disease; Group C, malignant disease). Among the initial symptoms and signs, abdominal pain was the most frequent in Group A (37%), and symptoms attributable to the primary disease in Groups B (44%) and C (75%). Definite or probable diagnosis was made in 45 of the 54 patients (81.8%) by ultrasound (US) examination carried out because of these symptoms and signs. Signs of portal hypertension were observed in 67% of patients; oesophageal varices were seen in 60%. Extrahepatic portal vein obstruction without portal hypertension signs was characterized by thick extensive hepatopetal collaterals or patency of some intrahepatic portal veins. Extrahepatic portal vein obstruction patients without portal hypertension remained free of its signs for more than 3 years of follow up and, in fact, EHPO without portal hypertension signs was a common occurrence. Emphasis is made on the diagnostic value of US examination which was useful in identifying the relation of clinical manifestation of EHPO to pathophysiology, and on the frequent lack of portal hypertension signs in this disease.