A case of congenital antithrombin III deficiency associated with extensive extrahepatic portal occlusion and esophagogastric varices.

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A 29-year-old man developed thrombosis of the superior mesenteric vein whose onset occurred in October 1980 with epigastralgia and underwent an extensive enterectomy. Thereafter he had several bouts of hematemesis and melena and was diagnosed to have congenital AT III deficiency 3 years and 6 months after the initiatory symptom appeared. Later, while he was on anticoagulant could successfully be arrested then with the administration of fresh blood and fresh frozen plasma.<BR>In January 1984, he was admitted with hematemesis, hepato-and splenomegaly. A through examination revealed esophagogastric varices and extensive portal occlusion with resultant cavernous transformation of portal vein.<BR>This case was thought to represent a rare instance in which, in the presence of congenital AT III deficiency as underlying pathology, repeated recurrences of superior mesenteric venous thrombosis led to selected occlusion of the splenic vein and portal trunk without causing generalized venous thrombosis since, to our knowledge, such a case has not been reported in the world literature.
社団法人日本肝臓学会の論文