A Case of Congenital Antithrombin III Deficiency Asociated with Extrahepatic Portal Occlusion and Esophgo-Gastric Varices

Abstract

A 41-year-old man was admitted because of hematemesis. He previously underwent enterectomy secondary to thrombosis of the superior mesenteric vein at the age of 25 years. His father had venous varices of the lower limbs and his brother was diagnosed as having superior mesenteric venous thrombosis. Antithrombin III (AT III) activity and antigen levels in the patient, his sister and his daughter were decreased. In the patient, endoscopy showed esophagogastric varices. Adominal angiography revealed complete occlusion and cavernous transformation of the portal vein. From these findings, he was diagnosed as having portal hypertension secondary to congenital AT III deficiency. Splenectomy and cardial resection were performed without thrombotic complication. AT III concentrate was infused from the day before the operation through the 8th day after the operation to maintain AT III level at 100-120%. Warfarin potassium and ticlopilidine hydrochloride were given to prevent from thrombotic attacks. We reviewed 38 families with congenital AT III deficiency reported in Japan and discussed the perioperative management of AT III levels in patients withcongenital AT III deficiency to prevent potential risk of thrombotic formation. Surgery was performed only in 3 cases diagnosed as congenital AT III deficiency.