A Case Report of Jejunal Necrosis caused by Superior Mesenteric Vein Thrombosis related to Congenital Protein S Deficiency

Junichi Yoshizawa¹⁾, Naohiko Koide¹⁾²⁾, Hiroyasu Saito¹⁾, Norihiko Furusawa¹⁾²⁾, Akira Kobayashi¹⁾, Akiyoshi Mochizuka³⁾, Fumihiro Ishida⁴⁾, Naoshi Nakamura⁵⁾ and Shinichi Miyagawa¹⁾

Department of Surgery¹⁾, Department of Critical and Intensive Care Medicine²⁾, Department of Laboratory Medicine³⁾ and Department of Internal Medicine Division of Hematology⁴⁾, Shinshu University School of Medicine
Department of Gastroenterology, Marunouchi Hospital⁵⁾

We report a case of jejunal necrosis caused by superior mesenteric vein thrombosis related to congenital protein S deficiency. A 53-year-old man complaining of lower abdominal pain was admitted to hospital. A physical examination revealed fever, abdominal distension, tenderness and rebound tenderness in the lower abdomen. Abdominal X-ray findings showed marked intestinal gas. Contrast-enhanced computed tomography showed dilatation and wall thickness of the small intestine and obstruction of the portal vein and the superior mesenteric vein. Emergency surgery was performed; a 120-cm section of the jejunum was necrotic, and the jejunal veins were obstructed by thrombi. We performed a jejunal resection of the necrotized region, created a jejunal anastomasis, and established an intestinal fistula. After the operation, anticoagulant therapy was performed using heparin and warfarin. The patient's free protein S antigen was markedly impaired, (28.2%), and that of his father, brother and son were also decreased. The superior mesenteric vein thrombosis in this case was thought to have been caused by congenital protein S deficiency.

Key words
superior mesenteric vein thrombosis, protein S deficiency, jejunal necrosis

Reprint requests
Junichi Yoshizawa Department of Surgery, Shinshu University School of Medicine
3-1-1 Asahi, Matsumoto, 390-8621 JAPAN

Accepted
January 25, 2006

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