上腸間膜静脈血栓症により腸管壊死をきたしたプロテインS欠乏症の1例

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  • A CASE OF PROTEIN S DEFICIENCY CAUSING INTESTINAL NECROSIS DUE TO THROMBOSIS OF CRANIAL MESENTERIC VEIN

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We report a case of thrombosis of cranial mesenteric vein caused by protein S deficiency, together with a review of the Japanese literature.<br> A 54-year-old man, who had had tenderness and spontaneous pain mainly in the lower abdomen since March 2004, complained of abdominal pain and vomiting and was admitted to the hospital with a diagnosis of acute abdomen. Abdominal plain CT scan visualized localized thickening of the small intestine, ascites, and an increased density of the superior mesenteric vein. Enhanced CT scan showed radiolucency in the superior mesenteric vein under the confluence of splenic vein. Intestinal necrosis due to thrombosis of cranial mesenteric vein was thus diagnosed, and a laparotomy was performed. Operative findings included bloody ascites and congestive changes and necrotized portions of the ileum. A partial excision of the small intestine by about 40cm in length, 120cm distal from the terminal ileum was performed. Blood coagulation tests revealed a low level of protein S. It is thought that protein S deficiency might cause thrombosis of cranial mesenteric vein with intestinal necrosis in this case.

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