A CASE OF MESENTERIC HEMATOMA ASSOCIATED WITH EHLERS-DANLOS SYNDROME

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  • Ｅｈｌｅｒｓ‐Ｄａｎｌｏｓ症候群に合併した腸間膜血腫の１例

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Ehlers-Danlos Syndrome (EDS) is a disorder of connective tissue, which is characterized by hyperextensible and atonic skin, hyperextensible joints and fragile skin and vessels. There are 10 variant forms of the disease. Especially type IV has life-threatening complication such as rupture and dissection of the artery, and perforation of the large intestine. We experienced a case of type IV EDS causing bleeding in the mesentery which demanded surgical excision.<br>A 29-year-old woman seen at the hospital because of lower abdominal pain was suspected to have abscess on an enhanced CT scan, and was operated on. Upon laparotomy, an egg-sized cystic lesion was present on mesentery side of the hepatic flexure of ascending colon. A right hemicolectomy was perfomed. On the resected material a capsulated hematoma was identified in the mesentery. Histologically, disappearance of smooth muscle structure of the vessels was identified, so that the fragility of vessels was suggested. The patient's postopetative course was uneventful, and there have been no signs of recurrence at present.<br>Many complications caused by fragile vessels have been reported in patients with type IV EDS, and accordingly utmost care must be exercised at surgery for these patient.

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