2017 Volume 37 Issue 1 Pages 099-102
A 25-year-old male patient was referred to our hospital with a history of lower abdominal pain. He had been diagnosed as having the vascular type of Ehlers-Danlos syndrome. Physical examination revealed slight tenderness and muscle guarding in the left lower abdomen, however, blood examination revealed no evidence of an inflammatory process. We suspected gastrointestinal perforation, which is often encountered in patients of Ehlers-Danlos syndrome. Abdominal CT revealed free air and a fluid collection in the lower abdominal space. We diagnosed acute peritonitis caused by perforation of the intestine, and performed emergency surgery. A pinhole perforation was found in the sigmoid colon. As the surrounding intestinal wall and mesenterium of the colon looked healthy, we performed wedge resection and suture of the perforated sigmoid colon. The postoperative course was uneventful and the patient was discharged 13 days after the surgery. Ehlers-Danlos syndrome is a hereditary disease characterized by lax tissues. The vascular type is the most serious type of the disease, as it is known to be complicated by vascular rupture, dissociation, and gastrointestinal perforation even at a young age of the patients. We report a case of Ehlers-Danlos syndrome, vascular type, in which we performed wedge resection and suture of the perforated sigmoid colon and obtained a good outcome.
