A Case of Vascular Ehlers-Danlos Syndrome Resulting in Death from a Ruptured Aneurysm after Colonic Perforation

Abstract

A 20-year-old-woman was admitted to our hospital due to lower abdominal pain, with suspected repeat ovarian bleeding. Two days later, she underwent emergent laparotomy for suspected colonic perforation. Since the serosal surface, as well as the colonic stump, was very fragile, and refractory oozing bleeding and subserosal hematomas were seen, open abdominal management was performed. Two days later, the abdominal wall was closed by suture, and a transverse colostomy was created after confirming neither bleeding nor organ necrosis. Although the patient's postoperative course was uneventful, sudden bleeding via the drainage tube was found 7 days after re-operation. Contrast-enhanced CT and angiography showed rupture of the left gastric artery and an aneurysm of the common hepatic artery. Coil embolization was successfully performed. However, the patient died due to repeat intra-abdominal bleeding just after re-admission to the intensive care unit. This patient was finally diagnosed as having vascular Ehlers-Danlos syndrome (vEDS) genetically and clinically by the major criteria of colon perforation and intra-abdominal aneurysm, as well as the minor criteria of joint hypermobility and dislocation. This rare case of vEDS is presented along with a review of the relevant literature.