ABSTRACT
Vascular Ehlers-Danlos Syndrome (EDS) causes fragility of the blood vessels, skin, and hollow organs, and the most common clinical feature is vascular rupture. We report a surgical case of a spontaneous ruptured dissection of the left common iliac artery (CIA) in a patient with vascular EDS. A 27-year-old man with documented vascular EDS with a mutation of the collagen type III alpha-1 [COL3A1] gene, presented with acute left lower abdominal pain. The enhanced computed tomography (CT) scan revealed a ruptured dissection of the left CIA with extravasation, and left iliopsoas hematoma. The enhanced CT performed 1 month previously showed no arterial aneurysm. The left CIA was anastomosed end-to-end using a 7 mm heparin-bonded polytetrafluorethylene (PTFE) graft (Fusion, MAQUET Cardiovascular LLC, USA). A PTFE graft was anastmosed end-to-side to the left external iliac artery (EIA). The distal side of the CIA was closed. The postoperative enhanced CT showed aneurysmal formation in the right inferior gluteal artery. The follow-up CT showed enlargement of the left EIA (from 7.5 to 12 mm in a diameter), and aneurysm formation in the proper hepatic artery. Therefore, a close follow-up based on noninvasive imagings is mandatory to detect life-threatening vascular complecations in patients with vascular EDS.