Surgical Repair of Spontaneous Ruptured Dissection of the Left Common Iliac Artery in a Patient with Vascular Ehlers-Danlos Syndrome

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  • Yoshida Kazufumi
    Department of Cardiovascular Surgery, Kobe City Medical Center General Hospital
  • Fukunaga Naoto
    Department of Cardiovascular Surgery, Kobe City Medical Center General Hospital
  • Koyama Tadaaki
    Department of Cardiovascular Surgery, Kobe City Medical Center General Hospital

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  • 急速な動脈瘤形成から左総腸骨動脈解離性動脈瘤破裂に至った血管型 Ehlers-Danlos 症候群の1手術例

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<p>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.</p>

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