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

Kazufumi Yoshida＊

Naoto Fukunaga＊

Tadaaki Koyama＊

（Department of Cardiovascular Surgery, Kobe City Medical Center General Hospital＊, Kobe, Japan）

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 7mm 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 12mm 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.

 

Jpn. J. Cardiovasc. Surg. 47:78-81（2018）

Keywords：vascular Ehlers-Danlos Syndrome;aneurysmal formation;surgical repair