Surgical Repair of Spontaneous Ruptured Dissection of the Left Common Iliac Artery in a Patient with Vascular Ehlers-Danlos Syndrome / 日本心臓血管外科学会雑誌

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.

Síndrome de Ehlers-Danlos vascular: complicaciones vasculares graves en enfermos con hipermovilidad articular / Vascular Ehlers-Danlos syndrome: severe vascular complications in patients with articular hypermobility

El Síndrome de Ehlers-Danlos Vascular es una de las Alteraciones Hereditarias del Tejido Conectivo. Es poco frecuente, pero es de alta morbilidad y mortalidad. Se debe a una mutación del gen COL3A1, que da debilidad del tejido colágeno, por lo que se pueden producir complicaciones graves afectando a la piel, tubo digestivo, hígado, pulmón y sistema vascular. Se pueden producir rupturas intestinales, neumotórax espontáneo, dilatación o ruptura aortica y anuerismas, lo que puede llevar a la muerte súbita del paciente.

The Vascular Ehlers-Danlos is one of Hereditary Diseases of Connective Tissue. Has low prevalence, but high morbidity and mortality. It is due to a COL3A1 gene mutation, producing tissue fragility, which can produce serious complications in the skin, digestive tract, liver, lungs and in the vascular system. It is frequently associated to intestinal ruptures, spontaneous pneumothorax, dilation or rupture of the aorta anuerism, which can lead to sudden death.