The General Infirmary at Leeds, Great George Street, West Yorkshire, Leeds, UK.
Eur J Vasc Endovasc Surg. 2012 Mar;43(3):341-2. doi: 10.1016/j.ejvs.2011.12.005. Epub 2012 Jan 5.
Vascular Ehlers-Danlos Syndrome (EDS) is a rare autosomal dominant condition resulting from a defect in type III procollagen synthesis. This causes the development of severe vascular pathologies, including arterial rupture and pseudoaneurysm formation. We present a case of a young boy previously diagnosed with vascular EDS due to a Gly975Val substitution in the collagen α1(III) chain presenting with a common femoral artery dissection secondary to minimal trauma. This was managed conservatively with serial duplex scans and gentle mobilization. At follow up the patient had returned to normal activities, with MRA and duplex scans showing complete resolution of the dissection.
血管型埃勒斯-当洛斯综合征(EDS)是一种罕见的常染色体显性遗传病,由 III 型前胶原合成缺陷引起。这会导致严重的血管病变,包括动脉破裂和假性动脉瘤形成。我们报告了一例年轻男孩的病例,该男孩先前因胶原 α1(III)链中的 Gly975Val 取代而被诊断为血管型 EDS,由于轻微创伤导致股总动脉夹层。通过连续的双功能超声扫描和轻柔的活动进行保守治疗。随访时,患者已恢复正常活动,磁共振血管造影和双功能超声扫描显示夹层完全愈合。