Imamura A, Nakamoto H, Inoue T, Yamada H, Okuno M, Takai S, Komada H, Kwon A H, Kamiyama Y
First Department of Surgery, Kansai Medical University, Moriguchi, Osaka, Japan.
Surg Today. 2001;31(1):85-9. doi: 10.1007/s005950170228.
Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and abnormalities of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized based on genetic, clinical, and biochemical characteristics. The spectrum of the disorder varies from mild to life-threatening vascular complications. EDS type IV is a particularly dangerous form with a lethal spontaneous rupture of the major arteries and aneurysmal formation. We present herein a case of a ruptured dissecting aneurysm in the bilateral iliac arteries caused by EDS type IV. A previously healthy 33-year-old man without any physical features of this connective tissue disorder experienced a metachronous vascular rupture two times. Successful synthetic bypass grafting was performed with great difficulty. The diagnosis of EDS type IV was made afterwards based on an electrophoresis analysis of a skin biopsy specimen which revealed a lack of type III collagen. Surgical intervention in cases of arterial complications in EDS type IV patients have been reported to be both difficult and frequently unsuccessful. The early clinical recognition of this syndrome is therefore of great importance due to the hazards of such surgical therapies.
埃勒斯-当洛综合征（EDS）是一种遗传性结缔组织疾病，其特征为皮肤过度伸展、关节活动过度以及心血管系统异常。根据遗传、临床和生化特征，目前已识别出10种类型及几种亚型的EDS。该疾病的范围从轻度到危及生命的血管并发症不等。IV型EDS是一种特别危险的形式，主要动脉会出现致命的自发性破裂和动脉瘤形成。我们在此报告一例由IV型EDS引起的双侧髂动脉夹层动脉瘤破裂病例。一名先前健康的33岁男性，没有这种结缔组织疾病的任何体征，却先后两次发生异时性血管破裂。成功进行了合成旁路移植术，但困难重重。之后通过对皮肤活检标本进行电泳分析，发现缺乏III型胶原蛋白，从而确诊为IV型EDS。据报道，对IV型EDS患者的动脉并发症进行手术干预既困难又常常不成功。因此，鉴于此类手术治疗的风险，早期临床识别该综合征非常重要。
