Cikrit D F, Miles J H, Silver D
J Vasc Surg. 1987 Feb;5(2):248-55.
The Ehlers-Danlos syndrome (EDs) is one of the most frequently inherited disorders of connective tissue. Type IV EDs, the arterial-ecchymotic type, is of concern to vascular surgeons because it is frequently associated with spontaneous catastrophic bleeding. This article summarizes our experience with five members of a family and reviews the 31 patients with type IV EDs described in the literature. The 22 male and 14 female patients had a mean age of 26 years. The 36 patients included 23 with easy "bruisability," 22 with hypermobility of their joints (especially the fingers), 13 with transparent skin, and 11 with excessive elasticity of the skin. The patients had 41 episodes of hemorrhage and 29 vascular surgical procedures. Twenty-nine of the patients had an aneurysm or a dissection, whereas eight patients had arteriovenous fistulas. Arteriography was associated with a complication rate of 67%. The prognosis for a patient with type IV EDs is poor; 44% die before surgery and 19% die during the operative period. Bleeding should be managed nonoperatively when possible. Fifty-one percent die before reaching 40 years of age. Arteriography should be avoided. The standard repair of aneurysms and perforations is unlikely to be successful because of marked vessel friability. Bleeding vessels should be repaired with buttressed sutures and no tension or should be ligated. Genetic counseling with assay of collagen production is recommended for family members.
埃勒斯-当洛综合征（EDS）是最常见的遗传性结缔组织疾病之一。IV型EDS，即动脉瘀斑型，是血管外科医生关注的问题，因为它常与自发性灾难性出血相关。本文总结了我们对一个家族中五名成员的经验，并回顾了文献中描述的31例IV型EDS患者。22名男性和14名女性患者的平均年龄为26岁。36例患者中，23例有容易“瘀伤”的情况，22例有关节（尤其是手指）活动过度，13例有皮肤透明，11例有皮肤弹性过度。患者有41次出血发作和29次血管外科手术。29例患者有动脉瘤或夹层，而8例患者有动静脉瘘。血管造影的并发症发生率为67%。IV型EDS患者的预后很差；44%在手术前死亡，19%在手术期间死亡。如有可能，出血应采用非手术治疗。51%在40岁之前死亡。应避免进行血管造影。由于血管明显脆弱，动脉瘤和穿孔的标准修复不太可能成功。出血血管应使用加强缝线进行修复且无张力，或者进行结扎。建议对家庭成员进行遗传咨询并检测胶原蛋白生成情况。
