Sauer L, Reilly L M, Goldstone J, Ehrenfeld W K, Hutton J E, Stoney R J
Division of Vascular Surgery, University of California, San Francisco 94143.
J Vasc Surg. 1990 Oct;12(4):488-95; discussion 495-6.
External iliac fibromuscular dysplasia is a rare and usually asymptomatic disorder. We report eight symptomatic patients seen over a 15-year period and review pathophysiologic mechanisms accounting for the three following distinct lower extremity ischemic sequelae: (1) Emboli--episodic focal digital ischemia (blue toe) was seen in three patients. Resection and primary anastomosis of focal iliac ulcerative fibromuscular dysplasia (one patient) or resection and replacement (two patients) removed the embolic source and relieved the symptoms. (2) Chronic ischemia--gradual onset of full leg claudication in four patients was treated by operative graduated intraluminal dilation in three patients and prosthetic bypass in one. Arteriography subsequently showed a remodeled lumen in the three patients who underwent dilation. (3) Dissection--acute onset leg ischemia resulted from presumed dissection of the external iliac segment. After 4 months of conservative management of antiplatelet agents and exercise, symptoms resolved completely, and arteriogram showed spontaneous restoration of a normal lumen in the dissected segment. The clinical presentation of fibromuscular dysplasia may mimic other arterial processes such as atherosclerosis. Diagnosis is made only by arteriography with specific magnification views of the external iliac arteries and careful surveillance of the renal arteries. Appropriate treatment should be tailored to the clinical presenting symptom. For microembolic disease, resection and replacement are required. For chronic ischemia, intraluminal dilation is generally sufficient and durable and has proved to be a simpler and acceptable alternative to replacement or bypass. In acute dissection, surgical intervention may be deferred if the limb is viable to allow spontaneous healing and remodeling. Persistent symptoms may be the only indication for intervention in this ischemic manifestation of external iliac fibromuscular dysplasia.
髂外纤维肌发育不良是一种罕见且通常无症状的疾病。我们报告了15年间诊治的8例有症状患者，并回顾了导致以下三种不同下肢缺血后遗症的病理生理机制：（1）栓子——3例患者出现发作性局灶性手指缺血（蓝趾）。对局限性髂溃疡性纤维肌发育不良进行切除并一期吻合（1例患者）或切除并置换（2例患者）消除了栓子来源并缓解了症状。（2）慢性缺血——4例患者逐渐出现全腿间歇性跛行，其中3例患者接受了手术性渐进性腔内扩张治疗，1例患者接受了人工血管旁路移植术。随后的血管造影显示，接受扩张治疗的3例患者管腔重塑。（3）夹层——推测髂外段夹层导致急性下肢缺血。在使用抗血小板药物和运动进行4个月的保守治疗后，症状完全缓解，血管造影显示夹层段管腔自发恢复正常。纤维肌发育不良的临床表现可能与其他动脉疾病如动脉粥样硬化相似。仅通过对髂外动脉进行特定放大倍数观察的血管造影以及对肾动脉的仔细监测才能做出诊断。应根据临床出现的症状进行适当治疗。对于微栓塞疾病，需要进行切除和置换。对于慢性缺血，腔内扩张通常就足够且持久，并且已被证明是替代置换或旁路移植的更简单且可接受的方法。在急性夹层中，如果肢体存活，可推迟手术干预以允许自发愈合和重塑。持续症状可能是髂外纤维肌发育不良这种缺血表现中进行干预的唯一指征。
