Väärämäki Suvi, Hautero Olli, Rajala Vesa, Nevalainen Pasi
Centre for Vascular Surgery, Tampere University Hospital, Faculty of Medicine and Life Sciences, Tampere, Finland.
Department of Vascular Surgery, Vaasa Central Hospital, Vaasa, Finland.
Vasc Endovascular Surg. 2025 Apr;59(3):315-319. doi: 10.1177/15385744241290007. Epub 2024 Oct 13.
Pseudoxanthoma elasticum (PXE) is a rare metabolic disease, causing calcification in the arterial media layer and further peripheral artery disease (PAD). A high rate of failure has been reported after endovascular and open surgical management of PAD among patients with PXE. Critical limb ischemia (CLI) rarely develops in PXE, and there are only few reports of its treatment.
We present a case report of a 57 year-old female diagnosed with pseudoxanthoma elasticum (PXE). She presented with critical limb ischemia (CLI) and was successfully treated with pedal bypass using the great saphenous vein.
Despite obtaining suboptimal outcomes through the initial approach of percutaneous transluminal angioplasty to treat critical limb ischemia, the subsequent bypass operation proved to be a success. At the first follow-up appointment at 1 month, the patient was asymptomatic and the ulceration had almost healed. The patient underwent an ultrasound examination at 3, 6, 12, and 24 months after discharge, and the surveillance was uncomplicated.
With a clear indication for surgery, limb-threatening ischemia can be successfully treated with distal bypass, if necessary, in patients with PXE similarly to atherosclerotic PADs. Appropriate diagnostic and surveillance imaging and the utilization of a multidisciplinary team are key components for effective management of PAD in patients with PXE.
弹性假黄瘤（PXE）是一种罕见的代谢性疾病，可导致动脉中膜层钙化并进而引发外周动脉疾病（PAD）。据报道，PXE患者的PAD在接受血管内治疗和开放手术治疗后失败率很高。PXE很少发生严重肢体缺血（CLI），关于其治疗的报道也很少。
我们报告一例57岁女性被诊断为弹性假黄瘤（PXE）的病例。她出现了严重肢体缺血（CLI），并通过大隐静脉进行足部搭桥手术成功治疗。
尽管通过经皮腔内血管成形术治疗严重肢体缺血的初始方法取得的效果欠佳，但随后的搭桥手术被证明是成功的。在术后1个月的首次随访中，患者无症状，溃疡几乎愈合。患者在出院后3、6、12和24个月接受了超声检查，监测过程无并发症。
对于有明确手术指征的患者，与动脉粥样硬化性PAD患者类似，必要时，PXE患者的肢体威胁性缺血可通过远端搭桥手术成功治疗。适当的诊断和监测成像以及多学科团队的利用是有效管理PXE患者PAD的关键组成部分。
