Scholarlink

<p>A 13-month-old female presented with acute right lower extremity discoloration, pain, and inability to bear weight. Examination revealed a purple/red limb with absent pulses and no Doppler signal. Imaging demonstrated aneurysmal dilation of the superior and inferior mesenteric arteries and right common iliac artery thrombosis with absent distal flow. Anticoagulation with a heparin infusion was initiated. During anesthesia for MRA of the head, neck, and body to evaluate vasculopathy, she developed a renovascular hypertensive crisis. Her limb worsened in appearance with progressive mottling, prompting left axillary access for thrombectomy and thrombolysis catheter placement which was effective. Aortogram and selective renal and visceral arteriograms revealed beaded appearance of the renal arteries suggesting fibromuscular dysplasia (FMD). Given demarcation of her ischemic right lower extremity, she underwent a right through-knee amputation. Pathology from the specimen confirmed extensive arteriopathy consistent with fibromuscular dysplasia. Persistent wound breakdown and lack of pulsatile flow required revision to an above-knee amputation with skin grafting and eventually revision amputation. She recovered well and is now ambulatory with a prosthesis. She remains on long term anticoagulation, antihypertensive therapy for renovascular hypertension, and beta blockade to possibly reduce visceral aneurysm rupture risk. This case illustrates a rare, severe presentation of FMD and underscores the need for prompt multidisciplinary evaluation and coordinated management.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Pediatric fibromuscular dysplasia presenting with acute right lower extremity limb ischemia requiring endovascular treatment and amputation – a case report

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年5月21日

查看全文
  • Amanda Louiselle,
  • Olivia Hoffman,
  • Kathleen Ludwig,
  • Ayesha Zia,
  • Angela Scheuerle,
  • Halima Janjua,
  • Dinesh Rakheja,
  • Jeanne Dillenbeck,
  • Sheena Pimpalwar,
  • Corey Gill,
  • Paymon Sanati-Mehrizy,
  • Russell B. Hawkins

摘要

A 13-month-old female presented with acute right lower extremity discoloration, pain, and inability to bear weight. Examination revealed a purple/red limb with absent pulses and no Doppler signal. Imaging demonstrated aneurysmal dilation of the superior and inferior mesenteric arteries and right common iliac artery thrombosis with absent distal flow. Anticoagulation with a heparin infusion was initiated. During anesthesia for MRA of the head, neck, and body to evaluate vasculopathy, she developed a renovascular hypertensive crisis. Her limb worsened in appearance with progressive mottling, prompting left axillary access for thrombectomy and thrombolysis catheter placement which was effective. Aortogram and selective renal and visceral arteriograms revealed beaded appearance of the renal arteries suggesting fibromuscular dysplasia (FMD). Given demarcation of her ischemic right lower extremity, she underwent a right through-knee amputation. Pathology from the specimen confirmed extensive arteriopathy consistent with fibromuscular dysplasia. Persistent wound breakdown and lack of pulsatile flow required revision to an above-knee amputation with skin grafting and eventually revision amputation. She recovered well and is now ambulatory with a prosthesis. She remains on long term anticoagulation, antihypertensive therapy for renovascular hypertension, and beta blockade to possibly reduce visceral aneurysm rupture risk. This case illustrates a rare, severe presentation of FMD and underscores the need for prompt multidisciplinary evaluation and coordinated management.