Review

Nonatherosclerotic limb ischemia: Prompt evaluation and diagnosis

John C. Eun, MD, William R. Hiatt, MD and Natalia O. Glebova, MD, PhD
Cleveland Clinic Journal of Medicine October 2016, 83 (10) 741-751; DOI: https://doi.org/10.3949/ccjm.83a.15102

Article Figures & Data

Figures

  • FIGURE 1
    FIGURE 1

    Computed tomography of the lower extremities shows right popliteal entrapment syndrome with distal runoff occlusions (A) and abnormal insertion of the gastrocnemius muscle (B, arrow) in the patient’s right leg. The left leg is normal. Intraoperative angiography shows a patent popliteal artery in the neutral position (C) with occlusion during forced dorsiflexion (arrow, D).

  • FIGURE 2
    FIGURE 2

    Partial thrombosis of the right popliteal artery aneurysm is seen on arterial duplex ultrasonography in grayscale (A) and with color flow (B) showing the flow lumen (red) and thrombus (arrow). Computed tomography of another patient (C, D) shows partial thrombosis of a right popliteal artery aneurysm (arrow) in transverse (C) and sagittal (D) views.

  • FIGURE 3
    FIGURE 3

    In this three-dimensional reconstruction of a computed tomographic scan (posterior view) in a patient with a left persistent sciatic artery, the arrow indicates dilation of an aneurysm.

  • FIGURE 4
    FIGURE 4

    A patient with phlegmasia cerulea dolens. The arrow points to the demarcation of ischemia.

  • FIGURE 5
    FIGURE 5

    This patient presented with severe claudication secondary to an infrarenal aortic occlusion in the setting of Takayasu arteritis (A, white arrow) and underwent bypass from the thoracic aorta to the left common iliac artery and the right common iliac artery with a bifurcated prosthetic graft. Note the small underfilled but noncalcified iliac arteries (A, red arrow), the relatively normal thoracic aorta (B, arrow), and the somewhat diseased juxtarenal aorta (C, arrow).

Tables

  • TABLE 1

    Nonatherosclerotic presentations of limb ischemia

    ConditionPresentationCauseWorkupTreatment
    Popliteal artery entrapment syndromeCalf claudication in a young patient without cardiovascular risk factorsCompression of the popliteal artery by muscles in the popliteal fossaArterial duplex ultrasonography and ankle-brachial index with and without plantar flexion
    Magnetic resonance imaging or computed tomography to define muscle and arterial anatomy    		Surgical release of the popliteal fossa
    Popliteal artery aneurysmLeg pain, coolness, numbness, weakness
    In severe cases, acute or chromic limb ischemia    		Aneurysmal degeneration and thrombosis with distal embolizationComputed tomographic angiography (CTA) or magnetic resonance angiography (MRA) in the acute presentationIntra-arterial thrombolysis followed by surgical bypass
    Cystic adventitial diseaseNew onset claudication in a patient not likely to have cardiovascular diseaseVessel narrowing secondary to mucincontaining cysts in adventitiaCTA or MRAComplete cyst resection and vessel reconstruction via bypass
    Persistent sciatic arteryLeg pain, coolness, numbness, weakness; buttock painFemoral arterial system fails to mature; sciatic arterial system remains and is prone to aneurysmal degeneration and atherosclerosisCTA or MRASurgical bypass for symptomatic lesions, close follow-up for aneurysmal degeneration with arterial duplex ultrasonography for asymptomatic lesions
    Phlegmasia cerulea dolensSudden onset of lower extremity pain, swelling, cyanosis, and arterial ischemiaExtensive acute ileofemoral venous thrombosisClinical suspicion with evidence of iliofemoral deep vein thrombosis
    Most would suggest venous duplex ultrasonography    		Aggressive fluid resuscitation, limb elevation, intravenous anticoagulation; catheter-directed thrombolysis followed by surgical thrombectomy if no improvement
    Buerger diseaseIn a cigarette smoker: claudication, ischemic foot pain at rest, non-healing digital ulcersNonatherosclerotic small-arterial segmental inflammatory diseaseShionoya criteria (see text)Cessation of all tobacco use including cigarettes, gum, and second-hand smoke
    Takayasu arteritisPulselessness, pulse or pressure discrepancy in the arms, unobtainable blood pressure measurement, limb fatigability and painVasculitis of the aorta and its main branchesMultiple diagnostic criteria have been proposed
    CTA or MRA for arterial imaging
    Inflammatory markers    		Anti-inflammatory and immunosuppressant drugs; surgical bypass if severe symptoms, but only when disease is quiescent
    Arterial thoracic outlet syndromeRanges from ischemia of the fingers due to microembolization to acute limb ischemia due to complete thrombosis of the subclavian arteryCompression of the subclavian artery as it exits the thoracic outlet; aneurysmal degeneration and thrombosisNoninvasive imaging with pulse volume recording followed by arterial duplex ultrasonography, chest radiography, and MRA and CTA with arms up and arms downManagement of the acute arterial injury, followed by removal of the first rib with potential arterial bypass
    External iliac endofibrosisUnilateral leg pain at near-maximal exercise in an elite athleteThickening of arterial intimaHistory
    Duplex ultrasonography CTA or MRA    		Avoidance of hip flexion
    Open surgery
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