Nonatherosclerotic presentations of limb ischemia
| Condition | Presentation | Cause | Workup | Treatment |
|---|---|---|---|---|
| Popliteal artery entrapment syndrome | Calf claudication in a young patient without cardiovascular risk factors | Compression of the popliteal artery by muscles in the popliteal fossa | Arterial 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 aneurysm | Leg pain, coolness, numbness, weakness In severe cases, acute or chromic limb ischemia | Aneurysmal degeneration and thrombosis with distal embolization | Computed tomographic angiography (CTA) or magnetic resonance angiography (MRA) in the acute presentation | Intra-arterial thrombolysis followed by surgical bypass |
| Cystic adventitial disease | New onset claudication in a patient not likely to have cardiovascular disease | Vessel narrowing secondary to mucincontaining cysts in adventitia | CTA or MRA | Complete cyst resection and vessel reconstruction via bypass |
| Persistent sciatic artery | Leg pain, coolness, numbness, weakness; buttock pain | Femoral arterial system fails to mature; sciatic arterial system remains and is prone to aneurysmal degeneration and atherosclerosis | CTA or MRA | Surgical bypass for symptomatic lesions, close follow-up for aneurysmal degeneration with arterial duplex ultrasonography for asymptomatic lesions |
| Phlegmasia cerulea dolens | Sudden onset of lower extremity pain, swelling, cyanosis, and arterial ischemia | Extensive acute ileofemoral venous thrombosis | Clinical 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 disease | In a cigarette smoker: claudication, ischemic foot pain at rest, non-healing digital ulcers | Nonatherosclerotic small-arterial segmental inflammatory disease | Shionoya criteria (see text) | Cessation of all tobacco use including cigarettes, gum, and second-hand smoke |
| Takayasu arteritis | Pulselessness, pulse or pressure discrepancy in the arms, unobtainable blood pressure measurement, limb fatigability and pain | Vasculitis of the aorta and its main branches | Multiple 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 syndrome | Ranges from ischemia of the fingers due to microembolization to acute limb ischemia due to complete thrombosis of the subclavian artery | Compression of the subclavian artery as it exits the thoracic outlet; aneurysmal degeneration and thrombosis | Noninvasive imaging with pulse volume recording followed by arterial duplex ultrasonography, chest radiography, and MRA and CTA with arms up and arms down | Management of the acute arterial injury, followed by removal of the first rib with potential arterial bypass |
| External iliac endofibrosis | Unilateral leg pain at near-maximal exercise in an elite athlete | Thickening of arterial intima | History Duplex ultrasonography CTA or MRA | Avoidance of hip flexion Open surgery |