Version May 2024
INTRODUCTION — Nonatheromatous popliteal artery diseases that can cause symptoms of claudication or, rarely, limb-threatening ischemia predominantly include popliteal artery entrapment syndrome and adventitial cystic disease. Affected patients are typically younger compared with those affected by peripheral artery disease and lack typical risk factors associated with atherosclerosis.
Treatment of symptomatic patients generally requires an open surgical approach to manage the vascular abnormality, although for some patients, medical management may be an option.
The clinical features, diagnosis, and treatment of nonatheromatous popliteal artery disease are reviewed.
The clinical features, diagnosis, and management of atherosclerotic lower extremity disease are reviewed elsewhere. Popliteal artery aneurysm is also reviewed separately. (See "Overview of lower extremity peripheral artery disease" and "Clinical features and diagnosis of lower extremity peripheral artery disease" and "Management of claudication due to peripheral artery disease" and "Popliteal artery aneurysm" and "Surgical and endovascular repair of popliteal artery aneurysm".)
ANATOMIC CONSIDERATIONS — The popliteal artery is a continuation of the femoral artery as it exits the adductor canal (Hunter canal), which is the aponeurotic passageway from the anterior thigh to posterior leg (figure 1). Within the popliteal fossa, the popliteal artery is the deepest structure and lies adjacent the knee joint capsule. Multiple geniculate branches provide blood supply to the knee joint (figure 2) as well as provide collateral flow to the leg during maximal knee flexion. Important muscles relevant to the pathologies discussed below (popliteal artery entrapment syndrome, adventitial cystic disease) include the popliteus muscle, which normally lies anterior to the popliteal artery, and the medial head of the gastrocnemius muscle of the calf, which lies medially in the distal portion of the popliteal space.
CLINICAL FEATURES AND DIAGNOSIS — The clinical presentation of nonatheromatous popliteal disease is primarily intermittent claudication (derived from the Latin word for limp), which is defined as a reproducible discomfort in a muscle group induced by exercise and relieved with rest. The severity of claudication symptoms depends upon the degree of obstruction, the effectiveness of the collateral circulation, and the level of exercise.
Diagnosis of vascular obstruction — Most patients with symptoms of claudication undergo noninvasive vascular testing, including physiologic testing with or without duplex ultrasound. Symptomatic patients with nonatheromatous popliteal disease often have normal ankle-brachial index values at rest but will have abnormal studies following exercise. As such, the first step in the evaluation of a patient with claudication and suspected nonatheromatous arterial disease should be exercise testing. (See "Noninvasive diagnosis of upper and lower extremity arterial disease", section on 'Exercise testing'.)
Imaging confirms vascular obstruction and etiology.
DIFFERENTIAL DIAGNOSIS — The differential diagnosis of nonatherosclerotic popliteal artery disease is primarily obstruction due to atherosclerosis (ie, peripheral artery disease [PAD]) causing claudication or other atypical symptoms. However, while PAD typically presents later in life, a subset of younger patients with more aggressive disease does exist. However, patients with PAD most often have typical risk factors for atherosclerotic disease (eg, smoking, hypertension, dyslipidemia, diabetes, chronic kidney disease), which are typically absent in those with nonatheromatous disease. (See "Clinical features and diagnosis of lower extremity peripheral artery disease".)
Nonatheromatous popliteal artery diseases often affect younger people, and the differential diagnosis is often quite broad (table 1) [1]. Claudication symptoms can frequently be mistaken for symptoms related to muscle strains or tears, stress fractures, or chronic compartment syndrome in affected younger patients [2]. In addition, patients with nonatheromatous vascular disease often have palpable pedal pulses at rest, which further confuses the issue [3]. Endofibrosis is another nonatheromatous arterial disease that affects the iliac artery related to repetitive trauma usually due to athletic activities [4].
Popliteal venous compression can coexist with nonatheromatous arterial diseases. Popliteal venous entrapment syndrome as an isolated entity is much less common compared with popliteal artery entrapment syndrome [5,6]. Other conditions that can cause lower extremity edema, which can be associated with nonatheromatous arterial diseases, include deep venous thrombosis and chronic venous insufficiency. In a young patient with unilateral swelling and no other risk factors for venous thrombosis, iliac vein obstruction should be considered. (See "Clinical manifestations and evaluation of edema in adults" and "Clinical manifestations of lower extremity chronic venous disease" and "Clinical presentation and diagnosis of the nonpregnant adult with suspected deep vein thrombosis of the lower extremity" and "May-Thurner syndrome".)
SPECIFIC SYNDROMES — While symptoms of ischemia and the general approach to evaluation are similar for nonatherosclerotic popliteal disease, treatment and prognosis are specific to each
syndrome. The most common nonatheromatous popliteal artery diseases, popliteal artery entrapment syndrome and adventitial cystic disease, are reviewed in detail below; other less common etiologies are briefly listed with links to separate topic reviews. (See 'Other nonatheromatous popliteal diseases' below.)
Popliteal artery entrapment syndrome — Popliteal artery entrapment syndrome (PAES) is a condition in which there is an abnormal anatomic relationship between the neurovascular structure of the popliteal fossa and nearby musculotendinous structures. Popliteal artery entrapment is the most common form, and popliteal artery compression by one of the calf muscles can reduce blood flow to the leg, causing ischemic symptoms. Symptoms of PAES are usually chronic, but acute limb ischemia (ALI) can also occur.
●PAES can occur as either a congenital or acquired condition. In the congenital form (types I to VI), one of the calf muscles develops in an abnormal position during fetal development, resulting in displacement or compression of the popliteal artery. In the acquired form of PAES (types F), one of the calf muscles becomes enlarged, resulting in compression of the popliteal artery. The acquired form is commonly referred to as functional PAES. (See "Popliteal entrapment syndromes", section on 'Classifications'.)
●Patients with PAES are typically young and male without atherosclerotic risk factors such as smoking, hypertension, hypercholesterolemia, and diabetes [7]. Symptomatic patients typically complain of classic intermittent claudication related to arterial compression [8,9]. ALI occurs in approximately 11 percent of patients [8]. Many cases are bilateral, although the exact prevalence of bilateral PAES is unknown (25 to 83 percent in the available literature [8-10]). (See "Popliteal entrapment syndromes", section on 'Clinical presentations'.)
●Duplex ultrasound can be used to establish a diagnosis of PAES. Cross-sectional imaging using CT or MR angiography will show the anatomic relationship neurovascular structures in the popliteal fossa to the gastrocnemius muscle. Surgical decompression of the popliteal space is the mainstay of treatment for symptomatic PAES. (See "Popliteal entrapment syndromes", section on 'Management approach' and "Popliteal entrapment syndromes", section on 'Summary and recommendations'.)
Adventitial cystic disease — Adventitial cystic disease (ACD) is a condition of unknown etiology that results in cysts containing gelatinous mucoid material located between the adventitial and medial layers of the artery, causing luminal stenosis or occlusion [11]. The cysts are usually unilocular, lack a cellular lining, and contain hyaluronic acid, protein, and proteoglycans [12,13].
●Patients with lower extremity ACD most commonly present with unilateral intermittent calf claudication [14]. The right and left lower extremity are affected with similar frequency [15]. As compared with PAD, however, patients with ACD and claudication frequently have prolonged post-exercise recovery time before pain resolution and may report a waxing and waning of symptoms due to fluctuating amounts of cyst content present at any given time. Patients can also present with ALI, pain, paresthesias, or a palpable tissue masses [14].
●While ACD should be considered in any patient lacking typical cardiovascular risk factors but presenting with claudication symptoms, particularly in younger patients. Vascular imaging can demonstrate the cystic lesion(s) of the affected artery and the severity of any associated stenosis or complete occlusion [14]. (See "Adventitial cystic disease", section on 'Clinical presentations' and "Adventitial cystic disease", section on 'Diagnostic evaluation'.)
●Treatment of symptomatic patients is warranted to prevent progression to a fixed lesion or to prevent limb-threatening ischemia due to thrombosis of the stenotic segment of the artery. Options for treatment include cyst aspiration, which is associated with recurrence in up to half of patients, or cyst excision with or without vascular reconstruction depending on the extent of the cystic changes [16]. (See "Adventitial cystic disease", section on 'Treatment'.)
Other nonatheromatous popliteal diseases — Other nonatheromatous diseases that can affect the popliteal artery and cause ischemic lower extremity symptoms include the following [17,18]:
Fibromuscular dysplasia — Fibromuscular dysplasia (FMD) most commonly affects the renal arteries and extracranial cerebrovascular arteries, but it can affect the popliteal artery, leading to ischemic symptoms due to embolism or related to formation of popliteal aneurysm. (See "Clinical manifestations and diagnosis of fibromuscular dysplasia" and "Popliteal artery aneurysm".)
Thromboangiitis obliterans (buerger disease) — Thromboangiitis obliterans (TAO) more typically affects the small vessels of the hands and feet. Large artery involvement can occur, but when it does, it rarely occurs in the absence of small vessel occlusive disease. Cannabis can lead to a vasculitis that is clinically similar to TAO [17]. (See "Thromboangiitis obliterans (Buerger disease)" and "Cannabis use and disorder: Epidemiology, pharmacology, comorbidities, and adverse effects".)
Medium and large vessel vasculitis — Arterial involvement occurs in 10 to 30 percent of patients with Behcet syndrome, largely related to aneurysm formation [18]. Other forms of vasculitis more typically involve the vessels of the upper extremity, but rarely the lower extremities can be involved [19]. (See "Clinical manifestations and diagnosis of Behçet syndrome", section on 'Arterial disease' and "Overview of and approach to the vasculitides in adults" and "Popliteal artery aneurysm".)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Acute extremity ischemia".)
SUMMARY AND RECOMMENDATIONS
●Nonatheromatous arterial diseases – Nonatheromatous popliteal artery diseases are uncommon conditions and include predominantly popliteal artery entrapment syndrome (PAES) and adventitial cystic disease (ACD). Affected patients are typically younger compared with those affected by peripheral artery disease (PAD) and lack typical risk factors associated with atherosclerosis. (See 'Clinical features and diagnosis' above.)
•Popliteal artery entrapment syndrome – Popliteal artery entrapment syndrome (PAES) is due to an abnormal anatomic relationship between the neurovascular structures of the popliteal fossa and nearby musculotendinous structures that can be congenital or acquired. PAES can be bilateral. PAES is classified based on the relationship of the medial head of the gastrocnemius muscle with the vascular structures, predominantly the popliteal artery. (See 'Popliteal artery entrapment syndrome' above.)
•Adventitial cystic disease – ACD is a condition of unknown etiology that results in cysts containing gelatinous mucoid material located between the adventitia and medial layers of the artery causing luminal stenosis or occlusion. ACD is more common in males compared with females and typically presents in the fourth or fifth decade of life. (See 'Adventitial cystic disease' above.)
●Clinical features and diagnosis – Nonatheromatous popliteal artery disease can reduce perfusion to the extremity typically causing chronic ischemic symptoms, but acute limb ischemia or other related symptoms can also occur. Patients with nonatheromatous popliteal disease predominantly present with intermittent claudication, which a reproducible discomfort in a muscle group induced by exercise and relieved with rest. Other symptoms include paresthesias, or limb swelling related to nerve or venous compression, respectively. Symptomatic patients often have normal ankle-brachial index (ABI) values at rest, but with exercise testing, ABI values markedly decrease. Vascular imaging (eg, ultrasound, cross-sectional imaging, angiography) confirms the diagnosis. (See 'Clinical features and diagnosis' above.)
●Differential diagnosis – The differential diagnosis of nonatherosclerotic disease is primarily obstruction due to atherosclerosis (ie, PAD) causing claudication or other atypical symptoms. Because nonatheromatous popliteal artery diseases usually affects younger people, the differential diagnosis of exertional leg pain is broad (table 1). (See 'Differential diagnosis' above.)
●Treatment – Patients who are asymptomatic do not require treatment. For symptomatic patients, treatment generally requires an open surgical approach to manage the vascular abnormality, although for some patients less invasive therapy, though not endovascular therapy, may be an option (eg, botulinum toxin for PAES; cyst aspiration for ACD). Long-term patency rates following surgical management of nonatheromatous arterial disease in the absence of thrombosis are generally excellent.
•Surgical treatment of PAES involves decompression of the popliteal space with reconstruction of the artery, if damaged. (See 'Popliteal artery entrapment syndrome' above.)
•Surgical treatment of ACD involves surgical excision of the cyst and interposition grafting using saphenous vein reconstruction. (See 'Adventitial cystic disease' above.)
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