ACHAIKI IATRIKI | 2021; 39(1):15–17
Editorial
Ioannis Ntouvas, Chrisanthi Papageorgopoulou, Konstantinos Nikolakopoulos
Department of Vascular Surgery, University Hospital of Patras, Patras, Greece
Received: 28 February 2020; Accepted: 16 March 2020
Corresponding author: Angeliki Zarkada, 16 Kononos street, 11634 Athens, Tel.: +30 6976 731819, E-mail: aggelikazark@hotmail.gr
Key words: Paediatric dysphagia, assessment, management, speech and language pathologist
Intermittent claudication (IC) is a symptom defined as reproducible fatigue, discomfort, or pain that occurs mainly in calf due to ischemia. It is a common symptom in older patients suffering from atherosclerotic peripheral vascular disease. However, intermittent claudication in young patients should prompt a search for causes other than atherosclerosis. The differential diagnosis includes well-known entities such as popliteal artery entrapment syndrome, cystic adventitial disease, fybromuscular dysplasia, Takayasu arteritis, Buerger disease and arterial compression by tumor.
Popliteal artery entrapment syndrome (PAES) is the most common cause of intermittent claudication in young patients. Its overall incidence ranges from 0.17% to 3.5% in the general population. Furthermore, the majority of patients (85%), are males, with almost 60% of cases occurring in young athletes during the third decade of life. In about 30% of patients, the disease has a bilaterally symptomatic presentation [1,2]. The term popliteal artery entrapment refers to popliteal artery compression caused by an abnormal anatomical relationship between the vessel and nearby musculotendinous structures or surrounding muscle hypertrophy. This arterial compression which initially causes intermittent claudication, might lead to chronic vascular microtraumas of the arterial wall with possible intramural hematoma or thrombus, distal embolization, aneurysm, dissections and thrombosis with acute distal ischemia [3]. PAES is further classified into six different types based on the relationship of the medial head of the gastrocnemius muscle with the popliteal artery [4]:
- Type I: An aberrant medial course of the popliteal artery around a normally positioned medial head of the gastrocnemius muscle (MHG)
- Type II: MHG attaches abnormally and more laterally on the femur causing the popliteal artery to pass medially and inferiorly
- Type III: Abnormal fibrous band or accessory muscle arising from the medial or lateral condyle encircling the popliteal artery
- Type IV: Popliteal artery lying in its primitive deep or axial position within the fossa, becoming compressed by the popliteus muscle or fibrous bands
- Type V: Entrapment of both the popliteal artery and vein due to any of the causes mentioned above
- Type VI: Muscular hypertrophy, resulting in a functional compression of both the popliteal artery and vein
The diagnosis of popliteal artery entrapment syndrome requires not only the depiction of the arterial changes but also the identification of the abnormal anatomic structures responsible for the entrapment. Although arterial compression can be shown on conventional angiography or ultrasonography with provoked maneuvers, such us plantar flexion or dorsiflexion, the underlying anatomic abnormality cannot be identified on either modality. Tailored MRI and MR angiography can reveal the abnormal muscular or fibrous attachment and the arterial findings necessary for diagnosis and surgical planning.
Treatment of PAES requires surgical release of the popliteal artery occasionally followed by a by-pass through the interposed saphenous vein graft in case that the popliteal artery is affected.
Cystic adventitial disease (CAD) is a rare vascular disorder. CAD predominantly affects the arteries, although rare reports of CAD of the veins have also been described. The majority (85%) of the cysts in CAD is found in the popliteal artery, but the disease may affect the external iliac, femoral, radial, ulnar, brachial, and axillary arteries as well. Bilateral disease has also been described [5]. CAD predominantly occurs in young to middle-aged population; however, the age of presentation ranges from 11 to 70 years old. CAD has a male predisposition, with a male-to-female ratio of 5:1 [6]. Various theories have been proposed for the origin of CAD, including a systemic disorder, repetitive trauma, and a persistent embryonic synovial track. Symptoms are caused by compression of the arterial lumen by a cystic collection of mucinous material inside the adventitia of the artery. The pain insists for as long as 20 minutes after the cessation of activity, in contrast to the rapid relief of pain that most patients with PAD-associated limb discomfort experience. CAD-related intermittent claudication may vary in clinical presentation or even disappear for several months and reappear without any clear inaugural event. On physical examination, peripheral pulse may or may not be absent at rest. The Ishikawa’s sign can be seen in CAD, which is the disappearance of the foot pulses with flexion of the knee. This differentiates CAD from the popliteal entrapment syndrome, where the pulse would disappear with contraction of the gastrocnemius muscle during active plantar flexion or passive dorsiflexion of the foot. Conventional angiography typically reveals a smoothly tapered narrowing of the mid-popliteal artery with otherwise normal-appearing lower extremity arteries. Ultrasonography, CT, or MRI may also show the cystic structure in the arterial wall. A number of techniques exist for the treatment of CAD, including surgical intervention, percutaneous aspiration, and percutaneous endovascular intervention. However, the decision to treat should be based on clinical and radiological presentations. Conservative treatment of CAD should be considered first, as the cysts in CAD may resolve spontaneously, or the patient may not experience significant discomfort to prompt for more invasive treatment options [7].
Endofibrosis of the iliac artery is another rare cause of arterial stenosis and intermittent claudication in young adults. It is reported most often in highly functioning and competitive cyclists and runners [8]. It is considered to result from repetitive trauma, predominantly of the external iliac artery. Symptoms include IC and a sensation of swelling or paresthesia in the proximal lower limb at the time of maximum exercise. Physical examination may be normal at rest, although a bruit may be heard over the ipsilateral pelvic fossa or inguinal region [9].
Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic arterial disease that occurs most commonly in women 20 to 60 years of age. The true prevalence of FMD is unknown. The renal arteries and the extracranial carotid arteries are mostly affected. However, less commonly, FMD may affect the iliac, femoral, or popliteal arteries. Lower-extremity involvement may result in IC, microembolisms, or (rarely) critical limb ischemia via dissection or rupture of the artery [10]. FMD is divided into several types according to which arterial layer is affected and by the arteriographic pattern of disease: medial fibroplasia, intimal fibroplasia, and adventitial (periarterial) hyperplasia. Medial fibroplasia is the most common type [11]. Angiographic studies will show a string-of-beads appearance reflecting multiple adjacent stenosis and focal aneurysm. Treatment of FMD depends on the location and the extension of the stenosis and includes by-pass surgery or endovascular treatment.
Takayasu arteritis, most commonly seen in young Asian women, is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation. Intermittent claudication as a result of aorta involvement, is a common symptom. The disease commonly emerges in the 2nd or 3rd decade of life [12]. Although the disease mostly affects the female population, the female:male ratio seems to decline from Eastern Asia to the West. Conventional angiography is the standard imaging tool in the evaluation of Takayasu’s arteritis, showing nonspecific focal stenoses. Whereas CT angiography, MRI, and MRA may show mural thickening in addition to narrowing of the lumen. Steroids are the mainstay of treatment with acceptable results. Surgery is recommended when the disease is in remission to avoid complications, which include restenosis, anastamotic failure, thrombosis, haemorrhage and infection. Endovascular treatment is another option [13,14].
Thromboangiitis obliterans or Buerger’s disease is a segmental occlusive inflammatory condition affecting small and medium sized arteries and veins of the upper and lower-extremities in heavy smokers [15]. The onset of Buerger’s disease occurs between 40 and 45 years of age, and men are most affected. Several different criteria have been proposed for the diagnosis of thromboangiitis obliterans. The most accepted are the criteria of Olin [16]:
- age under 45 years
- current or recent history of tobacco use
- the presence of distal-extremity ischemia indicated by claudication, pain at rest, ischemic ulcers or gangrenes and documented by non-invasive vascular testing
- exclusion of autoimmune diseases, hypercoagulable states and diabetes mellitus
- exclusion of a proximal source of emboli by echocardiography or arteriography
- consistent arteriographic findings in the clinically involved and non-involved limbs
Conventional angiography shows multilevel occlusions and segmental narrowing of the lower extremity arteries with extensive collateral flow, which has a characteristic corkscrew or “tree root” appearance. The only effective treatment of intermittent claudication in Buerger’s disease is smoking cessation.
Extrinsic artery compression by a tumor such us an osteochondroma, lipoma etc. is an extremely rare cause of intermittent claudication. Most reported cases of extrinsic compression are in veins, which have a thinner muscle layer in comparison to arteries and are consequently less resistant to vascular collapse. A radiograph of the affected extremity to exclude a bone lesion causing extrinsic compression of the nearby artery is of importance in patients with intermittent claudication but no signs of intrinsic vascular disease. Other imaging tests (CT or MRI) could complete the investigation [17].
In conclusion, intermittent claudication in young adults remains a diagnostic challenge. As it is mentioned above, many nosological entities may be responsible for this condition. In any case, the combination of thorough clinical examination and targeted imaging evaluation could lead to the cause of the intermittent claudication.
Conflict of interest disclosure
No potential conflict of interest
Declaration of funding sources
No financial support
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