Popliteal artery entrapment syndrome | |
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Normal course of the popliteal artery at the back of the knee | |
Symptoms | Numbness, discoloration, absent pulses, coolness |
Complications | Distal artery stenosis, limb amputation, arterial thromboembolism |
Duration | Chronic |
Causes | Congenital or functional |
Risk factors | Young athlete males. |
Treatment | Open surgical decompression |
Prognosis | Favorable |
The popliteal artery entrapment syndrome (PAES) is an uncommon pathology that occurs when the popliteal artery is compressed by the surrounding popliteal fossa myofascial structures. [1] This results in claudication and chronic leg ischemia. This condition mainly occurs more in young athletes than in the elderlies. [2] Elderlies, who present with similar symptoms, are more likely to be diagnosed with peripheral artery disease with associated atherosclerosis. [2] Patients with PAES mainly present with intermittent feet and calf pain associated with exercises and relieved with rest. [3] PAES can be diagnosed with a combination of medical history, physical examination, and advanced imaging modalities such as duplex ultrasound, computer tomography, or magnetic resonance angiography. Management can range from non-intervention to open surgical decompression with a generally good prognosis. [4] Complications of untreated PAES can include stenotic artery degeneration, complete popliteal artery occlusion, distal arterial thromboembolism, or even formation of an aneurysm.
In 1879, the syndrome was first described in a 64 years old male by Anderson Stuart, a medical student. [1] In 1959, Hamming and Vink first described the management of the PAES in a 12-year-old patient. The patient was treated with myotomy of the medial head of the gastrocnemius muscle and concomitant endarterectomy of the popliteal artery. They later reported four more cases and claimed that the incidence of this pathology in patients younger than 30 years old with claudication was 40%. Servello was the first to draw attention to diminished distal pulses observed with forced plantar- or dorsiflexion in patients with this syndrome. [5] In 1981, Bouhoutsos and Daskalakis reported 45 cases of this syndrome in a population of 20,000 Greek soldiers. [6] Over the last few decades, the increasing frequency with which popliteal artery entrapment is reported, strongly suggests greater awareness of the syndrome. [7]
In the general population, popliteal artery entrapment syndrome (PAES) has an estimated prevalence of 0.16%. [8] It is most commonly found in young, physically active males. [2] In fact, sixty percent of all cases of this syndrome occur in athletically active males under the age of 30. [9] The predilection of this syndrome presents in a male to female ratio of 15:1. [8] This discrepancy in prevalence may be overestimated due the findings that males are generally found to be more physically active than females or because a large portion of the data is from military hospitals that treat mostly male populations. [9] People, who participate in running, soccer, football, basketball, or rugby, are at increased risk. [10]
Newborns and young children are also at increased PAES risk due to congenital causes. During embryonic development, the medial head of gastrocnemius migrates medially and superiorly. This migration can cause structural abnormalities, such as irregular positioning of the popliteal artery, and can account for the rare instances of entrapment caused by the popliteus muscle. [9] Less than 3% of all people are born with this anatomical defect that progresses into PAES, and of those who are born with the anatomical defect, the majority never develop symptoms. [10] Bilateral presentation of PAES is found in approximately 30% of cases. [11]
PAES can be classified as either congenital or functional. [3] Analysis of human embryological development has shown that the popliteal artery and the medial head of the gastrocnemius muscle arise at approximately the same time. Because of that, abnormal development of muscle's position in relation to the nearby vessels can result in potential vascular compromise. [3] The varying types of PAES can be classified based on aberrant migration and resultant attachments of the medial head of the gastrocnemius muscle. Type VI PAES (functional PAES) describes a subtype that is due to repeated microtrauma resulting in the destruction of the internal elastic lamina and damage to the smooth muscles resulting in fibrosis and scar formation.
Type I | The popliteal artery courses more medially around a normally positioned medial head of the gastrocnemius muscle. [3] |
Type II | The medial head of the gastrocnemius muscle attaches more laterally to the femur. [3] |
Type III | Aberrant additional tendon of the gastrocnemius muscle encircles a normally positioned popliteal artery. [3] |
Type IV | The popliteal artery is compressed by the popliteus muscle or a fibrous brand. [3] |
Type V | The compression of both the popliteal artery and vein by any of the above causes. [3] |
Type VI | The normally positioned popliteal artery is entrapped by the gastrocnemius muscle hypertrophy. [3] |
Additionally, a more practical classification system was introduced by Heidelberg et al. [12] This system classifies PAES into three main types:
Patients with PAES are typically healthy young males without previous history of cardiovascular risk factors such as smoking, hypertension, hypercholesterolemia, or diabetes. [13] Typically, patients present with intermittent claudication that is worsened with exercise and relieved with rest. [3] Associated symptoms include numbness, discoloration, pallor, and coolness in the affected lower extremity. [13] Physical examination of suspected PAES may show hypertrophy of the calf muscles, as well as diminished, unequal, or absent pulses in the lower extremity upon plantar- or dorsiflexion. [14]
PAES should be suspected in young healthy male patients with clinical symptoms consistent with compression of the vascular structures and without significant cardiovascular risk factors such as smoking. [13] Multiple imaging modalities are used to confirm the diagnosis of PAES. [23] Based on a systemic review by Sinha et al, digital subtraction angiography (DSA) is the most common imaging used for PAES diagnosis, followed by ankle–brachial index (18 percent), computed tomography angiography (CTA) (12 percent), magnetic resonance angiography (MRA) (12 percent), duplex ultrasonography (DU) (10 percent), exercise ankle-brachial index (4 percent), and other modalities (4 percent). [23] According to a recent study by Willimas et al, a combination of DU and MRA is far superior in diagnosing PAES. [24]
Provocative maneuvers can be used to improve visualization of PAES on the imagines. [25] The patient is initially positioned supine with the legs straight, and then instructed to forcefully plantar-flex. A plantarflexion force of 0 to 70 percent maximum has been shown to maximize the sensitivity and specificity for PAES diagnosis. [25] The DU can be a quick, inexpensive, and noninvasive initial screening for PAES. Flow velocities in the popliteal artery will increase, as the popliteal artery is compressed, which is reflected on the DU. If DU is negative but there is still strong suspicion for PAES, MRA or CTA with provocative maneuvers are needed as follow-up imaging. MRA would demonstrate a focal occlusion or narrowing of the mid-popliteal artery, post-stenotic dilatation, or aneurysm of the distal popliteal artery. If MRA or CTA is [13] non-conclusive, DSA may be used as a further option with a high sensitivity (> 97%) for PAES diagnosis. [23]
Additionally, functional PAES in which the gastrocnemius hypertrophy causes arterial compression during exercise can be best evaluated with dynamic CT. [23] For dynamic CT, initial images are taken with the patient still. Further images are taken following a series of provocative maneuvers.
The outcome following the surgery is usually favorable. Successful resolution of PAES occurs in 77 percent of cases. [23] Surgical complications include deep vein thrombosis, hematoma, wound infection, or seroma. [23] After the surgery, patient is usually monitored using arterial duplex ultrasonography 1, 3, 6, and 12 months, and annually after that. [3]
Early detection and management of PAES can lead to a favorable outcome. [23] However, prolonged compression of popliteal artery can lead to extensive arterial damage and permanent claudication or limb loss. [28] Complications of PAES may include: [13]
Nonetheless, the course of PAES is often slow and takes time, thus, limbs loss is rarely seen, even in PAES patients. [13]
Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both diagnostic and therapeutic procedures through very small incisions or body orifices. Diagnostic IR procedures are those intended to help make a diagnosis or guide further medical treatment, and include image-guided biopsy of a tumor or injection of an imaging contrast agent into a hollow structure, such as a blood vessel or a duct. By contrast, therapeutic IR procedures provide direct treatment—they include catheter-based medicine delivery, medical device placement, and angioplasty of narrowed structures.
Peripheral artery disease (PAD) is a vascular disorder that causes abnormal narrowing of arteries other than those that supply the heart or brain. PAD can happen in any blood vessel, but it is more common in the legs than the arms.
Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.
A Baker's cyst, also known as a popliteal cyst, is a type of fluid collection behind the knee. Often there are no symptoms. If symptoms do occur these may include swelling and pain behind the knee, or knee stiffness. If the cyst breaks open, pain may significantly increase with swelling of the calf. Rarely complications such as deep vein thrombosis, peripheral neuropathy, ischemia, or compartment syndrome may occur.
The popliteal artery is a deeply placed continuation of the femoral artery opening in the distal portion of the adductor magnus muscle. It courses through the popliteal fossa and ends at the lower border of the popliteus muscle, where it branches into the anterior and posterior tibial arteries.
In medicine, aortoiliac occlusive disease is a form of central artery disease involving the blockage of the abdominal aorta as it transitions into the common iliac arteries.
Takayasu's arteritis (TA), also known as aortic arch syndrome, nonspecific aortoarteritis, and pulseless disease, is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, most commonly affecting young or middle-aged women of Asian descent, though anyone can be affected. It mainly affects the aorta and its branches, as well as the pulmonary arteries. Females are about 8–9 times more likely to be affected than males.
Arteritis is a vascular disorder characterized by inflammation of the walls of arteries, usually as a result of infection or autoimmune responses. Arteritis, a complex disorder, is still not entirely understood. Arteritis may be distinguished by its different types, based on the organ systems affected by the disease. A complication of arteritis is thrombosis, which can be fatal. Arteritis and phlebitis are forms of vasculitis.
Intermittent claudication, also known as vascular claudication, is a symptom that describes muscle pain on mild exertion, classically in the calf muscle, which occurs during exercise, such as walking, and is relieved by a short period of rest. It is classically associated with early-stage peripheral artery disease, and can progress to critical limb ischemia unless treated or risk factors are modified and maintained.
The popliteal vein is a vein of the lower limb. It is formed from the anterior tibial vein and the posterior tibial vein. It travels medial to the popliteal artery, and becomes the femoral vein. It drains blood from the leg. It can be assessed using medical ultrasound. It can be affected by popliteal vein entrapment.
The biceps femoris is a muscle of the thigh located to the posterior, or back. As its name implies, it consists of two heads; the long head is considered part of the hamstring muscle group, while the short head is sometimes excluded from this characterization, as it only causes knee flexion and is activated by a separate nerve.
The plantaris is one of the superficial muscles of the superficial posterior compartment of the leg, one of the fascial compartments of the leg.
Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory disease of the blood vessels that causes abnormal growth within the wall of an artery. FMD has been found in nearly every arterial bed in the body, although the most commonly affected are the renal and carotid arteries.
Acute limb ischaemia (ALI) occurs when there is a sudden lack of blood flow to a limb, within 14 days of symptoms onset. It is different from another condition which is more chronic called critical limb ischemia (CLD). CLD is the end stage of peripheral vascular disease where there is still some collateral circulation (alternate circulation pathways} that bring some blood to the distal parts of the limbs. While limbs in both acute and chronic limb ischemia may be pulseless, a chronically ischemic limb is typically warm and pink due to a well-developed collateral artery network and does not need emergency intervention to avoid limb loss.
A tibial plateau fracture is a break of the upper part of the tibia (shinbone) that involves the knee joint. This could involve the medial, lateral, central, or bicondylar. Symptoms include pain, swelling, and a decreased ability to move the knee. People are generally unable to walk. Complication may include injury to the artery or nerve, arthritis, and compartment syndrome.
A popliteal artery aneurysm (PAA) is a bulging (aneurysm) of the popliteal artery. A PAA is diagnosed when a focal dilation greater than 50% of the normal vessel diameter is found. PAAs are the most common aneurysm of the peripheral vascular system, accounting for 85% of all cases. PAAs are bilateral – occurring in both sides of the body – in some 50% of cases, and are often (40-50%) associated with an abdominal aortic aneurysm.
Chronic limb threatening ischemia (CLTI), also known as critical limb ischemia (CLI), is an advanced stage of peripheral artery disease (PAD). It is defined as ischemic rest pain, arterial insufficiency ulcers, and gangrene. The latter two conditions are jointly referred to as tissue loss, reflecting the development of surface damage to the limb tissue due to the most severe stage of ischemia. Compared to the other manifestation of PAD, intermittent claudication, CLI has a negative prognosis within a year after the initial diagnosis, with 1-year amputation rates of approximately 12% and mortality of 50% at 5 years and 70% at 10 years.
A knee dislocation is an injury in which there is disruption of the knee joint between the tibia and the femur. Symptoms include pain and instability of the knee. Complications may include injury to an artery, most commonly the popliteal artery behind the knee, or compartment syndrome.
Popliteal bypass surgery, more commonly known as femoropopliteal bypass or more generally as lower extremity bypass surgery, is a surgical procedure used to treat diseased leg arteries above or below the knee. It is used as a medical intervention to salvage limbs that are at risk of amputation and to improve walking ability in people with severe intermittent claudication and ischemic rest pain.
Arterial occlusion is a condition involving partial or complete blockage of blood flow through an artery. Arteries are blood vessels that carry oxygenated blood to body tissues. An occlusion of arteries disrupts oxygen and blood supply to tissues, leading to ischemia. Depending on the extent of ischemia, symptoms of arterial occlusion range from simple soreness and pain that can be relieved with rest, to a lack of sensation or paralysis that could require amputation.