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Leriche Syndrome

Leriches Syndrome

Leriche syndrome, also known as Leriche's syndrome is a type of occlusive peripheral artery disease that occurs at the bifurcation of the abdominal aorta into the common iliac arteries.


Presentation

Leriche syndrome classically presents in the form of a triad of specific symptoms. The most common of these is claudication, which is described as muscle cramping pain of the lower extremities present during exertion and alleviated by cessation of activity. Characteristically, for each particular patient the time after exertion at which claudication occurs is almost always constant. Cramping is experienced firstly in the calf muscles and may progress to involve muscles of the buttocks, hips and thighs. Claudication of thigh or buttock muscles is a sign of severe aortoiliac disease. Further physical examination may reveal cold, pale and weak extremities.

The second symptom in the triad is impotence manifested as difficulty or inability to maintain penile erection. This usually accompanies buttock claudication.

The third symptom in the triad is a weak or absent femoral pulse resulting from more proximal arterial occlusions [11]. Critical limb ischemia in the lower limbs rarely develops because of the presence of alternative blood supply sourced from a dense network of collateral blood vessels that form as the disease progresses slowly over time.

Intermittent Claudication
  • We describe the case of a 60-year-old male hypertensive patient who was admitted to our department with intermittent claudication. An echocardiography evaluation detected apical hypertrophy without an intracavity pressure gradient.[ncbi.nlm.nih.gov]
  • Medical management (smoking cessation, aspirin , statin, cilostazol ) is indicated in patients with intermittent claudication. Procedural treatm...[5minuteconsult.com]
  • Leriche's syndrome [lərēshs′] Etymology: René Leriche, French surgeon, 1879-1955 a vascular disorder marked by gradual occlusion of the terminal aorta, bilateral iliac arteries, or both; intermittent claudication in the buttocks, thighs, or calves; absence[medical-dictionary.thefreedictionary.com]
  • Presentation Intermittent claudication, pallor, and pain on lower limbs. Patient Data Age: 55 years Gender: Male Loading images...[radiopaedia.org]
  • Pain in the thighs, hips, and buttocks ( intermittent claudication ). Erectile dysfunction (impotence). Weak pulse in the thigh arteries (femoral arteries). Credits By Healthwise Staff Primary Medical Reviewer Rakesh K.[northshore.org]
Coronary Artery Disease
  • Further it is often associated with chronic renal failure and coronary artery disease. Diagnosis is normally made by computed tomography (CT) or magnetic resonance imaging (MRI).[ncbi.nlm.nih.gov]
  • Coronary artery disease in octogenarians is often diffuse and difficult to manage due to concomitant peripheral vascular disease.[ncbi.nlm.nih.gov]
  • Coronary artery disease (CAD) is the major determinant of preoperative morbidity and mortality for patients requiring major vascular surgery. The management of CAD in these patients is controversial.[ncbi.nlm.nih.gov]
  • His past medical history includes hyperlipidemia and coronary artery disease. He has a 20-pack-year smoking history. On physical exam, there are bilateral diminished 1 femoral pulses. His ankle-brachial index is 0.6 on the left and 0.7 on the right.[step2.medbullets.com]
  • artery disease Myocardial infarction Stroke PAD -- To view the remaining sections of this topic, please sign in or purchase a subscription -- TY - ELEC T1 - Leriche Syndrome ID - 816303 PB - 5-Minute Clinical Consult, Updating UR - ER -[unboundmedicine.com]
Renal Artery Stenosis
  • We encountered a patient with Leriche syndrome who had renovascular hypertension ascribed to a severe left renal artery stenosis.[ncbi.nlm.nih.gov]
  • Stenting for renal artery stenosis is well described in the literature. Bilateral renal artery stenting is not such a common procedure, however it is quite rare in patients with Leriche syndrome, as is the case we present.[ncbi.nlm.nih.gov]
  • Severe left renal artery stenosis was also found and considered, if untreated, to be an important factor in aggravation of his renal function.[ncbi.nlm.nih.gov]
  • Renal artery stenosis is causative in 2 - 5% patients with hypertension and accounts for 3 - 15% cases of chronic kidney disease (CKD).[ncbi.nlm.nih.gov]
  • Apical HCM combined with severe renal artery stenosis is very rare and has not previously been reported with Leriche syndrome.[ncbi.nlm.nih.gov]
Constitutional Symptom
  • Constitutional symptoms include raised inflammatory markers with fever, weight loss, or symptoms resembling connective tissue disease due to cytokine (interleukin-6) secretion by the myxoma itself, infection, or malignancy [ 1 ].[bmccardiovascdisord.biomedcentral.com]
Vascular Disease
  • Coronary artery disease in octogenarians is often diffuse and difficult to manage due to concomitant peripheral vascular disease.[ncbi.nlm.nih.gov]
  • We recommend that the surgeon make a most sincere effect to induce patients undergoing vascular operations for occlusive vascular diseases to give up smoking.[ncbi.nlm.nih.gov]
  • NOS , Peripheral vasc. disease NOS , Peripheral vascular disease (& [NOS]) (disorder) , Peripheral vascular disease (& [NOS]) , Peripheral vascular diseases , PERIPHERAL VASCULAR DISEASE , VASCULAR DISEASE, PERIPHERAL , DISEASE, PERIPHERAL VASCULAR ,[fpnotebook.com]
  • diseases I73.89 Other specified peripheral vascular diseases I73.9 Peripheral vascular disease, unspecified I74 Arterial embolism and thrombosis I74.0 Embolism and thrombosis of abdominal aorta I74.01 Saddle embolus of abdominal aorta I74.09 Other arterial[icd10data.com]
  • There was no prior history of peripheral vascular disease, coronary artery disease, hypercoagulable state or prior thrombosis formation.[vpjournal.net]
Heart Disease
  • A woman with ECG findings suspicious of ischemic heart disease was referred for coronary angiography, but this was impossible via the left or right iliac arteries because of total occlusion.[ncbi.nlm.nih.gov]
  • Author Affiliations †Field officer, Heart Disease Control Program, United States Public Health Service. ‡Associate professor of medicine, University of Pennsylvania School of Medicine.[nejm.org]
  • Many things can cause atherosclerosis, including: lack of exercise poor diet, especially diets that are high in fat family history of heart disease obesity smoking diabetes high blood pressure high cholesterol older age While Leriche syndrome is most[healthline.com]
  • Definition (CSP) condition in which there is a deviation from or interruption of the normal structure or function of the blood vessels outside the heart; diseases of the peripheral as opposed to the cardiac circulation.[fpnotebook.com]
Decreased Femoral Pulse
  • Signs and symptoms : Classically, Leriche syndrome is described in male patients as a triad of the claudication of the buttocks and thighs, absent or decreased femoral pulses & erectile dysfunction (impotence).[xpertdox.com]
  • When this condition was first described in the 1940s, a clinician could make the provisional diagnosis of Leriche syndrome in a patient with the triad of claudication, impotence and decreased femoral pulses.[westjem.com]
  • The classic triad of symptoms include claudication, impotence, and absent or decreased femoral pulses. It may be acute or chronic in onset.[vpjournal.net]
  • Signs and symptoms [ edit ] Classically, it is described in male patients as a triad of the following signs and symptoms : claudication of the buttocks and thighs absent or decreased femoral pulses erectile dysfunction This combination is known as Leriche[en.wikipedia.org]
Muscle Cramp
  • Drugs that help relieve muscle cramping (e.g. pentoxyfylline and cilostazol) are aslo prescribed.[symptoma.com]
  • These symptoms can include: Fatigue of both lower limbs Leg weakness or numbness Muscle cramping pain in the thigh, hips, and buttocks (intermittent claudication) Erectile difficulty in men Weak pulse in femoral arteries Cold and pale extremities Ulcers[belmarrahealth.com]
  • The symptom complex of claudication is defined as muscle cramps in the leg(s) that occur after exercise and are relieved by resting. In any individual patient, the exercise distance at which claudication occurs is quite constant.[emedicine.staging.medscape.com]
Leg Pain
  • We report about a 56-year-old man with dyspnoea and leg pain diagnosed with Leriche syndrome and chronic heart failure caused by dilated cardiomyopathy (DCM) with acute cardiac decompensation.[ncbi.nlm.nih.gov]
  • When patients come to hospital with complaints of leg pain, clinicians should consider vascular pathologies before reaching definitive diagnosis, using detailed patient history and comprehensive physical examination.[ncbi.nlm.nih.gov]

Workup

Peripheral arterial disease is diagnosed based on clinical history, physical examination findings and relevant diagnostic investigations. Symptoms are identified in the patient's history while suggestive signs are elicited objectively by the clinician. Examination of peripheral pulses alongside measurement of blood pressure is key. Pulse areas that are checked include the radial, brachial, femoral and popliteal. There is reduced or absent pulse in the arteries distal to the point of occlusion. Alongside checking for pulse, examination for patency of circulation in the extremities is also done by assessing for pallor and temperature changes on the skin over the extremities. Differences in blood pressure between one limb and the other or between a more proximal part and a distal part of the limb point towards the presence of a peripheral arterial disease.

Imaging tests are also used to achieve a diagnosis of occlusive arterial disease. The modality of choice is the non-invasive computed tomography (CT) angiography, which if not available may be substituted by magnetic resonance angiography (MRA) with contrast enhancement [12]. Another form of imaging is Doppler ultrasonography that is ideal for diagnosis of vascular occlusion. Radiographs can also be used for assessment. Invasive angiography is best employed if there is intention to carry out any form of surgical intervention on the occluded vessels.

Laboratory blood tests are done to rule out possible differential diagnoses that may present with vascular occlusion.

Treatment

The goal of treatment in Leriche syndrome is to correct the inadequate blood supply to the extremities by enhancing vascularization.

Surgical intervention

This involves procedures to either remove the occlusion or create a bypass around it. This may either be achieved through invasive surgery or by use of endovascular techniques that include aortoiliac endarterectomy, percutaneous transluminal angioplasty with or without stent placement, aortobifemoral bypass, axillary-bi-femoral and femoral-femoral bypass.

Medical intervention

The use of drugs to alleviate muscle claudication is advised. Pentoxyfylline and cilostazol are examples of recommended drugs. Antiplatelets are also administered as prophylaxis against hypercoagulation of blood.

Supportive treatment

This entails taking regular walking exercises under supervision with the aim of boosting use of oxygen by muscles in the lower extremities.

Treatment of underlying risk factors

Medical conditions such as hyperlipidemia, diabetes and hypertension must be well controlled because they predispose to development of peripheral arterial disease. Social habits like smoking require cessation as they impact negatively on the prognosis.

Prognosis

Being an aortoiliac disease, prognosis of Leriche syndrome is determined by considering patient outcomes in two ways: the rate of mortality associated with arterial surgical repair and the viability of such repairs over the course of time. The following is a table illustrating the different outcomes that determine mortality.

Surgical procedure Surgery related mortality within 30 days Rate of patency over 5 years
Aortoiliac TEA 2-3% 85-90%
Aortobifemoral bypass 2-3% 85-90%
Femorofemoral bypass 0-4% 44-85%
Axillobifemoral bypass 2-11% 19-50%

As an alternative to the conventional surgical procedures above, endovascular techniques are also employed in the management of aortoiliac disease. The most common interventions include placements of stents and percutaneous transluminal angioplasty (PTA). These two procedures are considered ideal for the management of isolated stenosis of the infrarenal aorta or common iliac arteries.

Impressive outcomes have been recorded when PTA is used to correct localized, segmental occlusion of the aorta with a success rate in 95% of the population and a 5 year rate of patency of 80-87%. Likewise, in iliac artery occlusive disease the success rate is 93-97% of the population and a 5 year patency rate of 54-85%. For both aortic and iliac stenosis, better outcomes have been recorded when intrarterial stents are used in isolation or in combination with PTA.

Comparison of the efficacy of endovascular techniques and open bypass surgical interventions for the treatment of aortoiliac disease reveal unique differences in clinical outcomes. Open bypass surgical interventions were linked to higher patency rates initially and over the course of 5 years. On the other hand, endovascular techniques were associated with lower 30 day mortality rates as well as lower rates of complication and reduced length of hospital stay [10].

Etiology

Leriche syndrome arises most frequently due to obstruction at the aortoiliac bifurcation by atherosclerotic plaques and thrombi. Vasculitis may also play a causative role [4]. Once obstruction sets in, several collateral pathways are formed to bypass it and allow vascular flow to the lower limbs [5]. These include:

  • The first collateral pathway formed by the mesenteric, hemorrhoidal and external iliac arteries.
  • The second collateral pathway constituted by the intercostal, subcostal, lumbar, superior gluteal, iliolumbar, internal and external iliac arteries. 
  • The third collateral pathway formed by the intercostal, subcostal, and lumbar arteries joining the circumflex arteries and external iliac arteries.
  • The fourth collateral pathway consisting of the subclavian arteries, internal thoracic arteries, superior and inferior epigastric arteries and external iliac arteries [6].

Epidemiology

Owing to the fact that half of the patient population diagnosed with peripheral arterial disease are symptom-less, little is known concerning the incidence and prevalence of Leriche syndrome. However, documented data shows that the syndrome occurs more commonly in patients with peripheral arterial disease especially with advancing age. Thus, a quarter of the US population at the age of 70 experience the disease.

Leriche syndrome most commonly presents in men, and risk factors include hypertension, diabetes mellitus, hyperlipidemia, and smoking [7].

Sex distribution
Age distribution

Pathophysiology

The presence of atherosclerotic plaques and thrombi in the infrarenal aorta, common iliac, internal and external iliac arteries leads to the development of Leriche syndrome [8] [9]. The syndrome then slowly progresses to involve both the proximal and distal portions [4]. Atherosclerosis in itself develops in a very intricate way and there is yet to be found a specific etiological basis for it. There are however a number of factors that have been associated with the development of atherosclerotic lesions.

The baseline that initiates the disease process in Leriche syndrome is injury to the arterial wall. The most common triggers are the following:

Mechanical factors

  • Intravascular force generated by hypertension
  • Low energy shear stress on the arterial wall

Chemical factors

  • Homocysteine
  • Nicotine
  • Lipemia
  • Abnormally high blood sugar

An arterial wall injury immediately triggers an inflammatory response with gravitation of macrophages to the site of the injury. Lipid accumulation onto these macrophages ensues with deposits of cholesterol, cholesterol esters and triglycerides collectively forming an atheroma. Any exposure of the atheroma contents to platelets circulating in blood triggers platelet aggregation. Glycogen receptors on the surface of platelets are activated causing them to bind to fibrin thus forming a new blood clot. The atheroma-clot complex forms a plaque on the arterial wall causing obstruction. Unstable plaques may break away forming atheromatous emboli that may eventually cause occlusion of the arterial lumen in distal vessels. Significant occlusion occurs when an atheroma increases in size to the extent that it covers at least 50% of the intraluminal arterial space. With such a stenosis, the velocity of blood flowing through the artery is bound to increase.

In the absence of exertion, oxygen requirements of the lower limbs are low and can be catered for even in the presence of a more proximal arterial stenosis. On the contrary, during exertion an oxygen debt is created within the skeletal muscles and this cannot be readily reversed due to obstructed blood flow caused by the stenosis. The effects of this obstruction range from mild symptoms of claudication to features of critical limb ischemia. The latter, however, is rarely associated with aortoiliac disease only, but is more common when arterial occlusion occurs in multiple arterial segments. At its worst, it may cause loss of limb tissue.

Leriche syndrome has varied presentations depending on the underlying pattern of atherosclerosis. The first pattern (type I atherosclerosis) affects only the infrarenal and common iliac arteries. It is more prevalent in women and is found in 5-10 % of the population with peripheral arterial disease. The second pattern (type II atherosclerosis) typically affects the infrarenal aorta, the common iliac and external iliac arteries but may progress to also include the common femoral arteries. The third pattern (type III atherosclerosis) is considered the most critical and most prevalent, found in 35% of patients with peripheral arterial disease. It shows involvement of the infrarenal aorta, iliac artery, femoral artery, popliteal artery and tibial artery.

Prevention

Prevention of Leriche syndrome lies in identifying and eliminating known or modifiable risk factors. Measures to be undertaken include:

Summary

The first time Leriche syndrome was ever described was in 1814 by Robert Graham [1]. However, it was the French surgeon Rene Leriche who was able to connect the disease process and its impact on physiology with the abnormal anatomy identified in Leriche syndrome. Henceforth the condition was described as a triad of symptoms: erectile dysfunction, claudication of the lower limbs and decreased or absent femoral pulses. Leriche later became the first person to publish on the subject after treating a 30 year old patient and successfully resolving his erectile dysfunction and pain associated with walking [2].

Leriche syndrome has a very significant occurrence in patients with peripheral arterial disease. In the latter condition, atherosclerosis leads to the formation of plaques in the infrarenal aorta, common iliac, internal and external iliac arteries. The pattern involves either one of the arteries or several of them concurrently. Depending on their size, plaques may cause arterial lumen obstruction with subsequent impairment of blood flow to the extremities. Other symptomatology occurs due to formation of thrombi and emboli from plaque disintegration. Obstruction in the more proximal vessels is easily recognized when palpation of the femoral artery reveals decreased or absent pulse.

Typically, Leriche syndrome is associated with a poorer prognosis as compared to more distal peripheral arterial diseases [3]. It is important then to have early recognition of the risk factors implicated in peripheral arterial disease for prompt initiation of non operative treatment. This measure will ensure general improvement of the patient's well-being.

Patient Information

Leriche syndrome is a disease that results from occlusion of peripheral arteries specifically at the point where the major blood vessel (aorta) divides to form its terminal branches, the common iliac arteries. This occlusion arises due to an obstruction of the artery by a plaque, a substance consisting of fats and clotted blood.

The symptoms and signs of this disease arise due to the reduced flow of blood into the regions beyond the occlusion (from the buttocks downwards to the legs). These features are typically:

  • Claudication: muscle cramping which begins at the calf muscles, and may extend up to the buttocks. It is triggered by a certain level of exercise and relieved by stopping the exercise.
  • Impotence due to an inability to sustain penile erection
  • Reduced or absent pulse in the groin area

A diagnosis is usually made when these features are present and after further clinical examination reveals deranged blood pressure and impaired circulation to the extremities as evidenced by cold, pale and weak limbs. Imaging tests are also used to confirm the diagnosis.

Leriche syndrome presents more commonly in males in the age bracket of 60 to 70 years. A number of factors predispose to the development of this condition. These include underlying high blood pressure, diabetes mellitus, high levels of blood cholesterol and smoking. Stopping these disorders from developing or progressing is important in the prevention of Leriche syndrome. It also demands for lifestyle modifications by engaging in regular exercise, maintaining a healthy weight and cessation of smoking.

For treatment of Leriche syndrome, certain medications (antiplatelets) are administered to prevent abnormal blood clot formation that would pose danger to the circulatory system. Drugs that help relieve muscle cramping (e.g. pentoxyfylline and cilostazol) are aslo prescribed. However, the mainstay of treatment in Leriche syndrome is surgery whereby the diseased part of the artery undergoes reconstruction or a bypass is created around it. Most of the surgical interventions are successful.

References

Article

  1. Jawor WJ, Plice SG. Thrombotic obliteration of the abdominal aorta; report of a case. Journal of the American Medical Association. 1952 May 10; 149 (2): 142–3.
  2. Leriche R, Morel A. The Syndrome of Thrombotic Obliteration of the Aortic Bifurcation. Annals of surgery. 1948 Feb; 127 (2): 193–206.
  3. Aboyans V, Desormais I, Lacroix P, Salazar J, Criqui MH, Laskar M. The general prognosis of patients with peripheral arterial disease differs according to the disease localization. J Am Coll Cardiol. 2010 Mar 2. 55(9):898-903.
  4. Takigawa M, Akutsu K, Kasai S et al. Angiographic documentation of aortoiliac occlusion in Leriche's syndrome. Can J Cardiol. 2008;24 (7): 568.
  5. Sebastià C, Quiroga S, Boyé R et-al. Aortic stenosis: spectrum of diseases depicted at multisection CT. Radiographics. 2003;23 Spec No (suppl 1): S79-91.
  6. Prager RJ, Akin JR, Akin GC, Binder RJ. Winslow's pathway: a rare collateral channel in infrarenal aortic occlusion. AJR Am J Roentgenol. 1977 Mar;128 (3): 485-7.
  7. Frederick M, Newman J, Kohlwes J. Leriche Syndrome. J Gen Intern Med. 2010;25(10):1102–1104.
  8. Indes JE, Pfaff MJ, Farrokhyar F, et al. Clinical outcomes of 5358 patients undergoing direct open bypass or endovascular treatment for aortoiliac occlusive disease: a systematic review and meta-analysis. J Endovasc Ther. 2013 Aug. 20 (4):443-55.
  9. Ruehm SG, Weishaupt D, Debatin JF. Contrast-enhanced MR angiography in patients with aortic occlusion (Leriche syndrome). J Magn Reson Imaging. 2000;11 (4): 401-10.
  10. Lee BY, Guerra J. Axillofemoral bypass graft in a spinal cord injured patient with impending gangrene. The Journal of the American Paraplegia Society. 1994;17 (4): 171–6.
  11. Lee W, Cheng Y, Lin H. Leriche syndrome. Int J Emerg Med. 2008;1(3):223.
  12. McKinsey JF. Extra-anatomic reconstruction. Surg. Clin. North Am. 1995; 75 (4): 731–40.

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Last updated: 2018-06-22 08:14