Leriche Syndrome


Article Author:
Kristen Brown


Article Editor:
Lorena Gonzalez


Editors In Chief:
Dustin Constant
Donald Kushner


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes


Updated:
2/18/2019 10:23:02 PM

Introduction

Leriche Syndrome (LS), also commonly referred to as aortoiliac occlusive disease (AIOD), is a product of atherosclerosis affecting the distal abdominal aorta, iliac arteries, and femoropopliteal vessels. [1] LS was first described in 1914 by Robert Grahman, but it was not until later that the trio of symptoms was documented as a syndrome. This was done by Henri Leriche, a French surgeon, and physiologist, now known as the father of LS. The extent and localization of atherosclerotic occlusions relative to these arteries determine the classification of the disease. AIOD is classified as Type I (confined to the distal abdominal aorta and common iliac arteries), Type II (predominately distal abdominal aorta with disease extension into common iliac and external iliac arteries), and Type III (affecting the aortoiliac segment and femoropopliteal vessels). [2] AIOD, when symptomatic, classically presents with a triad of claudication, impotence, and absence of femoral pulses. Claudication refers to cramping leg pain that is reproducible by exercise. This article will cover the etiology, epidemiology, pathophysiology, clinical presentation, evaluation, management options, differential diagnosis, prognosis, possible complications, and clinical pearls of LS in detail.

Etiology

Leriche Syndrome is caused by atherosclerosis. Modifiable risk factors for atherosclerosis include hypertension, diabetes mellitus, nicotine, hyperlipidemia, hyperglycemia, and homocysteine. Non-modifiable risk factors for atherosclerosis include age, gender, race, and family history. [3]

Epidemiology

Patients with peripheral arterial disease (PAD) may be asymptomatic (10%), so the exact prevalence and incidence of Leriche Syndrome are unknown. However, the prevalence of PAD increases in aging populations. The prevalence of PAD is 14% for patients older than 69 years of age. [4] Additionally, AIOD is more prevalent among males and the non-Hispanic black race.

Pathophysiology

Leriche Syndrome occurs secondary to atherosclerotic induced arterial wall injury leading to two out of three factors involved in Virchow triad, endothelial damage, and thrombosis. Once endothelial damage occurs, a resultant inflammatory response leads to lipid accumulation in smooth muscle cells and macrophages, which eventually leads to the formation of a plaque in the arterial lumen. Eventually, aortoiliac segments become stenotic by more than 50%, the resultant oxygen debt during exercise causes claudication. Once PAD becomes extensive in the aortoiliac segment, the majority of men with Leriche Syndrome present with impotence and sexual dysfunction secondary to reduced penile arterial flow. [3]

History and Physical

Patients commonly present with claudication, which is cramping in the lower extremities (hips, thighs, buttocks) reproducible by exercise. A detailed history is essential in determining the location, severity, and duration of symptoms. While impotence and sexual dysfunction may occur in the majority of patients, the hallmark of Leriche Syndrome is reduced or absent femoral pulses. [2] However, due to collateral vasculature, limb-threatening ischemia is not universal. [5]

Evaluation

A serum lipid profile (total cholesterol, LDL, HDL, TG) as well as HbA1c (if diabetic), lipoprotein A, and homocysteine levels should be obtained. [3] An ankle-brachial index (ABI) should also be performed to evaluate the perfusion of the lower extremities. An ABI is often the first screening test performed as it is non-invasive, inexpensive, and reliable. An ABI provides the ratio of the ankle systolic blood pressure divided by the brachial systolic blood pressure detected using a Doppler probe. An ABI lower than 0.9 is considered abnormal and indicates the patient has PAD significant enough to cause claudication. [6] Duplex ultrasonography and CTA are performed to determine the location and degree of stenosis while planning an intervention. [5] Coronary artery disease (CAD) is seen in 10% - 71% of patients with PAD, so obtaining an EKG as well to rule out CAD is recommended. [4]

Treatment / Management

Surgical treatment options for Leriche Syndrome include thromboendarterectomy (TEA), aortobifemoral bypass (AFB), and percutaneous transluminal angioplasty (PTA) with or without stenting. [7] [3] [8] AFB is preferred over TEA, mainly when intervention is necessary for arteries deep in the pelvis. Long-term patency of surgically repaired vessels in AFB have been recognized as superior to TEA and PTA with or without stenting in surgically fit patients. The long-term patency rates of AFB approach 85-90% at five years and 75-80% at ten years. [3] PTA and stenting is the intervention of choice in patients with multiple comorbidities, particularly those resulting in reduced lung function. Medical management includes smoking cessation, management of diabetes, antiplatelet and statin therapy, in addition to antihypertensive therapy. Implementation of a walking exercise program has been shown to improve walking ability by 50% - 200%. The exercise program should consist of daily 30-minute walking sessions. Intolerable claudication pain should be the endpoint for each session. [9] cessation and lowering glucose and lipid levels (LDL <100 mg/dL and HbA1c below 7%) are primary objectives of medical management. Cilostazol, a phosphodiesterase III inhibitor, may be administered to treat claudication symptoms of the patient. Cilostazol may also have benefit in graft patency and prevention of stenosis. [10] 

Differential Diagnosis

Numerous vascular conditions can mimic the symptoms of Leriche Syndrome and must be considered in the differential diagnosis. Arterial dissection, particularly in the iliac arteries may cause claudication and absent femoral pulses mimicking the symptom profile of Leriche Syndrome. [11] The presence of rest pain and a definitive time course of symptom onset would be useful in delineating the underlying pathology in conjunction with ultrasound and arteriographic imaging. Patients who have recently undergone instrumentation and graft placement are at increased risk for dissection and acute occlusion. Critical limb ischemia is not often seen in Leriche Syndrome, as its time course allows for the development of collateral vasculature. Signs of critical limb ischemia include sudden onset of pain in the affected extremity, paresthesias, paleness or coolness, and severely diminished or absent pulses. [12] Occlusion may result from thrombosis, emboli from a more proximal vascular region, or dissection. 

Prognosis

Without treatment, the prognosis of Leriche Syndrome is poor. However, with modern medicine outcomes are good. In some cases with slow progression or onset of LS, collaterals may develop as a self-compensating mechanism. [13]

Complications

Limb ischemia [14] is a potential complication of LS as well as heart failure, myocardial ischemia/infarction, gangrene, and even death. [15]  [16] [17]

Consultations

A quick consultation of either vascular surgery or interventional cardiology should be placed if MTS is suspected.

Deterrence and Patient Education

All patients should be encouraged to prevent/treat all modifiable risk factors such as hypertension, diabetes mellitus, nicotine, hyperlipidemia, hyperglycemia, and homocysteine. 

Pearls and Other Issues

With the complexity of LS is it highly recommended a purposeful interprofessional team be involved when LS is suspected. [18] Team members should include the patient's primary care physician, a cardiologist, an imaging specialist, a vascular surgeon, as well as an interventional cardiologist. The advantages of a team approach are to appropriately manage LS and improve patient satisfaction as well as the success of the intervention.

Enhancing Healthcare Team Outcomes

Multiple studies have been done investigating the benefit of utilizing a multidisciplinary team to enhance patient satisfaction as well as healthcare outcomes; the results are stunning. In lite of this new evidence, it now recommended that all institutions involved in percutaneous interventions or cardiothoracic interventions should set in place algorithms and encourage interdisciplinary communication. [18] Many institutions have complied with this recommendation by engaging in multidisciplinary conferences to discuss complex cases. In the case of LS, an interventional cardiologist, as well as a cardiothoracic surgeon, should be involved to ensure the best management option is undergone in a case by case manner.


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Leriche Syndrome - Questions

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An angiogram shows high-grade stenosis of the aorta at the aorto-iliac bifurcation. Which of the following is the most consistent symptom with the findings on the angiogram?



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A 68-year-old with severe COPD who requires home oxygen is unable to ambulate inside her home without experiencing severe hip pain. She was hospitalized 1 year ago for pneumonia and required mechanical ventilation for 3 weeks. An angiogram done for absent leg pulses reveals severe aorto-iliac disease with aortic occlusion up to the renal. Which of the following is the most appropriate vascular procedure for this patient?



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What diagnosis shows intermittent claudication, absent femoral pulses, and impotence?



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Which diagnosis is consistent with the presentation of impotence and coldness of the lower extremities with absence of femoral pulses?



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A 72-year-old male presents with complaints of erectile dysfunction. He has been married for over 40 years and has a normal sex drive but cannot achieve an erection. He does not remember when he last had a nocturnal erection. His past medical history is significant for CAD and HTN. He takes aspirin, metoprolol, and a statin. He is a current smoker and has a 48 pack-year smoking history. Review of symptoms is positive for pain in the bilateral lower extremities when walking long distances. His physical exam is significant for hairless, dry, shiny skin and barely palpable pedal pulses bilaterally. Examination of the genitalia and perineum is normal. Which of the following is the next best step in work up of his erectile dysfunction?



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Leriche Syndrome - References

References

Pascarella L,Aboul Hosn M, Minimally Invasive Management of Severe Aortoiliac Occlusive Disease. Journal of laparoendoscopic     [PubMed]
Wooten C,Hayat M,du Plessis M,Cesmebasi A,Koesterer M,Daly KP,Matusz P,Tubbs RS,Loukas M, Anatomical significance in aortoiliac occlusive disease. Clinical anatomy (New York, N.Y.). 2014 Nov;     [PubMed]
Frederick M,Newman J,Kohlwes J, Leriche syndrome. Journal of general internal medicine. 2010 Oct;     [PubMed]
Diehm C,Schuster A,Allenberg JR,Darius H,Haberl R,Lange S,Pittrow D,von Stritzky B,Tepohl G,Trampisch HJ, High prevalence of peripheral arterial disease and co-morbidity in 6880 primary care patients: cross-sectional study. Atherosclerosis. 2004 Jan;     [PubMed]
Ahmed S,Raman SP,Fishman EK, CT angiography and 3D imaging in aortoiliac occlusive disease: collateral pathways in Leriche syndrome. Abdominal radiology (New York). 2017 Sep;     [PubMed]
de Groote P,Millaire A,Deklunder G,Marache P,Decoulx E,Ducloux G, Comparative diagnostic value of ankle-to-brachial index and transcutaneous oxygen tension at rest and after exercise in patients with intermittent claudication. Angiology. 1995 Feb;     [PubMed]
Metcalfe MJ,Natarajan R,Selvakumar S, Use of extraperitoneal iliac artery endarterectomy in the endovascular era. Vascular. 2008 Nov-Dec;     [PubMed]
Krankenberg H,Schlüter M,Schwencke C,Walter D,Pascotto A,Sandstede J,Tübler T, Endovascular reconstruction of the aortic bifurcation in patients with Leriche syndrome. Clinical research in cardiology : official journal of the German Cardiac Society. 2009 Oct;     [PubMed]
Fakhry F,Spronk S,van der Laan L,Wever JJ,Teijink JA,Hoffmann WH,Smits TM,van Brussel JP,Stultiens GN,Derom A,den Hoed PT,Ho GH,van Dijk LC,Verhofstad N,Orsini M,van Petersen A,Woltman K,Hulst I,van Sambeek MR,Rizopoulos D,Rouwet EV,Hunink MG, Endovascular Revascularization and Supervised Exercise for Peripheral Artery Disease and Intermittent Claudication: A Randomized Clinical Trial. JAMA. 2015 Nov 10;     [PubMed]
Tara S,Kurobe H,de Dios Ruiz Rosado J,Best CA,Shoji T,Mahler N,Yi T,Lee YU,Sugiura T,Hibino N,Partida-Sanchez S,Breuer CK,Shinoka T, Cilostazol, Not Aspirin, Prevents Stenosis of Bioresorbable Vascular Grafts in a Venous Model. Arteriosclerosis, thrombosis, and vascular biology. 2015 Sep;     [PubMed]
Lequesne M,Zaoui A, [Misleading     [PubMed]
Yoon DH,Cho H,Seol SJ,Kim T, Right calf claudication revealing leriche syndrome presenting as right sciatic neuropathy. Annals of rehabilitation medicine. 2014 Feb;     [PubMed]
Morotti A,Busso M,Cinardo P,Bonomo K,Angelino V,Cardinale L,Veltri A,Guerrasio A, When collateral vessels matter: asymptomatic Leriche syndrome. Clinical case reports. 2015 Nov;     [PubMed]
Pillai J,Monareng T,Rangaka TB,Yazicioglu C,Jayakrishnan R,Veller MG, Aorto-internal iliac artery endovascular reconstruction for critical limb ischaemia: a case report. South African journal of surgery. Suid-Afrikaanse tydskrif vir chirurgie. 2015 Oct 8;     [PubMed]
JOHNSON JK, Ascending thrombosis of abdominal aorta as fatal complication of Leriche's syndrome. A.M.A. archives of surgery. 1954 Nov;     [PubMed]
Kashou AH,Braiteh N,Zgheib A,Kashou HE, Acute aortoiliac occlusive disease during percutaneous transluminal angioplasty in the setting of ST-elevation myocardial infarction: a case report. Journal of medical case reports. 2018 Jan 11;     [PubMed]
Keller K,Beule J,Oliver Balzer J,Coldewey M,Munzel T,Dippold W,Wild P, A 56-year-old man with co-prevalence of Leriche syndrome and dilated cardiomyopathy: case report and review. Wiener klinische Wochenschrift. 2014 Mar;     [PubMed]
Rubimbura V,Rostain L,Duval AM,Akakpo S,Boukantar M,Boiron P,Mouillet G,Gallet R,Belarbi A,Le Corvoisier P,Dubois-Randé JL,Teiger E, Outcomes and safety of same-day discharge after percutaneous coronary intervention: A 10-year single-center study. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography     [PubMed]

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