BASICS
DESCRIPTION
Leriche syndrome, also known as aortoiliac occlusive disease, is caused by atherosclerotic occlusive disease of the abdominal aorta and common iliac arteries, with symptoms of proximal claudication. The triad of symptoms commonly seen is erectile dysfunction in males, diminished pulses, and claudication of thighs (1).
EPIDEMIOLOGY
Incidence
Unknown; however, peripheral artery disease (PAD) increases with advancing age.
Prevalence
- Prevalence is unknown: 5 " 10% of patients with PAD
- Unique triad of symptoms (erectile dysfunction in males, diminished pulses, and claudication of thighs) appears in younger patients between 40 and 60 years, which are younger than for general PAD patients (1).
- Appears to be more common in males than females
ETIOLOGY AND PATHOPHYSIOLOGY
- Most common etiology is occlusive plaques in aortoiliac arteries. Cigarette smoking and hypercholesteremia are the two risk factors seen mostly in patients with occlusive disease.
- Less likely to be associated with diabetes than typical patterns of PAD
- Main factors contributing to occlusive disease include arterial wall damage by hypertension, nicotine, lipid accumulation leading to plaque formation, and development of atheroma.
- A rare cause is Takayasu disease, which is a nonspecific arteritis that can lead to occlusive disease of the aorta.
Genetics
Family history of hypercholesteremia and PAD
RISK FACTORS
- Tobacco use
- Male gender (1)
- Hypertension
- Hypercholesterolemia
- Family history of PAD
GENERAL PREVENTION
- Low-fat diet
- Exercise
- Low cholesterol
- Low blood pressure
- No cigarette smoking
COMMONLY ASSOCIATED CONDITIONS
- Hypertension
- Coronary artery disease
- Myocardial infarction
- Stroke
- PAD
DIAGNOSIS
HISTORY
- Male: erectile dysfunction or unable to complete intercourse or ejaculation dysfunction (1)[A]
- Proximal claudication symptoms: pain in thigh and buttock with walking and relieved by rest
- Weakness in lower extremities (1)[A]
- Associated with:
- Cigarette smoking
- Uncontrolled high blood pressure
PHYSICAL EXAM
- Distal signs: decreased capillary refill, cold feet, poor wound healing
- Decreased femoral and peripheral pulses
- No trophic changes (unlike distal PAD) (2)[A]
- Pallor of skin
DIFFERENTIAL DIAGNOSIS
- Peripheral neuropathy
- Compartment syndrome
- Cauda equina syndrome
- Spinal stenosis
- Venous stasis
- Deep vein thrombosis (rare bilaterally)
DIAGNOSTIC TESTS & INTERPRETATION
Initial Tests (lab, imaging)
- Ankle-brachial index (ABI) testing (3)
- ABI <0.3: critical ischemia
- <0.5: severe ischemia
- 0.50 to 0.80: moderate PAD
- 0.81 to 1.00: no significant disease or mild disease
- >1.00: normal
- >1.4: calcified vessel
- CT angiogram
Follow-Up Tests & Special Considerations
- MR angiogram
- Treadmill test: reproduction of claudication symptoms
- Lipid panel
- CBC/BMP
- Consider stress test to rule out coexistent coronary artery disease.
TREATMENT
GENERAL MEASURES
- Smoking cessation is mandatory.
- Surgical repair: aortofemoral bypass grafting or aortoiliac endarterectomy (4)[B]
- Surgical repair, especially aortofemoral bypass, has significant morbidity and mortality; however, it shows good long-term results in juxtarenal aortic occlusion (5).
- Endovascular surgery: angioplasty, stents, plaque debulking (1)[B]
- Endovascular repair has less morbidity/mortality; however, primary patency rates are inferior to that of surgery (1).
- Endovascular surgery should be considered in elderly patients and those patients with significant comorbidities (1).
- Currently, there are no published randomized controlled trials directly comparing surgical versus endovascular treatment (1).
MEDICATION
First Line
- Lifelong antiplatelet therapy (5)[A]
- Aspirin for all patients (75 to 325 mg/day)
- If aspirin is not tolerated, clopidogrel (75 mg/day) or ticlopidine (250 mg BID)
- Antihypertensives
- Statin therapy " based on 2013 ACC/AHA guidelines, high-intensity therapy should be offered (6)[A].
Second Line
No reports of efficacy of these agents in patients with Leriche syndrome:
- Pentoxifylline (Trental): conflicting efficacy to improving claudication symptoms when walking in PAD patients (5)[C]
- Cilostazol (Pletal): decreases some claudication symptoms in PAD patients (5)[C]
ISSUES FOR REFERRAL
- Prompt consultation with vascular surgery
- Consider cardiology referral for patients with symptoms of coronary artery disease.
ADDITIONAL THERAPIES
- Eliminating risk factors: smoking cessation, low-fat/low-cholesterol diet; statins
- Exercise regimen is essential.
INPATIENT CONSIDERATIONS
Admission Criteria/Initial Stabilization
- Signs of ischemia in lower extremities
- Renal insufficiency secondary to juxtarenal occlusions
- Severe pain unrelieved with rest
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
- Postoperative (7)
- Monitor for surgical site hematoma (bleeding is highest risk [4 " 17%]).
- Monitor for nephrotoxicity from contrast (increase in serum creatinine of at least 0.5 mg/dL within 48 hours of contrast).
- Cholesterol embolization: Look for livedo reticularis (lace-like purple mottling of skin in vascular pattern) of the skin.
- Close monitoring of blood pressure, cholesterol
DIET
Low-fat/low-cholesterol diet
PATIENT EDUCATION
- Stress the importance of exercise, healthy diet, and management of blood glucose and blood pressure.
- Smoking cessation
COMPLICATIONS
- Severe tissue loss, gangrene leading to amputation
- Acute renal failure secondary to decreased perfusion (7)
REFERENCES
11 Setacci C, Galzerano G, Setacci F, et al. Endovascular approach to Leriche syndrome. J Cardiovasc Surg (Torino). 2012;53(3):301 " 306.22 Leriche R, Morel A. The syndrome of thrombotic obliteration of the aortic bifurcation. Ann Surg. 1948;127(2):193 " 206.33 Al-Qaisi M, Nott D, King D, et al. Ankle brachial pressure index (ABPI): an update for practitioners. Vasc Health Risk Manag. 2009;5:833 " 841.44 Marrocco-Trischitta MM, Bertoglio L, Tshomba Y, et al. The best treatment of juxtarenal aortic occlusion is and will be open surgery. J Cardiovasc Surg (Torino). 2012;53(3):307 " 312.55 Sobel M, Verhaeghe R. Antithrombotic therapy for peripheral artery occlusive disease: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest. 2008;133(6)(Suppl):815S " 843S.66 Stone NJ, Robinson J, Lichtenstein AH, et al. 2013 ACC/AHA guideline on the treatment of blood cholesterol to reduce atherosclerotic risk in adults: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2013:63(25, Pt B):2889 " 2934.77 Fourneau I. How to avoid and manage complications in aorto-iliac interventions. J Cardiovasc Surg (Torino). 2012;53(3):325 " 331.
ADDITIONAL READING
- Arnold JR, Greenberg J, Reddy K, et al. Internal mammary artery perfusing Leriche 's syndrome in association with significant coronary arteriosclerosis: four case reports and review of literature. Catheter Cardiovasc Interv. 2000;49(4):441 " 444.
- Pittler MH, Ernst E. Ginkgo biloba extract for the treatment of intermittent claudication: a meta-analysis of randomized trials. Am J Med. 2000;108(4):276 " 281.
- Varcoe RL. Re-entry device use in the endovascular treatment of aorto-iliac occlusive disease. J Cardiovasc Surg (Torino). 2012;53(3):313 " 323.
CODES
ICD10
I74.09 Other arterial embolism and thrombosis of abdominal aorta
ICD9
444.09 Other arterial embolism and thrombosis of abdominal aorta
SNOMED
Leriche 's syndrome (disorder)
CLINICAL PEARLS
- Reducing risk factors such as cigarette smoking, hypertension, and hypercholesterolemia is key to prevention.
- Mainstay treatment involves either vascular surgery or endovascular surgery. No randomized comparative trial to access long-term outcomes
- High morbidity and mortality associated with surgery