Leriche Syndrome

An 86-year-old female with chronic back pain and lower extremity pain. Diagnosis? Eponym?• Radiology Case of the Week

Figure 1. An 86-year-old female with chronic back pain and lower extremity pain. Diagnosis? Eponym?

Figure 2. Sagittal (A) and coronal (B) CT angiography reformats. Patent proximal abdominal aorta (yellow arrows) and the occluded distal aorta (red arrows). Extensive calcification of the plaque indicates that the occlusion is chronic. Note the dilated inferior mesenteric artery with reverse flow (green arrows). Diagnosis: Chronic Leriche syndrome.

Figure 3. Axial CT angiography. A. Complete occlusion of the abdominal aorta below the level of the renal arteries (red arrow). B. Distal abdominal aorta is patent (yellow arrow) due to extensive collateral flow from superior mesenteric artery branches and reversed flow in a markedly dilated inferior mesenteric artery (green arrow).

Figure 4.

Doppler ultrasound of the common femoral arteries. A. Right (RT CFA). B. Left (LT CFA). Note the monophasic waveforms bilaterally indicating aortoiliac atherosclerotic stenotic disease or occlusion.

Epidemiology

The exact prevalence and incidence of Leriche syndrome are unknown. Leriche and Morel originally described it in young men, but older patients and women have since been reported [1,2]. It results from chronic aortoiliac atherosclerotic disease, so risk factors include age, smoking, hypertension, diabetes, and hyperlipidemia [3].

Clinical Findings

The classic triad is claudication, impotence in men, and absent or diminished femoral pulses [3]. Claudication is cramping pain in the buttocks, hips, and thighs that is reproduced by exercise. Reduced or absent femoral pulses are the hallmark finding. Limb-threatening ischemia is not universal, because collateral circulation develops as the disease progresses slowly [3]. An ankle-brachial index below 0.9 is abnormal and is usually the first test.

Pathology Leriche syndrome is caused by atherosclerotic plaque within the lumen. Stenosis greater than 50% produces claudication, because oxygen demand during exercise exceeds supply. The plaque can progress to complete occlusion of the distal aorta, and extensive calcification indicates a chronic process.

Classification Leriche syndrome is classified by the extent of disease [3]. Type I is confined to the distal abdominal aorta and common iliac arteries. Type II involves mainly the distal abdominal aorta and extends into the common and external iliac arteries. Type III involves the aortoiliac segment and the femoropopliteal vessels.

Radiographic Features

Duplex ultrasound and CT angiography define the location and degree of disease. Duplex ultrasound is noninvasive and is typically the first imaging test, often after the ankle-brachial index. CT angiography is the imaging modality of choice: it confirms the occlusion, shows plaque calcification, and maps the collateral pathways before treatment [4,5]. MR angiography is an alternative when iodinated contrast is a concern. In this 86-year-old woman with chronic back and lower extremity pain, ultrasound and CT angiography together showed a chronic aortic occlusion with the distal aorta reconstituted by collateral flow (Figs. 1-4).

Treatment and Prognosis

Management combines risk-factor control with revascularization when needed. Therapy includes supervised walking, smoking cessation, diabetes control, antiplatelet, statin and antihypertensive therapy. Revascularization options are aortobifemoral bypass, thromboendarterectomy, and angioplasty with or without stenting [6]. In fit patients, aortobifemoral bypass has the best long-term patency, about 85% to 90% at five years; endovascular treatment suits patients with multiple comorbidities. Prognosis is poor without treatment but good with modern therapy. Complications include limb ischemia, gangrene, myocardial infarction, and death.

Conclusion

Leriche syndrome is chronic atherosclerotic occlusion of the distal aorta and may present with nonspecific back and leg pain. Monophasic lower extremity waveforms on Doppler ultrasound should prompt CT angiography, which confirms the occlusion and its collateral pathways and guides treatment.

References: 

1. Leriche R, Morel A. The Syndrome of Thrombotic Obliteration of the Aortic Bifurcation. Ann Surg. 1948;127(2):193-206. DOI: 10.1097/00000658-194802000-00001

2. Kita K. Leriche syndrome (Aortoiliac occlusive disease). J Gen Fam Med. 2017;18(5):297-298. Published 2017 Apr 21. DOI: 10.1002/jgf2.63

3. Brown KN, Gonzalez L. Leriche Syndrome (Archived). In: StatPearls. Treasure Island (FL): StatPearls Publishing; February 13, 2023.  https://pubmed.ncbi.nlm.nih.gov/30855836/

4. Frederick M, Newman J, Kohlwes J. Leriche syndrome. J Gen Intern Med. 2010;25(10):1102-1104. DOI: 10.1007/s11606-010-1412-z

5. Ahmed S, Raman SP, Fishman EK. CT angiography and 3D imaging in aortoiliac occlusive disease: collateral pathways in Leriche syndrome. Abdom Radiol (NY). 2017;42(9):2346-2357. DOI: 10.1007/s00261-017-1137-0

6. Krankenberg H, Schlüter M, Schwencke C, et al. Endovascular reconstruction of the aortic bifurcation in patients with Leriche syndrome. Clin Res Cardiol. 2009;98(10):657 664. DOI: 10.1007/s00392-009-0052-y

Umaiza Ihsan, MD is a medical graduate of King Edward Medical University, Pakistan. She completed her internship at Mayo Hospital, Lahore, including a three month rotation in Radiology, which further strengthened her interest in diagnostic imaging. She has gained additional clinical exposure in Radiology through observerships at Mon Health Morgantown and the University of Maryland, Baltimore. She has a strong interest in Radiology, particularly diagnostic imaging and its role in clinical decision making, and is actively interested in clinical research, with experience in systematic reviews and medical research. All posts by Umaiza Ihsan, MD

Kevin M. Rice, MD is the president of Global Radiology CME and is a radiologist with Cape Radiology Group. He has held several leadership positions including Board Member and Chief of Staff at Valley Presbyterian Hospital in Los Angeles, California. Dr. Rice has made several media appearances as part of his ongoing commitment to public education. Dr. Rice's passion for state of the art radiology and teaching includes acting as a guest lecturer at UCLA. In 2015, Dr. Rice and Natalie Rice founded Global Radiology CME to provide innovative radiology education at exciting international destinations, with the world's foremost authorities in their field. In 2016, Dr. Rice was nominated and became a semifinalist for a "Minnie" Award for the Most Effective Radiology Educator. He was once again a semifinalist for a "Minnie" for 2021's Most Effective Radiology Educator by AuntMinnie.com. He has continued to teach by mentoring medical students interested in radiology. Everyone who he has mentored has been accepted into top programs across the country including Harvard, UC San Diego, Northwestern, Vanderbilt, and Thomas Jefferson.

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All posts by Kevin M. Rice, MD