What is Leriche Syndrome?
Leriche Syndrome (LS) is also known as aortoiliac occlusive disease (AIOD). It’s a condition caused by a disease called atherosclerosis, which clogs the arteries. This syndrome primarily affects the lower part of the abdominal aorta (which is the largest artery in your body), the iliac arteries (two large arteries that supply blood to your legs and lower body), and the femoropopliteal vessels (arteries that supply blood from your thigh to your knee).
This condition was first mentioned in 1914 by Robert Grahman, but it was Henri Leriche, a French doctor, known as the father of LS, who identified a set of three symptoms that define this syndrome. How LS is classified depends on the location and scope of the artery blockages. It can be classified into three types: Type I affects the lower part of the abdominal aorta and a pair of arteries called common iliac arteries. Type II predominantly involves the lower abdominal aorta with the condition spreading into the common iliac and external iliac arteries (that provide lower body and legs with blood), and Type III impacts both the aortoiliac segment (part of the arteries found near the lower end of the aorta) and femoropopliteal vessels.
When LS causes symptoms, patients typically experience a trio of issues: claudication, impotence, and a lack of pulse in the femoral artery (the main artery in the thigh). Claudication means that you experience a cramp-like leg pain that reappears when you exercise. This guide will help explain the causes, prevalence, functioning, symptoms, assessment, treatment options, differentiating it from other diseases, prognosis, potential complications, and important facts about LS.
What Causes Leriche Syndrome?
Leriche Syndrome is a condition that occurs due to a disease called atherosclerosis. Factors that you can change to lower your risk of developing atherosclerosis are high blood pressure, diabetes, smoking, high cholesterol levels, high blood sugar levels, and a chemical in the blood called homocysteine. On the other hand, factors that you can’t change include your age, gender, race, and family health history.
Risk Factors and Frequency for Leriche Syndrome
Peripheral arterial disease (PAD), which includes Leriche Syndrome, often doesn’t show symptoms in 10% of patients, making it difficult to determine its exact commonness. However, PAD tends to appear more frequently in older populations. For instance, 14% of individuals older than 69 years have PAD. It’s also worth noting that this condition is more common in men and non-Hispanic black individuals.
- Peripheral arterial disease (PAD), includes Leriche Syndrome, is often symptomless in 10% of patients. This makes its exact prevalence hard to determine.
- The frequency of PAD rises with age, with 14% of people older than 69 being affected.
- This condition is more commonly seen in men and non-Hispanic black individuals.
Signs and Symptoms of Leriche Syndrome
People with Leriche Syndrome often experience cramping in their lower body areas like the hips, thighs, or buttocks, especially during exercise. This is known as claudication. It’s important for these individuals to provide a thorough medical history to help pinpoint the exact location, severity, and how long they’ve been having these symptoms. Even though impotence and sexual dysfunction could happen in many patients, the key sign of Leriche Syndrome actually is weak or even non-existent pulses in the femoral artery (the primary artery in the thigh). But not everyone with this syndrome will face life-threatening circulation issues, thanks to additional or collateral blood vessels that can aid in blood flow.
Testing for Leriche Syndrome
To check for levels of cholesterol, bad (LDL) and good (HDL) cholesterol, and triglycerides in your blood – these fats can be a sign of heart disease, your doctor might order what’s called a serum lipid profile. If you have diabetes, your doctor should also check your HbA1c level, which gives an idea of your average blood sugar level over the past 3 months. Other tests that might be ordered include lipoprotein A, which can also be a risk factor for heart disease, and homocysteine, a type of protein that can damage your arteries.
Your doctor may also want to check how well blood is flowing to your legs using a test called ankle-brachial index (ABI). This is a simple, non-invasive test that measures the blood pressure at your ankle and compares it to the blood pressure in your arm, using a special kind of stethoscope called a Doppler probe. If your ABI score is lower than 0.9, that suggests that you might have peripheral artery disease (PAD) and the blood flow to your legs is less than it should be. This can cause leg pain when you walk, a symptom known as claudication.
If your doctor suspects you have PAD, they might also order imaging tests like duplex ultrasonography, which uses sound waves to create pictures of your arteries, or CT angiography (CTA) to get a clearer image of the arteries in your legs. These imaging tests can help pinpoint exactly where any blockages are in your arteries and how severe they might be, which can help in planning any treatments you might need.
It’s worth noting that many people with PAD also have coronary artery disease (CAD), which affects the arteries in your heart and can lead to heart attacks. So your doctor might also want to do an EKG, a simple test that checks the electrical activity of your heart, to see if you might have CAD as well.
Treatment Options for Leriche Syndrome
Leriche Syndrome is a condition affecting the large arteries in the body. There are several surgery options available for treating this condition. These include procedures such as Thromboendarterectomy, Aortobifemoral Bypass, and Percutaneous Transluminal Angioplasty, which may be performed with or without the insertion of a stent.
Aortobifemoral Bypass is often the preferred method, especially when the affected arteries are located deep within the pelvis. Studies have shown that this procedure offers better long-term results, with 85-90% of patients seeing improvements after five years, and 75-80% after ten years.
However, if a patient has multiple health issues—particularly those affecting their lung function—then Percutaneous Transluminal Angioplasty, with or without a stent, usually becomes the procedure of choice.
Treating Leriche syndrome also involves addressing lifestyle factors. It’s important for patients to quit smoking, manage any diagnosed diabetes, and engage in a daily exercise program—specifically, 30-minute walking exercises can help increase a patient’s ability to walk by 50% to 200%. The walking session should be continued until the pain becomes unbearable in each session.
Other important targets for managing this condition include maintaining control on blood sugar and cholesterol levels (LDL should be less than 100 mg/dL and HbA1c below 7%). As a part of treatment, medications like Cilostazol, a type of drug that helps open up the vessels and allows better blood flow, might be offered to manage symptoms. It may also help to maintain the open status of the graft and prevent any narrowing.
What else can Leriche Syndrome be?
Many blood vessel conditions can show symptoms similar to Leriche Syndrome. Therefore, these need to be taken into account when diagnosing. The list of these conditions includes, but is not limited to:
- Arterial dissection, especially in the iliac arteries, which can cause symptoms like leg pain and undetectable femoral pulses, similar to Leriche Syndrome
- Patients who have recently had medical procedures or graft placement, as these increase the risk for dissection and sudden blockage of blood vessels
- Critical limb ischemia which is rarely seen in Leriche Syndrome because its development over time allows for new blood vessels to form. Symptoms of critical limb ischemia can range from sudden pain in the affected limb, feelings of ‘pins and needles’, a pale or cold appearance, and hardly detectable or missing pulses
Blockages can occur due to blood clots, particles that have moved from another part of the body’s vascular system, or dissection. Symptoms of rest pain and a specific timeline of symptom onset can help differentiate the real medical issue. Ultrasound and specialized X-ray imaging of the blood vessels can also assist with this.
What to expect with Leriche Syndrome
If Leriche Syndrome isn’t treated, the outlook is usually quite grim. But with our modern medicines and treatments, the results are often positive. In some cases, where Leriche Syndrome progresses slowly or starts gradually, the body may develop collateral vessels as a natural way to compensate for the blocked arteries.
Possible Complications When Diagnosed with Leriche Syndrome
Ischemia, or inadequate blood supply to the limbs, is one possible complication that can happen with LS, in addition to heart failure. Other potential complications could be myocardial ischemia/infarction, which refers to heart muscle damage due to restricted blood flow, gangrene, or tissue death caused by lack of blood, and in severe cases, even death.
Possible Complications:
- Ischemia in the limbs
- Heart failure
- Damage to the heart muscle due to restricted blood flow (myocardial ischemia/infarction)
- Gangrene or tissue death caused by lack of blood
- Possible death in severe cases
Preventing Leriche Syndrome
All patients should be advised to manage and control risk factors that can be changed, such as high blood pressure (hypertension), diabetes (high blood sugar), smoking (nicotine intake), high levels of fats in the blood (hyperlipidemia), high levels of glucose in the blood (hyperglycemia), and high levels of a substance called homocysteine. Managing these factors can help reduce health risks.