Leriche Syndrome

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Leriche Syndrome
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Overview

Leriche syndrome, also known as aortoiliac occlusive disease, is a form of central artery disease characterized by thrombotic blockage of the abdominal aorta by atherosclerotic plaques as it transitions into the common iliac arteries. Leriche syndrome was first described by Robert Graham in 1914, but the condition with its triad of symptoms was ascribed to Rene Leriche, a French surgeon.

Causes:

Atherosclerosis is the most common cause of Leriche syndrome. Several risk factors include cigarette smoking, hypercholesterolemia, obesity, diabetes mellitus, alcoholism, and coagulation disorders. An uncommon cause of Leriche syndrome is Takayasu disease, a nonspecific arteritis that may cause obstruction of the abdominal aorta and its branches.

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). In the patients, any number of symptoms may present, depending on the disease extent, distribution and severity of the disease, such as muscle atrophy, slow wound healing or non-healing of wounds in the legs, and critical limb ischemia, manifesting as limb pain, bluish discoloration, paresthesia and edema. Fatigue of the lower limbs, pallor or coldness of both lower extremities may be observed.

Diagnosis:

Diagnosis requires patient's medical history, clinical examination and extensive work up involving serum lipid profile (total cholesterol, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, and triglycerides), homocysteine levels, blood sugar, glycosylated hemoglobin level (HbA1c), prothrombin time (PT), activated partial thromboplastin time (aPTT), platelet count, factor V Leiden, anticardiolipin antibody, factor II (prothrombin) C-20210a, protein C, protein S, and antithrombin III. Imaging studies include Computed tomography (CT) angiography, magnetic resonance angiography (MRA) or arterial duplex mapping. Doppler-derived segmental arterial pressures do not accurately reflect the severity of Leriche syndrome.

Management:

Treatment of Leriche syndrome involves revascularization typically either by angioplasty or a type of vascular bypass. Kissing balloon angioplasty with or without stent may be used. Aorto-iliac bypass graft, axillary-bifemoral bypass and femoral-femoral bypass may also be used. The 30-day operative mortality is 2-3%. Laparoscopically assisted aortofemoral bypasses (AFBs) have been performed with satisfactory results. Management protocols include complete cessation of smoking, careful regulation of serum glucose, hypertension control, control of low-density lipoprotein (LDL), total cholesterol and triglycerides with hepatic 3-methylglutaryl coenzyme A (HMG CoA) reductase inhibitors (statins). Pentoxifylline and cilostazol may alleviate the symptoms of claudication caused by Leriche syndrome. Pentoxifylline is a hemorheologic agent, which lowers blood viscosity, and is known to be effective in only 30-40% of patients and must be taken three times daily.