A 58-year-old man with a past medical history of hypertension, hyperlipidemia, and poorly controlled diabetes mellitus presents to the cardiology clinic complaining of sexual dysfunction. Patient is a 50 pack-year smoker but denies alcohol and illicit drug use. His family history is significant for a mother with multiple sclerosis and hyperlipidemia. He states he has not been able to produce an erection for a few months and prior to that, his erections were insufficiently rigid. He was seen by his primary care physician 6 months ago complaining of reduced exercise capacity due to pain in his thighs and buttocks. At that time, he was taken off his atorvastatin with little improvement in his symptoms. The patient has no symptoms at rest, but in the office, his pain is reproduced after walking 50 yards on a treadmill and unchanged when increasing the incline. Recent laboratory work demonstrates an elevated creatinine of 1.7 mg/dL (baseline ~1.0), glycohemoglobin 10.7%, hemoglobin 10.2 g/dL, and creatinine kinase 45 U/L. On physical exam, femoral and peripheral pulses are difficult to palpate and did not change during proactive maneuvers, such as dorsiflexion and plantar flexion. Lower extremity reflexes are intact, muscle strength and tone are normal, and straight leg raise is negative bilaterally. Patient endorses mildly diminished sensation to light touch on bottom of his feet. Right and left ankle-brachial index (ABI) values at rest are 0.59 on the right and 0.63 on the left, and with exertion are 0.50 and 0.51, respectively. Pulse volume recording showed a blunted waveform amplitude, and loss of dicrotic notch bilaterally at high thigh, low thigh, calf, and ankle.
The correct answer is: C. Leriche syndrome (aortoiliac occlusive disease)
Leriche syndrome, also known as aortoiliac occlusive disease, is a type of peripheral arterial disease where there is significant atherosclerosis occluding the distal aorta and extending into the common iliac and femoropopliteal circulation.1 Leriche syndrome also typically affects the internal iliac and pudendal artery. This patient's symptoms describe the classic triad found in Leriche syndrome: claudication in the thigh/buttocks (external and internal iliac arteries respectively), erectile dysfunction (internal pudendal artery off the internal iliac), and diminished femoral pulses. Risk factors for Leriche syndrome are similar to peripheral arterial diseases including: hyperlipidemia, hypertension, diabetes mellitus, and smoking.2
On physical exam, patients will have the normal stigmata of peripheral vascular disease including cool, dry, shiny, and hairless skin. A unique differentiator are blue toes on exam which are due to distal embolization of atherosclerotic disease. Patients may still have 1+ peripheral pulses despite complete upstream occlusion due to extensive collateralization.2
Initial screen test for Leriche syndrome include doppler ultrasound, pulse volume recordings, and ankle-brachial-indices.2 Definitive diagnosis can be made with computed tomography angiography, magnetic resonance angiography, and lower-extremity angiogram.3 Computed tomography angiography in a patient with Leriche syndrome demonstrates an infrarenal aortic occlusion with reconstitution of the iliac arteries from collaterals. For symptomatic patients, Leriche syndrome is treated with revascularization, which can be in the form of endovascular therapy, endarterectomy, or bypass surgery.1
(Answer A) A statin-induced myopathy can be considered in this patient due to the concern for rhabdomyolysis. He has an elevated creatinine; however, his creatine kinase was within normal limits. Additionally, a statin-induced myopathy would not cause erectile dysfunction. It is likely that the myalgias he had during his last primary care physician appointment were actually claudication symptoms from Leriche syndrome given there was no improvement after stopping his statin.
(Answer B) Popliteal artery entrapment is a progressive disease from repetitive mechanical compression of the artery that can cause intraluminal stenosis, post-stenotic dilatation and aneurysm formation. This may lead to thrombus formation, embolization, and even ischemia of the lower extremity. Physical examination should include palpitation of pulses at the dorsalis pedis and posterior tibial with the ankle in passive dorsiflexion and active plantar flexion. Loss of pulse during these maneuvers is diagnostic, however in this patient his pulses were diminished at rest and did not change with provocative maneuvers.
(Answer D) External iliac artery endofibrosis is associated with repetitive hip flexion exercise, such as in competitive cycling, cross-country skiing, and running. Patients have ischemic pain with cramping, tightness, distension, or weakness in the musculature of the affected limb in a unilateral fashion. Additionally, patients also have an audible anterior hip bruit with hip extension combined with their symptoms and are typically resolved within 5 minutes of exercise cessation. History and physical examination of this patient does not indicate external iliac artery endofibrosis as the patient experienced bilateral lower extremity claudication, femoral artery bruits present in both legs before and after exertion, with a history of erectile dysfunction.
(Answer E) Spinal stenosis typically effects individuals over the age of 60 years old and is caused by progressive disc degeneration. Patients with spinal stenosis present with "pseudoclaudication," where their pain is exacerbated by standing or walking. Many patients with spinal stenosis have relief from sitting or leaning forward (as when walking up a hill). This patient's symptoms were more typical of classic claudication as his pain was not positional and did not improve with increasing the incline on the treadmill.
References
- Lee WJ, Cheng YZ, Lin HJ. Leriche syndrome. Int J Emerg Med 2008;1:223.
- Frederick M, Newman J, Kohlwes J. Leriche syndrome. J Gen Intern Med 2010;25:1102–04.
- Mathur M, Huda N, Bashir R. Blockage below the belt: Leriche syndrome. Am J Med 2014;127:291–94.
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