In a previous article, I described a condition known as peripheral vascular disease (PVD). My article focused on the atherosclerotic causes (accumulation of fatty material inside the walls of arteries) of intermittent claudication.
Claudication is described as pain with walking, which is relieved with rest. Etiologies other than atherosclerosis that could cause limb pain include the musculoskeletal disorders such as arthritis, bursitis, tendonitis and plantar fasciitis, as well as neurogenic causes, including lumbar canal stenosis and peripheral neuropathy.
This article deals with many of the non-atherosclerotic causes of lower and upper limb pain. They include thromboangiitis obliterans (TAO), also known as Buerger’s disease; fibromuscular dysplasia (FMD); popliteal artery entrapment syndrome (PAES); cystic adventitial disease (CAD); external iliac artery endofibrosis (EIAE); and cannabis arteritis.
The diagnosis of these disorders requires a high index of suspicion. The test performed to determine the atherosclerotic causes of PVD – the ankle brachial/toe index – is normal.
These patients are young and athletic, and they present with exercise-induced claudication. They traditionally lack the normal risk factors for atherosclerosis.
TAO was first discovered in 1879 by Australian physician Felix von Winiwarter, and later by New York surgeon Leo Buerger. Buerger reported the clinical pathologic characteristics that would eventually bear his name. Buerger described the highly cellular nature of the thrombosis and perivascular inflammation that would prompt him to suggest the term, “thromboangiitis obliterans.”
Buerger’s disease is a non-atherosclerotic inflammatory vasculitis of small- and medium-sized arteries and veins of the upper and lower extremities. Of significant importance is that it occurs in young males who are heavy smokers. Recently, however, there has been an increasing prevalence in females.
Patients typically present with distal digital ischemia (a deficient supply of blood to a body part due to obstruction of inflow of arterial blood) usually involving multiple extremities and sadly, frequently requiring amputation. It is more commonly seen in the Middle East and Asia.
Of classic importance is the strong association with tobacco. Claudication in these patients is mostly in the foot, whereas in patients with PVD, the calf area is affected. Ulceration is not uncommon, as is infection.
Contrary to atherosclerotic disease, the upper extremities can be involved in Buerger’s disease. Patients can present with both, upper or lower extremity claudication, rest pain, Raynaud’s phenomenon, superficial thrombophlebitis and gangrene or ulcerations. Serological markers, including WSR and C-reactive protein, are normal. There is an increase in blood viscosity and high titers of anti-endothelial antibodies.
The Allen’s test may be abnormal. Biopsy and histological evaluation have a limited role in the diagnosis of Buerger’s disease. When available, findings include a highly inflammatory thrombus in both arteries and veins and inflammation of all three layers of the arterial wall. Duplex ultrasound and angiography show normal proximal arteries, but segmental disease of the distal arteries with the classic “corkscrew collaterals.”
Treatment involves abstinence of all tobacco products, including nicotine replacement. The use of protective footwear should prevent any pressure injuries to the heels and soles. Avoidance of the cold environment and medications that may lead to vasoconstriction is recommended.
Occasionally a surgical approach is warranted, although surgical revascularization is typically not feasible. Sympathectomy remains a valid treatment alternative for patients with refractory pain and ischemia despite maximal medical therapy.
Cannabis arteritis may be a form of TAO. It affects males generally less than 45 years of age. Claudication, Raynaud’s phenomenon and distal necrosis/gangrene of the legs with early disappearance of distal pulses may be seen. Treatment is the cessation of marijuana use.
FMD is a non-inflammatory disease that usually affects the medium-sized arteries. Most patients are women between the ages of 20 and 40. The pathogenesis is unknown.
There are five major types, which include medial, pre-medial and intimal fibroplasias and the interstitial and adventitial types. Medial fibroplasia is the most common type. The renal artery is most commonly affected. Other vessels include the carotid, vertebral, subclavian, mesenteric and iliac arteries.
Angiography demonstrates the so-called “string of beads” appearance, with the beads larger than the normal caliber of the artery. The clinical manifestations depend on the affected arterial bed. Resistant hypertension is the most common symptom if the renal arteries are affected.
Cerebrovascular involvement results in headaches, ringing in the ears, vertigo, transient ischemic attacks and intracranial aneurysms. FMD affecting the mesenteric arteries can result in persistent abdominal pain and weight loss.
Involvement of the extremities can result in claudication, chronic limb ischemia, embolization and aneurysms. Treatment includes the use of anti-platelet drugs and antihypertensive medications.
In resistant cases, an endovascular or surgical approach may be warranted.
PAES is a rare disorder of young athletic males. The disease is often bilateral with six known types. The pathogenesis involves a congenital abnormality between the popliteal artery and the medial head of the gastrocnemius muscle.
Overuse of this muscle results in secondary enlargement, resulting in compression of the artery as it passes through the muscle. Symptoms include calf and foot claudication brought on by exercise, nocturnal cramps and paresthesias.
Chronic limb ischemia with ulcers and blue toes secondary to thromboembolism can occur. Diagnosis is by history and with duplex scan. With dorsiflexion of the foot against resistance, the normal triphasic waveform converts to a biphasic waveform with altered flow velocities. Loss of the peripheral pulses during this maneuver may occur.
Treatment options are mostly surgical, including bypass grafting. There is no indication for stenting in this situation.
CAD is a disorder that affects males more frequently than females in their mid-40s. The presenting symptom is unilateral calf claudication. Painless swelling from secondary venous insufficiency may occur.
The location involves the popliteal artery in 85 percent of cases. However, the external iliac, common femoral and axillary arteries may also be involved.
The pathogenesis is the development of mucoid-filled cysts located in the adventitial layer of the blood vessel and subsequent pressure on the artery by the cyst. Patients have normal ABIs. They usually have intact popliteal and pedal pulses when the leg is in the neutral position.
Obliteration of the dorsal pedal and posterior tibial pulses is seen with flexion of the knee also known as Ishikawa’s sign. Duplex ultrasound may show a mass in the vessel wall. Angiography may show the “scimitar” or “hourglass” sign.
Treatment is by either ultrasound/CT-guided aspiration or surgical removal of the cyst. Stenting and percutaneous transluminal angioplasty are not indicated, while surgical bypass grafting is.
EIAE is seen in highly trained athletes,cyclists or rugby players. Patients are between 30 and 50 years of age with the left external iliac artery most commonly affected. Eighty-five percent of cases are unilateral.
The pathophysiology involves medial trauma caused by a strong-flexed thigh. Kinking due to excessive artery length, along with psoas muscle hypertrophy, may result in a dissection.
The pain is usually exercise-induced and located mostly in the thigh, with patients complaining of a lack of power. Symptoms are relieved with rest. The thigh may become swollen. The ABI can drop to less than 0.5 with exercise. Pulses are usually present and triphasic.
Therapy is either an endovascular or surgical approach.
Final note
I recently attended the Cattleman’s Gala, which raises money for young cancer victims. Throughout the evening, there was a host of auction items. Some prizes included a trip to the Las Vegas Rodeo or the Indy 500.
I was particularly impressed with one auction item and one individual. A one-of-a-kind iron bench with ceramic tiles handcrafted by several cancer victims was being auctioned off. The bench sat two to three people, and the sentimental value far outweighed the artistic abilities of each tile.
By the time the heat of the auction was over, an individual paid $36,000 for a piece of art with a material value of $500. The gentleman did not wish to be recognized. He was brought onstage by massive applause, where he received even more accolades from the crowd.
The gentleman then turned to the auctioneer and whispered in his ear. As the man walked offstage, the auctioneer informed the crowd that he had decided to give the chair back to charity for re-auction. The chair subsequently went for an additional $20,000.
I did not get a chance to meet this man. Somebody told me he had a child who died of cancer. He did not seem very wealthy or arrogant. His heart had simply been moved, along with mine.
I left in awe of that man’s gesture that night. It was truly “Jesus-like” in character. I will never forget that night, sir, whoever you are. I applaud you and thank you for letting me witness such an event.
I hope you all remember what Jesus once told us: “It is better to give than to receive.” That man certainly accomplished this that night.
Salvatore A. Barbaro, III, M.D., FACC, practices general and interventional cardiology at his private practice, located at 19234 Stone Hue, Ste. 104, San Antonio, Texas 78258. For more information, please call 210-490-4600, or send an email to barbarocardiology@sbcglobal.net.
