Other Uncommon Vascular Conditions

Our Beth Israel vascular specialists have extensive expertise and experience diagnosing and treating many less common vascular diseases, including the following:

Buerger's Disease (also known as thromboangiitis obliterans)

What is Buerger's disease?
This is a chronic condition caused by inflammation and tissue damage in the arteries of the hands and feet. Symptoms include pain in the fingers, hands, legs or feet, clammy and cold skin, and a diminished sense of heat and cold. As the disease progresses, people can develop gangrene in the hands and feet, which may require amputation.

What are the risk factors for Buerger's disease?
The disease is found mostly in men ages 20 to 40 who smoke cigarettes.

What are the treatment options for Buerger's disease?
The only treatment is to stop smoking.

Fibromuscular Dysplasia

What is fibromuscular dysplasia?
This condition develops when cells in the walls of arteries grow abnormally, causing blood vessels to narrow or bulge (aneurysm). Fibromuscular dysplasia often occurs in the carotid arteries, the two arteries in the neck bringing blood to the brain. It can also affect arteries within the brain, and those bringing blood to the kidneys. Narrowed arteries reduce or block blood flow to the brain, which can cause a stroke or "mini" stroke. Symptoms include high blood pressure, dizziness, ringing in the ears, weakness or numbness in the face, neck pain, or changes in vision.

What are the risk factors for fibromuscular dysplasia?
Women ages 14 to 50 are at greatest risk. Smoking and certain medications can worsen the disorder.

What are the treatment options for fibromuscular dysplasia?
Mild cases often respond to antiplatelet therapy such as aspirin or anticoagulants, and pain medication for headaches. In the case of severe narrowing of the blood vessels, treatment may include angioplasty, stenting or surgery to repair an aneurysm.

Marfan Syndrome

What is Marfan syndrome?
Marfan syndrome is a genetic (inherited) disorder of the connective tissue that can affect the skeleton, eyes, heart, or blood vessels because their connective tissue lacks strength due to its abnormal chemical makeup. Those affected have greater risk of developing a bulge in a weakened section of the aorta, which is the main blood vessel carrying blood away from the heart to the rest of the body. This bulge is called an aneurysm. The aorta can also become abnormally wide or enlarged. A bulge or enlargement of the aorta can rupture (burst), which can be fatal.

What are the risk factors for Marfan syndrome?
People usually inherit Marfan syndrome. However, in about 30 percent of cases, it appears spontaneously in people with no family history.

What are the treatment options for Marfan syndrome?
Vascular specialists carefully monitor people with Marfan syndrome, using tests including echocardiograms and CT scans to continually check the size of the aorta. Also, drugs such as beta-blockers can help reduce stress on the aorta by managing underlying heart problems. If an aneurysm enlarges or dissects, minimally invasive treatment options are used, such as endovascular stenting.

Raynaud's Disease

What is Raynaud's disease?
For a brief time, the body overreacts to cold or stress by abnormally narrowing the small blood vessels (vasospasm) in the fingers or toes, so that too little blood flows to those parts. During an episode, the affected part changes color, turning white (from inadequate blood supply), then blue (from lack of oxygen in tissues), then possibly red. Attacks last from a few minutes to over an hour. In severe cases, ulcers may develop on the finger pads.

What are the risk factors for Raynaud's disease?
Those with certain connective tissue disorders and autoimmune diseases are at greater risk. So are people who smoke, drink alcohol, or are infected with the bacteria Helicobacter pylori.

What are the treatment options for Raynaud's disease?
These include preventive measures such as wearing gloves (to keep fingers warm), avoiding cold, stopping smoking, avoiding stress, and medications that bring down high blood pressure, which helps open up blood vessels.

Scleroderma

What is scleroderma?
Also called systemic sclerosis, scleroderma is a chronic ailment that can be degenerative (worsening over time), causing abnormalities in the blood vessels as well as problems in the joints, skin and internal organs. Symptoms include pain and swelling of the fingertips, Raynaud's disease, joint pain, hardening of the skin, heart failure and abnormal heart rhythms.

What are the risk factors for scleroderma?
Scleroderma usually develops from combined genetic (inherited) and environmental factors, occurring three to four times more often in women than men.

What are the treatment options for scleroderma?
These may include anti-inflammatory medications or corticosteroids, penicillamine–a drug used to treat patients with rheumatoid arthritis, immunosuppressive medications, and physical or occupational therapy and exercise. On rare occasions, surgery is used to treat scleroderma.

Temporal Arteritis and Giant Cell Arteritis (GCA)

What is temporal arteritis and giant cell arteritis?
This is an inflammation of the lining of the arteries, the blood vessels carrying oxygen-rich blood away from the heart to the rest of the body. Most often, it affects the arteries in the head, especially those in the temples. If the inflammation affects the arteries in your neck, upper body and arms, it is called giant cell arteritis. Symptoms include headaches, jaw pain, and blurred or double vision. The most serious complications can be blindness and, less often, stroke.

What are the risk factors for temporal arteritis and giant cell arteritis?
The condition almost always affects older adults–the average age of onset is 70, and it rarely occurs in people younger than 50. Women are about two times more likely to develop the disease. People of Scandinavian origin are particularly at risk. In addition, people with the arthritic condition called polymyalgia rheumatic have increased risk of giant cell arteritis. The physician may perform a temporal artery biopsy on patients suspected of having temporal arteritis to confirm the diagnosis.

How is temporal arteritis or giant cell arteritis diagnosed?
Your doctor may recommend a temporal artery biopsy. During this procedure, a small piece of tissue is removed from your temporal artery (the artery passing through the temples) under local anesthesia. The tissue sample is studied for signs of inflammation and damage.

What are the treatment options for temporal arteritis or giant cell arteritis?
Prompt treatment with corticosteroid medications usually relieves symptoms, and may prevent loss of vision.

Thoracic Outlet Syndrome

What is thoracic outlet syndrome?
This condition develops when blood vessels and nerves passing through the narrow channel at the base of the neck and into the armpits and arm become compressed (abnormally narrowed) due to anatomic abnormalities. This can cause clots in arteries or veins. The arm and hand become swollen and discolored or pale and cool, tingle, ache or feel numb. The pulse may be weak or absent.

What are the risk factors for thoracic outlet syndrome?
These include poor posture, weak shoulder muscles, injury, and activities that involve repeatedly raising or holding the arms overhead. Sometimes the cause is unknown.

What are the treatment options for thoracic outlet syndrome?
Exercise and physical therapy can strengthen chest muscles, restore normal posture, and enlarge the passageway through which the blood vessels and nerves travel. Non-steroidal anti-inflammatory drugs (NSAIDs) can relieve pain. If the problem continues, our specialists may recommend surgery to release or remove the tissues pressing on the arteries and nerves.

For more information or to make an appointment with a vascular surgeon at Mount Sinai Beth Israel, click here to fill out an appointment scheduling form. A staff member will get back to you within 48 hours to schedule an appointment.

For An Appointment Call

Thomas R. Bernik, MD
Endovascular and Vascular Surgery
212.844.5555

Robert J. Grossi, MD
Vascular Surgery
212.844.5559

Gary A. Gwertzman, MD
Vascular Surgery and Wound Care
718.677.0109

Stephen P. Haveson, MD
Vascular Surgery and Wound Care
212.844.1330

Jennifer Svahn, MD
Vascular Surgery (Venous Disease)
212.420.5648

Vascular Labs
212.844.5555