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Unusual Vascular Conditions

NewYork-Presbyterian Hospital vascular specialists have considerable expertise diagnosing and treating a wide range of unusual vascular diseases, including those outlined below.

Raynaud's disease

In people with Raynaud's disease, blood flow is temporarily decreased to the fingers, and sometimes the nose, ears, toes, nipples, or knees in response to a trigger such as cold or stress. During an attack the fingers or other affected part change color from pale/white to blue to red, and may become swollen and painful when warmed. These attacks last from a few minutes to an hour or more. In severe cases ulcers may develop on the finger pads.

Risk Factors: People with certain connective tissue disorders and autoimmune diseases are at increased risk for Raynaud's disease, as are those who smoke, drink alcohol, or are infected with the bacteria Helicobacter pylori (H. pylori).

Treatment: Treatment may include preventive measures such as wearing gloves, avoiding cold, smoking cessation, avoiding trauma, and medications used to treat high blood pressure, which reduce blood vessel constriction.

Thoracic outlet syndrome

Thoracic outlet syndrome develops when the blood vessels and nerves that pass through the narrow passageway at the base of the neck to the armpit and arm (the brachial plexus or subclavian vessels) become compressed as a result of poor posture or anatomic abnormalities. The affected arm becomes pale and cool, and tingles, aches, or feels numb. The pulse is weak or absent.

Risk Factors: Poor posture, obesity, weak shoulder muscles, injury, and activities in which a person repeatedly raises or holds his or her arms overhead can all play a role in the development of thoracic outlet syndrome. In some cases no cause can be identified.

Treatment: Doctors treat thoracic outlet syndrome with exercise and physical therapy to strengthen chest muscles, restore normal posture, and enlarge the area through which the blood vessels and nerves pass. Non-steroidal anti-inflammatory drugs can relieve pain. If the problem persists, vascular specialists may recommend surgery to release or remove the structures compressing the arteries and nerves.

Marfan's syndrome

Marfan's syndrome is a genetic disorder of the connective tissues that can affect the skeleton, eyes, heart, or blood vessels. People with Marfan's syndrome are at risk of developing a bulge in a weakened spot in the aorta, the main blood vessel that carries blood from the heart to the rest of the body; this bulge is called an aneurysm. The aorta can also become dilated or enlarged. An aneurysm or dilation makes the aorta prone to dissection (tearing) or rupture, which can be fatal.

Risk Factors: Marfan syndrome is an inherited condition that usually develops in people with a family history of the syndrome. In up to a third of cases, though, the disorder appears spontaneously, in those without a familial link.

Treatment: Vascular specialists follow people with Marfan's syndrome regularly, using tests including echocardiograms to evaluate the size of the aorta. Drugs such as beta-blockers can help decrease stress on the aorta by managing underlying heart problems.

Fibromuscular dysplasia

In people with fibromuscular dysplasia, cells in the walls of arteries develop abnormally, causing the vessels to narrow or bulge. The condition often develops in the carotid arteries, the two blood vessels in the neck that carry blood to the brain. It can also affect the arteries within the brain, and those that supply the kidneys with blood. As the arteries narrow blood flow to the brain is reduced or blocked, sometimes causing a stroke or mini-stroke. Symptoms of fibromuscular dysplasia include high blood pressure, dizziness, headache, ringing in the ears, weakness or numbness in the face, neck pain, or changes in vision.

Risk Factors: Fibromuscular dysplasia develops most often in women ages 14 to 50. Smoking and certain medications can worsen fibromuscular dysplasia.

Treatment: Vascular specialists treat mild cases of fibromuscular dysplasia with antiplatelet therapy such as aspirin or anticoagulants and pain medication for headaches. If the narrowing is more severe treatment may include angioplasty, arterial stents, and surgery to treat aneurysms.

Difficult-to-control cholesterol

Cholesterol is a waxy substance that is both produced by the body and found in certain foods, especially meat and dairy products. When cholesterol levels in the blood are abnormally high, it can accumulate inside blood vessels, narrowing the channels through which blood flows. High cholesterol is linked to coronary artery disease, peripheral vascular disease (PVD), stroke, and high blood pressure.

Risk Factors: People develop abnormal cholesterol levels for many reasons including physical inactivity, obesity, age, sex, and a diet high in animal fats. Some inherited genetic disorders also cause the body to overproduce cholesterol and to eliminate it inefficiently.

Treatment: If regular exercise, a healthy diet, and cholesterol-lowering medications fail to normalize cholesterol levels, doctors may use techniques such as lipid apheresis to treat the problem. During lipid apheresis blood is withdrawn and circulated through filters that remove the damaging type of cholesterol (low-density lipoproteins). The filtered blood is then returned to the patient.

Buerger's Disease

Buerger's disease (thromboangiitis obliterans) is a chronic disease caused by inflammation and tissue damage in the arteries and veins in the hands and feet. Symptoms include pain in the finger, hands, legs, or feet, clammy and cold skin, and a diminished sense of heat and cold. As the disease progresses people with Buerger's can develop gangrene in their extremities and may require amputation.

Risk Factors: This disease is most common in men between the ages of 20 and 40 who smoke cigarettes.

Treatment: The only treatment for Buerger's is to stop smoking.

Scleroderma

Scleroderma, also called systemic sclerosis, is a chronic, degenerative disease that causes blood vessel abnormalities as well as joint, skin, and internal organ problems. Symptoms include thickening and swelling of the tips of the fingers; Raynaud's disease; joint pain; taut, dark skin on the face; spider veins; scarring of the skin on the fingers, wrists, or elbows causing immobility; and heart failure and abnormal heart rhythms.

Risk Factors: Scleroderma usually arises from a combination of genetic and environmental factors. The condition is three to four times more common in females than males.

Treatment: Treatment may include anti-inflammatory medications or corticosteroids, penicillamine, a drug used to treat patients with rheumatoid arthritis, immunosuppressive medications, and physical and occupational therapy and exercise.

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