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Lymphedema

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Overview of Lymphedema

The lymphatic system consists of a network of specialized lymphatic vessels and various tissues and organs throughout the body that contain lymphocytes (white blood cells) and other cells that help the body fight infection and disease. The lymphatic vessels are similar to veins but have thinner walls. Some of these vessels are very close to the skin surface and can be found near veins; others are just under the skin and in the deeper fatty tissues near the muscles and can be found near arteries. Muscles and valves within the walls of the lymphatic vessels near the skin surface help pick up fluid and proteins from tissues throughout the body and move the lymph in one direction, toward the heart. Lymph is slowly moved through larger and larger lymphatic vessels and passes through small bean-shaped structures called lymph nodes. Lymph nodes filter substances that can be harmful to the body and contain lymphocytes and other cells that activate the immune system to fight disease. Eventually, lymph flows into one of two large ducts in the neck region. The right lymphatic duct collects lymph from the right arm and the right side of the head and chest and empties into the large vein under the right collar bone. The left lymphatic duct or thoracic duct collects lymph from both legs, the left arm and the left side of the head and chest and empties into the large vein under the left collar bone.

The lymphatic system collects excess fluid and proteins from the body tissues and carries them back to the bloodstream. Proteins and substances too big to move through the walls of veins can be picked up by the lymphatic vessels because they have thinner walls. Edema may occur when there is an increase in the amount of fluid, proteins, and other substances in the body tissues because of problems in the blood capillaries and veins or a blockage in the lymphatic system.

Lymphedema may be either primary or secondary. Primary lymphedema is a rare inherited condition in which lymph nodes and lymph vessels are absent or abnormal. Secondary lymphedema can be caused by a blockage or cut in the lymphatic system, usually the lymph nodes in the groin area and the arm pit. Blockages may be caused by infection, cancer, or scar tissue from radiation therapy or surgical removal of lymph nodes. This summary discusses secondary lymphedema.

Acute versus gradual-onset lymphedema

There are four types of acute lymphedema. The first type of acute lymphedema is mild and lasts only a short time, occurring within a few days after surgery to remove the lymph nodes or injury to the lymphatic vessels or veins just under the collar bone. The affected limb may be warm and slightly red, but is usually not painful and gets better within a week after keeping the affected arm or leg supported in a raised position and by contracting the muscles in the affected limb (for example, making a fist and releasing it). The second type of acute lymphedema occurs 6 to 8 weeks after surgery or during a course of radiation therapy. This type may be caused by inflammation of either lymphatic vessels or veins. The affected limb is tender, warm or hot, and red and is treated by keeping the limb supported in a raised position and taking anti-inflammatory drugs. The third type of acute lymphedema occurs after an insect bite, minor injury, or burn that causes an infection of the skin and the lymphatic vessels near the skin surface. It may occur on an arm or leg that is chronically swollen. The affected area is red, very tender, and hot and is treated by supporting the affected arm or leg in a raised position and taking antibiotics. Use of a compression pump or wrapping the affected area with elastic bandages should not be done during the early stages of infection. Mild redness may continue after the infection. The fourth and most common type of acute lymphedema develops very slowly and may become noticeable 18 to 24 months after surgery or not until many years after cancer treatment. The patient may experience discomfort of the skin or aching in the neck and shoulders or spine and hips caused by stretching of the soft tissues, overuse of muscles, or posture changes caused by increased weight of the arm or leg.

Temporary versus chronic lymphedema

Temporary lymphedema is a condition that lasts less than 6 months. The skin indents when pressed and stays indented, but there is no hardening of the skin. A patient may be more likely to develop lymphedema if he or she has:

  • Surgical drains that leak protein into the surgical site.
  • Inflammation.
  • An inability to move the limb(s).
  • Temporary loss of lymphatic function.
  • Blockage of a vein by a blood clot or inflammation of a vein.

Chronic (long-term) lymphedema is the most difficult of all types of edema to treat. The damaged lymphatic system of the affected area is not able to keep up with the increased need for fluid drainage from the body tissues. This may happen:

  • After a tumor recurs or spreads to the lymph nodes.
  • After an infection and/or injury of the lymphatic vessels.
  • After periods of not being able to move the limbs.
  • After radiation therapy or surgery.
  • When early signs of lymphedema have not been able to be controlled.
  • When a vein is blocked by a blood clot.

A patient who is in the early stages of developing lymphedema will have swelling that indents with pressure and stays indented but remains soft. The swelling may easily improve by supporting the arm or leg in a raised position, gently exercising, and wearing elastic support garments. Continued problems with the lymphatic system cause the lymphatic vessels to expand and lymph flows back into the body tissues, making the condition worse. This causes pain, heat, redness, and swelling as the body tries to get rid of the extra fluid. The skin becomes hard and stiff and no longer improves with raised support of the arm or leg, gentle exercise, or elastic support garments.

Patients with chronic lymphedema are at increased risk of infection. No effective treatment is yet available for patients who have advanced chronic lymphedema. Once the body tissues have been repeatedly stretched, lymphedema may recur more easily.

Risk factors

Factors that can lead to the development of lymphedema include radiation therapy to an area where the lymph nodes were surgically removed, problems after surgery that cause inflammation of the arm or leg, the number of lymph nodes removed in surgery, and being elderly.

Patients who are at risk for lymphedema are those with:

  • Breast cancer if they have received radiation therapy or had lymph nodes removed. Radiation therapy to the underarm area after surgical removal of the lymph nodes and the number of lymph nodes removed increases the risk of lymphedema.
  • Surgical removal of lymph nodes in the underarm, groin, or pelvic regions.
  • Radiation therapy to the underarm, groin, pelvic, or neck regions.
  • Scar tissue in the lymphatic ducts or veins, under the collar bones, caused by surgery or radiation therapy.
  • Cancer that has spread to the lymph nodes in the neck, chest, underarm, pelvis, or abdomen.
  • Tumors growing in the pelvis or abdomen that involve or put pressure on the lymphatic vessels and/or the large lymphatic duct in the chest and block lymph drainage.
  • An inadequate diet or those who are overweight. These conditions may delay recovery and increase the risk for lymphedema.

Diagnosis

Specific criteria for diagnosing lymphedema do not yet exist. About half of patients with mild edema describe their affected arm or leg as feeling heavier or fuller than usual. To evaluate a patient for lymphedema, a medical history and physical examination of the patient should be completed. The medical history should include any past surgeries, problems after surgery, and the time between surgery and the onset of symptoms of edema. Any changes in the edema should be determined as well as any history of injury or infection. Knowing the medications a patient is taking is also important for diagnosis.

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Management of Lymphedema

Prevention

Patients at risk for lymphedema should be identified early, monitored, and taught self-care. A patient may be more likely to develop lymphedema if he or she eats an inadequate diet, is overweight, is inactive, or has other medical problems. To detect the condition early, the following should be examined:

  • Comparison of actual weight to ideal weight.
  • Measurements of the arms and legs.
  • Protein levels in the blood.
  • Ability to perform activities of daily living.
  • History of edema, previous radiation therapy, or surgery.
  • Other medical illnesses, such as diabetes, high blood pressure, kidney disease, heart disease, or phlebitis (inflammation of the veins).

It is important that the patient know about his or her disease and the risk of developing lymphedema. Poor drainage of the lymphatic system due to surgery to remove the lymph nodes or radiation therapy may make the affected arm or leg more susceptible to serious infection. Even a small infection may lead to serious lymphedema. Patients should be taught about arm, leg, and skin care after surgery and/or radiation (see Considerations for Teaching Patients Prevention and Control of Lymphedema list below). It is important that patients take precautions to prevent injury and infection in the affected arm or leg, since lymphedema can occur 30 or more years after surgery. Breast cancer patients who follow instructions about skin care and proper exercise after mastectomy are less likely to experience lymphedema.

Lymphatic drainage is improved during exercise, therefore exercise is important in preventing lymphedema. Breast cancer patients should do hand and arm exercises as instructed after mastectomy. Patients who have surgery that affects pelvic lymph node drainage should do leg and foot exercises as instructed. The doctor decides how soon patients should start exercising after surgery. Physiatrists (doctors who specialize in Physical Medicine and Rehabilitation) or physical therapists should develop an individualized exercise program for the patient.

Better recovery occurs when lymphedema is discovered early, so patients should be taught to recognize the early signs of edema and to tell the doctor about any of the following symptoms:

  • Feelings of tightness in the arm or leg.
  • Rings or shoes that become tight.
  • Weakness in the arm or leg.
  • Pain, aching, or heaviness in the arm or leg.
  • Redness, swelling, or signs of infection.

Considerations for Teaching Patients Prevention and Control of Lymphedema

  1. Keep the arm or leg raised above the level of the heart, when possible. Avoid making rapid circles with the arm or leg to keep blood from collecting in the lower part of the limb.
  2. Clean the skin of the arm or leg daily and moisten with lotion.
  3. Avoid injury and infection of the arm or leg:
    • Arms:
      • Use an electric razor for shaving.
      • Wear gardening and cooking gloves.
      • Use thimbles for sewing.
      • Take care of fingernails; do not cut cuticles.
    • Legs:
      • Keep the feet covered when outdoors.
      • Keep the feet clean and dry; wear cotton socks.
      • Cut toenails straight across; see a podiatrist as needed to prevent ingrown nails and infections.
    • Either arms or legs:
      • Suntan gradually; use sunscreen.
      • Clean cuts with soap and water, then use antibacterial ointment.
      • Use gauze wrapping instead of tape; do not wrap so tight that circulation is cut off.
      • Talk to the doctor about any rashes.
      • Avoid needle sticks of any type in the affected arm or leg.
      • Avoid extreme hot or cold such as ice packs or heating pads.
      • Do not overwork the affected arm or leg.
  4. Do not put too much pressure on the arm or leg:
    • Do not cross legs while sitting.
    • Wear loose jewelry; wear clothes without tight bands.
    • Carry a handbag on the unaffected arm.
    • Do not use blood pressure cuffs on the affected arm.
    • Do not use elastic bandages or stockings with tight bands.
    • Do not sit in one position for more than 30 minutes.
  5. Watch for signs of infection, such as redness, pain, heat, swelling, and fever. Call the doctor immediately if any of these signs appear.
  6. Do prescribed exercises regularly as instructed by the doctor or therapist.
  7. Keep regular follow-up appointments with the doctor.
  8. Check all areas of the arms and legs every day for signs of problems:
    • Measure around the arm or leg at regular time periods as suggested by the doctor or therapist.
    • Measure the arm or leg at the same two places each time.
    • Tell the doctor if the limb suddenly gets larger.
  9. The ability to feel touch, temperature, or pain in the affected arm or leg may be lessened. Use the unaffected limb to test temperatures for bath water or cooking.

Treatment

Lymphedema is treated by physical methods and with medication. Physical methods include supporting the arm or leg in a raised position; manual lymphatic drainage (a specialized form of very light massage that helps to move fluid from the end of the limb toward the trunk of the body); wearing custom-fitted clothes that apply controlled pressure around the affected limb; and cleaning the skin carefully to prevent infection. Lymphedema may be treated by combining several therapies. This is known as complex physical therapy (or complex decongestive therapy), which consists of manual lymphedema treatment, compression wrapping, individualized exercises, and skin care, followed by a maintenance program. Complex physical therapy must be performed by a professional trained in the techniques.

Surgery for treating lymphedema usually results in complications and is seldom recommended for cancer patients.

Compression garments

When pressure garments are used, they should cover the entire area of edema. For example, a stocking that reaches only to the knee tends to become tight and block the lymphatic vessels and veins if there is edema in the thigh. Pumps connected to cuffs that wrap around the arm or leg and apply pressure on and off may be helpful; however, some physicians and therapists feel these pumps are not effective and may make the edema worse. The cuff is inflated and deflated according to a controlled time cycle. This pumping action is believed to increase the movement of fluid in the veins and lymphatic vessels and keeps fluid from collecting in the arm or leg. Compression pumps should be used only under the supervision of a trained health care professional because high external pressure can damage the lymphatic vessels near the skin surface.

Drug therapy

Antibiotics may be used to treat and prevent infections. Other types of drugs such as diuretics or anticoagulants (blood thinners) are generally not helpful, and may make the problem worse.

Finding the exact cause of the swelling and treating it correctly is important. Edema often leads to infection, which then increases fluid and protein deposits in the tissues. If an infection is diagnosed, appropriate antibiotics should be given. Blood clots should be ruled out because massage and other therapy techniques to encourage drainage may cause the clots to move through the bloodstream and cause more serious heart or lung problems. If blood clots are found, they should be treated with anticoagulants.

Coumarin is a dietary supplement that has been studied as a treatment for lymphedema. In the United States, dietary supplements are regulated as foods not drugs. Supplements are not required to be approved by the Food and Drug Administration (FDA) before being put on the market. Because there are no standards for manufacturing consistency, dose, or purity, there may be considerable difference from one lot of dietary supplements to the next.

Coumarin was once used in some foods and medications in the United States. It was found to cause liver damage, and its use in foods and medications in the United States has been banned since the 1950s. Coumarin is available in several countries, but has not been approved for use in the United States or Canada.

Dietary Management

The nutritional status of the patient should be evaluated and appropriate dietary recommendations should be made. Blood protein levels and weight should be monitored regularly and patients should be encouraged to eat protein-rich foods.

Pain Management

Patients with lymphedema may experience pain caused by the swelling and pressure on nerves; loss of muscle tissue and function; or scar tissue causing shortening of muscles and less movement at joints. Pain may be treated with medications, relaxation techniques, and/or transcutaneous electrical nerve stimulation (TENS), however, the most successful treatment is to decrease the lymphedema.

Complications

The edema can make tissues less able to take in nutrients and more likely to be damaged if the affected limb is not moved for long periods. Therefore, patients with lymphedema should be monitored for areas of skin breakdown, especially over areas where there is very little tissue between the skin and bone (i.e., hips, knees, and elbows).

Bladder emptying problems can develop from lymphedema in the pelvic or groin areas. Patients with lymphedema who are also taking narcotic medications may develop bowel problems. Bowel and bladder status should be monitored regularly for any signs of urine retention or constipation.

Psychosocial considerations

Because lymphedema is disfiguring and sometimes painful and disabling, it can create mental, physical, and sexual problems. Several studies have noted that women who develop lymphedema after treatment for breast cancer have more mental, physical, and sexual difficulties than women who do not develop lymphedema. The added stresses associated with lymphedema may interfere with treatment that is often painful, difficult, and time-consuming.

Coping with lymphedema in the arm after breast cancer treatment is especially difficult for patients who have little social support. Some patients may react to the problem by withdrawing. It is also difficult for patients with painful lymphedema. Patients with lymphedema may be helped by group and individual counseling that provides information about ways to prevent lymphedema, the role of diet and exercise, advice for picking comfortable and flattering clothes, and emotional support.

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Complications of Lymphedema

In addition to the complications associated with chronic lymphedema noted in previous sections, a rare but fatal complication of lymphedema is lymphangiosarcoma, a tumor of the lymphatic vessels. The average time between mastectomy and the appearance of lymphangiosarcoma is about 10 years. After a patient develops lymphangiosarcoma, the average survival time is a little over 1 year.

The cause of lymphangiosarcoma is not known. It appears as one or more bluish-red bumps on the affected arm or leg. First, one purple-red, slightly raised area in the skin of the arm or leg appears. The patient usually describes it as a bruise. Later, more tumors appear, and the bumps grow. Death usually results from metastases to the lungs.

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Lymphedema