Related Terms

• Congenital lymphedema, elephantiasis, filarial elephantiasis, late-onset lymphedema, lymphatic obstruction, lymphedema praecox, lymphedema tarda, lymphoedema, Meige's disease, Milroy's disease.

Background

• Lymphedema is a condition in which swelling develops, usually in an arm or a leg, due to a compromised or impaired lymphatic system. The lymphatic system is a part of the immune system that fights infection and is also responsible for cleansing the tissues and maintaining balance of fluids in the body.

• The lymphatic system circulates a fluid called lymph throughout the body. The lymph collects bacteria, viruses, and waste products. The lymph travels through lymph vessels, which lead to lymph nodes. In the lymph nodes, the wastes are then filtered out by lymphocytes (white blood cells). Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin. Lymphedema occurs when the lymph system is damaged or blocked. When this happens, the lymph vessels are unable to adequately drain lymph fluid, usually from an arm or leg, although it can also affect other parts of the body.

• There are two types of lymphedema. Primary lymphedema is in inherited problem that causes improper development of the lymphatic system. Secondary lymphedema is caused by the removal or enlargement of the lymph nodes due to illness, surgery, or injury.

• The main symptom is persistent swelling, usually of an arm or leg. Symptoms may occur very slowly over time or more quickly in the case of infection or injury. Imaging tests may be used to diagnose lymphedema.

• Most of the underlying causes of lymphedema are irreversible. There is no cure for lymphedema. However, treatments are available to reduce the swelling and keep it to a minimum. Treatment focuses on minimizing the swelling, controlling pain, and preventing complications. Treatment includes physical methods, such as compression stockings, and medicines. In cases of severe lymphedema, surgery may be performed to remove excess tissue from the affected area. Medications are generally not used. Lymphedema may improve with time, although some swelling usually persists throughout an affected person's lifetime.

• Primary lymphedema is inherited and cannot be prevented. Patients at risk of developing secondary lymphedema can take measures to help prevent it. Patients having tumors removed and/or radiation therapy should find out if the procedure will involve the lymph nodes or lymph vessels. The chance of improving the condition is better if treatment begins early. Untreated lymphedema can lead to problems that cannot be reversed. Complications of lymphedema include skin infections, elephantiasis (extreme swelling), and cancer of the lymph vessels. Many of the complications may be prevented by vigilant skin care and hygiene.

Types of the Disease

• Primary lymphedema: Primary lymphedema is a rare inherited condition caused by problems with the development of the lymph vessels in the body. Primary lymphedema occur most frequently in women and usually affects the legs rather than the arms. Symptoms may occur at birth or later in life.

• Secondary lymphedema: Secondary lymphedema is the result of damage to the lymph system, which may be caused by infection, injury, cancer, removal of lymph nodes, radiation, or scar tissue.

Risk Factors

• Cancer: Patients with tumors that affect or block lymph ducts or lymph nodes or vessels in the neck, chest, underarm, pelvis, or abdomen are at an increased risk of developing secondary lymphedema.

• Cancer treatment: Cancer patients who have had removal and/or radiation of lymph nodes in the underarm, groin, pelvis, or neck are at risk of developing secondary lymphedema. Lymphedema often occurs in breast cancer patients who had all or part of their breast and underarm lymph nodes removed. Lymphedema may develop within days or many years after cancer treatment.

• Damaged skin: Damaged skin or scars from burns, injuries, or surgery increases the risk of developing secondary lymphedema.

• Family history: A family history of swollen legs or feet increases the risk of primary lymphedema, which is a genetic disorder. It is possible, however, that the condition was not diagnosed properly in previous generations.

• Obesity: Being overweight or obese increases the risk of developing lymphedema. Excess body weight causes fat cells to enlarge and new fat cells to be formed. These larger and new fat cells produce waste products that must be removed by the lymphatic system. Despite increased weight and body size, the number of lymph capillaries and vessels do not increase.

• Surgery: Lymphedema in the legs may occur after surgery performed in patients with uterine cancer, ovarian cancer, prostate cancer, lymphoma, vulvar cancer, or melanoma. Patients with a history of joint replacement surgery may be at risk for lymphedema, as there is a cluster of lymph nodes located near most major joints. Slow healing of the skin after surgery is a risk factor for lymphedema. Most surgery-related lymphedema develops within three years of surgery.

Causes

• General:

• The lymphatic system circulates a fluid called lymph throughout the body. The lymph collects bacteria, viruses, and waste products. The lymph travels through lymph vessels, which lead to lymph nodes. In the lymph nodes, the wastes are then filtered out by lymphocytes (white blood cells). Clusters of lymph nodes are found in the underarm, pelvis, neck, abdomen, and groin. Lymphedema occurs when the lymph system is damaged or blocked. When this happens, the lymph vessels are unable to adequately drain lymph fluid, usually from an arm or leg, although it can also affect other parts of the body. The cause of primary lymphedema is in inherited problem with development of the lymphatic system. Secondary lymphedema is caused by the removal or enlargement of the lymph nodes due to illness, surgery, or injury.

• Primary lymphedema:

• Late-onset lymphedema (lymphedema tarda): Late-onset lymphedema, also called lymphedema tarda, is a rare condition caused by an inherited genetic mutation. Symptoms usually do not appear until after 35 years of age.

• Meige's disease (lymphedema praecox): Meige's disease, also called lymphedema praecox, is caused by an inherited genetic mutation. It causes the lymph vessels to form without valves, which makes it difficult for the body to properly drain the lymph fluid from the arms and legs. Symptoms usually occur in childhood or at puberty.

• Milroy's disease (congenital lymphedema): Milroy's disease, also called congenital lymphedema, is caused by an inherited genetic mutation that causes incorrect formation of the lymph nodes. Symptoms occur in infancy.

• Secondary lymphedema:

• Cancer: A tumor growing near a lymph node or lymph vessel may become large enough to obstruct the flow of the lymph fluid.

• Infection: A bacterial infection may occur in the lymph vessels and lymph nodes and cause a blockage in the flow of lymph fluid. Lymphedema due to cellulitis, a skin infection, is more common in obese patients. A parasitic infection may also block lymph vessels. Infection-related lymphedema is most common undeveloped countries in tropical and subtropical regions.

• Injury: An injury that may damage the lymph node or lymph vessels and reduce drainage of lymph can cause lymphedema.

• Mastectomy (breast removal): Because lymphatic drainage of the arm passes through the armpit, in developed countries, one of the most common causes of lymphedema is removal of the breast and underarm lymph tissue in breast cancer patients. This causes lymphedema of the arm 10-15% of the time.

• Radiation: Radiation treatment in cancer patients may cause scarring and inflammation of the lymph nodes or lymph vessels, resulting in a blockage of the flow of lymph fluid.

• Surgery: Surgery may cause lymphedema to develop if the lymph nodes and lymph vessels are removed or damaged.

Signs and Symptoms

• General: The main symptom is persistent swelling, usually of an arm or leg. Symptoms may occur very slowly over time, or more quickly in the case of infection or injury.

• Arm or leg sensation: There is discomfort in the affected arm or leg. It may feel heavy, tight, ache, or itchy, or have a burning sensation.

• Infections: Recurring infections may occur in the affected arm or leg.

• Restricted range of motion: It may be difficult to moving a joint in the affected arm or leg.

• Skin changes: Hardening or thickening of the skin may occur in the affected arm or leg.

• Unexplained swelling: Swelling of the feet or ankles that is not due to an obvious cause such as injury may indicate primary lymphedema. The swelling may affect part or all of the arm or leg, including fingers and toes. Clothing, shoes, bracelets, watches, or rings may feel too small. The swelling may range from very minor swelling to extreme swelling that can make it impossible to use the affected arm or leg.

• Other: Other symptoms may include hair loss and trouble sleeping due to discomfort.

Diagnosis

• General: Swelling in an arm or leg may indicate lymphedema. A healthcare provider may check to be sure that the swelling is not due to other causes, such as a blood clot or an infection that does not involve lymph nodes. Arm or leg swelling in patients that have recently had cancer and/or surgery involving the lymph nodes usually indicates lymphedema. Following a physical exam, imaging tests may be used to determine the cause of swelling. During the physical exam, the healthcare provider will ask about a family history of swelling and any past illnesses and medications taken. The swollen arm or leg may be measured and compared to the other arm or leg.

• The severity of lymphedema is classified using a grading scale. In patients with stage I lymphedema, the arm or leg is swollen and feels heavy. Pressing on the swollen area leaves an imprint. This stage of lymphedema may get better on its own. In stage II lymphedema, the arm or leg is swollen and feels spongy. Tissue fibrosis may develop, which causes the arm or leg to feel hard. Pressing on the swollen area does not leave an imprint. Stage III lymphedema, also called lymphostatic elephantiasis, is the most advanced stage. The swollen limb may be very large. Stage III lymphedema is rare in breast cancer patients.

• Computerized tomography (CT): A CT scan produces images of the arm or leg in cross-sections and may show the areas of the lymphatic system that are blocked.

• Doppler ultrasound: A Doppler ultrasound assesses blood flow and pressure by bouncing high-frequency sound waves off of red blood cells.

• Lymphangiography: A lymphangiogram is an X-ray of the lymph nodes and lymph vessels where a dye or radioactive compound is injected into the body to enable viewing of the lymph nodes and lymph vessels, which are not usually seen on a normal X-ray.

• Lymphoscintigraphy: In a lymphoscintigraphy, a radioactive dye is injected and then scanned by a machine. A healthcare provider may then watch the dye moving through the lymph vessels and check for blockages.

• Magnetic resonance imaging (MRI): An MRI scan images the tissues in the affected arm or leg to check for lymph accumulation.

Complications

• General: Complications of lymphedema include skin infections, extreme swelling (elephantiasis), and cancer of the lymph vessels. Many of the complications may be prevented by vigilant skin care and hygiene.

• Depression: It may not be possible or comfortable to perform normal tasks using the affected arm or leg. This may lead to frustration and depression.

• Elephantiasis: Elephantiasis occurs when the affected arm or leg becomes so hardened with thickened skin that is difficult to move it. The skin on the affected arm or leg may be affected by persistent ulcers and repeated infections.

Treatment

• General: Lymphedema cannot be cured, as damage to the lymphatic system cannot be repaired. Treatment focuses on minimizing the swelling, controlling pain, and preventing complications. Treatment includes physical methods, such as compression stockings, and medicines. In cases of severe lymphedema, surgery may be performed to remove excess tissue from the affected area. Medications are generally not used. However, antibiotics may be used to treat and prevent infections. Diuretics or anticoagulants (blood thinners) should be avoided.

• Complete decongestant therapy (CDT): Complete decongestant therapy (also called combined physical therapy) occurs when several treatments for lymphedema are combined, such as massage, bandaging, exercises, and skin care. This combination therapy should be managed by a specialist and should be avoided in patients that have high blood pressure, diabetes, paralysis, heart failure, blood clots, or serious infections.

• Compression devices: Compression devices, also called pneumatic compressions, are sleeves that are worn over the affected arm or leg. The sleeve is connected to a pump that occasionally inflates the sleeve, putting pressure on the affected arm or leg, gently moving lymph fluid away from the fingers or toes. This prevents swelling by preventing fluid from building up in the arm or leg.

• Compression garments: Compression garments (also called pressure garments, compression sleeves, and lymphedema sleeves or stockings) are long sleeves or stockings that are made to compress the arm or leg, encouraging the flow of lymph fluid out of the affected arm or leg and preventing it from building up in the future. A patient may wrap the arm or leg with bandages or have custom-made compression garments. Wearing a pressure garment during exercise may help prevent further swelling in an affected limb. It is important to use pressure garments during air travel, because lymphedema can become worse at high altitudes.

• Exercise: Studies have shown that slow, carefully controlled exercise may prevent the development of lymphedema by encouraging the movement of the lymph fluid out of the affected arm or leg. In breast cancer survivors, upper-body exercise does not increase the risk that lymphedema will develop. Exercise should be designed and monitored by a physical therapist. Patients who have lymphedema should wear a well-fitting pressure garment. Avoid strenuous activity until complete recovery from radiation or surgery. Exercise should be stopped if swelling worsens. If exercise is stopped for a week or longer, it should be started again at a low level and increased slowly.

• Laser therapy: Laser therapy may help decrease lymphedema swelling and skin hardness after a mastectomy (breast removal).

• Massage: A special massage technique called manual lymph drainage (MLD) helps to drain excess lymph fluid out of the affected arm or leg. MLD involves special gentle hand strokes on the affected limb to move the lymph fluid to healthy lymph nodes, where it can drain. It should be performed by a professional with experience with lymphedema. Patients can be taught to do this type of massage therapy themselves. Massage should be avoided in patients that are undergoing radiation therapy, or have a skin infection, fever, active cancer, blood clots, or congestive heart failure, or where the skin is cut or wounded.

• Skin care: A cream or lotion should be used to keep the skin moist. Small cuts or breaks in the skin should be cleaned promptly and treated with an antibacterial ointment. Sunscreen and shoes should be used when outdoors. Sharp objects should be avoided. An electric razor should be used when shaving, gloves should be worn during gardening or cooking, and a thimble should be used when sewing.

• Surgery: In cases of severe lymphedema, surgery may be performed to remove excess tissue from the affected area. Surgery reduces severe swelling but does not cure lymphedema. Types of surgery include liposuction, removal of abnormal lymphatic tissue, and a transplant of normal lymphatic tissues to areas with abnormal lymphatic drainage. On rare occasions, a surgeon may bypass abnormal lymph tissue using vein grafts.

• Weight loss: In patients who are overweight, lymphedema related to breast cancer may improve with weight loss.

Integrative Therapies

• Unclear or conflicting scientific evidence:

• Aromatherapy: Aromatherapy refers to different therapies that use essential oils. These oils are either sprayed in the air, inhaled, or applied to the skin in managing lymphedema and lymphedema-related conditions. Clinical aromatherapy is used in conjunction with complete decongestive therapy (CDT), which is a physically complete approach of managing lymphedema. A blend of frankincense, grapefruit, hyssop, and lavender can be used to soften and release scars and improve circulation. Clinical research has evaluated the effects of self-massage and skin care using massage cream with aromatherapy oils in lymphedema patients. Although self-massage and skin care significantly improved symptom relief and well-being, it only slightly and nonsignificantly reduced limb volume. The aromatherapy oils did not seem to affect these measurements. Additional research is needed in this area.

• Carrier oils often used for lymphedema conditions contain proteins and can increase lymphatic load. These carriers should be avoided; examples are grape seed oil and safflower oil.

• Hesperidin: Hesperidin is a flavonoid found in citrus fruits such as lemons, oranges, and grapefruits before they ripen. Most studies of the effects of hesperidin have used combination products that contain other ingredients in addition to hesperidin. Hesperidin-containing products may have beneficial effects on blood vessels. A combination product (Cyclo 3 Fort®) containing Ruscus aculeatus root extract, hesperidin methyl chalcone (HMC), and ascorbic acid may help reduce swelling of the arm in women who have been treated for breast cancer. Additional research on the effect of hesperidin alone is needed.

• Use cautiously in patients with diabetes or hypoglycemia and those taking drugs, herbs, or supplements that affect blood sugar. Use cautiously in patients with bleeding disorders or those taking drugs that may increase the risk of bleeding. Use cautiously in patients with musculoskeletal disorders or those taking muscle relaxants. Use cautiously in patients prone to headaches. Use cautiously in patients with gastrointestinal disorders or those taking antacids or agents to treat nausea and vomiting. Use cautiously in patients taking agents processed by the liver's cytochrome P450 enzyme system, sedatives, CNS depressants, antiseizure agents, or cardiovascular agents, such as beta-blockers, calcium channel blockers, or agents that lower blood pressure. Avoid in patients with a known allergy or hypersensitivity to hesperidin, and hesperidin-containing foods and supplements, such as citrus. Avoid in pregnant or breastfeeding women.

• Iodine: Iodine is an element that is needed by the human body to make thyroid hormones. Footcare with Betadine®, a tincture of iodine, may help in the management of filarial lymphedema.

• Reactions can be severe, and deaths have occurred with exposure to iodine. Avoid iodine-based products if allergic or hypersensitive to iodine. Do no use for more than 14 days. Avoid Lugol solution and saturated solution of potassium iodide (SSKI, PIMA) with hyperkalemia (high amounts of potassium in the blood), pulmonary edema (fluid in the lungs), bronchitis, or tuberculosis. Use cautiously when applying to the skin, because it may irritate or burn tissues. Use sodium iodide cautiously with kidney failure. Avoid sodium iodide with gastrointestinal obstruction. Iodine is safe in recommended doses for pregnant or breastfeeding women. Avoid povidone-iodine for perianal preparation during delivery or postpartum antisepsis.

• Moxibustion: Moxibustion is a therapeutic method in traditional Chinese medicine, classical (five-element) acupuncture, and Japanese acupuncture, in which an herb, usually mugwort (Artemisia vulgaris), is burned above the skin or on the acupuncture points for the purpose of introducing heat into an acupuncture point to alleviate symptoms. It may be applied in the form of a cone, stick, or loose herb, or it may be placed on the head of an acupuncture needle, to manipulate the temperature gradient of the needle. Heat is believed to be a potent force for influencing the flow or qualities of qi through the body. Preliminary evidence from one small study suggests that moxibustion and acupuncture may reduce symptoms of lymphedema caused by intrapelvic lymph node dissection in gynecological cancer. However, the evidence is insufficient to make concrete recommendations at this time.

• Use cautiously over large blood vessels and thin or weak skin. Avoid with aneurysms, any kind of "heat syndrome," cardiac disease, convulsions or cramps, diabetic neuropathy, extreme fatigue and/or anemia, fever, inflammatory conditions, or allergic skin conditions. Avoid over ulcerated sores or skin adhesions. Avoid areas with an inflamed organ and inflamed areas in general; contraindicated acupuncture points; and the face, genitals, head, and nipples. Avoid in patients who have just finished exercising or taking a hot bath or shower. It is not advisable to bathe or shower for up to 24 hours after a moxibustion treatment. Use caution with elderly people with large vessels. Avoid if pregnant or breastfeeding.

• Physical therapy: Various types of physical therapy have been employed in the treatment of lymphedema, such as complex physical therapy (CPT), self home maintenance therapy (wearing a bandage or elastic garment and exercise), and pneumatic compression. No high-quality trials have shown the benefit of these therapies over other therapies or controls. Physical therapy has also been used in combination with sodium selenite application. Not all physical therapy programs are suited for everyone, and patients should discuss their medical history with a qualified healthcare professional before beginning any treatments. Physical therapy may aggravate preexisting conditions. Persistent pain and fractures of unknown origin have been reported. Physical therapy may increase the duration of pain or cause limitation of motion. Pain and anxiety may occur during the rehabilitation of patients with burns. Both morning stiffness and bone erosion have been reported in the physical therapy literature, although causality is unclear. Erectile dysfunction has also been reported. Physical therapy has been used in pregnancy, and although reports of major adverse effects are lacking in the available literature, caution is advised nonetheless. All therapies during pregnancy and breastfeeding should be discussed with a licensed obstetrician/gynecologist before initiation.

Prevention

• General: Primary lymphedema is inherited and cannot be prevented. Patients at risk of developing secondary lymphedema can take measures to help prevent it. Patients having tumors removed and/or radiation therapy should find out if the procedure will involve the lymph nodes or lymph vessels. The chance of improving the condition is better if treatment begins early. Untreated lymphedema can lead to problems that cannot be reversed.

• Avoid heating the affected arm or leg: Do not apply a heating pad to the swollen arm or leg.

• Cleanliness: Keep the skin on the affected arm or leg clean and check daily for any changes or breaks in the skin, which could lead to an infection. Fingernails should be kept trimmed and clean.

• Do not restrict blood flow: Body fluids should be able to move freely, especially through an affected limb or in areas where lymphedema may develop. For this reason, the following should be avoided: crossed legs, tight jewelry or clothing, carrying purses or bags over the affected arm, elastic bandages or blood pressure cuffs on the affected arm or leg, or swinging the affected arm or leg in rapid circles. The sitting position should be adjusted every 30 minutes.

• Elevation of the affected arm or leg: The affected arm or leg should be raised higher than the heart when possible.

• Exercise: Studies have shown that slow, carefully controlled exercise may prevent the development of lymphedema. In breast cancer survivors, upper-body exercise does not increase the risk that lymphedema will develop. Avoid strenuous activity until complete recovery from radiation or surgery.

• Foot care: Toenails should be cut straight across to prevent the risk of infection caused by ingrown toenails. A podiatrist (foot doctor) may be seen for foot infections. Feet should be kept clean and dry. Socks should be made of cotton.

• Prevent infection: Bacteria can enter the body through broken skin, such as a cut, scratch, burn, or insect bite. Avoid blood draws and vaccinations in the affected limb. Scratching the affected skin should be avoided. Fluid that is built up in arms or legs affected by lymphedema provides an environment where bacteria are able to grow. Contact a healthcare provider if signs of infection are found, such as redness, pain, swelling, heat, fever, or red streaks below the surface of the skin.

• Sentinel lymph node dissection: Sentinel lymph node dissection is a surgical procedure where the very first lymph node that filters fluid draining away from the area of the breast that contained the breast cancer is removed. In some women, this procedure may prevent lymphedema from developing after surgery for breast cancer.

Author Information

• This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography

Natural Standard developed the above evidence-based information based on a thorough systematic review of the available scientific articles. For comprehensive information about alternative and complementary therapies on the professional level, go to www.naturalstandard.com. Selected references are listed below.

1. Barclay J, Vestey J, Lambert A, et al. Reducing the symptoms of lymphoedema: is there a role for aromatherapy? Eur J Oncol Nurs. 2006 Apr; 10(2):140-9. View Abstract

2. Cheville AL, McGarvey CL, Petrek JA, et al. Lymphedema Management. Semin Radiat Oncol. 2003 Jul;13(3):290-301. View Abstract

3. Kanakura Y, Niwa K, Kometani K, et al. Effectiveness of acupuncture and moxibustion treatment for lymphedema following intrapelvic lymph node dissection: a preliminary report. Am J Chin Med 2002;30(1):37-43. View Abstract

4. Lymphatic Research Foundation. www.lymphaticresearch.org

5. Natural Standard: The Authority on Integrative Medicine. www.naturalstandard.com

6. Ogawa Y. Lymphedema. Nippon Rinsho. 2007 Jan;65(1):83-8. View Abstract

7. Rockson, SG. Lymphedema. Curr Treat Options Cardiovasc Med. 2006 Apr;8(2):129-136. View Abstract

8. Schook CC, Mulliken JB, Fishman SJ, et al. Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. Plast Reconstr Surg. 2011 Apr;127(4):1571-81. View Abstract

9. Tiwari A, Cheng KS, Button M, et al. Differential Diagnosis, Investigation, and current treatment of lower limb lymphedema. Arch Surg. 2003 Feb;138(2):152-61. View Abstract

10. Vascular Disease Foundation. www.vdf.org

11. Warren AG, Brorson H, Borud LJ, et al. Lymphedema: A Comprehensive Review. Ann Plast Surg. 2007 Oct;59(4):464-72 View Abstract