Lymphedema is an accumulation of lymphatic fluid in the interstitial tissue that causes swelling, most often in the arm(s) and/or leg(s), and occasionally in other parts of the body. Lymphedema can develop when lymphatic vessels are missing or impaired (primary), or when lymph vessels are damaged or lymph nodes removed (secondary).
When the impairment becomes so great that the lymphatic fluid exceeds the lymphatic transport capacity, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid not only causes tissue channels to increase in size and number, but also reduces oxygen availability in the transport system, interferes with wound healing, and provides a culture medium for bacteria that can result in lymphangitis (infection).
Lymphedema should not be confused with edema resulting from venous insufficiency, which is not lymph-edema. However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder which is treated in the same way as lymphedema.
What Causes Lymphedema?
Primary lymphedema, which can affect from one to as many as four limbs and/or other parts of the body, can be present at birth, develop at the onset of puberty (praecox) or in adulthood (tarda), all from unknown causes, or associated with vascular anomolies such as hemangioma, lymphangioma, Port Wine Stain, Klippel Trenaury.
Secondary lymphedema, or acquired lymphedema, can develop as a result of surgery, radiation, infection or trauma. Specific surgeries, such as surgery for melanoma or breast, gynecological, head and neck, prostate or testicular, bladder or colon cancer, all of which currently require removal of lymph nodes, put patients at risk of developing secondary lymphedema. If lymph nodes are removed, there is always a risk of developing lymphedema.
Secondary lymphedema can develop immediately post-operatively, or weeks, months, even years later. It can also develop when chemotherapy is unwisely administered to the already affected area (the side on which the surgery was performed) or after repeated aspirations of a seroma (a pocket of fluid which occurs commonly post-operatively) in the axilla, around the breast incision, or groin area. This often causes infection and, subsequently, lymphedema.
Another cause of lower extremity lymphedema is that resulting from the use of Tamoxifen. This medication can cause blood clots and subsequent DVT (deep venous thrombosis).
Radiation therapy, used in the treatment of various cancers and some AIDS-related diseases (such as Kaposi-Sarcoma), can damage otherwise healthy lymph nodes and vessels, causing scar tissue to form which interrupts the normal flow of the lymphatic fluid. Radiation can also cause skin dermatitis or a burn similar to sunburn. It is important to closely monitor the radiated area for any skin changes, such as increased temperature, discoloration (erythema) or blistering which can lead into the development of lymphedema. Be sure to keep the area soft with lotion recommended by your radiation oncologist.
Lymphedema can develop secondary to lymphangitis (an infection) which interrupts normal lymphatic pathway function. A severe traumatic injury in which the lymphatic system is interrupted and/or damaged in any way may also trigger the onset of lymphedema. Although extremely rare in developed countries, there is a form of lymphedema called Filariasis which affects as many as 200 million people worldwide (primarily in the endemic areas of southeast Asia, India and Africa). When the filarial larvae from a mosquito bite enters the lymphatic system, these larvae mature into adult worms in the peripheral lymphatic channels, causing severe lymphedema in the arms, legs and genitalia (also known as Elephantiasis).
Symptoms of Lymphedema
Lymphedema can develop in any part of the body or limb(s). Signs or symptoms of lymphedema to watch out for include: a full sensation in the limb(s), skin feeling tight, decreased flexibility in the hand, wrist or ankle, difficulty fitting into clothing in one specific area, or ring/wristwatch/bracelet tightness. If you notice persistent swelling, it is very important that you seek immediate medical advice (and get at least one second opinion) as early diagnosis and treatment improves both the prognosis and the condition.
Lymphedema develops in a number of stages, from mild to severe,referred to as Stage 1, 2 and 3:
Stage 1 (spontaneously reversible): Tissue is still at the “pitting” stage, which means that when pressed by fingertips, the area indents and holds the indentation. Usually, upon waking in the morning, the limb(s) or affected area is normal or almost normal size.
Stage 2 (spontaneously irreversible): The tissue now has a spongy consistency and is “non-pitting,” meaning that when pressed by fingertips, the tissue bounces back without any indentation forming). Fibrosis found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size.
Stage 3 (lymphostatic elephantiasis): At this stage the swelling is irreversible and usually the limb(s) is/are very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called “debulking” at this stage.
When lymphedema remains untreated, protein-rich fluid continues to accumulate, leading to an increase of swelling and a hardening or fibrosis of the tissue. In this state, the swollen limb(s) becomes a perfect culture medium for bacteria and subsequent recurrent lymphangitis (infections). Moreover, untreated lymphedema can lead into a decrease or loss of functioning of the limb(s), skin breakdown, chronic infections and, sometimes, irreversible complications. In the most severe cases, untreated lymphedema can develop into a rare form of lymphatic cancer called Lymphangiosarcoma (most often in secondary lymphedema).
Signs and symptoms of lymphangitis (infection) may include some or all of the following: rash, red blotchy skin, itching of the affected area, discoloration, increase of swelling and/or temperature of the skin, heavy sensation in the limb (more so than usual), pain, and in many cases a sudden onset of high fever and chills.
Treatment for infections: immediately discontinue ALL current lymphedema treatment modalities (including manual lymphatic drainage, bandaging, pumps, wearing of compression garments) and contact your physician as soon as possible. The antibiotics of choice for these types of lymphatic infections depend on the cultures.
Treatments for Lymphedema
Planning the treatment program depends on the cause of the lymphedema. For example: If the initial signs and symptoms of swelling are caused by infection (redness, rash, heat, blister or pain may indicate an infection), antibiotics will first need to be prescribed. Treating an infection often reduces some of the swelling and discoloration.
If the lymphedema is not caused by infection: Depending on the severity of the lymphedema, the recommended treatment plan should be determined using an approach based on the Complex Decongestive Therapy (CDT) methods which consist of: a) manual lymphatic drainage; b) bandaging; c) proper skin care & diet; d) compression garments (sleeves, stockings, devices such as Reid Sleeve, CircAid, Tribute, as well as other alternative approaches); e) remedial exercises; f) self-manual lymphatic drainage & bandaging, if instruction is available; g) continue to follow prophylactic methods at all times.
- Post-cancer surgery lymphedema patients who experience a sudden marked increase of swelling should immediately cease treatment and be checked by their physician for possible recurrent tumor or disease. Tumor growth can block the lymphatic flow causing a worsening of the condition. Although not yet proven in a controlled clinical study, many lymphedema specialists believe that patients with recurrent or metastatic disease should not undergo Complete Decongestive Therapy (CDT) in order not to promote the spreading of the cancer. Be sure to discuss this treatment with your doctor.
- Patients with a sudden onset of lymphangitis (infection) should immediately discontinue treatment (see page 4) until the infection is cleared. Patients with histories of vascular disease and who are taking anticoagulants, should have a Doppler and ultra-sound to rule out deep-venous thrombosis before being treated.
- Patients who have congestive heart failure must be monitored closely to avoid moving too much fluid too quickly, for which the heart may not be able to compensate.
- If pain is present, discontinue all treatment until the pain subsides or the underlying cause has been determined.
Also see information on VENOUS INSUFFICENCY and VENOUS SKIN ULCERS