What Is Lymphedema?
Lymphedema is an accumulation of lymphatic fluid in the interstitial tissues 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 lymph systems 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 or in adulthood, all from unknown causes, or associated with vascular anomalies.
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.
Aircraft flight has also been linked to the onset of lymphedema in patients post-cancer surgery (likely due to the decreased cabin pressure).
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).
- Approximately half of all cancer patients develop lymphedema.
- It is estimated that 83% to 90% of all patients with lymphedema are women.
- Lymphedema is one of the most misdiagnosed / undiagnosed disease processes in America.
- Is the skin smooth and swollen?
- Does the swelling affect only, or mainly, one leg or arm?
- Did you have an operation or radiology before the swelling started?
- Have you ever injured that part of the body?
- Are the backs of your feet or hands swollen?
- Are your natural skin folds deeper?
If you answered yes to 2 or more of these questions contact your healthcare practitioner.
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.
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 to 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.
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:
- manual lymphatic drainage
- proper skin care & diet
- compression garments (sleeves or stockings as well as other alternative approaches
- remedial exercises
- self-manual lymphatic drainage & bandaging if instruction is available
- Post-cancer surgery lymphedema patients who experience a sudden marked increase of swelling should immediately cease treatment and be checked by their physician. Tumor growth can block the lymphatic flow causing a worsening of the condition. Be sure to discuss this treatment with your doctor.
- Patients with a sudden onset of lymphangitis (infection) should immediately discontinue treatment 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. During treatment, these patients should be followed closely and regular laboratory tests should be performed.
- 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.
Revised © 2005 National Lymphedema Network. Permission to print and duplicate this page in its entirety for educational purposes only, not for sale. All other rights reserved.
The information above is adapted from Lymphedema: An Information Booklet, a classic NLN publication now in its 8th edition. This 16 page booklet addresses the lymphatic system, causes of lymphedema, symptoms, lymphangitis (infection), lymphedema risk reduction, treatments, contraindications, diagnostic studies, special warnings and reimbursement issues. Booklets are available through the NLN.