Lymphedema is an accumulation of lymphatic fluid in the interstitial tissue that may causes swelling in an extremity such as arm, legs, face abdomen or genitals.  Lymphedema can develop when lymphatic vessels are missing or impaired (primary), or when lymph vessels are damaged or removed (secondary).  The most common cause of Lymphedema is the result of surgery, lymph node removal and the side effects of radiation treatment.

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.

Lymphedema should not be confused with edema resulting from venous insufficiency.  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.

Aircraft flight may also be linked to the onset of lymphedema in patients post-cancer surgery (likely due to the decreased cabin pressure).  It is advisable to wear a compression garment (sleeve, stocking) when you fly, even if you do not have 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.

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.

Contraindications

1. Post-cancer surgery lymphedema patients who experience a sudden marked increase of swelling should immediately cease treatment and be checked by their physician.  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.
2. 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. During treatment, these patients should be followed closely and regular laboratory tests should be performed (prothrombentime).
3. 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.
4. If pain is present, discontinue all treatment until the pain subsides or the underlying cause has been determined.