Lymphedema is a very debilitating and progressive condition with no known cure. The unfortunate patient faces a lifelong struggle.
- In the United States, the highest incidence of lymphedema is observed following breast cancer surgery, particularly among those who undergo radiation therapy following axillary lymphadenectomy. Among this population, 10-40% develop some degree of ipsilateral upper extremity lymphedema.
- Worldwide, 140-250 million cases of lymphedema are estimated to exist, with filariasis being the most common cause.
Lymphedema may be classified as primary or secondary, based on underlying etiology.
Primary lymphedema represents a developmental abnormality of the lymphatic system. It is further subdivided into three forms depending on age at presentation. These conditions are most often sporadic, with no family history, and involve the lower extremity almost exclusively.
- Congenital lymphedema represents all forms that are clinically evident at birth. It accounts for 10-25% of all primary lymphedema cases. Females are affected twice as often as males. The lower extremity is involved 3 times more frequently than the upper extremity. Two thirds of patients have bilateral lymphedema, and this form may improve spontaneously with increasing age. There is a subset of patients with congenital lymphedema who have a familial sex-linked pattern of inheritance, which is termed Milroy disease. It accounts for 2% of primary lymphedema cases. The histology of the lymphatic channels often demonstrates an anaplastic pattern without subcutaneous lymphatic trunks but with normal dermal plexus.
- Lymphedema praecox is the most common form of primary lymphedema. It is also known as Meige disease. By definition, it becomes clinically evident after birth and before age 35 years. This condition accounts for 65-80% of all primary lymphedema cases and most often arises during puberty. Females are affected 4 times as often as males. About 70% of cases are unilateral, with the left lower extremity being involved more often than the right. Histologically, these patients are likely to demonstrate a hypoplastic pattern, with the lymphatics reduced in caliber and number.
- Lymphedema tarda does not become clinically evident until age 35 years or older. It is the rarest form of primary lymphedema and accounts for only 10% of cases. Histologically, patients are likely to demonstrate a hyperplastic pattern, with tortuous lymphatics increased in caliber and number. They often display absent or incompetent valves.
All three forms of primary lymphedema likely originate from a developmental abnormality that is present, but not always clinically evident, at birth. Some cases may become evident later in life when a triggering event or worsening of the condition causes the lymphatic transport capacity to exceed the volume of interstitial fluid formation, causing the patient to be unable to maintain normal lymphatic flow.
Secondary lymphedema represents an acquired dysfunction of otherwise normal lymphatics. Secondary lymphedema has an identifiable cause that destroys or renders inadequate the otherwise normal lymphatics.
- In the United States, it commonly results from damage or removal of regional lymph nodes through surgery (ie axillary dissection), radiation, infection, or tumor invasion or compression.
- Worldwide, the most common cause is filariasis, the direct infestation of lymph nodes by the parasite Wuchereria bancrofti.
- Other causes include vein stripping, peripheral vascular surgery, lipectomy, burns, burn scar excision, and insect bites.
- Recently, there has been an increasing number of morbidly obese patients presenting with lymphedema. "The rise in obesity is also contributing to the numbers of lymphedema patients. Ten years ago Dr. Fife didn't have any patients who weighed 500 pounds. Now, 2% or more of her patients are at this weight or heavier. Why morbid obesity leads to lymphedema is unknown, but one theory is that fat may block the system. "So as everyone gets fatter, we have more lymphedema," Dr. Fife said." --Dec 3, 2007 AMA Newsletter
If you are interested in the pathophysiology, please, read the eMedicine Article by Dr. Don R Revis, Jr, MD. You may also find more information at the links listed in the references below.
Patients present with varying degrees of severity, from mild swelling to severe disabling enlargement with potentially life-threatening complications. Lymphedema develops in a number of stages, from mild to severe:
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. The swollen limb(s) in this state becomes a perfect culture medium for bacteria and subsequent recurrent lymphangitis (infections). Furthermore, the 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).
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-- Intermittent pneumatic pump compression therapy may also be instituted on an outpatient basis or in the home. These manual lymphatic devices are most appropriate prior to fibrosclerotic evolution, and they assist in preventing fibrosclerotic evolution of the condition. These devices provide sequential active compression from distal to proximal, effectively milking the lymph from the extremity.
- Proper skin care & diet-- Meticulous hygiene is necessary to remove keratinaceous debris and bacteria. Cleanse the skin regularly and dry thoroughly. Regular inspection is necessary to identify any open wounds or developing cellulitis. Bland skin moisturizers applied conservatively may ameliorate cracking and furrowing.
- Compression garments (sleeves, stockings, devices such as Reid Sleeve, CircAid, Tribute, as well as other alternative approaches)-- should be worn continuously during the day. They may be removed at night when the extremity is elevated in bed, but they should be replaced promptly each morning. To encourage compliance, the elastic compression garments must fit appropriately. This requires that the garment be custom fit when the extremity is decompressed. The garment should also have graduated compression, increasing from distal to proximal, on the affected extremity.
- Remedial exercises
- Self-manual lymphatic drainage & bandaging, if instruction is available.
- Surgical treatment is palliative, not curative, and it does not obviate the need for continued medical therapy. It is reserved for those who do not improve with conservative measures or in cases where the extremity is so large that it impairs daily activities and prevents successful conservative management. The goals of surgical therapy are volume reduction to improve function, facilitation of conservative therapy, and prevention of complications.
If you are a patient with lymphedema:
Continue to follow prophylactic methods at all times to prevent further problems.
- Avoid exposure to extreme cold, which can be associated with rebound swelling, or chapping of skin.
- Avoid prolonged ( > 15 minutes) exposure to heat, particularly hot tubs and saunas.
- Avoid immersing limb in water temperatures above 102° F.
- Avoid constrictive clothing or jewelry that might have a tourniquet effect.
- If possible, avoid having blood pressure taken on the at-risk arm.
- Avoid prolonged standing or sitting or crossing your legs.
- Wear proper, well-fitting footwear.
- Skin Care - Avoid trauma/injury and reduce infection risk
- Keep extremity clean and dry.
- Apply moisturizer daily to prevent chapping/chaffing of skin.
- Attention to nail care; do not cut cuticles.
- Protect exposed skin with sunscreen and insect repellent.
- Use care with razors to avoid nicks and skin irritation.
- If possible, avoid punctures such as injections and blood draws.
- Wear gloves while doing activities that may cause skin injury (i.e., gardening, working with tools, using chemicals such as detergent).
- If scratches/punctures to skin occur, wash with soap and water, apply antibiotics, and observe for signs of infection (i.e. redness).
- If a rash, itching, redness, pain, increased skin temperature, fever or flu-like symptoms occur, contact your physician immediately.
FREE Lymphedema Alertband for the arm or leg are available here!
Lymphedema by Don R Revis, Jr, MD -- eMedicine Article
Lymphedema -- Vascular Web Home
Morbid Obesity And Lymphedema Management by Caroline E. Fife, MD, Susan Benavides, CLT-LANA, & Gordon Otto, PhD; LymphLink, Vol 19, No 3, July-Sept 2007
Lymphedema -- Mayo Clinic website
18 Steps to Prevention Revised: Lymphedema Risk-Reduction Practices By Saskia R.J. Thiadens, RN--National Lymphedema Network