About Lymphedema
Lymphedema : Etiology, Prevention, Treatment
Introduction:
Lymphedema is very common, affecting at least 3 million Americans. Some patients develop it after surgery or radiation therapy for various cancers (breast, prostate, bladder, uterus, melanoma, lymphoma) in which case it is referred to as secondary lymphedema. Other patients develop it without obvious cause at different stages in life (primary lymphedema), and still others develop it after trauma or deep vein thrombosis. In third world countries, parasites account for millions of cases.
Lymphedema is serious because of the disability it causes, because of the cosmetic deformities that are difficult to hide, because of the frequent complications that occur (cellulitis, lymphangitis, lymphorrhea, skin thickening, etc.) and because of the continuous worsening of the condition in untreated patients. It is also serious because of the pervasive lack of medical expertise in diagnosis and treatment of the condition and the tendency of clinicians to trivialize lymphedema in patients who have been treated for cancer.
Anatomy and Pathophysiology:
Lymph vessels are arranged in superficial and deep systems. The superficial vessels are very small, arranged in intricate networks in the dermis and have no valves. They connect with a deeper layer of larger diameter vessels that do contain valves and run alongside superficial subcutaneous veins. These in turn are linked to a still deeper layer of vessels that run just superficial to the muscle fascia and further help in the transport of lymph to the venous angle of the neck where the lymph system joins the blood circulation. There are few lymphatics within the muscle compartments; lymphedema is luckily not an illness that affects these deeper muscle layers.
Whenever the lymphatic transport system is impaired or undeveloped, large molecules, proteins in particular, accumulate in increasing quantity in the interstitial spaces. This causes an increase in colloid osmotic pressure and additional quantities of fluid in the interstitium: lymphedema.
Thus, the failure in lymphedema is that the lymph transport system is unable to carry protein, lipid and other molecules that are too large to be reabsorbed by the venous system.
We could also say that in lymphedema the patient experiences the swelling of an arm, leg, or other body part as a result of excess interstitial fluid rich with stagnant protein, a situation that leads to chronic low-grade inflammation, increasing fibrosclerotic changes in the tissues and susceptibility to cellulitis, erysipelas and lymphangitis.
Incidence:
About 50-70% of patients who have had axillary node surgery will develop lymphedema (1). Patients who have had axillary surgery plus radiation therapy are at even higher risk. It is estimated that at least 1-2 million breast cancer survivors are alive today after lymphadenectomy and that 400,000 of them cope daily with the disfigurement, discomfort and disability of arm and hand swelling (2).
In six different recent reports from three different countries on the incidence of lymphedema in patients who had different breast cancer treatments, Petrek and Lerner noted that the incidence of lymphedema is about 20% (16-26%) (3). They also noted that the incidence remains the same or higher in patients having breast conservation surgery because of the postoperative radiation reaching the axilla. Axillary radiation to the dissected axilla was a strong predictor of lymphedema in all studies that evaluated this issue.
The true numbers of patients suffering from any form of lymphedema is unknown. Based on the numbers above and other statistics, we estimate the incidence of lymphedema in the United States as follows:
- Secondary Lymphedema: 2-3 million
- Primary Lymphedema: 1-2 million
Presentation:
Whenever lymphatic load exceeds lymph transport capacity, lymphedema develops. The onset is gradual in some patients and sudden in others. The swelling usually occurs for the first time after a traumatic event, an infection in the body part at risk, great physical exertion or an airplane trip.
In secondary lymphedema, post-mastectomy lymphedema for instance, the lymphedema may affect the hand and forearm at first and later involve the shoulder and the ipsilateral trunk quadrant. Primary lymphedema, however, affects women more often than men and usually affects the lower extremities.
Once established, lymphedema cannot be cured. With treatment, however, the affected limb can be restored to normal size and contour, and with good care, the usual progression of the lymphedema can be avoided. Without treatment or with inadequate treatment, the lymphedema worsens year by year, the limb becomes more and more fibrotic and function and cosmetic appearance worsen.
Prevention:
Many lymphedema cases can be prevented. "At risk" patients must be taught how to avoid getting the condition. This group includes everyone who has had a regional node dissection or radiation therapy to a lymph-node bearing area: neck, axilla, groin, pelvis, retroperitoneum. If you are interested in further information about prevention, contact one of our offices to set up a consultation and learn how.
Patients in these categories as well as patients with primary lymphedema must be taught the importance of good skin and nail care, to avoid trauma and insect bites in the area at risk, to take special precautions on long airplane flights, to avoid hot temperatures (climate, sauna, baths), and to be sure that they do not have blood drawn, IV's started or blood pressure taken in the limb at risk (see pp. 899-900 in "Diseases of the Breast", Lippincott-Raven, 1996).
Treatment:
Lymphedema treatments vary from place to place.
The three basic treatments are:
- Pneumatic pumps
- Surgery of some kind
- Complete Decongestive Physiotherapy (CDP)
Note: There are no medications that are very useful in treating lymphedema. Diuretics are not recommended for lymphedema because their effect is transitory and over time, they lead to increased colloid osmotic tissue pressure, increased fibrosis and increased swelling.
Benzopyrones are being essayed in various countries. They lack FDA approval in the US and have serious adverse side effects. Furthermore, there are no long-term studies on the use of benzopyrones. Such studies are vital if one is treating a chronic condition that might require the use of these agents for many years.
Elastic sleeves and stockings are adjunctive measures that serve to contain the lymphedema. They are valuable in conjunction with one of the three basic treatment methods mentioned above.
Pneumatic pumps have been used for more than 20 years. Most patients find them to be ineffective and cease using them voluntarily because of disillusionment and lack of beneficial effect. If they do have value, this is seen only in early (Stage 1) lymphedema, prior to the buildup of fibrous tissue that develops in more advanced stages.
Surgical treatment for lymphedema patients is not recommended. The results are generally unpredictable, many of the procedures are long and require many hours of general anesthesia and the scarring that results interferes with later therapy. Some centers are using microsurgical procedures and the long term results of such procedures are awaited. Few surgeons have any expertise in lymphedema and it is wrong for any surgeon to do one or two cases a year and hope for a good result.
CDP, sometimes referred to as Complex Decongestive Physiotherapy or Combined Physiotherapy, dates back to 1882. Dr. Foeldi (5) popularized the procedure in the late 1970's and Dr. Lerner introduced it in the US a decade later.
CDP is a safe, rapid and very effective treatment. It consists of meticulous skin care and a lymphatic massage technique known as Manual Lymph Drainage. This "massage" phase is immediately followed by a multi-layered compression bandaging using minimally-elastic bandages and, once completed, by a series of gentle exercises designed to improve lymph flow and lymphatic microcirculation.
The main concept of CDP is to improve central lymph flow by opening non-functioning lympho-venous anastomoses and by stimulating collateral lymphatic channels to drain the swollen area into adjacent areas where lymph flow is normal.
CDP represents a major advance in lymphedema treatment. The swollen limb(s) are reduced to normal or close to normal in most every case. Post-treatment lymphoscintigraphy consistently demonstrates that lymph flow is more rapid and lymph back flow is decreased.
The real importance of CDP is that patients can be taught the CDP method during their treatment, that they can continue to improve at home without doctors or hospitals, and that the reduction in volume achieved can be maintained and even improved over time. This reduction in lymphedema improves the quality of life and reduces the number of episodes of cellulitis and other life-threatening infections. Lastly, CDP is undoubtedly the most cost-effective treatment available for lymphedema.
BIBLIOGRAPHY:
- The Breast Cancer Digest, National Cancer Institute, p.78, April 1984
- Petrek, JA and Lerner, R in Diseases of the Breast, Lippincott-Raven, 1996
- Idem
- Giuliano, AE: John Wayne Cancer Institute of St. John's Health Center, Santa Monica, CA
- Foeldi E, Foeldi M, Clodius L: The Lymphedema Chaos: A Lancet, Ann Pl. Surg. 22: 505-515, 1988