Lymphedema Treatment Services, Inc., Lauderhill, Fla., USA.
May 2004
Lymphedema is a complex, unappreciated, and chronically progressive medical condition. Appropriate management requires a correct diagnosis and an understanding of the underlying pathophysiology. Fluid retaining conditions and mechanical problems both can lead to edema. Diuretic therapy may be correct for certain patients but can be harmful to others. Although lymphedema is not curable, it can be controlled and managed.1,2 Multiple medical and surgical subspecialties are usually involved in treatment.
Lymphedema is an increasing medical problem in this country. This is partly the result of medical personnel having little or no training or understanding of the condition. Consequently, patients are frequently left untreated or treated incorrectly. Increasing longevity leads to complications that are part of the natural history of lymphedema and aging. These complications never had time to develop to a point of clinical significance in the past. However, now they are seen in greater numbers. Likewise, dermatological problems increase with age. Edema in elderly patients with loss of elasticity, collagen, and vascular supply now result in problems, the frequency of which has not been seen in the past.
Most lymphedema in this country is the result of surgical treatment for a malignant disease. Because malignancy is increasingly curable, the latent potential for developing lymphedema is a growing medical problem.
Skin care always has been an important part of lymphedema treatment. With the skin changes of aging, even basic skin care and standard treatments such as manual lymph drainage, compression wrapping, and the use of stockings and sleeves are becoming difficult or impossible in patients with certain dermatological/medical disorders. Further complicating matters is the fact that skin ulcerations can have multiple causes. What is clear, however, is that skin lesions and ulcerations, regardless of origin, are difficult or impossible to heal in an edematus extremity. Yet therapy to relieve edema in the presence of wounds may not be possible, creating a "catch 22."
The lymphatic and venous systems are closely related and interact with each other. Problems in one area will inevitably lead to problems in the other. Both lead to a final common pathway. The target organ is the skin. Often, the result is the need for wound care, but hopefully advancement to this stage can be prevented.
The Lymphatic System
Lymphatic "circulation" is a misnomer. Normal circulation involves arteries and veins with the heart the driving force. In true circulation, the arteries deliver nutrients and oxygen to the tissues and capillaries on the venous side return blood back to the heart and lungs to be replenished. Without going into membrane physiology and physics at any length, fluid constantly shifts in and out of the standard circulation to interstitial spaces outside the vascular system. This is the result of physical laws involving pressure gradients, colloidal osmotic pressure, and a concept called ultrafiltration. To oversimplify things, Nature likes to equalize things across a barrier. Approximately 10% of the normal circulatory fluid filters into the interstitial spaces. Our bodies are endowed with additional vessels known as lymphatics. These vessels begin in the tissues. They act like garbage pails, picking up large protein molecules and other materials which, because of their size, are unable to filter back into the normal venous circulation. This is actually a half "circulation" that lies in close conjunction with the venous system; it is quite intricate and complex. The fluid movement in the lymphatic system is not dependent on the pumping of the heart. The smaller lymphatics lead to larger ones that have smooth muscle and neurological connections.
Lymphangions are a segment of the lymphatic system. These vessels have valves and stretch receptors that contract and push fluid toward the heart. In addition, pulsations from nearby arteries and muscle contractions help propel lymphatic fluid back to the heart. On its course, lymphatic fluid passes through a series of lymph nodes, is purified, and ultimately returns to the general circulation.
Normally at rest, flow in the lymphatic system is minimal, but if the lymphatic workload is increased, the lymphatic circulation is capable of increasing its activity; in this manner, it acts as a self-contained pacemaker. Aside from its garbage pail nature, the lymphatic system performs a central immunological function. This is beyond the scope of this article.
Lymphedema, then, can develop for two main reasons: 1) disease, malfunction, or maldevelopment of the lymphatic system that renders it unable to handle a normal lymphatic load [low output]; or 2) a normal lymphatic system that is overwhelmed, usually the result of other medical conditions. In either case, the result will be increased protein and fluid in the interstitial spaces and the inability to absorb this fluid. Because this fluid is high in protein and other large molecules, it tends to draw additional fluid into these extra cellular spaces. The end result is edema, and this scenario sets in motion an inflammatory response leading to fibrosis and disruption of vascular integrity. The ultimate victim of all of this is the skin, not to mention the patient.
Lymphedema.
Edema, regardless of origin, ultimately affects the microcirculation. Tissue oxygenation and nutrition are effected. Changes in skin color are well known and common. With lymphedema, increased fibrosis, hardening of the skin, lobulations, and changes typical of elephantiasis occur. Blistering, weeping, and breakdown of the skin also are common and lead to cellulitis, further damaging the lymphatic system and microcirculation. In summary, this becomes a self-perpetuating nightmare. When skin breaks down and ulceration develops, especially in the presence of other diseases, the viability of an extremity can be threatened. Problems do not normally develop to this extent in typical lymphedema (either primary or secondary involving the upper extremities) except in the presence of malignant lymphedema. With venous insults such as axillary vein thrombosis, edema may develop, but the swelling is usually temporary. The same is apparently true for other upper extremity surgery involving the venous system alone, where edema of the upper extremity develops from disruption of the lymphatic system.3
The same is not true for skin disorders and ulcerations involving the lower extremities. The difference between arterial, venous, and stasis and pressure ulcers should be relatively easy to diagnose with a proper history and physical examination. As previously noted, it is medically important to determine the underlying reason for edema and ulcerations. Plastic surgical procedures including liposuction also can lead to lymphedema. In older people, especially those with "thin skin," ulceration of the skin can be difficult to heal. In these cases, even minimum trauma caused by compression stockings can lead to skin damage, making treatment difficult. Both edema therapy and preventive skin care are needed; creating a balance between both therapies can be challenging because, at times, they can be contradictory.
Chronic venous insufficiency (CVI) is a common cause of edema and ulceration of the skin. Lymphatic abnormalities exist in patients with CVI.4,5 Lymphedema is always present in advanced stages of CVI, and theories regarding the pathogenesis of skin ulceration in patients with this disorder are numerous.4,5 Skin grafts are unlikely to work on an edematous area. MLD, a gentle manual treatment that improves the activity of the lymph vessels and re-routes the lymph flow around the blocked areas into more centrally located lymph vessels that drain into the venous system, can reduce the pain, fibrosis, and postoperative morbidity in postsurgical cases.6 The bottom line is that ulcerations of the skin are not likely to heal in the presence of edema for whatever reason. Again, skin care, wound care, and edema must be addressed at the same time. While the authors' emphasis is on lymphedema, skin care always has been an important part of lymphedema treatment.
Treatment
Over the years, the authors have tried to emphasize the need for skin care in their patients. With the aging population, low pH moisturizing lotions and standard wound care methods are proving to be inadequate in patients whose skin has broken down or has that potential; hence, preventing the use of standard lymphedema treatment in many patients.
Recently, soft silicone technology products of various kinds (eg, Tendra, Mölnlycke Health Care, Newtown, Pa.) have been used according to patients' needs, type of wound, and amount of skin drainage Wound adherence has not been a problem and these products are easy to use. They have enabled clinicians to perform CDT, a comprehensive form of treatment for lymphedema involving physical techniques, compression wrapping, and patient education, as well as MLD, wrapping over the skin dressings. This has enabled lymphedema treatment; thereby, promoting wound healing while treating skin ulcerations. Silicone-based products also have been used in jeopardized skin as a preventive measure in conjunction with the usual wrappings and treatment for lymphedema. Thus far, this appears to be an effective approach.
Conclusion
Although patient numbers are small at the authors' facility and their experience may be somewhat anecdotal, lymphedema treatment in conjunction with soft silicone technology appears to be a workable combination and merits further investigation. - OWM
Acknowledgment
The authors are grateful to Anita King and Leora Krupnick for their invaluable assistance in preparing this article, as well as Joachim Luther and The Academy of Lymphatic Studies. They especially thank Robert Lerner, MD, who has been and continues to be an inspiration to all.Addressing the Pain is made possible through the support of Molnlycke Health Care, Newtown, Pa.
References
1. King MJ. Lymphedema - the role of the physician. Contact the author.2. King MJ, DeFalco E. Multiple radio tapes and articles. Contact the author.3. Foldi M, Foldi E, Clodius. The lymphedema chaos. Ann Plast Surg. 1989;22:505.4. Tehrani H. International Varicose Vein Congress. Miami Vein Center. Key Biscayne, Fla. September 21, 2003.5. Hartmann M. CVI. In: Weissleder, Schuchardt. Lymphedema - Diagnosis and Therapy. Viavital Verlag GmbH; 2001:266-2826. Cass LA, De Poli P. Manual Lymphatic Drainage Therapy: An Integral Component of Post-Operative Care in Plastic Surgery Patients. Abstract lecture. Northwestern University of Medical School.
Ostomy Wound Management
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