Never without compression!
Lymphedema is a chronic progressive disease, for the time being without curative possibility. The most important therapeutic measure is a consistent compression therapy, supplemented by manual lymphatic drainage. The required medical compression stocking depends on the clinical picture and should approximate the effect of a short compression bandage.
If at the capillary level the filtration is greater than the colloid osmotic pressure and the lymph drainage together, then edema develops. The cause may be increased filtration (inflammatory processes or venous hypertension), reduced colloid osmotic pressure (hypoalbuminemia) or a functional or anatomical lymphatic drainage disorder. The latter is called lymphedema. The lymph is always high in protein. Thus, lymphedema is characterized by high protein interstitial edema. Lymphedema is a chronic and normally progressive disease.
Primary lymphedema
Primary lymphedema (Figure 1) is usually sporadic, rarely hereditary or syndrome-associated. The cause is hypo- or hyperplasia of the lymphatic vessels or lymph node fibrosis. It is usually localized to the lower extremities and usually one-sided. The first clinical signs usually show up at puberty, but it can manifest itself shortly after birth or at a later date. The prevalence is poorly studied and reported in a study of 1.15: 100,000. Overall, this disease is rather rare. Women are affected about five times more often.
Secondary lymphedema
The cause of secondary lymphedema (Fig. 2) is an interruption of the lymphatics. One distinguishes between benign (trauma, infections) or malignant (cancer-related) causes. The most common cause in the Western world is certainly associated with tumor, as a result of surgical Lymphknotenstagings with radiotherapy, occasionally caused by cytostatics. When axillary lymph node dissection is necessary, the incidence of arm lymphedema is still 16% to 20% and in the case of lymphadenectomy para-aortic, iliac, or inguinal about 15%. Any chronic edema (be it chronic venous insufficiency, heart failure, or even obesity) eventually leads to a volume-related congestive failure of lymphatic transport capacity with consecutive secondary lymphoedema.
Diagnostics and clinic
The most important point in the diagnosis of lymphedema is to think of it in the differential diagnostic considerations. Of course, in a one-sided swollen limb, it is important to exclude all other causes, such as a thrombosis or a primary / recurrent tumor. In addition to the history of persistent swelling, the clinical sign is often a positive Stemmer sign (Figure 3). The skin over the relaxed toe can no longer be caught and lifted. The toes are edged, the toes wrinkles rarefied while coarser toe crotch wrinkles.
Over the years, extensive subcutaneous fibrosis and sclerosis can develop, as can cutaneous papillomatosis cutis lymphostatica (Figure 4). While the clinical signs in primary lymphoedema decrease from distal to proximal, there is a swelling tendency from secondary to secondary lymphoedema. The analogous clinical signs are also found on the upper extremity.
Clinical staging
The staging is done according to the International Society of Lymphology (ISL) or Földi (overview 1). This is called stage I, when the lymphoedema still spontaneously recovers overnight, from stage II, when no spontaneous recovery is observed (because there is already an increasing fibrosis), and from stage III, the elephantiasis. In addition to a gigantic limb, extensive lymphatic secondary changes can be observed, such as extensive fibrosis, papillomatosis, lymphocysts or ulcerations.
therapy options
The therapeutic options are limited. Individual patients are also expected to have surgical options in the near future. Good results appear to be shown by autologous lymph node transplantation in secondary lymphedema. The microsurgical system of lymphoid shunts, as has already been carried out at a few centers worldwide, also shows good results in initial studies. The use of lymphatic growth factors (VEGF) is being intensively researched.
Compression and manual lymphatic drainage or complex physical decongestive therapy
Established and in the near future the treatment of choice for most patients is the complex physical decongestive therapy (see Fig. 5 and 6a / b).
Manual lymphatic drainage stimulates the lymphatic vessels to maximum activity, which always begins clavicularly in the area of the mouth and progresses peripherally. The most important measure, however, is the absolutely consistent compression therapy. Liquid can not be compressed, but it can be moved. Compression increases the interstitial pressure, decreasing the amount of filtration and thus the burden of the interstitial fluid. Compression also shifts the interstitial fluid into the terminal resorptionscapable lymph capillaries and additionally supports lymphatic drainage. In the first intensive phase, which aims to de-dematify, manual lymphatic drainage is used daily, combined with a professionally designed short-stretch compression bandage. In addition, the therapists use fibrosolocking or pressure-enhancing foam elements. In the maintenance phase, the manual lymphatic drainage is applied individually adjusted weekly or intermittently. But always a consistent compression therapy must be carried out. In this phase, the compression stocking is used.
compression stocking
The task of the compression stocking is to exercise as continuous and permanent compression as possible in order to be able to counteract the increasing build-up during the course of the day. An ideal compression stocking would therefore be very easy and very elastic to create, but then rigid and inelastic in the wear phase. Because of these discrepant requirements, a compression stocking can never replace a good short-stretch compression bandage, but can approach it. Required is a compression stocking with the highest possible working pressure. These requirements are met best thick and tight / tight circular knitted compression stockings and all flat-knit compression stockings. Knitted compression stockings with a short-term behavior may well be used in mild forms of lymphedema. In advanced forms of lymphedema, especially in misshapen extremities, however, a so-called flat knit stocking should be mandatory. These are knitted two-dimensionally, which allows the variation of the mesh size with the same mesh size. By sewing together these compression stockings are significantly more expensive in production and price. However, they are the treatment of choice for all forms of advanced lymphedema.
Which compression pressure?
The few studies currently available to determine the optimal compression pressure indicate that moderate pressure (that is, below 30 mmHg on the arm) appears to be superior to higher pressure. Pressure values around 40 mmHg also seem ideal on the leg. In everyday life, however, it is important to work out the optimal stocking or the optimal stocking combination for the individual patient (see overview 2). An “experienced” lymphatic patient can quickly tell you where corrections, supplements or an increase in pressure are needed.
Approved and edited reprint from Ars medici 14/2013
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