Author: Joachim E. Zuther
(also published in NLN Newsletter Vol II, #2

Summary:
Lymphedema, caused by a low output failure, i.e. a reduced transport capacity (TC) of the lymphatic system, which in the case of lymphedema has fallen below the physiological level of the protein and water load, is a common and serious condition worldwide.

Complete Decongestive Physiotherapy (CDP) is the treatment of choice, even in the most advanced stages of lymphostatic edema - provided that both a physician with broad experience in clinical lymphology and a specially trained therapist are available.

A sufficient lymphatic system is able to return the physiological amount of protein and water load back to the venous system.

The lymphatic protein load consists of plasma proteins continuously leaving the blood capillaries. The fraction of water ultrafiltrated in the area of the blood capillaries which is not reabsorbed, is called the lymphatic load of water.

In the event of an increase of water and protein a healthy lymphatic system is, for some time, able to prevent the onset of edema by increasing its lymph time volume (LTV), i.e. lymph anions will increase their pulsation frequency and amplitude. This is called the lymphatic safety factor or safety valve function of the lymphatic system.

The highest lymph time volume is known as the transport capacity of the lymphatic system which is approximately ten times higher than the lymph time volume under physiological conditions (10).

Lymphedema arises due to an imbalance between the normal amount of protein load and the reduced transport capacity of the lymph vascular system. This condition, known as mechanical insufficiency, results in an accumulation of proteins in the interstitial tissue with subsequent fibrosclerotic changes.

Since in such cases the lymphatic system is not able to activate its lymphatic safety factor, other pathologic factors that produce an increased level of lymphatic load (inflammation, chronic venous insufficiency), can lead to even more serious complications such as ulcerations.

Common causes for mechanical insufficiencies in the case of secondary lymphedema are surgery, radiation, trauma or inflammation. The reason for an insufficient transport capacity in primary lymphedema are congenital malformations of the lymphatic system. Primary lymphedema can be present at birth or develop some time during the course of life (5,12).

ETIOLOGY

Stages of lymphedema
If the reduced transport capacity is still sufficient in managing the lymphatic load there is no clinical lymphedema present The time preceding the onset of lymphedema is called the "latency stage" (5).

The first stage of lymphedema (reversible stage) is characterized by a smooth texture of the tissue. The area affected with lymphedema is pitting and may vanish more or less over night. If the protein rich swelling persists, fibrosclerotic tissue changes will result in increased hardening of the tissues (stage II), elevation has no effect and in addition patients are prone to developing frequent infections which worsen the condition (4).

Typical for the third stage of lymphedema (lymphostatic elephantiasis), is an extreme increase of the swelling, combined with skin changes, loss of function and other complications.

Lymphedema, if left untreated, may lead to invalidity or even the development of angiosarcoma (Stewart Treves-Syndrome) (8).

LYPHEDEMA

Therapy
Since there is no cure for lymphedema (10), the goal of the therapy is to reduce the swelling and to maintain the reduction, i.e. to bring the lymphedema back to a stage of latency.

For a majority of patients this can be achieved by the skillful application of Complete Decongestive Physiotherapy, a non-invasive, safe and reliable method that shows good long term results in both primary and secondary lymphedema.

CDP is also cost-effective:
• it transfers the care from the doctor to the patient/family
• it significantly reduces the risk factors of developing cellulitis attacks, described by Olszewski as "Dermatolymphangioadenitis" (DLA), by improving lymph cysts, lympho cutaneous fistulas, varicose lymphatics or fungal infections (7)

Even though the basic steps of CDP had already been described by Winiwarter at the end of the last century, this therapy became widely accepted only during the past two and a half decades(1,2,13).

Numerous studies have proven the effectiveness of this therapy which is well established in European countries and is now becoming widely recognized in the United States (1,2,8,10).

CDP consists of four basic steps:
• Skin and nail care, that may also include topical and systemic antimycotic drug treatment (the skin must be free of infections before treatment can be started)
• Manual Lymph Drainage
• Compression therapy and
• Decongestive exercises

The treatment itself is done in two phases (8). In phase one the goal is to mobilize the accumulated protein- rich fluid and to initiate the reduction of fibrosclerotic tissues (if present). The average duration of this intensive phase is four weeks. The treatment is done twice a day, five days a week. Another important goal in this first phase is to instruct the patient in techniques designed to maintain and improve the success of the treatment (proper skin care, correct application of bandages, wearing of compression garments, etc.).

The first phase of the therapy is immediately followed by phase two, aimed to preserve and also to improve the success achieved in phase one. This phase is for the most part continued at the patients home. With a good patient compliance the volume reduction can not only be maintained but also improved by progressive reduction of fibrosclerotic tissues.

In more severe cases it is sometimes necessary to repeat phase one and if lymphedema is associated with other conditions, the individual steps of CDP will be modified accordingly.

STAGES OF LYMPHEDEMA AND THERAPEUTIC APPROACH

Manual Lymph Drainage is a gentle manual treatment technique which improves the activity of intact lymph vessels by mild mechanical stretches on the wall of lymph collectors (10). A better filling of lymph capillaries, achieved by a mild increase in tissue pressure during MLD, also results in a higher lymphangiomotoricity.

In most of the post-mastectomy patients, lymphedema not only includes the arm but also the ipsilateral trunk quadrant, since the collecting area of the axillary lymph nodes are the upper extremity and the homolateral upper trunk quadrant. In cases of secondary lymphedema of the lower extremities, the lower trunk quadrant and/or the genitalia may be involved in the lymphostasis because the inguinal lymph nodes receive lymph fluid from the leg, the ipsilateral lower quadrant of the trunk and the exterior genitals (10).

MLD is therefore performed in steps: the first step is to stimulate the lymph vessels in the non-affected contralateral trunk quadrant which results in a suction effect (6) on the lymphatics of the affected trunk quadrant. In the second step edema fluid is cautiously pushed from the congested quadrant into the quadrant free of edema via tissue channels, initial lymphatics and lymph vessels bridging the watersheds thus creating a connection between regional lymph nodes on the contralateral and ipsilateral sides.

After the trunk is decongested, the upper part of the extremity is treated and later on, the distal part and the hand/foot - always making sure not to overwhelm the drainage areas previously stimulated.

Many patients we see report that even though they received many treatments in "Manual Lymph Drainage" the lymphedema didn't improve and sometimes the limb size even increased. Asking the patient how the treatment was performed, in many cases we hear that the therapist performed an effleurage beginning at the fingers or toes or used massage techniques on the swollen extremity. As mentioned before, MLD is a very gentle manual technique consisting of four basic strokes and any combination of same. MLD has nothing to do with "classical" or "Swedish" massage and shouldn't be called massage. The word "massage" means "to knead" (Greek: massain), Manual Lymph Drainage does not have kneading elements and is generally applied suprafascially, whereas massage is usually applied to subfascial tissues.

Compression Therapy
Since the elastic fibers of the skin are damaged in lymphedema, it is mandatory to apply sufficient compression to the affected area in order to prevent reaccumulation of fluid. Compression therapy increases the tissue pressure (TP) which results in lower effective ultrafiltration and better reabsorption on the venous end of the blood capillaries. It also promotes the filling of initial lymph vessels, improves the function of the muscle pumps and helps to reduce fibrosclerotic tissue.

In phase I of the therapy compression is applied via short-stretch bandages.

Short-stretch bandages have a high working pressure (pressure the bandage exerts on the musculature working underneath) and a low resting pressure (pressure exerted on the tissue while resting). Long-stretch bandages have exactly opposite characteristics and are therefore not indicated in the treatment of lymphostatic edema since they tend to cut into the tissue while resting, causing a tourniquet effect and thus impeding sufficient lymph and blood flow. Long-stretch bandages also fail to produce an effective counterforce to the working muscles.

In order to avoid irritation on bony prominences and tendons, padding with cotton bandages or foam is applied underneath the bandages.

To enhance the reduction of fibrosclerosis, high density foam is frequently used in combination with short- stretch bandages.

Low pH-lotion to keep the skin moist and tubular bandages to avoid allergic reactions and to protect the bandage materials are also applied.

During phase I of CDP compression therapy during day and night is achieved by short-stretch bandages. In phase II the patient wears compression garments during the day and applies bandages for the night. Measurements for these elastic support garments should be taken at the end of phase I by the therapist or the supervising physician. An incorrectly fitted sleeve or stocking will have negative effects on the lymphedema itself and on the patients compliance. To achieve the best results with CDP good compliance of the patient is absolutely necessary.

The compression class and the type of garment (round or flat knit, style) depends on the severity of the swelling, the patients age and any other relevant factors.

In general the pressure of the garment should be as high as the patient can tolerate (14). For lower extremity lymphedema compression classes 111(30-40 mm/Hg) or IV (> 50 mm/Hg), for lymphedema of the upper extremities compression classes 1(10-20 mm/Hg) or II(20-30 mm/Hg), sometimes compression class Ill, are suitable. In some cases it might be necessary to apply even a greater compression than class IV which can be achieved by wearing two stockings on top of each other or by the application of bandages on top of a stocking.

To have the maximum effect, garments must be worn every day and replaced after six months.

Remedial exercises aid the lymphokinetic effects of joint and muscle pumps and should be performed by the patient wearing the compression bandage or the garment. The exercise program should be customized for each patient depending on the individual capacities. Exercises should be performed slowly and with both, the affected and non-affected extremity. Vigorous movements or exercises causing pain must be avoided.

When does CDT fail?
Phase I: malignant lymphedema artificial lymphedema improper treatment (MLD as the only treatment, no MLD or improper bandage) associated illnesses lack of compliance

Phase II: lack of compliance lack of hygiene reoccurrence of cancer associated illnesses

Conclusion:
Lymphedema can be treated successfully by a skilled therapist with extensive training in all components of Complete Decongestive Physiotherapy and good patient compliance. The treatment success must be monitored by circumferential and/or volumetric measurements.

If phase I of CDT is performed in the early "pitting" stage of lymphedema, total remission of the swelling is possible. In later stages of lymphedema phase I only reduces the swelling but fibrosclerotic tissue changes will still be present. For these more advanced stages phase II of Complete Decongestive Physiotherapy not only preserves the treatment success achieved in the intensive phase but also improves the edema overtime, restoring the limb to a normal or near normal size.