Pathologies of the Lymphatic System


What Is Lymphoedema

Lymphoedema is the name for an observable accumulation of fluid in interstitial spaces (between cells) that is caused by mechanical insufficiency(disturbance) of the lymphatic system.

This results in an incapacity of the lymphatic system to drain a sufficient volume of fluid, leading to a back-up which is referred to as “lymphoedema”.

In many cases, minor and/or major lymphatic vessels are disrupted by surgery, injury, radiotherapy or infection.

As a result of the damage to the lymphatic system, the remaining vessels are no longer capable of draining the fluid accumulating in tissue, and lymphoedema develops.


Secondary Lymphoedema

The most common cause for secondary lymphoedema in the Western world is cancer surgery involving removal and/or radiotherapy of the lymph nodes.

The probability of oedema occurring decreases continually following surgery, provided that the patient takes steps to avoid it. This means that patients absolutely must give the highest priority to preventing lymphoedema. Sometimes edema does not occur immediately after cancer treatment, but several months or even years later, despite the necessary precautions being taken; a medical specialist should be consulted in such cases to rule out the growth of any new tumors.

In many cases, the treatment of breast cancer requires the removal of lymph nodes. This surgical intervention often causes damage to the lymphatic system which compromises its effectiveness in draining fluids.

Fluids are no longer drained or are drained insufficiently.

Radiotherapy also affects the lymphatic system and its effectiveness. The imbalance between lymphatic load and lymph transport capacity following breast cancer surgery may result in a so-called secondary (=acquired) arm lymphedema. However, there are also cases of such edema – which is rarely painful – occurring in the chest.

In Western countries, the most common cause of secondary lymphedema is cancer surgery including removal and/or radiation of lymph nodes. The likelihood of edema formation diminishes as the post-operative period increases. To reduce the chances of developing lymphedema, patients need to follow very specific rules.If, however, a lymphedema does develop, it will require lifelong therapy. The treatment schedule will depend on the course of the disease. If left untreated, lymphedema will progress in most cases and can assume monstrous proportions (elephantiasis).

Lymphedema frequently does not occur immediately after surgery but appears months or sometimes even years later.

After many years of experience in this area we regret to have to say: “Once an edema, a lifetime of edema.” There is no known cure for lymphedema, but it is possible to manage and control the symptoms through consistent treatment and strict observence of precautionary rules.

In many cases, the treatment of abdominal cancer requires the removal of lymph nodes. This surgical intervention often causes damage to the lymphatic system which compromises its effectiveness in draining fluids.

There are rare cases where lymphedema involves the lower abdominal region or the genitals as well.  In Western countries, the most common cause of secondary lymphedema is cancer surgery including removal and/or radiation of lymph nodes.

The treatment schedule will depend on the course of the disease.

Secondary Lymphoedema is broken down into four classifications or stages:

Stage 0 or the Latent Stage – the patient feels that the limb is different, it may have odd sensation, feel heavier or feel like there is something under the skin. Whilst there may be no outward signs of lymphoedema, stemmer and pitting sign negative, no evidence of swelling to the eye there are pathological changes in the tissues as fluid and protiens are accumulating. We are in the very best position to begin treatment as this is the earliest indication that there are changes developing in the limb, the earlier you seek treatment, the less chance the condition has of progression.

Stage 1 – presents as a visable soft oedema, it reduces in size when the limb is elevated, it is a high protien oedema that has a change that is greater than 2cms in circumfrance and more than 200mls in fluid, this stage may spontaneously reverse however the best thing you can do to ensure that the condition does not progress is to have some decongestive therapy again this is the perfect time for us to effect change and prevent progression.

Stage 2 – raising the limb no longer improves the oedema. Tissues become fibrotic, fat cells become trapped and skin changes often take place such as pachyderma, papillomatosis. This stage does not spontaneously reverse and treatment is imperative to prevent further tissue strech occuring. This stage does not always have a pain response however the limb will feel very tight and uncomfortable, parts of the limb will feel quite hard, with MLD, bandaging and excercise softening of the fibrosis and reduction of fluid will occur. This stage is most often what a health care professional will see as a typical lymphoedema presentation.

Stage 3 – this stage is often referred to as lymphostatic elephantitis. An influx of inflammatory cells, growth of more fibre and fat, more and more protiens become trapped in the tissues. Extensive fibrosis can effect arteries, lymph vessels, veins. Reduced immune defence can effect the health of nails and skin leaving the limb at greater risk of infection and hypoxia. Pain will most certainly be present and severe skin changes will be present. Yes this is a condition that we can treat and care for and can certainly effect change but it can never be reversed.


Primary Lymphoedema

Primary lymphedema is an inherited abnormality of the lymphatic system. Its root causes are  lymph vessels are too few or too small, lymph nodes are too few or too small, lymph vessels and/or lymph nodes fail to function properly, or the causes are a combination of the above disorders

The lymphatic system has considerable reserve power, which is why it often takes years until its weakness becomes visible. It may be a simple sunburn which triggers system overload and is the last drop “to make the cup overflow”. As a consequence lymphedema will develop.Primary lymphedema usually begins in the distal leg area, i.e. the toes, in rare cases also in the hands. However, it may also develop in any other area of the body. Alongside the swelling, so typical of lymphedema, skin changes will also occur with progressing disease.

The skin in the edema area gradually thickens and coarsens.

If these changes occur at the base of the second toe, we talk of a
“positive Stemmer’s sign”.

The skin in the affected parts of the body frequently shows the typical changes associated with primary lymphedema:

  • Wart-like lesions (papillomatosis)
  • Excessive growth of horny tissue of the skin (hyperkeratosis)
  • Lymph vesicles
  • Fistula (the name given to an abnormal duct or passageway that links two cavities in the body, frequently opening through the skin)

These skin changes worsen with the duration and severity of edema. This is why lymphedema must be treated as early as possible to prevent such changes from developing.

Too late a therapy may result in these changes no longer being fully reversible. Primary lymphedema is a chronic disease which cannot be cured, but it can be very well managed by therapy.


Phlebo-Lymphoedema/Chronic Venous Insufficiency (CVI)

Possible causes of venous insufficiency include: a genetic predisposition to varicose veins thrombosis (or “post-thrombotic syndrome”)

The function of veins is to return blood to the heart. In other words, they pump blood “uphill”.

In order to avoid too much pressure building up, the veins in the legs in particular have special valves, spaced every 0.5 to 1cm apart, that prevent the blood from flowing backwards.

In the case of varicose veins, these valves no longer close properly. The pressure in the veins increases, particularly in the calves, since this section of the legs is farthest from the heart.

Long-term strain on the lymphatic system is considered to be the cause of phlebo-lymphedema. A greater volume of fluid from the blood vessels is forced (filtered) into tissue as a result of increased pressure in the veins. This excess tissue fluid is referred to by physicians as edema.

Patients suffer from congestion and swelling, particularly in the limbs. When the legs are propped up, the body reabsorbs the excess tissue fluid, draining it to the vessels once again. This means that in the morning, after getting up, the legs are not usually swollen any longer.

Generally there is a deep pitting in the lower ankle that indicates a lymphatic component. There will be a bluish/purple discolouration to the limb, varicosis, hemosiderin deposits, a sensation of heaviness/tension, frequent leg cramps and the condition will improve on elevation.

This condition is particularly vunerable to leg ulcers, thrombosis, embolisim and phlebitis.

However, if much bodily waste is retained in the tissue, propping up the legs is no longer a sufficient remedy. The tissue hardens and phlebo-lymphedema develops.

Therapy Symptoms caused by varicose veins (i.e. heavy legs, tingling sensation, cramps) and/or associated edema can be most effectively treated by wearing compression stockings.

Stockings extending to the knee are usually sufficient. In order to achieve the best results, it is necessary to wear the stockings consistently; patients should especially keep them on under warm conditions. Even so, many patients do not wear their compression stockings when temperatures are particularly high.

If the skin has already some pathological changes, the lymphvessel system is damaged too. In this case we the most effective therapy is the decongestive therapy which consists of Manual lymphdrainage, bandaging, skincare and exercises.



Lipoedema is a disorder of subcutaneous fat metabolism and distribution. The condition mostly affects women with an estimated 10-15% of the female population.  The condition affects mostly the lower limbs however can also be found in the upper limbs, it is a chronic and progressive disease that often has a hereditary link.

It is always bilateral and usually symmetrical, the tissues bruise more easily and those bruises resolve slowly, fat pads are seen around the thighs and knees. Ankles and feet do not become effected, the skin is extremely soft and pliable although tender to the touch often women are unable to tolerate massage or pressure to the areas.

Lipoedema has nothing to do with obesity and therefore can not be improved with diet unless there is an additional adipose tissue component, tjere is a tendency to develop hematoma with an increase in blood capillary permeability. The greater the permeability of the blood capillaries the more lymphatic fluid there is in the tissues, MLD can reduce the fluid from the tissues but not change the amount of adipose tissue. MLD will reduce the sensitivity of the tissues and reduce volume when there is a lymphatic component to the lipoedema.

The skin can appear orange peel like and adipose lumps can be palpated must under the surface of the skin, increased permibility and fragility of the blood capillaries are associated with lipoedema. Women often complain about the heaviness of the legs with a sense of tightness or fullness in the tissues. Weight control is essential with this disease, if obesity occurs with the liloedema then more often than not a secondary lymphoedema will occur hence the term lipo-lymphoedema.

Lipoedema is also broken down into stages in accordance with the disease

Stage 1 – the connective tissue is flooded.

Stage 2 – the connective tissue is softened.

Stage 3 – nodules form.

Stage 4 – nodules and orange-peel skin form.



Glyco-proteo-glycan accumulation in the tissues, a result of a thyroid dysfunction (hyper or hypo active), generally presents as a spongy honeycomb appearence, mostly symetrical and involves arms and legs.


Other Forms

Cyclic Idiopathic Syndrome – premenstral, no progressive characteristics, eyelids and face can swell and an overall sense of tension.

Pregnancy Oedema – oedema in pregnancy MLD is effective

Cardiac Oedema – absolutely contraindicated for MLD

Rheumatic Oedema – chronic polyarthritis, raynaud/scleroderma, bechterews disease, epicondylitis, tendinoses and bursitis all well indicated for MLD

Lipohypertrophy – no tendency to orthostatic oedema or hematoma, no pain, a pure adipose tissue dysfunction –  no indication for MLD

Madelungs Disease – accumulation of fat in trunk and upper limbs, more frequency with males and generally with history of alcohol abuse – no indication for MLD


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