Lymphoedema: Information on swelling, therapy and treatment of lymphoedema

Lymph, much like blood, consists of fluid (lymph plasma) and cells (corpuscular components). The difference between blood and lymph is that lymph carries away larger cells which cannot pass through the walls of blood vessels.

The smallest lymphatic vessels (known as lymphatic capillaries) have gaps between the cells of the lymphatic vessel wall at their ends, which is why, in comparison with the venous blood vessel system (which also transports fluid from tissues distant from the body towards the heart), larger components of the tissue fluid can be absorbed and transported.

Typical examples of larger cells transported by the lymph fluid are:

• pathogens (bacteria, fungi)
• Foreign bodies (e.g. including dyes injected into tissue)
• Proteins
• Fats (lipids)
• Immune cells (macrophages)

The lymphatic system consists of lymphatic vessels through which lymph is transported towards the heart. These lymphatic vessels are initially very small (lymphatic capillaries) and visible only under a microscope. The closer they get to the heart, the larger the lymphatic vessels become, until they are eventually visible to the naked eye. Ultimately, all the lymphatic vessels in the body flow into a large lymphatic vessel (ductus thoracicus), which empties into the subclavian vein just before the heart.

Filter stations are incorporated to ensure that all foreign bodies and pathogens are removed from the lymph fluid before it enters the blood vessel system. These are also known as lymph nodes and swell when there is an increased influx of pathogens that need to be broken down (e.g. in the case of inflammation). An example of this is palpable enlargement of the cervical lymph nodes in the case of upper respiratory tract infections or dental problems.

Lymphoedema is an accumulation of fluid in the intercellular spaces. The body produces approximately 2–3 litres of lymph fluid per day. Lymphoedema is caused by a failure to drain this lymph fluid properly. This leads to congestion in the interstitial spaces, with fluid increasingly accumulating in the tissue. Swelling very often occurs in one or both legs. However, other parts of the body, such as the arms, can also be affected. Lymphoedema can also occur in the neck, head, trunk or genitals, although it is less common in these areas.

There are various congenital and acquired causes and conditions that can lead to lymphoedema.

Lymphoedema should not be confused with lipoedema. Lipoedema is also a form of swelling, but it is caused not by an increased accumulation of fluid, but by an increase in fatty tissue.

How lymphoedema develops depends on the cause: a distinction is made between primary and secondary lymphoedema.

Primary lymphoedema is hereditary; it is therefore not caused by other illnesses or external factors. Medically, this is also referred to as hereditary (genetically determined) lymphoedema. In women, it is not uncommon for this to become apparent during pregnancy. In some cases, there are also conditions in which lymphatic vessels are missing in certain areas of the body, meaning they have not formed properly. This then leads to impaired drainage of lymph fluid.

Secondary lymphoedema can have various causes, such as metabolic disorders like diabetes mellitus or chronic skin conditions. Tumours (and tumour removal), accidents, and infections caused by viruses, bacteria, worms and fungi can also be included in this category. The same applies to radiotherapy, surgical scars or chronic venous insufficiency. 

Another cause of secondary lymphoedema is surgical procedures. In medical terms, this is referred to as iatrogenic lymphoedema (i.e. caused by a doctor). A common example of this is breast cancer surgery, during which affected lymph nodes in the armpit are removed. This is important to prevent the tumour from spreading further and, depending on the number of affected lymph nodes, to determine any necessary treatment measures (chemotherapy, radiotherapy).

However, when the lymph nodes are removed, important lymphatic vessels in the armpit are usually damaged, which is why the drainage of lymph fluid may subsequently be disrupted. The same applies to operations in the groin (e.g. for a narrowing of a blood vessel), during which lymphatic vessels may be damaged. As the lymphatic vessels are not visible to the naked eye, lymphoedema (also known as lymphatic fistulae) is a typical complication of surgical procedures.

Typical signs of lymphoedema include a bulging accumulation of fluid that is clearly visible. The leg or arm is severely swollen and appears puffy. Furthermore, this oedema can also be felt. Whilst in the early stages it is still possible to press a dent into the skin, in the advanced stages the oedema is firm. It is now no longer possible, or only with great difficulty, to press a dent into the tissue. One diagnostic method is the Stemmers’ sign. If the skin fold between the second and third toes – or fingers – can no longer be lifted, this test is considered positive. Lymphoedema is generally divided into several stages:

• Initial stage: In this early phase, there are as yet no significant symptoms; the oedema is only present to a subtle degree.
• First stage: The lymphoedema is now visible and can also be felt. It feels relatively soft, so that a dent can be pressed into the affected area. This dent recedes of its own accord after a short time. However, Stemmers’ sign is already positive at this stage.
• Second stage: It is no longer possible, or only to a limited extent, to indent the skin; the oedema becomes firmer. Whilst in the first stage it was still possible to achieve an improvement by elevating the affected limb, this measure is no longer effective at this stage.
• Third stage: Due to the increasingly severe swelling, mobility of the affected body part is severely restricted. The ‘swollen appearance’ has become even more pronounced. Furthermore, the skin may react with eczema or other conditions. In addition, wounds in this area heal only poorly. At this stage, the condition is referred to as elephantiasis.

In principle, it is advisable to consult your GP first, as they are best placed to know or investigate your medical history and the underlying causes. Lymphatic complications following surgical procedures should continue to be monitored by the surgeon who performed the original operation. Sometimes a further operation (known as a revision procedure) is necessary to close the lymphatic vessels that have been opened. This is usually carried out by means of so-called coagulation of the wound surfaces (thermal ablation); even during revision surgery, it is rarely possible to visualise the opened lymphatic vessels directly. For the diagnosis and treatment of lymphoedema, GPs, surgeons and lymphologists often work closely together.

The following treatment methods are available:

• in the early stages: elevation
• Compression therapy
• Lymphatic drainage
• Exercise therapy
• Surgical treatment

What options are available for compression therapy?

Treatment depends not only on the cause, but also on how far the lymphoedema or lymphoedemas have already progressed. At a very early stage, elevating the affected limb – such as the leg – can aid lymphatic drainage. In addition, consistent compression therapy is a top priority, as this can usually prevent the condition from progressing. In the case of open wounds, inflamed tissue and highly sensitive or painful skin, elasto-compressive bandaging is recommended initially. These must generally be changed several times a day, particularly in the case of heavily weeping wounds. The latter also usually require the application of special, skin-friendly and highly absorbent wound dressings. The expertise of a wound care specialist (wound manager) can be very helpful here. 

In cases where the skin appears normal (e.g. arm swelling due to lymphoedema following breast cancer surgery), wearing a medical compression stocking is recommended instead of an elastic-compression bandage. These are fitted at a medical supply shop. Such stockings are also available for the legs. However, these are very strong, firm stockings (compression class III, pressure approx. 40–45 mmHg), which can sometimes be difficult to put on and take off and should be done with the aid of a so-called donning aid. For venous disorders, less firm stockings (compression class II, approx. 30 mmHg) are generally sufficient and are easier to change. 

What happens during lymphatic drainage?

Ultimately, lymphatic drainage has also proved to be very effective. This is a specialised massage technique that stimulates the lymphatic system, thereby improving the drainage of fluid. This technique is also used following operations involving severe swelling and is carried out by a physiotherapist. 

Are there any medicines for lymphoedema?

In some cases, diuretics are prescribed to reduce the swelling. Diuretics are medicines that promote the excretion of water. Typical indications for diuretics include kidney and heart failure, where their use is often vital and life-saving. However, extreme caution is required when using them to treat lymphoedema, as these medicines can cause side effects of varying severity and cannot actually treat lymphoedema!