Lymphoedema

What is lymphoedema and which areas of the body are affected?

The lymphatic system is an integral part of the immune system and is made up of delicate lymphatic vessels and lymph nodes. Its main functions include the elimination of harmful cells and the transfer of white blood cells.

If this transport mechanism is impaired, it can lead to a condition known as lymphoedema.

Lymphoedema manifests itself in the form of visible and palpable swelling, which can occur on the arms, legs and/or trunk. However, not every swelling (e.g. lymph congestion) is automatically lymphoedema. Consequently, a professional physical examination is of central importance.

Lymphoedema is a chronic, progressive disease with the potential to cause disability.

Typical symptoms of lymphoedema are

• Swelling, which usually occurs on the arms or legs
• a feeling of tightness, especially when wearing tight-fitting clothing
• more noticeable and longer-lasting pressure points on the skin
• changes in the skin such as thickening or hardening
• dull pain in the affected areas
• the appearance of small blisters from which clear fluid escapes

Lymphoedema can occur anywhere on the body, although it is usually more common on the arms or legs.

The symptoms develop gradually over weeks or months and can even occur years after cancer treatment in cancer patients.

The symptoms associated with lymphoedema can have a significant impact on daily activities. This can be manifested, for example, by wearing unsuitable shoes or limited mobility.

In addition, patients have an increased risk of infections in the affected areas of the body, as the lymphatic system is weakened in this area and the skin is more susceptible to tears due to the swelling.

Early detection of lymphoedema is crucial in order to prevent or minimise the potential health complications of persistent congestion through appropriate treatment.

During a medical consultation and physical examination, the treating specialist can determine whether lymphoedema is present and at what stage. It is also important to rule out other possible causes of swelling and discomfort.

The stages of lymphoedema

The occurrence of lymphoedema can vary from person to person, as the structure of the lymphatic system varies from individual to individual. Even within the lymphatic system there can be differences, for example in the number of lymphatic vessels between the right and left side of the body, so lymphoedema can have different appearances even if it is caused by the same thing, such as surgery or radiotherapy.

A distinction is made between different stages of lymphoedema, which describe the severity of the condition:

Stage 0

At this earliest stage, no oedema is visible or palpable. Nevertheless, there is a latent risk of developing lymphoedema, as the conditions for this (damage caused by tumour surgery or radiation) are present. The duration of the latency stage is unpredictable; it can last for life or only for a short time. No treatment is required at this stage, but precautionary measures should be taken.

Stage 1

At this stage, visible and palpable lymphoedema is present, which increases during the day and partially disappears overnight. Signs of this can be that clothing or jewellery fits tighter in the evening or leaves marks. If such symptoms occur, it is advisable to consult a doctor, who can then arrange a referral for physiotherapy treatment. At this stage, a few measures are often enough to slow down the progression of the disease.

Stage 2

In stage 2, the lymphoedema persists overnight and is permanently visible and palpable. However, it does not have to be severe. At this stage, treatment in lymphological physiotherapy is required.

Stage 3

At this advanced stage, not only is permanent swelling visible, but complications such as skin changes (colour and appearance), occasional inflammation and considerable restrictions in everyday life also occur. Treatment at this stage is complex, but can also reduce major lymphoedema with complications. This leads to an improvement in the general condition.

The examination procedures

In modern medicine, advanced procedures are available to visualise and examine the lymphatic vessels:

• Examination of the vascular system (angiology)
• Ultrasound
• Oscillography (blood pressure measurement in the legs)
• Volumetry (determining the volume of the legs)
• Lymphoscintigraphy (diagnostic imaging procedure)
• Indocyanine green lymphangiography (visualisation of the lymph vessels)

These procedures play a particularly important role in the decision-making process when the question arises as to whether surgical treatment is necessary.

Lymphoedema occurs when the lymph fluid is no longer removed efficiently via the lymph channels between the cells - usually resulting in a backlog and flooding under the skin.

Oedema can also be caused by impaired heart or kidney function. Problems in the venous blood vessel system or the use of certain medications can also lead to oedema.

What types of lymphoedema are there?

There are two main types of lymphoedema : primary and secondary lymphoedema, with secondary lymphoedema being more common than primary lymphoedema.

Primary lymphoedema

Primary lymphoedema, also known as congenital lymphoedema, is the result of suboptimal development of the lymphatic system. This abnormality can occur in different parts of the body or it can be limited to a specific area.

In most cases, even if the lymphatic system is not optimally developed, sufficient lymph fluid can be removed over a longer period of time, so that lymphoedema only occurs after several years.

It is possible for wound healing oedema to develop after an accident and not completely resolve. In such cases, it is assumed that the lymphatic system was not fully functional to begin with, which is why this oedema is also considered primary lymphoedema.

Primary lymphoedema can be present from birth, although this is rarely the case. It often develops in response to a trigger, which can often only be identified by thorough investigation.

However, it can also occur without a recognisable trigger and its occurrence is not age-dependent. Typically, the legs are affected, sometimes also a whole side of the body.

Secondary lymphoedema

Secondary lymphoedema is an acquired condition that is often associated with cancer.

Tumour operations in which lymph nodes were removed and/or radiotherapy was required result in local and incurable damage to the lymphatic system. Lymph nodes have no regenerative capacity, which means that the lymphatic system may no longer be able to drain all lymphatic fluids from the affected tissue.

The lymphatic system is structured so that the lymph flows from an arm or a leg into the central lymph nodes in the corresponding section of the trunk. The effects of a lymphatic system disorder can only be seen in this specific area of the body.

For example, following the removal of a tumour from the left breast, lymphoedema may only occur in the left arm and/or the left part of the trunk, but not in the right arm or leg. Similarly, damage to the lymphatic system caused by the treatment of an abdominal tumour may never lead to lymphoedema in an arm.

In some cases, particularly in people with a large number of lymphatic vessels, lymphoedema may never occur despite tumour surgery with lymph node removal and radiotherapy. Studies on women after breast cancer have shown that only about one in five of them develop arm lymphoedema over time.

The development of secondary lymphoedema can occur immediately after cancer treatment or years later. This depends on the duration of the compensatory mechanisms.

It is important to emphasise that the development of lymphoedema does not necessarily have to be a consequence of cancer treatment.

What is the aim of treatment?

It is not usually possible to cure lymphoedema completely.

Treatment therefore focuses on controlling the swelling, improving the functionality of the affected extremities and preventing possible complications such as infections. In many cases, a lifestyle adjustment is essential for lasting improvements.

In physiotherapy, those affected receive instructions from specialists on massage techniques and special exercises that support the drainage of lymphatic fluid. The use of compression stockings may also be recommended. These stockings apply gentle pressure to the affected leg or arm, which distributes the accumulated fluid more evenly and allows it to be drained more efficiently by the lymphatic system.

The combination of lymphatic drainage and compression therapy is the gold standard in the conservative treatment of lymphoedema.

Surgical measures are only considered after at least one year of conservative physiotherapy. Patients affected by secondary lymphoedema must also have been tumour-free for at least one year. Under these conditions, it can be assessed whether a surgical procedure is promising and which technique should be used.

The surgical treatment of lymphoedema is the responsibility of plastic surgery. Thanks to major advances in surgical techniques, the following four procedures are now available:

• microsurgical lymph node transfer (VLNT)
• microsurgical lymphatic vessel transfer (VLVT)
• lymphovenous anastomosis (LVA)
• Liposuction

These procedures can greatly improve the quality of life of those affected. They are performed by our experienced specialist Prof Dr Mario Scaglioni .

Two main approaches in the surgical treatment of lymphoedema

• Removal of excess tissue
• Improvement of the lymphatic drainage

Reconstructive measures include techniques such as lymphovenous anastomosis (LVA), lymph node transplantation (VLNT) and microsurgical lymphatic vessel transfer (VLVT). Modern microsurgery procedures, in which high-precision instruments and powerful microscopes are used, enable the treatment of tiny lymphatic vessels. This involves connecting healthy lymphatic vessels with other small lymphatic or blood vessels in order to bypass blockages and normalise lymph flow.

In contrast, the respective procedures include interventions such as liposuction, in which excess fatty tissue is removed using vibrating, blunt cannulas. This procedure can effectively help to reduce the circumference of the arms or legs. However, the use of compression therapy is still essential after surgical treatment.

Lymphoedema treatments by Prof. Dr Mario Scaglioni, MD

With the help of microsurgery and supermicrosurgery, it is possible to restore lymphatic flow.

Lymphatic surgery is an essential part of cancer therapy. It not only has a high diagnostic value, but is also considered the gold standard of many oncological therapy concepts.

Lymphoedema as a complication of these therapies remains a challenging clinical problem. Lymphoedema of the lower extremities is far more common than that of the upper extremities, which is primarily associated with breast cancer and its treatment.

The treatment of lymphoedema is complex. However, the increasingly improved understanding of the lymphatic system and the introduction of microsurgery and supermicrosurgery have opened up new ways of treating lymphoedema.

Supermicrosurgery makes it possible to dissect the smallest lymphatic vessels with a diameter of 0.3 to 0.8 mm and thus restore their flow. The finest surgical instruments and a microscope with up to 40x magnification are used.

The techniques described below can be used to reconstruct the lymphatic drainage.

Creating new drainage channels with lymphovenous anastomosis (LVA)

The principle of lymphovenous anastomosis (LVA) enables fluid passage via the creation of new, extra-anatomical drainage pathways.We inject the dye indocyanine green (ICG) to preoperatively locate the corresponding lymphatic vessels in order to obtain a mapping of the insufficient lymphatic pathways.

The still active lymphatic vessels are then anastomosed to veins of the same calibre to restore the continuity of lymphatic drainage. It is also possible to anastomose lymphatic vessels directly with other surrounding lymphatic vessels.

Circumference reductions of almost 40 % and significant skin improvements can be achieved in the affected extremities.

This unique super-microsurgical technique requires many years of experience in microsurgery and the use of highly specialised instruments. In advanced stages of the disease, this surgical method is no longer feasible.

With the help of microsurgery and supermicrosurgery, it is possible to restore the lymph flow.

Lymphatic surgery is an essential part of cancer therapy. It not only has a high diagnostic value, but is also considered the gold standard of many oncological therapy concepts.

Lymphoedema as a complication of these therapies remains a challenging clinical problem. Lymphoedema of the lower extremities is far more common than that of the upper extremities, which is primarily associated with breast cancer and its treatment.

The treatment of lymphoedema is complex. However, the increasingly improved understanding of the lymphatic system and the introduction of microsurgery and supermicrosurgery have opened up new ways of treating lymphoedema.

Supermicrosurgery makes it possible to dissect the smallest lymphatic vessels with a diameter of 0.3 to 0.8 mm and thus restore their flow. The finest surgical instruments and a microscope with up to 40x magnification are used.

The techniques described below can be used to reconstruct the lymphatic drainage.

Creating new drainage channels with lymphovenous anastomosis (LVA)

The principle of lymphovenous anastomosis (LVA) enables fluid passage via the creation of new, extra-anatomical drainage pathways.We inject the dye indocyanine green (ICG) to preoperatively locate the corresponding lymphatic vessels in order to obtain a mapping of the insufficient lymphatic pathways.

The still active lymphatic vessels are then anastomosed to veins of the same calibre to restore the continuity of lymphatic drainage. It is also possible to anastomose lymphatic vessels directly with other surrounding lymphatic vessels.

Circumference reductions of almost 40 % and significant skin improvements can be achieved in the affected extremities.

This unique super-microsurgical technique requires many years of experience in microsurgery and the use of highly specialised instruments. In advanced stages of the disease, this surgical method is no longer feasible.

Transplanting healthy lymph nodes using microsurgical lymph node transfer (VLNT)

Lymph node transfer involves the removal of lymph nodes from a healthy region of the body, for example from the groin or collarbone area. These lymph nodes are removed together with the associated blood vessels and surrounding fatty tissue and then transferred to the affected limb. The blood vessels are microsurgically connected to the vascular system of the target area to ensure efficient blood flow to the transferred lymph nodes.

The transferred lymph nodes are able to absorb fluid from their surroundings and drain it via the blood vessel system. In the long term, new lymphatic channels can also be formed. Lymph node transfer not only reduces oedema, but also improves the local immunological situation. As a result, inflammations such as erysipelas rarely occur.

This procedure can reduce the extent of oedema by 60 to 80 % in the long term and significantly reduce the frequency of infections. However, it is important to note that it can take around a year for the full success of the procedure to be felt. Nevertheless, this procedure can also be performed on advanced and longstanding lymphoedema.

Alternatively, it is possible to restore the lymph flow in the affected extremities using free flap plasty ends. A free or pedicled skin flap with lymphatic vessels acts as a lymphatic bridge.

This treatment option utilises the spontaneous regeneration or reconnection of lymphatic vessels after a part has been replaced by the lymphatic bridge. The restoration of lymphatic channels after free tissue transfer has been confirmed in lymphoscintigraphic studies.

All of these diagnostic and therapeutic procedures require sophisticated techniques and instruments that are only offered in a few centres in Europe. We are the only clinic in the Zurich area to offer preoperative lymphatic vessel diagnostics and reconstructive lymphatic vessel surgery.

What follow-up treatment is required?

The exact follow-up treatment depends on the type of operation, the stage of the lymphoedema and the individual needs of the patient.

Ideal aftercare covers the following areas:

• Compression therapy: wearing compression stockings or sleeves can help to support lymph flow and reduce swelling.
• Physiotherapy: Physiotherapy exercises and lymphatic drainage techniques support mobility, lymphatic drainage and swelling control.
• Lifestyle changes: Patients can be instructed to focus on healthy eating, weight control and exercise to support lymphatic system health.
• Monitoring: Regular medical check-ups are necessary to monitor progress and make adjustments to the treatment strategy if necessary.

Lymphoedema is a chronic condition. Accordingly, certain risk factors must be taken into account - even after an operation. Experience has shown that heat (e.g. saunas), prolonged and heavy physical exertion and too many knocks/bumps can have the greatest negative effects on lymphoedema.