Lymphedema

What is lymphedema?

Lymphedema is a chronic inflammatory disease of the connective tissue, resulting from congenital or acquired damage to the lymph drainage system. This condition leads to swelling of the soft tissues due to impaired lymphatic drainage, commonly affecting the arms or presenting as painless swelling in the extremities.

All the cells in the body are surrounded by a fluid which delivers essential nutrients, oxygen, and specific signaling molecules are delivered to the cells and then transported away. The space in which the cells are located and surrounded by fluid is called connective tissue, (interstitium). Under physiological conditions, there is a balance between the fluid moving through the arterial blood vessel walls and its removal via the lymph vessels and veins. This metabolic process is responsible for the supply of nutrients and removal of waste from various tissues in the human body.

A damaged or disrupted lymphatic drainage system leads to the permanent accumulation and alteration of the connective tissue fluid, resulting in lymphedema.

What causes lymphedema?

There are two main types of lymphedemas: primary and secondary. Around 90% of cases are secondary lymphedema.

There are many causes of secondary lymphedema with the major reason being the removal of lymph nodes from the body. The most frequent cause is the removal of axillary lymph nodes following breast cancer. However, it is also common to remove lymph nodes in the pelvis, groin or around the pelvic/abdominal aorta, as well as cervical lymph nodes after cancer of the thyroid gland or in the ENT area.

Inflammations of the skin, major soft tissue injuries (post-traumatic lymphedema), or self-inflicted soft tissue damage (artificial lymphedema) can also lead to swelling of the legs and arms. In addition, untreated vein illnesses like varicose veins can lead to lymphatic drainage disorders and thus lymphedema. If left untreated, it is a progressive, chronic disease.

Primary lymphedema arises from genetic developmental disorders of the lymph nodes or lymphatic vessels. It can appear at birth, during puberty or pregnancy but becomes less common after the age of 35. Triggering factors include minor injuries such as cuts or punctures, insect bites, sunburns and interdigital fungal infections.

Symptoms of lymphedema

In lymphedema, regardless of the cause, there is swelling of the affected area, resulting in a bulging sensation due to the fluid filling. Generally, and particularly in the early stages, lymphedema is painless.

Signs of the development of arm/leg lymphedema may include:

• heavy arm/leg

• pulling pain

• swelling, which could subside overnight

• mild fatigue of the arm/leg

• more pronounced indentation marks from stockings (compared to the other side)

Ultimately, this results in a chronic swelling of the legs that is typically not painful. Arm edema tends to develop on the side in which the axillary lymph nodes were removed. Over time, as the swelling continues, various skin changes may appear such as warts, pigmentation, athlete's foot and fistulas, to name a few. The skin around the lymphedema is particularly susceptible to infections, such as erysipelas.

Affected patients are advised to avoid

• physical overexertion

• extreme heat

• extreme cold

• injuries, including injections by the doctor

• blood pressure measurements on the affected side

as not to worsen the lymphedema or contribute to its development.

Moreover, primary and secondary lymphedema can have different symptoms.

Primary lymphedema often occurs symmetrically, i.e. on both sides of the body, for instance on both feet. Another characteristic of primary lymphedema is the upward spreading, from the feet via the ankles to the lower leg, for example. The edema may also increase in warm seasons or during menstruation in women.

A typical symptom are so-called box toes, whereby the toes take on a square shape due to the increased pressure. As the toes are bulging with fluid, there is no longer a fold of skin to be seen; doctors refer to this as the Stemmer's sign.

A secondary lymphedema is usually unilateral and progresses from top to bottom, such as from the armpit towards the hand or from the groin towards the foot. However, the forefoot and toes are typically not affected, which allows for differentiation between the various types of lymphedemas.

What are the stages of lymphedema?

The progression of lymphedema can be divided into four stages:

In the latent stage, there is no clinical swelling, but this does not mean that that the lymphatic vessels are anatomically intact and physiologically functioning. Which is why this stage can also be referred to as subclinical lymphedema.

In stage I, there is visible soft swelling that subsides when the affected area is elevated. It is spontaneously reversible.

In stage II, there is visible edema that does not disappear with elevation. It is not spontaneously reversible. There are usually some tissue changes such as fibrosis or other skin changes.

In stage III, the edema is also referred to as lymphostatic elephantiasis. This is very pronounced swelling with severe changes to the skin. The edema is hard and bulging. There may be pain caused by nerve pressure damage. The local body defenses are disturbed, which can result in skin inflammation and fungal infections.

Which body parts can be affected?

Lymphedema of the legs

More than 90% of primary lymphedema affects the legs. Women are significantly more frequently affected by this than men. In this condition there may be either no or too few lymph vessels, underdeveloped lymph nodes or enlarged lymph vessels. This form of lymphedema typically begins with a swelling in the foot, ankle and lower leg area.

Another problem can be the defective positioning of the valves in the lymphatic channels, whose normal function is to prevent the backflow of lymph fluid. If these do not function properly, lymphedema can develop.

Secondary lymphedema can develop in both the legs and arms. There can be many different causes. Secondary lymphedema after cancer operations in the genital area and in the small pelvis usually begins at the root of the extremities (groin/thigh).

Lymphedema of the arm

Secondary lymphedemas arise in the arm mainly in women because of medical procedures: about 40% of patients who have had their lymph nodes or vessels removed in their armpits during breast cancer surgery develop lymphatic drainage issues. The risk of developing lymphedema is further increased by the additional radiation therapy. Due to this major side effect, lymph nodes are now solely removed following careful consideration of the benefits and risks rather than as a routine procedure. 

Lymphedema treatment

Lymphedema conservative therapy

Lymphedema cannot be treated at its source or cured. There are, however, ways to reduce the swelling/edema. The main treatment is known as combined physical decongestion therapy (CDT), which is divided into a decongestion phase and a maintenance phase, which - in principle - consist of several pillars:

• Lymphatic drainage

• Compression therapy

• Decongestive exercise therapy

• Skin care

• Self-management 

According to Dr. Vodder, the manual lymph drainage according to is a special type of massage. It uses very gentle, skin-stretching movements to manipulate the tissue and influence the lymph vessel motility. At the same time, the grip sequence is used to mechanically shift the lymphatic loads in the direction of the functional lymph drainage system. With this particular massage technique, the drainage of the lymphatic system is increased, potential skin changes like fibrosis are softened and promotes the return flow. This method is earned by physiotherapists and masseurs as a further qualification by completing a four-week training course.

In compression therapy, different bandages are applied to the affected extremity in the decongestion phase of complete decongestive therapy (CDT). The short-stretch bandages that are used are individually adjusted so that uniform pressure is exerted on the limb which prevents the reflux of lymph into the edematous extremity. The bandaging supports venous drainage and the softening of fibrosis.

During the maintenance phase of complete decongestive therapy, an individually fitted flat-knit compression stocking is applied. Its effect is like that of a bandage, but it is less customizable over time. The compression stocking must be exchanged and re-fitted after 4 to 6 months if worn regularly.

Movement therapy utilizes a physiological principle which is rhythmic compression and stretching of lymph vessels by moving muscles. This action massages the lymph fluid in its vessels towards the heart, with the lymph valves functioning like valves to ensure the correct direction and prevent backflow.

Skin care aims to prevent tears and injuries. This care includes regular foot baths and the application of moisturizing cream.

In general, the more optimal the self-management of lymphedema, the better. These include self-treatments such as breathing therapy as well as massage techniques in the underarm and groin area, daily physical exercise in compression stockings and reliable skin care of the edematous extremity. Dietary measures and psychological support are also part of self-management. The better the patient can deal with their chronic illness, the better the symptoms of lymphedema can be managed.

Lymphedema self-management

The aim of self-management in the case of a diagnosed lymphedema is to keep lymph congestion to a minimum for as long as possible. We recommend the following practices, which also apply to patients with existing lymphedema:

• Elevate legs and arms several times a day

• Carefully care for the skin

• Wear individually fitted compression stockings permanently and reliably

• Avoid physical overexertion

• Avoid exposing the affected extremity to excessive heat (sunbathing, hot oven, etc.)

• Avoid exposing the affected extremity to extreme cold (building a snowman with bare hands, etc.)

• Avoid injuries, including injections by the doctor

• Avoid blood pressure measurements on the affected side

• Do not wear constricting clothing and shoes

• Avoid insect bites

Lymphedema surgery

If the conservative methods do not work, new surgical techniques have been available for several years to treat lymphedema. Particularly in the early stages, before skin changes such as fibrosis occur, microsurgical procedures can be used to improve lymph transport. Lymph transport can be improved by lymphatic duct transplantation or lymph node transplantation. In both procedures, only the patient's own tissue is transplanted. The lymph nodes are removed from another part of the body beforehand. These microsurgical procedures can only be carried out after good preoperative diagnostics with a functional lymphoscintigraphy and sonography of the lymph vessels.

Which doctors and clinics specialize in lymphedema treatment?

The conservative treatment of lymphedema falls within the expertise of doctors with additional qualifications in lymphology. This qualification can be acquired as part of a four-week training course at the Dr. Vodder Academy in Walchsee, Austria. However, in Austria, the management of specialized hospitals or rehabilitation clinics for lymphedema patients is the responsibility of a specialist in physical medicine, internal medicine, or dermatology.

Once Stage II lymphedema has been diagnosed, a stay in a rehabilitation clinic for lymphedema patients is recommended. The therapies and training provided there help patients gain a strong understanding of self-management techniques. With good compliance, patients can effectively control their condition in an outpatient setting and sustain the improvements achieved during their inpatient stay.

At Primo Medico, we want to help patients find the most experienced and competent specialists for their particular condition. That is why all of the specialists and clinics listed here have been carefully reviewed and selected. All of them are experts in the field of lymphology and competent contacts for all aspects of lymphedema. Benefit from the experience of our specialists and schedule your first personal consultation quickly and easily.