Lymphedema Specialists and Information

Like blood, lymph consists of fluid (lymph plasma) and cells (corpuscular components). The difference between blood and lymph is that lymph transports larger cells that 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, compared to the venous blood vessel system (which also transports fluid from tissues distant from the heart toward the heart), larger components of the tissue fluid can be absorbed and transported.

Typical examples of larger cells transported by lymph fluid include:

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

The lymphatic system consists of lymphatic vessels through which lymph is transported toward 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 even 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.

To ensure that all foreign bodies and pathogens are removed from the lymph fluid before it enters the bloodstream, filtering mechanisms are in place. These are also called lymph nodes and swell when there is an increased influx of pathogens that need to be eliminated (e.g., during inflammation). An example of this is palpable enlargement of the cervical lymph nodes in cases of upper respiratory tract infections or dental problems.

Lymphedema is a buildup of fluid in the interstitial spaces. The body produces approximately 2–3 liters of lymph fluid per day. Lymphedema is caused by an inability to properly drain lymph fluid, leading to congestion in the interstitial spaces. Swelling very often occurs in one or both legs. However, other parts of the body, such as the arms, can also be affected. Lymphedema can also occur in the neck, head, trunk, or genital area, though it is less common in these regions.

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

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

A distinction is made between primary and secondary lymphedema. 

Primary lymphedema is hereditary; in other words, it is not caused by other diseases or external factors. Medically, this is also referred to as hereditary (genetically caused) lymphedema. In women, it often becomes noticeable during pregnancy. In some cases, there are also conditions in which lymphatic vessels are missing in certain areas of the body—that is, they were not properly formed. This then leads to impaired drainage of lymph fluid.

Secondary lymphedema can have various causes, such as metabolic disorders like diabetes mellitus or chronic skin conditions. Cancers (and cancer surgery), accidents, and infections caused by viruses, bacteria, worms, and fungi can also be included in this category. The same applies to radiation therapy, surgical scars, or chronic venous insufficiency. 

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

However, the removal of lymph nodes usually damages important lymphatic vessels in the armpit, which can subsequently disrupt the drainage of lymph fluid. The same applies to surgeries in the groin (e.g., for vascular stenosis), during which lymphatic vessels may be damaged. Since lymphatic vessels are not visible to the naked eye, lymphedema (also known as lymph fistulas) is a common complication of surgical procedures.

Lymphedema is very easy to spot due to the bulging accumulation of fluid. The leg or arm is thickly swollen and appears puffy. In addition, these swellings can also be felt. While it is still possible to press a dent into the skin in the early stages, the swelling becomes firm in advanced stages. It is then 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 over the second and third toes—or fingers—can no longer be lifted, this test is considered positive. Lymphedema is generally classified into several stages:

• Initial stage: In this early phase, there are no significant symptoms yet; the edema is only subtly present.
• First stage: The lymphedema is now visible and palpable. It feels relatively soft, so that a dent can be pressed into the affected area. This dent resolves on its own after a short time. However, Stemmers’ sign is already positive at this stage.
• Second stage: It is no longer possible—or only possible to a limited extent—to indent the skin; the edema becomes firmer. While elevating the affected body part could still bring about improvement in the first stage, 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. In addition, the skin may react with eczema or other conditions. Furthermore, wounds in this area heal only slowly. At this stage, the condition is referred to as elephantiasis.

In principle, it is recommended to first consult your primary care physician, who is best acquainted with your medical history and is best positioned to investigate the causes. Lymphatic complications following surgical procedures should continue to be monitored by the surgeon who performed the original procedure. Sometimes, another surgery (known as a revision procedure) is necessary to close the opened lymphatic vessels. This is usually done by coagulating the wound surfaces (thermal ablation); even during revision surgery, it is rarely possible to directly visualize the opened lymphatic vessels.

The following treatment methods are available:

• In the early stages: Elevation
• Compression therapy
• Lymphatic drainage
• Exercise therapy
• Surgical therapy

What options are available for compression therapy?

Treatment depends not only on the cause but also on how far the lymphedema has already progressed. In a very early stage, elevating the affected limb—such as the leg—can help promote lymphatic drainage. In addition, consistent compression therapy is a top priority, as it can usually prevent the disease from progressing. For open wounds, inflamed tissue, and highly sensitive or painful skin, elastic compression bandaging is recommended initially. These dressings generally need to be changed several times a day, especially 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 in this regard. 

In cases of unremarkable skin conditions (e.g., arm swelling due to lymphedema following breast cancer surgery), wearing a medical compression stocking is recommended instead of an elastocompressive bandage. This is fitted at a medical supply store. Such stockings are also available for the legs. However, these are very sturdy, firm stockings (compression class III, pressure approx. 40–45 mmHg), which can be difficult to put on and take off and should be done with 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 proven to be very effective. This is a special massage technique that stimulates the lymphatic system, thereby improving the drainage of fluid. This technique is also used after surgeries involving severe swelling and is performed by a physical therapist. 

Are there medications for lymphedema?

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