Lymphedema

Lymphedema is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system. Treatment includes medical compression, among others.

Lymphedema is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system. The lymph system returns interstitial fluid to the bloodstream. It has an important role in immune defense and acts as “waste disposal and recycling” for our bodies.

Many different factors can compromise the lymphatic system. Genetic predisposition, as well as secondary factors like cancer, infections, surgery, trauma, chemotherapy and radiotherapy and even obesity, can be causes of lymphedema.

Depending on the severity and stage of the disease, treatment of lymphedema includes decongestive therapy with medical compression, manual lymph drainage, skin care, exercises, surgery, and excisional procedures.

The lymphatic system

The lymphatic system is a large network of lymphatic vessels and lymph nodes that plays an important role in transporting lymph fluid, immune function, fluid homeostasis, blood cleaning, and blood filtering.

The lymphatic system is our “waste disposal and recycling” unit. It transports waste products and toxins out of the body. Body waste products include proteins, metabolic breakdown products, inflammatory products, and fat from the abdominal cavity.

The lymphatic vessels, which are spread throughout the body like a net, carry a clear fluid called lymph towards the thoracic duct, which drains into the blood circulation at the venous-lymphatic junctions in the neck.

How the lymphatic system works

Lymph is formed from the fluid that filters out of the blood circulation into the interstitium and taken up by the blind-ended lymph-sinuses. The lymph consists of interstitial fluid, proteins (smaller than albumin), fibrinogen, and other coagulation factors, small molecules and ions of the serum and interstitium, leucocytes, immunoglobulins, fat in form of chylomicrons, cellular debris, waste products, and bacteria.

The lymphatic system is responsible for the majority of fluid uptake from the interstitial spaces. This collection of fluid is carried out by the initial lymph-sinuses, that are blind-ended epithelial lined vessels with fenestrated openings that allow fluids and particles as large as cells or proteins smaller than albumin to enter.

The fluids are then sucked and pressed into lymph pre-collectors. The lymph collectors and continually larger lymph vessels have a lymph valve system and lymphangiones (e.g. enlarged units with smooth muscle cells between two lymph vessels).

Through active contraction of the lymphangione and restricted back flow of lymph due to the lymph vales, the lymph is transported in proximal direction through the lymph vessels and through lymph nodes.

Hundreds of lymph nodes are located within the human body (e.g. around the neck, intestinal tract or in the axilla or groin area) and are responsible for the removement of debris, regulation of the protein content of the lymph, the immune response, recirculation of lymphocytes, and re-absorption of water (approximately 5 to 8 liters per day).

Every day the lymphatic system takes up approximately 10 liters of interstitial fluid.

Intact lymphatic drainage

If the lymphatic drainage is intact, the initial lymph vessels, vessels with blind ends and windowed openings, transport the lymph fluid from the interstitial spaces.

Interrupted lymphatic drainage

Lymphedema can develop if the lymphatic drainage is interrupted, impaired, or if secretion of fluid into the interstitial tissue exceeds the uptake capacity of the lymph system.

What are lymphatic diseases and what is lymphedema?

Lymphatic disease is a class of disorders that directly affects the lymphatic system. When the lymphatic system is compromised – due to congenital malformation or damage to the lymphatic system (cancer, surgery, trauma) –, protein-rich lymph fluid can accumulate in the soft tissue, leading to swelling in one or more limbs, or in other body parts.

This condition is called lymphedema.

The following factors can lead to a progression or aggravation of existing lymphedema:

Surgery (lymph node removal for biopsies)
Trauma that might damage the lymphatics
Radiation and chemotherapy as cancer treatment
Serious infections of the skin or lymphatic channels (lymphangitis, cellulitis, erysipelas, microscopic parasite filarial larvae)
Obesity (it can damage the lymphatics)

Causes, symptoms, and stages of lymphedema

Symptoms of lymphedema

Swelling (called edema or congestion), mainly in the lower and upper extremities (including the fingers and toes), orin other regions of the body (e.g. neck, genital region, inguinal region, face, etc.)
Heaviness or tightness in the limbs
Restricted range of motion
Thick or hardened skin
Positive Stemmer’s sign: This means that a fold of skin cannot be pinched and lifted up at the base of the second toe, the base of the middle finger and other regions of the body where swelling is located.

Disorder subtypes

Lymphedema is classified as primary or secondary.

Primary lymphedema is thought to be the result of a congenital abnormality of the lymph conducting system. It is estimated that primary lymphedema represents 5 to 10% of lymphedema cases (Cooper, 2017).
Secondary or acquired lymphedema results from damage to the lymphatic system (lymphatic vessels and/or lymph nodes) or from functional deficiency. Infections from insect bites, serious wounds, or burns can cause lymphedema when they damage or destroy the lymphatic system. Any type of surgery, serious injury, or radiation for cancer treatment can also cause the onset of the disease. Secondary lymphedema is thought to represent 90 to 95% of lymphedema cases (Cooper, 2017).

Lymphedema staging

Depending on disease progression, lymphedema can be divided into three stages

Stage 0 (or Ia) refers to a latent or subclinical condition where swelling is not yet evident despite impaired lymph transport, subtle alterations in tissue fluid/composition, and changes in subjective symptoms. It may exist months or years before overt edema occurs.
Stage I represents an early accumulation of fluid relatively high in protein content which subsides with limb elevation. Pitting may occur (when pressed by the fingertips, the affected area indents and reverses with elevation).
Stage II signifies that limb elevation alone rarely reduces the tissue swelling and pitting is manifest. Later in Stage II, the limb may not pit as excess subcutaneous fat and fibrosis develop.
Stage III represents lymphostatic elephantiasis where pitting can be absent and trophic skin changes such as acanthosis, alterations in skin character and thickness, further deposition of fat and fibrosis, and overgrowths have developed.

Lymphedema stages in the leg

Lymphedema stages in the arm

Treatment of lymphedema

The management of lymphedema involves a multicomponent approach including physical and psychosocial interventions. The gold standard of lymphedema treatment is complex decongestive therapy with four cornerstones: manual lymphatic drainage (MLD), compression therapy, skin care, and exercise.

Depending on the severity and stage of the disease, other treatment options, like surgery or excisional procedures, might be applied.

Gold standard treatment: complex decongestive therapy

Complex deco ngestive therapy is performed in two phases

First phase of complex decongestive therapy

The first phase consists of specific manual massage (manual lymphatic drainage, MLD), application of medical compression (typically applied with multi-layered bandage wrapping), skin care, and sometimes deeper exercise techniques for certain patients by using muscle pumping exercises. The treatment goal of the first phase is to reach a maximum volume reduction in the affected edematous region(s).

Second phase of complex decongestive therapy

The second phase (initiated directly after Phase 1) aims to conserve and optimize the results obtained in the first phase. It consists of medical compression by a low-stretch elastic stocking or sleeve (see below), skin care, continued exercise, and repeated MLD as needed.

The cornerstones of complex decongestive therapy

Manual lymphatic drainage (MLD)

MLD is a type of massage applied to encourage the natural drainage of the lymph.
Manual lymph drainage uses a specific amount of pressure (less than 9 ounces per square inch or about 4 kPa) and rhythmic circular movements to stimulate lymph flow.

Bandaging

Used mainly in the first phase of the complex decongestive therapy together with manual lymphatic drainage (MLD) to reduce limb volume and soften tissue
Suitable for odd limb shapes
Time-consuming
Usually cannot be applied by the patient

Flat- and round-knit solutions

Compression hosiery is mainly used in the second phase of the complex decongestive therapy for the long-term management of lymphedema.

Least bulky compression option used only in stabilized limb volume conditions in the ideal case.
Flat-knit can fit more extreme limb shapes better than round-knit compression hosiery, therefore it is used in the more severe stages of lymphedema.
Round-knit is more suitable for the less severe stages of lymphedema.

Wraps (velcro/bra hooks)

Used during decongestion or in the stabilizing phase of the lymphedema treatment (mainly second phase, but sometimes in the first phase of complex decongestive therapy)
Easy to don and doff
Easily adjustable to vary the compression and to adapt to limb volume changes

Intermittent pneumatic compression (IPC)

IPC is a mechanical method that mimics MLD by massaging the fluid out of the limb
Should be only used in conjunction with MLD to favor truncal drainage
Stationary devices which hinder movement during treatment
Compression garments must be used to prevent re-accumulation of fluid after IPC treatment

Skin care

Chronic disturbance of lymph flow results in inflammation in the swollen body parts with increased activity and proliferation of the skin cells. The function of the immune system is reduced in chronic lymphedema patients. If the skin is not properly treated, severe inflammation or infections, such as cellulitis, can occur. Maintenance of skin integrity and careful management of skin problems in patients with lymphedema are important to minimize the risk of infections.

Skin care principles include:

Washing daily, using pH neutral soap, natural soap, or a soap substitute
If skin folds are present, ensuring they stay clean and dry
Monitoring the affected and unaffected skin for cuts, abrasions and bites
Applying emollients
Low pH lotions
Avoiding scented products

Exercise

Basic motions of the extremities (muscle pumping exercises), especially combined with external limb compression and preferably performed as daily life activities (walking, yoga, bicycling, climbing stairs), are useful.

Other treatment options for lymphedema

Surgery – can help to improve lymphatic flow

Microsurgical procedures: Lymphatic vessels are connected to venules to enhance lymphatic drainage.
Vascularized lymph node transfer: Lymph nodes with surrounding tissue are transferred from a part of the body to a damaged site. With the help of microsurgery, the nodes are connected to blood vessels; lymph vessels connect themselves to the implant.
Bypass: Lymph vessels from damaged areas are connected to areas that are still functioning

Liposuction

Excessive volume, mainly fat and protein, is removed with a liposuction-like procedure.

Surgical resection

Removal of excess skin and subcutaneous tissue of the lymphedematous limb (in extreme cases).

Kinesiotapes

For stimulation of lymph flow in patients with breast-cancer related lymphedema.

Nutrition

Helps to reduce weight
Helps to reduce inflammatory processes
Special diet plans available
Supplements like chitosan, grapeseed oil, and selenium are supposed to have a positive impact on the disease