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.
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 lymphangiones 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.
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:
Lymphedema is classified as primary or secondary.
Depending on disease progression, lymphedema can be divided into three stages
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.
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).
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.
Manual lymphatic drainage (MLD)
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.
Wraps (velcro/bra hooks)
Intermittent pneumatic compression (IPC)
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:
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.
Excessive volume, mainly fat and protein, is removed with a liposuction-like procedure.
Removal of excess skin and subcutaneous tissue of the lymphedematous limb (in extreme cases).
For stimulation of lymph flow in patients with breast-cancer related lymphedema.