Lymphedema

Understanding the lymphatic system and why lymphedema occurs

The Lymphatic System

The human vascular system is composed of three main parts: the arterial system, the venous system and the lymphatic system. Arteries carry oxygen to cells and veins carry deoxygenated blood back to the lungs and heart. This flow of blood is constantly providing oxygen and nutrient rich fluid to every cell in the body. This fluid exits the blood capillaries to nourish and bathe the cells and tissues. It is then absorbed by the lymphatic capillaries, which are finger-like tips of the lymphatic vasculature that are positioned near the blood capillaries.

This lymph fluid then flows through a series of lymph nodes that filter it before it returns back into the circulatory system. As the fluid is filtered, the immune cells within lymph nodes are able to detect and mount an immune response to any bacteria or viruses that have entered the body. Some of the lymphatic system’s main roles are to provide immune functions that protect against the environment, remove wastes from the fluid that bathes your cells, and manage fluid volume in the tissues.

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Up to 10 million Americans suffer from lymphedema and lymphatic diseases.

Lymphedema

Up to 10 million Americans, and hundreds of millions of people worldwide, suffer from lymphedema and lymphatic diseases. More people suffer from these diseases in the U.S. than suffer from Multiple Sclerosis, Muscular Dystrophy, ALS, Parkinson’s disease and AIDS – combined. Lymphedema is poorly understood by the general public and grossly under-diagnosed or misdiagnosed by medical professionals.

Simply put, lymphedema is caused by excessive fluid trapped in the tissue of the body. Lymphedema that persists in the tissues causes chronic inflammation that can change the way the skin looks and feels. While skin may feel flexible at first, it can progressively become thicker or firmer (fibrotic). This is a sign that the delicate lymphatic system is damaged and these areas of skin are more susceptible to infection.

Often, lymphedema is represented by severe disfigurement or only relative to cancer treatment and lymph node removal. However, all swelling is lymphatic edema and so lymphedema will most likely affect every person at some point in his or her life. It is important to understand the difference between transient and chronic lymphedema and also when intervention is needed to prevent the harmful effects of lymphatic failure. MCS are an important part of maintaining a healthy lymphatic system, as well as reducing the symptoms of lymphedema.

Lymphedema & Chronic Venous Disease

In the U.S., the number one cause of leg lymphedema is Chronic Venous Disease (CVD). CVD is a vascular disorder affecting more than 80% of the world’s population. This is also significantly impacted by obesity, which is a major contributor to both venous and lymphatic disease.

Over time, increased work of the lymphatic system causes damage to the delicate lymphatic capillaries and vessels. These vessels have to work harder when blood circulation is poor, resulting in high venous pressures, leading to swelling. The lymphatic capillaries must then absorb this excess fluid, filter it, and transport it back into the circulatory system.

Common symptoms of CVD include:

  • Tired, achy legs
  • Varicose veins
  • Spider veins
  • Heavy, swollen legs
  • Restless legs at night
  • Muscle cramps
  • Leg tingling
  • Skin discoloration (rusty or purplish)
  • Thickening of skin tissue

Primary vs. Secondary Lymphedema

There are two main categorizations of lymphedema: Primary and Secondary. Primary refers to lymphedema as a result of abnormal or insufficient development of the lymphatic vasculature. This can present at birth or later in life and has a genetic component. In contrast, secondary lymphedema is swelling that results from some other cause. These include all the risk factors already noted above other than genetic predisposition. In the arms, lymphedema is most often secondary to breast cancer, while in the legs it is most often secondary to CVD.

There are a number of other factors that lead to leg lymphedema, such as side effects of medications, heart or kidney disease, immobility, obesity and stroke. Acute injury, surgery, or infections also contribute to lymphedema. Fortunately, treatments for lymphedema can be effectively utilized with all these types of problems, under the guidance of a physician and carried out by a certified lymphedema therapist. It is important that any person with persistent swelling consult with a physician to medically manage all contributing factors of the edema before starting lymphedema treatment. In addition, MCS are especially important in the management of early stage lymphedema.

Signs and symptoms of early lymphedema include:

  • Feeling of heaviness, tightness, fullness or stiffness
  • Aching
  • Observable swelling, shiny or stretched skin
  • Clothing or jewelry such as sleeve, shoe or ring becoming tighter

Signs and symptoms of chronic lymphedema include:

  • Pitting edema: when the swollen tissue is pressed for 30-60 seconds, the skin will look indented
  • Cobblestone or lumpy appearance of the skin, similar to a navel orange
  • Skin tissue feeling heavy and full of fluid, and can become thickened with denser fibrotic tissue that may or may not indent with pressure
  • Brown or greyish scale build-up on the skin
  • Oozing of lymph from the skin

Stages of Lymphedema

Depending on the individual and severity of the condition, a limb may exhibit more than one stage of lymphedema.

Stage 0: A latent or sub-clinical 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 1: Early accumulation of fluid relatively high in protein content which subsides with limb elevation. Pitting may occur. An increase in various types of proliferating cells may also be seen.

Stage 2: Limb elevation alone rarely reduces the tissue swelling and pitting is manifested.

Late Stage 2: Limb may no longer pit as excess subcutaneous fat and fibrosis develop.

Stage 3: 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 warty overgrowths have developed.

Risk Factors for Leg Lymphedema:

  • Cancer treatment – surgery with inguinal lymph node dissection, pelvic radiotherapy
  • Recurrent soft tissue infections, cellulitis
  • Obesity
  • Varicose vein stripping and vein harvesting
  • Genetic predisposition/family history of chronic edema
  • Intrapelvic or intra-abdominal tumors that involve or directly compress lymphatic vessels
  • Orthopedic surgery
  • Poor nutritional status
  • Thrombophlebitis and chronic venous insufficiency, particularly post-thrombotic syndrome
  • Chronic skin disorders and inflammation
  • Concurrent illnesses such as phlebitis, hyperthyroidism, kidney or cardiac disease
  • Immobilization and prolonged limb dependency
  • Living in or visiting a lymphatic filariasis endemic area

Lymphedema Treatment

The gold standard of conservative treatment for lymphedema is Complete Decongestive Physiotherapy (CDT). Traditionally, CDT has been used to successfully treat chronic lymphedema, but it has also been shown to be beneficial to functional outcomes when used for many other applications, such as pre and post orthopedic surgery, cosmetic surgery, vein procedures, sports medicine, stroke rehab, sinus congestion and more.

CDT is a comprehensive treatment approach that includes an acute, decongestive phase to reduce the lymphedema, and a maintenance phase. CDT treatments include multi-layered lymphedema bandaging or decongestive garments, long-term management with appropriate compression garments, skin and nail care, wound care, therapeutic exercises, manual lymphatic drainage, education and instruction in a home program for life-long management.

In addition to CDT, a number of physical modalities augment treatments during the acute and long-term management of lymphedema. These may include use of intermittent pneumatic compression pumps, negative pressure devices, oscillation therapy, low level laser therapy and various other approaches.

There are now many surgical, microsurgical, and super micro surgical approaches for lymphedema treatment. These include vascularized lymph node transfer (VLNT), lymphaticovenous anastomosis (LVA), and lymphnodal venous anastomosis (LNVA) or lymphaticolymphatic bypass. The procedures may move healthy lymph nodes from one area of the body to assist in lymphatic drainage in an area where lymph nodes are not working well, or were removed or damaged. These procedures can also connect lymphatic vessels to small veins so that the lymph can drain directly into the venous system. Additionally, suction-assisted protein lipectomy (SAPL) is a procedure to remove excess fatty and proteinaceous solids that have accumulated within the affected limbs.

Staying Healthy

Whatever the underlying cause of swelling, it is important to incorporate a healthy lifestyle and address lymphedema problems early. MCS along with physical activity is critical. MCS are ideal for mild to moderate swelling and early intervention. As lymphedema progresses, more robust and containing garments are required. Compression garments work in conjunction with active muscle contractions to help move lymph through the deep lymphatic vessels. Wearing compression and exercise such as walking, yoga, progressive resistance training, or cycling can help keep the lymphatic system healthy.

Citations

  • Bjork R, Ehmann S. STRIDE professional guide to compression garment selection for the lower extremity. Journal of wound care. 2019 Jun 1;28(Sup6a):1-44.
  • Dean SM, Valenti E, Hock K, Leffler J, Compston A, Abraham WT. The clinical characteristics of lower extremity lymphedema in 440 patients. Journal of Vascular Surgery: Venous and Lymphatic Disorders. 2020 Jan 25.
  • Executive Committee. The diagnosis and treatment of peripheral lymphedema: 2016 consensus document of the International Society of Lymphology. Lymphology. 2016 Mar 21;49(4):170-84.
  • Földi M, Földi E, Strößenreuther C, Kubik S, editors. Földi’s textbook of lymphology: for physicians and lymphedema therapists. Elsevier Health Sciences; 2012 Feb 21.
  • Framework, L., 2006. Best practice for the management of lymphoedema. International consensus. London: MEP Ltd, pp.3-52.
  • https://lymphaticnetwork.org/
  • Mortimer PS, Rockson SG. New developments in clinical aspects of lymphatic disease. The Journal of clinical investigation. 2014 Mar 3;124(3):915-21.
  • Rabe E, Guex JJ, Puskas A, Scuderi A, Fernandez FQ. Epidemiology of chronic venous disorders in geographically diverse populations: results from the Vein Consult Program. International angiology: a journal of the International Union of Angiology. 2012 Apr;31(2):105-15.
  • Rockson SG, Rivera KK. Estimating the population burden of lymphedema. Annals of the New York Academy of Sciences. 2008 May;1131(1):147-54.

Understanding Lymphedema

Lymphedema