Lymphedema

Overview

Lymph vessels are part of a one-way transport system which facilitates the return of tissue fluids back into the bloodstream.  Along the way, this fluid is routed through lymph nodes which protect the body from infection and disease by storing and distributing the lymphocytes that attack dangerous pathogens (virus or microorganism).

Metabolic waste and plasma proteins that are too large to be returned or reabsorbed by the venous capillaries are also filtered through the lymph nodes.  Anytime this lymphatic transport mechanism is compromised, whether by surgery, radiation, congenital deficiency or malformation, stagnation and swelling will develop, proteins will accumulate and the affected area will be at great risk for infection.  

The condition is progressive and if it is left untreated the impairment becomes so great that the lymphatic fluid exceeds the lymphatic transport capacity and an abnormal amount of protein-rich fluid collects in the tissues of the affected area.  This stagnant, protein-rich fluid not only causes tissue channels to increase in size and number but also reduces oxygen availability in the transport system, interferes with wound healing, and provides a culture medium for bacteria that can result in lymphangitis (infection of lymphatic vessels).  Infection in the leg spreads much faster and must be treated as an emergency.  Infection scars down the remaining lymphatic channels making your swelling and edema many times worse.

Lymphedema should not be confused with edema resulting from venous insufficiency, which is not lymph-edema.  However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder which is treated in the same way as lymphedema .  Sometimes the venous disease must be treated as well.

Lymphedema is an accumulation of lymphatic fluid in the connective tissue between the cells of a structure (interstitial tissue) that causes swelling most often in the arms and/or legs and occasionally in other parts of the body.  Primary lymphedema can develop when lymphatic vessels are missing or impaired.  Secondary lymphedema can develop when lymphatic vessels are damaged or lymph nodes removed.  Infection in the legs or arms makes this many times worse.

What is the cause of lymphedema?

 There are two types of lymphedema:

  • Primary lymphedema is the result of a design flaw of the lymph vessels and is a rare, inherited condition.  It can be congenital and is apparent at birth, it may not become more apparent until puberty (lymphedema praecox), it may not become apparent until mid and later life (lymphedema tarda).
  • Secondary lymphedema results from identifiable damage to or obstruction of normally functioning lymph vessels and nodes. This can be from surgical excision and radical cancer surgery or from radiation treatment for cancer.

Worldwide lymphedema is most commonly caused by filariasis (a parasitic infection); but in the United States, lymphedema most commonly occurs in women who have had breast cancer surgery, particularly when followed by radiation treatment and infection.  It may also follow radiation to your pelvis and to the lymphatics that drain through your pelvis from your legs.

Primary lymphedema causes

Primary lymphedema is an abnormality of an individual’s lymphatic system and is likely present at birth, although symptoms may not become apparent until later in life.  Depending upon the age at which symptoms develop, three forms of primary lymphedema have been described.  Most primary lymphedema occurs without any known family history of the condition.

Familial lymphedema should be classified in two categories depending on onset:

  • Milroy’s disease, or congenital hereditary lymphedema, is present from birth, painless, without tendency to ulcerate, and may have cholestasis or intestinal lymphangiectasia associated with it.  This becomes apparent between birth and the first year of life.
  • Meige’s disease, hereditary lymphedema praecox, is lymphedema with onset in the first or second decade, often presenting with inflammation, and may have a number of associated related anomalies, or congenital defects.  This is generally considered to have an onset between ages of 1 and 35 years.

Congenital lymphedema is evident at birth, is more common in females, and accounts for 10%-25% of all cases of primary lymphedema.

Lymphedema praecox is the most common form of primary lymphedema making up 65%-80% of cases.  It is defined as lymphedema that becomes apparent after 1 year of age and before age 35 years and symptoms most often develop during puberty.  Lymphedema praecox is four times more common in females as in males.  

Primary lymphedema that becomes evident after 35 years of age is known as lymphedema tarda.  It is less common than congenital lymphedema and lymphedema praecox and accounts for 10% of cases of primary lymphedema.

Secondary lymphedema causes

Secondary lymphedema develops when a normally functioning lymphatic system is blocked or damaged.  In the United States breast cancer surgery, particularly when combined with radiation treatment, is the most common cause.  This results in one-sided (unilateral) lymphedema of the arm.  Any type of surgical procedure that requires removal of regional lymph nodes or lymph vessels can potentially cause lymphedema.  

Damage to lymph node and lymph vessels leading to lymphedema can also occur due to trauma, burns, radiation, infections, or compression or invasion of lymph nodes by tumors.

Filarial lymphedema

Worldwide filariasis is the most common cause of lymphedema.  Filariasis is the direct infestation of lymph nodes by the parasite Wuchereria bancrofti.  The disease is spread among persons by mosquitoes and affects millions of people in the tropics and sub-tropics of Asia, Africa, Western Pacific, and parts of Central and South America.  Infestation by the parasite damages the lymph system and leads to swelling in the arms, breasts, legs, and, for men, the genital area.  The entire leg, arm, or genital area may swell to several times its normal size.  Also, the swelling and the decreased function of the lymph system make it difficult for the body to fight infections.  Lymphatic filariasis is a leading cause of permanent disability in the world.  It is, therefore, important you inform your doctor of any travels you have made to those areas of the world.

Who is at risk for lymphedema?

Worldwide millions of people are afflicted with lymphedema.  Some are born with the disease and the symptoms may appear at birth or later in life.  This is called primary lymphedema and there is little research into its cause.

Anyone who has had cancer treatment that affects the lymph nodes, such as surgery for breast cancer, needs to be aware of the possibility of secondary lymphedema.  Secondary lymphedema is often the unintended consequence of life-saving cancer treatments.  

You may be at risk for filarial lympedema if you have ever traveled to any tropical countries, Asia, Africa, Western Pacific or parts of Central and South America and should be certain to inform your doctor of those trips and time spent in those countries.  I have only seen this in one patient who developed filariasis and lymphedema after visiting her family in Africa for only two weeks.

What are the complications of lymphedema?

Perhaps the greatest complication of lymphedema is infection.  Because lymphedema leads to immune deficiency in the affected limb or area, persons with this disease are increasingly subject to infection due to the inability of the stagnant lymph system to carry away impurities.  Inflammation of the skin and connective tissues, known as cellulitis, and inflammation of the lymphatic vessels (lymphangitis) are common complications of lymphedema.  Deep venous thrombosis (formation of blood clots in the deeper veins) is also a known complication of lymphedema.  Preventing infection is essential because each successive infection can further compromise the lymphatic and immune systems.  In the most severe cases, infections can be life threatening.

If left untreated, lymphedema can have other complications.  Severe swelling, discomfort, hardening of tissue (scar tissue of the subcutaneous tissues and fat), damage to skin, or even nerve damage due to the heavy weight of the limb that can threaten the usefulness of an arm or leg.  As this progresses and scar tissue becomes worse, the leg is obviously edematous but the tissues are hard and it will not pit (when you push your thumb in over your tibia, it will not leave an indentation).

Those who have had chronic, long-term lymphedema for more than 10 years have about a 10% chance of developing a cancer of the lymphatic vessels known as lymphangiosarcoma.  The cancer begins as a reddish or purplish lump visible on the skin and spreads rapidly.  This is an aggressive cancer that is treated by amputation of the affected limb.  Even with treatment, the prognosis is poor.

Finally, but of no less importance, persons with lymphedema may be affected by emotional problems such as depression or anxiety, particularly if they feel isolated and know little about the condition.

What are the signs and symptoms of lymphedema?

Lymphedema can develop in any part of the body or limbs.  Signs or symptoms of lymphedema to watch out for include:

  • A full sensation in the limb(s)
  • Skin feeling tight, decreased flexibility in the hand, wrist or ankle
  • Difficulty fitting into clothing in one specific area, or ring/wristwatch/bracelet tightness.

If you notice persistent swelling, it is very important that you seek immediate medical advice (and get at least one second opinion) as early diagnosis and treatment improves both the prognosis and the condition.  Early it may be suspected but hard to diagnose.

Lymphedema develops in a number of stages, from mild to severe, referred to as Stage 1, 2 and 3:

Stage 1 (spontaneously reversible)

Tissue is still at the “pitting” stage, which means that when pressed by fingertips, the area indents and holds the indentation.  Usually, upon waking in the morning, the limb(s) or affected area is normal or almost normal size.

Stage 2 (spontaneously irreversible)

The tissue now has a spongy consistency and is “non-pitting,” meaning that when pressed by fingertips, the tissue bounces back without any indentation forming).  Fibrosis found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size.

Stage 3 (lymphostatic elephantiasis)

At this stage the swelling is irreversible and usually the limbs are very large.  The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called “debulking” at this stage.

When lymphedema remains untreated, protein-rich fluid continues to accumulate leading to an increase of swelling and a hardening or fibrosis of the tissue.  In this state, the swollen limb(s) becomes a perfect culture medium for bacteria and subsequent recurrent lymphangitis (infections).  Moreover, untreated lymphedema can lead to a decrease or loss of functioning of the limbs, skin breakdown, chronic infections and sometimes irreversible complications.  In the most severe cases, untreated lymphedema can develop into a rare form of lymphatic cancer called lymphangiosarcoma (most often in secondary lymphedema).

Signs and symptoms of lymphangitis (infection)

This may include some or all of the following:

  • Rash
  • Red blotchy skin
  • Itching of the affected area
  • Discoloration
  • Increase of swelling and/or temperature of the skin
  • Heavy sensation in the limb (more so than usual)
  • Pain
  • In many cases a sudden onset of high fever and chills.

Treatment for infections:  Immediately discontinue ALL current lymphedema treatment modalities (including manual lymphatic drainage, bandaging, pumps, wearing of compression garments) and contact your physician immediately or go to the nearest emergency room.  Do not wait until Monday to be seen.  Some people keep prescriptions for antibiotics so they can get started immediately but the emergency room is the correct answer

NOTE:  Always carry antibiotics or a prescription with you  and especially when you travel.

How is lymphedema diagnosed?

A thorough medical history and physical examination are preformed to rule out other causes of limb swelling, such as edema due to congestive heart failure, kidney failure, blood clots or other conditions.  Often, the medical history of surgery or other conditions involving the lymph nodes will point to the cause and establish the diagnosis of lymphedema.

If the cause of swelling is not clear, other tests may be carried out to help determine the cause of limb swelling.

  • CT or MRI scans may be useful to help define lymph node architecture or to identify tumors or other abnormalities (to include the abdomen and chest).
  • Lymphoscintigraphy is a test that involves injecting a tracer dye into lymph vessels and then observing the flow of fluid using imaging technologies. It can illustrate blockages in lymph flow.  It is injected into a lymph channel in your foot.  If it is used, only a few stitches will be needed.
  • Doppler ultrasound is a soundwave test used to evaluate blood flow and can help identify any blood clot in the veins (deep vein thrombosis) that may be a cause of limb swelling.

What is the treatment for lymphedema?

There is no cure for lymphedema and planning the treatment program depends on the cause of the lymphedema.  For example, if the initial signs and symptoms of swelling are caused by infection (redness, rash, heat, blister or pain may indicate an infection), antibiotics will first need to be prescribed.  Treating an infection often reduces some of the swelling and discoloration.

If the lymphedema is not caused by infection and depending on the severity of the lymphedema, combined decongestive therapy (CDT) compression treatments can help reduce swelling and prevent scarring and other complications.  Examples of compression treatments are:

  • Elastic sleeves or stockings:  These must fit properly and provide gradual compression from the end of the extremity toward the trunk.  These are started after the size of the limb is treated to compress it to the smallest size that can be achieved is achieved.
  • Bandages:  Bandages that are wrapped more tightly around the end of the extremity and wrapped more loosely toward the trunk, to encourage lymph flow out of the extremity toward the center of the body
  • Pneumatic compression devices:  These are sleeves or stockings connected to a pump that provides sequential compression from the end of the extremity toward the body. These may be used in the clinic or in the home and are useful in preventing long-term scarring, but they cannot be used in all individuals such as those with congestive heart failure, deep venous thrombosis, or certain infections.
  • Manual compression:  Massage techniques, known as manual lymph drainage, can be useful for some people with lymphedema.  This is known as milking the leg and usually requires another family member to be trained to help you.
  • Exercises:  Exercises that lightly contract and stimulate arm or leg muscles may be prescribed by your doctor or physical therapist to help stimulate lymph flow.

Although combined decongestive therapy (CDT) has proved to be very successful, lymphedema therapists and educators are discovering the vital role of patient education in the prevention and management of this condition as there is no cure and long-term maintenance inevitably becomes the responsibility of the patient.  Those patients who have the opportunity to learn the basic anatomy and physiology of the lymphatic system better understand the risks and their own condition and they are more willing to make the commitment to learn and implement self-care techniques.

With daily self-care practices, the likelihood of recurrence of the worst edema and/or infection is greatly reduced and patients have the peace of mind that comes with knowing they can keep the condition under control and contribute to their own well-being.

In severe cases, surgical treatments for lymphedema are used to remove excess fluid and tissue, but no surgical treatment is able to cure lymphedema.  Surgery is absolutely the last resort and will leave your leg grossly disfigured.  You trade one disfigured leg for another disfigured leg and the lymphedema is still not cured.

Infections of skin and tissues associated with lymphedema must be promptly and effectively treated as an emergency with appropriate antibiotics to avoid spread to the bloodstream (sepsis).  

Again the need to always have on hand antibiotics or a prescription is emphasized.  Should signs of infection begin over a weekend, holiday or while traveling, time is of the essence to avoid any further loss of lymphatic channels.

If lymphedema is bad, you will also be more susceptible for topical fungal infections because the leg is always moist.

Prevention

It is important for any patient undergoing cancer treatment or anyone with a family history of primary lymphedema to understand the principles of lymphatic return.  If normal lymph transport capacity is ten times greater than normal lymph load (the accumulation of tissue fluid not reabsorbed by the venous capillaries), the body can quickly recover from traumatic injury or swelling.  If the transport capacity is permanently compromised for any reason and this 10:1 ratio decreases, the likelihood of developing chronic lymphedema increases.

A patient may remain in the latency stage of lymphedema for years following the initial insult until something as minor as a mosquito bite or an airplane ride pushes them over the edge and the swelling becomes obvious.  There are self-care techniques which can help restore lost transport capacity.  The well-informed patient who is willing to explore and implement these safeguards is more likely to discover lymphedema does not need to control their life.

Primary lymphedema cannot be prevented, but measures can be taken to reduce your risk of developing lymphedema if you are at risk for secondary lymphedema, such as after cancer surgery or radiation treatment.

The following steps may help reduce the risk of developing lymphedema in those at risk for secondary lymphedema:

  • Keep the affected arm or leg elevated above the level of the heart when possible and as often as possible (water runs downhill but not well with lymphedema but only slowly with lymphedema).  It  resolves much slower than edema from varicose veins or congestive heart failure.
  • Avoid tight or constricting garments or jewelry (also avoid the use of blood pressure cuffs on an affected arm).
  • Do not apply a heating pad to the affected area or use hot tubs, steam baths, etc.
  • Keep the body adequately hydrated.
  • Avoid heavy lifting and forceful activity with the affected limb but normal, light activity is encouraged.
  • Do not carry a heavy purse on an affected arm.
  • Practice thorough and careful skin hygiene.
  • Avoid insect bites and sunburns.

While nothing in medicine can be predicted with complete certainty, there are many ways to influence a chronic condition like lymphedema and improve the quality of a patient’s life, health, and emotional well-being.  There are now more and more lymphedema specialists who can assist with management not just of the outward signs and symptoms of lymphedema but the emotional stress that can also affect patients.

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