What is Lymphedema?
The lymphatic system is a network of tiny vessels and organs that help rid the body of toxin and waste. Its primary function is to transport lymph, a fluid rich in protein and in white blood cells and lymphocytes which are a primary agent in fighting infection throughout the body. Lymph travels through tiny and very fragile channels called lymphatics. These channels connect to lymph glands which act as a filtering organ and help our body fight infection.
When these small channels or the lymph glands themselves get damaged, lymph flow is obstructed, the filtering capacity of the lymphatic system is overwhelmed, and lymph fluid starts leaking out from these fragile and small channels into the surrounding tissues. Anytime this lymphatic transport mechanism is compromised, whether by surgery, radiation, hereditary deficiency or malformation, lymph fluid and proteins will accumulate and swelling will occur in the affected extremity.
Lymphedema (which literally means lymph swelling) results from a build-up of lymph fluid in the extremities, most frequently in the arms or legs. When allowed to get out of control, it can become quite disabling putting patients at much higher risk of serious infection.
At South Charlotte General and Surgery, our vascular specialists are experts in the diagnosis, management and advanced treatment of lymphedema and are here to help you understand and manage this difficult, and often lifelong condition – request an appointment today.
Is lymphedema a serious condition?
The condition may be progressive in some patients, and if left untreated, the impairment of the lymphatic system becomes so great that it can potentially create a situation where the oxygen availability to the tissues is markedly reduced system, setting the stage for delayed wound healing, and providing a culture medium for bacteria to grow. If an infection sets in in the lymphatics (a condition called lymphangitis), the 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.
What causes lymphedema?
Lymphedema may result from an underdeveloped and ineffective lymphatic system which starts at birth and progresses thru adolescence and adulthood (primary lymphedema), or as result of an obstruction or damage to the system by other conditions such as recurrent infections, surgery, trauma, or obstruction of the lymph glands by tumors or radiation (secondary lymphedema).
In the United States, lymphedema most commonly occurs in the arms of 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.
Worldwide lymphedema is most commonly caused by a parasitic infection transmitted by mosquito bites known as filariasis.
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.
- The so-called Stemmer's sign is a reliable diagnostic sign to recognize lymphedema. Try to pinch the skin at the base of your toe. If this is difficult, or even impossible, you have a "positive Stemmer's sign."
Furthermore, deeper natural skin folds over the joints, swelling over the backs of the hand and feet, and taut skin indicate lymphedema.
Lymphedema develops over time and may start as minimal swelling and gradually progress to the later stages. It is described in a number of stages, from mild to severe, referred to as Stage Pre, 1, 2 and 3.
Who is at risk for lymphedema?
Worldwide, many millions of people suffer from lymphedema. Some may be born with inadequate lymphatics, but their symptoms may only appear later in life as their body grows, and the capacity of the system becomes quickly surpassed. Risk factors for primary lymphedema are not yet known and the condition may develop with no family history.
Secondary lymphedema is often the unintended consequence of life-saving cancer treatments. 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.
You may be at risk for parasitic infection and lymphedema if you have ever traveled to any tropical countries, Asia, Africa, Western Pacific or parts of Central and South America. If you develop swelling in any extremity, you should be certain to alert your doctor of those trips and time spent in those countries.
What are the complications of lymphedema?
Patients with lymphedema are increasingly subject to infection due to the inability of the stagnant lymph system to filter waste and toxins. 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.
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). 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.
Always carry antibiotics or a prescription with you and especially when you travel.
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 swollen 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.
It is equally important to remember that 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.
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 - including:
- Doppler ultrasound
- CT scans or MRI scans
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
- Pneumatic compression devices
- Manual Massage techniques
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 should absolutely be the last resort as it may leave your leg disfigured. “You trade one swollen leg for a disfigured leg and the lymphedema may still not be cured.”
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.
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
- Avoid tight or constricting garments or blood pressure cuffs for longer than 3-5 minutes
- Keep the body adequately hydrated.
- Avoid heavy lifting and forceful activity with the affected limb but normal, light activity is encouraged.
- Practice thorough and careful skin hygiene.
- Avoid insect bites and sunburns.
If you think you suffer from chronic swelling of the arm or leg, please call us at South Charlotte General and Vascular Surgery and make an appointment to see one of our vascular experts.