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Lymphatic dysfunction means the lymphatic system is working poorly. The lymphatic system is made up of lymph nodes and lymph vessels that drain fluids from your body’s tissues.
The fluids carry toxins, immune cells, and waste products to your lymph nodes. Lymph vessels help maintain fluid balance in the body by returning filtered lymph fluid back to the bloodstream.
A poorly working lymphatic system causes tissues to become swollen with fluid. This is called lymphedema. It commonly results in swelling in your arms or legs. Other parts of the body can be affected, too.
You can be born with a lymphatic problem that causes lymphedema. This is called hereditary or primary lymphedema. It can also result from a variety of complex genetic conditions.
You can also develop lymphedema from a disease complication or injury. This is called secondary lymphedema. It’s a common side effect of cancer treatment.
Lymphatic dysfunction is a chronic condition for most people, but treatments are available to limit its progression.
There are several different causes of hereditary (primary) and secondary lymphatic dysfunction.
Hereditary (primary) lymphedema
Hereditary lymphedema is also known as primary lymphedema. It’s less common than secondary lymphedema. You’re more likely to have hereditary lymphedema if a family member also has it.
Meige disease is the most common form of hereditary lymphedema, accounting for 80 percent of all cases. It’s caused by a genetic mutation and affects the legs, arms, face, and larynx. It also causes yellow nails in some people.
Another type of hereditary lymphedema is known as Milroy disease. It can cause the structures that make up your lymphatic system to form incorrectly.
Treatment for breast cancer with a mastectomy is one of the most common causes of secondary lymphedema.
Surgeons often remove lymph tissue from under the arm when they’re removing cancerous breast tissue. Fluids draining from the arm must pass through the armpit. If lymph nodes are removed from this area, lymphatic dysfunction and swelling in the arm can occur.
Lymphedema can also occur after treating cancers of the head and neck. It can lead to swelling of the face, eyes, neck, and lips.
The main sign of lymphatic dysfunction is lymphedema. Lymphedema causes swelling in your arms or legs. Your fingers or toes may retain fluid and swell. The tissues of the head and neck may be affected, too.
The swelling can limit your range of motion. You may experience heaviness or a dull ache in the affected area. Lymphedema can also lead to:
- skin changes
- skin discoloration
- leaking of fluid from the skin
In the head and neck, lymphedema can affect vision and cause ear pain and nasal congestion. It can also cause problems with:
People with congenital lymphatic dysfunction may show symptoms in early childhood. It can also occur with the onset of puberty or in adulthood, even beyond age 35.
Symptoms of secondary lymphedema can appear any time after surgery. Most symptoms will appear within several months to a few years after surgery. However, it can also be significantly delayed.
Cellulitis is a type of bacterial skin infection. This is related to the skin changes that often go along with lymphedema, allowing bacteria to access deeper tissues.
Lymphangitis can be due to a bacterial, viral, or fungal infection of the lymph vessels.
Symptoms of infection include a streaky or blotchy red patch on the affected area. Other common symptoms include:
Your doctor will perform a physical exam and ask you about your medical history.
They may order imaging tests if they suspect lymphatic dysfunction. One possible test is a lymphangiogram, which is a type of X-ray that uses contrast dye to help show the path of your lymph nodes and lymph vessels more clearly.
Your doctor may inject the dye into an area between your toes or in the groin area. The X-ray images can reveal abnormalities in your lymphatic drainage system. An MRI may be used in place of traditional X-ray.
How is lymphedema classified?
Lymphedema is commonly classified into stages based on its severity:
- Stage 0 (latent). No visible changes are seen, but you may notice changes in sensation, often with achiness or tightness.
- Stage 1 (mild). Swelling in the affected area can change throughout the day. Tissue will hold an indentation when you press on it (pitting edema). There are no permanent changes in the skin.
- Stage 2 (moderate). There’s more permanent swelling where your tissue feels spongy to the touch. Inflammation and thickening of the skin occurs.
- Stage 3 (severe). There’s ongoing fluid retention. The affected area hardens and becomes very large. Skin changes are permanent, and there’s often loss of function.
The goals of treatment are to reduce swelling early and as much as possible and to retain range of motion and function of the affected area.
Compression is an important treatment for lymphedema. Compressing the affected limb encourages the lymphatic fluids to move toward your torso and out of the arm or leg in a more normal pattern of circulation.
Wrapping your arm or leg firmly with an elastic bandage or wearing a compression garment keeps continuous pressure on the swollen area. This brings down the size of the limb, decreases stress on the skin, and improves mobility.
Compression garments are specially designed socks, stockings, or sleeves that have a snug fit over the swollen limb. Your doctor may suggest buying compression garments of a certain grade or level of compression.
The grade or level will depend on the severity of swelling. Standard compression garments are available at most pharmacies and drugstores.
Levels of compression are measured in pressures of mm Hg (millimeters of mercury). The more compression you need, the higher the pressure should be.
Although there’s no industry standard, these are some
- Low (class 1): less than 20 mm Hg
- Medium (class 2): between 20 to 30 mm Hg
- High (class 3): greater than 30 mm Hg
Gradient compression is most often recommended. It can be built into a slip-on stocking or sleeve or achieved through a self-applied wrap. Gradient compression is designed so that it’s tightest at the furthest point of the arm or leg and becomes gradually looser further up the limb.
If built-in gradient compression garments aren’t available, a lymphedema specialist can show you how to use layers of wrapping to accomplish the same effect.
Tighter, more narrow wrapping with lots of overlap starts at the further point. Looser, wider, and less overlap are done as the wrapping moves further up the limb.
Another form of compression therapy is called pneumatic compression. Pneumatic compression involves vests or sleeves that are timed to inflate and deflate to stimulate the proper flow of lymphatic fluid.
Exercise can help manage lymphatic dysfunction. Your muscles contract during exercise, and these contractions put pressure on your lymph vessels. This helps fluid move through the vessels and reduces swelling.
Experts in lymphedema recommend a variety of exercises to help manage the condition:
- Simple range of motion exercises, such as knee bends or wrist rotations, are designed to maintain flexibility and mobility.
- Light repetitive exercises can also stop fluid from pooling in your arms or legs. Try walking, doing yoga, or low-impact aerobics like swimming.
Aim for 20 to 30 minutes of exercise most days of the week. Speak with your doctor before starting an exercise routine.
Medical and surgical procedures
It’s common to recommend comprehensive decongestive therapy (CDT) for the treatment of lymphedema. This includes several components, some already mentioned above:
- compression garments
- routine skin care
- limb exercises
- lymphatic drainage massage
Lymphatic drainage massage, also known as manual lymphatic drainage, is a type of massage therapy performed by a qualified lymphedema professional. Manipulating tissues allows the lymph fluids to drain more freely.
How often manual lymphatic drainage is required depends on the severity, location of swelling, and how much movement of the area is tolerated. It often starts out 5 days a week for 3 to 8 weeks, then is performed as frequently as necessary to maintain improvement.
You can also receive training from a professional to do it at home.
Don’t undergo manual drainage if you have cellulitis or other types of skin infections or skin breakdown.
The Food and Drug Administration (FDA) has approved low-level laser therapy for treating lymphedema related to breast cancer.
It’s recommended that a physical therapist or other health professional specializing in lymphedema apply this treatment.
Liposuction can be effective in cases of more advanced stages of lymphedema when other treatment options haven’t helped. A
However, following the procedure, it’s still necessary to use compression garments to keep swelling down.
Lymphedema that happens along with a bacterial infection will be treated first with antibiotics. Antibiotics can help control pain and swelling. They also help prevent the infection from spreading.
Your outlook depends on the stage, location, and cause of lymphedema, as well as your overall health.
Lymphedema is an ongoing condition that requires ongoing care. In most cases, you’ll deal with some level of swelling on a regular basis, but its progression can be reduced.
It’s important to work with a healthcare team of lymphedema specialists that include physical therapists and surgical and medical specialists. The best way to deal with lymphedema is to use a combination of treatments and management techniques.