Lymphedema: Causes, Diagnosis, and Treatment

Are you experiencing localized swelling, but don’t know why?

You’re not alone. In fact, lymphedema is a common condition that can be caused by a variety of things. It commonly affects arms and legs but can also occur in the chest wall, abdomen, neck, and genitals.

There are many treatments available for managing lymphedema. 

In this post, we’ll take a look at what causes lymphedema, as well as some common treatments.

We’ll also explore some lifestyle changes that can help reduce the risk of developing this condition.

What is Lymphedema?

Lymphedema is a condition that causes localized swelling.

The lymphatic system is a part of the immune system and vital for immune function and removing waste from our bodies.

It is a network of specialized vessels (lymph vessels) throughout the body whose purpose is to collect excess lymph fluid with proteins, lipids, and waste products from the tissues.

This fluid is then carried to the lymph nodes, which filter waste products and contain infection-fighting cells called lymphocytes. 

Lymphedema is typically caused by a blockage of this system or when lymph vessels are blocked, damaged, or unable to carry lymph fluid away from the tissues.

Types of Lymphedema

There are two types of lymphedema: primary and secondary.

Primary

Primary lymphedema occurs because of a genetic condition.

Aplasia, a birth anomaly where organs or limbs do not develop, can affect the lymphatic system.

In this case, parts of the system, such as lymph nodes, never developed in the body.

Hypoplasia, where parts of the lymphatic system are underdeveloped, and hyperplasia, where lymph collectors are larger than normal, can also cause lymphedema. 

Aplasia, hypoplasia, and hyperplasia can affect many different parts of the body and can be related to a number of other conditions.

The three main types of primary lymphedema are:

• Congenital lymphedema: Congenital lymphedema, also known as Milroy’s disease, is present at birth due to genetic factors. Congenital lymphedema is also associated with other genetic conditions, including Noonan syndrome and Turner syndrome. 
• Lymphedema praecox: Lymphedema praecox is another congenital condition, but the symptoms typically do not become apparent until puberty or later. This condition is also referred to as Meige disease, and it is the most common type of primary lymphedema. Swelling typically begins at the feet and progresses upward. 
• Lymphedema tarda: Lymphedema tarda, or late-onset lymphedema, is the third type of primary lymphedema. As the name suggests, this condition develops later in life. People with lymphedema tarda will not experience symptoms until after the age of 35. 

Secondary 

Secondary lymphedema is caused by another disease or outside factor like injury or obstruction to the lymphatic system.

Most secondary lymphedema cases are due to malignancy or related to the treatment of malignancy.

This includes surgical excision of lymph nodes, local radiation treatment, or medical therapy.

Breast cancer is the most common cancer associated with secondary lymphedema.

Causes

Cancer

Cancer can cause lymphedema by interfering with the lymphatic system.

Sometimes a cancerous tumor can get big enough to block the lymphatic system.

Lymphedema often occurs in breast cancer patients who had all or part of their breast removed and axillary (underarm) lymph nodes removed.

Lymphedema in the legs may occur after surgery for uterine cancer, prostate cancer, lymphoma, or melanoma. It may also occur with vulvar cancer or ovarian cancer.

Cancer Treatment

Surgery to remove cancer may also remove lymph nodes or some of the vessels that carry the lymph fluid.

This can cause the fluid to build up in surrounding tissues.

Radiation therapy, which is often used to treat cancer, can also damage lymph nodes and vessels.

Chemotherapy may also play a role in developing lymphedema by harming the lymph system.

Surgery Complication 

Complications from other surgeries can lead to lymphedema.

Generally speaking, people who undergo surgery that includes the removal of multiple lymph nodes are more likely to develop lymphedema.

However, even the removal of a single lymph node (eg, “sentinel lymph node” biopsy) comes with a risk.

Infection

Some infectious diseases can cause lymphedema. 

The most common cause is lymphatic filariasis, a parasitic infection which is sometimes called elephantiasis.

This disease occurs mostly in developing countries in tropical regions, and causes destruction of the lymph nodes that leads to severe swelling. 

Within the United States, most infections do not cause lymphedema, but in very rare cases, a severe bacterial skin infection or lymph node infection could lead to lymphedema.

And having lymphedema can increase your risk for skin infections, which in turn, could then make lymphedema worse. 

Heart Problems 

The lymphatic system is closely connected to the cardiovascular system.

Problems with heart structure or function, including congestive heart failure, which occurs when the heart cannot pump enough blood to meet the body’s needs, can affect this connection and lead to lymphedema.

Kidney Disease

Kidneys work to remove extra fluid and waste products from the body.

If there is impaired kidney function this can cause decreased ability of the body to remove the fluid.

This can back up to the entire system, causing swelling that may lead to lymphedema.

Trauma

Lymphedema occurs as a result of damage to the lymphatic system.

While it is rare, extensive burns or broken bones near an area of lymph nodes or vessels can injure them enough to lead to lymphedema.

Symptoms

The symptoms of lymphedema may include:

• Progressively increasing swelling in your arm, leg, or other part of your body
• Skin that feels tight and sometimes has a tingling sensation
• The arm or leg with lymphedema feels heavy
• Clothing and jewelry fit more tightly on the affected area
• The skin looks thicker or leathery

If you notice any swelling after cancer treatment, surgery, or an injury, that does not improve after a few weeks, talk to your healthcare provider about what is causing it and how best to treat it.

Risk Factors

There are a number of factors that can increase your risk of developing lymphedema, which includes:

• Family history: If someone in your family has had lymphedema, you may be more likely to develop the condition.
• Previous surgery: If you’ve had surgery to remove lymph nodes, you’re at an increased risk of developing lymphedema.
• Radiation therapy: Radiation therapy can damage the lymphatic system and increase your risk of developing lymphedema.
• Cancer treatment: Chemotherapy and other cancer treatments can also damage the lymphatic system and lead to lymphedema.
• Infection: While rare in the US, infections that damage the lymphatic system can lead to lymphedema. The most common infectious cause, lymphatic filariasis, is most common in tropical areas and developing countries, and rarely affects travelers or those living within the US.

Repeated episodes or untreated lymphedema can lead to other complications. These include:

• Skin infections (cellulitis): The trapped fluid and decreased blood flow to areas with lymphedema makes the affected areas prone to the growth of bacteria, and the smallest injury to the arm or leg can be an entry point for infection. Affected skin appears swollen and red and is typically painful and warm to the touch. See a healthcare provider right away if you are concerned you are developing an infection, as antibiotics may be needed.
• Sepsis: Untreated cellulitis (skin infection) can spread into the bloodstream and cause sepsis, a potentially life-threatening condition that occurs when the body’s response to an infection damages its own tissues. Sepsis requires emergency medical treatment. This is why it’s important to address any skin infections right away.
• Leakage through the skin: With severe swelling, lymph fluid can drain through small breaks in the skin causing oozing or blistering
• Skin changes: In some people with severe lymphedema, the skin of the affected limb can thicken and harden so it resembles the skin of an elephant.
• Psychological effects: Lymphedema can affect appearance, and this can have a psychological impact, especially in those who have been living with cancer. Lymphedema increases the risk of developing depression.

Diagnosis

A doctor or medical professional will try to rule out other possible causes of swelling, including a blood clot or an infection that does not involve the lymph nodes.

If the patient is at risk of lymphedema, for instance, if they recently had cancer surgery or treatment involving the lymph nodes, your healthcare provider may diagnose lymphedema based on the symptoms.

In general, no specific test is used to diagnose lymphedema, but other causes of swelling should be ruled out.

If there isn’t an obvious cause for the lymphedema, some imaging tests may be ordered to rule out other causes of swelling.

The following imaging techniques may be used:

• MRI scan
• CT scan
• Doppler ultrasound scan

Lymphoscintigraphy may also be used, which is when a radioactive dye is injected into the lymphatic system.

The nuclear scanner shows the dye’s movement through the lymphatic system and identifies any blockages.

Treatment

People with lymphedema are encouraged to follow a healthful lifestyle, including moving and exercising regularly.

However, in some cases, specialist help may be needed to exercise safely and effectively.

Home Remedies

People who are at risk of lymphedema following breast cancer surgery will not be at higher risk of lymphedema in the arm if they do gentle lifting exercises.

While the exercise was not shown to improve or cure lymphedema, those that participated in exercise gained strength and had lower rates of complications like infections.

The types of exercises that may be beneficial are those that:

• Enhance flexibility
• Increase range of motion
• Build strength

Also recommended is an aerobic exercise that focuses on the upper body, helps with healthy weight maintenance, and encourages deep breathing.

Medical Care

While there is no cure for lymphedema, treatments are designed to reduce the swelling and control discomfort and other symptoms.

Compression treatments can help reduce swelling and prevent infection 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.
• 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, 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.

Prevention

There are steps that can be taken to reduce the risk of lymphedema. They include:

• Understanding how lymphedema develops
• Following treatment plans after cancer surgery or other treatments that may affect the lymphatic system, such as radiation therapy or chemotherapy
• Managing any chronic medical conditions carefully and following a healthy lifestyle
• Avoiding injuries to the affected arm or leg, especially puncture wounds
• Wearing proper footwear with support for hiking and walking long distances, and not wearing high heels for extended periods of time
• Avoiding prolonged sun exposure and using sunscreen when outdoors
• Seeking early medical attention when symptoms of lymphedema arise
• 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

When to See a Medical Provider

Lymphedema should be treated as soon as possible to avoid serious complications.

Call a healthcare provider if:

• There is swelling that does not go away or continues to worsen
• The affected area feels warm to the touch or looks red and feels tender
• You develop a fever or chills
• There is an open wound on the affected limb that is not healing properly
• There is increased pain in the affected limb or difficulty moving it

If any of these symptoms occur, seek medical attention right away.

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