Lymphoedema

Lymphoedema is a chronic condition causing swelling in the body’s tissues that can affect any part of the body, though it most commonly occurs in the arms and legs. While there is no cure, the condition can be managed through various treatments and lifestyle changes.

Table of contents

• What is lymphoedema
• Types of lymphoedema
• Causes and risk factors
• Symptoms and signs
• How lymphoedema is diagnosed
• Treatment options
• Possible complications
• Prevention strategies

What is lymphoedema

Lymphoedema is a long-term condition where swelling occurs in the body’s tissues due to an accumulation of protein-rich fluid (lymph fluid) that is normally drained through the body’s lymphatic system^[1]. The lymphatic system is a network of channels and glands throughout the body that helps fight infection and remove excess fluid from tissues^[3].

When the lymphatic system doesn’t work properly, lymph fluid accumulates in the soft tissues and causes swelling. The chronic, progressive accumulation of this fluid exceeds the capacity of the lymphatic system to transport it^[5]. Lymphoedema most commonly affects the arms or legs, but it can also occur in the chest wall, abdomen, neck, genitals, face, and oral cavity^[1][4].

It’s estimated that lymphoedema affects more than 200,000 people in the UK^[3] and roughly 1 in 1,000 Americans, with the condition potentially affecting as many as 140 million to 250 million people worldwide^[17].

Types of lymphoedema

There are two main types of lymphoedema: primary and secondary^[2].

Primary lymphoedema is rare and comes from inherited or congenital conditions that cause a malformation of the lymphatic system, most often due to genetic mutation^[5]. Primary lymphoedema affects about 1 in 100,000 people in the United States^[2]. It can develop at different ages and is subdivided into three categories^[5]:

• Congenital lymphoedema: Present at birth or recognized within two years of birth. Infants may be born with Milroy’s disease, an inherited form of lymphoedema^[2].
• Lymphoedema praecox (also called Meige disease): Occurs at puberty, during pregnancy, or up until age 35^[2][5].
• Lymphoedema tarda: A rare, late-onset form that begins after age 35, with swelling usually limited to the legs^[2][5].

Secondary lymphoedema is much more common than primary lymphoedema^[3]. It occurs when the lymphatic system is damaged from surgery, trauma, or radiation therapy, or due to problems with the movement and drainage of fluid in the lymphatic system^[2]. Secondary lymphoedema is often associated with cancer and cancer treatment^[17].

Causes and risk factors

Lymphoedema is caused by a problem with the lymphatic system. Any type of problem that blocks the drainage of lymph fluid can cause lymphoedema^[1].

Primary lymphoedema can be due to a genetic condition where people are born with an abnormality of their lymphatic system^[4].

Secondary lymphoedema can be caused by^[4][7]:

• Cancer or tumors: A tumor can block the flow of lymph fluid or press against lymph nodes
• Surgery: Lymph nodes are often removed as part of cancer surgery, particularly breast cancer surgery. If you have more than four lymph nodes removed, the risk of developing lymphoedema rises to 15% to 25%^[6]
• Radiation therapy: Radiation can traumatize the lymphatic system and cause scar tissue that blocks the flow of lymph fluid^[6][7]
• Trauma or tissue damage
• Infection, including parasitic infections such as filariasis^[4]
• Obesity

Lymphoedema is most common in people who have had breast cancer treatment. Treatment for breast cancer often involves surgery that removes one or more lymph nodes in the underarm area, increasing the risk of lymphoedema^[7]. That said, not everyone who has breast cancer treatment develops lymphoedema^[2].

Patients who have axillary lymph node dissection have a lifetime risk of 15-25% of developing lymphoedema. For individuals whose surgery is limited to sentinel node techniques without radiation, the risk is about 6%^[6].

Secondary lymphoedema also often affects people with cancers that involve the lymph system or who have had treatment to the lymph nodes, such as vulval cancer, penile cancer, head and neck cancer, bowel cancer, prostate cancer, and cancer of the reproductive system^[3][4].

Other risk factors include having an infection, healing slowly after surgery, having lymph nodes removed, past surgeries or radiation, having advanced cancer, and being overweight or having obesity^[7].

Symptoms and signs

The main symptom of lymphoedema is swelling in all or part of a limb or another part of the body^[3]. Swelling may develop slowly over months or years, and you may not notice unusual swelling in lymphoedema’s early stages. Swelling from lymphoedema may also come on suddenly^[2].

At first, the swelling may be soft and easy to push in, leaving a dent, and may come and go. It may get worse during the day and go down overnight. Without treatment, it will usually become more severe and persistent^[3].

Signs and symptoms of lymphoedema include^[1][2][4]:

• Swelling of part or all of the arm or leg, including fingers or toes
• A feeling of heaviness or tightness in the affected area
• Difficulty with movement or restricted range of motion in the joints
• Aching and discomfort in the affected area
• You cannot see or feel the veins or tendons in your hands and feet
• Your arms or legs appear to be slightly different sizes
• It feels as if your joints are unusually tight or inflexible
• Your skin seems puffy or red
• Repeated skin infections
• Developing hard, tight, or thickened skin
• Folds developing in the skin
• Wart-like growths developing on the skin
• Fluid leaking through the skin
• It can be difficult to fit into clothes, and jewelry and watches can feel tight

Signs of lymphoedema in the head and neck may include swelling or heaviness in the face, mouth, or throat, and problems with swallowing and speech^[3].

Many people find that the swelling gets worse through the day and then gets better overnight, in the heat, with overuse of the limb, or with prolonged inactivity^[4].

How lymphoedema is diagnosed

If you experience the typical symptoms of lymphoedema, such as swelling in your arms and legs, you should see a doctor^[3]. It is very important to see a doctor right away to find out why you have swelling and make sure there is no other cause that needs immediate treatment, like a blood clot^[19].

In many cases, lymphoedema can be diagnosed from your symptoms and medical history, and by examining the affected body part. If you are at risk of lymphoedema—for instance, if you have recently had cancer surgery involving your lymph nodes—your doctor may diagnose lymphoedema based on your signs and symptoms^[9].

Your doctor may measure the swollen limb to compare it to your other limb. Usually, if your swollen arm or leg is 2 cm (about 4/5 of an inch) bigger than your other arm or leg, you have lymphoedema^[19].

If the cause of your lymphoedema is not obvious, your doctor may order imaging tests to get a look at your lymph system. Tests may include^[9]:

• MRI scan: Using a magnetic field and radio waves, an MRI produces 3D, high-resolution images of the involved tissue
• CT scan: This X-ray technique produces detailed, cross-sectional images of the body’s structures and can reveal blockages in the lymphatic system
• Ultrasound: This test uses sound waves to produce images of internal structures and can help find obstructions within the lymphatic system and vascular system
• Lymphoscintigraphy: During this test, the person is injected with a radioactive dye and then scanned by a machine. The resulting images show the dye moving through the lymph vessels, highlighting blockages

If you are being treated for cancer and are at risk of developing lymphoedema, you will be monitored for it afterwards^[3].

Treatment options

There is no cure for lymphoedema^[3][9]. However, it is usually possible to control the main symptoms using techniques to minimize fluid build-up and stimulate the flow of fluid through the lymphatic system. Treatment focuses on reducing the swelling and preventing complications^[9].

Healthcare providers can ease lymphoedema symptoms, and there are many things you can do to limit the impact lymphoedema may have on your quality of life^[2]. Once lymphoedema develops, it is a chronic condition that cannot be cured but can be treated to relieve swelling and improve your ability to function day to day. Lymphoedema is easier to control when treatment starts early^[7].

The recommended treatment for lymphoedema is decongestive lymphatic therapy (DLT), also known as complete decongestive therapy (CDT)^[10][13]. DLT is not a cure, but it can help control the symptoms^[10].

There are four main components to DLT^[10]:

• Compression therapy: Compression bandages or garments fitted over affected limbs act as a counterforce to muscles, which stimulates more effective lymph drainage. The combination of exercise and compression encourages the fluid to move out of the affected limb^[10]
• Skin care: Keeping the skin in good condition and reducing the chances of infection. Appropriate skin care is stressed to prevent recurrent cellulitis or lymphangitis. Meticulous hygiene is necessary to remove debris and bacteria^[14]
• Exercises: Using muscles in the affected limb to improve lymph drainage. Gentle contraction of the muscles in the arm or leg can help move the excess fluid out of the swollen limb^[9][10]
• Specialized massage techniques: Known as manual lymphatic drainage (MLD), this stimulates the flow of fluid in the lymphatic system and reduces swelling^[10]

DLT is an intensive phase of therapy, during which you may receive daily treatment for several weeks to help reduce the volume of the affected body part. This is followed by a maintenance phase where you are encouraged to take over your care using simple self-massage techniques, wearing compression garments, and continuing to exercise and look after your skin^[10].

Manual lymphatic drainage must only be performed by a specialist trained to do this. You might be shown simple lymphatic drainage (SLD), which is a simpler version of MLD you can do yourself. A specialist needs to teach you how to do this^[13].

Patients are also encouraged to lose weight if overweight, avoid even minor trauma, avoid constrictive clothing that might have a tourniquet effect, and elevate the affected extremity whenever possible, particularly at night^[14].

Medications

Lymphoedema greatly increases the risk of skin infections called cellulitis. Your doctor may prescribe antibiotics for you to keep on hand so that you can start taking them immediately once symptoms appear^[9].

Surgery

In a small number of cases, surgery may be used to treat lymphoedema. Surgical treatment is palliative, not curative, and it does not eliminate the need for continued medical therapy. It is rarely indicated as the primary treatment and is reserved for patients who do not improve with conservative measures or for cases in which the extremity is so large that it impairs daily activities^[14].

There are two main types of surgery^[10]:

• Removal of fat from the affected limb (liposuction)
• Restoration of the flow of fluid around the affected section of the lymphatic system—for example, by connecting the lymphatic system to nearby blood vessels (lymphaticovenular anastomosis)

These treatments may help reduce the size of areas of the body affected by lymphoedema in some people. They are only available in some specialist centers^[10].

Possible complications

Lymphoedema can be a serious medical condition. If you have lymphoedema, you may be at risk of developing infections that start in your skin. These infections may be life-threatening. Rarely, lymphoedema may also lead to lymphangiosarcoma, a very rare skin cancer^[2].

The most common complication of lymphoedema is cellulitis, a bacterial infection of the deep layer of skin^[3]. If you have lymphoedema, the build-up of fluid in your tissues makes you more vulnerable to infection^[3].

Symptoms of cellulitis can include^[3]:

• Redness and a feeling of heat in the skin (this may be harder to see on brown and black skin)
• Pain and increased swelling in the affected area
• A high temperature
• Chills

Antibiotics taken by mouth can usually be used to treat cellulitis, although severe cases may need treatment in hospital^[3].

Severe cases of lymphoedema can affect the ability to move the affected limb, increase the risks of skin infections and sepsis (a life-threatening response to infection), and can lead to skin changes and breakdown^[1].

Lymphoedema can also have a significant psychological impact. It can be devastating, causing psychological morbidity. Many people with lymphoedema feel self-conscious about the ways it changes their appearance^[2][11].

Prevention strategies

Although lymphoedema cannot be prevented entirely, there are ways to lower your risk or keep lymphoedema from getting worse^[7].

Before you have surgery or radiation treatment for cancer, ask your doctor what can be done to lower the chances that you will get lymphoedema^[19].

If you notice any swelling after cancer treatment, talk to your doctor about what is causing it and how best to treat it. Contact your doctor as soon as you notice heaviness, swelling, or other signs of lymphoedema^[7][19].

Important prevention and management strategies include^[3][18]:

• Taking good care of your skin to reduce your risk of developing an infection
• Moving and exercising regularly
• Having a healthy diet and lifestyle
• Losing weight if you are overweight
• Using specialized massage techniques
• Wearing compression garments as recommended
• Elevating the affected body part whenever possible