There are 2 types of lymphoedema – primary and secondary lymphoedema – which have different causes.

Primary lymphoedema

Primary lymphoedema is caused by alterations (mutations) in genes responsible for the development of the lymphatic system.

The faulty genes cause the parts of the lymphatic system responsible for draining fluid to not develop properly or not work as they should.

Primary lymphoedema often runs in families, although not every child born to someone with the condition will develop it themselves.

Secondary lymphoedema

Secondary lymphoedema develops in people who previously had a normal lymphatic system that then becomes damaged.

It can have a number of different causes. Some of the most common causes are explained below.

Surgery for cancer

Treatment for cancer can involve surgery to remove sections of the lymphatic system.

The surgeon will try to limit damage to your lymphatic system, although this is not always possible.

There's a particular risk of lymphoedema occurring after treatment for any cancer where lymph glands are removed.

Some of the more common cancers where this happens are:

• breast cancer
• skin cancer (melanoma)
• gynaecological cancers – such as cervical cancer and vulval cancer
• genitourinary cancers – such as prostate cancer or penile cancer

Radiotherapy

Radiotherapy uses controlled doses of high energy (radiation) to destroy cancerous tissue, but it can also damage healthy tissue.

If radiotherapy is needed to destroy cancerous cells in your lymphatic system, there's a risk that the lymphatic system could become permanently damaged and unable to drain fluid properly.

Infections

An infection, such as cellulitis, can sometimes cause lymphoedema. Severe cellulitis can damage the tissue around the lymphatic system, causing it to become scarred.

Filariasis can also cause lymphoedema. Lymphatic filariasis is a parasitic disease caused by microscopic, thread-like worms.

The adult worms only live in the human lymphatic system and block lymph drainage. It's a common cause of lymphoedema worldwide, but is not generally a risk in the UK.

Inflammation

Medical conditions that cause tissue to become red and swollen can also permanently damage the lymphatic system.

Conditions that can cause lymphoedema include:

• rheumatoid arthritis
• eczema

Venous diseases

Diseases that affect the flow of blood through the veins can cause lymphoedema in some people.

The abnormal or damaged veins can cause fluid to overflow from the veins into the tissue spaces.

This overwhelms and eventually exhausts the parts of the lymphatic system responsible for draining this fluid.

Some venous diseases that can lead to lymphoedema include:

• DVT (deep vein thrombosis) – a blood clot in 1 of the deep veins in the body
• swollen and enlarged veins (varicose veins) – where poor drainage of blood in the veins causes higher vein pressure and more fluid overflowing into the surrounding tissues

Obesity

Obesity is another possible cause of secondary lymphoedema.

People who are obese, particularly those who are severely obese, have an increased risk of developing swollen body parts.

It's not clear exactly why this is, but it's been suggested that the extra fatty tissue affects the lymphatic channels in some way, reducing the flow of fluid through them.

Check if you are obese on the BMI calculator.

In these cases, weight loss is an important part of treatment and even just starting to lose weight can make a big difference to the swelling.

Trauma and injury

In a small number of cases, lymphoedema can be caused by an accidental injury to the lymphatic system.

For example, it can sometimes occur after a road traffic accident where there's extensive bruising or soft tissue loss.

Immobility

Movement and exercise help lymph drainage because muscle activity surrounding the lymphatic vessels massages fluid into and along them.

Reduced movement can therefore lead to lymphoedema because the fluid in the lymphatic system does not get moved along.

For example, people who have limited mobility for a long period of time as a result of an illness, nerve damage or arthritis may be at risk of lymphoedema.

If you're being treated for cancer and are at risk of developing lymphoedema, you'll be monitored for the condition afterwards. Otherwise, see a GP if you experience symptoms of swelling.

In many cases, it's possible to diagnose lymphoedema by:

• asking about your symptoms and medical history
• examining the affected limb and measuring the distance around it to see if it's enlarged

The GP may refer you to a specialist lymphoedema treatment centre for further assessment.

Further tests

In most cases, further tests are not necessary, but they may occasionally be used to assess and monitor your condition.

Measuring limb volume

In some cases, tests to calculate the volume of an affected limb may be carried out.

These may include:

• using a tape measure – to measure the circumference of the limb at certain intervals to calculate its volume
• water displacement – where the affected limb is placed in a tank of water and the amount of water that's displaced is measured to calculate the volume of the limb
• perometry – where infrared light is used to measure the outline of an affected limb and calculate its volume

Bioimpedance testing

During a bioimpedance test, small metallic discs called electrodes are placed on different parts of your body.

The electrodes release a small, painless electric charge that's measured using a handheld device. Changes in the strength of the current can indicate the presence of fluid in your tissue.

Imaging tests

Imaging tests may also be used to help diagnose and monitor lymphoedema.

These include:

• a lymphoscintigram – where you're injected with a radioactive dye that can be tracked by a scanner; this shows how the dye moves through your lymphatic system and can check for any blockages
• a MRI scan – a strong magnetic field and radio waves are used to produce detailed images of the inside of your body
• an ultrasound scan – high-frequency sound waves are used to create an image of the inside of your body
• a CT scan – X-rays and a computer create detailed images of the lymph nodes

These scans can be used to create a clearer picture of the affected tissue.

The recommended treatment for lymphoedema is decongestive lymphatic therapy (DLT).

DLT is not a cure for lymphoedema, but it can help control the symptoms. Although it takes time and effort, the treatment can be used to bring lymphoedema under control.

Decongestive lymphatic therapy (DLT)

There are 4 components to DLT:

• compression bandages – to complement exercise by moving fluid out of the affected limb and minimise further build-up
• skin care – to keep the skin in good condition and reduce the chances of infection
• exercises – to use muscles in the affected limb to improve lymph drainage
• specialised massage techniques – known as manual lymphatic drainage (MLD); this stimulates the flow of fluid in the lymphatic system and reduces swelling

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 second phase called the maintenance phase. You'll be encouraged to take over your care using simple self massage techniques, wearing compression garments, and continuing to exercise.

This treatment phase aims to maintain the reduced size of the affected body part.

You'll have reviews every few months to check how your treatment is progressing.

Compression bandages and garments

Credit:

Alistair Heap / Alamy Stock Photo

Unlike blood circulation, the lymphatic system has no central pump, such as the heart, to move fluid to the lymph glands.

Instead, it uses the massaging effect of surrounding muscles to move the fluid. This is why exercise is important.

Compression bandages or garments, such as sleeves, gloves, stockings or tights, fitted over affected limbs act as a counterforce to muscles.

This stimulates more effective lymph drainage. The combination of exercise and compression encourages the fluid to move out of the affected limb.

Compression garments may also be applied after a massage session to prevent fluid accumulating in the limb again.

Velcro wraps may be used instead of bandages or compression garments. These are easier to apply yourself.

You'll be taught how to correctly apply compression garments or Velcro wraps so you can continue using them during the maintenance period.

Skin care

It's important to take good care of your skin to reduce your risk of developing an infection, such as cellulitis.

See preventing lymphoedema for more skin care advice.

Movement and exercises

Your lymphoedema care team will help devise an exercise and movement plan designed to strengthen and stimulate the muscles involved in lymph drainage.

They'll also help you lose weight if you're overweight. Your exercise plan will be tailored to your requirements and ability.

Your plan may involve specific limb exercises, as well as gentle activities that involve the whole body, such as swimming, cycling and walking.

Massage

To begin with, you may receive specialised massages called manual lymphatic drainage (MLD) – usually carried out by a specialist therapist – to move fluid from the swollen areas into working lymph nodes, where it can be drained.

Your lymphoedema therapist will also teach you a range of simpler massage techniques that you or your carer can use during the maintenance phase of treatment to help keep the swelling down. These self massage techniques are known as simple lymphatic drainage (SLD).

Surgery

In a small number of cases, surgery may be used to treat lymphoedema. There are 3 main types of surgery that may be useful for the condition:

• removal of sections of excess skin and underlying tissue (debulking)
• 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.

Liposuction

The accumulation of fat is a significant feature of lymphoedema swelling. Liposuction is where a thin tube is inserted through small cuts (incisions) in the skin to suck fat out of tissue.

It can be used to remove excess fat from an affected limb to help reduce its size.

After surgery, you'll have to wear a compression garment on the affected limb day and night for at least a year to help keep the swelling down.

It's not possible to completely prevent lymphoedema, but the following advice may help reduce your chances of developing it.

If you already have lymphoedema, this advice may stop it getting worse.

Skin care

The part of your body affected by lymphoedema is more vulnerable to infection of the build-up of fluid within the tissues.

Any cuts in your skin can allow bacteria to enter your body and may quickly develop into an infection.

Skin infections can also damage your lymphatic system and cause lymphoedema to develop.

You can reduce your chances of developing skin infections by:

• not having injections or blood pressure readings in the affected area whenever possible
• treating cuts and scratches immediately with an antiseptic cream
• using insect repellents to prevent insect bites
• moisturising the skin daily to keep it supple – a GP can prescribe a suitable cream
• avoiding very hot baths and showers – the heat from saunas, steam rooms and sun beds may increase the swelling
• using sunscreen with a high sun protection factor (SPF) to prevent sunburn
• wearing gloves for gardening and household tasks to avoid cuts if your upper limbs are affected
• using anti-fungal powder to prevent fungal infections in your skin or feet if your lower limbs are affected
• cutting your nails with nail clippers
• seeing a chiropodist for foot and nail care – make sure you tell them you have lymphoedema
• wearing shoes that fit correctly and provide support on the top of your feet if your lower limbs are affected
• using an electric razor if you need to shave to reduce the risk of cutting yourself
• not wearing tight fitting clothes or jewellery

Contact a GP as soon as possible if you develop symptoms of a possible skin infection. These symptoms may include redness or a feeling of heat in the skin.

Healthy lifestyle

Adopting a healthy lifestyle may help reduce your risk of developing lymphoedema, and may also help control the condition if you already have it.

This includes:

• eating a healthy, balanced diet
• maintaining a healthy weight – use the healthy weight calculator to work out whether you're a healthy weight for your height
• exercising regularly

Read more about how to lose weight.