Lymphoedema

Lymphoedema is a swelling which is the result of a damaged or weakened lymphatic system. This may be due to the lymphatic system not developing properly or from injury or damage following trauma or surgery (where the lymphatic system has been affected).

Lymphoedema most commonly affects the arms and legs but it can affect other parts of the body.

Recent research estimates that approximately 100,000 people in the UK may be suffering from this condition.

What is the lymphatic system and what does it do?

Blood flows through the body to nourish the tissues and any excess fluid is removed by the lymphatic system to be returned to the blood circulation.

There are many lymphatic channels and some, especially near the skin, are very small and delicate. As the channels go deeper into the body, the lymphatics become larger and drain through groups of lymph nodes (sometimes called glands).

The lymphatic system has an important role in fighting infection.

Lymphoedema develops for many reasons, some of which are as yet not fully understood.

Primary lymphoedema can develop at any age and is due to an 'inbuilt' problem with the lymphatic system, that may occur at birth or develop later in life. The most common problems are absent or obstructed lymphatics or scarring of the lymph nodes.

Secondary lymphoedema can occur when part of the lymphatic system gets damaged and may result from cancer treatment, injury or infection. Sometimes people with vein problems and ulcers get lymphoedema. Other conditions such as heart and kidney problems or limb paralysis can also lead to swelling.

Symptoms

These are some possible signs of lymphoedema but they can be due to other conditions so you need to get advice from an appropriately trained practitioner if you think you may have lymphoedema.

• swelling of one or more limb/s that does not go down over night and continues over 3 months
• swelling of other parts of the body such as the back, face, breast, hand/fingers, foot/toes, armpit, shoulder or genitals
• tightness of clothing or shoes, with indentations and creases appearing on the skin
• skin becomes hard and sometimes warm or hot to the touch

Treatment

Your lymphoedema practitioner, nurse or physiotherapist will talk to you individually about what treatments are suitable for you but these are usually based on four main areas:

• skin care and preventative care
• compression therapy (multi-layer bandaging and lymphoedema garments)
• manual lymph drainage (MLD) or patient self massage
• exercises

In the long term you will need to decide how you can adapt your lifestyle and get into a routine with doing the various self-treatments including daily skin care, wearing of lymphoedema garments, regular exercising and use of self-massage where necessary. It will be important to try to keep your body weight within normal limits. Making these self-treatments a natural part of your daily life is the key to successful control of your lymphoedema.

Some people require an Intensive Treatment programme combining bandaging and manual lymphatic drainage massage to reduce the swelling. This Intensive Treatment course is usually given over 2 or more weeks.

What can I do to help prevent problems?

• Every day wash and carefully dry your limb and use a good quality moisturiser to keep the skin supple and healthy
• Try to avoid scratching, burning or injuring your skin on the affected limb
• Treat any scratches, burns, insect bites or cuts with antiseptic to avoid infection and use insect repellent where required
• Take care when cutting or filing your nails to avoid damaging the skin and consult a chiropodist or podiatrist if you have problems with your toenails
• If you have arm swelling, wear gloves when gardening and use oven mitts when touching hot dishes
• If you have leg swelling, wear comfortable socks and shoes and try to avoid going barefoot
• Ensure that your underwear or clothing is not too tight to cause restriction
• Do not let anyone use the limb to give you an injection, take blood or take your blood pressure
• Try to move the limb as normally as possible, exercise as advised but avoid repetitive activities that make the swelling worse
• Be aware that hot weather, flights and sunburn can worsen the swelling
• Try to keep healthy, eat well and keep body weight within normal limits
• Consult a lymphoedema practitioner where possible

What is cellulitis and how can I prevent it?

Some people with lymphoedema get an infection called cellulitis. This can reoccur in some people making the lymphoedema worse. It is vital that cellulitis is properly treated and you should see your doctors immediately if you get any of these signs of infection:

• Red, hot, painful swollen limb
• Blotching, rash or red streaking (lymphangitis) present on the limb
• Other symptoms such as a temperature, flu-like symptoms and nausea

If you get cellulitis you should:

• Stop all lymphoedema treatments such as manual lymph drainage
• Remove compression garments or bandages and rest the limb
• Consult your doctor as soon as possible for antibiotics and take these regularly (usually for two weeks)
• Consult your lymphoedema practitioner, nurse or physiotherapist for other advice

Once the early symptoms are controlled, it may be possible to recommence lymphoedema treatment. Limb swelling will probably have increased due to the infection and compression therapy will be important in managing any changes.

Causes

Lymphoedema develops for many reasons, some of which are as yet not fully understood. By definition, 'lymphoedema' is due to insufficiency in the lymphatic system. However, several factors can contribute to swelling in an individual and therefore the term 'chronic oedema' reflects the broader aetiology of this condition.

Primary lymphoedema is due to an intrinsic abnormality in the lymphatic system including an absence of lymphatics (aplasia), obliteration of lymphatics or lymph node fibrosis. It can develop at any age and genetic studies have shown that it may be congenital and inherited (Milroy's disease). Meige's disease is used to describe inherited lymphoedema that develops between the ages of 2-35.

Secondary lymphoedema may occur following surgery, radiotherapy, injury or infection that damages or obstructs lymph drainage. Lymphoedema of the arm develops in 1 of 4 women following breast cancer treatment and can be a particular problem in recurrent and advanced cancer. Filarial infection is the most common lymphoedema worldwide but is predominately seen in underdeveloped countries. However, cellulitis or lymphangitis, particularly if they are recurrent, will damage lymphatics and can predispose to lymphoedema.

In some individuals, the amount of fluid accumulating in the tissue is increased due to alterations in capillary filtration. This can be due to venous hypertension, cardiac failure, chronic ulceration and infection, reduced mobility and limb dependency. Hypoproteinaemia, often associated with liver problems, raises the net capillary filtration rate, producing a generalised oedema. In all these situations, if the lymphatic system is unable to compensate for the excess fluid, swelling will ensue.

Diagnosis

If someone has swelling of > 3 months duration that does not significantly reduce on elevation, several others signs may be present to indicate that this is lymphoedema. Some examples of there are:

• Distortion of limb shape with skin folds evident
• Thickening of the skin and fibrosis of the underlying tissues leading to a positive Stemmer's sign (inability to pinch the skin at the base of the second toe)
• Lymphangiectasia - dilated skin lymphatics bulging from the skin similar to blisters
• Papillomatosis - an uneven appearance to the skin due to lymphangiectasia complicated by fibrosis
• Recurrent inflammation, cellulitis or lymphangitis in the swollen limb

In the early stages of lymphoedema, the swelling may be mild and can reduce overnight when in bed. This 'pitting' oedema is indented by finger pressure but the chronic accumulation of fluid, proteins, fats and various waste products can eventually result in fibrotic, 'non-pitting' tissues.

Other skin and tissue problems in lymphoedema include hyperkeratosis (thickened scaly areas), dermatitis, folliculitis and fungal infections, particularly in skin folds and between fingers and toes.

Lymphoscintigraphy, the injection of a radio-labelled protein into the interstitial tissues, provides images of lymphatics and lymph nodes and quantifies lymph flow. It is mainly used in primary lymphoedema or in children but may not provide any additional information to change the treatment plan. Investigations such as CAT scans, ultrasound and Magnetic Resonance Imaging may help to distinguish lymphoedema from other causes of swelling such as lipoedema and may be useful in patients with cancer. None of these investigations are routinely used although a full blood count, urea and electrolytes and ECG may be indicated in patients with a non-specific limb swelling of unknown origin.