Lymphoedema can be inherited or acquired. The acquired form can be due to cancer directly invading the lymphatics, or more commonly associated with the treatment of cancer, for example, post-surgery from axillary node clearance or post-radiotherapy.
Lymphoedema, unlike other forms of oedema, is usually associated with a high protein content in the tissues, which is usually greater than 5g/L but can be 20-30g/L or more.1 High protein content in the tissues can stimulate fibrosis. This type of oedema tends not to respond to traditional use of diuretics, but more to physical approaches and good skin care.
It is important to differentiate lymphoedema from other causes of oedema, for example, heart failure, renal failure and venous obstruction.
Instinctively when discussing lymphoedema, one thinks of a swollen limb. This is not always the case and a limb can be lymphoedematous but not swollen. Typically there are characteristic tissue and skin changes, the classic sign being non-pitting oedema.
Assessing the extent of the lymphoedema can be done by a number of means. Limb circumference can be measured at specific points and compared with the opposite limb. Alternatively, if a number of points are taken at which the circumference is measured, it is then possible to work out the volume by applying a geometric equation. However, this assumes the lymphoedematous limb behaves as a cylinder and most limbs are not that uniform.
Skin changes can include increased skin turgor, papillomatosis and hyperkeratosis.
2. Clinical features
Clinically, the patient can present with a swollen limb with skin changes.
The limb can be painful, especially if very heavy. It can be prone to infection and this is more common with lymph-oedema. Sometimes the limb feels so tight, patients describe the sensation as 'bursting'.
A heavy limb can make mobility difficult and an underestimated element of lymph-oedema is the psychological impact of a distorted body image.
Hypoalbuminaemia can contribute to oedema. In addition, signs of venous obstruction, such as dilated collateral veins, clinical and neurological signs leading to limb weakness can all lead to increased lymphoedema.
The diagnosis is not always easy to make clinically.
Typically, clinically the limb is brawny and does not easily pit on compression. This can be misleading, however. Checking renal function and albumin can be helpful in assessing other causes. A useful further investigation is a colour duplex ultrasound scan. This can help identify venous obstruction by showing impaired venous flow. MRI produces excellent soft tissue imaging and can differentiate true lymphoedema from that due to infiltrating tumour.2
Keeping the skin moist and preventing it from drying out is essential for good skin care, so use of emollients can be beneficial. Cleanliness and good skin hygiene are also important in preventing skin breaks.
Acute infectious episodes
One of the risks of a swollen tissue protein-rich arm is the development of cellulitis. It is advisable not to take bloods from the affected side, put in cannulae or carry out acupuncture; these all increase the risk of infection. Aggressive treatment with antibiotics for at least two weeks may be necessary.
The limb can be much heavier than normal. This can put a lot of strain on different parts of the body. For example, there may be strain on shoulder muscles if the arm is involved. A heavy leg can also cause back pain. Joints can be put under excess pressure.
Infected limbs can be acutely painful. Pressure on nerves such as the brachial plexus can precipitate nerve pain. Use of analgesia, using the WHO pain ladder as a guide, may be helpful. Elastic compression hosiery can also be beneficial. These can be worn during the day. Both sleeves and stockings can be used and usually last a few months.
Elevating the limb can help to improve drainage of fluid and the use of foam supports can help to find the best position.
Teaching the patient how to use self-massage can help to empty the lymphatics. This, together with associated deep breathing, can have some benefit. Avoid this, however, if the limb is infected or the patient has cutaneous metastases.
Gentle exercise using the affected limb can help the flow of lymphatic fluid. More specific exercises can be advised by a specialist therapist. Walking aids and special cutlery can improve patient functioning.
For very swollen limbs, use of pneumatic compression on a regular basis can reduce lymphoedema. This should be avoided in cardiac failure.
Having a severely swollen limb can be psychologically devastating. It can lead to altered body image perception, psychological distress and withdrawal from normal social interactions. This can result in isolation and subsequent depression, and may be exacerbated by the fact that the patient's normal clothes may not fit the affected limb.
A sensitive, supportive approach is essential. Exploring this with patients and helping them come to terms with their condition, as well as trying to improve the skin quality and diminish the swelling, can all be beneficial.
Lymphoedema can be difficult to treat.It is important to assess carefully and rule out cardiac, venous and other causes of oedema, because their management will be different.
Pain can be addressed by standard medication, such as opiates, or paracetamol.
Steroids, such as dexamethasone, can help if malignant infiltration is occurring in lymphatics, by reducing peri-tumour swelling.3
The mainstay of treatment, however, is good skin care, support hosiery, movement and massage. Measurement of treatment outcomes can include reduction in volume, circumference, acute infective episodes, tissue tone and truncal swelling.4 Good psychological support must not be neglected.
- Dr Auty is a GPSI in palliative care in Salford, Gtr Manchester
1. Twycross R, Wilcock A. Symptom management in advanced cancer (third edition). Oxford, Radcliffe Publishing, 2001.
2. Doyle D, Hanks G, Cherny NI et al. Oxford Textbook of Palliative Medicine (third edition). Oxford, Oxford University Press, 2001.
3. Back IN. Palliative Medicine Handbook (third edition). Cardiff, BPM Books, 2001.
4. Outcomes and outcome indicators of lymphoedema treatment. Progr Pall Care 1997: 5(6); 243-6.