Lymphoedema may manifest as swelling of one or more limbs and may include the corresponding quadrant of the trunk. Swelling may also affect other areas, eg head and neck, breast or genitalia.
Lymphoedema is the result of accumulation of fluid and other elements (eg proteins) in the tissue spaces due to an imbalance between interstitial fluid production and transport. It arises from congenital malformation of the lymphatic system, or damage to lymphatic vessels and/or lymph nodes.
Lymphoedema is a chronic condition that is not curable at present, but may be alleviated by appropriate management; if ignored, it can progress and become difficult to manage.
How many patients are affected?
At birth, around one person out of 6000 will develop primary lymphoedema; the overall prevalence of lymphoedema/chronic oedema has been estimated as 0.13-2%. In developed countries, the main cause of lymphoedema is widely assumed to be treatment for cancer.
However, it appears that about a quarter to a half of affected patients suffer from other forms of lymphoedema, eg primary lymphoedema and lymphoedema associated with poor venous function, trauma, limb dependency or cardiac disease.
What are the risk factors?
The true risk factor profile for lymphoedema is not known. There may be many factors that predispose an individual to developing lymphoedema or that predict the progression, severity and outcome of the condition.
Classification of lymphoedema
Lymphoedema is classified as primary or secondary.
- Primary lymphoedema is thought to be the result of a congenital abnormality of the lymph conducting system.
- Secondary or acquired lymphoedema results from damage to the lymphatic vessels and/or lymph nodes, or from functional deficiency. It may also be the result of high output failure of the lymphatic circulation.
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