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Lymphedema

Lymphedema

Lymphedema 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, neck, breast or genitalia. Lymphedema 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. 

Lymphedema is a chronic condition that is not curable at present, but may be alleviated by appropriate management; if not treated it can progress and become difficult to manage. 

How many patients are affected?

At birth, around one person out of 6 000 will develop primary lymphedema; the overall prevalence of lymphedema/chronic edema has been estimated as 0.13-2%. In developed countries, the main cause of lymphedema 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 lymphedema, eg primary lymphedema and lymphedema associated with poor venous function, trauma, limb dependency or cardiac disease. 

What are the risk factors?

The true risk factor profile for lymphedema is not known. There may be many factors that predispose an individual to developing lymphedema or that predict the progression, severity and outcome of the condition.

Classification of lymphedema

Lymphedema is classified as primary or secondary.

  • Primary lymphedema is thought to be the result of a congenital abnormality of the lymph conducting system.
  • Secondary or acquired lymphedema results from damage to the lymphatic vessels and/or lymph nodes, or from functional deficiency. Infections from insect bites, serious wounds or burns can cause lymphedema when they damage or destroy lymphatics. Any type of surgery, serious injury, or radiation for cancer treatment can also cause the onset of the disease. It may also be the result of high output failure of the lymphatic circulation.

Treatment of lymphedema

Lymphedema has different stages of development. Compression garments can be used as prophylaxis or as part of intial management in patients who have mild upper limb lymphedema. The main use of compression garments is in the long-term management of lymphedema, usually following a period of intensive therapy. In general, the level of compression used to treat lymphedema o fhte upper limb is lower than that required for lower limb lymphedema.1

Some patients - especially thos who are post breast cancer treatment - may wear a low compression armsleeve (15-20 mmHg) to minimize the risk of developing lymphedema. This may be worn in "high risk" times such as during flying, exercise, or overuse of the arm in consideration. For those patients who have mild edema, or well decongested extremities, an armsleeve of 20-30mmHg might be appropriate. For a patient experiencing a high level of edema, there are several ways to achieve reduction by decongesting the limb, including manual lymphatic drainage and/or compression bandaging.

The result can be sustained by compression garments. Compression garments can be used as initial management in patients who have mild upper limb lymphedema with minimal subcutaneaous tissue changes and shape distortion. Where there is considerable soft pitting edema, multi layer lymphoedema bandaging will be required to reduce and stabilize the swelling prior to the application of compression garments.

Lower pressure compression garments also have a role to play in managing symptoms in a palliative context. 

How to minimize the risk of developing lymphedema?

  • Take good care of skin and nails
  • Follow a balanced diet
  • Maintain optimal body weight
  • Avoid tight underwear, cloting, watches and jewellery
  • Wear comfortable supportive shoes
  • Undertake exercise/movement and limb elevation
  • Avoid injury at risk
  • Avoid exposure to extreme cold or heat
  • Use high factor sunscreen and insect repellent
  • Use mosquito nets in lymphathic filariasis endemic areas
  • Wear compression garments if prescribed or recommended

1) Lymphoedema Framework: International Consensus. Best Practice for the Managment of Lymphoedema. 2006, pages 19-39