When Does Lymphoedema Occur?

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If the lymphatic system fails to work normally, a build-up of fluid (lymph) develops in the affected area, which eventually becomes swollen.  This swelling is known as lymphoedema.

There are two types of lymphoedema: 

Primary lymphoedema is due to an abnormality of the lymphatic system, which is present from birth.  It often runs in families and can be due to an underdeveloped or abnormally functioning lymphatic system.  When the lymphatic system can no longer cope, swelling develops. This can occur in infants, children and adults. 

Secondary lymphoedema may occur following damage to a normal lymphatic system.  This can be as a result of surgery, e.g. the removal of lymph nodes during surgery for cancer, or as a side effect of radiation treatment for cancer.  It can also occur as a result of chronic venous insufficiency, or after injury, scarring, or infection of the lymphatic system.

Both types of lymphoedema progress through the same stages:

Stage 0 (Latent stage)

In this early stage, there is no obvious swelling. The lymphatic system has however been disturbed, e.g. by surgery, trauma, infection, etc., or by abnormalities present from birth, and there is a risk of lymphoedema developing at any time. It is therefore important that you are given advice about avoiding further damage to the lymphatic system so that the risk of lymphoedema developing can be reduced.

Stage l (Reversible stage)

During this stage, the lymphatic system has been overloaded, causing swelling to develop in the affected area.  This swelling can be indented by applying pressure (pitting oedema).  When the affected arm or leg is raised, the swelling will gradually reduce.  If left untreated at this stage, lymphoedema can progress to Stage II.

Stage II (Spontaneously irreversible stage)

In this stage, the swelling cannot be indented by applying pressure, and raising the affected area no longer reduces the swelling.  The texture of the swelling changes due to a gradual hardening of the tissues, which is known as fibrosis.

Stage III

In this, the most advanced stage, the swelling is severe, the skin becomes harder and large bulges may develop. There is also a much greater risk of infection and the development of wounds in the affected area.