A healthy lymphatic system moves fluid, macromolecules and formed elements from the interstitial spaces into the lymphatic vessels, once it enters the lymphatic system it is called lymph fluid. From the initial lymphatics, the lymph is transported through the lymphatic system and into the many lymph nodes along it pathways until it finally enters the blood stream at the subclavian veins (at the neck).
Lymphoedema is the swelling of localised soft tissues as a result of the accumulation of protein rich fluid in the extra cellular spaces. It occurs most frequently in the limbs, but can present in the breast, head, neck, abdomen, and genitalia. There are two types of Lymphoedema. Even though there is no cure for Lymphoedema, with the appropriate treatment the swelling can be reduced and a suitable maintenance programme can keep it under control.
Primary lymphoedema (LE) is caused by alterations (mutations) in genes responsible for the development of the lymphatic system. This mutation causes inadequate or non-function of the lymphatic vessels.
There are three types of primary LE:
Congenital Lymphoedema: which presents at birth, the baby is born with one or more limbs swollen, but often it is just the feet and toes. Seen mostly in Turner syndrome and Milroy’s disease. It accounts for less than 10% of cases. Twice as common in females. Only 2% of cases are hereditary.
Lymphoedema Praecox: develops in adolescence/puberty, again present with limb swelling, but may only affect one side of the body. Accounts of up to 80% of primary cases and is four times more common in females than in males.
Lymphoedema Tarda: onset is over the age of 35, accounts for 10% of cases and presents similarly to Lymphoedema Praecox
Secondary LE can occur following damage to the lymphatic system by surgery, injury or radiotherapy. It is most commonly seen following surgery for cancer of the breast, pelvis, resection for melanoma and head and neck surgery. Secondary LE can occur following injury or accident where the lymphatic pathway and nodes are damaged. It can also develop following parasitic infection and this is known as Filarisis, usually in the continents of Africa and India. Swelling of the legs can also develop as a result of venous insufficiency and varicose veins.