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Primary Lymphoedema

About one person in 6000 will develop primary lymphoedema.

Primary lymphoedema is a congenital abnormality occurring within the lymphatic system.

It may be classified according to lymphatic vessel appearance:
  • Aplasia - No collecting lymph vessels detected
  • Hypoplasia - A lower than normal number of lymph vessels detected
  • Hyperplasia -An increased number of lymphatic vessels detected

However, because of the wide range of features of Primary Lymphoedema a clinical classification is determined by age of onset of the swelling.
Congenital - Present at birth or within two years
  • Accounts for up to 25% of primary lymphoedema cases
  • It affects females twice as often as males
  • Is 3 times more likely to affect the legs than the arms
  • Most cases are bilateral – affecting both arms or both legs
     
Praecox - Between 2-35 years of age
  • The most common form of primary lymphoedema approximately 90% of all cases
  • Affects women at a ratio of men at 10 to 1
  • Usually emerges at puberty
  • Most cases are unilateral and limited to the foot or calf

 

Tarda - Appearing after the age of 35
  • Accounts for less than 10% of cases of lymphoedema
  • Also called Miege’ Syndrome

Treatment for primary lymphoedema involves classification and diagnosis by a trained lymphoedema therapist and an individualized treatment plan based on the four cornerstones of care in the management of lymphoedema: Manual Lymphatic Drainage, Compression, Exercise and Skin Care.

Medical taping is also very effective in Primary Lymphoedema Management.
 

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