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.