Key points

• The commonest cause of lymphoedema worldwide is filariasis

• In the Western world the commonest cause is malignancy and its treatment

• Oedema is initially pitting, but becomes non-pitting due to fibrosis of subcutaneous tissues

• Diagnosis is confirmed by isotope lymphangioscintigraphy

• Satisfactory treatment can usually be achieved by conservative measures that include manual drainage, compression hosiery, complex decongestive therapy and prevention of infection

There are various conditions that cause chronic lower limb swelling (Table 17.1). The two most common are chronic venous insufficiency and lymphoedema. Lymphoedema is a debilitating condition that has no cure. Several million people are affected worldwide.

Definition

Lymphoedema can be defined as the accumulation of fluid rich in protein in the skin and subcutaneous tissues due to a defect in the lymphatic system resulting in swelling of the limb.

Classification

Lymphoedema can be primary or secondary.

Primary

Primary lymphoedema can be classified based on the age of onset (Table 17.2). Women are more likely to be affected than men. The lower limbs are more frequently affected than the upper limbs.

• Congenital lymphoedema is present at birth and can be autosomally inherited (Milroy's disease). Oedema is present at birth and two-thirds of affected patients have bilateral lymphoedema. It accounts for 25% of all cases.

• Lymphoedema praecox presents up to the age of 35 years, usually during adolescence. It is the commonest form of congenital lymphoedema. Most patients have unilateral limb involvement.

• Lymphoedema tarda presents after the age of 35 years. This is the least common form and accounts for about 10% of cases.