Chronic peripheral oedema and lymphoedema

Peter S. Mortimer

in Oxford Textbook of Medicine

Fifth edition

Published on behalf of Oxford University Press

Published in print May 2010 | ISBN: 9780199204854
Published online May 2010 | e-ISBN: 9780199570973 | DOI:

Series: Oxford Textbooks

Chronic peripheral oedema and lymphoedema

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Lymph transport, not venous capillary reabsorption, is the main process responsible for interstitial fluid drainage. Oedema develops when the microvascular filtration rate exceeds lymph drainage for a sufficient period, either because the filtration rate is high or because lymph flow is low, or a combination of the two. In the United Kingdom the prevalence of lymphoedema is just over 0.1%, rising to 0.5% in those over 65 years of age.

Causes of lymphoedema

Lymph drainage may fail either because of a defect intrinsic to the lymph conducting pathways (primary lymphoedema), or because of irreversible damage from some factor(s) originating from outside the lymphatic system (secondary lymphoedema).

Primary lymphoedema—can be caused by mutations in the vascular endothelial growth factor receptor-3 gene (Milroy’s disease) or in the forkhead transcription factor gene FOXC2 (lymphoedema—distichiasis), but for most forms the genetic cause is unknown. Congenital lymphoedema may occur in isolation or as part of a syndrome.

Secondary lymphoedema—filariasis is by far the most common cause of lymphoedema worldwide. In the United Kingdom 25% of cases are due to malignancy.

Clinical features and management

Lymphoedema causes persistent swelling that does not resolve with overnight elevation and is associated with cellulitis. Response to diuretics is poor. The oedema does pit, but prolonged pressure is required to do so, and the skin is thickened and warty. The investigation of choice for confirming that oedema is primarily of lymphatic origin is lymphoscintigraphy (isotope lymphography).

No drug or surgical therapy is known to improve lymph drainage. Treatment relies on improving lymph drainage through the application of simple physiological principles known to stimulate lymph flow, while at the same time restoring any excessive capillary filtration to as near normal as possible. Patients with leg lymphoedema often notice that walking reduces swelling, and the addition of a bandage or stocking can enhance the effect of movement by creating an outer collar to the leg that resists expansion of the calf muscle during contraction. This generates a high interstitial pressure during muscle contractions to drive lymph filling and drainage, but allows low pressures during skeletal muscle relaxation and hence permits lymphatic vessel refilling before further muscle contraction repeats the cycle. Compression without movement (active or passive exercises) does not improve lymph drainage. Manual lymphatic drainage therapy, a specific form of lymphatic massage, operates on the same principle of stimulating alternating rises and falls in interstitial pressure and is used to decongest more proximal regions of the body.

Prevention and prompt treatment of cellulitis is crucial to the control of lymphoedema, because this contributes to further decline in lymph drainage, which in turn encourages more infection.

Chapter.  5702 words.  Illustrated.

Subjects: Cardiovascular Medicine

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