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Chronic peripheral oedema and lymphoedema 

Chronic peripheral oedema and lymphoedema

Chapter:
Chronic peripheral oedema and lymphoedema
Author(s):

Peter S. Mortimer

DOI:
10.1093/med/9780199204854.003.1618_update_001

Update:

This chapter has been extensively revised, including (1) discussion of newly discovered genetic causes of primary lymphedema, (2) new tables of causes of primary and secondary lymphedema, (3) classification and diagnostic algorithm for primary lymphatic dysplasis, with inclusion of molecular findings.

Updated on 29 Oct 2015. The previous version of this content can be found here.
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date: 23 April 2017

Lymph transport, not venous reabsorption, is the main process responsible for interstitial fluid drainage. All peripheral oedema is either absolute or relative lymph drainage failure. 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. Lymphoedema is strictly peripheral oedema due solely to a failure of lymph drainage. Most peripheral oedema arises from microvascular fluid filtration overwhelming lymph drainage, e.g. heart failure, but lymphoedema supervenes as lymph function declines if high filtration is sustained.

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— is caused by genetic faults in lymphatic development. These faults may be chromosomal abnormalities as seen in Turner’s syndrome, germline mutations such as in the vascular endothelial growth factor receptor-3 gene which causes Milroy’s disease or, as a somatic mutation as in congenital lymphatic malformations, but for most forms of lymphoedema the genetic cause is unknown.

For secondary lymphoedema—filariasis is by far the most common cause of lymphoedema worldwide. In the developed world most cases are secondary to cancer treatment and poor mobility.

Clinical features and management

Lymphoedema causes persistent swelling which is often associated with recurrent cellulitis. Response to diuretics is poor. The oedema does pit, but prolonged pressure may be required as the skin becomes thickened and warty. The investigation of choice for confirming lymphoedema is lymphoscintigraphy.

No drug or surgical therapy is known to improve lymph drainage. Current best practice aims to (1) improve lymph drainage through physiological principles known to stimulate lymph flow, and(2) control any excessive microvascular fluid filtration. Exercise and movement combined with compression form the basis of stimulating lymph flow. This generates a high interstitial pressure during muscle contractions to drive lymph drainage. Low pressures during skeletal muscle relaxation permit lymphatic vessel refilling before further muscle contraction repeats the cycle.

Cellulitis, often recurrent, occurs in up to 50% of lymphoedema cases. The mechanism is presumed to be disturbed immune cell trafficking. Each attack can cause further decline in lymph drainage and worse swelling. Prevention and prompt treatment of cellulitis is crucial to the control of lymphoedema.

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