Accumulation of excessive lymph fluid in the interstitial space due to an inability of the lymphatic system to carry lymph swelling of subcutaneous tissue (most common in the lower extremities, but can occur in the face, external genitalia or lower part of abdomen). High protein oedema (>1 gm/dl), as compared to low protein oedema of renal disease or congestive heart failure.
‘Elephantiasis’ – grossly enlarged/disfigured limb from lymphoedema resembling an elephant limb.
Primary (idiopathic) – (may be present at birth or develop later); eg Milroy’s disease, Turner’s syndrome, lymphoedema praecox (begins in puberty)
Secondary – due to interruption/occlusion of lymph flow - from infection (following lymphangitis and cellulitis – especially of recurrent); parasitic (eg filariasis); neoplasm – (eg carcinoma of the prostate or ovary common lymphatic occlusion); trauma (direct injury, surgical damage, eg surgical resection of lymph nodes); radiation damage to lymph nodes; fibrotic tissue constricting vessels; chronic venous insufficiency overloads lymphatic vessels; muscle paralysis ( lymph stasis due to lack of muscle pump)
Visible enlargement of extremity with painless oedema. Swelling starts insidiously. Colour is usually normal.
Pitting oedema becomes brawny non-pitting later
Skin thickens difficult to pinch or ‘pick up’ skin – usually most noticeable at base of second toe (Kaposi-Stemmer sign).
Lymphoedema praecox – usually begins around age puberty; F>M; usually starts as a painless swelling on dorsum of foot spreads to involve ankle and lower leg and rest of forefoot.
If secondary – show signs of aetiology; usually sudden onset and unilateral
Pain sometimes occurs
Differential diagnosis – chronic venous insufficiency; lipidaemia; congestive heart failure; arteriovenous fistulas; kidney disease
Long term complications – emotional problems from appearance of limb; gait alterations (eg knee and low back pathology); lymphangiosarcoma arising from damaged lymph vessels
Grade 1 – pitting oedema that is reversible of limb elevation
Grade 2 – non-pitting oedema which is non reversible with elevation (skin hardens due to fibrosis of tissues
Grade 3 - Elephantiasis
• no cure need to remove as much lymph from tissues as possible
• elevation, massage (manual lymph drainage), gentle exercise (to stimulate muscle pump) and elastic compression stockings
• pneumatic compression pumps (effective at reducing water content of tissues, but protein remains; patient generally needs to use daily for >2 hrs; used after manual lymph drainage)
• diuretic can be of some help (only remove water content of tissues; remaining protein content osmotically attracts water when diuretic is stopped)
• but some diuretics (eg frusemide), but not generally indicated unless severe- done with limb elevated and prophylaxis from deep vein thrombosis and monitored for electrolyte imbalances; strict bed rest in enforced in hospital followed by use of compression dressings on discharge
• surgical – can be a debulking procedure or microsurgical techniques to use veins to replace lymph vessels