Lymphoedema

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.

Aetiology:
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)

Clinical Features:
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

Grading:
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

Treatment:
• 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

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