Lymphoedema
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Direct answer
In India a leg that swells after repeated febrile episodes with lymphangitis and hardens over years is filarial until proved otherwise — Wuchereria bancrofti the commonest cause in the country — while the post-mastectomy arm after axillary dissection and radiotherapy is the other great group. Management follows the International Society of Lymphology staging: conservative decongestive therapy — manual lymphatic drainage, compression, exercise and meticulous skin care — is lifelong and comes first for everyone; surgery is an adjunct, physiologic (lymphovenous anastomosis, vascularised lymph node transfer) for early stages with patent channels, excisional (liposuction, staged excision, the Charles operation) for late fibrotic elephantiasis. Diuretics have no role.
What you must remember
- ISL stages: 0 latent; I fluid-rich, pitting, largely resolves with elevation; II spongy, late non-pitting with fat and fibrosis; III elephantiasis with trophic skin, papillomatosis, deep folds.
- Stemmer's sign — inability to pinch the dorsal skin at the base of the second toe — positive from stage II, and the bedside test worth quoting.
- Primary lymphoedema: congenita (Milroy disease), praecox before 35 (the commonest, including Meige disease), tarda after 35.
- Secondary causes in India: filariasis above all — the national elimination programme runs annual mass drug administration with diethylcarbamazine and albendazole — then post-axillary dissection arms, groin dissections, recurrent cellulitis.
- Stewart-Treves syndrome: lymphangiosarcoma in long-standing post-mastectomy lymphoedema, typically after a decade — a bruise-like nodule in a swollen arm needs biopsy.
- Investigations: lymphoscintigraphy standard; indocyanine green lymphography for channel patency; MRI or duplex to exclude pelvic malignancy and venous disease.
- Complete decongestive therapy: intensive phase (bandaging, manual drainage) then maintenance garments (about 20–40 mmHg arm, 30–50 mmHg leg) — for life.
- Physiologic surgery: lymphovenous anastomosis by supermicrosurgery for early disease with functioning channels; vascularised lymph node transfer (groin, gastroepiploic, thoracodorsal) for intermediate disease and irradiated fields.
- Excisional surgery: circumferential liposuction for fat-dominant disease; staged subcutaneous excision; the Charles operation for stage III filarial elephantiasis.
- Every cellulitis episode worsens the limb: treat tinea pedis and other entry sites; antibiotic prophylaxis after recurrent attacks.
One filarial leg through the decades
A 28-year-old labourer from an endemic district has transient ankle swelling after fever — acute filarial lymphangitis, treated under the national programme. He returns at 35 with stage II disease: heavy leg, Stemmer positive, non-pitting, recurrent erythema treated as cellulitis. The programme is complete decongestive therapy — intensive bandaging, a garment all day, daily washing with drying between the toes, tinea treatment, and penicillin prophylaxis after a third attack. Indocyanine green lymphography still shows functioning channels, so a lymphovenous anastomosis is offered — adjunctive, and the garment continues. At 48 he is stage III: elephantiasis with deep folds, papillomatosis and recurrent infection. Options are reasoned, not listed: liposuction addresses fat, but his burden is fibrosis; staged excision debulks the medial leg; the Charles operation is the end-stage answer, accepted with its rough grafted skin and hyperkeratosis because it removes the recurrent infection destroying his life.
Diuretics and missed fungus
Two errors dominate clinical practice and the viva. The first is the referral letter prescribing frusemide: diuretics strip intravascular volume while doing nothing for the protein-rich interstitial load, and may even promote fibrosis. The second is the ignored entry portal: tinea pedis is the engine of recurrent cellulitis, each attack destroying lymphatics; treating it, and daily washing with drying between the toes, is as therapeutic as any garment. Distinguish mimics — venous oedema (pitting, stasis changes, responds to elevation) and lipodema (bilateral, symmetric, sparing the feet, Stemmer negative) — because operating on lipodema as lymphoedema disappoints everyone. And the surgical humility point: an operation without lifelong compression fails; compression without surgery at least holds the line.
Frequently asked questions
What is Stemmer's sign, and when does it appear?
Inability to pinch a fold of skin at the base of the second toe, reflecting dermal thickening — positive from about ISL stage II, negative in early stage I and in lipodema. The quickest bedside discriminator.
Name the three clinical types of primary lymphoedema.
Congenita (Milroy disease), present at birth and often familial; praecox, before the age of 35, the commonest primary form including Meige disease; and tarda, after 35. All are diagnosed after secondary causes are excluded.
Which imaging decides suitability for lymphovenous anastomosis?
Indocyanine green near-infrared lymphography, showing functioning channels in real time — patent channels suit supermicrosurgical anastomosis, while a limb with none needs excisional surgery or node transfer. Lymphoscintigraphy remains the diagnostic standard.
What is the Charles operation, and when is it indicated?
Radical excision of all lymphoedematous skin and subcutaneous tissue down to fascia, resurfaced with split grafts — reserved for stage III elephantiasis, classically massive filarial disease. Its costs are conspicuous grafted skin and hyperkeratotic margins, so it is an end-stage, not a default, option.
Why do diuretics not work in lymphoedema?
Because the interstitial fluid is protein-rich and the defect is transport, not hydrostatic pressure; diuretics reduce intravascular volume and may increase interstitial protein concentration, promoting fibrosis. The effective "pharmacology" is compression, movement and skin care.