Surgery chapters
Lymphatic disorders and lymphoedema
Anatomy and function of the lymphatics, primary and secondary lymphoedema (including filariasis and post-cancer treatment), diagnosis with Stemmer's sign and lymphoscintigraphy, complications and conservative and surgical management.
In 30 seconds
- Lymphoedema is swelling from failed lymph drainage; it is protein-rich, starts in the foot and toes, pits early but not later, and gives a positive Stemmer's sign (cannot pinch the skin on the dorsum of the second toe).
- Primary forms are congenital (Milroy's, under 2 years), praecox (2-35 years, commonest primary, young women) and tarda (over 35); secondary forms are far commoner: filariasis worldwide and cancer surgery/radiotherapy (axillary or groin dissection) in richer countries.
- Treatment is mainly conservative: skin care, prompt antibiotics for cellulitis, exercise, multilayer bandaging then compression garments and manual lymphatic drainage; diuretics do not help; surgery is for selected cases.
On this page
Overview
The lymphatic system returns protein-rich tissue fluid to the blood. When drainage fails, fluid and protein build up in the skin and subcutaneous tissue: this is lymphoedema. It is chronic, disfiguring and prone to infection.
PGET usually has one MCQ here, mostly on causes, clinical signs, filariasis and management.
Anatomy and physiology
- Lymph from the legs, abdomen and left upper body drains via the cisterna chyli and thoracic duct into the left internal jugular and subclavian vein junction. The right head, neck and arm drain through the right lymphatic duct.
- Lymphatics run with veins (the superficial leg lymphatics follow the saphenous veins); there are none in brain or the cortex of bone.
- Lymph vessels have valves and contract rhythmically; muscle action and breathing also push lymph along.
- Normal lymph flow returns several litres of fluid and much of the filtered plasma protein each day.
Why lymphoedema is different from other oedema
| Feature | Lymphoedema | Most other oedema (heart, kidney, venous) |
|---|---|---|
| Cause | Low lymph drainage | High capillary filtration |
| Fluid | Protein-rich | Low protein |
| Tissue response | Inflammation, fibrosis, fat deposition, skin thickening | Little fibrosis early |
| Pitting | Pits early, non-pitting later | Pits |
| Elevation | Little effect once established | Usually improves |
| Compartment | Above the deep fascia (muscles spared) | Variable |
Classification
Primary lymphoedema (defect of the lymphatics themselves)
| Type | Age of onset | Features |
|---|---|---|
| Congenital | At birth or before 2 years | Commoner in males; often bilateral, whole leg. Familial form = Milroy's (Nonne-Milroy) disease (VEGFR-3 mutation) |
| Praecox | 2-35 years | Commonest primary type; three times commoner in females, peaks soon after puberty; usually one leg, to the knee. Familial form = Meige's disease |
| Tarda | After 35 years | Often with obesity |
- Lymphatic patterns on imaging: distal obliteration (commonest, mild, women), proximal obliteration, or hyperplasia with megalymphatics that can cause chylous reflux (chyluria, chylous ascites, chylothorax, chylous vesicles on skin).
- Syndromes with lymphoedema: Turner syndrome, Noonan syndrome, yellow nail syndrome (yellow nails, pleural effusion, lymphoedema), distichiasis syndrome (extra row of eyelashes).
Secondary lymphoedema (damage from outside) — the commonest form
| Cause | Examples |
|---|---|
| Parasitic | Filariasis (commonest cause worldwide) |
| Cancer treatment | Axillary node dissection and radiotherapy for breast cancer (commonest in richer countries); groin dissection for melanoma, vulval or penile cancer; pelvic radiotherapy |
| Malignant obstruction | Metastatic nodes, lymphoma, pelvic and genital tumours, Kaposi sarcoma |
| Infection | Recurrent cellulitis/lymphangitis, tuberculosis of nodes, fungal infection (tinea pedis) |
| Trauma | Degloving injuries, burns, extensive scarring |
| Venous disease | DVT, superficial thrombophlebitis; varicose vein stripping and vein harvesting damage lymphatics |
| Other | Podoconiosis (silica particles absorbed through bare feet in volcanic soil, Africa), rheumatoid arthritis, pretibial myxoedema, factitious (tourniquet) |
High-yield
Lymphoedema appearing for the first time after 50, especially if it starts in the thigh and spreads down, must prompt a search for pelvic or genital cancer (prostate, bladder, cervix, lymphoma).
Filariasis
- Caused mainly by the nematode Wuchereria bancrofti (most cases), also Brugia malayi; spread by mosquitoes (Culex and others). Endemic in parts of Africa, South and South-East Asia (including India) and South America; linked to poor sanitation.
- Adult worms live in lymphatics and nodes, causing obstruction and fibrosis from direct damage and the host immune response.
| Phase | Features |
|---|---|
| Acute | Fever, malaise, lymphangitis (spreading outwards, "retrograde"), lymphadenitis, funiculitis, epididymo-orchitis |
| Chronic | Lymphoedema of legs (also arm, breast), elephantiasis (massive warty limb), hydrocele (commonest chronic sign in men), scrotal lymphoedema, chyluria (milky urine), abdominal lymph varices |
- Diagnosis: night blood smear for microfilariae (nocturnal periodicity: sample around midnight), filarial antigen test (ICT card), eosinophilia, ultrasound "filarial dance sign" of live worms in lymphatics, microfilariae in chylous urine.
- Treatment: diethylcarbamazine (DEC) kills the parasites (often with albendazole; ivermectin is used where onchocerciasis coexists). It does not reverse established lymphoedema, which is then treated like other lymphoedema (hygiene, compression). Hydrocele needs surgery.
- Prevention: mass drug administration programmes, mosquito control, bed nets.
Clinical features of lymphoedema
- Swelling, heaviness, tightness of clothing, aching; often affects self-image and work.
- Leg lymphoedema starts in the foot:
- Squared-off toes
- "Buffalo hump" on the dorsum of the foot
- Loss of the hollows around the ankle
- Stemmer's sign: the skin over the dorsum of the second toe cannot be pinched up (a positive sign means lymphoedema)
- Early: soft and pitting, improves overnight. Later: firm, non-pitting, does not settle on elevation.
- Late skin changes: hyperkeratosis, warty papillomatosis, deep skin folds, lymphorrhoea (leaking clear lymph), lymphangiomas (lymph blisters), fungal infection between the toes.
Clinical grades
| Grade | Features |
|---|---|
| Latent | Abnormal lymphatics, no visible swelling |
| I | Pits; largely goes down with elevation and bed rest |
| II | Does not pit and does not settle with elevation; Stemmer's sign positive |
| III | Irreversible skin changes: fibrosis, papillae, warty growths (elephantiasis) |
- Severity is also judged by excess limb volume compared with the other limb (mild under 20%, moderate 20-40%, severe over 40%).
Differential diagnosis of a swollen leg
| Condition | Clue |
|---|---|
| DVT | Acute, painful, calf tenderness; duplex |
| Chronic venous insufficiency | Pigmentation, varicose veins, ulcers at the gaiter area; foot usually spared |
| Lipoedema | Women, onset at puberty, both legs symmetrically, feet spared (a "cuff" at the ankle), fat is tender and bruises easily, no pitting, Stemmer's negative |
| Cardiac, renal, liver failure, hypoproteinaemia | Bilateral pitting; systemic signs |
| Drugs (calcium-channel blockers) | Ankle oedema |
| Hypothyroidism (myxoedema) | Systemic features |
| Arteriovenous malformations, Klippel-Trenaunay | Limb overgrowth, port-wine stain |
Investigations
- Most cases are diagnosed clinically.
- Baseline tests: FBC, renal, liver and thyroid function, albumin, glucose, urine for protein and chyle, night blood film for microfilariae (in endemic areas), chest X-ray.
- Duplex ultrasound of veins to exclude DVT or venous disease.
- Isotope lymphoscintigraphy (technetium-labelled colloid injected into a toe web space; tracked by gamma camera): the investigation of choice when the diagnosis is uncertain. Shows slow or absent tracer transport and dermal back-flow.
- Contrast lymphangiography: now rarely used (it can damage lymphatics); kept for chylous reflux and before surgery.
- CT or MRI: to look for pelvic or abdominal malignancy obstructing lymphatics; MRI shows the typical honeycomb pattern above the fascia.
- Limb volume measurement (tape measure or water displacement) to follow treatment.
Complications
- Cellulitis / "acute inflammatory episodes": the commonest serious complication; each attack damages more lymphatics. Usually streptococcal.
- Fungal infection, eczema, ulcers, lymphorrhoea.
- Pain, loss of function, psychological distress.
- Lymphangiosarcoma (Stewart-Treves syndrome): rare, aggressive malignant tumour of long-standing lymphoedema, classically in the arm about 10 years after mastectomy; purple-red nodules or non-healing bruises. Poor prognosis.
- Other cancers in lymphoedematous skin: Kaposi sarcoma, squamous cell carcinoma, lymphoma.
Management
Treatment is lifelong and mainly non-surgical; there is no cure.
Decongestive lymphoedema therapy (DLT)
Phase 1 (intensive, 2-4 weeks) then Phase 2 (maintenance, lifelong). Components:
| Component | Details |
|---|---|
| Skin care | Wash and dry carefully (especially between toes), moisturise with emollients, treat athlete's foot, avoid cuts, insect bites, sunburn; never walk barefoot |
| Exercise | Movement while wearing compression pumps lymph; keep weight down |
| Multilayer lymphoedema bandaging (MLLB) | In the intensive phase: reduces swelling, restores limb shape, stops lymphorrhoea and softens tissues |
| Compression garments | The mainstay of long-term control; higher pressures than for venous disease. Check ABPI first: no compression if ABPI is under 0.5 |
| Manual lymphatic drainage (MLD) | Special gentle massage by a therapist that moves lymph towards working lymphatics; simple self-massage for maintenance |
| Pneumatic compression pumps | Multi-chamber devices may help some patients |
Treating cellulitis (acute inflammatory episodes)
- Oral amoxicillin is first choice (clarithromycin or erythromycin if penicillin allergic); add flucloxacillin if staphylococcal signs; IV antibiotics if septic or not settling within about 48 hours.
- Bed rest and elevation; stop massage and compression during the acute attack; avoid NSAIDs (linked to necrotising fasciitis); thromboprophylaxis if immobile.
- Patients with two or more attacks a year may take long-term prophylactic penicillin V; many carry a standby course to start at the first sign of trouble.
Exam trap
Diuretics have no role in pure lymphoedema; the fluid is protein-rich and diuretics only cause electrolyte problems. They help only when another cause (e.g. heart failure) coexists.
Exam trap
In a woman with lymphoedema, do not strip varicose veins found on duplex: removing the saphenous veins also damages the lymphatics that run with them and can make the swelling worse.
Drugs
- DEC (with albendazole) for active filarial infection.
- Benzopyrones are of doubtful benefit.
Surgery (for a minority)
Indications: severe, disabling swelling or deformity not controlled by good conservative care, recurrent infection, lymphorrhoea, or massive genital lymphoedema.
| Type | Examples |
|---|---|
| Reducing (excisional) operations | Charles procedure: remove the whole thickness of skin and fat, together with the deep fascia, around the limb and then cover with split-skin grafts (very poor cosmetic result, used for massive elephantiasis). Homans procedure: raise skin flaps and remove a wedge of subcutaneous tissue (medial and lateral sides done months apart). Thompson's operation: buries a dermal flap to create new lymphatic connections |
| Liposuction | For non-pitting, fat-dominant arm or leg lymphoedema; must be followed by lifelong compression |
| Bypass / physiological | Lymphaticovenular anastomosis (LVA) with microsurgery; vascularised lymph node transfer; older methods (omental pedicle, enteromesenteric bridge) |
High-yield
Surgery does not cure lymphoedema; patients still need compression garments for life after any operation.
Prevention after cancer surgery
- Sentinel node biopsy instead of full axillary clearance where possible; avoid combining axillary surgery with axillary radiotherapy.
- Early arm exercises; skin care; prompt treatment of infection; avoid injections, blood tests and BP cuffs on the affected arm where practical.
Other lymphatic conditions
- Lymphangitis: red streaks running towards the regional nodes from an infected wound (usually streptococcal), with tender nodes and fever. Treat with antibiotics (penicillin) and elevation.
- Lymphangioma: dilated lymph channels: lymphangioma circumscriptum (small skin vesicles like frogspawn) and cystic hygroma (cystic lymphatic malformation in the posterior triangle of a child's neck, brilliantly transilluminant). Treated with sclerotherapy (e.g. OK-432, bleomycin) or excision.
- Chylous disorders: chylothorax (thoracic duct injury in chest or neck surgery: milky effusion with high triglycerides), chylous ascites, chyluria (filariasis). Managed with drainage, a low-fat diet with medium-chain triglycerides (absorbed directly into the portal blood, bypassing lymphatics), sometimes total parenteral nutrition or duct ligation/embolisation.
Exam focus
Expect: the commonest cause of lymphoedema worldwide (filariasis) and in developed countries (breast cancer treatment), primary types by age (Milroy's congenital, praecox commonest, tarda), Stemmer's sign and foot involvement, lymphoedema versus lipoedema, lymphoscintigraphy as the investigation of choice, night blood smear and DEC for filariasis with hydrocele and chyluria, cellulitis treatment with amoxicillin and prophylaxis, no role for diuretics, compression after ABPI check, Charles procedure, Stewart-Treves lymphangiosarcoma, cystic hygroma transillumination and MCT diet for chylous leaks.
Quick revision
- Lymphoedema: protein-rich, starts in the foot, Stemmer's sign positive.
- Primary: congenital (Milroy's, under 2), praecox (2-35, commonest, young women), tarda (over 35).
- Secondary (commoner): filariasis worldwide; breast cancer treatment in richer countries.
- New lymphoedema after 50: look for pelvic cancer.
- Lymphoscintigraphy = investigation of choice; night smear for microfilariae.
- DEC kills filariae but does not reverse the swelling.
- Mainstay: skin care, exercise, bandaging, compression, MLD.
- Cellulitis: amoxicillin; prophylactic penicillin V for frequent attacks; no NSAIDs.
- No diuretics. Charles procedure for massive elephantiasis.
- Stewart-Treves: lymphangiosarcoma in chronic post-mastectomy lymphoedema.
Written by The House Job from standard references (Bailey & Love).
FAQ
What is Stemmer's sign?
Inability to pinch and lift a fold of skin over the dorsum of the second toe because of thickening and fibrosis; it is positive in lymphoedema and negative in lipoedema.
What is the commonest cause of lymphoedema?
Worldwide it is filariasis caused by Wuchereria bancrofti; in developed countries it is treatment for breast cancer, especially axillary surgery combined with radiotherapy.
Why are diuretics not used for lymphoedema?
The swelling is protein-rich fluid trapped by failed lymph drainage, not salt and water overload, so diuretics remove little of it and cause electrolyte problems.
How is filariasis diagnosed?
By finding microfilariae in a blood smear taken around midnight, or by filarial antigen tests, often with eosinophilia; ultrasound can show moving adult worms.
Can lymphoedema be cured by surgery?
No. Surgery can reduce bulk or improve drainage in selected patients, but compression garments and skin care are still needed for life.