Lymphoedema

Causes of limb swelling

Bilateral pitting oedema

  • Heart failure
  • Renal disease
  • Proteinuria
  • Cirrhosis
  • Carcinomatosis
  • Nutritional

Painful unilateral pitting oedema

  • Deep venous thrombosis
  • Superficial thrombophlebitis
  • Cellulitis
  • Trauma
  • Ischaemia

Painless unilateral oedema

  • Post-phlebitic limb
  • Extrinsic compression of the deep veins
  • Deep venous incompetence
  • Lymphoedema
  • Immobility

Lymphoedema

  • Lymphoedema presents with gradual limb swelling
  • Due to progressive failure of lymphatic system

Primary lymphoedema

  • Congenital (age < 1 year) - familial or non-familial
  • Praecox (age < 35 years) - familial or non-familial
  • Tarda (age > 35 years)

Secondary lymphoedema

  • Malignant disease
  • Surgery - axillary surgery or groin dissection
  • Radiotherapy
  • Infection - parasitic (e.g. filariasis)

Bilateral lymphoedema

Picture provided by Eldeeb Mabrouk, University Hospital, Alexandria, Egypt

Filariasis

Picture provided by Vikram Kate, Jawaharial Institute of Postgraduate Medical Education and Research, Pondicherry, India

Pathology

  • Primary lymphoedema is the result of a spectrum of lymphatic disorders
  • Can be due to aplasia, hypoplasia or hyperplasia of lymphatics
  • In 80% obliteration of distal lymphatics occurs
  • A proportion of patients have a family history (Milroy's disease)
  • In 10% proximal occlusion of lymphatics in abdomen and pelvis is seen
  • In 10% lymphatic valvular incompetence develops
  • Chronic lymphoedema results in subcutaneous fibrosis
  • Fibrosis can be worsened by secondary infection

Clinical features

  • The initial presentation is usually with peripheral oedema worse on standing
  • Begins distally and progresses proximally
  • Limb usually feels heavy
  • Can be unilateral or bilateral
  • Primary lymphoedema is more common in women and is usually bilateral
  • With secondary lymphoedema the underlying cause if often apparent
  • Examination shows non-pitting oedema
  • The skin often has hyperkeratosis, fissuring and secondary infection
  • Ulceration is rare

Investigations

  • Chronic venous insufficiency should be excluded with doppler ultrasound
  • Lymphoedema and its cause can be confirmed with:
    • Lymphoscintigraphy
    • CT or MRI scanning
    • Lymphangiography
  • Lymphoscintigraphy is usually the investigation of choice
  • Has a sensitivity > 90% and specificity of 100%
  • Normal lymphoscintigraphy excludes a diagnosis of lymphoedema
  • Lymphangiography is painful and rarely required

Management

  • The aims of treatment are to:
    • Reduce limb swelling
    • Improve limb function
    • Reduce the risk of infection

Conservative treatment

  • General skin care will reduce risk of infection
  • Swelling can be reduced by elevation
  • Physiotherapy and manual lymph drainage may help
  • External pneumatic compression will also reduce swelling
  • Once swelling is reduced compression stockings should be applied
  • Antibiotics should be given at the first sign of infection
  • Drugs (e.g. diuretics) are of no proven benefit

Surgery

  • Surgery consists of two approaches
    • Debulking operations
    • Bypass procedures
  • Debulking operations include:
    • Homan's operation - elliptical excisions of skin and subcutaneous tissue with primary closure
    • Charles' operation - radical excision of skin and subcutaneous tissue with skin grafts
  • Both produce good functional results
  • Cosmesis is often poor
  • Bypass operations include:
    • Skin and muscle flaps
    • Omental bridges
    • Enteromesenteric bridges
    • Lymphaticolymphatic anastomosis
    • Lymphaticovenous anastomosis
Filiriasis Postoperative appearance

Bibliography

Ko D S,  Lerner R,  Klose G,  Cosimi A B.  Effective treatment of lymphedema of the extremities.  Arch Surg 1998;  133:  452-458.

Mortimer P S.  Swollen lower limb - lymphoedema.  Br Med J 2000;  320:  1527-1530.

Tiwari A,  Cheng KS,  Button M,  Myint F,  Hamilton G. Differential diagnosis, investigation and current treatment of lower limb lymphoedema. Arch Surg  2003; 138: 152-61.

 

 
 

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