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Lymphoedema

Scope

Lymphoedema is a progressive and chronic condition. It arises from congenital malformation of the lymphatic system (primary lymphoedema), or secondary from damage to lymphatic vessels and/or lymph nodes. Figures are estimated at 3.93/1000, (England 2016), with other studies estimating higher, as Wales (2017) projected 5.49/1000.

Out of scope

  • Acute oedema
  • Chronic oedema that can be managed in Primary Care
  • Measuring hosiery service
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History and Examination

  • Medical history to establish if primary or secondary
  • Family history

Signs and Symptoms

  • Swelling commonly of one or more limbs that may include the corresponding quadrant of the trunk or less common to other body areas, that has been present for more than three months
  • Swelling of limbs affects hands and feet
  • Affects both male and females
  • Stemmer's sign may be positive
  • Aching in affected part
  • Skin changes – hyperkeratosis, lymphangectasia, papillomatosis
  • Stages:
    • Early (mild) - soft pitting oedema
    • Moderate - tissue firming with pitting, skin changes
    • Late (severe) - tissues firm and non-pitting, with skin changes
  • No improvement with diuretics (a trial of diuretics is not recommended unless there is known associated cardiac failure).

Differential Diagnoses

  • Acute and chronic oedema

Obesity

Where a patient presents with chronic swelling, of more than 3 months the type/cause of the swelling should be established.

Unknown cause

Investigations/assessment should be undertaken to exclude the following causes:

  • Iatrogenic – Steroids, NSAIDS, Calcium antagonists
  • Anaemia
  • Presence or recurrence of tumour
  • Congestive heart failure
  • Vena Cava obstruction
  • Deep vein Thrombosis
  • Renal Failure
  • Liver Failure
  • Hypothyroidism
  • Hypoproteinaemia

Where these causes have been excluded or treated consider either

Secondary Lymphoedema as a result of:

  • Trauma/Tissue damage
  • Malignant disease
  • Venous disease
  • Infection
  • Inflammation
  • Endocrine disease
  • Immobility and dependence
  • Lipoedema

Or

Primary lymphoedema .

  • This is usually diagnosed after the exclusion of secondary lymphoedema.

  • Skin care – to prevent injury and infection
  • Exercise – to improve lymph flow
  • Elevation – of legs when sitting, sleeping in a bed
  • Compression bandaging/garments
  • Weight management – lymphoedema is exacerbated by weight gain/obesity. Patients should be encouraged to maintain and/or reduce their weight. Patients with a BMI over 40 should be offered referral to a dietician or weight management

Patients with acute cellulitis or active infection should first be treated in line with local formulary guidance (the British Lymphology Society (BLS) also have guidelines for cellulitis in lymphoedema patients).

Referral Criteria

  • Patients should have been appropriately investigated as above to ensure correct diagnosis.
  • Patients are unlikely to benefit from Lymphoedema treatment if they have: a BMI over 40 who are unwilling to see a dietician or follow a Weight Management programme, unwilling to wear compression or a likelihood of non-concordance.

North Devon/North Cornwall – Age 18 years +. 

Referral Instructions:

North Devon/North Cornwall – by letter or referral form, with medical history, medication and the results of any investigations.

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