Lymphoedema

Lymphoedema is the accumulation of tissue fluid in the extracellular compartment (Interstitial space) due to impaired drainage of lymph in the presence of normal capillary function.

Commonly lymphoedema is seen in the lower limbs, but can be seen in the upper limbs and scrotum. In the upper limbs it is particularly seen in post-mastectomy patients.



Characteristics of lymphoedema

  • Protein rich fluid
  • Confined to the epifasical space (Skin and subcutaneous tissues)
    • Muscles compartments are free of oedema
  • Defect in lymphatic drainage
  • Normal capillary function 
 
 
Causes Of Lymphoedema
  • Primary
    • Familial 
      • Lymphoedema congenital (Less than 1 year of age)
        • Milroy’s disease
          • Inheritance – Autosomal dominant 
          • Mutation in the FLT4 gene
          • Bilateral disease
          • Brawny edema of both lower limbs
            • (Brawny oedema – Swelling offers resistance but leaves no impressions)
          • Develops from birth or before puberty
      • Lymphoedema precox (1 – 35 years of age)
        • Meige disease 
          • Inheritance – Autosomal dominant 
          • But no genes are associated with Meige disease
          • Lymphoedema develops around puberty 
            • Unilateral / bilateral disease 
            • Upper limbs can also be affected
          • Develops between puberty and before 35 years of age
      • Lymphoedema tada (After 35 years of age)
        • Associated with obesity
        • Lymph nodes are replaced by fibrofatty tissues
    • Syndromic 
      • Klippel-Trenaunay syndrome
        • Inheritance – Sporadic
        • Characterized by….
          • Congenital varicose veins present 
          • Lymphoedema 
          • Limb length discrepancy – Leading to limping gait
      • Lymphedema distichiasis syndrome
        • Inheritance – Autosomal dominant 
        • Mutation of the FOXC2 gene
        • Characterized by….
          • Lymphoedema – Develops around puberty
          • Extra eyelashes
  • Secondary
    • Infection 
      • Parasitic infection (Filariasis)
      • Bacterial infection 
    • Lymphatic malignancy 
    • Following groin or axillary surgery
    • Radiotherapy to lymph nodes
    • Surgical removal of lymph nodes 

Lymphoedema

 

 
Stages Of Lymphoedema
  • Stage 1 
    • Soft pitting oedema
      • Due to accumulation of lymph in the interstitial space
  • Stage 2
    • Oedema become more pronounced 
    • Develops non pitting oedema
    • Soft to firm in consistency 
      • Accumulation of fibroblasts, adipocytes and macrophages in the affected tissues causing an inflammatory reaction leading to structural changes in the tissues
  • Stage 3 
    • Skin changes (Fibrosis, papillae formation, scarring)
      • Inflammatory process further damages the functioning lymphatics 
      • Progressive worsening of lymphatic drainage
 
 
Clinical Presentation Of  Lymphoedema
  • Swelling of the limb
    • Loss of ankle contour 
    • Buffalo hump on the dorsum of the foot 
    • Initially develops pitting oedema which becomes non pitting oedema later on 
  • Pain in the affected site
    • Dull aching / bursting / burning paint  
  • Paraesthesia (Pins and needles sensation)
  • Concomitant infective episodes
  • Skin changes 
    • Fibrosis
    • Dermal thickening
    • Ezcema
  • Stemmer’s sign
    • Early sign of lymphoedema 
    • Skin cannot be pinched on the dorsum of the second toe
      • Due to subcutaneous fibrosis 

Stemmers sign

Fig : Stemmer’s Sign

Investigations Of Lymphoedema
  • Usually it is a clinical diagnosis
  • But in case of uncertainty, investigations may be required
  • Lymphoscintigraphy
    • Radioactive substance is injected subcutaneously into the foot, 
    • Monitor its progression using a gamma camera
    • A delay in transit – Confirms the diagnosis
  • Lymphangiography
    • Done prior to lymphatic reconstruction
    • Radio-opaque dye is inserted into a lymphatic channel in the lower limb under direct exposure 
    • Radiographs are taken to visualize the lymphatic system
 
 
Treatment Of Lymphoedema
  • Conservative management 
    • Elevation of the limb
    • Compression with pressure bandages and stockings
    • Lymphopress 
      • Serial pneumatic compression device 
    • Identify and treat the cause 
    • Antibiotics – Infections should be identified early and treated aggressively (Cellulitis)
    • No place for diuretics
  • Surgery – Poor outcome
    • Indications for surgery 
      • Failure of conservative management 
      • Marked disability
      • Deformity of the limb
      • Proximal lymphatic obstruction with patent distal lymphatics 
      • Lymphocutaneous fistulae
      • Megalymphatics
    • Surgical procedures
      • Homan’s procedure
        • Debulking surgery
        • Overlying skin is preserved and is used for primary closure of the wound
        • Wedge of tissue is removed 
        • Limb is reduced by a third
        • Overlying skin has to be in good condition
      • Charles’ operation
        • Debulking surgery 
        • All skin and subcutaneous tissue is removed up to the deep fascia
        • Split skin grafts are used for closure of the exposed fascia 
        • Greater reduction than Homan’s procedure
        • Done in cases where overlying skin is not in good condition
      • Lymphovenous anastamosis
        • By pass procedure 
        • Done when there is proximal obstruction with patent distal lymphatics 
        • Lymphatic channels are anastamosed to the subdermal venules 
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