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)
 
      
     
    
    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
 
        
     
    Dr. Pankaj Patel a vascular surgeon has expertise in peripheral vascular diseases, varicose veins and deep vein thrombosis