Lymphedema : Types, Clinical Features and Complications

The role of the lymphatic system is to collect the excess fluid, proteins, and toxins from cells and tissues and return them to the bloodstream. When there is any condition that affects the lymphatic system, it will not work properly and that leads to the accumulation of fluid and begins to swell it is called lymphedema. This swelling can affect the arms and legs but it can affect any area of the body. The lymphedema can be studied under 2 categories, these are primary lymphedema and secondary lymphedema.

Primary Lymphedema

• It is caused by congenital malformation.
• It is further divided into 3 categories:

1. Congenital Lymphedema
   • It is seen in less than one year of age.
   • Lower limb, genitalia, and face are involved in this type.
   • It is usually bilateral and multiple limbs are involved.
   • The familial version of congenital lymphedema is called MILROY's disease.
2. Lymphedema Praecox
   • It is the most common type of primary lymphedema. It is found in more than 90% of cases.
   • The age group is from 1 to 35 years.
   • It is mostly seen in females.
   • It is usually unilateral and involves the calf and foot.
   • Its familial version is known as Meig’s disease.
3. Lymphedema Tarda
   • It is usually seen in age groups greater than 35 to 40 years of age.
   • It is a rare kind of primary lymphedema.

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Secondary Lymphedema

• It is the most common type of lymphedema.
• It occurs secondary to lymphatic obstruction or lymphatic disruption anywhere in the body.
• Worldwide the most common cause of lymphatic obstruction is filariasis.
• The Organism responsible is Wuchereria bancrofti.
• The most common cause of lymphatic disruption is lymph node dissection. The lymph nodes involved are axillary lymph node dissection or inguinal lymph node dissection.
• The most common cause of secondary lymphedema in the USA is axillary lymph node dissection.
• The other causes of lymphatic disruption are Trauma, Malignancy, and radiotherapy.

There is one more classification of lymphedema known as Brunner's clinical classification of lymphedema. It is divided into grades.

• Subclinical or latent
  • Excess interstitial fluid
  • Histological abnormality in lymphatic and lymph nodes.
  • No clinically apparent lymphedema
• Grade I- Pitting edema
• Grade II- Non-pitting edema
• Grade III- Edema with irreversible skin changes.

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Clinical Features Of Chronic Lymphedema

• Buffalo Hump- It is a sign used to describe swelling present over the dorsum of the foot.
• Square Toes- The toes appear square-shaped because of the prolonged confinement of the foot into footwear.
• Stemmer’s Sign- The skin over the dorsum of the foot cannot be pinched because of subcutaneous fibrosis.

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Complications of Chronic Lymphedema

• Limb Swelling- The patient will be in severe discomfort and pain.
• Increased Risk of Infection- The patient will be at increased risk of bacterial infection, fungal infection, and Cellulitis. 
• There will be skin thickening and discoloration due to the development of Cellulitis and lymphangitis.
• The patient will be at increased risk of malignancy if the patient has lymphedema for a long duration. 
• The lymphangiosarcoma can develop.

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Diagnosis of Lymphedema

• Diagnosis is mainly clinical.
• There should be proper history taking and an examination should be done in case of mild swelling and absence of complicating features.
• Investigation is required in the case of atypical presentation. If the patient presents with severe lymphedema with complications and multifactorial swellings then proper investigation should be done.
• The investigation performed is lymphangiography.

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Treatment of Lymphedema

• Conservative treatment is preferred because of good efficacy and decrease the risk of complications.
• Conservative treatment includes:
  • Compression stockings
  • Limb elevation and regular checkups
  • Antibiotics in infections like cellulitis.
  • Specialized physical therapy.
• Surgery
  • It is not commonly used because of poor efficacy and increased risk of complications.
  • There are some conditions where surgery is inevitable. The indications of surgery are:
    • Severe functional impairment
    • Recurrent lymphangitis
    • Severe pain
    • Lymphedema is resistant to medical therapy.
• These surgical options are reconstructive operation and excisional operation.

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