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Lymphoedema

Lymphoedema, also referred to as lymphoedema or lymphatic oedema, is a chronic condition characterised by localised swelling due to a compromised lymphatic system. This system plays a very important role in the body's immune function and in returning interstitial fluid to the bloodstream.

Lymphoedema is often a complication of cancer treatments or parasitic infections, and can also arise from genetic disorders. It is incurable and progressive, but various treatments can manage and alleviate symptoms, improving patient outcomes.

Signs and Symptoms

The hallmark of lymphoedema is soft tissue swelling (oedema), typically affecting the extremities. Initially, the swelling may be mild and reversible with elevation. As the condition progresses, the swelling becomes more pronounced and persistent. Advanced lymphoedema can lead to skin changes such as discolouration, verrucous (wart-like) hyperplasia, hyperkeratosis, papillomatosis, dermal thickening, and ulceration. Patients are also at increased risk of skin infections, including erysipelas.

Lymphoedema seen on CT scan
Lymphoedema seen on CT scan

Complications

When the lymphatic system's transport capacity is exceeded, protein-rich fluid accumulates in tissues, causing enlargement of tissue channels and reducing oxygen availability. This can lead to chronic infections, lymphangitis, lymphadenitis, and skin ulcers. In rare cases, prolonged lymphoedema may result in lymphangiosarcoma, a form of cancer known as Stewart–Treves syndrome.

Causes and Risk Factors

Lymphoedema can be classified as primary or secondary. Primary lymphoedema is often hereditary and associated with syndromes like Turner syndrome and Milroy's disease. Secondary lymphoedema is commonly due to lymph node damage from cancer treatments, particularly breast cancer. Other causes include surgery, radiation therapy, infections like cellulitis, and certain medications such as tamoxifen.

Lower extremity lymphoedema
Lower extremity lymphoedema

Diagnosis

Diagnosis is typically based on clinical signs and symptoms, supported by imaging studies when necessary. Swelling is assessed through limb measurements, visual inspection, and palpation. Imaging techniques like lymphoscintigraphy and indocyanine green lymphography can be used in complex cases or when surgical intervention is considered.

Severity of upper extremity lymphoedema in different stages
Severity of upper extremity lymphoedema in different stages
Severity of lower extremity lymphoedema in different stages
Severity of lower extremity lymphoedema in different stages

Treatment

Although there is no cure for lymphoedema, treatment can improve the condition significantly. The mainstay of lymphoedema management includes:

  1. Compression Therapy: This involves the use of compression garments and bandages to manage swelling and maintain limb shape. Garments are usually worn during the day and can be custom-fitted by professionals.
Lymphoedema compression sleeve on a mannequin
Lymphoedema compression sleeve on a mannequin
  1. Manual Lymphatic Drainage (MLD): This specialised massage technique helps promote lymph fluid movement and reduce swelling.
  2. Exercise: Regular physical activity, especially resistance training, helps maintain lymphatic flow and reduces swelling.
  3. Intermittent Pneumatic Compression (IPC): This therapy uses a pneumatic sleeve to enhance lymphatic drainage, often used in conjunction with other treatments like MLD.
  4. Good Skin Care: Maintaining skin integrity is very important to prevent infections, which can exacerbate lymphoedema.

Surgical Options

In severe cases, surgical interventions like Suction Assisted Lipectomy (SAL) and Vascularized Lymph Node Transfers (VLNT) may be considered. These procedures aim to reduce limb volume and improve lymphatic function.

Emerging Therapies

Low-level laser therapy (LLLT) has shown promise in reducing arm volume in post-mastectomy lymphoedema. New pharmacological approaches, such as the drug QBX258, are under investigation for their potential benefits in managing lymphoedema.

Epidemiology

Lymphoedema affects approximately 200 million people globally, underscoring the need for effective management strategies to improve patient quality of life.


Self-assessment MCQs (single best answer)

What is the primary function of the lymphatic system?



Which of the following is a common cause of secondary lymphoedema?



What is a hallmark symptom of lymphoedema?



Which imaging technique is often used in the diagnosis of complex lymphoedema cases?



Which therapy involves the use of a pneumatic sleeve to enhance lymphatic drainage?



Which of the following is NOT a skin change associated with advanced lymphoedema?



What is the potential serious complication of prolonged lymphoedema?



Which of the following is a hereditary condition associated with primary lymphoedema?



What is the role of compression garments in lymphoedema management?



Which emerging therapy has shown promise in reducing arm volume in post-mastectomy lymphoedema?



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