Lymphedema

Lymphedema is the abnormal accumulation of lymph in the interstitium. It is a progressive and chronic condition caused by compromised lymphatic vessels or lymph nodes, resulting in edema and subcutaneous fibroadipose deposition. Primary lymphedema is rare and results from congenital abnormalities in lymphatic development; it is a characteristic clinical feature of Turner syndrome. Secondary lymphedema is more common than primary lymphedema and is caused by damage to the lymphatic system, e.g., due to tumors, surgery, infection, or radiation therapy. Lymphedema manifests with progressive limb swelling, skin changes, and recurrent skin and soft tissue infections. Diagnosis is primarily clinical; lymphoscintigraphy may be performed if there is diagnostic uncertainty. Management involves manual lymphatic drainage, compression, exercise, and skincare. Surgery may be considered if conservative management is unsuccessful.

Lymphedema is the abnormal accumulation of highly viscous lipid-rich and protein-rich lymph in the interstitium due to compromised lymphatic vessels or lymph nodes.

• Primary lymphedema: (rare): idiopathic, resulting from congenital abnormalities in lymphatic vessels, e.g., in Turner syndrome
• Secondary lymphedema: caused by damage to the lymphatic system ^[1][2]
  • Tumors
  • Surgery
  • Inflammation
  • Trauma
  • Radiation therapy
  • Infections, e.g.:
    • Recurrent erysipelas
    • Leprosy
    • Syphilis
    • Granuloma inguinale
    • Lymphatic filariasis
  • Chronic venous insufficiency ^[2]
  • BMI > 50 kg/m^{2 [3]}

Inguinal or axillary lymph node injury is the most significant risk factor for lymphedema. ^[1]

Typical features ^[1]

• Onset of secondary lymphedema: typically 12–18 months after damage to the lymphatic system ^[1]
• Painless unilateral or bilateral swelling of limbs (pitting in early stages and nonpitting in later stages)
  • Swelling of toes and dorsal feet with deep flexion creases
  • Stemmer sign: inability to lift and pinch thickened skin at the base of the second toe ^[4]
• Hyperkeratosis (brawny induration)
• Lymphorrhea: leaking of lymph through the skin
• Absence of cutaneous ulceration

Lymphedema involves the toes, unlike venous edema, which typically spares the toes.

Severe chronic lymphedema can result in elephantiasis.

Clinical features by stage ^[5][6]

• Stage 0 (latent stage): reduced capacity of lymphatic vessels, no swelling
• Stage I: reversible pitting edema
• Stage II: gradual fibrosis, irreversible pitting or nonpitting edema ^[1]
• Stage III: hardened fibrotic skin, nonpitting edema

• Lymphedema is primarily a clinical diagnosis. ^[2]
• Consider targeted studies to rule out differential diagnoses, e.g., duplex ultrasound to rule out chronic venous insufficiency. ^[2][5]
• Obtain lymphoscintigraphy if primary lymphedema is suspected or if there is diagnostic uncertainty. ^[7]
• MR lymphangiography may be used to evaluate late-stage lymphedema. ^[6]

• Vascular malformations
• Chronic venous insufficiency
• Heart failure
• Lipedema
• Myxedema
• Obesity
• Posttraumatic swelling
• See also “Common causes of peripheral edema.”

The differential diagnoses listed here are not exhaustive.

Treatment of the underlying cause and conservative management are indicated for all patients with lymphedema.

Conservative management ^[2][5]

The following exercises and techniques can be trialed individually or combined as part of complete decongestive therapy.

• Manual lymphatic drainage: a form of massage that is applied in the direction of the heart to increase the natural drainage of lymph
• Compression garments, e.g., multilayer compression stockings (applied by a specialist)
• Elevation of the involved limb
• Muscle pumping exercises, e.g., walking rather than taking escalators
• Sequential pneumatic compression ^[2][8]
• Skincare, e.g.:
  • Cleansing and use of emollients to prevent and identify infection
  • Treatment of lymph stasis-related skin and soft tissue infections

Multilayer compressive bandages for lymphedema should be applied by a trained professional (e.g., a physiotherapist or clinical lymphologist) as they can be harmful or ineffective when applied incorrectly. ^[5]

Surgery ^[5][6]

If conservative management is unsuccessful, refer for surgical evaluation.

• Removal of deposited fibroadipose tissue and fluid, e.g.:
  • Excision
  • Liposuction
• Lymphovenous bypass surgery
• Vascularized lymph node transfer