• Accumulation of lymphatic fluid in the interstitial tissue causing swelling
  • Lymphedema can develop when lymphatic vessels are missing or impaired (primary) or when lymph vessels are damaged or lymph nodes are removed (secondary).
  • Most common in the lower limb(s) (80%) but also can occur in the arm(s), face, trunk, and external genitalia



  • Predominant sex: female > male
  • 13% of patients with breast cancer treated with surgery; 42% of those treated with surgery and radiation therapy; 25% after GYN cancer surgery
  • Milroy disease presents at birth; estimated to be between 1/6,000 and 1/300 live births
  • Meige disease develops during puberty.


  • 120 million people worldwide are affected with primary lymphatic filariasis.
  • 10 million people are affected by nonfilarial secondary lymphedema in the United States.

Etiology and Pathophysiology

Secondary lymphedema:

  • Postoperative: gradual failure of distal lymphatics, which have to “pump” lymph at a greater pressure through damaged proximal ducts
  • Risk is higher with postoperative radiation because radiation reduces regrowth of ducts due to fibrous scarring.
  • Trauma; recurrent infection; malignancy, including metastatic disease, and marked obesity
  • Lymphangiogenesis inhibition and tissue fibrosis promotion by T cells appear to promote lymphedema.
  • Developing countries: Most common cause is filariasis (Wuchereria bancrofti).


  • Milroy disease: autosomal dominant; diagnosed either at birth or the 1st year of life
  • Lymphedema praecox has onset between the ages of 1 and 35 years.
  • Lymphedema tarda occurs in those >35 years of age.
  • Genetics referral: primary and lymphedema tarda

Risk Factors

  • Filariasis: most common cause worldwide
  • Lymphadenectomy or radiation therapy for malignancy (mastectomy, melanoma)
  • Prior trauma, serious burns, infection of affected limb
  • Obesity (body mass index: >50 kg/m2)
  • Inflammatory disorders: arthritis, sarcoidosis, dermatitis

General Prevention

Healthy body weight; treatment of congestive heart failure, early recognition of infection and cancer, and venous insufficiency

Commonly Associated Conditions

Venous disease, morbid obesity, regional cancer, filarial disease (Africa and Asia)

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