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- 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
- Predominant age: any age
- 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 lymphatic filariasis in 73 countries but no primary infections in United States.
- 10 million people are affected by nonfilarial secondary lymphedema in the United States.
Etiology and Pathophysiology
- 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
- Filariasis: most common cause worldwide
- Mastectomy, melanoma surgery
- Prior trauma, serious burns, infection of affected limb
- Lymphadenectomy or radiation therapy for malignancy
- Obesity (>50 body mass index)
- Inflammatory disorders: arthritis, sarcoidosis, dermatitis
Healthy body weight maintenance; treatment of congestive heart failure (CHF), early recognition of infection and cancer, and venous insufficiency
Commonly Associated Conditions
Venous disease, morbid obesity, regional cancer, filarial disease (Africa and Asia)