Lymphedema
Basics
Description
- 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
Epidemiology
Incidence
- 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.
Prevalence
- 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).
Genetics
- 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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Citation
Domino, Frank J., et al., editors. "Lymphedema." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema.
Lymphedema. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema. Accessed September 28, 2023.
Lymphedema. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema
Lymphedema [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 September 28]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema.
* Article titles in AMA citation format should be in sentence-case
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