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Lymphedema

Lymphedema is a topic covered in the 5-Minute Clinical Consult.

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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
  • 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.

Prevalence
  • 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

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
  • 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

General Prevention

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)

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Citation

Stephens, Mark B., et al., editors. "Lymphedema." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema.
Lymphedema. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema. Accessed April 21, 2019.
Lymphedema. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema
Lymphedema [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 21]. 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
TY - ELEC T1 - Lymphedema ID - 116967 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116967/all/Lymphedema PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -