What is lymphedema?

Accumulation of protein-rich fluid in the interstitial space due to lymphatic dysfunction.

image    How common is lymphedema?

Approximately 150 million people are affected worldwide.

image    What structures are most commonly involved?

The extremities, followed by the genitalia.

image    Does lymphedema affect the superficial or deep areas of the extremity?

Only the subcutaneous tissues are involved.

image    What is the natural history of lymphedema?

Adipose and fibrous tissues are produced which cause enlargement of the limb.

image    How is lymphedema classified?

Primary or secondary.


Types of lymphedema. (Left) A 24-year-old female developed primary lymphedema of the lower extremity during adolescence. (Right) A 70-year-old female with upper extremity lymphedema following mastectomy, axillary lymphadenectomy, and radiation for breast cancer.

image    What is primary lymphedema?

Lymphedema that results from a congenital malformation of the lymphatic system.

image    How common is primary lymphedema?

Rare. The prevalence is approximately 1:100,000.

image    When does primary lymphedema present?

Males usually present in infancy, females typically develop swelling in adolescence.

image    What structures are most commonly affected by primary lymphedema?

Lower extremities (90%), genitalia (16%), and upper extremities (10%).

image    Is there a genetic cause of primary lymphedema?

Yes, mutations in VEGFR3, FOXC2, SOX18, and CCBE1 cause some familial/syndromic forms of lymphedema.

image    What is Milroy disease?

Lymphedema of one or both lower extremities present at birth, with either a family history of disease or a mutation in VEGFR3.

image    What is Meige disease?

Lymphedema of one or both lower extremities presenting in adolescence with a family history of the disease; a genetic mutation has not been identified.

image    What is secondary lymphedema?

Lymphedema that results from injury to the lymphatic system.

image    What is the most common cause of lymphedema worldwide?

Parasitic infection (filariasis), causing lower extremity lymphedema.

image    What is the most common cause of lymphedema in developed countries?

Upper extremity lymphedema secondary to axillary lymphadenectomy and radiation treatment for breast cancer.

image    What percentage of women with breast cancer who have axillary lymphadenectomy and radiation develop lymphedema?

Approximately 33%.

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Aug 28, 2016 | Posted by in Reconstructive surgery | Comments Off on Lymphedema
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