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Lymphedema pp 59–77Cite as

Primary Lymphedema

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Abstract

Primary lymphedema is idiopathic and affects approximately 1/100,000 people. Males most commonly present in infancy, and females usually develop the condition in adolescence. Primary lymphedema also may occur in adulthood. One or both lower extremities are typically affected. Although the disease usually is sporadic, it can be syndromic and inherited. The involved limb enlarges over time because of subcutaneous adipose deposition. Morbidity includes decreased self-esteem, recurrent infections, difficulty fitting clothing, and reduced function of the area. Definitive diagnosis is achieved using lymphoscintigraphy, which also can determine the severity of lymphatic dysfunction. Management includes protecting the limb from incidental trauma, exercise, and compression. Rarely, operative intervention is indicated to reduce morbidity.

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Authors and Affiliations

  1. Department of Plastic and Oral Surgery, Boston Children’s Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA, 02115, USA

    Arin K. Greene M.D., M.MSc.

Authors
  1. Arin K. Greene M.D., M.MSc.
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Correspondence to Arin K. Greene M.D., M.MSc. .

Editor information

Editors and Affiliations

  1. Department of Plastic and Oral Surgery, Boston Children’s Hospital, Boston, Massachusetts, USA

    Arin K. Greene

  2. Division of Plastic Surgery, Beth Israel Deaconess Medical Center, Brookline, Massachusetts, USA

    Sumner A. Slavin

  3. Plastic and Reconstructive Surgery, Skåne University Hospital, Malmö, Sweden

    Håkan Brorson

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© 2015 Springer International Publishing Switzerland

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Greene, A.K. (2015). Primary Lymphedema. In: Greene, A., Slavin, S., Brorson, H. (eds) Lymphedema. Springer, Cham. https://doi.org/10.1007/978-3-319-14493-1_7

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  • DOI: https://doi.org/10.1007/978-3-319-14493-1_7

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-14492-4

  • Online ISBN: 978-3-319-14493-1

  • eBook Packages: MedicineMedicine (R0)

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