PEDIATRICS Vol. 76 No. 2 August 1985, pp. 206-218
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Primary Lymphedema in Children and Adolescents: A Follow-up Study and Review

David M. Smeltzer MD1, Gunnar B. Stickler MD1, and Alexander Schirger MD1

1 From the Mayo Medical School and Department of Pediatrics and Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota

Primary lymphedema, a disorder causing persistent swelling in an extremity, is rare in children and adolescents; it affects 1.15/100,000 persons less than age 20 years. It primarily affects girls near menarche. The records of 125 children and adolescents, aged 0 to 20 years, who were examined at the Mayo Clinic were analyzed; 99 of these patients were contacted to obtain follow-up data. The influences of estrogen and inflammation are thought to be important etiologic factors in primary lymphedema. The diagnosis can be made on the basis of a thorough history and physical examination. Lymphangiograms, venograms, and biopsies add nothing to the diagnosis because of the low incidence of tumor in children and adolescents. Conservative treatment is recommended: a Jobst-type stocking, elevation, and proper foot care. Diuretics are not recommended. Careful psychologic counseling, especially in adolescents, is highly recommended.

Key Words: primary lymphedema • lymphedema praecox • congenital lymphedema

Submitted on May 4, 1984
Accepted on October 27, 1984


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