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Primary Lymphedema: Clinical Features and Management in 138 Pediatric Patients

Schook, Carolyn C. B.A.; Mulliken, John B. M.D.; Fishman, Steven J. M.D.; Grant, Frederick D. M.D.; Zurakowski, David Ph.D.; Greene, Arin K. M.D., M.M.Sc.

Plastic and Reconstructive Surgery: June 2011 - Volume 127 - Issue 6 - p 2419-2431
doi: 10.1097/PRS.0b013e318213a218
Pediatric/Craniofacial: Original Articles
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Background: Lymphedema results from maldevelopment of the lymphatic system (primary) or injury to lymphatic vasculature (secondary). Primary lymphedema is far less common than the secondary condition. The purpose of this study was to determine the clinical features of primary lymphedema in the pediatric age group.

Methods: The authors' Vascular Anomalies Center database was reviewed for patients evaluated between 1999 and 2010 with onset of lymphedema before 21 years of age. Cause, sex, age of onset, location, and familial/syndromic association were determined. Morbidity, progression, and treatment were analyzed.

Results: Lymphedema was confirmed in 142 children: 138 cases (97.2 percent) were primary and four (2.8 percent) were secondary. Analysis of the primary cohort showed that 58.7 percent of the patients were female. Age of onset was infancy, 49.2 percent; childhood, 9.5 percent; or adolescence, 41.3 percent. Boys most commonly presented in infancy (68.0 percent), whereas girls usually developed swelling in adolescence (55.3 percent). Lymphedema involved an extremity (81.9 percent), genitalia (4.3 percent), or both (13.8 percent). The lower limb was most commonly affected (91.7 percent), and 52.9 percent had bilateral lower extremity disease. Eleven percent of patients had familial or syndromic lymphedema. Cellulitis occurred in 18.8 percent of children; 13.0 percent required hospitalization. The majority of patients (57.9 percent) had progression of their disease. Treatment was compression garments alone (75.4 percent) or in combination with pneumatic compression (19.6 percent); 13.0 percent had operative intervention.

Conclusions: Pediatric primary lymphedema usually involves the lower extremities. Boys typically are affected at birth, and girls most often present during adolescence. Most patients do not have major morbidity, are successfully managed by compression, and do not require surgical treatment.

Boston, Mass.

From the Departments of Plastic and Oral Surgery, Surgery, and Radiology, Vascular Anomalies Center, Children's Hospital Boston, Harvard Medical School.

Received for publication October 12, 2010; accepted December 21, 2010.

Arin K. Greene, M.D., M.M.Sc., Department of Plastic and Oral Surgery, Vascular Anomalies Center, Children's Hospital Boston, 300 Longwood Avenue, Boston, Mass. 02115, arin.greene@childrens.harvard.edu

Disclosure: The authors have no financial interest to declare in relation to the content of this article.

©2011American Society of Plastic Surgeons