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Updated: Jun 1, 2026
A Murine Tail Lymphedema Model
Published on: February 10, 2021
Primary lymphedema: clinical features and management in 138 pediatric patients
Carolyn C Schook1, John B Mulliken, Steven J Fishman
1Boston, Mass. From the Departments of Plastic and Oral Surgery, Surgery, and Radiology, Vascular Anomalies Center, Children's Hospital Boston, Harvard Medical School.
Insights
Pediatric primary lymphedema, often affecting lower limbs, presents differently in boys and girls. Most children manage well with compression garments, rarely needing surgery.
Area of Science:
- Pediatric Medicine
- Genetics and Genomics
- Vascular Biology
Background:
- Lymphedema arises from lymphatic system maldevelopment (primary) or injury (secondary).
- Primary lymphedema is significantly rarer than secondary forms.
- Understanding pediatric primary lymphedema is crucial due to its distinct presentation.
Purpose of the Study:
- To investigate the clinical characteristics of primary lymphedema in pediatric patients.
- To identify patterns in onset, location, and associations of pediatric primary lymphedema.
- To analyze morbidity, progression, and treatment outcomes for this condition.
Main Methods:
- Retrospective review of a Vascular Anomalies Center database (1999-2010).
- Inclusion criteria: lymphedema onset before 21 years of age.
- Data collected: cause, sex, age of onset, location, familial/syndromic links, morbidity, progression, and treatment.
Main Results:
- 142 children diagnosed with lymphedema; 138 (97.2%) were primary.
- Lower extremities most commonly affected (81.9%), often bilaterally (52.9%).
- Boys presented in infancy (68.0%), girls in adolescence (55.3%); 11% had familial/syndromic associations. Cellulitis occurred in 18.8%.
Conclusions:
- Pediatric primary lymphedema predominantly affects lower extremities.
- Distinct age-of-onset patterns exist for boys (infancy) and girls (adolescence).
- Conservative management with compression is effective; surgery is infrequently required.
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.
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