Primary lymphedema: Clinical features and management in 138 pediatric patients

Carolyn C. Schook, John B. Mulliken, Steven J. Fishman, Frederick D. Grant, David Zurakowski, Arin K. Greene

Research output: Contribution to journalArticle

65 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)2419-2431
Number of pages13
JournalPlastic and Reconstructive Surgery
Volume127
Issue number6
DOIs
StatePublished - Jun 2011

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Lymphedema
Pediatrics
Lower Extremity
Age of Onset
Morbidity
Lymphatic System
Clothing
Cellulitis
Genitalia
Blood Vessels
Disease Progression
Hospitalization
Cohort Studies
Therapeutics
Extremities
Age Groups
Parturition
Databases
Wounds and Injuries

ASJC Scopus subject areas

  • Surgery

Cite this

Schook, Carolyn C. ; Mulliken, John B. ; Fishman, Steven J. ; Grant, Frederick D. ; Zurakowski, David ; Greene, Arin K. / Primary lymphedema : Clinical features and management in 138 pediatric patients. In: Plastic and Reconstructive Surgery. 2011 ; Vol. 127, No. 6. pp. 2419-2431.
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Schook, CC, Mulliken, JB, Fishman, SJ, Grant, FD, Zurakowski, D & Greene, AK 2011, 'Primary lymphedema: Clinical features and management in 138 pediatric patients', Plastic and Reconstructive Surgery, vol. 127, no. 6, pp. 2419-2431. https://doi.org/10.1097/PRS.0b013e318213a218

Primary lymphedema : Clinical features and management in 138 pediatric patients. / Schook, Carolyn C.; Mulliken, John B.; Fishman, Steven J.; Grant, Frederick D.; Zurakowski, David; Greene, Arin K.

In: Plastic and Reconstructive Surgery, Vol. 127, No. 6, 06.2011, p. 2419-2431.

Research output: Contribution to journalArticle

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T2 - Clinical features and management in 138 pediatric patients

AU - Schook, Carolyn C.

AU - Mulliken, John B.

AU - Fishman, Steven J.

AU - Grant, Frederick D.

AU - Zurakowski, David

AU - Greene, Arin K.

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N2 - 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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