Primary lymphedema of childhood: Treatment results from a tertiary center

Abstract

Background: Primary lymphedema is the most common form of lymphedema presenting in the pediatric age group. Childhood lymphedema is caused by hereditary or congenital malformations in the lymphatic system that can manifest at birth or during childhood or adolescence.

Objectives: Complex decongestive therapy (CDT) is the cornerstone of conservative management of lymphedema in both adult and pediatric lymphedema patients, although pediatric treatment guidelines are still lacking. In this study we aimed to assess the effects of CDT on pediatric patients.

Methods: Childhood lymphedema patients who presented to the lymphedema rehabilitation unit of our university hospital before the age of 18 and who were treated for lymphedema with CDT were included in the study. Data on patient demographics, disease characteristics, and treatment duration were recorded. Limb volumes were calculated from patient measurements using a spreadsheet software (Limb Volume Calculator) that utilized the geometric formula for volume of a truncated cone. Measurements were taken before treatment and also weekly after initiation of treatment. Percent excess volume (PEV) was used instead of absolute volume difference to define the severity of lymphedema.

Results: A total of 34 limbs from 24 patients were included in the study. The mean age of the patients was 10.1 ± 4.9 years and 14 (58.3 %) were female. Most patients had one affected limb but 16 had bilateral lower-extremity lymphedema. The mean duration of treatment with CDT was 153.6 ± 155.8 days. Excess volume percentage change between pre-treatment PEV (602.8 ± 713.8) and post-treatment PEV (514.6 ± 699.1) was found to be statistically significant (p < 0.05).

Conclusion: Pediatric lymphedema management is a difficult and less well studied area in lymphedema rehabilitation. Our data support the use of CDT, which is a safe and effective treatment method, for pediatric lymphedema patients.

Keywords: Complex decongestive therapy; Lymphedema; Pediatric lymphedema; Rehabilitation.