Complex Decongestive Therapy-based Management of Primary Lymphedema

Primary lymphedema is related to a developmental defects (malformation, dysplasia) of the lymphatic vessels or lymph nodes. Many studies have reported an association between several genes and primary lymphedema. Although no curative surgical or medical intervention is available for lymphedema, current standard for treatment of either primary or secondary lymphedema is two-phase complex decongestive therapy (CDT). Many studies have revealed the short-term and long-term benefits of CDT such as decreasing edema volume and improving quality of life (QOL) in patient with secondary lymphedema on limb or even head/neck area. The ultimate goals of treatment for patients with primary lymphedema are to improve not only physical impairments of affected limb or area but also QOL and functional disabilities. Especially, the treatment of children with primary lymphedema should be performed with a long-term plan, utilizing CDT. This article will explore the two-phase CDT approach according to involved area especially lower limb and discuss clinical experience on primary lymphedema treatment, comparing it with characteristics of secondary lymphedema.