[Primary lymphedema in childhood]

Lymphedema results from impaired lymphatic transport with increased limb volume and is divided into primary and secondary forms. In children, primary lymphedema is the most frequent, with a sporadic, rarely familial form or associated with complex malformative or genetic disorders. Diagnosis of lymphedema is mainly clinical and lymphoscintigraphy is useful to assess the lymphatic function of both limbs precisely. The main differential diagnosis is overgrowth syndrome. Erysipelas (cellulitis) is the main complication, but psychological or functional discomfort may occur throughout the course of lymphedema. Lymphedema management is based on multilayer low-stretch bandage, skin care, and eventually manual lymph drainage. The objective of treatment is to reduce lymphedema volume and then stabilize it. Multilayer low-stretch bandage and elastic compression are the cornerstone of treatment. Parent's motivation, including self-management, is required to ensure the child's compliance and improve quality of life.