Diagnosis and therapy in children with lymphoedema

Lymphoedema (LE) is a disorder characterized by persistent swelling caused by impaired lymphatic drainage because of various aetiologies, including lymphatic injury and congenital functional or anatomical defects.

Literature review and expert opinion about diagnosis and treatment of LE in children.

LE is rare in children, with a prevalence of about 1.15/100,000 persons, 20 years old. The management of LE in children differs considerably from adults in terms of origin, co-morbidity and therapeutic approach. The objective of this presentation is to discuss practical issues related to clinically relevant information on the diagnosis, aetiology, work-up and treatment of LE in children. In contrast to adults, who usually experience secondary LE because of acquired lymphatic failure, most cases in children have a primary origin. The diagnosis can be made mainly on the basis of careful personal and family history, and physical examination. LE in children can be part of a syndrome if there are other concomitant phenotypic abnormalities and if a genetic defect is recognizable. Treatment of LE is mostly conservative utilizing decongestive LE therapy including compression therapy, directed exercises, massage and skincare. In the neonate, initial observation alone may be sufficient, as delayed lymphatic development and maturation can result in spontaneous improvement. The role of parents is crucial in providing the necessary input.

We present a review emphasizing a practical approach to treating a child with LE according to current publications and our own experience.