The swollen leg and primary lymphoedema.

Children who present with unilateral or bilateral swelling of the legs are often suspected of having a deep venous thrombosis. The incidence of deep venous thrombosis in children is low and lymphoedema may be a more appropriate diagnosis. Lymphoedema can be primary or secondary. In childhood, primary lymphoedema is more common and may be seen associated with other congenital abnormalities, such as cardiac anomalies or gonadal dysgenesis. Primary hypoplastic lymphoedema is the most often encountered type. It is more common in girls, especially around puberty, and is typically painless. Atypical presentations produce diagnostic confusion and may require imaging to confirm the presence, extent, and precise anatomical nature of the lymphatic dysplasia. This article describes four patients presenting with limb pain and reviews the clinical features and imaging options in children with suspected lymphoedema.