Lymphedema develops due to the abnormality of the transport capacity of the lymphatic system. Clinically lymphedema is not only a cosmetic deformity, but also a disabling and distressing condition.

Primary lymphedema is an inborn defect caused by such condition as absence or underdevelopment of the lymphatic system. It occurs in approximately 1 in 10,000 persons less than 20 years of age, with females being affected more frequently than males. Secondary lymphedema is an acquired condition resulting from loss or obstruction of the previously normal lymphatic system due to infection, tumor, filariasis and other miscellaneous conditions.

Clinical symptoms and signs depend on the duration and the severity of the lymphedema. In the early stage of disease, the edema is soft and pits easily with pressure (pitting edema) and may decrease or disappear with elevation of the limbs. In the advanced stage, the skin texture turns woody as the surrounding tissue becomes indurated and fibrotic.

Lymphangiography was introduced in the early 19th century. But there are several drawbacks to this procedure, including the complexity of the procedure, irradiation by contrast agent that may result in lymphangitis and potentially worsen the lymphedema. Lymphoscintigraphy is easier to perform than lymphangiography and is not reported to cause lymphangitis. Lymphoscintigraphy is gradually replacing lymphangiography.

The mainstay of the treatment is complex decongestive physical therapy including leg elevation, elastic or rigid compression, manual lymph drainage, and intermittent pneumatic compression. When the function of the limb is significantly impaired, surgical reduction is considered. The surgery is not curative, but it can make the disorder more manageable by complex decongestive physical therapy.