Lymphedema is a condition that arises from the accumulation of lymphatic fluid due to an obstruction, absence or dysfunction of the lymphatic system.
Causes
Lymphedema may be inherited (primary) or caused by injury to the lymphatic vessels (secondary).
While the exact cause of primary lymphedema is still unknown, it generally occurs due to poorly-developed or missing lymph nodes and/or channels in the body. Lymphedema may be present at birth (congenital), develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). Lower-limb primary lymphedema is most common, occurring in one or both legs. Some cases of lymphedema may be associated with other vascular abnormalities.
Secondary lymphedema affects both men and women. In women, it is most prevalent in the upper limbs after breast surgery and lymph nodes dissection, occurring in the arm on the side of the body in which the surgery is performed. Head and neck lymphedema can be caused by surgery or radiation therapy for tongue or throat cancer. It may also occur in the lower limbs or groin after surgery for colon, ovarian or uterine cancer in which removal of lymph nodes or radiation therapy is required. Surgery and/or treatment for prostate, colon and testicular cancers may result in secondary lymphedema, particularly when lymph nodes have been removed or damaged.
Stages
The most common method of staging was defined by the Fifth WHO Expert Committee on Filariasis:
- Stage 0 (latent): The lymphatic vessels have sustained some damage which is not yet apparent. Transport capacity is still sufficient for the amount of lymph being removed. Lymphedema is not present.
- Stage 1 (spontaneously reversible): Tissue is still at the "pitting" stage: when pressed by the fingertips, the affected area indents and holds the indentation. Usually upon waking in the morning, the limb or affected area is normal or almost normal in size.
- Stage 2 (spontaneously irreversible): The tissue now has a spongy consistency and is considered "non-pitting:" when pressed by the fingertips, the tissue bounces back without any indentation. Fibrosis found in Stage 2 Lymphedema marks the beginning of the hardening of the limbs and increasing size.
- Stage 3 (lymphostatic elephantiasis): At this stage, the swelling is irreversible and usually the limb(s) or affected area is very large. The swelling and tissue fibrosis cause the skin to thicken and lose its normal elasticity. These changes create tissue folds that limit mobility, are disfiguring, and encourage fungal infections and open wounds that are difficult to heal.
Diagnosis
Assessment of the lower extremities begins with a visual inspection of the legs. Color, presence of hair, visible veins, size of the legs and any sores or ulcerations should be noted. Stemmer's sign: a thickened skin fold at the base of the second toe or second finger that is a diagnostic sign for lymphedema. A positive result occurs when this tissue cannot be lifted but can only be grasped as a lump of tissue. In a negative result, it is possible to lift the tissue normally.
Lymphoscintigraphy is a simple and noninvasive functional test for the evaluation of the lymphatic system. Is the radionuclide technique of imaging the lymphatic system using interstitially injected radiopharmaceutical particles, was first introduced in 1953 and is now the gold standard for assessing the lymphatics.
Treatment
Treatment for lymphedema varies depending on the severity of the edema and the degree of fibrosis of the affected limb. Most people with lymphedema follow a daily regimen of treatment as suggested by their physician or certified lymphedema therapist. The most common treatments for lymphedema are a combination of the use of manual lymphatic drainage, compression bandaging and garment and intermittent sequential gradient pumps. Complex decongestive physiotherapy is a system of manual lymphatic drainage massage, application of multi-layer compression bandages, skin care and education.
