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Overview :

Lymphedema involves blockage of the lymph vessels, with a resulting accumulation of lymphatic fluid in the interstitial tissues of the body. The lymphatic system consists of lymph vessels and lymph nodes throughout the body. The lymph vessels collect lymphatic fluid, which consists of protein, water, fats, and wastes from cells. The lymph vessels transport the fluid to the lymph nodes, where waste materials and foreign materials are filtered out from the fluid. The fluid is then returned to the blood. When the vessels are damaged or missing, the lymph fluid cannot move freely throughout the system but accumulates. This accumulation of fluid results in abnormal swelling of the arm(s) or leg(s), and occasionally swelling in other parts of the body.

Lymphedema is a very serious condition. There is no cure for lymphedema and once it develops, it can be a long-term, uncomfortable, and sometimes painful condition requiring daily treatment. When lymphedema is not treated, the protein-rich fluid continues to accumulate, leading to even more swelling and hardening (referred to as fibrosis) of the tissues. This fluid is a good culture medium for bacteria, thus resulting in reoccurring infections when there are injuries to the skin, decrease or loss of functioning of the affected limbs, and skin breakdown. Infections, referred to lymphangitis, can affect the connective tissue under the skin. Repeated infections may result in scarring, which in turn makes the tissue susceptible to more swelling and infection. Over time, these infections result in tissue hardening (i.e., fibrosis), which is a characteristic of advanced chronic lymphedema. In very severe cases, untreated lymphedema may even result in a rare form of lymphatic cancer called lymphangiosarcoma.

Lymphedema affects approximately 100 million people worldwide, including at least 3 million people in the United States.

Symptoms of lymphedema include:

  • swelling of an affected limb, which may develop gradually or suddenly
  • tightness of the skin and a feeling of heaviness in the affected area
  • discomfort or a feeling of "pins and needles" in the affected area
  • pitting edema, which can be identified by observing a temporary indentation in the swollen area when pressure is placed on the affected area
  • aching in the adjacent shoulder or hip due to the increasing weight of the swelling limb
  • tight fitting of a ring, wristwatch, or bracelet, without a gain in weight.

In 90% of the cases, lymphedema is diagnosed through observations, measurements, and symptoms. The remaining 10% require the use of more complex diagnostic tests such as lymphoscintigraphy. Lymphoscintigraphy is a technique in which a radioactive substance that concentrates in the lymphatic vessels is injected into the affected tissue and is mapped using a gamma camera, which images the location of the radioactive tracer. Magnetic resonance imaging (MRI), computed tomography (CT) scanning, and duplex ultrasound are imaging techniques that are also sometimes used as diagnostic tools for lymphedema.

There are three stages of lymphedema:

  • Stage 1 (spontaneously reversible)'tissue is still at the pitting stage and soft to the touch. Upon waking in the morning, the limbs or affected areas are of normal or almost normal size
  • Stage 2 (spontaneously irreversible)'tissue is non-pitting and no longer soft to the touch, fibrosis begins to form, and the limbs increase in size
  • Stage 3 (lymphostatic elephantiasis)'swelling is irreversible and the affected areas are very swollen. The skin hardens and begins to break down, fibrosis is more extensive, and patients may need surgery to remove some of the swollen tissues.

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