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Lymphedema

Lymphedema (or "Lymphoedema", "lymphatic obstruction") is a condition of localized fluid retention caused by a compromised lymphatic system. The lymphatic system (often referred to as the body's "second" circulatory system) collects and filters the interstitial fluid of the body. Lymphedema has been barely recognized as being a serious health problem; however, this is slowly changing due to education and awareness. Still, doctors and medical staff who practice in fields where this disease is uncommon may fail to correctly diagnose the condition due to the apparent lack of information regarding this disease. more...

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Causes

Lymphedema may be inherited (primary) or caused by injury to the lymphatic vessels (secondary). It was most frequently seen after surgery and radiation therapy, which may cause unintended damage to the lymphatic drainage system. It was especially common after surgery or radiation therapy were used in combination to treat breast cancer. Lymphedema may also be associated with accidents or certain diseases or problems that may inhibit the lymphatic system from proper function. Many cancer patients find this condition may develop after their therapy has concluded. The symptoms of Primary Lymphedema may not become apparent for many years. In the lower extremity it will be unilateral or bilateral.

Lymphedema affects both men and women. In women, it was most prevalent in the upper limbs after breast cancer surgery and lymph node dissection, occuring in the arm on the side of the body in which the surgery is performed, and in the lower limbs or groin after hysterectomy surgery. In men, lower-limb Lymphedema is most common, occuring in one or both legs or occasionally in the groin.

Aircraft flight has been linked to the onset of Secondary Lymphedema in patients who have had post-cancer surgery (likely due to decreased cabin pressure). Some cases of lower-limb Lymphedema have been associated with the use of Tamoxifen, which can also cause blood clots and deep vein thrombosis (DVT).

Symptoms

When the lymphatic impairment becomes so great that the lymph fluid exceeds the lymphatic system's ability to transport it, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid causes tissue channels to increase in size and number, reducing the availability of oxygen. This interferes with wound healing and provides a rich culture medium for bacterial growth that can result in lymphangitis (infection).

Symptoms may include severe fatigue, a heavy swollen limb or localized fluid accumulation in other body areas, deformity ("elephantiasis"), discoloration of the skin overlying the Lymphedema, recurrent episodes of cellulitis, and in severe cases, skin ulcers and infections. Each case is different. In certain exceptionally-severe cases, prolonged, untreated Lymphedema can lead to a form of cancer known as Lymphangiosarcoma.

Lymphedema may also result in psychological distress. The normal, daily-living lifestyle can become severely limited.

Lymphedema should not be confused with edema arising from venous insufficiency, which is not Lymphedema. However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder which is treated the same way as Lymphedema (see Treatment below).

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Lymphedema
From Gale Encyclopedia of Medicine, 4/6/01 by J. Ricker Polsdorfer

Definition

Lymphedema is the swelling of tissues (edema), usually the feet and legs, due to lymphatic obstruction.

Description

Lymphatic fluid seeps out of the blood circulation into the tissues. It returns to the heart through separate channels called lymphatics, carrying waste products and germs. On its way to the heart, it passes through lymph nodes, where infecting germs (including some cancers) are attacked by the body's defense mechanisms.

If lymphatic channels are obstructed or inadequate, fluid backs up and causes edema. Tissue fluid can also return to the circulation through tissues, without using the lymphatics, but gravity hinders this flow. So lymphedema is usually confined to the feet and legs.

Causes & symptoms

There are several types of congenital abnormalities associated with other birth defects of the lymphatics, which cause this condition. One in 10,000 people have this type of lymphedema.

Lymphatics can be damaged or obstructed by many different agents. Repeated bouts of blood poisoning can scar the vessels. Surgery to remove cancerous lymph nodes or radiation therapy can damage them. Cancer itself, as it invades the lymph system, as well as several other infectious and inflammatory conditions, can result in blockage of lymph flow. The most common worldwide cause of lymphedema is a group of worms known as filaria. Filaria can be found in most of the developing regions of the world. They enter humans through insect bites, mostly mosquitoes, and take up residence in lymphatic channels, irritating them enough to scar them and impair their ability to carry lymph. Long-standing lymphatic filariasis can cause massive swelling of the legs, earning the name elephantiasis.

Diagnosis

Since other types of swelling may look similar to lymphedema, precise diagnostic tools must be used. Ultrasound, computed tomography scans (CT), and magnetic resonance imaging (MRI) scans may help with diagnosis. Lymphangiography may be needed to clarify the cause.

Treatment

Physical activity can pump some of the fluid out of the tissues. Compression stockings are of some value, as are devices that actively squeeze fluid out of tissues. Diuretics may alleviate some of the edema. Because the ability of the skin to defend itself is hampered by the swelling, infections are more common. It is therefore important to care for wounds and to treat infections early.

When caused by infection, lymphedema can be treated by eliminating the underlying infection with antibiotics.

Reconstructing lymphatic channels using microvascular surgery has recently achieved some success.

Prognosis

If congenital, lymphedema is a progressive and lifelong condition. If secondary or caused by an underlying disease or infection, lymphedema can be treated by treating the disease.

Prevention

When traveling in regions known to have filaria, avoidance of insect bites is crucial. Prompt and effective treatment of the infection will prevent the consequences.

Key Terms

Blood poisoning
Infection that has escaped local defenses and spread into the circulation.

Further Reading

For Your Information

    Books

  • Creager, Mark A. and Victor A. Dzau. "Vascular diseases of the extremities." In Harrison's Principles of Internal Medicine. Edited by Kurt Isselbacher, et al. New York: McGraw-Hill, 1998, pp.1405-1406.
  • Kontos, Hermes A. "Vascular diseases of the limbs." In Cecil Textbook of Medicine. Edited by J. Claude Bennett and Fred Plum. Philadelphia: W. B. Saunders, 1996, pp.357.
  • Nutman, Thomas B. and Peter F. Weller. "Filariasis and related infections." In Cecil Textbook of Medicine. Edited by J. Claude Bennett and Fred Plum. Philadelphia: W. B. Saunders, 1996.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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