Lymphedema

Lymphedema, or swelling caused by lymph accumulation, can have a variety of causes, from genetic malformations of the lymph system to cancer, surgery, or infection.

• Lymphedema is swelling in one or more of the arms or legs that arises due to damage or poor function of the lymphatic system.
• The lymphatic system is a network of vessels that course throughout the body to collect excess fluid as well as waste products. The fluid is filtered at the lymph nodes, which are important in fighting infection and are a key part of the lymphatic system. Eventually, the excess fluid removed from the tissues is drained into the bloodstream.
• Lymphedema most commonly affects one of the extremities only, but in some cases, both arms or legs are affected.
• Lymphedema may be classified as primary lymphedema or secondary lymphedema.

Primary Lymphedema

• Lymphedema can occur due to a defect in the function of the lymphatic system, although this is not common. In this situation, the lymphedema is referred to as primary lymphedema.
• Depending upon when in life the signs and symptoms develop, primary lymphedema is termed congenital lymphedema (present from the time of birth), lymphedema praecox, or Meige disease.
• Milroy disease is one specific type of primary lymphedema that is inherited in a sex-linked genetic pattern.

Secondary Lymphedema

• Much more commonly, lymphedema occurs because of damage or destruction of a lymphatic system that was previously functioning normally (secondary lymphedema).
• The most common cause of lymphedema in the U.S. is breast cancer surgery, especially in combination with radiation therapy, which can cause lymphedema of the arm on the side of the body affected by cancer. '
• Other surgeries, such as vein stripping, peripheral vascular surgery, scar excisions, or any procedure that potentially damages lymph nodes and vessels can result in lymphedema.
• Worldwide, filariasis is the most common cause of lymphedema.
  • Filariasis is an infestation of lymph nodes by the parasite Wuchereria bancrofti, which is transmitted among humans by mosquitoes.
  • Filariasis is a significant public health problem affecting millions in tropical and subtropical regions of Asia, Africa, the Western Pacific, and parts of Central and South America. In people suffering from filariasis, the entire leg, arm, or genital area may swell to several times its normal size, causing long-term disability.
  • Other conditions characterized by damage to lymph nodes can also cause lymphedema, including infiltration of lymph nodes by cancer or damage due to trauma, burns, radiation, compression, or infection.

The swelling of lymphedema may be so slight as to barely be noticed, or the swelling may be severe and disfiguring. Pronounced swelling can be accompanied by fatigue when moving the involved extremity, as well as embarrassment.

Over the long-term, the excess fluid and proteins in the tissues cause chronic inflammation and scarring. The swelling is firm and does not retain an indentation (pit) when the skin is compressed by a finger (non-pitting edema). The skin in the involved area can become scaly or cracked, or may develop an orange-peel appearance (peau d'orange). Tenderness and soreness can accompany the swelling and skin changes. Loss of mobility may also occur.

Lymphedema also increases the susceptibility to infection in the affected area. Bacterial infections of the skin and of the subcutaneous tissues (the tissues underlying the skin) are the most common type of infections that occur in affected areas.

It is appropriate to seek medical care if you believe you have lymphedema.

The diagnosis of lymphedema is often obvious given the history of a surgical procedure or other condition that includes damage to lymph nodes. A careful physical examination and medical history will be necessary to rule out other conditions that can cause limb swelling, such as kidney or heart failure.

In some cases, specialized imaging tests may be ordered to confirm the diagnosis or obtain information about the cause of lymphedema. These may include:

• CT or MRI scans
• Doppler ultrasound scans, which can identify deep blood clots that may cause limb swelling
• Lymphoscintigraphy, which is a test that illustrates the flow of fluid in lymph vessels. A tracer dye is injected into lymph vessels prior to imaging studies.

Lymphedema is not curable; although there are treatments that can help minimize the symptoms and ease discomfort.

Your health care practitioner can advise you about the best ways to care for your affected limb(s). In general, it is important to practice good skin hygiene and avoid heavy lifting or strenuous activity with the affected limb.

• Tight clothing and jewelry should also be avoided.
• Take care to avoid sunburn, insect bites, cuts, abrasions, and dehydration, since all of these can worsen lymphedema.

Various surgical procedures have been carried out, but no operation is able to cure lymphedema. When surgery is performed, the goal of treatment is the removal of excess fluid and/or scar tissue.

Medical treatment consists primarily of compression therapies to help stimulate the flow of lymph in the affected area. These must be practiced in cooperation with your health care practitioner or lymphedema therapist to ensure that they are performed correctly.

There are different types of compression therapies available, ranging from compression garments and bandages to massage techniques and pneumatic compression devices.

• A general principle of compression therapies is that the pressure applied is greatest at the distal end (hand or foot) of the extremity and gradually decreases toward the center of the body.
• Your therapist may also recommend light exercises for the affected body part to help stimulate lymph drainage.

Medications are useful in the treatment of filariasis, which is the primary cause of lymphedema worldwide but rare in the U.S. Filariasis is treated with the drug diethylcarbamazine.

• Antibiotics may be necessary for the treatment of secondary infections of the skin and subcutaneous tissues (see below) that are a frequent complication of lymphedema.

Primary lymphedema cannot be prevented; it is possible to take steps to reduce your risk of developing secondary lymphedema if you are at risk.

• Avoid heavy lifting (including carrying heavy purses) with an affected arm.
• Drink plenty of fluids; dehydration can worsen lymphedema.
• Avoid environmental irritants in the affected area, such as insect bites or stings, and sunburn.
• Practice good skincare and hygiene.
• Don't wear tight clothing or jewelry on the affected limb. Even the use of blood pressure cuffs on an affected arm should be avoided.

As described above, lymphedema cannot be cured, although treatments are available to help manage the condition and lessen the severity of symptoms. Because the immune system is weakened in areas affected by lymphedema, bacterial infections often develop in the skin or in the tissues beneath the skin. These infections must be treated promptly to avoid spreading to other parts of the body.

A rare cancer of the lymphatic vessels known as lymphangiosarcoma can develop as a result of long-term lymphedema. Individuals who have had long-term lymphedema for 10 years or more have some risk of developing this cancer.

• Lymphangiosarcoma appears as a reddish or purplish lump on the skin and spreads rapidly.
• Treatment is amputation of the affected limb.