Lymphedema

Alternative names
Lymphatic obstruction

Definition
Lymphatic obstruction involves blockage of the lymph vessels, which drain fluid from tissues throughout the body and allow immune cells to travel where they are needed.

Causes, incidence, and risk factors

There are many causes of lymphatic obstruction, which is also known as lymphedema (swelling of the lymph passages). These include certain infections (for example, chronic cellulitis or parasitic infections such as filariasis), trauma, tumors, certain surgeries, and radiation therapy. In addition, there are rare forms of congenital (present from birth) lymphedema, which probably result from abnormalities in the development of the lymphatic vessels.

In Western societies, one of the most common causes of lymphedema is mastectomy with axillary dissection (removal of the breast and underarm lymph tissue for breast cancer). This can cause lymphedema of the arm in 10-15% of patients. This occurs because the lymphatic drainage of the arm passes through the axilla (armpit), and tissue in the axilla has been removed during mastectomy.

Recurrent cellulitis, or infection of the skin, can damage lymphatic vessels and lead to lymphedema of the affected area. This is more commonly seen in obese patients.

Symptoms
Chronic swelling, usually of the arm or leg, is the clinical hallmark of lymphatic obstruction.

Signs and tests

Physical exam and history are most important for diagnosis. In some cases, lymphangiography may be performed. This involves injection of dye into lymphatic vessels followed by x-rays of the area. Lymphangiography requires surgical isolation of the lymph vessels to be injected and is therefore a difficult procedure.

Lymphoscintigraphy is an alternative technique in which a radioactive substance that concentrates in the lymphatic vessels is injected into the affected tissue and mapped using a gamma camera, which images the location of the radioactive tracer. Lymphoscintigraphy is less invasive and more easily performed and is often used as a substitute for lymphangiography.

MRI is being explored as a means to image lymphatic vessels.

Treatment

The mainstays of therapy for lymphedema include range of motion exercises, compression (usually with multi-layered bandages), and manual lymph drainage (MLD). A physical therapist trained in these techniques is the appropriate person to see for treatment. Manual lymph drainage is a light massage therapy technique that involves moving the skin in particular directions based on the structure of the lymphatic system. This helps encourage drainage of the fluid and waste through the appropriate channels.

Other important aspects of treatment include skin care to prevent injuries, infection, and skin breakdown, as well as light exercise and movement programs. The latter should be carefully designed by the physical therapist to encourage drainage without developing swelling from overexertion which could worsen lymphedema.

Compression may also be accomplished by wearing compression stockings on the affected area or using an intermittent pneumatic compression pumps. Your doctor and appropriately-trained physical therapist will decide which compression methods would be best.

Surgical therapy is used in some cases, but has met with limited success and requires significant experience and technical expertise. Also, the physical therapy methods described above will still be needed after surgery to reduce lymphedema.

Surgical options include removal of tissue containing abnormal lymphatics, liposuction, and less commonly, transplant of tissue from areas with normal lymphatic tissues to areas with abnormal lymphatic drainage. Rarely, bypass of abnormal lymphatic tissue is attempted, in some cases using vein grafts. Such procedures are frequently unsuccessful, and are usually performed only under experimental protocols.

Expectations (prognosis)
Lymphedema is a chronic disease that usually requires lifelong management. In some cases, lymphedema improves with time, but some degree of swelling usually persists for years after onset.

Complications
In addition to swelling of the affected limb, skin breakdown and formation of chronic wounds and ulcers are the primary complications of chronic lymphedema in severe cases. Thus, patients must be vigilant about skin care and hygiene. There is also a small risk of developing a lymph tissue-related cancer

Calling your health care provider
See your doctor if you have ongoing swelling of your arms, legs, or lymph nodes.

Prevention

Sentinel lymph node dissection is a method that some surgeons are using in order to prevent the development of lymphedema following surgery for breast cancer.

Johns Hopkins patient information

Last revised: December 3, 2012
by Levon Ter-Markosyan, D.M.D.

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