Lymphedema

From Wikipedia, the free encyclopedia

**Lymphedema**
*Classifications and external resources*
ICD-10	I89.0, Q82.0, I97.2
ICD-9	457.0, 457.1, 757.0

Lymphedema (AmE), also known as "Lymphoedema" (BrE), or "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. The danger with lymphedema comes from the constant risk of developing an uncontrolled infection in the affected limb. Still, physicians 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.

1 Causes
2 Symptoms
3 Staging and severity
4 Illustration
5 Treatment
6 Sources
7 External links

[edit] Causes

Lymphedema may be inherited (primary) or caused by injury to the lymphatic vessels (secondary). It is most frequently seen after lymph node dissection, surgery and/or radiation therapy, in which damage to the lymphatic system is caused during the treatment of cancer, most notably breast cancer. In many cancer patients this condition does not develop until months or even years after their therapy has concluded. Lymphedema may also be associated with accidents or certain diseases or problems that may inhibit the lymphatic system from functioning properly. In tropical areas of the world, a common cause of secondary Lymphedema is filariasis, a parasitic infection.

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, develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). Some cases of Lymphedema may be associated with other vascular abnormalities. In the lower extremity it will be unilateral or bilateral. If it is bilateral, one leg may be worse than the other.

Lymphedema affects both men and women. In women, it is most prevalent in the upper limbs after breast cancer surgery and lymph node dissection, occurring in the arm on the side of the body in which the surgery is performed. It may also occur in the lower limbs or groin after surgery for colon, ovarian or uterine cancer in which removal of lymph nodes is required. In men, lower-limb Primary Lymphedema is most common, occurring in one or both legs. Surgery and/or treatment for prostate, colon and testicular cancers may result in Secondary Lymphedema, particularly where lymph nodes have been removed or damaged.

The onset of Secondary Lymphedema in patients who have had cancer surgery has also been linked to aircraft flight (likely due to decreased cabin pressure). For breast cancer survivors, wearing a prescribed and properly-fitted low-compression sleeve and gauntlet may help decrease swelling during flight.

Some cases of lower-limb Lymphedema have been associated with the use of Tamoxifen, due to the blood clots and deep vein thrombosis (DVT) that can be caused by this medication.

[edit] 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. Because the lymphatic fluids are basically stagnant, toxins and pathogens can build up after an injury and overwhelm the local defense system without completely activating an immune response.

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).

[edit] Staging and severity

Whether Primary or Secondary, Lymphedema develops in a number of stages, from mild to severe:

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 "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 tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called "debulking."

Lymphedema can also be categorized by its severity (usually referenced to a healthy extremity):

Grade 1 (mild edema): Lymphedema involves the distal parts such as a forearm and hand or a lower leg and foot. The difference in circumference is less than 4 centimeters, and other tissue changes are not yet present.

Grade 2 (moderate edema): Lymphedema involves an entire limb or corresponding quadrant of the trunk. Difference in circumference is more than 4 but less than 6 centimeters. Tissue changes, such as pitting, are apparent. The patient may experience erysipelas.

Grade 3a (severe edema): Lymphedema is present in one limb and its associated trunk quadrant. The difference in circumference is greater than 6 centimeters. Significant skin alterations, such as cornification or keratosis, cysts and/or fistulae, are present. Additionally the patient may experience repeated attacks of erysipelas.

Grade 3b (massive edema): The same symptoms as Stage 3a except that two or more extremities are affected.

Grade 4 (gigantic edema): Also known as elephantiasis. In this stage of Lymphedema, the affected extremities are huge due to almost complete blockage of the lymph channels. Elephantiasis may also affect the head and face.

[edit] Illustration

Presented here is a case of unilateral hereditary Lymphedema which had been present for 25 years without treatment:

Comparison of normal and swollen limb

Size of swollen foot, toes underneath

Another view of lymphedemic foot

Foot and leg (held vertically)

Presented here is a case of combined Primary Lymphedema and Lipedema(Stage 3) before and after treatments: Below Photos courtesy of Amy's Lymphedema Story

Stage 3 Lymphedema front view before treatments

Stage 3 Lymphedema back view before treatments

Stage 3 Lymphedema front view after treatments, 65 pounds lost in 14 days

Stage 3 Lymphedema back view after treatments, 65 pounds lost in 14 days

[edit] Treatment

The treatment of choice for lymphedema is Complete Decongestive Therapy (CDT). CDT consists of manual lymphatic drainage (MLD), short stretch compression bandaging, therapeutic exercise, and skin care. MLD was pioneered by Dr. Emil Vodder in the 1930s for the treatment of chronic sinusitis and other immune disorders, and is now recognized as a primary tool in Lymphedema management. Therapists can today receive certification through special classes conducted by various organizations specializing in MLD.

The MLD component of treatment consists of gentle, rhythmic massaging of the skin to stimulate the lymph nodes to open and drain. The treatment is very comfortable and nonaggressive. A typical session will involve drainage of the neck, abdomen, trunk, and involved extremity and lasts approximately 40 to 60 minutes, depending on the severity and extent of the lymphedema.

Compression bandaging is the application of several layers of compression garments to the involved area(s), which includes one or more short-stretch bandages. Short-stretch bandages are preferred over long-stretch bandages (such as Ace(r) bandages), as the long-stretch bandages cannot produce the higher tension necessary to safely reduce Lymphedema. The bandages are in most cases comfortable for wear during sleep and rest, unlike Ace(r) wraps which can become very tight and cause cutoff of blood circulation without regular body movements. During activity, the short-stretch bandages provide increased resistance against the affected areas and, therefore, help to soften fluid-swollen areas as the muscles push out during exercise and daily activities.

Therapeutic exercise while compression bandaging is worn is used to help improve lymphatic flow. Also, some patients with chronic Lymphedema or large, swollen areas will have poor strength or range of movement. These patients benefit from exercise prescribed specifically for them by their therapist to help improve their function and comfort.

Skin care is an important component of CDT. People with Lymphedema or who have had lymph nodes removed are at a higher risk for infections of the affected areas. Teaching higher risk sufferers about the signs and symptoms of infections is key to early identification and treatment. Untreated infections can further damage an already impaired lymphatic system and lead to more severe Lymphedema and skin ulcers.

Initially, CDT involves frequent visits to a certified therapist with a doctor's prescription. Once the Lymphedema is reduced, increased patient participation is required for ongoing care, along with the use of compression garments to further reduce the swelling.

Compression pumps are often used in the treatment of Lymphedema, but special care needs to be taken to ensure that the involved trunk quadrant is properly treated with manual lymphatic drainage before the application of a compression pump. If adequate treatment of the trunk is not carried out, the edema may be pushed into the upper portion of the arm or leg, or into the genitals. If a patient's Lymphedema worsens during a course of compression pump therapy, reassessment for adequate trunk MLD is necessary. However, newer compression pump garments provide compression well into the trunk and/or abdomen and groin areas. Although very beneficial, many patients find it inconvenient or expensive to attend regular MLD treatment sessions. A 2002 Stanford University article suggests that patients who received MLD and compression pump therapy experienced greater overall limb reduction.

Even after MLD, the patient may only be bandaging up to the axilla or groin. Unless the patient, is monitored during post MLD self bandaging, reassessment for adequate trunk MLD may need to be re-evaluated as necessary.

A new adjunct treatment is being taught to therapists utilizing a special type of tape called Kinesio Taping. It is used to help soften edemas.

[edit] Sources

"Lymphology: An Emerging Field of U.S. Medicine" - Annual Conference 2006 Kansas City, October http://www.lymphology.org
Lymphedema Diagnosis & Treatment, LLTretbar, MD and BB Lee, MD: Springer Verlag 2006
National Lymphedema Network | "What is Lymphedema?"
Schingale, Franz-Josef, "Lymphoedema, Lipoedema, Diagnosis and Therapy: A Guide for Those Affected," 2003, Hannover: Schlütersche GmbH & Co. KG.