Lymphovenous Canada: What are Lymphatic Disorders?

Lymphatic disorders can include: lymphedema, Klippel-Trenaunay (KT) syndrome, lymphangioma, hemangioma, and vascular malformations. Individuals may be born with these conditions, or develop them later in life. Lymphedema is present in about 70% of Turner Syndrome - a chromosomal disorder in which part or all of one of a woman's X chromosomes is missing. It is thought that the malfunction of the lymphatic system may be involved in such auto immune diseases such as arthritis and Crohn's disease.

Many individuals acquire lymphovenous disorders such as lymphedema as a result of the removal of lymph nodes during surgery or through damage to the nodes and lymphatic vessels following radiation used in the treatment of cancer. Other causes may include trauma to the limb or parasitic infiltration of the lymph nodes and vessels (such as lymphatic filariasis, common in tropical areas). In fact, it is estimated that as many as one quarter of world's population is affected by lymphatic filariasis.

3-5 million people are estimated to be effected by secondary lymphedema in the U.S.

There are few Canadian statistics which exist on the numbers of people with secondary lymphedema - however 3-5 million people are estimated to be affected with secondary lymphedema in the United States. In December 2000 the U.S. National Institutes of Health gave the following description and statistical breakdown of the incidence of lymphedema:

There are two major types of lymphedema: (the less common) primary (congenital)lymphedema and (most common) secondary lymphedema (caused by tissue injury, scarring, lymph node removal, or infection). The incidence of primary lymphedema has been estimated in the U.S. to be between 1/6000 to 1/300 live births. For primary lymphedema, there is an early onset form (Milroy's disease) which is relatively rare and presents at birth. A more common type (Meige's disease) develops during puberty, representing approximately 80 percent of all cases (of primary lymphedema). A third form, lymphedema tarda, occurs after the age of 35...

The largest percentage of this group are breast cancer survivors. The American Cancer Society (Understanding and Managing Lymphedema After Cancer Treatment, page 20, 2006) suggests that 1 in 5 of breast cancer survivors in the U.S. are at risk of getting lymphedema.

Obesity has been suggested as a major predictive factor for arm edema in breast cancer

Obesity has been suggested as a major predictive factor for arm edema in breast cancer. At the May 2, 2006 annual meeting of the American Society of Breast Disease (ASBD) Lucy K. Helyer, MD, surgical oncology fellow, Princess Margaret Hospital University Health Network in Canada reported on recent findings from their study which confirms earlier research in this area.

Dr. Helyer followed 137 women with breast cancer who underwent sentinel node biopsies. Close to 36% of patients who were obese developed lymphedema in contrast to about 16% of overweight patients, about 7% of normal weight patients and none in underweight patients.

Other studies have previously shown a link between obesity and lymphedema. These include: Arm edema in conservatively managed breast cancer: obesity is a major predictive factor, Therapeutic Radiology, 1991, Jul: 180(1):18; Obesity and cancer: the risks, science, and potential management strategies, Oncology 2005 Jun; 19(7): 871-81). Authors Say and Donegan (A biostatistical evaluation of complications from mastectomy. Surg. Gynecol, 1974) have suggested that individuals who are overweight often have prolonged operations and multiple transfusions, leading to arm edema, which they attributed to poorer vascularity of overweight patients. Other authors such as Haagensen (Diseases of the Breast, Philadelphia: Saunders, 1971) have suggested that infection is more difficult to avoid in patients who are obese.

In the Lymphlink Question Corner on obesity, the U.S. National Lymhedema Network notes that "it would be fair to extrapolate similar risks to leg LE (lymphedema) and excess weight too."

The 2007 publication Lymphedema available from the U.S. National Cancer Insitute (NCI) makes the following commments on lymphedema prevalence rates:

"...the reported incidence of lymphedema varies because of discrepancies in its definition and measurement and because of variations in time since treatment after which subjects are assessed for this complication. The incidence also varies according to treatment received and limb involved. With these caveats understood, there appears to be an overall incidence of arm edema after breast cancer therapy of 26%. A survey of 1,151 women treated with radiation for breast cancer reported lymphedema in 23% of subjects at 0 to 2 years posttreatment and 45% at 15 or more years posttreatment. Among breast cancer patients treated with surgery alone, prevalence increased from 20% at 0 to 2 years postsurgery to 30% at 15 or more years postsurgery. In a retrospective study, women who underwent radiation therapy following treatment for vulvar cancer with removal of lymph nodes were at higher risk (47%) for developing lower limb lymphedema (LLL) than were survivors of ovarian cancer (7%), uterine cancer (17.7%), or cervical cancer (17.5%). Of those with LLL, 70.8% had onset within 6 months of surgery.

Factors that contribute to the development of lymphedema are irradiation of the dissected nodal basin, postoperative wound complications and subsequent cellulitis of the limb, the extent of node dissection, and advanced age.

The following are risk factors for the development of lymphedema:
- Breast cancer, if patients have received radiation therapy or undergone node dissection. Radiation therapy to the axilla following axillary node dissection increases the incidence of lymphedema. A review of several studies reports lymphedema in approximately 41% (range, 21%-51%) of patients who underwent axillary radiation and surgery, compared with 17% (range, 6%-39%) of those receiving axillary surgery without radiation.[7] The extent of axillary dissection also increases the risk of lymphedema.
- Nodal dissection of axillary, inguinal, or pelvic regions.
- Radiation therapy of axillary, inguinal, or pelvic regions or supraclavicular (mantle field) radiation.
- Scarring of the left or right subclavian lymphatic ducts and veins by either surgery or radiation."

In 1997 the National Cancer Institute (NCI) in the U.S. reported that other cancer survivors at risk for lymphedema also included those who have undergone surgery and/or radiation treatment for malignant melanoma of the upper or lower extremities, prostate cancer, gynecologic cancers, ovarian, testicular/prostate cancers, colorectal, pancreatic, or liver cancers.

World wide as many as 120 million people are affected with lymphatic filariasis

World wide as many as 120 million people are affected with lymphatic filariasis according to the World Health Organization (WHO), with over 40 million people seriously incapacitated and disfigured by the disease. The extreme form of this disease is referred to as "Elephantiasis".

WHO reports that,"one-third of the people infected with the disease live in India, one third are in Africa and most of the remainder are in South Asia, the Pacific and the Americas. In tropical and subtropical areas where lymphatic filariasis is well-established, the prevalence of infection is continuing to increase. A primary cause of this increase is the rapid and unplanned growth of cities, which creates numerous breeding sites for the mosquitoes that transmit the disease...

In its most obvious manifestations, lymphatic filariasis causes enlargement of the entire leg or arm, the genitals, vulva and breasts. In endemic communities, 10-50% of men and up to 10% of women can be affected. The psychological and social stigma associated with these aspects of the disease are immense. In addition, even more common than the overt abnormalities is hidden, internal damage to the kidneys and lymphatic system caused by the filariae."

No cure currently exists, however a range of options are available to keep the swelling under control

While no cure currently exists, there are a range of options available which can keep the swelling associated with lymphedema under control, thus reducing the possibility of infection and secondary skin change. See Compression page for more information in this area. These options can also help to maintain the motion and function of the affected limbs. (Note: after these treatments are used, limbs/affected areas of the body must be wrapped either in low stretch bandages or compression garments to maintain the reduction.)

• Extremity pumps - through the use of air pressure chambers in a garment placed around the affected limb, lymph is moved out of the affected area to areas closer to the larger lymph channels. Some treatment experts have suggested that the pressure in pumps should not exceed 30-60 pounds of pressure. Click here for information and research on the use of pneumatic extremity pumps in the treatment of lymphedema.
• Manual Lymph Drainage (MLD), Complex physical therapy (CPT), Complete Decongestive Therapy (CDT) - types of lymphatic massage performed by specially trained therapists, used to encourage the excess fluid in congested, swollen areas of the body to move to areas where it can drain away normally.
• Wrapping with low-stretch bandages - generally carried out to help obtain the lowest level of swelling ("dry limb") so that compression garments can be fitted. Many individuals frequently wear bandages during the times they are not wearing compression garments (such as sleeping) to keep their swelling under control or use them when their limbs are too large to fit into compression garments.
• CircAid, MedAssist, Reid Sleeves, foam inserts, etc.these products control swelling through adjustable straps and focussed pressure. Visit the Suppliers page for more information in this area.
• Compression garments - support stockings and sleeves used to contain swelling after treatment with extremity pumps or massage.

OTHER TYPES OF TREATMENT:

• The maintenance of a healthy weight and diet - a balanced diet with low sodium intake.
• Exercise - recent studies with suggest that exercise can be helpful to persons with lymphedema.
• Meticulous and regular skin hygiene and nail care
• Alcohol injections - to certain veins and laser treatments to port wine stains may be appropriate for K-T disorders.
• Drugs - when infections take place, antibiotics must be taken immediately - in chronic cases of repeated infections antibiotics may be prescribed for long periods. Although the use of benzopyrones have not been proven effective (liver damage and death have been associated with this drug - see Health Watch), research is currently underway to determine if there are other drugs which might effective in controlling lymphedema(see:Studies on potential drug use on lymphedema.
• Surgery - does not "cure" lymphedema and is not used except in exceptional situations where more conservative treatments do not work. Notes the American Cancer Society in its 2006 book Understanding and Managing Lymphedema after Cancer Treatment (see the Library section of the Lymphovenous Canada web site for more information on this publication):
..."Surgery involves potential risk, complications, and cost, and may further damage the lymph system or exacerbate lymphedema... Surgery may cause complications such as infection, may leave swollen areas, causes scarring, and necessitates wearing of compression complications afterward (pgs. 98-100)." Types of surgery include: debulking procedures and liposuction, connecting blood vessels to lymph vessels or nodes, transplanting lymph notes, and microsurgery.
• Diuretics - not generally used in the treatment of lymphedema.

With all lymphatic disorders it is important to avoid the use of needles in the affected area, unless there is no other choice.

There are a number of other disorders which may look similar to some lymphovenous disorders such as lymphedema but are not the same. These include a condition known as "lipodema" which is an abnormal accumulation of fat in the hips and legs to the ankles (see the Research & Advisories section for more information on this topic), and swelling which can result from blood clots.

An examination by your doctor and/or specialists, along with a number of diagnostic tests, can help to verify your condition. It is recommended that you determine what your condition might be before embarking on any treatment program.