Although Lymphedema has afflicted the population for centuries, little was understood about the disease. Only in the past 10 to 15 years have clinicians begun to seriously focus on it's treatment.
The lymphatic system, an offshoot of the circulatory system, develops embryologically. Fluids and protein, lipids, fat soluble vitamins, and immune cells circulate within lymphatic vessels and nodes. If lymphatic drainage is impaired, edema and protein collect in soft tissues providing a natural medium for infection.
Lymphodema is the swelling of body parts, most often an extremity, caused by the abnormal accumulation of lymph fluid.
Primary cases of Lymphedema occur from idiopathic or unknown causes. They may be present at birth (lymphedema congenital), occur later in life (lymphedema praecox) or develop after age 35 (lymphedema tarda). Some cases are familial as well as congenital.
Primary Lymphedema is more common in females and occurs more often in lower extremities.
Causes of Primary Lymphedema:
- Congenital or Hereditary Lymphedema
- Milroy's Disease
- Idiopathic Lymphedema
- Lymphedema Praecox
- Lymphedema Tarda
- Turner's Disease
- Park-Weber Syndrome
- Klipple-Trenaunay Syndrome
Secondary cases of Lymphodema is caused by injury, scarring or exclusion of the lymphatic vessels - usually as a result of previous radiation and/or surgery of the lymphatics. Occasionally, secondary Lymphedema is caused by trauma or chronic infection of the lymphatic system.
Causes of Secondary Lymphedema:
- Postsurgical Lymphedema from cancer surgery (breast, prostate, ovarian and uterine)
- DES Daughters
- Traumatic (accidents)
- RSD (Reflex Sympathetic Dystrophy)
- Postinflammatory Lymphedema
- Liver Disease
Lymphedema and Cancer
As cancer rates increase throughout the world, so does the incidence of Lymphedema.
Breast or abdominal surgery can result in secondary Lymphedema. Treatment, such as radiation and/or surgery commonly given for carcinoma of the breast, uterus, bladder, ovary, prostate or testicle, as well as other malignant melanomas, can produce secondary Lymphedema.
Surgical removal of a tumor and the adjacent lymph nodes and vessels can often block lymph fluid from flowing through the system. Radiation therapy is one of the leading causes of secondary Lymphedema. It can damage healthy lymph nodes and cause scar tissue to form, interrupting the normal flow of the lymphatic system.
Lymphedema is the chronic swelling or feeling of tightness in the arm or hand due to an accumulation of lymphatic fluid in the soft tissue of the arm. The condition occurs when lymph vessels, which normally carry excess fluid out of the limbs and back into central circulation, have had their flow interrupted. Axillary (underarm) lymph node removal is commonly performed on breast cancer patients to stage or treat their cancer. However, between 15% and 20% of breast cancer patients who undergo axillary lymph node removal develop lymphedema. According to the American Cancer Society, of the two million breast cancer survivors in the U.S., approximately 400,000 must cope with lymphedema on a daily basis.
Lymphedema of Foot and Leg also may occur. It may also be caused by chronic infection, usually involving the legs. Less commonly, lymphedema is primary (i.e., due to the genetic make-up). Children with primary lymphedema may be born with it (congenital lymphedema) or it may become evident in their teens (lymphedema praecox) or in adulthood (lymphedema tarda).
Chronic lymphedema may result in minor swelling and discomfort. Occasionally it leads to a grave disability and disfigurement. Lymphedema may be precipitated, or made worse, by a skin infection. Skin infections can be difficult to treat in someone with pre-existing lymphedema.