Lymphedema is the build-up of fluid in soft body tissues when the lymph system is damaged or blocked. Lymphedema usually affects an arm or leg, but it can also affect other parts of the body. Lymphedema can cause long-term physical, psychological, and social problems for patients.
When the lymph system is working as it should, lymph flows through the body and is returned to the bloodstream. When part of the lymph system is damaged or blocked, fluid cannot drain from nearby body tissues. Fluid builds up in the tissues and causes swelling.
There are two types of lymphedema:
The two types of primary lymphedema are idiopathic (unknown cause) and hereditary. Hereditary lymphedema is caused by either an autosomal dominant or recessive inheritance. It can be associated with syndromes as well. The most common form of primary congenital lymphedema is lymphedema praecox, or Milroy’s Disease. It is a hereditary from of lymphedema with autosomal dominant inheritance. All patients with lymphedema should be evaluated by a genetic counselor.
Scientists are closer to establishing the genetic basis for these disorders. Some mutations in the vascular endothelial growth factor receptor (VEGFR3) have been found and others are being investigated.
Congenital lymphedema, which is the rarest form of primary lymphedema, appears early in life. It generally involves more than one limb, but rarely extends above the knee. Swelling enlarges at a slower rate than body growth. It becomes less pronounced with age, and only supportive therapy is required in two-thirds of cases. The remaining one-third of patients have a poor prognosis and frequently require surgical interventions.
Lymphedema praecox, which is the most common form of primary lymphedema, is seen mostly in females. It occurs in late childhood and adolescence, and swelling usually begins concurrently with a growth spurt. It extends to the groin and is sometimes associated with skin and nail changes. A significant increase in swelling may occur over time, resulting in limbs that are markedly enlarged.
Other conditions may cause the same symptoms as lymphedema. A doctor should be consulted if any of the following problems occur:
These symptoms may occur very slowly over time or more quickly if there is an infection or injury to the arm or leg. Daily activities and the ability to work or enjoy hobbies may be affected by lymphedema.
Lymphedema can occur after any cancer or treatment that affects the flow of lymph through the lymph nodes, such as removal of lymph nodes. It may develop within days or many years after treatment. Most lymphedema develops within three years of surgery.
Taking preventive steps may keep lymphedema from developing. The chance of improving the condition is better if treatment begins early. Untreated lymphedema can lead to problems that cannot be reversed. If lymphedema has developed, these steps may keep it from getting worse:
Damage to the lymph system cannot be repaired. The goal of treatment is to control the swelling, help patients continue with activities of daily living, decrease pain, and improve the ability to move and use the limb (arm or leg) with lymphedema. Drugs are not usually used for long-term treatment of lymphedema.
Treatment of lymphedema may include the following:
When lymphedema is severe and does not get better with treatment, other problems may be the cause.