Although most common in infants, lymphedema may occur or reoccur at any age, and may be associated with the initiation of therapy with growth hormone or estrogen.
Lymphedema is present in about 70% of Turner Syndrome patients and the hands and feet are most commonly affected . It is the result of the underdevelopment of the lymphatic system before birth [1,p.14]. As the child matures, this swelling is managed using with the same treatment methods that are used to control other types of lymphedema.
What is Lymphedema?
- An abnormal swelling due to the presence of excess lymphatic fluid within the tissues. This swelling occurs when the lymphatic system malfunctions or is damaged and lymphatic fluid cannot drain as quickly as produced.
- Affects an estimate 100 million men, women, and children around the world including at least 3 million Americans.
- Occurs most commonly in the extremities (arms, or legs); however, it can also affect the trunk, breast, abdomen, neck, head, and or genitals.
- The fluid that causes the swelling is protein-rich and this makes the tissue easily susceptible to infections.
- A chronic condition that can be treated but not cured. Early treatments can usually effectively control the condition.
- Without treatment, symptoms become progressively more serious.
- The onset can be sudden, gradual, or delayed so that it appears years after the causative event.
The two principle types are PRIMARY lymphedema and SECONDARY lymphedema.
Primary Lymphedema is a hereditary abnormality of the lymphatic system that occurs in approximately one in six thousand people. Although the cause is not well understood, these abnormalities often include hypoplasia or hyperplasia. The swelling of primary lymphedema usually starts distally (at the far end of the limb) and spreads proximally (upward toward the body).
Primary congenital lymphedema is also known as Nonne- Milroy’s disease or Milroy’s disease it is either present at birth or develops within the first two years of life.
- Lymphedema praecox, also known as Meige disease, is primary lymphedema in which the symptoms begin at the time of puberty. Most cases of primary lymphedema present symptoms at this age and this condition affect mostly girls with symptoms in the lower extremities.
- Lymphedema tarda, is a primary lymphedema that occurs in adults and affects both males and females. The onset of symptoms in sudden, with no apparent cause, and can affect one or both of the lower extremities.
Secondary Lymphedema is the result of a damaged or blocked lymphatic system. The swelling usually begins near the body and spread towards the far end of the limb. Causes include:
- Cancer treatment that involves the removal of the lymph node or their destruction due to radiation or chemotherapy.
- Scars due to burns radiation treatment, or other damage to large areas of the skin.
- Trauma including all types of accidents, injuries, and surgery that damage to the lymphatic system.
- Those with chronic venous insufficiency.
- Those who are obese.
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To read more, scroll to page(s) G36-G37 in the Clinical Guidelines found here.