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Lymphedema

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.
Newborn lymphedema
Presentation of newborn lymphedema

 

Lymphedema is present in about 70% of Turner Syndrome patients and the hands and feet are most commonly affected [4]. The form of lymphedema 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?

  • Lymphedema is 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.
  • Lymphedema affects an estimate 100 million men, women, and children around the world including at least 3 million Americans.
  •  Lymphedema 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 of lymphedema is protein-rich and this makes the tissue easily susceptible to infections.
  • Lymphedema is a chronic condition that can be treated but not cured. Early treatments can usually effectively control the condition.
  • Without treatment, the lymphedema symptoms become progressively more serious. The seriousness of the condition is described as Stages of Lymphedema.
  • The onset of lymphedema 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

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

Secondary Lymphedema is the result of a damaged or blocked lymphatic system. The swelling of secondary lymphedema usually begins near the body and spread towards the far end of the limb. Causes of secondary lymphedema include:

  • Cancer treatment that involves the removal of the lymph node or their destruction due to radiation or chemotherapy. Shown here is secondary lymphedema following cancer treatment.
  • 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 can develop lymphedema as a secondary condition.
  • Those who are obese can develop lymphedema as a secondary condition.

READ MORE ON LYMPHEDEMA DIAGNOSIS AND MANAGEMENT Read more…

Lymphatic Education & Research Network 
www.lymphaticnetwork.org
Registry & Tissue Bank
https://lernregistry.stanford.edu/
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