About Lymphedema

Overview

Lymphedema is a chronic, progressive swelling of a region of the body due to an obstruction of the lymphatic system. The obstruction can be congenital, either an anatomical or functional problem, or caused by an injury, surgery, radiation, burns, infection, or tumor.   Congenital lymphedema is called Primary Lymphedema whereas lymphedema caused by other factors is called Secondary Lymphedema. Regardless of the cause, lymphedema is a lifelong condition requiring daily management.

There are two categories of lymphedema: lymphostatic edema and lymphodynamic edema. Lymphostatic edema is a protein rich edema resulting from the lymphatic system not being able to handle the lymph-obligatory load (the amount of lymph fluid in the tissue). It is like a swampy river in the summer with standing water lying around, outside the riverbanks. Examples of a lymphostatic edema include swelling of a limb following radiation therapy or surgery.

Lymphodynamic edema is swelling caused by too much fluid in the tissue with no problems associated with the lymphatic system. It is like a river in the spring with more water than the riverbanks can contain. Examples include pregnancy.

Lymphostatic edema can be managed with Combined Decongestive Therapy (CDT) to stimulate increased lymphatic flow. Lymphodynamic edema is not improved by CDT as the lymphatic system is already functioning at 100% capacity.

Initial signs and symptoms of lymphedema typically include:

  • A sensation of fullness or heaviness in the limb or region
  • A sense of tightness in the skin
  • Tightness in regular clothing or jewelry
  • Decreased flexibility or mobility of a joint

Over time lymphedema causes the bio-mechanical properties of the skin and tissue, in the affected region, to change. Skin can become hardened and inflamed, and subcutaneous tissue can become fibrotic. As a result it is important to determine changes in the tissue, as well as fluid volume changes in the region.

Clinical assessment of lymphedema should include the physical examination of skin and tissue texture, inflammation, pitting, enlarged skin folds, and any other dermatological conditions, such as wounds or papillomas (benign growths) in addition to determining the volume of the region. There are a number of ways to undertake the tissue assessment, including:

  • Stemmer sign,
  • Tonometry, and/or
  • Bio-impedence.

Limb volume can be determined by:

  • Circumference measurement of the limb,
  • Water displacement, or
  • Perometry.

Tape measurement of the limb circumstance is the most common practice. Vodder School International recommends a measurement every 4 cm along the limb, the measurement is then squared, totaled with all other measurements, and divided by 3.1416 in order to determine the volume of the region.

Following this assessment the severity of a lymphedema can be categorized into the following four stages:

  • Stage 0 (Latency Stage) – no apparent swelling is visible although excess fluid is beginning to accumulate. The tissue feels consistent with non-edemic tissue upon palpation.
  • Stage 1 – the tissue is soft and non-pitting upon palpation, swelling is alleviated with elevation and there are no secondary changes associated with the tissue.
  • Stage 2 – tissue becomes increasingly dense or hardened with some pitting evident upon palpation. Risk of infection is increased.
  • Stage 3 – tissue becomes dense or hardened with noticeable pitting upon palpation due to the extreme build-up of fluid and protein.   The condition of the skin thickens and deteriorates.

Primary Lymphedema

Primary lymphedema is a condition that people are born with, although it may not be evident until later in life. Primary lymphedema can onset at basically three stages in life: 0 – 6 months of age, around puberty (praecox), or after age 35 years (tarda). Primary lymphedema can occur as a result of an anatomical problem or a functional problem.

Anatomical problems include situations such as:

  • missing lymph vessels,
  • missing smooth muscle which wrap pre-collector/collector vessels,
  • missing nodes,
  • inadequate number of vessels, or
  • inadequate nodes in a region of the body.

Functional problems include:

  • smooth muscles on lymph vessels not functioning, and
  • lymph values not working properly.

Recent developments in the nuclear medical field, particularly lymphoscintigraphy, now offer a technique for imaging lymph vessels and nodes to detect abnormalities in the lymphatic system. In addition, progress has been made in genetic testing to determine the presence of chromosome abnormalities, such as Turner’s syndrome and Milroy’s disease. Most inherited lymphedema, however, is not detected on a gene or chromosome test.

Secondary Lymphedema

Secondary lymphedema is caused by a disruption to the lymphatic system, and is the most prevalent form of lymphedema.   Typical disruptions include damage caused by:

  • surgical procedures,
  • cancer,
  • radiation,
  • injury or trauma,
  • infections, or
  • inflammation.

Secondary lymphedema can occur shortly after the lymphatic system has been damaged or it can become symptomatic weeks, months or even years later. As a result individuals at risk for lymphedema, due to any of the above factors, should take precautions to avoid overtaxing the lymphatic system. This may include some or all of the following practices:

  • take precautions to avoid injury to any region of the body at risk,
  • avoid medical procedures (such as blood pressure cuffs, drawing blood, vaccinations) on limbs at risk,
  • wear compression garments when vigorously exercising,
  • wear compression garments when travelling by air,
  • avoid heat including hot baths, hot tubs, steams or saunas,
  • avoid tight clothing,
  • elevate at risk limbs when resting, and/or
  • use low ph cleansers on your skin and clean cuts, scraps, or cracks in the skin that could lead to infection immediately.