Stages of Lymphedema

There are 4 stages which are considered in the diagnosis and treatment of Lymphedema.  

Stage 0, Latency stage 

This stage pertains to oncology patients who have had cancer treatment involving Lymphadenectomy (Lymph Node Dissection) and/or Radiation Therapy. Following these procedures, patients are considered to have Stage 0 Lymphedema. When lymph nodes are removed, or lymphatic structures are altered, the lymph transport capacity is reduced.

Following these procedures, patients have a "Limb At Risk" - and this risk is life-long. Post operative cancer patients develop Secondary Lymphedema 5, 10, even upwards of 20 years and beyond their initial cancer treatment.

The lymphatic system functions less efficiently as we age, therefore, any clinically induced compromise to the lymphatic system can result in Lymphatic insuffiencies or Lymphedema as time goes on.

Most patients in this stage are externally asymptomatic, showing no signs of swelling. Lymphedema is generally unilateral  (affecting only one limb) and asymmetrical. However, estasblished Lymphedema can develop over time.

The following signs and symptoms are indicative of Latent Lymphedema:

• An achy, full or heavy feeling in one or more limbs. These feelings could be present even though there is no visible swelling;

• General fatigue or fatigue upon exertion on limb(s), especially if no similar feeling is felt in the unaffected extremity

• Unexplained but palpablle sensations, such as tingling, numbness, etc.

• Marginal swelling may or may not appear and if so, it is usually random rather than episodic.

Stage 1 

This stage of Lymphedema is often referred to as Spontaneous, "Reversible" Lymphedema. Swelling can appear for no specific reason and last throughout the day. It is often difficult for patients to identify a precipitating factor. The tissue of the affected extremity will often appear marginally larger in circumference than the unaffected extremity and a pitting response (indentation in the tissue) can occur with applied or sustained pressure. Patient's will often notice that the swelling abates when they awake in the morning. Recurrent swelling may present throughout the next day or may not present for several days or weeks following the initial episode. Because the swelling in this Stage tends to be both marginal and intermittent, many patients as well as their clinicians deny the need for applied clinical intervention until or if the swelling progresses. However, it is actually very important to seek clinical attention and treatment following the first sign of initial swelling. In other words:

If your extremity at risk for Lymphedema swells, even marginally, seek clinical attention right away and do not wait to see if the swelling progresses

The following characteristics are indicative of Stage 1 Lymphedema:

• Marginal intermittent swelling that abates more than being present; this swelling can occur at any point along the affected extremity and can present in any pattern

• Pitting response

• Elevation generally reduces swelling

• Normal limb contour can be marginally altered

• Swelling can abate entirely during the night and into the following morning, and may not recur for some time.

Stage 2

Lymphedema in this stage is often referred to as Spontaneously "Irreversible" Lymphedema, because at this point, the affected limb remains swollen permanently. Unlike Stage 1 where swelling is often intermittent and could abate in the morning, here the swelling becomes unmanageable and as a result, secondary tissue changes generally occur.

The following are characteristics of Stage 2 Lymphedema:

• Permanent and progressive swelling;

• The tissue produces only a minimal or no pitting response upon pressure because fibrosclerotic changes begin.

• The limb begins to feel hard and  full, the tissue generally feels like hard dough or like soft rubber;

• Range of motion could be compromised through advancement of this stage;

• The normal limb contour is altered marginally or significantly, depending on the progression of swelling;

• The patient becomes more susceptible to infections such as cellulitis, due to adverse metabolic changes in the tissue;

• Psycho-social issues can begin to impact quality-of-life.

Stage 3

Stage 3 Lymphedema or "Lymphostatic Elephantiasis", is the most advanced and severe stage of Lymphedema. In addition to the secondary tissue changes seen in Stage 2 Lymphedema, such as fibrosis, induration and loss of limb countour, in Stage 3 Lymphedema the following characteristics are seen:

• Tertiary skin pathologies such as: Hyperkeratosis, papillomatosis and fungal infections;

• Significant obliteration of normal limb contour and in some cases complete limb deformity, due to hypertropy (overgrowth) of subcutaneous tissues;

• Increased risk for infection, or cellulitis;

• Excessive weeping of fluid from the limb;

• Tissue maceration;

• Ulcerations/wound formation;

• Restricted range of motion or inhibition of ambulation due to extreme induration (hardening/thickening) of the tissues;

• Compromised hygiene resulting in a malodorous tagging of the affected extremity.

The characteristics associated with Stage 3 Lymphedema can cause social isolation, occupational cessation, physical disability, developmental obesity due to increased inactivity and  financial indigence.

In the most extreme cases, Stage 3 Lymphedema can result in Lymphangiosarcoma, extensive debulking surgeries or rarely, amputation of the extremity.