Lymphedema is an abnormal accumulation of protein-rich fluid in the interstitium, which causes chronic inflammation and reactive fibrosis of the affected tissues. Primary lymphedema is congenital and is the result of lymphatic dysplasia that may present at birth but more often develops later in life (peri-pubertal onset). Secondary lymphedema is the result of lymphatic system failure secondary to a trauma or injury to the system. In this country, it most often occurs after surgery or radiation therapy to treat cancer and results in a mechanical insufficiency, meaning that the lymphatic system itself is damaged and cannot keep up with the demands of the additional fluid and proteins in the interstitium. Secondary lymphedema can also occur due to obesity, chronic venous insufficiency, cardiac edema, cardiac bypass, orthopedic surgery, liposuction, etc. and begins initially with a dynamic insufficiency (fluid overload but intact lymphatics). Over time, this progresses to a combined dynamic and mechanical insufficiency when the lymphatic system has been overtaxed to the point where permanent damage begins to occur.
Dynamic insufficiencies are low protein edemas consisting mostly of water and typically reverse with elevation. Mechanical insufficiencies are high protein edemas and will not respond as well, or at all, to elevation or pneumatic pumps because neither causes the proteins in the interstitium to be reabsorbed. This is when complete decongestive therapy (CDT) is needed, which consists of manual lymph drainage (MLD), multi-layer compression bandaging, long term use of compression garments, remedial exercise, skin/nail care, and patient education for prevention and maintenance since lymphedema is a chronic condition and there is no cure.
We can help people with mechanical, dynamic, or mechanical/dynamic insufficiencies but the course of treatment will be different and we will expect different outcomes based on the patient’s type of edema. Those who have a dynamic insufficiency (fluid overload) with no damage to the lymphatic system will respond very well to compression garments, exercise, and education and may be able to skip the MLD and bandaging. However, for those with a high protein or mixed edema, the gold standard of treatment is CDT.
Early Treatment is Important
Lymphedema progresses at different unpredictable rates. Some limbs may stay in the congested state without developing fibrosis for quite a while and others develop fibrosis more quickly. Once fibrosis develops, it becomes more difficult to achieve good outcomes, so early treatment is crucial.
Patient education: patients are instructed to contact a healthcare provider immediately if they begin to experience feelings of heaviness, tightness, notice that clothing or jewelry are fitting more tightly, or if the at-risk area becomes hot or red. A patient who has no visible edema may still be in the initial stage 0 of lymphedema and referral for treatment is important even in early stages.
Signs and Symptoms of Various Edemas
Chronic venous insufficiency: Without appropriate therapy, venous edema triggers a lymphatic failure (mechanical insufficiency) and results in a combined phlebolymphostatic edema.
Traits: Gaiter distribution, non-pitting (indentation occurs but quickly refills), brawny (leathery or woody texture), hemosiderin staining (dark blue, brown or purple discoloration of the gaiter distribution), fibrosis of the subcutaneous tissue, ulcers are common
Cardiac Edema: (Caused by Right Heart Failure) CHF creates hypertension in the venous limb of the system, continued circulation increases blood capillary pressure at the microcirculation level resulting in back pressure and edema.
Traits: Most fluid builds up distally, always bilateral, pitting (but will refill quickly), complete resolution with elevation, no pain, jugular venous distention, orthopnea, paroxysmal nocturnal dyspnea, or dyspnea on exertion.
Acute Deep Vein Thrombosis: Sudden onset, usually unilateral, pain, cyanosis, swelling, distention of surface veins and pulmonary embolism.
Malignant lymphedema: May be indication of new onset or recurrent cancer.
Traits: Pain, paresthesia, paralysis, proximal onset, rapid development and continuous progression, shiny tight skin, swelling in nodules in supraclavicular fossa, hematoma-like discoloration, ulcers, non-healing open wounds.
Stages of Lymphedema:
Stage 2: accumulation of protein-rich edema fluid, pitting becomes progressively more difficult, connective tissue proliferation (fibrosis).
Stage 3: accumulation of protein-rich edema fluid, non-pitting, fibrosis and sclerosis, skin changes (papillomas, hyperkeratosis etc.).