Lymphedema

Lymphedema is a condition that arises from the accumulation of lymphatic fluid due to an obstruction, absence or dysfunction of the lymphatic system.

Causes

Lymphedema may be inherited (primary) or caused by injury or damage to the lymphatic vessels (secondary).

While the exact cause of primary lymphedema is still unknown, it generally occurs due to poorly-developed or missing lymph nodes and/or channels in the body. Lymphedema may be present at birth (congenital), develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). Lower-limb primary lymphedema is most common, occurring in one or both legs. Some cases of lymphedema may be associated with other vascular abnormalities. 

Secondary lymphedema affects both men and women. In women, it is most prevalent in the upper limbs after breast surgery and lymph nodes dissection, occurring in the arm on the side of the body in which the surgery is performed. Head and neck lymphedema can be caused by surgery or radiation therapy for tongue or throat cancer. It may also occur in the lower limbs or groin after surgery for colon, ovarian or uterine cancer in which removal of lymph nodes or radiation therapy is required. Surgery and/or treatment of prostate, colon and testicular cancers may result in secondary lymphedema, particularly when lymph nodes have been removed or damaged.

Secondary lymphedema has a number of other causes such as tumor recurrence or cancer infiltration of the lymph nodes, infection, burns, obesity that can result in massive localized lymphedema, lipedema; trauma/injury, immobility such as stroke/CVA, congestive heart failure, chronic venous insufficiency (phlebo-lymphedema) and portal hypertension.

Stages

The most common method of staging was defined by the Fifth WHO Expert Committee on Filariasis:

  • Stage 0 (latent): The lymphatic vessels have sustained some damage which is not yet apparent. Transport capacity is still sufficient for the amount of lymph being removed. Lymphedema is not present.
  • Stage 1 (spontaneously reversible): Tissue is still at the “pitting” stage when pressed by the fingertips, the affected area indents and holds the indentation. Usually upon waking in the morning, the limb or affected area is normal or almost normal in size.
  • Stage 2 (spontaneously irreversible): The tissue now has a spongy consistency and is considered “non-pitting” when pressed by the fingertips, the tissue bounces back without any indentation. Fibrosis found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size.
  • Stage 3 (lymphostatic elephantiasis): At this stage, the swelling is irreversible and usually the limb(s) or affected area is very large. The swelling and tissue fibrosis cause the skin to thicken and lose its normal elasticity. These changes create tissue folds that limit mobility, are disfiguring, and encourage fungal infections and open wounds that are difficult to heal.

Diagnosis

Assessment of the lower extremities begins with a visual inspection of the legs. Color, presence of hair, visible veins, size of the legs and any sores or ulcerations should be noted. Stemmer’s sign: a thickened skin fold at the base of the second toe or second finger that is a diagnostic sign for lymphedema. A positive result occurs when this tissue cannot be lifted but can only be grasped as a lump of tissue. In a negative result, it is possible to lift the tissue normally.

If the cause of your lymphedema is not obvious, imaging tests can be ordered to determine what’s causing the condition:

  • Magnetic resonance imaging (MRI) –  using magnetic fields and radio waves, an MRI produces 3-D, high-resolution images. An MRI gives a better look at the tissues in your arm or leg. MRI offers strong qualitative and quantitative parameters in the diagnosis of lymphedema and lipo-lymphedema (lipedema).
  • Computerized tomography (CT) scan – is an X-ray technique that produces detailed, cross-sectional images of your body’s structures. CT scans can reveal areas of the lymphatic system that may be blocked.
  • Doppler ultrasound – used to evaluate the health of blood vessels, looks at blood flow and pressure by bouncing high-frequency sound waves (ultrasound) to capture images of structures inside the body. Ultrasound can be helpful in finding venous and arterial obstructions.
  • Lymphoscintigraphy – simple and noninvasive functional test for the evaluation of the lymphatic system and diagnosis of lymphedema. Is the radionuclide technique of imaging the lymphatic system using interstitially injected radio-pharmaceutical particles, was first introduced in 1953 and is now the gold standard for assessing the lymphatics. Lymphoscintrigraphy may be used to predict the outcome of treatment and also may be a useful preoperative investigation in the selection of patients for microvascular procedures.

Treatment

Treatment for lymphedema varies depending on the severity of the edema and the degree of fibrosis of the affected limb. Most people with lymphedema follow a daily regimen of treatment as suggested by their physician and certified lymphedema therapist.

The lymphedema physician (lymphologist) through a history and physical examination can help differentiate between the different kinds of “edemas”. Edema or swelling of an arm or leg can be related to conditions or diseases of the heart, kidney, liver, lymphatic system, venous system disorders such as chronic venous disease and deep venous thrombosis; and/or side effect of some medications.

The most common treatments for lymphedema are a combination of the use of manual lymphatic drainage, compression bandaging and garment and intermittent sequential gradient pumps. Complete decongestive therapy (CDT) is a system of manual lymphatic drainage massage (MLD), application of multi-layer compression bandages, therapeutic exercises, skin care and patient education on self care and prevention of complications like cellulitis, cutaneous lymphoadenitis, fungal infections, lymphorrhea, skin breakdown, ulcers and other skin conditions.

Prevention

  • Do not allow anyone to take blood or your blood pressure on the affected arm or leg.
  • Wear a MEDICAL BRACELET warning of lymphedema or risk for developing lymphedema.
  • Maintain a healthy weight and eat well-balanced, avoiding high salt and fatty foods.
  • Use sunscreen when outdoors, test first on unaffected area for skin reaction.
  • Avoid direct use ice of HEATING PADS to the affected area, prolonged sun exposure, hot tubs or saunas.
  • Avoid skin scratch, cuts, burns or bug bites to the affected arm or leg to prevent port of entry for bacteria.
  • Use an electric razor to shave.
  • Keep your affected arm or leg clean, use a mild unscented soap and apply a mild unscented body LOTION FOR DRY SKIN that will not block your pores.
  • Avoid crossing your legs or carrying items on your shoulder if the area is at risk.
  • Keep hands and feet protected by wearing gloves and shoes specially when gardening and HOUSE CLEANING.
  • When air traveling use your COMPRESSION GARMENT, move around to prevent blood clots specially in long flights, perform exercises with the affected limb(s), drink water and limit alcohol consumption.