Lymphedema, also known as lymphatic obstruction, is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system. The lymphatic system returns the interstitial fluid to the thoracic duct and then to the bloodstream, where it is recirculated back to the tissues. Tissues with lymphedema are at risk of infection.
Symptoms may include severe fatigue, a heavy swollen limb or localized fluid accumulation in other body areas, including the head or neck, discoloration of the skin overlying the lymphedema, and eventually deformity (elephantiasis).
Lymphedema should not be confused with edema arising from venous insufficiency, which is not lymphedema. However, untreated venous insufficiency can progress into a combined venous/lymphatic disorder which is treated the same way as lymphedema.
Presented here is an extreme case of severe unilateral hereditary lymphedema which had been present for 25 years without treatment.
CAUSES & RISK FACTORS
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Lymphedema (also see Elephantiasis) may be inherited (primary) or caused by injury to the lymphatic vessels (secondary). It is most frequently seen after lymph node dissection, surgery and/or radiation therapy, in which damage to the lymphatic system is caused during the treatment of cancer, most notably breast cancer. In many patients with cancer this condition does not develop until months or even years after therapy has concluded. Lymphedema may also be associated with accidents or certain diseases or problems that may inhibit the lymphatic system from functioning properly. In tropical areas of the world, a common cause of secondary lymphedema is filariasis, a parasitic infection. It can also be caused by a compromising of the lymphatic system resulting from cellulitis.
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, develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). In men, 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 cancer surgery and lymph node 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 for prostate, colon and testicular cancers may result in secondary lymphedema, particularly when lymph nodes have been removed or damaged.
The onset of secondary lymphedema in patients who have had cancer surgery has also been linked to aircraft flight (likely due to decreased cabin pressure). For breast cancer survivors, wearing a prescribed and properly fitted low-compression sleeve and gauntlet may help decrease swelling during flight.
Some cases of lower-limb lymphedema have been associated with the use of tamoxifen, due to the blood clots and deep vein thrombosis (DVT) that can be caused by this medication. Resolution of the blood clots or DVT is needed before lymphedema treatment can be initiated.
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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. Lack of hair may indicate an arterial circulation problem. If swelling is observed, the calf circumference should be measured with a tape measure. This measurement can be compared to future measurements to see if the swelling is getting better. Determine if elevating the legs makes the swelling go away. Pressure should be applied with the fingertips over the ankle to determine the degree of swelling. The assessment should also include a check of the popliteal, femoral, posterior tibial, and dorsalis pedis pulses. When checking the femoral pulse, feel for the inguinal nodes and determine if they are enlarged. Enlargement of the nodes lasting more than three weeks may indicate infection or some other disease process requiring further medical attention.The diagnosis or early detection of lymphedema is difficult. The first signs may be subjective observations such as "my arm feels heavy" or "I have difficulty these days getting rings on and off my fingers". These may be symptomatic of early stage of lymphoedema where accumulation of lymph is mild and not detectable by any difference in arm volume or circumference (see severity above). As lymphedema develops further, definitive diagnosis is commonly based upon an objective measurement of difference between the affected or at-risk limb at the opposite unaffected limb, e.g. in volume or circumference. Unfortunately, there is no generally accepted worldwide criterion of difference definitively diagnostic although a volume of difference of 200 ml between limbs or a 4 cm (at a single measurement site or set intervals along the limb) is often used. Recently the technique of bioimpedance measurement (a method that measures the amount of fluid in a limb) has been shown to have greater sensitivity than these existing methods and holds promise as a simple diagnostic and screening tool. Impedance analysers specifically designed for this purpose are now commercially available.
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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 or certified lymphedema therapist. The most common treatments for lymphedema are a combination of the daily use of intermittent sequential multi-chambered overlapping gradient pumps, manual compression lymphatic massage, compression garments or bandaging. Complex decongestive physiotherapy is an empiric system of lymphatic massage, skin care, and compressive garments. Although a combination treatment program may be ideal, any of the treatments can be done individually.
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