Lymphedema ó an overview
Lymphedema refers to swelling that occurs most often in arms or legs when a blockage in the lymphatic system prevents the lymph fluid from draining adequately.
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). Some cases of lymphedema may be associated with other vascular abnormalities.
In pediatric patients 97 percent present primary lymphedema and 58 percent are females. Age of onset usually is more frequent at infancy and adolescence. Boys most commonly presented in infancy, whereas girls usually developed swelling in adolescence.
Lymphedema involves an extremity in 82 percent of cases, genitalia 4 percent, or both in 13 percent. The lower limb was most commonly affected and about 50 percent had bilateral lower extremity disease. Familial or syndromic lymphedema is found in about 10 percent of patients.
It is common occurrence association of primary lymphedema with other abnormalities and genetic defects such as hereditary congenital lymphedema (Milroyís disease); Meige lymphedema syndrome (also known as familial lymphedema praecox), yellow nail syndrome, Turner syndrome, Noonan syndrome, neurofibromatosis type I, Klinefelterís syndrome (XXY syndrome), congenital absence of nails, lymphedema-distichiasis syndrome; primary intestinal lymphangiectasia (Waldmannís disease), Trisomy 13, 10 and 21; hypotrichosis-lymphedematelangiectasia, Klippel-Trenaunay Syndrome (KTS), etc.
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 there was 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, lipo-lymphedema; trauma/injury, immobility such as stroke/CVA, congestive heart failure, chronic venous insufficiency (phlebo-lymphedema) and portal hypertension.
Worldwide, Filariasis is the most common cause of lymphedema. Filariasis is infestation of lymph nodes by the parasite Wuchereria bancrofti, which is transmitted among humans by mosquitoes. Filariasis is a significant public health problem affecting millions in tropical and subtropical regions of Asia, Africa, the Western Pacific, and parts of Central and South America.
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.
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.
Stemmerís sign: a thickened skin fold at the base of the second toe or second finger that is a diagnostic sign of lymphedema. A positive result occurs when this tissue cannot be lifted but can only be grasped as a lump of tissue normally.
If the cause of the lymphedema is not obvious, imaging tests can be ordered to determine whatís causing the condition:
Magnetic resonance imaging (MRI): MRI characteristically shows diffusive subcutaneous edema, reticular lymphangiectasis and ďchannelsĒ with sequestered lymph. MRI scan clearly displayed the proliferative and extended lymphatic vessels, trunks and chylocyst. MRI offers strong qualitative and quantitative parameters in the diagnosis of lymphedema and lipo-lymphedema (lipedema).
Computerized tomography (CT) scan: CT scan images can show an increase in the thickness of the subcutaneous tissue compartment and a characteristic ďhoneycombĒ appearance of the subcutaneous tissue.
Doppler ultrasound: used to evaluate the blood vessels, and is important in defining venous insufficiency, deep venous thrombosis as well as arterial system obstruction.
Lymphoscintigraphy: simple and noninvasive functional test for the evaluation of the lymphatic system. Is the radionuclide technique of imaging the lymphatic system using interstially injected radiopharmaceutical particles, was first introduced in 1953 and is now the gold standard for assessing the lymphatics. The images can demonstrate dermal diffusion (dermal backflow) or retention at the injection site of the tracer with poorly defined lymphatic trunks and delayed or no visualization of regional lymph nodes.
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, venous system disorders such as chronic venous disease and deep venous thrombosis; and side effect of some medications such as but not limited to:
Antidepressants, including MAO inhibitors such as phenelzine and tranylcypromine; and tricyclics such as nortriptyline, desipramine and amitriptyline
Blood pressure medicines such as nifedipine, amlodipine, diltiazem, felodipine and verapamil
Hormones like estrogen in birth control pills or hormone replacement therapy; and testosterone
NSAIDís such as ibuprofen, naproxen etc
Antiepileptic drugs commonly used for neuropathic pain such a pregabalin, gabapentin etc.
The most common treatments for lymphedema are a combination of the use of manual lymphatic drainage, compression bandaging and garment; and intermittent sequential gradient devices. Complex decongestive therapy (CDT) is a system of manual lymphatic drainage massage, application of multi-layer compression bandages, therapeutic exercises, skin care, prevention of complications and patient education.
The goal is to reduce and control the lymphedema and prevent serious skin complications such as ulcers and cutaneous lymphoadenitis. Early detection and proper management is the key factor to control the disease.
For additional information visit www.slideshare.net/physmedi/lymphedema-an-overview; www.lymphedemaphysmedi.com
Margarita Correa MD
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