Meige Lymphedema
From WikiGenetics
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[edit] Name of Condition
[edit] Definition
Meige is a non-hereditary form of lymphedema which is not pressent at birth. A protein rich lymphedema (swelling) forms at some point after birth and may be many years before manifesting. It should not be confused with Nonne-Milroy type which is Hereditary.
[edit] Description, Signs and Symptoms
symptoms typically appear later in teen years however they can appear at any time. The most common age of symptome appearance is 17. Symptoms include the typical symptoms of lymphedema: swelling, tightness, heat, decreased range of motion. Most commonly this rare condition produces swelling that starts in the feet and ankles and moves up the leg as the lymphatic becomes damaged by years of lymphostatic hypertension. Cellulitis and Erysipelas are common infections with lymphedema and are the cause of further damage to the functioning parts of the lymphatic system.
[edit] Inheritance
Non-Hereditary
[edit] Demographics
sporadic
[edit] Diagnosis
lymphoscintography remains the gold standard for diagnosis. Radiographic images when properly done will reveal displasia in the lymphatic system.
[edit] Treatment and Management
Combined Decongestive Therapy remains the gold standard to the treatment of lymphedema. This therapy involves the use of Manual lymphatic Drainage techniques along with compression bandaging to achive a reduction in the edema. After an intensive course of therapy which typicaly lasts 3-6 weeks, custom compression garments can be fitted to maintain the reduction. With good compliance and regular maintenance treatments, a relatively normal life can be achieved. In rare instances a poor response to therapy result in only a small reduction. Lymph-channel transfers which is an experimental surgical procedures, has shown promise for the treatment of resistent lymphedema.
[edit] Prognosis
At pressent there is no cure. The only measure of therapy found effective is Combined Decongestive Therapy. Even the experimental surgical procedures still leave the patient with lymphedema, however somewhat reduced. Drugs have no positive effect on lymphedema that has ever been proven. The typical attempt made by most doctors to treat with drugs is the use of diretics. A temporary reduction may be seen with diratics however this will not last. In fact the conditions usually worsens as a result of the use of these drugs due to tissue dehydration and the increased concentration of proteins withing the edema.
