{"id":127,"date":"2014-02-19T07:32:51","date_gmt":"2014-02-19T07:32:51","guid":{"rendered":"http:\/\/lymphoedemacenter.com\/?page_id=127"},"modified":"2022-01-05T09:26:03","modified_gmt":"2022-01-05T09:26:03","slug":"congenital-lymphedema","status":"publish","type":"page","link":"https:\/\/lymphoedemacenter.com\/?page_id=127","title":{"rendered":"Congenital lymphedema"},"content":{"rendered":"

Lymphedema is a pathologic condition that results from a disturbance of the lymphatic system, with localized fluid retention and tissue swelling. Swelling can affect a single limb, multiple limbs, genitalia or the face, abdomen.<\/p>\n\n\n\n

Primary lymphedema<\/strong> is a congenital disorder<\/strong>, due to a malformation of lymph vessels and\/or nodes (hypotrophic or hypertrophic).<\/p>\n\n\n\n

Congenital lymphedema can appear at birth or during the first years of the life. In most of the cases it develops during puberty, perhaps due to the hypotrophy of the lymphatic system which is too small for the growing body, or dysplasia.<\/p>\n\n\n\n

Lymphatic anomalies include a variety of developmental and\/or functional defects affecting the lymphatic vessels: sporadic and familial forms of primary lymphedema, chylothorax and chylous ascites, lymphatic malformations, and overgrowth syndromes with a lymphatic component. <\/p>\n\n\n\n

Germline mutations have been identified in at least 20 genes that encode proteins acting around VEGFR-3 signaling but also downstream of other tyrosine kinase receptors. These mutations exert their effects via the RAS\/MAPK and the PI3K\/AKT pathways and explain more than a quarter of the incidence of primary lymphedema, mostly of inherited forms. More common forms may also result from multigenic effects or post-zygotic mutations.<\/p>\n\n\n\n

DIAGNOSIS<\/h2>\n\n\n\n

The clinical aspect and the history are important.<\/p>\n\n\n\n

Complementary Investigations<\/h3>\n\n\n\n

Nowadays, radiological evaluation of lymphedema patients can be done through lympho MRI.<\/p>\n\n\n\n

PDE (Photodynamic eye) provides information on the superficial lymphatic network.<\/p>\n\n\n\n

Magnetic Resonance Lymphography (MRL), with T3 weighted imaging, allows visualization of the lymphatic system anatomy with greater sensitivity than lymphoscintigraphy<\/a>, without the need of any injection.<\/p>\n\n\n\n

\"\"<\/a>
Lymphangioscintigraphy is dynamic but provides poor results.<\/em><\/figcaption><\/figure><\/div>\n\n\n\n
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Lymphatic MRI provides a better visualisation of the lymphatic system anatomy<\/em><\/figcaption><\/figure><\/div>\n\n\n\n

Physiotherapic Treatment<\/h2>\n\n\n\n

Physiotherapy (manual drainages, pressotherapy, compression, bandages) is the usual treatment for chronic lymphedema. It is not a curative therapy, but helps to control the evolution of the disease.<\/p>\n\n\n\n

Surgical Treatment<\/h2>\n\n\n\n

ALNT Indication<\/h3>\n\n\n\n

Patients with HYPOPLASIA of the lymphatic system, pain and chronic infections are the preferred candidates for the procedure.<\/p>\n\n\n\n

The flap<\/a> is inserted in the area where the nodes and the lymphatic vessels are insufficient.<\/p>\n\n\n\n

The healthy nodes contain a growth hormone (VGEF 3) witch induces neoformation of lymphatic ducts. They also help fighting again infections.<\/p>\n\n\n\n

In patients with distal lymphedema<\/strong> :<\/p>\n\n\n\n