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Lymphedema

LYMPHOEDEMA
Definition
Lymphoedema may be defined as abnormal limb swelling caused by the accumulation of increased amounts of high protein ISF secondary to defective lymphatic drainage in the presence of (near) normal net capillary filtration

Risk factor for lymphedema

Causes of primary lymphedema

inherited abnormality of the lymphatic system, sometimes termed ‘congenital lymphatic dysplasia’.
sporadic cases of primary lymphoedema occur in the presence of a (near-)normal lymphatic system and are actually examples of secondary lymphoedema .the triggering factor may be trivial (but repeated) bacterial and/or fungal infections, insect bites, barefoot walking (silica), deep venous thrombosis (DVT) or episodes of superficial thrombophlebitis.


Classification of primary lymphedema
Primary lymphoedema is usually classified on the basis of :
appar- ent genetic susceptibility :Primary lymphoedema is often subdivided into those cases in which there appears to be a genetic susceptibility or element to the disease, and those in which there is not. The former may be further divided into those cases that are familial (hereditary) and those cases that are syndromic. It is either inherited or sporadic. Syndromic lymphoedema may be sporadic and chromosomal (Turner’s (XO karyotype), Klinefelter’s (XXY), Down’s (trisomy 21) syndrome) or clearly inherited and related to an identified or presumed single-gene defect (lymphoedema–distichiasis (autosomal dominant defect in FOXC2 gene)), or of uncertain genetic aetiology (yellow-nail and Klippel–Trenaunay–Weber’s syndromes).

Continue classification of primary edema….

Age of onset:
Lymphoedema congenita (onset at or within two years of birth) is more in males, more likely bilateral and involve the whole leg.
Lymphoedema praecox (onset from 2 to 35 years) is more common in females, peak incidence shortly after menarche, more likely to be unilateral and usually only extends to the knee.
Lymphoedema tarda develops, by definition, after the age of 35 years and is often associated with obesity, with lymph nodes being replaced with fibrofatty tissue. The cause is unknown. Lymphoedema developing for the first time after 50 years should prompt a thorough search for underlying (pelvic, genitalia) malignancy. It is worth noting that, in such patients, lymphoedema often commences proximally in the thigh rather than distally.

Continue classification of primary edema….

Lymphoangiographic classification :Browse has classified primary lymphoedema on the basis of lymphangiographic findings . These findings may be related to the clinical presentations . Some patients with lymphatic hyperplasia possess megalymphatics in which lymph or chyle refluxes freely under the effects of gravity against the physiological direction of flow. The megalymphatics usually end in thin-walled vesicles on the skin, serous surfaces (chylous ascites, chylothorax), intestine (protein-losing enteropathy), kidney or bladder (chyluria) .see the following table.

Classification of secondary lymphedema: