Lymphedema refers to an accumulation of liquids (high protein concentration) in the subcutaneous tissue (interstitial space) determined by a reduced lymphatic transposition capacity. It is a pathology present in about 300,000,000 cases worldwide (half of which is primitive).
The lymphedema can be Congenital or Acquired. In the first case it is linked to a reduced development of the local lymphatic system or to an altered functioning of the valvular systems; in the second one it is linked to trauma or surgical/radiotherapy lesions of lymph node pathways or stations or to damage dued to infections (like Erysipelas).
Lymphedema sometimes also occur in the case of functional overload of the regional lymphatic system as in the case of the post-thrombotic syndrome (it is a process that is established after deep vein thrombosis), of chylous ascites or kilo thorax.
Primitive (or congenital) lymphedema is an autosomal dominant disorder with variable expressiveness; in the same family both genetic mutations and anatomical alterations are present but in some members the anatomical defect is clinically evident during life while not in others. It can also be present at birth or in the second decade of life or after.
The diagnosis of lymphedema is clinical and instrumental (high-resolution ultrasound and lymphoscintigraphy).
The treatment depends on the cause of it and consist in ‘conservative’ therapies (like mechanical-pressure therapy, manual lymphatic drainage, multi-layer bandages, ultrasound, shock waves, vacuum therapy, etc.) or, in the most serious cases, microsurgical therapy that ranges from the connection of lymphatic vessels of 0.3-0.8 mm veins of varying caliber to liposuction in cases where the edema does not respond to physical treatments