Authors report modern diagnostic and therapeutic procedures used in the correct assessment and treatment of congenital lymphatic and chylous disorders. Lymphatic dysplasias can be clinically represented only by peripheral lymphedema or be associated with more complex dysfunctions of chyliferous vessels and the thoracic duct (chylous ascitis, chylothorax, etc.) It is, therefore, useful to perform a complete diagnostic evaluation of each patient before carrying out any therapeutical approach. Lymphoscintigraphy, lymphangio-MR, oil contrast lymphography, and lymphangio-CT are the common diagnostic tools used in these cases, variable associated depending above all on the complexity of the pathology. From the therapeutical point of view, microsurgical methods proved to bring successful and long lasting results, both with derivative lymphatic-venous anastomoses and reconstructive lymphatic-venous-lymphatic anastomoses. Better long-term results are obtained in earlier stages.
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