Primary Lymphedema and Lymphatic Malformation
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Primary lymphedema is defined as edema caused by lymphatic dysplasia or/and dysfunction due to congenital or unknown factors. The clinical manifestations of primary lymphedema are variable. Based on MR lymphangiography, lymphatic system malformations in primary lymphedema can be classified as (1) lymph nodes affected with nodal structural abnormality, (2) lymph vessel affected with hypoplasia/aplasia, (3) both lymph vessel and lymph node affected with subgroups, and (4) lymphatic dysfunction. The classification proposed may be helpful in further studies focused on the development of more appropriate target-oriented treatment.
KeywordsPrimary Lymphedema Lymphatic Malformations Lymphatic Dysfunction Yellow Nail Syndrome Chylous Reflux
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