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Disease of the lymphatics can be either developmental or acquired. Developmental disorders include heritable forms of lymphatic pathology, such as Milroy's disease, as well as congenital vascular malformations, such as Klippel-Trenaunay syndrome (Table 40-1). Acquired forms of lymphatic disease may arise from damage to, or disruption of, the lymphatics by trauma, infection, neoplasms, or iatrogenic causes. These iatrogenic sources of lymphatic vascular insufficiency include trauma induced by surgery or radiotherapy. Heritable forms of lymphedema are uncommon, with the highest frequency associated with Klinefelter's syndrome.1 Globally, lymphatic filariasis contributes the greatest share of the lymphatic disease burden,2 but in the developed world, where filariasis is distinctly uncommon, most lymphatic pathology arises as a direct consequence of the treatment of malignant melanoma and breast and pelvic malignancies. Lymphedema is a common complication of treatment and has increased as the number of cancer survivors has grown.3

TABLE 40-1.Complex Vascular Malformations247

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