Current concepts and future directions in the diagnosis and management of lymphatic vascular disease

Despite the central, complex role for the lymphatic system in the maintenance of human health, the biology of this important and complex vasculature has been relatively under-investigated. However, the last decade has witnessed a substantial growth in the elucidation of lymphatic structural biology and the function of this system in health and in disease. These newly gained insights can be used to formulate our evolving concepts about the diagnostic and therapeutic approaches to patients with lymphatic vascular disorders. In lymphedema, there is a spectrum of disease that extends from primary (heritable) to secondary (acquired) causes. Once detected, the presence of lymphatic edema mandates very specific modalities of intervention, predominated by physiotherapeutic techniques. In addition, a physiological basis for adjunctive, intermittent pneumatic compression has been established, and these modalities may be indicated in selected patient populations. The acknowledgement of a unique biology in lymphatic edemas is, increasingly, guiding research efforts within this field. Increasing investigative attention is being directed toward animal models of lymphatic vascular disease. As insight into the complex biology of the lymphatic vasculature continues to expand through focused biomedical investigation, the translation of these mechanistic insights into targeted, rationally conceived therapeutics will become increasingly feasible.