STATE-OF-THE-ART PAPER
Diagnosis and Management of Lymphatic Vascular Disease
Stanley G. Rockson, MD*
Stanford Center for Lymphatic and Venous Disorders, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California
Manuscript received May 19, 2008;
revised manuscript received May 28, 2008,
accepted June 3, 2008.
* Reprint requests and correspondence: Dr. Stanley G. Rockson, Stanford Center for Lymphatic and Venous Disorders, Division of Cardiovascular Medicine, Stanford University School of Medicine, 300 Pasteur Drive, Stanford, California 94305 (Email: srockson{at}cvmed.stanford.edu).
The lymphatic vasculature is comprised of a network of vessels that is essential both to fluid homeostasis and to the mediation of regional immune responses. In health, the lymphatic vasculature possesses the requisite transport capacity to accommodate the fluid load placed upon it. The most readily recognizable attribute of lymphatic vascular incompetence is the presence of the characteristic swelling of tissues, called lymphedema, which arises as a consequence of insufficient lymph transport. The diagnosis of lymphatic vascular disease relies heavily upon the physical examination. If the diagnosis remains in question, the presence of lymphatic vascular insufficiency can be ascertained through imaging, including indirect radionuclide lymphoscintigraphy. Beyond lymphoscintigraphy, clinically-relevant imaging modalities include magnetic resonance imaging and computerized axial tomography. The state-of-the-art therapeutic approach to lymphatic edema relies upon physiotherapeutic techniques. Complex decongestive physiotherapy is an empirically-derived, effective, multicomponent technique designed to reduce limb volume and maintain the health of the skin and supporting structures. The application of pharmacological therapies has been notably absent from the management strategies for lymphatic vascular insufficiency states. In general, drug-based approaches have been controversial at best. Surgical approaches to improve lymphatic flow through vascular reanastomosis have been, in large part, unsuccessful, but controlled liposuction affords lasting benefit in selected patients. In the future, specifically engineered molecular therapeutics may be designed to facilitate the controlled regrowth of damaged, dysfunctional, or obliterated lymphatic vasculature in order to circumvent or mitigate the vascular insufficiency that leads to edema and tissue destruction.
Key Words: lymphatic • lymphedema • vascular insufficiency • edema • lymphangiogenesis • lymphatic vascular disease
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Abbreviations and Acronyms
| | CDPT = complex decongestive physiotherapy | | KTS = Klippel-Trenaunay syndrome | | LAM = lymphangioleiomyomatosis | | MLD = manual lymphatic drainage | | VEGF = vascular endothelial growth factor | | VEGFR = vascular endothelial growth factor receptor |
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