Diagnosis and Management of Lymphatic Vascular Disease

Stanley G. Rockson, MD

Disclosures

J Am Coll Cardiol. 2008;52(10):799-806. 

In This Article

Abstract and Introduction

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.

The lymphatic vasculature, an integral component of the mammalian circulation, is comprised of a network of vessels that is essential both to fluid homeostasis and to the mediation of regional immune responses.[1] This vasculature consists of a series of conduits to interconnect the body's interstitial spaces with the lymphoid organs (thymus, spleen, and lymph nodes), and the central circulation, respectively. The vessels are structurally and functionally specialized to mediate the collection and homeostatic regulation of the protein-enriched fluid that is excluded from the venous end of the blood capillary.[2] The distinctive structural attributes of the lymphatic capillary network support this vital physiological task: in contrast to the blood circulation, the endothelial monolayers of the lymphatic capillaries display loose junctions that facilitate the entry of fluid, macromolecules, and cells.[3] In parallel to its role in extracellular homeostasis, the lymphatic vasculature promotes the traffic of immune cells and fosters lymphocyte population growth.[4]

Unlike the circulation of body fluids through the blood vasculature, lymphatic flow occurs through a low pressure system.[5] Interstitial fluid gains entry through the initial lymphatics that abut the interstitial space. These structures coalesce into conduits of increasing caliber that, ultimately, become invested with a smooth muscle coat and possess the capacity for rhythmic contractility; these collecting vessels eventually drain their fluid content (lymph) into the central vasculature, chiefly through the thoracic duct.[2]

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