Diagnosis and Management of Lymphatic Vascular Disease

Abstract

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.

Endothelial progenitor cell transplantation decreases lymphangiogenesis and adverse myocardial remodeling in a mouse model of acute myocardial infarction

“…Increased lymphatic flow rate might be associated with interstitial edema caused by LAD ligation. Moreover, lymphatic flow impairment caused by lymphatic disruption or flow resistance may occur in some diseases such as AMI and mediastinal lymphadenopathy, in which functional lymphangiogenesis (lymphatic vessel regeneration) will improve cardiac lymph flow (Rockson, 2008). In our study, potent lymphangiogenetic factors including VEGF-C and D, Prox-1, and Lyve-1 were significantly expressed in the peri-infarction areas after AMI.…”

Bone morphogenetic protein 9 (BMP9) controls lymphatic vessel maturation and valve formation

“…Hypoplasia, disruption or dysfunction of the lymphatic vessels, impair the ability of the lymphatic vasculature to collect and transport fluids and lead to lymphedema. 1,2 The mammalian lymphatic system has been shown to originate from embryonic veins. Lymphatic vessel development starts at embryonic days (E) 9.5 to 10.5 in mice.…”

Effects of physiotherapy combined with sirolimus in a patient with vascular malformation: A case report

“…Hammil et al 4 showed that the use of the mTOR (mammalian target of sirolimus) inhibitor sirolimus, with its tolerable side effects, significantly improved clinical conditions of the patients. In this study, it is also showed that sirolimus is effective and reliable in the treatment of life-threatening vascular anomalies, and it should be used as an important treatment approach 5 .…”

“…Complete decongestive physiotherapy (CDT), which is the most common and the most effective treatment method in lymphedema, is a multicomponent approach aiming to decrease the extremity volume, maintain the skin health, and support the structures 5 . Manual therapy is used in physiotherapy for pain and joint limitations 8 .…”