Szuba A, Rockson SG.
Lymphedema: classification, diagnosis and therapy.
Vasc Med 1998;3(2):145-56

Division of Cardiovascular Medicine, Stanford University School of Medicine, Falk
Cardiovascular Research Center, CA 94305, USA.

This review presents the diagnostic features, the pathophysiology and the
available therapies for lymphedema. This disease is often able to be diagnosed by
its characteristic clinical presentation, yet, in some cases, ancillary tests
might be necessary to establish the diagnosis, particularly in the early stages
of the disease and in edemas of mixed etiology. These diagnostic modalities are
also useful in clinical studies. Available modalities include isotopic
lymphoscintigraphy, indirect and direct lymphography, magnetic resonance imaging,
computed tomography and ultrasonography. Lymphedema may be primary or secondary
to the presence of other disease and/or to the consequences of surgery. Primary
lymphedema may occur at any phase of life but it most commonly appears at
puberty. Secondary lymphedema is encountered more often. The most prevalent
worldwide cause of lymphedema is filariasis, which is particularly common in
south-east Asia. In the USA, postsurgical lymphedema of the extremity prevails.
Complications of chronic limb lymphedema include recurrent cellulitis and
lymphangiosarcoma. Most patients are treated conservatively, by means of various
forms of compression therapy, including complex physical therapy, pneumatic pumps
and compressive garments. Volume reducing surgery is performed rarely. Lymphatic
microsurgery is still in an experimental stage, although a few centers
consistently report favorable outcomes.