Review
Lymphedema

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Abstract

Lymphedema is a set of pathologic conditions that are characterized by the regional accumulation of excessive amounts of interstitial protein-rich fluid. These occur as a result of an imbalance between the demand for lymphatic flow and the capacity of the lymphatic circulation. Lymphedema can result from either primary or acquired (secondary) disorders. In this review, the pathophysiology, classification, natural history, differential diagnosis, and treatment of lymphedema are discussed.

Section snippets

Pathophysiology of lymphedema

In contrast with venous edema, in which enhanced capillary pressure can indirectly stimulate lymph production, lymphedema is caused by a reduction in lymphatic transport. Several anatomic problems can lead to lymphatic stasis, including lymphatic hypoplasia and functional insufficiency or absence of lymphatic valves (8). Some patients may have an impairment in the intrinsic contractility of the lymphangion (the segmentally contracting, functional vascular unit of the lymphatic circulation) (8).

Classification of lymphedema

The simplest classification of lymphedema relies upon a differentiation between primary and secondary causes (15). Primary lymphedemas are often classified according to the age at which the edema first appeared. Congenital lymphedema is apparent at birth or becomes recognized within the first 2 years of life. Lymphedema praecox is most commonly detected at the time of puberty, but may appear as late as the third decade of life. Lymphedema tarda typically appears after age 35 years. Recent

Natural history of lymphedema

The natural history of lymphedema is uncertain, particularly as it may be asymptomatic. For example, as late as 3 years after radical mastectomy and axillary node dissection, more than 20% of women remain free of any clinical evidence of lymphatic impairment, despite the extensive iatrogenic destruction of the lymphatic architecture in these patients 31, 32. Similarly, in many forms of primary lymphedema, there may be a protracted phase of apparently normal lymphatic function, despite an

Diagnosis of lymphedema

In most patients with advanced lymphedema, the characteristic clinical presentation and physical findings establish the diagnosis with near certainty (41). However, early in the natural history, or with presentations of mild or intermittent swelling, it may be more difficult to distinguish lymphedema from other edematous states. Several physical features distinguish lymphedema from other causes of chronic edema of the extremities. Among these are the classic changes of cutaneous and

Chronic venous insufficiency and postphlebitic syndrome

This common condition is often confused with lymphedema of the legs. Its distinguishing clinical features include aching discomfort in the lower extremities during sitting or standing and chronic pruritus, particularly overlying the incompetent communicating veins (56). Physical findings include cutaneous deposits of hemosiderin, dusky discoloration and venous engorgement with dependence, cutaneous varicosities, and if advanced, ulceration of the skin.

Myxedema

This special form of edema arises when

General therapeutic measures

Lymphedema is a chronic condition that requires lifelong attention. Meticulous attention to control of edema may reduce the likelihood of disease progression and limit the incidence of soft-tissue infections (41). Aggressive implementation of decongestive lymphatic therapy is the mainstay of most therapeutic recommendations 61, 62, 63. This complex form of physical therapy integrates meticulous skin care, massage, exercise, and use of compressive elastic garments. Decongestive lymphatic therapy

Acknowledgements

The assistance of Dr. Andrzej Szuba in the preparation of the figures is gratefully ackowledged.

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