Elsevier

Clinics in Dermatology

Volume 32, Issue 5, September–October 2014, Pages 685-691
Clinics in Dermatology

Towards an effective management of chronic lymphedema

https://doi.org/10.1016/j.clindermatol.2014.04.017Get rights and content

Abstract

Lymph conduit perturbation causes lymph stasis and the local accumulation of interstitial fluid. Lymphedema, a chronic and debilitating disorder, remains incurable despite the advances in the description of its pathomechanism and the improvement of conservative and nonsurgical treatments. The gold standard of lymphedema treatment is multicomponent decongestive physiotherapy. Manual lymph drainage, compression bandaging, skin care, and exercises constitute the therapeutic regimen that could be adjusted with intermittent pneumatic compression. Prophylaxis could give a major benefit to risk group patients; however, the assessment of preventive approaches postulates further clinical trials. Surgery represents an emerging stakeholder in lymphedema care, although, the partnership with adjunctive nonsurgical therapy is still alive. Liposuction proved to be one of the most promising technique with the clearance of the lymph stasis-related adipose tissue. Regeneration of lymphatic tunnels with lymphovenous anastomoses or the transplantation of lymph vessels or small veins is based on long-term experience. The success of lymph node transplantation is still under evaluation, but this novel technique has produced notable improvements.

Introduction

Lymphatic vasculature plays an essential role in the regulation of tissue fluid homeostasis; the transport of lipids, fat-soluble vitamins, proteins, and other large particles; and the afferent loop of the immunosurveillance.[1], [2] The lymphatic circulation provides the normal conduit for the return of interstitial fluid and proteins to the blood circulation; thus, sustained lymph stasis leads to the accumulation of protein and cellular metabolites in the extracellular space, generating an increase in tissue colloid osmotic pressure and then concomitant water accumulation along with an elevation of the interstitial hydraulic pressure.[2], [3] Chronic lymphedema is mostly an incurable and disabling lifelong condition that could develop either congenitally or be provoked by acquired factors.2 Primary lymphedema comprises genetic damage (eg, mutations of lymphatic vessel and lymph node developmental genes),3 whereas secondary lymphedema predominantly results from bacterial or parasitic infections, iatrogenic interventions (surgery, irradiation), malignancies, traumatic injuries, or chronic venous insufficiency.2

Early stages of lymphedema are predominantly characterized by interstitial fluid accumulation or mild tissue changes, whereas advanced lymphedema is accompanied by adipose tissue hypertrophy and excessive fibrosis.2 Despite tremendous efforts in the exploration of its pathomechanism and treatment, lymphedema remains a barely curable disease, and therapeutic options are relatively limited.2

Section snippets

Diagnosis

An accurate diagnosis is essential for appropriate therapy. In most cases, lymphedema could be relevantly diagnosed on the basis of clinical history and picture and physical examination. Confounding conditions such as gross obesity; endocrinopathies and other metabolic dysfunctions; lipedema or lipodystrophies; venous insufficiency; cardiac, renal, or respiratory insufficiencies; and the adverse effects of various medications could complicate the clinical picture.[2], [3] A thorough and

Prophylaxis

Total or segmental limb volume changes (≥3%) should alert caregivers to the early development of lymphedema.9 One study found that immediate intervention with compression garments resulted in a rapid evacuation of edema from the arms of women with breast cancer.9 This observation has spurred further clinical trials to assess the efficacy of compression stockings in lymphedema prevention. A recent paper emphasized the role of manual lymph drainage (MLD) as a prophylactic tool against breast

Conservative (nonoperative) treatment

Compression therapy has been recognized as an effective treatment in the management of venous and lymphatic disease for thousands of years.[11], [12], [13], [14], [15], [16], [17] Until recently, evidence of efficacy was based on empirical study and little objective research had been performed. More, detailed information has now been obtained from published clinical series and randomized controlled trials. There is obviously an upper limit beyond which further increase of compression pressure

Conclusions

Lymphedema is usually a chronic disorder with a severe impact on the quality of life. Instrumental diagnosis and follow-up has recently been simplified with the launch of near-infrared fluorescent imaging visualization of lymphatics. The available conservative and interventional treatment options do not allow a complete cure of the disease; however, new techniques are under development. The introduction of gene therapy in human application might anticipate a breakthrough in lymphedema therapy.

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      The International Society of Lymphology recommends monitoring the affected limb for changes in size or appearance, maintenance of an ideal body weight, limb elevation, prevention of infection, and the avoidance of limb constriction.9 Further therapy emphasizes techniques to move excess fluid into circulation, including compression bandages, manual lymphatic drainage, complete decongestive therapy, and intermittent pneumatic compression.10 In general, there is a limited role for pharmacotherapy, including diuretics, with the exception of antibiotics for recurrent infection.9

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