Significance: Lymphedema is a common, distressing and debilitating condition with various etiologies. Effective diagnosis, assessment, and management rely on evidence-based clinical practice guidelines ("guidelines"). This study aims to describe and compare international guidelines on lymphedema diagnosis, assessment, and management. Recent Advances: The review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and registered on the International Prospective Register of Systematic Reviews (PROSPERO). Systematic searches of electronic literature databases and the web were completed in December 2020 for lymphedema guidelines published in English since 2000. Quality was assessed using the Appraisal of Guidelines for Research and Evaluation (AGREE)-II reporting checklist. Synthesis took a narrative approach to compare guideline recommendations and associated levels of evidence. Critical Issues: This systematic review of 1,564 articles and 159 web pages yielded 14 guidelines. All guidelines were from high-income countries. Ten focused exclusively on lymphedema, and four on cancer. Most (n = 13) guidelines recommended an integrated medical, psychological assessment, and physical examination, with a limb volume measurement of >10% in the affected limb compared, confirming a lymphedema diagnosis. Recommended management involved Complex Decongestive Therapy (CDT) followed by self-management using skincare, self-lymphatic drainage massage, exercise, and compression. Future Directions: The underlying etiology of lymphedema appeared to make little difference to guideline recommendations regarding care. High-quality guidelines are available to guide lymphedema care. However, their suitability for low-resource settings is unclear.
Keywords: complex decongestive therapy; lymphedema; manual lymphatic drainage; practice guidelines; systematic review.