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International Angiology 2013 December;32(6):541-74


language: English

Diagnosis and Treatment of Primary Lymphedema. Consensus Document of the International Union of Phlebology (IUP)-2013

Lee B. B. 1, Andrade M. 2, Antignani P. L. 3, Boccardo F. 4, Bunke N. 5, Campisi C. 4, Damstra R. 5, Flour M. 6, Forner-Cordero J. 7, Gloviczki P. 8, Laredo J. 9, Partsch H. 10, Piller N. 11, Michelini S. 12, Mortimer P. 13, Rabe E. 14, Rockson S. 15, Scuderi A. 16, Szolnoky G. 17, Villavicencio J. L. 18

1 Center for Vein, Lymphatics, and Vascular Malformation, Division of Vascular Surgery, Department of Surgery, Georgetown University School of medicine, Washingto DC, USA; 2 Department of Surgery, University of São Paulo Medical School, São Paulo, Brazil; 3 Vascular Center, Villa Claudia, Rome, Italy; 4 Unit of Lymphatic Surgery, Dpt. of Surgery, Section of Lymphology and Microsurgery, University Hospital “S.Martino”, University of Genoa, Genoa, Italy; 5 Department of Dermatology, Phlebology and Lympho-vascular Medicine, Nij Smellinghe hospital, Drachten, Netherlands; 6 Vascular Centre,Multidisciplinary Diabetic Foot Clinic, University Hospital Leuven, Leuven, Belgium; 7 Lymphedema Unit, Rehabilitation Department, Hospital Universitario La Fe, Valencia, Spain; 8 Mayo Clinic College of Medicine, Chair, Division of Vascular and Endovascular Surgery, Director, Gonda Vascular Center, Mayo Clinic, Rochester, MN, USA; 9 Center for Lymphedema and Vascular Malformation, Division of Vascular Surgery, Department of Surgery, George Washington University School of Medicine, WashingtonWashington DC, USA; 10 Emeritus Head of the Dermatological Department of the Wilhelminen Hospital, Vienna, Austria; 11 Director Lymphoedema Research Unit, Department of Surgery, School of Medicine Flinders University and Flinders Medical Centre, Adelaide, South Australia; 12 Chief of Department of Vascular Rehabilitation San Giovanni Battista Hospital, Rome, Italy; 13 Clinical Science Division (Dermatology) St George’s, University of London, London, UK”; 14 Department of Dermatology, University of Bonn, Sigmund-Freud-Str. 25, D-53105 Bonn, Germany; 15 Allan and Tina Neill Professor of Lymphatic Research and Medicine, Director of the Stanford Center for Lymphatic and Venous Disorders, Chief of Consultative Cardiology, Stanford University School of Medicine, Stanford, CA, USA; 16 Santa Lucinda Hospital (Sorocaba – Brazil) – Pontificial Catholic University os Sao Paulo, Brazil, President of UIP; 17 Department of Dermatology and Allergology, University of Szeged, Szeged, Hungary; 18 Department of Surgery, Uniformed Services University School of Medicine, and Director Emeritus Venous and Lymphatic Teaching Clinic, Walter Reed Army Medical Center, Bethesda, MD, USA


Primary lymphedema can be managed effectively as a form of chronic lymphedema by a sequenced and targeted treatment and management program based around a combination of Decongestive Lymphatic Therapy (DLT) with compression therapy, when the latter is desired as an adjunct to DLT. Treatment in the maintenance phase should include compression garments, self-management, including self-massage, meticulous personal hygiene and skin care, in addition to lymphtransport-promoting excercises and activities, and, if desired, pneumatic compression therapy applied in the home. When conservative treatment fails, or gives sub-optimal outcomes, the management of primary lymphedema can be improved, where appropriate, with the proper addition of surgical interventions, either reconstructive or ablative. These two surgical therapies can be more effective when fully integrated with manual lymphatic drainage (MLD)-based DLT postoperatively. Compliance with a long-term commitment to MLD/DLT and particularly compression postoperatively is a critical factor in determining the success of any new treatment strategy involving either reconstructive or palliative surgery. The future of management of primary lymphedema has never been brighter with the new prospect of gene-and perhaps stem-cell oriented management.

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