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International Angiology 2010 October;29(5):454-70


language: English

Diagnosis and treatment of primary lymphedema. Consensus Document of the International Union of Phlebology (IUP)-2009

Lee B. 1, Andrade M. 2, Bergan J. 3, Boccardo F. 4, Campisi C. 4, Damstra R. 5, Flour M. 6, Gloviczki P. 7, Laredo J. 8, Piller N. 9, Michelini S. 10, Mortimer P. 11, Villavicencio J. L. 12

1 Center for Vein, Lymphatics, and Vascular Malformation, Division of Vascular Surgery, Department of Surgery, Georgetown University School of Medicine, Washington DC, USA; 2 Department of Surgery, University of São Paulo Medical School, São Paulo, Brazil; 3 Department of Surgery, UCSD School of Medicine, University of California, San Diego, CA, USA; 4 Unit of Lymphatic Surgery, Department 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 Department of Dermatology, Vascular Centre, Multidisciplinary Diabetic Foot Clinic, University Hospital Leuven, Leuven, Belgium; 7 Division of Vascular and Endovascular Surgery and Gonda Vascular Center, Mayo Clinic, Rochester, MN, USA; 8 Center for Vein, Lymphatics, and Vascular Malformation, Division of Vascular Surgery, Department of Surgery, Georgetown University School of medicine, Washington DC, USA; 9 Lymphoedema Assessment Clinic, Department of Surgery, School of Medicine Flinders University, South Australia; 10 Department of Vascular Rehabilitation, San Giovanni Battista Hospital, Rome, Italy; 11 Dermatological Medicine, Cardiac and Vascular Sciences (Dermatology) St George’s, University of London, London, UK; 12 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 safely as one of the chronic lymphedemas by a proper combination of DLT with compression therapy. Treatment in the maintenance phase should include compression garments, self management including the compression therapy, self massage and meticulous personal hygiene and skin care in addition to lymph-transport promoting excercises. The management of primary lymphedema can be further improved with proper addition of surgical therapy either reconstructive or ablative. These two surgical therapies can be effective only when fully integrated with MLD-based DLT postoperatively. Compliance with a long-term commitment of DLT postoperatively is the most critical factor determining the success of any new treatment strategy with either reconstructive or palliative surgery. The future of management of primary lymphedema caused by truncular lymphatic malformation has never been brighter with the new prospect of gene-oriented management.

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