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

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.