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Italian Journal of Vascular and Endovascular Surgery 2021 March;28(1):42-9

DOI: 10.23736/S1824-4777.21.01500-X

Copyright © 2021 EDIZIONI MINERVA MEDICA

lingua: Inglese

Review on target treatment of Klippel-Trenaunay Syndrome

Byung-Boong LEE 1, Massimo VAGHI 2

1 Department of Surgery, Center for Lymphedema and Vascular Malformations, George Washington University, Washington, WA, USA; 2 Department of Vascular Surgery, Center for the Study and the Cure of Vascular Malformations, Garbagnate Milanese, Milan, Italy



Klippel-Trenaunay Syndrome (KTS) represents the archetype of vascular malformations and its eponym has been used for the individuation of practically all forms of vascular malformations. This is due to two reasons: the first is that the clinical picture of all different forms of vascular malformations represents the tip of an iceberg and the deep part may be discovered only using various diagnostic tools. The second is that it is a rare disease and also the physician treating this pathology are rare and have a difficulty to share the knowledge in the field of vascular malformations. KTS is a complex syndrome characterized by overgrowth of soft tissues, bone elongation or shortening, presence of skin naevus, anomalies in the superficial and deep veins, lymphatic vessels anomalies, visceral involvement. The patient may have all the described signs or the part but for the diagnosis of KTS the contemporary presence of at least two signs between tissue overgrowth, skin naevus and dilatation of superficial vein is mandatory. Regarding the hemodynamic aspects KTS is a slow-flow malformation on contrary to Parkes Weber Syndrome, which is a fast flow malformation, which remains the most crucial issue for the management. But such difficulty to differentiate clinically these two syndromes represents not only the lack of knowledge in this field in the first 80 years of the last century but also the lack of indifference. Hence proper understanding of its nature remains critical.


KEY WORDS: Klippel-Trenaunay-Weber syndrome; Vascular malformations; Lymphedema; Hypertrophy

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