ABSTRACT
Klippel-Trenaunay syndrome is a complex disease still missing proper pathophysiological comprehension. The first concern is a comprehensive diagnostic workup in multidisciplinary settings to identify and accurately characterize all the lesions involved; to absolutely exclude arterial flow; to carefully evaluate the deep venous system for competence, patency, deep vein thrombosis, aplasia, and hypoplasia, and the superficial venous system for valvular competence/reflux and/or the presence of the marginal vein/embryonic vein; as well as to assess the coagulopathic status of a patient. Following this comes the second concern, that is, to create no harm with the management of the venous component, and in this sense, an attempt in favor of a conservative treatment must always be considered. At the same time, the genetic nature of the disease urges an adequate lifestyle after an eventual vein procedure. Endovenous techniques now offer the chance for minimally invasive approaches, confining the open surgical option to a limited number of cases not suitable for thermal ablation or sclerotherapy treatment. Data in the literature show the benefits of the venous procedure only after failed conservative treatment following a proper diagnostic and therapeutic strategy workflow, rigorously developed by multidisciplinary expert teamwork.
