As is known, 1900 Klippel and Trenaunay summarized naevus teleangiectatisuc lateralis, vein ectasias and isolated gigantism to a triad. Seven years later Weber described the same syndrom adding the symptom of arterio-venous fistulas, that he clinically diagnosed. Some authors deduce from this symptomatology two syndroms ; on one hand the Klippel and Trenaunay syndrom and on the other the P. F. Weber-syndrom. As it is shown, in spite of an immense variability of symptoms, arterio-venous short circuits can regularly be demonstrated-clinically, arteriographically or histomorphologically. In addition to this some casuistical examples are presented. Referring to this reports the authors do not agree on separation of Klippel-Trenaunay and Weber syndrom. Considering to therapeutical consequences -- namely surgical -- it should be always cleared up angiographically if hemodynamically relevant arterio-venous fistulas are present. (Due to this fact a divergence to Weber's characterization is given). Relative to the authors opinion the presence of hemodynamically and so therapeutically relevant or obviously hemodynamically non-relevant arterio-venous fistulas does not allowed the separation in two syndroms. Out of this reason the authors nomenclature the described symptomatology as Klippel-Trenaunay-Weber-Syndrom.

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