[Klippel-Trenaunay syndrome and phacomatoses].
Several mechanisms can be invoked in order to explain the appearance in a limb of the characteristic triad : naevus, varices and osteodystrophy. Many cases can be explained by the haemodynamic disturbance associated with venous hypertension caused by a venous malformation combined with arteriovenous microcommunications. These are the true Klippel and Trenaunay Syndromes. In a few rare cases the haemodynamic disturbance is caused by one or more arteriovenous fistulae with major flows. These are the Parkes-Weber-Syndromes. Finally, in the more complex syndromes there is the combined triad of naevus, varices and osteodystrophy, together with other genetic disturbances. These are the Phacomatoses.
D000798:Angiomatosis / Q000503:physiopathology*; D001165:Arteriovenous Malformations / Q000150:complications; D001848:Bone Diseases, Developmental / Q000209:etiology; D005855:Germ Layers / Q000473:pathology*; D006439:Hemodynamics; D006801:Humans; D006973:Hypertension / Q000150:complications; D007715:Klippel-Trenaunay-Weber Syndrome / Q000196:embryology / Q000503:physiopathology*; D009506:Nevus / Q000196:embryology; D014648:Varicose Veins / Q000196:embryology; D014680:Veins / Q000002:abnormalities
Syndrome de Klippel et Trenaunay et phacomatoses.