Summary

The Klippel-Trénaunay syndrome (KTS) is a congenital disorder of vascular malformations characterized by the clinical trias of naevi flammei, varicosis and hypertrophy of the affected limb. According to its clinical development KTS can be diagnosed directly post partum or becomes apparent in the course of the development particularly regarding incomplete forms. The most described symptoms next to swelling are pain and augmented sweating of the affected extremity. Complications of KTS are bleeding from mechanically traumatised vascular malformations or widened vessels, but also thromboembolic events are reported. We report on two patients with KTS and one patient with angiodysplasia, who developed deep vein thromboses in the affected limb at young age. None of the patients suffered from thrombophilia or other predisposing risk factors for thrombosis. KTS and other angiodysplastic disorders seem to be a risk factor for thromboembolic complications even at young age. A comprehensive counceling of the patients concerning the prevention of complications and especially the symptoms of thromboembolic events is important for early diagnosis and immediate therapy.