RENOVASCULAR HYPERTENSION IN WILLIAMS-BEUREN-SYNDROME

Patients with Williams-Beuren-Syndrome characterized by the combinatio n of typical morphology, supravalvular aortic stenosis and mental reta rdation. The typical cardiac malformation,the supravalvular aortic ste nosis may be absent. Progressive peripheral vascular lesions as renal artery stenosis, coronary vascular abnormalities and cerebrovascular i schemia were not commonly observed in the past. Case report: We presen t a 12 year old girl with Williams-Beuren-Syndrome without typical sig ns of cardiac involvement who developed renovascular hypertension afte r 9 years of follow up due to stenosis of a renal artery. It was succe ssfully treated by aorto renal bypass. Discussion: In the absence of t ypical cardiac involvement Patients with Williams-Beuren Syndrome can develop progressive vascular lesions as renal artery stenosis,coronary artery stenosis or cerebro-vascular lesions. Therefore irrespective o f cardiac involvement patients with Willliams-Beuren syndrome should b e followed up with special regards to this complication.