[Williams-Beuren syndrome and renovascular arterial hypertension]

The Williams and Beuren's syndrome associates a particuliar facies, a mental debility, a supravalvular aortic stenosis, and frequent vascular anomalies of pulmonary artery as well as aorta and its branches. Relations between this syndrome and congenital idiopathic hypercalcemia seem now determined; the hypertension which has been frequently observed in this last case, seems more unusual in the case of Williams and Beuren's syndrome. The authors report a case about this syndrome in which the hypertension seemed linked to a stenosis of the left renal artery and which has been treated surgically. A year later the result is satisfactory but the evolutive potential of arterial lesions described in this syndrome does not necessory lead to a final recovery. Besides there are in this disease hypertensions which are relevant to an other etiology (more especially aortic hypoplasia) and stenosis of renal arteries without hypertension.