PT - JOURNAL ARTICLE AU - W. Schurch AU - F. H. Messerli AU - J. Genest AU - R. Lefebvre AU - P. Roy AU - P. Carter AU - J. M. Rojo-Ortega TI - Arterial hypertension and neurofibromatosis: renal artery stenosis and coarctation of abdominal aorta DP - 1975 Nov 08 TA - Canadian Medical Association Journal PG - 879--885 VI - 113 IP - 9 4099 - http://www.cmaj.ca/content/113/9/879.short 4100 - http://www.cmaj.ca/content/113/9/879.full SO - CMAJ1975 Nov 08; 113 AB - A 10-year-old girl had arterial hypertension, generalized neurofibromatosis, coarctation of the abdominal aorta and multiple stenoses at the origin of each renal artery. After resection of the stenotic areas and reimplantation of the renal arteries in the aorta, her arterial pressure decreased substantially. However, hypertension recurred and radiologic follow-up 4 1/2 years later showed distinct progression of the coarctation and renewed stenosis of all renal arteries at their origin. The stenotic areas showed eccentric intimal proliferation, frequently bulging into the lumen, with small nodular aggregates of smooth muscle cells and proliferation of fibrous tissue containing spindle-shaped nuclei in a palisading pattern. Hypertension associated with neurofibromatotic vascular disease has been described in 47 other patients in the literature. These patients have been young (mean age, 14 years) and predominantly male. In contrast to fibromuscular dysplasia, in which 95% of all stenoses are found in the distal two thirds of the renal arteries, in vascular neurofibromatosis more than 50% of the stenoses are found at the origin.