Schürch W, Messerli F H, Genest J, Lefebvre R, Roy P, Carter P, Rojo-Ortega J M
Can Med Assoc J. 1975 Nov 8;113(9):879-85.
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.
一名10岁女孩患有动脉高血压、全身性神经纤维瘤病、腹主动脉缩窄以及每条肾动脉起始处的多处狭窄。在切除狭窄区域并将肾动脉重新植入主动脉后，她的动脉压大幅下降。然而，高血压复发，4年半后的放射学随访显示缩窄明显进展，且所有肾动脉起始处再次出现狭窄。狭窄区域可见偏心性内膜增生，常向管腔内突出，伴有平滑肌细胞的小结节状聚集以及呈栅栏状排列的含梭形核的纤维组织增生。文献中还描述了其他47例与神经纤维瘤病性血管疾病相关的高血压患者。这些患者较为年轻（平均年龄14岁），且以男性为主。与纤维肌性发育异常不同，纤维肌性发育异常中95%的狭窄发生在肾动脉远端三分之二处，而在血管性神经纤维瘤病中，超过50%的狭窄发生在起始处。
