Arterial Stiffness in a Toddler with Neurofibromatosis Type 1 and Refractory Hypertension

Figure 1

CTA at diagnosis showed severe segmental aortic stenosis arising before the origin of the upper mesenteric artery extending for a length of 3–4 cm with lumen diameter of 0.2–0.3 cm at the narrowest part and a poststenotic diameter of 0.6–0.7 cm. Two right renal arteries were demonstrated, both with ostial stenosis, as well as an auxiliary stenotic artery perfusing the lower pole of the right kidney. Left renal artery also presented ostial stenosis. Furthermore, an enlarged riolan arcade resulted in an increased blood flow to the upper mesenteric artery from the lower mesenteric artery providing collateral circulation.