A 12-year-old boy presented with hypertension and intermittent lower extremity claudication. Breath-hold high spatial resolution MRA at 3.0 T (gradient recalled echo sequence; 10 ml of Magnevist) revealed abdominal aortic coarctation (Fig. 1, thin long arrow), severely narrowed right renal artery and atrophy of the ipsilateral kidney (Fig. 1b, arrowhead), and hypertrophied inferior mesenteric artery (Fig. 1a, thick short arrow) feeding the superior mesenteric artery (Fig. 1a, thin short arrow) through the arc of Riolan (Fig. 1a, arrowhead). The child had previously undergone placement of an aorto-aortic graft (Fig. 1, thick long arrow).

Fig. 1
figure 1

3-D volume-rendered MR angiograms (Vitrea 3.6; Vital Images, Minneapolis, MN)

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Midaortic syndrome (MAS) is a rare disease caused by segmental narrowing of the abdominal or distal descending thoracic aorta associated with concomitant stenoses involving the renal (63%) and visceral (33%) arteries [1]. MAS can be congenital or acquired caused by giant cell or Takayasu arteritis, retroperitoneal fibrosis, Williams syndrome, fibromuscular dysplasia, neurofibromatosis, and mucopolysaccharidosis [1, 2]. Noninvasive diagnosis is made by MRA or CT angiography [2]. Surgical bypass grafting is the optimal method of treatment [1].