Presentation
Hypertension and renal failure.
Patient Data
Age: 12 years
Gender: Female
From the case:
Williams syndrome with midaortic syndrome
Frontal
Cardiac silhouette is mildly enlarged. Absent aortic arch and descending aorta silhouette.
From the case:
Williams syndrome with midaortic syndrome
Axial C+ arterial phase
The descending thoracic and abdominal aorta is very small caliber. Origins of the renal arteries, celiac trunk and superior mesenteric arteries are also narrowed. Enlarged arc of Riolan.
Case Discussion
Williams syndrome can be a cause of midaortic syndrome via an elastin arteriopathy in approximately 50% of patients. Midaortic syndrome is a rare cause (0.5-2%) of aortic coarctations. There is severe narrowing of the abdominal aorta, often with involvement of the celiac trunk and superior mesenteric artery (~30%) and renal arteries (90%).
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