CTICasesMiddle Aortic Syndrome
Imaging findings
Contrast-enhanced chest CT in a patient with neurofibromatosis type 1 (NF1) and scoliosis demonstrates multiple paraspinal, mediastinal, and intercostal neurofibromas. There is severe, focal narrowing and hypoplasia of the abdominal aorta (middle aortic syndrome), which is bypassed by a surgical jump graft. The celiac artery is occluded proximally and reconstituted via collateral pathways.
Key takeaways
Middle aortic syndrome is a rare vascular disorder characterized by segmental narrowing of the distal thoracic and abdominal aorta, often involving the renal and visceral branches. It can be congenital or acquired, and is associated with neurofibromatosis type 1 (NF1), Williams syndrome, and Takayasu arteritis. In NF1, the narrowing is caused by intimal proliferation and fibromuscular dysplasia unique to the disease, leading to renovascular hypertension.
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