CTICasesAbdominal Aortic Stenosis in Neurofibromatosis Type 1
[...]. Abdominal Aortic Stenosis in Neurofibromatosis Type 1. CTICases. Published January 27, 2022. https://str-case-explorer.pages.dev/c/4sauh
Imaging findings
CT angiography shows severe, focal narrowing of the suprarenal abdominal aorta (mid-aortic syndrome) in a young patient. Extensive paraspinal soft tissue masses representing plexiform neurofibromas are noted along the sympathetic chain and vagus nerve roots.
Key takeaways
Neurofibromatosis type 1 (NF1) is associated with vascular manifestations, including renal artery stenosis and abdominal/thoracic coarctation (mid-aortic syndrome). NF1 should be considered in the differential diagnosis of early-onset hypertension in young patients alongside Williams syndrome and Takayasu arteritis.
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