CTICasesAneurysmal Aortic Dissection and Rupture in Marfan Syndrome
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[...]. Aneurysmal Aortic Dissection and Rupture in Marfan Syndrome. CTICases. Published March 26, 2014. https://cticases.com/c/bjjh9
Imaging findings
CT angiogram in a patient with Marfan syndrome shows a complex aortic dissection of the descending aorta with a near-completely collapsed true lumen. Serial imaging shows rapid expansion of the false lumen by one centimeter over two days, followed by acute rupture, manifest on radiography as apical capping and a massive, left-sided tension hemothorax.
Key takeaways
Patients with Marfan syndrome are at extremely high risk for aggressive aortic disease, including dissection and rapid aneurysmal expansion. Aortic dimensions can change dramatically over very short intervals, and rapid enlargement of the false lumen is a warning sign of impending catastrophic rupture and exsanguination into the pleural space.
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