I’m a 30-year-old woman. I’m taller than your average girl, probably thinner than your average girl, and more active than your average girl. Yeah I run an ice cream business for a living, but like to think I’m healthier than your average girl, too. No prior medical history. Nothing.
Observational studies and case reports published since the early 1980s show that patients with spontaneous internal carotid artery dissection may also have a history of stroke in their family and/or hereditary connective tissue disorders, such as Marfan syndrome, Ehlers-Danlos syndrome, autosomal dominant polycystic kidney disease, pseudoxanthoma elasticum, fibromuscular dysplasia, and osteogenesis imperfecta type I.
However, although an association with connective tissue disorders does exist, most people with spontaneous arterial dissections do not have associated connective tissue disorders. Also, the reports on the prevalence of hereditary connective tissue diseases in people with spontaneous dissections are highly variable, ranging from 0% to 0.6% in one study to 5% to 18% in another study.
Among 1,049 patients diagnosed with aortic dissection in a multinational registry, patients with Marfan's syndrome were typically younger and had unique presentations. Despite their younger age at presentation, patients with Marfan's syndrome and aortic dissection had a high mortality rate, similar to patients without Marfan's syndrome, an older patient cohort. Our data support the importance of aneurysm surveillance and prophylactic surgical intervention for patients with Marfan's syndrome to potentially reduce the risk of mortality.
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