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Intracranial Aneurysms and Marfan Syndrome
Quick Takes
- In a single-center, retrospective, observational study of patients with Marfan syndrome (MFS) who underwent brain imaging (including for neurological symptoms), there was an approximate 12% prevalence of intracranial aneurysm (ICA).
- Compared to people without MFS who underwent brain imaging and were considered to be a control group, detected ICAs among MFS patients were larger, more frequently located in the vertebrobasilar artery, and more often fusiform in morphology.
Study Questions:
What are the characteristics of intracranial aneurysm (ICA) detected among patients with Marfan syndrome (MFS)?
Methods:
In a single-center retrospective study, 118 patients with MFS who underwent brain imaging between 2007 and 2020 were identified and classified based on the presence or absence of ICA. Patients with MFS were included if they underwent computed tomography angiography (CTA) of the brain vessels with an indication of MFS, or if they underwent brain CTA or magnetic resonance angiography (MRA) for any neurological symptoms. Demographic and clinical data were acquired from medical records. Two readers independently reviewed all brain images regarding the presence, morphology, size, and location of an ICA. ICA characteristics among patients with MFS were compared to ICAs found in a control group of individuals who paid for their own brain scan at the same tertiary hospital between 2011 and 2012.
Results:
A total of 15 ICAs were detected among 14 patients with MFS, yielding a prevalence of 11.9%. Compared to MFS patients without ICA, MFS patients with ICA were older (44 ± 12 years with ICA vs. 37 ± 14 years without ICA, p = 0.04) and more often women (71% with ICA vs. 43% without ICA, p = 0.047). All ICAs were unruptured, and during the available follow-up interval (mean 54 ± 43 months), there was no record of subarachnoid hemorrhage. Among the control group of 2,118 people who paid for a brain scan, 95 ICAs were detected among 79 people who were taken to be healthy. The mean ICA diameter was larger among patients with MFS than in the control group (4.2 ± 1.8 mm vs. 3.0 ± 1.6 mm, p = 0.004). ICAs among patients with MFS more frequently were located in the vertebrobasilar artery (33% in MFS vs. 2.1% in the control group, p = 0.002) and more common of fusiform morphology (13% in MFS vs. 1.1% in the control group, p = 0.048).
Conclusions:
The authors concluded that this large cohort study demonstrated a high prevalence and different features of ICA among patients with MFS, which they believe may support an association between ICA and MFS.
Perspective:
This single-center, retrospective, observational study found an approximate 12% prevalence of ICA among patients with MFS. However, because patients with MFS were included in the analysis if brain imaging was performed for neurological symptoms, this might not represent the prevalence of ICA among an unselected (asymptomatic) population of patients with MFS. The control group is similarly problematic, having been selected based on self-payment for brain imaging, with retrospective assessment of overall health; based on data available in this manuscript, the prevalence of ICA among a healthy control population is unknown. Although it is interesting that the location and morphology of ICA among patients with MFS were different than in the control group, additional study would be necessary to define the true incidence of ICA among patients with MFS, and whether ICA among patients with MFS is different compared to a matched control group without MFS.
Clinical Topics: Cardiovascular Care Team, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Vascular Medicine, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Angiography, Computed Tomography, Magnetic Resonance Imaging, Nuclear Imaging
Keywords: Coronary Angiography, Diagnostic Imaging, Heart Defects, Congenital, Intracranial Aneurysm, Magnetic Resonance Angiography, Medical Records, Neuroimaging, Neurologic Examination, Prevalence, Subarachnoid Hemorrhage, Tertiary Care Centers, Tomography, X-Ray Computed, Vascular Diseases
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