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Intracranial Aneurysms in Patients with Marfan Syndrome: A Multicenter Propensity-Matched Analysis

Ricardo A Domingo1, Carlos Perez-Vega1, Shashwat Tripathi2

  • 1Department of Neurologic Surgery, Mayo Clinic, Jacksonville, Florida, USA.

World Neurosurgery
|August 23, 2021
PubMed
Abstract

Insights

Marfan syndrome patients with a history of coronary artery disease and current smoking status face an increased risk of intracranial aneurysms (IAs). Neurovascular screening is recommended for all Marfan syndrome patients, especially those with these risk factors.

Area of Science:

  • Cardiovascular Research
  • Neurology
  • Genetics

Background:

  • Marfan syndrome (MFS) is a genetic disorder affecting connective tissue, increasing the risk of aortic and cardiovascular complications.
  • Intracranial aneurysms (IAs) are a less commonly recognized complication in MFS patients.
  • Identifying risk factors for IA in MFS is crucial for early detection and management.

Purpose of the Study:

  • To determine the prevalence and risk factors associated with intracranial aneurysms (IAs) in patients diagnosed with Marfan syndrome (MFS).
  • To characterize the clinical presentation and management of IAs in this patient population.

Main Methods:

  • Retrospective analysis of 159 Marfan syndrome patients with brain imaging between 1980 and 2020.
  • Comparison of patients with and without intracranial aneurysms using logistic regression and propensity score matching.
  • Evaluation of various clinical factors, including smoking status, coronary artery disease, hypertension, and diabetes.

Main Results:

  • The prevalence of intracranial aneurysms (IAs) in the studied Marfan syndrome (MFS) cohort was 11.3%.
  • Current tobacco smoking (OR: 4.20) and a history of coronary artery disease (CAD) (OR: 5.79) were significantly associated with increased odds of IA.
  • After propensity score matching, history of CAD (61.1% vs. 22.2%) and current smoker status (33.3% vs. 0%) remained significantly higher in the IA group.

Conclusions:

  • Intracranial aneurysms (IAs) occur in 11.3% of Marfan syndrome (MFS) patients, with a 5.5% rate of de novo formation.
  • A history of coronary artery disease (CAD) and current smoking are significant risk factors for IA development in MFS.
  • Routine neurovascular screening is advised for all MFS patients, with particular emphasis on those who smoke or have a history of heart disease.
Keywords:
AneurysmEndovascularHemorrhageIntracranialMarfanSmokingSurgery

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