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Intracranial aneurysms in sickle cell disease: A systematic review and case-control study

Author(s):

Ramazan Jabbarli*, Thiemo Florin Dinger, Daniela Pierscianek, Marvin Darkwah Oppong, Bixia Chen, Philipp Dammann, Karsten H Wrede, Klaus Kaier, Martin Köhrmann, Michael Forsting, Christoph Kleinschnitz and Ulrich Sure  

Abstract:


Background: The exact causes of intracranial aneurysms (IAs) are still unknown. However, certain diseases are known to be associated with IAs.

Objective: To analyze the differences in IA characteristics in the general population and in individuals with sickle-cell disease (SCD).

Methods: We systematically searched PubMed, Scopus, Web of Science, and Cochrane Library for data on SCD patients with IAs. We compared IA characteristics of SCD patients with those from 2451 healthy IA carriers from our observational cohort.

Results: 129 SCD patients with IAs were identified in 42 studies. The SCD patient cohort was characterized by younger age (mean 27.1 vs 54.9 years, p<0.0001) and lower female prevalence (57.7% vs 68.4%, p=0.0177). The prevalence (47% vs 34.5%, p=0.004) and the number (3.02 vs 2.56 IAs/patient, p=0.004) of multiple IAs was also higher in the SCD cohort. Unruptured IAs (3.27 vs 6.16 mm, p<0.0001), but not ruptured IAs (7.8 vs 7.34 mm, p=0.9086) were significantly smaller in the SCD cohort. In addition, IAs were more frequently located in the internal carotid artery (45% vs 29%, p<0.0001) or posterior circulation (43% vs 20%, p<0.0001). Higher age (≥30 years, p=0.007), IA size ≥7 mm (p=0.008), and location in posterior circulation (p=0.01) were independently associated with subarachnoid hemorrhage in SCD.

Conclusions: There is a distinct demographic and radiographic pattern of IA in SCD. Risk factors for IA rupture in SCD are mostly congruent with those in healthy individuals.

Keywords:

intracranial aneurysm, subarachnoid hemorrhage, risk factors, sickle-cell disease, rupture, growth.

Affiliation:

Department of Neurosurgery, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen, Institute for Medical Biometry and Statistics, University of Freiburg, Clinic for Neurology, University Hospital of Essen, Institute for Diagnostic and Interventional Radiology, University Hospital of Essen, Clinic for Neurology, University Hospital of Essen, Department of Neurosurgery, University Hospital of Essen



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