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Published on: March 14, 2017
Intracranial Aneurysms in Sickle Cell Disease
Ramazan Jabbarli1, Thiemo F Dinger1, Daniela Pierscianek1
1Department of Neurosurgery, University Hospital of Essen, Essen, Germany.
Insights
Intracranial aneurysms (IAs) in sickle-cell disease (SCD) patients present differently than in the general population, often appearing younger and more numerous. Rupture risk factors in SCD patients largely align with those in healthy individuals.
Area of Science:
- Neurology
- Vascular Surgery
- Hematology
Background:
- Intracranial aneurysms (IAs) etiology remains unclear, though associations with certain diseases exist.
- Sickle-cell disease (SCD) is a known condition linked to increased IA risk.
Purpose of the Study:
- To compare the demographic and radiographic characteristics of intracranial aneurysms (IAs) in patients with sickle-cell disease (SCD) versus the general population.
- To identify specific risk factors for IA rupture in individuals with SCD.
Main Methods:
- Systematic literature search of PubMed, Scopus, Web of Science, and Cochrane Library for studies on SCD patients with IAs.
- Comparison of IA characteristics between 129 SCD patients and 2451 healthy IA carriers from an observational cohort.
Main Results:
- SCD patients with IAs were younger (mean 27.1 vs 54.9 years) with a lower female prevalence.
- Multiple IAs were more prevalent (47% vs 34.5%) and numerous (3.02 vs 2.56 IAs/patient) in the SCD cohort.
- IAs were smaller in SCD patients (unruptured: 3.27 vs 6.16 mm) and more frequently located in the internal carotid artery or posterior circulation.
Conclusions:
- Intracranial aneurysms in SCD patients exhibit distinct demographic and radiographic patterns compared to the general population.
- Key risk factors for IA rupture in SCD, including age, size, and location, are similar to those observed in healthy individuals.
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 were 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.
Conclusion:
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.
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