A new systematic review published in the British Journal of Neurosurgery has found that patients with sickle cell disease (SCD) have a higher incidence of unruptured intracranial aneurysms (UIAs) than the general population.

The study noted that the reviewed studies tended to have poor methodological quality, highlighting the need for well-designed randomized trials to better understand the relationship between UIAs and SCD.

“UIAs are defined as abnormal dilatation on the arterial wall of the cerebral vessels and current estimates put the numbers at just 3.2% of adults,” the authors explained. “The presence of morphological, pathological, and racial risk factors that coexist with SCD can influence rupture rates, justifying the need for a radiological surveillance protocol that can be structured based on the incidence and prevalence of UIAs in this population.”

The authors conducted a literature review of English language articles on patients with SCD and confirmed radiological findings of UIAs. Ten articles ultimately fulfilled the inclusion criteria and were analyzed.

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Due to the wide variability and heterogeneous nature of the studies, the team standardized incidence rates using a common denominator; however, incidence rates of UIAs still varied widely by study. It was clear that incidence rates were higher in this patient group, at approximately 4.1% compared to 3.2% in the general population.

Most of the aneurysms in this patient group were small (<3 mm) and were treated conservatively with radiological monitoring. Most remained stable over time and maintained the same characteristics of UIAs in the general population in terms of their location, multiplicity, and size.

The authors caution that the studies’ heterogeneity and small sample sizes limit the ability to draw firm conclusions and that there is a need for larger, well-designed multicenter trials exploring the association between UIAs and SCD.


Essiet E, Shapey J, Kailaya-Vasan A. The incidence and prevalence of unruptured intracranial aneurysms in sickle cell disease patients: a systematic review. Br J Neurosurg. Published online August 8, 2022. doi:10.1080/02688697.2022.2104217