The incidence and prevalence of unruptured intracranial aneurysms in sickle cell disease patients: a systematic review

Abstract

Background Unruptured Intracranial Aneurysms (UIAs) pose a significant risk of morbidity in the general population and much more so among sickle cell disease (SCD) patients. Meanwhile, the proportion of these patients with UIAs is not established just as the course and characteristics of the aneurysms are not well known. Aim To estimate the prevalence, incidence and characteristics of UIAs in SCD patients and compare same with the metrics and features in the general population. Methods The Data repositories, Medline (PubMed), Embase and Web of science were systematically searched from January 1st, 1990, to July 31st, 2021. Publications that passed an inclusion test were reviewed for data on the incidence and prevalence of UIAs, aneurysm characteristics and outcomes in SCD patients extracted. Findings from the included studies were appraised, using the Methodological Index for Non-randomized studies score (MINORS). The results were descriptively analysed. Given the marked heterogeneity of retrieved data, results were reported as standardized values, including the mean weighted annual incidence rate. Results 105 SCD patients with 186 UIAs were identified in 10 retrospective studies. Mean age ranged from 10.5 to 40.18 across studies with adult (>18 years) predominance. The prevalence of UIAs in SCD was 4.1% (95%CI 3.6 and 4.6) incidence rate was 1290.3/100,000 patient-years (95% CI 1018.0–1562.6). Aneurysms tended to be small (60%), anterior (76.1%), multiple (45.7%), and managed conservatively (62%) with mostly good outcomes (95%). The average MINORS score was 9.4 ± 3.1 for non-comparative studies (n = 8) and 19.5 ± 0.7 for comparative studies (n = 2). Conclusion UIAs have a definite relationship with SCD, with higher incidence figures relative to the general population. Aneurysmal characteristics although largely similar, tend to be smaller in SCD patients. The low methodological quality of reviewed studies informs the need for well-designed prospective randomized controlled studies to better understand the mechanics of this relationship.