• Maite E Houwing, Rowena L Grohssteiner, Dremmen Marjolein H G MHG Department of Pediatric Radiology, Erasmus MC - Sophia Children's Hospital, Rotterdam, The Netherlands., Ferdows Atiq, Wichor M Bramer, Anne P J de Pagter, C Michel Zwaan, Tonya J H White, Meike W Vernooij, and Marjon H Cnossen.
• Department of Pediatric Haematology and Oncology, Erasmus MC - Sophia Children's Hospital, NC-825, Wytemaweg 80, 3015 CN, Rotterdam, The Netherlands. m.houwing@erasmusmc.nl.
• Bmc Med. 2020 Dec 22; 18 (1): 393.

Background And PurposeSilent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences.MethodsThe databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019.ResultsThe search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSβ0 SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSβ0 genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSβ+ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association.ConclusionsThis systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.

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