Retrospective cohort study reveals brain MRI abnormalities in 30% of pediatric sickle cell patients, indicating a protective association with hydroxyurea therapy.
Background: Sickle cell anemia (SCA) is a severe healthcare issue in Saudi Arabia. A considerable proportion of SCD patients may experience neurological complications over their lifetime, including infarction, silent ischemia, intracranial hemorrhage, then brain atrophy, and cognitive impairment. Aim: To identify the clinical and brain-imaging findings in SCD subjects with suspected neurological complications in Saudi Arabia and the impact of Hydroxyurea (HU) and chronic transfusion program. Methods: This cohort was undertaken at King Fahad Medical City, Riyadh, between October 2022 and September 2023. A total of 100 consecutive patients aged 2–18 years with homozygous sickle cell disease were included. All subjects underwent an MRI. Result: Abnormal brain MRI was found among 30% of the subjects, and the most common abnormal findings were SCI (16%) and CVA + moyamoya (9%). Of the patients, 46% received HU, and 25% received chronic transfusion programs. The HU group has less CVA + Moya-Moya and less SCI (p = 0.002) and tended to have normal TCD (p = 0.001). TCD was commonly abnormal among 66.7% of patients with CVA + moyamoya and 56.3% of SCI patients (p < 0.001). Conclusion: Neurologic sequences of SCD are a major health issue in Saudi Arabia and impact children’s quality of life. The most common complications were SCI and CVA combined with moyamoya.
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