Prevalence of Stroke and Outcome in Relation to Risk Factors and Comorbidities in Children with Sickle Cell Disease

Abstract: BACKGROUND: Stroke is a major cause of morbidity in children with sickle cell disease (SCD). We aimed to characterize clinical and demographic features, risk factors, and outcomes of pediatric SCD patients who developed stroke at a tertiary center in Saudi Arabia. METHODOLOGY: We conducted a retrospective, single-center review of children (2–14 years) with confirmed SCD and overt stroke (first or recurrent) verified by neuroimaging. Extracted data included demographics, comorbidities (acute chest syndrome, vaso-occlusive crisis), stroke characteristics, imaging, acute management, and outcomes. RESULTS: Thirty-seven children were identified (mean age at stroke 6.9 years; 64.9% girls). Ischemic stroke predominated (91.9%); recurrence occurred in 32.4%. A family history of stroke was present in 24.3%. At presentation, hemoglobin was <8 g/dL in 73.0%. Eight patients had documented COVID-19 infection – three concurrent with stroke onset and five within the preceding months – suggesting both immediate and delayed thrombo-inflammatory effects. Although a formal transcranial Doppler (TCD) program exists, TCD was performed in only 21.6% and was abnormal in 37.5% of those tested. All patients received exchange transfusion acutely; 27.0% were on hydroxyurea before stroke, and poststroke hydroxyurea use rose to 86.5% (64.9% good adherence; 21.6% poor), with 13.5% remaining off therapy. Higher baseline platelet counts were associated with recurrence (mean 528.7 vs. 363.9 × 10 9 /L; P = 0.032). CONCLUSION: Pediatric SCD strokes occurred at a young age with substantial recurrence. Findings highlight implementation gaps – underutilized TCD and delayed hydroxyurea initiation – despite availability, and suggest closer surveillance for children with high platelet counts or recent systemic infection, particularly within regional and national sickle cell programs.