Characteristics and outcomes of stroke hospitalizations in patients with sickle cell disease and moyamoya syndrome

Objectives: Stroke is the leading cause of death in patients with Sickle cell disease (SCD). Here, we detail the burden of Moyamoya syndrome (MMS) as a cause of stroke in patients with SCD.

Materials and methods: A review of SCD-related hospital discharges was conducted utilizing the National Inpatient Sample. Rates of stroke hospitalization, risk factors, procedures, and outcomes were compared between patients with SCD-MMS and SCD alone. Univariate analyses including T-test, Wilcoxon Rank-Sum test, Chi-square were performed to compare risk factors and outcomes. Multivariable regression was used to identify predictors of stroke unique to each population.

Results: Stroke occurred in 9.8% of SCD-MMS hospitalizations versus 0.5% of those involving patients with SCD alone (OR = 20.71, p < 0.001). Patients with SCD-MMS developed stroke at younger ages and with fewer comorbidities compared to those with SCD alone. Stroke hospitalizations in SCD-MMS involved a greater number of procedures (90.5% vs. 79.3%, p = 0.007), but were more likely to result in favorable discharge (58.5% vs. 44.2%, p = 0.005). The presence of anemia during hospitalization was a significant risk factor for stroke in both cohorts. Long-term antiplatelet use was protective against stroke (OR = 0.42, p = 0.008) only in the SCD-MMS cohort.

Conclusions: MMS confers a 20-fold increased risk of stroke among patients with SCD and appears to be an important cause of recurrent stroke in this population. Anemia is one of the most significant risk factors for stroke, while antiplatelet use appears to confer a protective benefit.