Pediatric Sickle-cell Disease-associated Moyamoya Syndrome: A Nationwide Analysis of Risk Factors Associated with In-hospital Complications

Introduction: Sickle-cell disease-associated moyamoya syndrome (SCD-MMS) carries a high risk for recurrent strokes and cerebrovascular morbidity in pediatric populations. Recent studies have suggested that surgical revascularization can reduce the rate of recurrent stroke in adults; however, an in-depth analysis of complications associated with SCD-MMS, particularly in the pediatric population, does not exist. Greater understanding of the risk factors for in-hospital complications in SCD-MMS will aid physicians in optimizing treatment plans.

Methods: A national database of pediatric hospital admissions was examined across the years 2003-2019. ICD-9 and ICD-10 diagnosis codes were analyzed to isolate discharges with a primary diagnosis of SCD-MMS. Patient demographics, comorbidities, and hospital characteristics were examined for associations with in-hospital complication incidence. Univariate and multivariate logistic regression analyses were performed.

Results: In total, 274 admissions for SCD-MMS were identified. Sixty-four (23.4%) admissions were associated with transfusion therapy, and 86 (31.4%) were associated with surgical revascularization. Ten of these SCD-MMS admissions (3.6%) experienced a total of eleven in-hospital complications. After multivariate regression, both comorbid chronic lung disease (8% incidence, adjusted OR 5.3 [1.1, 26.9], P=0.04) and surgical revascularization (7% incidence, adjusted OR 10.2 [2.0, 52.4], P=0.006) were associated with development of in-hospital complications. Among hospitalizations including revascularization, univariate regression demonstrated that only chronic lung disease was associated with development of complications (OR 7.43 [1.24, 44.61], P=0.03).

Conclusion : In this nationwide database of pediatric SCD-MMS hospitalizations, comorbid chronic lung disease and surgical revascularization were associated with development of in-hospital complications. The 7% in-hospital complication rate for revascularization should be discussed with patients, but may be tolerated given recent studies suggesting reduced rates of recurrent stroke after revascularization. Even so, patients with comorbid chronic lung disease are at high risk of complications with or without revascularization and may particularly benefit from medical optimization and close monitoring during their stay.