Risk factors associated with in-hospital complications for pediatric sickle-cell disease-associated moyamoya syndrome: a nationwide cross-sectional study

Robert C Osorio; Kunal P Raygor; Lorenzo Rinaldo; Christine K Fox; Neha Bhasin; Adib A Abla; Nalin Gupta

doi:10.1007/s00381-024-06363-2

Risk factors associated with in-hospital complications for pediatric sickle-cell disease-associated moyamoya syndrome: a nationwide cross-sectional study

Childs Nerv Syst. 2024 Mar 26. doi: 10.1007/s00381-024-06363-2. Online ahead of print.

Authors

Robert C Osorio¹, Kunal P Raygor¹, Lorenzo Rinaldo¹, Christine K Fox², Neha Bhasin³, Adib A Abla¹, Nalin Gupta^{4

5}

Affiliations

¹ Department of Neurological Surgery, University of California San Francisco, 550 16th Street, 5th Floor, San Francisco, CA, USA.
² Department of Neurology, University of California San Francisco, San Francisco, CA, USA.
³ Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA.
⁴ Department of Neurological Surgery, University of California San Francisco, 550 16th Street, 5th Floor, San Francisco, CA, USA. nalin.gupta@ucsf.edu.
⁵ Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA. nalin.gupta@ucsf.edu.

PMID: 38530413
DOI: 10.1007/s00381-024-06363-2

Abstract

Purpose: Sickle-cell disease-associated moyamoya syndrome (SCD-MMS) carries a high risk for recurrent strokes and cerebrovascular morbidity in children. However, few data are available about complications that occur in children hospitalized with SCD-MMS. The purpose of this analysis was to determine the risk factors for in-hospital complications in pediatric SCD-MMS admissions, and thus aid physicians in optimizing future 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 identify discharges with a primary diagnosis of SCD-MMS and identify in-hospital complications, defined as complication-associated diagnostic codes logged during the same admission. Patient demographics, comorbidities, and hospital characteristics were examined using univariate and multivariate logistic regression analyses to determine associations with in-hospital complications.

Results: In total, 274 admissions with a primary diagnosis of SCD-MMS were identified. During 64 (23.4%) admissions, transfusion therapy was given, and in 86 admissions (31.4%), surgical revascularization was performed. In 10 admissions (3.6%), a total of 11 in-hospital complications were identified. After multivariate regression, both comorbid chronic lung disease (adjusted OR 5.3 [1.1, 26.9], P = 0.04) and surgical revascularization (adjusted OR 10.2 [2.0, 52.4], P = 0.006) were associated with development of complications.

Conclusions: In this nationwide database of pediatric SCD-MMS hospitalizations, comorbid chronic lung disease and surgical revascularization were associated with development of in-hospital complications. Patients with comorbid chronic lung disease or who are admitted for revascularization may warrant closer monitoring and greater medical optimization during the hospitalization.

Keywords: Complications; Moyamoya syndrome; Pediatrics; Sickle-cell disease.