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

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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.

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Data availability

Data is available through the Kids’ Inpatient Database (KID), a large pediatric inpatient database curated every 3–4 years by the Healthcare Cost and Utilization Project (HCUP) and created by the Agency for Healthcare Research and Quality. Data included in this study was aggregated from the KID across the years 2003–2019.

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Acknowledgements

The authors would like to acknowledge Donna M. Hays for her assistance in identifying appropriate diagnosis and procedure codes for use in this study.

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Authors and Affiliations

  1. Department of Neurological Surgery, University of California San Francisco, 550 16th Street, 5th Floor, San Francisco, CA, USA

    Robert C. Osorio, Kunal P. Raygor, Lorenzo Rinaldo, Adib A. Abla & Nalin Gupta

  2. Department of Neurology, University of California San Francisco, San Francisco, CA, USA

    Christine K. Fox

  3. Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA

    Neha Bhasin & Nalin Gupta

Authors
  1. Robert C. Osorio
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by RCO, KPR and NG. The first draft of the manuscript was written by RCO and KPR, and all authors edited and commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Nalin Gupta.

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This is an observational study using publicly available, anonymized data. IRB approval was not necessary for use of this de-identified dataset.

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Osorio, R.C., Raygor, K.P., Rinaldo, L. et al. Risk factors associated with in-hospital complications for pediatric sickle-cell disease-associated moyamoya syndrome: a nationwide cross-sectional study. Childs Nerv Syst (2024). https://doi.org/10.1007/s00381-024-06363-2

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