Validation of the Ruptured Arteriovenous Malformation Grading Scale in a pediatric cohort

Joseph H. Garcia, Department of Neurological Surgery, University of California, San Francisco.
Caleb Rutledge, Department of Neurological Surgery, University of California, San Francisco.
Ethan A. Winkler, Department of Neurological Surgery, University of California, San Francisco.
Luis Carrete, Department of Neurological Surgery, University of California, San Francisco.
Ramin A. Morshed, Department of Neurological Surgery, University of California, San Francisco.
Alex Y. Lu, Department of Neurological Surgery, University of California, San Francisco.
Satvir Saggi, Department of Neurological Surgery, University of California, San Francisco.
Christine K. Fox, Pediatric Stroke and Cerebrovascular Disease Center, Department of Neurology, University of California, San Francisco.
Heather J. Fullerton, Pediatric Stroke and Cerebrovascular Disease Center, Department of Neurology, University of California, San Francisco.
Helen Kim, Center for Cerebrovascular Research, Department of Anesthesia and Perioperative Care, University of California, San Francisco.
Daniel L. Cooke, Department of Radiology and Biomedical Imaging, University of California, San Francisco.
Steven W. Hetts, Department of Radiology and Biomedical Imaging, University of California, San Francisco.
Michael T. Lawton, Department of Neurological Surgery, University of California, San Francisco.Follow
Nalin Gupta, Department of Neurological Surgery, University of California, San Francisco.
Adib A. Abla, Department of Neurological Surgery, University of California, San Francisco.

Document Type

Article

Abstract

OBJECTIVE: Pediatric brain arteriovenous malformations (AVMs) are the leading cause of spontaneous intracranial hemorrhage (SICH) in children. Although the incidence of SICH is low in pediatric populations, such events cause substantial morbidity. The recently created Ruptured Arteriovenous Malformation Grading Scale (RAGS) is proposed as a reliable and novel grading system to specifically serve as a predictor of clinical outcomes in patients following AVM rupture, similar to the Hunt and Hess (HH) grade for ruptured aneurysms. While these data are promising, pediatric patients were notably absent from the original study validating the RAGS. Therefore, correlation of the RAGS score with clinical outcomes following AVM rupture in individuals younger than 18 years of age using the RAGS score is needed. The objective of this study was to validate the RAGS in a cohort of pediatric patients with AVMs who presented with hemorrhage, thereby demonstrating the score's generalizability, and expanding its external validity. METHODS: A cohort of children with ruptured AVMs were retrospectively reviewed. Using disability, measured by the modified Rankin Scale (mRS), as the response variable, the area under the receiver operating characteristic curve (AUROC) was calculated for patients based on their RAGS scores for three time periods. The AUROC values were then compared with those generated by two commonly used clinical grading systems, the HH classification and Glasgow Coma Scale. RESULTS: A total of 81 children who presented with ruptured AVMs were included in the study, with a mean follow-up duration of 4 years. The RAGS score outperformed other clinical grading scales in predicting mRS scores, with AUROC values of 0.81, 0.82, and 0.81 at three distinct follow-up periods. CONCLUSIONS: The RAGS score correlated well with the clinical outcome after AVM rupture in pediatric patients. Additional validation studies across multiple treatment centers are needed to further demonstrate the generalizability of the scoring system.

Publication Date

2-25-2022

Publication Title

Journal of neurosurgery. Pediatrics

E-ISSN

1933-0715

First Page

1

Last Page

5

PubMed ID

35213838

Digital Object Identifier (DOI)

10.3171/2022.1.PEDS21466

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