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Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Validation of the Ruptured Arteriovenous Malformation Grading Scale in a pediatric cohort
Joseph H Garcia1, Caleb Rutledge1, Ethan A Winkler1
11Department of Neurological Surgery, University of California, San Francisco.
Insights
The Ruptured Arteriovenous Malformation Grading Scale (RAGS) effectively predicts outcomes in pediatric patients with ruptured brain arteriovenous malformations (AVMs). This validated scale improves upon existing methods for assessing hemorrhage severity in children.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Medical Imaging
Background:
- Pediatric brain arteriovenous malformations (AVMs) are a significant cause of spontaneous intracranial hemorrhage (SICH) in children, leading to considerable morbidity.
- The Ruptured Arteriovenous Malformation Grading Scale (RAGS) was developed to predict clinical outcomes post-AVM rupture, analogous to the Hunt and Hess (HH) grade for aneurysms.
- Pediatric patients were excluded from the initial RAGS validation study, necessitating evaluation of its applicability in this demographic.
Purpose of the Study:
- To validate the RAGS score in a cohort of pediatric patients who experienced AVM rupture and hemorrhage.
- To assess the generalizability and expand the external validity of the RAGS score in the pediatric population.
- To compare the predictive performance of the RAGS score against established grading systems like the HH classification and Glasgow Coma Scale.
Main Methods:
- Retrospective review of a cohort of 81 children with ruptured AVMs.
- Utilized the modified Rankin Scale (mRS) to measure disability as the outcome variable.
- Calculated the area under the receiver operating characteristic curve (AUROC) for RAGS scores at three time points and compared it with HH and Glasgow Coma Scale scores.
Main Results:
- The RAGS score demonstrated strong predictive capability for mRS scores in pediatric patients.
- AUROC values for the RAGS score were consistently high at 0.81, 0.82, and 0.81 across three follow-up periods.
- The RAGS score outperformed the HH classification and Glasgow Coma Scale in predicting clinical outcomes.
Conclusions:
- The RAGS score is a reliable tool for predicting clinical outcomes in pediatric patients following brain AVM rupture.
- Further multi-center validation studies are recommended to confirm the broad generalizability of the RAGS scoring system.
- The RAGS score offers a valuable addition to the assessment of ruptured AVMs in children.
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.
