title: Toward a comprehensive assessment of functional outcomes in pediatric patients with brain arteriovenous malformations: the Pediatric Quality of Life Inventory.
abstract: OBJECTIVE Brain arteriovenous malformations (bAVMs) are rare in pediatric patients but represent the most common cause of hemorrhagic stroke in this population. Pediatric patients demonstrate superior outcomes in comparison with adult patients with similar lesions and presentations. Most studies of clinical outcomes of pediatric bAVMs use the modified Rankin Scale (mRS), despite a lack of validation in pediatric patients. METHODS The authors interviewed the parents of 26 pediatric patients who underwent multimodality bAVM treatment and administered the Pediatric Quality of Life Inventory (PedsQL)-a well-validated tool for pediatric outcomes that quantifies performance in a physical, emotional, social, and school domains. They also reviewed clinical information from the patients' medical charts. Statistical analysis was performed using a log-transformed t-test, the Mann-Whitney exact test, the Kruskal-Wallis test, and Spearman correlation. In addition, the literature was reviewed for prior reports of clinical outcome of pediatric cases of bAVM. RESULTS The average PedsQL health-related quality of life score was 71 ± 24, with an average age at diagnosis of 12.5 years and an average follow-up period of 6.8 years. Seventeen patients (65%) presented with hemorrhage and 4 (15%) with seizures. PedsQL scores correlated strongly and at a statistically significant level (p < 0.001) with mRS, Pediatric Overall Performance Category (POPC), Pediatric Cerebral Performance Category (PCPC), and Glasgow Outcome Scale scores. Multivariate modeling validated special education, corrective devices, and cure status as significant predictors of PedsQL scores. Statistically significant risk factors for undergoing placement of a ventriculoperitoneal shunt included lower Glasgow Coma Scale motor scores on admission (p = 0.042), cerebellar location (p = 0.046), and nidus volume (p = 0.017). Neither treatment modality nor location statistically affected clinical outcomes at follow-up. CONCLUSIONS There have been few studies of long-term clinical outcomes of bAVM in pediatric patients, and previously published studies have used conventional metrics that have been validated in the adult population, such as the mRS. Although these metrics can serve as reasonable surrogates, an accurate understanding of overall health-related quality of life is contingent on utilizing validated toolsets, such as the PedsQL.
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Isaac Josh
authors.initials: IJ
authors.lastname: Abecassis
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: John D
authors.initials: JD
authors.lastname: Nerva
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Jason
authors.initials: J
authors.lastname: Barber
authors.affiliation: Radiation Oncology, and.
authors.forename: Jason
authors.initials: J
authors.lastname: Rockhill
authors.affiliation: Departments of 1 Neurological Surgery.
authors.forename: Richard G
authors.initials: RG
authors.lastname: Ellenbogen
authors.affiliation: Departments of 1 Neurological Surgery.|Radiology, University of Washington, Seattle, Washington.
authors.forename: Louis J
authors.initials: LJ
authors.lastname: Kim
authors.affiliation: Departments of 1 Neurological Surgery.|Radiology, University of Washington, Seattle, Washington.
authors.forename: Laligam N
authors.initials: LN
authors.lastname: Sekhar
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000175:diagnosis* / Q000453:epidemiology* / Q000523:psychology; D008297:Male; D011788:Quality of Life / Q000523:psychology; D020127:Recovery of Function; D012189:Retrospective Studies; D011795:Surveys and Questionnaires / Q000592:standards*; D016896:Treatment Outcome; D055815:Young Adult
keywords: AVM = arteriovenous malformation; CNS = central nervous system; EVD = external ventricular drain; GCS = Glasgow Coma Scale; GOS = Glasgow Outcome Scale; HRQL = health-related quality of life; ICH = intracranial hemorrhage; PCPC = Pediatric Cerebral Performance Category; POPC = Pediatric Overall Performance Category; PedsQL = Pediatric Quality of Life Inventory; SAH = subarachnoid hemorrhage; TBI = traumatic brain injury; VP = ventriculoperitoneal; WFNS = World Federation of Neurosurgical Societies; bAVM = brain AVM; clinical outcomes; functional status; mRS = modified Rankin Scale; neurological surgery outcomes; pediatric brain arteriovenous malformations; vascular disorders