Introduction: Pediatric brain arteriovenous malformations (bAVMs) are rare and represent the most common cause of hemorrhagic stroke. Pediatric patients demonstrate superior outcomes in comparison to adult patients with similar lesions and presentations. Most series of pediatric bAVM clinical outcomes utilize the modified Rankin scale (mRS), despite a lack of validation in pediatric patients.

Methods: We identified 40 pediatric patients treated with multimodality therapy. We contacted the parents of the patients and administered the Pediatric Quality of Life Inventory (PedsQL) – a well-validated tool in pediatric outcomes that quantifies performance in a physical, emotional, social, and school domains. We also reviewed clinical information from the patients’ medical charts. We also reviewed the literature for prior clinical outcome reports in pediatric bAVM (Table 1).

Results: Average age at time of intervention was 11.7 years (See Table 2), with an average follow up time of 6.6 years. Sixty three percent of patients presented with hemorrhage. mRS at presentation ranged from 0 to 5, with a mean value of 2.2, and 1.3 at most recent follow up. The average health related quality of life (HRQL) score was 69.3 +/-25. This is lower than mild TBI (82), higher than spina bifida (60), and similar to pediatric stroke and brain tumor (see Table 3). The mRS at most recent follow up was statistically significantly related to the “Physical” subscore of the PedsQL survey (p=0.013), but not as closely corrrelated to the overall PedsQL score (p=0.075). Hemorrhagic presentation, Spetzler-Martin grade, surgical intervention, angiographic cure, and discharge location did not correlate with PedsQL scores. Patients with a ventriculoperitoneal shunt had a lower PedsQL score (p=0.017).

Conclusions: Long-term clinical outcomes for pediatric bAVM patients are under-analyzed. An accurate understanding of HRQL is contingent on utilizing validated toolsets, such as the PedsQL. Important cognitive assessments are missed with conventional follow up.

Patient Care: This study highlights the variety of metrics reported in the literature for documenting “clinical outcomes” in pediatric neurosurgical patients in general, and pediatric bAVM patients more particularly. It also demonstrates an inability of the modified rankin score (mRS) system to capture overall HRQL. More thorough evaluation of pediatric bAVM patients can set the stage for improved neuropsychological interventions and clinical outcomes.

Learning Objectives: (1) To review the concept of “functional clinical outcome” as it relates to prior pediatric neurosurgical series, as well as prior clinical reports of pediatric bAVM outcomes. (2) To evaluate health related quality of life (HRQL) scores for pediatric bAVM patients (3) To screen for variables that are predictive of poor clinical outcomes, in an effort to better optimize patient care.

References: 1. Anderson V, Spencer-Smith M, Leventer R, Coleman L, Anderson P, Williams J, Greenham M, Jacobs R: Childhood brain insult: can age at insult help us predict outcome? Brain 132: 45-56, 2009 2. B, Kolb: Synaptic plasticity and the organization of behaviour after early and late brain injury. Can J Exp Psychol 53: 62-76, 1999 3. Bhat SR, Goodwin TL, Burwinkle TM, Lansdale MF, Dahl GV, Huhn SL, Gibbs IC, Donaldson SS, Rosenblum RK, Varni JW, Fisher PG: Profile of daily life in children with brain tumors: an assessment of health-related quality of life. J Clin Oncol 23: 5493-5500, 2005 4. Bull KS, Liossi C, Peacock JL, Yuen HM, Kennedy CR, Children's Cancer Leukaemia Group: Screening for cognitive deficits in 8 to 14-year old children with cerebellar tumors using self-report measures of executive and behavioral functioning and health-related quality of life. Neuro Oncol: 2015 5. Darsaut TE, Guzman R, Marcellus ML, Edwards MS, Tian L, Do HM, Chang SD, Levy RP, Adler JR, Marks MP, Steinberg GK: Management of pediatric intracranial arteriovenous malformations: experience with multimodality therapy. Neurosurgery 69: 540-556; discussion 556, 2011 6. deVeber GA, MacGregor D, Curtis R, Mayank S: Neurologic outcome in survivors of childhood arterial ischemic stroke and sinovenous thrombosis. J Child Neurol 15: 316-324, 2000 7. Garza-Mercado R, Cavazos E, Tamez-Montes D: Cerebral arteriovenous malformations in children and adolescents. Surg Neurol 27: 131-140, 1987 8. Ghotra SK, Johnson JA, Qiu W, Newton A, Rasmussen C, Yager JY: Age at stroke onset influences the clinical outcome and health-related quality of life in pediatric ischemic stroke survivors. Dev Med Child Neurol 57: 1027-1034, 2015 9. Gross BA, Storey A, Orbach DB, Scott RM, Smith ER: Microsurgical treatment of arteriovenous malformations in pediatric patients: the Boston Children's Hospital experience. J Neurosurg Pediatr 15: 71-77, 2015 10. Kuhlthau KA, Pulsifer MB, Yeap BY, Rivera Morales D, Delahaye J, Hill KS, Ebb D, Abrams AN, Macdonald SM, Tarbell NJ, Yock TI: Prospective study of health-related quality of life for children with brain tumors treated with proton radiotherapy. J Clin Oncol 30: 2079-2086, 2012 11. Max JE, Bruce M, Keatley E, Delis D: Pediatric stroke: plasticity, vulnerability, and age of lesion onset. J Neuropsychiatry Clin Neurosci 22: 30-39, 2010 12. Nerva JD, Kim LJ, Barber J, Rockhill JK, Hallam DK, Ghodke BV, Sekhar LN: Outcomes of Multimodality Therapy in Pediatric Patients With Ruptured and Unruptured Brain Arteriovenous Malformations. Neurosurgery: 2015 13. Ramachandra P, Palazzi KL, Skalsky AJ, Marietti S, Chiang G: Shunted hydrocephalus has a significant impact on quality of life in children with spina bifida. PM R 5: 825-831, 2013 14. Rivara FP, Vavilala MS, Durbin D, Temkin N, Wang J, O'Connor SS, Koepsell TD, Dorsch A, Jaffe KM: Persistence of disability 24 to 36 months after pediatric traumatic brain injury: a cohort study. J Neurotrauma 29: 2499-2504, 2012 15. Sanchez-Mejia RO, Chennupati SK, Gupta N, Fullerton H, Young WL, Lawton MT: Superior outcomes in children compared with adults after microsurgical resection of brain arteriovenous malformations. J Neurosurg 105: 82-87, 2006 16. Singhal A, Adirim T, Cochrane D, Steinbok P: Pediatric patients with poor neurological status and arteriovenous malformation hemorrhage: an outcome analysis. J Neurosurg Pediatr 7: 462-467, 2011 17. Swanson JO, Vavilala MS, Wang J, Pruthi S, Fink J, Jaffe KM, Durbin D, Koepsell T, Temkin N, Rivara FP: Association of initial CT findings with quality-of-life outcomes for traumatic brain injury in children. Pediatr Radiol 42: 974-981, 2012 18. Varni JW, Burwinkle TM, Seid M, Skarr D: The PedsQL 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr 3: 329-341, 2003 19. Varni JW, Limbers CA: The pediatric quality of life inventory: measuring pediatric health-related quality of life from the perspective of children and their parents. Pediatr Clin North Am 56: 843-863, 2009 20. Varni JW, Seid M, Kurtin PS: PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care 39: 800-812, 2001 21. Wong ST, Fong D: Ruptured brain arteriovenous malformations in children: correlation of clinical outcome with admission parameters. Pediatr Neurosurg 46: 417-426, 2010