title: Long-term hemorrhagic risk in pediatric patients with arteriovenous malformations.
abstract: OBJECTIVE Compared with the general population, the specific natural history of arteriovenous malformations (AVMs) in pediatric patients is less well understood. Furthermore, few pediatric studies have compared posttreatment hemorrhagic risk and functional outcome across different treatment modalities. The objective of this study was to elucidate these points. METHODS The authors retrospectively reviewed all pediatric patients with AVMs evaluated at their institution between 1990 and 2013. The AVM natural history was represented by hemorrhagic risk during the observation period. For treated patients, the observation period was defined as the interval between diagnosis and treatment. Posttreatment hemorrhagic risk and functional outcomes were also assessed. RESULTS A total of 124 pediatric patients with AVMs were evaluated, and 90 patients (72.6%) were retained through follow-up. The average patient age was 13.3 ± 3.8 years, with a mean follow-up period of 9.95 years. The overall AVM obliteration rate was 59.7%. Radiosurgery had an obliteration rate of 49.0%. Thirteen patients were managed conservatively. Four patients under observation hemorrhaged during a total interval of 429.4 patient-years, translating to an annual risk of 0.9%. Posttreatment hemorrhagic risk by treatment modalities were categorized as follows: surgery ± embolization (0.0%), radiosurgery ± embolization (0.8%), embolization alone (2.8%), surgery + radiosurgery ± embolization (3.5%), and observation (0.8%). A significantly higher risk of posttreatment hemorrhage was observed for patients with hemorrhagic presentation (p = 0.043) in multivariate analysis. Seizure presentation, frontal lobe location, nonheadache presentation, and treatment modality were significantly associated with increased risk of poor functional outcomes. CONCLUSIONS In this study of pediatric patients with AVMs, the natural history of hemorrhage was relatively low at 0.9%. Resection remained the optimal management for hemorrhage control and functional outcome perseverance in these pediatric patients with AVMs. AVM obliteration is a valid treatment goal, especially for patients with ruptured presentation, to prevent further hemorrhages later in life.
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Wuyang
authors.initials: W
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Heather
authors.initials: H
authors.lastname: Anderson-Keightly
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Erick M
authors.initials: EM
authors.lastname: Westbroek
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Justin M
authors.initials: JM
authors.lastname: Caplan
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Xiaoming
authors.initials: X
authors.lastname: Rong
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Alice L
authors.initials: AL
authors.lastname: Hung
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Geoffrey P
authors.initials: GP
authors.lastname: Colby
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Alexander L
authors.initials: AL
authors.lastname: Coon
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Rafael J
authors.initials: RJ
authors.lastname: Tamargo
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Judy
authors.initials: J
authors.lastname: Huang
authors.affiliation: Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors.forename: Edward S
authors.initials: ES
authors.lastname: Ahn
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004621:Embolization, Therapeutic; D005260:Female; D005500:Follow-Up Studies; D006470:Hemorrhage / Q000453:epidemiology*; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000453:epidemiology* / Q000628:therapy; D053208:Kaplan-Meier Estimate; D008297:Male; D015999:Multivariate Analysis; D019635:Neurosurgical Procedures; D016016:Proportional Hazards Models; D016634:Radiosurgery; D012189:Retrospective Studies; D012306:Risk; D013997:Time Factors; D016896:Treatment Outcome
keywords: AVM = arteriovenous malformation; CI = confidence interval; HR = hazard ratio; RE = radiosurgery ± embolization; SE = surgery ± embolization; SM = Spetzler-Martin; SR = surgery + radiosurgery ± embolization; arteriovenous malformation; hemorrhage; mRS = modified Rankin Scale; natural history; vascular disorders