Natural history of high-grade pediatric arteriovenous malformations: implications for management options

Cerebral arteriovenous malformations are a common cause of pediatric intracranial hemorrhage. Often, small, superficial, lesions are treated surgically; however, more complex, deeper, eloquently located lesions portend higher-risk features and suffer from limitations in treatment. We sought to examine our institution's experience with the natural history of these high-grade arteriovenous malformations to explore outcomes with conservative treatment.

A retrospective chart review was performed to identify all pediatric cases of intracranial arteriovenous malformations seen at our institution from 2005 to 2018. Subjects with Spetzler-Martin grade IV or V lesions, treated conservatively, were examined for primary outcomes including rupture rate, progression, and functional outcomes.

A total of 14 patients were included in the study, of which, 78.57% were classified as Spetzler-Martin grade IV and 21.43% Spetzler-Martin grade V. All patients in this study were treated conservatively, with surveillance, followed for a mean of 32.17 months (range 9.43-79.10). 7.14% experienced delayed hemorrhage or re-rupture, 7.14% had hydrocephalus, and 14.29% had seizures. Neurological sequelae included weakness, visual impairment, speech impairment, sensory changes, and dystonia; functionally independent outcomes, defined as modified Rankin Score of 0-2, were seen in 85.71% of patients.

Our experience suggests that patients with large, deep lesions have significant morbidity with high rates of rupture and subsequent neurologic deficits. However, intervention of these lesions may carry high risk, and the literature suggests such lesions may have less favorable outcomes when treated. We propose conservative treatment for high-grade arteriovenous malformations as a viable option with good functional outcomes in a cohort often without good options for conventional treatment.