Natural history and treatment of deep-seated brain arteriovenous malformations in pediatric patients

Pediatric deep brain arteriovenous malformations (bAVMs) represent a unique management challenge given their higher cumulative risk of hemorrhage as well as a higher risk of treatment. Better understanding of hemorrhage risk in this patient population will lead to a better decision-making process for patient management.

The authors retrospectively reviewed their institutional bAVM database from 1990 to 2019 and included patients younger than 21 years who had deep-seated bAVMs. They present the annual hemorrhage risk, during the natural history and after treatment, and functional outcomes.

Thirty-one pediatric patients were included in this study (13 males and 18 females) with a mean age of 11.8 (SD 4.4) years. The most frequent presenting symptoms were headache (54.8%), weakness (38.7%), and seizure (22.6%). The mean follow-up duration was 13.14 (SD 12.5) years, during which 7 (22.6%) AVMs were obliterated, 10 (32.3%) individuals experienced hemorrhage, and the modified Rankin Scale score worsened in 8 (25.8%) patients. The annual natural history risk of hemorrhage was 3.24% per patient, and the overall annual hemorrhage risk after treatment was 1.98% per patient. In particular, the risk was reduced to 0.64% per patient in the stereotactic radiosurgery (SRS) group. Non-White race showed a trend of higher rupture at presentation (OR 5 [95% CI 0.84-41.68], p = 0.09). Female sex was associated with higher odds (OR 13.076 [95% CI 1.424-333.591], p = 0.048) and SRS was associated with lower odds (OR 0.122 [95% CI 0.011-0.862], p = 0.049) of follow-up hemorrhage.

Given the substantial cumulative risk of lifelong hemorrhagic stroke in pediatric patients, timely definitive treatment is warranted. SRS may be beneficial when the risk-benefit profile is deemed acceptable.