Pediatric cerebral arteriovenous malformation: an institutional experience from 25 years of treatment

OBJECTIVE In pediatric cerebral arteriovenous malformation (AVM), the long-term outcome data are scarce. The aim of this study was to analyze the clinical and radiological long-term outcomes from an institutional experience of children and adolescents treated for AVM over 25 years. METHODS The authors reviewed clinical and radiological data from a consecutive series of patients (age ≤ 18 years) treated for AVM at a single institution from 2000 to 2024, with at least 1 year of follow-up. RESULTS Overall, 96 patients were treated for AVM (48 males and 48 females, mean age 12.8 ± 4.8 years). Spetzler-Martin grades were I in 18 patients (19.4%), II in 33 (35.5%), and III in 30 (32.3%), and 14.6% of patients had an infratentorial location. Overall, 8.3% of patients were asymptomatic and 54.2% presented with hemorrhage. Moreover, 11.5% of patients experienced new or repeated hemorrhage during the initial observation or ongoing treatment. The stand-alone treatment was microsurgery in 38.5% of patients, radiosurgery in 28.1%, and embolization in 2.1%, while 9.4% of patients were treated conservatively. The treatment was multimodal in 21.9% and repeated in 49.4% of patients. The obliteration rate in patients with completed treatment (n = 75) was 80% (higher in those presenting with hemorrhage: 91.1% vs 63.3%, p = 0.003). The obliteration rate was 88.6% after microsurgery and 57.9% after radiosurgery (higher in the hemorrhagic group: 87.5% vs 36.4%, p = 0.026). Recurrence occurred in 8.3% of patients at a median of 60 months (range 36–68 months), and following microsurgery in all cases. A favorable clinical outcome (modified Rankin Scale scores 0–2) was achieved in 83.2% of patients, nonsignificantly less in those with hemorrhagic presentation (76.5% vs 90.9%, p = 0.061). The overall mortality rate was 5.3%, without a significant difference between the hemorrhagic and nonhemorrhagic groups. Hydrocephalus, hemorrhage volume, nidus size, infratentorial location, and Spetzler-Martin, supplementary, and combined grades were negative predictors of a good outcome in the univariate analysis, with the combined grade being the strongest predictor. Hemorrhage was a strong predictor of obliteration in the multivariate model, whereas radiosurgery and the number of treatments were predictors of nonobliteration. CONCLUSIONS Although more than half of children and adolescents with AVM presented with hemorrhage, most patients had a favorable clinical outcome. In our experience, microsurgery and radiosurgery resulted in the highest obliteration rates, particularly in hemorrhagic cases, while the role of endovascular treatment was only supplementary.