Seizure Burden In Children With Brain Vascular Malformations, A Retrospective Single Center Study.

Introduction: Brain arteriovenous malformations (bAVM) and cavernomas are the most common vascular malformations causing hemorrhagic stroke in children. Understanding seizure burden associated with these conditions is important for long term management.

Methods: Retrospective cohort study of a consecutively enrolled cohort of children diagnosed with bAVM and cavernomas presenting to a tertiary care pediatric center between 2013-2022.

Results: Seventy-three children were included (46:bAVM; 27:cavernomas). Median age at diagnosis was 13 years (IQR=7.5). Forty-eight were males (28:bAVM; 20:cavernomas). In the bAVM group, 5 children had a genetic diagnosis of hereditary hemorrhagic telangiectasia and 1 of RASA-1 mutation. Six children with cavernomas had KRIT-1 mutation. A total of 55/73 (75%) had supratentorial vascular malformations. Seizures at presentation were observed in 5/46 (11%) children with bAVM and 8/27 (30%) with cavernomas. A total of 16/46 (35%) in the bAVM cohort and 5/27 (19%) in the cavernoma cohort were discharged on maintenance anti-seizure medication. Seizures at one year follow-up were seen in 8/46 (17%) of bAVM and 3/27 (11%) cavernomas; 13/46 (28%) bAVM and 5/27 (19%) cavernomas were on one drug and 2/73 children required two. Drug-resistant epilepsy was observed in 4/73 (5%) of total cohort; 3/46 (6%) bAVM and 1/27 (4%) cavernomas.

Conclusion: Low seizure burden was observed in this pediatric cohort with bAVM and cavernomas. This has important implications when counselling parents regarding epilepsy following diagnosis of brain vascular malformation, and long-term medical management.