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Clinical features and outcome in pediatric arteriovenous malformation: institutional multimodality treatment

Pepper, Joshua
Lamin, Saleh
Thomas, Allan
Walsh, A Richard
Rodrigues, Desiderio
Lo, William B
Solanki, Guirish A
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2022-12-29
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Purpose: Intracranial arteriovenous-malformation (AVM) is a relatively rare condition in pediatrics, yet is a major cause of spontaneous intracranial hemorrhage with a risk of fatal hemorrhage reported to be between 4 and 29%. Little is known about vessel morphology and optimum treatment modalities including multimodality combination therapy and prognosis in children. Methods: A retrospective review of all children presenting to our institution from 2006 to 2020 that had an AVM was undertaken. Results: A total of 50 children were identified with median age of 11 (range 1-16) years. The mean follow-up was 7.6 years. Forty-one children presented as an emergency and of those, 40 had hemorrhage identified on initial brain imaging. The average nidus size was 25 mm, drainage was superficial in 51% of cases, and located in eloquent cortex in 56%. The supplemental Spetzler-Martin grading indicated 78% (39/50) were grade 4 and above (moderate to high risk). Primary treatment modalities included embolization in 50% (25) or SRS in 30% (15) and surgery in 20% (10).The AVM was obliterated on follow-up DSA in 66% children. Three children had post-treatment hemorrhage, two related to embolization and one the day following SRS, giving a re-bleed rate of 6%. The GOSE was available for 32 children at long term follow and 94% had a good outcome (GOSE 5-8). Two children died due to acute hemorrhage (4%). Conclusion: The majority of children with AVM present with hemorrhage. The rebleed rate during definitive treatment is low at 6% over the study period. The selective use of the 3 modalities of treatment has significantly reduced mortality and severe disability.
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Pepper J, Lamin S, Thomas A, Walsh AR, Rodrigues D, Lo WB, Solanki GA. Clinical features and outcome in pediatric arteriovenous malformation: institutional multimodality treatment. Childs Nerv Syst. 2023 Apr;39(4):975-982. doi: 10.1007/s00381-022-05800-4. Epub 2022 Dec 29
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