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.


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.

Keywords: AVM; Arteriovenous malformation; Embolization; Pediatric; Stereotactic radiosurgery; Surgery.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Follow-Up Studies
  • Humans
  • Infant
  • Intracranial Arteriovenous Malformations* / complications
  • Intracranial Arteriovenous Malformations* / diagnostic imaging
  • Intracranial Arteriovenous Malformations* / therapy
  • Intracranial Hemorrhages / surgery
  • Prognosis
  • Radiosurgery* / methods
  • Retrospective Studies
  • Treatment Outcome