Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia.
Publication/Presentation Date
7-1-2024
Abstract
OBJECTIVE: The brain arteriovenous malformation (BAVM) nidus compactness score (CS), determined on angiography, predicts BAVM recurrence after surgical resection among children with sporadic BAVMs. We measured the angiographic CS for BAVMs among children with hereditary hemorrhagic telangiectasia (HHT) to determine CS characteristics in this population.
METHODS: A pediatric interventional neuroradiologist reviewed angiograms to determine the CS of BAVMs in children with HHT recruited to the BVMC. CS is based on overall nidus and perinidal anomalous vessel compactness. CS categories included 1 = diffuse nidus, 2 = intermediate nidus, and 3 = compact nidus.
RESULTS: Forty-eight of 78 children (61.5%) with HHT and brain vascular malformations had a conventional angiogram; 47 (97.9%) angiograms were available. Fifty-four BAVMs were identified in 40 of these 47 children (85.1%). Of 54 BAVMs in children with HHT, CS was 1 in 7 (13%), 2 in 29 (53.7%), and 3 in 18 BAVMs (33.3%) compared with CS of 1 in six (26.1%), 2 in 15 (65.2%), and 3 in 2 BAVMs (8.7%) among 23 previously reported children with sporadic BAVMs, p = 0.045 (Fisher's exact). Seven children with HHT had intracranial hemorrhage: 4 had CS = 3, 1 had CS = 2, and 2 had CS = 1.
CONCLUSIONS: A range of CSs exists across HHT BAVMs, suggesting it may be an angiographic measure of interest for future studies of BAVM recurrence and hemorrhage risk. Children with HHT may have more compact niduses compared to children with sporadic BAVMs. Additional research should determine whether CS affects hemorrhage risk or post-surgical recurrence risk in HHT-associated BAVMs, which could be used to direct BAVM treatment.
Volume
40
Issue
7
First Page
2101
Last Page
2108
ISSN
1433-0350
Published In/Presented At
Beslow, L. A., Vossough, A., Kim, H., Nelson, J., Lawton, M. T., Pollak, J., Lin, D. D. M., Ratjen, F., Hammill, A. M., Hetts, S. W., Gossage, J. R., Whitehead, K. J., Faughnan, M. E., Krings, T., & Brain Vascular Malformation Consortium HHT Investigator Group (2024). Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 40(7), 2101–2108. https://doi.org/10.1007/s00381-024-06366-z
Disciplines
Medicine and Health Sciences | Pediatrics
PubMedID
38517485
Department(s)
Department of Pediatrics
Document Type
Article