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22-03-2024 | Brain Arteriovenous Malformation | Research

Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia

Authors: Lauren A. Beslow, Arastoo Vossough, Helen Kim, Jeffrey Nelson, Michael T. Lawton, Jeffrey Pollak, Doris D. M. Lin, Felix Ratjen, Adrienne M. Hammill, Steven W. Hetts, James R. Gossage, Kevin J. Whitehead, Marie E. Faughnan, Timo Krings, Brain Vascular Malformation Consortium HHT Investigator Group

Published in: Child's Nervous System

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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.
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Metadata
Title
Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia
Authors
Lauren A. Beslow
Arastoo Vossough
Helen Kim
Jeffrey Nelson
Michael T. Lawton
Jeffrey Pollak
Doris D. M. Lin
Felix Ratjen
Adrienne M. Hammill
Steven W. Hetts
James R. Gossage
Kevin J. Whitehead
Marie E. Faughnan
Timo Krings
Brain Vascular Malformation Consortium HHT Investigator Group
Publication date
22-03-2024
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-024-06366-z