TY - JOUR
T1 - Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia
AU - Brain Vascular Malformation Consortium HHT Investigator Group
AU - Beslow, Lauren A.
AU - Vossough, Arastoo
AU - Kim, Helen
AU - Nelson, Jeffrey
AU - Lawton, Michael T.
AU - Pollak, Jeffrey
AU - Lin, Doris D.M.
AU - Ratjen, Felix
AU - Hammill, Adrienne M.
AU - Hetts, Steven W.
AU - Gossage, James R.
AU - Whitehead, Kevin J.
AU - Faughnan, Marie E.
AU - Krings, Timo
AU - Atherton, Mary E.
AU - Chakinala, Murali M.
AU - Clancy, Marianne S.
AU - Henderson, Katharine
AU - Hetts, Steven
AU - Hountras, Peter
AU - Iyer, Vivek
AU - Kasthuri, Raj S.
AU - Lin, Doris
AU - Mager, Johannes Jurgen
AU - Marchuk, Douglas A.
AU - McWilliams, Justin P.
AU - McDonald, Jamie
AU - Pawlikowska, Ludmila
AU - Swanson, Karen
AU - Vethanayagam, Dilini
AU - Weinsheimer, Shantel
AU - White, Andrew J.
AU - Wilcox, Pearce
N1 - Publisher Copyright:
© The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2024.
PY - 2024/7
Y1 - 2024/7
N2 - 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.
AB - 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.
KW - Angiography
KW - Brain arteriovenous malformation
KW - Hereditary hemorrhagic telangiectasia
UR - http://www.scopus.com/inward/record.url?scp=85195196069&partnerID=8YFLogxK
U2 - 10.1007/s00381-024-06366-z
DO - 10.1007/s00381-024-06366-z
M3 - Article
C2 - 38517485
AN - SCOPUS:85195196069
SN - 0256-7040
VL - 40
SP - 2101
EP - 2108
JO - Child's Nervous System
JF - Child's Nervous System
IS - 7
ER -