Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia

Lauren A. Beslow, Neurology and Pediatrics, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Penn HHT Center of Excellence and Children's Hospital of Philadelphia Hereditary Hemorrhagic Telangiectasia Program, 3401 Civic Center Boulevard, Philadelphia, 19104, PA, USA. beslow@chop.edu.
Arastoo Vossough, Radiology, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
Helen Kim, Center for Cerebrovascular Research, Anesthesia, University of California, San Francisco, CA, USA.
Jeffrey Nelson, Center for Cerebrovascular Research, Anesthesia, University of California, San Francisco, CA, USA.
Michael T. Lawton, Neurosurgery, Barrow Neurological Institute, Phoenix, AZ, USA.
Jeffrey Pollak, Vascular & Interventional Radiology, Yale University School of Medicine, New Haven, CT, USA.
Doris D. Lin, Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Felix Ratjen, Paediatrics and Paediatric Respiratory Medicine, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada.
Adrienne M. Hammill, Cancer and Blood Diseases Institute, Division of Hematology, Cincinnati Children's Hospital Medical Center, and Division of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Steven W. Hetts, Division of Neurointerventional Radiology, University of California, San Francisco, CA, USA.
James R. Gossage, Departments of Critical Care Medicine and Pulmonary Medicine, Augusta University, Augusta, GA, USA.
Kevin J. Whitehead, Division of Cardiovascular Medicine, Department of Internal Medicine, University of Utah Health, Salt Lake City, UT, USA.
Marie E. Faughnan, Toronto HHT Centre, Department of Medicine, St. Michael's Hospital and Li Ka Shing Knowledge Institute, University of Toronto, Toronto, ON, Canada.
Timo Krings, Division of Neuroradiology, Toronto Western Hospital and University of Toronto, Toronto, ON, Canada.

Document Type

Article

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.

Medical Subject Headings

Humans; Telangiectasia, Hereditary Hemorrhagic (complications, diagnostic imaging, epidemiology); Child; Male; Female; Intracranial Arteriovenous Malformations (diagnostic imaging, complications, surgery); Child, Preschool; Adolescent; Cerebral Angiography; Infant

Publication Date

7-1-2024

Publication Title

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

E-ISSN

1433-0350

Volume

40

Issue

7

First Page

2101

Last Page

2108

PubMed ID

38517485

Digital Object Identifier (DOI)

10.1007/s00381-024-06366-z

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