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Brain AVM compactness score in children with hereditary hemorrhagic telangiectasia.
Beslow, Lauren A; Vossough, Arastoo; Kim, Helen; Nelson, Jeffrey; Lawton, Michael T; Pollak, Jeffrey; Lin, Doris D M; Ratjen, Felix; Hammill, Adrienne M; Hetts, Steven W; Gossage, James R; Whitehead, Kevin J; Faughnan, Marie E; Krings, Timo.
Afiliación
  • Beslow LA; 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,
  • Vossough A; Radiology, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
  • Kim H; Center for Cerebrovascular Research, Anesthesia, University of California, San Francisco, CA, USA.
  • Nelson J; Center for Cerebrovascular Research, Anesthesia, University of California, San Francisco, CA, USA.
  • Lawton MT; Neurosurgery, Barrow Neurological Institute, Phoenix, AZ, USA.
  • Pollak J; Vascular & Interventional Radiology, Yale University School of Medicine, New Haven, CT, USA.
  • Lin DDM; Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
  • Ratjen F; Paediatrics and Paediatric Respiratory Medicine, The Hospital for Sick Children and University of Toronto, Toronto, ON, Canada.
  • Hammill AM; 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.
  • Hetts SW; Division of Neurointerventional Radiology, University of California, San Francisco, CA, USA.
  • Gossage JR; Departments of Critical Care Medicine and Pulmonary Medicine, Augusta University, Augusta, GA, USA.
  • Whitehead KJ; Division of Cardiovascular Medicine, Department of Internal Medicine, University of Utah Health, Salt Lake City, UT, USA.
  • Faughnan ME; Toronto HHT Centre, Department of Medicine, St. Michael's Hospital and Li Ka Shing Knowledge Institute, University of Toronto, Toronto, ON, Canada.
  • Krings T; Division of Neuroradiology, Toronto Western Hospital and University of Toronto, Toronto, ON, Canada.
Childs Nerv Syst ; 40(7): 2101-2108, 2024 Jul.
Article en En | MEDLINE | ID: mdl-38517485
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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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Telangiectasia Hemorrágica Hereditaria / Angiografía Cerebral / Malformaciones Arteriovenosas Intracraneales Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Childs Nerv Syst Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: Panamá Pais de publicación: Alemania

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Telangiectasia Hemorrágica Hereditaria / Angiografía Cerebral / Malformaciones Arteriovenosas Intracraneales Límite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male Idioma: En Revista: Childs Nerv Syst Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: Panamá Pais de publicación: Alemania