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Natural history of familial cerebral cavernous malformation syndrome in children: a multicenter cohort study

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dc.contributor.authorGeraldo, Ana Filipa
dc.contributor.authorAlves, Cesar Augusto P. F.
dc.contributor.authorLuis, Aysha
dc.contributor.authorTortora, Domenico
dc.contributor.authorGuimarães, Joana
dc.contributor.authorAbreu, Daisy
dc.contributor.authorReimão, Sofia
dc.contributor.authorPavanello, Marco
dc.contributor.authorde Marco, Patrizia
dc.contributor.authorScala, Marcello
dc.contributor.authorCapra, Valeria
dc.contributor.authorVaz, Rui
dc.contributor.authorRossi, Andrea
dc.contributor.authorSchwartz, Erin Simon
dc.contributor.authorMankad, Kshitij
dc.contributor.authorSeverino, Mariasavina
dc.date.accessioned2022-10-11T15:38:15Z
dc.date.available2022-10-11T15:38:15Z
dc.date.issued2022
dc.description© The Author(s) 2022. Open Access This article is licensed under a Creative Commons Attri- bution 4.0 International License, which permits use, sharing, adapta- tion, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.pt_PT
dc.description.abstractPurpose: There is limited data concerning neuroimaging findings and longitudinal evaluation of familial cerebral cavernous malformations (FCCM) in children. Our aim was to study the natural history of pediatric FCCM, with an emphasis on symptomatic hemorrhagic events and associated clinical and imaging risk factors. Methods: We retrospectively reviewed all children diagnosed with FCCM in four tertiary pediatric hospitals between January 2010 and March 2022. Subjects with first available brain MRI and [Formula: see text] 3 months of clinical follow-up were included. Neuroimaging studies were reviewed, and clinical data collected. Annual symptomatic hemorrhage risk rates and cumulative risks were calculated using survival analysis and predictors of symptomatic hemorrhagic identified using regression analysis. Results: Forty-one children (53.7% males) were included, of whom 15 (36.3%) presenting with symptomatic hemorrhage. Seven symptomatic hemorrhages occurred during 140.5 person-years of follow-up, yielding a 5-year annual hemorrhage rate of 5.0% per person-year. The 1-, 2-, and 5-year cumulative risks of symptomatic hemorrhage were 7.3%, 14.6%, and 17.1%, respectively. The latter was higher in children with prior symptomatic hemorrhage (33.3%), CCM2 genotype (33.3%), and positive family history (20.7%). Number of brainstem (adjusted hazard ratio [HR] = 1.37, P = 0.005) and posterior fossa (adjusted HR = 1.64, P = 0.004) CCM at first brain MRI were significant independent predictors of prospective symptomatic hemorrhage. Conclusion: The 5-year annual and cumulative symptomatic hemorrhagic risk in our pediatric FCCM cohort equals the overall risk described in children and adults with all types of CCM. Imaging features at first brain MRI may help to predict potential symptomatic hemorrhage at 5-year follow-up.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationNeuroradiology. 2022 Oct 6. doi: 10.1007/s00234-022-03056-ypt_PT
dc.identifier.doi10.1007/s00234-022-03056-ypt_PT
dc.identifier.eissn1432-1920
dc.identifier.issn0028-3940
dc.identifier.urihttp://hdl.handle.net/10451/54741
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherSpringer Naturept_PT
dc.relation.publisherversionhttps://www.springer.com/journal/234pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectBrain imagingpt_PT
dc.subjectCavernous malformationpt_PT
dc.subjectFamilial cavernous malformation syndromept_PT
dc.subjectMagnetic resonance imagingpt_PT
dc.titleNatural history of familial cerebral cavernous malformation syndrome in children: a multicenter cohort studypt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.titleNeuroradiologypt_PT
person.familyNameGeraldo
person.familyNameabreu
person.familyNameReimão
person.givenNameAna Filipa
person.givenNamedaisy
person.givenNameSofia
person.identifier.ciencia-id0110-31E1-DEE3
person.identifier.ciencia-idCB19-3E68-EBCB
person.identifier.orcid0000-0002-9663-4387
person.identifier.orcid0000-0002-7979-6456
person.identifier.orcid0000-0002-8822-3166
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication23cf72dd-520e-4567-a100-3b43893b2782
relation.isAuthorOfPublication4f324601-fcfa-4e3f-a203-4d6cd91991ee
relation.isAuthorOfPublication72ade4b9-4353-45ce-9334-eddd2a2045c0
relation.isAuthorOfPublication.latestForDiscovery23cf72dd-520e-4567-a100-3b43893b2782

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