Apert syndrome: Analysis of associated brain malformations and conformational changes determined by surgical treatment

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dc.contributor.authorYacubian-Fernandes, A. [UNESP]
dc.contributor.authorPalhares Neto, Aristides Augusto [UNESP]
dc.contributor.authorGiglio, A.
dc.contributor.authorGabarra, Roberto Colichio [UNESP]
dc.contributor.authorZanini, S.
dc.contributor.authorPortela, L.
dc.contributor.authorPlese, J. P P
dc.contributor.institutionUniversidade de São Paulo (USP)
dc.contributor.institutionHospital Alemão Oswaldo Cruz
dc.contributor.institutionUniversidade Estadual Paulista (Unesp)
dc.date.accessioned2014-05-27T11:21:03Z
dc.date.available2014-05-27T11:21:03Z
dc.date.issued2004-03-01
dc.description.abstractApert Syndrome, also called acrocephalosyndactylia type 1, is characterized by craniostenosis with early fusion of sutures of the vault and/ or cranial base, associated to mid-face hypoplasia, symmetric syndactylia of the hands and feet and other systemic malformations. CNS malformations and intracranial hypertension are frequently observed in these patients. Early surgical treatment aims to minimize the deleterious effects of intracranial hypertension. Fronto-orbital advancement, the usual surgical technique, increases the intracranial volume and improves the disposition of encephalic structures previously deformed by a short skull. This study analyzes CNS alterations revealed by magnetic resonance in 18 patients presenting Apert Syndrome, and the conformational alterations in the encephalic structures after surgical treatment. The patients' age in February 2001 ranged from 14 to 322 months (m=107). Image study included brain magnetic resonance showing ventricular enlargement in five cases (27.8%), corpus callosum hypoplasia in five cases (27.8%), septum pellucidum hypoplasia in five cases (27.8%), cavum vergae in two cases (11.1%) and, arachnoid cyst in the posterior fossa in two cases (11.1%). Absence of CNS alterations was noted in 44.4% of cases. A corpus callosum morphologic index was established by dividing its height by its length, which revealed values that ranged from 0.4409 to 1.0237. The values of this index were correlated to the occurrence or absence of surgical treatment (p=0.012; t=2.83). Data analysis allowed the conclusion that the corpus callosum morphologic measure quantified the conformational alterations of the cerebral structures determined by the surgical treatment.en
dc.description.affiliationDepartment of Craniofacial Surgery Hosp. Reabilit. Anomalias Craniofac. University of Sao Paulo (USP), Rua Silvio Marchione 3-20, 900 Bauru SP
dc.description.affiliationDepartment of Neuroradiology Hospital Alemão Oswaldo Cruz, São Paulo, SP
dc.description.affiliationDepartment of Neurosurgery Hospital das Clínicas University of Sao Paulo (USP), São Paulo, SP
dc.description.affiliationDept. of Neurology and Psychiatry Hospital das Clínicas UNESP, Botucatu, SP
dc.description.affiliationDepartment of Surgery Hospital das Clínicas University of the State of Sao Paolo, Botucatu, SP
dc.description.affiliation, R. Bandeirantes 7-36, Bauru SP, CEP 17015011
dc.description.affiliationUnespDept. of Neurology and Psychiatry Hospital das Clínicas UNESP, Botucatu, SP
dc.format.extent116-122
dc.identifierhttp://dx.doi.org/10.1016/S0150-9861(04)96978-7
dc.identifier.citationJournal of Neuroradiology, v. 31, n. 2, p. 116-122, 2004.
dc.identifier.doi10.1016/S0150-9861(04)96978-7
dc.identifier.issn0150-9861
dc.identifier.lattes6664825590096912
dc.identifier.scopus2-s2.0-1642359042
dc.identifier.urihttp://hdl.handle.net/11449/67684
dc.identifier.wosWOS:000220339800005
dc.language.isoeng
dc.relation.ispartofJournal of Neuroradiology
dc.relation.ispartofjcr2.706
dc.relation.ispartofsjr0,985
dc.rights.accessRightsAcesso restrito
dc.sourceScopus
dc.subjectAcrocephalosyndactylia type I
dc.subjectApert Syndrome
dc.subjectBrain anomalies
dc.subjectCorpus callosum
dc.subjectMR imaging
dc.subjectacrocephalosyndactyly
dc.subjectbrain arachnoid cyst
dc.subjectbrain malformation
dc.subjectbrain tomography
dc.subjectbrain ventricle dilatation
dc.subjectclinical article
dc.subjectcontrolled study
dc.subjectcorpus callosum
dc.subjectcorrelation analysis
dc.subjectdata analysis
dc.subjectdisease association
dc.subjectfemale
dc.subjectheight
dc.subjecthuman
dc.subjectmale
dc.subjectneuroimaging
dc.subjectneurosurgery
dc.subjectnewborn
dc.subjectnuclear magnetic resonance imaging
dc.subjectseptum pellucidum
dc.subjectskull
dc.subjectAcrocephalosyndactylia
dc.subjectAdolescent
dc.subjectAdult
dc.subjectBrain Diseases
dc.subjectCentral Nervous System
dc.subjectChild
dc.subjectChild, Preschool
dc.subjectFemale
dc.subjectHumans
dc.subjectInfant
dc.subjectMagnetic Resonance Imaging
dc.subjectMale
dc.subjectMolecular Conformation
dc.subjectSurgical Procedures, Elective
dc.subjectTreatment Outcome
dc.titleApert syndrome: Analysis of associated brain malformations and conformational changes determined by surgical treatmenten
dc.typeArtigo
unesp.author.lattes6664825590096912
unesp.author.lattes8588587034751584[2]
unesp.author.orcid0000-0002-3484-862X[2]
unesp.campusUniversidade Estadual Paulista (Unesp), Faculdade de Medicina, Botucatupt

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Artigos - Neurologia, Psicologia e Psiquiatria - FMB