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Submitted by Guilherme Lemeszenski (guilherme@nead.unesp.br) on 2013-08-22T18:48:25Z No. of bitstreams: 1 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) Made available in DSpace on 2013-08-22T18:48:25Z (GMT). No. of bitstreams: 1 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) Previous issue date: 2007-06-01 Made available in DSpace on 2013-09-30T19:38:15Z (GMT). No. of bitstreams: 2 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) S0004-282X2007000300020.pdf.txt: 23956 bytes, checksum: 323426ac4fe836f350cf3ce924373237 (MD5) Previous issue date: 2007-06-01 Submitted by Vitor Silverio Rodrigues (vitorsrodrigues@reitoria.unesp.br) on 2014-05-20T13:36:00Z No. of bitstreams: 2 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) S0004-282X2007000300020.pdf.txt: 23956 bytes, checksum: 323426ac4fe836f350cf3ce924373237 (MD5) Made available in DSpace on 2014-05-20T13:36:00Z (GMT). No. of bitstreams: 2 S0004-282X2007000300020.pdf: 387188 bytes, checksum: b3716f5d41df3e4ebf4e8c506a7b3883 (MD5) S0004-282X2007000300020.pdf.txt: 23956 bytes, checksum: 323426ac4fe836f350cf3ce924373237 (MD5) Previous issue date: 2007-06-01 A síndrome de Crouzon é caracterizada por deformidade craniana, alterações faciais e exoftalmia. O retardo no desenvolvimento neuropsicomotor é observado em alguns casos. Este estudo tem como objetivo analisar a influência do momento da cirurgia, da classe sócio-econômica associada ao nível educacional dos pais e da ocorrência de malformações do sistema nervoso central no desenvolvimento cognitivo destes pacientes correlacionando estes achados à qualidade de vida deles e de suas famílias. Foram estudados 11 pacientes com diagnóstico de síndrome de Crouzon com idade entre um ano e quatro meses e treze anos. A avaliação multidisciplinar dos pacientes incluiu, avaliação social, avaliação cognitiva, estudo do encéfalo por ressonância magnética e avaliação da qualidade de vida. O quociente de inteligência variou de 46 a 102 (m=84,2) e foi correlacionado de forma inversa com o Fator 4 do Questionário de Recursos e Estresse Simplificado (incapacidade da criança); não se correlacionou com as alterações encefálicas, com a condição sócio-econômica dos pais e nem com o momento do tratamento neurocirúrgico. Crouzon syndrome is characterized by cranial and facial abnormalities and exophtalmos. Mental retardation is sometimes observed. The objective of this study was to correlate brain malformations, timing for surgery and also social classification of families and parents education to the neuropsychological evaluation and to the quality of life of these families. Eleven patients with Crouzon syndrome were studied, whose ages were between 16 and 132 months. The multidisciplinary evaluation included : social evaluation, cognitive evaluation, brain studies by magnetic ressonance imaging and quality of life evaluation. The intelligence quotient values observed were between 46 and 102 (m=84.2) and was correlated (inverted correlation) to the factor IV of the short-form of the Questionnaire on Resources and Stress. Mental development was not correlated to brain malformation, neither to the age at time of operation or to the level of family environment and parents education. Universidade de São Paulo Hospital de Reabilitação de Anomalias Craniofaciais Departamento de Cirurgia Craniofacial Hospital Alemão Oswaldo Cruz Departamento de Neuroradiologia USP Hospital das Clínicas Departamento de Neurocirurgia Universidade Estadual Paulista Hospital das Clínicas Departamento de Neurologia e Psiquiatria UNESP Hospital das Clínicas Departamento de Cirurgia e Ortopedia USP Faculdade de Odontologia Departamento de Fonoaudiologia Universidade Estadual Paulista Hospital das Clínicas Departamento de Neurologia e Psiquiatria UNESP Hospital das Clínicas Departamento de Cirurgia e Ortopedia

Crouzon syndrome is characterized by cranial and facial abnormalities and exophtalmos. Mental retardation is sometimes observed. The objective of this study was to correlate brain malformations, timing for surgery and also social classification of families and parents education to the neuropsychological evaluation and to the quality of life of these families. Eleven patients with Crouzon syndrome were studied, whose ages were between 16 and 132 months. The multidisciplinary evaluation included : social evaluation, cognitive evaluation, brain studies by magnetic resonance imaging and quality of life evaluation. The intelligence quotient values observed were between 46 and 102 (m=84.2) and was correlated (inverted correlation) to the factor IV of the short-form of the Questionnaire on Resources and Stress. Mental development was not correlated to brain malformation, neither to the age at time of operation or to the level of family environment and parents education.