<p>Children with myelomeningocele (MMC) usually have problems with daily life activities, but the background to their problems is not altogether obvious. An understanding of the possible causes of activity problems is a prerequisite for the effectiveness of physical therapy. The overall aim of the present studies was to identify impairments above the cele level, which might influence mobility in children with MMC (study I) and to analyse sitting posture (study II) and the movement characteristics of reaching movements (study III and IV). </p><p>In total, 41 children and young adults with MMC and without mental retardation were investigated. Study I comprised 32 children, 6-11 years. Mobility and the caregiver assistance required for mobility were quantified according to the Paediatric Evaluation of Disability Inventory (PEDI) and correlation between mobility and neurological impairment, hand function and cognitive function were calculated. The results showed that nine children achieved independent mobility and that there was a moderate and significant correlation between the need for physical assistance and high cele level, impaired hand function and impaired cognitive function. In those children who used a wheelchair, only poor hand strength was significantly correlated with the need for caregiver assistance.</p><p>Study II comprised 11 children, 10-13 years, and a control group of 20 healthy children. Sitting posture was investigated by using a force plate and analysed from the frequency and the amplitude of the postural sway. The reaction forces before and during rapid arm lift were also analysed. The result showed that children with MMC had significantly lower sway frequency compared to the controls. In both groups, the ground reaction forces were registered before the children lifted their arms. Study III and IV comprised 31 children and young adults, 9-19 years and 31 matched controls. Reaching movements were investigated with a digitising tablet, linked to a computer. The ability to program and execute reaching movements was analysed and also the ability to adapt reaching to new visuomotor conditions. The results showed that the MMC group had poorer precision, less straight movements and shorter deceleration phases as compared to the controls. In both groups the movements were pre-programmed. In addition, adaptation of reaching to new visuomotor conditions was poor in the MMC group as compared to the controls. </p><p>In conclusion we found that impairments above the cele level influenced mobility and the control of sitting posture in children with MMC. Reduced precision and co-ordination of reaching, and also difficulties with motor adaptation, could partly explain thier problems with hand activities. These findings need to be considered in therapy programs for children and young adults with MMC.</p>

Children with myelomeningocele (MMC) usually have problems with daily life activities, but the background to their problems is not altogether obvious. An understanding of the possible causes of activity problems is a prerequisite for the effectiveness of physical therapy. The overall aim of the present studies was to identify impairments above the cele level, which might influence mobility in children with MMC (study I) and to analyse sitting posture (study II) and the movement characteristics of reaching movements (study III and IV). In total, 41 children and young adults with MMC and without mental retardation were investigated. Study I comprised 32 children, 6-11 years. Mobility and the caregiver assistance required for mobility were quantified according to the Paediatric Evaluation of Disability Inventory (PEDI) and correlation between mobility and neurological impairment, hand function and cognitive function were calculated. The results showed that nine children achieved independent mobility and that there was a moderate and significant correlation between the need for physical assistance and high cele level, impaired hand function and impaired cognitive function. In those children who used a wheelchair, only poor hand strength was significantly correlated with the need for caregiver assistance. Study II comprised 11 children, 10-13 years, and a control group of 20 healthy children. Sitting posture was investigated by using a force plate and analysed from the frequency and the amplitude of the postural sway. The reaction forces before and during rapid arm lift were also analysed. The result showed that children with MMC had significantly lower sway frequency compared to the controls. In both groups, the ground reaction forces were registered before the children lifted their arms. Study III and IV comprised 31 children and young adults, 9-19 years and 31 matched controls. Reaching movements were investigated with a digitising tablet, linked to a computer. The ability to program and execute reaching movements was analysed and also the ability to adapt reaching to new visuomotor conditions. The results showed that the MMC group had poorer precision, less straight movements and shorter deceleration phases as compared to the controls. In both groups the movements were pre-programmed. In addition, adaptation of reaching to new visuomotor conditions was poor in the MMC group as compared to the controls. In conclusion we found that impairments above the cele level influenced mobility and the control of sitting posture in children with MMC. Reduced precision and co-ordination of reaching, and also difficulties with motor adaptation, could partly explain thier problems with hand activities. These findings need to be considered in therapy programs for children and young adults with MMC.

Orientador: Maria da Consolação Gomes Cunha Fernandes Tavares / Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Educação Fisica / Made available in DSpace on 2018-08-09T17:26:42Z (GMT). No. of bitstreams: 1 Catusso_RenataLobo_M.pdf: 1391611 bytes, checksum: d3b214284db7ac81ebd3f02b854697be (MD5) Previous issue date: 2007 / Resumo: O contato com o próprio corpo e com o mundo, experimentado por crianças com mielomeningocele (MM) apresenta características bem diferentes do habitual em decorrência principalmente dos déficits motores e sensitivos e do estigma de sua deficiência física. A resiliência é uma capacidade universal que permite a uma pessoa, grupo ou comunidade prevenir, minimizar ou ajustar-se aos efeitos das adversidades. O objetivo dessa pesquisa foi refletir sobre a resiliência e a imagem corporal de adolescentes e adultos com MM de uma instituição especializada no atendimento da área da deficiência física, Associação Campineira de Recuperação da Criança Paralítica, na cidade de Campinas - SP. A pesquisa foi de natureza qualitativa, descritiva exploratória, incluindo uma pesquisa de campo, na qual se aplicou a Escala de Resiliência adaptada por Pesce e colaboradores (2005). Os escores da escala oscilam de 25 a 175 pontos, com valores altos indicando elevada resiliência. Há seis questões relacionadas à Imagem Corporal, elaboradas especialmente para esse trabalho e um questionário de identificação. Participaram 12 sujeitos, dentre eles 04 são do sexo masculino e 08 do sexo feminino, com idade entre 15 a 34 anos. A maioria tem religião. Dos 12 sujeitos, 04 trabalham, 04 não trabalham e 04 são estudantes. Nível de escolaridade dos sujeitos foi do ensino fundamental incompleto à pós-graduação. A renda familiar foi de um a seis salários mínimos, sendo que a maioria recebe o Benefício de Prestação Continuada (BPC). Apenas 01 sujeito não faz uso de cadeira de rodas. Podemos constatar que os sujeitos apresentaram alto potencial de resiliência (94 ¿ 163). Fatores relacionados à capacidade de resolução de ações e valores ficaram acima da média (valores de 49 a 98). Os fatores relacionados à independência e determinação, apenas 02 sujeitos ficaram abaixo da média (valores entre 15 e 28). Um sujeito apresentou o fator autoconfiança e a capacidade de adaptação abaixo da média. Quanto à Imagem Corporal, a maioria diz que gostaria de modificar alguma parte do corpo. As partes mais apreciadas pelos sujeitos foram os olhos e o rosto. E as partes que menos gostaram foram: a barriga e os seios, por serem grandes. Um dos sujeitos apontou como partes do corpo a serem reconstruídas se caso pudesse: a medula, a bexiga e o intestino. A ¿estética¿ e a ¿função¿ foram elementos determinantes dos sentimentos de ¿gostar¿ ou ¿não gostar¿ do corpo. Os resultados desta pesquisa corroboram a relação entre aparência/função e imagem corporal. O conhecimento e o desenvolvimento da resiliência e da imagem corporal são conceitos que contribuem na intervenção satisfatória do profissional de educação física junto a essa população, através do fortalecimento dos pilares básicos da resiliência: afetividade, independência, iniciativa, humor, criatividade e auto-estima / Abstract: The contact with the own body and with the world, as experienced by children with myelomeningocele (MM), shows characteristics significantly different from the usual, mainly as a result of motor and sensitive deficiencies and the related stigma. Resilience is an universal ability which allows a person, a group or a community to prevent, minimize and adapt to the adversities. This research¿s aim is to reflect on resilience and body image on the basis of data obtained from a sample of adolescent and adult patients with MM from a specialized institution, named Associação Campineira de Recuperação da Criança Paralítica, located in Campinas, state of São Paulo in the area of physical deficiencies. This research constitutes of a qualitative and exploratory-descriptive one including a field-work research consisting in the application of the Resilience Scale adapted by Pesce et al. (2005). The scale¿s scores vary from 25 to 175 points, in which higher values being indicative of greater resilience. There are six questions and an identification questionnaire related to body image, especially drawn up for this work. There were 12 subjects, four male and eight female, age ranging from 15 to 34. Most of them are religious. Four are workers, four are students and four others do not have any activity. Academic exposure from incomplete fundamental course to post-graduation. Family income from one to six minimum wages The great majority receives Continuous Monthly Benefit. All of them are wheelchair users except for one. The subjects show high degrees of resilience (94-163), scoring high in items related to action resolution and values, between 49 and 98. In independence and determination factors only two subjects scored below average (values between 15 and 28). One subject showed the factor self-confidence and adaptation below average. As to Body Image, most of the subjects declared that they would like to change some part of the body. The most appreciated parts of the body were the eyes and the face. The parts they appreciated less were the belly and the breasts, these for being big. One subject pointed the medulla, the bladder and the intestines as parts of the body to be reconstructed, if possible. ¿esthetics¿ and ¿function¿ were decisive for the ¿like¿ and ¿dislike¿ preference. The results of this research corroborate the relation between appearance/function and body image. The knowledge and the advance of resilience and of body image are concepts that contribute in the satisfactory interference of a physical education professional with this population through the strengthening of the basic pillars of resilience, affection, independence, initiative, humour, creativity, and self-confidence / Mestrado / Atividade Fisica, Adaptação e Saude / Mestre em Educação Física

Crianças com mielomeningocele (MMC) apresentam anormalidades primárias no fechamento do tubo neural com extrusão de tecido nervoso. Alterações secundárias, como a paraplegia, podem comprometer o desempenho cardiopulmonar e a função autonômica cardiovascular, sendo ainda maior, nos indivíduos que são dependentes exclusivamente de cadeira de rodas. Embora a literatura científica tenha amplamente estudado as manifestações clínicas dos diferentes níveis da MMC, pouca atenção tem sido dada à disfunção cardiopulmonar. A análise da função autonômica cardiovascular assim como a avaliação da capacidade cardiopulmonar, pelo consumo máximo de oxigênio (VO2max), tem sido utilizada como forma de predizer fatores de risco relacionados à saúde, mas o teste cardiopulmonar que é o padrão ouro, requer especificidades que limitam sua ampla utilização. Objetivos: Analisar a capacidade cardiopulmonar de crianças e adolescentes cadeirantes com mielomeningocele e comparar com a do seu respectivo controle, e aplicação de teste indireto a fim de estimar o VO2max nas crianças e adolescentes com MMC. Métodos: Participaram desse estudo transversal 22 crianças e adolescentes, de ambos os sexos, com idade entre 8 e 15 anos, separadas em dois grupos: Grupo Mielomeningocele (GM), composto por 11 crianças e adolescentes com mielomeningocele que não deambulam (estritamente usuários de cadeira de rodas); e Grupo Controle (GC), composto por 11 crianças e adolescentes saudáveis pareados por sexo, idade, massa corporal e/ou estatura. Foram obtidos dados antropométricos; composição corporal por bioimpedância elétrica; classificação do nível de maturação pelo índice de Tanner; nível de atividade física pelo Questionário Internacional de Nível de Atividade Física; avaliação da força muscular isométrica dos abdutores de ombro, flexores e extensores de cotovelo, pelo dinamômetro Handheld e força muscular de preensão palmar pelo dinamômetro de Bulbo; avaliação da Variabilidade da Frequência Cardíaca e da Pressão Arterial (VFC e VPA) e da Sensibilidade Barorreflexa Espontânea (SBRE); teste cardiopulmonar em cicloergômetro de membro superior (teste direto = TD); e, teste indireto (TI) seguindo método proposto por Franklin et al.,(1990) para estimar VO2max. Resultados: os grupos não apresentaram diferença estatística com relação à antropometria e força muscular (p > 0,05), porém o GM apresentou incremento de aproximadamente 6kg de massa gorda (p 0,05). No teste cardiopulmonar, o GM apresentou reduzido valor de VO2pico comparado ao seu controle indicando diferença significativa entre os grupos (p 0,05). O VO2pico correlacionou-se positivamente com dados antropométricos, massa magra e força muscular. Os valores de VO2max estimado pelo TI foram menores que os valores reais obtidos no TD, sofrendo uma variação de 24 à 56%; por outro lado, os dados de FCmax e FC de repouso não apresentaram diferença entre os testes. Com relação à função autonômica cardiovascular, os parâmetros espectrais da VFC não apresentaram diferença significativa entre os grupos, mas os valores de baixa frequência na VPA e a SBRE estavam reduzidos no GM (p < 0,05). Conclusão: crianças e adolescentes com MMC apresentam menor capacidade cardiopulmonar que seus controles saudáveis, e reduzida sensibilidade barorreflexa espontânea com baixo controle simpático na VPA, indicando predisposição a eventos cardíacos tardios. E ainda, o método indireto selecionado não se mostrou ideal para estimar o VO2max nos voluntários do presente estudo, subestimando os valores reais, mas permitiu alcançar valores de FCmax prevista. Este pode ser um modelo de teste de esforço máximo, que necessita ser explorado nessa população. Uma proposta de condicionamento cardiopulmonar necessita ser desenvolvida para esta população. / Children with myelomeningocele (MMC) present primary abnormalities in neural tube with nerve tissue extrusion. Secondary alterations, such as paraplegia, can compromise the cardiopulmonary performance and cardiac autonomic function, mainly in individuals who are exclusively dependent on a wheelchair. Although the scientific literature has extensively studied the clinical manifestations of MMC and their different neural lesion levels, poor attention has been addressed to cardiopulmonary dysfunction. Evaluation of cardiac autonomic function and cardiopulmonary capacity using maximum oxygen uptake (VO2max) has been used to predict risk factors related to health, but this gold standard technique requires specificities that limit its widespread use. Objectives: To analyze the difference cardiopulmonary capacity of children and adolescents with myelomeningocele unable to walk and his or her matched control by detecting data that predispose to cardiovascular risks, and using an indirect test in order to estimate the VO2max in children with MMC. Methods: Twenty two (n = 11) children and adolescents participated of this cross-sectional study, both sexes, aged 8 15 years, separated into two groups: Group Myelomeningocele (GM), with 11 children and adolescents with myelomeningocele unable to walk (wheelchair users); and Control Group (CG), with 11 healthy children and adolescents matched for sex, age, weight and/or height. The data obtained were: Anthropometric data; body composition by bioelectrical impedance analysis; index maturation classification by Taner; level of physical activity by IPAQ; isometric muscle strength of shoulder abductors, elbow flexors and extensors using Handheld dynamometer, and grip muscle strength using bulb dynamometer; Spontaneous baroreflex sensitivity (SBS) and heart rate and blood pressure variability (HRV and BPV) evaluation; cardiopulmonary exercise testing on a cycle ergometer of upper limb (direct test = DT); and indirect test following the method proposed by Franklin et al., (1990) to estimate VO2max. Results: Anthropometry data and muscle strength did not present significant difference between analyzed groups (p > 0.05). GM presented approximately 6kg increased fat mass than CG (p 0.05). By cardiopulmonary test, GM presented reduced VO2peak compared to GC, indicating a significant difference between groups (p 0.05). The VO2peak have positive correlation with anthropometric data and fat free mass and muscle strength. The VO2max in DT varied between 2456% higher than those on the IT, but rest and maximum HR mean value comparisons showed no statistical difference between the tests. Considering cardiac autonomic function, no difference was observed to spectral parameters of HRV, but low frequency of BPV and the SBS were significantly lower in GM (p < 0.05). Conclusion: Children with MMC present reduced cardiopulmonary capacity than their healthy peers and reduced spontaneous baroreflex sensitivity and sympathetic control on BPV, indicating some predisposition to risk of cardiovascular event. And yet, VO2max values estimated by the chosen IT underestimated the DT values and were not applicable to this population; however, they may represent a potential model to test maximal exercise and obtain maximal HR values, which need to be explored further in this population.

abstract: This thesis reviews the initial cases of fetal surgery to correct myelomeningocele, a severe form of spina bifida, and discusses the human and social dimensions of the procedure. Myelomeningocele is a fetal anomaly that forms from improper closure of the spinal cord and the tissues that surround it. Physicians perform fetal surgery on a developing fetus, while it is in the womb, to mitigate its impacts. Fetal surgery to correct this condition was first performed experimentally in the mid-1990and as of 2020, it is commonly performed. The initial cases illuminated important human and social dimensions of the technique, including physical risks, psychological dimensions, physician bias, and religious convictions, which affect decision-making concerning this fetal surgery. Enduring questions remain in 2020. The driving question for this thesis is: given those human and social dimensions that surround fetal surgery to correct myelomeningocele, whether and when is the surgery justified? This thesis shows that more research is needed to answer or clarify this question. / Dissertation/Thesis / Masters Thesis Biology 2020

No description available.

Introdução: Pacientes com mielomeningocele apresentam elevada mortalidade e desenvolvem déficits neurológicos que ocorrem, primariamente, pelo desenvolvimento anormal da medula e de raízes nervosas e, secundariamente, por complicações adquiridas no período pós-natal. O desafio no cuidado desses pacientes é o reconhecimento precoce dos recém-nascidos de risco para evolução desfavorável a fim de estabelecer estratégias terapêuticas individualizadas. Objetivo: Este estudo tem como objetivo identificar marcadores prognósticos de curto prazo para recém-nascidos com mielomeningocele. As características anatômicas do defeito medular e da sua correção neurocirúrgica foram analisadas para esta finalidade. Métodos: Foi realizado um estudo de coorte retrospectiva com 70 pacientes com mielomeningocele em topografia torácica, lombar ou sacral nascidos entre janeiro de 2007 a dezembro de 2013 no Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Pacientes com infecção congênita, anomalias cromossômicas e outras malformações maiores não relacionadas à mielomeningocele foram excluídos da análise. As características anatômicas da mielomeningocele e a sua correção neurocirúrgica foram analisadas quanto aos seguintes desfechos: reanimação neonatal, tempo de internação, necessidade de derivação ventricular, deiscência da ferida operatória, infecção da ferida operatória, infecção do sistema nervoso central e sepse. Para a análise bivariada dos desfechos qualitativos com os fatores de interesse foram empregados testes do qui-quadrado e exato de Fisher. Para a análise do desfecho quantitativo, tempo de internação hospitalar, foram empregados testes de Mann-Whitney. Foram estimados os riscos relativos e os respectivos intervalos com 95% de confiança. Foram desenvolvidos modelos de regressão linear múltipla para os desfechos quantitativos e regressão de Poisson para os desfechos qualitativos. Resultados: Durante o período do estudo 12.559 recém-nascidos foram admitidos no Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Oitenta pacientes foram diagnosticados com mielomeningocele, com incidência de 6,4 casos para cada 1.000 nascidos vivos. Dez pacientes foram excluídos da análise devido à mielomeningocele em topografia cervical (n = 1), à cardiopatia congênita (n = 4), à trissomia do cromossomo 13 (n = 1), à onfalocele (n = 3) e à encefalocele (n = 1). Ocorreram três óbitos (4,28%). Mielomeningocele extensa foi associada a infecção do sistema nervoso central, a complicação de ferida operatória e a maior tempo de internação hospitalar. Os pacientes com mielomeningocele em topografia torácica apresentaram tempo de internação, em média, 39 dias maior que aqueles com defeito em topografia lombar ou sacral. Houve maior necessidade de reanimação em sala de parto entre os pacientes com macrocrania ao nascer. A correção cirúrgica realizada após 48 horas de vida aumentou em 5,7 vezes o risco de infecção do sistema nervoso central. Entre os pacientes operados nas primeiras 48 horas de vida não foi observado benefício adicional na correção cirúrgica realizada em \"tempo zero\". A ausência de hidrocefalia antenatal foi um marcador de bom prognóstico. Nestes pacientes, a combinação dos desfechos necessidade de derivação ventricular, complicações infecciosas, complicações de ferida operatória e reanimação em sala de parto foi 70% menos frequente. Conclusão: Este estudo permitiu identificar marcadores prognósticos de curto prazo em recém-nascidos com mielomeningocele. Os defeitos medulares extensos e a correção cirúrgica após 48 horas de vida influenciaram negativamente na evolução de curto prazo. As lesões extensas foram associadas a maiores taxas de infecção do sistema nervoso central, a complicações de ferida operatória e a internação hospitalar prolongada. A correção cirúrgica realizada após 48 horas de vida aumentou significativamente a ocorrência de infecção do sistema nervoso central. Ausência de hidrocefalia antenatal foi associada a menor número de complicações nos primeiros dias de vida / Introduction: Patients with myelomeningocele have a high mortality and develop neurological disabilities that occur primarily by a defective spinal cord and nerve root development and, secondarily, by acquired post-natal complications. The challenge in the post-natal management of myelomeningocele is the early recognition of cases at risk for complications in order to establish individualized treatment strategies. Objective: This study aims to identify short-term prognostic markers for newborns with myelomeningocele. Anatomical characteristics of the spinal defect and technical aspects of the neurosurgical repair were analyzed for this purpose. Methods: A retrospective cohort study was conducted in 70 patients with thoracic, lumbar or sacral myelomeningocele born between January 2007 and December 2013 in the Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Patients with congenital infection, chromosomal abnormalities and other major malformations unrelated to myelomeningocele were excluded from our analysis. Features of myelomeningocele anatomy and neurosurgical treatment were analyzed for the following outcomes: neonatal resuscitation, length of hospital stay, need for ventricular shunt, wound dehiscence, wound infection, central nervous system infection and sepsis. Relationships between qualitative outcomes and factors of interest were examined using chi-square or Fisher\'s exact tests. The relationships with the quantitative outcome duration of hospital stay were evaluated using the Mann-Whitney tests. Relative risks were estimated with 95% confidence intervals. Multivariate linear regression was used to evaluate the quantitative outcomes and a Poisson regression model was used for the qualitative outcomes. Results: During the study period a total of 12,559 neonates were born in Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Eighty patients were diagnosed with myelomeningocele resulting in an incidence of 6.4 cases per 1000 live births. Ten patients were excluded from our analysis due to cervical myelomeningocele (n = 1), congenital heart disease (n = 4), trisomy 13 (n = 1), omphalocele (n = 3) and encephalocele (n = 1). Three deaths were observed in the study period (4,28%). Large myelomeningocele was associated with central nervous system infection, wound complications and longer hospital stay. Patients with thoracic myelomeningocele required longer hospital stay, on average 39 days longer when compared to patients with lumbar or sacral defects. There was a positive correlation between the need for resuscitation at the delivery room and the presence of macrocrania at birth. Late surgical repair performed after 48 hours of life increased in 5.7 times the risk of central nervous system infection. Among patients operated within the first 48 hours, no additional benefit in interventions held in \"time zero\" was observed. Absence of antenatal hydrocephalus was a favorable prognostic marker. In these cases, the combination of need for ventricular drainage, sepsis, central nervous system infection, complications of surgical site and intervention in the delivery room were 70% lower. Conclusion: This study allowed us to identify short-term prognostic markers for newborns with myelomeningocele. Extensive spinal cord defect and surgical repair after 48 hours of life negatively influenced short-term outcomes. Extensive lesions were associated with higher rates of central nervous system infections, surgical wound complications and prolonged hospital stay. Interventions performed 48 hours after birth significantly increased occurrence of central nervous system infections. Absence of antenatal hydrocephalus was associated with fewer complications in the first days of life

Introdução: Pacientes com mielomeningocele apresentam elevada mortalidade e desenvolvem déficits neurológicos que ocorrem, primariamente, pelo desenvolvimento anormal da medula e de raízes nervosas e, secundariamente, por complicações adquiridas no período pós-natal. O desafio no cuidado desses pacientes é o reconhecimento precoce dos recém-nascidos de risco para evolução desfavorável a fim de estabelecer estratégias terapêuticas individualizadas. Objetivo: Este estudo tem como objetivo identificar marcadores prognósticos de curto prazo para recém-nascidos com mielomeningocele. As características anatômicas do defeito medular e da sua correção neurocirúrgica foram analisadas para esta finalidade. Métodos: Foi realizado um estudo de coorte retrospectiva com 70 pacientes com mielomeningocele em topografia torácica, lombar ou sacral nascidos entre janeiro de 2007 a dezembro de 2013 no Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Pacientes com infecção congênita, anomalias cromossômicas e outras malformações maiores não relacionadas à mielomeningocele foram excluídos da análise. As características anatômicas da mielomeningocele e a sua correção neurocirúrgica foram analisadas quanto aos seguintes desfechos: reanimação neonatal, tempo de internação, necessidade de derivação ventricular, deiscência da ferida operatória, infecção da ferida operatória, infecção do sistema nervoso central e sepse. Para a análise bivariada dos desfechos qualitativos com os fatores de interesse foram empregados testes do qui-quadrado e exato de Fisher. Para a análise do desfecho quantitativo, tempo de internação hospitalar, foram empregados testes de Mann-Whitney. Foram estimados os riscos relativos e os respectivos intervalos com 95% de confiança. Foram desenvolvidos modelos de regressão linear múltipla para os desfechos quantitativos e regressão de Poisson para os desfechos qualitativos. Resultados: Durante o período do estudo 12.559 recém-nascidos foram admitidos no Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Oitenta pacientes foram diagnosticados com mielomeningocele, com incidência de 6,4 casos para cada 1.000 nascidos vivos. Dez pacientes foram excluídos da análise devido à mielomeningocele em topografia cervical (n = 1), à cardiopatia congênita (n = 4), à trissomia do cromossomo 13 (n = 1), à onfalocele (n = 3) e à encefalocele (n = 1). Ocorreram três óbitos (4,28%). Mielomeningocele extensa foi associada a infecção do sistema nervoso central, a complicação de ferida operatória e a maior tempo de internação hospitalar. Os pacientes com mielomeningocele em topografia torácica apresentaram tempo de internação, em média, 39 dias maior que aqueles com defeito em topografia lombar ou sacral. Houve maior necessidade de reanimação em sala de parto entre os pacientes com macrocrania ao nascer. A correção cirúrgica realizada após 48 horas de vida aumentou em 5,7 vezes o risco de infecção do sistema nervoso central. Entre os pacientes operados nas primeiras 48 horas de vida não foi observado benefício adicional na correção cirúrgica realizada em \"tempo zero\". A ausência de hidrocefalia antenatal foi um marcador de bom prognóstico. Nestes pacientes, a combinação dos desfechos necessidade de derivação ventricular, complicações infecciosas, complicações de ferida operatória e reanimação em sala de parto foi 70% menos frequente. Conclusão: Este estudo permitiu identificar marcadores prognósticos de curto prazo em recém-nascidos com mielomeningocele. Os defeitos medulares extensos e a correção cirúrgica após 48 horas de vida influenciaram negativamente na evolução de curto prazo. As lesões extensas foram associadas a maiores taxas de infecção do sistema nervoso central, a complicações de ferida operatória e a internação hospitalar prolongada. A correção cirúrgica realizada após 48 horas de vida aumentou significativamente a ocorrência de infecção do sistema nervoso central. Ausência de hidrocefalia antenatal foi associada a menor número de complicações nos primeiros dias de vida / Introduction: Patients with myelomeningocele have a high mortality and develop neurological disabilities that occur primarily by a defective spinal cord and nerve root development and, secondarily, by acquired post-natal complications. The challenge in the post-natal management of myelomeningocele is the early recognition of cases at risk for complications in order to establish individualized treatment strategies. Objective: This study aims to identify short-term prognostic markers for newborns with myelomeningocele. Anatomical characteristics of the spinal defect and technical aspects of the neurosurgical repair were analyzed for this purpose. Methods: A retrospective cohort study was conducted in 70 patients with thoracic, lumbar or sacral myelomeningocele born between January 2007 and December 2013 in the Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Patients with congenital infection, chromosomal abnormalities and other major malformations unrelated to myelomeningocele were excluded from our analysis. Features of myelomeningocele anatomy and neurosurgical treatment were analyzed for the following outcomes: neonatal resuscitation, length of hospital stay, need for ventricular shunt, wound dehiscence, wound infection, central nervous system infection and sepsis. Relationships between qualitative outcomes and factors of interest were examined using chi-square or Fisher\'s exact tests. The relationships with the quantitative outcome duration of hospital stay were evaluated using the Mann-Whitney tests. Relative risks were estimated with 95% confidence intervals. Multivariate linear regression was used to evaluate the quantitative outcomes and a Poisson regression model was used for the qualitative outcomes. Results: During the study period a total of 12,559 neonates were born in Centro Neonatal do Instituto da Criança do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo. Eighty patients were diagnosed with myelomeningocele resulting in an incidence of 6.4 cases per 1000 live births. Ten patients were excluded from our analysis due to cervical myelomeningocele (n = 1), congenital heart disease (n = 4), trisomy 13 (n = 1), omphalocele (n = 3) and encephalocele (n = 1). Three deaths were observed in the study period (4,28%). Large myelomeningocele was associated with central nervous system infection, wound complications and longer hospital stay. Patients with thoracic myelomeningocele required longer hospital stay, on average 39 days longer when compared to patients with lumbar or sacral defects. There was a positive correlation between the need for resuscitation at the delivery room and the presence of macrocrania at birth. Late surgical repair performed after 48 hours of life increased in 5.7 times the risk of central nervous system infection. Among patients operated within the first 48 hours, no additional benefit in interventions held in \"time zero\" was observed. Absence of antenatal hydrocephalus was a favorable prognostic marker. In these cases, the combination of need for ventricular drainage, sepsis, central nervous system infection, complications of surgical site and intervention in the delivery room were 70% lower. Conclusion: This study allowed us to identify short-term prognostic markers for newborns with myelomeningocele. Extensive spinal cord defect and surgical repair after 48 hours of life negatively influenced short-term outcomes. Extensive lesions were associated with higher rates of central nervous system infections, surgical wound complications and prolonged hospital stay. Interventions performed 48 hours after birth significantly increased occurrence of central nervous system infections. Absence of antenatal hydrocephalus was associated with fewer complications in the first days of life

Myelomeningocele (MM) is a disease that causes motor and sensitive disabilities. Health problems associated to this condition may lead mothers the main caregivers to situations of stress, anxiety and depression that may impair their health-related quality of life (HRQL). The aim of this study was to assess the HRQL of mothers of children and adolescents with MM in comparison to mothers of healthy controls and to observe its association with clinical and demographic variables of patients and mothers. The study group consisted of 50 mothers of children and adolescents with MM under 18 years of age in treatment at the Associação de Assistência à Criança Deficiente (AACD-MG). Mothers of healthy controls were matched by patients gender and age in the proportion of 2:1. Patients were grouped by level of neurological lesion and status of functional ambulation. Clinical and demographic data were obtained by interview and medical records. The HRQL of mothers was assessed by interview through the Medical Outcomes Study 36-Item Short Form Health Survey (SF-36). SF-36 reliability was evaluated by Cronbach s alpha coefficient. The Beck Depression Inventory (BDI) was self-applied to assess depressive symptoms. SF-36 scores were compared between groups of mothers of patients with MM and controls (Mann-Whitney test). Effect sizes (ES) were calculated to measure the m agnitude of significant differences. Clinical and demographic variables were compared (Mann-Whitney and Kruskal-Wallis tests) and correlated (Spearman correlation coefficient) with SF-36 scores. Mothers median age was 35.9 years (study group) and 33.5 years (control group). The median age of children with MM was 6.0 years. Most of the group consisted of children (90.0%), females (58.0%) and patients with lower lumbar neurological lesion (50.0%), non-ambulators (44.0%). Cronbach s alpha ranged from 0.6 to 0.8 for the SF-36 domains and components. In comparison to mothers of healthy controls, mothers of children/adolescents with MM had significantly lower scores in the physical component and in three SF-36 domains: role limitations due to physical problems, global health and role limitations due to social problems (p<0.05). Effect size was large (ES>0.8) in the following domains: role limitations due to physical problems and role limitations due to social problems. There was no difference in SF-36 scores according to patients level of neurological lesion and functional ambulation status (p>0.05). Among all patients and mothers clinical and demographic variables, patients gender (female), catheterization and presence of chronic disease in mothers were associated to impaired HRQL (p<0.05). The intensity of depressive symptoms was correlated to all domains and components of the SF-36 (p<0.05). In conclusion, mothers of children and adolescents with MM had a mental and mostly physical impairment in their HRQL in comparison to mothers of healthy controls. The impact was similar among the different levels of neurological lesion or functional ambulation status. Few associations were found among other clinical and demographic variables. / A Mielomeningocele (MM) ocasiona dificuldades motoras e sensitivas. Os diversos problemas de saúde associados a essa condição podem gerar na mãe, principal cuidador, estresse, ansiedade, preocupação excessiva, depressão e impactar sua qualidade de vida relacionada à saúde (QVRS). Diante disso, este estudo teve como objetivo avaliar a QVRS de mães de crianças/adolescentes com MM em relação às mães de crianças/adolescentes saudáveis, e verificar a sua associação com variáveis clínicas e demográficas dos pacientes e das mães. Participaram do estudo 50 mães de crianças e adolescentes até 18 anos de idade e diagnóstico de MM, em acompanhamento na Associação de Assistência à Criança Deficiente (AACD-MG) e mães de crianças e adolescentes saudáveis, pareadas por sexo e idade dos pacientes na proporção de 2:1. Os pacientes foram classificados em grupos segundo o nível de lesão neurológica e padrão de deambulação. Os dados demográficos e clínicos foram obtidos por entrevista e completados com informações contidas no prontuário médico. A QVRS das mães foi avaliada por meio de entrevista pelo Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) e a confiabilidade foi verificada pelo coeficiente de alfa Cronbach. O Inventário de Depressão de Beck (IDB) foi respondido de maneira auto-aplicada para verificar os sintomas depressivos. Os escores do SF-36 foram comparados entre os grupos de mães de pacientes com MM e controle (teste de Mann-Whitney), o tamanho do efeito (T.E) foi calculado para medir a magnitude das diferenças significativas. As variáveis demográficas e clínicas foram comparadas (teste de Mann-Whitney e Kruskal-Wallis) e correlacionadas (coeficiente de correlação de Spearman) com os escores do SF-36. A mediana de idade das mães de crianças/adolescentes com MM foi de 35,9 anos e no controle de 33,5 anos. Em relação às crianças/adolescentes com MM, a mediana das idades foi de 6,0 anos, com predomínio de crianças (90,0%) e do sexo feminino (58,0%). Houve predomínio do nível de lesão neurológica lombar baixo (50%) e não deambulador (44%). O coeficiente de alfa Cronbach.variou de 0,6 a 0,8 para os domínios e componentes do SF-36. As mães de crianças/adolescentes com MM obtiveram escores do SF-36 significativamente menores no componente físico e em 3 domínios: aspectos físicos, estado geral de saúde e aspectos sociais (p<0,05). O tamanho do efeito foi grande (T.E&#8805;0,8) nos domínios aspectos físicos e aspectos sociais. Não ocorreu diferença entre os pacientes segundo o nível de lesão neurológica e o padrão de deambulação (p>0,05). Dentre as variáveis demográficas e clínicas das mães e pacientes avaliadas, houve associação com prejuízo na QVRS das mães segundo, sexo do paciente (feminino), realização do cateterismo e presença de doença crônica nas mães (p<0,05). A intensidade de sintomas depressivos correlacionou-se com todos os domínios e componentes do SF-36, sendo a variável que mais se associou com os escores do SF-36 (p<0,05). Concluiu-se que mães de crianças/adolescentes com MM apresentam prejuízo mental e principalmente físico na QVRS em comparação às mães de crianças/adolescentes saudáveis. O impacto foi semelhante entre os diferentes níveis de lesão ou padrões de deambulação. Poucas associações foram encontradas entre as outras variáveis demográficas e clínicas analisadas. / Mestre em Ciências da Saúde

This was an exploratory-descriptive study of fifty children afflicted with myelomeningocele, ages one through six, who were known to the Myelomeningocele Clinic of the Crippled Children’s Division. The study identified the degree of multiple physical, emotional, and environmental stress factors that families must be prepared to cope with. The study identified eleven factors felt to play an important role in family dynamics and how they related to the families response to their child with myelomeningocele. The factors were tested and found to be valid by the use of a pre-test on ten case records. Medical records were then obtained from the Crippled Children's Division for chart review purposes and the appropriate material was recorded. Scores were developed that indicated the degree of stress ranging from minimal involvement to maximum involvement. The study found that a majority of the families in the sample live within commuting distance to needed medical services, have transportation available to them and generally utilize the necessary medical care appropriately. The remainder of the study showed, however, that families could be expected to face a variety of other problems that could only serve to increase family stress. Most of the families had limited financial resources. Over one-half of the families needed special education for their children. A majority of families had no medical insurance. Fifty-eight percent of the families were found to have additional stressful problems to cope with e. g., marital stress, sibling rivalry, additional ill members, etc. Added to this was the information that the child with myelomeningocele was found to be greatly involved in a multiplicity of medical problems at many different levels of functioning. e. g., orthopedic, bowel, neurosurgical, etc., that would be expected to add to the already stressful family dynamics. Among the recommendations developed was a plea for the expansion of the satellite clinic concept, development of parent groups on a geographical basis, development of educational programs for educators and community service personnel, brief orientation programs for parents with the goal of helping them understand and integrate the health care system that they find themselves in.