Myelomeningocele : Surgical treatment and results

Protzenko

et al. 2017

Arq. Neuro-Psiquiatr.

ARTICLEThe mortality rate after hospital discharge in patients with myelomeningocele decreased after implementation of mandatory flour fortification with folic acid Redução da taxa de mortalidade após a alta hospitalar em pacientes com mielomeningocele depois da implementação da fortificação obrigatória das farinhas com ácido fólico Myelomeningocele (MMC), or spina bifida cystica, is the most common neural tube defect, developing between days 21 and 28 after fertilization 1,2 . The lack of a protective lining exposes the neural tissue to gradual destruction due to the deleterious effects of amniotic fluid over the neural plate 3 .The prevalence of MMC varies according to the geographic, racial, and ethnic characteristics of the population being studied, and may differ greatly between different regions of the same continent or country 4,5 . In Brazil, the prevalence of Chiari II malformation (CMII) is estimated to range between 1.4 and 1.5 per 10,000 births 6,7 . The cause of MMC is multifactorial, but folic acid (FA) deficiency is the main risk factor. Chromosomal and genetic abnormalities, maternal hyperthermia during the early stages of pregnancy, use of ABSTRACTObjective: To evaluate the mandatory folic acid fortification of flour on mortality rates after the hospital discharge of children born with myelomeningocele, the most affected age group and the most frequent cause of death. Methods: A retrospective study of 383 children born with myelomeningocele from January 1990 to December 2013 in a high-fetal-risk reference hospital. Results: A total of 39 patients died (10.1%), of which 23 (6%) died after discharge. Most children who died were younger than 12 months of age. The most frequent cause of death was infection of the central nervous system, followed by urinary tract sepsis and infections of the respiratory system. Symptomatic Chiari II malformation was the most frequent comorbidity factor. Conclusion: Although there was no significant difference in infant mortality before and after folic acid fortification, there was a significant reduction in deaths after hospital discharge in babies born after implementation of mandatory folic acid fortification Keywords: meningomyelocele; mortality; folic acid. RESUMOObjetivo: Avaliar a fortificação obrigatória de farinhas com ácido fólico nas taxas de mortalidade após a alta hospitalar de crianças nascidas com mielomeningocele, a faixa etária mais atingida e a causa mais freqüente de morte. Métodos: Estudo retrospectivo de 383 crianças nascidas com mielomeningocele de janeiro de 1990 a dezembro de 2013, em um hospital de referência de alto risco fetal. Resultados: Um total de 39 pacientes morreram (10,1%), dos quais 23 (6%) morreram após a alta. A maioria das crianças que morreram não alcançaram 12 meses de idade. A causa mais frequente de morte foi infecção do sistema nervoso central, seguido por sépsis, infecções do trato urinário e do sistema respiratório. A malformação de Chiari Tipo II foi a comorbidade mais freqüente. Conclusão: Apesar de não haver diferença ...

mentioning

confidence: 99%

The mortality rate after hospital discharge in patients with myelomeningocele decreased after implementation of mandatory flour fortification with folic acid

Protzenko

et al. 2017

Arq. Neuro-Psiquiatr.

Clinical and ultrasonographic criteria for using ventriculoperitoneal shunts in newborns with myelomeningocele

Melo¹,

Pacheco²,

Melo³

et al. 2015

Arq. Neuro-Psiquiatr.

Objective Hydrocephalus is one of the main complications associated with myelomeningocele (MM). This study aimed to identify clinical and ultrasonographic criteria for using ventriculoperitoneal (VP) shunts in this group of patients.Method A retrospective cohort study, based on established protocol for VP shunt implant in hydrocephalic children with MM. Parameters used to guide the indication of VP shunts included measurement of head circumference (HC), evaluation of fontanels, and measurement of lateral ventricular atrium (LVA) width by transcranial ultrasonography.Results 43 children were included in the analysis, of which 74% had hydrocephalus and required a VP shunt. These children had LVA width ≥ 15 mm, showed increased HC, or had bulging fontanels.Conclusion VP shunt is required in children with increased HC (≥ 2 standard deviation regarding age group), bulging fontanels, or LVA width of ≥ 15 mm after the closure of MM.

Perfil dos Pacientes Portadores de Mielomeningocele na Cidade de Mogi das Cruzes

Bergamaschi¹,

Faria²,

Santos³

2001

Rev Neurocienc

original RESUMOA mielomeningocele (MMC) é uma malformação congênita frequente e ocorre entre a terceira e quinta semana de gestação. O objetivo deste trabalho foi verificar o perfil e o nível sócio-econômico dos pacientes na cidade de Mogi das Cruzes. Foram aplicados dois questionários aos pais de crianças com MMC, um direcionado à formação do perfil dos pacientes e outro para observar o nível sócio-econômico. Os resultados demonstraram que houve maior prevalência no sexo feminino, com maior incidência na região lombar baixa. A maioria dos casos foi diagnosticado no período pré-natal e submetidos a correção nas primeiras 48 horas de nascimento. A hidrocefalia estava presente em todos os casos, sendo a troca da derivação ventrículo peritoneal realizada na metade deles. Todas as crianças tiveram infecção urinária, e a maioria deformidades ortopédicas, tendo o maior índice, o pé torto congênito. Dos casos em análise quase 50% foram submetidos a cirurgias ortopédicas corretivas. Embora todos utilizassem órtese suropodálica, apenas 62,5% realizavam fisioterapia. A maioria estudava em escolas regulares e pertencentes da classe social C. Apenas 56,25% dos pais apresentavam conhecimento adequado sobre a patologia. Concluiu-se a grande importância do conhecimento do perfil dos pacientes para que os profissionais possam realizar de forma mais adequada seus tratamentos e orientações. ABSTRACTMyelomeningocele (MMC) is a congenital malformation and often occurs between the third and fifth week of gestation. The objective of this work was to verify the profile and socioeconomic level of the patients with MMC in Mogi das Cruzes. Two questionnaires were applied to parents of children with MMC, one seeking profile and the other the socioeconomic level of the patients. The results showed higher prevalence among females, with higher incidence in lower lumbar region. Most cases were diagnosed in the prenatal period and submitted to the correction within 48 hours of birth. Hydrocephalus was present in all cases, ventriculoperitoneal shunt was done in half of the cases. All children had urinary infection, the most presented orthopedic deformities, the majority have presented congenital clubfoot. Almost 50% of the analyzed cases performed surgery to repair it. Although all used ankle-foot orthoses, only 62.5% practiced physiotherapy. For the social economic and cultural aspect the most were studying in regular schools and socially they belong to class C. Only 56.25% of parents had good knowledge about the disease which victimize their children. It was concluded the great importance of knowing the patient profile so that professionals can perform more adequately their treatments and guidelines.