Opciones quirúrgicas para la cobertura de defectos del cierre del tubo neural. Serie de casos / Surgical options for coverage of neural tube closure defects. Case series

Introducción. Los defectos del tubo neural (DNT) son la segunda causa más prevalente de malformaciones congénitas, solo detrás de las cardiopatías congénitas. Los DTN abiertos comprenden el 80% de estas malformaciones. El mielomeningocele es el defecto abierto más común del tubo neural. La mayoría de los defectos son lo suficientemente pequeños como para soportar un cierre por primera o por segunda intención, pero el cierre primario puede no ser posible en aproximadamente 25% de los casos, lo cual es evidente en defectos grandes. Pacientes y métodos. El trabajo a continuación tiene como objetivo mostrar la experiencia en el manejo quirúrgico posnatal a través de una serie de caso con 3 pacientes con diagnóstico de defectos de cierre del tubo neural en distintas localizaciones anatómicas sometidos a cirugía en forma conjunta entre el Servicio de Neurocirugía y Cirugía Plástica del Parque de la Salud en la ciudad de Posadas Misiones. Resultados. En nuestra serie los colgajos realizados para la cobertura de mielomeningoceles fueron eficaces, evolucionando en primera instancia con complicaciones menores. La resolución del caso de encefalocele occipital con un colgajo miocutáneo de trapecio diferido resultó ser una opción válida y confiable. Conclusión. El uso de colgajos regionales para la reconstrucción de este tipo de malformaciones congénitas es una opción válida y reproducible, con bajo porcentaje de complicaciones.

Introduction. Neural tube defects (NTD) are the second most prevalent cause of congenital malformations, only behind congenital heart defects. Open NTDs comprise 80% of these malformations. Myelomeningocele is the most common open neural tube defect. Most defects are small enough to support a first or second intention closure, but primary closure may not be possible in approximately 25% of cases, being evident in large defects. Patients and methods. The following work aims to show the experience in postnatal surgical management through a case series with 3 patients diagnosed with neural tube closure defects in different anatomical locations undergoing joint surgery between the Neurosurgery and Plastic Surgery Service of Parque de la Salud in the city of Posadas, Misiones. Results. In our series, the flaps made to cover myelomeningoceles were effective, evolving in the first instance with minor complications. Resolution of the occipital encephalocele case with a delayed trapezius myocutaneous flap proved to be a valid and reliable option. Conclusion. The use of regional flaps for the reconstruction of this type of congenital malformations is a valid and reproducible option, with a low percentage of complications.

Diferencias en la duración del reflejo rectoanal inhibitorio en pacientes con constipación funcional refractaria y mielomeningocele / Differences in rectoanal inhibitory reflex duration between patients with refractory functional constipation and myelomeningocele

Introducción. Habitualmente, durante la manometría anorrectal, en lo correspondiente al reflejo rectoanal inhibitorio (RRAI) solo se pesquisa su presencia o ausencia. Estudios han reportado que su análisis detallado puede brindar datos de interés. Nuestra hipótesis es que la medición del RRAI puede dar información para reconocer causas orgánicas (médula anclada, lipoma, etc.) en pacientes en los que previamente se consideró como de causa funcional. Objetivos. Comparar la duración del reflejo rectoanal inhibitorio en la manometría anorrectal de pacientes con constipación funcional refractaria (CFR) y mielomeningocele (MMC). Población y métodos. Estudio observacional, transversal, analítico (2004-2019). Pacientes constipados crónicos con incontinencia fecal funcional y orgánica (mielomeningocele). Se les realizó manometría anorrectal con sistema de perfusión de agua y se midió la duración del RRAI con diferentes volúmenes (20, 40 y 60 cc). Grupo 1 (G1): 81 CFR. Grupo 2 (G2): 54 MMC. Se excluyeron pacientes con retraso madurativo, esfínter anal complaciente, agenesia sacra y aquellos no colaboradores. Resultados. Se incluyeron 135 sujetos (62 varones). La mediana de edad fue G1:9,57 años; G2: 9,63 años. Duración promedio G1 vs. G2 con 20 cc: 8,89 vs. 15,21 segundos; con 40 cc: 11.41 vs. 21,12 segundos; con 60 cc: 14,15 vs. 26,02 segundos. La diferencia de duración del RRAI entre ambos grupos con diferentes volúmenes fue estadísticamente significativa (p = 0,0001). Conclusión. La duración del RRAI aumenta a mayor volumen de insuflación del balón en ambas poblaciones. Pacientes con MMC tuvieron mayor duración del RRAI que aquellos con CFR. En los pacientes con RRAI prolongado, debe descartarse lesión medular.

Introduction. Usually, during anorectal manometry, only the presence or absence of rectoanal inhibitory reflex (RAIR) is investigated. Studies have reported that a detailed analysis may provide data of interest. Our hypothesis is that RAIR measurement may provide information to detect organic causes (tethered cord, lipoma, etc.) in patients in whom a functional cause had been previously considered. Objectives. To compare RAIR duration in anorectal manometry between patients with refractory functional constipation (RFC) and myelomeningocele (MMC). Population and methods. Observational, analytical, cross-sectional study (2004­2019). Patients with chronic constipation and functional and organic fecal incontinence (myelomeningocele). The anorectal manometry was performed with a water-perfused system, and the duration of RAIR was measured with different volumes (20, 40, and 60 cc). Group 1 (G1): 81 RFC. Group 2 (G2): 54 MMC. Patients with developmental delay, compliant anal sphincter, sacral agenesis and non-cooperative patients were excluded. Results. A total of 135 individuals were included (62 were male). Their median age was 9.57 years in G1 and 9.63 years in G2. Average duration in G1 versus G2 with 20 cc: 8.89 versus 15.21 seconds; 40 cc: 11.41 versus 21.12 seconds; 60 cc: 14.15 versus 26.02 seconds. The difference in RAIR duration with the varying volumes was statistically significant (p = 0.0001). Conclusion. RAIR duration was longer with increasing balloon inflation volumes in both populations. RAIR duration was longer in patients with MMC than in those with RFC. Spinal injury should be ruled out in patients with prolonged RAIR.

Procedimiento clínico-quirúrgico en una paciente embarazada con una derivación urológica compleja. Reporte de un caso / Clinical-surgical approach in a pregnant patient with complex urological diversion. A case report

Resumen ANTECEDENTES: El mielomeningocele es el defecto del tubo neural más frecuente; se registra un caso por cada 1000 nacidos vivos. Es más frecuente en mujeres, que resultan con secuelas discapacitantes, entre ellas las urológicas. Pueden subsanarse con diferentes técnicas quirúrgicas y derivaciones urológicas complejas que las predispone a infecciones de vías urinarias de repetición y, cuando hay embarazo, complicaciones materno-fetales y dificultad para el acceso quirúrgico, en caso de cesárea. CASO CLÍNICO: Paciente con 14 semanas de embarazo, 27 años de edad, con diagnóstico de mielomeningocele y vesicoplastia, con múltiples infecciones urinarias y ruptura de membranas pretérmino. Finalización del embarazo por cesárea a las 33 semanas, por indicación de las condiciones fetales. La técnica de la cesárea se modificó para no dañar la cistoplastia y evitar complicaciones con las adherencias en la pelvis. CONCLUSIONES: El mejor pronóstico materno-fetal en pacientes con derivación urológica compleja se consigue con la búsqueda intencionada de infecciones urinarias y tratamiento oportuno y adecuado, además de una planificación multidisciplinaria al momento de la finalización del embarazo.

Abstract BACKGROUND: Myelomeningocele is the most common neural tube defect; one case per 1000 live births is reported. It is more frequent in females, resulting in disabling sequelae, including urological sequelae. They can be corrected with different surgical techniques and complex urological derivations that predispose them to repeated urinary tract infections and, when there is pregnancy, maternal-fetal complications and difficulty for surgical access, in case of cesarean section. CLINICAL CASE: Patient 14 weeks pregnant, 27 years old, diagnosed with myelomeningocele and vesicoplasty, with multiple urinary tract infections and preterm rupture of membranes. Termination of pregnancy by cesarean section at 33 weeks, due to fetal conditions. The cesarean section technique was modified so as not to damage the cystoplasty and to avoid complications with adhesions in the pelvis. CONCLUSIONS: The best maternal-fetal prognosis in patients with complex urologic diversion is achieved with the intentional search for urinary tract infections and timely and adequate treatment, in addition to multidisciplinary planning at the time of termination of pregnancy.

Presentación de un meningocele lumbosacro en un adulto / Case Presentation of a ruptured lumbosacral meningocele in an adult patient

La espina bífida, o mielodisplasia, es una anomalía de origen multifactorial congénita que se presenta con mayor frecuencia durante el desarrollo embrionario. Se produce por el cierre parcial de los pliegues neurales conjuntamente con una fusión defectuosa de los arcos vertebrales. Su clínica es variable e incluye una serie de manifestaciones como expresión del compromiso neurológico. El diagnóstico se basa en la presencia de las manifestaciones clínicas ayudado de estudios imagenológicos. La corrección quirúrgica del defecto es la conducta terapéutica que se preconiza como adecuada. Se presenta el caso de una paciente de 34 años de edad con mielomeningocele no corregido en edad infantil que se presenta con complicaciones infecciosas y secuelas neurológicas. Se administró esquema de antibioticoterapia con varios antibióticos de amplio espectro, y se decidió intervenir quirúrgicamente, con lo que se logró el cierre del orificio comunicante de la región espinal con el exterior. Además, se reconstruyó la zona aledaña al mielomeningocele para evitar posible sepsis del Sistema Nervioso Central. La paciente tuvo una evolución favorable y fue dada de alta hospitalaria a los 7 días posteriores a la intervención quirúrgica sin secuelas neurológicas(AU)

Spine bifida, or myelodysplasia, is a multifactorial congenital anomaly that occurs most frequently during embryonic development. It is produced by the partial closure of the neural folds together with a defective fusion of the vertebral arches. Its clinic is variable and includes a series of manifestations as an expression of neurological compromise. The diagnosis is based on the presence of clinical manifestations, aided by imaging studies. Surgical correction of the defect is the therapeutic conduct that is recommended as adequate. This report presents the case of a 34-year-old patient with uncorrected myelomeningocele in childhood who presented with infectious and neurological sequelae complications(AU)

Historical Clinical Outcomes of Children with Myelomeningocele Meeting the Criteria for Fetal Surgery: A Retrospective Cohort Survey of Brazilian Patients / Resultados clínicos históricos de crianças com mielomeningocele com critérios para cirurgia fetal: Um estudo de coorte retrospectivo de pacientes brasileiros

Abstract Objective To analyze the historical clinical outcomes of children with myelomeningocele (MMC) meeting the criteria for fetal surgery, but who underwent postnatal primary repair. Methods Data from children undergoing postnatal MMC repair between January 1995 and January 2015 were collected from the Neurosurgery Outpatient Clinic's medical records. Children were included if they had ≥1 year of postoperative follow-up andmet the criteria for fetal surgery. The children's data were then stratified according to whether they received a shunt or not. The primary outcome was mortality, and secondary outcomes were educational delays, hospitalization, recurrent urinary tract infections (UTIs), and renal failure. Results Over the 20-year period, 231 children with MMC were followed up. Based on clinical data recorded at the time of birth, 165 (71.4%) qualify of fetal surgery. Of the 165 patients, 136 (82.4%) underwent shunt placement. The mortality rate was 5.1% in the group with shunt and 0% in the group without, relative risk (RR) 3.28 (95% confidence interval, 95% CI, 0.19-55.9). The statistically significant RRs for adverse outcomes in the shunted group were 1.86 (95% CI, 1.01-3.44) for UTI, 30 (95% CI, 1.01-537) for renal failure, and 1.77 (95% CI, 1.09-2.87) for hospitalizations. Conclusion Children with MMC qualifying for fetal surgery who underwent shunt placement were more likely to have recurrent UTIs, develop renal failure, and be hospitalized. Since approximately half of the shunt procedures could be avoided by fetal surgery, there is a clinical benefit and a possible financial benefit to the implementation of this technology in our setting.

Resumo Objetivo Analisar os resultados clínicos históricos de crianças commielomeningocele (MMC) com critérios para cirurgia fetal,mas que foram submetidas a cirurgia pós-natal. Métodos Dados de crianças submetidas à correção deMMCpós-natal entre janeiro de 1995 e janeiro de 2015 foram coletados nos prontuários do Ambulatório de Neurocirurgia. Foram incluídas crianças se tivessem ≥ 1 ano de acompanhamento pósoperatório e atendessem os critérios para cirurgia fetal. As informações dessas crianças foram então estratificadas de acordo com se receberam ou não derivação do líquido cefalorraquidiano. O desfecho primário foi a mortalidade e os desfechos secundários foram atrasos educacionais, hospitalização, infecções recorrentes do trato urinário einsuficiência renal. Resultados Durante o período de 20 anos, 231 crianças com MMC foram acompanhadas. Com base nos dados clínicos registrados no momento do nascimento, 165 (71,4%) atendiam critérios para a cirurgia fetal. Dos 165 pacientes, 136 (82,4%) foram submetidos à colocação de derivação do líquido cefalorraquidiano. A taxa de mortalidade foi de 5,1% no grupo com derivação do líquido cefalorraquidiano e 0% no grupo sem risco relativo (RR) 3,28 (intervalo de confiança 95%, IC 95%, 0,19-55,9). Os RRs estatisticamente significativos para resultados adversos no grupo com derivação do líquido cefalorraquidiano foram 1,86 (IC 95%, 1,01-3,44) para infecção do trato urinário, 30 (IC 95%, 1,01-537) para insuficiência renal e 1,77 (IC 95%, 1,09-2,87) para hospitalizações. Conclusão Crianças com MMC com critérios para cirurgia fetal submetidas à colocação de derivação do líquido cefalorraquidiano eram mais propensas a ter infecções recorrentes do trato urinário, desenvolver insuficiência renal e serem hospitalizadas. Como aproximadamente metade dos procedimentos de derivação poderiam ser evitados por cirurgia fetal, há um benefício clínico e um possível benefício financeiro com a implementação dessa tecnologia em nosso meio.

Avaliação dos fatores relacionados ao sucesso da terceiro ventriculostomia endoscópica na hidrocefalia associada à mielomeningocele / Evaluation of factors related to the success of endoscopic third ventriculostomy in hydrocephalus associated with myelomeningocele

Introdução: A mielomeningocele (MMC) é o defeito do tubo neural compatível com a vida mais comum, e uma de suas complicações mais frequentes é a ocorrência de hidrocefalia, principalmente em pacientes cuja correção do defeito se deu no período pós-natal. O tratamento padrão para esta população ainda é a inserção de uma derivação ventrículo-peritoneal (DVP), associada a complicações e maior morbimortalidade. Alternativamente à DVP, destaca-se a terceiro ventriculostomia endoscópica (TVE), procedimento sem a necessidade de dispositivo implantável, mas que apresenta uma taxa de sucesso muito variável na literatura médica. Objetivos: Avaliar a taxa de sucesso da TVE no tratamento da hidrocefalia associada à MMC, identificar possíveis fatores clínicos e/ou radiológicos relacionados ao sucesso do procedimento e identificar se o sucesso estimado pelo Endoscopic Third Ventriculostomy Success Score (ETVSS) foi comparável à real taxa de sucesso. Método: Vinte e um pacientes com o diagnóstico de MMC, nascidos entre 2015 e 2021 e submetidos a TVE para o tratamento da hidrocefalia foram incluídos nesse estudo. Pacientes com correção fetal da MMC foram excluídos. Dados clínicos como o perímetro cefálico ao nascimento e no pré-operatório prematuridade, presença de DVP, número de revisões e presença de infecção prévias, idade no momento da TVE, além de fatores radiológicos como o índice de Evans, ângulo clivo-occipital, volume estimado da fossa posterior e volume estimado do compartimento supratentorial foram analisados. Resultados: A taxa de sucesso em seis meses foi de 61,9%, a taxa geral de sucesso foi de 57,1% e o ETVSS médio calculado foi de 46,7%. O único fator com aparente relevância para o sucesso da TVE foi a idade, pacientes com mais de 6 meses completos apresentaram uma taxa de sucesso de 76,9% enquanto os mais novos apresentaram uma taxa de sucesso de 25% (OR: 0,03; IC 95% 0,01-0,35; p = 0,013). Não foi identificado nenhum fator radiológico associado ao sucesso do procedimento. Conclusões: O único fator de relevância estatística com o sucesso da TVE foi a idade, sendo que pacientes com idade inferior a 6 meses completos apresentaram uma probabilidade maior de falha. Já o ETVSS subestimou a taxa de sucesso do procedimento em 6 meses ­ o que demonstra a necessidade de modificações para seu aprimoramento em pacientes mais jovens.

Introduction: Myelomeningocele (MMC) is the most common neural tube defect compatible with life, and one of its most frequent complications is the occurrence of hydrocephalus (HCF), especially in patients whose defect correction took place in the postnatal period. The standard treatment for this population is still the insertion of a ventriculoperitoneal shunt (VPS), but it is associated with complications and higher morbidity and mortality. As an alternative to DVP, endoscopic third ventriculostomy (ETV) stands out, it is a procedure without the need for an implantable device, but its success rate is inconsistent in the medical literature. Objectives: To evaluate the success rate of EVT in the treatment of hydrocephalus associated with MMC and to identify possible clinical and/or radiological features associated with ETV success. Methods: Twenty-one patients with myelomeningocele, born between 2015 and 2021, that underwent EVT for hydrocephalus were included in this study. Patients with fetal MMC correction were excluded. Clinical data such as head circumference at birth and preoperatively, prematurity, presence of previous VPS, number of previous revisions, presence of a previous infection, age at the time of ETV, in addition to radiological factors such as the Evans index, clivo-occipital angle, the estimated volume of the posterior fossa and estimated volume of the supratentorial compartment were analyzed. Results: The six-month success rate was 61.9%, the overall success rate was 57.1%, and the mean calculated ETVSS was 46.7%. The only factor with apparent relevance to the success of ETV was age: the patients older than six full months had a success rate of 76.9%, while the younger ones had a success rate of 25% (OR: 0.03; 95% CI 0.01-0.35; p = 0.013). No radiological features were identified as associated with the procedure's success. Conclusions: The only factor of statistical relevance with the success of TVE was age, with patients younger than six full months of age having a higher probability of failure. The ETVSS, on the other hand, underestimated the procedure's success rate at six months, which shows a need for modifications to improve its results when applied in younger patients.

Comparison between two methods of measuring pelvic obliquity in cerebral palsy and myelomeningocele / Comparação entre dois métodos de medida de obliquidade pélvica em paralisia cerebral e mielomeningocele / Comparación entre dos métodos de medición de la oblicuidad pélvica en parálisis cerebral y mielomeningocele

ABSTRACT Objective: To evaluate the intra- and inter-observer reproducibility of the evaluation of the pelvic obliquity (PO) in patients with neuro-muscular deformities via the method that uses the iliac crests and the method that uses the upper endplate of S1 and to determine whe ther there is a relationship between the methods. Methods: The digitized panoramic radiographies of thirty patients with cerebral paralysis or myelomeningocelein outpatient monitoring were evaluated by four examiners: two experienced spinal surgeons and two fellows. Two radiographs were excluded because analysis was impossible. All exams were obtained in accordance with the periodic monitoring protocol in the sitting position, using digitized film and a film-focus distance of 110 cm Results: High intra- and inter-observer agreement was observed both for method that uses the iliac crests and the method that evaluates the S1 endplate. However, no significant relationship between the two methods was observed. Conclusions: The methods evaluated had good reproducibility and agreement among the observers. It was confirmed that, on account of the existent linear relation, it is possible to estimate the value of the iliac crest method knowing the value obtained by the S1 plateau multiplied by 0.76. There was no agreement between the iliac crest and S1 plateau PO evaluation methods. Level of evidence IV; Retrospective cross-sectional study.

RESUMO Objetivo: Estimar a reprodutibilidade intra e interobservadores da avaliação da obliquidade pélvica (OP) nos pacientes com deformidades neuromusculares pelo método que usa as cristas ilíacas e pelo método que usa o platô superior de S1e avaliar se há relação entre os métodos. Métodos: Trinta radiografias panorâmicas digitalizadas de pacientes com paralisia cerebral e mielomeningocele acompanhados em ambulatório foram avaliadas por quatro examinadores, sendo dois cirurgiões de coluna experientes e doisfellows. Foram excluídas duas radiografias que impossibilitavam a análise. Todos os exames foram obtidos conforme protocolo de acompanhamento periódico, na posição sentada, em filme digitalizado e distância foco-filme de 110 cm. Resultados: Observou-se alta concordância intra e interobservadores tanto do método que usa as cristas ilíacas quanto do método que avalia o platô de S1. No entanto, não foi observada relação significativa entre os dois métodos. Conclusões: Os métodos avaliados apresentam boa reprodutibilidade e concordância entre os observadores. Verificou-se que, por conta da relação linear existente, é possível estimar o valor do método das cristas ilíacas conhecendo o valor obtido pelo método do platô de S1 multiplicado por 0,76. Não houve concordância entre o método de avaliação da OP pelas cristas ilíacas em comparação com o método de avaliação pelo platô de S1. Nível de evidência IV; Estudo transversal retrospectivo.

RESUMEN Objetivo: Estimar la reproducibilidad intra e interobservador de la evaluación de la oblicuidad pélvica (OP) en pacientes con deformidades neuromusculares por el método que utiliza las crestas ilíacas y por el método que utiliza la meseta superior de S1 y evaluar si existe una relación entre los métodos. Métodos: Treinta radiografías panorámicas digitales de pacientes con parálisis cerebral y mielomeningocele acompañadosen clínica ambulatoria fueron evaluados por cuatro examinadores, dos cirujanosde columna de vasta experiencia y dos fellows. Se excluyeron dos radiografías porque imposibilitaban el análisis. Todos los exámenes se obtuvieron según el protocolo de acompañamiento periódico, en posición sentada, sobre película digitalizada y distancia foco-película de 110 cm. Resultados: Se observó una alta concordancia intra e interobservador tanto en el método que utiliza las crestas ilíacas como en el que evalúa la meseta de S1. Sin embargo no se observó ninguna relación significativa entre los dos métodos. Conclusiones: Los métodos evaluados presentaron buena reproducibilidad y concordancia entre los observadores. Se observó que, debido a la relación lineal existente, es posible estimar el valor del método de las crestas ilíacas conociendo el valor obtenido por el método demeseta de S1 multiplicado por 0,76. No hubo concordancia entre el método de evaluación de la OP por las crestas ilíacas en comparación conel método de evaluación por meseta de S1. Nivel de evidencia IV; Estudio transversal retrospectivo.

Experiencia con el uso de toxina botulínica en niños con vejiga hiperactiva neurogénica: Una serie de casos / Experience with Botulinum Toxin in Children with Neurogenic Overactive Bladder: A Case Series

Objetivo Describir la experiencia con la administración de inyección de toxina botulínica en niños con vejiga hiperactiva neurogénica refractaria a manejo de primera línea. Materiales y Métodos Estudio observacional descriptivo (serie de casos) que incluyó a 14 niños con diagnóstico de vejiga hiperactiva neurogénica refractarios a tratamiento de primera línea sometidos a administración intravesical de toxina botulínica entre 2015 y 2021; se realizó el seguimiento teniendo en cuenta las variables clínicas, con evaluación de la respuesta y de los eventos adversos. Se reportaron frecuencias absolutas y porcentajes para las variables cualitativas; para las variables cuantitativas, se reportaron medidas de tendencia central y dispersión. Resultados Se incluyeron 6 niños y 8 niñas, con una media de edad 10,1 (desviación estándar [DE]: ± 4,4) años. Todos los pacientes fueron tratados previamente con anticolinérgico y cateterismos limpios intermitentes, con una media de uso de 2,8 (DE: ± 1.0) pañales al día; 11 (78,5%) pacientes tenían antecedente de infección urinaria, 13 (92,8%), estreñimiento, y 2 (15,3%), incontinencia fecal. En la ecografía, 7 (50,0%) pacientes presentaban engrosamiento de las paredes vesicales, y 6 (42,8%), hidronefrosis. Tras el procedimiento, 1 paciente presentó infección urinaria como complicación, 6 presentaron una respuesta completa, 7, respuesta parcial, y 1 paciente no obtuvo respuesta con la primera inyección, con un tiempo promedio efectivo de la terapia 8 (DE: ± 6,3) meses. Una segunda inyección fue necesaria en 6 (42.8%) pacientes, y, de estos, 3 (50%) requirieron una tercera inyección. Conclusión La inyección de toxina botulinica intravesical como terapia de segunda línea de manejo para vejiga hiperactiva neurogénica tiene buenos resultados, con bajas tasas de complicaciones.

Objective To describe the experience with the administration of botulinum toxin injection in children with neurogenic overactive bladder who were refractory to the first-line management. Materials and Methods A descriptive observational study (case series) which included 14 children with a diagnosis of neurogenic overactive bladder who were refractory to the first-line treatment and were aubmitted to the intravesical administration of botulinum toxin between 2015 and 2021. Follow-up was performed taking into account the clinical variables, wth an evaluation of the response and the adverse events. Absolute frequencies and percentages were reported for the qualitative variables; for the quantitative variables, measures of central tendency and dispersion were reported. Results We included 6 boys and 8 girls with a mean age of 10.1 (standard deviation [SD]: ± 4.4) years. All patients were previously treated with anticholinergics and clean intermittent catheterizations, with a mean use of 2.8 (SD: ± 1.0) diapers per day; 11 (78.5%) had a history of urinary tract infection, 13 (92.8%), constipation, and 2 (15.3%), fecal incontinence. On ultrasound, 7 (50.0%) patients presented bladder wall thickening, and 6 (42.8%), hydronephrosis. After the procedure, 1 patient presented urinary tract infection as a complication, 6 presented complete response, 7, partial response, and 1 patient did not obtain a response with the first injection, with an effective mean time of therapy of 8 (SD: ± 6.3) months. A second injection was required by 6 (42.8%) patients, and of these, 3 (50%) required a third injection. Conclusion Intravesical botulinum toxin injection as a second-line management therapy for neurogenic overactive bladder yileds good results, with low rates of complications.

Cirugía de escoliosis en pacientes con mielomeningocele / Scoliosis surgery in patients with myelomeningocele

Resumen: Objetivo: Evaluar en forma retrospectiva los resultados obtenidos y las complicaciones presentadas en el tratamiento quirúrgico de pacientes con escoliosis neuropática secundaria a mielomeningocele. Material y métodos: Entre Julio de 1991 y Julio de 2018 fueron tratados quirúrgicamente 40 pacientes consecutivos con diagnóstico de escoliosis neuropática secundaria a mielomeningocele, a quienes se les realizó artrodesis vertebral. Resultados: El promedio de observación fue de 10 años (rango de 2-27 años). Fueron 19 pacientes masculinos y 21 femeninos, con un promedio de edad de 13 años. La magnitud promedio de la curva escoliótica fue de 90o en el prequirúrgico y de 43o en el último control. Corrección promedio de 52%. La magnitud promedio de la oblicuidad pélvica fue de 19o en el prequirúrgico y de 9o en el último control. Corrección promedio de 53%. La magnitud preoperativa del balance coronal fue de 28.4 mm, al último control fue de 17 mm. Corrección promedio de 40%. La magnitud promedio de la cifosis fue de 50o en el prequirúrgico y de 41o en el último control. Corrección promedio de 18%. El balance sagital prequirúrgico fue de 63.3 mm, al último control fue de 38.3 mm. Corrección promedio de 40%. Hubo 13 complicaciones (32.5%), la infección fue la complicación observada con mayor frecuencia. Conclusión: Los principales objetivos de la estabilización quirúrgica en pacientes con mielomeningocele son obtener una columna estable, balanceada y sin dolor. Sin embargo, el tratamiento quirúrgico de estos pacientes continúa siendo difícil y está asociado a un alto porcentaje de complicaciones.

Abstract: Objective: The aim of the study was to evaluate the outcomes and complications in patients with myelomeningocele who have undergone spinal fusion for neuromuscular scoliosis. Material and methods: Retrospective study of 40 consecutive patients with myelomeningocele with neuromuscular scoliosis who underwent spinal arthrodesis, treated at our center between July 1991 and July 2028. Results: Mean follow up was 10 years. There were 19 male and 21 females. The average age at operation was 13 years. The mean preoperative scoliosis curve was 90o. At last follow up, the mean scoliosis curve was 43o. Mean correction of 52%. The average of pelvic obliquity was 19o. At last follow up de obliquity was 9o. Mean correction of 53%. The mean preoperative coronal balance was 28.4 mm. At the last follow up it was 17 mm. Mean correction of 40%. The mean preoperative kyphosis was 50o. At the last follow up it was 41o. Mean correction of 18%. The mean preoperative sagittal balance was 63.3 mm. At the last follow up it was 38.3 mm. Mean correction of 40%. There were 13 complications (32.5%), with infection being the most frequently observed complication. Conclusions: The goals of the spinal surgery in patients with MMC are to obtain a stable, balanced and painless spinal fusion. Although the surgical treatment of these patients remains difficult, it is associated with high complication rate.

Intervenciones de prevención y tratamiento en recién nacidos con defectos del tubo neural / Prevention and treatment interventions in newborns with neural tube defects

Introducción: los defectos de tubo neural son anomalías congénitas del sistema nervioso central; estas malformaciones elevan el grado de morbimortalidad en los recién nacidos durante los diez primeros años de vida. Objetivo: definir las intervenciones que el profesional de enfermería puede realizar en el recién nacido con defectos del tubo neural tomando en cuenta aspectos de prevención y tratamiento. Material y métodos: se realizó una búsqueda sistematizada en las bases de datos de PudMed y BVS de julio-septiembre 2021, que incluyo artículos completos relacionados con intervenciones de enfermería a recién nacidos con defectos del tubo neural publicados entre 2016-2021 y artículos médicos de revisión literaria. Resultados: se seleccionaron 41 artículos para definir las intervenciones, a saber: a. intervenciones preventivas con el consumo de ácido fólico, control prenatal y educación sanitaria, b. intervenciones relacionadas al tratamiento enfocadas al cuidado de las derivaciones ventriculoperitoneales y cuidado de heridas quirúrgicas, c. intervenciones ante complicaciones. Discusión: las tasas de mortalidad y discapacidad infantil han incrementado en los últimos años a pesar de los avances en la medicina preventiva, por lo que toma relevancia el consumo de ácido fólico, educación sanitaria y métodos de cuidado hospitalarios. Conclusiones: la enfermera neonatal puede participar con cuidados estandarizados en beneficio de los recién nacidos considerando intervenciones para evitar retraso en el crecimiento y desarrollo de los hitos motores y cognitivos, reducir complicaciones y mejorar las posibilidades de una óptima condición de vida.

Introduction: neural tube defects are congenital anomalies of the central nervous system; These malformations increase the degree of morbidity and mortality in newborns during the first ten years of life. Objective: define the interventions that the nursing professional can be carried out in newborns with neural tube defects, considering aspects of prevention and treatment. Material and methods: systematized search was carried out in the databases of PudMed and BVS during July-September 2021, which included complete articles related to nursing interventions for newborns with neural tube defects published between 2016-2021 and medical literature review articles. Results: forty-one articles were selected to define the interventions: a. Preventive interventions in the consumption of folic acid, prenatal control and health education, b. Interventions related to treatment focused on the care of ventriculoperitoneal shunt and surgical wound care, c. interventions for complications. Discussion: infant mortality and disability rates have increased in recent years despite advances in preventive medicine, which is why the consumption of folic acid, health education and hospital care methods are relevant. Conclusions: neonatal nurse can participate with standardized care for the benefit of newborns, considering interventions to avoid delayed growth and development of motor and cognitive milestones, reduce complications and improve the chances of an optimal life condition.

Sexuality of Female Spina Bifida Patients: Predictors of a Satisfactory Sexual Function / Sexualidade feminina em pacientes com espinha bífida: preditores de uma função sexual satisfatória

Abstract Objective To assess the sexual function of women with spina bifida (SB), and to verify the factors that influence their sexual function. Methods A cross-sectional study in which a validated female-specific questionnaire was applied to 140 SB female patients from four different cities (Porto Alegre, Brazil; and Barcelona, Madrid, and Málaga, Spain) between 2019 and 2020. The questionnaires collected data on the clinical characteristics of SB, and female sexual function was assessed using the 6-item version of the Female Sexual Function Index (FSFI-6) validated to Portuguese and Spanish. Results Half of the patients had had sexual activity at least once in the life, but most (57.1%) did not use any contraception method. Sexual dysfunction was present in most (84.3%) patients, and all sexual function domains were impaired compared those of non-neurogenic women. The presence of urinary and fecal incontinence significantly affected the quality of their sexual activity based on the FSFI-6. Conclusion The specific clinical aspects of the SB patients, such as urinary and fecal incontinence, should be properly addressed by their doctors, since they are associated with reduced sexual activity and lower FSFI-6 scores in the overall or specific domains. There is also a need to improve gynecological care among sexually-active SB patients, since most do not use any contraceptive methods and are at risk of inadvertent pregnancy.

Resumo Objetivo Analisar a função sexual de pacientes do sexo feminino com espinha bífida (EB), e avaliar quais fatores influenciam na função sexual. Métodos Uma pesquisa transversal em que um questionário validado para mulheres foi aplicado em 140 pacientes com EB de quatro cidades diferentes (Porto Alegre, Brasil; e Barcelona, Madri e Málaga, Espanha) entre 2019 e 2020. Os questionários coletaram dados sobre características clínicas da espinha bífida, e a função sexual feminina foi avaliada com a versão de seis itens do Índice de Funcionamento Sexual Feminino (IFSF-6) nas versões validadas para português e espanhol. Resultados Metade das pacientes havia praticado atividade sexual pelo menos uma vez na vida, mas a maioria (57.1%) não utilizava nenhum método contraceptivo. A disfunção sexual estava presente na maioria das pacientes (84.3%), sendo todos os domínios de função sexual prejudicados em comparação com os de mulheres não neurogênicas. A presença de incontinência urinária e fecal afetou significativamente a qualidade da atividade sexual das pacientes. Conclusão Aspectos clínicos específicos da EB, como incontinência urinária e fecal, devem ser adequadamente abordados pelos médicos assistentes, visto que estão associados à redução na atividade sexual e piores resultados no IFSF-6. Também é necessário melhorar o atendimento ginecológico das pacientes sexualmente ativas, uma vez que a maioria não utiliza métodos contraceptivos e corre o risco de gravidez inadvertida.

Actualización del diagnóstico prenatal y cirugía fetal del mielomeningocele / Update on prenatal diagnosis and fetal surgery for myelomeningocele

A partir del estudio seminal Management of Myelomeningocele Study en el año 2011, el cual demostró que la reparación prenatal del defecto del mielomeningocele antes de la semana 26 mejoraba los resultados neurológicos, la cirugía fetal fue incorporada dentro de las opciones de estándar de cuidado. Así, el diagnóstico prenatal del mielomeningocele dentro de la ventana terapéutica se convirtió en un objetivo obligatorio y, por ello, se intensificó la investigación de estrategias de tamizaje, sobre todo, en el primer trimestre. Además, se desarrollaron distintas técnicas de cirugía fetal para mejorar los resultados neurológicos y disminuir los riesgos maternos. El objetivo de la siguiente revisión es actualizar los avances en tamizaje y diagnóstico prenatal en el primer y segundo trimestre, y en cirugía fetal abierta y fetoscópica del mielomeningocel

A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved neurological outcomes; based on this study, fetal surgery was introduced as a standard of care alternative. Thus, prenatal myelomeningocele diagnosis within the therapeutic window became a mandatory goal; therefore, research efforts on screening strategies were intensified, especially in the first trimester. In addition, different fetal surgery techniques were developed to improve neurological outcomes and reduce maternal risks. The objective of this review is to provide an update on the advances in prenatal screening and diagnosis during the first and second trimesters, and in open and fetoscopic fetal surgery for myelomeningocele

Puesta al día en electroestimulación vesical y vejiga neurógena en pediatría: una revisión sistemática / Update on vesical electrostimulation and neuropatic bladder in pediatrics: A sistematic review

Introducción y objetivo: La electroestimulación vesical ha surgido como nueva alternativa en el manejo de pacientes adultos con disfunciones vesicales de origen neuropático. A pesar de que numerosos trabajos han probado la eficacia y la seguridad de esta terapia en este grupo de edad, todavía son escasos los estudios que demuestran su éxito en la vejiga neuropática pediátrica. Material y métodos. Realizamos una revisión sistemática de los estudios que evalúan el impacto de la electroestimulación vesical en pacientes pediátricos (0-18 años) con vejiga de origen neurógeno. La búsqueda identificó 9.716 artículos potencialmente elegibles. Finalmente, 11 trabajos publicados entre 1992 y 2019 cumplieron los criterios necesarios para formar parte del estudio y fueron seleccionados para la presente revisión sistemática. Resultados: No encontramos una tendencia común entre los resultados obtenidos de los estudios incluidos en la presente revisión. A pesar de que la mayoría de ellos demostró individualmente una tasa de eficacia óptima y una alta seguridad, la superioridad de esta terapia frente a los fármacos anticolinérgicos no pudo establecerse. Por tanto, hasta la fecha, sus resultados continúan siendo controvertidos. Además, la metodología entre los diferentes estudios fue muy diversa, lo que dificultó la comparación entre los resultados obtenidos de cada uno de ellos. Conclusiones: Dado el potencial riesgo de insuficiencia renal que presentan los pacientes pediátricos con vejiga neurógena, las alternativas terapéuticas utilizadas en su manejo deben tener una eficacia demostrada. Así, ante la ausencia de evidencia con respecto a la electroestimulación vesical en este grupo de pacientes, por el momento su utilización debería ser limitada y excepcional.

Introduction and objective: Bladder neuromodulation has emerged as a new alternative in the management of adults with bladder dysfunctions from a neuropathic origin. Despite the fact that, numerous studies have proven the efficacy and safety of this therapy in adults’ patients, there are however, still few studies that demonstrate its success in paediatric neuropathic bladder. Materials and methods: We perform a systematic review of the studies which analysed the impact of bladder electrostimulation in paediatric patients (0 to 18 years) with a neurogenic origin. The search identified 9716 potentially articles. Finally, 11 papers published between 1992 and 2019 fulfilled the necessary criteria to be part of our study and were selected for the systematic review. Results: We did not find a common trend among the results obtained from the studies included in the present review. Most of the studies demonstrated an optimal efficacy rate and high safety, but however the superiority of this therapy over anticholinergic drugs could not be established due the presented results. For this fact, these results continue to be controversial. Furthermore, the methodology between the different studies was very huge, which made it difficult to compare the results obtained from each one of them. Conclusions: Given the potential risk of renal failure presented by paediatric patients with neurogenic bladder, the therapeutic alternatives used in the management of this disease must have a proven efficacy. For this reason, and after the review, due the absence of evidence regarding bladder electrotherapy in this group of patients, its use should be limited and exceptional. Bladder neuromodulation has emerged as a new alternative in the management of adult patients with bladder dysfunctions of neuropathic origin...(AU)

Evaluación del proceso de atención urológica de pacientes pediátricos con mielomeningocele / Evaluation of the urological care process of pediatric patients with myelomeningocele

Introducción: El mielomeningocele (MMC) es una de las malformaciones congénitas más severas compatible con la vida. El 90% de los pacientes presenta vejiga neurogénica que debe ser evaluada y tratada precozmente. Objetivos: Describir la evaluación y tratamiento nefrourológico recibido por pacientes con MMC hasta el momento de la primera consulta en el Hospital Garrahan (periodo pre-ingreso). Describir la evaluación realizada y el tratamiento urológico implementado a partir del ingreso al hospital Garrahan (periodo post-ingreso). Evaluar la prevalencia de Enfermedad Renal Crónica (ERC). Población y Métodos: Se realizó un estudio con diseño clínico analítico, retrospectivo, longitudinal sobre pacientes con MMC de 1 mes a 18 años derivados al Hospital Garrahan para atención ambulatoria en los años 2011 y 2012. Resultados: Se incluyeron115 pacientes. Al momento de la derivación al hospital ("pre-ingreso") 7% de los pacientes habían logrado completar evaluación nefrourológica, (ecografía vesicorenal, urodinamia, Cistouretrografía, Centellograma renal y Creatininemia). Tratamiento: 33% vaciaban vejiga por CIL o vesicostomía y 21% recibían Oxibutinina. A partir del ingreso al seguimiento en el Garrahan 83% lograron completar la evaluación, y en función del resultado de la misma se indicó CIL en 87% y Oxibutinina en el 66% de los pacientes. La prevalencia de ERC al ingreso fue de 43%; la mayoría en estadio I. Conclusiones: La mayoría de los pacientes con MMC fueron derivados al hospital de tercer nivel con evaluaciones urológicas incompletas y sin el tratamiento adecuado de la vejiga neurogénica. El inicio del seguimiento interdisciplinario en un hospital de alta complejidad facilitó la realización de las evaluaciones necesarias y la implementación del tratamiento adecuado (AU)

Introduction: Myelomeningocele (MMC) is one of the most severe congenital malformations compatible with life. Of all the patients, 90% presents with a neurogenic bladder requiring early evaluation and treatment. Objectives: To describe the uronephrological evaluation and treatment received by patients with MMC up to the first consultation at Garrahan Hospital (pre-follow-up period). To describe the urological evaluation and treatment implemented from referral to Garrahan Hospital (follow-up period). To evaluate the prevalence of chronic kidney disease (CKD). Population and Methods: A retrospective, longitudinal study with a clinical, analytical design was conducted in patients with MMC between 1 months and 18 years of age referred to Garrahan Hospital for outpatient care in 2011 and 2012. Results: 115 patients were included. At the time of referral to the hospital ("pre-follow-up") 7% of the patients had undergone complete uronephrological evaluation (kidney-bladder ultrasonography, urodynamic studies, cystourethrography, renal scintigraphy, and creatininemia levels). Treatment: 33% emptied their bladder by CIC or vesicostomy and 21% received oxybutynin. From follow-up initiation at Garrahan Hospital, 83% underwent complete evaluation, and based on the results CIC was indicated in 87% and oxybutynin in 66% of the patients. On admission, prevalence of CKD was 43%; with stage I in the majority of the patients. Conclusions: The majority of the patients with MMC were referred to a third-level hospital with incomplete urological studies and without adequate treatment of the neurogenic bladder. Initiation of interdisciplinary follow-up at a tertiary hospital allowed for the necessary studies and implementation of adequate treatment (AU)

Corrección de mielomeningocele intrautero: Reporte del primer caso en Ecuador / Case report: First intrauterine myelomeningocele correction in Ecuador

This is the case of a 26 year old patient, at week 27 of gestational age and with a prenatal diagnosis of myelomeningocele. It was decided to enter the clinic to perform the first correction of the spina bifida defect in Ecuador, with the support of a multidisciplinary team of national and foreign professionals. This procedure was carried out within international parameters and standards for the management of this pathology, with the equipment, supplies and trained personnel to carry this surgery to a successful outcome, both for the fetus and its mother. It is demonstrated that our country has the capacity of both human resources and equipment to solve highly complex pathologies.

Se trata del caso de una paciente de 26 años, en la semana 27 de edad gestacional y con un diagnóstico prenatal de mielome- ningocele. Se decide ingresar a la clínica para realizar la primera corrección de defecto de espina bífida del Ecuador, con apoyo de un equipo multidisciplinario de profesionales nacionales y extranjeros. Este procedimiento se llevó a cabo dentro de parámetros y estándares internacionales de manejo de esta patología, contando con los equipos, insumos y el personal capacitado para llevar esta cirugía a un desenlace exitoso, tanto para el feto como su madre. Queda demostrado que en nuestro país se cuenta con la capacidad tanto de recursos humanos y equipos para resolver patologías de gran complejidad.

Mielomeningocele: actualización para la práctica clínica / Myelomeningocele: update for clinical practice

El mielomeningocele es una de las malformaciones congénitas más frecuentes en Honduras, que se caracteriza por un cierre anormal de la lámina posterior de los cuerpos vertebrales, con la exposición de las estructuras nerviosas. El propósito de esta revisión fue recopilar información que apoye la práctica clínica oportuna en pacientes con mielomeningocele, especialmente en la literatura médica hondureña, reforzada con literatura de otras revistas médicas nacionales e internacionales, en la base de datos PubMed y LILACS, para el periodo 1985- 2018. En conclusión, dar un manejo oportuno proporciona un mejor pronóstico y calidad de vida a los pacientes con defectos del tubo neural...(AU)

Seeking tirelessly for better health and life conditions for the child with myelomeningocele / Buscando incansavelmente por melhores condições de saúde e vida para o filho com mielomeningocele / Buscando sin cesar mejores condiciones de salud y de vida para el niño con mielomeningocele

Objective: to understand the experience of families in the care of children with myelomeningocele. Method: a qualitative research study, which adopted Symbolic Interactionism and Grounded Theory as its theoretical and methodological framework, and the semi-structured interview as a data collection instrument. Twenty-eight participants from thirteen families living in a municipality in the inland of São Paulo took part in the study. Results: the family tirelessly seeks better health and life conditions for the child with myelomeningocele; aiming at the child's autonomy, it is mobilized to the necessary treatments, to learn the care, to adapt the routine, as well as to supply for all the child's needs so that it reaches potential development, looking for a less dependent future with inclusion and social ascension. Conclusion: family nursing shows potential support in the face of myelomeningocele, due to its ability to apprehend the family system, evaluate it and enable intervention proposals in the care process. In this study, rehabilitation nursing was also emphasized, since it goes together with the child and the family who experience myelomeningocele.

Objetivo: compreender a experiência de famílias no cuidado de crianças com mielomeningocele. Método: pesquisa qualitativa, que adotou como referencial teórico e metodológico o Interacionismo Simbólico e a Teoria Fundamentada nos Dados, e a entrevista semiestruturada como instrumento de coleta de dados. Vinte e oito participantes de treze famílias residentes em um município do interior paulista integraram o estudo. Resultados: a família busca incansavelmente por melhores condições de saúde e vida para o filho com mielomeningocele; objetivando a autonomia da criança, se mobiliza aos tratamentos necessários, a aprender os cuidados, a adaptar a rotina, assim como suprir por todas as necessidades da criança a fim de que esta alcance desenvolvimento potencial, prospectando um futuro menos dependente com inclusão e ascensão social. Conclusão: a enfermagem familiar mostra-se apoio potencial frente à condição da mielomeningocele, devido à sua capacidade de apreender o sistema familiar, avaliá-lo e possibilitar proposições de intervenção no processo de cuidado. Neste estudo enfatizou-se também a enfermagem de reabilitação, uma vez que esta caminha conjuntamente à criança e à família que experienciam a mielomeningocele.

Objetivo: comprender la experiencia de las familias en el cuidado de niños con mielomeningocele. Método: investigación cualitativa, que adoptó el Interaccionismo Simbólico y la Teoría Fundamentada como marco teórico y metodológico, y la entrevista semiestructurada como instrumento de recolección de datos. Participaron del estudio 28 participantes de trece familias residentes en una ciudad del interior de São Paulo. Resultados: la familia busca sin cesar mejores condiciones de vida y salud para el niño con mielomeningocele; con el objetivo de estimular la autonomía del niño, se realizan los tratamientos necesarios, se aprenden los cuidados, se adecua la rutina y se satisfacen todas las necesidades del niño para que alcance su desarrollo potencial, proyectando un futuro menos dependiente con inclusión y ascensión social. Conclusión: la enfermería familiar muestra un potencial apoyo frente a la condición de mielomeningocele, debido a su capacidad para aprehender el sistema familiar, evaluarlo y posibilitar propuestas de intervención en el proceso de atención. En este estudio también se enfatizó la enfermería de rehabilitación, ya que va de la mano del niño y la familia que padecen mielomeningocele.

Seeking tirelessly for better health and life conditions for the child with myelomeningocele / Buscando incansavelmente por melhores condições de saúde e vida para o filho com mielomeningocele / Buscando sin cesar mejores condiciones de salud y de vida para el niño con mielomeningocele

Objective: to understand the experience of families in the care of children with myelomeningocele. Method: a qualitative research study, which adopted Symbolic Interactionism and Grounded Theory as its theoretical and methodological framework, and the semi-structured interview as a data collection instrument. Twenty-eight participants from thirteen families living in a municipality in the inland of São Paulo took part in the study. Results: the family tirelessly seeks better health and life conditions for the child with myelomeningocele; aiming at the child's autonomy, it is mobilized to the necessary treatments, to learn the care, to adapt the routine, as well as to supply for all the child's needs so that it reaches potential development, looking for a less dependent future with inclusion and social ascension. Conclusion: family nursing shows potential support in the face of myelomeningocele, due to its ability to apprehend the family system, evaluate it and enable intervention proposals in the care process. In this study, rehabilitation nursing was also emphasized, since it goes together with the child and the family who experience myelomeningocele.

Objetivo: compreender a experiência de famílias no cuidado de crianças com mielomeningocele. Método: pesquisa qualitativa, que adotou como referencial teórico e metodológico o Interacionismo Simbólico e a Teoria Fundamentada nos Dados, e a entrevista semiestruturada como instrumento de coleta de dados. Vinte e oito participantes de treze famílias residentes em um município do interior paulista integraram o estudo. Resultados: a família busca incansavelmente por melhores condições de saúde e vida para o filho com mielomeningocele; objetivando a autonomia da criança, se mobiliza aos tratamentos necessários, a aprender os cuidados, a adaptar a rotina, assim como suprir por todas as necessidades da criança a fim de que esta alcance desenvolvimento potencial, prospectando um futuro menos dependente com inclusão e ascensão social. Conclusão: a enfermagem familiar mostra-se apoio potencial frente à condição da mielomeningocele, devido à sua capacidade de apreender o sistema familiar, avaliá-lo e possibilitar proposições de intervenção no processo de cuidado. Neste estudo enfatizou-se também a enfermagem de reabilitação, uma vez que esta caminha conjuntamente à criança e à família que experienciam a mielomeningocele.

Objetivo: comprender la experiencia de las familias en el cuidado de niños con mielomeningocele. Método: investigación cualitativa, que adoptó el Interaccionismo Simbólico y la Teoría Fundamentada como marco teórico y metodológico, y la entrevista semiestructurada como instrumento de recolección de datos. Participaron del estudio 28 participantes de trece familias residentes en una ciudad del interior de São Paulo. Resultados: la familia busca sin cesar mejores condiciones de vida y salud para el niño con mielomeningocele; con el objetivo de estimular la autonomía del niño, se realizan los tratamientos necesarios, se aprenden los cuidados, se adecua la rutina y se satisfacen todas las necesidades del niño para que alcance su desarrollo potencial, proyectando un futuro menos dependiente con inclusión y ascensión social. Conclusión: la enfermería familiar muestra un potencial apoyo frente a la condición de mielomeningocele, debido a su capacidad para aprehender el sistema familiar, evaluarlo y posibilitar propuestas de intervención en el proceso de atención. En este estudio también se enfatizó la enfermería de rehabilitación, ya que va de la mano del niño y la familia que padecen mielomeningocele.

Infant hydrocephalus in sub-Saharan Africa: Impact of perioperative care in the Zanzibar archipelago. / Hidrocefalia infantil en el África subsahariana: impacto de los cuidados perioperatorios en el archipiélago de Zanzíbar.

INTRODUCTION: Child hydrocephalus in low- and middle-income countries represents one of the most sensitive ethical and health problems facing international health development. The most optimistic estimates indicate that 200,000 newborns annually will develop hydrocephalus or be born with a neural tube defect in East, Central and South Africa (ECSA). It is estimated that less than 10% of these children will be operated by ventriculoperitoneal shunts, and in general in poor quality conditions or with a very high complication rate. OBJECTIVE: To describe the general characteristics, epidemiology and demographic data of childhood hydrocephalus of patients treated at the NED Institute in the Zanzibar archipelago, and assess the clinical details and medium-term results of the impact of the set-up nursing care. MATERIAL AND METHODS: This is a descriptive and analytical observational study of a retrospective nature, in patients diagnosed and treated with childhood hydrocephalus, in the period from September 2016 to September 2018. With the implementation of a series of perioperative nursing protocols in these patients, the results obtained were described and analyzed. RESULTS: A total of 96 patients were treated for childhood hydrocephalus. 51% (n=49) of these patients were male, with a mean age of 9.25 months. All the mothers of the patients were monitored during pregnancy, but only 8% were treated with folic acid during pregnancy. 81% of children were born through vaginal delivery or uncomplicated spontaneous delivery. Regarding the etiology, 27.1% of treated hydrocephalus was associated with an infectious cause and 35.4% with an unknown cause. 67 ventriculoperitoneal shunt surgery and 15 endoscopic ventriculostomies were performed. The complication rate was 23.17%. CONCLUSIONS: The results of this research indicate that childhood hydrocephalus in Zanzibar has etiology, evolution and complications that are similar to or less than those described to date in East Africa. Implementing a series of perioperative protocols and standardized nursing care positively influences the results obtained. Currently, the Mnazi Mmoja Surgical NED Institute is one of the few centers in East Africa with an exhaustive record of healthcare activity and is the first health center that offers further training to nurses.

Anestesia para reparación antenatal de mielomeningocele: Presentación de un caso / Anesthesia for myelomenigocele antenatal repair: Case report

INTRODUCTION: The advance in the methods of prenatal diagnosis and surgical techniques have allowed the development of fetal surgery, achieving identification and early treatment of anomalies invalidating extrauterine life. Myelomeningocele (MMC) is the most frequent neural tube defect and its intrauterine correction has demonstrated benefits. OBJECTIVE: To publicize the anesthetic management of a prenatal correction of MMC performed in a public hospital in Chile. CASE REPORT: 31-year-old woman, pregnancy of 25 weeks of gestational age, fetus carrying MMC lumbosacral, who underwent open correction. Procedure performed with incidents under general anesthesia with remifentanil and sevoflorane MAC in 2 and tocolytic prophylaxis. At 48 hours post operative, he presented an acute pulmonary edema compatible (EPA), which was successfully resolved with depletive therapy for 24 hours in the Intensive Care Unit, without the need for mechanical ventilation or use of vasoactive drugs. Discharged one week later in good condition, with interruption of pregnancy by elective caesarean section at 37 weeks, with a newborn without stigmas of neurological sequelae. CONCLUSIONS: The mother-fetus binomial is a challenge for the anesthetist. In intrauterine surgery the need for knowledge about the pharmacology of tocolytics, placental uterine physiology and the complications of the procedure are added.

INTRODUCCIÓN: El avance en los métodos de diagnóstico prenatal y las técnicas quirúrgicas han permitido el desarrollo de la cirugía fetal, logrando identificación y tratamiento precoz de anomalías invalidantes para la vida extrauterina. El mielomeningocele (MMC) es el defecto del tubo neural más frecuente y su corrección intrauterina tiene beneficios demostrados. OBJETIVO: Dar a conocer el manejo anestésico de una corrección prenatal de MMC realizada en un hospital público de Chile. CASO CLÍNICO: Mujer de 31 años, embarazo de 25 semanas de edad gestacional, feto portador de MMC lumbosacro, que se sometió a una corrección por vía abierta. Procedimiento realizado con incidentes bajo anestesia general con remifentanilo y sevoflorano MAC en 2 y profilaxis tocolítica. A las 48 horas postoperatorias presentó cuadro compatible con edema pulmonar agudo (EPA), que se resolvió exitosamente con terapia depletiva por 24 horas en Unidad de Cuidados Intensivos, sin necesidad de ventilación mecánica ni uso de drogas vasoactivas. Dada de alta una semana después en buenas condiciones. El embarazo se interrumpió por cesárea electiva a las 37 semanas, con un recién nacido sin estigmas de secuela neurológica. CONCLUSIONES: El binomio madre-feto es un reto para el anestesista. En cirugía intraútero se suma la necesidad de conocimientos sobre la farmacología de los tocolíticos, fisiología útero placentaria y las complicaciones propias del procedimiento.