Resultados a largo plazo de la cistoplastia de ampliación: una evaluación retrospectiva de 54 niños / Long-term outcomes of augmentation cystoplasty: A retrospective view of 54 children

Introducción y objetivos: A lo largo de los años los médicos que se ocupan de los trastornos urológicos pediátricos han recurrido a la ampliación vesical (AV), un procedimiento quirúrgico altamente desafiante desarrollado para preservar las funciones renales en diversos casos de anomalías urinarias congénitas. Este estudio tiene como objetivo revelar los resultados de la AV a muy largo plazo en una amplia muestra de pacientes pediátricos, así como el papel de la enfermedad subyacente en el pronóstico renal tras la AV.Materiales y métodosSe realizó un estudio transversal retrospectivo en 54 niños con anomalías urinarias congénitas que fueron sometidos a una AV. Se evaluó la técnica de ampliación utilizada, la localización de la implantación del uréter, las complicaciones y los hallazgos ecográficos. Se recogieron datos sobre los niveles de creatinina sérica en los registros preoperatorios y posteriores a la AV anuales.ResultadosDe los 54 niños 33 (61,1%) eran varones. Los diagnósticos fueron disrafismo espinal (DE) (n=13), válvula uretral posterior (VUP) (n=8), extrofia vesical (n=32) y traumatismo (n=1). La duración media del seguimiento fue de 18 (3-31) años. Las comparaciones de los niveles de creatinina sérica entre los grupos revelaron que, a pesar de que no existían diferencias significativas entre el grupo de extrofia vesical y el de VUP en el periodo preoperatorio ni en el primer año postoperatorio, los casos con VUP presentaban niveles de creatinina sérica significativamente más altos en los siguientes años posteriores a la cirugía. Además, en comparación con el grupo de DE, los sujetos con VUP tenían niveles significativamente más altos en el segundo (p=0,035) y décimo año (p=0,006) del postoperator. (AU)

Introduction and objectives: Throughout the years, clinicians dealing with pediatric urology disorders have resorted to bladder augmentation (BA), a demanding surgical procedure, to preserve renal functions in sundry congenital urinary tract defects. This study aimed to reveal the very long-term outcomes of BA in a large sample of pediatric patients and the role of underlying disease on renal prognosis after BA.Materials and methodsA retrospective cross-sectional study was conducted on 54 children with congenital urinary defects who underwent BA. The utilized augmentation technique, the location of ureter implantation, complications, and ultrasonography findings were analyzed. Data on serum creatinine levels were collected from preoperative records and anniversaries following BA.ResultsAmong 54 children, 33 (61.1%) were boys. Diagnoses were spinal dysraphism (SD) (n=13), posterior urethral valve (PUV) (n=8), bladder exstrophy (n=32) and trauma (n=1). The median follow-up duration was 18 (3-31) years. The comparisons of serum creatinine levels between groups revealed that, despite no meaningful difference was present between bladder exstrophy and PUV group in the preoperative period and postoperative 1st year, cases with PUV had significantly higher levels of serum creatinine levels in the following postoperative years. Therewithal compared with the SD group, subjects with PUV had significantly higher levels at the postoperative 2nd year (P=.035) and 10th year (P=.006).ConclusionsIn our study, significantly long-term follow-up outcomes could facilitate the pre- and postoperative approach for enterocystoplasty in children. According to our results, it is noteworthy that kidney functions are at high risk of worsening in subjects with PUV and underwent BA. (AU)

Cerebrospinal fluid hypotension following fall in a child: Case report / Hipotensión del líquido cefalorraquídeo tras caída en un niño: caso clínico

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI (AU)

La hipotensión del LCR surge en el contexto de una fuga de dicho líquido que causa presión intracraneal negativa. Las fracturas del sacro son originadas por traumatismos de alta energía que a menudo no se diagnostican. Un niño de diez años acudió con dolor de cadera tras una caída. Podía mover las piernas, no reportando ningún dolor en las mismas, ni irradiación o falta de control del esfínter. El examen neurológico resultó normal. Cuando se le pidió que se pusiera de pie comenzó a sufrir cefalea biparietal, náuseas y vómitos, que mejoraban al tumbarse. La TC mostró un meningocele intrasacro oculto. La RM reveló rastros de LCR a lo largo de la columna, fractura en S3 con laceración potencial del meningocele y apertura de una fístula de LCR. Nuestro diagnóstico fue hipotensión de LCR, secundaria a la apertura de la fístula. El diagnóstico resultó difícil. El niño presentó síntomas de hipotensión del LCR sin causa evidente. El descubrimiento del meningocele nos llevó a conjeturar la apertura de una fístula, un diagnóstico infrecuente que fue confirmado posteriormente mediante RM (AU)

Long-term outcomes of augmentation cystoplasty: A retrospective view of 54 children.

INTRODUCTION AND OBJECTIVES: Throughout the years, clinicians dealing with pediatric urology disorders have resorted to bladder augmentation (BA), a demanding surgical procedure, to preserve renal functions in sundry congenital urinary tract defects. This study aimed to reveal the very long-term outcomes of BA in a large sample of pediatric patients and the role of underlying disease on renal prognosis after BA. MATERIALS AND METHODS: A retrospective cross-sectional study was conducted on 54 children with congenital urinary defects who underwent BA. The utilized augmentation technique, the location of ureter implantation, complications, and ultrasonography findings were analyzed. Data on serum creatinine levels were collected from preoperative records and anniversaries following BA. RESULTS: Among 54 children, 33 (61.1%) were boys. Diagnoses were spinal dysraphism (SD) (n = 13), posterior urethral valve (PUV) (n = 8), bladder exstrophy (n = 32) and trauma (n = 1). The median follow-up duration was 18 (3-31) years. The comparisons of serum creatinine levels between groups revealed that, despite no meaningful difference was present between bladder exstrophy and PUV group in the preoperative period and postoperative 1st year, cases with PUV had significantly higher levels of serum creatinine levels in the following postoperative years. Therewithal compared with the SD group, subjects with PUV had significantly higher levels at the postoperative 2nd year (P = .035) and 10th year (P = .006). CONCLUSIONS: In our study, significantly long-term follow-up outcomes could facilitate the pre- and postoperative approach for enterocystoplasty in children. According to our results, it is noteworthy that kidney functions are at high risk of worsening in subjects with PUV and underwent BA.

Cerebrospinal fluid hypotension following fall in a child: Case report.

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI.

Lipomielomeningocele y médula anclada en un neonato: a propósito de un hallazgo cutáneo / Lipomyelomeningocele and tethered cord in neonate: a cutaneous finding / Lipomielomeningocele e medula ancorada em rescem nacido: um achado cutâneo

Introducción: el disrafismo espinal oculto comprende las anomalías congénitas caracterizadas por la fusión incompleta del tubo neural, en las que la lesión se encuentra cubierta por piel sin observarse exposición del tejido nervioso. Existen estigmas cutáneos que se asocian a su presencia, siendo los lipomas congénitos en la línea media posterior altamente sugerentes de lesión espinal. Su principal complicación es la asociación con el síndrome de médula anclada, que puede causar un daño neurológico irreversible. Caso clínico: recién nacida de sexo femenino, durante la exploración en Maternidad detectamos un estigma cutáneo del tipo lipoma en la región sacra sospechoso de disrafismo espinal oculto, confirmándose posteriormente, mediante estudio con ecografía y resonancia magnética, la presencia de lipomielomeningocele y médula anclada. Conclusiones: el reconocimiento de los marcadores cutáneos, que constituyen a veces la única manifestación de la enfermedad en pacientes asintomáticos, posibilitaría un diagnóstico precoz y manejo individualizado con posible corrección quirúrgica según el caso, que podría prevenir el daño neurológico irreversible asociado a la médula anclada.

Introduction: hidden spinal dysraphism involves congenital anomalies characterized by an incomplete fusion of the neural tube, where the lesion is covered by skin and the nervous tissue is not exposed. Some skin stigmas are linked with this spinal injury, mainly congenital lipomas in the posterior midline of the lesion. Hidden spinal dysraphism's main complication could be tethered cord syndrome, which can cause irreversible neurological damage. Clinical case: female newborn showing a lipoma-like skin stigma in the sacral region, looking like hidden spinal dysraphism, which was later confirmed through ultrasound and magnetic resonance imaging, which showed lipomyelomeningocele and a tethered cord. Conclusions: early detection of these skin markers is sometimes the only tool to early diagnosis and personalized treatment in asymptomatic patients. It enables a possible surgical remediation and may prevent the irreversible neurological damage linked to the tethered cord.

Introdução: o disrafismo espinhal oculto envolve anomalias congênitas caracterizadas por uma fusão incompleta do tubo neural, onde a lesão é coberta por pele e o tecido nervoso não fica exposto. Alguns estigmas de pele estão relacionados a essa lesão espinhal, principalmente lipomas congênitos na linha média posterior da lesão. A principal complicação do disrafismo espinhal oculto pode ser a síndrome da medula ancorada, a qual pode causar danos neurológicos irreversíveis. Caso clínico: recém-nascida apresenta estigma cutâneo semelhante a lipoma na região sacral, semelhante a disrafismo espinhal oculto, posteriormente confirmado por ultrassonografia e ressonância magnética, o que evidenciou lipomielomeningocele e medula ancorada. Conclusões: a detecção precoce desses marcadores cutâneos às vezes é a única ferramenta para o diagnóstico precoce e tratamento personalizado em pacientes assintomáticos. Permite uma possível correção cirúrgica e pode prevenir os danos neurológicos irreversíveis ligados à medula ancorada.

Validity of the VO2peak prediction model to Brazilian youth with spina bifida / Validação de um modelo preditivo do VO2pico em jovens brasileiros com espinha bífida

Objective:To validate a peak oxygen uptake (VO2peak) prediction model in Brazilian youth with spina bifida. Methods:Twenty participants with spina bifida performed a graded arm crank test to measure VO2peak. The VO2peakvalues predicted by the equation "VO2peak(mL/min) = 194 + 18 × peak workload ­110 × sex" were compared to the VO2peakvalues measured. Results:The predicted VO2peakwas not different from the measured VO2peak. A high correlation was found between both VO2peak values, and the Bland-Altman analysis did not show a significant difference, demonstrating agreement between the values. Conclusions:The VO2peak prediction model in Brazilian youth with spina bifida was validated, being an advantageous alternative to assess and follow physical fitness and prescribe exercise training intensity.

Objetivo: Validar uma equação preditiva do consumo pico de oxigênio (VO2pico) em jovens brasileiros com espinha bífida. Métodos: Vinte participantes com espinha bífida realizaram um teste ergoespirométrico de membros superiores para medir o VO2pico. Os valores de VO2pico preditos pela equação "VO2pico (mL/min) = 194 + 18 × carga pico ­ 110 × sexo" foram comparados com o VO2pico medido. Resultados: O VO2pico predito pela equação não foi diferente do VO2pico medido. Foi encontrada alta correlação entre os valores de VO2pico e, a análise Bland Altman não mostrou diferença significativa, demonstrando concordância entre os valores. Conclusão: A equação preditiva do VO2pico é válida para jovens brasileiros com espinha bífida e é uma alternativa vantajosa para obter e acompanhar o condicionamento físico e prescrever a intensidade de treinamento nesses indivíduos.

Spinal dermal sinus complication in children: case series and literature review / Complicação do seio dérmico espinhalem crianças: séries de casos e revisão de literatura / Complicacióndel seno dérmico espinal en niños: serie de casos y revisión de la literatura

ABSTRACT Introduction: Dermal sinus (DS) is a rare dysraphism. It can be asymptomatic, become infected, and produce severe neurological symptoms. Our objective is to present a series of pediatric cases with spinal DS complicated byinfections (DSCI), describe the findings correlated with the anatomy in a stillbirth, as well as the associated pathologies and their treatment. Method: We analyzeddifferent variables in the clinical histories of 5 children with spinal DSCI. In addition, an anatomical dissection of a stillbirth with lumbar DS was performed. Results: Two males and 3 femaleswith DSCI and a mean age of 2 years and 9 months were included: 2 lumbar (one in the midline and the other in theparamedian region), 1 in the thoracic region, 1 in the upper cervical region,and 1 in the lumbosacral region. The forms of presentation were 3 meningeal profiles (one with pain andlocalized swelling) and 3 neurological deficits (one associated with the meningeal profile and another associated with pain and a tumor). In all cases, the tract of the DS was identified by magnetic resonance imaging (MRI).Associated lesions included 1 dorsal intramedullary dermoid cyst, 1 tethered lumbar spinal cord with syringomyelia, 1 partial cervical medullary disconnection, and 2 spinal dysraphisms. Four were operated on and one died of infectious complications before surgery. In the 12-week-old male fetus with lumbar DS, a permeable tract to the subarachnoid space was verified. Conclusions: DSCIsshould bestudied with MRI to identify their tracts, infectious complications of thecentral nervous system, associated malformations, inclusion tumors, and to enabledifferential diagnosis. Once diagnosed, they should be urgently treated both surgically and with prolonged antibiotictherapy. Level of Evidence IV; Therapeutic Study (Treatment Outcome Investigation)

RESUMO Introdução: O seio dérmico (SD) é uma disrafia rara. Pode ser assintomático, sofrer infecção e produzir quadros graves.Nosso objetivo é apresentar uma série de casos pediátricos com SDs espinhais complicados por infecções (SDCI),descrever os achados correlacionados com a anatomia em um natimorto, as patologias associadas e seu tratamento. Método: Foram analisadasas variáveis da históriaclínica em 5 crianças com SCDI espinhal.Além disso, foi feita uma dissecção anatômica de um natimorto com SD. Resultados: Foram incluídos 2 meninose 3 meninas, com média de idade de 2 anos e 9 meses, com SDCI: 2 lombares(um na linha média e um paramediano), 1 na região torácica, 1 na região cervical superior e 1 lombossacral. As formas de apresentação foram 3 quadros meníngeos, 1 com dor e tumefação lombar local e 3 déficits neurológicos (um associado ao quadro meníngeo e outro associado à dor e tumor). Em todos os casos, o trajeto do SD foi identificado por ressonância magnética (RM).Aslesões associadas foram 1 cisto dermoide intramedular dorsal, 1 medula ancorada lombar com siringomielia, 1 desconexão medular cervical parcial e 2 disrafias espinhais. Quatro participantes foram operados e um foi a óbito decorrente de complicações infecciosas antes da cirurgia.No feto masculino de 12 semanas com SD lombar, foi verificado um trajeto permeável até o espaçosubaracnóideo. Conclusões: Os SDCIs devem ser estudados com RM para identificar o seu trajeto, complicações infecciosas do sistema nervoso, malformações associadas, tumores de inclusão e permitir o diagnóstico diferencial. Eles devem ser tratados cirurgicamente com urgência uma vez diagnosticados e com tratamento prolongado com antibióticos. Nível de evidência IV; EstudosTerapêuticos- Investigação dos Resultados do Tratamento

RESUMEN Introducción: El seno dérmico (SD) es una disrafia infrecuente. Puede ser asintomático, infectarse y producircuadros severos. Nuestro objetivo es presentar una serie de casos pediátricos con SD espinalescomplicados porinfecciones (SDCI), describir los hallazgoscorrelacionadoscon la anatomía en un mortinato, las patologías asociadas y su tratamiento. Método: Seanalizaron diferentes variables enlas historias clínicas de 5 niños con SDCI espinales. Además, se realizó unadisección anatómicade un mortinato con SD lumbar. Resultados: Se incluyeron2varones y 3 mujeres,de 2 años y 9 meses de edad promedio, con SDCI:2 lumbares (uno en línea media y otro paramediano), 1 en región torácica, 1 cervical superior y 1 lumbosacro. Las formas de presentación fuerontres cuadros meníngeos, 1 con dolor y tumefacción local lumbar y 3 déficits neurológicos (uno asociado a cuadro meníngeo y otro asociado a dolor y tumor). En todos los casos se identificó el trayecto del SD por resonancia magnética (IRM). Como lesiones asociadas hubo1 quiste dermoide intramedular dorsal,1médula lumbar anclada con siringomielia,1 desconexión medular cervical parcial y 2 disrafias espinales. Cuatro fueron operados y uno falleció por complicaciones infecciosas antes de la cirugía. En el feto masculino de 12 semanas con SD lumbar, se verificóun trayecto permeable hasta el espacio subaracnoideo. Conclusiones: Los SDCI se deben estudiarconIRM, para identificarsu trayecto, las complicaciones infecciosas del sistema nervioso, las malformaciones asociadas,los tumores de inclusión y permitirel diagnóstico diferencial. Se deben tratar quirúrgicamente con urgencia una vez diagnosticados y con antibiótico-terapia prolongada. Nivel de Evidencia IV; Estudio Terapéutico (Investigación de Resultado de Tratamiento).

Cerebrospinal fluid hypotension following fall in a child: Case report.

CSF hypotension arises in the context of a leak of CSF which causes negative intracranial pressure. Sacral fractures result from high-energy trauma which are frequently underdiagnosed. A ten-year-old boy presented with hip pain, after a fall. He mobilized both lower limbs, reported no leg pain, irradiation nor lack of sphincter control. The neurological examination was normal. When asked to stand, he began biparietal headache, nausea and vomiting, which improved laying down. CT scan showed an occult intrasacral meningocele; the MRI revealed collections of CSF along the spine, a S3 fracture with potential laceration of the meningocele and opening of a CSF fistula. Our diagnosis was the CSF hypotension, secondary to the fistula opening. The diagnosis was challenging. The child first presented with symptoms of CSF hypotension without evident cause. The discovery of the meningocele led us to hypothesize the opening of a fistula, a rare diagnosis, later confirmed by MRI.

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)

Neurocirugía transicional: ¿una nueva subespecialidad? / Transitional neurosurgery: a new subspecialty?

Introducción: El proceso de transición de un paciente de la etapa pediátrica a la adulta es un proceso dinámico, complejo y planificado, que incluye la transferencia propiamente dicha de una institución pediátrica a una adulta. El aumento de la sobrevida de niños y adolescentes con patologías crónicas, la falta de acuerdos formales entre instituciones de salud y la falta de enfoque multidisciplinario de estos casos son los principales problemas a tener en cuenta. El objetivo del presente trabajo es describir y proponer una respuesta a las situaciones y dificultades que se encuentran en la actualidad en la salud pública durante el proceso de transición de pacientes pediátricos con patología neuroquirúrgica crónica y de pacientes adultos con patología congénita. Para tal fin se deben considerar factores sociales, económicos y comunicacionales. Material y Método: Estudio observacional, transversal y retrospectivo de pacientes con patología neuroquirúrgica transicional desde el 01 de enero de 2017 al 31 de diciembre de 2018. En total se revisaron las historias clínicas de 47 pacientes del Hospital "El Cruce". Resultados: De los 47 pacientes observados, con un rango etario entre 17 y 42 años, realizamos una división de éstos en 2 grupos: GRUPO 1: pacientes adultos operados en la edad pediátrica que requieren un seguimiento crónico de su patología de origen; y GRUPO 2: pacientes adultos con patología congénita. En el GRUPO 1, observamos 38 pacientes (24 masculinos y 14 femeninos) de los cuales 24 fueron operados y 14 no operados (solo seguimiento clínico). Dentro de este grupo, el 63% de los pacientes (n=29) presentó como su patología de base para su seguimiento la hidrocefalia. En el GRUPO 2, observamos a 9 pacientes (2 masculinos y 7 femeninos) de los cuales 7 fueron operados y 2 no operados. Todos los pacientes de este último grupo presentaron como diagnóstico de base un disrafismo espinal. Conclusión: Se debe lograr una transición planificada para el bienestar de los jóvenes con necesidades especiales de atención de salud. Actualmente no hay acuerdos interinstitucionales formales para el seguimiento y atención de estos pacientes.Consideramos que existe un grupo de pacientes que se beneficiarían con la creación de una nueva subespecialidad neuroquirúrgica: la neurocirugía transicional. La misma debería desarrollarse en hospitales generales, de alta complejidad, donde coincidan en el servicio de neurocirugía de adultos, neurocirujanos con formación pediátrica

Introduction: The transition process of a patient from pediatric to adult stage is a dynamic, complex, and planned process which, strictly speaking, includes the transfer from a pediatric to an adult institution. The increased rate of survival of children and adolescents with chronic pathologies, lack of formal agreements between health institutions and lack of multidisciplinary approach to these cases are the main issues to consider. The purpose of this paper is to describe and provide a response to situations and difficulties that currently exist in the public health during the transition process of pediatric patients with chronic neurosurgical pathology and adult patients with congenital pathology. For this purpose, communication, social, and economic factors must be considered. Methods: Observational, cross-sectional, and retrospective study of patients with transitional neurosurgical pathology from January 1, 2017, to December 31, 2018. In total, 47 patient's medical records were reviewed from "El Cruce" Hospital. Results: A total of 47 patients, with an age range between 17 and 42 years, were observed. We divided our study population into 2 groups: GROUP 1: adult patients who have been operated in pediatric age and require chronic follow-up of their origin pathology; and GROUP 2: adult patients with congenital pathology. In GROUP 1, we observed 38 patients (24 male and 14 female) of whom 24 were performed neurosurgery, and 14 were not. Within this group, 63% of the patients (n = 29) presented hydrocephalus as their basic pathology for monitoring. In GROUP 2, we observed 9 patients (2 male and 7 female) of whom 7 were operated and 2 were not. All patients in this last group presented spinal dysraphism as their basic diagnosis. Conclusion: A planned transition for the well-being of young patients with special health care needs must be achieved. Currently, there are no formal institutional agreements for the monitoring and care of these patients. We believe that there is a group of patients who would benefit from the creation of a new neurosurgical sub-specialty: transitional neurosurgery. It should be developed in high complexity general hospitals, where neurosurgeons with pediatric training could be part of a general neurosurgery department.

Lumbar pseudo-tail associated with dermal sinus - A case report.

BACKGROUND: Lumbosacral and coccygeal skin covered appendages are a rare malformation, considered human-tails. CASE REPORT: The authors describe the case of a full term newborn girl with lumbar skin appendage and a normal neurologic examination. The magnetic resonance of the spine revealed a dermal sinus continuous with the skin appendage and with extension into to the spinal canal. Due to the infection risk, a surgery was performed on the third day of life, with dermal sinus ligation and appendage removal. At 12 months of follow-up the girl has a normal neurologic examination. CONCLUSIONS: With the description of this case the authors aim to emphasize that although rare, lumbar skin appendages can be associated with spinal dysraphism and other lesions, requiring extensive work-up and long-term surveillance.

Diagnostic yield of lumbosacral magnetic resonance imaging requested by paediatric urology consultations. / Rentabilidad diagnóstica de la resonancia magnética lumbosacra solicitada desde las consultas de urología pediátrica.

OBJECTIVES: In the historical series, the diagnostic yield of lumbosacral magnetic resonance imaging to rule out occult spinal dysraphism (or occult myelodysplasia), requested by paediatric urology, ranged from 2% to 15%. The aim of this study was to define our cost-effectiveness in children with urinary symptoms and to define endpoints that increase the possibility of finding occult spinal dysraphism. PATIENTS AND METHODS: A screening was conducted on patients with urinary dysfunction for whom an magnetic resonance imaging was requested by the paediatric urology clinic, for persistent symptoms after treatment, voiding dysfunction or other clinical or urodynamic findings. We analysed clinical (UTI, daytime leaks, enuresis, voiding dysfunction, urgency, renal ultrasonography, lumbosacral radiography, history of acute urine retention, skin stigma and myalgia) and urodynamic endpoints (hyperactivity or areflexia, voiding dysfunction, interrupted pattern, accommodation value and maximum flow). A univariate analysis was conducted with SPSS 20.0. RESULTS: We analysed 21 patients during the period 2011-2015. The median age was 6 years (3-10). Three patients (14.3%) had occult spinal dysraphism: one spinal lipoma, one filum lipomatosus and one caudal regression syndrome with channel stenosis. The endpoints with statistically significant differences were the myalgias and the history of acute urine retention (66.7% vs. 5.6%, P=.04; OR= 34; 95%CI: 1.5-781 for both endpoints). CONCLUSIONS: The diagnostic yield of magnetic resonance imaging requested for children with urinary dysfunctions without skin stigma or neuro-orthopaedic abnormalities is low, although nonnegligible. In this group, the patients with a history of acute urine retention and muscle pain (pain, «cramps¼) can experience a greater diagnostic yield or positive predictive value.

[Simultaneous diagnosis of pseudomeningocele, tethered cord syndrome and cerebrospinal fluid fistula: Report of a case]. / Diagnóstico simultáneo de pseudomeningocele, síndrome de médula anclada y fístula de líquido cefalorraquídeo: a propósito de un caso.

The clinical case is presented on a patient with an extensive sacral dysraphism, a history of myelomeningocele surgical repair in her childhood, as well as tethered cord syndrome. The patient was also diagnosed with pseudomeningocele and a cerebrospinal fluid cutaneous fístula. A surgical approach was used, with encouraging results being obtained in the clinical outcome of the patient. A review of the literature was performed to support the surgical decision in this case.

Advances in fetal surgery / Avanços em cirurgia fetal

ABSTRACT This paper discusses the main advances in fetal surgical therapy aiming to inform health care professionals about the state-of-the-art techniques and future challenges in this field. We discuss the necessary steps of technical evolution from the initial open fetal surgery approach until the development of minimally invasive techniques of fetal endoscopic surgery (fetoscopy).

RESUMO São discutidos os principais avanços na terapia cirúrgica do feto, tendo como objetivo informar profissionais da área da saúde sobre qual o estado atual da arte e quais seus desafios futuros. São debatidos os passos que foram necessários para a evolução da técnica inicial, quando a cirurgia fetal era realizada a céu aberto, até o desenvolvimento de técnicas minimamente invasivas, de cirurgia endoscópica fetal (fetoscopia).

USE OF VIRTUAL QUESTIONNAIRE AND DISSEMINATION AS A DATA COLLECTION STRATEGY IN SCIENTIFIC STUDIES / USO DE CUESTIONARIO Y DIVULGACIÓN VIRTUALES COMO ESTRATEGIA PARA LA RECOGIDA DE DATOS DE ESTUDIOS CIENTÍFICOS / USO DE QUESTIONÁRIO ONLINE E DIVULGAÇÃO VIRTUAL COMO ESTRATÉGIA DE COLETA DE DADOS EM ESTUDOS CIENTÍFICOS

ABSTRACT This study describes the use of online questionnaires and their virtual dissemination as a data collection strategy in Germany. The online questionnaire was elaborated using a virtual platform, creating a link that was disseminated in virtual environments. Participants were 100 Germans with Spina Bifida; 149 people visited the link, 83 of them coming from virtual forums, 27 from emails, 26 from social networks, ten from the German association of spina bifida site and three from the university website. With 55.7% of the participants, the virtual forum was the most agile dissemination medium to capture the data. This data collection mode should be used in future research, involving different countries, due to the speed, saving, good use of answers and help to overcome the language barrier. Public policies that support people's access to the Internet are necessary, so that these studies can be conducted successfully.

RESUMEN Este estudio describe el uso de un cuestionario online y su divulgación virtual como una estrategia de recolección de datos. El instrumento online fue desarrollado utilizando una plataforma virtual, creando un link que se publicó en ambientes virtuales. Participaron 100 alemanes con Espina Bífida. 149 personas tuvieron acceso al link, 83 oriundos de foros virtuales, 27 de correos electrónicos, 26 de redes sociales, 10 provenientes de la asociación alemana de espina bífida y 3 del website de la universidad. Con el 55,7% de los participantes, el medio de divulgación más ágil para la captura fue el foro virtual. Se sugiere que este método sea utilizado para investigaciones futuras que involucren varios países, esto debido a la velocidad, la economía, el buen uso de respuestas y por permitir a superar la barrera del idioma. Se requieren políticas públicas que apoyen el acceso de las personas a Internet, para que estos estudios se realicen con éxito.

RESUMO Este estudo teve como objetivo descrever o uso de questionário online e sua divulgação virtual como estratégia de coleta de dados na Alemanha. O questionário online foi elaborado com uma plataforma virtual, criando um link que foi divulgado em ambientes virtuais. Participaram 100 indivíduos alemães com Espinha Bífida; 149 pessoas visitaram o link, dessas 83 oriundas de fóruns virtuais, 27 e-mails, 26 redes sociais, dez do site da associação da espinha bífida alemã e três do site da universidade. Com 55,7% dos participantes, o meio de divulgação mais ágil para captação foi o fórum virtual. Sugere-se que esse modo de coleta de dados seja utilizado para pesquisas futuras, envolvendo diversos países, devido à rapidez, economia, bom aproveitamento de respostas e por permitir ultrapassar a barreira linguística. Políticas públicas que apoiem o acesso da população à internet são necessárias, para que esses estudos sejam realizados com êxito.

Fetoscopy for meningomyelocele repair: past, present and future / Cirurgia fetal endoscópica para correção de mielomeningocele: passado, presente e futuro

ABSTRACT Meningomyelocele is a malformation with high prevalence, and one of its main comorbidities is Arnold-Chiari malformation type II. The intrauterine repair of this defect has been studied to reduce the progressive spinal cord damage during gestation. The purpose of the present review was to describe the evolution of fetal surgery for meningomyelocele repair. Searches on PubMed database were conducted including articles published in the last 10 years. Twenty-seven articles were selected, 16 experimental studies and 11 studies in humans. A recent study demonstrated that the fetal correction results in better prognosis of neurological and psychomotor development, but open surgery, which has being used widely, has considerable maternal risks. Studies in animal and human models show that the endoscopic approach is feasible and leads to lower maternal morbidity rates. Two endoscopic techniques are currently under assessment - one in Germany and another in Brazil, and we believe that the endoscopic approach will be the future technique for prenatal repair of this defect.

RESUMO A meningomielocele é uma malformação de alta incidência e, dentre suas principais comorbidades, está a malformação de Arnold-Chiari tipo II. A fim de reduzir os danos progressivos durante a gestação, tanto a nível medular, quanto sobre a fossa posterior, a correção intrauterina desse defeito vem sendo estudada. A presente revisão teve por objetivo descrever a evolução da cirurgia fetal para a correção da meningomielocele. Foi realizada uma pesquisa na base de dados PubMed, incluindo artigos publicados nos últimos 10 anos. Foram selecionados 27 artigos, sendo 16 de estudos experimentais e 11 sobre pesquisa em humanos. Um estudo recente demonstrou que a correção pré-natal resulta em melhor prognóstico neuropsicomotor, porém a abordagem a céu aberto, que vem sendo amplamente utilizada, possui um risco materno considerável. Estudos, tanto em modelo animal, quanto em humanos, mostram que a abordagem endoscópica é factível e apresenta menor morbidade materna. No momento, duas técnicas de abordagem endoscópica estão sendo estudadas, uma na Alemanha, e outra no Brasil, e acreditamos que a via endoscópica será o futuro da correção pré-natal desse defeito.

Atuação no intra operatório da correção cirúrgica de mielomeningocele a "céu aberto" intraútero / Practice in the intraoperative period of surgical correction of a “sky open” myelomeningocele in utero

Relatar a experiência da atuação da enfermeira no intraoperatório de correção de mielomeningoceles a céu aberto intraútero. Método: Relato de experiência da prática em um hospital privado de São Paulo. Resultados: A enfermeira possui papel fundamental na previsão e na provisão de materiais e equipamentos para a cirurgia, no acompanhamento da paciente na admissão no Centro Cirúrgico e no auxílio durante o ato anestésico-cirúrgico. Conclusão: Fatores primordiais para o sucesso da cirurgia são o conhecimento e o domínio da equipe sobre as fases da cirurgia e o atendimento a pacientes gestantes, considerando as possíveis complicações que podem envolver a mãe ou o feto...

Comparação entre duas técnicas cirúrgicas para correção intra-uterina de meningomielocele em feto de ovelha / Comparison between two surgical technique for prenatal correction of meningomyelocele in sheep

Introdução: A incidência de defeitos do tubo neural é de cerca de 1/1000 nascimentos. Na mielomenngocele, o tratamento intra-uterino utilizando a técnica neurocirúrgica clássica, apresenta melhor prognóstico neurológico, do que o tratamento pós natal, mas está associado a complicações maternas e fetais. Novas técnicas de correção estão sendo estudadas para diminuir a morbidade materna e fetal. Objetivo: Comparar os efeitos sobre a medula de duas técnicas cirúrgicas de correção intra-uterina de um defeito semelhante à mielomeningocele , em fetos de ovelha. Métodos: Em 15 fetos foi criado um defeito semelhante à mielomeningocele (laminectomia e excisão de dura-máter) no 90° dia de gestação. O tipo de correção foi randomizado. No grupo 1, o defeito foi corrigido usando a técnica neurocirúrgica clássica, com a sutura de três camadas (dura-máter, músculo e pele), realizada por um neurocirurgião. No grupo 2, um especialista em Medicina Fetal, utilizou uma técnica simplificada, colocando um fragmento de celulose Biosintética sobre a medula e suturando apenas a pele sobre a celulose. Próximo ao termo da gestação (132° dias), os fetos foram sacrificados para análise anatomopatológica. Resultados: Ocorreram 1 morte materna, 3 casos de trabalho de parto precoce e 4 tardios. Um total de 10 casos foram viáveis para avaliação anatomopatológica 10 casos, 6 no grupo 1 e 4 no grupo 2. No grupo 1, todos os casos mostraram aderência da medula à cicatriz (meningoadesão) e perda da arquitetura medular por destruição do funículo posterior e 5 de 6 casos apresentaram perda da visualização da substância cinzenta. No grupo 2, observamos em todos os casos, a formação de uma neoduramater, separando o tecido nervoso do músculo adjacente, sendo que o funículo posterior e a substância cinzenta estavam preservados. Conclusão: A técnica simplificada foi superior à técnica neurocirúrgica, com maior preservação da medula e evitando as aderências do tecido nervoso. Nossos...

Introduction: The incidence of neural tube defects is about 1/1000 births. In myelomeninocele, the intrauterine treatment with the classical neurosurgical technique suggest better neurological prognosis for the fetus, than postnatal treatment, but it is associated with maternal and fetal complications. New correction techniques are being studied to decrease maternal and fetal morbidity. Objective: To compare the effects on the medulla of two surgical techniques for intrauterine correction of myelomeningocele-like defect in sheep. Methods: In 15 pregnant sheep a myelomeningocele -like defect (laminectomy and dural excision) was created in the lumbar region in 90o day gestation. The type of correction was randomized. In group 1 the defect was corrected using the classic neurosurgical technique of three layers suture (dura-mater, muscle and skin closure) performed by a neurosurgeon. In group 2, a fetal medicine specialist used a biosynthetic cellulose patch to protect the medulla and only the skin was sutured above it. Near term (132o day gestation) fetuses were sacrificed for pathological analysis. Results: One maternal death occurred, early preterm labour, and late preterm labour occurred in 3 and 4 cases, respectively. A total of 10 cases were available for pathological analysis, 6 in group 1 and 4 in group 2. In group 1, all the cases showed a adherence of the medulla to the scar (meningoneural adhesion) and a destruction of the normal architecture of nervous tissue, whithout view of posterior funiculus and in 5 of 6 cases without view of grey matter. In group 2, in all the cases were observed a formation of an organized tissue involving the cellulose patch, a neoduramater, separating the nervous tissue of adjacent muscle, preserving the posterior funicullus and grey matter. Conclusion: The simplified new technique was better than the classical neurosurgical technique. It preserved the nervous tissued and avoided the adherence of the medulla to the scar....

Improvement of motor function and decreased need for postnatal shunting in children who had undergone intrauterine myelomeningocele repair / Melhora em função motora e necessidade reduzida de shunts pós-natais em crianças submetidas a cirurgia fetal uterina da mielomeningocele

Objective To compare neuromotor development between patients who did and those who did not undergo intrauterine myelomeningocele repair. Methods Children with myelomeningocele aged between 3.5 and 6 years who did undergo intrauterine repair (Group A, n=6) or not (Group B; n=7) were assessed for neuromotor development at both anatomical and functional levels, need for orthoses, and cognitive function. Results Intrauterine myelomeningocele repair significantly improved motor function. The functional level was higher than the anatomical level by 2 or more spinal segments in all children in Group A and 2 children in Group B, with a significant statistical difference between groups (p<0.05). Five children in Group A and one in Group B were community ambulators. Conclusion Despite the small sample, it was observed that an improvement of motor function and decreased need for postnatal shunting in the 6 children who had undergone intrauterine myelomeningocele repair. .

Objetivo Comparar o desenvolvimento neuromotor de pacientes submetidos à cirurgia fetal intrauterina da mielomeningocele ao de pacientes não submetidos ao procedimento. Métodos Foram avaliados: o desenvolvimento neuromotor (descrevendo o nível anatômico e motor funcional), o tipo de marcha, a necessidade de órteses e o nível cognitivo de crianças com mielomeningocele entre 3,5 e 6 anos de idade, submetidas (Grupo A; n=6) ou não submetidas (Grupo B; n=7) à cirurgia fetal intra-uterina. Resultados A função motora apre-sentou melhora significante, com nível funcional mais elevado em dois ou mais segmentos em relação ao nível anatômico em todas as crianças do Grupo A e em duas crianças do Grupo B, com diferença estatística entre os grupos (p<0.05). Cinco crianças do Grupo A e uma do grupo B eram deambuladoras comunitárias. Conclusão Apesar da pequena amostragem, nos 6 casos de cirurgia prenatal observouse melhora da função motora e menor necessidade de shunts pós-natais. .

Comparison between two surgical techniques for prenatal correction of meningomyelocele in sheep / Comparação entre duas técnicas cirúrgicas de correção intrauterina da mielomeningocele em fetos de ovelha

OBJECTIVE: To compare the classical neurosurgical technique with a new simplified technique for prenatal repair of a myelomeningocelelike defect in sheep. METHODS: A myelomeningocele-like defect (laminectomy and dural excision) was created in the lumbar region on day 90 of gestation in 9 pregnant sheep. Correction technique was randomized. In Group 1 the defect was corrected using the classic neurosurgical technique of three-layer suture (dura mater, muscle and skin closure) performed by a neurosurgeon. In Group 2, a fetal medicine specialist used a biosynthetic cellulose patch to protect the spinal cord and only the skin was sutured above it. Near term (day 132 of gestation) fetuses were sacrificed for pathological analysis. RESULTS: There were two miscarriages and one maternal death. In total, six cases were available for pathological analysis, three in each group. In Group 1, there were adherence of the spinal cord to the scar (meningo-neural adhesion) and spinal cord architecture loss with posterior funiculus destruction and no visualization of grey matter. In Group 2, we observed in all cases formation of a neo-dura mater, separating the nervous tissue from adjacent muscles, and preserving the posterior funiculus and grey matter. CONCLUSION: The new simplified technique was better than the classic neurosurgical technique. It preserved the nervous tissue and prevented the adherence of the spinal cord to the scar. This suggests the current technique used for the correction of spina bifida in humans may need to be reassessed.

OBJETIVO: Comparar a técnica neurocirúrgica clássica a uma nova técnica simplificada, para correção de mielomeningocele, em fetos de ovelhas. MÉTODOS: Em 9 fetos, foi criado um defeito semelhante à mielomeningocele (laminectomia e excisão de dura-máter) no 90º dia de gestação. O tipo de correção foi randomizado. No Grupo 1, o defeito foi corrigido usando a técnica neurocirúrgica clássica, com a sutura de três camadas (dura-máter, músculo e pele), realizada por um neurocirurgião. No Grupo 2, um especialista em Medicina Fetal utilizou a técnica simplificada, colocando um fragmento de celulose biossintética sobre a medula e suturando apenas da pele sobre a celulose. Próximo ao termo da gestação (132 dias), os fetos foram sacrificados para análise anatomopatológica. RESULTADOS: Ocorreram dois casos de aborto e uma morte materna, restando seis casos para avaliação - três em cada grupo. No Grupo 1, todos os casos mostraram aderência da medula à cicatriz (meningoadesão) e perda da arquitetura medular, com destruição do funículo posterior e perda da visualização da substância cinzenta. No Grupo 2, observou-se, em todos os casos, a formação de uma neodura-máter, separando o tecido nervoso do músculo adjacente, sendo que o funículo posterior e a substância cinzenta estavam preservados. CONCLUSÃO: A técnica simplificada foi superior à neurocirúrgica, com maior preservação da medula e evitando as aderências do tecido nervoso. Os presentes achados sugerem que a técnica utilizada atualmente na correção de mielomeningocele em fetos humanos deva ser reavaliada.