Management of neurogenic bladder dysfunction in children update and recommendations on medical treatment

ABSTRACT Introduction: Defective closure of the neural tube affects different systems and generates sequelae, such as neurogenic bladder (NB). Myelomeningocele (MMC) represents the most frequent and most severe cause of NB in children. Damage of the renal parenchyma in children with NB acquired in postnatal stages is preventable given adequate evaluation, follow-up and proactive management. The aim of this document is to update issues on medical management of neurogenic bladder in children. Materials and Methods: Five Pediatric Urologists joined a group of experts and reviewed all important issues on "Spina Bifida, Neurogenic Bladder in Children" and elaborated a draft of the document. All the members of the group focused on the same system of classification of the levels of evidence (GRADE system) in order to assess the literature and the recommendations. During the year 2020 the panel of experts has met virtually to review, discuss and write a consensus document. Results and Discussion: The panel addressed recommendations on up to date choice of diagnosis evaluation and therapies. Clean intermittent catheterization (CIC) should be implemented during the first days of life, and antimuscarinic drugs should be indicated upon results of urodynamic studies. When the patient becomes refractory to first-line therapy, receptor-selective pharmacotherapy is available nowadays, which leads to a reduction in reconstructive procedures, such as augmentation cystoplasty.

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)

Study of serum and urinary markers of the reninangiotensin-aldosterone system in myelomeningocele patients with renal injury detected by DMSA

ABSTRACT Introduction: The Renin-Angiotensin-Aldosterone System (RAAS) has been suggested as a possible marker of renal injury in chronic diseases. This study proposes to analyze the serum and urinary markers of the RAAS in myelomeningocele patients with renal function abnormalities detected on DMSA. Material and Methods: Seventeen patients followed in our institution that presented with renal injury on DMSA. We review nephrologic and urologic clinical aspects and evaluated ultrassonagraphy, voiding urethrocystography and urodynamics. Urinary and serum samples were collected to evaluate possible correlations of renal lesions with RAAS. Control group urine and serum samples were also sent for analysis. Results: Serum ACE 2 activity means in relation to urodynamic findings were the only values that had a statistically significant difference (p = 0.040). Patients with normal bladder pattern presented higher ACE 2 levels than the high risk group. Statistical analysis showed that the study group (SG) had a significantly higher mean serum ACE than the CG. The means of ACE 2 and urinary ACE of the SG and CG were not statistically different. The ROC curve for serum ACE values had a statistically significant area for case and non-case differentiation, with 100% sensitivity and 53% specificity for values above 60.2 mg/dL. No statistically significant areas were observed in relation to ACE 2 and urinary ACE values between SG and CG. Conclusion: The analysis of serum ACE, ACE 2 and urinary ACE were not significant in patients with myelomeningocele and neurogenic bladder with renal injury previously detected by renal DMSA.

Series de casos de malformación de Arnold Chiari: Hospital Nacional Mario Catarino Rivas, 2008-2018 / Arnold Chiaris malformation case series: Hospital Nacional Mario Catarino Rivas, 2008-2018

La malformación de Arnold Chia-ri (MAC) es una enfermedad por primera vez descrita en 1883 y clasificada en 1891 en cuatro grupos.Se ha reportado una prevalencia de malformación de Chiari tipo 1 de 0.24-0.9% de los adultos que se realizan un resonancia magnética cerebral por cualquier motivo. Los sín-tomas se relacionan a hidrocefalia obstructiva, movimientos oculares anormales y mielomeningocele espinal. Su causa es multifactorial y las manifestaciones clínicas muy variadas. Se relaciona con síndromes de craneosinostosis, síndromes osteopáticos, desórdenes del tejido conectivo, anomalías vertebrales, anomalías cráneofaciales, síndrome de Williams, síndrome de Noonan y neurofibromatosis. El diagnóstico se realiza por resonancia magnética.Casos clínicos: Se presenta una serie de 3 casos de pacientes que acudieron al Hospital Mario Catarino Rivas (HMCR) en el periodo de 2008-2018. El motivo de consulta fue diferente en cada paciente, siendo: vómito, pérdida de fuerza muscular, disfagia, dolor en miembro superior derecho, paresia y parestesia de miembro inferior y ataxia, aumento del perímetro cefálico. El tipo de malformación fue tipo1 en dos casos, tipo2 en un caso.Solo dos de los pacientes se operaron, teniendo evolución posquirúrgica satisfactoria. Conclusión: La MAC es una patología poco estudiada en el país. No existe una forma de presentación global, por lo que siempre hay que tenerla en cuenta cuando se presente un paciente con alguna de esta sintomatología...(AU)

Composición corporal y gasto energético en población de niños y adolescentes con mielomeningocele / Body composition and energy expenditure in a population of children and adolescents with myelomeningocele

Introducción: El mielomeningocele es un defecto congénito con cierre incompleto del tubo neural. Presenta alteraciones en la composición corporal y alta prevalencia de obesidad. Es difícil detectar el indicador más apropiado para diagnóstico nutricional por impresición de las medidas antropométricas. Objetivo: Describir en una población de pacientes con mielomeningocele seguidos en el "Hospital Garrahan", la composición corporal, gasto energético en reposo y trastornos metabólicos, comparando los pacientes con mielomeningocele obesos con una población control con obesidad multifactorial. Población y Métodos: Se realizó antropometría, impedanciometría, pliegues cutáneos, perímetro braquial, calorimetría indirecta y determinaciones bioquímicas a todos los pacientes con mielomeningocele entre junio/2013-abril/2014, previa firma del Consentimiento Informado. Resultados: Se evaluaron 131 pacientes de 0,718,6 años, clasificados según Score-Z de Índice de Masa Corporal en 15% bajo peso, 42% normopeso, 12% sobrepeso y 31% obesidad. Se encontró alta correlación (r²0,74) entre %masa grasa por impedanciometría vs calculado con pliegues cutaneos. Los pacientes con % masa grasa elevada vs %masa grasa normal tuvieron mayor score-Z de Indice de Masa Corporal (1,07 vs -0,27 p0,0001) aunque ambos valores se encontraban dentro de parámetros normales. Hubo menor gasto energético en reposo en los pacientes con mielomeningocele obesos comparado con el esperado y con obesos multifactoriales. Conclusiones: Se encontró alto porcentaje de sobrepeso/obesidad en la población con mielomeningocele. Los pliegues cutáneos serían más apropiados para detectar obesidad. Los pacientes con mielomeningocele obesos presentaron gasto energetico en reposo menor al esperado y a los controles. La indicación de energía debe ser personalizada.

Introduction. Myelomeningocele is a congenital defect that occurs when the neural tube fails to close completely. It causes body composition alterations and a high prevalence of obesity. It is difficult to detect the most adequate indicator for a nutritional diagnosis due to the impossibility of recording accurate anthropometric measurements. Objective. To describe body composition, resting energy expenditure and metabolic disorders in a population of patients with myelomeningocele managed at "Hospital Garrahan" by comparing obese patients with myelomeningocele and a control population with multifactorial obesity. Population and methods. An anthropometry, an impedance analysis, skinfold equations, arm circumference equations, indirect calorimetry, and biochemical determinations were done to all patients with myelomeningocele between June 2013 and April 2014, once the informed consent had been signed. Results. 131 patients aged 0.7-18.6 years were assessed; they were classified according to their body mass index Z-score into low weight (15%), normal weight (42%), overweight (12%), and obese (31%). A high correlation (r: 20.74) was observed between the fat mass % measured by impedance analysis versus that estimated using skinfolds. Patients with a high fat mass % had a higher body mass index Z-score than those with a normal fat mass % (1.07 versus -0.27, p: 0.0001) although both values were within normal parameters. A lower resting energy expenditure was observed among obese patients with myelomeningocele than predicted and in comparison with multifactorial obese controls. Conclusions. A high percentage of overweight/obesity was found in the population with myelomeningocele. Skinfold equations would be more adequate to detect obesity. Obese patients with myelomeningocele had a lower resting energy expenditure than predicted and in comparison with controls. Energy indication should be customized.

Alterações auditivas em recém-nascidos e lactentes com mielomeningocele / Hearing impairment in newborns and infants with myelomeningocele

RESUMO Objetivo Verificar a ocorrência de alterações auditivas em recém-nascidos e lactentes com mielomeningocele e/ou Síndrome de Arnold Chiari tipo II. Métodos Foram incluídos na amostra 160 neonatos atendidos em unidade de terapia intensiva neonatal, distribuídos em três grupos: Grupo Estudo 1, formado por 42 neonatos com Síndrome de Arnold Chiari tipo II; Grupo Estudo 2, constituído por 38 neonatos com mielomeningocele e Grupo Controle, com 80 recém-nascidos sem síndromes, malformações e/ou riscos para alteração auditiva. Todos os pacientes realizaram a triagem auditiva neonatal com emissões otoacústicas evocadas por estímulo transiente e foram submetidos à pesquisa do potencial evocado auditivo de tronco encefálico com estímulo clique. Quando houve falha nas emissões, a pesquisa dos limiares eletrofisiológicos foi realizada mediante potencial evocado auditivo de tronco encefálico com tone burst . A análise estatística foi feita por meio de testes paramétricos e os dados encontrados foram descritos nos resultados, considerando o nível de significância p<0,05. Resultados Houve maior ocorrência de perda coclear, alteração central e neuropatia auditiva nos grupos estudados. No que se refere aos valores médios das latências absolutas e dos intervalos interpicos, nos três grupos, por orelha, houve aumento das latências absolutas das ondas III e V e dos intervalos interpicos III-V e I-V , no grupo de lactentes com Síndrome de Arnold Chiari tipo II. Conclusão Recém-nascidos e lactentes com Síndrome de Arnold Chiari tipo II e mielomeningocele apresentaram maior ocorrência de perda coclear, espectro da neuropatia auditiva e alterações centrais.

ABSTRACT Purpose To characterize Auditory and oral language performance in children under 2 years of age with Hearing and Language Development Scale (EDAL-1) and to compare the results to those of hearing children up to two years of age. Methods This is an experimental study of descriptive nature. A total of 141 children were divided into two groups: control group with 92 normal hearing, and the experimental group with 49 deaf children followed audiologically during the first two years after implantation. Normal hearing children underwent auditory assessment and parents responded to EDAL-1. The children implanted, every three months, underwent otorhinolaryngological and speech-language evaluation, and the parents answered EDAL-1. Results In both groups we observed upward responses, with improvement as chronological and auditory age increases. Results obtained in the study group are statistically worse than in the control group, but with a tendency to grow. EDAL-1 was easy to apply and allowed to monitor the performance of the implanted children. Conclusion Oral and auditory performance in deaf children with cochlear implants was characterized by EDAL-1, and it was shown to be growing as the time of auditory stimulation increased, a fact also seen in hearing children. Despite this, the results of the hearing children were better than those of the deaf children implanted.

Cirugía precoz en la prevención de la sepsis del recién nacido con mielomeningocele / Precocious surgery in preventing the sepsis of newborn with meningomyelocele

El mielomeningocele es una malformación congénita por defecto del cierre del tubo neural, se produce en las primeras semanas de crecimiento intrauterino. Consiste en una masa quística que incluye tejido nervioso y meninges, acompañadas de una fusión incompleta de los arcos vertebrales. Produce severos daños neurales y puede asociarse a otras malformaciones. Su reparación, en las primeras horas de vida, es necesaria para evitar complicaciones que pueden comprometer la vida del niño o causar mayor discapacidad. Se reportó un caso de un recién nacido con diagnóstico de mielomeningocele fisurado, a nivel lumbar. Se intervino quirúrgicamente en las primeras 6 h de vida; se disecó el saco dural y se separaron las raíces, logrando el cierre completo de la duramadre. Se reparó la piel con afrontamiento de los bordes y adecuada cicatrización de la herida. Fue aplicada antibioticoterapia profiláctica con cefalosporina de tercera generación. El infante egresó a los 10 días de operado sin complicaciones neuroquirúgicas asociadas (AU).

Meningomyelocele is a congenital malformation by defect of neural tube closing, produced in the first weeks of intrauterine grow. It is a cystic mass that includes nervous tissues and meninges, together with an incomplete fusion of the vertebral arches. It produces severe neural damages and could be associated to other malformations. It is necessary to repair it during the first hours after birth to avoid complications that could compromise the child’s life or cause more disability. The case of a new-born child diagnosed with fissured meningomyelocele at the lumbar level is presented. He was operated in the first six hours after birth; the dural sac was dissected and the roots separated, reaching the complete dura mater closure. The skin was repaired with edges affronting and adequate wound healing. Prophylactic antibiotic therapy with third generation cephalosporin was applied. The child was discharged 10 days alter the surgery without associated neurosurgical complications (AU).

Benefits from using an ankle-foot orthosis in children with myelomeningocele / Benefícios do uso da órtese tornozelo-pé nas crianças com mielomeningocele

Myelomeningocele (MMC) or spina bifida is a defect of the neural tube in which the spinal cord, its envelopes (meninges), and vertebral arches develop abnormally in the beginning of gestation, and owing to this failure of closure there appear a series of congenital defects and associated comorbidies, impairing in several aspects the functioning of the life of children with MMC. The congenital clubfoot has been found the most common orthopaedic anomaly in patients with MMC. The ankle-foot orthosis (AFO) is an orthopaedic device commonly used by these children to minimize the sequelae caused by this anomaly. OBJECTIVE: Identify the functional benefits brought about by the use of the AFO to children with MMC, as reported by their guardians. METHOD: Descriptive, transversal study. Convenience sample consisting of 25 guardians of children with MMC who were using/had used an AFO. RESULTS: Eighty percent of the guardians have reported at least one benefit brought about by the use of the AFO, among them: improvement in foot position (68%), foot growth (40%), improvement in foot balance (32%), and balance sitting position (15%). CONCLUSION: The use of the AFO by children with MMC can provide several benefits reported by their guardians.

A mielomeningocele (MMC) ou espinha bífida é um defeito do tubo neura no qual a medula espinal, seus envoltórios (meninges) e os arcos vertebrais desenvolvem-se anormalmente no início da gestação e, como consequências, temos uma série de defeitos congênitos e comorbidades associadas prejudicando a funcionalidade em diversos aspectos da vida das crianças com MMC. O pé torto congênito foi apontado como a anomalia ortopédica mais comum nos pacientes com MMC e a órtese tornozelo-pé (OTP) é um aparelho ortopédico que pode ser usado nessas crianças para amenizar as sequelas geradas por essa anomalia. OBJETIVO: Identificar os benefícios funcionais trazidos pelo uso da OTP em crianças com MMC relata-dos pelos seus responsáveis. MÉTODO: Estudo descritivo, transversal. Amostra de conveniência composta por responsáveis de 25 crianças com MMC que usavam/usaram a OTP que responderam a uma entrevista estruturada que contemplava as variáveis estudadas. RESULTADOS: Oitenta por cento dos responsáveis relataram pelo menos um benefício causado pelo uso da OTP, dentre eles: melhorou a postura do pé (68%), o pé cresceu (40%), melhorou o equilíbrio em pé (32%), melhorou o equilíbrio sentado (15%). CONCLUSÃO: O uso da OTP em crianças com MMC pode proporcionar benefícios identificados pelos responsáveis.

Prevalencia de alergia al látex en una población de pacientes con diagnóstico de mielomeningocele / Prevalence of latex allergy in a population of patients diagnosed with myelomeningocele

Introducción: La alergia al látex se encuentra dentro de las primeras causas de anafilaxia en el quirófano. La prevalencia de esta enfermedad es más elevada en pacientes con mielomenigocele. Existen escasos datos epidemiológicos en pacientes argentinos. Objetivo: Estimar la prevalencia de la sensibilidad y de la alergia al látex en una población de pacientes con mielomeningocele y describir los factores de riesgo asociados. Pacientes y métodos: Estudio descriptivo, transversal, observacional. Se analizaron los antecedentes familiares y personales de alergia, el número de procedimientos quirúrgicos, la historia de síntomas ante el contacto con látex o alimentos con reactividad cruzada, el recuento de eosinófilos, la inmunoglobulina E total y la inmunoglobulina E específica mediante pruebas serológicas y cutáneas para el látex, los aeroalérgenos y las frutas con reactividad cruzada. Resultados: Se evaluaron 82 pacientes con diagnóstico de mielomeningocele, 41 del sexo masculino (50%), con edad promedio de 15,3 ± 7,66 años. Del total de los pacientes, 2 no realizaron las pruebas cutáneas y serológicas. De los 80 restantes, 16 (19,51%) presentaban alergia al látex, 46 (57,5%) no eran alérgicos y 18 (22%) fueron sensibles pero no alérgicos. Se observó que haber tenido más de 5 cirugías representaba un factor de riesgo asociado a alergia al látex (p= 0,035). No se encontró una asociación significativa con el resto de las variables evaluadas. Conclusión: El estudio estima que la prevalencia de alergia al látex en esta población de pacientes es de 19,51% y que el factor de riesgo más importante para el desarrollo de esta patología es el antecedente de haber sido sometido a más de 5 intervenciones.

Introduction: Latex allergy is one of the main reasons of anaphylaxis in the operating room. The prevalence of this condition is higher among patients with myelomeningocele. Epidemiological data obtained from Argentine patients is scarce. Objective: To estimate the prevalence of latex sensitivity and latex allergy in a population of patients with myelomeningocele and to describe associated risk factors. Population and Methods: Descriptive, crosssectional, observational study. Family and personal history of allergy, number of surgeries, history of symptoms caused by having been in contact withlatex or cross-reactive foods, eosinophil count, measurement of total immunoglobulin E and specific immunoglobulin E levels by means of skin and serologic testing for latex, aeroallergens and cross-reactive fruit. Results: Eighty-two patients diagnosed with myelomeningocele were assessed: 41 were males and their average age was 15.3 ? 7.66 years old. Out of all patients, two did not complete skin and serologic testing. Among the remaining 80 patients, 16 (19.51%) had latex allergy, 46 (57.5%) were not allergic, and 18 (22%) showed sensitivity but not allergy. Having undergone more than five surgeries was a risk factor associated with latex allergy (p= 0.035). No significant association was observed with the remaining outcome measures. Conclusion: According to this study, the prevalence of latex allergy in this population of patients is 19.51% and the most important risk factor for this condition is a history of having undergone more than five surgeries.

Fluorodeoxyglucose-positron emission tomography/computed tomography imaging of squamous cell carcinoma arising in a meningomyelocele

No abstract available.

Surgical technique of retrograde ventricle-sinus shunt is an option for the treatment of hydrocephalus in infants after surgical repair of myelomeningocele / A derivação ventrículo-sinusal retrógrada é uma opção para o tratamento de hidrocefalia em lactentes após correção de mielomeningocele

ABSTRACT Introduction Treatment of hydrocephalus is accomplished primarily through a ventricular-peritoneal shunt (VPS). This study aims to describe the application of retrograde ventricle-sinus shunt (RVSS) in patients with hydrocephalus after surgical treatment of myelomeningocele. Method A prospective, randomized and controlled pilot study. We consecutively enrolled 9 patients with hydrocephalus after surgical repair of myelomeningocele from January 2010 to January 2012. These patients underwent elective RVSS or VPS. Five underwent RVSS and 4 underwent VPS. Patients were followed for one year with quarterly evaluations and application of transcranial Doppler. Results RVSS group showed outcomes similar to those of VPS group. Doppler revealed significant improvement when comparing preoperative to postoperative period. RVSS group had significantly higher cephalic perimeter than VPS group. Neuropsychomotor development, complications and subjective outcomes did not differ between groups. Conclusion RVSS shunt is viable; it is an alternative option for the treatment of hydrocephalus.

RESUMO O tratamento da hidrocefalia é realizado principalmente através de uma derivação ventrículo-peritoneal (DVP). Nosso objetivo é descrever a aplicação da derivação ventrículo-sinusal retrógrada (DVSR) em pacientes com hidrocefalia após o tratamento cirúrgico de mielomeningocele. Método Estudo prospectivo, randomizado e controlado. Selecionados consecutivamente 9 pacientes com hidrocefalia após correção cirúrgica de mielomeningocele de janeiro de 2010 a janeiro de 2012. Eles foram submetidos à DVSR ou DVP. Cinco foram submetidos à DVSR e 4 à DVP. Foram seguidos por 1 ano com realização trimestral de avaliações e aplicação do Doppler transcraniano. Resultados O grupo DVSR apresentou desfechos semelhantes ao grupo DVP. O Doppler mostrou melhora significativa quando comparado o pré-operatório com o pós-operatório. O grupo DVSR apresentou perímetro cefálico significativamente maior que o grupo DVP. O desenvolvimento neuropsicomotor e complicações não diferiram entre os grupos. Conclusão A derivação ventrículo-sinusal retrógrada é viável; ela é uma opção para o tratamento de hidrocefalia.

Clinical and ultrasonographic criteria for using ventriculoperitoneal shunts in newborns with myelomeningocele / Critérios clínicos e ultrassonográficos para a indicação de derivação ventrículo peritoneal em neonatos portadores de mielomeningocele

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

Objetivo Identificar os critérios clínicos e ultrassonográficos para a recomendação do implante de derivações ventrículo peritoneais (DVP) em neonatos portadores de mielomeningocele (MM).Método Estudo de coorte retrospectivo, com base no protocolo estabelecido para o implante de DVP em crianças com hidrocefalia associada a MM. Parâmetros utilizados para orientar a indicação de DVP incluíram a medida da circunferência craniana (CC), a avaliação das fontanelas e a medida da largura lateral do átrio ventricular (LAV), avaliado por ultrassonografia transcraniana.Resultados 43 crianças foram incluídas na análise, dos quais 74% tinham hidrocefalia com recomendações para uso de DVP.Conclusão O aumento da CC e o abaulamento de fontanelas foram os principais critérios para a indicação de DVP. A DVP é necessária em crianças com aumento da CC (≥ 2 desvios padrões para a idade), fontanelas abauladas, ou LAV ≥ 15 mm após o fechamento cirúrgico da MM.

Surgical results of strabismus correction in patients with myelomeningocele / Resultados cirúrgicos da correção do estrabismo em pacientes com mielomeningocele

Purpose: Myelomeningocele is one of the most common birth defects. It is associated with severe neurological deficiencies, and ocular changes, such as strabismus, are very common. The purpose of this study was to describe indications for strabismus surgery in patients with myelomeningocele and to evaluate the results achieved with surgical correction. Methods: We retrospectively reviewed records of all patients with myelomeningocele who underwent surgery for strabismus correction in a 5-year period in an institution for disabled children. Results: The main indications for strabismus surgery were esotropia and A-pattern anisotropia. Excellent surgical results were achieved in 60.9% of patients, satisfactory in 12.2%, and unsatisfactory in 26.9%. Conclusion: Patients with myelomeningocele and strabismus had a high incidence of esotropia and A-pattern anisotropia. Strabismus surgery in these patients had an elevated percentage of excellent and satisfactory results, not only for the ocular deviation, but also for improvement of head posture. .

Objetivo: A mielomeningocele é um dos mais frequentes defeitos do nascimento e está associada a disfunções neurológicas severas. Alterações oculares como estrabismo são muito comuns nesses pacientes. O objetivo deste estudo foi descrever as principais indicações de cirurgia de estrabismo em pacientes com mielomeningocele e avaliar os resultados atingidos com a correção cirúrgica. Métodos: Foi realizado estudo restrospectivo com revisão de prontuários de todos os pacientes com mielomeningocele submetidos à cirurgia para correção de estrabismo em um período de 5 anos em uma instituição de assistência a crianças deficientes. Resultados: As principais indicações para cirurgia de estrabismo foram esotropia e anisotropia com padrão em A. Resultados cirúrgicos excelentes foram alcançados em 60,9% dos pacientes, satisfatórios em 12,2% e insatisfatórios em 26,9%. Conclusão: Pacientes com mielomeningocele e estrabismo têm uma alta incidência de esotropia e anisotropia com padrão em A. A cirurgia de estrabismo nesta população teve uma elevada porcentagem de resultados excelentes e satisfatórios, não somente em relação ao desvio ocular, mas também na melhora na posição viciosa de cabeça .

Anestesia neuroaxial en trabajo de parto y cesárea en pacientes con antecedentes de mielomeningocele operado / Neuraxial anesthesia in labor and cesarean delivery in patients with a history of myelomeningocele operated

Los desórdenes del desarrollo del tubo neural son el segundo mayor grupo de malformaciones congénitas conocidas y con una incidencia de 1/1000 nacidos vivos. El meningomielocele es una de las malformaciones más frecuentes. Se define como una falla en el cierre del tubo neural con herniación de meninges y elementos neurales. El embarazo en estas pacientes es complicado por las deformidades físicas y complicaciones neurológicas secundarias, pudiendo dificultar la técnica anestésica neuroaxial en el trabajo de parto y operación cesárea. Existen escasos reportes de pacientes con meningomielocele en trabajo de parto y analgesia neuroaxial. Presentamos una revisión de esta patología y las técnicas anestésicas utilizadas en el trabajo de parto y operación cesárea de pacientes con antecedente de meningomielocele, basados en un caso clínico del cual participamos.

Neural tube defects are the second most common type of congenital birth defects with an incidence of 1/1000 newborns. Meningomyelocele is one of the most common clinical presentations. It is defined as a failed closure of the neural arch with herniation of the meninges and neural elements. Pregnancy among these patients can be complicated with physical deformity and coexisting neurological defects, which may challenge neuroaxial anesthetic technique in obstetric labor and cesarean delivery. There are few reports involving patients with meningomyelocele in obstetric labor and neuroaxial anesthesia. We discuss some key points of this disease and the anesthetic technique of choice in obstetric labor and cesarean delivery in patients with history of meningomyelocele, based on a case in which we participated.

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. .

Agenesia sacra asociada a disrrafismo espinal e hidrocefalia / Sacra agenesia associated to spinal dysraphism and hydrocephaly

Introducción: la agenesia sacra es una malformación congénita rara que forma parte del síndrome de regresión caudal. Se caracteriza por un grupo de anomalías en las cuales la columna caudal está ausente. Esta enfermedad es la malformación más frecuente en los hijos de madres diabéticas, además se ha relacionado con otros factores predisponentes, como deficiencias de ácido fólico, de vitaminas, uso de insulina en el embarazo, e incluso, la hipoxia. Entre un 30-40 por ciento de pacientes con agenesia sacra completa, pueden tener asociado un mielomeningocele, y el desplazamiento de las raíces nerviosas empeora los trastornos neurológicos. En estos casos, la hidrocefalia, muchas veces también asociada a malformación Chiari tipo II, está ya presente al nacer. Caso clínico: se presenta el caso de un neonato con agenesia sacra asociada a disrrafismo espinal e hidrocefalia. La intervención quirúrgica fue precoz, se le colocó derivación ventrículo peritoneal y se realizó la reparación del defecto del tubo neural. La evolución posoperatoria fue favorable, aunque persistieron los déficits neurológicos preoperatorios. Conclusiones: no se hallaron factores predisponentes en este paciente y el análisis del cariotipo fue normal. Las anomalías óseas de miembros inferiores fueron las más llamativas, así como la presencia de hidrocefalia asociada a malformación Chiari tipo II y mielomeningocele. El tratamiento a estos casos requiere de un enfoque multidisciplinar, y la reparación quirúrgica del mielomeningocele debe ser precoz para conseguir una evolución favorable. Las formas graves pueden ocasionar una muerte temprana neonatal, en cambio, los niños que sobreviven, generalmente presentan inteligencia normal

Introduction: sacra agenesia is a rare congenital malformation as part of the caudal regression syndrome. It is characterized by a group of anomalies in which the caudal cord is absent. This disease is the most common malformation found in children from diabetic mothers but it has also been related to other predisposing factors such as folic acid deficiencies, vitamin deficiencies, use of insulin at pregnancy and even hypoxia. Thirty to forty percent of patients with complete sacra agenesia can also have myelomeningocele, and the displacement of nerve roots worsens the neurological disorders. In these cases, hydrocephaly, many times associated to Chiari malformation type II, is also present at birth. Clinical case: a neonate with sacra agenesia associated to spinal dysraphism and hydrocephaly. Surgical intervention was performed early, a peritoneal ventricular derivation was placed and the neural tube defect was repaired. The post-surgery evolution was favorable, but the preoperative neurological deficits persisted. Conclusions: there were no predisposing factors in this patient and the analysis of the cariotype was normal. The bone anomalies of the lower members were the most remarkable aspects as well as the hydrocephaly associated to Chiari malformation type II and myelomeningocele. The treatment of these cases requires multidisciplinary approach and surgical repair of the myelomeningocele at early phase to achieve favorable evolution. The most severe forms can cause early neonatal death; however, those surviving children generally present normal intelligence coefficient

Perfil clínico-epidemiológico dos pacientes tratados com mielomeningocele em um hospital universitário de Curitiba / Clinical and epidemiological profile of patients treated with myelomeningocele at a university hospital in Curitiba

Objetivo: O objetivo deste trabalho é traçar o perfil clínico-epidemiológico dos recém-nascidos portadores de mielomeningocele tratados no serviço de neurocirurgia do Hospital Universitário Evangélico de Curitiba (HUEC) e comparar esses dados com a literatura. Método: Foram revistos os prontuários e descrições de cirurgia de todos os pacientes operados para tratamento de mielomeningocele no período de janeiro de 2007 a junho de 2012, compondo uma amostra de 26 pacientes. Resultados: Os pacientes estudados foram predominantemente do sexo masculino, com lesão ao nível da região lombar. Em 21 pacientes evidenciou-se a presença de hidrocefalia, e outras quatro crianças apresentaram outras malformações do sistema nervoso central. A maioria dos pacientes apresentou bexiga neurogênica, e outros nove recém-nascidos tiveram anormalidades ortopédicas. Conclusão: A mielomeningocele é um evento que cursa com alta morbidade principalmente devido às complicações neurológicas, urológicas, ortopédicas, entre outras, assim é de suma importância tanto estimular a prevenção quanto realizar o diagnóstico e tratamento de forma mais precoce possível.

Objective: The aim of this paper is to map the clinical and epidemiological profile of the newborn with myelomeningocele treated in the neurosurgery service of the Hospital Universitário Evangélico of Curitiba (HUEC) and compare this data with the literature. Method: We reviewed the hospital files, surgical and out-patient notes of all patients operated on for the treatment of myelomeningocele from January 2007 to June 2012, composing a sample of 26 patients. Results: The patients studied were predominantly male with an injury in the lumbar region. In 21 patients revealed the presence of hydrocephalus, and other four children had other malformations of the central nervous system. Most patients had neurogenic bladder, and other nine neonates had orthopedic abnormalities. Conclusion: Myelomeningocele is an event that leads to high morbidity mainly due to neurological, urological, orthopedic and others, so it is of paramount importance both to stimulate the prevention and perform the diagnosis and treatment as early as possible.