Intracranial Tumors in the First Year of Life.

Intracranial tumors in the first year of life are rare and, in this age group, are the second most common type of pediatric cancer after leukemias. As the more common solid tumor in neonates and infants, they present some peculiarities such as the high incidence of malignancies. Routine ultrasonography made easier to detect intrauterine tumors, but diagnosis can be delayed due to the lack or scarcity of recognizable symptoms. These neoplasms are often very large and highly vascular. Their removal is challenging, and there is a higher rate of morbidity and mortality than seen in older children, adolescents, and adults. They also differ from older children with respect to location, histological features, clinical behavior, and management. Pediatric low-grade gliomas represent 30% of the tumors in this age group and comprise circumscribed and diffuse tumors. They are followed by medulloblastoma and ependymoma. Other non-medulloblastoma embryonal neoplasms, former known as PNETS, are also commonly diagnosed in neonates and infants. Teratomas have an expressive incidence in newborns but decline gradually until the end of the first year of life. Immunohistochemical, molecular, and genomic advances are impacting the understanding and targeting of the treatment of some tumors, but, despite all these advances, the extent of resection remains the most important factor in the prognosis and survival of almost all types of tumors. The outcome is difficult to estimate, and 5-year survival ranges from one-quarter to three-quarters of the patients.

Identifying abusive head trauma and its mimics: diagnostic nuances.

BACKGROUND: Abusive head trauma (AHT) is the most serious injury inflicted to the nervous system of neonate an infant with a high incidence of disabilities. The authors present two cases in which the initial manifestations and neurologic status were misinterpreted and stress that clinical presentation and imaging can be variable and confuse the examiner. DISCUSSION: Subdural hemorrhage (SDH) in this age group raises high suspicion of non-accidental trauma but have been reported in other situations such as several bleeding disorders. Although rare, hematological diseases should be considered when other data of maltreatment are lacking. CONCLUSION: Differential diagnosis is important to avoid underdiagnosing AHT and to prevent morbidity if a pre-existing hematological disease is misdiagnosed.

Hydrocephalus and occipital encephaloceles: presentation of a series and review of the literature.

INTRODUCTION: Encephaloceles are rare congenital malformations of the central nervous system in which brain tissue is extruded from a defect in the skull. Hydrocephalus can occur in 60 to 90% of patients with posterior encephaloceles when compared to other types of this malformation. This article aims to present a series of posterior encephaloceles and its association with hydrocephalus as well as promote a review of the pertinent literature. MATERIAL AND METHODS: A retrospective study of our series based on hospital charts of 50 patients with posterior encephaloceles was performed. Data on sex, location of encephalocele, presence of associated malformations, presence of neural tissue within the malformation, presence of hydrocephalus and microcephaly were recorded. RESULTS: There were 29 females and 21 males. There were 25 (50%) supratorcular, 8 (16%) torcular, and 17 (34%) infratorcular lesions. Mean age of encephalocele primary repair was 8 days (range 2-120 days). Hydrocephalus was diagnosed in 25 (50%) of the cases. Ventriculoperitoneal shunt was inserted in 24 patients. The mean age at VP shunt insertion was 1.3 months (range 0.3-9 months). Endoscopic third ventriculostomy was successfully performed in one patient. Dandy-Walker malformation and ventriculomegaly prior to encephalocele surgical correction were positively associated with hydrocephalus (p values 0.05 and 0.01, respectively). Chiari III malformation was found in 2 cases, both requiring CSF shunt for treatment of hydrocephalus and are stable in follow-up. Microcephaly was present in 9 cases. The known mortality rate was 8%. CONCLUSIONS: Hydrocephalus is common in patients with posterior encephaloceles, being more frequent in the supratorcular type, especially when associated to Dandy-Walker, Chiari III malformation, and pre-existing ventriculomegaly. The severity of giant encephaloceles, when associated to torcular types and microcephaly, is a limiting factor for development of hydrocephalus, due both to the rapid evolution of natural history and the structural changes in microcephaly.

Reviewing the prognostic factors in myelomeningocele.

OBJECTIVE: The goal of this study was to analyze the factors that have an impact on morbidity and mortality in patients with myelomeningocele (MMC). METHODS: A retrospective cohort study was conducted to analyze factors associated with MMC that influence the morbidity and mortality of the disease. Data were collected from medical records of children who underwent the primary repair of MMC at the Fernandes Figueira Institute-Oswaldo Cruz Foundation (IFF-Fiocruz) between January 1995 and January 2015, with a minimum follow-up of 1 year. The following variables were analyzed: demographic characteristics (gestational age, sex, and birth weight); clinical features (head circumference at birth, anatomical and functional levels of MMC, hydrocephalus, symptomatic Chiari malformation type II, neurogenic bladder, and urinary tract infection [UTI]); and surgical details such as timing of repair of MMC, age at first shunt placement, shunt surgery modality (elective or emergency), concurrent surgery (correction of MMC and shunt insertion in the same surgical procedure), incidence and cause of shunt dysfunction, use of external ventricular drain, transfontanelle puncture, surgical wound complications prior to shunting, and endoscopic treatment of hydrocephalus. RESULTS: A total of 231 patients with MMC were included in the analysis. Patients were followed for periods ranging from 1 to 20 years, with a mean of 6.9 years. The frequency of shunt placement was observed mainly among patients with MMC at the highest spinal levels (p < 0.01). The main causes of morbidity and mortality in patients with MMC were shunt failures, diagnosed in 91 of 193 cases (47.2%) of hydrocephalus, and repeated UTIs, in 129 of 231 cases (55.8%) of MMC; these were the main causes of hospitalization and death. Head circumference ≥ 38 cm at birth was found to be a significant risk factor for shunt revision (p < 0.001; 95% CI 1.092-1.354). Also, the lumbar functional level of MMC was associated with less revision than upper levels (p < 0.014; 95% CI 0.143-0.805). There was a significant association between recurrent UTI and thoracic functional level. CONCLUSIONS: Macrocephaly at birth and higher levels of the defect have an impact on worse outcome and, therefore, are a challenge to the daily practice of pediatric neurosurgery.

Management opinions from different centers (Rio de Janeiro).

INTRODUCTION: The surgical treatment of Chiari type 1 (CM1) malformation is controversial and depends largely on the preference of the surgeon. The evolution of neuroimaging resulted in an increased number of asymptomatic patients incidentally diagnosed. PURPOSE: To study retrospectively a population of 24 symptomatic patients with CM1 operated between 1999 and 2017 in which intraoperative ultrasonography (IOUS)-assisted posterior fossa-C1 decompression was used to decide whether the dura mater should be opened (CVD+) or not (CVD). RESULTS: Most of the patients complained of headache or neck pain, 15 had hydrosyringomyelia and 14 had some spinal cord involvement. Patients were categorized in improved, unchanged, or worse according the preoperative signs and symptoms. Overall, 19 patients improved, 3 deteriorated, and 2 remained unchanged. Among these, 4 out 5 had syringohydromyelia. CONCLUSIONS: IOUS-assisted posterior fossa-C1 decompression is our preferred option to treat CM1. Children submitted to intradural procedures, initially or subsequently, had increased postoperative complications. CSF fistula or pseudomeningocele was the major cause of complication. The final result seems to correlate with the preoperative neurological status.

Pediatric neurocysticercosis.

BACKGROUND: Neurocysticercosis (NCC) is an infestation of the nervous system caused by encysted larvae of Taenia solium. NCC is an important acquired cause of epilepsy and other neurological manifestations especially in endemic areas. NCC in children has pleomorphic manifestations depending on the location, number, viability of the cysts, and host response. Even with advancing knowledge of the disease manifestations, many aspects related to diagnosis and treatment, particularly in children, still remain controversial and pose challenges to clinical practice. There is no gold standard test to diagnose NCC and the management recommendations are still emerging. This review provides an overview of diagnosis of NCC in children and its management with special focus on current challenges and future prospects. DISCUSSION: In developing countries, NCC is important not only because of its frequency but also because of high morbidity and mortality rates associated, especially in cases in which it progresses to increased intracranial pressure. Because of its pleomorphic presentation, NCC should be considered in the differential diagnosis of a number of neurological conditions. Treatment with cysticidal therapy leads to reduction in seizure frequency and a faster resolution of lesions. CONCLUSIONS: We have summarized the current approaches to diagnosis and treatment of NCC, recent advances in understanding the biology of NCC, and how one can take advantage of these new insights to formulate the next generation of clinical trials.

The congenital Zika virus infection: still a puzzle.

BACKGROUND: As a new disease, some features of the congenital Zika virus infection are not yet fully understood. The current Brazilian outbreak brought up an unexpected increase in the number of microcephaly cases as this strain is essentially neurotropic and associated with devastating effects on the developing central nervous system. OBJECT: This focus session aims to discuss the several issues related to the epidemiology, diagnosis, clinical features, and treatment of the congenital Zika virus infection.

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

OBJECTIVE:: To evaluate the mandatory folic acid fortification of flour on mortality rates after the hospital discharge of children born with myelomeningocele, the most affected age group and the most frequent cause of death. METHODS:: A retrospective study of 383 children born with myelomeningocele from January 1990 to December 2013 in a high-fetal-risk reference hospital. RESULTS:: A total of 39 patients died (10.1%),of which 23 (6%) died after discharge. Most children who died were younger than 12 months of age. The most frequent cause of death was infection of the central nervous system, followed by urinary tract sepsis and infections of the respiratory system. Symptomatic Chiari II malformation was the most frequent comorbidity factor. CONCLUSION:: Although there was no significant difference in infant mortality before and after folic acid fortification, there was a significant reduction in deaths after hospital discharge in babies born after implementation of mandatory folic acid fortification.

The mortality rate after hospital discharge in patients with myelomeningocele decreased after implementation of mandatory flour fortification with folic acid / Redução da taxa de mortalidade após a alta hospitalar em pacientes com mielomeningocele depois da implementação da fortificação obrigatória das farinhas com ácido fólico

ABSTRACT Objective: To evaluate the mandatory folic acid fortification of flour on mortality rates after the hospital discharge of children born with myelomeningocele, the most affected age group and the most frequent cause of death. Methods: A retrospective study of 383 children born with myelomeningocele from January 1990 to December 2013 in a high-fetal-risk reference hospital. Results: A total of 39 patients died (10.1%),of which 23 (6%) died after discharge. Most children who died were younger than 12 months of age. The most frequent cause of death was infection of the central nervous system, followed by urinary tract sepsis and infections of the respiratory system. Symptomatic Chiari II malformation was the most frequent comorbidity factor. Conclusion: Although there was no significant difference in infant mortality before and after folic acid fortification, there was a significant reduction in deaths after hospital discharge in babies born after implementation of mandatory folic acid fortification

RESUMO Objetivo: Avaliar a fortificação obrigatória de farinhas com ácido fólico nas taxas de mortalidade após a alta hospitalar de crianças nascidas com mielomeningocele, a faixa etária mais atingida e a causa mais freqüente de morte. Métodos: Estudo retrospectivo de 383 crianças nascidas com mielomeningocele de janeiro de 1990 a dezembro de 2013, em um hospital de referência de alto risco fetal. Resultados: Um total de 39 pacientes morreram (10,1%), dos quais 23 (6%) morreram após a alta. A maioria das crianças que morreram não alcançaram 12 meses de idade. A causa mais frequente de morte foi infecção do sistema nervoso central, seguido por sépsis, infecções do trato urinário e do sistema respiratório. A malformação de Chiari Tipo II foi a comorbidade mais freqüente. Conclusão: Apesar de não haver diferença significativa na frequência de mortalidade de recém-nascidos, antes e depois da fortificação obrigatória com ácido fólico, houve uma redução significativa no número de mortes após a alta hospitalar em bebês nascidos após a implementação da fortificação obrigatória das farinhas com ácido fólico.

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.

Laparoscopic assisted ventriculoperitoneal shunt revisions as an option for pediatric patients with previous intraabdominal complications.

UNLABELLED: Multiple shunt failure is a challenge in pediatric neurosurgery practice and one of the most feared complications of hydrocephalus. OBJECTIVE: To demonstrate that laparoscopic procedures for distal ventriculoperitoneal shunt failure may be an effective option for patients who underwent multiple revisions due to repetitive manipulation of the peritoneal cavity, abdominal pseudocyst, peritonitis or other situations leading to a "non reliable" peritoneum. METHOD: From March 2012 to February 2013, the authors reviewed retrospectively the charts of six patients born and followed up at our institution, which presented with previous intra-peritoneal complications and underwent ventriculoperitoneal shunt revision assisted by video laparoscopy. RESULTS: After a mean follow-up period of nine months, all patients are well and no further shunt failure was identified so far. CONCLUSION: Laparoscopy assisted shunt revision in children may be, in selected cases, an effective option for patients with multiple peritoneal complications due to ventriculo-peritoneal shunting.

Laparoscopic assisted ventriculoperitoneal shunt revisions as an option for pediatric patients with previous intraabdominal complications / Revisão de derivações ventrículo-peritoneais assistidas por videolaparoscopia como uma opção para pacientes pediátricos com complicações intra-abdominais

Multiple shunt failure is a challenge in pediatric neurosurgery practice and one of the most feared complications of hydrocephalus. Objective: To demonstrate that laparoscopic procedures for distal ventriculoperitoneal shunt failure may be an effective option for patients who underwent multiple revisions due to repetitive manipulation of the peritoneal cavity, abdominal pseudocyst, peritonitis or other situations leading to a “non reliable” peritoneum. Method: From March 2012 to February 2013, the authors reviewed retrospectively the charts of six patients born and followed up at our institution, which presented with previous intra-peritoneal complications and underwent ventriculoperitoneal shunt revision assisted by video laparoscopy. Results: After a mean follow-up period of nine months, all patients are well and no further shunt failure was identified so far. Conclusion: Laparoscopy assisted shunt revision in children may be, in selected cases, an effective option for patients with multiple peritoneal complications due to ventriculo-peritoneal shunting. .

Múltiplas disfunções de derivações ventrículo-peritoneais em pacientes crônicos são complicações temidas no tratamento das hidrocefalias e um desafio na prática neurocirúrgica. Objetivo: Demonstrar que a abordagem laparoscópica para o tratamento das obstruções distais das derivações ventrículo-peritoneais é uma opção eficaz em pacientes submetidos a múltiplas revisões, manipulação repetitiva da cavidade abdominal, pseudocisto abdominal, peritonite ou outras complicações indutoras de peritônio “não confiável”. Método: Os autores revisaram retrospectivamente, de março de 2012 a fevereiro de 2013, os prontuários de seis pacientes nascidos e acompanhados em um hospital pediátrico, que apresentaram múltiplas complicações intraperitoneais e tiveram a revisão de derivações ventrículo-peritoneais assistida por videolaparoscopia. Resultados: Todos os pacientes melhoraram clinicamente e nenhuma outra disfunção foi identificada após um período de acompanhamento médio de 9 meses. Conclusão: Revisão distal de derivações ventrículo-peritoneais assistida por videolaparoscopia em crianças é, em casos selecionados, uma opção eficaz para pacientes crônicos com história de múltiplas complicações peritoneais. .

Contribuiçäo ao estudo dos disrafismos espinais císticos cervicais e torácicos altos com proposta de classificaçäo / Contribuition to the study of the cervical and upper thoracic cystic spinal dysraphisms with proposal to classification

Objetivo: Apresentar uma casuística de portadores de disrafismos espinhais císticos cervicais e torácicos altos, discutir seus diversos aspectos, em especial as diferenças com as mielomeningoceles; discutir as denominações utilizadas e propor uma classificação baseada no conteúdo do cisto Métodos: Estudo retrospectivo de 16 casos operados no Instituto Fernandes Figueira (M.S.-Fiocruz, Rio de Janeiro), Escola Paulista de Medicina (UNIFESP) e Hospital das Clínicas (USP). Resultados: Todos os pacientes foram operados e o intervalo entre o nascimento e a intervenção cirúrgica variou entre 6h e nove meses. O achado mais freqüente, em treze casos, foi uma haste de tecido, mais ou menos vascularizado, que conectava a superfície posterior da medula ao revestimento do saco. Nos três casos restantes, identificaram-se mielocistoceles. Uma paciente veio a falecer. Os demais não apresentaram intercorrências pós-operatórias. Seis pacientes (37,5 por cento) desenvolveram hidrocefalia que necessitasse tratamento cirúrgico e quatro eram portadores da malformação de Chiari do tipo li. Seis pacientes tinham hidromielia. Quinze pacientes estão sendo regularmente acompanhados. Dois foram reoperados, um deles devido a ancoramento medular por remanescente da haste. Os achados histopatológicos foram de três tipos: hastes neurogliais, hastes fibrovasculares e mielocistoceles. Conclusões: Disrafismos espinhais císticos cervicais e torácicos são raros diferem das mielomeningoceles em suas características clínicas e estruturais. As denominações a eles atribuídas são, na maioria dos casos, inadequadas, de modo que o autor propõe chama-los, genericamente, Disrafismos Espinais Císticos Cervicais e Torácicos Altos (DECCT). O autor propõe uma classificação, de acordo com os achados no interior do cisto: o Tipo I incluiria os disrafismos com haste; o Tipo li, as mielocistoceles e o Tipo III, as meningoceles verdadeiras, desprovidas de qualquer estrutura no interior do cisto

Acompanhamento ambulatorial de pacientes com mielomeningocele em um hospital pediátrico / Ambulatory follow-up of patients with myelomeningocele in a pediatric hospital

A mielomeningocele constitui a mais freqüente malformaçäo congênita do sistema nervosos e, a despeito de sua complexidade e acometimento de múltiplos órgäos, é compatível a sobrevida prolongada. Por esses motivos, o acompanhamento de pacientes com essa malformaçäo assume vital importância no que dis respeito à qualidade de sua sobrevida. Com o objetivo de avaliar a qualidade do acompanhamento de portadores de spina bifida cística tratados em um hospital pediátrico, 54 pacientes foram examinados e seus familiares entrevistados. A inexistência de um centro multidisciplinar de tratamento de portadores de defeito de fechamento do tubo neural na cidade do Rio de Janeiro obrigou os pacientes a se deslocarem para locais geograficamente distantes enter si em busca de tratamento complementar em várias especialidades correlatas, com reflexos negativos na qualidade de sobrevida. Desta maneira, apenas uma quarta parte dos pacientes era capaz de se locomover e a chamada deambulaçäo comunitária era praticamente nenhuma. Apenas 2/3 se submetiam regularmente a fisioterapia e apenas a metade era regularmente acompanhada por ortopedistas. Quase 50 por cento dos pacientes näo foram orientados a procurar assistência urológica e 75 por cento apresentavam incontinência urinária. A incidência de infecçöes urinárias de repetiçäo foi 72,2 por cento. As complicaçöes e intercorrências neurocirúrgicas propriamente ditas tiveram incidências comparáveis às observadas na literatura. Concluímos que a qualidade de sobrevida dos pacientes estudados é significativamente afetada por fatores sócio-econômicos e pela ausência de centros multidisciplinares de tratamento

Empiema espinhal subdural: relato de um acso / Spinal subdural empyema: a case report

A raridade do empiema subdural pode ser verificada pela escassez de casos descritos na literatura. Os autores apresentam mais um caso, mostrando as dificuldades diagnósticas principalmente quando näo há aparente porta de entrada. Enfatizam, nestas circunstâncias, a importância de certos sinais clínicos, o valor da punçäo lombar e da imagem por ressonância nuclear magnética na elucidaçäo diagnóstica. Este último exame näo foi mencionado anteriormente na literatura consultada sobre o assunto. O tratamento cirúrgico, associado à antibioticoterapia, mostrou-se bastante eficaz, principalmente se realizado precocemente