Posterior fossa choroidplexus papilloma in the pediatric population: case series and literature review / Papiloma de plexo coroide na fossa posterior na população pediátrica: série de casos e revisão da literatura

Abstract Choroid plexus papillomas (CPPs) are rare benign neoplasms which are particularly uncommon in the posterior fossa in children. We herein present a case series of five patients treated at a tertiary care hospital. A comprehensive literature review was also carried out. The patients treated at the tertiary care hospital were aged between 4 and 16 years. Gross total resection (GTR) was initially achieved in two patients. All patients showed clinical improvement. Moreover, 27 articles published between 1975 and 2021 were selected for the literature review, totaling 46 patients; with the 5 patients previously described, the total sample was composed of 51 cases, With a mean age was 8.2 years. The lesions were located either in the fourth ventricle (65.3%) or the cerebellopontine angle (34.7%). Hydrocephalus was present preoperatively in 66.7% of the patients, and a permanent shunt was required in 31.6% of the cases. The GTR procedure was feasible in 64.5%, and 93.8% showed clinical improvement. For CPPs, GTR is the gold standard treatment and should be attempted whenever feasible, especially because the role of the adjuvant treatment remains controversial. Neuromonitoring is a valuable tool to achieve maximal safe resection. Hydrocephalus is common and must be recognized and promptly treated. Most patients will need a permanent shunt. Though there is still controversy on its efficacy, endoscopic third ventriculostomy is a safe procedure, and was the authors' first choice to treat hydrocephalus.

Resumo Os papilomas do plexo coroide (PPCs) são neoplasias benignas raras e, na população pediátrica, são particularmente incomuns na fossa posterior. Apresentamos uma série de casos de cinco pacientes atendidos em um hospital terciário. Além disso, foi realizada uma ampla revisão da literatura. Os pacientes atendidos no hospital terciário tinham entre 4 e 16 anos. Ressecção macroscópica total (RMT) foi inicialmente realizada em dois pacientes. Todos os pacientes apresentaram melhora clínica. Além disso, 27 artigos publicados entre 1975 e 2021 foram selecionados para a revisão da literatura, totalizando 46 pacientes. Somados à série de casos atuais, encontramos 51 pacientes, com média de idade de 8,2 anos. As lesões localizavam-se no quarto ventrículo (65,3%) ou no ângulo pontocerebelar (34,7%). Hidrocefalia estava presente no pré-operatório em 66,7% dos pacientes, e derivação ventricular permanente foi necessária em 31,6% dos casos. A RMT foi possível em 64,5%, e 93,8% tiveram melhora clínica. Para os CPPs, a RMT é o tratamento padrão-ouro e deve ser tentado sempre que possível, especialmente porque ainda existem controvérsias quanto ao papel do tratamento adjuvante. A neuromonitorização é uma ferramenta importante para se atingir a máxima ressecção segura. A hidrocefalia é comumente vista nesses pacientes e deve ser identificada e tratada. A maioria dos pacientes irá precisar de uma derivação permanente. Apesar de persistirem controvérsias sobre sua eficácia, a terceiro-ventriculostomia endoscópica foi a primeira escolha para tratar a hidrocefalia na experiência dos autores e é uma opção segura.

A Systematic Review of Benefits and Risks of Fetal Surgery for Congenital Cardiac Defects Such as Pulmonary Valve Stenosis and Critical Aortic Stenosis

ABSTRACT Introduction: Congenital heart diseases (CHDs) constitute the most prevalent congenital pathology, and they are a consequence of structural and functional abnormalities during fetal development. The etiology of CHD involves the interaction of genetic and environmental factors. Fetal cardiac surgery aims at preventing natural pathways of CHD in utero, mitigating progression to more complex abnormalities. The goal of this review was to demonstrate the benefits and risks of fetal interventions in the two most prevalent CHDs, pulmonary stenosis and pulmonary atresia with an intact ventricular septum, but also critical aortic stenosis and hypoplastic left heart syndrome. Methods: Original and relevant articles were selected by meta-aggregation to perform a qualitative analysis of fetal cardiac interventions for pulmonary stenosis and critical aortic stenosis. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument (or JBI-QARI) was used for data quality appraisal. Results: Of 61 potential articles, 13 were selected, and nine were finally included. Discussion: The present review demonstrated that fetal cardiac surgery increases right ventricular growth and hemodynamic flow in pulmonary stenosis, whereas in critical aortic stenosis it enables growth of the left ventricle and increases left ventricular pressure. However, it has a high complication rate, along with considerable morbidity and mortality. Conclusion: The benefits of fetal cardiac surgery for pulmonary stenosis and critical aortic stenosis are well-described in the literature; however, there is a significant risk of complications which can be reduced by the surgeon's technical expertise and well-structured hospital facilities.

Management of Chiari I Deformity in Children and Adolescents: A Report from the Consensus Taskforce of the Brazilian Society of Pediatric Neurosurgery

Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.

Neuroendoscopic surgery in children: an analysis of 200 consecutive procedures / Neuroendoscopia em crianças: uma análise de 200 procedimentos consecutivos

Neuroendoscopic surgery in children has particular features and is associated with different success rates (SR). The aim of this study was to identify putative factors that could influence the outcome in pediatric patients.

Clinical data of 177 patients under 18 years of age submitted to 200 consecutive neuroendoscopic procedures from January 2000 to January 2010 were reviewed. The overall success rate was 77%. Out of the patients with successful outcomes, 46% were under six months, 68% were between six months and one year of age, and 85% older than one year. Neuroendoscopic techniques provide very good results for a wide number of indications in children. Tumor-related cerebrospinal fluid (CSF) circulation problems and aqueductal stenosis seem to be particularly well suited to neuroendoscopic treatment regardless of the patient's age. Patients' age and etiology of hydrocephalus were associated with a different outcome. In all cases, surgical experience is extremely important to reduce complications. .

A cirurgia neuroendoscópica em crianças apresenta particularidades e está associada a diferentes taxas de sucesso (TS). O objetivo deste estudo consistiu em identificar fatores que pudessem influir no resultado do tratamento em pacientes pediátricos. Dados clínicos de 177 pacientes com idade inferior a 18 anos submetidos a 200 procedimentos neuroendoscópicos consecutivos entre janeiro de 2000 e janeiro de 2010 foram revisados. A taxa de sucesso global foi de 77%. Os pacientes com idade inferior a seis meses apresentaram taxa de sucesso de 46%; pacientes entre seis meses e um ano de vida obtiveram êxito em 68% dos casos; dentre os maiores de um ano, 85% dos procedimentos foram bem-sucedidos. Técnicas neuroendoscópicas proporcionam muito bons resultados para uma grande variedade de indicações em crianças. Independentemente da faixa etária, o tratamento endoscópico apresenta-se particularmente adequado para problemas da circulação liquórica relacionados a tumores e à estenose aquedutal. A faixa etária dos pacientes e a etiologia da hidrocefalia estão associadas a diferentes resultados. Em todos os casos, experiência neurocirúrgica é extremamente importante para a redução das complicações. .

Traumatismos raquimedulares penetrantes em adolescentes / Traumatismos raquimedulares penetrantes en adolescentes / Penetrating spinal cord injuries in adolescents

OBJETIVO: analisar a conduta em uma série de adolescentes com traumatismos raquimedulares penetrantes (TRMp) e, confrontando com a literatura, estabelecer a melhor estratégia terapêutica para esses pacientes. MÉTODOS: avaliação retrospectiva de prontuários médicos de dez pacientes, nove do sexo masculino e um do feminino, com TRMp e menores de 18 anos, tratados à nível hospitalar. Analisou-se a anamnese, o exame neurológico e os exames de imagem. A conduta terapêutica variou entre cirurgia ou conservador. RESULTADOS: a média de idade foi de 16 anos (13 a 18 anos). A mediana de hospitalização inicial foi de 11 dias (4 a 180 dias). Quatro pacientes foram submetidos à laminectomia, um ao desbridamento da lesão e os demais a tratamento conservador. Só um paciente, foi submetido à laminectomia, que migrou para Frankel E. Nenhum paciente apresentou infecção da ferida ou instabilidade tardia da coluna vertebral. CONCLUSÃO: as lesões medulares secundárias desempenham pouca importância no resultado neurológico, o que leva à definição clínica do quadro na admissão hospitalar da maioria dos casos. Houve uma nítida predominância de TRMp em adolescentes do sexo masculino. Não ocorreu diferença significativa entre as condutas realizadas em relação ao resultado neurológico, à infecção e instabilidade. O tratamento deve ser individualizado, não negligenciando o tratamento clínico e considerando a cirurgia apenas em casos específicos.

OBJECTIVE: to analyze the management of a series of adolescents with penetrating spinal cord injuries (pSCI) and comparing to literature, provide the best therapeutic strategy to these patients. METHODS: retrospective evaluation of medical reports of ten consecutive cases of pSCI, nine from the male sex and one from the female. All the patients were under 18 years old. The clinical history, neurologic evaluation and the images exams were analyzed. The therapeutic management varied between surgery and conservative. RESULTS: the average age was 16 years old (form 13 to 18 years). The median time spent at the first hospitalization was 11 days (from 4 to 180 days). Four patients were submitted to laminectomy, one to wound debridement and five to conservative treatment. Only one patient submitted to laminectomy had neurological improvement. No patients presented in this series showed, in the follow-up, wound infection or spinal instability. CONCLUSION: secondary spinal cord injuries are less relevant to the neurological status, which means that the sequelae are established at hospital admission in most of the cases. There was a clear predominance of pSCI in male adolescents. There was no significant difference between the cases that were managed clinically or surgically, concerning the neurological outcome, infection and instability. The therapy must be individualized, the clinical management must not be neglected and surgery must be considered only in specific cases.

OBJETIVO: analizar la conducta en una serie de adolescentes con traumatismos raquimedulares penetrantes (TRMp) y confrontar con la literatura, además de establecer la mejor estrategia terapéutica para eses pacientes. MÉTODOS: evaluación retrospectiva de historias clínicas de diez pacientes, nueve masculinos y un femenino de TRMp menores de 18 años, tratados a nivel hospitalar. Se analizó la anamnesis, el examen neurológico y los exámenes de imagen. La conducta terapéutica varió entre cirugía o tratamiento conservador. RESULTADOS: el promedio de edad fue de 16 años (13 a 18 años). La media de hospitalización inicial fue de 11 días (4 a 180 días). Cuatro pacientes fueron sometidos al tratamiento conservador. Solo un paciente sometido a laminectomía migró para Frankel E. Ningún paciente presentó infección de la herida o inestabilidad tardía de la columna vertebral. CONCLUSIÓN: las lesiones medulares secundarias desempeñan poca importancia en el resultado neurológico, lo cual lleva a la definición clínica del cuadro en la admisión hospitalar de la mayoría de los casos. Hubo una nítida predominancia de TRMp en adolescentes del sexo masculino. No ocurrió diferencia significativa entre las conductas realizadas en relación al resultado neurológico, infección e inestabilidad. El tratamiento debe ser individualizado, sin negligencia en el tratamiento clínico y considerando la cirugía sólo para casos específicos.