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1.
Childs Nerv Syst ; 39(10): 2719-2728, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37462810

RESUMO

PURPOSE: Pediatric hydrocephalus is a common and challenging condition. To date, the ventriculoperitoneal shunt (VPS) is still the main lifesaving treatment option. Nonetheless, it remains imperfect and is associated with multiple short- and long-term complications. This paper is a reflective review of the current state of the VPS, our knowledge gaps, and the future state of shunts in neurosurgical practice. METHODS AND RESULTS: The authors' reflections are based on a review of shunts and shunt-related literature. CONCLUSION: Overall, there is still an urgent need for the neurosurgical community to actively improve current strategies for shunt failures and shunt-related morbidity. The authors emphasize the role of collaborative efforts amongst like-minded clinicians to establish pragmatic approaches to avoid shunt complications.


Assuntos
Derivações do Líquido Cefalorraquidiano , Hidrocefalia , Criança , Humanos , Derivações do Líquido Cefalorraquidiano/efeitos adversos , Derivação Ventriculoperitoneal/efeitos adversos , Hidrocefalia/etiologia , Próteses e Implantes/efeitos adversos
2.
Childs Nerv Syst ; 39(12): 3445-3455, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37284980

RESUMO

PURPOSE: Cerebrospinal fluid (CSF) shunt failures in children are devastating. The primary aims of this study are to, firstly, review our institutional series of ventriculoperitoneal shunt (VPS) insertions and identify factors associated with shunt failure. METHODS: This is a single-institution, retrospective study conducted over a 12-year period. All patients under 18 years old with VPS inserted were included. Variables of interest such as patient characteristics, hydrocephalus aetiology, shunt implant details, and outcomes were subjected to statistical analyses. RESULTS: A total of 214 VPS patients were selected for this study. The mean age at VPS insertion was 6 months with a mean follow-up duration of 44 months. The most common type of hydrocephalus was obstructive (n = 142, 66.4%), and the most frequent aetiology was tumour-related (n = 66, 30.8%). The 30-day shunt failure rate was 9.3%: 9 infections (4.2%), 7 occlusions (3.3%), and 4 others (1.9%). After multivariable analysis, only recent central nervous system (CNS) infection prior to VPS insertion remained significant (OR 15.4 (1.3-175), p = 0.028). CONCLUSION: This is the first, large-scale local study focused on the shunt failure in Singaporean children. Significant findings in our study demonstrate that recently treated CNS infection is a factor associated with 30-day shunt failure while the values of CSF constituents were not contributory.


Assuntos
Hidrocefalia , Derivação Ventriculoperitoneal , Criança , Humanos , Adolescente , Lactente , Derivação Ventriculoperitoneal/efeitos adversos , Estudos Retrospectivos , Singapura/epidemiologia , Hidrocefalia/cirurgia , Hidrocefalia/etiologia , Hospitais
3.
Childs Nerv Syst ; 38(12): 2437-2444, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36239781

RESUMO

PURPOSE: Abusive head trauma (AHT) is a major cause of morbidity and mortality in children. Studies on pediatric head injury observe that AHT patients often have a higher incidence of malignant cerebral oedema and, overall, worse prognosis. There are limited studies with a focus on the outcome of decompressive surgery in children with AHT. This is a study undertaken to review our institutional experience on the role of decompressive surgery in AHT patients and objectively assess its outcomes, in corroboration with current literature. METHODS: This is an ethics-approved, retrospective study. Inclusion criteria consist of all children with a diagnosis of AHT managed by the Neurosurgical Service, KK Women's and Children's Hospital. Demographical and clinical variables are incorporated in the statistical analyses. RESULTS: From 2011 to 2021, a total of 7 patients required decompressive surgery for AHT. Mean age of the cohort was 17.1 months (with the majority of patients being male (n = 5, 71.4%). During the follow-up period, there was 1 mortality (14.3%), 3 patients developed cerebral palsy (42.9%), and 3 patients had post-traumatic epilepsy (42.9%). With regards to functional outcome, 4 patients (57.1%) had a favorable KOSCHI score at 6 months follow-up. CONCLUSION: Decompressive surgery in children with AHT presents with its own unique challenges. We therein present our neurosurgical experience in decompressive surgery for this extremely vulnerable group of patients. Given the potential role of decompressive surgery in AHT, the development of an objective marker to select such patients who may benefit most from intervention should be the way forward.


Assuntos
Maus-Tratos Infantis , Traumatismos Craniocerebrais , Humanos , Criança , Masculino , Feminino , Lactente , Estudos Retrospectivos , Singapura/epidemiologia , Maus-Tratos Infantis/diagnóstico , Traumatismos Craniocerebrais/cirurgia , Traumatismos Craniocerebrais/epidemiologia , Hospitais
4.
Childs Nerv Syst ; 38(12): 2397-2407, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-35851614

RESUMO

PURPOSE: Abusive head trauma (AHT) is a serious problem in children. The aims of this study are to identify risk factors that correlate with outcomes for those requiring neurosurgical intervention for very young children with AHT, assessment of variables associated with outcomes, and corroboration of our results with literature. METHODS: This is an ethics-approved, retrospective study. Inclusion criteria consisted of patients aged 2 years old or less with a diagnosis of AHT managed by the Neurosurgical Service, KK Women's and Children's Hospital. Demographical and clinical variables are incorporated in the statistical analyses. Logistic regression was applied to statistically significant variables for the risk prediction model. RESULTS: From 2000 to 2020, 24 patients required surgery for AHT. Timepoint was set at 12 months post-diagnosis. Univariate analyses demonstrated that patients with mild TBI were likely to have a favourable GOS-E Peds (p = 0.01), whereas radiological presence of cerebral oedema (p < .001), development of scar epilepsy (p = 0.021), and progression to cerebral palsy (p = 0.001) were associated with unfavourable GOS-E Peds. CONCLUSION: This is the first study focused on neurosurgical outcomes for very young children with AHT in Singapore. We advocate multidisciplinary efforts to improve outcomes for this devastating condition.


Assuntos
Maus-Tratos Infantis , Traumatismos Craniocerebrais , Humanos , Criança , Feminino , Lactente , Pré-Escolar , Estudos Retrospectivos , Maus-Tratos Infantis/diagnóstico , Singapura/epidemiologia , Traumatismos Craniocerebrais/epidemiologia , Traumatismos Craniocerebrais/cirurgia , Hospitais
5.
Acta Neurochir (Wien) ; 164(4): 1157-1160, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35015155

RESUMO

BACKGROUND: Congenital complex spinal lipomas (CSL) are challenging lesions to treat. Cerebrospinal fluid (CSF) leaks are feared complications due to the risk of infection, and subsequent scarring that may promote retethering. Much has been written in the literature on the surgical technique of CSL resection with less emphasis placed on the prevention and management of CSF leak. METHOD: The authors describe the nuances in the prevention and management of CSF leaks in the context of CSL, including the operative approach, resection, closure and recommended postoperative care. CONCLUSION: CSF leaks are complications that can be minimized with deliberate steps and meticulous surgical technique.


Assuntos
Dura-Máter , Lipoma , Vazamento de Líquido Cefalorraquidiano/etiologia , Dura-Máter/cirurgia , Humanos , Lipoma/complicações , Lipoma/cirurgia , Procedimentos Neurocirúrgicos/métodos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos
6.
Br J Neurosurg ; : 1-7, 2022 Dec 23.
Artigo em Inglês | MEDLINE | ID: mdl-36564943

RESUMO

PURPOSE: Tethered cord due to focal nondisjunction of primary neuralisation (FNPN) is a rare form of spinal dysraphism. We present our institutional experience in managing children diagnosed with FNPN. MATERIALS AND METHODS: This is a single institution, retrospective study approved by the hospital ethics board. Patients below 18 years of age diagnosed with CDS, LDM or their mixed lesions, and subsequently underwent intervention by the Neurosurgical Service, KK Women's and Children's Hospital, are included. RESULTS: From 2001 to 2021, 16 FNPN patients (50% males) were recruited. Eight of them had CDS (50.0%), seven had LDM (43.8%), and one patient had a mixed CDS and LDM lesion (6.2%). The average duration of follow up was 5.7 years and the mean age of surgery was 6 months old. Thirteen patients underwent prophylactic intent surgery (81.2%) and three had therapeutic intent surgery (18.8%). All patients did not have new neurological deficit or required repeat surgery for cord retethering. We observed that detethering surgery performed at or less than three months old was associated with having a wound infection (p = .022). CONCLUSIONS: Our study reports that early recognition and timely intervention are mainstays of management for FNPN. We advocate a multi-disciplinary approach for good outcomes.

7.
J Stroke Cerebrovasc Dis ; 31(3): 106283, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-34998042

RESUMO

OBJECTIVES: Paediatric brain arteriovenous malformation (bAVM) is a rare and distinct clinical entity. There is a growing body of literature that support the success of multimodality approaches for this difficult condition. The authors aim to firstly, describe our institutional experience with a consecutive series of patients and next, corroborate our results with current literature. MATERIAL AND METHODS: This is a single institution, retrospective study conducted over a 20-year period. Patients less than 19 years old with bAVM were included. Variables of interest included patient demographics, clinical presentation, neuroimaging features, bAVM characteristics and treatment modality. Functional outcomes were measured with modified Rankin scale (mRS). RESULTS: There were 58 paediatric bAVMs, presenting at a mean age of 8.7 ± 4.2 years, and followed up for a mean duration of 7.7 years. Thirty-six patients (62.1%) underwent microsurgical resection, 10 patients had stereotactic radiosurgery (17.2%) and 2 patients had endovascular treatment (3.4%). 50 patients (86.2%) had a favourable outcome at 1-year follow up. Microsurgical resection and SRS had similar obliteration rates (resection 83.3%; SRS 80.0%) and recurrence (resection 10.0%; SRS 12.5%). There were 6 cases of bAVM recurrence (12.8%). This subgroup was noted to be less than 7.5 years old at presentation (OR 15.0, 95% CI 1.56 - 144), and less likely to present with bAVM rupture (OR 0.11, 95% CI 0.01 - 0.96). CONCLUSION: This study describes our experience in managing paediatric bAVM, whereby monomodal therapy can still be effective. Of note, we also demonstrate the role of extended surveillance to detect recurrence.


Assuntos
Malformações Arteriovenosas Intracranianas , Criança , Pré-Escolar , Hospitais Pediátricos , Humanos , Malformações Arteriovenosas Intracranianas/terapia , Estudos Retrospectivos , Singapura , Resultado do Tratamento
8.
BMC Cancer ; 20(1): 554, 2020 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-32539808

RESUMO

BACKGROUND: Metastatic medulloblastoma (MB) portends a poor prognosis. Amongst the 4 molecular subtypes, Group 3 and Group 4 patients have a higher incidence of metastatic disease, especially involving the neuroaxis. At present, mechanisms underlying MB metastasis remain elusive. Separately, inflammation has been implicated as a key player in tumour development and metastasis. Cytokines and their inflammation-related partners have been demonstrated to act on autocrine and, or paracrine pathways within the tumour microenvironment for various cancers. In this study, the authors explore the involvement of cerebrospinal fluid (CSF) cytokines in Group 3 and 4 MB patients with disseminated disease. METHODS: This is an ethics approved, retrospective study of prospectively collected data based at a single institution. Patient clinicpathological data and corresponding bio-materials are collected after informed consent. All CSF samples are interrogated using a proteomic array. Resultant expression data of selected cytokines are correlated with each individual's clinical information. Statistical analysis is employed to determine the significance of the expression of CSF cytokines in Group 3 and 4 patients with metastatic MB versus non-metastatic MB. RESULTS: A total of 10 patients are recruited for this study. Median age of the cohort is 6.6 years old. Based on Nanostring gene expression analysis, 5 patients have Group 3 as their molecular subtype and the remaining 5 are Group 4. There are 2 non-metastatic versus 3 metastatic patients within each molecular subtype. Proteomic CSF analysis of all patients for both subtypes show higher expression of CCL2 in the metastatic group versus the non-metastatic group. Within the Group 3 subtype, the MYC-amplified Group 3 MB patients with existing and delayed metastases express higher levels of CXCL1, IL6 and IL8 in their CSF specimens at initial presentation. Furthermore, a longitudinal study of metastatic Group 3 MB observes that selected cytokines are differentially expressed in MYC-amplified metastatic Group 3 MB, in comparison to the non-MYC amplified metastatic Group 3 MB patient. CONCLUSION: This study demonstrates higher expression of selected CSF cytokines, in particular CCL2, in metastatic Group 3 and 4 MB patients. Although our results are preliminary, they establish a proof-of-concept basis for continued work in a larger cohort of patients affected by this devastating disease.


Assuntos
Biomarcadores Tumorais/líquido cefalorraquidiano , Neoplasias Cerebelares/patologia , Citocinas/líquido cefalorraquidiano , Meduloblastoma/diagnóstico , Biomarcadores Tumorais/imunologia , Encéfalo/diagnóstico por imagem , Neoplasias Cerebelares/líquido cefalorraquidiano , Neoplasias Cerebelares/imunologia , Neoplasias Cerebelares/cirurgia , Criança , Pré-Escolar , Citocinas/imunologia , Feminino , Seguimentos , Perfilação da Expressão Gênica , Humanos , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Meduloblastoma/líquido cefalorraquidiano , Meduloblastoma/secundário , Meduloblastoma/cirurgia , Estudo de Prova de Conceito , Estudos Prospectivos , Proteômica , Estudos Retrospectivos
9.
BMC Neurol ; 20(1): 415, 2020 Nov 14.
Artigo em Inglês | MEDLINE | ID: mdl-33187494

RESUMO

BACKGROUND: Germinomas (IG) account for up to 50% of all intracranial germ cell tumours. These tumours are reputed to be more prevalent in Oriental populations in comparison to Western cohorts. Biological characteristics of IG in other ethnic groups are unknown. Singapore is a multi-ethnic country with diverse cultures. Owing to inter-racial heterogeneity, the authors hypothesize there are molecular differences between paediatric IG patients in our local population. The aims of this study are exploratory: firstly, to identify molecular characteristics in this tumour type and circulating CSF unique to different racial cohorts; and next, to corroborate our findings with published literature. METHODS: This is a single-institution, retrospective study of prospectively collected data. Inclusion criteria encompass all paediatric patients with histologically confirmed IG. Excess CSF and brain tumour tissues are collected for molecular analysis. Tumour tissues are subjected to a next generation sequencing (NGS) targeted panel for KIT and PDGRA. All CSF samples are profiled via a high-throughput miRNA multiplexed workflow. Results are then corroborated with existing literature and public databases. RESULTS: In our cohort of 14 patients, there are KIT exon variants in the tumour tissues and CSF miRNAs corroborative with published studies. Separately, there are also KIT exon variants and miRNAs not previously highlighted in IG. A subgroup analysis demonstrates differential CSF miRNAs between Chinese and Malay IG patients. CONCLUSION: This is the first in-depth molecular study of a mixed ethnic population of paediatric IGs from a Southeast Asian cohort. Validation studies are required to assess the relevance of novel findings in our study.


Assuntos
Neoplasias Encefálicas , Germinoma , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/metabolismo , Criança , Germinoma/genética , Germinoma/metabolismo , Humanos , MicroRNAs/líquido cefalorraquidiano , MicroRNAs/genética , MicroRNAs/metabolismo , Proteínas Proto-Oncogênicas c-kit/genética , Estudos Retrospectivos , Singapura
10.
BMC Cancer ; 19(1): 182, 2019 Feb 28.
Artigo em Inglês | MEDLINE | ID: mdl-30819134

RESUMO

BACKGROUND: Primary paediatric epidural sarcomas are extremely rare. Overall, there remains a paucity of knowledge in paediatric epidural sarcomas owing to the infrequent number of cases. The Archer FusionPlex Sarcoma Kit (ArcherDX, Inc) is a next-generation sequencing assay that has been reported to be a useful technique to detect recurrent fusion in sarcomas. We report the molecular exploration of 3 primary paediatric epidural sarcomas-one in the cranium (mesenchymal chondrosarcoma) and 2 in the spine (mesenchymal chondrosarcoma and Ewing sarcoma respectively). CASE PRESENTATION: This is a study approved by the hospital ethics board. Clinico-pathological information from 3 consenting patients with primary epidural sarcomas was collected. These selected tumours are interrogated via Archer FusionPlex Sarcoma Kit (ArcherDX, Inc) for genomic aberrations. Results were validated with RT-PCR and Sanger sequencing. All findings are corroborated and discussed in concordance with current literature. Our findings show 2 variants of the HEY1-NCOA2 gene fusion: HEY1 (exon 4)-NCOA2 (exon 13) and HEY1 (exon 4)-NCOA2 (exon 14), in both mesenchymal chondrosarcoma patients. Next, the Ewing sarcoma tumour is found to have EWSR1 (exon 10)-FLI1 (exon 8) translocation based on NGS. This result is not detected via conventional fluorescence in situ testing. CONCLUSIONS: This is a molecularly-centered study based on 3 unique primary paediatric epidural sarcomas. Our findings to add to the growing body of literature for these exceptionally rare and malignant neoplasms. The authors advocate global collaborative efforts and in-depth studies for targeted therapy to benefit affected children.


Assuntos
Neoplasias Epidurais/diagnóstico , Sarcoma/diagnóstico , Fatores Etários , Biomarcadores Tumorais , Biópsia , Criança , Condrossarcoma Mesenquimal/diagnóstico , Condrossarcoma Mesenquimal/genética , Análise Mutacional de DNA , Neoplasias Epidurais/genética , Feminino , Humanos , Imageamento por Ressonância Magnética , Sarcoma/genética , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/genética , Avaliação de Sintomas
11.
Childs Nerv Syst ; 35(10): 1777-1783, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31175390

RESUMO

PURPOSE: The natural history of Chiari I malformation (C1M) in the paediatric population is poorly understood. There are conflicting reports with regards to surgical indications, operative techniques and peri-operative prognostic indicators. In this paper, we report our institutional experience in the management of paediatric C1M. METHODS: The workflow process which includes preferred imaging modalities, surgical techniques and indications for intervention is discussed. In particular, we describe one of our current projects-an in-house designed Seow Operative Score (SOS) as a feasibility scoring system for neurosurgical intervention in our local cohort of paediatric C1M patients. RESULTS: In our series, we have 2 groups: 10 non-operated patients versus 19 operated patients. In the non-operated group, the majority of patients had a SOS of 0 to 1. One patient had a score of 2.5 and was kept under close surveillance. Follow-up imaging demonstrated resolution of the cerebellar herniation and intraspinal syrinx. In the operated group, 17 patients had a SOS of 3 or more. Two patients had a SOS of 2. For these 2, 1 developed progressive symptoms, and the other had an extensive cervico-thoracic syrinx. Decision was made for surgery after a period of surveillance. CONCLUSIONS: In this paper, we report our institutional experience in managing paediatric C1M and, at the same time, highlight salient points of our practices. Meanwhile, we advocate collective global efforts and in-depth research for better disease understanding of this challenging condition.


Assuntos
Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/cirurgia , Tomada de Decisão Clínica/métodos , Procedimentos Neurocirúrgicos/métodos , Procedimentos Neurocirúrgicos/normas , Índice de Gravidade de Doença , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Estudos de Viabilidade , Feminino , Humanos , Lactente , Masculino
12.
Childs Nerv Syst ; 35(6): 1021-1027, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-30937530

RESUMO

PURPOSE: Diffuse leptomeningeal glioneuronal tumour (DLGNT) is an extremely rare tumour involving the neuroaxis. At present, its exact pathogenesis and associated factors remain incompletely characterised. Recent molecular investigations in a small cohort have offered some insights into this disease. However, the role of germline findings has not yet been fully explored in affected patients. The authors present a case of DLGNT, focusing on the clinical and molecular features with reference to current disease knowledge. METHODS: A 4-year-old male presented with raised intracranial pressure symptoms secondary to extensive leptomeningeal disease of the brain and spine. Preliminary impression was that of an inflammatory lesion. RESULTS: A lumbar biopsy of the lesion confirmed DLGNT on routine diagnostic examination. Further molecular analysis of his tumour and blood demonstrated a previously unreported TP53 exon 5 (c.147V > I) germline variant. Based on the latest DLGNT molecular subtyping classification, his tumour falls into the group with better clinical outcome. However, his germline findings may add an extra layer of uncertainty to his overall prognosis. CONCLUSION: Given that much remains unknown in many rare paediatric tumours at this stage, isolated findings found in an individual may be of significance. Supplementary genetic information, together with tumour molecular analysis, add to our current understanding of this uncommon disease. This case highlights the benefit of combined clinical and molecular efforts, including germline testing, especially for children affected by such challenging neoplasms.


Assuntos
Neoplasias do Sistema Nervoso Central/genética , Neoplasias Meníngeas/genética , Neoplasias Neuroepiteliomatosas/genética , Proteína Supressora de Tumor p53/genética , Antineoplásicos/uso terapêutico , Neoplasias do Sistema Nervoso Central/patologia , Neoplasias do Sistema Nervoso Central/terapia , Pré-Escolar , Humanos , Masculino , Neoplasias Meníngeas/patologia , Neoplasias Meníngeas/terapia , Neoplasias Neuroepiteliomatosas/patologia , Neoplasias Neuroepiteliomatosas/terapia , Procedimentos Neurocirúrgicos/métodos , Polimorfismo de Nucleotídeo Único
14.
Childs Nerv Syst ; 34(10): 1871-1880, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-29968000

RESUMO

INTRODUCTION: Brain abscesses affect all age groups and are not peculiar to a particular country, race, or geographical location. It is a disease that, in the past, carried a high morbidity and mortality. With improvements in medical technology and expertise, outcomes have improved tremendously. The causative organisms vary vastly and have evolved with time. Treatment of brain abscesses is primarily with antimicrobial therapy but surgery plays a vital role in achieving better outcomes. CONTENT: In this article, we review the literature to find out how the epidemiology of this disease has changed through the years and re-visit the basic pathological process of abscess evolution and highlight the new research in the biochemical pathways that initiate and regulate this process. We also highlight how magnetic resonance imaging and its various modalities have improved diagnostic accuracy. Finally, we discuss the pros and cons of traditional open surgery versus newer minimally invasive methods.


Assuntos
Abscesso Encefálico/diagnóstico por imagem , Abscesso Encefálico/epidemiologia , Abscesso Encefálico/patologia , Humanos
15.
Childs Nerv Syst ; 33(3): 457-465, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28190209

RESUMO

PURPOSE: Posterior fossa syndrome (PFS) is a serious complication faced by neurosurgeons and their patients, especially in paediatric medulloblastoma patients. The uncertain aetiology of PFS, myriad of cited risk factors and therapeutic challenges make this phenomenon an elusive entity. The primary objective of this study was to identify associative factors related to the development of PFS in medulloblastoma patient post-tumour resection. METHODS: This is a retrospective study based at a single institution. Patient data and all related information were collected from the hospital records, in accordance to a list of possible risk factors associated with PFS. These included pre-operative tumour volume, hydrocephalus, age, gender, extent of resection, metastasis, ventriculoperitoneal shunt insertion, post-operative meningitis and radiological changes in MRI. Additional variables included molecular and histological subtypes of each patient's medulloblastoma tumour. Statistical analysis was employed to determine evidence of each variable's significance in PFS permanence. RESULTS: A total of 19 patients with appropriately complete data was identified. Initial univariate analysis did not show any statistical significance. However, multivariate analysis for MRI-specific changes reported bilateral DWI restricted diffusion changes involving both right and left sides of the surgical cavity was of statistical significance for PFS permanence. CONCLUSION: The authors performed a clinical study that evaluated possible risk factors for permanent PFS in paediatric medulloblastoma patients. Analysis of collated results found that post-operative DWI restriction in bilateral regions within the surgical cavity demonstrated statistical significance as a predictor of PFS permanence-a novel finding in the current literature.


Assuntos
Neoplasias Cerebelares/cirurgia , Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/patologia , Imagem de Difusão por Ressonância Magnética , Meduloblastoma/cirurgia , Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Bases de Dados Bibliográficas/estatística & dados numéricos , Feminino , Escala de Coma de Glasgow , Humanos , Masculino , Análise Multivariada , Período Pós-Operatório , Estudos Retrospectivos
16.
Lancet Oncol ; 17(4): 484-495, 2016 04.
Artigo em Inglês | MEDLINE | ID: mdl-26976201

RESUMO

BACKGROUND: Patients with incomplete surgical resection of medulloblastoma are controversially regarded as having a marker of high-risk disease, which leads to patients undergoing aggressive surgical resections, so-called second-look surgeries, and intensified chemoradiotherapy. All previous studies assessing the clinical importance of extent of resection have not accounted for molecular subgroup. We analysed the prognostic value of extent of resection in a subgroup-specific manner. METHODS: We retrospectively identified patients who had a histological diagnosis of medulloblastoma and complete data about extent of resection and survival from centres participating in the Medulloblastoma Advanced Genomics International Consortium. We collected from resections done between April, 1997, and February, 2013, at 35 international institutions. We established medulloblastoma subgroup affiliation by gene expression profiling on frozen or formalin-fixed paraffin-embedded tissues. We classified extent of resection on the basis of postoperative imaging as gross total resection (no residual tumour), near-total resection (<1·5 cm(2) tumour remaining), or sub-total resection (≥1·5 cm(2) tumour remaining). We did multivariable analyses of overall survival and progression-free survival using the variables molecular subgroup (WNT, SHH, group 4, and group 3), age (<3 vs ≥3 years old), metastatic status (metastases vs no metastases), geographical location of therapy (North America/Australia vs rest of the world), receipt of chemotherapy (yes vs no) and receipt of craniospinal irradiation (<30 Gy or >30 Gy vs no craniospinal irradiation). The primary analysis outcome was the effect of extent of resection by molecular subgroup and the effects of other clinical variables on overall and progression-free survival. FINDINGS: We included 787 patients with medulloblastoma (86 with WNT tumours, 242 with SHH tumours, 163 with group 3 tumours, and 296 with group 4 tumours) in our multivariable Cox models of progression-free and overall survival. We found that the prognostic benefit of increased extent of resection for patients with medulloblastoma is attenuated after molecular subgroup affiliation is taken into account. We identified a progression-free survival benefit for gross total resection over sub-total resection (hazard ratio [HR] 1·45, 95% CI 1·07-1·96, p=0·16) but no overall survival benefit (HR 1·23, 0·87-1·72, p=0·24). We saw no progression-free survival or overall survival benefit for gross total resection compared with near-total resection (HR 1·05, 0·71-1·53, p=0·8158 for progression-free survival and HR 1·14, 0·75-1·72, p=0·55 for overall survival). No significant survival benefit existed for greater extent of resection for patients with WNT, SHH, or group 3 tumours (HR 1·03, 0·67-1·58, p=0·89 for sub-total resection vs gross total resection). For patients with group 4 tumours, gross total resection conferred a benefit to progression-free survival compared with sub-total resection (HR 1·97, 1·22-3·17, p=0·0056), especially for those with metastatic disease (HR 2·22, 1·00-4·93, p=0·050). However, gross total resection had no effect on overall survival compared with sub-total resection in patients with group 4 tumours (HR 1·67, 0·93-2·99, p=0·084). INTERPRETATION: The prognostic benefit of increased extent of resection for patients with medulloblastoma is attenuated after molecular subgroup affiliation is taken into account. Although maximum safe surgical resection should remain the standard of care, surgical removal of small residual portions of medulloblastoma is not recommended when the likelihood of neurological morbidity is high because there is no definitive benefit to gross total resection compared with near-total resection. FUNDING: Canadian Cancer Society Research Institute, Terry Fox Research Institute, Canadian Institutes of Health Research, National Institutes of Health, Pediatric Brain Tumor Foundation, and the Garron Family Chair in Childhood Cancer Research.


Assuntos
Neoplasias Encefálicas/classificação , Neoplasias Encefálicas/cirurgia , Meduloblastoma/classificação , Meduloblastoma/cirurgia , Prognóstico , Adulto , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patologia , Canadá , Criança , Pré-Escolar , Terapia Combinada , Progressão da Doença , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Meduloblastoma/genética , Meduloblastoma/patologia , Estudos Retrospectivos
17.
Neurosurg Focus ; 41(5): E7, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27798980

RESUMO

OBJECTIVE The introduction of ventriculoperitoneal shunts changed the way hydrocephalus was treated. Whereas much is known about the causes of shunt failure in the first few years, there is a paucity of data in the literature regarding the cause of late shunt failures. The authors conducted a study to find out the different causes of late shunt failures in their institution. METHODS A 10-year retrospective study of all the patients who were treated in the authors' hospital between 2006 and 2015 was conducted. Late shunt failures included those in patients who had to undergo shunt revision more than 5 years after their initial shunt insertion. The patient's notes and scans were reviewed to obtain the age and sex of the patient, the time it took for the shunt to fail, the reason for failure, and the patient's follow-up. RESULTS Forty-six patients in the authors' institution experienced 48 late shunt failures in the last 10 years. Their ages ranged from 7 to 26 years (12.23 ± 4.459 years [mean ± SD]). The time it took for the shunts to fail was between 6 and 24 years (mean 10.25 ± 3.77 years). Reasons for failure resulting in shunt revision include shunt fracture in 24 patients (50%), shunt blockage in 14 patients (29.2%), tract fibrosis in 6 patients (12.5%), shunt dislodgement in 2 patients (4.2%), and shunt erosion in 2 patients (4.2%). Postoperative follow-up for the patients ranged from 6 to 138 months (mean 45.15 ± 33.26 months). CONCLUSIONS Late shunt failure is caused by the effects of aging on the shunt, and the complications are different from early shunt failure. A large proportion are complications associated with shunt calcification. The authors advocate a long follow-up for pediatric patients with shunts in situ to monitor them for various causes of late shunt failure.


Assuntos
Falha de Equipamento , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/epidemiologia , Centros de Atenção Terciária/tendências , Derivação Ventriculoperitoneal/tendências , Adolescente , Adulto , Criança , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Singapura/epidemiologia , Fatores de Tempo , Derivação Ventriculoperitoneal/efeitos adversos , Adulto Jovem
18.
Childs Nerv Syst ; 31(4): 609-14, 2015 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-25537110

RESUMO

PURPOSE: Cerebral arteriovenous malformation (AVM) is traditionally thought of as a congenital diagnosis. In recent years, there has been infrequent reports of this neurovascular condition presenting as de novo entities. METHODS: The authors report two cases of pediatric patients who present with de novo cerebral AVMs. In both cases, the patients had magnetic resonance imaging (MRI) of the brain done as part of the work-up for first-onset seizures. At that point in time, the scans were unremarkable. After a latent period of approximately 6 and 9 years respectively, a repeated MRI brain scan showed evidence of de novo AVM for each patient. RESULTS: Both patients did not have radiological evidence of cerebral AVM during their first presentation of seizures. However, a repeated MRI brain scan after a period of 6 and 9 years demonstrated new findings of cerebral AVM for each patient. CONCLUSIONS: Currently, the disease of cerebral de novo AVM remain as an ambiguous condition that is poorly understood. With the advances in molecular diagnostics, there are possibilities of exploring biochemical profiles for better understanding of the origin of cerebral AVMs. However, in the meantime, owing to the unpredictable nature of cerebral AVMs, clinicians should have increased awareness of this unique condition. This is especially important, as definitive treatment is available to prevent devastating neurological sequelae from cerebral AVM rupture.


Assuntos
Malformações Arteriovenosas Intracranianas/diagnóstico , Malformações Arteriovenosas Intracranianas/terapia , Criança , Eletroencefalografia , Feminino , Humanos , Lactente , Angiografia por Ressonância Magnética , Imageamento por Ressonância Magnética , Masculino
20.
Neurosurg Focus Video ; 10(1): V17, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38283817

RESUMO

Selective dorsal rhizotomy (SDR) is an established neurosurgical technique for children with spastic diplegia secondary to cerebral palsy. Meticulous intraoperative testing of individual nerve roots with electromyography in tandem with the on-site neurorehabilitation team is recommended for good clinical outcomes. The standard approach requires the neurosurgeons to spend extended time under the traditional operating microscope. In this video, the authors describe the use of a 3D exoscope system for SDR. Overall, the 3D exoscope improves ergonomics and reduces musculoskeletal fatigue for the operating neurosurgeons. Furthermore, it provides excellent visualization of important structures, allowing safe and efficient completion of the procedure. The video can be found here: https://stream.cadmore.media/r10.3171/2023.10.FOCVID23105.

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