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1.
Acta Neurochir (Wien) ; 166(1): 166, 2024 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-38565800

RESUMO

BACKGROUND: The health-related quality of life (HRQoL) and cognition are important indicators for the quality of survival in patients with high-grade glioma (HGG). However, data on long-term survivors and their caregivers are scarce. We aim to investigate the interaction between cognition and HRQoL in long-term survivors, their caregivers' evaluations, and the effect on caregiver strain and burden. METHODS: 21 long-term HGG (8 WHO grade III and 13 WHO grade IV) survivors (survival ≥ 5 years) and 15 caregivers were included. Cognition (verbal memory, attention, executive functioning, and language), HRQoL, anxiety and depression, caregiver strain, and caregiver burden were assessed with standardized measures. Questionnaires were completed by patients and/or their caregivers. RESULTS: Mean survival was 12 years (grade III) and 8 years (grade IV). Cognition was significantly impaired with a large individual variety. Patients' general HRQoL was not impaired but all functioning scales were deviant. Patient-proxy agreement was found in most HRQoL subscales. Three patients (14%) showed indications of anxiety or depression. One-third of the caregivers reported a high caregiver strain or a high burden. Test scores for attention, executive functioning, language, and/or verbal memory were correlated with perceived global health status, cognitive functioning, and/or communication deficits. Caregiver burden was not related to cognitive deficits. CONCLUSIONS: In long-term HGG survivors maintained HRQoL seems possible even when cognition is impaired in a large variety at the individual level. A tailored approach is therefore recommended to investigate the cognitive impairments and HRQoL in patients and the need for patient and caregiver support.


Assuntos
Glioma , Qualidade de Vida , Humanos , Qualidade de Vida/psicologia , Cuidadores/psicologia , Glioma/psicologia , Inquéritos e Questionários , Cognição , Sobreviventes/psicologia
2.
Spine Deform ; 12(2): 507-511, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38097876

RESUMO

Biallelic pathogenic variants of the RIPPLY2 gene have been recognized to cause a subtype of autosomal recessive spondylocostal dysostosis (SCDO6), characterized by predominant cervical spine malformation with minor or absent involvement of the ribs. To date, RIPPLY2 associated SCDO6 has been described in ten patients in five studies with accompanying clinical symptoms varying from transient and recurrent torticollis to flaccid quadriplegia. Here, we describe two additional patients in one family in which the c.A238T:p.Arg80* RIPPLY2 mutation in the homozygous state, was associated with severe malformation of the posterior elements of the cervical vertebral column. In both cases neurological symptoms occurred early in life due to spinal cord compromise. These two cases, in keeping with previous reports, highlight the early and progressive natural history of cervical deformity in this rare skeletal dysplasia and the need for close neurological and radiological surveillance. Surgical decision-making needs to carefully balance the need for early intervention to protect spinal cord function on one hand, with the problem of bone malformation and skeletal immaturity on the other.


Assuntos
Anormalidades Múltiplas , Traumatismos da Medula Espinal , Humanos , Irmãos , Anormalidades Múltiplas/cirurgia , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Vértebras Cervicais/anormalidades , Traumatismos da Medula Espinal/complicações
3.
PLoS One ; 18(6): e0287175, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37379312

RESUMO

INTRODUCTION: We performed a systematic review and meta-analysis on the incidence of secondary tethered spinal cord (TSC) between prenatal and postnatal closure in patients with MMC. The objectives was to understand the incidence of secondary TSC after prenatal surgery for MMC compared to postnatal surgery for MMC. MATERIAL AND METHODS: On May 4, 2023, a systematic search was conducted in Medline, Embase, and the Cochrane Library to gather relevant data. Primary studies focusing on repair type, lesion level, and TSC were included, while non-English or non-Dutch reports, case reports, conference abstracts, editorials, letters, comments, and animal studies were excluded. Two reviewers assessed the included studies for bias risk, following PRISMA guidelines. TSC frequency in MMC closure types was determined, and the relationship between TSC occurrence and closure technique was analyzed using relative risk and Fisher's exact test. Subgroup analysis revealed relative risk differences based on study designs and follow-up periods. A total of ten studies, involving 2,724 patients, were assessed. Among them, 2,293 patients underwent postnatal closure, while 431 received prenatal closure for the MMC defect. In the prenatal closure group, TSC occurred in 21.6% (n = 93), compared to 18.8% (n = 432) in the postnatal closure group. The relative risk (RR) of TSC in patients with prenatal MMC closure versus postnatal MMC closure was 1.145 (95%CI 0.939 to 1.398). Fisher's exact test indicated a statistically non-significant association (p = 0.106) between TSC and closure technique. When considering only RCT and controlled cohort studies, the overall RR for TSC was 1.308 (95%CI 1.007 to 1.698) with a non-significant association (p = .053). For studies focusing on children up until early puberty (maximum 12 years follow-up), the RR for tethering was 1.104 (95%CI 0.876 to 1.391), with a non-significant association (p = 0.409). CONCLUSION AND DISCUSSION: This review found no significant increase in relative risk of TSC between prenatal and postnatal closure in MMC patients, but a trend of increased TSC in the prenatal group. More long-term data on TSC after fetal closure is needed for better counseling and outcomes in MMC.


Assuntos
Meningomielocele , Humanos , Feminino , Gravidez , Meningomielocele/cirurgia , Feto , Procedimentos Neurocirúrgicos/métodos , Incidência , Medula Espinal
4.
Prenat Diagn ; 43(3): 355-358, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36627260

RESUMO

OBJECTIVE: To develop a realistic simulation model for laparotomy-assisted fetoscopic spina bifida aperta (SBa) surgery, to be used for training purposes and preoperative planning. METHODS: The predefined general requirement was a realistic model of an exteriorized uterus, allowing all neurosurgical steps of the intervention. The uterus was modelled using ultrasound and MRI images of a 25 weeks' gravid uterus, consisting of flexible polyurethane foam coated with pigmented silicone. The fetal model, contained an opening on the dorsal side for a customizable spinal insert with all the aspects of a SBa, including a cele, placode, and myofascial and skin layer. The model was assessed in a series of validation experiments. RESULTS: Production costs are low, uterus and fetus are reusable. Placental localization and the level and size of the spinal defect are adjustable, enabling case-specific adaptations. All aspects of the simulator were scored close to realistic or higher for both appearance and functional capacities. CONCLUSIONS: This innovative model provides an excellent training opportunity for centers that are starting a fetoscopic SBa repair program. It is the first simulation model with adjustable spinal defect and placental localisation. Further objective validation is required, but the potential for using this model in preoperative planning is promising.


Assuntos
Meningomielocele , Espinha Bífida Cística , Gravidez , Feminino , Humanos , Meningomielocele/diagnóstico por imagem , Meningomielocele/cirurgia , Placenta/diagnóstico por imagem , Placenta/cirurgia , Espinha Bífida Cística/cirurgia , Fetoscopia/métodos , Feto/diagnóstico por imagem , Feto/cirurgia
5.
Front Oncol ; 13: 1218297, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38260852

RESUMO

Background: Only a small group of patients with glioblastoma multiforme (GBM) survives more than 36 months, so-called long-term survivors. Recent studies have shown that chromosomal instability (CIN) plays a prognostic and predictive role among different cancer types. Here, we compared histological (chromosome missegregation) and bioinformatic metrics (CIN signatures) of CIN in tumors of GBM typical survivors (≤36 months overall survival), GBM long-term survivors and isocitrate dehydrogenase (IDH)-mutant grade 4 astrocytomas. Methods: Tumor sections of all gliomas were examined for anaphases and chromosome missegregation. Further CIN signature activity analysis in the The Cancer Genome Atlas (TCGA)-GBM cohort was performed. Results: Our data show that chromosome missegregation is pervasive in high grade gliomas and is not different between the 3 groups. We find only limited evidence of altered CIN levels in tumors of GBM long-term survivors relative to the other groups, since a significant depletion in CIN signature 11 relative to GBM typical survivors was the only alteration detected. In contrast, within IDH-mutant grade 4 astrocytomas we detected a significant enrichment of CIN signature 5 and 10 activities and a depletion of CIN signature 1 activity relative to tumors of GBM typical survivors. Conclusions: Our data suggest that CIN is pervasive in high grade gliomas, however this is unlikely to be a major contributor to the phenomenon of long-term survivorship in GBM. Nevertheless, further evaluation of specific types of CIN (signatures) could have prognostic value in patients suffering from grade 4 gliomas.

6.
Epilepsy Behav Rep ; 16: 100508, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34917922

RESUMO

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy but there is limited literature characterizing the disease burden despite this being crucial for disease management strategies, and for designing and interpreting clinical trials. We searched the Vagus Nerve Stimulation (VNS) Therapy Patient Outcome Registry including over 7000 patients with drug­resistant epilepsy (DRE). Propensity Score Matching (PSM) matched LGS-DRE patients and non-LGS-DRE patients and frequencies of individual seizure types were assessed. The PSM population included 705 and 1410 DRE patients with and without LGS. 40% of the LGS-DRE group had polypharmacy with 3 antiseizure medications (ASM) while 42% in non-LGS-DRE had polypharmacy with 2 ASMs. Median total monthly seizure frequency was over double in the LGS group: 90 (IQR, 28-312) versus 40 (IQR, 10-150); p < 0.001. This analysis suggests that seizure frequency in LGS patients who later receive VNS is more than double than in non-LGS DRE patients with mostly bilateral tonic-clonic seizures contributing to this difference. Furthermore, ASM burden with poorer seizure control may be greater in LGS patients, however data collection ceased in 2003 and therefore does not take recent ASMs approved for LGS into account. This analysis offers quantitative insight into the burden of disease in patients with LGS.

7.
J Neurooncol ; 153(1): 121-131, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-33881726

RESUMO

OBJECTIVE: The aim of this work is to define competencies and entrustable professional activities (EPAs) to be imparted within the framework of surgical neuro-oncological residency and fellowship training as well as the education of medical students. Improved and specific training in surgical neuro-oncology promotes neuro-oncological expertise, quality of surgical neuro-oncological treatment and may also contribute to further development of neuro-oncological techniques and treatment protocols. Specific curricula for a surgical neuro-oncologic education have not yet been established. METHODS: We used a consensus-building approach to propose skills, competencies and EPAs to be imparted within the framework of surgical neuro-oncological training. We developed competencies and EPAs suitable for training in surgical neuro-oncology. RESULT: In total, 70 competencies and 8 EPAs for training in surgical neuro-oncology were proposed. EPAs were defined for the management of the deteriorating patient, the management of patients with the diagnosis of a brain tumour, tumour-based resections, function-based surgical resections of brain tumours, the postoperative management of patients, the collaboration as a member of an interdisciplinary and/or -professional team and finally for the care of palliative and dying patients and their families. CONCLUSIONS AND RELEVANCE: The present work should subsequently initiate a discussion about the proposed competencies and EPAs and, together with the following discussion, contribute to the creation of new training concepts in surgical neuro-oncology.


Assuntos
Oncologia Cirúrgica , Competência Clínica , Bolsas de Estudo , Humanos , Internato e Residência
8.
Neurosurg Focus ; 50(4): E12, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33794489

RESUMO

The aim of this study was to report on a single center's experience with spring-assisted cranial vault expansion (SAE) in patients with Crouzon syndrome and sagittal suture synostosis. Strip craniotomy with SAE has resulted in successful outcomes with low complication and revision rates in patients with isolated scaphocephaly. However, recent experience suggests that outcomes in patients with Crouzon syndrome and sagittal synostosis (SS) who undergo SAE are less favorable compared with the outcomes of those who undergo frontobiparietal (FBP) expansion. The authors reviewed both operations performed at a single center and noticed an upward expansion of the skull, which may be related to ventriculomegaly, with concurrent intracranial hypertension and poor aesthetic outcome. All patients diagnosed with Crouzon syndrome and SS who were treated with SAE required a revision FBP operation. Based on this outcome, the authors consider Crouzon syndrome a contraindication for correcting SS with springs.


Assuntos
Disostose Craniofacial , Craniossinostoses , Hipertensão Intracraniana , Procedimentos de Cirurgia Plástica , Disostose Craniofacial/diagnóstico por imagem , Disostose Craniofacial/cirurgia , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Craniotomia , Humanos , Lactente , Hipertensão Intracraniana/cirurgia , Crânio/diagnóstico por imagem , Crânio/cirurgia
9.
Prenat Diagn ; 41(8): 949-956, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-33778976

RESUMO

Since the completion of the Management of Myelomeningocoele Study, maternal-fetal surgery for spina bifida has become a valid option for expecting parents. More recently, multiple groups are exploring a minimally invasive approach and recent outcomes have addressed many of the initial concerns with this approach. Based on a previously published framework, we attempt to delineate the developmental stage of the surgical techniques. Furthermore, we discuss the barriers of performing randomized controlled trials comparing two surgical interventions and suggest that data collection through registries is an alternative method to gather high-grade evidence.


Assuntos
Fetoscopia/normas , Meningomielocele/cirurgia , Procedimentos Neurocirúrgicos/normas , Adulto , Feminino , Fetoscopia/métodos , Fetoscopia/estatística & dados numéricos , Humanos , Meningomielocele/epidemiologia , Procedimentos Neurocirúrgicos/métodos , Procedimentos Neurocirúrgicos/estatística & dados numéricos , Gravidez , Disrafismo Espinal/cirurgia
10.
Childs Nerv Syst ; 37(6): 2025-2031, 2021 06.
Artigo em Inglês | MEDLINE | ID: mdl-33604718

RESUMO

OBJECTIVE: Lumbosacral lipomas (LSL) of the 'dorsal' type have been associated with more favourable outcomes compared with other conus region lipomas. We hypothesised that integrity of the conus on MRI underpins the improved prognosis in this subgroup of LSL patients. METHODS: The definition of 'dorsal lipomas' included lipomas with attachment to the conus, but where the conus could be delineated on MRI (Morota type 1) as reported by Morota et al. (J Neurosurg Pediatr 19:428-439, 2017). Additional inclusion criteria included asymptomatic status at presentation, age >3 years at follow-up, and neurological and urological evaluation at presentation and at last follow-up. Lipoma extent and conus level were recorded. Outcome measures were the need for untethering surgery and neuro-urological status at last follow-up. Urological outcomes were defined by continence and efficacy of bladder emptying. RESULTS: Twenty-six children were included (median age 8.7 years). Conus level was low (at or below L2) in 92%. Nine required untethering surgery: 5 prophylactic, 4 because of clinical deterioration. Twenty-five children were continent at last follow-up, one had stress incontinence, and none required catheterisation. One had persisting ankle weakness after surgery requiring orthotic support. CONCLUSIONS: In LSL of the conus, visualisation of the conus on MRI is associated with good urological and motor outcomes. The integrity of the conus appears to be a more important prognostic factor than anatomical level. An observational approach to this group of LSL patients does not appear to compromise outcomes. These findings support a selective approach to untethering surgery.


Assuntos
Caramujo Conus , Lipoma , Neoplasias da Medula Espinal , Neoplasias da Coluna Vertebral , Animais , Criança , Pré-Escolar , Humanos , Lactente , Lipoma/diagnóstico por imagem , Lipoma/cirurgia , Região Lombossacral/diagnóstico por imagem , Região Lombossacral/cirurgia , Imageamento por Ressonância Magnética , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/cirurgia , Resultado do Tratamento
11.
J Plast Reconstr Aesthet Surg ; 74(5): 1087-1092, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33189617

RESUMO

BACKGROUND: The aim of this study is to identify if certain types of craniosynostosis and/or surgical procedures are more likely to require a secondary intracranial procedure because of insufficient correction of skull shape or raised ICP after initial surgery. METHODS: All consecutive cases with craniosynostosis that were operated at our center between January 2010 and January 2019 were included and the number of secondary operations of the vault were determined, as well as diagnosis, the indication and type and timing of initial surgery. Monobloc and facial bipartition procedures were excluded. RESULTS: Over a nine year period, 790 vault corrections were performed in 780 patients of which 38 procedures were reoperations in 35 patient because of raised intracranial pressure or an insufficient esthetic result of the skull shape. Particularly patients with a multisutural or syndromic craniosynostosis are represented in this group, as well as three surgical procedures: 1. biparietal outfracturing for sagittal synostosis; 2. endoscopic stripcraniectomy with helmet therapy for unicoronal, multisutural or syndromic craniosynostosis; 3. conventional occipital expansion for syndromic craniosynostosis. CONCLUSIONS: The risk of a second intracranial correction for insufficient outcome of skull shape or for raised ICP is related to type of synostosis and type of initial surgical technique. Particularly multisutural and syndromic craniosynostosis are more likely to require repeat surgery for these indications. Concerning initial technique, biparietal outfracturing does not correct sagittal synostosis sufficiently, stripcraniectomy with helmet therapy appears to undercorrect unicoronal, multisutural and syndromic synostosis, and conventional occipital expansion for multisutural and syndromic synostosis has poorer outcome than occipital expansion with distraction.


Assuntos
Craniossinostoses/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Craniossinostoses/classificação , Humanos , Lactente , Recém-Nascido , Procedimentos Cirúrgicos Minimamente Invasivos , Reoperação
12.
Neurosurg Focus ; 49(5): E9, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33130612

RESUMO

OBJECTIVE: Neurosurgery is historically seen as a high-risk medical specialty, with a large percentage of neurosurgeons facing complaints during their careers. The Dutch medicolegal system is characterized by a strong emphasis on informal mediation, which can be accompanied or followed by disciplinary actions. To determine if this system is associated with a low overall risk for medical litigation through disciplinary law, the authors conducted a review of disciplinary cases involving neurosurgeons in the Netherlands. METHODS: The authors reviewed legal cases that had been filed against consultant neurosurgeons and neurosurgical residents under the Dutch disciplinary law for medical professions between 2009 and 2019. RESULTS: A total of 1322 neurosurgical care-related cases from 2009 to 2019 were reviewed. Fifty-seven (4.3%) cases were filed against neurosurgeons (40 first-instance cases, 17 appeal cases). In total, 123 complaints were filed in the 40 first-instance cases. Most of these cases were related to spine surgery (62.5%), followed by cranial surgery (27.5%), peripheral nerve surgery (7.5%), and pediatric neurosurgery (2.5%). Complaints were filed in all stages of care but were mostly related to preoperative and intraoperative care. CONCLUSIONS: The risk for medically related litigation in neurosurgery in the Netherlands through disciplinary law is low but not negligible. Although the absolute number of cases is low, spinal neurosurgery was found to be a risk factor for complaints. The relatively high number of cases that involved the sharing of information suggests that specific improvements-focusing on communication-can be made in order to lower the risk for future litigation.


Assuntos
Imperícia , Neurocirurgia , Criança , Humanos , Países Baixos/epidemiologia , Neurocirurgiões , Coluna Vertebral
13.
Neurosurg Focus ; 47(4): E10, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31574464

RESUMO

OBJECTIVE: Improvements in imaging and surgical technological innovations have led to the increasing implementation of fetal surgical techniques. Open fetal surgery has demonstrated more favorable clinical outcomes in children born with open myelomeningocele (MMC) than those following postnatal repair. However, primarily because of maternal risks but also because of fetal risks, fetal surgery for MMC remains controversial. Here, the authors evaluated the contemporary management of MMC in the hope of identifying barriers and facilitators for neurosurgeons in providing fetal surgery for MMC. METHODS: An online survey was emailed to members of the Congress of Neurological Surgeons (CNS) and the International Society for Pediatric Neurosurgery (ISPN) in March 2019. The survey focused on 1) characteristics of the respondents, 2) the practice of counseling on and managing prenatally diagnosed MMC, and 3) barriers, facilitators, and expectations of fetal surgery for MMC. Reminders were sent to improve the response rate. RESULTS: A total of 446 respondents filled out the survey, most (59.2%) of whom specialized in pediatric neurosurgery. The respondents repaired an average of 9.6 MMC defects per year, regardless of technique. Regardless of the departments in which respondents were employed, 91.0% provided postnatal repair of MMC, 13.0% open fetal repair, and 4.9% fetoscopic repair. According to the surgeons, the most important objections to performing open fetal surgery were a lack of cases available to become proficient in the technique (33.8%), the risk of maternal complications (23.6%), and concern for fetal complications (15.2%). The most important facilitators according to advocates of prenatal closure are a decreased rate of shunt dependency (37.8%), a decreased rate of hindbrain herniation (27.0%), and an improved rate of motor function (18.9%). Of the respondents, only 16.9% agreed that open fetal surgery should be the standard of care. CONCLUSIONS: The survey results showed diversity in the management of patients with MMC. In addition, significant diversity remains regarding fetal surgery for MMC closure. Despite the apparent benefits of open fetal surgery in selected pregnancies, only a minority of centers and providers offer this technique. As a more technically demanding technique that requires multidisciplinary effort with less well-established long-term outcomes, fetoscopic surgery may face similar limited implementation, although the surgery may pose fewer maternal risks than open fetal surgery. Centralization of prenatal treatment to tertiary care referral centers, as well as the use of sophisticated training models, may help to augment the most commonly cited objection to the implementation of prenatal closure, which is the overall limited caseload.


Assuntos
Meningomielocele/cirurgia , Neurocirurgiões , Procedimentos Neurocirúrgicos , Rombencéfalo/anormalidades , Feminino , Fetoscopia/métodos , Feto/cirurgia , Humanos , Procedimentos Neurocirúrgicos/métodos , Gravidez
14.
Neurosurg Focus ; 47(4): E3, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31574477

RESUMO

OBJECTIVE: Myelomeningocele (MMC) is the most common form of spina bifida, with a lifelong impact on the quality of life for infants born with this condition. In recent decades, fetal surgery has evolved from an experimental therapy to standard of care for many centers in the world. In this study, the authors aimed to provide an overview of the current management and outcomes for infants with MMC managed at their institution. This then provides a center-specific historical cohort for comparison with future antenatal-treated MMC cases. METHODS: This is a retrospective, single-institution cohort study including all consecutive MMC cases between January 1, 2000, and June 1, 2018, at Erasmus MC. Outcome data included closure of the defect (location, timing, and surgical parameters), hydrocephalus management, Chiari malformation type II (CMTII) management, incidence of spinal cord tethering and outcome, motor outcomes, and continence. RESULTS: A total of 93 patients were included with predominantly lumbosacral lesions. Two patients died during follow-up. Hydrocephalus was present in 84%, with a 71% ventriculoperitoneal shunt reoperation rate. Surgery was performed in 12% for a tethered spinal cord at a mean age of 8 years. Decompression surgery was performed in 3 patients for CMTII. Special education in 63% was significantly associated with hydrocephalus (p < 0.015). Nineteen percent of patients were able to walk independently, and 47% were nonambulators. Social continence for urine was obtained in 75% of patients, 4% had fecal incontinence. CONCLUSIONS: This study provides an overview of current MMC outcomes at the authors' center and will serve as a historical cohort for comparison with future fetal surgery cases operated on at the center in the coming years. Apart from a relatively low surgical untethering rate, the authors' outcome data are comparable to those in the literature. Hydrocephalus is highly prevalent in postnatally treated MMC patients; in this study as in much of the literature, hydrocephalus is correlated with a low cognitive function. Fetal surgery for MMC halves the need for shunt treatment in a select group of MMC pregnancies, constituting a major indication for us to undergo the transition to a fetal surgery center. The fetal benefits of open antenatal surgery for MMC are well established, yet long-term data on especially tethered spinal cord are eagerly awaited.


Assuntos
Malformação de Arnold-Chiari/cirurgia , Hidrocefalia/cirurgia , Meningomielocele/cirurgia , Disrafismo Espinal/cirurgia , Criança , Descompressão Cirúrgica/efeitos adversos , Feminino , Humanos , Lactente , Masculino , Gravidez , Qualidade de Vida , Reoperação/efeitos adversos , Estudos Retrospectivos , Derivação Ventriculoperitoneal/efeitos adversos
15.
Ned Tijdschr Geneeskd ; 1632019 05 09.
Artigo em Holandês | MEDLINE | ID: mdl-31140766

RESUMO

To act or not to act? Developments in prenatal and postnatal care for children with spina bifida aperta Until the middle of the twentieth century, newborns with spina bifida aperta had low chances of survival. Advances in the treatment of hydrocephalus, among other conditions, led to increased chances of survival during the 1960s. This also revealed the downsides of the treatment of spina bifida patients since some considered the quality of life of a number of these patients to be unacceptable. But withholding treatment also had negative consequences, leading to an ethical deadlock. Over the past thirty years - besides postnatal closure of the neural tube defect - more emphasis has been put on selective pregnancy termination and sporadic active termination of life in newborns with very severe forms of spina bifida. At the same time, new treatment strategies, such as foetal surgery, are being developed. With this historical overview, we illustrate the way in which technological developments and ethical dilemmas are constantly affecting each other.


Assuntos
Aborto Eugênico , Eutanásia Ativa , Terapias Fetais , Espinha Bífida Cística/terapia , Aborto Eugênico/ética , Eutanásia Ativa/ética , Terapias Fetais/ética , Humanos , Recém-Nascido , Cuidado Pós-Natal , Cuidado Pré-Natal , Qualidade de Vida
16.
Br J Neurosurg ; 33(3): 287-289, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28617059

RESUMO

An 18-year-old patient developed multiple infarcts, nine days after endoscopic fenestration of a large arachnoid cyst. We consider vasospasm to be the most likely cause, presumably triggered by a chemical meningitis. Although mostly seen after subarachnoid haemorrhage, vasospasm can also occur after traumatic brain injury, brain surgery or meningitis.


Assuntos
Cistos Aracnóideos/cirurgia , Isquemia Encefálica/etiologia , Vasoespasmo Intracraniano/etiologia , Adolescente , Humanos , Imageamento por Ressonância Magnética , Masculino , Imagem Multimodal , Neuroendoscopia/efeitos adversos , Complicações Pós-Operatórias/etiologia , Tomografia Computadorizada por Raios X
17.
Neurosurg Focus ; 44(1): E7, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29290136

RESUMO

OBJECTIVE The health care costs for instrumented spine surgery have increased dramatically in the last few decades. The authors present a novel noninstrumented surgical approach for patients with isthmic spondylolisthesis, with clinical and radiographic results. METHODS Charts of patients who underwent this technique were reviewed. The procedure consisted of nerve root decompression by reconstruction of the intervertebral foramen. This was achieved by removal of the pedicle followed by noninstrumented posterolateral fusion in which autologous bone graft from the right iliac crest was used. Outcomes regarding radicular complaints, bony fusion, progression of the slip, and complications were evaluated using patient history and radiographs obtained at follow-up intervals of 3-18 months after surgery. RESULTS A total of 58 patients with a mean age of 47 years were treated with this method. Partial removal of the pedicle was performed in 93.1% of the cases, whereas in 6.9% of the cases the entire pedicle was removed. The mean duration of surgery was 216.5 ± 54.5 minutes (range 91-340 minutes). The mean (± SD) duration of hospitalization was 10.1 ± 2.9 days (range 5-18 days). After 3 months of follow-up, 86% of the patients reported no leg pain, and this dropped to 81% at last follow-up. Radiographic follow-up showed bony fusion in 87.7% of the patients. At 1 year, 5 patients showed progression of the slip, which in 1 patient prompted a second operation within 1 year. No major complications occurred. CONCLUSIONS Treatment of isthmic spondylolisthesis by reconstruction of the intervertebral neuroforamen and posterolateral fusion in situ is a safe procedure and has comparable results with the existing techniques. Cost-effectiveness research comparing this technique to conventional instrumented fusion techniques is necessary to evaluate the merits for both patients and society.


Assuntos
Descompressão Cirúrgica , Vértebras Lombares/cirurgia , Radiculopatia/cirurgia , Espondilolistese/cirurgia , Adulto , Idoso , Análise Custo-Benefício , Descompressão Cirúrgica/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Fusão Vertebral/métodos , Resultado do Tratamento
19.
Oncotarget ; 7(36): 58435-58444, 2016 Sep 06.
Artigo em Inglês | MEDLINE | ID: mdl-27533080

RESUMO

BACKGROUND: Glioblastoma is the most malignant tumor of the central nervous system and still lacks effective treatment. This study explores mutational biomarkers of 11 drugs targeting either the RTK/Ras/PI3K, the p53 or the Rb pathway using 25 patient-derived glioblastoma stem-like cell cultures (GSCs). RESULTS: We found that TP53 mutated GSCs were approximately 3.5 fold more sensitive to dual inhibition of mammalian target of rapamycin complex 1 and 2 (mTORC1/2) compared to wild type GSCs. We identified that Bcl-2(Thr56/Ser70) phosphorylation contributed to the resistance of TP53 wild type GSCs against dual mTORC1/2 inhibition. The Bcl-2 inhibitor ABT-263 (navitoclax) increased sensitivity to the mTORC1/2 inhibitor AZD8055 in TP53 wild type GSCs, while sensitivity to AZD8055 in TP53 mutated GSCs remained unchanged. CONCLUSION: Our data suggest that Bcl-2 confers resistance to mTORC1/2 inhibitors in TP53 wild type GSCs and that combined inhibition of both mTORC1/2 and Bcl-2 is worthwhile to explore further in TP53 wild type glioblastomas, whereas in TP53 mutated glioblastomas dual mTORC1/2 inhibitors should be explored.


Assuntos
Neoplasias Encefálicas/genética , Resistencia a Medicamentos Antineoplásicos , Glioblastoma/genética , Alvo Mecanístico do Complexo 1 de Rapamicina/antagonistas & inibidores , Alvo Mecanístico do Complexo 2 de Rapamicina/antagonistas & inibidores , Proteínas Proto-Oncogênicas c-bcl-2/antagonistas & inibidores , Proteína Supressora de Tumor p53/metabolismo , Compostos de Anilina/farmacologia , Biomarcadores Tumorais , Neoplasias Encefálicas/tratamento farmacológico , Neoplasias Encefálicas/metabolismo , Linhagem Celular Tumoral , Sobrevivência Celular , Estudos de Coortes , Análise Mutacional de DNA , Ensaios de Seleção de Medicamentos Antitumorais , Glioblastoma/tratamento farmacológico , Glioblastoma/metabolismo , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Morfolinas/química , Mutação , Células-Tronco Neoplásicas/citologia , Fosfatidilinositol 3-Quinases/metabolismo , Fosforilação , Proteoma , Sulfonamidas/farmacologia , Serina-Treonina Quinases TOR/metabolismo
20.
World Neurosurg ; 92: 586.e9-586.e13, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27178238

RESUMO

BACKGROUND: The out-of-body experience (OBE), during which a person feels as if he or she is spatially removed from the physical body, is a mystical phenomenon because of its association with near-death experiences. Literature implicates the cortex at the temporoparietal junction (TPJ) as the possible anatomic substrate for OBE. CASE DESCRIPTION: We present a patient who had an out-of-body experience during an awake craniotomy for resection of low-grade glioma. During surgery, stimulation of subcortical white matter in the left TPJ repetitively induced OBEs, in which the patient felt as if she was floating above the operating table looking down on herself. CONCLUSIONS: We repetitively induced OBE by subcortical stimulation near the left TPJ during awake craniotomy. Diffusion tensor imaging tractography implicated the posterior thalamic radiation as a possible substrate for autoscopic phenomena.


Assuntos
Craniotomia/efeitos adversos , Alucinações/etiologia , Tálamo/fisiopatologia , Vigília , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Imagem de Tensor de Difusão , Estimulação Elétrica/efeitos adversos , Feminino , Lateralidade Funcional , Glioma/diagnóstico por imagem , Glioma/cirurgia , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Articulação Temporomandibular/diagnóstico por imagem , Articulação Temporomandibular/fisiopatologia
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