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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.

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., Key Points What's already known about this topic? The use of fetoscopy for spina bifida repair is adopted by an increasing number of centers worldwide. On the other hand, its efficacy has not been established within a randomized controlled trial and thus it is still considered experimental by others. What does this study add? Herein we describe a framework which could be used to determine the developmental stage of novel interventions in the field of maternal‐fetal surgery. Furthermore, we discuss the difficulties of gathering high level evidence for a fetoscopic closure of the spinal defect in fetuses with a spina bifida.

OBJECTIVEIntracranial hypertension is a major concern in children with syndromic craniosynostosis (sCS). Cerebral venous hypertension caused by cerebral venous outflow obstruction is believed to contribute to intracranial hypertension. The authors therefore hypothesized that cerebral venous volume would be increased in those children with sCS and intracranial hypertension.METHODSIn a case series of 105 children with sCS, of whom 32 had intracranial hypertension, cerebral MRI techniques were used to quantify the volume of the superior sagittal sinus, straight sinus (StrS), and both transverse sinuses.RESULTSLinear regression showed that total cerebral venous volume increased by 580.8 mm3 per cm increase in occipitofrontal head circumference (p < 0.001). No significant difference was found between the intracranial hypertension group and the nonintracranial hypertension group (p = 0.470). Multivariate ANOVA showed increased StrS volume (as a proportion of total volume) in the intracranial hypertension group (8.5% vs 5.1% in the nonintracranial hypertension group, p < 0.001). Multivariate logistic regression showed that a 100-mm3 increase in StrS volume is associated with increased odds of having intracranial hypertension by 60% (OR 1.60, 95% CI 1.24–2.08).CONCLUSIONSAlthough intracranial hypertension was not associated with total cerebral venous volume increase, it was associated with an isolated increase in StrS volume. Hence, it is unlikely that general cerebral venous outflow obstruction is the mechanism of intracranial hypertension in sCS. Rather, these findings indicate either a central cerebral vulnerability to intracranial hypertension or a mechanism involving venous blood redistribution.

BACKGROUND Although prognosis is poor in high-grade glioma (HGG) patients, some become a long-term survivor (LTS). Health-related quality of life (HRQOL) and cognition are seen as indicators for quality of survival in HGG but data in LTS and their caregivers are scarce. We aim to investigate cognition and HRQOL in LTS with evaluation by their proxies. MATERIAL AND METHODS We included LTS with initial HGG-diagnosis between 1999–2014. Cognition was assessed with: Boston Naming Test, shortened Token Test, verbal (Category and Letter) Fluency, Diagnostic Instrument of Mild Aphasia, Hopkins Verbal Learning Test and Trail Making Test (A, B, B/A) and HRQOL with: EORTC Quality of Life Questionnaire (C30) and Brain Cancer Module (BN20). 15 Proxies evaluated patients’ HRQOL with: C30, BN20 and they filled in a burden scale: Caregiver Strain Index (CSI). RESULTS 21 patients with grade III or IV glioma and 7 years (range 5–20 years) median survival after diagnosis participated. Compared to normative data patients deviated significantly on HVLT (direct recall, delayed recall, recognition true positives), TMT (A, B and B/A), BNT and CF (p12 years survival compared to patients with CONCLUSION Most LTS suffer from cognitive impairments. Despite worse prognosis and cognitive impairments, at group level patients report relatively intact QoL, which is supported by their proxies. However, at the individual level severity of impairments is variable and high caregiver strain is reported in one-third of the proxies. Objective cognitive functioning was only modestly associated with subjective experience in patients. A patient-tailored approach is recommended to investigate individual impairments and needs for rehabilitation and support for patients and also for their carers.

Summary 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.

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.

OBJECTIVENeurosurgery 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.METHODSThe 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.RESULTSA 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.CONCLUSIONSThe 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.

The authors describe spontaneous externalization of a ventriculo-peritoneal shunt through the navel.

Introduction Sudden unexpected death in patients with epilepsy (SUDEP) remains a poorly understood entity, and it is unclear whether the same pathomechanisms underlie all sudden deaths occurring in patients with epilepsy. One aspect not included in current models of SUDEP is the role of increased intracranial pressure (ICP) which can be observed immediately upon seizure activity in neurosurgical practice. Methods We conducted a systematic review of the occurrence of edema in patients with epilepsy reported to have died of sudden death who underwent brain autopsy or postmortem brain imaging and discuss how increased ICP may contribute to clinical features of SUDEP. Results 19 eligible studies comprising a total of 623 patients were identified. Edema—mostly mild or moderate—was reported in 17% of cases and 74% of studies. 1% (n = 6) of the overall cases were clearly identified as having Dravet syndrome or an SCN1A mutation. In these patients, edema was found in 4 (67%) of cases. Conclusion Edema is regularly found in patients with epilepsy classified to have died from SUDEP. We argue that seizures preceding SUDEP may in certain cases elicit acute edema which may represent an additional contributing factor in the cascade of events leading to sudden death of patients with epilepsy. Furthermore, we hypothesize that mild edema may especially progress to severe edema in patients with sodium channel mutations which may represent an important mechanism to investigate in the context of understanding the significantly elevated risk of SUDEP in patients with SCN1A mutations., Seizures may in certain cases elicit acute edema which may represent an additional contributing factor in the cascade of events leading to SUDEP. We hypothesize that mild edema may especially progress to severe edema in certain patients e.g. those with sodium channel mutations which may represent an important mechanism to investigate in the context of understanding the significantly elevated risk of SUDEP in patients with SCN1A mutations.

// Subramanian Venkatesan 1 , Marlous Hoogstraat 2, 3 , Ester Caljouw 4 , Tessa Pierson 1 , Jochem K.H. Spoor 1 , Lona Zeneyedpour 5 , Hendrikus J. Dubbink 6 , Lennard J. Dekker 5 , Marielle van der Kaaij 1 , Jenneke Kloezeman 1 , Lotte M.E. Berghauser Pont 1 , Nicolle J.M. Besselink 2, 3, 7 , Theo M. Luider 5 , Jos Joore 4 , John W. Martens 8, 9 , Martine L.M. Lamfers 1 , Stefan Sleijfer 3, 8, 9 , Sieger Leenstra 1, 10 1 Department of Neurosurgery, Brain Tumor Center Erasmus MC, Rotterdam, The Netherlands 2 Department of Medical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands 3 Center for Personalized Cancer Treatment (CPCT), University Medical Center Utrecht, Utrecht, The Netherlands 4 Pepscope BV, Utrecht, The Netherlands 5 Department of Neurology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands 6 Department of Pathology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands 7 Department of Genetics, University Medical Center Utrecht, Utrecht, The Netherlands 8 Department of Medical Oncology, Erasmus MC Cancer Institute, Rotterdam, The Netherlands 9 Cancer Genomics Netherlands, Erasmus MC Cancer Institute, Rotterdam, The Netherlands 10 Department of Neurosurgery, St. Elisabeth Hospital Tilburg, Tilburg, The Netherlands Correspondence to: Sieger Leenstra, email: s.leenstra@erasmusmc.nl Keywords: brain tumor, personalized medicine, genetic biomarkers, small molecule kinase inhibitors, resistance Received: January 11, 2016 Accepted: July 26, 2016 Published: August 11, 2016 ABSTRACT 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.

OBJECTIVEThe 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.METHODSCharts 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.RESULTSA 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.CONCLUSIONSTreatment 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.