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OBJECTIVE Dandy-Walker malformation (DWM) is a disorder that most neurologists and neurosurgeons will manage at some point during their careers. It is characterized by partial or complete dysgenesis of the cerebellar vermis. Outcomes are highly variable and range from functionally normal to severely disabled. Predicting these outcomes has classically been focused on the radiological findings that constitute DWM. Other anomalies that can be commonly found in these patients are potentially more indicative of outcome than the tenet markers of DWM. Furthermore, hydrocephalus is an ever-present danger in these patients, many of whom will be admitted to the hospital due to this condition. This study aims to identify these items as potential predictors of outcome. METHODS All referrals from antenatal anatomy scans between 1992 and 2013 that were suspicious for DWM were reviewed. Neurosurgery archives were reviewed for outpatient letters and other correspondence. The number of DWM diagnoses was quantified. Outcomes were judged based on patient status, ranging from death to attending normal school. The presence of any other anomalies was quantified and measured against patient outcomes. RESULTS Cyst size and the presence of another CNS anomaly were shown to portend worse outcomes. Non-CNS anomalies and hydrocephalus were not predictive of worse outcomes. Furthermore, of all the treatments assessed, ventriculoperitoneal shunts were shown to be the most effective in this data set. CONCLUSIONS Results from this study suggest a pivot in how prognoses in DWM should be established and how parents should be counseled, along with a view of hydrocephalus and its treatment that challenges the current literature.

A multitude of operations exists for the treatment of patients with sagittal craniosynostosis presenting after 6 months of age, of which total cranial vault remodeling now provides the most reliable aesthetic outcome. As our national craniofacial center evolved and began to develop a comprehensive multi-disciplinary team to manage complex surgical cases, we offered cranial vault distraction as an alternative to more invasive surgery for late presentation nonsyndromic scaphocephaly. The authors conducted a retrospective review of all patients undergoing this procedure.An average distraction distance of 39.4 mm was achieved in 15 patients aged 1 to 9 years. As a result, the cephalic index changed an average of 4%. The mean transfusion volume in the perioperative period was 32.47% of estimated blood volume. There were 2 complications requiring further operative intervention and distraction was stopped early in 1 patient.The authors propose that internal calvarial distraction is a viable alternative to total cranial vault remodeling for the management of late presentations of sagittal craniosynostosis.

BACKGROUND Resorbable plates are commonly used in cranial vault reconstruction surgery. There are few published papers examining their safety profile. The authors examined the prevalence of wound complications associated with the use of resorbable plates (Inion CPS Fixation System) in pediatric patients undergoing cranial vault reconstruction. METHODS A retrospective review of patients (n = 182) who underwent cranial vault reconstruction using resorbable plate fixation was undertaken. All procedures were performed by a single Craniofacial Surgeon at the National Pediatric Craniofacial Center from 2008 to 2016. Wound complications were identified from a prospectively maintained database and medical note review. Several key patient characteristics and surgical variables were also recorded and tested for associations with wound complications. RESULTS A total of 58.8% (107 of 182) of patients were male with a median age at surgery of 16.2 months. Overall, 12.1% (22 of 182) experienced a postoperative wound complication requiring hospital admission. A total of 2.73% (5 of 182) of the patients that returned to theatre had remnants of plates removed. The authors had a mean time from primary operation to secondary reoperation of 103 days. In univariate statistical analysis, females were more likely to develop a wound complication. However, in stratified analyses excluding patients with an underlying genetic syndrome, increasing age, and lower weight but not gender were associated with wound complications. CONCLUSIONS A 12.1% (22 of 182) wound complication rate with the use of the Inion CPS Fixation System was observed. Inion plates appear to have an equivalent safety profile to other fixation devices. Increasing age and lower weight were associated with an increased risk of wound complications in nonsyndromic patients.

Endoscopic extended transsphenoidal surgery (EETSS) has gained popularity for treatment of craniopharyngiomas. The aim of this study is to assess the outcome of endoscopic extended transsphenoidal surgery (EETSS) for newly diagnosed paediatric craniopharyngiomas. Patient details were obtained from a prospective database of all endoscopic transnasal operations performed by a single surgeon. Outcomes including visual function, pituitary function, body mass index (BMI), postoperative neurological deficit, extent of resection and recurrence on follow-up were obtained. Obesity was defined as BMI percentile of equal to or greater than 95%. Between January 2011 and January 2020, 15 of 16 children (5–18 years old) with newly diagnosed craniopharyngiomas underwent EETSS. Four patients had a conchal-type sphenoid sinus. Gross total resection (GTR) was achieved in 4 patients and near total resection (NTR) in 5 patients. The remaining 6 had subtotal resection (STR). Postoperative radiotherapy was used in 6 patients (4 with STR, 2 with NTR). There were no postoperative deaths, strokes or CSF leaks. Normalisation of visual fields (VF) occurred in 9/13 patients with preoperative VF defects. One patient developed a new visual field defect. During a median follow-up period of 74 (8–104) months, 2 patients have required further surgery for tumour progression following initial STR, where a tumour remnant was left in situ to preserve the pituitary stalk. 6/11 patients developed new anterior pituitary dysfunction as a result of surgery and 9/12 developed new diabetes insipidus (DI). At the time of last follow-up, 14/15 children had anterior panhypopituitarism, 13/15 had DI and 1 patient developed new onset obesity. Two patients, who were obese preoperatively, were no longer obese at last follow-up. EETSS can be performed as the first option in the majority of children with newly diagnosed craniopharyngioma, despite factors such as small nose, non-pneumatised sphenoid sinus, small sella or purely suprasellar tumour location. Preservation of the pituitary stalk at the expense of leaving residual tumour may not be in the best interests of the patient.

Background While the concerns regarding the long-term cognitive effects of repeated sports related head injury have become a major source of debate, it is not uncommon for these patients to require neurosurgical interventions in the acute setting. The aim of this study was to provide a unique insight into the acute nature and neurosurgical management of sports and exercise related traumatic brain injury. Methods We retrospectively analysed electronic records of all referrals made between July 2016 and December 2018 to the National Neurosurgical Centre at Beaumont Hospital to identify instances of sport and exercise related traumatic brain injuries (TBI). A sub-group analysis was carried out on patients transferred to the tertiary centre requiring neurosurgical/neuro-critical care. Results Over the 30-month period, 194 patients (mean age: 36) were referred with sports and exercise related TBI, of which 56 were transferred to our unit (26 adults, 30 paediatrics). The most frequently encountered sporting activities were cycling, gaelic football, horse riding and rugby. Injuries included cerebral contusions, subdural haematomas, extradural haematomas and skull fractures. Neurosurgical intervention via intracranial pressure monitoring (ICP), decompressive craniectomy and elevation of depressed skull fracture was required in 28 out of 194 patients (14.4%). 85.7% (n = 48) of patients had a discharge Glasgow Coma Scale (GCS) of 15. 4 patients had a tracheostomy in place at discharge and there were 4 mortalities. Conclusions Sports and exercise activities, ranging from contact team sports to individual activities, are a common cause of traumatic brain injury and maybe associated with a significant morbidity and mortality.

There are no previously published reports regarding the epidemiology and characteristics of moyamoya disease or syndrome in Ireland. To examine patient demographics, mode of presentation and the outcomes of extracranial-intracranial bypass surgery in the treatment of moyamoya disease and syndrome in Ireland. All patients with moyamoya disease and syndrome referred to the National Neurosurgical Centre during January 2012–January 2019 were identified through a prospective database. Demographics, clinical presentation, radiological findings, surgical procedures, postoperative complications and any strokes during follow-up were recorded. Twenty-one patients were identified. Sixteen underwent surgery. Median age at diagnosis was 19 years. Fifteen were female. Mode of presentation was ischaemic stroke in nine, haemodynamic TIAs in eight, haemorrhage in three and incidental in one. Sixteen patients had Moyamoya disease, whereas five patients had moyamoya syndrome. Surgery was performed on 19 hemispheres in 16 patients. The surgical procedures consisted of ten direct (STA-MCA) bypasses, five indirect bypasses and four multiple burr holes. Postoperative complications included ischaemic stroke in one patient and subdural haematoma in one patient. The median follow-up period in the surgical group was 52 months; there was one new stroke during this period. Two patients required further revascularisation following recurrent TIAs. One patient died during follow-up secondary to tumour progression associated with neurofibromatosis type 1. Moyamoya is rare but occurs in Caucasians in Ireland. It most commonly presents with ischaemic symptoms. Surgical intervention in the form of direct and indirect bypass is an effective treatment in the majority of cases.

Extradural haematoma (EDH) is a serious neurosurgical emergency in children, which confers significant morbidity and mortality rates. The objective of this study was to retrospectively evaluate the role of post-operative imaging in children with EDH who were managed surgically in a national paediatric neurosurgical unit over a 9-year period (January 2008 to December 2016). A retrospective case review of paediatric patients who underwent surgical evacuation of extradural haematoma between January 2008 and December 2016 was performed. This included demographic and clinical details, indications for post-operative imaging and outcomes. Seventy patients underwent surgical management of EDH during this time period, with a male preponderance (69%) and a mean age of 8 years. The commonest location of haematoma in this cohort was in the parietal region (n = 24), with a mean maximum thickness of 25.9 mm and mean volume of 57 ml. Post-operative imaging was performed in 84% of patients. However, only one patient had a change in the course of their post-operative management as a result of post-operative imaging findings. Post-operative imaging in asymptomatic paediatric patients after evacuation of EDH could therefore be avoided as a routine investigation.

A specialist fetal neurosurgical clinic was set up in order to improve patient care in a tertiary referral fetal medicine centre. The clinic provides a targeted clinical service for women diagnosed with fetal neurological abnormalities. The service consists of fetal MRI, fetal ultrasound and joint assessment and counselling from neurosurgery and fetal medicine teams. We aimed to review this service that provides MDT expertise directly to parents and record the cases and pregnancy outcomes involved. This is a prospective study of clinic data from Jan 2013 to Dec 2017. Information includes ultrasound scan findings, MRI results, karyotype results and pregnancy outcome data including post mortem results and data from the paediatric neurosurgery service at the affiliated children’s hospital. From 2013 to 2017, there were 1852 major fetal anomalies diagnosed antenatally at the tertiary referral fetal medicine service and n = 306/1852 [16%] were primarily neurological in origin. The neurosurgical clinic reviewed 125 patients since 2013. The most common reasons for referral were spina bifida, n = 60 [48%] and isolated ventriculomegaly n = 43 [34%]. Other reasons for referral include agenesis of the corpus callosum n = 4 [3%], encephalocoele n = 5 [4%] and intracranial mass lesions n = 3 [2.4%]. Cases with borderline ventriculomegaly and cases with known chromosomal or genetic abnormalities were not typically referred to the clinic. Full outcome data were available on 110 of 125 women seen. Thirty-two women [29%] underwent invasive testing and 14 women [12.7%] had a termination of pregnancy. Multidisciplinary antenatal counselling supported with in utero MRI provides families with optimum information to inform them of likely neonatal outcome.

Purpose: Diffuse intrinsic pontine glioma is the most aggressive form of high grade glioma in children with no effective therapies. There have been no improvements in survival in part due poor understanding of underlying biology, and lack of representative in vitro and in vivo models. Recently, it has been found feasible to use both biopsy and autopsy tumors to generate cultures and xenograft models. Methods: To further model development, we evaluated the collective international experience from 8 collaborating centers to develop DIPG pre-clinical models from patient-derived autopsies and biopsies. Univariate and multivariate analysis was performed to determine key factors associated with the success of in vitro and in vivo PDX development. Results: In vitro cultures were successfully established from 57% of samples (84.2% of biopsies and 38.2% of autopsies). Samples transferred in DMEM media were more likely to establish successful culture than those transported in Hibernate A. In vitro cultures were more successful from biopsies (84.2%) compared with autopsies (38.2%) and as monolayer on laminin-coated plates than as neurospheres. Primary cultures successfully established from autopsy samples were more likely to engraft in animal models than cultures established from biopsies (86.7% vs. 47.4%). Collectively, tumor engraftment was more successful when DIPG samples were directly implanted in mice (68%), rather than after culturing (40.7%). Conclusion: This multi-center study provides valuable information on the success rate of establishing patient-derived pre-clinical models of DIPG. The results can lead to further optimization of DIPG model development and ultimately assist in the investigation of new therapies for this aggressive pediatric brain tumor.

This paper reviews CNS involvement secondary to malignant-mixed Müllerian tumor or uterine carcinosarcoma, a rare aggressive biphasic Müllerian tumor. We report a cerebellar metastasis with epithelial and mesenchymal components, demonstrating heterologous rhabdomyogenic and chondroblastic differentiation. The patient had undergone total abdominal hysterectomy and bilateral salpingo-oophorectomy for palliation of symptomatic chemotherapy-resistant node-positive disease. CNS involvement is rare, and prognostically poor, and suggestively poorer in predominantly sarcomatous metastases. Multimodal therapy is indicated; in solitary metastases, surgical resection or stereotactic radiosurgery is included, followed by whole brain radiotherapy. In unresectable brain metastases, stereotactic radiosurgery and whole brain radiotherapy warrant consideration in up to 2–3 metastases. In multiple metastases, palliative steroid therapy or cranial irradiation may be considered. Combination or platinum-based chemotherapy (i.e., ifosfamide-paclitaxel or carboplatin-paclitaxel) is indicated in all stages, with a role in both disease cure and control-directed management. Targeted therapeutics have thus far not demonstrated significant clinical efficacy.

Summary Background Insertion of a ventriculoperitoneal shunt for hydrocephalus is one of the commonest neurosurgical procedures worldwide. Infection of the implanted shunt affects up to 15% of these patients, resulting in prolonged hospital treatment, multiple surgeries, and reduced cognition and quality of life. Our aim was to determine the clinical and cost-effectiveness of antibiotic (rifampicin and clindamycin) or silver shunts compared with standard shunts at reducing infection. Methods In this parallel, multicentre, single-blind, randomised controlled trial, we included patients with hydrocephalus of any aetiology undergoing insertion of their first ventriculoperitoneal shunt irrespective of age at 21 regional adult and paediatric neurosurgery centres in the UK and Ireland. Patients were randomly assigned (1:1:1 in random permuted blocks of three or six) to receive standard shunts (standard shunt group), antibiotic-impregnated (0·15% clindamycin and 0·054% rifampicin; antibiotic shunt group), or silver-impregnated shunts (silver shunt group) through a randomisation sequence generated by an independent statistician. All patients and investigators who recorded and analysed the data were masked for group assignment, which was only disclosed to the neurosurgical staff at the time of operation. Participants receiving a shunt without evidence of infection at the time of insertion were followed up for at least 6 months and a maximum of 2 years. The primary outcome was time to shunt failure due the infection and was analysed with Fine and Gray survival regression models for competing risk by intention to treat. This trial is registered with ISRCTN 49474281. Findings Between June 26, 2013, and Oct 9, 2017, we assessed 3505 patients, of whom 1605 aged up to 91 years were randomly assigned to receive either a standard shunt (n=536), an antibiotic-impregnated shunt (n=538), or a silver shunt (n=531). 1594 had a shunt inserted without evidence of infection at the time of insertion (533 in the standard shunt group, 535 in the antibiotic shunt group, and 526 in the silver shunt group) and were followed up for a median of 22 months (IQR 10–24; 53 withdrew from follow-up). 32 (6%) of 533 evaluable patients in the standard shunt group had a shunt revision for infection, compared with 12 (2%) of 535 evaluable patients in the antibiotic shunt group (cause-specific hazard ratio [csHR] 0·38, 97·5% CI 0·18–0·80, p=0·0038) and 31 (6%) of 526 patients in the silver shunt group (0·99, 0·56–1·74, p=0·96). 135 (25%) patients in the standard shunt group, 127 (23%) in the antibiotic shunt group, and 134 (36%) in the silver shunt group had adverse events, which were not life-threatening and were mostly related to valve or catheter function. Interpretation The BASICS trial provides evidence to support the adoption of antibiotic shunts in UK patients who are having their first ventriculoperitoneal shunt insertion. This practice will benefit patients of all ages by reducing the risk and harm of shunt infection. Funding UK National Institute for Health Research Health Technology Assessment programme.

The practice of trepanning (referred to today as a craniotomy) dates back to the Neolithic period. Reasons for drilling a hole through the skull evolved from releasing evil spirits and curing insanity to practical management of head injuries in ancient Greece and Rome. Today, craniotomy or drilling a burr hole through the skull is very much the purview of the neurosurgeon. Yet one could argue that the procedure itself is more ‘bone surgery’ than ‘brain surgery’. Nevertheless, despite the fact that head injury is a common presentation at district general hospitals and traumatic extra-axial haemorrhages are encountered often, the straightforward skillset required to drill a burr hole as a pretransfer, temporising, life-saving measure is seldom taught and has never gained traction. What we advocate in this article is the adaptation and novel application of an old, tried and tested technique in new hands. The critical pathophysiological turning point of any expanding extra-axial haemorrhage is the inflection point on the volume/Intracranial pressure (ICP) curve beyond which compensation is impossible. The subsequent rising ICP initiates a predictable continuum of clinical signs signalling progressive herniation. There are few emergencies as time-critical as a patient with an isolated, expanding extradural haemorrhage embarking on a trajectory of rostrocaudal deterioration and inevitable death. In many cases, the tragedy is compounded by the knowledge that such a patient probably has a healthy underlying brain, often evidenced by a lucid period after trauma. Our emergency department is attached to a small 300-bed District General Hospital (DGH) on the rural North West coast of Ireland. We are 262 km distant by road from a national neurosciences department that can, at best, be reached in 2 hours and 30 min. Quality improvement review of years of dismal outcomes in patients such as those described earlier with potentially remediable pathology prompted research and development of the skillset we are now able to offer, an old technique in new hands.

Introduction Differentiation between plagiocephaly and craniosynostosis continues to provide a challenge for the clinician. Historically, initial investigations would have included a skull x-ray. However, it is widely accepted that this investigation is difficult to interpret and its value in diagnosis of craniosynostosis is debated. Aside from creating the burden of additional investigations, it also exposes children to ionising radiation. Aim and Objectives Examine the correlation between reported skull x-rays and clinical diagnosis of craniosynostosis to establish their diagnostic value and value in management of abnormal skull shape. Methods Retrospective chart review conducted as a 2 part study in children who were referred with a skull x-ray. Part A: Referrals of abnormal skull shapes to the National Pediatric Craniofacial Center (NPCC), Temple Street Children’s Hospital, between 1st January 2015 and 30th May 2017 Part B: Children who underwent surgery for a confirmed craniosynostosis between 1st January 2011 - 25th October 2017. Results and Findings Part A: 300 children were referred with 59 skull x-rays. This represented 20% of all patients referred during the time period. Of these 44 (75%) were found to be a match with 15 (25%) not matching the final clinical diagnosis. Part B: 274 children underwent surgery for a confirmed craniosynostosis between 1st January 2011 - 25th October 2017. 63 pts had skull x-rays on referral- this represents 23% of all operated children in the time period. Of these 41 (63%) were found to be a match with 17 (29%) not matching the final clinical diagnosis. 5 (8%) were inconclusive. Conclusions Part A: In 25% of the children referred to the NPCC with abnormal skull shape, their clinical diagnosis did not match their x-ray report. As such, skull x-rays did not contribute to their management. Part B: In 35% of children who underwent surgery for craniosynostosis, their clinical diagnosis did not match their radiological diagnosis. In the remaining children who had a skull x-ray performed and underwent surgery for craniosynostosis, the majority (88%) had a subtype of craniosynostosis which our clinical team who feel confident to diagnosis clinically without imaging. As such, it can be said that in 92% of children who underwent surgery at the NPCC the x-ray did not contribute to their management. We recommend clinicians should check with the NPCC with respect to the protocol for x-rays where craniosynostosis is suspected as in most cases the diagnosis is clinically obvious to the craniofacial surgeon.

Background Craniosynostosis is a congenital condition resulting from premature fusion of the cranial sutures resulting in a phenotypic headshape. Nonsyndromic craniosynostosis, involving a single suture only; is the most common presentation involving the fusion of one of the following sutures; sagittal, metopic, coronal or lambdoid. The National Paediatric Craniofacial Centre in Temple Street Children’s University Hospital is the designated craniofacial centre in the Republic of Ireland. The treatment of craniosynostosis involves appearance-changing surgery designed to normalise the headshape. As with most other designated craniofacial centres the aim is to perform surgery as close to 12 months of age as possible for these children. The clinical presentation of craniosynostosis as with most rare diseases can result in an over-reliance on different types of radiology imaging, a subsequent later referral and surgery being performed at a later age. The aim of this review was to report the referral patterns of children with metopic, coronal and lambdoid nonsyndromic craniosynostosis presenting to the NPCC. Methods A retrospective chart review of children who underwent surgery for metopic, coronal or lambdoid craniosynostosis between January 2009 and December 2018 was undertaken. Data collected included: patient demographics, hospital of birth, referrer specialty, date of referral and any radiology investigations performed. Children subsequently diagnosed with a syndrome were excluded. Results 130 patients were included in the review. 80% (n=104) were referred before 10 months of age. There was a high use of radiology imaging with 71% (n= 92) of all children having at least one radiology investigation prior to referral. 15% (n=19) had multiple radiology investigations performed. Paediatricians and Neonatologists referred the largest number of children; 58% (n=75) and 22% (n=28) respectively. Surgery was performed before 15 months of age for 44% (n=57) of children and before 18 months of age for a further 26% (n=34) of all children. Conclusion The incidence of metopic, coronal and lambdoid craniosynostosis is in keeping with reported rates in Europe. Although most children were referred before 10 months of age complex referral patterns and use of radiology imaging were associated with later referrals, leading to subsequent delays in surgery performed. It is hoped that an increased awareness of recognising and referring children with craniosynostosis will lead to subsequent treatment as early as possible. The NPCC encourages that referral letters for children with suspected craniosynostosis are accompanied by photographs of the child’s headshape and not radiology investigations.

Introduction There is a paucity of knowledge regarding Sleep Disordered Breathing (SDB) in paediatric patients with Spina Bifida/Myelomeningocoele (SB/MMC). Undiagnosed, untreated SDB has detrimental effects on growth, development (1) and cognition (2) which is especially important to recognise in those with other co-morbidities as is the case in SB/MMC. Aim There are currently 317 patients aged 0–16 years attending the Spina Bifida service at Temple Street Children’s University (TSCUH). Fourteen of 317 patients (4.4%) currently require Non-Invasive Ventilation (NIV) therapy for SDB under the care of the Respiratory Team. We hypothesize that a subpopulation of the SB/MMC patients are at risk of undiagnosed SDB. We aimed to initially review the demographics of the sub-group of SB/MMC patients currently requiring NIV by assessing for potential predictors of SDB. Method We used up to date medical records of the patients under our care to look for potential predictors for SDB, in particular hydrocephalus with VP shunt, Chiari II malformation and intellectual disability (ID). We then compared our data to what is known about the general SB/MMC population. Results 100% of SB patients with SDB requiring NIV had hydrocephalus with VP shunt compared to 80% in general SB/MMC patients. 92% also had Chiari II malformation. 50% had a moderate to severe ID in contrast to 25–25% of the general SB/MMC population. Conclusion A higher proportion of SB/MMC patients known to have SDB have hydrocephalus and ID compared to the SB/MMC population mean. At present there is no standardised screen for SDB in our SB/MMC patients. The results of this review have prompted a clinical audit of all of SB/MMC patients at TSCUH to identify those at higher risk of SDB and ultimately determine the prevalence of SDB in this population. References ‘How do we recognize the child with OSAS?’ Joosten KF, et al. Pediatric Pulmonology 2017 Feb;52(2):260–271. ‘Association of Sleep-Disordered Breathing With Cognitive Function and Risk of Cognitive Impairment: A Systematic Review and Meta-analysis.’ Leng Y, et al. JAMA Neurology 2017 Oct 1;74(10):1237–1245.

The authors describe the case of a 3-year-old boy with a giant congenital vertex hemangioma who underwent presurgical embolization with Onyx (ethylene-vinyl alcohol copolymer dissolved in dimethyl sulfoxide) and Glubran (N-butyl-2-cyanoacrylate). This vascular tumor had no intracranial vascular communication as assessed by pre-embolization MRI and catheter angiography. All embolizations were performed by direct percutaneous injection. One week following the last embolization procedure the child presented with a 24-hour history of ataxia and extrapyramidal tremor. He was diagnosed with a possible immune-mediated reaction to Onyx or Glubran, which was treated with an urgent surgical excision of the hemangioma followed by intravenous administration of immunoglobulin and steroids. To the authors' knowledge, this is the first case of possible immune-mediated toxicity secondary to either Onyx or Glubran administration. This case highlights the need for awareness of potential toxic reactions to these embolic agents in the treatment of hemangiomas in the pediatric patient.

Minimizing normal-tissue radiation exposure is especially important in the pediatric population as children appear to be particularly sensitive to postradiation vasculopathies after conventional photon radiotherapy. Given the limited scattering effect and low-dose radiation delivery to the surrounding tissues with proton beam radiotherapy, this modality is considered to be an effective treatment for pediatric skull-base tumors compared to conventional radiotherapy, and to have fewer adverse side effects. We report 2 cases of radiation-induced moyamoya syndrome following proton beam therapy in pediatric patients. To our knowledge, only a few other reported cases of radiation-induced moyamoya syndrome following proton beam therapy exist in the current literature. While rare, radiation-induced moyamoya syndrome can occur in the pediatric population with newer techniques like proton beam radiotherapy. Accordingly, patients and their families should be informed about this potential complication prior to all forms of radiation treatment.

OBJECTIVEThe aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors’ service.METHODSThe authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients’ neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale–Extended (GOS-E Peds) obtained 1 year after the last operation.RESULTSFive patients with a bleeding diathesis who underwent surgery for intracranial hematoma were identified; the diagnosis was hemophilia A in 3 cases, idiopathic thrombocytopenic purpura in 1 case, and severe aplastic anemia in 1 case. Intracerebral hematoma (ICH) (n = 4) and acute subdural hematoma (n = 1) were confirmed on radiological investigations. In 2 of the 4 patients with ICH, the diagnosis of bleeding diathesis was made for the first time on presentation. Four patients (all male) were younger than 2 years; the patient with severe aplastic anemia and spontaneous ICH was 15 years old and female. The duration of symptoms varied from 24 hours to 5 days. Neurological examination at 1 year’s follow-up showed complete recovery (GOS-E Peds score of 1) in 3 cases and mild weakness (GOS-E Peds score of 2) in 2 cases.CONCLUSIONSNeurosurgical management of patients with bleeding diathesis should be carried out in a tertiary-care setting with multidisciplinary team management, including members with expertise in neuroimaging and hematology, in addition to neurosurgery. Early diagnosis and prompt treatment of a bleeding diathesis is crucial for full neurological recovery.

Background The most common condition treated by paediatric neurosurgeons is hydrocephalus. Despite having significant improvements in treatment and management, hydrocephalus continues to have a considerable impact on the long-term outcome in children. The objective of designing Electronic Integrated Text, Visual and Audio Questionnaire (EITVAQ) was to develop a child-friendly tool, anticipating it will be a better tool to measure child health status from a child’s perspective. Methods This was a prospective crossover pilot study that compared the outcome and completion rate between EITVAQ and a printed text 2-paged paper questionnaire (TEXT). Children with hydrocephalus from 8 years old to 16 years old were invited to participate in this study from November 2015 to June 2016. EITVAQ was created to be visual and audio automated using macrosenabled function in Microsoft PowerPoint. Time of completion for each format was recorded. All data were compiled and organised for statistical analysis using Software Package using Statistical Analysis (SPSS). Findings 24 children participated in this study. There was a 100% completion rate for EITVAQ with only 37.5% completing the TEXT format. The mean time to complete EITVAQ was 7 min and the mean time for TEXT was 15 min. The mean score for EITVAQ was was 16% higher in comparison to a mean score of HOQ completed by proxy. The result suggested participants with more surgical procedures tend to have a higher incompletion rate of TEXT format by 18.9%. However, there was no correlation found between GMFCS score and the incompletion rate. Such results suggested that the incompletion rate in TEXT format is likely secondary to a central cause rather than a physical cause. Conclusions In this study, the results supported that EITVAQ is a better tool to evaluate the health status of paediatric hydrocephalus patients. Such encouraging results call for future research in this area to develop EITVAQ as a validated tool and to be used widespread across hydrocephalus patients to identify health status issues, to enable them to access appropriate early intervention and to improve their quality of life.

BACKGROUND: Ireland has the highest rate of melanoma related deaths in Europe. Despite the incidence of melanoma reaching record highs there remains a paucity of information in Ireland regarding the factors associated with melanoma brain metastasis (MBM). METHODS: Patients diagnosed with MBM in Ireland were retrospectively identified in Beaumont Hospital between 1999 and 2018. Patient demographics; year of detection of MBM, age at diagnosis of primary melanoma, age at detection of MBM, anatomical location of primary melanoma, BRAF mutation analysis and the number of metastases were investigated. Follow up data was also derived, including overall survival (OS). RESULTS: The incidence of malignant melanoma has increased by 158% over the past 20 years with 1,092 and 422 cases diagnosed in 2018 and 1999, respectively.128 patients with melanoma brain metastases were identified during this period. The median OS after detection of MBM was 5 months (95% CI 0.641–9.359 months). There was a male predominance (n= 77/128; 60%) with a median age of death at 58 years (n=67; range 16–82 years). Although females had a significantly longer time between diagnosis of primary melanoma and detection of MBM compared to males, 4 and 2 years respectively (p=0.02442), there was no significant age difference at death between males and females (p= 0.41294). BRAF mutation was an independent prognostic factor with an improved overall survival compared to those without the mutation, of 8 months and 3.5 months respectively (p=0.0012). Although non significant, the primary location of melanoma, leptomeningeal disease and number of cerebral metastases were all important considerations in this group. CONCLUSIONS: Male predominance and BRAF mutation represent important factors in this population group. The results of this study add to our knowledge concerning outcomes in melanoma brain metastases in Ireland, and may be useful in clinical planning, educational programs and future treatments.

Intraparenchymal meningiomas are rare. To date, no such lesion has been reported within the basal ganglia of a paediatric patient. Here, we describe the case of a 15-year-old-boy who presented with symptoms referable to a cystic, calcified, left basal ganglia intraparenchymal meningioma and discuss the surgical management of this lesion.

Object The frequency with which routine preoperative blood test results predict perioperative or postoperative complications is insignificant. The unnecessary ordering of routine tests increases the financial costs and patients' distress. The authors evaluated the effects of routine preoperative testing on patient management and the overall financial costs. Methods The authors retrospectively reviewed the medical records and laboratory data for 355 children admitted to the neurosurgical department for elective procedures over a 5-year period (January 2008–December 2012). They excluded all patients admitted for imaging or surgical procedures requiring local anesthesia, and they recorded the results of preoperative and previous (up to 6 months before surgery) blood tests and any abnormalities noted. Results As a result of the 3489 blood tests ordered preoperatively for 328 (94.6%) of the 355 patients, 29 abnormalities (9%) were detected. Most of these abnormal values were near the reference range, and none significantly affected the progression of scheduled procedures. For only 1 patient (0.28%) was the procedure cancelled because of an abnormality (preoperative partial thromboplastin time), which further testing showed to be a false-positive result. The cost of these tests over 5 years was 5205–10,410 euros ($6766–$13,533 US). Conclusions Preoperative tests should be selectively requested on the basis of clinical indication.

The field of neuroendoscopy is rapidly expanding with increasing indications for endoscopic third ventriculostomy (ETV). As a treatment for hydrocephalus, ETV has the advantage of providing a more physiological cerebrospinal fluid diversion without shunt hardware which reduces the risk of recurrent infection and malfunction. The success rate of ETV has been increasing with decreasing morbidity and mortality. Originally, ETV was indicated for cases of obstructive hydrocephalus, however the indications are expanding. To highlight this, we present a small series of cases were ETV is not traditionally indicated and was a treatment of last choice.

Chiari I malformation may be treated with foramen magnum decompression (FMD). We aim to describe the symptoms with which patients initially present, and to determine the number and type of complications occurring after FMD for Chiari I malformation.Retrospective review of medical records for patients who had FMD performed for Chiari I malformation between January 2009 and December 2011. Post-operative outcomes were recorded and analysed. Patient demographic details and other relevant medical conditions were also noted.Between January 2009 and December 2011, 54 FMDs were performed for Chiari I malformation. Among them, 40(74%) patients were female and 14 patients (26%) were male. The majority of patients (42.6%) were aged 16-39 years and 24.07% of patients were children aged16 years. A total of 30(55.6%) patients had documented evidence of a syrinx pre-operatively. 18(33.3%) patients developed complications. Nine of these developed multiple complications while nine had a single problem. One mortality was reported. Ten (18.5%) patients developed hydrocephalus requiring shunting. Two patients developed subdural collections requiring evacuation associated with hydrocephalus. Six (11.1%) patients developed post-operative infections: two CNS infections; one wound infection; and three other infections.FMD for Chiari I malformation is a procedure which carries risk. In particular, the risk of developing post-operative hydrocephalus requiring permanent shunting is relatively high. ICP monitoring prior to FMD may be required to definitively rule out raised intracranial pressure.

Over the years, there has been increasing awareness of the radiation doses from medical investigation and treatment modalities and the long-term effect of this radiation. In no other patient population is this more of an issue than in the paediatric population who are more radiosensitive and have a longer life span in which to express any negative effects of radiation. In children under the age of one, the anterior fontanelle is an acoustic window to intracranial structures allowing for the use of cranial ultrasound (CRUSS) instead of CT. To determine the pattern of CT and Cranial ultrasound used in patients aged one or younger with shunt treated hydrocephalus. A retrospective review of patients who had a shunt inserted before the age of one and their imaging. Effective radiation doses were calculated for those who had CT scans. One hundred thirty-five patients were included with 227 CTs and 124 CRUSS conducted. In the follow-up period after shunt insertion, 92 patients had CTs while 14 were followed with CRUSS and 51 patients required a shunt revision before the age of one. The average effective radiation dose per scan was 2.76 mSv. Children with an open fontanelle and shunt can be followed reliably with CRUSS in order to reduce their exposure to radiation.

The endoscopic third ventriculostomy success score (ETVSS) is a model, which provides each patient with a prediction of the outcome of endoscopic third ventriculostomy. The objective of this study was to determine if there is clinical value to the use of the ETVSS in the decision for ETV.Prospectively collected data on all ETV procedures with the Republic of Ireland in children ≤16 years of age, totalling 112, from 2008 to 2014 was analysed. The percentage chance of success at six months was retrospectively calculated according to the ETVSS. A multivariable model, comprising the risk factors from the ETVSS - age, aetiology and previous shunt - was created and its performance compared to that of the ETVSS.The ETVSS achieved an AUC of 0.61 (95% CI: 0.49-0.71) with a sensitivity and specificity of 50% and 76%, respectively, at its optimal cutoff. The ETVSS was not significantly well calibrated in this cohort and there was a limited net benefit on decision curve analysis in comparison with the strategy of performing ETV in all patients. The multivariable model achieved an AUC of 0.67 (95% CI: 0.56-0.78), was well calibrated and was associated with a superior net benefit over that of the ETVSS.The ETVSS represents the future of patient risk stratification with an easy to use, individualised approach for each patient. The ETVSS has performed adequately in this study. However, through the addition of novel risk factors, the continuous updating of the model and recalibration where needed, the ETVSS can become a tool that the paediatric neurosurgeon cannot do without.

We present a very rare case of indirect cavernous carotid fistula (CCF) in a 12-year-old girl. Indirect CCF is extremely rare in the paediatric population. A 12-year-old girl presented with a 7-month history of frontal headaches and intermittent left-sided proptosis. On examination, she had dilated and engorged scleral veins on the left eye, mild dysdiadochokinesia and past pointing on the left side. A brain computer tomography with contrast, brain magnetic resonance imaging (MRI) and interventional radiography (IR) cerebral angiogram confirmed the diagnosis of CCF. The CCF was embolized and a follow-up brain MRI and an IR cerebral angiogram were conducted over the course of 8 months that revealed no evidence of residual CCF. CCF, though rare in the paediatric population, should be highly considered in the differential diagnosis when dilated scleral veins, proptosis and dysdiadokinesis are present in the clinical setting. Prompt treatment has good prognostic results.

There are two errors and one omission in the original article. Author Gottardo's correct name is Nicholas G. Gottardo, author Hulleman's correct affiliation is no. 3 (VUMC, Amsterdam), and the Acknowledgements should include the following sentence: "We would like to thank Dr Angel Montero Carcaboso (Hospital Sant Joan de Deu, Barcelona, Spain) for generously supplying the HSJD-DIPG007 cells."

BACKGROUND: The Electronic Integrated Text, Visual and Audio Questionnaire (EITVAQ) was designed towards developing a disease-specific tool to measure child health status from a child’s perspective. EITVAQ’s outcome is anticipated to be positive, introducing a child-friendly health measurement tool. METHODS: This was a prospective crossover pilot study that compared EITVAQ to a printed text 2-paged paper questionnaire (TEXT) format quality of life questionnaire in paediatric hydrocephalus patients. The study was conducted from November 2015 to June 2016 in the Neurosurgical Outpatient Department, Temple Street, Children University Hospital. Data was compiled for both EITVAQ and TEXT and analyzed using Software Package using Statistical Analysis (SPSS). FINDINGS: There was a 100% rate of completion for EITVAQ. However, only 9 (37.5%) of the patients managed to complete the text format. The mean time to complete EITVAQ was 7 minutes. There are 32 questions found in EITVAQ. The mean score for EITVAQ was 132.92 (83%) satisfactory score. Using Pearson Chi-Square Test, the P-Value is 0.341. Although this is not a significantly substantial value, however, patient with less surgical procedure were able to complete EITVAQ 18.9% more than patient with more than 5 surgical procedures. Furthermore, there was no real correlation between the gross motor function classification system (GMFCS) score and the inability to complete the TEXT format. CONCLUSIONS: In this study, the results support that EITVAQ is a better tool to evaluate the health status of paediatric hydrocephalus patients as it is tailored according to their disability needs.

Ventriculoperitoneal (VP) shunt insertion is the most common cerebrospinal fluid (CSF) diversionary procedure used for the treatment of chronic hydrocephalus. Sterile CSF ascites is a rare complication of VP shunt insertion. This can arise from either an overproduction of CSF or inadequate filtration of CSF at the level of the peritoneum. By either mechanism, the development of CSF ascites requires an intact VP shunt. The authors discuss two paediatric cases diagnosed with suprasellar pilocytic astrocytomas treated with platinum-based chemotherapy, who subsequently developed sterile CSF ascites. We review the literature with regard to CSF malabsorption and discuss it as a contributing factor to shunt malfunction. CSF malabsorption with resultant ascites is a rare complication of VP shunting with many etiologies. Two common predisposing factors included the use of platinum-based chemotherapeutic agents, as well as the specific neuropathology. Further analysis of these two entities is needed in order to elucidate their role in contributing to the development of CSF ascites in this patient cohort.

The authors report a case of high-grade glioma with unusual pathology that has not previously been reported in glioma pathology. The 5-year-old patient presented to the emergency department with a 1-day history of a right temporal swelling on a background history of increasing nausea and vomiting for the preceding 5 months. A computed tomography brain was performed, which showed a large right-sided temporoparietal lesion. The patient underwent surgery to remove the mass and pathology confirmed anaplastic astrocytoma (WHO Grade 3). This report focuses on prognostic factors in high-grade glioma, particularly on pathological indicators, namely epidermal growth factor receptor, O6-methylguanine-DNA-methyltransferase expression and BRAF V600D mutation.

The objectives of this study are to ascertain the clinical outcome and overall survival of holoprosencephaly (HPE) patients diagnosed antenatally and postnatally, to determine the accuracy of antenatal diagnosis and to determine the role of neurosurgical intervention in HPE. This is a retrospective review over a 10-year period. Sixty-three patients were included in the study, 45 were diagnosed by antenatal radiological imaging and 18 were diagnosed by postnatal radiological imaging. Patient data was drawn from Temple Street Children’s University Hospital (the national paediatric neurosurgery centre), the National Maternity Hospital in Holle’s Street, Dublin, and Our Lady of Sick Children Hospital, Dublin. The study was carried out through a review of antenatal and postnatal radiological imaging and reports, clinical charts, GP letters from patient follow-up and telephone conversations with parents of HPE patients. Four patients in the antenatal diagnosis group had follow-up foetal MRI confirming HPE. Twelve in this group had radiological follow-up postnatally, and in five of these, HPE was confirmed. The remaining seven were identified as false positive. Alobar HPE constituted 55 % (21/38) of patients with 95 % mortality. Fifty-one percent had a normal karyotype. The overall survival in the antenatal diagnosis group was 13 %. In the postnatal group, 18 patients were identified, 67 % (12/18) lobar and 33 % (6/18) semilobar. Normal karyotype was found in 72 % (13/18), with an overall survival rate of 56 % (10/18). Neurosurgical intervention in both groups mainly consisted of CSF diversion in the form of ventriculoperitoneal (VP) or cystoperitoneal shunt (CP) (13/67). Foetal MRI should be routinely performed in suspected cases of HPE, and reliance on ultrasound alone in the antenatal period may not be sufficient. In our study, there is a high early mortality noted in severe cases of HPE, while milder forms of HPE in children tend to survive beyond infancy albeit with associated complications that required neurosurgical intervention and medical management for other associated systemic anomalies.

Background Hemorrhage in medulloblastoma (MB) is a rare clinical entity with the undeniable potential for catastrophic repercussions. This case report highlights the first incidence of intraoperative subarachnoid hemorrhage (SAH) in a patient with MB secondary to an intracranial aneurysm. Case Description A 6-year-old girl with a left cerebellar mass, fourth ventricle effacement, and triventriculomegally had an external ventricular drain inserted, at which time blood stained cerebral spinal fluid was drained. After insertion of an external ventricular drain, imaging showed diffuse SAH. Post tumor resection scans showed a small residuum of tumor. At repeat tumor resection after chemoradiotherapy, a 2-mm internal carotid aneurysm was noted. Conclusions Intraoperative hemorrhage in MB is a very rare occurrence. We describe the first case of hemorrhage in MB secondary to an intracranial aneurysm. MB has a predisposition to bleed spontaneously that can have catastrophic repercussions. Sudden clinical deterioration after insertion of external ventricular drainage should be susceptive of intracerebral hemorrhage. In cases of uncertain etiology, investigation of SAH with cerebral angiography is recommended.

OBJECTIVE Elevated serum levels of lipoprotein(a), a risk factor for atherosclerosis (AS), are also associated with the presence of asymptomatic intracranial aneurysms. AS is present in some aneurysms, but its contribution to aneurysm formation and growth is unclear. Apolipoprotein(a) [apo(a)], the active moiety of lipoprotein(a), is present in atherosclerotic circle of Willis vessels but not in normal circle of Willis vessels. We wished to determine whether apo(a) is present in intracranial aneurysms independently of AS. METHODS With a purified anti-apo(a) monoclonal antibody, aneurysms (n = 25) and feeding vessels (n = 23) were examined for apo(a) expression by immunohistochemical analysis. Circle of Willis arteries with and without AS (n = 19), cavernous angiomas (n = 5), and arteriovenous malformations (n = 6) acted as control samples. RESULTS AS was present in 32% of aneurysms, and all of those aneurysms demonstrated mural immunopositivity for apo(a). However, aneurysms devoid of AS also demonstrated apo(a) immunopositivity. Apo(a) was demonstrated in 86% of available feeding vessels. Apo(a) deposition was not observed in cavernous angiomas but was present in arteriovenous malformations. Eleven Circle of Willis arteries (57.9%) were devoid of AS and demonstrated no apo(a) immunostaining, whereas the eight (42.1%) with AS were immunopositive for apo(a). CONCLUSION Apo(a) expression in intracranial aneurysms may occur independently of AS. Apo(a) in feeding vessels suggests a possible role for apo(a) in early events leading to aneurysm formation. Multilayered transmural apo(a) deposition in established aneurysms suggests apo(a) involvement in aneurysm growth, possibly via cycles of injury and repair. The absence of apo(a) in cavernous angiomas suggests that such injury might be pressure-mediated.

✓The authors describe a case of progressive neurological deficit caused by syringomyelia in a 7-year-old boy with a normally positioned conus medullaris. This deficit responded favorably to surgical untethering of the filum terminale, with subsequent clinical and radiological improvement. The authors discuss the implications of their findings in the context of the current understanding of the pathophysiology of tethered cord syndrome, particularly in relation to the ongoing debate in the neurosurgical literature.

The authors report two cases of spontaneous intracranial haemorrhage after elective craniotomy for resection of cerebral tumour. Both patients had mechanical aortic valve prostheses and were on regular warfarin therapy. In both cases, warfarin therapy was discontinued 5 days prior to surgery and unfractionated heparin administered intravenously until 12 h before surgery. Both patients were re-anticoagulated with subcutaneous low molecular weight heparin within the first week postcraniotomy-both developed life-threatening intracranial haemorrhage requiring urgent evacuation. The authors emphasize the risk of re-anticoagulation without postoperative imaging and the disadvantages of therapeutic dose, low molecular weight heparin in the postoperative period.

Pilocytic astrocytomas in the supratentorial compartment make up 20 % of all brain tumours in children with only 5 % of these arising in the suprasellar region. Optic pathway gliomas or suprasellar gliomas are often seen in neurofibromatosis type 1 (NF1) patients. Given their location, suprasellar pilocytic astrocytomas are challenging to manage surgically with high morbidity rates from surgical resection. We assess our cohort of patients with suprasellar pilocytic astrocytoma and document our experience.A retrospective review of patients diagnosed with suprasellar glioma between 2000-October 2012. We included patients diagnosed with optic pathway glioma based on radiological features (with or without biopsy) and those who had a biopsy confirming pilocytic astrocytoma.Fifty-three patients included (sporadic tumours 24 and NF1 related 29). Fifteen sporadic and four NF1 patients were biopsied. Twelve sporadic and 13 NF1 patients were initially treated with chemotherapy while only 1 patient had radiotherapy initially. Progression was noted in 58 % of the sporadic group and 24 % of the NF1 group. The only significant factor for progression was NF1 status (p = 0.026).Management should be guided by individual patient circumstance. In our cohort, chemotherapy did not significantly improve progression free survival; however, NF1 status significantly correlated with the decreased progression.

This report describes a novel method of repair for a large thoracolumbar myelomeningocele with an associated lumbar kyphosis in the neonate. A Caucasian male child was born at term with an antenatal diagnosis of hydrocephalus and spina bifida. Lumbar spine X-ray showed a significant kyphosis at L2–L3 level. Kyphectomy was performed and a cervical plate was used to reduce the gibbus deformity and maintain a rigid construct. Local rotation flaps were elevated and advanced to cover the defect. Wound was well healed at 3-month follow-up and the patient remained shunt-free at 1 year. To our knowledge, this is the first description of myelomeningocele repair with kyphectomy and posterior fixation in a neonate.

Ventriculoperitoneal (VP) shunt insertion is one of the most common neurosurgical procedures for the treatment of chronic hydrocephalus. Although regarded as a relatively benign procedure, several complications including obstruction, infection and mechanical failure can be seen during the postoperative stage. Symptomatic sterile cerebrospinal fluid (CSF) ascites and hydrothoracies are rare complications of VP shunt surgery. The paucity of cases makes identifying the aetiological factors difficult, particularly without catheter tip migration. It is most likely that several factors interact to reduce the absorption of CSF. The authors discuss the case of a 5-year-old girl who developed CSF ascites and a pleural effusion after starting chemotherapy for a suprasellar pilocytic astrocytoma, 2 years post-VP shunt insertion, due to a secondary obstructive hydrocephalus. After the initial management of the presenting symptoms, the child9s VP shunt was subsequently changed to a ventriculo-atrial shunt and the patient made an unremarkable recovery. We also review the literature pertaining to this rare complication, assessing identifiable risk factors and surgical management options.

LG-48. PAEDIATRIC LOW GRADE GLIOMAS IMPACT OF GROSS TOTAL RESECTION IN A SINGLE CENTRE COHORT Noelle Cullinan1, John Caird2, Michael Capra1, Jane Pears1, Cormac Owens1, andDarachCrimmins2; DepartmentofHaematology/Oncology,Our Lady’s Children’s Hospital, Dublin, Ireland; Department of Paediatric Neurosurgery, Children’s University Hospital, Temple Street, and Beaumont Hospital, Dublin, Ireland AIMS: Completely excised paediatric low grade gliomas (LGGs) are associated with excellent survival rates. Complete resection is not always achievable. We reviewed the Irish neurosurgical experience of paediatric LGGs over a 10-year period. METHODS: We performed a retrospective review of all paediatric patients with LGG diagnosed in the Republic of Ireland between 2005-2014. RESULTS: 112 children were diagnosed over a 10-year period (52 male (46%), 60 female (53%), Age range 0.23–15.87 years, Mean 7.03 years, Median 6.3 years). 111 children had surgical procedures – 14 (13%) had biopsies alone, 16 (14%) had biopsies followed by later resections, while 79 (71%) had upfront craniotomies with resection. We were unable to identify the type of procedure in 2 children. 1 child had a radiological diagnosis of aLGG but no confirmatorybiopsy.79 patientshadupfront craniotomies. In 77/79, all required information was available. 40/77 (52%) had Gross Total Resection (GTR) confirmed by post-operative imaging and 37/77 (48%) had incomplete resections. Of those who had GTR confirmed (excluding patients with Neurofibromatosis Type 1), 3 patients (4%) relapsed or had disease progression in follow-up, with early progression noted, all within 15 months after resection. All others are currently disease free in follow up (96%). CONCLUSION: In those with confirmed GTR on post-operative imaging, recurrence, if it occurred, was early. We propose that scanning intervals can be increased after 2 years and ongoing need for imaging surveillance in those with confirmed GTR should be reviewed. Neuro-Oncology 18:iii78–iii96, 2016. doi:10.1093/neuonc/now075.48 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

Aims We reviewed severe head injuries in children and adolescents who were treated in two national neurosurgical centres in 2013 and 2014. We studied the epidemiology, severity, care processes and clinical outcomes of these injuries. Methods The TARN (Trauma Audit and Research Network) database was used to collect data for admissions of 72 h or greater from two national neurosurgical centres. Results 117 patients with a severe head injury were admitted with a gender breakdown of 83 males and 34 females (2.4:1). 82 (61%) patients were transferred from another hospital. 64 (54%) of all head injuries were as a result of a fall, of which 45 (38%) were from less than 2 metres. Falls from less than 2 metres accounted for 27 (71%) injuries to children less than 2 years of age. Of 26 head injuries under 12 months of age, 5 (19%) were felt to be non-accidental injuries. There was a significant gender bias for mechanism of injury with 22 males and 8 females as a result of a vehicle collision (1.3:1) and 19 males and 1 female suffering a blow (19:1). The degree of head injury was scored using ISS (Injury Severity Score) and GCS (Glasgow Coma Scale). These showed that 85 (73%) had an ISS over 15, 19 (16%) between 9–15 and 13 (11%) between 1–8. 16 patients had a GCS under 8. The overall mortality was 5%. Morbidity was significant in that 21 survivors (18.9%) required intubation and 40 (34.1%) required one or more neurosurgical procedures. 21 (19%) patients had neuro-disability post-discharge all of which had an ISS > 15. Conclusion In our cohort, falls were the most common cause of severe head injury. The mortality in our cohort was 5% reflecting the experience in tertiary neurosurgical centres. The large burden of care that these injuries place on the hospital system is evident by the high number of neurosurgical interventions. It is evident that ISS is a good prognostic indicator as all those with neuro-disability scored greater than 15. In infants, non-accidental injury should always be considered.

We describe a case of acute functional deterioration in a 13-year-old girl with severe spastic diplegia (GMFCS III) and a new diagnosis of diffuse intrinsic pontine glioma (DIPG). She presented with acute deterioration in mobility and motor function over 1 month, which was associated with dysarthria, dysphagia and behavioural change. Her mother had noticed subtle functional deterioration over the 2 months prior to this. Her physiotherapist who was concerned about her acute functional deterioration referred her for emergency review. Neurological imaging revealed a diffuse pontine lesion consistent with DIPG. She was subsequently referred to oncology. She deteriorated further, clinically, over the next few days and following discussion with the team; her family opted for palliative treatment, given the poor prognosis associated with DIPG.

One-third of all primary central nervous system tumors in adults are meningiomas. Rarely, meningiomas occur at multiple sites, usually occurring in individuals with type 2 neurofibromatosis (NF2). We sequenced the exomes of three unrelated individuals with familial multiple spinal meningiomas without NF2 mutations. We identified two individuals with heterozygous loss-of-function mutations in the SWI/SNF chromatin-remodeling complex subunit gene SMARCE1. Sequencing of SMARCE1 in six further individuals with spinal meningiomas identified two additional heterozygous loss-of-function mutations. Tumors from individuals with SMARCE1 mutations were of clear-cell histological subtype, and all had loss of SMARCE1 protein, consistent with a tumor suppressor mechanism. Our findings identify multiple-spinal-meningioma disease as a new discrete entity and establish a key role for the SWI/SNF complex in the pathogenesis of both meningiomas and tumors with clear-cell histology.

Although uncommon, there is significant morbidity and mortality associated with paediatric spinal glioblastoma. The paucity of cases makes treatment options difficult. The current recommended standard of care is biopsy followed by adjuvant chemo-radiotherapy, with emerging data supporting the role of safe gross total resection. The purpose of this paper is to provide a single-institution case study and to discuss current and future therapeutic treatment strategies. A 14-year-old boy presented with a 2-year history of intermittent back pain with recent progressively worsening motor and sensory deficits of the right side. Pre-operative MRI revealed an enhancing intra-medullary tumour extending from C2 to C7. During the operative case, no tumour–cord margin could be identified, and the patient underwent a subtotal excision. Histopathology confirmed glioblastoma. In the subsequent weeks, the patient's clinical condition deteriorated. Adjuvant therapy was declined by the family, and the patient died 9 weeks after initial presentation. Despite major advances in surgical techniques, peri-operative neuro-imaging as well as chemo-radiotherapy, the prognosis of a paediatric intra-medullary high-grade spinal tumour remains poor. Detailed analysis of our understanding of tumour dynamics in this patient group is important in establishing future therapeutic strategies.

Intra-extracranial schwannomas arising unrelated to major cranial nerves are exceedingly rare neoplasms. We report the case of a 23-year-old male who presented with a 9 month history of progressive temporal swelling which was excised and found histologically to be a schwannoma. A succinct review of the relevant literature is presented.