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Fetal ventriculomegaly is associated with varying degrees of genetic and structural abnormalities. The objective was to present the experience of fetal ventriculomegaly in a large European center in relation to: 1. grade of ventriculomegaly; 2. additional chromosomal/structural abnormalities; and 3. perinatal survival rates.This was a prospective observational study of patients referred with fetal ventriculomegaly from January 2011 to July 2020. Data were obtained from the hospital database and analyzed to determine the rate of isolated ventriculomegaly, associated structural abnormalities, chromosomal/genetic abnormalities, and survival rates. Data were stratified into three groups; mild (Vp = 10-12 mm), moderate (Vp = 13-15 mm) and severe (Vp 15 mm) ventriculomegaly.There were 213 fetuses included for analysis. Of these 42.7% had mild ventriculomegaly, 44.6% severe and 12.7% had moderate ventriculomegaly. Initial ultrasound assessment reported isolated ventriculomegaly in 45.5% fetuses, with additional structural abnormalities in 54.5%. The rate of chromosomal/genetic abnormalities was high,16.4%. After all investigations, the true rate of isolated VM was 36.1%. The overall survival was 85.6%. Survival was higher for those with isolated VM across all groups (P 0.05).Ventriculomegaly is a complex condition and patients should be counselled that even with apparently isolated VM, there remains the possibility of additional genetic and/or structural problems being diagnosed in up to 10% of fetuses.

We present the case of a pregnant 32-year-old woman who presented with a giant fetal facial tumour at 22 weeks. The mass, initially 4 × 3.5 × 3 cm in size, was largely cystic with a small solid component. It subsequently increased to 9 × 9 × 10 cm. Significant compression effects on the fetal orbit, temple and infratemporal fossa, with potential compression of the optic nerve, were noted on ultrasound and MRI. The cyst required drainage twice in the pregnancy: firstly to reduce the compression effects and secondly to facilitate caesarean delivery. Postnatally, the baby had significant compression and displacement of the craniofacial skeleton from the mass effect. Postnatal histology revealed a diagnosis of a teratoma. This case highlights the complexities and challenges surrounding the diagnosis and management of a giant fetal facial tumour.

Severe fetal ventriculomegaly (VM) is defined as an enlargement of the atria of the lateral cerebral ventricles (Vp) of greater than 15 mm. While it is well established that it confers significant risk of morbidity and mortality to the neonate, there is limited information pertaining to the caesarean delivery rates and the obstetric management of these complex cases. The aim of this study was twofold: firstly, to determine survival rates in fetuses with severe VM, and secondly to determine the caesarean delivery rates in continuing pregnancies. We explore the obstetric challenges associated with these difficult cases.This was a prospective observational study of patients with antenatal severe VM, attending the Department of Fetal Medicine, National Maternity Hospital, Dublin, Ireland, from 1st January 2011 to 31st July 2020. Data were obtained from the hospital database and those with severe VM (Vp 15 mm) were identified. The rates of chromosomal abnormalities, the survival rates and the caesarean delivery (CD) rates for the overall group were then determined. The data were then further sub-divided into two groups: 1. Vp 20 mm and 2. Vp 20 mm, and the results compared. Statistical analysis was performed using the Chi-Square test.A total of N = 95 pregnancies with severe VM were included for analysis, of which additional structural abnormalities on ultrasound were apparent in 67/95 (70.5%) and 28/95 (29.5%) had isolated severe VM. Chromosomal abnormalities were diagnosed in 15/95 (15.8%) of cases, with (2/28) 7.1% in the isolated SVM group versus (13/67) 19.4% in the non-isolated SVM group. The overall survival rate (excluding TOP) was 53/74 (71.6%), with 20/23 (86.9%) in the isolated SVM group. The overall CD rate was 47/72 (65.3%), which was significantly higher than the CD for the hospital during the same time period of 25.4% (P 0.01). The data were subdivided into Vp 20 and Vp 20 and those with a Vp 20 had higher rates of additional intracranial findings on ultrasound (Vp 20 13/41 (31.7%) versus Vp 20 32/54 (59.3%) (P 0.05)) and macrocrania (Vp 20 14/41 (34.1%) versus Vp 20 35/54 (64.8%) (P 0.05)). No significant difference was observed in the overall survival or CD rates between the two groups.In conclusion this study reports significant fetal morbidity and mortality with severe VM with high CD rates observed in this cohort. Significant challenges exist in relation to the obstetric management and counseling of parents regarding an often uncertain neonatal prognosis. In continuing pregnancies with significant macrocrania delivery plans should be individualized to improve neonatal outcomes where possible and minimize harm to the mother.

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.

Biomarkers such as calcium channel binding protein S100 subunit beta (S100B), glial fibrillary acidic protein (GFAP), ubiquitin c-terminal hydrolase L1 (UCH-L1) and neuron-specific enolase (NSE) have been proposed to aid in screening patients presenting with mild traumatic brain injury (mTBI). As such, we aimed to characterise their accuracy at various thresholds. MEDLINE, SCOPUS and EMBASE were searched, and articles reporting the diagnostic performance of included biomarkers were eligible for inclusion. Risk of bias was assessed using the QUADAS-II criteria. A meta-analysis was performed to assess the predictive value of biomarkers for imaging abnormalities on CT. A total of 2939 citations were identified, and 38 studies were included. Thirty-two studies reported data for S100B. At its conventional threshold of 0.1 μg/L, S100B had a pooled sensitivity of 91% (95%CI 87-94) and a specificity of 30% (95%CI 26-34). The optimal threshold for S100B was 0.72 μg/L, with a sensitivity of 61% (95% CI 50-72) and a specificity of 69% (95% CI 64-74). Nine studies reported data for GFAP. The optimal threshold for GFAP was 626 pg/mL, at which the sensitivity was 71% (95%CI 41-91) and specificity was 71% (95%CI 43-90). Sensitivity of GFAP was maximised at a threshold of 22 pg/mL, which had a sensitivity of 93% (95%CI 73-99) and a specificity of 36% (95%CI 12-68%). Three studies reported data for NSE and two studies for UCH-L1, which precluded meta-analysis. There is evidence to support the use of S100B as a screening tool in mild TBI, and potential advantages to the use of GFAP, which requires further investigation.

Paediatric high grade glioma and diffuse midline glioma (including DIPG) are comprised of multiple biological and clinical subgroups, the majority of which urgently require novel therapies. Patient-derivedin vitroprimary cell cultures represent potentially useful tools for mechanistic and preclinical investigation based upon their retention of key features of tumour subgroups under experimental conditions amenable to high-throughput approaches. We present 17 novel primary cultures derived from patients in London, Dublin and Belfast, and together with cultures established or shared from Barcelona, Brisbane, Rome and Stanford, assembled a panel of 52 models under 2D (laminin matrix) and/or 3D (neurospheres) conditions, fully credentialed by phenotypic and molecular comparison to the original tumour sample (methylation BeadArray, panel/exome sequencing, RNAseq). In screening a subset of these against a panel of ~400 approved chemotherapeutics and small molecules, we identified specific dependencies associated with tumour subgroups and/or specific molecular markers. These includedMYCN-amplified cells and ATM/DNA-PK inhibitors, and DIPGs withPPM1Dactivating truncating mutations and inhibitors of MDM2 or PARP1. Specific mutations inPDGFRAwere found to confer sensitivity to a range of RTK inhibitors, though not all such mutations conferred sensitivity to targeted agents. Notably, dual PDGFRA/FGFR and downstream pathway MEK inhibitors showed profound effects against both PDGFRA-sensitising mutant and FGFR1-dependent non-brainstem pHGG and DIPG. In total, 85% cells were found to have at least one drug screening hit in short term assays linked to the underlying biology of the patient’s tumour, providing a rational approach for individualised clinical translation.

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.

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.

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.

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.

Background To date, children with hydrocephalus continue to have a considerable long-term outcome. However, current literature on health-related quality of life (HRQOL) among children with hydrocephalus are limited. This serves a call for research to validate a suitable HRQOL for children with hydrocephalus measuring the physical, emotional, social and cognitive well being. Objective To validate a technology-enhanced quality of life questionnaire (EITVAQ) as an effective assessment tool measuring the current well-being of a child with hydrocephalus (child-centred) To adopt a technology-enhanced quality of life assessment tool (EITVAQ) into a phone/tablet application to gain widespread usage To explore the use of technology to increase the learning potential and obtaining a new skill among children with hydrocephalus Methods This is a prospective study which took place since January 2018 to January 2019. This study received ethical approval All data was compiled and analysed using SPSS. The validation process involved: Content Validity, Feasibility and Reliability. A cohort of 70 healthy children from various primary and secondary schools participated as a control. Finally, a small cohort of children with hydrocephalus participated in technology-enhanced learning programme (TELP) to learn a new music skill and created a music video. Results This study reports the result of the validation process. In total there were 132 participants. Content validity was achieved by the nominal group technique. In this study, technology enhanced quality of life questionnaire (EITVAQ) is a feasible tool, having a high satisfaction rate of 78%, a response rate of 76.5% and an average completion time of 4 mins and 26 seconds. EITVAQ had a score of 0.92 (Cronbach Alpha). When comparing with the control, the two lowest scores were the social and emotional aspect. The two major differences in scores among both groups were the physical and cognitive well-being. All participants who participated in the TELP managed to complete the task learning the skills of creating a music video. Conclusion EITVAQ, an interactive and child-friendly tool to assess quality of life is now validated. It aims to be used widely among children with hydrocephalus, providing a baseline assessment to allow us to understand more about a child’s quality of life from their own perspective. This study concludes that technology has a huge potential in helping children with hydrocephalus and various neuro-disability to integrate into society.

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.

Dandy–Walker Syndrome (DWS) is a constellation of congenital anomalies of the central nervous system consisting of cerebellar vermis hypoplasia or agenesis, cystic dilatation of the fourth ventricle, enlarged posterior fossa, and a high tentorium. Hydrocephalus is a common occurrence in DWS and its treatment varies between shunting-ventriculoperitoneal or cystoperitoneal or both, endoscopic cyst fenestration, and third ventriculostomy. Chronic cerebral herniation is a known complication of treatment in DWS; however, we present an unusual case of central brain herniation. A 2 year old boy with shunted DWS presented with status epilepticus. Initial CT brain scan showed no increase in ventricle or cyst size; however, tapping the shunt reservoir did not yield any CSF prompting a shunt revision. Postoperatively, he was very slow to wake and subsequently experienced an episode of fixed pupils and extensor posturing. MRI brain demonstrated severe herniation of both thalami through the tentorium. This child had chronic central brain herniation (CCBH) secondary to the shunting of his cyst. To our knowledge, this is the first case of CCBH following treatment of DW cyst

Object McLone and Knepper's unified theory of Chiari malformation Type II (CM-II) describes how the loss of CSF via the open posterior neuropore fails to create adequate distending pressure for the developing rhomboencephalic vesicle. The authors of the present article describe the relationship between the posterior fossa volume and intracranial cerebellar volume as being related to the distance from the obex of the fourth ventricle to the myelomeningocele lesion using a common mathematical model, the Hagen-Poiseuille law. Methods All newborns who required closure of a myelomeningocele at the authors' institution between 2008 and 2011 and who were between 4 weeks premature and 2 months, corrected gestational age, at the time of MRI were included in this study. Volumes and measurements were obtained from axial and sagittal T2-weighted MR images of the brain and spine. Results: A total of 56 newborn infants met the inclusion criteria. There was a direct linear relationship between both posterior fossa volume and cerebellar volume and the spinal level of the myelomeningocele lesion (p = 0.0012 and p = 0.0041, respectively). There was a negative linear relationship between the cerebellar descent, the spinal level of the lesion, and posterior fossa volume and cerebellar volume. These relationships strengthen in patients with no syringomyelia and are not significant in those groups with syringomyelia. The results of a 1-way ANOVA for the 3 groups did not reach significance. Conclusions Using a linear equation derived from the Hagen-Poiseuille law that describes pressure in the fourth ventricle as being directly related to the length of the central canal from the obex to the myelomeningocele lesion, the authors were able to explain the directly observed linear relation between posterior fossa volume, intracranial cerebellar volume, and cerebellar descent to the level of the spinal lesion. As this model assumes a uniform radius of the central canal they were able to validate this model when they observed a strengthening in relationships in the no syringomyelia group and statistically insignificant correlations in the groups with syringomyelia. They therefore propose that the spinal level of the lesion is one of the major determinants of posterior fossa volume, intracranial cerebellar volume, and cerebellar ectopia.

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.