Patient advice regarding participation in sport in children with disorders of cerebrospinal fluid (CSF) circulation: a national survey of British paediatric neurosurgeons.

BACKGROUND: Management of children with disorders of cerebrospinal fluid (CSF) circulation is a common aspect of paediatric neurosurgical practice. Sport and physical activity play an integral role in the lives of patients in this age group. However, there is little evidence to support the dissemination of appropriate advice to children regarding such activities. The aim of this study was to evaluate the perspectives of clinicians across the UK regarding the participation of children with disorders of CSF circulation in sports. METHODS: Questionnaires were distributed to Consultant Paediatric Neurosurgeons practising across the UK via the Society of British Neurological Surgeons (SBNS). Five different patient scenarios were supplied, and participants were asked to choose whether they would advise participation in the following sports: Taekwondo, rugby, skiing, and football. RESULTS: An overall response rate of 66.7% (36 out of 54 paediatric neurosurgeons) was achieved. The following percentages of clinicians advocated football, rugby, Taekwondo, and skiing across all scenarios: 96%, 75%, 77%, and 97%, respectively. The majority of responders (91.2%) relied on personal experience when providing advice, whilst 50% used available literature and 19.4% used available guidelines. CONCLUSIONS: There is a paucity of evidence in the literature to support the dissemination of appropriate advice to children with disorders of CSF circulation regarding participation in sports. Our findings demonstrate that the majority of clinicians rely on personal experience to make such decisions, emphasizing the necessity of larger scale studies to inform evidence-based guidelines.

Subdural haematoma in neonates following forceps-assisted delivery: case series and review of the literature.

PURPOSE: Subdural haematoma (SDH) is a recognised complication of forceps-assisted delivery (FAD). There are no guidelines regarding its management. This study aims to provide a better insight into the management and outcomes of neonatal SDH post-FAD. METHODS: Retrospective review of our neonatal database and systematic review of the literature for neonatal cases that presented with SDH after FAD. Retrospective neurosurgical database search for cases of neonatal SDH post-FAD managed in our unit between January 2007 and January 2017. Systematic review of the literature was performed using PRISMA guidelines. The inclusion criteria are as follows: (1) neonates; (2) forceps-assisted delivery; (3) evidence of SDH on imaging, with or without other traumatic lesions. RESULTS: A literature search yielded nine studies with 30 patients meeting our inclusion criteria. In addition, four cases were identified from our institutional database. Forty-two percent (n = 14) had their SDH managed surgically, with subsequent full neurological recovery in 57%. In comparison, 95% (n = 18) of the conservatively managed patients made a full recovery. Hydrocephalus was present in 1/19 and 11/14 of the conservatively managed and surgically managed patients respectively. CONCLUSIONS: Conservative management can lead to a full neurological recovery in SDH following FAD in neonates. However, a significant minority may still need neurosurgical intervention for the SDH or subsequent hydrocephalus; therefore, we advocate early transfer to a specialist neuroscience centre.

Neurosurgical aspects of abusive head trauma management in children: a review for the training neurosurgeon.

The management of head trauma in children from suspected abuse is multidisciplinary. The primary role of neurosurgeons is to manage the acute clinical situation. They also have a secondary role in assisting others to determine the cause of the injury and prevent recurrences. This article aims to guide the trainee neurosurgeon on management of this patient group according to current literature.

One year failure rates for de-novo ventriculo-peritoneal shunts in under 3-month-old children.

Introduction: Ventriculo-peritoneal shunts (VPS) are still the mainstay treatment for hydrocephalus in children. It is generally accepted that VPS failure and infection rates are higher for neonates than for older children. We compared our 1-year failure and infection rates in under 3-month-old children compared with older children in our department. Results: We identified 58 children under 3 months of age who underwent VPS insertion between January 2007 and December 2016. They had a 29.3% (17) shunt failure rate over the first year. There were two confirmed shunt infections (3.4%). Discussion: The 1-year shunt failure rate at our institution for VPS insertion in children over 3 months is 26.1% and the infection rate is 4.3% (9). The literature suggests that the outcome for VPS in younger children is worse than for older children. Our work shows similar outcomes for all children compared to those under 3 months at time of VPS insertion alone. Conclusion: Children under 3-months-old undergoing VPS insertion should not automatically expect an increased 1-year failure or infection rate compared with older children. The reasons for this may be as a result of increased subspecialisation, the more widespread use of antibiotic-impregnated catheters and improved neonatal care.

Pituitary xanthogranulomas: clinical features, radiological appearances and post-operative outcomes.

BACKGROUND: Xanthogranulomas are inflammatory masses most commonly found at peripheral sites such as the skin. Sellar and parasellar xanthogranulomas are rare and present a diagnostic challenge as they are difficult to differentiate from other sellar lesions such as craniopharyngiomas and Rathke's cleft cysts pre-operatively. Their radiological imaging features are yet to be clearly defined, and clinical outcomes after surgery are also uncertain. This study reviews clinical presentation, radiological appearances, and clinical outcomes in a cohort of patients with pituitary xanthogranulomas. METHODS: A prospectively maintained pituitary surgery database was screened for histologically confirmed pituitary xanthogranulomas between May 2011-December 2016. Retrospective case note assessments were then performed by three independent reviewers. Patient demographics, clinical presentations, imaging, and clinical outcomes were analysed. RESULTS: During the study period 295 endoscopic endonasal pituitary surgeries were performed. Six patients had confirmed pituitary xanthogranulomas (2%). Patients most commonly presented with visual field deficits and/or endocrine dysfunction. Common imaging features included: a cystic consistency, hyperintensity on T1-weighted MR images, and contrast enhancement either peripherally (n = 3) or homogenously (n = 3). The most common pre-operative endocrine deficits were hyperprolactinaemia and hypoadrenalism (at least one of which was identified in 4/6 patients; 66%). Thirty-three percent (2/6) of patients presented with diabetes insipidus. The most common post-operative endocrinological deficits were adrenocortical dysfunction (66%) and gonadotropin deficiency (66%). Visual assessments normalised in all six patients post-operatively. Gross total resection was achieved in all patients, and at median follow up of 33.5 months there were no cases of tumour recurrence. CONCLUSIONS: The prevalence of pituitary xanthogranulomas in our series is higher than that suggested in the literature. Surgery restored normal vision to all cases, however four patients (67%) required long-term hormonal replacement post-operatively. Imaging features such peripheral rim enhancement, a suprasellar tumour epicentre, and the absence of both calcification or cavernous sinus invasion were identified as potential indicators that together should alert clinicians to the possibility of pituitary xanthogranuloma when assessing patients with cystic sellar and parasellar tumours.

Neurocutaneous melanosis presenting with hydrocephalus and malignant transformation: case-based update.

INTRODUCTION: Neurocutaneous melanosis (NCM) is a sporadic condition characterised by congenital melanocytic nevi and melanocytic thickening of the leptomeninges. It is believed to result from congenital dysplasia of melanin-producing cells within the skin and leptomeninges. The management of cutaneous manifestations remains controversial; for neurological manifestations, outcome remains poor even with the use of radiotherapy and chemotherapy. PATIENTS AND METHODS: We describe the case of a 5-month-old boy who presented with giant congenital melanocytic nevus and hydrocephalus. MR imaging and CSF immunohistochemistry confirmed leptomeningeal melanosis. We discuss the diagnosis, treatment and prognosis of this rare disorder in the light of recent published literature. RESULTS: Patient required placement of right-sided ventriculoperitoneal shunt to control hydrocephalus. The patient tolerated the procedure well and was discharged home with normal neurological function. A presumptive diagnosis of NCM was made based on the MR characteristics, CSF cytology and clinical presentation. He received trametinib, a MAPK/Erk kinase inhibitor for 7 months. At 30 months of age, he developed left-sided weakness and status epilepticus requiring paediatric intensive care unit admission and ventilator support. The patient eventually succumbed to malignant transformation of leptomeningeal disease. CONCLUSION: Cutaneous manifestations of NCM are usually congenital, and neurological manifestations develop early in life. Patients with large or multiple congenital nevi should therefore be investigated early to facilitate treatment. MR imaging is the investigation of choice which can further assist in performing biopsy. Symptomatic NCM is refractory to radiotherapy and chemotherapy and has a poor prognosis. A multidisciplinary approach is necessary in the management of NCM patients.

Outcomes of ventriculoperitoneal shunt insertion in the management of idiopathic intracranial hypertension in children.

PURPOSE: The ventriculoperitoneal (VP) shunt has become the procedure of choice for treatment of idiopathic intracranial hypertension (IIH). We aimed to assess the efficacy of frameless stereotactic placement of VP shunts for the management of medically resistant IIH in children and to assess the role of gender and obesity in the aetiology of the condition. METHODS: This is a retrospective analysis of the case notes of 10 patients treated surgically at the University Hospital of Wales in Cardiff, from May 2006 to September 2012. RESULTS: VP shunts were successful in relieving headache, papilloedema and stabilising vision. No sex predilection was identified, and increased BMI was a feature throughout the population, regardless of age. CONCLUSIONS: Neuronavigated VP shunt insertion is an effective mode of treatment for medically resistant IIH in children. The aetiological picture in children does not seem to be dominated by obesity, as in adults. Literature on childhood IIH is sparse, and larger scale, comparative studies would be of benefit to treating clinicians.

Ventriculo-peritoneal shunt independence following successful treatment of Gram negative (E. coli) ventriculitis: Case report and review of the literature.

We report a case of tumour-related hydrocephalus in a child treated with a ventriculo-peritoneal shunt which subsequently became infected with gram negative bacteria (Escherichia coli). After successful treatment of the infection the patient became shunt independent and has remained so for over 2 years. Gram negative ventriculitis is associated with diminished cerebro-spinal fluid production and we discuss the literature to date regarding this phenomenon.

A rare case of multicystic disseminated astrocytoma with pilomyxoid characteristics in a 4-year-old child.

Pilomyxoid astrocytomas are a more aggressive variant of pilocytic astrocytoma. Over the last 14 years, there has been increasing evidence to suggest that these tumours are distinct pathological entities to pilocytic astrocytomas. Radiological features of these tumours are slowly emerging in the neuroradiological literature. We report a unique radiological appearance of a multicystic, disseminated astrocytoma with pilomyxoid characteristics presenting in a 4-year-old boy and highlight the importance of considering this diagnosis with similar imaging.

Vagal nerve stimulation in children under 12 years old with medically intractable epilepsy.

OBJECTIVE: This study aims to assess the efficacy and safety of vagal nerve stimulation (VNS) in children less than 12 years old operated on at the University Hospital Wales. METHOD: Retrospective review of patients undergoing VNS insertion, over a 3-year period, was undertaken. All children had a minimum follow-up period of 2 years. Sixteen patients were identified via the paediatric epilepsy surgery database. A case note review and telephone evaluation was conducted. Seizure frequency using the McHugh classification was the primary outcome measure, with anti-epileptic drug (AED) use as a secondary outcome measure. RESULTS: There were 10 males and 6 females. The mean time with epilepsy prior to surgery was 5.7 years and the mean age at the time of surgery was 7.6 years. Overall, nine (56 %) children experienced a reduction in their seizure frequency of 50 % or more. Of these, four (25 %) had a reduction of more than 80 %. Seven children (44 %) had no reduction in their seizure frequency, although two of these patients reported benefit regarding seizure control and post-ictal recovery. The VNS system was removed in two patients due to infection and no benefit, respectively. Half of the cohort (50 %) reduced the number of anti-epileptic drugs post-surgery, and there was an overall mean reduction of AED of 0.5. CONCLUSION: This study suggests that VNS is a safe and effective adjuvant therapy in children under 12 years old, with over half reporting significant benefit. Further studies are needed to enable preoperative selection of patients in order to maximise the potential benefit.

The influence of different forms of early childcare on children's emotional and behavioural development at school entry.

BACKGROUND: Over the past few decades there has been a dramatic increase in maternal employment and, as a result, an increase in the use of non-maternal childcare in the early years. The purpose of this longitudinal study was to examine, in a large representative English sample, the influence of different forms of childcare on children's behavioural and emotional development around the age of school entry. METHODS: A sample of 991 families, originally recruited when the children were 3 months old, was assessed around school entry age at 51 months. The main outcome variable was the children's emotional and behavioural functioning, measured by questionnaire completed by both mothers and teachers. A range of repeated assessments were carried out at different time points, including direct observation of the quality of maternal caregiving and observations of the quality of non-parental care, and amount of time spent in different forms of care. RESULTS: The strongest and most consistent influences on behaviour and emotional problems were derived from the home, including lower socio-demographic status, poorer maternal caregiving, parental stress/maternal mental health problems, as well as child gender (being a boy). Non-parental childcare had small effects on child outcome. One finding that did emerge was that children who spent more time in group care, mainly nursery care, were more likely to have behavioural problems, particularly hyperactivity. CONCLUSIONS: These findings suggest that interventions to enhance children's emotional and behavioural development might best focus on supporting families and augmenting the quality of care in the home.

The incidence of infection for adults undergoing supra-tentorial craniotomy for tumours without hair removal.

AIMS & OBJECTIVES: The aim of the study was to look at the infection rate in adults undergoing craniotomies without hair removal and compare the results with the usual practice of pre-operative shaving/clipping. MATERIALS & METHODS: One-hundred consecutive adult patients who had elective supra-tentorial craniotomy for tumour were studied prospectively. A uniform policy of performing surgery without hair removal was adopted. Patients were followed up prospectively to look for surgical site infection. The rate of infection was determined and the results were compared with the published data on similar procedures where hair removal was carried out before surgery. RESULTS: A total of three patients developed surgical-site infection. One infection occurred in the glioma and two in the meningioma subgroup. The rate of infection is comparable to the published figures where pre-operative hair removal was performed routinely. CONCLUSIONS: Cranial surgery with hair left in place does not pre-dispose to an increased infection risk for adults undergoing tumour surgery.

One year failure rate for de-novo ventriculo-peritoneal shunts in children from a small volume paediatric neurosurgical unit.

It is the belief of some that results of shunt surgery for the treatment of hydrocephalus in children are improved if the surgery is performed in high-volume centres. Currently in the UK paediatric neurosurgery is undergoing a service review. As part of this review a set of standards of care are being drafted which state that 1-year failure rates and infection rates for de-novo ventriculo-peritoneal shunts in children should be less than 40% and 10%, respectively. Our de-novo shunt infection rate (4.3%) and our 1-year failure rate (28.6%) are well within the standards set by this process and comparable to published literature from much higher volume centres.

The efficacy of endoscopic third ventriculostomy in children 1 year of age or younger: A systematic review and meta-analysis.

PURPOSE: Hydrocephalus is a major cause of morbidity in the pediatric population, with potentially severe consequences if left untreated. Two viable strategies for management of non-communicating hydrocephalus are endoscopic third ventriculostomy (ETV) and ventriculoperitoneal shunting. However, there is uncertainty over the safety and efficacy of ETV in younger infants aged 1 year or below. In this systematic review, we aim to elucidate the success rate and procedural risks of ETV in this age group. METHODS: A multi-database (PubMed, Embase, Web of Science) literature search between January 1990 and April 2018 was performed in accordance with PRISMA guidelines. Eligible studies were included if they (i) examined non-communicating hydrocephalus; (ii) quantified the success/failure rates of ETV; and (iii) assessed outcomes in children 1 year of age or younger. RESULTS: A total of 19 articles with 399 patients were eligible for inclusion. Mean age at procedure was 4.2 months (range 34 weeks gestation to 12 months), with 116 females and 143 males. Commonest underlying aetiology was congenital aqueductal stenosis (AS) (60.4%). Remaining causes included post-haemorrhagic, post-infection, Chiari malformations, malignancies and others. Overall and AS mean success rates were 51.6% and 56.5% respectively. Overall complication rate was 10.0%, consisting mainly of CSF leak, infection, and haemorrhage. Younger age was significantly associated with poorer ETV success rate when divided into <6 months and 6-12 months of age (44.4 vs 66.7%; p = 0.0007). Underlying pathology had no significant association with ETV outcome when divided into AS and other pathologies (p = 0.53). CONCLUSIONS: Age is significantly associated with ETV success rates. Pathology-dependent effects were not found in this age group. Despite a lower ETV success rate at younger ages (44.4 vs 66.7%), it offers a comparable safety profile that is independent of age. ETV remains a viable treatment option for non-communicating hydrocephalus for infants aged 1 year or younger.

A comparison of father-infant interaction between primary and non-primary care giving fathers.

BACKGROUND: This study examined the socio-demographic characteristics and attitudes of primary care giving fathers and non-primary care giving fathers and the quality of their interaction with their infants. METHOD: Two groups of fathers of 11.9-month old infants were compared - 25 primary care giving fathers (20 h per week or more of sole infant care) and 75 non-primary care giving fathers - with regard to socio-demographic characteristics, attitudinal differences and father-infant interaction during play and mealtimes. The quality of father-child interaction in relation to the total number of hours of primary care provided by fathers was also examined. RESULTS: Primary care giving fathers had lower occupational status and earned a smaller proportion of the family income but did not differ in educational level or attitudes compared with non-primary care giving fathers. There were no differences between the partners of the two groups of fathers on any variables, and their infants did not differ in temperament. Primary care giving fathers and their infants exhibited more positive emotional tone during play than non-primary care giving fathers, although fathers did not differ in responsivity. There were no differences between the groups during mealtimes. There was a positive association between total number of child care hours provided by all fathers and infant positive emotional tone. CONCLUSIONS: Primary and non-primary care giving fathers were similar in many respects, but primary care giving fathers and their infants were happier during play. This suggests a possible link between the involvement of fathers in the care of their children and their children's emotional state. The finding of a trend towards increased paternal happiness with increased hours of child care suggests that there may also be a gain for fathers who are more involved in the care of their infants. Further research is needed to determine whether these differences ultimately have an effect on children's development.

Why do some fathers become primary caregivers for their infants? A qualitative study.

BACKGROUND: Despite recent advances, research on father-infant relations is still relatively lacking. One hitherto neglected area is the subjective experience of primary caregiving (PCG) fathers, including their reasons for taking on a PCG role. METHOD: Twenty-five PCG fathers, defined as those providing sole child care for their 1-year-old infant for at least 20 waking hours a week, completed semi-structured interviews as part of a large two-centre English study of child care. Interviews were tape-recorded, transcribed and analysed using emergent theme analysis. Themes with a bearing on the fathers' explicit and implicit reasons for providing relatively high levels of sole child care were grouped using an iterative process of independent analysis, comparison and rechecking. RESULTS: Reasons included mother's and father's employment situation and relative earning power, the absence of acceptable alternative child care, perceptions of societal values, maternal and paternal health, family history and ideological values. Flexibility of paternal employment, or its relative unimportance, and motivation or willingness on the part of the father to share in child care were invariably present. For some, the fact of one or other of their own parents having been emotionally distant or physically absent had led to a determination to do things differently with their own children. Fathers reported that they valued the increased time spent with their child, even if they had not been consciously motivated beforehand. CONCLUSIONS: Fathers describe a wide range of reasons for becoming primary caregivers for their infant, and generally experience themselves as being actively involved in that decision. If fathers are to take an increasing role in child care, societal values and financial pressure may need to be addressed.

Spaced training improves learning in Ts65Dn and Ube3a mouse models of intellectual disabilities.

Benefits of distributed learning strategies have been extensively described in the human literature, but minimally investigated in intellectual disability syndromes. We tested the hypothesis that training trials spaced apart in time could improve learning in two distinct genetic mouse models of neurodevelopmental disorders characterized by intellectual impairments. As compared to training with massed trials, spaced training significantly improved learning in both the Ts65Dn trisomy mouse model of Down syndrome and the maternally inherited Ube3a mutant mouse model of Angelman syndrome. Spacing the training trials at 1 h intervals accelerated acquisition of three cognitive tasks by Ts65Dn mice: (1) object location memory, (2) novel object recognition, (3) water maze spatial learning. Further, (4) spaced training improved water maze spatial learning by Ube3a mice. In contrast, (5) cerebellar-mediated rotarod motor learning was not improved by spaced training. Corroborations in three assays, conducted in two model systems, replicated within and across two laboratories, confirm the strength of the findings. Our results indicate strong translational relevance of a behavioral intervention strategy for improving the standard of care in treating the learning difficulties that are characteristic and clinically intractable features of many neurodevelopmental disorders.

Choreathetoid movement as an unusual presentation of subdural haematoma.

Subdural haematomas can present with a wide variety of symptoms. An atypical presentation can be movement disorders. The key feature is that the history of onset is more rapid than with neurological conditions such as Parkinson's disease. The symptoms can also be an acute worsening of pre-existing disorder. The case discussed involved an 80 year old woman with bilateral choreathetoid movements attributed to a unilateral chronic subdural haematoma. The haematoma was surgically drained and the symptoms resolved. Sporadic reports of similar cases of movement disorders associated with subdural haematomas exist in the literature, but rarely do unilateral haematomas present with bilateral symptoms. Pressure effects, neurotransmitter abnormality and ischaemia have been postulated as reasons for this type of presentation. Unexplained and sudden movement disorders might warrant imaging to rule out a subdural haematoma as the cause.

The influence of maternal depression, caregiving, and socioeconomic status in the post-natal year on children's language development.

BACKGROUND: Post-natal depression is common and has been associated with adverse effects on children's later emotional and behavioural development. The evidence for effects on children's cognitive development is unclear but this could potentially be a major public health issue. The aim was to examine whether maternal depression and maternal caregiving during the first year of life are associated with children's subsequent language development. METHODS: One thousand two hundred and one women were recruited from antenatal and post-natal baby clinics in two areas in England, and followed up until their babies were 3 years. Mothers and children were assessed by questionnaire, interview and home observation; 999 children's language was assessed at 36 months, although 55 were excluded principally because they had been exposed to insufficient English. RESULTS: In bivariate analyses maternal depressive symptomatology in the post-natal year but not at 36 months was associated with poorer child language at 36 months; maternal caregiving, was positively associated with language. Structural Equation Modelling revealed that depression was associated with poorer caregiving but was not independently associated with language. Higher quality caregiving at 10 months was associated with better language. When the sample was split by socioeconomic factors the effects of depression on caregiving were stronger in the less advantaged group. In both groups poorer quality early caregiving predicted lower language outcome. CONCLUSIONS: Post-natal depression had a negative effect on caregiving, which in turn affected language; post-natal depression did not have an additional direct effect on language. Socioeconomic factors moderated the effects of depression on caregiving. When targeting interventions at mothers with post-natal depression, it may be strategic to focus on lower socioeconomic groups at higher risk.

Diffuse brainstem gliomas in children: should we or shouldn't we biopsy?

The decision to biopsy diffuse pontine gliomas in children remains controversial. There have been many publications over the last 30 years aiming to address this issue. The prognosis for these patients remains extremely poor regardless of treatment and many authors advocate that biopsy carries significant risk for little or no clinical benefit. However, with an increasing knowledge of tumour biology and genetics there is the potential for specific treatments tailored for individual tumours based on their biological or genetic characteristics. The progress of such science in the first instance requires histological diagnosis as part of well conducted clinical trials, then, when treatments have been developed, biopsy samples will be needed to identify the tumours that may respond to such treatments. The authors believe that there is an increasing need for performing a biopsy of these lesions.