Spontaneous intracerebral haemorrhage secondary to 5-ALA-induced thrombocytopaenia in a paediatric patient: case report and literature review.

INTRODUCTION: The primary objective of neurosurgical management of malignant gliomas is maximal safe resection of the tumour. One of the main obstacles in achieving this is the ability to accurately discriminate between tumour edges and the surrounding healthy brain tissue. The use of fluorescence-guided surgery utilising 5-aminolevulinic acid (5-ALA), first introduced more than 20 years ago, has become an invaluable adjunct in high-grade glioma surgery in adults. However, as 5-ALA is not licensed for use in paediatric patients, the safety profile for such use remains undetermined. CASE REPORT: We describe the case of a 4-year-old boy who underwent 5-ALA-guided resection of a fourth ventricle anaplastic ependymoma. Although complete resection was achieved and the patient awoke from surgery well with no neurological deficits, the patient developed acute transaminitis, anaemia, thrombocytopaenia and coagulopathy postoperatively. The patient had a sudden neurological deterioration on postoperative day 2; imaging revealed that he had suffered a spontaneous right frontal intracerebral haemorrhage. The patient returned to theatre for surgical decompression and evacuation of the haematoma, and ultimately went on to make a full recovery. CONCLUSION: The use of 5-ALA in paediatric patients can be helpful in maximising surgical resection, but the associated safety profile remains undefined. Further research is urgently warranted in order to characterise the efficacy and risk of the use of 5-ALA in the paediatric population.

Effectiveness of subtemporal decompression for the management of slit ventricle syndrome.

OBJECTIVES: To assess the effectiveness of subtemporal decompression in the management of slit ventricle syndrome. METHODS: We conducted a retrospective review of all patients with slit ventricle syndrome (SVS) who underwent subtemporal decompression (STD) at our centre between 2010 and 2021. Cases were identified using the hospital database. Medical records for each patient were reviewed, including operative and radiological reports. RESULTS: Fifteen patients underwent STD for the management of SVS. Median age at time of STD was 9.18 years. Aetiology of hydrocephalus consisted of spinal dysraphism (5), idiopathic (4), post-infectious (1), post-haemorrhagic (3), secondary to tumour (1), and craniofacial anomalies (1). Median age at first shunt insertion was 3.4 months. Median pre-operative period assessed, from initial shunt insertion to STD, was 4.54 (interquartile range [IQR] 3.12-10.47) years. Twelve patients underwent ≥1 shunt revision prior to STD. All patients had a diagnosis of SVS at time of STD. Presenting symptoms, for the admission in which STD was performed, included nausea (9), vomiting (8), lethargy (8), headache (12), irritability (5), and visual disturbances (6). One third underwent shunt revision at the time of STD. Two patients developed post-operative complications requiring further surgery (meningitis requiring shunt revision: 1; wound debridement: 1). Three patients developed uncomplicated post-operative pyrexia, which was managed with antibiotics. Median duration of post-operative follow-up was 5.4 (IQR 1.73-8.54) years. Eleven patients underwent ≥1 shunt related procedure following STD. Wilcoxon signed-rank test demonstrated a significant difference in number of shunt related procedures before (median = 5, IQR 1-8) and after (median = 3, IQR 0-5) STD (Z = -2.083, p = .037). All patients reported subjective symptom improvement post-operatively. Thirteen patients experienced symptom recurrence at a median duration of 10 months post-operatively. CONCLUSIONS: STD was associated with a reduction in the amount of shunt related procedures required in this group of patients with SVS. Further study is required to confirm this association.

Clinical characteristics and outcomes of children with non-sinogenic Brain infections.

BACKGROUND: Central Nervous System (CNS) suppurative infections are serious but rare conditions in the paediatric population. Data on long-term neurocognitive and quality of life outcomes in children recovering from these infections are lacking. METHODS: A retrospective cohort review of children <16 years with non-sinogenic infections undergoing neurosurgery was conducted. Data for patients admitted to Children's Health Ireland at Temple St between 2008-2021 were analysed for clinical and microbiological profiles. Follow-up reviews evaluating neurological and academic sequelae and quality of life were performed. Categorical variables were analysed for unfavourable outcome with a p < 0.05 significance value. RESULTS: Forty patients were included with a mean age of 4.5 years and equal gender distribution. Fever (68%) and vomiting (58%) were the most common presenting complaints. Only fourteen (35%) patients presented with the classic triad of fever, headache, and focal neurological deficit. Meningitis/Encephalitis was the most common cause of suppurative infection (40%). Predisposing factors included congenital heart disease (18%), prematurity (15%) and immunocompromised status (10%). More patients received an initial Burr hole aspiration (73%) than Craniotomy (27%). The re-operation rate was higher in the craniotomy group (45%) compared to the burr hole group (34%), but this was not statistically significant (p = 0.522). Four patients died (10%) including two intra-hospital deaths (5%). Male gender (p = 0.047) and multiple abscesses (p = 0.041) were associated with unfavourable outcome at discharge. Mobility impairment was the most affected determinant of quality of life. CONCLUSION: CNS suppurative infections are associated with long-term neurocognitive sequelae in children. Multiple abscesses and male gender are associated with unfavourable GOS on discharge. Children are still left with mobility impairment (25%), personality changes (23%) and intellectual disability (18%) at an average of 5 years. Long-term follow up with multidisciplinary input is required. Further research should focus on evaluating long-term HRQoL in children.

Severe fetal ventriculomegaly: Fetal morbidity and mortality, caesarean delivery rates and obstetrical challenges in a large prospective cohort.

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Endoscopic extended transsphenoidal surgery for newly diagnosed paediatric craniopharyngiomas.

PURPOSE: 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. METHODS: 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%. RESULTS: 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. CONCLUSIONS: 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.

Role of routine post-operative CT brain following evacuation of extradural haematoma in children: a single-centre experience.

PURPOSE: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: Post-operative imaging in asymptomatic paediatric patients after evacuation of EDH could therefore be avoided as a routine investigation.

Correction to: International experience in the development of patient-derived xenograft models of diffuse intrinsic pontine glioma.

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."

International experience in the development of patient-derived xenograft models of diffuse intrinsic pontine glioma.

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.

Holoprosencephaly: antenatal and postnatal diagnosis and outcome.

OBJECTIVES: 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. DESIGN: This is a retrospective review over a 10-year period. PATIENTS: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Routine cerebrospinal fluid analysis during 'de novo' ventriculoperitoneal shunt insertion: Single Institution Experience.

Background Cerebrospinal fluid (CSF) sampling is performed during ventriculoperitoneal (VP) shunting procedure surgery for early detection of infection. We have performed a retrospective study to determine whether routine intraoperative CSF sampling at new VP shunt insertion has predicted shunt infection or changed subsequent surveillance or management. Method From January 2011 to December 2013, 99 patients underwent first time VP shunt insertion at our hospital. The data collected from Beaumont Hospital Information System and operating theatre logbook. The reviewed data were patient demographics, the clinical condition requiring VP shunt procedure, shunt type, date of VP shunt insertion, date of VP shunt infection or malfunction and laboratory data. Results A first time VP shunt insertion procedure was performed in 99 patients. During the VP shunt insertion procedure, 64 patients had CSF sampling for microbiological analysis. The CSF culture was negative in all samples. All patients were followed up for 12 months. During the follow-up period, a total of 15 patients underwent VP shunt revision. Three of the shunt revisions were secondary to infection. The three infected shunt cases had sterile CSF cultures at the time of insertion. Conclusion There appears to be no correlation between CSF sampling during first time VP shunt insertion and the later development VP shunt infection. This questions the need for routine CSF sampling at the time of insertion. This would be confirmed as a part of future randomised trials.

Monitoring the changing pattern of delivery of paediatric epilepsy surgery in England--an audit of a regional service and examination of national trends.

PURPOSE: The demand for paediatric epilepsy surgery in the UK greatly exceeds the number of operations performed. Hence, Children's Epilepsy Surgery Service (CESS) was commenced in 2012. This study is aimed to characterise the changes in service delivery in the North East of England Paediatric Neuroscience Network and nationally. METHODS: A retrospective cohort study of paediatric epilepsy surgery in Leeds between 2005 and 2012 is presented followed by analysis of British Paediatric Neurosurgical Group (BPNG) data before and after CESS commissioning. RESULTS: During the study period, 42 children underwent epilepsy surgery in Leeds. The commonest aetiologies were neoplasm (33%), focal cortical dysplasia (19%) and mesial temporal sclerosis (19%). Seizure outcome was 71 % EngelI and 83% EngelI+II. Complications included one infection (2%), two temporary (5%) and one permanent (2%) motor deficits, three new/worsened visual field deficits (7%). There were six re-craniotomies (14%). The BPNG data show a 48% increase in paediatric epilepsy surgery in England between 2009 (90 cases) and 2012 (133 cases), and a 20% fall in 2013 (106 cases)--the first calendar year for CESS. On average, 64% of all operations were performed in London. CONCLUSIONS: The number of children receiving surgery for epilepsy in England had increased annually up to, and declined after, the establishment of CESS centres. The yearly caseload in neurosurgical units outside of London is small. The outcomes from Leeds are comparable to those published elsewhere. Other UK units are encouraged to publish outcomes to facilitate patient, commissioner and provider decision making.

Suprasellar pilocytic astrocytoma: one national centre's experience.

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

ETV as a last resort.

INTRODUCTION: The field of neuroendoscopy is rapidly expanding with increasing indications for endoscopic third ventriculostomy (ETV). DISCUSSION: 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. CONCLUSION: 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.

Foramen magnum decompression for Chiari I malformation: a procedure not to be underestimated.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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 aged < 16 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. CONCLUSIONS: 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.

The open fontanelle: a window to less radiation.

INTRODUCTION: 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. AIM: To determine the pattern of CT and Cranial ultrasound used in patients aged one or younger with shunt treated hydrocephalus. METHODS: 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. RESULTS: 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. CONCLUSION: Children with an open fontanelle and shunt can be followed reliably with CRUSS in order to reduce their exposure to radiation.

Multiple Abscesses in the Frontal, Temporal and Brainstem regions in a 4.5-year-Old Girl- An Illustrative Case Report.

Background: Brainstem located abscesses are rare in the pediatric population. Diagnosis of brain abscess can be challenging as patients may present with nonspecific symptoms and the classical triad of headache, fever, and focal neurological deficit is not always present. Treatment can be conservative or a combination of surgical intervention with antimicrobial therapy. Case Description: We present the first case of a 4.5-year-old girl with acute lymphoblastic leukemia that developed infective endocarditis (IE) and subsequently developed 3 suppurative collections (frontal, temporal, and brainstem). The patient had negative cerebrospinal, blood, and pus culture growth and subsequently underwent burr-hole drainage of the frontal and temporal abscesses with a 6-week course of intravenous antibiotic therapy with an uneventful postoperative course. At 1 year, the patient is left with minor right lower limb hemiplegia and no cognitive sequelae. Conclusion: The decision to surgically intervene for brainstem abscesses is dependent on surgeon and patient factors including the presence of multiple collections, midline shift, the aim of source identification in sterile cultures, and the patient's neurological condition. Patients with hematological malignancies should be monitored closely for IE which is a risk factor for hematogenous spread of brainstem located abscesses.

Prenatal findings and associated survival rates in fetal ventriculomegaly: A prospective observational study.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

A multidisciplinary fetal neurosurgical service-5 years of fetal outcomes from a national referral centre.

BACKGROUND: 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. AIMS: We aimed to review this service that provides MDT expertise directly to parents and record the cases and pregnancy outcomes involved. METHODS: 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. RESULTS: 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. CONCLUSION: Multidisciplinary antenatal counselling supported with in utero MRI provides families with optimum information to inform them of likely neonatal outcome.

Antibiotic-impregnated ventriculoperitoneal shunts--a multi-centre British paediatric neurosurgery group (BPNG) study using historical controls.

BACKGROUND: Ventriculoperitoneal shunt infection remains a significant problem. The introduction of antibiotic-impregnated shunt (AIS) systems in the prevention of shunt infection may represent a potential advance; however, there are no randomized controlled trials to establish a robust evidence-based practice. Previously published single-institution cohort studies have provided varying results on the efficacy of AIS systems in the prevention of shunt infection. In this study, we evaluate combined outcomes from three paediatric neurosurgical units in the use of AIS systems for paediatric patients with hydrocephalus. METHODS: The three units established independent databases with data collected from varying time frames. All procedures, where a complete AIS system or part was implanted into patients from 0-16 years in age, were included. The primary outcome measure was shunt infection rate. Shunt procedures were classified as de novo (DNS) and clean revision (CRS). An infant (<1 year) de novo insertion subgroup was also analyzed. AIS shunts were compared to a historical control of non-AIS shunts and results were analysed by centre using an odds ratio with a 95% confidence interval and combined across centres by meta-analysis. RESULTS: A total of 581 AIS implantation procedures were performed in all three units. The comparative non-AIS historical cohort comprised of 1,963 procedures. The pooled effect estimate indicated a clinical advantage for AIS shunts compared to non-AIS shunts, odds ratio (OR), 0.60 (95% CI 0.38, 0.93). The de novo infant group comprised 153 AIS systems, and 465 de novo shunts in the historical non-AIS cohort. Again the pooled effect estimate indicated a clinical advantage for AIS shunts compared to non-AIS shunts, OR 0.38 (95% CI, 0.17; 0.85); however, there was a large overlap of confidence intervals in the results from the different sites indicating the uncertainty in the treatment effect estimates. Over 80% of organisms were gram positive in the infected AIS cohort with a median time to infection of 19 days. Two rifampicin-resistant organisms and three MRSA organisms were detected. CONCLUSION: Data from this exclusively paediatric multi-centre historical control study suggest that AIS may significantly reduce infection rates in de novo and clean revision shunt implants. Although the possibility of bias cannot be excluded due to study design, this is the largest study on an exclusively paediatric cohort comparing standard shunts to AIS implants. Future double-blinded RCTs are needed to confirm AIS efficacy.