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Dissemination of diffuse intrinsic pontine glioma (DIPG) outside the central nervous system is exceptional. Here, we present a child diagnosed with DIPG who developed seeding along the track of the ventriculoperitoneal shunt and review the literature on this unusual occurrence.

Purpose: The aim of the study was to evaluate established risk factors and define new inflammation-associated factors associated with postoperative ventriculoperitoneal shunt placement., Methods: The electronic medical records of children who underwent surgery for a tumor in the posterior fossa between January 2009 and January 2018 were retrospectively analyzed. Factors evaluated include age, clinical symptoms, tumor type, extent of surgical tumor resection, treatment with EVD and/or ETV, radiological findings, postoperative serum CRP, and leucocyte levels. Tumor tissue was stained immunohistochemically with antibodies against CD3, and leucocyte counts were performed. Patients with pre- or postoperative signs of infection or confirmation of a concurrent infection were excluded from some analyses., Results: Seventy patients ages 0.4-20.8 years (median, 8.2) were included. Forty-five of 70 (65.3%) presented postoperative radiological signs of hydrocephalus. Fifteen of 70 (21.4%) patients required shunt placement postoperatively. Shunt placement was significantly associated with age < 3 years at diagnosis (p = 0.013), perioperative EVD placement (p < 0.001), signs of hydrocephalus in postoperative imaging (p = 0.047), a frontooccipital horn ratio (FOHR) > 0.46 within the first 72 h postoperatively (p < 0.001), and the presence of intraventricular blood postoperatively (p = 0.007). Six patients who underwent shunting had serum CRP levels > 40 mg/l (p = 0.030) within the first 48 h postoperatively. Tumor type or extent of resection did not correlate with shunt placement., Conclusions: Several established and new factors associated with shunt placement after posterior fossa tumor surgery could be identified. Additional studies are needed to explore the aseptic inflammation pathways involved with increased CRP levels and shunt placement.

Purpose: To develop a better understanding of pediatric intracranial aneurysms (PIAs)., Methods: All PIAs treated in our center from January 2012 to April 2017 were retrospectively included. Clinical data, treatment summaries, and follow-up outcomes were retrieved and analyzed., Results: A total of 66 PIAs were found in 64 patients with a mean age of 11.4 ± 5.7 years, 68.8% of whom were male. The most common symptoms were seizure (n = 7, 63.6%) for the 0-5 age group and headache (n = 38, 71.7%) for the 6-18 age group. Fifty-one PIAs (77.3%) were located in the anterior circulation, with the middle cerebral artery (MCA) being the most common site (n = 28, 42.4%). Fifteen patients (23.4%) had PIAs that were pseudoaneurysms, and nine of them (60%) had a combined history of head trauma. Thirty-five patients (54.7%) had distal arterial aneurysms, and 21 of them (60%) presented with seizure. During a mean follow-up time of 1.6 ± 1.2 years, 79.7% of patients (n = 51) had favorable outcomes, 5 patients (7.8%) died, and the remaining 8 patients (12.5%) had unfavorable outcomes with severe neurological deficits., Conclusion: Apart from characteristics consistent with previous studies, several new findings regarding PIAs were reported, including a difference in the most common symptoms in different age groups, the MCA as the predominant location of PIAs, the high ratio of pseudoaneurysms and their head trauma etiology, and the incidence of distal arterial aneurysms and their relationship with the risk of seizures.

Background: Endoscopic third ventriculostomy (ETV) and ventriculo-peritoneal shunt (VPS) although recognized surgical options for non-communicating hydrocephalus have debatable applications., Objective: We analysed a prospective cohort of age-matched children with non-tumor, non-communicating hydrocephalus treated with the two surgical modalities using clinically measurable parameters., Methods: A single institution analysis of age-matched patients with non-communicating hydrocephalus treated with VPS or ETV over a 3-year period. Occipitofrontal circumference (OFC), milestone, shunt independence as well as complication profiles of patients were recorded and analysed. Mean follow-up period was 1.27 ± 0.19 years 95%CI). Data analysis were performed using SPSS version 15, Chicago, IL. Statistical tests were set at 95% significance level., Results: Fifty-five patients were enrolled, 25 patients had ETV, while 30 had VPS. Mean age was 2.3 ± 0.7 years (95% CI) with a range of 3 months to 4.5 years. Aqueductal stenosis was the most common indication. OFC profile decline was significant among the VPS group when compared with ETV group at 3 months follow-up (χ 2  = 7.59, df = 1, p < 0.05). There was no difference among the two treatment groups χ 2  = 2.47, df = 1, p > 0.05) in milestone profile. Thirteen percent of VPS, compared to (4%) ETV patients, had sepsis (χ 2  = 4.59, df = 1 p < 0.05). Ninety-two percent of ETV patients remained shunt free, while 80% of shunted patients achieved ETV independence. Two patients died among the VPS group compared to one patient in the ETV group., Conclusion: VPS compared to ETV is associated with an earlier milestone and OFC response. ETV is associated with lower rates of sepsis and mortality.

Objective: Treatment monitoring and outcome evaluation in pediatric hydrocephalus require gapless documentation regarding surgical and clinical follow-up data beginning from day 1 of treatment in order to apply high quality of care. Endoscopic procedures, shunt insertion and revision surgeries, and individual modifications of valve hardware or pressure settings during follow-up as well as established outcome measurements are highly relevant for complete illustration of the patient's hydrocephalus histories. A digital tool to capture, organize, and analyze comprehensive treatment-related data was estimated long overdue, consequentially developed, and implemented in daily pediatric neurosurgical routine., Methods: We established a self-contained, network-capable database application to supply and back up clinical information of complete surgical treatment history with implant status and follow up for all institutional pediatric hydrocephalus patients from 1995 to date. The application content has been prospectively complemented since 2012 during daily pediatric neurosurgical routine. Beside surgical data, neurological outcome and quality of life assessment were integrated according to validated scales to be recordable 2, 3, and 5 years after initial surgical intervention for prospective administration. The application is in continuous and problem-free use since implementation offering homogeneous and structured real-time information of surgical and corresponding neurological hydrocephalus-related data. By using an automatized data extraction tool, an exemplary surgical outcome evaluation reviewing institutional ventriculo-peritoneal shunt (VPS) treatment in infants over a period of more than 20 years was performed. To validate applicability, the Registry was successfully implemented in an external institution under identical conditions continuously serving for the same purpose until today., Results: Upon completion of the developing process, the application was successfully implemented into routine clinical workflow of our institution. In total, 579 pediatric hydrocephalus patients entered into the Registry with collectively 1874 corresponding hydrocephalus-related surgeries (9% neuro-endoscopic procedures, 18% temporary CSF-diversions, 73% shunt surgeries) so far. For exemplary surgical outcome analysis, the total volume of complex data sets could easily be reduced stepwise in regard to requested inclusion criteria. The selection process generated conclusive data of 256 institutional pediatric VPS patients providing a median follow-up of 8.5 years. Surgical outcome was evaluated in regard to hydrocephalus etiology, applied valve design, valve augmentation, cause of initial malfunction, time to initial shunt revision, and number of total revisions., Conclusion: The pediatric hydrocephalus registry application delivers easy access to contemporary and up-to-date clinical information during daily clinical routine and proves comprehensive value for various scientific purposes. Institutional hydrocephalus etiologies, treatment modalities, and surgical outcome could be reviewed for a selected pediatric patient collective during an interval of more than 20 years and confirmed initial shunt treatment within the first year of age, communicating hydrocephalus and a history of prematurity as significant variables for unfavorable shunt survival and long-term revision rate. At our institution, the Registry emerged to an essential and sustainable tool to capture, organize, and analyze patterns of care in pediatric hydrocephalus patients of all etiologies and treatment modalities. Because of its adaptable and reliable predicate, a prospective multi-center utilization is currently in preparation.

Introduction: Chronic overdrainage affects shunted patients producing a variety of symptoms that may be misdiagnosed. The best known symptoms are so-called shunt-related headaches. There is mounting evidence that changes in cerebrospinal venous system dynamics are a key factor to the pathophysiology of chronic overdrainage syndrome., Clinical Presentation: We report the case of a 29-year-old woman with a shunt since the postnatal period suffering from chronic but the most severe intermittent headache attacks, despite an open shunt and with unchanged ventricular width during attacks. Intracranial pressure (ICP) recordings were performed during headache attacks and thereafter., Diagnosis and Managment: Massively increased ICPs, a continuous B wave "storm," and severely compromised intracranial compliance despite an open shunt were found, a scenario that was always self-limiting with the resolution of symptoms after several hours. When mobilized to the upright position, her ICPs dropped to - 17 mm Hg, proving shunt overdrainage., Outcome and Conclusions: Symptomatology can only be explained by sudden venous entrapment following chronic venous distention as a result of chronic overdrainage. Subsequent therapeutic management with an overdrainage preventing shunt and satisfying clinical outcome with complete ceasing of headache attacks adds insight into the pathophysiology of chronic overdrainage syndrome.

Purpose: The purpose of this study was to determine whether drainage and revision are an effective treatment for abdominal pseudocyst associated ventriculoperitoneal (VP) shunt failure by estimating the total rate of secondary shunt failure., Methods: We performed a retrospective review of children with hydrocephalus diagnosed with and treated for an abdominal pseudocyst at the Children's Hospital, London Health Sciences Centre (LHSC) between January 1, 2000 and May 31, 2016 (ethics approval # 108136). Patients with a VP shunt were included if (i) the development of an abdominal pseudocyst at age 2 to 18 years was identified, (ii) treatment of the pseudocyst by either interventional radiology (IR) or surgical drainage, and (iii) revision of the VP shunt. Demographic data and details of pseudocyst formation/ treatment as well as subsequent failures were identified., Results: Twelve patients who had a VP shunt developed abdominal pseudocyst and met inclusion criteria. A 91% shunt failure rate after drainage and shunt revision was identified. Three patients had the pseudocyst drained in interventional radiology and then externalized due to shunt infection. Nine patients were treated by surgical revision. Ten patients experienced recurrent shunt failure following initial drainage of the pseudocyst: pseudocyst reoccurrence (n = 3), distal obstruction from adhesions (n = 1), and uncleared infection (n = 6)., Conclusion: The results suggest that pseudocyst drainage and shunt revision is ineffective in providing long-term resolution of shunt problems.

Introduction: Management of pregnancy and delivery of a patient with a history of myelomeningocele requires a multidisciplinary team approach., Case Report: We report a case of pregnancy and delivery by a patient who had a history of myelomeningocele surgical repair, ventriculoperitoneal (VP) shunt, and bladder augmentation enterocystoplasty. Regarding types of delivery style, anesthesiologists recommended a Cesarean section under general anesthesia. However, urologists recommended a vaginal delivery because they were concerned that she would require a nephrostomy because of severe adhesion between her uterus and the neobladder if she had a Cesarean section., Discussion: In a pregnant myelomeningocele patient with a VP shunt, neurosurgeons are expected to manage the VP shunt during pregnancy and delivery. The possible types of delivery style and the best options based on the neurological deficit should be discussed together with a medical team.

Purpose: It is established in the literature that disparities exist in the quality of healthcare for patients from disadvantaged backgrounds and lower socioeconomic status. There may be roadblocks within the field of neurosurgery preventing equal access and quality of care. Our goal was to study the similarities between pediatric patients with shunted hydrocephalus of different insurance types and race., Methods: A retrospective chart review was performed on all pediatric patients who underwent ventriculoperitoneal shunting from 1990-2010 at our institution. Race and insurance type were recorded and assessed against specific outcome measures to statistically compare complication rates., Results: A complete record was found for 373 patients who received 849 shunting procedures at our institution. No differences were found between racial groups and insurance type for overall shunt survival, total revision number, or average time to failure. However, nonwhite patients spent an average of 3 days longer in the hospital at initial shunting (p = 0.04), and those with public insurance stayed for 5 days longer (p = 0.002). Patients with public insurance were more likely to present with shunt failure from outside hospitals (p = 0.005) and be born prematurely (p < 0.001). Private patients were more likely to have a neoplasm present at time of initial shunt placement (p = 0.003)., Conclusion: While overall revision rate was not affected by race or insurance status, there were significant delays in discharge for patients with public insurance. Moreover, potential disparities in outpatient access to primary physicians and specialists may be affecting care.

Ventriculoperitoneal shunting constitutes the standard procedure for draining cerebrospinal fluid (CSF) in children with hydrocephalus. Ventriculoatrial and ventriculopleural shunting are alternative methods of CSF drainage, which have gained less acceptance. Ventriculopleural shunts are seldom used owing to justified fears of pneumothorax and symptomatic effusions of CSF. The addition of an antisiphon device to standard shunt systems seems to have prevented CSF pleural effusion. From 1988 to 1998, we treated each of six hydrocephalic children with a ventriculopleural shunt. In five cases we used new-technology valves designed to prevent the effects of siphoning with current differential pressure valves. Peritoneal adhesions, recent peritonitis, ascites, and obstruction of a previous ventriculoatrial shunt were the indications for pleural shunting. After a mean follow-up period of 2.5 years all shunts were functioning adequately. Only one patient showed transient symptoms of CSF overdrainage, which were corrected by up-grading the valve setting with the magnet. A late death was unrelated to the pleural shunting procedure. The use of valves of a new design designed to prevent overdrainage seems to account for the satisfactory outcomes observed in this series. We suggest that ventriculopleural shunting should be considered as the preferred alternative to peritoneal drainage in children with intra-abdominal adhesions or with a history of recent peritoneal infection.