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Objective: In pediatric hydrocephalus, shunts tend to result in smaller postoperative ventricles compared with those following an endoscopic third ventriculostomy (ETV). The impact of the final treated ventricle size on neuropsychological and quality-of-life outcomes is currently undetermined. Therefore, the authors sought to ascertain whether treated ventricle size is associated with neurocognitive and academic outcomes postoperatively., Methods: This prospective cohort study included children aged 5 years and older at the first diagnosis of hydrocephalus at 8 Hydrocephalus Clinical Research Network sites from 2011 to 2015. The treated ventricle size, as measured by the frontal and occipital horn ratio (FOR), was compared with 25 neuropsychological tests 6 months postoperatively after adjusting for age, hydrocephalus etiology, and treatment type (ETV vs shunt). Pre- and posttreatment grade point average (GPA), quality-of-life measures (Hydrocephalus Outcome Questionnaire [HOQ]), and a truncated preoperative neuropsychological battery were also compared with the FOR., Results: Overall, 60 children were included with a mean age of 10.8 years; 17% had ≥ 1 comorbidity. Etiologies for hydrocephalus were midbrain lesions (37%), aqueductal stenosis (22%), posterior fossa tumors (13%), and supratentorial tumors (12%). ETV (78%) was more commonly used than shunting (22%). Of the 25 neuropsychological tests, including full-scale IQ (q = 0.77), 23 tests showed no univariable association with postoperative ventricle size. Verbal learning delayed recall (p = 0.006, q = 0.118) and visual spatial judgment (p = 0.006, q = 0.118) were negatively associated with larger ventricles and remained significant after multivariate adjustment for age, etiology, and procedure type. However, neither delayed verbal learning (p = 0.40) nor visual spatial judgment (p = 0.22) was associated with ventricle size change with surgery. No associations were found between postoperative ventricle size and either GPA or the HOQ., Conclusions: Minimal associations were found between the treated ventricle size and neuropsychological, academic, or quality-of-life outcomes for pediatric patients in this comprehensive, multicenter study that encompassed heterogeneous hydrocephalus etiologies.

Central nervous system (CNS) tumors in children are a devastating diagnosis and delay in diagnosis is well documented in the literature. The aim of this study was to document and characterize time to diagnosis of CNS tumors among children 0 to 17 years of age in a pediatric center. A retrospective chart review was conducted of medical records of children with CNS tumors from 2000 to 2016 in British Columbia, Canada and 148 reports were available for review. Average age at diagnosis was 87.8 months (SD=59.7; median=72). One third (30%) were diagnosed after a single visit to a health care provider and 11 (7.7%) after more than 4 visits. Median time to diagnosis (prediagnostic symptomatic interval [PSI]) was 62 days (average 197±341 d; range, 0 to 2047 d). Longest period was time from first symptom to first health care provider visit (PSI1, median 37 d). Tumors in the posterior fossa and symptoms of ataxia or paresis were associated with a significantly shorter PSI. CNS tumors in children continue to pose a diagnostic challenge with variability in time to diagnosis. Our population-based study suggests variability in time to diagnosis with a need for education of families to identify symptoms associated with CNS tumors., Competing Interests: The authors declare no conflict of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: The craniometrics of head circumference (HC) and ventricular size are part of the clinical assessment of infants with hydrocephalus and are often utilized in conjunction with other clinical and radiological parameters to determine the success of treatment. We aimed to assess the effect of endoscopic third ventriculostomy (ETV) and shunting on craniometric measurements during the follow-up of a cohort of infants with symptomatic triventricular hydrocephalus secondary to aqueductal stenosis., Methods: We performed a post hoc analysis of data from the International Infant Hydrocephalus Study (IIHS)-a prospective, multicenter study of infants (< 24 months old) with hydrocephalus from aqueductal stenosis who were treated with either an ETV or shunt. During various stages of a 5-year follow-up period, the following craniometrics were measured: HC, HC centile, HC z-score, and frontal-occipital horn ratio (FOR). Data were compared in an analysis of covariance, adjusting for baseline variables including age at surgery and sex., Results: Of 158 enrolled patients, 115 underwent an ETV, while 43 received a shunt. Both procedures led to improvements in the mean HC centile position and z-score, a trend which continued until the 5-year assessment point. A similar trend was noted for FOR which was measured at 12 months and 3 years following initial treatment. Although the values were consistently higher for ETV compared with shunt, the differences in HC value, centile, and z-score were not significant. ETV was associated with a significantly higher FOR compared with shunting at 12 months (0.52 vs 0.44; p = 0.002) and 3 years (0.46 vs 0.38; p = 0.03) of follow-up., Conclusion: ETV and shunting led to improvements in HC centile, z-score, and FOR measurements during long-term follow-up of infants with hydrocephalus secondary to aqueductal stenosis. Head size did not significantly differ between the treatment groups during follow-up, however ventricle size was greater in those undergoing ETV when measured at 1 and 3 years following treatment.

Purpose: The aim of this study was to evaluate the outcomes and complications for individual surgeons at British Columbia Children's Hospital for the treatment of Chiari I Malformation (CMI) in children., Methods: This was a retrospective review of patients with CMI who had surgery from 1986 to 2015. We assessed the Chicago Chiari Outcome Scores (CCOS) and complication rates by surgeon., Results: Seventy patients, 38 males and 32 females, underwent posterior fossa decompression including 14 extradural and 56 intradural approaches. Syringomyelia was present in 74.3%. Most syringomyelia improved with no difference between intradural and extradural surgeries. After initial surgery, 13 patients (18.6%) had complications including 2/14 (14.3%) of extradural and 11/56 (19.6%) of intradural surgeries. Two patients required surgical intervention for complications whereas 11 had transient complications. The complication rate by surgeon ranged from 11 to 20% for extradural (2 surgeons only) and 10.5 to 40% for intradural surgeries (4 surgeons). The CCOS ranged from 12 to 15 for extradural and 6 to 16 for intradural. The CCOS ranges for surgeons 1 and 2 were 12-15 and 13-15 respectively for extradural. The CCOS ranges for surgeons 1, 2, 3, and 4 were 12-16, 6-15, 12-16, and 12-16 respectively for intradural. Thirteen patients had a second surgery for CMI. The final CCOS was good in 86% and moderate in 14%., Conclusion: There was variability in surgeries performed at BCCH by different surgeons, with variations in CCOS and complication rates. This information is important during decision making, consent process, and for quality improvement.

Objective: Endoscopic third ventriculostomy combined with choroid plexus cauterization (ETV+CPC) has been adopted by many pediatric neurosurgeons as an alternative to placing shunts in infants with hydrocephalus. However, reported success rates have been highly variable, which may be secondary to patient selection, operative technique, and/or surgeon training. The objective of this prospective multicenter cohort study was to identify independent patient selection, operative technique, or surgical training predictors of ETV+CPC success in infants., Methods: This was a prospective cohort study nested within the Hydrocephalus Clinical Research Network's (HCRN) Core Data Project (registry). All infants under the age of 2 years who underwent a first ETV+CPC between June 2006 and March 2015 from 8 HCRN centers were included. Each patient had a minimum of 6 months of follow-up unless censored by an ETV+CPC failure. Patient and operative risk factors of failure were examined, as well as formal ETV+CPC training, which was defined as traveling to and working with the experienced surgeons at CURE Children's Hospital of Uganda. ETV+CPC failure was defined as the need for repeat ETV, shunting, or death., Results: The study contained 191 patients with a primary ETV+CPC conducted by 17 pediatric neurosurgeons within the HCRN. Infants under 6 months corrected age at the time of ETV+CPC represented 79% of the cohort. Myelomeningocele (26%), intraventricular hemorrhage associated with prematurity (24%), and aqueductal stenosis (17%) were the most common etiologies. A total of 115 (60%) of the ETV+CPCs were conducted by surgeons after formal training. Overall, ETV+CPC was successful in 48%, 46%, and 45% of infants at 6 months, 1 year, and 18 months, respectively. Young age (< 1 month) (adjusted hazard ratio [aHR] 1.9, 95% CI 1.0-3.6) and an etiology of post-intraventricular hemorrhage secondary to prematurity (aHR 2.0, 95% CI 1.1-3.6) were the only two independent predictors of ETV+CPC failure. Specific subgroups of ages within etiology categories were identified as having higher ETV+CPC success rates. Although training led to more frequent use of the flexible scope (p < 0.001) and higher rates of complete (> 90%) CPC (p < 0.001), training itself was not independently associated (aHR 1.1, 95% CI 0.7-1.8; p = 0.63) with ETV+CPC success., Conclusions: This is the largest prospective multicenter North American study to date examining ETV+CPC. Formal ETV+CPC training was not found to be associated with improved procedure outcomes. Specific subgroups of ages within specific hydrocephalus etiologies were identified that may preferentially benefit from ETV+CPC.

OBJECTIVEAt British Columbia Children's Hospital (BCCH), pediatric patients with nonsyndromic craniosynostosis are admitted directly to a standard surgical ward after craniosynostosis surgery. This study's purpose was to investigate the safety of direct ward admission and to examine the rate at which patients were transferred to the intensive care unit (ICU), the cause for the transfer, and any patient characteristics that indicate higher risk for ICU care.METHODSThe authors retrospectively reviewed medical records of pediatric patients who underwent single-suture or nonsyndromic craniosynostosis repair from 2011 to 2016 at BCCH. Destination of admission from the operating room (i.e., ward or ICU) and transfer to the ICU from the ward were evaluated. Patient characteristics and operative factors were recorded and analyzed.RESULTSOne hundred fourteen patients underwent surgery for single-suture or nonsyndromic craniosynostosis. Eighty surgeries were open procedures (cranial vault reconstruction, frontoorbital advancement, extended-strip craniectomy) and 34 were minimally invasive endoscope-assisted craniectomy (EAC). Sutures affected were sagittal in 66 cases (32 open, 34 EAC), coronal in 20 (15 unilateral, 5 bilateral), metopic in 23, and multisuture in 5. Only 5 patients who underwent open procedures (6%) were initially admitted to the ICU from the operating room; the reasons for direct admission were as follows: the suggestion of preoperative elevated intracranial pressure, pain control, older-age patients with large reconstruction sites, or a significant medical comorbidity. Overall, of the 107 patients admitted directly to the ward (75 who underwent an open surgery, 32 who underwent an EAC), none required ICU transfer.CONCLUSIONSOverall, the findings of this study suggest that patients with nonsyndromic craniosynostosis can be managed safely on the ward and do not require postoperative ICU admission. This could potentially increase cost savings and ICU resource utilization.

Objectives: Choosing Wisely Canada is an evidence-based, patient-focused, physician-led campaign to improve the delivery of medical care in Canada. The goal of this study was to produce Canadian recommendations for physicians treating patients with selected paediatric neurosurgery issues., Methods: Paediatric neurosurgeons practicing in Canada were invited to participate. Suggestions were obtained using an anonymous questionnaire, and then ranked anonymously by the participating surgeons. Suggestions that received consensus from participants were discussed at the 2016 annual Canadian Pediatric Neurosurgery Study Group meeting. Suggestions that were not evidence based, or that would not have a substantive population impact were eliminated. All remaining suggestions were anonymously ranked by the group and the top five recommendations were submitted to Choosing Wisely Canada., Results: The final five recommendations include: 1) don't order a computed tomography scan to investigate macrocephaly (order an ultrasound or magnetic resonance imaging scan); 2) don't image a midline dimple related to the coccyx in an asymptomatic infant or child; 3) don't use computed tomography scans for routine imaging of children with hydrocephalus. Fast sequence nonsedated magnetic resonance imaging scans or ultrasounds provide adequate information to assess patients without exposing them to radiation or an anesthetic; 4) don't recommend helmets for mild to severe positional flattening; 5) don't do routine surveillance imaging for incidentally discovered Chiari I malformation., Conclusions: Five Choosing Wisely Canada recommendations were produced to support care of patients with paediatric neurosurgical issues. While these recommendations will apply to the majority of children with the involved conditions, occasionally, deviation from these recommendations may be clinically indicated.

OBJECTIVE CSF shunt infection requires both surgical and antibiotic treatment. Surgical treatment includes either total shunt removal with external ventricular drain (EVD) placement followed by new shunt insertion, or distal shunt externalization followed by new shunt insertion once the CSF is sterile. Antibiotic treatment includes the administration of intravenous antibiotics. The Hydrocephalus Clinical Research Network (HCRN) registry provides a unique opportunity to understand reinfection following treatment for CSF shunt infection. This study examines the association of surgical and antibiotic decisions in the treatment of first CSF shunt infection with reinfection. METHODS A prospective cohort study of children undergoing treatment for first CSF infection at 7 HCRN hospitals from April 2008 to December 2012 was performed. The HCRN consensus definition was used to define CSF shunt infection and reinfection. The key surgical predictor variable was surgical approach to treatment for CSF shunt infection, and the key antibiotic treatment predictor variable was intravenous antibiotic selection and duration. Cox proportional hazards models were constructed to address the time-varying nature of the characteristics associated with shunt surgeries. RESULTS Of 233 children in the HCRN registry with an initial CSF shunt infection during the study period, 38 patients (16%) developed reinfection over a median time of 44 days (interquartile range [IQR] 19-437). The majority of initial CSF shunt infections were treated with total shunt removal and EVD placement (175 patients; 75%). The median time between infection surgeries was 15 days (IQR 10-22). For the subset of 172 infections diagnosed by CSF culture, the mean ± SD duration of antibiotic treatment was 18.7 ± 12.8 days. In all Cox proportional hazards models, neither surgical approach to infection treatment nor overall intravenous antibiotic duration was independently associated with reinfection. The only treatment decision independently associated with decreased infection risk was the use of rifampin. While this finding did not achieve statistical significance, in all 5 Cox proportional hazards models both surgical approach (other than total shunt removal at initial CSF shunt infection) and nonventriculoperitoneal shunt location were consistently associated with a higher hazard of reinfection, while the use of ultrasound was consistently associated with a lower hazard of reinfection. CONCLUSIONS Neither surgical approach to treatment nor antibiotic duration was associated with reinfection risk. While these findings did not achieve statistical significance, surgical approach other than total removal at initial CSF shunt infection was consistently associated with a higher hazard of reinfection in this study and suggests the feasibility of controlling and standardizing the surgical approach (shunt removal with EVD placement). Considerably more variation and equipoise exists in the duration and selection of intravenous antibiotic treatment. Further consideration should be given to the use of rifampin in the treatment of CSF shunt infection. High-quality studies of the optimal duration of antibiotic treatment are critical to the creation of evidence-based guidelines for CSF shunt infection treatment.

OBJECTIVE High-quality data comparing endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) to shunt and ETV alone in North America are greatly lacking. To address this, the Hydrocephalus Clinical Research Network (HCRN) conducted a prospective study of ETV+CPC in infants. Here, these prospective data are presented and compared to prospectively collected data from a historical cohort of infants treated with shunt or ETV alone. METHODS From June 2014 to September 2015, infants (corrected age ≤ 24 months) requiring treatment for hydrocephalus with anatomy suitable for ETV+CPC were entered into a prospective study at 9 HCRN centers. The rate of procedural failure (i.e., the need for repeat hydrocephalus surgery, hydrocephalus-related death, or major postoperative neurological deficit) was determined. These data were compared with a cohort of similar infants who were treated with either a shunt (n = 969) or ETV alone (n = 74) by creating matched pairs on the basis of age and etiology. These data were obtained from the existing prospective HCRN Core Data Project. All patients were observed for at least 6 months. RESULTS A total of 118 infants underwent ETV+CPC (median corrected age 1.3 months; common etiologies including myelomeningocele [30.5%], intraventricular hemorrhage of prematurity [22.9%], and aqueductal stenosis [21.2%]). The 6-month success rate was 36%. The most common complications included seizures (5.1%) and CSF leak (3.4%). Important predictors of treatment success in the survival regression model included older age (p = 0.002), smaller preoperative ventricle size (p = 0.009), and greater degree of CPC (p = 0.02). The matching algorithm resulted in 112 matched pairs for ETV+CPC versus shunt alone and 34 matched pairs for ETV+CPC versus ETV alone. ETV+CPC was found to have significantly higher failure rate than shunt placement (p < 0.001). Although ETV+CPC had a similar failure rate compared with ETV alone (p = 0.73), the matched pairs included mostly infants with aqueductal stenosis and miscellaneous other etiologies but very few patients with intraventricular hemorrhage of prematurity. CONCLUSIONS Within a large and broad cohort of North American infants, our data show that overall ETV+CPC appears to have a higher failure rate than shunt alone. Although the ETV+CPC results were similar to ETV alone, this comparison was limited by the small sample size and skewed etiological distribution. Within the ETV+CPC group, greater extent of CPC was associated with treatment success, thereby suggesting that there are subgroups who might benefit from the addition of CPC. Further work will focus on identifying these subgroups.

Background: High-dose steroid administration is no longer recommended in the treatment of acute traumatic brain injury (TBI) as it failed to prove beneficial in improving patients' outcome. However, a masked benefit of steroid administration in TBI management was that it provided corticosteroid replacement therapy in patients with TBI-related central adrenal insufficiency., Case Presentation: We report the case of a 12-year-old boy who suffered a severe TBI from a motor vehicle accident that resulted in complete deficiency of anterior pituitary function. Central adrenal insufficiency was not ruled out by a near normal response to a low-dose ACTH test performed on D11., Conclusion: Consideration should be given to the empirical treatment of TBI pediatric patients with stress doses of corticosteroids if injury to the hypothalamus or pituitary gland is possible until a formal assessment of the hypothalamic-pituitary-adrenal axis can be made.

OBJECTIVE Accurate placement of ventricular catheters may result in prolonged shunt survival, but the best target for the hole-bearing segment of the catheter has not been rigorously defined. The goal of the study was to define a target within the ventricle with the lowest risk of shunt failure. METHODS Five catheter placement variables (ventricular catheter tip location, ventricular catheter tip environment, relationship to choroid plexus, catheter tip holes within ventricle, and crosses midline) were defined, assessed for interobserver agreement, and evaluated for their effect on shunt survival in univariate and multivariate analyses. De-identified subjects from the Shunt Design Trial, the Endoscopic Shunt Insertion Trial, and a Hydrocephalus Clinical Research Network study on ultrasound-guided catheter placement were combined (n = 858 subjects, all first-time shunt insertions, all patients < 18 years old). The first postoperative brain imaging study was used to determine ventricular catheter placement for each of the catheter placement variables. RESULTS Ventricular catheter tip location, environment, catheter tip holes within the ventricle, and crosses midline all achieved sufficient interobserver agreement (κ > 0.60). In the univariate survival analysis, however, only ventricular catheter tip location was useful in distinguishing a target within the ventricle with a survival advantage (frontal horn; log-rank, p = 0.0015). None of the other catheter placement variables yielded a significant survival advantage unless they were compared with catheter tips completely not in the ventricle. Cox regression analysis was performed, examining ventricular catheter tip location with age, etiology, surgeon, decade of surgery, and catheter entry site (anterior vs posterior). Only age (p < 0.001) and entry site (p = 0.005) were associated with shunt survival; ventricular catheter tip location was not (p = 0.37). Anterior entry site lowered the risk of shunt failure compared with posterior entry site by approximately one-third (HR 0.65, 95% CI 0.51-0.83). CONCLUSIONS This analysis failed to identify an ideal target within the ventricle for the ventricular catheter tip. Unexpectedly, the choice of an anterior versus posterior catheter entry site was more important in determining shunt survival than the location of the ventricular catheter tip within the ventricle. Entry site may represent a modifiable risk factor for shunt failure, but, due to inherent limitations in study design and previous clinical research on entry site, a randomized controlled trial is necessary before treatment recommendations can be made.

OBJECTIVE Endoscopic third ventriculostomy (ETV) is now established as a viable treatment option for a subgroup of children with hydrocephalus. Here, the authors report prospective, multicenter results from the Hydrocephalus Clinical Research Network (HCRN) to provide the most accurate determination of morbidity, complication incidence, and efficacy of ETV in children and to determine if intraoperative predictors of ETV success add substantially to preoperative predictors. METHODS All children undergoing a first ETV (without choroid plexus cauterization) at 1 of 7 HCRN centers up to June 2013 were included in the study and followed up for a minimum of 18 months. Data, including detailed intraoperative data, were prospectively collected as part of the HCRN's Core Data Project and included details of patient characteristics, ETV failure (need for repeat hydrocephalus surgery), and, in a subset of patients, postoperative complications up to the time of discharge. RESULTS Three hundred thirty-six eligible children underwent initial ETV, 18.8% of whom had undergone shunt placement prior to the ETV. The median age at ETV was 6.9 years (IQR 1.7-12.6), with 15.2% of the study cohort younger than 12 months of age. The most common etiologies were aqueductal stenosis (24.8%) and midbrain or tectal lesions (21.2%). Visible forniceal injury (16.6%) was more common than previously reported, whereas severe bleeding (1.8%), thalamic contusion (1.8%), venous injury (1.5%), hypothalamic contusion (1.5%), and major arterial injury (0.3%) were rare. The most common postoperative complications were CSF leak (4.4%), hyponatremia (3.9%), and pseudomeningocele (3.9%). New neurological deficit occurred in 1.5% cases, with 0.5% being permanent. One hundred forty-one patients had documented failure of their ETV requiring repeat hydrocephalus surgery during follow-up, 117 of them during the first 6 months postprocedure. Kaplan-Meier rates of 30-day, 90-day, 6-month, 1-year, and 2-year failure-free survival were 73.7%, 66.7%, 64.8%, 61.7%, and 57.8%, respectively. According to multivariate modeling, the preoperative ETV Success Score (ETVSS) was associated with ETV success (p < 0.001), as was the intraoperative ability to visualize a "naked" basilar artery (p = 0.023). CONCLUSIONS The authors' documented experience represents the most detailed account of ETV results in North America and provides the most accurate picture to date of ETV success and complications, based on contemporaneously collected prospective data. Serious complications with ETV are low. In addition to the ETVSS, visualization of a naked basilar artery is predictive of ETV success.

OBJECTIVE Although patients with lumbosacral lipomas may be asymptomatic at presentation, most develop neurological symptoms over time. Given the challenges in examining infants, the authors sought to determine whether MRI would be helpful in identifying patients who are more likely to deteriorate early in life and who would potentially benefit from early surgical intervention. METHODS A retrospective review of all patients with lumbosacral lipomas who were seen at the authors' institution between 1997 and 2013 and who were managed without prophylactic surgery was performed. The clinical history and imaging results for each patient were reviewed in detail and then correlated to the pattern of and age at clinical deterioration. RESULTS Twenty-four patients were identified. Nine worsened within the first 18 months of life (early deterioration), and 15 patients deteriorated or remained stable after 30 months (late deterioration/stable). No patients worsened between 18 and 30 months of age. Patients who deteriorated early were more likely to have large intradural lipomas that filled the canal, increased during the 1st year of life, and compressed neurological structures. Some had a syrinx extending above the neural-lipoma interface. Syrinxes in patients with early deterioration were large and expanded in infancy. Patients with early deterioration had motor deficits at the time of deterioration, whereas patients with late deterioration developed mixed urological and motor dysfunction. CONCLUSIONS Patients with large lipomas displacing the cord and an enlarging syrinx have a propensity for early clinical deterioration. Given this, their families may be counseled that 1) the risk of deterioration in infancy may be higher than in infants without these features, and 2) they require more diligent observation. Intervention before deterioration in these infants should also be considered. Patients without these features may be safely observed to a lesser extent.

OBJECTIVE The ophthalmological outcomes of children treated for pineal tumors have received limited attention in the literature. METHODS This paper reviews the outcomes of 29 children treated for pineal and posterior third ventricular tumors in the contemporary era using chemotherapy, radiotherapy, and resection as defined by the histology and/or marker profile of the tumor. RESULTS At the time of diagnosis, all patients except 1 had hydrocephalus and all had ophthalmological involvement. Papilledema was found in 69% of patients. Seventy-five percent of patients had partial or complete Parinaud's syndrome, and diplopia or blurred vision was noted in the remaining patients. Visual acuity was impaired in 8 patients. Outcomes were dependent on the histology of the tumor and the treatment required. Those patients who did not requiring resection showed a lower rate of ophthalmological worsening during treatment and greater long-term improvement, in particular with respect to up-gaze palsy. Patients who underwent resection for postchemotherapy residual disease or primary resection showed greater worsening during treatment and lesser degrees of recovery. All patients with impaired visual acuity improved with treatment. CONCLUSIONS As the mortality of germ cell and other pineal tumors decreases, posttreatment morbidity remains, specifically that related to convergence nystagmus, accommodation, and diplopia. In addition to survival, ophthalmological morbidity should be reported in studies concerning the outcomes of treatment for pineal neoplasms.

Introduction: Many patients with lumbosacral lipoma are asymptomatic; however, a significant proportion will have neurological deficits present at birth. Implication of these deficits with respect to natural history and management are not well understood., Methods: A retrospective review of all infants with lumbosacral lipoma seen at BCCH between 1997 and 2013 was carried out. The study population was stratified on the presence of a congenital, non-progressive deficit and subdivided on treatment approach. The subsequent developments of deficits resulting in untethering procedures were recorded., Results: Of the 44 infants in this study, 24 patients had no neurologic deficit while 20 patients had a fixed, non-progressive deficit evident at birth. Ten of 24 patients without a neurological deficit at birth underwent a prophylactic untethering with 3 eventually requiring repeat untethering after, on average, 62.7 months. Eleven of 14 asymptomatic, monitored patients required untethering for clinical deterioration. Two required a second untethering procedure after 48.7 months. Ten of 20 infants with congenital deficits present at birth underwent prophylactic untethering, and 4 required further surgery after 124 months. Ten patients underwent observation with 8 eventually requiring surgery. Two required repeat untethered after 154 months. The complication rates and operative burden for patients are similar whether prophylactic or delayed surgery is performed., Conclusion: The presence of congenital neurologic deficit does not affect the likelihood of deterioration in patients managed expectantly; prophylactic detethering of these patients did not prevent delayed neurologic deterioration. Comparing the need for repeat surgery in prophylactically untethered patients with initial untethering of patients operated upon at the time of deterioration, prophylactic untethering may confer a benefit with respect to subsequent symptomatic tethering if complication rates are low. However, in a setting with multidisciplinary follow-up, a period of observation for patients and intervention when patients become symptomatic is an acceptable approach for patients with or without congenital deficits.

Introduction: Our center previously reported low transfusion rates for craniosynostosis surgery by two experienced neurosurgeons using standard intraoperative techniques and acceptance of low hemoglobin levels. This study evaluated whether low rates were maintained over the last 10 years and if a less experienced neurosurgeon, trained in and practicing in the same environment, could achieve similar outcomes., Methods: All craniosynostosis operations performed in children between 2004 and 2015 were reviewed retrospectively. Transfusion rates were calculated. Analyses examined the relationship of transfusion to craniosynostosis type, surgical procedure, redo operation, surgeon, and perioperative hemoglobin levels., Results: Two hundred eighteen patients were included: 71 open sagittal, 28 endoscopic-assisted sagittal, 32 unicoronal, 14 bicoronal, 42 metopic, and 31 multisuture. Median age at operation was 9.1 months. Overall transfusion rate was 24 %: 17 % open sagittal, 7 % endoscopic-assisted sagittal, 6 % unicoronal, 21 % bicoronal, 45 % metopic, and 45 % multisuture. The timing of transfusions were 75, 21, and 4 % for intraoperative, postoperative, and both, respectively. Patients not receiving transfusion had a mean lowest hemoglobin of 87 g/l (range 61-111) intraoperatively and 83 g/l (range 58-115) postoperatively. Mean lowest hemoglobin values were significantly lower in those necessitating intraoperative (75 g/l, range 54-102) or postoperative (59 g/l, range 51-71) transfusions. There was no significant difference in transfusion rate between less and more experienced surgeons. There were no cardiovascular complications or mortalities., Conclusion: In craniosynostosis surgery, reproducible, long-term low blood transfusion rates were able to be maintained at a single center by careful intraoperative technique and acceptance of low intraoperative and postoperative hemoglobin levels in hemodynamically stable patients. Furthermore, low rates were also achieved by an inexperienced neurosurgeon in the group. This suggests that these results may be achievable by other neurosurgeons, who follow a similar protocol.

Sagittal craniosynostosis, the most common single suture craniosynostosis, is treated by numerous surgical techniques. Minimally invasive endoscopy-assisted procedures with postoperative helmeting are being used with reports of good cosmetic outcomes with decreased morbidity, shortened hospital stay, and less blood loss and transfusion. This procedure uses small skin incisions, which must be properly placed to provide safe access to the posterior sagittal and lambdoid sutures. However, the lambda is often hard to palpate through the skin due to the abnormal head shape. The authors describe their experience with the use of intraoperative, preincision ultrasound localization of the lambda in patients with scaphocephaly undergoing a minimally invasive procedure. This simple technique can also be applied to other operations where proper identification of the cranial sutures is necessary.

Object: Cerebellar cavernous malformations (CCMs) have not been specifically described in the pediatric age group. Authors of this study, after considering the published literature, describe the characteristic clinical, radiological, and surgical features of CCM in children. METHOSDS: Patients younger than 18 years of age who were known to have CCM and had undergone surgery between 1992 and 2014 at the authors' institution were reviewed. Pediatric CCM cases reported in the literature (case reports and cases included in series on CMs in the pediatric age group) were also analyzed for specific features of this entity., Results: Four male patients and 1 female patient (2.5-14 years of age) with CCM presented acutely with severe headache followed by cerebellar dysfunction. In all patients, neuroimaging (cranial CT and MRI) demonstrated hemorrhagic cerebellar lesions with heterogeneous T1 and T2 signal intensities and hyperintense blooming on susceptibility-weighted imaging. The lesions reached large sizes exhibiting spherical, cystic, and often "pseudotumoral" morphology. In 3 patients, developmental venous anomalies (DVAs) were found. In 4 of the 5 patients, the CCMs and hematomas were totally removed. All patients had a clinically excellent functional outcome without surgical complication and with complete resolution of their presenting symptoms., Conclusions: Cerebellar CMs occur in all pediatric age groups and display characteristic clinical and imaging features. In children, CCMs reach large sizes and can result in massive hemorrhage, often leading to a possible diagnosis of hemorrhage into a tumor. An associated DVA is quite common. Surgery is a safe and efficient treatment option with excellent outcomes in patients.

Background: Counts are the commonest method used to ensure that all sponges and neuropatties are removed from a surgical site before closure. When the count is not reconciled, plain radiographs of the operative site are taken to determine whether the missing patty has been left in the wound. The purpose of this study was to describe the detectability of commonly used neuropatties in the clinical setting using digital technologies., Methods: Neuropatties were implanted into the anterior and posterior cranial fossae and the thoracolumbar extradural space of a mature male cadaver. Four neuropatty sizes were used: 3 × 1 in, 2 × ½ in, ½ × ½ in, and ¼ × ¼ in. Neuropatties, with size and location chosen at random, were placed in the surgical sites and anteroposterior/posterior-anterior and lateral radiographs were taken using standard portable digital radiographic equipment. Six clinicians reviewed the digital images for the presence or absence of neuropatties. The readers were not aware of the number and size of the patties that were included in each image., Results: The detectability of neuropatties is dependent on the size of the neuropatty's radiopaque marker and the operative site. Neuropatties measuring 2 × ½ in and 3 × 1 in were detected reliably regardless of the operative site. ¼ × ¼ in neuropatties were poorly detected by neurosurgeons and radiologists in all three operative sites. Readers of various experience and background were similar in their ability to detect neuropatties under these conditions., Conclusions: Under simulated operating room conditions and using currently available neuropatties and plain radiograph imaging technology, small ¼-in and ½-in neuropatties are poorly visible/detectable on digital images., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Authors of this report describe 2 patients who had undergone shunt insertion for hydrocephalus and who, at 6 weeks or 9 months after their last revision, presented with symptoms of shunt dysfunction and CSF collections at the valve site. At the ensuing shunt revision in both patients, the silicone housing was fractured and the Siphonguard was disconnected from the Codman Hakim Precision flat-bottom valve. The cause of these failures was not clear since manipulation, bending, and twisting of the valves were not thought to have occurred during implantation. A review of the FDA's Manufacturer and User Facility Device Experience (MAUDE) database revealed 58 cases of silicone housing failure in the Codman Hakim Precision valve with integrated Siphonguard since the year 2000. A single report was found in the databases administered by the Canadian Medical Devices Sentinel Network (CMDSNet). The Codman Hakim Precision valves with integrated Siphonguard are delicate devices that do not withstand the intraoperative handling tolerated by other valves. When these valves are implanted, gentle handling and wide exposures are needed to minimize the risk of valve damage. Valves should be handled according to the manufacturer's instructions. However, in light of this particular pattern of failure, it is recommended that the manufacturer redesign this valve to provide handling tolerance that is characteristic of other valves on the market. The featured cases illustrate the importance of the surgeon's role in postmarket surveillance of medical devices and reporting device failures to the responsible agencies and manufacturers.

Purpose: About 5% of pediatric intracranial germ cell tumors and 20% of non-germinomatous germ cell tumors (NGGCT) progress to growing teratoma syndrome (GTS) following chemoradiotherapy. The growing teratoma is thought to arise from the chemotherapy-resistant, teratomatous portion of a germ cell tumor and is commonly benign but may undergo malignant transformation., Methods: Two pediatric patients whose intracranial NGGCTs progressed to growing teratomas during chemotherapy and later transformed to secondary malignant tumors after partial resection and radiation therapy (RT)., Results: Both tumors were diagnosed by MRI scans and elevated serum and CSF markers. Following normalization of tumor markers with chemotherapy and initial decrease in tumor volume, subsequent imaging showed regrowth during chemotherapy with pathology revealing benign teratoma. RT was administered. Several years following this treatment, further growth was seen with pathology indicating malignant carcinoma in one patient and malignant rhabdomyosarcoma in the other. The patient with carcinoma received palliative care while the patient with the sarcoma received further resection, intensive chemotherapy, and an autologous stem cell transplant and is currently in remission, 36 months since malignant transformation., Conclusion: Malignant transformation of presumed residual teratoma has been seldom reported. Treatment of NGGCT involves platinum-based chemotherapy with craniospinal RT and boost to the primary site, with cure rates of around 80%. Teratomas are characteristically chemotherapy and RT resistant and are treated surgically. In the event that residual or growing teratoma is suspected, a complete resection should be considered early in the management as there is a risk of malignant transformation of residual teratoma.

Visual loss following surgery for craniopharyngioma is usually the result of operative injury or tumor recurrence. The authors present the case of a 12-year-old boy who developed progressive visual field constriction 11 years after gross-total resection of a solid and cystic craniopharyngioma. No tumor recurrence was evident on multiple MRI studies, and it was only at surgical exploration that the diagnosis of optochiasmatic arachnoiditis was established. Lysis of the adhesions around the optic nerves and chiasm resulted in substantial and sustained visual improvement.

If you were to have an operation tomorrow, would you want your surgical team members to feel comfortable speaking up, to defy hierarchy, to interact with each other just as well as they perform technical aspects of the procedure? Would you want to feel like part of the team? Your answers to these admittedly leading questions are based on the culture of the surgical team and the interdependence of team members and are at the heart of a current debate around the surgical checklist's effectiveness. In British Columbia (BC), many individuals responded to the paper by Urbach et al. (2014) that described the minimal impact on patient mortality after implementation of the surgical safety checklist in Ontario. They wrote to the Surgical Quality Action Network (SQAN) to express their perspectives, and interestingly, some refuted and others supported the conclusions. Given the strong reaction this study created in the surgical community, a number of key stakeholders have prepared a response in order to provide another perspective to the article and emphasize the checklist's value for improving the culture of surgical teams.

Introduction: Serial change in ventricular size is recognized as an imperfect indicator of ongoing hydrocephalus in children. Potentially, other radiographic features may be useful in determining the success of hydrocephalus interventions. In this study, optic nerve sheath diameter (ONSD), optic nerve tortuosity, and optic disk bulging were assessed as indicators of hydrocephalus control in children who underwent endoscopic third ventriculostomy (ETV) or posterior fossa tumor resection., Methods: Sixteen children underwent ETV or tumor resection for treatment of hydrocephalus. T2-weighted axial magnetic resonance images of the orbit were obtained, and the ONSD was measured posterior to the optic globe, pre- and post-intervention. Evidence of optic disk bulging and optic nerve tortuosity was also assessed. Ventricular size was estimated using the frontal and occipital horn ratio (FOR)., Results: There was a significant reduction in the ONSD post-ETV (n = 9) and after tumor resection (n = 7). Average preoperative ONSD was 6.21 versus 5.71 mm postoperatively (p = 0.0017).There was also an 88% (p = 0.011) and 60% (p = 0.23) reduction in optic disk bulging and tortuosity, respectively. The FOR normalized in the tumor resection group but not the ETV group. After intervention, all patients showed improvement in signs and symptoms of hydrocephalus., Conclusion: In our study population, ONSD decreased in response to measures to reduce hydrocephalus. Optic disk bulging also appears to resolve. Serial reduction in ONSD, and optic disk bulging may be indicators of improved hydrocephalus following pediatric neurosurgical interventions.

Object: Monitoring and recording of complications in pediatric neurosurgery are important for quality assurance and in particular for improving outcomes. Lack of accurate or mutually agreed upon definitions hampers this process and makes comparisons between centers, which is an important method to improve outcomes, difficult. Therefore, the Canadian Pediatric Neurosurgery Study Group created definitions of complications in pediatric neurosurgery with consensus among 13 Canadian pediatric neurosurgical centers., Methods: Definitions of complications were extracted from randomized trials, prospective data collection studies, and the medical literature. The definitions were presented at an annual meeting and were subsequently recirculated for anonymous comment and revision, assembled by a third party, and re-presented to the group for consensus., Results: Widely used definitions of shunt failure were extracted from previous randomized trials and prospective studies. Definitions for wound infections were extracted from the definitions from the Centers for Disease Control and Prevention. Postoperative neurological deficits were based on the Pediatric Stroke Outcome Measure. Other definitions were created and modified by consensus. These definitions are now currently in use across the Canadian Pediatric Neurosurgery Study Group centers in Morbidity and Mortality data collection and for subsequent comparison studies., Conclusions: Coming up with consensus definitions of complications in pediatric neurosurgery is a first step in improving the quality of outcomes. It is a dynamic process, and further refinements are anticipated. Center to center comparison will hopefully allow significant variations in outcomes to be identified and acted upon.

Introduction: Cavum velum interpositum (CVI) is commonly an incidental asymptomatic finding on imaging studies. Encystment can occur and, in most situations, is also asymptomatic. Clinical symptoms occurring in patients with CVI cysts have been reported infrequently with the result that the relationship of these symptoms and the cyst are usually unclear. This report contributes to the knowledge base of symptoms that can occur in patients with CVI and the response of symptoms to effective treatment., Patients and Methods: We report the clinical outcomes of a 3-year-old male patient and a 13-year-old female patient with symptoms and CVI cysts on imaging who were treated successfully with endoscopic fenestration., Results: The developmental delay and occasional headache present in the 3-year-old male patient resolved after endoscopic fenestration; however, the 13-year-old patient who had neuropsychiatric symptoms did not improve., Conclusions: Our cases add to the literature describing the response to cyst treatment in symptomatic patients harboring CVI cysts. Symptoms due to CSF pathway obstruction may respond to cyst fenestration, while the response of symptoms in patents who do not have clear CSF circulation disorders is less predictable.

Morquio syndrome, or mucopolysaccharidosis type IV, is a rare enzyme deficiency disorder and results in skeletal dysplasia. Odontoid dysplasia is common among affected patients, resulting in atlantoaxial instability and spinal cord compression. Surgical treatments include decompression and prophylactic fusion, during which intraoperative neuromonitoring is important to alert the surgical team to changes in cord function so that they can prevent or mitigate spinal cord injury. This report describes a 16-year-old girl with Morquio syndrome who developed paraplegia due to thoracic spinal cord infarction during foramen magnum and atlantal decompression. This tragic event demonstrates the following: 1) that patients with Morquio syndrome are at risk for ischemic spinal cord injury at levels remote from areas of maximal anatomical compression while under anesthesia in the prone position, possibly due to impaired cardiac output; 2) the significance of absent motor evoked potential responses in the lower limbs with preserved upper-limb responses in an ambulatory patient; 3) the importance of establishing intraoperative neuromonitoring baseline assessments prior to turning patients to the prone position following induction of anesthesia; and 4) the importance of monitoring cardiac output during prone positioning in patients with chest wall deformity.

Background: Identifying adverse events and near misses is essential to improving safety in the health care system. Patients are capable of reliably identifying and reporting adverse events. The effect of a patient safety reporting system used by families of pediatric inpatients on reporting of adverse events by health care providers has not previously been investigated., Methods: Between Nov. 1, 2008, and Nov. 30, 2009, families of children discharged from a single ward of British Columbia's Children's Hospital were asked to respond to a questionnaire about adverse events and near misses during the hospital stay. Rates of reporting by health care providers for this period were compared with rates for the previous year. Family reports for specific incidents were matched with reports by health care providers to determine overlap., Results: A total of 544 familes responded to the questionnaire. The estimated absolute increase in reports by health care providers per 100 admissions was 0.5% (95% confidence interval -1.8% to 2.7%). A total of 321 events were identified in 201 of the 544 family reports. Of these, 153 (48%) were determined to represent legitimate patient safety concerns. Only 8 (2.5%) of the adverse events reported by families were also reported by health care providers., Interpretation: The introduction of a family-based system for reporting adverse events involving pediatric inpatients, administered at the time of discharge, did not change rates of reporting of adverse events and near misses by health care providers. Most reports submitted by families were not duplicated in the reporting system for health care providers, which suggests that families and staff members view safety-related events differently. However, almost half of the family reports represented legitimate patient safety concerns. Families appeared capable of providing valuable information for improving the safety of pediatric inpatients.

Introduction: Some investigators have suggested that medulloblastomas can be distinguished from other cerebellar neoplasms by demonstrating "restricted diffusion" on the Apparent Diffusion Coefficient (ADC) map obtained from diffusion-weighted imaging (DWI) sequences on magnetic resonance imaging. Previous authors have postulated that this observed restricted diffusion is a reflection of very high cell density. There has been a tendency to assert that pediatric medulloblastoma uniformly demonstrates restricted diffusion on DWI. However, our clinical observation has been that there are pediatric medulloblastomas that exhibit normal or even increased diffusion on DWI. The current study was undertaken primarily to determine whether restricted diffusion is uniformly present in pediatric medulloblastoma and secondly to look for pathological features that might distinguish medulloblastomas with and without restricted diffusion., Methods: The DWI characteristics of pathologically confirmed medulloblastomas diagnosed at our institution were retrospectively reviewed. The ADC was obtained in two non-overlapping, solid, non-hemorrhagic, non-necrotic regions of tumor and averaged. An ADC below 1 × 10(-3) mm(2)/s was considered to represent restricted diffusion. A detailed pathologic review of each tumor was conducted., Results: Ten cases of medulloblastoma were reviewed, of which two demonstrated average ADCs above 1 × 10(-3) mm(2)/s (1.223 and 1.169 × 10(-3) mm(2)/s, respectively), indicating no restricted diffusion. Pathologic review revealed that both of these non-restricting cases displayed a lack of reticulin deposition by light microscopy., Conclusion: DWI does not appear to be an entirely reliable means of distinguishing medulloblastomas from other cerebellar neoplasms. Histologically, restricted diffusion in medulloblastomas may be related to reticulin deposition.

Object: The criteria for identifying patients in whom endoscopic third ventriculostomy (ETV) provides control of hydrocephalus remain in evolution. In particular, it is not clear when ETV would be effective if intraventricular obstruction is not found preoperatively. The authors postulated that 1) displacement of the third ventricle floor inferiorly into the interpeduncular cistern and displacement of the lamina terminalis anteriorly into the lamina terminalis cistern could predict clinical success of ETV, and 2) improvement in these displacements would correlate with the success of ETV., Methods: Magnetic resonance imaging in 38 consecutive patients treated between 2004 and 2010 was reviewed to assess displacement of the lamina terminalis and third ventricular floor prior to and following ETV. Displacements of the floor and lamina terminalis were judged qualitatively and quantitatively, using a newly created index, the Third Ventricular Morphology Index (TVMI). The association between the aforementioned morphological features and clinical success of ETV was analyzed., Results: Ninety-six percent of patients in whom the authors preoperatively observed displacement of the lamina terminalis and the third ventricular floor were successfully treated with ETV. Displacements of the third ventricular floor and lamina terminalis, as judged qualitatively, correlated with the clinical success of ETV. The TVMI correlated with the qualitative assessments of displacement. Postoperative decrease in the TVMI occurred in the majority of successfully treated patients. Changes in third ventricular morphology preceded changes in other measures of third and lateral ventricular volume following ETV., Conclusions: Assessment of third ventricular floor and lamina terminalis morphology is useful in predicting clinical success of ETV and in the follow-up in treated patients. The TVMI provided a quantitative assessment of the third ventricular morphology, which may be useful in equivocal cases and in research studies.

Purpose: A possible benefit of endoscopic third ventriculostomy (ETV) is that families might harbor less concern and anxiety compared to shunt. This has not yet been demonstrated, however. Our goal was to compare parental concern in a large sample of children with hydrocephalus treated with ETV or shunt, using our previously developed measure of parental concern, the Hydrocephalus Concerns Questionnaire for Parents (HCQ-P)., Methods: The parents of children 5-18 years old with previously treated hydrocephalus at three Canadian pediatric neurosurgery centers completed the HCQ-P. HCQ-P scores were compared between those who were initially treated with ETV and those initially treated with shunt. A multivariable linear regression analysis was used to adjust for center, current age, age at initial hydrocephalus surgery, seizures, etiology, hydrocephalus complications, and quality of life., Results: Six hundred three families participated (58 ETV [9.6%], 545 shunt [90.4%]). In unadjusted comparison, ETV parents had lower overall concern (HCQ-P = 0.41 versus 0.51, p = 0.02). After adjustment for multiple patient factors, ETV parents still had lower concern (p = 0.03) but the only questions for which there was a still a statistically significant difference were those related to concerns about shunt/ETV complications., Conclusions: Parents of children who have had ETV experience less concern than those who have had shunt and this is due almost exclusively to less concern about hydrocephalus treatment complications. While this could be interpreted as a beneficial aspect of ETV treatment, it remains important for neurosurgeons to ensure that parents are not being overly complacent about the possibility of ETV failure requiring urgent treatment.

Object: Microfibrillar collagen hemostat (MCH; trade name Avitene) is a partially water-insoluble acid salt of purified bovine corium collagen. This agent has been widely used to control hemorrhage at surgery, and especially during pediatric neurosurgeries at the authors' institution. Despite its effectiveness, rare case reports detailing adverse inflammatory reactions to MCH have been documented. Based primarily on MR imaging, postoperative reactions have most commonly elicited clinical differential diagnoses of tumor recurrence or abscess. According to the literature, MCH induces a very characteristic mixed inflammatory response that is rich in eosinophils; in light of these observations, many authors have suggested an allergy-based pathogenesis., Methods: The authors retrospectively reviewed 3 pediatric neurosurgical cases treated at their institution, wherein a common histomorphological inflammatory reaction to MCH was elicited at the site of prior craniotomy., Results: Case 1 is that of a 10-year-old girl whose diagnosis was a right temporal lobe ganglioglioma, classified as WHO Grade I. Case 2 is that of a 9-year-old boy whose diagnosis was a left parietal lobe anaplastic ependymoma, classified as WHO Grade III. Finally, Case 3 is that of a 15-year-old girl whose diagnosis was focal cortical dysplasia Type IIA affecting the left occipital lobe. Each patient presented with new or recurrent seizures 5–6 weeks after the initial resection. The postsurgical reactions incited by MCH mimicked the radiological appearance of either an abscess (Cases 2 and 3) or recurrent tumor (Case 1). Histologically, the mixed inflammatory infiltrate was typified by the presence of MCH-centric necrotizing granulomas that were surrounded by a palisade of macrophages and often several eosinophils., Conclusions: The findings are in keeping with previous case reports describing the clinicopathological features of adverse reactions occurring due to MCH. Based on the authors' observations, the possibility of an idiopathic inflammatory reaction to MCH should be considered when either seizures, a typical radiological appearance (that is, consistent with tumor recurrence or abscess formation), or both arise shortly after initial surgery. A conservative treatment approach to this type of inflammatory lesion appears to be the most appropriate management strategy.

Object: Quality of life (QOL) studies comparing treatment with endoscopic third ventriculostomy (ETV) and CSF shunting are very limited. The authors compared QOL outcomes following these 2 treatments in a large cohort of children with hydrocephalus by using multivariable statistical techniques to adjust for possible confounder variables., Methods: The families of children between 5 and 18 years of age with previously treated hydrocephalus at 3 Canadian pediatric neurosurgery centers completed measures of QOL: the Hydrocephalus Outcome Questionnaire (HOQ) and the Health Utilities Index Mark 3 (HUI3). Medical records and recent brain imaging studies were reviewed. A linear regression analysis was performed with the QOL measures as the dependent variable. In multivariable analyses, the authors assessed the independent effect of initial hydrocephalus treatment (ETV vs shunting) while adjusting for the treatment center, current patient age, age at initial treatment, etiology of hydrocephalus, total number of days spent in the hospital for initial treatment, total number of days spent in the hospital for subsequent hydrocephalus complications, functioning ETV at follow-up assessment, frequency of seizures, and current ventricle size., Results: Data from 603 patients were available for analysis. Fifty-eight patients had undergone ETV as their primary treatment and 545 had undergone CSF shunting. Endoscopic third ventriculostomy patients were slightly younger at the follow-up assessment, were older at the first surgery, and spent fewer days in the hospital for hydrocephalus complications. Without adjustment for any confounders, treatment with ETV was associated with significantly higher HOQ physical scores and HUI3 scores. After multivariable adjustment, however, there was no significant difference in any outcome measure. A functioning ETV at the time of the follow-up assessment was not significant in any model., Conclusions: Treatment with either ETV or CSF shunting does not appear to be associated with any substantial difference in QOL outcome after adjusting for prognostic factors. Further study is needed to definitively determine the relative QOL benefit of either procedure, if any.

Purpose: Adverse event reporting systems allow healthcare institutions to detect and prevent recurrence of avoidable patient harm. It is known that standard reporting systems, which are initiated by clinicians, detect only a minority of chart-documented adverse events. The objective of the study was to develop a web-based system, the Family Reporting System (FRS), to elicit adverse event reports from families of children admitted to hospital through survey methodology and human factors engineering techniques., Measurements: Face validity and usability were measured via standardized survey instruments. Utility was measured via the rate, typology, degree of harm, likelihood of recurrence, quality of information, and inter-rater agreement analysis of the reported events., Results: The FRS has good face validity, excellent usability, and good clinical utility., Conclusion: The application of survey and human factors methodologies to the design of an electronic system is an effective means of developing an electronic adverse event reporting system for the use of families of pediatric patients., (Copyright 2010 Elsevier Ireland Ltd. All rights reserved.)

Background: Patients have been shown to report accurate observations of medical errors and adverse events. Various methods of introducing patient reporting into patient safety systems have been published with little consensus among researchers on the most effective method. Terminology for use in patient safety reporting has yet to be standardised., Methods: Two databases, PubMed and MEDLINE, were searched for literature on patient reporting of medical errors and adverse events. Comparisons were performed to identify the optimal method for eliciting patient initiated events., Results: Seventeen journal publications were reviewed by patient population, type of healthcare setting, contact method, reporting method, duration, terminology and reported response rate., Conclusion: Few patient reporting studies have been published, and those identified in this review covered a wide range of methods in diverse settings. Definitive comparisons and conclusions are not possible. Patient reporting has been shown to be reliable. Higher incident rates were observed when open-ended questions were used and when respondents were asked about personal experiences in hospital and primary care. Future patient reporting systems will need a balance of closed-ended questions for cause analysis and classification, and open-ended narratives to allow for patient's limited understanding of terminology. Establishing the method of reporting that is most efficient in collecting reliable reports and standardising terminology for patient use should be the focus of future research.

Objective: To prospectively evaluate the results of endoscopic choroid plexus cauterization (ECPC) and ventriculoperitoneal shunts (VPSs) in infants with hydranencephaly or near hydranencephaly., Methods: We prospectively collected clinical data from all untreated hydranencephalic and near hydranencephalic children from October 2006 to March 2008. All patients treated were randomly divided into 2 groups, ECPC or VPS, and submitted to either endoscopic choroid plexus cauterization or ventriculoperitoneal shunt placement., Results: Seventeen patients were entered into the study. ECPC was completed in 9 patients; the procedure successfully controlled excessive head circumference and signs of increased intracranial pressure in 8 of these patients (88.8%). One endoscopic procedure in a hydranencephalic child failed after 7 months, resulting in VPS placement. Thus, of the 10 patients randomized to ECPC, 8 were treated successfully by ECPC (80%), and 2 went on to have a VPS. There were no complications related to this method of treatment. Seven children were randomized to the VPS group; and of these, 2 patients (28.5%) required shunt revisions during follow-up. There were no complications related to shunt placement. There was no difference in the success rate between patients randomized to ECPC and VPS, but the ECPC was more economical., Conclusion: ECPC is an acceptable alternative to VPS for treatment of hydranencephaly and near hydranencephaly. It is a single, definitive, safe, effective, and economical treatment that may avoid the complications of shunting.

Introduction: Choroid plexus hyperplasia (CPH) is a rare cause of cerebrospinal fluid (CSF) overproduction and shunt-resistant hydrocephalus in infants. If treated with a ventriculoperitoneal (VP) shunt, these patients secondarily develop CSF accumulation along the shunt tract and within the peritoneum. The surgical management of this condition is not as clearly defined as in the case of a choroid plexus papilloma or carcinoma., Case Report: An 8-day-old male patient presented with bulging fontanelle, head circumference = 42 cm (>98th percentile), and cranial ultrasound demonstrating communicating hydrocephalus with enlarged choroid plexuses. A VP shunt was inserted, and secondarily progressive hydrocephalus, shunt tract fluid accumulation, and ascites developed. The infant underwent staged bilateral plexectomy and is tolerating CSF diversion. The pathology confirmed CPH., Discussion: Of the cases reported in the literature, treatments have included CSF shunting, endoscopic coagulation, and craniotomy with plexectomy. CSF shunting was required in the majority but not all. Only those having undergone bilateral choroid plexectomy have been rendered shunt free.

Introduction: Surgical resection is generally recommended for cervicomedullary tumors, but morbidity of resection may be significant. This study sought to identify MRI characteristics that might predict morbidity and extent of resection., Materials and Methods: A retrospective review was performed of MRI findings, histopathology, extent, and morbidity of resection in cervicomedullary gliomas undergoing resection during 1985-2008., Results: Of 78 brainstem tumors, nine cervicomedullary tumors undergoing resection were identified: two pilocytic astrocytomas, two gangliogliomas, and five grade II astrocytomas. Mean age was 6.3 years (range 1.7-11.2 years). Initial treatment was surgery in seven: biopsy (1), <25% resection (4), and 25-50% resections (2). Bulbar worsening occurred in five of six patients with interposed areas of non-enhancement versus one of three patients without interposed non-enhancing tissue (P = 0.014). Additionally, bulbar worsening occurred in five of five patients with a poorly defined tumor/brainstem interface and abnormal low T1 signal extending beyond obvious tumor into the brainstem versus one of four with a well-defined tumor margin (P = 0.008). Following chemo- or radiotherapy, the definition of the brainstem/tumor interface improved. In four patients undergoing surgery after chemo/radiotherapy, more extensive resections were achieved without neurologic worsening: >80% in three and 30% in one., Conclusion: A less aggressive initial surgical approach, supplemented by postoperative chemotherapy, designed to preserve brainstem function, is proposed for patients with interposed non-enhancing tissue continuous with normal cervical cord or medulla and/or a poorly defined ventral tumor/brainstem interface with abnormal low T1 signal extending beyond obvious tumor into the brainstem.

Object: Approximately 30% of children with posterior fossa tumors exhibit hydrocephalus after tumor resection. Recent literature has suggested that prophylactic endoscopic third ventriculostomy diminishes the risk of this event. Because the majority of patients will not have postoperative hydrocephalus, a preoperative clinical prediction rule that identifies patients at high or low risk for postresection hydrocephalus would be helpful to optimize the care of these children., Methods: The authors evaluated a derivation cohort of 343 consecutive children with posterior fossa tumors who underwent treatment between 1989 and 2003. Multivariate methods were used on these data to generate the Canadian Preoperative Prediction Rule for Hydrocephalus. The rule's estimated risk of postresection hydrocephalus was compared with risk observed in 111 independent patients in the validation cohort., Results: Variables identified as significant in predicting postresection hydrocephalus were age < 2 years (score of 3), papilledema (score of 1), moderate to severe hydrocephalus (score of 2), cerebral metastases (score of 3), and specific estimated tumor pathologies (score of 1). Patients with scores > or = 5 were deemed as high risk. Predicted probabilities for the high- and low-risk groups were 0.73 and 0.25, respectively, from the derivation cohort, and 0.59 and 0.14 after prevalence adjustment compared with the observed values of 0.42 and 0.17 in the validation cohort., Conclusions: A patient's score on the Preoperative Prediction Rule for Hydrocephalus will allow improved patient counseling and surgical planning by identifying patients at high risk of developing postresection hydrocephalus. These patients might selectively be exposed to the risks of preresection CSF diversion to improve outcome.

Introduction: The association between hypopituitarism, Chiari I malformation, and syringomyelia has been recently recognized. Most of the reported patients suffered perinatal injury or asphyxia., Materials and Methods: We present the case of a premature child without identifiable perinatal injury, who was recognized to have growth hormone (GH) deficiency, Chiari I malformation, and syringohydromyelia., Conclusion: There was a resolution of syrinx and tonsillar herniation after GH replacement.

Objective: To study the factors associated with health-related quality of life (HRQL) in Canadian children with hydrocephalus, using a comprehensive model of determinants of child health, including socioeconomic factors., Study Design: A cross-sectional study was performed between November 2005 and November 2006 at 3 Canadian pediatric hospitals. Parents of children with hydrocephalus age 5 to 18 years completed the Hydrocephalus Outcome Questionnaire (HOQ) and the Health Utilities Index Mark 3 (HUI-3)., Results: A consecutive sample of 340 subjects participated from a total of 366 eligible children (mean age, 11.6 +/- 3.6 years; mean time from the diagnosis of hydrocephalus, 10.0 +/- 4.6 years). Adjusted multivariate linear regression models demonstrated that the most important determinants of poorer HRQL included lower family income, lower parental education, worse family functioning, seizures, myelomeningocele, and prolonged treatment for cerebrospinal fluid shunt obstruction., Conclusions: Despite a national universal health care system, socioeconomic disparities remain important as determinants of HRQL. Given the absence of a parallel private health care system in Canada, this suggests that the impact of socioeconomic factors is related to issues other than access to care.