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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.

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.

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.

Objective: Endoscopic third ventriculostomy (ETV) is currently the principal alternative to cerebrospinal fluid shunt placement in the management of pediatric hydrocephalus. Cost-effectiveness analysis can help determine the optimal strategy for integrating these different approaches., Methods: All patients (n = 28) who underwent ETV at British Columbia's Children's Hospital between 1989 and 1998 were matched for age, pathogenesis, and number of previous shunt procedures, with patients treated with cerebrospinal fluid shunts. To perform a cost-effectiveness analysis, hydrocephalus-related resource consumption and outcome (determined as the number of hydrocephalus treatment-free days during follow-up) were then retrospectively identified. Cost data were linked to resource use to provide a total cost for all resources used. Costs and outcomes were discounted annually at 5% by standard economic analysis methods., Results: Twenty-four of 28 ETV patients had obstructive hydrocephalus. Over equivalent follow-up periods (median, 35 mo), the ETV success rate (defined by need for reoperation) was 54%. One hydrocephalus-related death and one hemiparesis occurred in the ETV group. No permanent procedure-related morbidity or mortality was seen in the shunt group. The cost/effect ratios for the two groups were similar. The additional incremental resource use by the shunt group included six readmissions and eight reoperations. ETV mean costs per patient were $10,570 +/- $7628, versus $10,922 +/- $8722 for the shunt group (Canadian dollars for the year 2000). Costs accrued more quickly for the shunt group as time passed. The additional incremental outcome benefit to the endoscopy group was 86 treatment-free days (3.07 d per patient [95% confidence interval, -7.56 to 13.70 d]). Neither of these differences was statistically significant., Conclusion: In this matched cohort, ETV was not significantly less costly or more effective over a median 35 months of follow-up, with a 54% initial ETV success rate, even before the additional morbidity and mortality encountered were taken into account. The time course for the accrued costs suggests that a larger cohort, longer follow-up, or higher success rates are needed to demonstrate the cost-effectiveness of this therapy.

Introduction: Tethering is assumed to be the primary cause of deterioration seen in children with transitional lipomyelomeningocele as they age. The inevitability of deterioration has led to recommendations for prophylactic interventions to stabilize or prevent further clinical deterioration., Objective: Determine the frequency and patterns of functional deterioration observed after successful untethering in patients with transitional lipomyelomeningocele and compare functional outcomes with what is known regarding untreated patients., Methods: Fifty patients having transitional LMMC, treated at a single institution and followed in a multidisciplinary clinic were retrospectively reviewed to determine their clinical status prior to untethering, and the time to development of new symptoms or signs following untethering., Results: 82% of patients were diagnosed and 78% underwent untethering prior to one year of age. All patients had a cutaneous lumbosacral lipoma, 22 patients were considered normal at presentation and 28 showed abnormalities on clinical examination. Forty-nine patients were untethered successfully and all were available for follow-up ranging from 2 to 138 months (mean 39 months). Acute morbidity was limited to transient neurogenic bladder dysfunction and minor wound complications. Late clinical deterioration occurred in the majority of patients. Orthopedic and neurological deterioration occurred over the first 60 months following untethering and urological deterioration occurred thereafter., Conclusions: Functional loss after untethering is common and the pattern of loss is likely a reflection of the ability to detect abnormalities in this infant population. Untethering does not usually result in permanent acute morbidity, and does not prevent longer-term functional deterioration. The ratio of asymptomatic to symptomatic patients at follow-up in this operative series is similar to age-matched historical series of untreated patients.