Finding the Needle in the Hay Stack: Population-based Study of Prediagnostic Symptomatic Interval in Children With CNS Tumors.

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.

Outcomes and complications for individual neurosurgeons for the treatment of Chiari I malformation at a children's hospital.

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.

Choosing Wisely Canada: Pediatric Neurosurgery Recommendations.

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.

The natural history and management of patients with congenital deficits associated with lumbosacral lipomas.

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.

Minimizing blood transfusions in the surgical correction of craniosynostosis: a 10-year single-center experience.

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.

Growing teratoma syndrome in intracranial non-germinomatous germ cell tumors (iNGGCTs): a risk for secondary malignant transformation­a report of two cases.

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.

What would I want for my surgery?

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.

Does optic nerve sheath diameter on MRI decrease with clinically improved pediatric hydrocephalus?

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.

Identification by families of pediatric adverse events and near misses overlooked by health care providers.

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.

Endoscopic fenestration of cavum velum interpositum cysts: a case study of two symptomatic patients.

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.

Impact of ventricle size on neuropsychological outcomes in treated pediatric hydrocephalus: an HCRN prospective cohort study.

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.

Diffusion-weighted imaging and pathological correlation in pediatric medulloblastomas-"They are not always restricted!".

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.

Does treatment with endoscopic third ventriculostomy result in less concern among parents of children with hydrocephalus?

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.

Clinical, radiologic and pathologic features and outcome following surgery for cervicomedullary gliomas in children.

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.

Predictors of success for combined endoscopic third ventriculostomy and choroid plexus cauterization in a North American setting: a Hydrocephalus Clinical Research Network study.

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.

Medical, social, and economic factors associated with health-related quality of life in Canadian children with hydrocephalus.

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.

Resolution of syringomyelia and Chiari malformation after growth hormone therapy.

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.

Examining the need for routine intensive care admission after surgical repair of nonsyndromic craniosynostosis: a preliminary analysis.

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.