Patient-reported outcome measures more than fifteen years after treatment of sagittal or metopic craniosynostosis: a prospective cohort study.

PURPOSE: To evaluate the long-term anthropometric measurements, cosmetic satisfaction, and other patient-reported outcome measures (PROMs) of patients who underwent surgical treatment or observation only of sagittal or metopic single-suture craniosynostosis (SSC). METHODS: A prospective study was designed for all patients diagnosed with non-syndromic sagittal and metopic craniosynostosis at the British Columbia Children's Hospital, Vancouver, Canada, in the period July 1986 to July 2006. After a minimum of 15 years post-diagnosis, all eligible patients were invited to fill out the Craniofacial Surgery Outcomes Questionnaire (CSO-Q) and to attend a scheduled follow-up appointment for the collection of anthropometric measurements. A descriptive analysis of the cosmetic results was performed. Statistical analyses compared the differences in anthropometric measurements between treated and non-treated patients. RESULTS: Of the 253 eligible patients, 52 participants were willing to share patient data for use in the study. Of those 52 former patients, 36 (69.2%) filled out and returned the CSO-Q and 23 (44.2%) attended the follow-up appointment. The mean follow-up period between surgical treatment and the CSO-Q was 20.2 ± 2.5 years and between surgical treatment and the follow-up appointment was 20.9 ± 2.7 years. In patients with sagittal SSC, the mean cephalic index (CI) was significantly larger in treated than in non-treated patients (74.6 versus 69.1, p = 0.04), while the mean pupillary distance and forehead to back index were significantly smaller (pupillary distance 6.0 cm versus 6.7 cm [p = 0.04] and forehead to back index 19.6 cm versus 21.1 cm [p = 0.03]). Focusing more on the patient reported outcome measures, overall cosmetic satisfaction was found to be high (80.6%) and no differences were found between sagittal and metopic synostosis patients, nor between treated or non-treated craniosynostosis patients. Overall outcomes regarding self-esteem (RSES) and fear of negative evaluation (FNE) were comparable with population based outcomes. CONCLUSION: This is the first prospective study of sagittal and metopic craniosynostosis patients regarding long-term anthropometric outcome and patient reported outcome measures, including patients who were treated surgically and those who received observation only. Although study participation two decades after initial diagnosis was difficult to obtain, our data provide a platform from which one can develop an inclusive and uniform approach to assess patients' subjective cosmetic satisfaction using the CSO-Questionnaire and might be useful in preoperative counseling and psychosocial care for patients and their families.

Optic nerve sheath diameter correlates with both success and failure of hydrocephalus treatment in pediatric patients with pineal region lesions.

BACKGROUND: Pineal region lesions in children are heterogenous pathologies often symptomatic due to occlusive hydrocephalus and thus elevated intracranial pressure (ICP). MRI-derived parameters to assess hydrocephalus are the optic nerve sheath diameter (ONSD) as a surrogate for ICP and the frontal occipital horn ratio (FOHR), representing ventricle volume. As elevated ICP may not always be associated with clinical signs, the adjunct of ONSD could help decision making in patients undergoing treatment. The goal of this study is to assess the available magnetic resonance imaging (MRI) of patients with pineal region lesions undergoing surgical treatment with respect to pre- and postoperative ONSD and FOHR as an indicator for hydrocephalus. METHODS: Retrospective data analysis was performed in all patients operated for pineal region lesions at a tertiary care center between 2010 and 2023. Only patients with pre- and postoperative MRI were selected for inclusion. Clinical data and ONSD at multiple time points, as well as FOHR were analyzed. Imaging parameter changes were correlated with clinical signs of hydrocephalus before and after surgical treatment. RESULTS: Thirty-three patients with forty operative cases met the inclusion criteria. Age at diagnosis was 10.9 ± 4.6 years (1-17 years). Hydrocephalus was seen in 80% of operative cases preoperatively (n = 32/40). Presence of hydrocephalus was associated with significantly elevated preoperative ONSD (p = 0.006). There was a significant decrease in ONSD immediately (p < 0.001) and at 3 months (p < 0.001) postoperatively. FOHR showed a slightly less pronounced decrease (immediately p = 0.006, 3 months p = 0.003). In patients without hydrocephalus, no significant changes in ONSD were observed (p = 0.369). In 6/6 patients with clinical hydrocephalus treatment failure, ONSD increased, but in 3/6 ONSD was the only discernible MRI change with unchanged FOHR. CONCLUSIONS: ONSD measurements may have utility in evaluating intracranial hypertension due to hydrocephalus in patients with pineal region tumors. ONSD changes appear to have value in assessing hydrocephalus treatment failure.

Cosmetic satisfaction and patient-reported outcomes following surgical treatment of single-suture craniosynostosis: a systematic review.

PURPOSE: This study provides a systematic review on cosmetic satisfaction and other patient-reported outcomes (PROMs) of patients who underwent surgical treatment of SSC. METHODS: A systematic review of all articles published from inception to 1 June 2022 was performed. Articles were included if they reported on subjective assessment of cosmetic satisfaction or other PROMs by patients or their families using questionnaires or interviews. RESULTS: Twelve articles, describing 724 surgical treatments of SSC, met the inclusion criteria. Cosmetic satisfaction was evaluated in the following ways: 1) use of the VAS score, binary questions or a 5-point scale to rate general, facial or skull appearance; 2) use of an aesthetic outcome staging in which personal opinion was added to the treating surgeon's opinion; and 3) use of an evaluation of anatomical proportions of the skull and face. A trend towards an overall improvement in cosmetic satisfaction following surgical treatment of SSC was observed. Reported PROMs included general health, socioeconomic status, patients' and their families' rating of the normalcy and noticeability of their appearance and how much this bothered them, and patients' answers to the Youth Quality of Life with Facial Differences (YQOL-FD) questionnaire. No clear overall trend of the reported PROMs was identified. CONCLUSION: This systematic review illuminates that there is a wide variation in outcomes for evaluating cosmetic satisfaction and other PROMs of patients who underwent surgical treatment of SSC, suggesting that further research is needed to develop an inclusive and uniform approach to assess these outcomes.

Outcomes of a radiation sparing approach in medulloblastoma by subgroup in young children: an institutional review.

OBJECTIVE: To describe disease outcomes including overall survival and relapse patterns by subgroup in young pediatric patients treated for medulloblastoma with a radiation-sparing approach. METHODS: Retrospective analysis of clinical outcomes includes treatment, relapse, and salvage therapy and late effects in children treated for medulloblastoma with a radiation-sparing approach at British Columbia Children's Hospital (BCCH) between 2000 and 2020. RESULTS: There were 30 patients (median age 2.8 years, 60% male) treated for medulloblastoma with a radiation-sparing approach at BCCH. Subgroups included Sonic Hedgehog (SHH) (n = 14), group 3 (n = 7), group 4 (n = 6), and indeterminate status (n = 3). Three- and 5-year event-free survival (EFS) were 49.0% (30.2-65.4%) and 42.0% (24.2-58.9%) and overall survival (OS) 66.0% (95% CI 46.0-80.1%) and 62.5% (95% CI 42.5 and 77.2%), respectively, with a median follow-up of 9.5 years. Relapse occurred in 12/25 patients following a complete response, of whom six (group 4: n = 4; group 3: n = 1; unknown: n = 1) were successfully salvaged with craniospinal axis (CSA) RT and remain alive at a median follow-up of 7 years. Disease/treatment-related morbidity included endocrinopathies (n = 8), hearing loss n = 16), and neurocognitive abnormalities (n = 9). CONCLUSIONS: This radiation sparing treatment approach for young patients with medulloblastoma resulted in a durable cure in most patients with SHH subgroup medulloblastoma. In those patients with groups 3 and 4 medulloblastoma, relapse rates were high; however, most group 4 patients were salvaged with RT.

Systematic review of spinal deformities following multi-level selective dorsal rhizotomy.

PURPOSE: Cerebral palsy is a common neurological disorder that involves spasticity of the extremities and can lead to lifelong disability. Selective dorsal rhizotomy (SDR) can improve spasticity and quality of life in these patients, but it may be associated with the development of spinal deformity. Risk factors for spinal deformity after SDR have not yet been systematically examined. METHODS: Medline, Embase, and Web of Science databases were queried for clinical studies reporting incidence of new or worsening spinal deformity, including scoliosis, after SDR. Variables that represent possible risk factors for deformity were correlated with reported incidence of deformity. RESULTS: Twenty-two articles for a total of 1485 patients met the inclusion criteria for this study. Deformity occurs among all patients with a weighted mean incidence of 28.0%. Scoliosis appears to be the most common deformity occurring with a weighted mean incidence of 31.6%. There is substantial heterogeneity between studies, limiting our analysis. Significant positive correlation was found between percent of patients that developed any type of deformity and the ratio of female to male patients, p = 0.02. Significant positive correlation was also found between percent of patients that develop scoliosis and the ratio of female to male patients, p < 0.01, and between scoliosis and the number of years to follow-up, p < 0.01. CONCLUSION: Spinal deformity is an important potential complication of SDR with scoliosis being the most common type of deformity. The major risk factor for postoperative deformity is female sex. Deformity was also found to significantly increase with extended follow-up, indicating a slow process that should be carefully monitored.

Cerebellar mutism after posterior fossa tumor resection in children: a multicenter international retrospective study to determine possible modifiable factors.

PURPOSE: A preliminary survey of pediatric neurosurgeons working at different centers around the world suggested differences in clinical practice resulting in variation in the risk of pediatric cerebellar mutism (CM) and cerebellar mutism syndrome (CMS) after posterior fossa (PF) tumor resection. The purposes of this study were (1) to determine the incidence and severity of CM and CMS after midline PF tumor resection in children treated at these centers and (2) to identify potentially modifiable factors related to surgical management (rather than tumor biology) that correlate with the incidence of CM/CMS. METHODS: Attending pediatric neurosurgeons at British Columbia's Children's Hospital (BCCH) and neurosurgeons who completed a pediatric neurosurgery fellowship at BCCH were invited to provide data from the center where they currently practiced. Children aged from birth to less than 18 years who underwent initial midline PF tumor resection within a contemporary, center-selected 2-year period were included. Data was obtained by retrospective chart and imaging review. Modifiable surgical factors that were assessed included pre-resection surgical hydrocephalus treatment, surgical positioning, ultrasonic aspirator use, intraoperative external ventricular drain (EVD) use, surgical access route to the tumor, and extent of resection. CM was defined as decreased or absent speech output postoperatively and CMS as CM plus new or worsened irritability. RESULTS: There were 263 patients from 11 centers in 6 countries (Canada, Germany, the Netherlands, India, Indonesia, and the USA). Median age at surgery was 6 years (range < 1 to 17 years). The overall incidence of postoperative CM was 23.5% (range 14.7-47.6% for centers with data on ≥ 20 patients). The overall incidence of CMS was 6.5% (range 0-10.3% for centers contributing data on ≥ 20 patients). A multivariate logistic regression on the full data set showed no significant association between pre-resection surgical hydrocephalus treatment, prone position, ultrasonic aspirator use, EVD use, telovelar approach, complete or near total resection, or treating center and either postoperative CM or CMS. CONCLUSIONS: While there was variation in surgical management of midline PF tumors among centers participating in this study, the factors in management that were examined did not predict postoperative CM or CMS.

Cranioplasty after craniectomy in pediatric patients-a systematic review.

INTRODUCTION: Complications following cranioplasty with either autografts or cranial implants are commonly reported in pediatric patients. However, data regarding cranioplasty strategies, complications and long-term outcomes are not well described. This study systematically reviews the literature for an overview of current cranioplasty practice in children. METHODS: A systematic review of articles published from inception to July 2018 was performed. Studies were included if they reported the specific use of cranioplasty materials following craniectomy in patients younger than 18 years of age, and had a minimum follow-up of at least 1 year. RESULTS: Twenty-four manuscripts, describing a total of 864 cranioplasty procedures, met the inclusion criteria. The age of patients in this aggregate ranged from 1 month to 20 years and the weighted average was 8.0 years. The follow-up ranged from 0.4 months to 18 years and had a weighted average of 40.4 months. Autologous bone grafts were used in 484 cases (56.0%). Resorption, infection and/or hydrocephalus were the most frequently mentioned complications. In this aggregate group, 61 patients needed a revision cranioplasty. However, in 6/13 (46%) papers studying autologous cranioplasties, no data was provided on resorption, infection and revision cranioplasty rates. Cranial implants were used in 380 cases (44.0%), with custom-made porous hydroxyapatite being the most commonly used material (100/380, 26.3%). Infection and migration/fracturing/loosening were the most frequently documented complications. Eleven revision cranioplasties were reported. Again, no data was reported on infection and revision cranioplasty rates, in 7/16 (44%) and 9/16 (56%) of papers, respectively. CONCLUSION: Our systematic review illuminates that whether autografts or cranial implants are used, postcranioplasty complications are quite common. Beyond this, the existing literature does not contain well documented and comparable outcome parameters, suggesting that prospective, long-term multicenter cohort studies are needed to be able to optimize cranioplasty strategies in children who will undergo cranioplasty following craniectomy.

Cranioplasties following craniectomies in children-a multicenter, retrospective cohort study.

OBJECTIVE: Complications following pediatric cranioplasty after craniectomy with either autologous bone flaps or cranial implants are reported to be common, particularly bone flap resorption. However, only sparse data are available regarding cranioplasty strategies, complications, and outcomes. This manuscript describes a Canadian-Dutch multicenter pediatric cohort study with autografts and cranial implant cranioplasties following craniectomies for a variety of indications. METHODS: The study included all children (< 18 years) who underwent craniectomy and subsequent cranioplasty surgeries from 2008 to 2014 (with a minimum of 1-year follow-up) at four academic hospitals with a dedicated pediatric neurosurgical service. Data were collected regarding initial diagnosis, age, time interval between craniectomy and cranioplasty, bone flap storage method, type of cranioplasty for initial procedure (and redo if applicable), and the postoperative outcome including surgical site infection, wound breakdowns, bone flap resorption, and inadequate fit/disfigurement. RESULTS: Sixty-four patients (46 males, average age 9.7 ± 5.5 years) were eligible for inclusion, with mean follow-up of 82.3 ± 31.2 months after craniectomy. Forty cranioplasties (62.5%) used autologous bone re-implant, 23 (57.5%) of which showed resorption. On average, resorption was documented at 434 days (range 62-2796 days) after reimplantation. In 20 cases, a revision cranioplasty was needed. In 24 of the post-craniectomy cases (37.5%), a cranial implant was used with one of ten different implant types. Implant loosening prompted a complete revision cranioplasty in 2 cases (8.3%). Cranial implants were associated with low morbidity and lower reoperation dates compared to the autologous cranioplasties. CONCLUSION: The most prominent finding in this multicenter cohort study was that bone flap resorption in children remains a common and widespread problem following craniectomy. Cranioplasty strategies varied between centers and evolved over time within centers. Cranial implants were associated with low morbidity and low reoperation rates. Still, longer term and prospective multicenter cohort studies are needed to optimize cranioplasty strategies in children after craniectomies.

What attributes define excellence in a trauma team? A qualitative study

Background: Hospital trauma teams consist of a diverse spectrum of health care professionals who work together to deliver quality care. Although the qualities of a well-performing trauma team are often believed to be self-evident, there is little objective information about the most desirable personal and professional characteristics associated with quality trauma care. The aim of this study was to determine the traits and characteristics deemed of greatest value for a trauma team leader and a trauma team member in the adult trauma care setting. Methods: Semistructured interviews were conducted with trauma team leaders and trauma team members at a tertiary Canadian trauma centre. Standard qualitative research methodology was used. Interviews were recorded, transcribed and analyzed via an inductive analysis approach. Results: Thematic saturation was achieved after 5 interviews, and 6 further interviews were conducted to ensure that a breadth of trauma care disciplines were included. Six attributes were identified to be of greatest value for trauma team leaders: communication, role clarity, experience, anticipation, management and decisiveness. Four attributes were identified to be of greatest value for trauma team members: engagement, efficiency, experience and collaboration. We further characterized the language defining the ranking of performance for each of these attributes. Conclusion: Results of this qualitative study involving an experienced and diverse spectrum of trauma team practitioners provide insight into the characteristics that are critical to establishing a "good" trauma team. These findings can be used to inform future determinations of the quality of trauma teams, the education of trauma practitioners and continuing medical education training and assessment tools.

Contexte: Les équipes de traumatologie des hôpitaux sont formées de professionnels de la santé de divers horizons qui travaillent ensemble pour offrir des soins de qualité. Bien que les attributs d'une bonne équipe de traumatologie soient souvent vus comme étant évidents, il existe peu de données objectives sur les caractéristiques personnelles et professionnelles les plus fortement associées à des soins traumatologiques de qualité. Cette étude avait pour but de déterminer les traits et caractéristiques les plus recherchés chez les chefs et les membres d'équipes de traumatologie pour adultes. Méthodes: Nous avons mené des entrevues semi-structurées auprès de chefs et de membres d'équipes de traumatologie, dans un centre tertiaire de traumatologie canadien. Une méthode de recherche qualitative standard a été utilisée. Les entrevues ont été enregistrées et transcrites, puis analysées selon une approche inductive. Résultats: Le seuil de saturation thématique a été atteint après 5 entrevues, mais nous avons mené 6 entrevues supplémentaires pour garantir une variété dans les disciplines représentées. Six attributs ont été relevés pour les chefs d'équipe de traumatologie : communication, clarté du rôle, expérience, anticipation, gestion et esprit de décision. Quatre attributs ont été relevés pour les membres de l'équipe : engagement, efficacité, expérience et collaboration. Pour chaque attribut, nous avons caractérisé avec précision les termes définissant la qualité des soins prodigués. Conclusion: Les résultats de cette étude qualitative, qui rassemblait des professionnels de la traumatologie expérimentés et d'horizons diversifiés, mettent en lumière les caractéristiques essentielles à la mise en place d'une « bonne ¼ équipe de traumatologie. Ils pourront servir dans l'évaluation des équipes, dans la formation des praticiens et à la création de cours et d'outils d'évaluation pour l'éducation médicale continue.

International survey on the management of Chiari 1 malformation and syringomyelia: evolving worldwide opinions.

INTRODUCTION: In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M. MATERIALS AND METHODS: Pediatric neurosurgeons worldwide were surveyed, using an e-mail list provided by the ISPN communication committee chairperson. Respondents were given scenarios similar to the 2003 C1M survey in order to determine opinions regarding whether to surgically intervene, and if so, with which operations. RESULTS: Of 300 surveys electronically distributed, 122 responses were received (40.6% response rate)-an improvement over the 30.8% response rate in 2003. Pediatric neurosurgeons from 34 different countries responded. There was broad consensus that non-operative management is appropriate in asymptomatic C1M (> 90%) as well as asymptomatic C1M with a small syrinx (> 65%). With a large syrinx, a majority (almost 80%) recommended surgical intervention. Scoliotic patients with CIM were generally offered surgery only when there was a large syrinx. There has been a shift in the surgical management over the past decade, with a bone-only decompression now being offered more commonly. There remains, however, great variability in the operation offered. CONCLUSION: This survey, with a relatively strong response rate, and with broad geographic representation, summarizes current worldwide expert opinion regarding management of pediatric C1M. Asymptomatic C1M and C1M with a small syrinx are generally managed non-operatively. When an operation is indicated, there has been a shift towards less invasive surgical approaches.

MRI in mild pediatric traumatic brain injury: diagnostic overkill or useful tool?

PURPOSE: Magnetic resonance imaging (MRI) is a sensitive imaging tool which lacks the burden of ionizing radiation. It is not established as primary diagnostic tool in traumatic brain injury (TBI). The purpose of this study was to evaluate the usefulness of MRI as initial imaging modality in the emergency management of mild pediatric TBI. METHODS: Children (0-18 years, sub-divided in four age-groups) with mild TBI who received MRI in the emergency department were identified. Clinical characteristics and trauma mechanisms were evaluated retrospectively. Univariate and multivariate logistic regression analyses were used to identify clinical factors that might be indicative for trauma sequelae on MRI scans. RESULTS: An institutional case series of 569 patients (322 male/247 female; age < 18years; (GCS ≥ 13), who received MRI for mild TBI, was analyzed. Multi-sequence imaging (including T2, T2*, FLAIR, and diffusion-weighted sequences) was feasible without sedation in 96.8% of cases (sedation, 1.8%; general anesthesia, 1.4%). MRI revealed trauma-associated findings in 13% of all cases; incidental findings were detected in 4.7%. In our cohort, GCS deterioration, scalp hematoma, clinical signs of skull base fractures, and horseback riding accidents were related to structural trauma sequelae on MRI. CONCLUSIONS: MRI is a practical primary imaging tool for evaluating children with mild TBI in the emergency department. The presented analyses demonstrated that in our institution, MRI imaging is performed frequently in the emergency department. It resulted mostly in normal findings. This may reflect uneasiness of when to perform imaging in mild TBI and appears retrospectively as an "overdo." There are clinical factors that are more likely associated with MRI-positive findings. Their reliability has to be evaluated in prospective studies in order to formulate further decision rules of when to perform MRI imaging or not.

Manifestations of Tuberous Sclerosis Complex: The Experience of a Provincial Clinic.

BACKGROUND: Tuberous sclerosis complex (TSC) is a neurocutaneous disorder with a wide spectrum of manifestations. Recent consensus recommendations stress the importance of multidisciplinary management of children with TSC. The objective of this study was to examine the manifestations of TSC at a large referral centre to determine the care needs of this population. METHODS: A retrospective, systematic chart review was performed of children with TSC managed at British Columbia Children's Hospital. Patients were identified through epilepsy and clinical neurophysiology databases. RESULTS: The study population comprised 81 patients, born between 1987 and 2014, who were a median of 10 years (range, 0.2-23.2) at most recent follow-up. Epilepsy occurred in 91% of patients, including 32% with a history of infantile spasms. Nineteen patients underwent epilepsy surgery, nine (47%) of whom were seizure-free at most recent follow-up. Overall, 61% of epilepsy patients had been seizure-free for at least 1 year at the time of last follow-up. Neuropsychiatric disorders were diagnosed in 49% of children, with autism (25%), attention deficit hyperactivity order (19%) and anxiety (16%) being the most common. Cardiac rhabdomyomata occurred in 35% of children and renal angiomyolipomas were seen in 43%. A total of 91% had skin manifestations. CONCLUSION: This study outlines the multisystem manifestations of TSC, observed through a large pediatric referral center. Epilepsy and neuropsychiatric disorders are the major source of morbidity in this age group and provide many challenges to the treating clinician. Because a subset of the study population is still quite young, the prevalence of neuropsychiatric disorders is likely underestimated.

Correction to: Challenges in minor TBI and indications for head CT in pediatric TBI-an update.

The published version of this article unfortunately contained an error.An error in the Abstract was introduced during corrections stage when a "Conclusion" section appended is from a different article and was not provided from authors.

Challenges in minor TBI and indications for head CT in pediatric TBI-an update.

INTRODUCTION: Pediatric head trauma is one of the commonest presentations to emergency departments. Over 90% of such head injuries are considered mild, but still present risk acute clinical deterioration and longer term morbidity. Identifying which children are at risk of clinically important brain injuries remains challenging and much of the data on minor head injuries is based on the adult population. CHALLENGES IN PEDIATRICS: Children, however, are different, both anatomically and in terms of mechanism of injury, to adults and, even within the pediatric group, there are differences with age and stage of development. IMAGING: CT scans have added to the repertoire of clinicians in the assessment of pediatric head injury population, but judicious use is required given radiation exposure, malignancy risk, and resource constraints. Guidelines and head injury rules have been developed, for adults and children, to support decision-making in the emergency department though whether their use is applicable to all population groups is debatable. Further challenges in mild pediatric head trauma also include appropriate recommendations for school attendance and physical activity after discharge. FURTHER DEVELOPMENTS: Concern remains for second-impact syndrome and, in the longer term, for post-concussive syndrome and further research in both is still needed. Furthermore, the development of clinical decision rules raises further questions on the purpose of admitting children with minor head injuries and answering this question may aid the evolution of clinical decision guidelines. CONCLUSIONS: The next generation of catheter with homogeneous flow patterns based on parametric designs may represent a step forward for the treatment of hydrocephalus, by possibly broadening their lifespan.

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.

Hypothermia for Traumatic Brain Injury in Children-A Phase II Randomized Controlled Trial.

OBJECTIVES: To perform a pilot study to assess the feasibility of performing a phase III trial of therapeutic hypothermia started early and continued for at least 72 hours in children with severe traumatic brain injury. DESIGN: Multicenter prospective randomized controlled phase II trial. SETTING: All eight of the PICUs in Australia and New Zealand and one in Canada. PATIENTS: Children 1-15 years old with severe traumatic brain injury and who could be randomized within 6 hours of injury. INTERVENTIONS: The control group had strict normothermia to a temperature of 36-37°C for 72 hours. The intervention group had therapeutic hypothermia to a temperature of 32-33°C for 72 hours followed by slow rewarming at a rate compatible with maintaining intracranial pressure and cerebral perfusion pressure. MEASUREMENTS AND MAIN RESULTS: Of 764 children admitted to PICU with traumatic brain injury, 92 (12%) were eligible and 55 (7.2%) were recruited. There were five major protocol violations (9%): three related to recruitment and consent processes and two to incorrect temperature management. Rewarming took a median of 21.5 hours (16-35 hr) and was performed without compromise in the cerebral perfusion pressure. There was no increase in any complications, including infections, bleeding, and arrhythmias. There was no difference in outcomes 12 months after injury; in the therapeutic hypothermia group, four (17%) had a bad outcome (pediatric cerebral performance category, 4-6) and three (13%) died, whereas in the normothermia group, three (12%) had a bad outcome and one (4%) died. CONCLUSIONS: Early therapeutic hypothermia in children with severe traumatic brain injury does not improve outcome and should not be used outside a clinical trial. Recruitment rates were lower and outcomes were better than expected. Conventional randomized controlled trials in children with severe traumatic brain injury are unlikely to be feasible. A large international trials group and alternative approaches to trial design will be required to further inform practice.

Intraoperative ultrasound in pediatric brain tumors: does the surgeon get it right?

PURPOSE: Intraoperative ultrasound (iUS) is a valuable tool-inexpensive, adds minimal surgical time, and involves minimal risk. The diagnostic predictive value of iUS is not fully characterized in Pediatric Neurosurgery. Our objective is to determine if surgeon-completed iUS has good concordance with post-operative MRI in estimating extent of surgical resection (EOR) of pediatric brain tumors. METHODS: We reviewed charts of all pediatric brain tumor resections (single institution 2006-2013). Those with iUS and postoperative imaging (<1 week) were included. The surgeon's estimation of the EOR based on iUS and the post-operative neuroimaging results (gold standard) were collected, as well as information about the patients/tumors. RESULTS: Two hundred two resections were reviewed and 58 cases were included. Twenty-six of the excluded cases utilized iUS but did not have EOR indicated. The concordance of interpretation between iUS and post-operative MRI was 98.3%. Of 43 cases where iUS suggested gross total resection, 42 were confirmed on MRI (negative predictive value (NPV), 98%). All 15 cases where iUS suggested subtotal resection were confirmed on MRI (positive predictive value (PPV), 100 %). Agreement between iUS and post-operative imaging had an overall Kappa score of 0.956, signifying almost perfect agreement. CONCLUSION: The results from this study suggest that iUS is reliable with both residual tumor (PPV-100%) and when it suggests no residual (NPV-98%) in tumors that are easily identifiable on iUS. However, tumors that were difficult to visualize on iUS were potentially excluded, and therefore, these results should not be extrapolated for all brain tumor types.

Impact of preoperative laboratory investigation and blood cross-match on clinical management of pediatric neurosurgical patients.

PURPOSE: Studies in the adult literature suggest that preoperative laboratory investigations and cross-match are performed unnecessarily and rarely lead to changes in clinical management. The purposes of this study were the following: (1) to explore whether preoperative laboratory investigations in neurosurgical children alter clinical management and (2) to determine the utilization of cross-matched blood perioperatively in elective pediatric neurosurgical cases. METHODS: We reviewed pediatric patient charts for elective neurosurgery procedures (June 2010-June 2014) at out institution. Variables collected include preoperative complete blood count (CBC), electrolytes, coagulation, group and screen, and cross-match. A goal of the review was to identify instances of altered clinical management, as a consequence of preoperative blood work. The number of cross-matched blood units transfused perioperatively was also determined. RESULTS: Four hundred seventy-seven electively scheduled pediatric neurosurgical patients were reviewed. Preoperative CBC was done on 294, and 39.8 % had at least one laboratory abnormality. Electrolytes (84 patients) and coagulation panels (241 patients) were abnormal in 23.8 and 24.5 %, respectively. The preoperative investigations led to a change in clinical management in three patients, two of which were associated with significant past medical history. Group and screen test was performed in 62.5 % of patients and 57.9 % had their blood cross-matched. Perioperative blood transfusions (71 % of these patients were under 3 years of age) were received by 3.6 % of patients (17/477). The cross-match to transfusion ratio was 16. CONCLUSION: This study suggests that the results of preoperative laboratory exams have limited value, apart from cases with oncology and complex preexisting conditions. Additionally, cross-matching might be excessively conducted in elective pediatric neurosurgical cases.

Evolution of a Pediatric Primary Cerebral ALK-1-Positive Anaplastic Large Cell Lymphoma on Serial MRI.

BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare central nervous system tumor, especially in the pediatric population. There are fewer than 20 described cases of pediatric primary central nervous system anaplastic large cell lymphoma. The child described in our case report demonstrated a dramatic evolution of this tumor in the first 4 weeks on serial imaging. METHODS: Serial MRI imaging was performed followed by biopsy and chemotherapy. RESULTS: Initial imaging revealed a T2 hyperintense lesion in the frontal lobe with abnormally enhancing sulci and minimal surrounding edema and diffusion restriction. Serial imaging revealed progressive increase in the degree of gadolinium enhancement, and the hyperintense T2 edema progressed markedly to exert mass effect. The lesion itself grew marginally. Biopsy revealed an anaplastic large cell lymphoma, only described in 14 previous pediatric patient case reports. The patient was successfully treated with chemotherapy and autologous stem cell transplant. CONCLUSIONS: Our case demonstrates the rapidity with which a PCNSL lesion can develop, and the evolution of the imaging characteristics prior to definitive diagnosis and treatment. Serial imaging by MRI may help differentiate the behavior of a PCNSL from other imitating lesions.

CSF complications following intradural spinal surgeries in children.

BACKGROUND: Cerebrospinal fluid (CSF) leakage is a complication of intradural spinal surgery and is associated with poor wound healing and infection. The incidence of CSF leak is reported at ∼16% in adults, but little information is available in children. PURPOSE: The aim of this study is to determine the CSF leak rate and predisposing factors after intradural pediatric spinal surgeries. METHODS: This study was a retrospective chart review of 638 intradural spinal operations at BC Children's Hospital. CSF leak was defined as pseudomeningocele or CSF leak through incision. Primary operations to untether lipomyelomeningoceles, myelomeningocele/meningocele closure, and Chiari decompressions were excluded. RESULTS: CSF leaks occurred in 7.1%, with 3% having overt CSF leaks through skin (OCSF leak). CSF leaks, specifically OCSF leaks, were associated with postoperative wound infection (P = 0.0016). Sixteen of 45 cases of CSF leak required reoperation. The type of dural suture used, site of operation, or use of fibrin glue did not affect CSF leak rates. Previous spinal surgery (P < 0.0001), use of dural graft (P = 0.0043), method of dural suturing (P = 0.0023), and procedure performed (P < 0.001) were associated with postoperative CSF leakage. Patients with CSF leak were older than those without leak (98 vs. 72 months, P = 0.002). CONCLUSIONS: Our results provide evidence on intraoperative factors that may predispose to CSF leaks after spinal intradural surgery and may help guide surgical practice. This study confirms that the pediatric population shares many of the same risk factors for CSF leak as in adult populations. Further research is needed to explain how specific factors are associated with CSF leaks.