An apple a day keeps the doctor away: pediatric scurvy case report and mini review.

Scurvy is a rare nutritional disorder caused by deficiency of ascorbic acid (vitamin C). It is often under-diagnosed in clinical settings, especially in North America where population statistics are unavailable. However, scurvy is more common than previously thought and appears to be re-emerging in children with developmental delays. Here, we review the pertinent literature and present a case of a previously healthy, 5-year-old, non-verbal boy who presented with multiple, acute, and subacute spontaneous epidural hemorrhages managed by neurosurgical intervention. He remained in hospital for 17 days and was seen in follow-up 3 weeks post-operatively having returned to his neurological baseline. Our case suggests the importance of considering scurvy in patients who have developmental delays and poor nutritional status.

Conservative management for pediatric craniocervical Pott's disease: A case study and literature review.

Craniocervical Pott's disease is rare, particularly in the pediatric population. The importance of motion preservation in this age group renders managing this disease even more challenging. The literature regarding craniocervical Pott's disease comes from endemic regions. Most authors will agree on early surgical intervention in cases of neurological compromise or severe instability, while patients with minimal symptoms will do well with conservative management. Controversy remains when patients are mildly symptomatic but with imaging findings concerning for significant instability. Here, we present the case of a 15-year-old male presenting with craniocervical tuberculoma with radiographic instability and advanced bony destruction without overt neurological deficits. He was managed with a rigid cervical collar and completed 1 year of anti-tuberculosis therapy. At 1-year follow-up, he had an intact range of motion, was pain-free, and remained neurologically intact. Although this case suggests good outcomes with conservative management are possible, more long-term follow-up is required to assess the need for delayed surgical intervention in this unique population.

Natural history of Chiari I malformation with syrinx and dilatation of the central canal in the pediatric population: the CHEO experience.

PURPOSE: Given that syrinx is often considered an indication of surgery in children with Chiari I malformation (CM1), understanding of the natural history of these patients is very challenging. In this study, we investigate the natural history of children with CM1 that have syrinx and/or prominence of the central canal on presentation. METHODS: All pediatric Chiari I patients who had syrinx and/or prominence of the central canal who underwent MR imaging of the head and spine from 2007 to 2020 were reviewed. Patients were divided into 3 groups (early surgery, delayed surgery, and conservative management). We focused on those patients who did not initially undergo surgery and had at least 1 year of clinical follow-up. We assessed if there were any radiological features that would correlate with need for delayed surgical intervention. RESULTS: Thirty-seven patients met the inclusion criteria. Twenty-one patients were female and 16 were male. The mean age at presentation was 8.7 (5.8 SD). Fourteen (38%) patients had early surgical intervention, with a mean of 2.5 months after initial presentation, 8 (16%) had delayed surgery due to new or progressive neurological symptoms and 46% of patients did not require intervention during follow-up. The length of tonsillar herniation and the position of the obex were associated with the need of surgery in patients who were initially treated conservatively. CONCLUSION: In pediatric patients with CM1 with syringomyelia and prominence of the central canal, conservative treatment is initially appropriate when symptoms are absent or mild. Close follow-up of patients with CM1 and dilatation of the central canal who have an obex position below the foramen magnum and greater tonsillar herniation is suggested, as these patients show a trend towards clinical deterioration over time and may require earlier surgical intervention.

Global trends and decision-making in the management of arachnoid cysts.

OBJECTIVE: In pediatric patients, middle cranial fossa (MCF) arachnoid cysts are often discovered incidentally on imaging in asymptomatic patients during workup for other indications. This study aims to describe current management gestalt and threshold for surgical intervention by surveying an international cohort of neurosurgeons. METHODS: A web-based survey was circulated via email list of attendants of the 2019 Canadian Pediatric Neurosurgery Study Group (CPNSG) and International Society of Pediatric Neurosurgery (ISPN) mailing list. The survey consisted of 8 clinical scenarios involving patients with MCF arachnoid cysts. Demographic variables of respondents and their decisions regarding management for each scenario were analyzed using R computing software. RESULTS: A total of 107 respondents were included. Cysts in asymptomatic patients (92%), younger age at diagnosis (81%), and presence of a mild learning delay were predominantly managed non-surgically (80.7 ± 9.4%). Patients with cyst enlargement, headaches, new seizures, or hemorrhage were divided between non-surgical (55.8 ± 3.3%) and surgical (44.2 ± 2.9%) management. Patients with contralateral hemiparesis were treated predominantly surgically (67%). For both Galassi I and II, papilledema was favored as the primary indication for surgical intervention in 54% of patients. Those inclined to surgery (n = 17) were more likely to practice and train outside North America compared to those not pro-surgical (adjusted P = 0.092). CONCLUSION: Incidental MCF arachnoid cysts in asymptomatic patients and younger age of diagnosis are predominantly managed non-surgically. Mild learning delay was not considered an indication to intervene. In contrast, radiological progression, hemorrhagic evolution, or non-focal neurological deficits lead to uncertainty in management, while focal neurological deficits and papilledema with MCF cysts were favored to be intervened surgically. Among the provider level factors, only location of training and practice trended towards a pro-surgery approach.

Tone management: An environmental scan of current management practices across Canada.

BACKGROUND: Currently, there are no standardized approaches to care or evaluation for tone dysfunction in Canada. The study authors hypothesize that there is significant practice variation across the country. This environmental scan is aimed to describe the current practice for management of paediatric patients with hypertonia across Canada. METHODS: A web-based survey was developed by the authors with a multi-disciplinary approach and sent to representative paediatric rehabilitation sites in each province in Canada. Disciplines at the rehabilitation sites surveyed included all or some of the following disciplines: physiatry, neurology, neurosurgery, plastic surgery, orthopaedic surgery, physiotherapy and occupational therapy. All statistical analyses were performed using the R statistical software version 4.0. Fifteen rehabilitation sites were contacted, and 12 sites were used for the final analysis. RESULTS: Cerebral palsy was found to be the most common diagnosis for tone dysfunction, with 58% of sites diagnosing greater than 20 new patients per year. In 67% of sites, patients were seen within a formal multidisciplinary clinic to manage hypertonia. All 12 sites utilized oral baclofen and gabapentin, and 92% of sites utilized trihexyphenidyl. Botulinum toxin injections were offered at 50% of sites. Upper and lower extremity surgical procedures were offered in 83% of the sites. CONCLUSION: The information gained from this study provides some insight into the current practice across Canada for children with hypertonia. This study may assist in the development of a national, standardized strategy to tone management, potentially facilitating more equitable access to care for patients.

Automated Implementation of the Edinburgh Visual Gait Score (EVGS) Using OpenPose and Handheld Smartphone Video.

Recent advancements in computing and artificial intelligence (AI) make it possible to quantitatively evaluate human movement using digital video, thereby opening the possibility of more accessible gait analysis. The Edinburgh Visual Gait Score (EVGS) is an effective tool for observational gait analysis, but human scoring of videos can take over 20 min and requires experienced observers. This research developed an algorithmic implementation of the EVGS from handheld smartphone video to enable automatic scoring. Participant walking was video recorded at 60 Hz using a smartphone, and body keypoints were identified using the OpenPose BODY25 pose estimation model. An algorithm was developed to identify foot events and strides, and EVGS parameters were determined at relevant gait events. Stride detection was accurate within two to five frames. The level of agreement between the algorithmic and human reviewer EVGS results was strong for 14 of 17 parameters, and the algorithmic EVGS results were highly correlated (r > 0.80, "r" represents the Pearson correlation coefficient) to the ground truth values for 8 of the 17 parameters. This approach could make gait analysis more accessible and cost-effective, particularly in areas without gait assessment expertise. These findings pave the way for future studies to explore the use of smartphone video and AI algorithms in remote gait analysis.

Referral practices for epilepsy surgery in pediatric patients: A North American Study.

OBJECTIVE: Patients with epilepsy who do not respond to two trials of appropriate antiepileptic drugs are considered to have drug-resistant epilepsy (DRE). The International League Against Epilepsy recommends patients with DRE be referred for surgical evaluation; however, prior literature suggests this is an underutilized intervention, especially in the pediatric setting. This study captures practices of North American pediatric neurologists regarding the management of DRE and factors that may promote or limit referrals for epilepsy surgical evaluation. METHODS: A REDCap survey was distributed via the Child Neurology Society mailing list to pediatric neurologists practicing in North America. Ethics approval from the Children's Hospital of Eastern Ontario Research Ethics Board was granted prior to the start of data collection. RESULTS: Ninety-eight responses were included in the analysis; 77% of participants currently practice in the United States; 73.5% of respondents reported they would refer a patient for surgical consultation after two failed medications. Of all potential predictors tested in a binary logistic regression model, only annual referral volume predicted whether participants refer patients after three or more failed medications. Centers with high referral volume were 37% more likely to adhere to the guideline of referral after two failed medications. SIGNIFICANCE: Pediatric neurologists demonstrate fair knowledge of formal recommendations to refer patients for surgical evaluation after two failed medication trials, although referral rates remain unacceptably low. Participants continue to report that they would not refer patients with generalized electroencephalographic findings for surgical evaluation; this should continue to be addressed. Other modifiable factors reported, especially family perceptions of epilepsy surgery, should be prioritized when developing tools to enhance effective referrals and increase utilization of epilepsy surgery in the management of pediatric DRE.

Efficacy of Selective Dorsal Rhizotomy and Intrathecal Baclofen Pump in the Management of Spasticity.

BACKGROUND: Neurosurgical indications and interventions provided in the management of spasticity have evolved significantly over time. Selective dorsal rhizotomy (SDR) and intrathecal baclofen (ITB) pumps have been used to improve mobility, reduce lower extremity spasticity, and increase quality of life in patients with various diagnoses. METHODS: Studies describing ITB and SDR outcomes in adult and pediatric patients were identified from Medline and Embase databases. Only publications between January 1990 to January 2021 were included. Combinations of search terms 'Selective Dorsal Rhizotomy', 'Selective Posterior Rhizotomy', 'functional posterior rhizotomy', 'intrathecal baclofen pump', and 'spasticity' were used. Only studies in English language and those that included parameters for lower extremity outcome (i.e., spasticity, ambulation) were included. Only studies describing follow-up 12 months or greater were included. Case reports, reviews without primary data, or inaccessible publications were excluded. RESULTS: Two hundred and ninety publications between January 1990 to January 2021 were identified. Of these, 62 fit inclusion and exclusion criteria for a total of 1291 adult and 2263 patients. Etiologies in adult and pediatric populations varied substantially with multiple sclerosis, cerebral palsy, and trauma comprising the majority of causes for spasticity in adult patients. In pediatric patients, cerebral palsy was the predominant etiology of spasticity. While outcomes after SDR and ITB varied, both are effective for long-term tone reduction. SDR appeared to have a greater effect on function compared to baseline when comparing relatively similar subgroups. The complication rates for either intervention were significant; ITB had a much greater incidence of wound and hardware adverse events, whereas SDR was associated with a not insignificant incidence of new bladder or sensory deficit. CONCLUSION: ITB and SDR have demonstrated efficacy and utility for tone reduction in a variety of conditions. The selection of a specific intervention may have a variety of determining features including the etiology of spasticity, age of patient, as well as balancing benefit and complication profiles of each technique. Appropriate patient selection is essential for providing optimal patient outcomes.

Pediatric Chiari I malformation: novel and traditional measurements associated with syrinx and surgery.

PURPOSE: Multiple imaging parameters have been examined to estimate the presence of syrinx and the need for surgery in Chiari I patients (CM1); however, no consistent or definitive criteria have been proposed. The objective of this study was to review existing and identify novel radiological and clinical characteristics of CM1 patients that associate syrinx development and surgical intervention. METHODS: Patients with Chiari I malformation diagnosed on imaging between 0 and 18 years were retrospectively reviewed from January 1, 2007 to February 12, 2020. Participants were included if they had a baseline MRI of the head and spine prior to surgical intervention if required. Forty age-matched controls with cranial imaging were identified for comparison. Imaging parameters and clinical symptoms were recorded. RESULTS: A total of 122 CM1 patients were included in this study. Of the 122 patients, 28 (23%) had syrinx, and 27 (22%) had surgery. The following imaging parameters associated with syrinx and surgical intervention were identified: midbrain length (P < 0.001; P = 0.032), the obex position (P = 0.002; P < 0.001) and medullary kinking (P = 0.041; P < 0.001). Among the clinical features, the presence of overall pain (P = 0.017; P = 0.042), neck pain (P = 0.005; P = 0.027), and sensory dysfunction (P < 0.001) were found to be strongly associated with syrinx and surgery. CONCLUSION: While further investigation is needed, these specific radiological and clinical parameters should be considered when evaluating CM1 patients and may be used to guide further management.

Quality Indicators for Children With Traumatic Brain Injury After Transition to an American College of Surgeons Level I Pediatric Trauma Center.

OBJECTIVE: The aim of the study was to compare quality indicators, including frequency of acute surgical and emergent interventions, and resource utilization before and after American College of Surgeons (ACS) level I trauma verification among children with moderate or severe traumatic brain injury (TBI). METHODS: This is a retrospective review of patients younger than 18 years treated for moderate or severe TBI, as determined by International Classification of Disease codes. Our institution obtained ACS level I trauma verification in 2013. Outcomes during the pre-ACS (June 2003-May 2008), interim (June 2008-May 2013), and post-ACS (June 2013-May 2018) periods were compared via nonparametric tests. Tests for linear trend were conducted using Cochran-Armitage tests for categorical data and by linear regression for continuous variables. RESULTS: There were 677 children with moderate or severe TBIs (pre-ACS, 125; interim, 198; post-ACS, 354). Frequency of any surgical intervention increased significantly in the post-ACS period (12.2%) compared with interim (5.1%) and pre-ACS periods (5.6%, P = 0.007). More children in the post-ACS period required intracranial pressure monitoring (P = 0.017), external ventricular drain placement (P = 0.003), or endotracheal intubation (P = 0.001) compared with interim and pre-ACS periods. There was no significant change in time to operating room (P = 0.514), frequency of decompression (P = 0.096), or time to decompression (P = 0.788) between study periods. The median time to head CT decreased significantly in the post-ACS period (26 minutes; interquartile range [IQR], 9-60) compared with interim (36 minutes; IQR, 21-69) and pre-ACS periods (53 minutes; IQR, 36-89; P < 0.001). Frequency of repeat head computed tomography decreased significantly in the post-ACS period (30.2%) compared with interim (56.1%) and pre-ACS periods (64.0%, Ptrend = 0.044). CONCLUSIONS: Transition to an ACS level I trauma verification was associated with improvements in quality indicators for children with moderate or severe TBI.

Effect of Gene Mutation on Seizures in Surgery for Tuberous Sclerosis Complex.

BACKGROUND: Tuberous sclerosis complex (TSC) is a rare genetic disorder that commonly leads to drug-resistant epilepsy in affected patients. This study aimed to determine whether the underlying genetic mutation (TSC1 vs. TSC2) predicts seizure outcomes following surgical treatments for epilepsy. METHODS: We retrospectively assessed TSC patients using the TSC Natural History Database core registry. Data review focused on outcomes in patients treated with surgical resection or vagus nerve stimulation. RESULTS: A total of 42 patients with a TSC1 mutation, and 145 patients with a TSC2 mutation, were identified. We observed a distinct clinical phenotype: children with TSC2 mutations tended to be diagnosed with TSC at a younger age than those with a TSC1 mutation (p < 0.001), were more likely to have infantile spasms (p < 0.001), and to get to surgery at a later age (p = 0.003). Among this TSC2 cohort, seizure control following resective epilepsy surgery was achieved in less than half (47%) the study sample. In contrast, patients with TSC1 mutations tended to have more favorable postsurgical outcomes; seizure control was achieved in 66% of this group. CONCLUSION: TSC2 mutations result in a more severe epilepsy phenotype that is also less responsive to resective surgery. It is important to consider this distinct clinical disposition when counseling families preoperatively with respect to seizure freedom. Larger samples are required to better characterize the independent effects of genetic mutation, infantile spasms, and duration of epilepsy as they relate to seizure control following resective or neuromodulatory epilepsy surgery.

Systematic review on use and efficacy of selective dorsal rhizotomy (SDR) for the management of spasticity in non-pediatric patients.

PURPOSE: Selective dorsal rhizotomy (SDR) has been used to improve mobility and reduce lower extremity spasticity in patients with a various CNS conditions. Incidentally, literature on SDR has been performed in the pediatric population as such there is a paucity of research on the use in adult patients. METHODS: Studies describing SDR in adults were identified from Medline and Embase databases. Combinations of search terms "Selective Dorsal Rhizotomy," "Selective Posterior Rhizotomy," and "Adult" were used. Only literature in English language on patients over the age of 18 years and that included measures for lower extremity outcome (i.e., spasticity, mobility) were included. Case reports, reviews without primary data, or inaccessible publications were excluded. RESULTS: One hundred twenty-nine publications between 1970 and 2019 were identified. Twelve of these publications fit the inclusion criteria (n = 141 patients). In series where it was reported, SDR resulted in ambulatory improvement (54%, n = 44 out of 81), reduced spasticity (75.2%, n = 106 out of 141), and minimized muscle and joint pain (74.5%, n = 64 out of 86). SDR also showed improvement in parameters of the activities of daily life. 92.3% (n = 48 out of 52) of patients post-SDR developed new lower limb paresthesia. CONCLUSION: The success and efficacy appear durable in the short-term, but further follow-up is necessary to validate these findings. The goal of the intervention dictates the ideal adult patient for SDR. Patients seeking ambulatory improvement, any etiology of spasticity besides MS, seem favorable. Positive locomotive predictors include the ability to isolate lower extremity function, lack of contractures, lower limb strength, and post-SDR physiotherapy.

A protocol of situation-dependent transfusion, erythropoietin and tranexamic acid reduces transfusion in fronto-orbital advancement for metopic and coronal craniosynostosis.

PURPOSE: Assess the effect of a protocol of preoperative erythropoietin (EPO) and ferrous sulfate in addition to perioperative tranexamic acid (TXA) on blood transfusions in patients with coronal or metopic craniosynostosis undergoing cranial vault remodeling (CVR) with fronto-orbital advancement (FOA). METHODS: Retrospective review of all coronal and metopic craniosynostosis patients undergoing CVR and FOA from March 2010 to June 2019 was performed. Before 2014 ("Control group"), all patients received blood transfusion at the start of surgery. In 2014, a protocol of preoperative EPO and ferrous sulfate with perioperative TXA and non-automatic transfusion was instituted ("Study group"). Patient demographics and anthropometrics, perioperative hemoglobin (Hb) levels, and transfusion details were collected and compared. RESULTS: Thirty-six patients met inclusion criteria. Twenty-one patients were in the control group, and 15 in the Study group. Nineteen patients had metopic synostosis, 11 had unicoronal synostosis, and 6 had bicoronal synostosis. There were no significant differences between groups in demographics, operative time, intraoperative crystalloid volume, craniofacial syndromes, or sutures affected. The Study group had higher preoperative Hb (13.9 ± 1.0 vs. 12.6 ± 0.8 g/dL, p < 0.001), lower intraoperative Hb nadir (7.4 ± 1.8 vs. 9.2 ± 1.2 g/dL) lower intraoperative transfusion rate (66.7% vs. 100%, p = 0.008), lower postoperative transfusion rate (0% vs 28.6%, p = 0.03), and exposure to fewer unique units of packed red blood cells (0.7 ± 0.6 vs. 1.5 ± 0.9 units). CONCLUSION: Our protocol resulted in decreased transfusion needs. These results add valuable information to the growing body of work on transfusion reduction in craniosynostosis surgery.

Long term outcome of Selective Dorsal Rhizotomy for the management of childhood spasticity-functional improvement and complications.

BACKGROUND: Selective dorsal rhizotomy (SDR) for the management of lower extremity spasticity is a surgical technique that has existed since the 1900s. While much evidence supports its efficaciousness in reducing tone in the short term, limited information exists detailing the long-term outcome and evolution over time of patients undergoing SDR. METHODS: All publications with 10 years or more of outcome data on patients undergoing SDR were identified from Medline and Embase databases using the search term "Rhizotmy." Only publications that were in English included patients with cerebral palsy under the age of 21 and discussed SDR for lower extremity spasticity were included. Case reports, reviews without primary data, or publications not accessible online were excluded from the review. RESULTS: A total of 2128 publications were initially identified, of which 19 papers describing 1054 patients fit inclusion and exclusion criteria. GMFCS in most patients improved or remained stable over time after surgery, although durability of improvement and final outcome was dependent on initial functional status. Tone was noted to also substantially improve although a small proportion required additional oral or injectable pharmacologic agents and an even smaller proportion required baclofen pumps. Upper extremity function was also noted to improve substantially after SDR. A significant number of patients were independent for activities of daily living and were engaged in secondary education or gainful employment. A significant proportion of patients still required orthopedic surgery to the lower extremities after SDR, although the incidence of spinal deformity was not observed to be substantially elevated over that of natural history. CONCLUSION: SDR offers substantial improvements to a number of domains beyond pure tone reduction. These changes are durable over time, although patient selection is crucial in identifying those patients that will have the most benefit. Long-term follow-up is in important in this population given the potential need for further interventions that still exist in many patients.

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.

Interest and participation in global neurosurgery: a survey of Canadian neurosurgery residents.

OBJECTIVE: The lack of access to primary neurosurgical care presents a significant burden for patients in low- and middle-income countries (LMICs). Efforts to decrease the inequities in access to surgical care have become an increasing focus of initiatives and advocacy within the international neurosurgery community. Although residents may represent highly active members of international teams, the extent of their availability for and interest in global neurosurgery opportunities remains unclear. The objectives of this study were to assess the degree of participation, the level of interest, and the perceived barriers for residents of Canadian neurosurgery programs in engaging in neurosurgery initiatives in LMICs. METHODS: A cross-sectional survey was administered to 144 residents from all accredited Canadian neurosurgery residency programs. The survey was used to determine the level of participation and interest in global neurosurgery initiatives. RESULTS: The survey response rate from residents at Canadian neurosurgery programs was 45/144 (31%), although not all respondents answered every question; thus, the denominator for each question is not always 45. Most neurosurgery residents (n = 30, 68%) were unaware of any global neurosurgery opportunities available during residency. Despite 64% of residents having an interest in participation (strongly agree/agree; n = 28), with most residents planning on participating as attending staff (strongly agree/agree; n = 28, 64%), only 3 residents (7%) had taken part in LMIC neurosurgery initiatives. The most cited perceived limitations for participation were lack of time (n = 37, 84%) and finances (n = 26, 59%). CONCLUSIONS: Among surveyed residents from Canadian neurosurgery programs, there was a low participation rate in global neurosurgery initiatives and limited knowledge of available opportunities, despite relatively high interest. Many respondents identified time and financial constraints as barriers to participation. Many residents felt that interest could increase were there a structured program dedicated to global neurosurgery available in their residency program.

Minimizing transfusion in sagittal craniosynostosis surgery: the Children's Hospital of Minnesota Protocol.

PURPOSE: To assess the success of a protocol using preoperative erythropoietin (EPO) and iron with perioperative tranexamic acid (TXA) in reducing blood transfusion in sagittal craniosynostosis surgery. METHODS: A retrospective chart review of all sagittal craniosynostosis patients undergoing open repair at our institution since 2010 was conducted. A novel protocol of preoperative EPO with iron and perioperative TXA, along with a shift away from automatic transfusion, was initiated in 2014. Perioperative hemoglobin levels, length of stay, and transfusion rates were compared between the historical control and the study group receiving the protocol. RESULTS: A total of 36 patients met inclusion criteria. Twenty-eight patients were male and 8 were female. Twenty-two patients were in the control group receiving neither TXA nor EPO and automatically received a transfusion, while 14 were in the study group and received the full protocol. There were no significant demographic differences between groups. Within the control group, 100% of patients were transfused compared with 14.3% of the study group (p < 0.0001). The study group also had a shorter postoperative length of stay in the hospital (mean, 3.4 days; range, 3-6) than the control (mean, 4 days; range, 2-5.5, p = 0.038). The study group had a higher preoperative hemoglobin than the control (13.6 vs. 11.8 g/dL, p = 0.0001). CONCLUSION: Our protocol of preoperative EPO and iron with perioperative TXA increased the preoperative hemoglobin and was associated with a low transfusion rate without negatively impacting postoperative course.

Dynamic MRI in the Evaluation of Atlantoaxial Stability in Pediatric Down Syndrome Patients.

BACKGROUND/AIMS: Down syndrome is the most common inherited disorder. Some patients develop atlantoaxial instability. Existing screening guidelines were developed prior to availability of MRI. We present predictors for deficit using dynamic MRI of the craniocervical junction. METHODS: A retrospective review of Down syndrome patients from 2001 to 2015 was carried out. Patients were considered symptomatic if they had clinical deficits or signal change on MRI. Measurements were taken at the atlantoaxial junction and structural abnormalities noted. Analysis was performed with SPSS. RESULTS: A total of 36 patients were included. Patients averaged 93 months of age with a follow-up of 57 months. No asymptomatic patients developed myelopathy during follow-up. During dynamic imaging, symptomatic patients had greater changes in space available for the cord (SAC) (5.2 vs. 2.7 mm; p < 0.001) and atlantodental interval (ADI) (2.8 vs. 1.3 mm; p = 0.04). These patients were also more likely to have a bony anomaly (50 vs. 13%; p = 0.03). CONCLUSION: This study characterizes the range of motion seen on dynamic MRI and provides parameters that can be used to distinguish patients at risk for neurologic injury. Changes greater than 3 mm in ADI or 5 mm in SAC during dynamic MRI or any bony abnormality warrants further investigation. Patients without these features may be able to avoid an unnecessary intervention.

Cranial Rhabdomyosarcoma Masquerading as Infectious Mastoiditis: Case Report and Literature Review.

BACKGROUND: Rhabdomyosarcoma originating in the mastoid is rare and may be misdiagnosed as an infectious mastoiditis due to overlapping clinical and imaging features. We aimed to identify distinguishing characteristics to facilitate earlier diagnosis and treatment. METHOD: Here we describe a case report and a systematic review of 23 reports describing previous cases of mastoid rhabdomyosarcoma. We compare these patients to a systematic review of patients with infectious mastoiditis and identify distinguishing clinical features. RESULTS: A total of 43 patients with rhabdomyosarcoma of the mastoid were identified and compared with patients with mastoiditis. Rhabdomyosarcoma patients were more likely to present with a mass (86%) or cranial nerve dysfunction (83.7%), while mastoiditis patients were more likely to have fever (72.4%), pain (48.2%), and present at a younger age (4.4 vs. 6.1 years). The average lifespan with rhabdomyosarcoma of the mastoid was 7.1 months after diagnosis, with 41.7% of patients alive at the time of report. CONCLUSIONS: Based on abstracted and aggregated information, we identified unique features observed more frequently in each of rhabdomyosarcoma and mastoiditis. These predictive features allow for the differentiation of each diagnosis and avoid the delay of proper treatment.

Proliferative Index in Pediatric Pilocytic Astrocytoma by Region of Origin and Prediction of Clinical Behavior.

BACKGROUND/AIMS: Pilocytic astrocytomas are common pediatric tumors. Molecular profiles vary with location of origin. Comparisons of proliferation have not been reported. We sought to identify differences in growth by region and whether these predict clinical behavior. METHODS: A retrospective review of all patients undergoing surgery for a pilocytic astrocytoma at Children's Hospital LA from 2003 to 2015 was completed. Tumor location, determined by imaging, was stratified into infratentorial, supratentorial, or optic pathway. Proliferation was measured by Ki-67 immunostaining. A p value of 0.05 was deemed significant. RESULTS: 77 patients were identified. 51 had posterior fossa tumors, 12 had supratentorial tumors, and 14 had optic pathway tumors. Mean Ki-67 score was 3.67, 4.09, and 3.83%, respectively (p = 0.82). Ki-67 of ≥4% trended towards recurrence (p = 0.11), incomplete resection (p = 0.15), and younger age at presentation (p = 0.04). Ki-67 was weakly correlated with shorter survival after surgery (r = -0.103, p = 0.41). Partial resection strongest predicted recurrence (p < 0.001; OR = 13.0). CONCLUSION: Proliferative index does not change by location. Higher cell proliferation was seen in younger patients and associated with shorter time to and a higher risk of recurrence. Further study is needed to identify predictors for clinical behavior. Importance of Study: This study provides a detailed analysis of the proliferative indices of tumors arising from characteristic locations within the brain. With recent advances in our understanding of the differences in molecular and genetic profiles despite similar histologic diagnoses, we felt that it was important to review whether there were unique components of tumor behavior that could be identified. In turn, we sought to determine whether tumor behavior could be used to predict the clinical course. This knowledge is important, given that not every tumor may undergo complete surgical resection, and that some lesions may require more aggressive upfront adjuvant therapy or be closely monitored for recurrence.