Predictors of syrinx presentation and outcomes in pediatric Chiari malformation type I: a single institution experience of 218 consecutive syrinx patients.

PURPOSE: Chiari I malformation (CM-I) in pediatric patients can impose substantial neurologic and functional impairment. Additionally, the presence of syrinx is often a harbinger of clinical compromise, but little attention has been devoted to identifying features associated with syrinx development and the clinical impact of syrinx resolution. Therefore, this study aims to identify clinical and radiographic variables associated with preoperative syrinx presence and postoperative syrinx reduction in pediatric patients with CM-I and determine the relationship between postoperative syrinx reduction and clinical symptom improvement. METHODS: The authors performed a retrospective analysis of 435 consecutive pediatric patients who underwent surgical treatment of CM-I from 2001 to 2021 at a single tertiary pediatric medical center. All patients underwent pre- and postoperative MRI, and clinical and radiographic variables were recorded and subject to inferential analysis. RESULTS: Syrinx at presentation was independently associated with symptoms of spinal cord dysfunction at presentation (OR 2.17 (95% CI 1.05-4.48); p = 0.036), scoliosis (OR 5.33 (2.34-10.86); p = 0.001), and greater pB-C2 (posterior basion to C2 distance) measurement length (OR 1.14 (95% CI 1.01-1.30); p = 0.040). Syrinx at presentation was inversely associated with tussive headaches at presentation (OR 0.27 (95% CI 0.16-0.47); p = 0.001) and cranial nerve deficits at presentation (OR 0.49 (95% CI 0.26-0.92); p = 0.025). Postoperatively, patients with radiographic evidence of syrinx improvement had greater rates of symptom improvement (93.1% vs 82.1%; p = 0.049), better CCOS scores (15.4 vs 14.2; p = 0.001), and decreased rates of readmission (6.0% vs 25.0%, p = 0.002) and reoperation (0.5% vs 35.7%; p = 0.001). The difference in syrinx resolution was similar but not statistically significant (10.3% vs 16.7%; p = 0.251). AO joint anomaly (OR 0.20, 95% CI 0.04-0.95; p = 0.026) and foramen magnum diameter (OR 1.12, 95% CI 1.00-1.25; p = 0.049) were the only independent predictors of syrinx improvement, and surgical technique was the only predictor for syrinx resolution (OR 2.44, 95% CI 1.08-5.50; p = 0.031). Patients that underwent tonsil reduction surgery whose syrinx improved had a wider foramen magnum diameter than those whose did not improve (34.3 vs 31.7; p = 0.028). CONCLUSIONS: Radiographic syrinx improvement is associated with greater rates of symptom improvement and less readmissions and reoperations for CM-I. AO joint anomalies and narrower foramen magnums were independent risk factors for the lack of syrinx improvement. These novel insights will help guide preoperative patient counseling, pre- and intraoperative surgical decision-making, and postoperative clinical prognostication in the treatment of pediatric CM-I.

Risk factors for clinical and radiological worsening following Chiari malformation type I surgery in the pediatric population.

OBJECTIVE: Foramen magnum (FM) decompression with or without duraplasty is considered a common treatment strategy for Chiari malformation type I (CM-I). The authors' objective was to determine a predictive model of risk factors for clinical and radiological worsening after CM-I surgery. METHODS: A retrospective review of electronic health records was conducted at an academic tertiary care hospital from 2001 to 2019. A multivariable Cox proportional hazards regression model was used to determine the risk factors. The Kaplan-Meier estimate was plotted to delineate outcomes based on FM size. FM was measured as the preoperative distance between the basion and opisthion and dichotomized into < 34 mm and ≥ 34 mm. Syrinx was measured preoperatively and postoperatively in the craniocaudal and anteroposterior directions using a T2-weighted MRI sequence. RESULTS: A total of 454 patients (231 females [50.9%]) with a median (range) age of 8.0 (0-18) years were included in the study. The median duration of follow-up was 21.0 months (range 3.0-144.0 years). The model suggested that patients with symptoms consisting of occipital/tussive headache (HR 4.05, 95% CI 1.34-12.17, p = 0.01), cranial nerve symptoms (HR 3.46, 95% CI 1.16-10.2, p = 0.02), and brainstem/spinal cord symptoms (HR 3.25, 95% CI 1.01-11.49, p = 0.05) had higher risk, whereas those who underwent arachnoid dissection/adhesion lysis had 75% lower likelihood (HR 0.25, 95% CI 0.10-0.64, p = 0.004) of clinical worsening postoperatively. Similarly, patients with evidence of brainstem/spinal cord symptoms (HR 7.9, 95% CI 2.84-9.50, p = 0.03), scoliosis (HR 1.04, 95% CI 1.01-2.80, p = 0.04), and preoperative syrinx (HR 16.1, 95% CI 1.95-132.7, p = 0.03) had significantly higher likelihood of postoperative worsening of syrinx. Patients with symptoms consisting of occipital/tussive headache (HR 5.44, 95% CI 1.86-15.9, p = 0.002), cranial nerve symptoms (HR 2.80, 95% CI 1.02-7.68, p = 0.04), and nonspecific symptoms (HR 6.70, 95% CI 1.99-22.6, p = 0.002) had significantly higher likelihood, whereas patients with FM ≥ 34 mm and those who underwent arachnoid dissection/adhesion lysis had 73% (HR 0.27, 95% CI 0.08-0.89, p = 0.03) and 70% (HR 0.30, 95% CI 0.12-0.73, p = 0.008) lower likelihood of reoperation, respectively. The Kaplan-Meier curve showed that patients with FM size ≥ 34 mm had significantly better clinical (p = 0.02) and syrinx (p = 0.03) improvement postoperatively when the tonsils were resected. CONCLUSIONS: These results showed that preoperative and intraoperative factors may help to provide better clinical decision-making for CM-I surgery. Patients with FM size ≥ 34 mm may have better outcomes when the tonsils are resected.

Postoperative surveillance of pediatric cerebellar pilocytic astrocytoma.

The purpose of this study was to identify the optimal frequency and duration of magnetic resonance imaging follow-up in children who had gross totally resected cerebellar pilocytic astrocytomas (CPAs). Our hypothesis was that following two MR examinations, separated by at least 3 months, showing no evidence of tumor, gross totally resected CPAs did not recur and no further imaging follow-up was necessary. Retrospective review of Neuro-Oncology database from 1/2000 to 7/2013 yielded 53 patients with CPAs that had preoperative imaging and >2 years post-operative imaging follow-up available. Pilocytic astrocytomas with brainstem involvement and patients with neurofibromatosis type I were excluded. Preoperative tumor volumes were calculated. The dates and reports of the examinations were tabulated. The median number of follow-up examinations was 9 over a median follow-up time of 6.05 years (2.07-12.28 years). Two consecutive MR examinations over at least a 3 month span demonstrated the smallest negative likelihood ratio of future recurrence (0.15). There was no association of recurrence with preoperative tumor volume. Among the 35 patients with gross total resection of their tumor and greater than two negative follow-up examinations, one recurrence (2.9 %) was identified, occurring 6.4 years after initial resection. Gross totally resected pediatric CPAs can recur, but this is exceedingly rare. Frequent surveillance (every 3-6 months) is suggested in patients with CPAs until absence of tumor is concluded on imaging and documented on two consecutive studies spaced at least 3 months apart. The likelihood of recurrence thereafter is low.

Management of infectious intracranial aneurysms in the pediatric population.

INTRODUCTION: Infectious intracranial aneurysms (IIAs) account for approximately 15 % of all pediatric intracranial aneurysms. Histologically, they are pseudoaneurysms that develop in response to an inflammatory reaction within the adventitia and muscularis layers, ultimately resulting in disruption of both the internal elastic membrane and the intima. The majority of pediatric IIAs are located within the anterior circulation, and they can be multiple in 15-25 % of cases. BACKGROUND: The most common presentation for an IIA is intracerebral and/or subarachnoid hemorrhage. In children with a known diagnosis of infective endocarditis who develop new neurological manifestations, it is imperative to exclude the existence of an IIA. The natural history of untreated infectious aneurysms is ominous; they demonstrate a high incidence of spontaneous rupture. High clinical suspicion, prompt diagnosis, and adequate treatment are of paramount importance to prevent devastating neurological consequences. DISCUSSION: The prompt initiation of intravenous broad-spectrum antibiotics represents the mainstay of treatment. Three questions should guide the management of pediatric patients with IIAs: (a) aneurysm rupture status, (b) the presence of intraparenchymal hemorrhage or elevated intracranial pressure, and (c) relationship of the parent vessel to eloquent brain tissue. Those three questions should orient the treating physician into either antibiotic therapy alone or in combination with microsurgical or endovascular interventions. This review discusses important aspects of the epidemiology, the diagnosis, and the management of IIAs in the pediatric population.

Tectal Lesions in Children: A Long-Term Follow-Up Volumetric Tumor Growth Analysis in Surgical and Nonsurgical Cases.

BACKGROUND/AIMS: Different tectal masses have been described; most are low-grade gliomas. Only 20-30% of all lesions grow, as shown on follow-up MRIs, requiring surgical resection at some point. The aim of this study is to describe the experience of a single institution managing pediatric patients with tectal lesions. METHODS: We retrospectively studied and analyzed 40 children with tectal lesions managed from 1990 to 2006; the mean age at diagnosis was 9.4 years. A volumetric classification was used to analyze tumor growth trends. More than 1 year of imaging follow-up was available for 23 patients. RESULTS AND CONCLUSION: Medium- and large-volume-size lesions were associated with the need for surgery. About half of the nonsurgical lesions grew at least 50% over a period of 4.5 years and did not require surgical resection.

Management of cerebrospinal fluid pseudocysts in the laparoscopic age.

OBJECTIVE: Abdominal CSF pseudocysts are an uncommon but challenging complication of ventriculoperitoneal shunts. Pseudocysts consist of a loculated intraperitoneal compartment that inadequately absorbs CSF and may be infected or sterile at diagnosis. The treatment goal is to clear infection if present, reduce inflammation, and reestablish long-term function in an absorptive (intraperitoneal) space. This aim of this paper was to study the efficacy of primary laparoscopic repositioning of the distal shunt catheter for treatment of sterile abdominal CSF pseudocysts. METHODS: All patients treated for abdominal CSF pseudocysts at Dallas Children's Health from 1991 to 2021 were retrospectively reviewed. Patient history and pseudocyst characteristics were analyzed, with a primary outcome of pseudocyst recurrence at 1 year. RESULTS: Of 92 primary pseudocysts, 5 initial treatment strategies (groups) were used depending on culture status, clinical history, and surgeon preference: 1) shunt explant/external ventricular drain (EVD) placement (23/92), 2) distal tubing externalization (13/92), 3) laparoscopic repositioning (35/92), 4) open repositioning (4/92), and 5) other methods such as pseudocyst drainage or direct revision to another terminus (17/92). Seventy pseudocysts underwent shunt reimplantation in the peritoneal space. The 1-year peritoneal shunt survival for groups 1 and 2 combined was 90%, and 62% for group 3. In group 3, 1-year survival was better for those with normal systemic inflammatory markers (100%) than for those with high markers (47%) (p = 0.042). In a univariate Cox proportional hazards model, the risk of pseudocyst recurrence was increased if the most recent abdominal procedure was a nonshunt abdominal surgery (p = 0.012), and it approached statistical significance with male sex (p = 0.054) and elevated inflammatory markers (p = 0.056. Multivariate Cox analysis suggested increased recurrence risk with male sex (p = 0.05) and elevated inflammatory markers (p = 0.06), although the statistical significance threshold was not reached. The length of hospital stay was shorter for laparoscopic repositioning (6 days) than for explantation/EVD placement (21 days) (p < 0.0001). Ultimately, 62% of patients had a peritoneal terminus at the last follow-up, 33% (n = 30) had an extraperitoneal terminus (19 pleura, 8 right heart, and 3 gallbladder), and 5 patients were shunt free. CONCLUSIONS: Some sterile pseudocysts with normal systemic inflammatory markers can be effectively treated with laparoscopic repositioning, resulting in a significantly shorter hospitalization and modestly higher recurrence rate than shunt explantation.

A survey of the members of the American Society of Pediatric Neurosurgeons and factors associated with well-being.

OBJECTIVE: The goal of this study was to survey the members of the American Society of Pediatric Neurosurgeons (ASPN) to assess the prevalence and associated risks of burnout among pediatric neurosurgeons. The authors aimed to identify the factors that most significantly contributed to this risk to provide a baseline group of characteristics to improve physician well-being. METHODS: Institutional Review Board approval from the University of Arizona was obtained, and the 7-question and 9-question Mayo Physician Well-Being Index (WBI) was distributed to members of the ASPN (n = 275). This index screens for many different aspects of distress for physicians, including burnout risk, stress, depression, fatigue, suicidal ideation, and low career satisfaction. RESULTS: An analysis of 111 pediatric neurosurgeons (111/275 [40% response rate]) was completed. Respondent ages were distributed, with those aged 56-60 years representing the highest proportion (20%). University practice represented a majority (72%). One-third (32%) of respondents reported practicing greater than 25 years, and most physicians in the survey were married (76%). One-third of surgeons spend 61-70 hours working per week (33%), and a plurality are on call between 6 and 10 days per month (42%). Most surgeons reported treating fewer than 200 cases per year (37% reported 100-150 cases; 23%, 151-200). Most pediatric neurosurgeons (63%) stated their annual salary was sufficient. Analysis of each WBI question was performed to identify which factors specifically contributed to the risk of burnout. An overwhelming majority of respondents reported that they make significant efforts to do at least one thing each week that brings them joy (97%), and they either agree or strongly agree that they perform meaningful work (98% of all participants, 97% of females, and 98% of men, p = 0.010). Nearly half of all respondents (49%) reported feelings of burnout and a majority of them were female (67% of women and 42% of men, p = 0.021). Time, environment, case volumes, and quality-of-life concerns are all factors that significantly contribute to the overall risk of burnout and well-being. CONCLUSIONS: This survey study of the ASPN membership revealed a 49% rate of burnout with females at higher risk (67%). Factors associated with burnout were salary, more than 10 days on call per month, electronic medical record stressors, and work-life incongruity. The aforementioned notwithstanding, respondents believe pediatric neurosurgery is a meaningful career. This study provides evidence supporting a moral imperative toward recognition of burnout symptoms and a pivot point toward implementing change.

Common pediatric cerebellar tumors: correlation between cell densities and apparent diffusion coefficient metrics.

PURPOSE: To test whether there is correlation between cell densities and apparent diffusion coefficient (ADC) metrics of common pediatric cerebellar tumors. MATERIALS AND METHODS: This study was reviewed for issues of patient safety and confidentiality and was approved by the Institutional Review Board of the University of Texas Southwestern Medical Center and was compliant with HIPAA. The need for informed consent was waived. Ninety-five patients who had preoperative magnetic resonance imaging and surgical pathologic findings available between January 2003 and June 2011 were included. There were 37 pilocytic astrocytomas, 34 medulloblastomas (23 classic, eight desmoplastic-nodular, two large cell, one anaplastic), 17 ependymomas (13 World Health Organization [WHO] grade II, four WHO grade III), and seven atypical teratoid rhabdoid tumors. ADCs of solid tumor components and normal cerebellum were measured. Tumor-to-normal brain ADC ratios (hereafter, ADC ratio) were calculated. The medulloblastomas and ependymomas were subcategorized according to the latest WHO classification, and tumor cellularity was calculated. Correlation was sought between cell densities and mean tumor ADCs, minimum tumor ADCs, and ADC ratio. RESULTS: When all tumors were considered together, negative correlation was found between cellularity and mean tumor ADCs (ρ = -0.737, P < .05) and minimum tumor ADCs (ρ = -0.736, P < .05) of common pediatric cerebellar tumors. There was no correlation between cellularity and ADC ratio. Negative correlation was found between cellularity and minimum tumor ADC in atypical teratoid rhabdoid tumors (ρ = -0.786, P < .05). In atypical teratoid rhabdoid tumors, no correlation was found between cellularity and mean tumor ADC and ADC ratio. There was no correlation between the ADC metrics and cellularity of the pilocytic astrocytomas, medulloblastomas, and ependymomas. CONCLUSION: Negative correlation was found between cellularity and ADC metrics of common pediatric cerebellar tumors. Although ADC metrics are useful in the preoperative diagnosis of common pediatric cerebellar tumors and this utility is generally attributed to differences in cellularity of tumors, tumor cellularity may not be the sole determinant of the differences in diffusivity.

Cerebellar tonsil reduction for surgical treatment of Chiari malformation type I in children.

OBJECTIVE: The goal of this study was to review the efficacy and safety of different surgical techniques used for treatment of Chiari malformation type I (CM-I) in children. METHODS: The authors retrospectively reviewed 437 consecutive children surgically treated for CM-I. Procedures were classified into four groups: bone decompression (posterior fossa decompression [PFD]) and duraplasty (PFD with duraplasty [PFDD]), PFDD with arachnoid dissection (PFDD+AD), PFDD with tonsil coagulation of at least one cerebellar tonsil (PFDD+TC), and PFDD with subpial tonsil resection of at least one tonsil (PFDD+TR). Efficacy was measured as a greater than 50% reduction in the syrinx by length or anteroposterior width, patient-reported improvement in symptoms, and rate of reoperation. Safety was measured as the rate of postoperative complications. RESULTS: The mean patient age was 8.4 years (range 3 months to 18 years). In total, 221 (50.6%) patients had syringomyelia. The mean follow-up was 31.1 months (range 3-199 months), and there was no statistically significant difference between groups (p = 0.474). Preoperatively, univariate analysis showed that non-Chiari headache, hydrocephalus, tonsil length, and distance from the opisthion to brainstem were associated with the surgical technique used. Multivariate analysis demonstrated that hydrocephalus was independently associated with PFD+AD (p = 0.028), tonsil length was independently associated with PFD+TC (p = 0.001) and PFD+TR (p = 0.044), and non-Chiari headache was inversely associated with PFD+TR (p = 0.001). In the treatment groups postoperatively, symptoms improved in 57/69 (82.6%) PFDD patients, 20/21 (95.2%) PFDD+AD patients, 79/90 (87.8%) PFDD+TC patients, and 231/257 (89.9%) PFDD+TR patients, and differences between groups were not statistically significant. Similarly, there was no statistically significant difference in postoperative Chicago Chiari Outcome Scale scores between groups (p = 0.174). Syringomyelia improved in 79.8% of PFDD+TC/TR patients versus only 58.7% of PFDD+AD patients (p = 0.003). PFDD+TC/TR remained independently associated with improved syrinx outcomes (p = 0.005) after controlling for which surgeon performed the operation. For those patients whose syrinx did not resolve, no statistically significant differences between surgery groups were observed in the length of follow-up or time to reoperation. Overall, there was no statistically significant difference between groups in postoperative complication rates, including aseptic meningitis and CSF- and wound-related issues, or reoperation rates. CONCLUSIONS: In this single-center retrospective series, cerebellar tonsil reduction, by either coagulation or subpial resection, resulted in superior reduction of syringomyelia in pediatric CM-I patients, without increased complications.

Delivery of Nusinersen Through an Ommaya Reservoir in Spinal Muscular Atrophy.

OBJECTIVE: Spinal muscular atrophy (SMA) is a common rare neuromuscular disease responsible for very high mortality during infancy and high morbidity during childhood and adolescence. It is caused by autosomal recessive mutations in the survival motor neuron gene. In 2016, the Food and Drug Administration approved the first disease modifying therapy for use in all patients of any age. Nusinersen is an antisense oligonucleotide that showed dramatic benefits with achievement of motor milestones in infants and improved gross motor function in children. METHODS: This was a retrospective chart review of all SMA patients seen at a single site between 2016 and 2020 for treatment with nusinersen. RESULTS: We report 8 patients who underwent placement of an Ommaya reservoir and lumbosacral catheter for drug delivery. Complications included infection and revisions due to catheter separation. One patient required fluoroscopy for injections because of location of port site. CONCLUSION: We conclude that placement of an Ommaya port is a viable option for patients who have challenges for access to intrathecal space. Practical innovations have the potential to control administration costs, achieve therapeutic value, and promote patient safety.

Thirty-day readmission risk after intracranial tumor resection surgeries in children.

OBJECTIVE: The risk of readmission after brain tumor resection among pediatric patients has not been defined. The authors' objective was to evaluate the readmission rates and predictors of readmission after pediatric brain tumor resection. METHODS: Nationwide Readmissions Database (NRD) data sets from 2010 to 2014 were searched for unplanned readmissions within 30 days of the discharge date after pediatric brain tumor resection. Patient demographic variables included sex, age, expected payment source (Medicaid or private insurance), and median annual household income. Readmission events for chemotherapy, radiation therapy, or further tumor resection were not included. RESULTS: Of 282 patients (12.7%) readmitted within 30 days of the index event, the median time to readmission was 10 days (IQR 5-19 days). The most common reason for readmission was hydrocephalus, which accounted for 19% of readmission events. Other CNS-related complications (24%), surgical site infections or septicemia (14%), seizures (7%), and hematological disorders (7%) accounted for other major readmission events. The median charge for readmission events was $35,431, and the median length of readmission stay was 4 days. In multivariate regression, factors associated with a significant increase in readmission risk included Medicaid as the primary payor, discharge from the index event with home health services, and fluid and electrolyte disorders during the index event. CONCLUSIONS: More than 10% of pediatric brain tumor patients have unplanned readmission events within 30 days of discharge after tumor resection. Medicaid patients and those with preoperative or early postoperative fluid and electrolyte disturbances may benefit from early or frequent outpatient visits after tumor resection.

Inchoate guidelines of endoscopic resection of colloid cysts.

Colloid cyst are cystic lesions in the third ventricle and could render patients asymptomatic. However, there is an inherent risk of symptomatic progression, acute decompensation, and sudden death. Therefore, there is no clear consensus as how to observe or when to treat a newly diagnosed patient with a colloid cyst. The authors' objective is to identify the risk factors and then develop a risk stratification score to guide neurosurgeons during acute or chronic presentation. Radiological imaging characteristics have been outlined for the risk stratification as well preoperative evaluation. A baseline neuropsychological evaluation is helpful to obtain during an incidental presentation because history and neurological examination could be inconclusive in these cases. Radiological imaging with an MRI brain scan plays a vital role for the initial screening (determination of the cyst size, exact location, and the imaging characteristics) as well as for the preoperative planning. Stereotactic guidance is a high yield, followed by neuroendoscopic resection of the colloid cyst has been an established approach to resect these lesions. Modified colloid cyst risk scoring (mCCRS) system is robust and detailed for the optimal risk stratification of colloid cyst presentation. Stereotactic guided neuroendoscopic resection of the colloid cyst is a safe and efficacious approach to manage these lesions. The intended use, crucial steps involved, and the limitations of the technique have been discussed especially with a focus on the recurrence. Moreover, a comprehensive treatment algorithm has been presented.

Diffuse intrinsic pontine gliomas: Diagnostic approach and treatment strategies.

Diffuse intrinsic pontine gliomas (DIPG) are high grade gliomas of the brainstem with fatal outcomes. Radiation is known to be partially effective to control the immediate flare but relapse is frequent. There has been ongoing research to study the role of molecular subgroups and identification of specific targets but this is not possible with histopathological diagnosis alone. The authors' objective is to highlight the need for and discuss ongoing molecular research. There is an inherent need for the availability of tumor tissue to be able to conduct research studies. The authors advocate the use of neuronavigation assisted stereotactic technique for tumor biopsy. The technique is feasible with a predefined surgical trajectory. After obtaining tissue diagnosis further work can be performed to isolate and identify histone protein genetic mutations and methylation changes responsible for DIPG molecular subgrouping. Moreover, convection enhanced delivery of therapeutic agents is being developed for better instillation of future drug agents. Despite identification of genetic/epigenetic mutations, growth factors, receptors, and tissue biomarkers, the oncogenesis of DIPG remains elusive. The authors' effort to provide a comprehensive review on DIPG to better understand the disease, need for tissue diagnosis, described surgical technique, and need for pre-clinical and clinical future research is novel.

Health care transition in pediatric neurosurgery: a consensus statement from the American Society of Pediatric Neurosurgeons.

OBJECTIVE: The number of children with complex medical conditions surviving to adulthood is increasing. A planned transition to adult care systems is essential to the health maintenance of these patients. Guidance has been established for the general health care transition (HCT) from adolescence to adulthood. No formal assessment of the performance of pediatric neurosurgeons in HCT has been previously performed. No "best practice" for this process in pediatric neurosurgery currently exists. The authors pursued two goals in this paper: 1) define the current state of HCT in pediatric neurosurgery through a survey of the membership of the American Society of Pediatric Neurosurgeons (ASPN) on current methods of HCT, and 2) develop leadership-endorsed best-practice guidelines for HCT from pediatric to adult neurosurgical health care. METHODS: Completion of the Current Assessment of Health Care Transition Activities survey was requested of 178 North American pediatric neurosurgeons by using a web-based questionnaire to capture HCT practices of the ASPN membership. The authors concurrently conducted a PubMed/MEDLINE-based literature review of HCT for young adults with special health care needs, surgical conditions, and/or neurological conditions for the period from 1990 to 2018. Selected articles were assembled and reviewed by subject matter experts and members of the ASPN Quality, Safety, and Advocacy Committee. Best-practice recommendations were developed and subjected to peer review by external expert groups. RESULTS: Seventy-six responses to the survey (43%) were received, and 62 respondents (82%) answered all 12 questions. Scores of 1 (lowest possible score) were recorded by nearly 60% of respondents on transition policy, by almost 70% on transition tracking, by 85% on transition readiness, by at least 40% on transition planning as well as transfer of care, and by 53% on transition completion. Average responses on all core elements were < 2 on the established 4-point scale. Seven best-practice recommendations were developed and endorsed by the ASPN leadership. CONCLUSIONS: The majority of pediatric neurosurgeons have transition practices that are poor, do not meet the needs of patients and families, and should be improved. A structured approach to transition, local engagement with adult neurosurgical providers, and national partnerships between pediatric and adult neurosurgery organizations are suggested to address current gaps in HCT for patients served by pediatric neurosurgeons.

Dental management of scleroderma patients using pentoxifylline plus vitamin E with and without TheraBite® to reduce trismus: Two case reports and brief review of literature.

To provide evidence supporting the off label use of pentoxifylline and vitamin E especially by dentists with TheraByte to reduce trismus in scleroderma patients.

Margin-Free Fractionated Stereotactic Radiation Therapy for Pediatric Brain Tumors.

PURPOSE: Conventional radiation therapy (RT) to pediatric brain tumors exposes a large volume of normal brain to unwarranted radiation causing late toxicity. We hypothesized that in well demarcated pediatric tumors lacking microscopic extensions, fractionated stereotactic RT (SRT), without target volume expansions, can reduce high dose normal tissue irradiation without affecting local control. METHODS AND MATERIALS: Between 2008 and 2017, 52 pediatric patients with brain tumors were treated using the CyberKnife (CK) with SRT in 180 to 200 cGy per fraction. Thirty representative cases were retrospectively planned for intensity modulated RT (IMRT) with 4-mm PTV expansion. We calculated the volume of normal tissue within the high or intermediate dose region adjacent to the target. Plan quality and radiation dose-volume dosimetry parameters were compared between CK and IMRT plans. We also reported overall survival, progression-free survival (PFS), and local control. RESULTS: Tumors included low-grade gliomas (n = 28), craniopharyngiomas (n = 16), and ependymomas (n = 8). The volumes of normal tissue receiving high (≥80% of prescription dose or ≥40 Gy) or intermediate (80% > dose ≥50% of the prescription dose or 40 Gy > dose ≥25 Gy) dose were significantly smaller with CK versus IMRT plans (P < .0001 for all comparisons). With a median follow-up of 3.7 years (range, 0.1-9.0), 3-year local control was 92% for all patients. Eight failures occurred: 1 craniopharyngioma (marginal), 2 ependymomas (both in-field), and 5 low-grade gliomas (2 in-field, 1 marginal, and 2 distant). CONCLUSIONS: Fractionated SRT using CK without target volume expansion appears to reduce the volume of irradiated tissue without majorly compromising local control in pediatric demarcated brain tumors. These results are hypothesis generating and should be tested and validated in prospective studies.

Concomitant ruptured anterior circulation and unruptured posterior circulation aneurysms: Treatment strategy and review of literature.

Basilar artery apex or bifurcation is the most common location for aneurysms arising from posterior cerebral circulation. Reports of unruptured aneurysms of the basilar bifurcation associated with ruptured anterior circulation aneurysms are rare. The presence of multiple intracranial aneurysms poses a significantly high risk to management than a single aneurysm due several factors involved. Surgical management is considered the best treatment modality for most aneurysmal types and location with quite a few limitations when applicable. Authors have conducted a literature review of anterior and posterior circulation concomitant aneurysms and report their own experience with a case of anterior communicating artery blister type aneurysmal rupture presented with the symptoms and signs of subarachnoid hemorrhage concomitant with an unruptured basilar artery bifurcation aneurysm. Moreover, the anomalous origin of thalamoperforators at the basilar apex instead of the posterior cerebral artery makes it reasonably challenging for the microsurgical clipping. Discussed is the clinical presentation, radiological studies obtained, surgical approach utilized with an adequate exposure of the entire circle of Willis as well as the critical decision making when managing these challenging cases.

Oculomotor Schwannomas: A Systematic Review and Report of Two Pediatric Cases Treated with Fractionated Cyberknife Stereotactic Radiotherapy.

OBJECTIVE: Pediatric oculomotor nerve schwannomas are rare and challenging lesions due to the high morbidity associated with surgical intervention and their proximity to critical structures limiting the opportunity for stereotactic radiosurgery. We aim to report and review the novel use of fractionated Cyberknife (Accuray, Inc., Sunnyvale, California, USA) stereotactic radiotherapy in pediatric patients with oculomotor schwannomas. METHODS: A systematic review of PubMed, Embase, and Cochrane was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Two patients, ages 8 and 10 years, with tumor volumes of 0.1 cm3 and 0.2 cm3, respectively, were treated with fractionated Cyberknife radiotherapy at our institution. A total dose of 45-50 Gy was administered over 25 fractions (1.8-2.0 Gy per fraction) to the 82%-84% isodose line. Serial magnetic resonance imaging was obtained for long-term follow-up (56-58 months). RESULTS: We found 14 articles published between 1982 and 2018 that reported a total of 18 pediatric patients with intracranial oculomotor schwannomas. No previously described cases of pediatric intracranial oculomotor schwannomas were treated with radiation therapy. In both of our patients, radiographic tumor control was achieved at a mean follow-up of 57 months, with 1 patient displaying a decrease in tumor volume. Neither patient exhibited any worsening of their presenting symptoms, nor did either patient develop any new neurocognitive deficits following treatment. CONCLUSIONS: Fractionated Cyberknife radiotherapy is an effective and well-tolerated treatment option for intracranial oculomotor nerve schwannomas with excellent tumor control rates, similar to surgical and radiosurgical techniques, while sparing critical surrounding structures.

Predictors of 90-Day Readmission in Children Undergoing Spinal Cord Tumor Surgery: A Nationwide Readmissions Database Analysis.

OBJECTIVE: A fair number of hospital admissions occur after 30 days; thus, the true readmission rate could have been underestimated. Therefore, we hypothesized that the 90-day readmission rate might better characterize the factors contributing to readmission for pediatric patients undergoing spinal tumor resection. METHODS: The Nationwide Readmissions Database was used to study the patient demographic data, comorbidities, admissions, hospital course, spinal tumor behavior (malignant vs. benign), complications, revisions, and 30- and 90-day readmissions. RESULTS: Of the 397 patients included in the 30-day cohort, 43 (10.8%) had been readmitted. In comparison, the 90-day readmission rate was significantly greater; 52 of 325 patients were readmitted (16.0%; P < 0.04). Patients aged 16-20 constituted the largest subgroup. However, the highest readmission rate was observed for patients aged <5 years (30-day, 21.7%; 90-day, 26.4%). Medicaid patients were more likely to be readmitted than were private insurance patients (30-day odds ratio [OR], 3.3 [P < 0.001]; 90-day OR, 2.29 [P < 0.02]). In both cohorts, patients with malignant tumors required readmission more often than did those with benign tumors (30-day OR, 2.78 [P < 0.02]; 90-day OR, 1.92 [P = 0.08]). In the 90-day cohort, the patients had been readmitted 26.4 days after discharge versus 10.6 days in the 30-day cohort. Within the 90-day cohort, 18.6% of the readmissions were for spinal reoperation, 28.3% for chemotherapy or hematologic complications, and 25.6% for other central nervous system disorders. The median charges for each readmission were ∼$50,000 and ∼$40,000 for the 30- and 90-day cohorts, respectively. Medicaid insurance, malignant tumors, and younger age were significant predictors of readmission in the 90-day cohort. CONCLUSIONS: The prevalence and charges associated with unplanned hospital readmissions after spinal tumor resection were remarkably high. Younger age, Medicaid insurance, malignant tumors, and complications during the initial admission were significant predictors of 90-day readmission.

Imaging characteristics of atypical teratoid-rhabdoid tumor in children compared with medulloblastoma.

OBJECTIVE: The purpose of our study was to compare the imaging characteristics of atypical teratoid-rhabdoid tumor with medulloblastoma and seek distinguishing features that can aid in preoperative diagnosis. MATERIALS AND METHODS: Preoperative MRI examinations of 55 patients (36 medulloblastomas and 19 atypical teratoid-rhabdoid tumors) were analyzed retrospectively. Imaging characteristics of atypical teratoid-rhabdoid tumor and medulloblastoma were assessed with conventional MRI and CT. Diffusion-weighted imaging (DWI) was available in 27 patients (19 medulloblastomas and eight atypical teratoid-rhabdoid tumors). Apparent diffusion coefficient (ADC) values were calculated for 14 medulloblastomas and six atypical teratoid-rhabdoid tumors. RESULTS: Both atypical teratoid-rhabdoid tumors in general and infratentorial atypical teratoid-rhabdoid tumors presented at a younger age than medulloblastomas. Eleven of 19 atypical teratoid-rhabdoid tumors were infratentorial. Cerebellopontine angle (CPA) involvement was more frequent (8/11, 72.7%) in atypical teratoid-rhabdoid tumor than in medulloblastoma (4/36, 11.1%) (p < 0.001). Intratumoral hemorrhage was more common in atypical teratoid-rhabdoid tumor (9/19, 47.4%) than in medulloblastoma (2/36, 5.6%) (p < 0.0001). All atypical teratoid-rhabdoid tumors and all medulloblastomas for which DWI was available displayed increased signal intensity on DWI compared with normal brain parenchyma. The mean ADC values for tumor types were not significantly different. CONCLUSION: Atypical teratoid-rhabdoid tumor presents at a younger age than medulloblastoma. Although atypical teratoid-rhabdoid tumor and medulloblastoma display similar imaging characteristics on conventional MRI, CPA involvement and intratumoral hemorrhage are more common in atypical teratoid-rhabdoid tumor. If a pediatric posterior fossa mass that displays restricted diffusion is involving the CPA, atypical teratoid-rhabdoid tumor is a more likely consideration than medulloblastoma.