Publications by authors named "Matthew Smyth"

202 Publications

Neonatal hydrocephalus: an atypical presentation of malignant infantile osteopetrosis.

Childs Nerv Syst 2021 Sep 14. Epub 2021 Sep 14.

Department of Neurological Surgery and Pediatrics, St. Louis Children's Hospital, Washington University in St. Louis, Saint Louis, MO, USA.

Purpose: Autosomal recessive osteopetrosis has a variable presentation, most commonly including failure to thrive, hypocalcemia, seizures, hepatosplenomegaly, hydrocephalus, vision or hearing loss, and cytopenias. Multiple symptoms are usually seen at presentation. The variability of presentation often delays diagnosis and subsequent treatment. Here, we present a case of an infant with this condition who initially presented with triventricular hydrocephalus with Chiari I malformation. This alone is not a common presentation of this disease, and we present this case to highlight autosomal recessive osteopetrosis as a potential diagnosis in infants presenting with hydrocephalus and discuss the other associated symptoms, management, and prognosis of this condition.

Case Report: The patient was a full-term infant with a routine newborn period. At 6 months, the infant had macrocephaly and frontal bossing with a bulging fontanelle. She was found to have hydrocephalus with moderate ventriculomegaly involving the third and lateral ventricles with an associated Chiari 1 malformation. The infant was asymptomatic at the time. The infant was promptly referred to neurosurgery and underwent an uncomplicated ventriculoperitoneal shunt placement. Post-operative X-rays showed increased density of the skull with other bone changes suggestive of autosomal recessive osteopetrosis. Subsequent lab work and imaging studies were consistent with this condition. The diagnosis was confirmed by genetic testing, and the patient has undergone treatment with hematopoietic stem cell transplant.

Conclusion: Hydrocephalus is a common feature of this condition, typically seen in conjunction with other systemic symptoms and laboratory findings. Our patient had a limited initial presentation of triventricular hydrocephalus with Chiari I malformation and was otherwise clinically asymptomatic. There is limited literature of such a presentation, and we highlight this case to increase awareness, as timely diagnosis of these patients is critical for treatment and future outcomes.
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http://dx.doi.org/10.1007/s00381-021-05345-yDOI Listing
September 2021

Comparison of the real-world effectiveness of vertical versus lateral functional hemispherotomy techniques for pediatric drug-resistant epilepsy: A post hoc analysis of the HOPS study.

Epilepsia 2021 Sep 12. Epub 2021 Sep 12.

Division of Neurosurgery, Department of Surgery, BC Children's Hospital and University of British Columbia, Vancouver, British Columbia, Canada.

Objective: This study was undertaken to determine whether the vertical parasagittal approach or the lateral peri-insular/peri-Sylvian approach to hemispheric surgery is the superior technique in achieving long-term seizure freedom.

Methods: We conducted a post hoc subgroup analysis of the HOPS (Hemispheric Surgery Outcome Prediction Scale) study, an international, multicenter, retrospective cohort study that identified predictors of seizure freedom through logistic regression modeling. Only patients undergoing vertical parasagittal, lateral peri-insular/peri-Sylvian, or lateral trans-Sylvian hemispherotomy were included in this post hoc analysis. Differences in seizure freedom rates were assessed using a time-to-event method and calculated using the Kaplan-Meier survival method.

Results: Data for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittal hemispherotomy and 600 (89.3%) underwent lateral peri-insular/peri-Sylvian or trans-Sylvian hemispherotomy. Seizure freedom was obtained in 62.4% (95% confidence interval [CI] = 53.5%-70.2%) of the entire cohort at 10-year follow-up. Seizure freedom was 88.8% (95% CI = 78.9%-94.3%) at 1-year follow-up and persisted at 85.5% (95% CI = 74.7%-92.0%) across 5- and 10-year follow-up in the vertical subgroup. In contrast, seizure freedom decreased from 89.2% (95% CI = 86.3%-91.5%) at 1-year to 72.1% (95% CI = 66.9%-76.7%) at 5-year to 57.2% (95% CI = 46.6%-66.4%) at 10-year follow-up for the lateral subgroup. Log-rank test found that vertical hemispherotomy was associated with durable seizure-free progression compared to the lateral approach (p = .01). Patients undergoing the lateral hemispherotomy technique had a shorter time-to-seizure recurrence (hazard ratio = 2.56, 95% CI = 1.08-6.04, p = .03) and increased seizure recurrence odds (odds ratio = 3.67, 95% CI = 1.05-12.86, p = .04) compared to those undergoing the vertical hemispherotomy technique.

Significance: This pilot study demonstrated more durable seizure freedom of the vertical technique compared to lateral hemispherotomy techniques. Further studies, such as prospective expertise-based observational studies or a randomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes.
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http://dx.doi.org/10.1111/epi.17021DOI Listing
September 2021

Early implementation of stereoelectroencephalography in children: a multiinstitutional case series.

J Neurosurg Pediatr 2021 Sep 3:1-8. Epub 2021 Sep 3.

1Department of Neurological Surgery, University of Alabama at Birmingham, Alabama.

Objective: Pediatric stereoelectroencephalography (SEEG) has been increasingly performed in the United States, with published literature being limited primarily to large single-center case series. The purpose of this study was to evaluate the experience of pediatric epilepsy centers, where the technique has been adopted in the last several years, via a multicenter case series studying patient demographics, outcomes, and complications.

Methods: A retrospective cohort methodology was used based on the STROBE criteria. ANOVA was used to evaluate for significant differences between the means of continuous variables among centers. Dichotomous outcomes were assessed between centers using a univariate and multivariate logistic regression.

Results: A total of 170 SEEG insertion procedures were included in the study from 6 different level 4 pediatric epilepsy centers. The mean patient age at time of SEEG insertion was 12.3 ± 4.7 years. There was no significant difference between the mean age at the time of SEEG insertion between centers (p = 0.3). The mean number of SEEG trajectories per patient was 11.3 ± 3.6, with significant variation between centers (p < 0.001). Epileptogenic loci were identified in 84.7% of cases (144/170). Patients in 140 cases (140/170, 82.4%) underwent a follow-up surgical intervention, with 47.1% (66/140) being seizure free at a mean follow-up of 30.6 months. An overall postoperative hemorrhage rate of 5.3% (9/170) was noted, with patients in 4 of these cases (4/170, 2.4%) experiencing a symptomatic hemorrhage and patients in 3 of these cases (3/170, 1.8%) requiring operative evacuation of the hemorrhage. There were no mortalities or long-term complications.

Conclusions: As the first multicenter case series in pediatric SEEG, this study has aided in establishing normative practice patterns in the application of a novel surgical technique, provided a framework for anticipated outcomes that is generalizable and useful for patient selection, and allowed for discussion of what is an acceptable complication rate relative to the experiences of multiple institutions.
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http://dx.doi.org/10.3171/2021.5.PEDS20923DOI Listing
September 2021

A comparison of machine learning classifiers for pediatric epilepsy using resting-state functional MRI latency data.

Biomed Rep 2021 Sep 23;15(3):77. Epub 2021 Jul 23.

Division of Pediatric Neurosurgery, McGovern Medical School at UTHealth, Houston, TX 77030, USA.

Epilepsy affects 1 in 150 children under the age of 10 and is the most common chronic pediatric neurological condition; poor seizure control can irreversibly disrupt normal brain development. The present study compared the ability of different machine learning algorithms trained with resting-state functional MRI (rfMRI) latency data to detect epilepsy. Preoperative rfMRI and anatomical MRI scans were obtained for 63 patients with epilepsy and 259 healthy controls. The normal distribution of latency z-scores from the epilepsy and healthy control cohorts were analyzed for overlap in 36 seed regions. In these seed regions, overlap between the study cohorts ranged from 0.44-0.58. Machine learning features were extracted from latency z-score maps using principal component analysis. Extreme Gradient Boosting (XGBoost), Support Vector Machines (SVM), and Random Forest algorithms were trained with these features. Area under the receiver operating characteristics curve (AUC), accuracy, sensitivity, specificity and F1-scores were used to evaluate model performance. The XGBoost model outperformed all other models with a test AUC of 0.79, accuracy of 74%, specificity of 73%, and a sensitivity of 77%. The Random Forest model performed comparably to XGBoost across multiple metrics, but it had a test sensitivity of 31%. The SVM model did not perform >70% in any of the test metrics. The XGBoost model had the highest sensitivity and accuracy for the detection of epilepsy. Development of machine learning algorithms trained with rfMRI latency data could provide an adjunctive method for the diagnosis and evaluation of epilepsy with the goal of enabling timely and appropriate care for patients.
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http://dx.doi.org/10.3892/br.2021.1453DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8330002PMC
September 2021

Cross-Sectional Analysis of Quality of Life in Pediatric Patients with Inflammatory Bowel Disease in British Columbia, Canada.

J Pediatr 2021 Jul 19. Epub 2021 Jul 19.

Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Faculty of Medicine, British Columbia Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada; British Columbia Children Hospital Research Institute, Vancouver, British Columbia, Canada; Department of Cellular and Physiological Sciences, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address:

Objectives: To evaluate quality of life (QoL) in a large cohort of pediatric patients with inflammatory bowel disease (IBD) and to identify the clinical factors that influence QoL.

Study Design: This cross-sectional study analyzes a quality improvement initiative in 351 pediatric patients with IBD in British Columbia, Canada using the self-reported Pediatric Quality of Life Inventory (PedsQL) 4.0 generic scale. The questionnaire was completed at outpatient clinic and biologic infusion appointments. Statistical analysis included the t test, ANOVA, and multilinear regressions to evaluate the relationships between clinical factors and QoL.

Results: Mean (SE) QoL scores (79.95 [0.84]) fell between previously described healthy and chronically ill populations. Disease activity was the most significant predictor of QoL, with patients in remission scoring similar (84.42 [0.87]) to well established healthy norms, and those with moderately or severely active disease having some of the lowest published PedsQL scores (63.13 [3.27]), lower than most other chronic pediatric conditions. Twenty-five patients with moderately or severely active disease at the time of survey completion had follow-up surveys identified 1 year later and had a significant improvement of both their disease activity (P < .005) and their PedsQL scores (follow-up survey mean 76.13 [3.11]). Additional clinical factors independently associated with poor QoL were school nonattendance (15.5% decrease in QoL, P < .001), immune-modulator selection (methotrexate conferring a 9.5% lower mean QoL score than azathioprine, P = .005), and female gender (P = .031).

Conclusion: Pediatric patients with IBD experience a QoL significantly impacted by multiple clinical factors including current severity of IBD symptoms.
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http://dx.doi.org/10.1016/j.jpeds.2021.07.036DOI Listing
July 2021

Alterations in resting-state functional connectivity in pediatric patients with tuberous sclerosis complex.

Epilepsia Open 2021 Sep 3;6(3):579-587. Epub 2021 Aug 3.

Department of Neurology, Washington University, St. Louis, MO, USA.

Objective: To investigate resting-state functional connectivity (FC) in pediatric patients with tuberous sclerosis complex and intractable epilepsy requiring surgery.

Methods: Resting-state functional MRI was utilized to investigate functional connectivity in 13 pediatric patients with tuberous sclerosis complex (TSC) and intractable epilepsy requiring surgery.

Results: The majority of patients demonstrated a resting-state network architecture similar to those reported in healthy individuals. However, preoperative differences were evident between patients with high versus low tuber burden, as well as those with good versus poor neurodevelopmental outcomes, most notably in the cingulo-opercular and visual resting-state networks. One patient with high tuber burden and poor preoperative development and seizure control had nearly normal development and seizure resolution after surgery. This was accompanied by significant improvement in resting-state network architecture just one day postoperatively.

Significance: Although many patients with tuberous sclerosis complex and medically refractory epilepsy demonstrate functional connectivity patterns similar to healthy children, relationships within and between RSNs demonstrate clear differences in patients with higher tuber burden and worse outcomes. Improvements in resting-state network organization postoperatively may be related to epilepsy surgery outcomes, providing candidate biomarkers for clinical management in this high-risk population.
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http://dx.doi.org/10.1002/epi4.12523DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8408601PMC
September 2021

Preoperative imaging patterns and intracranial findings in single-suture craniosynostosis: a study from the Synostosis Research Group.

J Neurosurg Pediatr 2021 Jun 25:1-7. Epub 2021 Jun 25.

1Department of Neurosurgery, and.

Objective: The diagnosis of single-suture craniosynostosis can be made by physical examination, but the use of confirmatory imaging is common practice. The authors sought to investigate preoperative imaging use and to describe intracranial findings in children with single-suture synostosis from a large, prospective multicenter cohort.

Methods: In this study from the Synostosis Research Group, the study population included children with clinically diagnosed single-suture synostosis between March 1, 2017, and October 31, 2020, at 5 institutions. The primary analysis correlated the clinical diagnosis and imaging diagnosis; secondary outcomes included intracranial findings by pathological suture type.

Results: A total of 403 children (67% male) were identified with single-suture synostosis. Sagittal (n = 267), metopic (n = 77), coronal (n = 52), and lambdoid (n = 7) synostoses were reported; the most common presentation was abnormal head shape (97%), followed by a palpable or visible ridge (37%). Preoperative cranial imaging was performed in 90% of children; findings on 97% of these imaging studies matched the initial clinical diagnosis. Thirty-one additional fused sutures were identified in 18 children (5%) that differed from the clinical diagnosis. The most commonly used imaging modality by far was CT (n = 360), followed by radiography (n = 9) and MRI (n = 7). Most preoperative imaging was ordered as part of a protocolized pathway (67%); some images were obtained as a result of a nondiagnostic clinical examination (5.2%). Of the 360 patients who had CT imaging, 150 underwent total cranial vault surgery and 210 underwent strip craniectomy. The imaging findings influenced the surgical treatment 0.95% of the time. Among the 24% of children with additional (nonsynostosis) abnormal findings on CT, only 3.5% required further monitoring.

Conclusions: The authors found that a clinical diagnosis of single-suture craniosynostosis and the findings on CT were the same with rare exceptions. CT imaging very rarely altered the surgical treatment of children with single-suture synostosis.
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http://dx.doi.org/10.3171/2021.2.PEDS2113DOI Listing
June 2021

Extradural decompression versus duraplasty in Chiari malformation type I with syrinx: outcomes on scoliosis from the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2021 Jun 18:1-9. Epub 2021 Jun 18.

25Division of Pediatric Neurosurgery, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA.

Objective: Scoliosis is common in patients with Chiari malformation type I (CM-I)-associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression.

Methods: A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty.

Results: In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion.

Conclusions: In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.
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http://dx.doi.org/10.3171/2020.12.PEDS20552DOI Listing
June 2021

A multicenter validation of the condylar-C2 sagittal vertical alignment in Chiari malformation type I: a study using the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2021 Jun 4:1-7. Epub 2021 Jun 4.

1Division of Pediatric Neurosurgery, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.

Objective: The condylar-C2 sagittal vertical alignment (C-C2SVA) describes the relationship between the occipitoatlantal joint and C2 in patients with Chiari malformation type I (CM-I). It has been suggested that a C-C2SVA ≥ 5 mm is predictive of the need for occipitocervical fusion (OCF) or ventral brainstem decompression (VBD). The authors' objective was to validate the predictive utility of the C-C2SVA by using a large, multicenter cohort of patients.

Methods: This validation study used a cohort of patients derived from the Park-Reeves Syringomyelia Research Consortium; patients < 21 years old with CM-I and syringomyelia treated from June 2011 to May 2016 were identified. The primary outcome was the need for OCF and/or VBD. After patients who required OCF and/or VBD were identified, 10 age- and sex-matched controls served as comparisons for each OCF/VBD patient. The C-C2SVA (defined as the position of a plumb line from the midpoint of the O-C1 joint relative to the posterior aspect of the C2-3 disc space), pBC2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body), and clival-axial angle (CXA) were measured on sagittal MRI. The secondary outcome was the need for ≥ 2 CM-related operations.

Results: Of the 206 patients identified, 20 underwent OCF/VBD and 14 underwent repeat posterior fossa decompression. A C-C2SVA ≥ 5 mm was 100% sensitive and 86% specific for requiring OCF/VBD, with a 12.6% misclassification rate, whereas CXA < 125° was 55% sensitive and 99% specific, and pBC2 ≥ 9 was 20% sensitive and 88% specific. Kaplan-Meier analysis demonstrated that there was a significantly shorter time to second decompression in children with C-C2SVA ≥ 5 mm (p = 0.0039). The mean C-C2SVA was greater (6.13 ± 1.28 vs 3.13 ± 1.95 mm, p < 0.0001), CXA was lower (126° ± 15.4° vs 145° ± 10.7°, p < 0.05), and pBC2 was similar (7.65 ± 1.79 vs 7.02 ± 1.26 mm, p = 0.31) among those who underwent OCF/VBD versus decompression only. The intraclass correlation coefficient for the continuous measurement of C-C2SVA was 0.52; the kappa value was 0.47 for the binary categorization of C-C2SVA ≥ 5 mm.

Conclusions: These results validated the C-C2SVA using a large, multicenter, external cohort with 100% sensitivity, 86% specificity, and a 12.6% misclassification rate. A C-C2SVA ≥ 5 mm is highly predictive of the need for OCF/VBD in patients with CM-I. The authors recommend that this measurement be considered among the tools to identify the "high-risk" CM-I phenotype.
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http://dx.doi.org/10.3171/2020.12.PEDS20809DOI Listing
June 2021

Management of sagittal synostosis in the Synostosis Research Group: baseline data and early outcomes.

Neurosurg Focus 2021 04;50(4):E3

Divisions of1Pediatric Neurosurgery, Primary Children's Hospital, and.

Objective: Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research.

Methods: All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables.

Results: Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group.

Conclusions: The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.
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http://dx.doi.org/10.3171/2021.1.FOCUS201029DOI Listing
April 2021

Hemispherectomy Outcome Prediction Scale: Development and validation of a seizure freedom prediction tool.

Epilepsia 2021 05 13;62(5):1064-1073. Epub 2021 Mar 13.

Department of Pediatrics, BC Children's Hospital and University of British Columbia, Vancouver, British Columbia, Canada.

Objective: To develop and validate a model to predict seizure freedom in children undergoing cerebral hemispheric surgery for the treatment of drug-resistant epilepsy.

Methods: We analyzed 1267 hemispheric surgeries performed in pediatric participants across 32 centers and 12 countries to identify predictors of seizure freedom at 3 months after surgery. A multivariate logistic regression model was developed based on 70% of the dataset (training set) and validated on 30% of the dataset (validation set). Missing data were handled using multiple imputation techniques.

Results: Overall, 817 of 1237 (66%) hemispheric surgeries led to seizure freedom (median follow-up = 24 months), and 1050 of 1237 (85%) were seizure-free at 12 months after surgery. A simple regression model containing age at seizure onset, presence of generalized seizure semiology, presence of contralateral 18-fluoro-2-deoxyglucose-positron emission tomography hypometabolism, etiologic substrate, and previous nonhemispheric resective surgery is predictive of seizure freedom (area under the curve = .72). A Hemispheric Surgery Outcome Prediction Scale (HOPS) score was devised that can be used to predict seizure freedom.

Significance: Children most likely to benefit from hemispheric surgery can be selected and counseled through the implementation of a scale derived from a multiple regression model. Importantly, children who are unlikely to experience seizure control can be spared from the complications and deficits associated with this surgery. The HOPS score is likely to help physicians in clinical decision-making.
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http://dx.doi.org/10.1111/epi.16861DOI Listing
May 2021

Comparing Health Administrative and Clinical Registry Data: Trends in Incidence and Prevalence of Pediatric Inflammatory Bowel Disease in British Columbia.

Clin Epidemiol 2021 11;13:81-90. Epub 2021 Feb 11.

Department of Pediatrics, BC Children's Hospital, Vancouver, British Columbia, Canada.

Purpose: Canada maintains robust health administrative databases and British Columbia Children's Hospital (BCCH), as the only tertiary care pediatric hospital in British Columbia (BC), maintains a comprehensive clinical inflammatory bowel disease (IBD) registry. To evaluate the strengths and weaknesses of utilizing health administrative and clinical registry data to study the epidemiology of IBD in BC, we conducted a population-based retrospective cohort study of all children <18 years of age who were diagnosed with IBD between 1996 and 2008 in BC.

Methods: IBD cases from health administrative data were identified using a combination of IBD-coded physician encounters and hospitalizations while a separate IBD cohort was identified from the BCCH clinical registry data. Age and gender standardized incidence and prevalence rates were fitted to Poisson regression models.

Results: The overall incidence of pediatric IBD identified in health administrative data increased from 7.1 (95% CI 5.5-9.2) in 1996 to 10.3 (95% CI 8.2-12.7) per 100,000 children in 2008. Similarly, the incidence of the BCCH cohort increased from 4.3 (95% CI 3.0-6.0) to 9.7 (95% CI 7.6-12.1) per 100,000. Children aged 10-17 had the highest rise in incidence in both data sources; however, the administrative data identified significantly more 10-17-year-olds and significantly less 6-9-year-olds (p<0.05) compared to clinical registry data.

Conclusion: While the application of both health administrative and clinical registry data demonstrates that the incidence of IBD is increasing in BC, we identify strengths and limitations to both and suggest that the utilization of either data source requires unique considerations that mitigate misclassification biases.
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http://dx.doi.org/10.2147/CLEP.S292546DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7886108PMC
February 2021

Dural augmentation approaches and complication rates after posterior fossa decompression for Chiari I malformation and syringomyelia: a Park-Reeves Syringomyelia Research Consortium study.

J Neurosurg Pediatr 2021 Feb 12:1-10. Epub 2021 Feb 12.

3Division of Pediatric Neurosurgery, University of Alabama at Birmingham, AL.

Objective: Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM.

Methods: The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft.

Results: A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain.

Conclusions: In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.
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http://dx.doi.org/10.3171/2020.8.PEDS2087DOI Listing
February 2021

The Role of SPECT and PET in Epilepsy.

AJR Am J Roentgenol 2021 03 21;216(3):759-768. Epub 2021 Jan 21.

Department of Neurology, Washington University School of Medicine, St. Louis, MO.

The purpose of this article is to summarize the role of molecular imaging of the brain by use of SPECT, FDG PET, and non-FDG PET radiotracers in epilepsy. Quantitative image analysis with PET and SPECT has increased the diagnostic utility of these modalities in localizing epileptogenic onset zones. A multi-modal platform approach integrating the functional imaging of PET and SPECT with the morphologic information from MRI in presurgical evaluation of epilepsy can greatly improve outcomes.
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http://dx.doi.org/10.2214/AJR.20.23336DOI Listing
March 2021

Morphologic Severity of Craniosynostosis: Implications for Speech and Neurodevelopment.

Cleft Palate Craniofac J 2021 Jan 20:1055665620984643. Epub 2021 Jan 20.

Division of Plastic and Reconstructive Surgery, Washington University in St. Louis, MO, USA.

Background: Single-suture craniosynostosis (SSC) can be associated with neurodevelopmental deficits. We examined the correlation between morphologic severity and incidence of speech-language or psychological concerns.

Methods: In 62 patients (33 sagittal, 17 metopic, and 12 unicoronal), morphologic severity was determined via preoperative computed tomography (CT). Severity metrics for sagittal, metopic, and unicoronal synostosis were adjusted cephalic index (aCI), interfrontal angle (IFA), and anterior cranial fossa area ratio (ACFR), respectively. Speech-language and psychological concerns were assessed at age ≥4.5 years and defined as recommendation for therapy or monitoring.

Results: Mean assessment age was 5.7 years; 32% had a speech-language concern and 44% had a psychological concern; 44% had neither. Sagittal: Mean aCI of those with a speech-language concern (0.62) and those without (0.62) were equivalent ( = .580), as were mean aCI of those with a psychological concern (0.62) and those without (0.62; = .572). Metopic: Mean IFA with (117.9) and without (125.2) a speech-language concern were equivalent ( = .326), as were mean IFA with (120.2) and without (123.2) a psychological concern ( = .711). Unicoronal: Mean ACFR with (0.65) and without (0.69) a psychological concern ( = .423) were equivalent. However, mean ACFR with (0.74) and without (0.63) a speech-language concern were not ( = .022*). Bivariate rank correlation showed significant association between morphologic severity and speech-language score only for unicoronal synostosis (ρ = .722; = .008*).

Conclusion: A significant portion of patients with SSC had speech-language or psychological concerns. We found no correlation between morphologic severity and incidence of speech-language or psychological concerns for patients with sagittal or metopic synostosis. Morphological severity did correlate with speech concerns in patients with unicoronal synostosis.
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http://dx.doi.org/10.1177/1055665620984643DOI Listing
January 2021

Convolutional Neural Networks for Pediatric Refractory Epilepsy Classification Using Resting-State Functional Magnetic Resonance Imaging.

World Neurosurg 2021 05 6;149:e1112-e1122. Epub 2021 Jan 6.

Department of Pediatric Surgery and Neurosurgery, McGovern Medical School at UTHealth, Houston, Texas, USA.

Objective: This study aims to evaluate the performance of convolutional neural networks (CNNs) trained with resting-state functional magnetic resonance imaging (rfMRI) latency data in the classification of patients with pediatric epilepsy from healthy controls.

Methods: Preoperative rfMRI and anatomic magnetic resonance imaging scans were obtained from 63 pediatric patients with refractory epilepsy and 259 pediatric healthy controls. Latency maps of the temporal difference between rfMRI and the global mean signal were calculated using voxel-wise cross-covariance. Healthy control and epilepsy latency z score maps were pseudorandomized and partitioned into training data (60%), validation data (20%), and test data (20%). Healthy control individuals and patients with epilepsy were labeled as negative and positive, respectively. CNN models were then trained with the designated training data. Model hyperparameters were evaluated with a grid-search method. The model with the highest sensitivity was evaluated using unseen test data. Accuracy, sensitivity, specificity, F1 score, and area under the receiver operating characteristic curve were used to evaluate the ability of the model to classify epilepsy in the test data set.

Results: The model with the highest validation sensitivity correctly classified 74% of unseen test patients with 85% sensitivity, 71% specificity, F1 score of 0.56, and an area under the receiver operating characteristic curve of 0.86.

Conclusions: Using rfMRI latency data, we trained a CNN model to classify patients with pediatric epilepsy from healthy controls with good performance. CNN could serve as an adjunct in the diagnosis of pediatric epilepsy. Identification of pediatric epilepsy earlier in the disease course could decrease time to referral to specialized epilepsy centers and thus improve prognosis in this population.
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http://dx.doi.org/10.1016/j.wneu.2020.12.131DOI Listing
May 2021

Occipital-Cervical Fusion and Ventral Decompression in the Surgical Management of Chiari-1 Malformation and Syringomyelia: Analysis of Data From the Park-Reeves Syringomyelia Research Consortium.

Neurosurgery 2021 01;88(2):332-341

Department of Neurosurgery, University of Minnesota Medical School, Minneapolis, Minnesota.

Background: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology.

Objective: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD.

Methods: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD.

Results: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups.

Conclusion: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.
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http://dx.doi.org/10.1093/neuros/nyaa460DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7803430PMC
January 2021

Comparison of 2 Sagittal Craniosynostosis Repair Techniques: Spring-Assisted Surgery Versus Endoscope-Assisted Craniectomy With Helmet Molding Therapy.

Cleft Palate Craniofac J 2021 06 23;58(6):678-686. Epub 2020 Oct 23.

Division of Plastic and Reconstructive Surgery, 12321The Warren Alpert Medical School of Brown University, Providence, RI, USA.

Introduction: This study compares anthropometric outcomes of 2 sagittal synostosis repair techniques: spring-assisted surgery and endoscope-assisted craniectomy with molding helmet therapy.

Methods: Patients undergoing spring-assisted surgery (n = 27) or endoscope-assisted craniectomy with helmet therapy (n = 40) at separate institutions were retrospectively reviewed. Pre- and 1-year postoperative computed tomography (CT) or laser scans were analyzed for traditional cranial index (CI), adjusted cranial index (aCI), and cranial vault volume (CVV). Nine patient-matched scans were analyzed for measurement consistency.

Results: The spring-assisted group was older at both time points ( < .050) and spring-assisted group CVV was larger preoperatively and postoperatively ( < .01). However, the change in CVV did not differ between the groups ( = .210). There was no difference in preoperative CI (helmet vs spring: 70.1 vs 71.2, = .368) between the groups. Postoperatively, helmet group CI (77.0 vs 74.3, = .008) was greater. The helmet group also demonstrated a greater increase in CI (6.9 vs 3.1, < .001). The proportion of patients achieving CI of 75 or greater was not significantly different between the groups (helmet vs spring: CI, 65% vs 52%, = .370). There was no detectable bias in CI between matched CT and laser scans. Differences were identified between scan types in aCI and CVV measurements; subsequent analyses used corrected CVV and aCI measures for laser scan measures.

Conclusions: Both techniques had equivalent proportions of patients achieving normal CI, comparable effects on cranial volume, and similar operative characteristics. The study suggests that there may be greater improvement in CI in the helmet group. However, further research should be performed.
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http://dx.doi.org/10.1177/1055665620966521DOI Listing
June 2021

Use of fast-sequence spine MRI in pediatric patients.

J Neurosurg Pediatr 2020 Sep 18:1-6. Epub 2020 Sep 18.

1Department of Neurological Surgery and.

Objective: The immediate and long-term risk of anesthesia in the pediatric population is controversial. Traditional spine MRI protocols require the patient to remain still during the examination, and in young children this frequently results in the need for sedation administration. The authors' goal was to develop an abbreviated spine MRI protocol to reduce sedation administration in young patients undergoing spine MRI.

Methods: After IRB approval, the medical records of all pediatric patients who underwent a fast spine MRI protocol between 2017 and 2019 were reviewed. The protocol consisted of T2-weighted half-Fourier acquisition single-shot turbo spin echo, T1-weighted turbo spin echo, and T2-weighted STIR sequences acquired in the sagittal plane. The total acquisition time was 2 minutes with no single sequence acquisition longer than 60 seconds. Interpretability of the scans was assessed in accordance with the radiology report in conjunction with the neurosurgeon's clinical notes.

Results: A total of 47 fast spine MRI sessions were performed in 45 patients. The median age at the time of the MRI was 2.4 years (25th-75th quartile, 1.1-4.3 years; range 0.16-18.58 years). The most common indication for imaging was to rule out or follow a known syrinx (n = 30), followed by the need to rule out or follow known spinal dysraphism (n = 22). There were no uninterpretable or unusable scans. Eight of 47 scans were noted to have moderate motion artifact limitations with respect to the quality of the scan. Seven patients underwent a subsequent MRI with a sedated standard spine protocol within 1 year from the fast scan, which confirmed the findings on the fast MRI protocol with no new findings identified.

Conclusions: The authors report the first pediatric series of a fast spine MRI protocol for use in young patients. The protocol does not require sedation and is able to identify and monitor syrinx, spinal dysraphism, and potentially other intraspinal anomalies.
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http://dx.doi.org/10.3171/2020.5.PEDS20137DOI Listing
September 2020

Using Histopathology to Assess the Reliability of Intraoperative Magnetic Resonance Imaging in Guiding Additional Brain Tumor Resection: A Multicenter Study.

Neurosurgery 2020 12;88(1):E49-E59

Washington University School of Medicine, St. Louis, Missouri.

Background: Intraoperative magnetic resonance imaging (iMRI) is a powerful tool for guiding brain tumor resections, provided that it accurately discerns residual tumor.

Objective: To use histopathology to assess how reliably iMRI may discern additional tumor for a variety of tumor types, independent of the indications for iMRI.

Methods: A multicenter database was used to calculate the odds of additional resection during the same surgical session for grade I to IV gliomas and pituitary adenomas. The reliability of iMRI for identifying residual tumor was assessed using histopathology of tissue resected after iMRI.

Results: Gliomas (904/1517 cases, 59.6%) were more likely than pituitary adenomas (176/515, 34.2%) to receive additional resection after iMRI (P < .001), but these tumors were equally likely to have additional tissue sent for histopathology (398/904, 44.4% vs 66/176, 37.5%; P = .11). Tissue samples were available for resections after iMRI for 464 cases, with 415 (89.4%) positive for tumor. Additional resections after iMRI for gliomas (361/398, 90.7%) were more likely to yield additional tumor compared to pituitary adenomas (54/66, 81.8%) (P = .03). There were no significant differences in resection after iMRI yielding histopathologically positive tumor between grade I (58/65 cases, 89.2%; referent), grade II (82/92, 89.1%) (P = .98), grade III (72/81, 88.9%) (P = .95), or grade IV gliomas (149/160, 93.1%) (P = .33). Additional resection for previously resected tumors (122/135 cases, 90.4%) was equally likely to yield histopathologically confirmed tumor compared to newly-diagnosed tumors (293/329, 89.0%) (P = .83).

Conclusion: Histopathological analysis of tissue resected after use of iMRI for grade I to IV gliomas and pituitary adenomas demonstrates that iMRI is highly reliable for identifying residual tumor.
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http://dx.doi.org/10.1093/neuros/nyaa338DOI Listing
December 2020

School-Aged Anthropometric Outcomes After Endoscopic or Open Repair of Metopic Synostosis.

Pediatrics 2020 09 11;146(3). Epub 2020 Aug 11.

Division of Plastic and Reconstructive Surgery, Department of Surgery and

Background And Objectives: Metopic craniosynostosis can be treated by fronto-orbital advancement or endoscopic strip craniectomy with postoperative helmeting. Infants younger than 6 months of age are eligible for the endoscopic repair. One-year postoperative anthropometric outcomes have been shown to be equivalent, with significantly less morbidity after endoscopic treatment. The authors hypothesized that both repairs would yield equivalent anthropometric outcomes at 5-years postoperative.

Methods: This study was a retrospective chart review of 31 consecutive nonsyndromic patients with isolated metopic craniosynostosis treated with either endoscopic or open correction. The primary anthropometric outcomes were frontal width, interfrontal divergence angle, the Whitaker classification, and the presence of lateral frontal retrusion. Peri-operative variables included estimated blood loss, rates of blood transfusion, length of stay, and operating time.

Results: There was a significantly lower rate of lateral frontal retrusion in the endoscopic group. No statistically significant differences were found in the other 3 anthropometric outcomes at 5-years postoperative. The endoscopic group was younger at the time of surgery and had improved peri-operative outcomes related to operating time, hospital stay and blood loss. Both groups had low complication and reoperation rates.

Conclusions: In our cohort of school-aged children with isolated metopic craniosynostosis, patients who underwent endoscopic repair had superior or equivalent outcomes on all 4 primary anthropometric measures compared with those who underwent open repair. Endoscopic repair was associated with significantly faster recovery and decreased morbidity. Endoscopic repair should be considered in patients diagnosed with metopic craniosynostosis before 6 months of age.
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http://dx.doi.org/10.1542/peds.2020-0238DOI Listing
September 2020

Impact of Intraoperative Magnetic Resonance Imaging and Other Factors on Surgical Outcomes for Newly Diagnosed Grade II Astrocytomas and Oligodendrogliomas: A Multicenter Study.

Neurosurgery 2020 12;88(1):63-73

Department of Neurosurgery, Washington University School of Medicine in St. Louis, St. Louis, Missouri.

Background: Few studies use large, multi-institutional patient cohorts to examine the role of intraoperative magnetic resonance imaging (iMRI) in the resection of grade II gliomas.

Objective: To assess the impact of iMRI and other factors on overall survival (OS) and progression-free survival (PFS) for newly diagnosed grade II astrocytomas and oligodendrogliomas.

Methods: Retrospective analyses of a multicenter database assessed the impact of patient-, treatment-, and tumor-related factors on OS and PFS.

Results: A total of 232 resections (112 astrocytomas and 120 oligodendrogliomas) were analyzed. Oligodendrogliomas had longer OS (P < .001) and PFS (P = .01) than astrocytomas. Multivariate analyses demonstrated improved OS for gross total resection (GTR) vs subtotal resection (STR; P = .006, hazard ratio [HR]: .23) and near total resection (NTR; P = .02, HR: .64). GTR vs STR (P = .02, HR: .54), GTR vs NTR (P = .04, HR: .49), and iMRI use (P = .02, HR: .54) were associated with longer PFS. Frontal (P = .048, HR: 2.11) and occipital/parietal (P = .003, HR: 3.59) locations were associated with shorter PFS (vs temporal). Kaplan-Meier analyses showed longer OS with increasing extent of surgical resection (EOR) (P = .03) and 1p/19q gene deletions (P = .02). PFS improved with increasing EOR (P = .01), GTR vs NTR (P = .02), and resections above STR (P = .04). Factors influencing adjuvant treatment (35.3% of patients) included age (P = .002, odds ratio [OR]: 1.04) and EOR (P = .003, OR: .39) but not glioma subtype or location. Additional tumor resection after iMRI was performed in 105/159 (66%) iMRI cases, yielding GTR in 54.5% of these instances.

Conclusion: EOR is a major determinant of OS and PFS for patients with grade II astrocytomas and oligodendrogliomas. Intraoperative MRI may improve EOR and was associated with increased PFS.
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http://dx.doi.org/10.1093/neuros/nyaa320DOI Listing
December 2020

Evaluation of the Patient-Practitioner Consultation on Surgical Treatment Options for Patients With Craniosynostosis.

J Craniofac Surg 2020 Jul-Aug;31(5):1186-1190

*Division of Plastic and Reconstructive Surgery, Department of Surgery, St. Louis Children's Hospital, Washington University School of Medicine in St. Louis, St. Louis, MO †Department of Neurosurgery, Division of Pediatric Neurosurgery, Primary Children's Hospital, University of Utah, Salt Lake City, UT ‡Neurological Surgery §Division of Plastic Surgery, Seattle Children's Hospital, University of Washington, Seattle, WA ||Department of Neurological Surgery, Columbia University, Morgan Stanley Children's Hospital of New York-Presbyterian, New York, NY ¶Division of Plastic Surgery and Reconstructive Surgery, University of Utah, Salt Lake City, UT #Division of Neurosurgery, British Columbia Children's Hospital, University of British Columbia, Vancouver, Canada **Plastic Surgery, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA ††Division of Plastic Surgery, Columbia University Medical Center, New York-Presbyterian Hospital, New York, NY ‡‡Department of Neurosurgery, St. Louis Children's Hospital, Washington University School of Medicine in St. Louis, St. Louis, MO.

Introduction: Endoscope-assisted craniectomy and spring-assisted cranioplasty with post-surgical helmet molding are minimally invasive alternatives to the traditional craniosynostosis treatment of open cranial vault remodeling. Families are often faced with deciding between techniques. This study aimed to understand providers' practice patterns in consulting families about surgical options.

Methods: An online survey was developed and distributed to 31 providers. The response rate was 84% (26/31).

Results: Twenty-six (100%) respondents offer a minimally invasive surgical option for sagittal craniosynostosis, 21 (81%) for coronal, 20 (77%) for metopic, 18 (69%) for lambdoid, and 12 (46%) for multi-suture. Social issues considered in determining whether to offer a minimally invasive option include anticipated likelihood of compliance (23 = 88%), distance traveled for care (16 = 62%) and financial considerations (6 = 23%). Common tools to explain options include verbal discussion (25 = 96%), 3D reconstructed CT scans (17 = 65%), handouts (13 = 50%), 3D models (12 = 46%), hand drawings (11 = 42%) and slides (10 = 38%). Some respondents strongly (7 = 27%) or somewhat (3 = 12%) encourage a minimally invasive option over open repair. Others indicate they remain neutral (7 = 27%) or tailor their approach to meet perceived needs (8 = 31%). One (4%) somewhat encourages open repair. Despite this variation, all completely (17 = 65%), strongly (5 = 19%) or somewhat agree (4 = 15%) they use shared decision making in presenting surgical options.

Conclusion: This survey highlights the range of practice patterns in presenting surgical options to families and reveals possible discrepancies in the extent providers believe they use shared decision making and the extent it is actually used.
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http://dx.doi.org/10.1097/SCS.0000000000006327DOI Listing
October 2020

3D pediatric cranial bone imaging using high-resolution MRI for visualizing cranial sutures: a pilot study.

J Neurosurg Pediatr 2020 Jun 12:1-7. Epub 2020 Jun 12.

2Mallinckrodt Institute of Radiology, and.

Objective: There is an unmet need to perform imaging in young children and obtain CT-equivalent cranial bone images without subjecting the patients to radiation. In this study, the authors propose using a high-resolution fast low-angle shot golden-angle 3D stack-of-stars radial volumetric interpolated breath-hold examination (GA-VIBE) MRI sequence that is intrinsically robust to motion and has enhanced bone versus soft-tissue contrast.

Methods: Patients younger than 11 years of age, who underwent clinical head CT scanning for craniosynostosis or other cranial malformations, were eligible for the study. 3D reconstructed images created from the GA-VIBE MRI sequence and the gold-standard CT scan were randomized and presented to 3 blinded reviewers. For all image sets, each reviewer noted the presence or absence of the 6 primary cranial sutures and recorded on 5-point Likert scales whether they recommended a second scan be performed.

Results: Eleven patients (median age 1.8 years) underwent MRI after clinical head CT scanning was performed. Five of the 11 patients were sedated. Three clinicians reviewed the images, and there were no cases, either with CT scans or MR images, in which a reviewer agreed a repeat scan was required for diagnosis or surgical planning. The reviewers reported clear imaging of the regions of interest on 99% of the CT reviews and 96% of the MRI reviews. With CT as the standard, the sensitivity and specificity of the GA-VIBE MRI sequence to detect suture closure were 97% and 96%, respectively (n = 198 sutures read).

Conclusions: The 3D reconstructed images using the GA-VIBE sequence in comparison to the CT scans created clinically acceptable cranial images capable of detecting cranial sutures. Future directions include reducing the scan time, improving motion correction, and automating postprocessing for clinical utility.
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http://dx.doi.org/10.3171/2020.4.PEDS20131DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7736460PMC
June 2020

Endoscopic treatment of combined metopic-sagittal craniosynostosis.

J Neurosurg Pediatr 2020 Apr 17:1-9. Epub 2020 Apr 17.

1Division of Plastic & Reconstructive Surgery, Department of Surgery, and.

Objective: Combined metopic-sagittal craniosynostosis is traditionally treated with open cranial vault remodeling and fronto-orbital advancement, sometimes in multiple operations. Endoscopic treatment of this multisuture synostosis presents a complex challenge for the surgeon and orthotist.

Methods: The authors retrospectively analyzed the preoperative and 1-year postoperative CT scans of 3 patients with combined metopic-sagittal synostosis, all of whom were treated with simultaneous endoscope-assisted craniectomy of the metopic and sagittal sutures followed by helmet therapy. Established anthropometric measurements were applied to assess pre- and postoperative morphology, including cranial index and interfrontal divergence angle (IFDA). Patients' measurements were compared to those obtained in 18 normal controls.

Results: Two boys and one girl underwent endoscope-assisted craniectomy at a mean age of 81 days. The mean preoperative cranial index was 0.70 (vs control mean of 0.82, p = 0.009), corrected postoperatively to a mean of 0.82 (vs control mean of 0.80, p = 0.606). The mean preoperative IFDA was 110.4° (vs control mean of 152.6°, p = 0.017), corrected postoperatively to a mean of 139.1° (vs control mean of 140.3°, p = 0.348). The mean blood loss was 100 mL and the mean length of stay was 1.7 days. No patient underwent reoperation. The mean clinical follow-up was 3.4 years.

Conclusions: Endoscope-assisted craniectomy with helmet therapy is a viable single-stage treatment option for combined metopic-sagittal synostosis, providing correction of the stigmata of trigonoscaphocephaly, with normalization of the cranial index and IFDA.
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http://dx.doi.org/10.3171/2020.2.PEDS2029DOI Listing
April 2020

Tocilizumab in patients with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis (APTITUDE): a multicentre, single-arm, phase 2 trial.

Lancet Rheumatol 2020 Mar 7;2(3):e135-e141. Epub 2020 Feb 7.

Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, UK.

Background: Uveitis associated with juvenile idiopathic arthritis is a cause of major ocular morbidity. A substantial proportion of children are refractory to systemic methotrexate and TNF inhibitors. Our aim was to study the safety and efficacy of tocilizumab in children with juvenile idiopathic arthritis-associated uveitis refractory to both methotrexate and TNF inhibitors.

Methods: This multicentre, single-arm, phase 2 trial was done following a Simon's two-stage design at seven tertiary hospital sites in the UK. Patients aged 2-18 years with active juvenile idiopathic arthritis-associated uveitis were eligible. All patients had been on a stable dose of methotrexate for at least 12 weeks and had not responded to treatment with a TNF inhibitor. Patients weighing 30 kg or more were treated with 162 mg subcutaneous tocilizumab every 2 weeks for 24 weeks, and participants weighing less than 30 kg were treated with 162 mg every 3 weeks for 24 weeks. The primary outcome was treatment response defined as a two-step decrease, or decrease to zero, from baseline in the level of inflammation (anterior chamber cells) at week 12, per the standardisation of uveitis nomenclature criteria. A phase 3 trial would be justified if more than seven patients responded to treatment. An interim analysis was planned to assess whether the trial would be stopped for futility, with futility defined as two or fewer treatment responses among ten participants. Adverse events were collected up to 30 calendar days after treatment cessation. The primary analysis was done in the intention-to-treat population and the safety analysis was done in all patients who started the treatment. This trial is registered with the International Standard Randomised Controlled Trial Number registry (ISRCTN95363507) and EU Clinical Trials Register (EudraCT 2015-001323-23).

Findings: 22 participants were enrolled to the trial between Dec 3, 2015, and March 9, 2018, and 21 participants received treatment. One participant was found to be ineligible immediately after enrolment and was therefore withdrawn. Seven of 21 (median unbiased estimate of proportion 34% [95% CI 25-57]) responded to treatment (p=0·11). Safety results were consistent with the known safety profile of tocilizumab.

Interpretation: The primary endpoint was not met, and thus the results do not support a phase 3 trial of tocilizumab in patients with juvenile idiopathic arthritis-associated uveitis. Importantly, data on the use of tocilizumab in clinical practice is now captured in national registries. Despite this trial not meeting the threshold required to justify a larger phase 3 trial, several patients responded to treatment; as such, tocilzumab might still be a therapeutic option in some children with uveitis refractory to anti-TNF drugs, given the absence of other treatment options.

Funding: Versus Arthritis and the National Institute for Health Research Clinical Research Network: Children.
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http://dx.doi.org/10.1016/S2665-9913(20)30008-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7134526PMC
March 2020

Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2020 Apr 10:1-7. Epub 2020 Apr 10.

1Department of Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri.

Objective: In patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.

Methods: A large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.

Results: Of 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.

Conclusions: In this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.
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http://dx.doi.org/10.3171/2020.1.PEDS18755DOI Listing
April 2020

Magnetic resonance-guided stereotactic laser ablation therapy for the treatment of pediatric brain tumors: a multiinstitutional retrospective study.

J Neurosurg Pediatr 2020 Mar 27:1-9. Epub 2020 Mar 27.

Departments of7Neurosurgery and.

Objective: This study aimed to assess the safety and efficacy of MR-guided stereotactic laser ablation (SLA) therapy in the treatment of pediatric brain tumors.

Methods: Data from 17 North American centers were retrospectively reviewed. Clinical, technical, and radiographic data for pediatric patients treated with SLA for a diagnosis of brain tumor from 2008 to 2016 were collected and analyzed.

Results: A total of 86 patients (mean age 12.2 ± 4.5 years) with 76 low-grade (I or II) and 10 high-grade (III or IV) tumors were included. Tumor location included lobar (38.4%), deep (45.3%), and cerebellar (16.3%) compartments. The mean follow-up time was 24 months (median 18 months, range 3-72 months). At the last follow-up, the volume of SLA-treated tumors had decreased in 80.6% of patients with follow-up data. Patients with high-grade tumors were more likely to have an unchanged or larger tumor size after SLA treatment than those with low-grade tumors (OR 7.49, p = 0.0364). Subsequent surgery and adjuvant treatment were not required after SLA treatment in 90.4% and 86.7% of patients, respectively. Patients with high-grade tumors were more likely to receive subsequent surgery (OR 2.25, p = 0.4957) and adjuvant treatment (OR 3.77, p = 0.1711) after SLA therapy, without reaching significance. A total of 29 acute complications in 23 patients were reported and included malpositioned catheters (n = 3), intracranial hemorrhages (n = 2), transient neurological deficits (n = 11), permanent neurological deficits (n = 5), symptomatic perilesional edema (n = 2), hydrocephalus (n = 4), and death (n = 2). On long-term follow-up, 3 patients were reported to have worsened neuropsychological test results. Pre-SLA tumor volume, tumor location, number of laser trajectories, and number of lesions created did not result in a significantly increased risk of complications; however, the odds of complications increased by 14% (OR 1.14, p = 0.0159) with every 1-cm3 increase in the volume of the lesion created.

Conclusions: SLA is an effective, minimally invasive treatment option for pediatric brain tumors, although it is not without risks. Limiting the volume of the generated thermal lesion may help decrease the incidence of complications.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7885863PMC
March 2020

Factors associated with syrinx size in pediatric patients treated for Chiari malformation type I and syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

J Neurosurg Pediatr 2020 Mar 6:1-11. Epub 2020 Mar 6.

31Department of Neurosurgery, University of Michigan, Ann Arbor, Michigan.

Objective: Factors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.

Methods: Using the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.

Results: The authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16-0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34-1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12-0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02-2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31-3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67-5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.

Conclusions: These data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.
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March 2020
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