Publications by authors named "Richard G Ellenbogen"

203 Publications

Pediatric hemispherectomy outcome: Adaptive functioning, intelligence, and memory.

Epilepsy Behav 2021 Sep 16;124:108298. Epub 2021 Sep 16.

Neurosciences, Seattle Children's Hospital, United States; Center for Integrated Brain Research, Seattle Children's Hospital, United States; Department of Neurology, University of Washington School of Medicine, United States; Division of Pediatric Neurology, Seattle Children's Hospital, United States.

Objective: Our purpose was to characterize neuropsychological evaluation (NP) outcome following functional hemispherectomy in a large, representative cohort of pediatric patients.

Methods: We evaluated seizure and NP outcomes and medical variables for all post-hemispherectomy patients from Seattle Children's Hospital epilepsy surgery program between 1996 and 2020. Neuropsychological evaluation outcome tests used were not available on all patients due to the diversity of patient ages and competency that is typical of a representative pediatric cohort; all patients had at least an adaptive functioning or intelligence measure, and a subgroup had memory testing.

Results: A total of 71 hemispherectomy patients (37 right; 34 females) yielded 66 with both preoperative (PREOP) plus postoperative (POSTOP) NPs and 5 with POSTOP only. Median surgery age was 5.7 (IQR 2-9.9) years. Engel classification indicated excellent seizure outcomes: 59 (84%) Class I, 6 (8%) Class II, 5 (7%) Class III, and 1 (1%) Class IV. Medical variables - including seizure etiology, surgery age, side, presurgical seizure duration, unilateral or bilateral structural abnormalities, secondarily generalized motor seizures - were not associated with either Engel class or POSTOP NP scores, though considerable heterogeneity was evident. Median PREOP and POSTOP adaptive functioning (PREOP n = 45, POSTOP n = 48) and intelligence (PREOP n = 29, POSTOP n = 36) summary scores were exceptionally low and did not reveal group decline from PREOP to POSTOP. Fifty-five of 66 (85%) cases showed stability or improvement. Specifically, 5 (8%) improved; 50 (76%) showed stability; and 11 (16%) declined. Improve and decline groups showed clinically interesting, but not statistical, differences in seizure control and age. Median memory summary scores were low and also showed considerable heterogeneity. Overall median PREOP to POSTOP memory scores (PREOP n = 16, POSTOP n = 24) did not reveal declines, and verbal memory scores improved. Twenty six percent of intelligence and 33% of memory tests had verbal versus visual-spatial discrepancies; all but one favored verbal, regardless of hemispherectomy side.

Significance: This large, single institution study revealed excellent seizure outcome in 91% of all 71 patients plus stability and/or improvement of intelligence and adaptive functioning in 85% of 66 patients who had PREOP plus POSTOP NPs. Memory was similarly stable overall, and verbal memory improved. Medical variables did not predict group NP outcomes though heterogeneity argues for further research. This study is unique for cohort size, intelligence plus memory testing, and evidence of primacy of verbal over visual-spatial development, despite hemispherectomy side. This study reinforces the role of hemispherectomy in achieving good seizure outcome while preserving functioning.
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http://dx.doi.org/10.1016/j.yebeh.2021.108298DOI Listing
September 2021

Signature morpho-electric, transcriptomic, and dendritic properties of human layer 5 neocortical pyramidal neurons.

Neuron 2021 Sep;109(18):2914-2927.e5

Allen Institute for Brain Science, Seattle, WA 98109, USA; Department of Neurological Surgery, University of Washington School of Medicine, Seattle, WA 98195, USA.

In the neocortex, subcerebral axonal projections originate largely from layer 5 (L5) extratelencephalic-projecting (ET) neurons. The unique morpho-electric properties of these neurons have been mainly described in rodents, where retrograde tracers or transgenic lines can label them. Similar labeling strategies are infeasible in the human neocortex, rendering the translational relevance of findings in rodents unclear. We leveraged the recent discovery of a transcriptomically defined L5 ET neuron type to study the properties of human L5 ET neurons in neocortical brain slices derived from neurosurgeries. Patch-seq recordings, where transcriptome, physiology, and morphology were assayed from the same cell, revealed many conserved morpho-electric properties of human and rodent L5 ET neurons. Divergent properties were often subtler than differences between L5 cell types within these two species. These data suggest a conserved function of L5 ET neurons in the neocortical hierarchy but also highlight phenotypic divergence possibly related to functional specialization of human neocortex.
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http://dx.doi.org/10.1016/j.neuron.2021.08.030DOI Listing
September 2021

Commentary: A Crowdsourced Consensus on Supratotal Resection Versus Gross Total Resection for Anatomically Distinct Primary Glioblastoma.

Neurosurgery 2021 Sep 1. Epub 2021 Sep 1.

Department of Neurological Surgery, University of Washington School of Medicine, Seattle, Washington, USA.

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http://dx.doi.org/10.1093/neuros/nyab324DOI Listing
September 2021

Evaluating the Utility of Routine Computed Tomography Scans after Cranial Vault Reconstruction for Children with Craniosynostosis.

Plast Reconstr Surg 2021 Jul;148(1):63e-70e

From the Department of Craniomaxillofacial Surgery, Erasmus University; the Department of Radiology, Department of Surgery, Division of Plastic Surgery, and Department of Neurological Surgery, University of Washington; and the Department of Pediatrics, Division of Craniofacial Medicine, Seattle Children's Hospital.

Background: Postoperative computed tomography scans allow for evaluation of the structural results of cranial vault reconstruction and potential surgical concerns. The authors evaluated the clinical utility of routine postoperative scans to identify relevant surgical findings in children treated for craniosynostosis.

Methods: The authors conducted a retrospective study of postoperative computed tomography reports for patients with craniosynostosis following cranial vault reconstruction during a 9-year period at their tertiary care pediatric hospital. They categorized postoperative computed tomography findings as typical, atypical, or indeterminate. Images with reported indeterminate or atypical findings were reviewed and verified by a pediatric neuroradiologist and a pediatric neurological surgeon. Clinical outcomes of patients with abnormal postoperative images were assessed with chart review for clinical relevance.

Results: Postoperative computed tomography radiology reports for 548 operations in 506 participants were included. Most participants had single-suture craniosynostosis (89 percent), were male (64 percent), and under 1 year of age (78 percent). Surgically concerning scans were described in 52 reports (<9.5 percent), and the research team's pediatric neuroradiologist confirmed abnormal findings in 36 (6.5 percent). Potentially relevant abnormal findings included subdural blood (n = 18), subarachnoid blood (n = 4), intraparenchymal findings (n = 6), bone abnormalities (n = 5), vascular injury (n = 3), and increased ventricular size (n = 2). Most cases with abnormal findings did not require additional observation nor intervention. Only three cases (of 548; 0.55 percent) required further intervention, which included additional medical management (n = 2) and return to the operating room (n = 1).

Conclusion: Abnormal findings on routine computed tomography scans after cranial vault reconstruction are uncommon and rarely result in an urgent surgical or medical intervention.

Clinical Question/level Of Evidence: Diagnostic, IV.
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http://dx.doi.org/10.1097/PRS.0000000000008056DOI Listing
July 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

United States Medicolegal Progress and Innovation in Telemedicine in the Age of COVID-19: A Primer for Neurosurgeons.

Neurosurgery 2021 08;89(3):364-371

Department of Neurological Surgery, University of Washington, Seattle, Washington, USA.

Telemedicine has received increased attention in recent years as a potential solution to expand clinical capability and patient access to care in many fields, including neurosurgery. Although patient and physician attitudes are rapidly shifting toward greater telemedicine use in light of the COVID-19 pandemic, there remains uncertainty about telemedicine's regulatory future. Despite growing evidence of telemedicine's utility, there remain a number of significant medicolegal barriers to its mass adoption and wider implementation. Herein, we examine recent progress in state and federal regulations in the United States governing telemedicine's implementation in quality of care, finance and billing, privacy and confidentiality, risk and liability, and geography and interstate licensure, with special attention to how these concern teleneurosurgical practice. We also review contemporary topics germane to the future of teleneurosurgery, including the continued expansion of reciprocity in interstate licensure, expanded coverage for homecare services for chronic conditions, expansion of Center for Medicare and Medicaid Services reimbursements, and protections of store-and-forward technologies. Additionally, we discuss recent successes in teleneurosurgery, stroke care, and rehabilitation as models for teleneurosurgical best practices. As telemedicine technology continues to mature and its expanse grows, neurosurgeons' familiarity with its benefits, limitations, and controversies will best allow for its successful adoption in our field to maximize patient care and outcomes.
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http://dx.doi.org/10.1093/neuros/nyab185DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8344865PMC
August 2021

The Delayed Neuropathological Consequences of Traumatic Brain Injury in a Community-Based Sample.

Front Neurol 2021 16;12:624696. Epub 2021 Mar 16.

Department of Laboratory Medicine and Pathology, University of Washington School of Medicine, Seattle, WA, United States.

The late neuropathological effects of traumatic brain injury have yet to be fully elucidated, particularly with respect to community-based cohorts. To contribute to this critical gap in knowledge, we designed a multimodal neuropathological study, integrating traditional and quantitative approaches to detect pathologic changes in 532 consecutive brain autopsies from participants in the Adult Changes in Thought (ACT) study. Diagnostic evaluation including assessment for chronic traumatic encephalopathy (CTE) and quantitative immunoassay-based methods were deployed to examine levels of pathological (hyperphosphorylated) tau (pTau) and amyloid (A) β in brains from ACT participants with ( = 107) and without ( = 425) history of remote TBI with loss of consciousness (w/LOC). Further neuropathological assessments included immunohistochemistry for α-synuclein and phospho-TDP-43 pathology and astro- (GFAP) and micro- (Iba1) gliosis, mass spectrometry analysis of free radical injury, and gene expression evaluation (RNA sequencing) in a smaller sub-cohort of matched samples (49 cases with TBI and 49 non-exposed matched controls). Out of 532 cases, only 3 (0.6%-none with TBI w/LOC history) showed evidence of the neuropathologic signature of chronic traumatic encephalopathy (CTE). Across the entire cohort, the levels of pTau and Aβ showed expected differences for brain region (higher levels in temporal cortex), neuropathological diagnosis (higher in participants with Alzheimer's disease), and genotype (higher in participants with one or more ε4 allele). However, no differences in PHF-tau or Aβ were identified by Histelide with respect to the history of TBI w/LOC. In a subset of TBI cases with more carefully matched control samples and more extensive analysis, those with TBI w/LOC history had higher levels of hippocampal pTau but no significant differences in Aβ, α-synuclein, pTDP-43, GFAP, Iba1, or free radical injury. RNA-sequencing also did not reveal significant gene expression associated with any measure of TBI exposure. Combined, these findings suggest long term neuropathological changes associated with TBI w/LOC may be subtle, involve non-traditional pathways of neurotoxicity and neurodegeneration, and/or differ from those in autopsy cohorts specifically selected for neurotrauma exposure.
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http://dx.doi.org/10.3389/fneur.2021.624696DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8008107PMC
March 2021

siRNA nanoparticle suppresses drug-resistant gene and prolongs survival in an orthotopic glioblastoma xenograft mouse model.

Adv Funct Mater 2021 Feb 6;31(6). Epub 2020 Nov 6.

Department of Materials Science and Engineering, University of Washington, Seattle, WA 98195, United States; Department of Neurological Surgery, University of Washington, Seattle, WA 98195, United States.

Temozolomide (TMZ) is the standard of care chemotherapy drug for treating glioblastomas (GBMs), the most aggressive cancer that affects people of all ages. However, its therapeutic efficacy is limited by the drug resistance mediated by a DNA repair protein, O-methylguanine-DNA methyltransferase (MGMT), which eliminates the TMZ-induced DNA lesions. Here we report the development of an iron oxide nanoparticle (NP) system for targeted delivery of siRNAs to suppress the TMZ-resistance gene (MGMT). We show that our NP is able to overcome biological barriers, bind specifically to tumor cells, and reduce MGMT expression in tumors of mice bearing orthotopic GBM serially-passaged patient-derived xenografts. The treatment with sequential administration of this NP and TMZ resulted in increased apoptosis of GBM stem-like cells, reduced tumor growth, and significantly-prolonged survival as compared to mice treated with TMZ alone. This study introduces an approach that holds great promise to improve the outcomes of GBM patients.
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http://dx.doi.org/10.1002/adfm.202007166DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7942690PMC
February 2021

Sport-Related Structural Brain Injury and Return to Play: Systematic Review and Expert Insight.

Neurosurgery 2021 05;88(6):E495-E504

Department of Neurosurgery, Johns Hopkins University Medical Center, Baltimore, Maryland, USA.

Background: Sport-related structural brain injury (SRSBI) is intracranial pathology incurred during sport. Management mirrors that of non-sport-related brain injury. An empirical vacuum exists regarding return to play (RTP) following SRSBI.

Objective: To provide key insight for operative management and RTP following SRSBI using a (1) focused systematic review and (2) survey of expert opinions.

Methods: A systematic literature review of SRSBI from 2012 to present in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and a cross-sectional survey of RTP in SRSBI by 31 international neurosurgeons was conducted.

Results: Of 27 included articles out of 241 systematically reviewed, 9 (33.0%) case reports provided RTP information for 12 athletes. To assess expert opinion, 31 of 32 neurosurgeons (96.9%) provided survey responses. For acute, asymptomatic SRSBI, 12 (38.7%) would not operate. Of the 19 (61.3%) who would operate, midline shift (63.2%) and hemorrhage size > 10 mm (52.6%) were the most common indications. Following SRSBI with resolved hemorrhage, with or without burr holes, the majority of experts (>75%) allowed RTP to high-contact/collision sports at 6 to 12 mo. Approximately 80% of experts did not endorse RTP to high-contact/collision sports for athletes with persistent hemorrhage. Following craniotomy for SRSBI, 40% to 50% of experts considered RTP at 6 to 12 mo. Linear regression revealed that experts allowed earlier RTP at higher levels of play (β = -0.58, 95% CI -0.111, -0.005, P = .033).

Conclusion: RTP decisions following structural brain injury in athletes are markedly heterogeneous. While individualized RTP decisions are critical, aggregated expert opinions from 31 international sports neurosurgeons provide key insight. Level of play was found to be an important consideration in RTP determinations.
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http://dx.doi.org/10.1093/neuros/nyab041DOI Listing
May 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 Judicious Use of Stereotactic Radiosurgery and Hypofractionated Stereotactic Radiotherapy in the Management of Large Brain Metastases.

Cancers (Basel) 2020 Dec 29;13(1). Epub 2020 Dec 29.

Department of Radiation Oncology, University of Washington School of Medicine, Seattle, WA 98195, USA.

Brain metastases are the most common intracranial malignant tumor in adults and are a cause of significant morbidity and mortality for cancer patients. Large brain metastases, defined as tumors with a maximum dimension >2 cm, present a unique clinical challenge for the delivery of stereotactic radiosurgery (SRS) as patients often present with neurologic symptoms that require expeditious treatment that must also be balanced against the potential consequences of surgery and radiation therapy-namely, leptomeningeal disease (LMD) and radionecrosis (RN). Hypofractionated stereotactic radiotherapy (HSRT) and pre-operative SRS have emerged as novel treatment techniques to help improve local control rates and reduce rates of RN and LMD for this patient population commonly managed with post-operative SRS. Recent literature suggests that pre-operative SRS can potentially half the risk of LMD compared to post-operative SRS and that HSRT can improve risk of RN to less than 10% while improving local control when meeting the appropriate goals for biologically effective dose (BED) and dose-volume constraints. We recommend a 3- or 5-fraction regimen in lieu of SRS delivering 15 Gy or less for large metastases or resection cavities. We provide a table comparing the BED of commonly used SRS and HSRT regimens, and provide an algorithm to help guide the management of these challenging clinical scenarios.
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http://dx.doi.org/10.3390/cancers13010070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7795798PMC
December 2020

Evolution of Cranioorbital Shape in Nonsyndromic, Muenke, and Saethre-Chotzen Bilateral Coronal Synostosis: A Case-Control Study of 2-Year Outcomes.

Plast Reconstr Surg 2021 01;147(1):148-159

From the Craniofacial Image Analysis Lab, Craniofacial Center, Seattle Children's Hospital; and the Division of Plastic Surgery and the Department of Neurological Surgery, University of Washington.

Background: The purpose of this study was to quantify change in cranioorbital morphology from presentation, after fronto-orbital advancement, and at 2-year follow-up.

Methods: Volumetric, linear, and angular analyses were performed on computed tomographic scans of consecutive bilateral coronal synostosis patients. Comparisons were made across three time points, between syndromic and nonsyndromic cases, and against normal controls. Significance was set at p < 0.05.

Results: Twenty-five patients were included: 11 were nonsyndromic, eight had Saethre-Chotzen syndrome, and six had Muenke syndrome. Total cranial volume was comparable to normal, age-matched control subjects before and 2 years after surgery despite an expansion during surgery. Axial and sagittal vector analyses showed advancement and widening of the lower forehead beyond control values with surgery and comparable anterior position, but increased width compared to controls at 2 years. Frontal bossing decreased with a drop in anterior cranial height and advanced lower forehead position. Middle vault height was not normalized and turricephaly persisted at follow-up. Posterior fossa volume remained lower at all three time points compared to control subjects. Supraorbital retrusion relative to anterior corneal position was overcorrected by surgery, with values comparable to those of control subjects at 2 years because of differential growth. There was no difference at 2 years between syndromic and nonsyndromic groups.

Conclusions: Open fronto-orbital advancement successfully remodels the anterior forehead but requires overcorrection to be comparable to normal at 2 years. Although there are differences in syndromic cases at presentation, they do not result in significant morphometric differences on follow-up. Posterior fossa volume remains lower at all time points.

Clinical Question/level Of Evidence: Therapeutic, IV.
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http://dx.doi.org/10.1097/PRS.0000000000007494DOI Listing
January 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

Medicolegal issues in abusive head trauma for the pediatric neurosurgeon.

Neurosurg Focus 2020 11;49(5):E23

1Department of Neurological Surgery, University of Washington; and.

The purpose of this article is to serve as a rational guide for the pediatric neurosurgeon in navigating common medicolegal issues that arise in the management of abusive head trauma (AHT). Many of these issues may be unfamiliar or unpleasant to surgeons focused on addressing disease. The authors begin with a brief history on the origins of the diagnosis of AHT and the controversy surrounding it, highlighting some of the facets of the diagnosis that make it particularly unique in pediatric neurosurgery. They then review some special medical considerations in these patients through the perspective of the neurosurgeon and provide several examples as illustration. The authors discuss how to appropriately document these cases in the medical record for expected legal review, and last, they provide an overview of the legal process through which the neurosurgeon may be called to provide testimony.
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http://dx.doi.org/10.3171/2020.8.FOCUS20599DOI Listing
November 2020

Expansile duraplasty and obex exploration compared with bone-only decompression for Chiari malformation type I in children: retrospective review of outcomes and complications.

J Neurosurg Pediatr 2020 Oct 30:1-8. Epub 2020 Oct 30.

1Department of Neurological Surgery and.

Objective: While a select population of pediatric patients with Chiari malformation type I (CM-I) remain asymptomatic, some patients present with tussive headaches, neurological deficits, progressive scoliosis, and other debilitating symptoms that necessitate surgical intervention. Surgery entails a variety of strategies to restore normal CSF flow, including increasing the posterior fossa volume via bone decompression only, or bone decompression with duraplasty, with or without obex exploration. The indications for duraplasty and obex exploration following bone decompression remain controversial. The objective of this study was to describe an institutional series of pediatric patients undergoing surgery for CM-I, performed by a single neurosurgeon. For patients presenting with a syrinx, the authors compared outcomes following bone-only decompression with duraplasty only and with duraplasty including obex exploration. Clinical outcomes evaluated included resolution of syrinx, scoliosis, presenting symptoms, and surgical complications.

Methods: A retrospective review was conducted of the medical records of 276 consecutive pediatric patients with CM-I operated on at a single institution between 2001 and 2015 by the senior author. Imaging findings of tonsillar descent, associated syrinx (syringomyelia or syringobulbia), basilar invagination, and clinical assessment of CM-I-attributable symptoms and scoliosis were recorded. In patients presenting with a syrinx, clinical outcomes, including syrinx resolution, symptom resolution, and impact on scoliosis progression, were compared for three surgical groups: bone-only/posterior fossa decompression (PFD), PFD with duraplasty (PFDwD), and PFD with duraplasty and obex exploration (PFDwDO).

Results: PFD was performed in 25% of patients (69/276), PFDwD in 18% of patients (50/276), and PFDwDO in 57% of patients (157/276). The mean follow-up was 35 ± 35 months. Nearly half of the patients (132/276, 48%) had a syrinx. In patients presenting with a syrinx, PFDwDO was associated with a significantly higher likelihood of syrinx resolution relative to PFD only (HR 2.65, p = 0.028) and a significant difference in time to symptom resolution (HR 2.68, p = 0.033). Scoliosis outcomes did not differ among treatment groups (p = 0.275). Complications were not significantly higher when any duraplasty (PFDwD or PFDwDO) was performed following bone decompression (p > 0.99).

Conclusions: In this series of pediatric patients with CM-I, patients presenting with a syrinx who underwent expansile duraplasty with obex exploration had a significantly greater likelihood of syrinx and symptom resolution, without increased risk of CSF-related complications, compared to those who underwent bone-only decompression.
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http://dx.doi.org/10.3171/2020.6.PEDS20376DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8085180PMC
October 2020

Human macrophages engineered to secrete a bispecific T cell engager support antigen-dependent T cell responses to glioblastoma.

J Immunother Cancer 2020 10;8(2)

Department of Neurological Surgery, University of Washington, Seattle, Washington, USA

Background: Targeted and effective treatment options are needed for solid tumors, including glioblastoma (GBM), where survival rates with standard treatments are typically less than 2 years from diagnosis. Solid tumors pose many barriers to immunotherapies, including therapy half-life and persistence, tumor penetrance, and targeting. Therapeutics delivered systemically may not traffic to the tumor site. If cellular therapies or drugs are able to access the tumor site, or can be delivered directly within the tumor, treatments may not persist for the duration necessary to reduce or eliminate tumor burden. An approach that allows durable and titratable local therapeutic protein delivery could improve antitumor efficacy while minimizing toxicities or unwanted on-target, off-tissue effects.

Methods: In this study, human monocyte-derived macrophages were genetically engineered to secrete a bispecific T cell engager (BiTE) specific to the mutated epidermal growth factor variant III (EGFRvIII) expressed by some GBM tumors. We investigated the ability of lentivirally modified macrophages to secrete a functional BiTE that can bind target tumor antigen and activate T cells. Secreted BiTE protein was assayed in a range of T cell functional assays in vitro and in subcutaneous and intracranial GBM xenograft models. Finally, we tested genetically engineered macrophages (GEMs) secreting BiTE and the proinflammatory cytokine interleukin (IL)-12 to amplify T cell responses in vitro and in vivo.

Results: Transduced human macrophages secreted a lentivirally encoded functional EGFRvIII-targeted BiTE protein capable of inducing T cell activation, proliferation, degranulation, and killing of antigen-specific tumor cells. Furthermore, BiTE secreting macrophages reduced early tumor burden in both subcutaneous and intracranial mouse models of GBM, a response which was enhanced using macrophages that were dual transduced to secrete both the BiTE protein and single chain IL-12, preventing tumor growth in an aggressive GBM model.

Conclusions: The ability of macrophages to infiltrate and persist in solid tumor tissue could overcome many of the obstacles associated with systemic delivery of immunotherapies. We have found that human GEMs can locally and constitutively express one or more therapeutic proteins, which may help recruit T cells and transform the immunosuppressive tumor microenvironment to better support antitumor immunity.
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http://dx.doi.org/10.1136/jitc-2020-001202DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7597484PMC
October 2020

Genetically engineered macrophages persist in solid tumors and locally deliver therapeutic proteins to activate immune responses.

J Immunother Cancer 2020 10;8(2)

Ben Towne Center for Childhood Cancer Research, Seattle Children's Research Institute, Seattle, Washington, USA

Background: Though currently approved immunotherapies, including chimeric antigen receptor T cells and checkpoint blockade antibodies, have been successfully used to treat hematological and some solid tumor cancers, many solid tumors remain resistant to these modes of treatment. In solid tumors, the development of effective antitumor immune responses is hampered by restricted immune cell infiltration and an immunosuppressive tumor microenvironment (TME). An immunotherapy that infiltrates and persists in the solid TME, while providing local, stable levels of therapeutic to activate or reinvigorate antitumor immunity could overcome these challenges faced by current immunotherapies.

Methods: Using lentivirus-driven engineering, we programmed human and murine macrophages to express therapeutic payloads, including Interleukin (IL)-12. In vitro coculture studies were used to evaluate the effect of genetically engineered macrophages (GEMs) secreting IL-12 on T cells and on the GEMs themselves. The effects of IL-12 GEMs on gene expression profiles within the TME and tumor burden were evaluated in syngeneic mouse models of glioblastoma and melanoma and in human tumor slices isolated from patients with advanced gastrointestinal malignancies.

Results: Here, we present a cellular immunotherapy platform using lentivirus-driven genetic engineering of human and mouse macrophages to constitutively express proteins, including secreted cytokines and full-length checkpoint antibodies, as well as cytoplasmic and surface proteins that overcomes these barriers. GEMs traffic to, persist in, and express lentiviral payloads in xenograft mouse models of glioblastoma, and express a non-signaling truncated CD19 surface protein for elimination. IL-12-secreting GEMs activated T cells and induced interferon-gamma (IFNγ) in vitro and slowed tumor growth resulting in extended survival in vivo. In a syngeneic glioblastoma model, IFNγ signaling cascades were also observed in mice treated with mouse bone-marrow-derived GEMs secreting murine IL-12. These findings were reproduced in ex vivo tumor slices comprised of intact MEs. In this setting, IL-12 GEMs induced tumor cell death, chemokines and IFNγ-stimulated genes and proteins.

Conclusions: Our data demonstrate that GEMs can precisely deliver titratable doses of therapeutic proteins to the TME to improve safety, tissue penetrance, targeted delivery and pharmacokinetics.
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http://dx.doi.org/10.1136/jitc-2020-001356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7594542PMC
October 2020

Issues of consent and assent in pediatric neurosurgery.

Childs Nerv Syst 2021 01 17;37(1):33-37. Epub 2020 Oct 17.

Department of Neurological Surgery, University of Washington, 325 Ninth Ave., Seattle, WA, 98104, USA.

Background: Consent and assent are important concepts to understand in the care of pediatric neurosurgery patients. Recently it has been recommended that although pediatric patients generally do not have the legal capacity to make medical decisions, they be encouraged to be involved in their own care. Given the paucity of information on this topic in the neurosurgery community, the objective is to provide pediatric neurosurgeons with recommendations on how to involve their patients in medical decision-making.

Methods: We review the essential elements and current guidelines of consent and assent for pediatric patients using illustrative neurosurgical case vignettes.

Results: The pediatric population ranges widely in cognitive and psychological development making the process of consent and assent quite complex. The role of the child or adolescent in medical decision-making, issues associated with obtaining assent or dissent, and informed refusal of treatment are considered.

Conclusion: The process of obtaining consent and assent represents a critical yet often overlooked aspect to care of pediatric neurosurgical patients. The pediatric neurosurgeon must be able to distill immensely complex and high-risk procedures into simple, understandable terms. Furthermore, they must recognize when the child's dissent or refusal to treatment is acceptable. In general, allowing children to be involved in their neurosurgical care is empowering and gives them both identity and agency, which is the vital first step to a successful neurosurgical intervention.
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http://dx.doi.org/10.1007/s00381-020-04907-wDOI Listing
January 2021

Continuous improvement in patient safety and quality in neurological surgery: the American Board of Neurological Surgery in the past, present, and future.

J Neurosurg 2020 Oct 16:1-7. Epub 2020 Oct 16.

22Department of Neurosurgery, David Geffen School of Medicine at UCLA, Los Angeles, California.

The American Board of Neurological Surgery (ABNS) was incorporated in 1940 in recognition of the need for detailed training in and special qualifications for the practice of neurological surgery and for self-regulation of quality and safety in the field. The ABNS believes it is the duty of neurosurgeons to place a patient's welfare and rights above all other considerations and to provide care with compassion, respect for human dignity, honesty, and integrity. At its inception, the ABNS was the 13th member board of the American Board of Medical Specialties (ABMS), which itself was founded in 1933. Today, the ABNS is one of the 24 member boards of the ABMS.To better serve public health and safety in a rapidly changing healthcare environment, the ABNS continues to evolve in order to elevate standards for the practice of neurological surgery. In connection with its activities, including initial certification, recognition of focused practice, and continuous certification, the ABNS actively seeks and incorporates input from the public and the physicians it serves. The ABNS board certification processes are designed to evaluate both real-life subspecialty neurosurgical practice and overall neurosurgical knowledge, since most neurosurgeons provide call coverage for hospitals and thus must be competent to care for the full spectrum of neurosurgery.The purpose of this report is to describe the history, current state, and anticipated future direction of ABNS certification in the US.
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http://dx.doi.org/10.3171/2020.6.JNS202066DOI Listing
October 2020

Early Effects of COVID-19 Pandemic on Neurosurgical Training in the United States: A Case Volume Analysis of 8 Programs.

World Neurosurg 2021 01 13;145:e202-e208. Epub 2020 Oct 13.

Department of Neurosurgery, University of Louisville, Louisville, Kentucky, USA.

Objective: To determine the impact of the 2019 novel coronavirus disease (COVID-19) pandemic on operative case volume in 8 U.S. neurosurgical residency training programs in early 2020 and to survey these programs regarding training activities during this period.

Methods: A retrospective review was conducted of monthly operative case volumes and types for 8 residency programs for 2019 and January through April 2020. Cases were grouped as elective cranial, elective spine, and nonelective emergent cases. Programs were surveyed regarding residents' perceptions of the impact of COVID-19 on surgical training, didactics, and research participation. Data were analyzed for individual programs and pooled across programs.

Results: Across programs, the 2019 monthly mean ± SD case volume was 211 ± 82; 2020 mean ± SD case volumes for January, February, March, and April were 228 ± 93, 214 ± 84, 180 ± 73, and 107 ± 45. Compared with 2019, March and April 2020 mean cases declined 15% (P = 0.003) and 49% (P = 0.002), respectively. COVID-19 affected surgical case volume for all programs; 75% reported didactics negatively affected, and 90% reported COVID-19 resulted in increased research time. Several neurosurgery residents required COVID-19 testing; however, to our knowledge, only 1 resident from the participating programs tested positive.

Conclusions: This study documents a significant reduction in operative volume in 8 neurosurgery residency training programs in early 2020. During this time, neurosurgery residents engaged in online didactics and research-related activities, reporting increased research productivity. Residency programs should collect data to determine the educational impact of the COVID-19 pandemic on residents' operative case volumes, identify deficiencies, and develop plans to mitigate any effects.
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http://dx.doi.org/10.1016/j.wneu.2020.10.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7550889PMC
January 2021

Developing microsurgical milestones for psychomotor skills in neurological surgery residents as an adjunct to operative training: the home microsurgery laboratory.

J Neurosurg 2020 Sep 4:1-11. Epub 2020 Sep 4.

Objective: A variety of factors contribute to an increasingly challenging environment for neurological surgery residents to develop psychomotor skills in microsurgical technique solely from operative training. While adjunct training modalities such as cadaver dissection and surgical simulation are embraced and practiced at our institution, there are no formal educational milestones defined to help residents develop, measure, and advance their microsurgical psychomotor skills in a stepwise fashion when outside the hospital environment. The objective of this report is to describe an efficient and convenient "home microsurgery lab" (HML) assembled and tested by the authors with the goal of supporting a personalized stepwise advancement of microsurgical psychomotor skills.

Methods: The authors reviewed the literature on previously published simulation practice models and designed adjunct learning modules utilizing the HML. Five milestones were developed for achieving proficiency with each graduated exercise, referencing the Accreditation Council for Graduate Medical Education (ACGME) guidelines. The HML setup was then piloted with 2 neurosurgical trainees.

Results: The total cost for assembling the HML was approximately $850. Techniques for which training was provided included microinstrument handling, tissue dissection, suturing, and microanastomoses. Five designated competency levels were developed, and training exercises were proposed for each competency level.

Conclusions: The HML offers a unique, entirely home-based, affordable adjunct to the operative neurosurgical education mandated by the ACGME operative case logs, while respecting resident hospital-based education hours. The HML provides surgical simulation with specific milestones, which may improve confidence and the microsurgical psychomotor skills required to perform microsurgery, regardless of case type.
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http://dx.doi.org/10.3171/2020.5.JNS201590DOI Listing
September 2020

A Comparison of Subgaleal Versus Subperiosteal Dissection in Open Cranial Vault Expansion for Sagittal Craniosynostosis.

World Neurosurg 2020 11 22;143:108-113. Epub 2020 Jul 22.

Division of Plastic Surgery, Department of Surgery, University of Washington School of Medicine, Seattle, Washington, USA; Department of Oral and Maxillofacial Surgery, University of Washington School of Dentistry, Seattle, Washington, USA. Electronic address:

Background: The aim of this study was to evaluate surgical outcomes for patients with sagittal craniosynostosis undergoing open cranial vault remodeling with a modified pi procedure comparing subgaleal versus subperiosteal dissection.

Methods: A retrospective chart review was performed for children between the ages of 3 and 7 months with sagittal craniosynostosis undergoing open cranial vault expansion at Seattle Children's Hospital. Patient demographics, operative variables, and postoperative outcomes including the surface area of bony cranial defects at 2-year follow-up were evaluated.

Results: Over a 3-year period, 35 patients between the ages of 3 and 7 months underwent surgical correction of sagittal craniosynostosis using our institutional adaptation of the modified pi technique. Twenty-five patients underwent exposure via a subgaleal (SG) approach, 10 patients had a subpericranial (SP) exposure. Compared with the SP group, the SG group had significant lower estimated blood loss and a shorter operating time (P < 0.05). There were no significant differences with regard to hospital length of stay or postoperative complications (P ≥ 0.48). At 2 years postoperatively, there were no significant differences in the size of the largest cranial defects (SG: 1.1 ± 0.1 cm versus 3.7 ± 0.1 cm, P = 0.40); no patients required a secondary cranioplasty.

Conclusions: Open posterior and middle cranial vault expansion is a safe and efficient method of open cranial vault expansion in sagittal craniosynostosis regardless of the plane of dissection. Elevation of the scalp flaps in the SG plane is a minor technical modification that can reduce blood loss and operative times.
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http://dx.doi.org/10.1016/j.wneu.2020.07.099DOI Listing
November 2020

Children with DIPG and high-grade glioma treated with temozolomide, irinotecan, and bevacizumab: the Seattle Children's Hospital experience.

J Neurooncol 2020 Jul 16;148(3):607-617. Epub 2020 Jun 16.

Division of Hematology/Oncology, Department of Pediatrics, Seattle Children's Hospital, University of Washington, 4800 Sand Point Way NE, M/S MB.8.501, PO Box 5371, Seattle, WA, 98105, USA.

Introduction: Beyond focal radiation, there is no consensus standard therapy for pediatric high-grade glioma (pHGG) and outcomes remain dismal. We describe the largest molecularly-characterized cohort of children with pHGG treated with a 3-drug maintenance regimen of temozolomide, irinotecan, and bevacizumab (TIB) following radiation.

Methods: We retrospectively reviewed 36 pediatric patients treated with TIB at Seattle Children's Hospital from 2009 to 2018 and analyzed survival using the Kaplan-Meier method. Molecular profiling was performed by targeted DNA sequencing and toxicities, steroid use, and palliative care utilization were evaluated.

Results: Median age at diagnosis was 10.9 years (18 months-18 years). Genetic alterations were detected in 26 genes and aligned with recognized molecular subgroups including H3 K27M-mutant (12), H3F3A G34-mutant (2), IDH-mutant (4), and hypermutator profiles (4). Fifteen patients (42%) completed 12 planned cycles of maintenance. Side effects associated with chemotherapy delays or modifications included thrombocytopenia (28%) and nausea/vomiting (19%), with temozolomide dosing most frequently modified. Median event-free survival (EFS) and overall survival (OS) was 16.2 and 20.1 months, with shorter survival seen in DIPG (9.3 and 13.3 months, respectively). Survival at 1, 2, and 5 years was 80%, 10% and 0% for DIPG and 85%, 38%, and 16% for other pHGG.

Conclusion: Our single-center experience demonstrates tolerability of this 3-drug regimen, with prolonged survival in DIPG compared to historical single-agent temozolomide. pHGG survival was comparable to analogous 3-drug regimens and superior to historical agents; however, cure was rare. Children with pHGG remain excellent candidates for the study of novel therapeutics combined with standard therapy.
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http://dx.doi.org/10.1007/s11060-020-03558-wDOI Listing
July 2020

Evaluating angioarchitectural characteristics of glial and metastatic brain tumors with conventional magnetic resonance imaging.

J Clin Neurosci 2020 Jun 17;76:46-52. Epub 2020 Apr 17.

Departments of Neurological Surgery, University of Washington, Seattle, WA, USA. Electronic address:

Primary and metastatic brain tumors can overlap in traditional imaging features detected on preoperative conventional magnetic resonance imaging (MRI). The research objective was to determine whether morphological vascular characteristics present in routine preoperative imaging using traditional MRI sequences are predictive of primary versus metastatic brain tumors; secondarily to determine association of conventional and vascular-related imaging parameters with intraoperative blood loss, pathological invasion, and World Health Organization (WHO) tumor grade. A retrospective review analyzed 100 consecutive intracranial tumor surgeries, 50 WHO grade II-IV gliomas and 50 intracranial metastases. Two blinded expert readers independently evaluated preoperative MRIs, obtained via standard morphological imaging sequences, for adjacent or intra-tumoral arterial aneurysm, peritumoral venous ectasia, prominence, or engorgement ("aberrant peritumoral vessels"), and prominent intra-tumoral flow voids. Multivariate analysis was performed to develop models predictive of glioma and glioblastoma (GBM). Aberrant peritumoral vessels and prominent intra-tumoral flow voids were statistically significant predictors of glioma in univariate analyses (p = 0.048, p = 0.001, respectively) and when combined in multivariate analysis (OR = 5.23, p = 0.001), particularly for GBM (OR = 9.08, p < 0.001). Multivariate modeling identified prominent intra-tumoral flow voids and FLAIR invasion as the strongest combined predictors of gliomas and GBM. Aberrant peritumoral vessels and larger tumor volume predicted higher intraoperative blood loss in all analyses. No vascular-related parameters predicted pathological invasion on multivariate analysis. Aberrant peritumoral vessels and prominent intra-tumoral flow voids were predictive of gliomas, specifically GBM. These vascular characteristics, evaluated on routine clinical preoperative MRI imaging, may aid in distinguishinggliomafrom brainmetastases andmay predict intraoperative blood loss.
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http://dx.doi.org/10.1016/j.jocn.2020.04.051DOI Listing
June 2020

Pediatric functional hemispherectomy: operative techniques and complication avoidance.

Neurosurg Focus 2020 04;48(4):E9

1Department of Neurological Surgery, University of Washington.

Functional hemispherectomy/hemispherotomy is a disconnection procedure for severe medically refractory epilepsy where the seizure foci diffusely localize to one hemisphere. It is an improvement on anatomical hemispherectomy and was first performed by Rasmussen in 1974. Less invasive surgical approaches and refinements have been made to improve seizure freedom and minimize surgical morbidity and complications. Key anatomical structures that are disconnected include the 1) internal capsule and corona radiata, 2) mesial temporal structures, 3) insula, 4) corpus callosum, 5) parietooccipital connection, and 6) frontobasal connection. A stepwise approach is indicated to ensure adequate disconnection and prevent seizure persistence or recurrence. In young pediatric patients, careful patient selection and modern surgical techniques have resulted in > 80% seizure freedom and very good functional outcome. In this report, the authors summarize the history of hemispherectomy and its development and present a graphical guide for this anatomically challenging procedure. The use of the osteoplastic flap to improve outcome and the management of hydrocephalus are discussed.
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http://dx.doi.org/10.3171/2020.1.FOCUS19889DOI Listing
April 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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http://dx.doi.org/10.3171/2020.1.PEDS19493DOI Listing
March 2020

Maintenance of Certification and the Platinum Rule: An Existential Crisis.

Mayo Clin Proc 2020 02;95(2):228-230

Mayo Clinic College of Medicine and Science, Rochester, MN.

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http://dx.doi.org/10.1016/j.mayocp.2019.07.021DOI Listing
February 2020

Surgical Versus Endovascular Management of Ruptured and Unruptured Intracranial Aneurysms: Emergent Issues and Future Directions.

World Neurosurg 2020 Apr 30;136:17-27. Epub 2019 Dec 30.

Department of Neurological Surgery, University of Washington, Seattle, Washington, USA. Electronic address:

Ideal management of unruptured intracranial aneurysms (UIAs) and ruptured intracranial aneurysms (RIAs) is a controversial issue. Over the last few decades, a significant paradigm shift has occurred away from open microsurgical clipping toward endovascular coil embolization. Multiple studies have been performed with mixed results. Some studies suggest that endovascular treatment produces better clinical and functional outcomes, but is associated with increased need for retreatment. Other studies report increased durability in aneurysms treated with microsurgical clipping, but that clipping may be associated with worse functional outcomes in some cases. Further complicating the dialogue are variable costs associated with different treatment modalities, including country-dependent cost differences. Here we provide a review of some of the major studies comparing open surgery versus endovascular treatment for both RIAs and UIAs to distill their key findings and corresponding implications for clinical practice. We relate these research results to our institution's experience with RIAs and UIAs and describe our approach to treatment of these conditions. Finally, we discuss implications of the paradigm shift for both open and endovascular surgery, including educational initiatives directed toward preserving important microsurgical tenets in the setting of diminishing surgical volume.
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http://dx.doi.org/10.1016/j.wneu.2019.12.127DOI Listing
April 2020

Acute traumatic presentation of Chiari I malformation with central cord syndrome and presyrinx in an infant.

Surg Neurol Int 2019 27;10:253. Epub 2019 Dec 27.

Department of Neurological Surgery, University of Washington, WA, United States.

Background: Chiari I malformation (CM-I) typically presents in late childhood and early adulthood. Often these lesions are asymptomatic and discovered incidentally. Patients typically present with tussive headaches and focal neurological findings, especially when associated with syringomyelia. Here, an 11-month-old child with a severely symptomatic CM-I required surgery (e.g., suboccipital craniectomy and C1/2 laminectomy) within the 1 year of life.

Case Description: An 11-month-old infant presented with acute bilateral upper extremity weakness following a ground-level fall. The magnetic resonance imaging of the cervical spine showed crowding at the craniocervical junction with 7 mm of cerebellar tonsillar herniation/descent, and swelling/edema of the cervical spinal cord with a presyrinx. The patient underwent an urgent suboccipital craniectomy and C1/2 laminectomy under intraoperative neuromonitoring; the motor evoked potentials in the upper and lower extremities partially recovered intraoperatively. One day postoperatively, bilateral upper extremity strength improved; 4 weeks later, he recovered full neurological function. The follow-up MR also showed complete resolution of the previously noted presyrinx.

Conclusion: Acute neurological deficits may occur in infants with CM-I who, following trauma, sustain the equivalent of a central cord syndrome. Neurosurgical evaluation with MR should prompt timely/appropriate surgical decompression (e.g., suboccipital craniectomy and C1/2 laminectomy).
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http://dx.doi.org/10.25259/SNI_256_2019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6935965PMC
December 2019
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