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Background: Absence of hydrocephalus on neuroimaging may impart a false sense of security for patients with pineal cysts. In this case series, we characterize a subset of patients with pineal cysts having an occult presentation. Unifying features of worsening paroxysmal headaches suggesting intermittent obstructive hydrocephalus and radiographic evidence of third ventricular invagination characterize these patients as high risk., Objective: To define features of occult, high-risk pineal cysts and outcomes of endoscopic cyst fenestration., Methods: Charts were retrospectively reviewed for patients with pineal cysts evaluated at our institution between 2018 and 2021 who underwent endoscopic cyst fenestration. To capture cysts presenting as occult, patients were excluded if hydrocephalus was noted at presentation. Relevant clinical history, imaging, operative data, and clinical outcomes were reviewed., Results: Of 50 pineal cyst patients, 4 satisfied inclusion criteria. All patients presented with worsening paroxysmal headaches. In addition, 75% (3/4) also experienced intermittent syncope. Patients exhibited no hydrocephalus (n = 3) or fluctuating ventricular size on longitudinal imaging (n = 1). In all cases, high-resolution sagittal 3-dimensional T2 magnetic resonance imaging demonstrated invagination of the cyst anteriorly into the posterior third ventricle. All patients underwent endoscopic cyst fenestration with complete symptom resolution (mean follow-up of 20.6 months; range 3.5-37.4 months)., Conclusion: The clinical history for occult, high-risk pineal cysts is notable for worsening paroxysmal headaches and episodic alterations of consciousness suggesting intermittent obstructive hydrocephalus. Because ventricular size can appear normal on standard imaging protocols, clinical suspicion should trigger workup with high-resolution magnetic resonance imaging designed to detect these cysts. Endoscopic cyst fenestration is a safe and efficacious management strategy., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Objective: Craniopharyngioma is a benign but surgically challenging brain tumor. Controversies exist regarding its ideal treatment strategy, goals of surgery, efficacy of radiation, and the long-term outcomes of these decisions. The authors of this study performed a detailed analysis of factors predictive of the extent of resection and recurrence in large series of craniopharyngiomas removed via an endoscopic endonasal approach (EEA) with long-term follow-up., Methods: From a prospective database of all EEAs done at Weill Cornell Medical College by the senior author from 2004 to 2022, a consecutive series of histologically proven craniopharyngiomas were identified. Gross-total resection (GTR) was generally the goal of surgery. Radiation was often given if GTR had not been achieved. The stalk was preserved if not infiltrated with tumor but was sacrificed to achieve GTR. Intentional subtotal resection (STR) was performed in select cases to avoid hypothalamic injury., Results: Among the 111 identified cases were 88 adults and 23 children. Newly diagnosed cases comprised 58.6% of the series. GTR was attempted in 77.5% of the patients and among those cases was achieved in 89.5% of treatment-naive tumors and 72.4% of recurrent tumors. An inability to achieve GTR was predicted by prior surgical treatment (OR 0.13, 95% CI 0.03-0.6, p = 0.009), tumor diameter ≥ 3.5 cm (OR 0.11, 95% CI 0.02-0.53, p = 0.006), and encasement of the optic nerve or a major artery (OR 0.11, 95% CI 0.01-0.8, p = 0.03). GTR with stalk preservation maintained some anterior pituitary function in 64.5% of cases and prevented diabetes insipidus in 25.8%. After a median follow-up of 51 months (IQR 17-80 months), the recurrence rate after GTR was 12.5% compared with 38.5% after non-GTR. The median recurrence-free survival was 5.5 years after STR, 8.3 years after near-total resection (≥ 98%), and not reached after GTR (p = 0.004, log-rank test). GTR was the strongest predictor of recurrence-free survival (OR 0.09, 95% CI 0.02-0.42, p = 0.002), whereas radiation did not show a statistically significant impact (OR 1.17, 95% CI 0.45-3.08). In GTR cases, the recurrence rate was higher if the stalk had been preserved (22.6%) as opposed to a sacrificed stalk (4.9%; OR 5.69, 95% CI 1.09-29.67)., Conclusions: The study data show that GTR should be the goal of surgery in craniopharyngiomas if it can be achieved safely. Although stalk preservation can maintain some endocrine function, the risk of recurrence is higher in such cases. Radiation may not be as effective as previously reported.

Glioblastomas are among the deadliest human cancers and are highly vascularized. Angiogenesis is dynamic during brain development, almost quiescent in the adult brain but reactivated in vascular-dependent CNS pathologies, including brain tumors. The oncofetal axis describes the reactivation of fetal programs in tumors, but its relevance in endothelial and perivascular cells of the human brain vasculature in glial brain tumors is unexplored. Nucleolin is a regulator of cell proliferation and angiogenesis, but its roles in the brain vasculature remain unknown. Here, we studied the expression of Nucleolin in the neurovascular unit in human fetal brains, adult brains, and human gliomas in vivo as well as its effects on sprouting angiogenesis and endothelial metabolism in vitro. Nucleolin is highly expressed in endothelial and perivascular cells during brain development, downregulated in the adult brain, and upregulated in glioma. Moreover, Nucleolin expression correlated with glioma malignancy in vivo. In culture, siRNA-mediated Nucleolin knockdown reduced human brain endothelial cell (HCMEC) and HUVEC sprouting angiogenesis, proliferation, filopodia extension, and glucose metabolism. Furthermore, inhibition of Nucleolin with the aptamer AS1411 decreased brain endothelial cell proliferation in vitro. Mechanistically, Nucleolin knockdown in HCMECs and HUVECs uncovered regulation of angiogenesis involving VEGFR2 and of endothelial glycolysis. These findings identify Nucleolin as a neurodevelopmental factor reactivated in glioma that promotes sprouting angiogenesis and endothelial metabolism, characterizing Nucleolin as an oncofetal protein. Our findings have potential implications in the therapeutic targeting of glioma.

Objective: Endonasal endoscopic odontoidectomy (EEO) is an alternative to transoral surgery for symptomatic ventral compression of the anterior cervicomedullary junction (CMJ), allowing for earlier extubation and feeding. Because the procedure destabilizes the C1-2 ligamentous complex, posterior cervical fusion is often performed concomitantly. The authors' institutional experience was reviewed to describe the indications, outcomes, and complications in a large series of EEO surgical procedures in which EEO was combined with posterior decompression and fusion., Methods: A consecutive, prospective series of patients who underwent EEO between 2011 and 2021 was studied. Demographic and outcome metrics, radiographic parameters, extent of ventral compression, extent of dens removal, and increase in CSF space ventral to the brainstem were measured on the preoperative and postoperative scans (first and most recent scans)., Results: Forty-two patients (26.2% pediatric) underwent EEO: 78.6% had basilar invagination, and 76.2% had Chiari type I malformation. The mean ± SD age was 33.6 ± 3.0 years, with a mean follow-up of 32.3 ± 4.0 months. The majority of patients (95.2%) underwent posterior decompression and fusion immediately before EEO. Two patients underwent prior fusion. There were 7 intraoperative CSF leaks but no postoperative CSF leaks. The inferior limit of decompression fell between the nasoaxial and rhinopalatine lines. The mean ± SD vertical height of dens resection was 11.98 ± 0.45 mm, equivalent to a mean ± SD resection of 74.18% ± 2.56%. The mean increase in ventral CSF space immediately postoperatively was 1.68 ± 0.17 mm (p < 0.0001), which increased to 2.75 ± 0.23 mm (p < 0.0001) at the most recent follow-up (p < 0.0001). The median (range) length of stay was 5 (2-33) days. The median time to extubation was 0 (0-3) days. The median time to oral feeding (defined as, at minimum, toleration of a clear liquid diet) was 1 (0-3) day. Symptoms improved in 97.6% of patients. Complications were rare and mostly associated with the cervical fusion portion of the combined surgical procedures., Conclusions: EEO is safe and effective for achieving anterior CMJ decompression and is often accompanied by posterior cervical stabilization. Ventral decompression improves over time. EEO should be considered for patients with appropriate indications.

Objective: Syringomyelia (syrinx) associated with Chiari malformation type I (CM-I) is commonly managed with posterior fossa decompression, which can lead to resolution in most cases. A persistent syrinx postdecompression is therefore uncommon and challenging to address. In the setting of radiographically adequate decompression with persistent syrinx, the authors prefer placing fourth ventricular subarachnoid stents that span the craniocervical junction particularly when intraoperative observation reveals arachnoid plane scarring. The objective of this study was to evaluate the safety and efficacy of a fourth ventricle stent for CM-I-associated persistent syringomyelia, assess dynamic changes in syrinx dimensions, and report stent-reduction durability, clinical outcomes, and procedure-associated complications., Methods: The authors performed a single-institution, retrospective review of patients who underwent fourth ventricular subarachnoid stent placement for persistent CM-I-associated syringomyelia following a prior posterior fossa decompression. The authors' institutional Chiari database contains 600 cases with 149 decompressions for CM-I-associated syringomyelia, of which 13 met criteria for inclusion. Data on patient demographics, clinical presentation and outcomes, and MRI findings were collected. The maximal syrinx diameter was estimated by calculating the area of an elliptical cross-section in the largest axial plane from preoperative, immediately postoperative, and late postoperative T2-weighted MR images., Results: All 13 patients experienced a significant decrease in mean syrinx area from the preoperative to the late postoperative MRI (mean syrinx diameter 114.1 ± 81.8 mm2 vs 24.5 ± 23.8 mm2, p < 0.001). The mean time until late postoperative MRI was 19.7 months (range 2.0-70.7 months). The syrinx area reduced on average by 75.0% ± 23.9% at the time of the last postoperative scan. Syrinx resolution was variable, with 4 patients (30.8%) achieving near-complete resolution (> 90%, grade III reduction), 7 patients (50%) having 50%-90% reduction (grade II), and 2 patients (14.3%) having < 50% decrease (grade I). One patient experienced catheter migration into the left brachium pontis with an associated cyst at the tip of the catheter that decreased in size on follow-up imaging., Conclusions: Placement of fourth ventricular subarachnoid stents spanning the craniocervical junction in patients with persistent CM-I-associated syringomyelia after posterior fossa decompression is a safe therapeutic option and significantly reduced the mean syrinx area, with a greater reductive effect seen over longer follow-up periods.

Pediatric brain tumors are the leading cause of cancer-related death in children in the United States and contribute a disproportionate number of potential years of life lost compared to adult cancers. Moreover, survivors frequently suffer long-term side effects, including secondary cancers. The Children's Brain Tumor Network (CBTN) is a multi-institutional international clinical research consortium created to advance therapeutic development through the collection and rapid distribution of biospecimens and data via open-science research platforms for real-time access and use by the global research community. The CBTN's 32 member institutions utilize a shared regulatory governance architecture at the Children's Hospital of Philadelphia to accelerate and maximize the use of biospecimens and data. As of August 2022, CBTN has enrolled over 4700 subjects, over 1500 parents, and collected over 65,000 biospecimen aliquots for research. Additionally, over 80 preclinical models have been developed from collected tumors. Multi-omic data for over 1000 tumors and germline material are currently available with data generation for > 5000 samples underway. To our knowledge, CBTN provides the largest open-access pediatric brain tumor multi-omic dataset annotated with longitudinal clinical and outcome data, imaging, associated biospecimens, child-parent genomic pedigrees, and in vivo and in vitro preclinical models. Empowered by NIH-supported platforms such as the Kids First Data Resource and the Childhood Cancer Data Initiative, the CBTN continues to expand the resources needed for scientists to accelerate translational impact for improved outcomes and quality of life for children with brain and spinal cord tumors., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. David S. Ziegler is a consultant, or on the advisory board, of Bayer, AstraZeneca, Accendatech, Novartis, Day One, FivePhusion, Amgen, Alexion, and Norgine. Angela J. Waanders is on the advisory board of Alexion and Day One., (Copyright © 2022. Published by Elsevier Inc.)

Background: The prognosis for metastatic and recurrent tumors of the central nervous system (CNS) remains dismal, and the need for newer therapeutic targets and modalities is critical. The cell surface glycoprotein B7H3 is expressed on a range of solid tumors with a restricted expression on normal tissues. We hypothesized that compartmental radioimmunotherapy (cRIT) with the anti-B7H3 murine monoclonal antibody omburtamab injected intraventricularly could safely target CNS malignancies., Patients and Methods: We conducted a phase I trial of intraventricular 131 I-omburtamab using a standard 3 + 3 design. Eligibility criteria included adequate cerebrospinal fluid (CSF) flow, no major organ toxicity, and for patients > dose level 6, availability of autologous stem cells. Patients initially received 74 MBq radioiodinated omburtamab to evaluate dosimetry and biodistribution followed by therapeutic 131 I-omburtamab dose-escalated from 370 to 2960 MBq. Patients were monitored clinically and biochemically for toxicity graded using CTCAEv 3.0. Dosimetry was evaluated using serial CSF and blood sampling, and serial PET or gamma-camera scans. Patients could receive a second cycle in the absence of grade 3/4 non-hematologic toxicity or progressive disease., Results: Thirty-eight patients received 100 radioiodinated omburtamab injections. Diagnoses included metastatic neuroblastoma (n = 16) and other B7H3-expressing solid tumors (n = 22). Thirty-five patients received at least 1 cycle of treatment with both dosimetry and therapy doses. Acute toxicities included < grade 4 self-limited headache, vomiting or fever, and biochemical abnormalities. Grade 3/4 thrombocytopenia was the most common hematologic toxicity. Recommended phase 2 dose was 1850 MBq/injection. The median radiation dose to the CSF and blood by sampling was 1.01 and 0.04 mGy/MBq, respectively, showing a consistently high therapeutic advantage for CSF. Major organ exposure was well below maximum tolerated levels. In patients developing antidrug antibodies, blood clearance, and therefore therapeutic index, was significantly increased. In patients receiving cRIT for neuroblastoma, survival was markedly increased (median PFS 7.5 years) compared to historical data., Conclusions: cRIT with 131 I-omburtamab is safe, has favorable dosimetry and may have a therapeutic benefit as adjuvant therapy for B7-H3-expressing leptomeningeal metastases., Trial Registration: clinicaltrials.gov NCT00089245, August 5, 2004., (© 2022. The Author(s).)

Background: Safe sampling of central nervous system tumor tissue for diagnostic purposes may be difficult if not impossible, especially in pediatric patients, and an unmet need exists to develop less invasive diagnostic tests., Methods: We report our clinical experience with minimally invasive molecular diagnostics using a clinically validated assay for sequencing of cerebrospinal fluid (CSF) cell-free DNA (cfDNA). All CSF samples were collected as part of clinical care, and results reported to both clinicians and patients/families., Results: We analyzed 64 CSF samples from 45 pediatric, adolescent and young adult (AYA) patients (pediatric = 25; AYA = 20) with primary and recurrent brain tumors across 12 histopathological subtypes including high-grade glioma (n = 10), medulloblastoma (n = 10), pineoblastoma (n = 5), low-grade glioma (n = 4), diffuse leptomeningeal glioneuronal tumor (DLGNT) (n = 4), retinoblastoma (n = 4), ependymoma (n = 3), and other (n = 5). Somatic alterations were detected in 30/64 samples (46.9%) and in at least one sample per unique patient in 21/45 patients (46.6%). CSF cfDNA positivity was strongly associated with the presence of disseminated disease at the time of collection (81.5% of samples from patients with disseminated disease were positive). No association was seen between CSF cfDNA positivity and the timing of CSF collection during the patient's disease course., Conclusions: We identified three general categories where CSF cfDNA testing provided additional relevant diagnostic, prognostic, and/or therapeutic information, impacting clinical assessment and decision making: (1) diagnosis and/or identification of actionable alterations; (2) monitor response to therapy; and (3) tracking tumor evolution. Our findings support broader implementation of clinical CSF cfDNA testing in this population to improve care., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objective: Virtual learning has made neurosurgery education more available to medical students (MSs) of all stages than ever before. We aimed to evaluate the impact of on-site learning in order to assess whether a return to this method of teaching, when safe, is warranted for MSs of all years., Methods: Registrants of the 2019 MS Neurosurgery Training Camps were sent precourse and postcourse surveys to assess changes in self-assessed confidence in concrete neurosurgical skills. Data were analyzed using a 2-tailed paired Student's t-test for continuous variables. A P value <0.05 was considered significant., Results: The 2019 Training Camp had 105 attendees, of whom 94 (89.5%) completed both surveys. Students reported statistically significant improvements in every surveyed skill area, except for understanding what is and is not sterile in an operating room. The cohort of MS 3/4 students indicated a postcourse decrease in confidence in their ability to understand what is and is not sterile in an operating room (93.69 ± 16.41 vs. 86.20 ± 21.18; P < 0.05). MS 3/4 students did not benefit in their ability to perform a neurologic examination or tie knots using a 1-handed technique., Conclusions: Neurosurgical education initiatives for MSs should continue to be developed. Hands-on neurosurgical training experiences for MSs serve as a valuable educational experience. Improvement in training models will lead to capitalizing on MS education to better improve readiness for neurosurgical residency without concern for patient safety., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Purpose: Programmable ventriculoperitoneal shunts (pVP shunts) are increasingly utilized for intraventricular chemotherapy, radioimmunotherapy, and/or cellular therapy. Shunt adjustments allow optimization of drug concentrations in the thecal space with minimization in the peritoneum. This report assesses the success of the pVP shunt as an access device for intraventricular therapies. Quantifying intrathecal drug delivery using scintigraphy by pVP shunt model has not been previously reported., Methods: We performed a single-institution, retrospective analysis on patients with CNS tumors and pVP shunts from 2003 to 2020, noting shunt model. pVP flow was evaluated for consideration of compartmental radioimmunotherapy (cRIT) using In-111-DTPA scintigraphy. Scintigraphy studies at 2-4 h and at 24 h quantified ventricular-thecal and peritoneal drug activity., Results: Twenty-two CSF flow studies were administered to 15 patients (N = 15) with diagnoses including medulloblastoma, metastatic neuroblastoma, pineoblastoma, and choroid plexus carcinoma. Six different types of pVP models were noted. 100% of the studies demonstrated ventriculo-thecal drug activity. 27% (6 of 22) of the studies had no peritoneal uptake visible by imaging. 73% (16 of 22) of the studies had minimal relative peritoneal uptake (< 12%). 27% (6 of 22) of the studies demonstrated moderate relative peritoneal uptake (12-37%). No studies demonstrated peritoneal uptake above 37%., Conclusions: All patients had successful drug delivery of In-111-DTPA to the ventriculo-thecal space. 73% of the patients had minimal relative (< 12%) peritoneal drug uptake. Though efficacy varies by shunt model, low numbers preclude conclusions regarding model superiority. CSF flow scintigraphy studies assesses drug distribution of In-111-DTPA, informing CSF flow for delivery of intraventricular therapies., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: Patients with Ehlers-Danlos syndrome (EDS) are at elevated risk for soft tissue complications when undergoing decompression with or without fusion of the craniocervical junction. We have previously shown that muscle flap closure can decrease reoperative rates. This study investigated whether myofascial flap closure improved clinical outcomes after simple or complex surgery of the craniocervical junction in EDS patients specifically., Methods: We performed a retrospective chart review of EDS patients who had undergone surgery for Chiari malformation at the Weill Cornell Medical Center between 2013 and 2020. Postoperative complications were recorded, including infection, wound dehiscence, seroma, hematoma, hardware removal, cerebrospinal fluid (CSF) leak, reoperation, and pseudomeningocele. Patients were stratified by type of closure and type of surgery. Fisher exact test was used for statistical comparison., Results: Between 2013 and 2020, 62 EDS patients who had surgery of the cervicocranial junction were reviewed. Of these, 31 patients had complex surgery with myofascial flap closure and 22 had simple surgery with traditional closure. The mean age at the time of surgery was 21.3 years. There were no significant differences in wound complications or reoperation rates between the simple surgery and complex surgery groups. In addition, there were no significant differences in complications between complex surgery with flap closure and simple surgery with traditional closure. Our CSF cutaneous fistula rate was 0%, considerably lower than rates reported in the literature, and, in one case, a patient developed a postoperative pseudomeningocele secondary to a dural leak, but the myofascial flap closure prevented its progression., Conclusions: Patients with EDS undergoing surgery of the cervicocranial junction may benefit from myofascial flap closure. Flap closure reduced complications after complex surgery of the craniocervical junction to the level of simple surgery. Our CSF leak rate was exceptionally low and only one patient experienced pseudomeningocele. Myofascial flaps are safe to perform in the EDS cohort and prevented CSF cutaneous fistula formation., Competing Interests: Conflicts of interest and source of funding: none declared., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM)., Methods: The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups., Results: A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD., Conclusions: PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

Purpose: Pediatric pituitary adenomas (pPAs) are uncommon. Thus, their presentation and outcomes after treatment are less well-understood than those of pituitary adenomas in adulthood (aPAs)., Methods: A retrospective chart review was conducted for all patients who underwent endoscopic endonasal transsphenoidal surgery (EETS) for pPA at NewYork-Presbyterian Hospital/Weill Cornell Medicine (NYP/WCM) from 2005-2020. Eleven patients were identified, and information pertaining to age, sex, adenoma characteristics, procedural details, and outcomes was reviewed. A systematic review of the literature was also performed to compare outcomes of EETS versus microscopic endonasal transsphenoidal surgery (METS) for pPA., Results: From 2005-2020, 11 patients underwent EETS for pPA at NYP/WCM. Mean age at operation was 14.9 ± 2.7 years, and 5 patients (45.5%) were male. 10 adenomas (90.9%) were hormone-producing. Of the functional adenomas, 8 (80.0%) were PRL-secreting and 2 (20.0%) were GH-secreting. Maximum adenoma diameter (MAD) ranged from 1.2-5.1 cm, with a median of 1.55 cm. Cavernous sinus invasion (CSI) occurred in 2 patients with macroprolactinoma. Gross total resection (GTR) was achieved in 10 (90.9%). Biochemical remission occurred in 5/10 (50.0%). Post-operative complications were documented in 8 cases (72.7%) and included diabetes insipidus, hypopituitarism, sinusitis, weight gain, cerebrospinal fluid leak, meningitis, and hydrocephalus. Systematic literature review of 105 microscopic and 175 endoscopic cases revealed high frequency of hormone-producing tumors (83.6%) and similar rates of GTR (82.4% vs 85.1%) and biochemical cure (75.8% vs 64.3%)., Conclusions: pPAs are more likely to be hormone producing and may be more aggressive and difficult to cure than aPAs. EETS is an effective treatment, although complication rates may be higher than in adult populations., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Intracranial hypotension (IH) manifests with orthostatic headaches secondary to cerebrospinal fluid (CSF) hypovolemia. Common iatrogenic etiologies include lumbar punctures and spinal surgery. Although much rarer, structural defects such as osteophytes and herniated calcified discs can violate dural integrity, resulting in CSF leak., Observations: The authors reported the case of a 32-year-old woman who presented with progressively worsening orthostatic headaches. During an extensive examination, magnetic resonance imaging of her thoracic spine revealed a cervicothoracic ventral epidural collection of CSF, prompting a dynamic computed tomography myelogram, which not only helped to confirm severe cerebral hypotension but also suggested underlying pathology of a dorsally projecting disc osteophyte complex at T2-3. Conservative and medical management failed to alleviate symptoms, and a permanent surgical cure was eventually sought. The patient underwent a transdural thoracic discectomy with dural repair, which resulted in resolution of her symptoms., Lessons: Clear guidelines regarding the management strategy of IH secondary to disc osteophyte complexes are yet to be established. A thorough literature review noted only 24 reported cases between 1998 and 2019, in which 13 patients received surgery. There is a 46% symptom resolution rate with conservative management, lower than that for iatrogenic etiologies. For patients in whom conservative management failed, surgical intervention proved effective in resolving symptoms, with a success rate of 92.3%.

Background: As our molecular understanding of pediatric central nervous system (CNS) tumors evolves, so too do diagnostic criteria, prognostic biomarkers, and clinical management decision making algorithms. Here, we explore the clinical utility of wide-breadth assays, including whole-exome sequencing (WES), RNA sequencing (RNA-seq), and methylation array profiling as an addition to more conventional diagnostic tools for pediatric CNS tumors., Methods: This study comprises an observational, prospective cohort followed at a single academic medical center over 3 years. Paired tumor and normal control specimens from 53 enrolled pediatric patients with CNS tumors underwent WES. A subset of cases also underwent RNA-seq (n = 28) and/or methylation array analysis (n = 27)., Results: RNA-seq identified the driver and/or targetable fusions in 7/28 cases, including potentially targetable NTRK fusions, and uncovered possible rationalized treatment options based on outlier gene expression in 23/28 cases. Methylation profiling added diagnostic confidence (8/27 cases) or diagnostic subclassification endorsed by the WHO (10/27 cases). WES detected clinically pertinent tier 1 or tier 2 variants in 36/53 patients. Of these, 16/17 SNVs/INDELs and 10/19 copy number alterations would have been detected by current in-house conventional tests including targeted sequencing panels., Conclusions: Over a heterogeneous set of pediatric tumors, RNA-seq and methylation profiling frequently yielded clinically relevant information orthogonal to conventional methods while WES demonstrated clinically relevant added value primarily via copy number assessment. Longitudinal cohorts comparing targeted molecular pathology workup vs broader genomic approaches including therapeutic selection based on RNA expression data will be necessary to further evaluate the clinical benefits of these modalities in practice., (© The Author(s) 2022. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Background: Radiographic characterization of Chiari malformation (CM) has historically focused on caudal tonsillar herniation (CH) below the foramen magnum. Previously, we published evidence linking ventral tonsillar herniation (VH) and medullary symptoms in very young children. We sought to extend that investigation by studying the radiographic and clinical significance of VH in adults diagnosed with CM., Methods: We retrospectively reviewed adults with cerebellar ectopia who underwent posterior fossa decompression with or without duraplasty (PFD/D) at our institution. VH was defined as tonsils crossing a line bisecting the caudal medulla at the level of the foramen magnum on axial MRI. Degree of VH was measured as distance between this bisecting line and the ventral tip of the herniated tonsil. Dorsal brainstem compression was qualitatively determined by assessing for obliteration of CSF space between the dorsal brainstem and the tonsils., Results: Out of 89 cases reviewed, 54 had some degree of VH. Compared with those without VH, the VH group was significantly older in age and more likely to also present with dorsal brainstem compression and headaches. No correlation was observed between degrees of CH and VH in the VH group. The degree of VH significantly decreased 3 months after PFD/D., Conclusions: VH is relatively common in CM patients and might be an important independent radiographic metric to evaluate and consider as part of the decision-making process, especially in those presenting with Chiari-like symptomatology referable to the medulla but who do not meet the traditional criteria of cerebellar ectopia greater than 5 mm., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: CSF-venous fistulas (CVF) may cause incapacitating positional headaches resulting from spontaneous intracranial hypotension/hypovolemia (SIH). Their etiology remains unknown, although unrecognized local trauma may precipitate SIH. In addition, they are diagnostically challenging despite various imaging tools available. Here, we present CVF identification using magnetic resonance myelography (MRM) and elaborate on their surgical management techniques., Methods: Retrospective charts of confirmed and treated CVF patients with attention to their diagnostic imaging modalities and management techniques were further reviewed., Results: Six cases were identified of which three are presented here. There were two females and one male patient. All had fistulas on the left side. Two were at T7-T8 while the third was at T9-T10 level. Two underwent hemilaminotomies at the T7-T8 while the third underwent a foraminotomy at T9 level to access the fistula site. All CVF were closed with a combination of an aneurysm clip and a silk tie. On follow-up, all had complete resolution of symptoms with no evidence of recurrence., Conclusion: Of the various imaging modalities available, MRM is particularly sensitive in localizing CVF spinal nerve level and their laterality. In addition, the technique of aneurysm clip ligation and placement of a silk tie is curative for these lesions., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Surgical Neurology International.)

Introduction: Tethered cord syndrome (TCS) is an amalgamation of neurological, urological, orthopedic, and dermatologic signs and symptoms with radiographic evidence of a thickened filum and low-lying conus. Surgical sectioning of the filum and disconnection of any tethering entities such as dermal sinus tracts or lipomas has been shown to improve outcomes. The manifestation of TCS symptoms in the absence of a low-lying conus has been referred to as occult tethered cord syndrome (OTCS) and is much less well reviewed in the literature. To date, there has only been one randomized controlled trial examining the effect of intervention in OTCS; therefore, contemporary data is often elicited from limited cohorts., Objective: To perform a comprehensive literature review of management in OTCS and evaluate treatment response rates to sectioning of the filum terminale., Results: Seventeen papers met inclusion criteria for our review. Sample sizes ranged from 8 to 60 children, and results were mixed, often dependent on study design, definition of typical OTCS symptoms, and follow-up intervals. Symptomatic improvement was observed in > 50% of patients for all but one study; however, the recurrence rates were highly variable., Conclusion: The data regarding the efficacy of surgical treatment in OTCS is mixed and merits more rigorous scientific examination with strict and clear parameters regarding symptomatic operationalization and follow-up time points to monitor for TCS recurrence., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Delivery of drugs intraarterially to brain tumors has been demonstrated in adults. In this study, the authors initiated a phase I trial of superselective intraarterial cerebral infusion (SIACI) of bevacizumab and cetuximab in pediatric patients with refractory high-grade glioma (diffuse intrinsic pontine glioma [DIPG] and glioblastoma) to determine the safety and efficacy in this population., Methods: SIACI was used to deliver mannitol (12.5 ml of 20% mannitol) to disrupt the blood-brain barrier (BBB), followed by bevacizumab (15 mg/kg) and cetuximab (200 mg/m2) to target VEGF and EGFR, respectively. Patients with brainstem tumors had a balloon inflated in the distal basilar artery during mannitol infusion., Results: Thirteen patients were treated (10 with DIPG and 3 with high-grade glioma). Toxicities included grade I epistaxis (2 patients) and grade I rash (2 patients). There were no dose-limiting toxicities. Of the 10 symptomatic patients, 6 exhibited subjective improvement; 92% showed decreased enhancement on day 1 posttreatment MRI. Of 10 patients who underwent MRI at 1 month, 5 had progressive disease and 5 had stable disease on FLAIR, whereas contrast-enhanced scans demonstrated progressive disease in 4 patients, stable disease in 2, partial response in 2, and complete response in 1. The mean overall survival for the 10 DIPG patients was 519 days (17.3 months), with a mean posttreatment survival of 214.8 days (7.2 months)., Conclusions: SIACI of bevacizumab and cetuximab was well tolerated in all 13 children. The authors' results demonstrate safety of this method and warrant further study to determine efficacy. As molecular targets are clarified, novel means of bypassing the BBB, such as intraarterial therapy and convection-enhanced delivery, become more critical. Clinical trial registration no.: NCT01884740 (clinicaltrials.gov).

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.

Objective: A meta-analysis was performed to understand disparities in the representation of female authorship within the neurosurgical literature and implications for career advancement of women in neurosurgery., Methods: Author names for articles published in 16 of the top neurosurgical journals from 2002 to 2019 were obtained from MEDLINE. The gender of each author was determined using automated prediction methods. Publication trends were compared over time and across subdisciplines. Female authorship was also compared to the proportionate composition of women in the field over time., Results: The metadata obtained from 16 major neurosurgical journals yielded 66,546 research articles. Gender was successfully determined for 96% (127,809/133,578) of first and senior authors, while the remainder (3.9%) were unable to be determined through prediction methods. Across all years, 13.3% (8826) of articles had female first authorship and 9.1% (6073) had female senior authorship. Female first authorship increased significantly over time from 5.8% in 2002 to 17.2% in 2019 (p < 0.001). Female senior authorship also increased significantly over time, from 5.5% in 2002 to 12.0% in 2019 (p < 0.001). The journals with the highest proportions of female first authors and senior authors were the Journal of Neurosurgery: Pediatrics (33.5%) and the Asian Journal of Neurosurgery (23.8%), respectively. Operative Neurosurgery had the lowest fraction of female first (12.4%) and senior (4.7%) authors. There was a significant difference between the year-by-year proportion of female neurosurgical trainees and the year-by-year proportion of female neurosurgical first (p < 0.001) and senior (p < 0.001) authors. Articles were also more likely to have a female first author if the senior author of the article was female (OR 2.69, CI 2.52-2.86; p < 0.001). From 1944 to 2019, the Journal of Neurosurgery showed a steady increase in female first and senior authorship, with a plateau beginning in the 1990s., Conclusions: Large meta-analysis techniques have the potential to effectively leverage large amounts of bibliometric data to quantify the representation of female authorship in the neurosurgical literature. The proportion of female authors in major neurosurgical journals has steadily increased. However, the rate of increase in female senior authorship has lagged behind the rate of increase in first authorship, indicating a disparity in academic advancement in women in neurosurgery.

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.

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., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Introduction: Children aged 0-6 years with Chiari malformation (CM) often present with atypical symptoms and require revision surgery more often than older children. We studied characteristics and outcomes of CM patients in this age-group who underwent one or more revision surgeries to assess how often revision surgery is necessary and successful in this age-group., Methods: We retrospectively reviewed patients who were diagnosed with CM 1 or CM 1.5 and surgically treated with posterior fossa decompression (PFD) with or without duraplasty before their 7th birthday. Basic demographics, preoperative presentation, operative details, and postoperative outcomes were analyzed., Results: Forty patients (mean age 3.2 ± 1.7 years, 35% female) were reviewed. The most common presenting symptoms were headache, dysphagia, and respiratory problems. Eight patients required one or more revision surgeries 11.6 ± 7.6 months on average after their initial surgery. Comparing the revision and no revision groups, dysautonomia was significantly more common prior to initial surgery in the children requiring revision (37.5 vs. 3.1%, p = 0.02). The revision group also trended toward more dysphagia (75.0 vs. 46.9%, p = 0.24) and respiratory problems (75.0 vs. 40.6%, p = 0.12). The most frequent reasons for reoperation were symptom recurrence (6/8), residual posterior fossa compression (3/8), significant scar tissue formation (2/8), ventral brainstem compression (1/8), and suspected craniocervical instability (1/8). Of the 8 children undergoing reoperation, surgery achieved symptom resolution or improvement in 5, while 3 had unchanged symptoms. Two patients underwent a third surgery, after which 1 showed improvement and the other did not. This last patient showed short-term improvement after a fourth surgery but had symptom recurrence 12 months later., Discussion/conclusion: Oropharyngeal and respiratory problems are particularly common in children aged 0-6 years with CM. Presentation with dysautonomia or other signs of brainstem compression will often predict an additional surgery will be needed after an initial PFD. Symptom recurrence is the most frequent reason for reoperation, and revision surgeries lead to improved clinical outcomes in the majority but not all of these young patients. Surgery in very young children is successful, but reoperation should be integrated into an up-front discussion algorithm particularly in children with severe symptoms., (© 2021 The Author(s) Published by S. Karger AG, Basel.)

Background: The pathophysiological connection between Chiari malformation and syringomyelia is accepted. Debate remains, however, how can we best define changes in syringomyelia following surgery., Objective: To introduce a grading system focusing on syrinx reduction based on routinely and reproducible radiological information, and provide a suggestion of the application of this scale for prediction of patient's prognoses., Methods: Data from 48 patients with Chiari malformation and syringomyelia were compiled. We calculated syrinx cross-sectional area by approximating an ellipse in the largest axial plane. We compared the percentage of reduction or enlargement following surgery. The percentage change was grouped into four grades: Grade 0 = Increasing size, grade I ≤ 50% reduction, grade II = 50% to 90% reduction, grade III ≥ 90% reduction., Results: A total of 89.6% of patients had syrinx improvement after surgery. A total of 5 patients were grade 0, 14 were grade I, 20 patients were grade II, and 9 patients met criteria for grade III. The mean postoperative syrinx area was 24.1 mm2 (0-169 mm2) with a mean syrinx reduction of 62.7%., Conclusion: Radiological improvement of syringomyelia can be mathematically defined and standardized to assist in communication in outcome-based trials. Radiological resolution is expected most patients., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Objective: Early surgical intervention for pediatric refractory epilepsy is increasingly advocated as surgery has become safer and data have demonstrated improved outcomes with early seizure control. There is concern that the risks associated with staged invasive electroencephalography (EEG) in very young children outweigh the potential benefits. Here, the authors present a cohort of children with refractory epilepsy who were referred for invasive monitoring, and they evaluate the role and safety of staged invasive EEG in those 3 years old and younger., Methods: The authors conducted a retrospective review of children 3 years and younger with epilepsy, who had been managed surgically at two institutions between 2001 and 2015. A cohort of pediatric patients older than 3 years of age was used for comparison. Demographics, seizure etiology, surgical management, surgical complications, and adverse events were recorded. Statistical analysis was completed using Stata version 13. A p < 0.05 was considered statistically significant. Fisher's exact test was used to compare proportions., Results: Ninety-four patients (45 patients aged ≤ 3 [47.9%]) and 208 procedures were included for analysis. Eighty-six procedures (41.3%) were performed in children younger than 3 years versus 122 in the older cohort (58.7%). Forty-two patients underwent grid placement (14 patients aged ≤ 3 [33.3%]); 3 of them developed complications associated with the implant (3/42 [7.14%]), none of whom were among the younger cohort. Across all procedures, 11 complications occurred in the younger cohort versus 5 in the older patients (11/86 [12.8%] vs 5/122 [4.1%], p = 0.032). Two adverse events occurred in the younger group versus 1 in the older group (2/86 [2.32%] vs 1/122 [0.82%], p = 0.571). Following grid placement, 13/14 younger patients underwent guided resections compared to 20/28 older patients (92.9% vs 71.4%, p = 0.23)., Conclusions: While overall complication rates were higher in the younger cohort, subdural grid placement was not associated with an increased risk of surgical complications in that population. Invasive electrocorticography informs management in very young children with refractory, localization-related epilepsy and should therefore be used when clinically indicated.

Background: Baclofen pumps provide treatment of symptoms of spasticity for disease processes such as cerebral palsy and traumatic brain injury. These devices provide continuous infusion or periodic dosing of intrathecal baclofen (ITB). Traditionally, these pumps have been placed subcutaneously. Subcutaneous device placement has been associated with infection and extrusion. Baclofen pumps are large and range from 8 to 10 cm in diameter and 4 to 8 cm in width. Patients requiring device placement typically have a paucity of subcutaneous tissue. Cachexia coupled with the size and bulk of these devices leads to increased protusion and friction. Submuscular placement provides a well-vascularized pocket that directs the device inward eliminating protrusion and decreasing the potential for soft tissue breakdown., Methods: A retrospective chart review of ITB pump placement in a submuscular plane by a single plastic surgeon at a major academic center in conjunction with a neurosurgeon was performed. Inclusion criteria were cases of primary placement or replacement of ITB pumps and spasticity requiring ITB. Major complications included infection, extrusion, and reoperation., Results: Five patients during a 5-month period were treated with submuscular placement of ITB pumps. Average age of patients included was 18.4 years. Average preoperative body mass index was 18.8 kg/m, with values ranging from 15.8 to 20.1 kg/m. Medical histories of patients included diagnoses of cerebral palsy and traumatic brain injury causing spasticity. The most frequently cited reason for plastic surgical consultation preoperatively was cachexia. Two patients had previous baclofen pumps placed subcutaneously. Average follow-up was 6 months and ranged from 3 to 13 months. There were no major complications. One patient had a small seroma that spontaneously resolved. No patient had wound healing problems, and there were no extrusions of implanted devices or reoperations., Conclusion: Submuscular placement of baclofen pumps provides a well-vascularized and stable environment for device placement that minimizes the dangers of pump extrusion and infection. Patients who require treatment with ITB commonly have severe cachexia, which makes subcutaneous device placement high risk. Submuscular placement should be performed in all patients with body mass index less than 20 kg/m.

Introduction: The benefits of decompression and fusion for patients with craniocervical instability are well described. However, complications such as wound breakdown and need for unplanned reoperation frequently occur. Recent studies have shown advantages of myofascial flap closure for various spinal procedures. This study investigated whether closure with myofascial flaps after surgery for craniocervical instability decreases complications with further subgroup analysis of patients with Ehlers-Danlos syndrome (EDS)., Methods: A retrospective review of patients presenting to Weill Cornell Medical Center from 2010 to 2017 for craniocervical surgery was performed. All patients who underwent craniocervical surgery, regardless of plastic surgical involvement, were included in the study. Data including patient demographics, comorbidities, EDS diagnosis, surgical history, complications, and follow-up information were collected and analyzed., Results: Data from 57 patients were analyzed. Eighteen patients (31.6%) had craniocervical surgery without myofascial flap closure, whereas 39 (68.4%) had surgery with flap closure. In the nonflap group, 9 patients required unplanned reoperation (50%). In the flap group, there were 5 patients requiring unplanned reoperation (15%). For reoperation, the Fisher exact test 2-tailed P value is 0.0096. Of those 57 patients, 24 had EDS: 5 (20.8%) had no flap closure, whereas 19 (79.2%) had flap closure. In the no-flap group, 3 patients required unplanned reoperation (60%). In the flap group, 5 patients required unplanned reoperation (21%). For reoperation, the Fisher exact test 2-tailed P value is 0.1265., Conclusions: Patients undergoing surgery for craniocervical instability may benefit from myofascial flap closure even if they have EDS. Mobilizing well-vascularized tissue can decrease rates of reoperation.

An 8-year-old child presented with fatigue, weight loss, and visual deterioration. MRI demonstrated a craniopharyngioma with compression of the optic chiasm and extensive edema on the hypothalamus and optic radiations. The tumor was completely removed via an endoscopic endonasal approach. Postoperatively, vision improved and hypothalamic edema completely resolved within 5 days. This video demonstrates the technical nuances of the surgery and discusses the impact of surgery on the hypothalamic nuclei in pediatric patients. The video can be found here: https://youtu.be/wxkBmhTPi6c., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2020, The Authors.)

Chiari malformation comprises a spectrum of congenital malformations characterized by a herniation of the cerebellar tonsils below the foramen magnum. Chiari malformation type I (CM-I) is the most prevalent subtype seen in clinical practice. This condition variably compresses the cerebellum and medulla-spinal cord junction secondary to malformation of the posterior fossa. Most neurologists and neurosurgeons recognize the sensorimotor and lower brainstem manifestations that result in the clinical picture of CM-I. The effects of CM-I on cognitive functioning, however, and their impact on neuropsychological performance are poorly understood, despite having long been recognized. This article reviews neuropsychological deficits demonstrated by individuals with CM-I, and explores cerebellocortical neuroanatomic pathways to provide possible rationale for the neurocognitive impairments present in affected individuals., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: To review indications and techniques for the endoscopic endonasal approach to the craniovertebral junction (CVJ), analyze postoperative outcomes, and discuss important technical considerations., Methods: A retrospective analysis was performed on all patients undergoing endonasal endoscopic approaches to the CVJ from May 2007 to June 2017. Demographic information, presenting symptoms, imaging results, treatment course, postoperative functional status, and follow-up were recorded., Results: There was a total of 30 patients in this series, with a mean follow-up of 11.7 months. The average age was 33.6 years (range, 5-75 years), with 18 females and 12 males. The majority of patients ( n  = 22, 73.3%) had Chiari malformation type 1 with basilar invagination and symptomatic cervicomedullary compression as the indication for surgery. Intraoperative cerebrospinal fluid leak (CSF) was noted in 3 cases of odontoid resection and a single case of skull base resection. There were no postoperative CSF leaks. Overall, 81% of patients resumed regular diet by post-operative day 2 (range, 0-8 days). Severe postoperative dysphagia occurred in two cases with one requiring gastrostomy tube placement and another utilizing total parenteral nutrition for support prior to eventual gastrostomy. On average, patients were extubated by postoperative day 0.93 (range 0-3 days), with 85% extubated by postoperative day 1. A tracheotomy was required in one patient., Conclusion: The endonasal endoscopic approach is a valuable technique for access to the CVJ with minimal disruption of respiratory and alimentary function., Competing Interests: None., (© 2020 The Authors.)

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.

Objective: Endoscopic endonasal approaches (EEAs) to the skull base have evolved over the last 20 years to become an essential component of a comprehensive skull base practice. Many case series show a learning curve from the earliest cases, in which the authors were inexperienced or were not using advanced closure techniques. It is generally accepted that once this learning curve is achieved, a plateau is reached with little incremental improvement. Cases performed during the early steep learning curve were eliminated to examine whether the continued improvement exists over the "tail end" of the curve., Methods: A prospectively acquired database of all EEA cases performed by the senior authors at Weill Cornell Medicine/NewYork-Presbyterian Hospital was reviewed. The first 200 cases were eliminated and the next 1000 consecutive cases were examined to avoid the bias created by the early learning curve., Results: Of the 1000 cases, the most common pathologies included pituitary adenoma (51%), meningoencephalocele or CSF leak repair (8.6%), meningioma (8.4%), craniopharyngioma (7.3%), basilar invagination (3.1%), Rathke's cleft cyst (2.8%), and chordoma (2.4%). Use of lumbar drains decreased from the first half to the second half of our series (p <0.05) as did the authors' use of fat alone (p <0.005) or gasket alone (p <0.005) for dural closure, while the use of a nasoseptal flap increased (p <0.005). Although mean tumor diameter was constant (on average), gross-total resection (GTR) increased from 60% in the first half to 73% in the second half (p <0.005). GTR increased for all pathologies but most significantly for chordoma (56% vs 100%, p <0.05), craniopharyngioma (47% vs 0.71%, p <0.05) and pituitary adenoma (67% vs 75%, p <0.05). Hormonal cure for secreting adenomas also increased from 83% in the first half to 89% in the second half (p <0.05). The rate of any complication was unchanged at 6.4% in the first half and 6.2% in the latter half of cases, and vascular injury occurred in only 0.6% of cases. Postoperative CSF leak occurred in 2% of cases and was unchanged between the first and second half of the series., Conclusions: This study demonstrates that contrary to popular belief, the surgical learning curve does not plateau but can continue for several years depending on the complexity of the endpoints considered. These findings may have implications for clinical trial design, surgical education, and patient safety measures.

Recent work has highlighted the tumor microenvironment as a central player in cancer. In particular, interactions between tumor and immune cells may help drive the development of brain tumors such as glioblastoma multiforme (GBM). Despite significant research into the molecular classification of glioblastoma, few studies have characterized in a comprehensive manner the immune infiltrate in situ and within different GBM subtypes.In this study, we use an unbiased, automated immunohistochemistry-based approach to determine the immune phenotype of the four GBM subtypes (classical, mesenchymal, neural and proneural) in a cohort of 98 patients. Tissue Micro Arrays (TMA) were stained for CD20 (B lymphocytes), CD5, CD3, CD4, CD8 (T lymphocytes), CD68 (microglia), and CD163 (bone marrow derived macrophages) antibodies. Using automated image analysis, the percentage of each immune population was calculated with respect to the total tumor cells. Mesenchymal GBMs displayed the highest percentage of microglia, macrophage, and lymphocyte infiltration. CD68 + and CD163 + cells were the most abundant cell populations in all four GBM subtypes, and a higher percentage of CD163 + cells was associated with a worse prognosis. We also compared our results to the relative composition of immune cell type infiltration (using RNA-seq data) across TCGA GBM tumors and validated our results obtained with immunohistochemistry with an external cohort and a different method. The results of this study offer a comprehensive analysis of the distribution and the infiltration of the immune components across the four commonly described GBM subgroups, setting the basis for a more detailed patient classification and new insights that may be used to better apply or design immunotherapies for GBM.

Context: Multiple angles of the craniocervical junction (CCJ) are associated with pathological conditions and surgical outcomes, including the clivo-axial angle (CXA), clival slope (CS), and sagittal axis (XS). However, there are varying normative ranges reported and a paucity of data analyzing the effects of imaging modality, age, and gender on these angles., Setting and Design: A retrospective review of computed tomographic (CT) and magnetic resonance imaging (MRI) scans in fifty adults without CCJ pathology from 2014 to 2019., Methods: Age, gender, indication, and hours between scans were recorded. Two-blinded observers measured all angles. Analysis between angles from the same patient was performed using the Wilcoxon signed-rank test. Multivariable linear regression was used to test for associations between average angles and age or gender., Results: Average age and time between scans were 41.3 and 14.3 h, respectively, with 94% performed due to trauma. On CT, average CXA, CS, and XS were 162.1°, 118.4°, and 81.3°, respectively. On MRI, they were 159.8°, 117.2°, 85.3°, respectively. There were statistically significant differences between CXA and XS ( P < 0.01) based on imaging modality. On CT, there was a significant increase in XS by 1.93°° and decrease in CS by 1.88°° and on MRI, there was a significant increase in CXA by 1.93°° and decrease in CS by 2.75°° corresponding with a 10-year advancement of age. Gender did not have an effect., Conclusion: There are significant differences in angular measurements of the CCJ between CT and MRI from the same patient, as well as changes in normative values based on age., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Craniovertebral Junction and Spine.)

Purpose: Occipitocervical instrumentation is infrequently required for stabilization of the axial and subaxial cervical spine in very young children. However, when it is necessary, unique surgical considerations arise in children when compared with similar procedures in adults., Methods: The authors reviewed literature describing fusion of the occipitocervical junction (OCJ) in toddlers and share their experience with eight cases of young children (age less than or equal to 4 years) receiving occiput to axial or subaxial spine instrumentation and fixation. Diagnoses and indications included severe or secondary Chiari malformation, skeletal dysplastic syndromes, Klippel-Feil syndrome, Pierre Robin syndrome, Gordon syndrome, hemivertebra and atlantal occipitalization, basilar impression, and iatrogenic causes., Results: All patients underwent occipital bone to cervical spine instrumentation and fixation at different levels. Constructs extended from the occiput to C2 and T1 utilizing various permutations of titanium rods, autologous rib autografts, Mersilene sutures, and combinations of autografts with bone matrix materials. All patients were placed in rigid cervical bracing or halo fixation postoperatively. No postoperative neurological deficits or intraoperative vascular injuries occurred., Conclusion: Instrumented arthrodesis can be a treatment option in very young children to address the non-traumatic craniocervical instability while reducing the need for prolonged external halo vest immobilization. Factors affecting fusion are addressed with respect to preoperative, intraoperative, and postoperative decision-making that may be unique to the toddler population.

Objective: Gross-total resection (GTR) of craniopharyngiomas (CPs) is potentially curative and is often the goal of surgery, but endocrinopathy generally results if the stalk is sacrificed. In some cases, GTR can be attempted while still preserving the stalk; however, stalk manipulation or devascularization may cause endocrinopathy and this strategy risks leaving behind small tumor remnants that can recur., Methods: A retrospective review of a prospective cohort of patients who underwent initial resection of CP using the endoscopic endonasal approach over a period of 12 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital, was performed. Postresection integrity of the stalk was retrospectively assessed using operative notes, videos, and postoperative MRI. Tumors were classified based on location into type I (sellar), type II (sellar-suprasellar), and type III (purely suprasellar). Pre- and postoperative endocrine function, tumor location, body mass index, rate of GTR, radiation therapy, and complications were reviewed., Results: A total of 54 patients who had undergone endoscopic endonasal procedures for first-time resection of CP were identified. The stalk was preserved in 33 (61%) and sacrificed in 21 (39%) patients. GTR was achieved in 24 patients (73%) with stalk preservation and 21 patients (100%) with stalk sacrifice (p = 0.007). Stalk-preservation surgery achieved GTR and maintained completely normal pituitary function in only 4 (12%) of 33 patients. Permanent postoperative diabetes insipidus was present in 16 patients (49%) with stalk preservation and in 20 patients (95%) following stalk sacrifice (p = 0.002). In the stalk-preservation group, rates of progression and radiation were higher with intentional subtotal resection or near-total resection compared to GTR (67% vs 0%, p < 0.001, and 100% vs 12.5%, p < 0.001, respectively). However, for the subgroup of patients in whom GTR was achieved, stalk preservation did not lead to significantly higher rates of recurrence (12.5%) compared with those in whom it was sacrificed (5%, p = 0.61), and stalk preservation prevented anterior pituitary insufficiency in 33% and diabetes insipidus in 50%., Conclusions: While the decision to preserve the stalk reduces the rate of postoperative endocrinopathy by roughly 50%, nevertheless significant dysfunction of the anterior and posterior pituitary often ensues. The decision to preserve the stalk does not guarantee preserved endocrine function and comes with a higher risk of progression and need for adjuvant therapy. Nevertheless, to reduce postoperative endocrinopathy attempts should be made to preserve the stalk if GTR can be achieved.

Basilar invagination is defined as abnormal upward and/or posterior displacement of the odontoid leading to ventral compression of the cervicomedullary junction. This condition leads to lower cranial neuropathies, sensorimotor deficits, and myelopathy. These symptoms can persist even after posterior decompression, which is an indication for ventral decompression. Transoral approaches to the upper cervical spine carry significant morbidity, limiting their utility. The endonasal approach to the upper cervical spine presents an alternative for patients with amenable anatomy. In this report, we present a case of a patient with type 1 Chiari malformation with persistent symptoms despite adequate posterior decompression through suboccipital craniectomy and C1 laminectomy. A retroflexed odontoid and dorsal clival bone lip contributed to persistent cervicomedullary compression. To address this, we performed a 2-stage procedure: an occiput-to-C4 fusion followed by endoscopic endonasal approach for dorsal clivusectomy, C1 anterior arch resection, and odontoidectomy. In the associated video, Supplemental Digital Content 1 (http://links.lww.com/CLINSPINE/A52), we demonstrate the step-by-step approach for this anterior approach including positioning, dissection through the nasopharyngeal fascia, identification of bony landmarks using an intraoperative CT scanner with 3-dimensional navigation guidance, and drilling/bony decompression of the dorsal clivus, C1, and C2. We also discuss key pearls, pitfalls, and preoperative/postoperative considerations critical to successful outcomes.

Objective: Epidermoid cysts (ECs) commonly extend to involve the ventral cisterns of the cranial base. When present, symptoms arise due to progressive mass effect on the brainstem and adjacent cranial nerves. Historically, a variety of open microsurgical approaches have been used for resection of ECs in this intricate region. In recent years, the endoscopic endonasal approach (EEA) has been proposed as an alternative corridor that avoids crossing the plane of the cranial nerves. To date, there is a paucity of data in the literature regarding the safety and efficacy of the EEA in the treatment of ECs of the ventral cranial base., Methods: The authors reviewed a prospectively acquired database of EEAs for resection of ECs over 8 years at Weill Cornell, NewYork-Presbyterian Hospital. All procedures were performed by the senior authors. Standardized clinical and radiological parameters were assessed before and after surgery. Statistical tests were used to determine the impact of previous surgery and tumor volume on extent of resection and recurrence as well as the method of closure on rate of CSF leak., Results: Between January 2009 and February 2017, 7 patients (4 males and 3 females; age range 16-70 years) underwent a total of 8 surgeries for EC resection utilizing the EEA. Transplanum and transclival extensions were performed in 3 and 5 patients, respectively. Methods of closure incorporated a gasket seal in 6 of 8 procedures and a nasoseptal flap in 7 of 8 procedures. Gross-total resection (GTR) was achieved in 43% of patients, and near-total resection (> 95%) was obtained in another 43%. Complications included diabetes insipidus (n = 2), postoperative CSF leak (n = 2), transient third cranial nerve palsy (n = 1), and epistaxis (n = 1). With a mean follow-up of 43.5 months, recurrence has been observed in 2 of 7 patients. In 1 case, reoperation for recurrence was required 71 months following the initial surgery. Use of the gasket-seal technique with nasoseptal flap coverage significantly correlated with the absence of postoperative CSF leakage (p = 0.018). GTR was achieved in 25% of the patients who had prior surgeries and in 50% of patients without previous resections. The mean volume of cysts in which GTR was achieved (4.3 ± 1.8 cm3) was smaller than that in which subtotal or near-total resection was achieved (12.2 ± 11 cm3, p = 0.134)., Conclusions: The EEA for resection of ECs of the ventral cranial base is a safe and effective operative strategy that avoids crossing the plane of the cranial nerves. In the authors' experience, gasket-seal closure with nasoseptal flap coverage has been associated with a decreased risk of postoperative CSF leakage.

Cindy Campbell, a bereaved parent who lost her son to a rare pediatric brain tumor, shares her experience and frustration over the lack of treatment options and minimal research funding in pediatric oncology. She invites Dr. Jeffrey P. Greenfield to reflect on the situation and share his professional experiences pertaining to advances in oncogenomics and pediatric brain tumors. They share a passion for making this technology available to all pediatric brain tumor patients in the future and using data to inform treatment protocols and improve outcomes., (© 2018 Campbell and Greenfield; Published by Cold Spring Harbor Laboratory Press.)

Background: High-risk and recurrent medulloblastoma (MB) is associated with significant mortality. The murine monoclonal antibody 3F8 targets the cell-surface disialoganglioside GD2 on MB. We tested the efficacy, toxicity, and dosimetry of compartmental radioimmunotherapy (cRIT) with intraventricular 131 I-labeled 3F8 in patients with MB on a phase II clinical trial., Methods: Patients with histopathologically confirmed high-risk or recurrent MB were eligible for cRIT. After determining adequate cerebrospinal fluid (CSF) flow, patients received 2 mCi (where Ci is Curie) 124 I-3F8 or 131 I-3F8 with nuclear imaging for dosimetry, followed by up to four therapeutic (10 mCi/dose) 131 I-3F8 injections. Dosimetry estimates were based on serial CSF and blood samplings over 48 hr plus region-of-interest analyses on serial imaging scans. Disease evaluation included pre- and posttherapy brain/spine magnetic resonance imaging approximately every 3 months for the first year after treatment, and every 6-12 months thereafter., Results: Forty-three patients received a total of 167 injections; 42 patients were evaluable for outcome. No treatment-related deaths occurred. Toxicities related to drug administration included acute bradycardia with somnolence, headache, fatigue, and CSF pleocytosis consistent with chemical meningitis and dystonic reaction. Total CSF absorbed dose was 1,453 cGy (where Gy is Gray; 350.0-2,784). Median overall survival from first dose of cRIT was 24.9 months (95% confidence interval [CI]:16.3-55.8). Patients treated in radiographic and cytologic remission were at a lower risk of death compared to patients with radiographically measurable disease (hazard ratio: 0.40, 95% CI: 0.18-0.88, P = 0.024)., Conclusions: cRIT with 131 I-3F8 is safe, has favorable dosimetry to CSF, and when added to salvage therapy using conventional modalities, may have clinical utility in maintaining remission in high-risk or recurrent MB., (© 2017 Wiley Periodicals, Inc.)

The combined petrosal approach is an essential technique for the skull base neurosurgeon. In this manuscript, the authors provide a brief history of the development of this approach, technical instruction with consideration of important landmarks, and a literature review of the broad range of clinical applications for this approach. The combined petrosal approach was performed bilaterally in 6 cadaveric injected specimens. The relationship of middle and posterior fossa dura, venous sinuses, cranial nerves (CNs), and the vascular anatomy were studied. Additionally, the authors performed a systematic review of the literature to elucidate the clinical applications, technical considerations, outcomes, and complications associated with the combined petrosal approach. Several critical details of the approach are outlined. The approach offers a magnified view of this region and inflicts minimal damage to the temporal bridging veins while entering the transverse sinus no more than 5mm anterior to Citelli's angle. The approach allows for visualization of CN IV as an important anatomical landmark, while preserving the venous integrity of posterior cavernous sinus. The literature review surveys 11 studies that provide a range of clinical applications for the approach. The authors conclude that the combined petrosal approach is one of the most versatile skull base approaches to the middle and posterior fossa, and it can be used to address complex pathologies of the petroclival region. Detailed knowledge of its technical nuances and applications is essential for any skull base surgeon., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Purpose: Diffuse intrinsic pontine glioma (DIPG) is universally fatal without proven therapy other than radiation therapy for palliation. Representative animal models will play an essential role in the preclinical stage of future therapy development. To address the shortage of representative models, we created a novel infiltrative brainstem glioma model in rats based on glioblastoma spheroids., Methods: Cells dissociated from glioblastoma spheroids grown from surgical specimens were implanted into the brainstem of NIH nude rats. Animals were serially assessed clinically and radiographically with magnetic resonance imaging (MRI). Tumors were further characterized using histology, immunohistochemistry, and cytogenetics., Results: Tumor generation was successful in all animals receiving glioblastoma spheroid cells. The rats survived 17-25 weeks before severe symptoms developed. The tumors showed as diffuse hyperintense lesions on T2-weighted images. Histologically, they demonstrated cellular heterogeneity, and infiltrative and invasive features, with cells engorging vascular structures. The tumors were shown to comprise immature human origin glial tumor cells, with human epidermal growth factor receptor (EGFR) gene amplification and gain., Conclusions: This study showed that cells from glioblastoma spheroids produced infiltrative gliomas in rat brainstem. The rat brainstem gliomas are radiographically and histologically accurate compared to DIPG. These tumors develop over several months that would allow sequential clinical and radiographic assessments of therapeutic interventions. This study demonstrated in principle the feasibility of developing patient-specific animal models based on putative cancer stem cells from biopsy or resection samples.

We review several newer modalities to monitor the brain in children with acute neurologic disease in the pediatric intensive care unit, such as partial brain tissue oxygen tension (PbtO2), jugular venous oxygen saturation (SjvO2), near infrared spectroscopy (NIRS), thermal diffusion measurement of cerebral blood flow, cerebral microdialysis, and EEG. We then discuss the informatics challenges to acquire, consolidate, analyze, and display the data. Acquisition includes multiple data types: discrete, waveform, and continuous. Consolidation requires device interoperability and time synchronization. Analysis could include pressure reactivity index and quantitative EEG. Displays should communicate the patient's current status, longitudinal and trend information, and critical alarms., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: This case report describes a transadnexal transorbital roof approach to the anterior cranial fossa to treat an epidural abscess in a patient who had previously undergone extensive craniofacial reconstruction secondary to fibrous dysplasia. A standard frontal craniotomy or even a supraciliary craniotomy would have been burdensome due to the positioning of the porous polyethylene implant as well as extensive scar tissue and adhesions from prior surgical procedures., Methods: We performed a retrospective review of the case of a 15-year-old male with an epidural abscess treated at a university hospital., Results: The patient tolerated the procedure well with no postoperative complications. Surgical operative time was approximately 1.5 h with minimal blood loss and the patient was discharged on postoperative day 2. Six weeks after antibiotic therapy (intravenous ampicillin/sulbactam, 2,000 mg q.i.d. for 6 days total), an MRI showed complete resolution of the epidural abscess. At that time the patient had no visual changes, extraocular muscle dysfunction, headaches, periorbital cellulitis, or further seizures and is on no antiepileptic therapy., Conclusion: Minimally invasive approaches to treat complex problems in certain circumstances can yield excellent results and limit morbidity encountered by more extensive procedures. In the future, more formal studies with a larger subset of patients will help determine what role these approaches will ultimately have., Level of Evidence V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Purpose: Lennox-Gastaut syndrome (LGS) is an epileptogenic disorder that arises in childhood and is typically characterized by multiple seizure types, slow spike-and-wave complexes on EEG and cognitive impairment. If medical treatment fails, patients can proceed to one of two palliative surgeries, vagus nerve stimulation (VNS) or corpus callosotomy (CC). Their relative seizure control rates in LGS have not been well studied. The purpose of this paper is to compare seizure reduction rates between VNS and CC in LGS using meta-analyses of published data., Methods: A systematic search of Pubmed, Ovidsp, and Cochrane was performed to find articles that met the following criteria: (1) prospective or retrospective study, (2) at least one patient diagnosed with Lennox-Gastaut syndrome, and (3) well-defined measure of seizure frequency reduction. Seizure reduction rates were divided into seizure subtypes, as well as total seizures, and categorized as 100%, >75%, and >50%. Patient groups were compared using chi-square tests for categorical variables and t-test for continuous measures. Pooled proportions with 95% confidence interval (95% CI) of seizure outcomes were estimated for total seizures and seizure subtypes using random effects methods., Results: 17 VNS and 9 CC studies met the criteria for inclusion. CC had a significantly better outcome than VNS for >50% atonic seizure reduction (80.0% [67.0-90.0%] vs. 54.1% [32.1-75.4%], p<0.05) and for >75% atonic seizure reduction (70.0% [48.05-87.0%] vs. 26.3% [5.8-54.7%], p<0.05). All other seizure types, as well as total number of seizures, showed no statistically significant difference between VNS and CC., Conclusions: CC may be more beneficial for LGS patients whose predominant disabling seizure type is atonic. For all other seizure types, VNS offers comparable rates to CC., (Copyright © 2012 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)