Citations

OBJECTIVE The authors’ goal was to use a multicenter, observational cohort study to determine whether supramarginal resection (SMR) of FLAIR-hyperintense tumor beyond the contrast-enhanced (CE) area influences the overall survival (OS) of patients with isocitrate dehydrogenase–wild-type (IDH-wt) glioblastoma after gross-total resection (GTR). METHODS The medical records of 888 patients aged ≥ 18 years who underwent resection of GBM between January 2011 and December 2017 were reviewed. Volumetric measurements of the CE tumor and surrounding FLAIR-hyperintense tumor were performed, clinical variables were obtained, and associations with OS were analyzed. RESULTS In total, 101 patients with newly diagnosed IDH-wt GBM who underwent GTR of the CE tumor met the inclusion criteria. In multivariate analysis, age ≥ 65 years (HR 1.97; 95% CI 1.01–2.56; p < 0.001) and contact with the lateral ventricles (HR 1.59; 95% CI 1.13–1.78; p = 0.025) were associated with shorter OS, but preoperative Karnofsky Performance Status ≥ 70 (HR 0.47; 95% CI 0.27–0.89; p = 0.006), MGMT promotor methylation (HR 0.63; 95% CI 0.52–0.99; p = 0.044), and increased percentage of SMR (HR 0.99; 95% CI 0.98–0.99; p = 0.02) were associated with longer OS. Finally, 20% SMR was the minimum percentage associated with beneficial OS (HR 0.56; 95% CI 0.35–0.89; p = 0.01), but > 60% SMR had no significant influence (HR 0.74; 95% CI 0.45–1.21; p = 0.234). CONCLUSIONS SMR is associated with improved OS in patients with IDH-wt GBM who undergo GTR of CE tumor. At least 20% SMR of the CE tumor was associated with beneficial OS, but greater than 60% SMR had no significant influence on OS.

Abstract The optic canal (OC) is a bony channel that transmits the optic nerve (ON) and ophthalmic artery (OphA) as they course through the lesser wing of the sphenoid bone to the orbital apex. The OC is involved in a variety of intracranial and extracranial pathologies, 1 and opening of the canal may be necessary in order to achieve adequate exposure, better disease control, and vision preservation. 2 Depending on the location of the pathology and its relationship with the optic nerve, the OC may be decompressed through an open transcranial approach or an endoscopic endonasal approach. 1,3 OC drilling can be tailored based on the location of the pathology and its extension. Anterior clinoid process and optic strut drilling can be added based on these factors as well. 4,5 In this video, we demonstrate the steps of OC drilling in both transcranial microscopic and endoscopic endonasal approaches through a combination of animated illustrations and operative videos. We present 4 cases, including 2 transcranial microscopic and 2 endoscopic endonasal approaches, 6 demonstrating OC decompression and its technical nuances. Each case was selected to represent the range of pathologies relevant to OC drilling to allow for a complete understanding of the techniques and concepts required for optimal treatment. An informed written consent has been obtained from each of the patients in this publication. Video © Mayo Foundation for Medical Education and Research. All rights reserved. Images at 1:03-1:10 © The Rhoton Collection. Images at 3:56-4:07 Reprinted from World Neurosurgery , vol 124, Caporlingua et al, 3 Copyright 2019, with permission from Elsevier. Images at 9:02-9:48 reprinted from Bendok et al, 6 by permission of the Congress of Neurological Surgeons.

Background: Disorders of consciousness (DoC) includes coma, vegetative state (VS), minimally conscious state (MCS), and emergence from the MCS. Aneurysmal rupture with high-grade SAH, traumatic brain injury, and neoplastic brain lesions are some of the frequent pathologies leading to DoC. The diagnostic errors among these DoC are as high as ranging from 25% to 45%, with a probable error in the conclusion of patients’ state, treatment choice, end-of-life decision-making, and prognosis. Some studies also reported that 37–43% of patients were misdiagnosed in VS while demonstrating signs of awareness. Despite its wide acceptance, Coma Recovery Scale-Revised (CRS-r) remained underused or inappropriately utilized, which may lead to substandard or unprofessional patient care. Literature is rare on the knowledge of CRS-r among physicians published from India and across the globe. Therefore, we carried out the present study to ascertain physicians’ knowledge on CRS-r and raise awareness about its justifiable clinical utilization. We also explored the factors associated with this perceived level of experience among participants and recommend frequent physicians’ training for care of patients with DoC. Methods: An institution-based cross-sectional online survey was conducted from June 8 to July 7, 2020, among Ninety-six physicians recruited using a convenient sampling technique. Twenty-item, validated, reliable, and a pilot-tested questionnaire was used to assess the knowledge regarding CRS-r and collect socio-demographic variables. The analysis was performed using the Statistical Package for the Social Sciences version 23. Bivariate and multivariate logistic regression analyses were employed to assess the association of participants’ socio-demographic variables and their parent department of work with the knowledge. P < 0.05 was considered statistically significant in the multivariate analysis. Results: A total of Ninety-six participants were included in the analysis, and only 33.3% of them were found to have adequate knowledge of CRS-r. Multivariate analysis revealed that age (adjusted odds ratio [AOR] = 31.66; 95% CI: 6.25–160.36), gender (AOR = 44.16; 95% CI: 7.43–268.23), and parent department of working (AOR = 0.148; 95% CI: 0.06–0.39) were significantly associated with the knowledge. Conclusion: Knowledge of the physicians on CRS-r is found to be exceptionally low. It has a strong tendency to adversely affect patients’ optimal care with disorders of consciousness (DoC). Therefore, it is crucial to expand physicians’ knowledge and awareness regarding CRS-r to adequately screen patients with DoC.

Abstract Adolescent idiopathic scoliosis (AIS) is an abnormal lateral curvature of the spine that arises during the pubescent growth spurt. AIS mainly affects females in the age group of 10 to 16 yr, with a prevalence of about 1% to 3% in the at-risk population. 1 Treatment options vary depending on disease presentation and severity. Mild curvature mainly requires periodic observation for disease progression, whereas more moderate curvature can necessitate bracing or corrective surgery. 2 Here, we present the use of a temporary rod and neuroimaging for the correction of Lenke type 1 spinal curvature in an AIS patient. An inferior facetectomy is performed, and a Lenke probe is used for entry into the pedicle and vertebral body. The channel is sounded before and after tapping to check for adequate walls prior to insertion of the pedicle screw. The ARTIS pheno is brought into the field, which uses fluoroscopy to create a 3-dimensional (3D) representation of the instrumentation within the spine. A temporary rod is placed in the concavity, and a combination of corrective techniques, including a rod roll, apical translation, and reduction, is performed to bring up the concavity, derotate the spine, and translate the spine. The permanent rod is then placed in the convexity, and compression is performed to bring down the scapular prominence. The temporary rod is then removed, and a final rod is placed to complete the 3D correction. The patient consented to the procedure, and an informed written consent was obtained from the patient to use her photographs and video recordings for publication.

Abstract OBJECTIVE Glioblastoma (GBM) is the most common malignant primary brain tumor in adults, with males more commonly affected than females(1.6:1). Despite advancements in treatments, prognosis is dismal with a median overall survival of 15 months. Our aim was to investigate sex as a variable in GBM patient survival after receiving incremental levels of standard-of-care treatment regimens – different extents of surgical resection and different numbers of cycles of adjuvant temozolomide chemotherapy. METHODS Drawing from our extensive multi-institutional brain tumor repository, we investigated GBM subjects with overall survival (OS), extent of resection (EOR), number of temozolomide (TMZ) cycles, and sex data (n=620, males: n=387, females: n=233). Cox proportional hazard ratios were computed to investigate the multivariable predictive value of the patient variables with OS. Patients were then divided into groups based on their sex, EOR (either biopsy, subtotal resection (STR) or gross total resection (GTR)), and TMZ cycles (I: &lt; 6 cycles, II: 7-11 cycles and III: &gt;12 cycles). RESULTS We observed that STR was beneficial for females (HR=0.52; CI=0.33-0.83; p-value=0.013), while for males the benefit was not detected (HR=0.73; CI=0.46-1.15; p-value=0.173) for STR but was detectable for GTR (HR=0.58, CI=0.37-0.90; p-value=0.014). Females receiving 7-11 cycles of TMZ showed a survival benefit (HR=0.52; CI=0.12-0.53; p-value=0.048) while males in the same group did not (HR=0.74; CI=0.46-1.19; p-value=0.21), in comparison to those in group I of TMZ cycles. No sex differences were identified in patients receiving &lt; =6 cycles or &gt;=12 cycles. CONCLUSION Together, our results contribute to the growing literature that sex differences exist in GBM patients, even in response to standard-of-care therapies. This should be accounted for when designing clinical trials for GBM so that we may advance our pursuit to deliver personalized medicine.

Abstract INTRODUCTION Leptomeningeal disease (LMD) is an aggressive, late-stage complication of systemic cancers that maintains a poor prognosis with an average survival of 3-6 months. Ommaya reservoirs (OR) are an accessible alternative to serial lumbar punctures for the delivery of intrathecal chemotherapy in the treatment of LMD. However, widespread use of OR has been limited by reports of complications, limited physicians trained in their use, and the stigma of brain surgery. Here, we assess survival and complication rates for patients who received intrathecal chemotherapy through OR for LMD treatment. METHODS After IRB approval, chart review of patients who received at least one administration of intrathecal chemotherapy through OR for LMD treatment between 2017- 2020 at Mayo Clinic in Phoenix, Arizona was conducted. Charts were reviewed for demographics, treatment type, complications, and outcomes. RESULTS A total of 10 patients were included. The median overall survival from date of first OR injection was 110.5 days (95% CI: 64 – not estimable). The median time to progression from the first injection was 55.5 days (95% CI: 45 – 753). A total of 82 OR injections were conducted for an average of approximately 8 injections per patient. Two patients (20%) experienced mild adverse events – grade 2 or lower by Common Terminology Criteria for Adverse Events with a single injection. The majority of injections involved no adverse events with the overall risk of any complication at 2.4%. None of the patients experienced infection, a commonly reported complication of OR use. CONCLUSIONS Our results support the inclusion of intrathecal chemotherapy via OR into the treatment paradigm in patients with leptomeningeal malignancies. This approach is feasible, safe, and median survival is comparable with data reported in published series. The overall complication rate in patients receiving intrathecal chemotherapy through OR is very low including the risk of infection.

AbstractBackgroundDual diagnoses in genetics practice are not uncommon and patients with dual diagnosis often present with complex and challenging phenotypes. A combination of meticulous phenotyping and molecular genetic techniques are essential in solving these diagnostic odysseys.MethodsClinical features and genetic workup of a patient presenting with incidental schwannomatosis.ResultsA 19‐year‐old male presented with incidental painless schwannomatosis in the background of macrocephaly, distinctive facies, and learning disability. Comprehensive genetic testing with gene panel and chromosomal microarray led to a dual diagnosis of LZTR1‐related schwannomatosis and 7q11.23 duplication syndrome.ConclusionWe emphasize the need for high index of suspicion and comprehensive genetic testing in complex phenotypes. Interrogation of the interplay between the pathogenic variants in multiple genes could improve our understanding of the pathophysiologic pathways and contribute to therapeutic discoveries.

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.