Faculty Bibliography

BACKGROUND AND OBJECTIVES/OBJECTIVE:Tuberculum sellae meningiomas are resected via an expanded endonasal (EEA) or transcranial approach (TCA). Which approach provides superior outcomes is debated. The Magill-McDermott (M-M) grading scale evaluating tumor size, optic canal invasion, and arterial involvement remains to be validated for outcome prediction. The objective of this study was to validate the M-M scale for predicting visual outcome, extent of resection (EOR), and recurrence, and to use propensity matching by M-M scale to determine whether visual outcome, EOR, or recurrence differ between EEA and TCA. METHODS:Forty-site retrospective study of 947 patients undergoing tuberculum sellae meningiomas resection. Standard statistical methods and propensity matching were used. RESULTS:The M-M scale predicted visual worsening (odds ratio [OR]/point: 1.22, 95% CI: 1.02-1.46, P = .0271) and gross total resection (GTR) (OR/point: 0.71, 95% CI: 0.62-0.81, P < .0001), but not recurrence ( P = .4695). The scale was simplified and validated in an independent cohort for predicting visual worsening (OR/point: 2.34, 95% CI: 1.33-4.14, P = .0032) and GTR (OR/point: 0.73, 95% CI: 0.57-0.93, P = .0127), but not recurrence ( P = .2572). In propensity-matched samples, there was no difference in visual worsening ( P = .8757) or recurrence ( P = .5678) between TCA and EEA, but GTR was more likely with TCA (OR: 1.49, 95% CI: 1.02-2.18, P = .0409). Matched patients with preoperative visual deficits who had an EEA were more likely to have visual improvement than those undergoing TCA (72.9% vs 58.4%, P = .0010) with equal rates of visual worsening (EEA 8.0% vs TCA 8.6%, P = .8018). CONCLUSION/CONCLUSIONS:The refined M-M scale predicts visual worsening and EOR preoperatively. Preoperative visual deficits are more likely to improve after EEA; however, individual tumor features must be considered during nuanced approach selection by experienced neurosurgeons.

UNLABELLED:The superior hypophyseal arteries (SHAs) are well known in anatomical and surgical literature, with a well-established role in supply of the anterior hypophysis and superjacent optic apparatus. However, due to small size and overlap with other vessels, in vivo imaging by any modality has been essentially non-existent. Advances in high resolution cone beam CT angiography (CBCTA) now enables this deficiency to be addressed. This paper presents, to the best of our knowledge, the first comprehensive in vivo imaging evaluation of the SHAs. METHODS:Twenty-five CBCTA studies of common or internal carotid arteries were obtained for a variety of clinical reasons. Dedicated secondary reconstructions of the siphon were performed, recording the presence, number, and supply territory of SHAs. A spectrum approach, emphasizing balance with adjacent territories (inferior hypophyseal, ophthalmic, posterior and communicating region arteries) was investigated. RESULTS:The SHAs were present in all cases. Supply of the anterior pituitary was nearly universal (96%) and almost half (44%) originated from the 'cave' region, in excellent agreement with surgical literature. Optic apparatus supply was more difficult to adjudicate, but appeared present in most cases. The relationship with superior hypophyseal aneurysms was consistent. Patency following flow diverter placement was typical, despite a presumably rich collateral network. Embryologic implications with respect to the ophthalmic artery and infraoptic course of the anterior cerebral artery are intriguing. CONCLUSIONS:SHAs are consistently seen with CBCTA, allowing for correlation with existing anatomical and surgical literature, laying the groundwork for future in vivo investigation.

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE/UNASSIGNED:The extreme lateral approach is useful for both extradural and intradural anterior and anterolateral lesions at the lower clivus down to the level of C2. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT/UNASSIGNED:The patient is evaluated with MRI, computed tomography (CT), and an angiogram. Special attention is given to vascular (vertebral artery course, dominance, tumor feeders) and bony (occipital condyle, jugular tubercle, foramen magnum and extent of bony involvement) anatomy. ESSENTIALS STEPS OF THE PROCEDURE/UNASSIGNED:The patient is positioned lateral with the head flexed and tilted down without axial rotation. A hockey-stick incision is performed, and the myocutaneous flap is raised. A retrocondylar craniectomy is performed. The extradural vertebral artery is exposed for proximal control. A C1 hemilaminectomy is performed. Cephalad/caudal exposure and drilling of the occipital condyle are determined per case. The dura is opened, and the vertebral artery is released at the dural entry point to facilitate the tumor removal. The tumor is debulked and delivered inferoventrally away from the neuroaxis and cranial nerves. After removing the tumor, the dura is closed using an allograft.The patients consented to the procedure and to the publication of their images. PITFALLS/AVOIDANCE OF COMPLICATIONS/UNASSIGNED:• Cranial nerve deficits• Craniocervical instability• Postoperative hydrocephalus• Postoperative pseudomeningocele. VARIANTS AND INDICATIONS FOR THEIR USE/UNASSIGNED:A transmastoid extension of the craniectomy allows access further rostrally in the clivus. For C1-2 chordomas, the approach is extended inferiorly, and the vertebral artery is mobilized out of the C1-2 transverse foramina. For tumors involving the joints, an occipitocervical stabilization is required.Images in video reused with permission as follows: image at 00:16 from Revuelta Barbero et al, Endoscopic endonasal transclival-medial condylectomy approach for resection of a foramen magnum meningioma: 2-dimensional operative video, Oper Neurosurg, 16(2), 2018, by permission from the Congress of Neurological Surgery; images at 00:30, and top image at 00:52 reused from Wen et al, Microsurgical anatomy of the transcondylar, supracondylar, and paracondylar extensions of the far-lateral approach, J Neurosurg, 87(4), 1997, with permission from JNSPG; bottom images at 00:52 from Muthukumar et al, A morphometric analysis of the foramen magnum region as it relates to the transcondylar approach, Acta Neurochir, 147(8), 2005, by permission from Springer Nature.

BACKGROUND:Surgical management of skull base chordomas has changed significantly in the past 2 decades, most notably with use of the endoscopic endonasal approach (EEA), although high quality outcome data using these modern approaches remain scarce. OBJECTIVE:To evaluate outcomes in a large series of patients treated by a single surgeon, using primarily the EEA. METHODS:Between 2006 and 2020, 68 patients with skull base chordoma underwent resection using mostly the EEA. Complications, outcomes, and potential contributing factors were evaluated using Kaplan-Meier survival analysis and univariable and multivariable Cox proportional hazards models. RESULTS:Overall 5-year survival was 76.3% (95% CI 61.5%-86.0%), and 5-year progression-free survival was 55.9% (95% CI 40.0%-69.0%). In multivariable analysis, radical resection was associated with significant reduction in risk of death (hazard ratio [HR] 0.04, 95% CI 0.005-0.33, P = .003) and disease progression (HR 0.05, 95% CI 0.01-0.18, P < .001). Better preoperative function status reduced risk of death (HR 0.42 per 10-point increase in Karnofsky Performance Scale, 95% CI 0.28-0.63, P < .001) and progression (HR 0.60 per 10-point increase in Karnofsky Performance Scale, 95% CI 0.45-0.78, P < .001). Localization at the clivus reduced risk of death (HR 0.02, 95% CI 0.002-0.15, P < .001) and progression (HR 0.24, 95% CI 0.09-0.68, P = .007) compared with tumors at the craniovertebral junction. CONCLUSION/CONCLUSIONS:In multivariable analysis, overall survival and progression-free survival of chordoma resection was most positively affected by radical resection, better preoperative functional status, and tumor location at the clivus rather than craniovertebral junction.

BACKGROUND:The most significant paradigm shift in surgical management of skull base chordomas has been the adoption of the endoscopic endonasal approach, but the impact on patient outcomes compared with open skull base approaches remains unclear. OBJECTIVE:To compare a large series of patients treated by a single surgeon using primarily endoscopic endonasal approaches with previously published outcomes by the same surgeon using open skull base approaches. METHODS:Between 2006 and 2020, 68 patients with skull base chordoma underwent resection using primarily endoscopic endonasal approaches. Outcomes and complications were compared with previously published results of resection of chordomas from 1991 to 2005 using open skull base approaches. RESULTS:Compared with the prior cohort, the current principally endoscopic cohort demonstrated similar rates of OS (P = .86) and progression-free survival (P = .56), but patients undergoing first-time resection had significantly higher rates of radical resection (82.9% compared with 64.3%, P = .05) and required fewer staged surgeries (9.8% compared with 33.3%, P = .01). CONCLUSION/CONCLUSIONS:There was no difference in survival rates for patients treated in the current era, primarily using endoscopic endonasal techniques, compared with previously published results using open skull-base approaches by the same surgeon. Although use of endoscopic endonasal approach resulted in higher rates of radical resection, patients undergoing first-time resection and fewer staged surgeries were required.

Sphenoorbital meningiomas are a challenge to access and reconstruct. Although there is much neurosurgical literature on resection of such tumors, there is little discussion on the best methods for the reconstruction of consequent defects, which are often extensive due to large areas of hyperostosis requiring resection. We performed a retrospective analysis of patients who underwent resection and reconstruction of a sphenoorbital meningioma by the senior authors (C.S. and D.A.S.) between 2010 and 2020. Surgical access in all cases included an orbitozygomatic osteotomy. The study cohort consisted of 23 patients (20 female, 3 male) with an average age of 50 (range: 37-72) years at the time of surgery. Most patients had progressive proptosis before the ablative operation. Orbital reconstruction was with a combined titanium-Medpor implant in 18 patients, split calvarial bone graft in 3 patients, and a Medpor implant in 2 patients. Calvarial reconstruction was performed with titanium mesh in 21 patients, split calvarial bone graft and titanium mesh in 1 patient, and craniotomy bone and titanium plate in 1 patient. Reoperation was required in 7 patients due to hypoglobus or enophthalmos (N=2), orbital implant malposition (N=1), abscess (N=1), pain (N=1), intracranial fat graft modification (N=1), and soft tissue deformities (N=2). Our experience demonstrates that sphenoorbital meningiomas can require broad areas of resection of the skull base and calvarium and necessitate comprehensive reconstruction of the anterior cranial fossa, orbital walls, and cranium. Collaboration between craniofacial surgeons and neurosurgeons can achieve optimal results.

BACKGROUND/UNASSIGNED:Central nervous system (CNS) cancer is the 10th leading cause of cancer-associated deaths for adults, but the leading cause in pediatric patients and young adults. The variety and complexity of histologic subtypes can lead to diagnostic errors. DNA methylation is an epigenetic modification that provides a tumor type-specific signature that can be used for diagnosis. METHODS/UNASSIGNED:We performed a prospective study using DNA methylation analysis as a primary diagnostic method for 1921 brain tumors. All tumors received a pathology diagnosis and profiling by whole genome DNA methylation, followed by next-generation DNA and RNA sequencing. Results were stratified by concordance between DNA methylation and histopathology, establishing diagnostic utility. RESULTS/UNASSIGNED:Of the 1602 cases with a World Health Organization histologic diagnosis, DNA methylation identified a diagnostic mismatch in 225 cases (14%), 78 cases (5%) did not classify with any class, and in an additional 110 (7%) cases DNA methylation confirmed the diagnosis and provided prognostic information. Of 319 cases carrying 195 different descriptive histologic diagnoses, DNA methylation provided a definitive diagnosis in 273 (86%) cases, separated them into 55 methylation classes, and changed the grading in 58 (18%) cases. CONCLUSIONS/UNASSIGNED:DNA methylation analysis is a robust method to diagnose primary CNS tumors, improving diagnostic accuracy, decreasing diagnostic errors and inconclusive diagnoses, and providing prognostic subclassification. This study provides a framework for inclusion of DNA methylation profiling as a primary molecular diagnostic test into professional guidelines for CNS tumors. The benefits include increased diagnostic accuracy, improved patient management, and refinements in clinical trial design.