International Tuberculum Sellae Meningioma Study: Preoperative Grading Scale to Predict Outcomes and Propensity-Matched Outcomes by Endonasal Versus Transcranial Approach.

BACKGROUND AND OBJECTIVES: 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: 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.

International Tuberculum Sellae Meningioma Study: Surgical Outcomes and Management Trends.

BACKGROUND AND OBJECTIVES: Tuberculum sellae meningiomas (TSMs) can be resected through transcranial (TCA) or expanded endonasal approach (EEA). The objective of this study was to report TSM management trends and outcomes in a large multicenter cohort. METHODS: This is a 40-site retrospective study using standard statistical methods. RESULTS: In 947 cases, TCA was used 66.4% and EEA 33.6%. The median maximum diameter was 2.5 cm for TCA and 2.1 cm for EEA ( P < .0001). The median follow-up was 26 months. Gross total resection (GTR) was achieved in 70.2% and did not differ between EEA and TCA ( P = .5395). Vision was the same or better in 87.5%. Vision improved in 73.0% of EEA patients with preoperative visual deficits compared with 57.1% of TCA patients ( P < .0001). On multivariate analysis, a TCA (odds ratio [OR] 1.78, P = .0258) was associated with vision worsening, while GTR was protective (OR 0.37, P < .0001). GTR decreased with increased diameter (OR: 0.80 per cm, P = .0036) and preoperative visual deficits (OR 0.56, P = .0075). Mortality was 0.5%. Complications occurred in 23.9%. New unilateral or bilateral blindness occurred in 3.3% and 0.4%, respectively. The cerebrospinal fluid leak rate was 17.3% for EEA and 2.2% for TCA (OR 9.1, P < .0001). The recurrence rate was 10.9% (n = 103). Longer follow-up (OR 1.01 per month, P < .0001), World Health Organization II/III (OR 2.20, P = .0262), and GTR (OR: 0.33, P < .0001) were associated with recurrence. The recurrence rate after GTR was lower after EEA compared with TCA (OR 0.33, P = .0027). CONCLUSION: EEA for appropriately selected TSM may lead to better visual outcomes and decreased recurrence rates after GTR, but cerebrospinal fluid leak rates are high, and longer follow-up is needed. Tumors were smaller in the EEA group, and follow-up was shorter, reflecting selection, and observation bias. Nevertheless, EEA may be superior to TCA for appropriately selected TSM.

Clival Chordomas in the Endoscopic Endonasal Era: Comparison With Management With Open Skull Base Approaches.

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: 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.

Clival Chordomas in the Endoscopic Endonasal Era: Clinical Management, Outcomes, and Complications.

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: 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.

Intraoperative monitoring of facial corticobulbar motor evoked potentials: Methodological improvement and analysis of 100 patients.

OBJECTIVE: A) To describe an improved methodology for continuously monitoring the functional integrity of facial nerve by eliciting facial corticobulbar motor-evoked potentials (FCoMEP) and B) To establish the prognosis of facial nerve function based on changes in FCoMEP during skull base surgery. METHODS: Intraoperative monitoring of FCoMEP performed in 100 patients. Previously published methodology has been improved upon by a) doing preoperative mapping of the facial nerve, b) facilitating the corticobulbar tract (CBT) by continuous transcranial electrical stimulation (TES) at 2 Hz repetition rate, c) recording from multiple facial nerve innervated muscles, and d) eliciting blink reflex (BR). We analyzed changes in FCoMEP, comparing them with the clinical facial nerve outcome scored with the House-Brackman (HB) scale. RESULTS: The monitorability rate was 100%. Out of 100 patients, nine presented a new facial deficit after surgery. Eight of these showed significant changes in FCoMEP. In four patients FCoMEPs were lost; they presented a complete facial paralysis from which they did not recover. To discriminate the prognosis of patients, ROC analysis identified a cut-off at 65% for FCoMEPs amplitude decrease with a sensitivity of 89% and specificity of 99%. In four patients FCoMEP showed a decrease in amplitude greater than 65%, and they presented mild/moderate facial paresis that was transient. One patient did not present changes in FCoMEP but had a mild facial paresis from which the patient recovered. CONCLUSIONS: The improved methodology allows the maximum rate of monitorability and minimizes false positive and false negative results. This study shows that prognosis of facial nerve may be reliably established based on FCoMEP parameters. SIGNIFICANCE: We improved the previously described methodology for continuously monitoring the functional integrity of the facial nerve by increasing the monitorability rate, and we describe the impact of FCoMEP intraoperative management of facial nerve. This method may permit establishing the short-term and long-term prognosis of facial nerve function in skull base surgery.

Skull Base Aerosol Generating Cases Amidst the COVID-19 Pandemic: An Experience from the Epicenter.

Soon after the World Health Organization declared the severe acute respiratory syndrome coronavirus 2 a global health emergency on January 30, 2020, New York City was plagued by the virus and its health system and economy pushed to their limits. The majority of the limited neurosurgical data in relation to COVID-19 is anecdotal and the higher theoretical risk of transmission of the virus among skull base aerosol generating (SBAG) cases has not been investigated or discussed in a neurosurgical population. We discuss a series of 13 patients who underwent 15 SBAG surgical procedures during the peak of COVID-19 in our hospital system and the protocols use perioperatively for their procedures. Our data support that with proper preoperative testing, a well-delineated surgical algorithm, and appropriate personal protective equipment, emergent/urgent cases can be done safely in hospitals that are currently experiencing high volumes of COVID-19 cases as we did in March to May of 2020.

Volumetric growth rates of untreated cavernous sinus meningiomas.

OBJECTIVE: Meningiomas that arise primarily within the cavernous sinus are often believed to be more indolent in their growth pattern. Despite this perceived growth pattern, disabling symptoms can arise even with small tumors. While research has been done on cavernous sinus meningiomas (CSMs) and their treatment, very little is known about their natural growth rates. With a better understanding of the growth rate of CSM, patient treatment and guidance can be can optimized and individualized. The goal of this study was to determine volumetric growth rates of untreated CSMs. METHODS: Thirty-seven patients with 166 MR images obtained between May 2004 and September 2019 were reviewed, with a range of 2-13 MR images per patient (average of 4.5 MR images per patient). These scans were obtained over an average follow-up period of 45.9 months (median 33.8, range 2.8-136.9 months). All imaging prior to any intervention was included in this analysis. Volumetric measurements were performed and assessed over time. RESULTS: The estimated volumetric growth rate was 23.3% per year (95% CI 10.2%-38.0%, p < 0.001), which is equivalent to an estimated volume doubling time (VDT) of 3.3 years (95% CI 2.1-7.1 years). There was no significant relationship between growth rate and patient age (p = 0.09) or between growth rate and patient sex (p = 0.78). The median absolute growth rate was 41% with a range of -1% to 1793%. With a definition of "growth" as an increase of greater than 20% during the observed period, 65% of tumors demonstrated growth within their observation interval. Growth rates for each tumor were calculated and tumors were segmented based on growth rate. Of 37 patients, 22% (8) demonstrated no growth (< 5% annual growth, equivalent to a VDT > 13.9 years), 32% (12) were designated as slow growth (annual growth rate 5%-20%, VDT 3.5-13.9 years), 38% (14) were found to have medium growth (annual growth rate 20%-100%, VDT 0.7-3.5 years), and 8% were considered fast growing (annual growth rate > 100%, VDT < 0.7 years). CONCLUSIONS: This study evaluated CSM volumetric growth rates. A deeper understanding of the natural history of untreated CSMs allows for better counseling and management of patients.

GNA11 Mutation in an Intracranial Melanocytoma with Orbital Involvement and Nevus of Ota.

The prognostic value of mutations in G-protein genes GNAQ and GNA11 in patients with intracranial and orbital melanocytomas is unknown. The authors present a case of GNA11 mutation (GNA11Q209L) in a 32-year-old male suffering from a meningeal melanocytoma with orbital involvement and ipsilateral Nevus of Ota. The patient underwent gamma knife stereotactic radiosurgery without biopsy and later partial transcranial resection of the melanocytic tumor that was subject to immunohistochemical and molecular analysis. A 50-gene next-generation sequencing panel revealed a 626A>T mutation in the GNA11 gene. One year later, intracranial extension of the melanocytoma necessitated a ventriculoperitoneal shunt and immunotherapy. Future work is needed to determine how GNA11 mutations in melanocytomas influence prognosis and monitoring strategies.

Phase 0 Clinical Trial of Everolimus in Patients with Vestibular Schwannoma or Meningioma.

Inhibition of mTORC1 signaling has been shown to diminish growth of meningiomas and schwannomas in preclinical studies, and clinical data suggest that everolimus, an orally administered mTORC1 inhibitor, may slow tumor progression in a subset of patients with neurofibromatosis type 2 (NF2) with vestibular schwannoma. To assess the pharmacokinetics, pharmacodynamics, and potential mechanisms of treatment resistance, we performed a presurgical (phase 0) clinical trial of everolimus in patients undergoing elective surgery for vestibular schwannoma or meningiomas. Eligible patients with meningioma or vestibular schwannoma requiring tumor resection enrolled on study received everolimus 10 mg daily for 10 days immediately prior to surgery. Everolimus blood levels were determined immediately before and after surgery. Tumor samples were collected intraoperatively. Ten patients completed protocol therapy. Median pre- and postoperative blood levels of everolimus were found to be in a high therapeutic range (17.4 ng/mL and 9.4 ng/mL, respectively). Median tumor tissue drug concentration determined by mass spectrometry was 24.3 pg/mg (range, 9.2-169.2). We observed only partial inhibition of phospho-S6 in the treated tumors, indicating incomplete target inhibition compared with control tissues from untreated patients (P = 0.025). Everolimus led to incomplete inhibition of mTORC1 and downstream signaling. These data may explain the limited antitumor effect of everolimus observed in clinical studies for patients with NF2 and will inform the design of future preclinical and clinical studies targeting mTORC1 in meningiomas and schwannomas.

Orbital Rosai-Dorfman disease initially diagnosed as IgG4-related disease: a case report.

Inflammatory orbital lesions include a broad list of diagnoses, many of them with overlapping clinical and radiographic features. They often present a diagnostic conundrum, even to the most experienced orbital specialist, thus placing considerable weight on surgical biopsy and histopathological analysis. However, histopathological diagnosis is also inherently challenging due to the rarity of these lesions and the overlaps in histologic appearance among distinct disease entities. We herein present the case of an adolescent male with a subacutely progressive orbital mass that generated a significant diagnostic dilemma. Early orbital biopsy was consistent with a benign fibro-inflammatory lesion, but corticosteroid therapy was ineffective in halting disease progression. After an initial substantial surgical debulking, histopathological analysis revealed several key features consistent with IgG4-related disease (IgG4-RD), a systemic fibro-inflammatory process typically accompanied by multifocal tumor-like lesions. Surprisingly, within months, there was clear evidence of clinical and radiographic disease progression despite second-line rituximab treatment, prompting a second surgical debulking. This final specimen displayed distinctive features of Rosai-Dorfman disease (RDD), a systemic inflammatory disease characterized by uncontrolled histiocytic proliferation. Interestingly, certain features of this re-excision specimen were still reminiscent of IgG4-RD, which not only reflects the difficulty in differentiating RDD from IgG4-RD in select cases, but also illustrates that these diagnoses may exist along a spectrum that likely reflects a common underlying pathogenetic mechanism. This case emphasizes the importance of surgical biopsy or resection and histopathological analysis in diagnosing-and, ultimately, treating-rare, systemic inflammatory diseases involving the orbit, and, furthermore, highlights the shared histopathological features between RDD and IgG4-RD.

Intraosseous Petrous Apex Schwannoma: Case Report and Review of Literature.

BACKGROUND: Intraosseous petrous apex schwannomas are an exceedingly rare entity; little is known about their epidemiology, natural history, and postoperative outcomes. CASE DESCRIPTION: Here, we present the fourth known case of a primary intraosseous schwannoma of the petrous apex: a 68-year-old woman presenting with diplopia, facial numbness, progressive intermittent vertigo, tinnitus, diminished hearing, and ataxia. She underwent a transtemporal approach for subtotal resection of the tumor with subsequent stereotactic radiosurgery. CONCLUSIONS: Our 2-year follow-up demonstrates slow growth and success of multimodal management in the treatment of these tumors. We review the 3 prior reports of petrous apex schwannomas and identify unifying radiographic and clinical characteristics to aid future diagnostic considerations of lesions of the petrous apex.

The posterior nasoseptal flap: A novel technique for closure after endoscopic transsphenoidal resection of pituitary adenomas.

BACKGROUND: While effective for the repair of large skull base defects, the Hadad-Bassagasteguy nasoseptal flap increases operative time and can result in a several-week period of postoperative crusting during re-mucosalization of the denuded nasal septum. Endoscopic transsphenoidal surgery for pituitary adenoma resection is generally not associated with large dural defects and high-flow cerebrospinal fluid (CSF) leaks requiring extensive reconstruction. Here, we present the posterior nasoseptal flap as a novel technique for closure of skull defects following endoscopic resection of pituitary adenomas. This flap is raised in all surgeries during the transnasal exposure using septal mucoperiosteum that would otherwise be discarded during the posterior septectomy performed in binostril approaches. METHODS: We present a retrospective, consecutive case series of 43 patients undergoing endoscopic transsphenoidal resection of a pituitary adenoma followed by posterior nasoseptal flap placement and closure. Main outcome measures were extent of resection and postoperative CSF leak. RESULTS: The mean extent of resection was 97.16 ± 1.03%. Radiographic measurement showed flap length to be adequate. While a defect in the diaphragma sellae and CSF leak were identified in 21 patients during surgery, postoperative CSF leak occurred in only one patient. CONCLUSIONS: The posterior nasoseptal flap provides adequate coverage of the surgical defect and is nearly always successful in preventing postoperative CSF leak following endoscopic transsphenoidal resection of pituitary adenomas. The flap is raised from mucoperiosteum lining the posterior nasal septum, which is otherwise resected during posterior septectomy. Because the anterior septal cartilage is not denuded, raising such flaps avoids the postoperative morbidity associated with the larger Hadad-Bassagasteguy nasoseptal flap.

Intraoperative fat placement in surgically refractory parasellar neoplasms to facilitate stereotactic radiosurgery.

BACKGROUND: In this article, we report on the technique of placing fat in between a sellar or parasellar neoplasm and the optic chiasm to possibly protect the optic chiasm from sequelae of radiation. METHODS: A review was performed on three patients, each of whom had planned subtotal resection with fat placed near their optic chiasm to facilitate future radiosurgery. RESULTS: Follow-up on our three patients varied from 6 months to 3 years post-stereotactic radiosurgery. The fat remained stable and in place. The tumors either remained stable or reduced in size. No infections, postoperative marker dependent neurological complications or unusual symptoms were encountered. CONCLUSIONS: Placement of fat between a parasellar neoplasm and the optic chiasm appears to be a safe approach to help define the tumor chiasm space, helping to facilitate radiosurgery. Future experience is warranted to determine the efficacy of this technique.

Partial labyrinthectomy presigmoid transpetrosal resection of petroclival meningioma.

A petroclival meningioma represents a challenging tumor to resect in the cerebellopontine angle and ventral to the brain stem. Multiple cranial nerves and blood vessels may be intimately involved with the tumor. A partial labyrinthectomy presigmoid transpetrosal approach can facilitate resection while preserving hearing. This approach allows for a direct line of sight along the petrous bone while sealing the canals can preserve hearing. In this video operative manuscript, we demonstrate a step-by-step illustration of a partial labyrinthectomy presigmoid transpetrosal resection of a petroclival meningioma. This approach affords the best chance of hearing preservation and an opportunity for maximum tumor resection. The video can be found here: http://youtu.be/29I4KEXz1vY .

A novel sling technique for microvascular decompression of a rare anomalous vertebral artery causing cervical radiculopathy.

Cervical radiculopathy secondary to compression from congenital anomalous vertebral arteries (VAs) is a known entity. Patients present with a variety of symptoms ranging from upper-extremity numbness to true occipital neuralgia. Treatment options for extracranial tortuous VAs include conservative management or some form of surgical microvascular decompression (MVD). The authors report on a patient with a congenital anomalous VA loop causing cervical nerve root compression. Successful MVD was conducted with relief of the patient's symptoms. A novel sling technique was used for mobilization of the VA. To the authors' knowledge, this is the first MVD described utilizing this technique.

Contralateral mini-craniotomy for clipping of bilateral ophthalmic artery aneurysms using unilateral proximal carotid control and Sugita head frame.

OBJECTIVE: Conventional surgical treatment of bilateral ophthalmic aneurysms would require bilateral craniotomies and bilateral neck dissections for proximal control of the cervical internal carotid artery (ICA). We present a semiemergent case where bilateral ophthalmic artery aneurysms were clipped using a unilateral mini-pterional craniotomy and contralateral proximal cervical ICA control while employing the Sugita head frame. CLINICAL PRESENTATION: A 37-year-old female presented with progressive right retro-orbital headaches. MRI/A revealed a right carotid-ophthalmic aneurysm as well as a small aneurysm on the left carotid-ophthalmic segment. Given the young age and medial orientation of the right aneurysm, direct surgical clipping was planned. It was our thought that a contralateral approach would afford us the best chance to clip the right medially pointing aneurysm fully without optic nerve retraction while having proximal control via exposure of contralateral cervical ICA. METHODS: After gaining proximal ICA control from right neck dissection, the Sugita frame was rotated to allow for a left pterional craniotomy. The right medially pointing ophthalmic aneurysm was clipped without optic nerve retraction. After dissection of the distal dural ring and gaining proximal control, the left aneurysm was clipped. Postoperatively, the patient remained intact without any visual complaints, and both aneurysms were obliterated on angiography. CONCLUSION: Our case illustrates safety and control while clipping bilateral ophthalmic artery aneurysms via a unilateral mini-pterional approach and utility of the Sugita head frame.

Operative nuances of side-to-side in situ posterior inferior cerebellar artery-posterior inferior cerebellar artery bypass procedure.

BACKGROUND: An intracranial posterior circulation revascularization procedure in the form of a side-to-side in situ posterior inferior cerebellar artery (PICA)-PICA bypass operation was introduced in 1991. This elegant and apparently low-risk operation is performed infrequently. Thus, the operative nuances used in this procedure have not been well reported, limiting the scope of treatment modalities of vertebral artery-PICA aneurysms and vertebral dissections. OBJECTIVE: To repair an incidental right-sided PICA aneurysm noted in a 51-year-old woman in magnetic resonance imaging and subsequent angiography. METHODS: The patient underwent side-to-side in situ PICA-PICA bypass surgery. RESULTS: Immediate indocyanine green angiography suggested that the PICA distal to the aneurysms was filling in a retrograde fashion through the bypass. On the following day, the patient was taken for coil embolization of the aneurysm. However, angiography images revealed that the aneurysm was spontaneously thrombosed, the proximal PICA was patent, and the PICA distal to the aneurysms was filling in a retrograde fashion, as suspected in intraoperative indocyanine green angiography. No further treatments were done. The patient recovered fully. CONCLUSION: We describe in detail the preoperative evaluation, decision process, and operative techniques for a side-to-side in situ PICA-PICA bypass operation, which is a relatively safe and elegant posterior circulation bypass procedure.