Characterization of Pediatric Infratentorial Arteriovenous Malformations: A Retrospective, Multicenter Cohort Study.

BACKGROUND AND OBJECTIVES: Infratentorial arteriovenous malformations (AVMs) harbor different characteristics compared with supratentorial AVMs. This study aims to explore the unique characteristics of pediatric infratentorial AVMs and their response to single session stereotactic radiosurgery (SRS). METHODS: The International Radiosurgery Research Foundation database of pediatric patients with AVM (age <18 years) who underwent SRS was retrospectively reviewed. Baseline demographics, AVM characteristics, outcomes, and complications post-SRS were compared between infratentorial and supratentorial pediatric AVMs. Unfavorable outcome was defined as the absence of AVM obliteration, post-SRS hemorrhage, or permanent radiation-induced changes at last follow-up. RESULTS: A total of 535 pediatric AVMs managed with SRS with a median follow-up of 67 months (IQR 29.0-130.6) were included, with 69 being infratentorial and 466 supratentorial. The infratentorial group had a higher proportion of deep location (58.4% vs 30.3%, P = <.001), deep venous drainage (79.8% vs 61.8%, P = .004), and prior embolization (26.1% vs 15.7%, P = .032). There was a higher proportion of hemorrhagic presentation in the infratentorial group (79.7% vs 71.3%, P = .146). There was no statistically significant difference in the odds of an unfavorable outcome (odds ratio [OR] = 1.36 [0.82-2.28]), AVM obliteration (OR = 0.85 [0.5-1.43]), post-SRS hemorrhage (OR = 0.83 [0.31-2.18]), or radiologic radiation-induced changes (OR = 1.08 [0.63-1.84]) between both cohorts. No statistically significant difference on the rates of outcomes of interest and complications were found in the adjusted model. CONCLUSION: Despite baseline differences between infratentorial and supratentorial pediatric AVMs, SRS outcomes, including AVM obliteration and post-SRS hemorrhage rates, were comparable amongst both groups. SRS appears to have a similar risk profile and therapeutic benefit to infratentorial pediatric AVMs as it does for those with a supratentorial location.

The Promise, Mystery, and Perils of Stenting for Symptomatic Internal Jugular Vein Stenosis: A Case Series.

BACKGROUND AND OBJECTIVES: Cerebral venous outflow disorders (CVDs) secondary to internal jugular vein (IJV) stenosis are becoming an increasingly recognized cause of significant cognitive and functional impairment in patients. There are little published data on IJV stenting for this condition. This study aims to report on procedural success. METHODS: A single-center retrospective analysis was performed on patients with CVD that underwent IJV stenting procedures. RESULTS: From 2019 to 2023, 29 patients with CVD underwent a total of 33 IJV stenting procedures. Most patients (20; 69%) had an underlying connective tissue disorder diagnosis. The mean age of the included patients was 36.3 years (SD 12.4), 24 were female (82.8%), and all were Caucasian except for 2 patients (27; 93.0%). Twenty-eight procedures (85%) involved isolated IJV stenting under conscious sedation, whereas 5 procedures (15%) involved IJV stenting and concomitant transverse sinus stenting under general anesthesia. Thirteen (39%) patients underwent IJV stenting after open IJV decompression and styloidectomy. Three patients had stents placed for stenosis below the C1 tubercle, one of which was for carotid compression. Periprocedural complications occurred in 11 (33%), including intracardiac stent migration in 1 patient, temporary shoulder pain/weakness in 5 (15%), and persistent and severe shoulder pain/weakness in 2 patients (6%). Approximately 75% of patients demonstrated improvement after stenting although only 12 patients (36%) had durable improvement over a mean follow-up of 4.5 months (range 6 weeks-3.5 years). CONCLUSION: Our experience, along with early published studies, suggests that there is significant promise to IJV revascularization techniques in these patients; however, stenting carries a high complication rate, and symptom recurrence is common. Most neurointerventionalists should not be performing IJV stenting unless they have experience with these patients and understand technical nuances (stent sizing, anatomy, patient selection), which can maximize benefit and minimize risk.

Surgical outcomes in large vestibular schwannomas: should cerebellopontine edema be considered in the grading systems?

PURPOSE: Large (> 3 cm) vestibular schwannomas pose complexity in surgical management because of narrow working corridors and proximity to the cranial nerves, brainstem, and inner ear structures. With current vestibular schwannoma classifications limited in information regarding cerebellopontine edema, our retrospective series examined this radiographic feature relative to clinical outcomes and its possible role in preoperative scoring. METHODS: Of 230 patients who underwent surgical resection of vestibular schwannoma (2014-2020), we identified 107 patients with Koos grades 3 or 4 tumors for radiographic assessment of edema in the middle cerebellar peduncle (MCP), brainstem, or both. Radiographic images were graded and patients grouped into Koos grades 3 or 4 or our proposed grade 5 with edema. Tumor volumes, radiographic features, clinical presentations, and clinical outcomes were evaluated. RESULTS: The 107 patients included 22 patients with grade 3 tumors, 39 with grade 4, and 46 with grade 5. No statistical differences were noted among groups for demographic data or complication rates. Unlike grades 3 and 4 patients, grade 5 patients presented with worse hearing (p < 0.001), larger tumors (p < 0.001), lower rates of gross total resection (GTR), longer hospital stays, and higher rates of balance dysfunction. CONCLUSION: With edema detected in 43% of this cohort, special considerations are warranted for grade 5 vestibular schwannomas given the preoperative findings of worse hearing, lower GTR rates, longer hospital stays, and 96% who pursued postoperative balance therapy. We propose that grade 5 with edema offers a more nuanced interpretation of a radiographic feature that holds relevance to treatment selection and patient outcomes.

Fusion of Lateral Calvarial Sutures on Volume-Rendered Computed Tomography Reconstructions in Patients With Known Craniosynostosis.

INTRODUCTION: After treating a child with familial sagittal craniosynostosis, clinocephaly, and bilateral parietomastoid/posterior squamosal suture fusion, the authors wondered if major-suture synostosis and clinocephaly were associated with abnormal fusion of minor lateral calvarial sutures. METHODS: The authors reviewed all preoperative volume-rendered head computed tomography reconstructions performed for craniosynostosis at their institution from 2010 through 2014 and determined whether the sphenoparietal, squamosal, and parietomastoid sutures were open, partially fused, or fused. The authors determined whether any sutures were abnormally fused based upon a previous study from their center, in which abnormal fusion was defined as either 1 of 3 abnormal fusion patterns or abnormally-early fusion. The authors then determined the rate of abnormal fusion of these sutures and whether abnormal fusion was associated with (1) major-suture craniosynostosis, (2) type of craniosynostosis (sutures involved; single-suture versus multisuture; syndromic versus nonsyndromic), and (3) clinocephaly. RESULTS: In 97 included children, minor lateral sutures were abnormally fused in 8, or 8.2%, which was significantly higher than in children without craniosynostosis from our earlier study. Abnormal minor lateral suture fusion was not associated with the type of single-suture synostosis or with multisuture synostosis but was associated with syndromic synostosis. Four of 8 children with abnormal minor lateral suture fusion had multisuture synostosis and 6 had syndromic synostosis. Lateral sutures were abnormally fused in 1 of 4 subjects with clinocephaly, which was not significant. CONCLUSION: Abnormal minor lateral calvarial suture fusion is significantly associated with major-suture craniosynostosis, especially syndromic synostosis.

Spinal cord tethering after selective dorsal rhizotomy below the conus medullaris.

BACKGROUND: Two techniques for selective dorsal rhizotomy (SDR) involve stimulating and sectioning nerve rootlets either below the conus medullaris or above the entrances to their respective dural root sleeves. In general, both techniques lead to sustained improvements in lower extremity spasticity with low complication rates. To our knowledge, spinal cord tethering has not been previously reported as a complication of SDR using either technique. METHODS: We review the presentation, treatment, and outcome of two patients who developed symptomatic spinal cord tethering after SDR below the conus. RESULTS: A 4-year-old male and a 6-year-old female each with a history of spastic diparetic cerebral palsy underwent L1-L2 osteoplastic laminectomy and SDR below the conus. Both surgeries went well with no known complications. Both patients initially did well, with marked improvement in their tone and gait. There were no significant immediate postoperative bowel or bladder problems. The male patient presented 10 months after surgery with new-onset urinary incontinence. A urological evaluation was performed but no imaging was performed. He re-presented 8 months later with worsened urinary incontinence, new fecal incontinence, and worsened gait. An MRI showed signs of tethering, including a holocord syrinx. He underwent two untethering surgeries as well syrinx fenestration, and although his gait improved, it remained worse than it had been several months after surgery. The incontinence did not improve. The second patient also developed urinary incontinence a year after SDR. Due to our experience with the first patient, we obtained an MRI immediately and found evidence of tethering. She underwent untethering and her incontinence improved. CONCLUSION: We report two cases of spinal cord tethering after SDR below the conus, a previously unreported complication. It is important to realize that this complication can occur after SDR.

Concomitant Temozolomide plus radiotherapy for high-grade and recurrent meningioma: a retrospective chart review.

BACKGROUND: High-grade and recurrent meningiomas are often treatment resistant and pose a therapeutic challenge after surgical and radiation therapy (RT) failure. Temozolomide (TMZ) is a DNA alkylating agent that appears to have a radiosensitizing effect when used in combination with RT and may be worthwhile in meningioma treatment. Thus, we investigated the potential efficacy of concomitant RT plus TMZ compared to historical controls of just RT used in the treatment of high-grade and recurrent meningiomas. METHODS: We performed a retrospective analysis of patients with meningioma treated at the University of Colorado with TMZ chemoradiation. Progression free survival (PFS) and overall survival (OS) were calculated from the start of chemoradiation to local recurrence or death, respectively. RESULTS: Eleven patients (12 tumors) were treated with chemoradiation with a median follow-up of 41.5 months. There were two WHO grade 1, eight grade 2 and two grade 3 meningiomas. Three patients died during the follow-up period-one being disease related (11.1%). Two patients had meningioma recurrence-at 2.3 months (WHO grade 3), and 5.4 years (WHO grade 2). Three-year OS and PFS for grade 2 meningiomas were each 88%. Historical controls demonstrate a 3-year median OS and PFS of 83% and 75.8%, respectively. CONCLUSIONS: Treatment options are limited for meningiomas after local failure. In this study, TMZ chemoradiation demonstrated no significant difference in PFS and OS in the treatment of grade 2 meningiomas compared to historic controls. Further study is warranted to find novel methods for the treatment of malignant and recurrent meningiomas.

Microenvironment changes in arteriovenous malformations after stereotactic radiation.

Cerebral arteriovenous malformations are dysplastic vascular tangles with aberrant vascular dynamics and can result significant morbidity and mortality. A myriad of challenges are encountered when treating these lesions and are largely based on nidal size, location, and prior hemorrhage. Currently, stereotactic radiosurgery is an accepted form of treatment for small to medium sized lesions and is especially useful in the treatment of lesions in non-surgically assessable eloquent areas of the brain. Despite overall high rates of nidal obliteration, there is relatively limited understand on the mechanisms that drive the inflammatory and obliterative pathways observed after treatment with stereotactic radiosurgery. This review provides an overview of arteriovenous malformations with respect to stereotactic radiosurgery and the current understanding of the mechanisms that lead to nidal obliteration.

Management of post-operative delirium following stereoelectroencephalography electrode placement for drug resistant epilepsy: Lessons learned from two case reports.

Post-operative delirium (POD) represents a unique challenge in the care of any surgical patient but is especially challenging in neurosurgical inpatient management due to a host of potentially significant predisposing factors. Patients undergoing stereoencephalography (SEEG) for diagnosis of drug resistant epilepsy are at unique risk due to safety concerns, yet POD has been underdiscussed in this population. Patients should be counseled pre-operatively about their risk and subsequent steps be taken post-operatively. We present two cases of POD status-post SEEG and propose a mechanism by which future post-operative care be coordinated by the physician, patient, and patient's family.