Citations

AbstractHemifacial spasm is a neuromuscular disorder caused by compression of the facial nerve at the nerve root entry zone, often due to ectatic or aberrant vasculature. Pathologic compression of the nerve–brainstem interface results in involuntary, paroxysmal contractions of ipsilateral facial muscles that may cause considerable impairments in quality of life. For those with severe symptoms, have positive imaging demonstrating vascular compression, or who fail other management modalities,1 2 3 4 microvascular decompression offers potential definitive treatment.5 6 Traditionally, nonabsorbable packing agent is used to pack between the nerve and offending vascular structure. However, for large and more complex arterial structures, simple nonabsorbable padding is often not sufficient.In this operative video, we demonstrate microvascular decompression for intractable hemifacial spasm in a 52-year-old female using a specialized sling tacked to the petrous dura for management of a large, ectatic vertebral artery. Following a standard left retrosigmoid craniotomy, an atheromatous ectatic vertebral artery was identified. We fashioned a bovine pericardium sling around the vessel and used a permanent aneurysm clip to secure it to an incision portion of petrous dura. We subsequently identified potential additional facial nerve root compression by anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA) branches, which were elevated and secured using Teflon felt packing. Following elevation of all three vessels, the lateral spread response resolved. At 2 weeks postoperatively, the patient reported substantial relief in her hemifacial spasms and endorsed highly improved quality of life.

Objective The treatment paradigm of vestibular schwannoma (VS) focuses on preservation of neurologic function, with small tumors increasingly managed with active surveillance. Often, tumor size and hearing outcomes are poorly correlated. The aim of the current work was to describe the natural history of hearing among patients with nongrowing VS during observational management. Study Design Historical cohort study. Patients Adults with sporadic VS. Intervention Wait-and-scan management. Main Outcome Measure Maintenance of serviceable hearing (SH) after diagnosis. Results Among 228 patients with nongrowing VS, 157 patients had SH at diagnosis. Rates of maintaining SH (95% CI; number still at risk) at 1, 3, and 5 years after diagnosis were 94% (89–98; 118), 81% (74–89; 65), and 78% (71–87; 42), respectively. Poorer hearing at diagnosis (hazard ratio [HR] per 10 dB hearing level increase in pure-tone average of 2.51, p < 0.001; HR per 10% decrease in word recognition score of 1.70, p = 0.001) was associated with increased likelihood of developing non-SH during observation. When controlling for baseline hearing status, tumors measuring 5 mm or greater in the internal auditory canal or with cerebellopontine angle extension were associated with significantly increased risk of developing non-SH (HR, 4.87; p = 0.03). At 5 years after diagnosis, 95% of patients with nongrowing intracanalicular VS measuring less than 5 mm maintained SH. Conclusions Hearing worsens during periods of nongrowth in sporadic VS. Patients with small (<5 mm) intracanalicular tumors demonstrate robust maintenance of SH over time, reinforcing the consideration of initial observation in this patient subset.

Abstract OBJECTIVE This study aimed to analyze the hemorrhagic risk of melanoma brain metastases following SRS. METHODS A retrospective analysis was conducted using a prospective institutional database between 1990 and 2021. Lesional hemorrhage was categorized as definite or possible, and hemorrhage severity was graded using CTCAE. RESULTS The study identified 291 patients with 1083 lesions. The mean age of patients was 60 ± 15 years, with 61% being male. Median follow-up was 11 months, corresponding to 581 patient-years. Among the patients, 82 (28%) experienced 135 lesional hemorrhages (13%). Of these, 88 lesions in 61 patients were grade 1-2 hemorrhages, 42 lesions in 25 patients were grade 3, and five lesions in five patients progressed from grade 1 to 3. Surgical intervention was required for 17 lesions (2%) in 16 patients (5%). Lesional hemorrhage was associated with radiosurgery performed between 2015 and 2021 (OR, 0.373; 95% CI, 0.231-0.60; p &lt; 0.001), increasing marginal dose (OR, 0.89; 95% CI, 0.82-0.98; p = 0.017), antiplatelet use (OR, 0.192; 95% CI, 0.082-0.45; p &lt; 0.001), and WBRT (OR, 0.51; 95% CI, 0.330-0.77; p = 0.0017). The cumulative lesional hemorrhage risk reached 17–20% at 36 months following radiosurgery, with 95-96% of hemorrhages occurring within the initial year. Median OS was 11 months (95% CI, 9-13 months). Patients who received radiosurgery between 2015 and 2021 exhibited a median OS of 17 months (95% CI, 10-24 months; p &lt; 0.001), while those without hemorrhage had a median OS of 12 months (95% CI, 10-14 months; p = 0.054). CONCLUSIONS Definitive hemorrhage risk was 17% for melanoma brain metastases after SRS within the first three years, with 95% of lesional hemorrhages occurring within the initial year. Surgical intervention was necessary in 5% of patients. Hemorrhage risk and overall survival improved after 2015.

OBJECTIVE Arginine vasopressin deficiency (AVD) following neurosurgical procedures for pituitary disorders is common and can delay discharge. Copeptin, a stable surrogate marker of arginine vasopressin, may predict postoperative AVD. The authors’ aim was to assess the optimal postoperative sampling time and cut-point concentration of copeptin to predict the development of postsurgical AVD. METHODS Adults without preexisting AVD who were undergoing surgery for a pituitary lesion between February 2020 and April 2022 were eligible for study inclusion. Two samples were drawn from each patient postoperatively to assess the copeptin concentration using an immunofluorescent assay. Samples were denoted as "early" (within 6 hours of extubation) or "postoperative day 1" (POD1; within 10–30 hours of extubation). Patients were evaluated for the development of AVD. RESULTS One hundred ninety-two patients (54.2% female) with a median age of 54.5 years (IQR 39.8–67.0 years) were included in the study. The median copeptin concentration at both time points was significantly lower in those with AVD (transient or permanent; n = 22, 11.5%) than in those without (early: 4.9 vs 18.7 pmol/L, p < 0.001; POD1: 3.4 vs 4.9 pmol/L, p < 0.001) but did not differ in those who developed transient versus permanent AVD. The optimal copeptin cut point for the prediction of AVD was < 8.5 pmol/L for early samples (sensitivity 0.70, specificity 0.80, positive predictive value [PPV] 0.29, negative predictive value [NPV] 0.96) and < 4.3 pmol/L for POD1 samples (sensitivity 0.82, specificity 0.63, PPV 0.22, NPV 0.96). In early samples, a copeptin cutoff of 22.9 pmol/L increased the sensitivity for the detection of AVD to 95% with an NPV of 99%. The proportion of patients who had AVD was higher (60.0% vs 8.8%, p < 0.001) and the copeptin concentration lower (early: 4.3 vs 17.0 pmol/L, p < 0.001; POD1: 2.7 vs 4.9 pmol/L, p < 0.001) among those who had undergone surgery for a craniopharyngeal duct pathology versus a pituitary adenoma. Although copeptin was lower in patients with persistent Cushing’s disease than in those in remission, the difference did not reach statistical significance (early p = 0.11, POD1 p = 0.52). Furthermore, the copeptin concentration could not predict the development of syndrome of inappropriate secretion of antidiuretic hormone. Patients without AVD who had received stress dose steroids intraoperatively had lower median early copeptin (11.7 vs 19.1 pmol/L, p = 0.027). CONCLUSIONS In early postoperative copeptin samples, the optimal copeptin cut point for AVD diagnosis was < 8.5 pmol/L, and a level > 22.9 pmol/L had predicative utility in excluding AVD. Caution should be used when interpreting copeptin results, as patients administered glucocorticoids intraoperatively without AVD had lower median copeptin concentrations.

OBJECTIVE Chordomas are rare tumors that often recur regardless of surgery with negative margins and postoperative radiotherapy. The predictive accuracy of widely used immunohistochemical (IHC) markers in addressing the recurrence of skull base chordomas (SBCs) is yet to be determined. This study aimed to investigate IHC markers in the prediction of recurrence after SBC resection with adjuvant radiation therapy. METHODS The authors reviewed the records of patients who had treatment for SBC between January 2017 and June 2021 across the Mayo Clinic in Minnesota, Florida, and Arizona. Exclusion criteria included patients who had no histopathology or recurrence as an outcome. Histopathological markers included cytokeratin A1/A3 only, epithelial membrane antigen (EMA), S100 protein, pan-cytokeratin, IN1, GATA3, CAM5.2, OSCAR, and chondroid. Information from patient records was abstracted, including treatment, clinical and radiological follow-up duration, demographics, and histopathological factors. Decision tree and random forest classifiers were trained and tested to predict the recurrence based on unseen data using an 80/20 split. RESULTS A total of 38 patients with a diagnosis of SBC who underwent resection (gross-total resection: 42.1%; and subtotal resection: 57.9%) and radiation therapy were extracted from the medical records. The mean patient age was 48.2 (SD 19.6) years; most patients were male (n = 23; 60.5%) and White (n = 36; 94.7%). Pan-cytokeratin was associated with an increased risk of postoperative recurrence (OR 14.67, 95% CI 2.44–88.13; p = 0.00517) after resection and adjuvant radiotherapy. The decision tree analysis found pan-cytokeratin–positive tumors to have a 78% chance of being classified as a recurrence, with an accuracy of 75%. The distribution of minimal depth in the prediction of postoperative recurrence indicates that the most important variables were pan-cytokeratin, followed by cytokeratin A1/A3 and EMA. CONCLUSIONS The authors’ machine learning algorithm identified pan-cytokeratin as the largest contributor to recurrence among other IHC markers after SBC resection. Machine learning may facilitate the prediction of outcomes in rare tumors, such as chordomas.

OBJECTIVE The authors report a single-surgeon experience with anterior clinoid meningiomas (ACMs) and propose a novel scoring system to predict visual outcomes based on preoperative risk factors. METHODS A cohort study of all ACMs that were surgically treated by a single surgeon between 2003 and 2021 was performed. Visual function was assessed by an ophthalmologist pre- and postoperatively. Based on the combination of visual fields and visual acuity, 4 visual grades were described. Favorable visual outcomes were defined as mild visual deficit or intact vision postoperatively. Unfavorable visual outcomes were defined as a severe or moderate visual deficit. Predictors of unfavorable visual outcomes were identified using multivariable logistic regression analysis. A scoring system was then created using the resulting β coefficient. A receiver operating characteristic curve analysis was performed to identify a cutoff point on the grading score for stratifying patients at risk for unfavorable visual outcomes. RESULTS Fifty-two patients met all inclusion criteria. Twenty-five (48%) patients presented with intact vision, and 27 (51%) presented with some visual dysfunction. Postoperative favorable visual outcomes were achieved in 39 patients (75%). Among the 27 patients presenting with visual dysfunction, 14 (52%) experienced improvement after surgery. No new visual deficits were observed among the 25 patients with intact vision at baseline. Nine patients (17%) had a reversible complication. Multivariable analysis showed that severe preoperative visual deficit (OR 13.03, 95% CI 2.64–64.39; p = 0.002), radiographic evidence of optic nerve (ON) encasement (OR 4.20, 95% CI 1.06–16.61; p = 0.04), intraoperative evidence of ON invasion (OR 17.31, 95% CI 2.91–102.86; p = 0.002), an average ganglion cell layer thickness of ≤ 70 µm (OR 21.54, 95% CI 2.94–159.04; p = 0.003), and an average retinal nerve fiber layer thickness of ≤ 80 µm (OR 13.68, 95% CI 1.91–98.00; p = 0.009) were associated with unfavorable visual outcome. The predictive score included the following factors: abnormal optical coherence tomography (OCT) findings, radiographic evidence of ON encasement by the tumor, and severe preoperative visual deficit. A score ≥ 4 of 6 points was demonstrated to be the cutoff associated with unfavorable visual outcome, with a sensitivity of 80%, specificity of 88%, positive predictive value of 80%, negative predictive value of 88%, and area under the curve of 0.847 (95% CI 0.674–1.0; p = 0.003). CONCLUSIONS The authors have designed a practical and novel scoring system to predict visual outcomes in patients with ACMs. This scoring system may guide preoperative discussions with patients and timely surgical intervention to yield optimal visual function outcomes. Although most patients have excellent neurosurgical outcomes, severe baseline visual deficits, ON encasement, and characteristic OCT abnormalities are associated with unfavorable visual function after ACM resection.

Objective To compare cochlear implant (CI) and auditory brainstem implant (ABI) performance in patients with NF2-related schwannomatosis (NF2) Study Design Historical cohort. Setting Tertiary academic center. Patients A total of 58 devices among 48 patients were studied, including 27 ABIs implanted from 1997 to 2022 and 31 CIs implanted from 2003 to 2022. Three patients had bilateral ABIs, three had bilateral CIs, three had an ABI on one side and a CI on the other, one had a CI that was later replaced with an ipsilateral ABI, and one had an ABI and CI concurrently on the same side. Interventions CI or ABI ipsilateral to vestibular schwannoma. Main Outcome Measures Open-set speech perception, consonant-nucleus-consonant word scores, and AzBio sentence in quiet scores. Results Among all patients, 27 (47%) achieved open-set speech perception, with 35 (61%) daily users at a median of 24 months (interquartile range [IQR], 12–87 mo) after implantation. Comparing outcomes, CIs significantly outperformed ABIs; 24 (77%) CIs achieved open-set speech perception compared with 3 (12%) ABIs, with median consonant-nucleus-consonant and AzBio scores of 31% (IQR, 0–52%) and 57% (IQR, 5–83%), respectively, for CIs, compared with 0% (IQR, 0–0%) and 0% (IQR, 0–0%), respectively, for ABIs. Patients with ABIs were younger at diagnosis and at implantation, had larger tumors, and were more likely to have postoperative facial paresis. Conclusion Many patients with NF2-associated vestibular schwannoma achieved auditory benefit with either a CI or an ABI; however, outcomes were significantly better in those patients who were able to receive a CI. When disease and anatomy permit, hearing rehabilitation with a CI should be considered over an ABI in these patients. Tumor management strategies that increase the ability to successfully use CIs should be strongly considered given the high risk of losing bilateral functional acoustic hearing in this population.

Objective The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. Study Design Survey. Setting A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. Results House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. Conclusions Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

BACKGROUND AND OBJECTIVES: Schwannomas originating from the lower cranial nerves (LCNS) are rare and pose a significant surgical challenge. Resection is the mainstay treatment; however, risk of treatment morbidity is considerable, and the available literature regarding differential treatment outcomes in this vulnerable population is sparse. METHODS: A single-institution cohort study and systematic literature review of LCNS were performed. RESULTS: Fifty-eight patients were included: 34 underwent surgical resection and 24 underwent stereotactic radiosurgery (SRS). The median age at diagnosis was 48 years (range 17-74). Presenting symptoms were dysphagia (63%), dysarthria/hypophonia (47%), imbalance (33%), and hearing loss/tinnitus (30%). Tumor size was associated with surgical resection, as compared with initial SRS (4.1 cm vs 1.5 cm, P = .0001). Gross total resection was obtained in 52%, with tumor remnants predominantly localized to the jugular foramen (62%). Post-treatment worsening of symptoms occurred in 68% of surgical and 29% of SRS patients ( P = .003). Postoperative symptoms were mostly commonly hypophonia/hoarseness (63%) and dysphagia (59%). Seven patients (29%) had new neurological issues after SRS treatment, but symptoms were overall milder. The median follow-up was 60 months (range 12-252); 98% demonstrated meaningful clinical improvement. Eighteen surgical patients (53%) underwent adjuvant radiation at a median of 5 months after resection (range 2-32). At follow-up, tumor control was 97% in the surgical cohort and 96% among SRS patients. CONCLUSION: Although LCNS resection is potentially morbid, most postoperative deficits are transient, and patients achieve excellent tumor control—particularly when paired with adjuvant SRS. For minimally symptomatic patients undergoing surgical intervention, we advise maximally safe resection with intracapsular dissection to preserve nerve integrity where possible. For residual or as a primary treatment modality, SRS is associated with low morbidity and high rates of long-term tumor control.

OBJECTIVE The objective of this study was to clarify the detailed clinical course of recurrent clival chordoma and the outcomes of each treatment modality. METHODS A single-center retrospective analysis was conducted on patients seen for recurrent clival chordoma. The cohort was identified from those who underwent surgery, stereotactic radiosurgery, or proton therapy at the authors’ institution between 1990 and 2022. RESULTS A total of 95 recurrences in 40 patients with a median (interquartile range [IQR]) follow-up of 43 (18–79) months were identified. The median (IQR) age at the time of diagnosis was 48 (36–62) years, and 55% of patients were male. Twenty-three patients were treated with surgery followed by adjuvant radiation before the first recurrence. The median (range) number of recurrences per patient was 2 (1–8), and the median (IQR) time to the first recurrence was 29 (9–51) months. The recurrences were treated with one or more of the following therapies: surgery, radiation, systemic therapy, and laser interstitial thermal therapy (LITT). Surgery was performed for 44 recurrences in 25 patients. Radiation was used to treat 42 recurrences in 28 patients. Patients with recurrences treated with surgery plus radiation had the longest progression-free survival (PFS) (median [95% CI] overall survival [OS] 120 [0–245] months, p < 0.01, log-rank test). Patients with recurrences but without prior radiation had longer PFS than those patients with prior radiation. The median (95% CI) OS after the first recurrence was 68 (54–82) months, 5-year OS after the first recurrence was 48%, and 10-year OS was 27%. Multivariate Cox regression analysis showed that mortality after the first recurrence was significantly associated with no adjuvant radiation (HR 0.149, 95% CI 0.038–0.59, p = 0.0067), older age at the time of the first recurrence (HR 1.04, 95% CI 1.01–1.08, p = 0.021), and total number of recurrences (p = 0.032). Seven patients received systemic therapy, and the median (95% CI) OS of these patients since initiation of systemic therapy was 31 (11–51) months. Imatinib and/or nivolumab were used in 6 patients (15%). One patient (3%) was treated with LITT for his fourth recurrence. CONCLUSIONS Despite the aggressive nature of recurrent chordoma, 14 of 29 patients (48%) survived for more than 5 years after the initial recurrence using combined therapies. Multiple treatment options may contribute to the long-term survival of patients with this intractable tumor.