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Giselle E. K. Malina, Daniel M. Heiferman, Loren N. Riedy, Caroline C. Szujewski, Elhaum G. Rezaii, John P. Leonetti and Douglas E. Anderson

OBJECTIVE

Sporadic unilateral vestibular schwannomas are rare in the pediatric population. Little has been reported in the literature on the presentation, tumor size, response to surgical treatment, and recurrence rates in these younger patients. The authors’ goal was to describe their institutional experience with pediatric sporadic vestibular schwannomas and to conduct a meta-analysis of the existing literature to provide further insight into the presentation, tumor characteristics, and surgical outcomes for these rare tumors to help direct future treatment strategies.

METHODS

The authors performed a retrospective review of all patients 21 years of age or younger with unilateral vestibular schwannomas and without neurofibromatosis type 2 who underwent resection by the senior authors between 1997 and 2019. A systematic review of the literature and meta-analysis was also performed by entering the search terms “pediatric” and “vestibular schwannoma” or “acoustic neuroma,” as well as “sporadic” into PubMed. Presentation, treatment, clinical outcomes, and follow-up were analyzed.

RESULTS

Fifteen patients were identified at the authors’ institution, ranging in age from 12 to 21 years (mean 16.5 years). Common presenting symptoms included hearing loss (87%), headache (40%), vertigo (33%), ataxia (33%), and tinnitus (33%). At the time of surgery, the mean tumor size was 3.4 cm, with four 1-cm tumors. Four patients had residual tumor following their first surgery, 3 (75%) of whom had significant radiographic regrowth that required further treatment. The literature review identified an additional 81 patients from 26 studies with patient-specific clinical data available for analysis. This resulted in a total of 96 reported patients with an overall average age at diagnosis of 12.1 years (range 6–21 years) and an average tumor size of 4.1 cm.

CONCLUSIONS

Pediatric vestibular schwannomas present similarly to those in adults, although symptoms of mass effect are more common, as these tumors tend to be larger at diagnosis. Some children are found to have small tumors and can be successfully treated surgically. Residual tumors in pediatric patients were found to have a higher rate of regrowth than those in their adult counterparts.

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Joshua J. Wind, John P. Leonetti, Michael J. M. Raffin, Marc T. Pisansky, Brian Herr, Justin D. Triemstra and Douglas E. Anderson

Object

No extant literature documents the analysis of patient perceptions of hearing as a corollary to objective audiometric measures in patients with vestibular schwannoma (VS), or acoustic neuroma. Therefore, using objective audiometric data and patient perceptions of hearing function as outlined on a questionnaire, the authors evaluated the hearing of patients who underwent VS resection.

Methods

This investigation involved a retrospective review of 176 patients who had undergone VS resections in which hearing preservation was a goal. Both pre- and postoperative audiometry, expressed as a speech discrimination score (SDS) and pure tone threshold average (PTA), were performed, and the results were analyzed. Intraoperative auditory brainstem responses were also recorded. Eighty-seven of the patients (49.4%) completed a postoperative questionnaire designed to assess hearing function in a variety of social and auditory situations. Multiple linear regression analyses were completed to compare available audiometric results with questionnaire responses for each patient.

Results

One hundred forty-two patients (80.7%) had PTA and SDS audiometric data pertaining to the surgically treated ear; 94 of these patients (66.2%) had measurable postoperative hearing, as defined by a PTA < 120 dB or SD > 0%. Eighty-seven patients (49.4%) completed the retrospective questionnaire, and 74 of them had complete audiometric data and thus were included in a comparative analysis. Questionnaire data showed major postoperative subjective hearing decrements, even among patients with the same pre- and postoperative objective audiometric hearing status. Moreover, the subscore reflecting hearing while exposed to background noise, or the “cocktail party effect,” characterized the most significant patient-perceived hearing deficit following VS resection.

Conclusions

The authors' analysis of a patient-perceived hearing questionnaire showed that hearing during exposure to background noise, or the cocktail party effect, represents a significant postoperative hearing deficit and that patient perception of this deficit has a strong relation with audiometric data. Furthermore, questionnaire responses revealed a significant disparity between subjective hearing function and standard audiometrics such that even with similar levels of audiometric data, subjective measures of hearing, especially the cocktail party effect, decreased postoperatively. The authors posit that the incorporation of patient-perceived hearing function evaluation along with standard audiometry is an illustrative means of identifying subjective hearing deficits after VS resection and may ultimately aid in specific and subsequent treatment for these patients.

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Ryan P. Morton, Paul D. Ackerman, Marc T. Pisansky, Monika Krezalek, John P. Leonetti, Michael J.M. Raffin and Douglas E. Anderson

Object

Preservation of facial nerve function in vestibular schwannoma (VS) resections remains a significant operative challenge. Delayed facial palsy (DFP) is one specific challenge yet to be fully elucidated. The aim of this study was to evaluate DFP among VS resection cases to identify significant prognostic factors associated with its incidence and clinical recovery.

Methods

This investigation involves a retrospective review of 104 cases of VS resection that occurred between December 2005 and May 2007. Patients who developed DFP were compared with patients exhibiting no facial palsy postoperatively with regard to surgical approach, severity and day of palsy onset, tumor size, intraoperative facial nerve monitoring, and postoperative recovery and treatment. Patients who demonstrated immediate facial palsy (IFP) following VS resection were also analyzed. Furthermore, specific analyses were performed in 2 distinct DFP patient groups: those who developed DFP after postoperative Day 3 (“late onset DFP”), and those whose palsy worsened after initial DFP identification (“deteriorators”).

Results

Of the 104 patients who underwent VS resection, 25.0% developed DFP and 8.6% demonstrated IFP postoperatively. The DFP group did not differ significantly in any measure when compared with patients with no postoperative facial palsy. However, patients with DFP presented with significantly smaller tumor sizes than patients with IFP. This IFP group averaged significantly smaller intraoperative facial nerve responses than patients without facial palsy, and larger tumor sizes than both the DFP and no facial palsy groups. Within the DFP group, patients with late onset DFP showed diminished intraoperative facial nerve responses when compared with the total DFP patient population. In total, 25 (96.2%) of 26 patients with DFP and 7 (77.8%) of 9 patients with IFP recovered to normal or near-normal facial function (House-Brackmann Grade I or II) at longest clinical follow-up.

Conclusions

Although patients with DFP did not exhibit any distinguishable characteristics when compared with patients without postoperative facial palsy, our analysis identified significant differences in patients with palsy presenting immediately postoperatively. Further study of patients with DFP should be undertaken to predict its incidence following VS resection.

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Darian R. Esfahani, Marc T. Pisansky, Rima M. Dafer and Douglas E. Anderson

Neuropathic facial pain can be a debilitating condition characterized by stabbing, burning, dysesthetic sensation. With a large range of causes and types, including deafferentation, postherpetic, atypical, and idiopathic, both medicine and neurosurgery have struggled to find effective treatments that address this broad spectrum of facial pain. The authors report the use of motor cortex stimulation to alleviate 3 distinct conditions associated with intractable facial pain: trigeminal deafferentation pain following rhizotomy, deafferentation pain secondary to meningioma, and postherpetic neuralgia. Functional MR imaging was used to localize facial areas on the precentral gyrus prior to surgery. All 3 patients experienced long-lasting complete or near-complete resolution of pain following electrode implantation. Efficacy in pain reduction was achieved through variation of stimulation settings over the course of treatment, and it was assessed using the visual analog scale and narrative report. Surgical complications included moderate postsurgical incisional pain, transient cerebral edema, and intraoperative seizure. The authors' results affirm the efficacy and broaden the application of motor cortex stimulation to several forms of intractable facial pain.

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Ahmad Khaldi, Vikram C. Prabhu, Douglas E. Anderson and Thomas C. Origitano

Object

This study was conducted to evaluate the value of postoperative CT scans in determining the probability of return to the operating room (OR) and the optimal time to obtain such scans to determine the effects of surgery.

Methods

Between January and December 2006 (12 months), all postoperative head CT scans obtained for 3 individual surgeons were reviewed. Scans were divided into 3 groups, which were determined by the preference of each surgeon: Group A (early scans—scheduled between 0 and 7 hours); Group B (delayed scans—scheduled between 8 and 24 hours); and Group C (urgent scans—ordered because of a new neurological deficit). The initial scans were reviewed and analyzed in 2 different fashions. The first was to analyze the efficacy of the scans in predicting return to the OR. The second was to determine the optimal time for obtaining a scan. The second analysis was a review of serial postoperative scans for expected versus unexpected findings and changes in the acuity of these findings over time.

Results

In 251 (74%) of 338 cases, the patients had postoperative head CT scans within 24 hours of surgery. Analysis 1 determined the percent of patients returning to the OR for emergency treatment based on postoperative scans: Group A (early)—133 patients, with 0% returning to the OR; Group B (delayed)—108 patients, with 0% returning to the OR; and Group C (urgent)—10 patients, with 30% returning to the OR (p < 0.05). Analysis 2 determined the optimal timing of postoperative scans and changes in scan acuity: Group A (early scan) had an 11% incidence of change in acuity on subsequent scans. Group B (delayed scan) had a 3% incidence of change in acuity on follow-up scans (p < 0.05).

Conclusions

Routine postoperative scans at 0–7 hours or at 8–24 hours are not predictive of return to the OR, whereas patients with a new neurological deficit in the postoperative period have a 30% chance of emergency reoperation based on CT scans. In addition, early postoperative scans (0–7 hours) fail to predict CT changes, which might evolve over time and may influence postoperative medical management.

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Brian P. Walcott, Ganesh Sivarajan, Bronislava Bashinskaya, Douglas E. Anderson, John P. Leonetti and Thomas C. Origitano

Object

Vestibular schwannomas (VSs) are rare in the pediatric population. Most often, these lesions manifest as a bilateral disease process in the setting of neurofibromatosis Type 2. Even in the absence of additional clinical diagnostic criteria, the presentation of a unilateral VS in a young patient may be a harbinger of future penetrance for this hereditary tumor syndrome.

Methods

The authors retrospectively reviewed the charts of a cohort of 7 patients who presented with apparently sporadic, unilateral VSs. These patients had previously undergone surgery via translabyrinthine, retrosigmoid, or combined approaches. Clinical outcomes were reviewed with emphasis on facial nerve function and follow-up for signs and symptoms of a heritable disorder.

Results

All patients underwent microsurgical resection in a multidisciplinary effort by the senior authors. The average tumor size was 4.57 cm, with an average duration of symptoms prior to definitive diagnosis of 31.2 months. The tumor size at the time of presentation followed a trend different from reports in adults, while the duration of symptoms did not. At a follow-up average of 6.3 years (range 1–12 years), 100% of patients demonstrated good facial function (House-Brackmann Grade I or II). No patient in this cohort demonstrated symptoms, objective signs, or genetic analysis indicating the presence of neurofibromatosis Type 2.

Conclusions

Diagnosis and management of sporadic, unilateral VSs in children is complicated by clinical presentations and surgical challenges unique from their adult counterparts. Careful consideration should be given to a heritable genetic basis for sporadic unilateral VS in the pediatric population. Results of genetic testing do not preclude the necessity for long-term follow-up and systemic investigation. In patients who present with large tumors, preliminary experience leads the authors to suggest that a combined retrosigmoid-translabyrinthine approach offers the greatest opportunity for preservation of facial nerve function.

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Joshua J. Wind and Douglas E. Anderson

The history of psychosurgery is described and analyzed. This historical perspective largely begins with analysis of the work of Egas Moniz in the development of the leukotomy, and follows the rise and fall of its popularity in the 1900s. The reemergence of psychosurgical procedures and the development of new therapeutic technologies such as vagus nerve stimulation and deep brain stimulation are discussed. In addition, an introduction to the field of neuroethics is provided, given its importance in any discussion about surgical therapy for psychiatric patients.

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O. Howard Reichman, Edward A. M. Duckworth, Douglas E. Anderson and Thomas C. Origitano

✓The conventional wisdom resulting from the international, multicenter, trial of extracranial–intracranial bypass surgery is that this procedure offers no benefit. Because of the complex and unique circumstances of some, clinical experience and judgment must sometimes overrule some statistical conclusions.

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Pierre-Hugues Roche, Nicolas Lari, Jean-Marc Thomassin and Jean Régis

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Douglas E. Anderson, John Leonetti, Joshua J. Wind, Denise Cribari and Karen Fahey

Object. Vestibular schwannoma surgery has evolved as new therapeutic options have emerged, patients' expectations have risen, and the psychological effect of facial nerve paralysis has been studied. For large vestibular schwannomas for which extirpation is the primary therapy, the goals remain complete tumor resection and maintenance of normal neurological function. Improved microsurgical techniques and intraoperative facial nerve monitoring have decreased the complication rate and increased the likelihood of normal to near-normal postoperative facial function. Nevertheless, the impairment most frequently reported by patients as an adverse effect of surgery continues to be facial nerve paralysis. In addition, patient assessment has provided a different, less optimistic view of outcome. The authors evaluated the extent of facial function, timing of facial nerve recovery, patients' perceptions of this recovery and function, and the prognostic value of intraoperative facial nerve monitoring following resection of large vestibular schwannomas; they then analyzed these results with respect to different surgical approaches.

Methods. The authors retrospectively reviewed a database of 67 patients with 71 vestibular schwannomas measuring 3 cm or larger in diameter. The patients had undergone surgery via translabyrinthine, retrosigmoid, or combined approaches. Clinical outcomes were analyzed with respect to intraoperative facial nerve activity, responses to intraoperative stimulation, and time course of recovery.

Eighty percent of patients obtained normal to near-normal facial function (House—Brackmann Grades I and II). Patients' perceptions of facial nerve function and recovery correlated well with the clinical observations.

Conclusions. Trends in the data lead the authors to suggest that a retrosigmoid exposure, alone or in combination with a translabyrinthine approach, offers the best chance of facial nerve preservation in patients with large vestibular schwannomas.