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W. Scott Jellish, John Murdoch and John P. Leonetti

The anesthetic management of complex skull base surgical procedures provides unique problems and concerns for the neuroanesthesiologist. Positioning to access the skull base could put the patient at risk for peripheral nerve injury and some of the positions may increase the risk for air emboli. In addition, tumor pathology and involvement with vital structures could increase the chances for substantive blood loss, destruction of associated nerves or vessels, and may require temporary occlusion of the carotid artery necessitating intraoperative neuroprotection. Neurophysiological monitors may also be used to safeguard nerve function and anesthetic techniques must be adjusted to accommodate their use. Finally, postoperative morbidity may be affected by surgical approach to the skull base and the anesthesiologist should be aware of which approach may produce a greater incidence of pain, nausea, and vomiting in the postoperative period.

The authors discuss the anesthetic concerns and management for complex cranial base surgery. Different approaches will be discussed and comparisons of perioperative parameters between these approaches will be made with data provided by retrospective chart review of more than 600 skull base procedures performed at the authors' institution over the last 10 years. This information should help guide decision making concerning anesthetic management for these skull base procedures.

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Sam J. Marzo, John P. Leonetti and Guy Petruzzelli

Object

Bell palsy remains the most common cause of facial paralysis. Unfortunately, this term is often erroneously applied to all cases of facial paralysis.

Methods

The authors performed a retrospective review of data obtained in 11 patients who were treated at a university-based referral practice between July 1988 and September 2001 and who presented with acute facial nerve paralysis mimicking Bell palsy. All patients were subsequently found to harbor an occult skull base neoplasm. A delay in diagnosis was demonstrated in all cases. Seven patients died of their disease, and four patients are currently free of disease.

Conclusions

Although Bell palsy remains the most common cause of peripheral facial nerve paralysis, patients in whom neoplasms invade of the facial nerve may present with acute paralysis mimicking Bell palsy that fails to resolve. Delays in diagnosis and treatment in such cases may result in increased rates of mortality and morbidity.

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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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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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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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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.