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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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Wael F. Asaad, Brian P. Walcott, Brian V. Nahed and Christopher S. Ogilvy

Brainstem cavernous malformations (CMs) are complex lesions associated with hemorrhage and neurological deficit. In this review, the authors describe the anatomical nuances relating to the operative techniques for these challenging lesions. The resection of brainstem CMs in properly selected patients has been demonstrated to reduce the risk of rehemorrhage and can be achieved relatively safely in experienced hands.

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Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Navid Redjal and Jean-Valery Coumans

Object

Previous methods to determine stroke prevalence, such as nationwide surveys, are labor-intensive endeavors. Recent advances in search engine query analytics have led to a new metric for disease surveillance to evaluate symptomatic phenomenon, such as influenza. The authors hypothesized that the use of search engine query data can determine the prevalence of stroke.

Methods

The Google Insights for Search database was accessed to analyze anonymized search engine query data. The authors' search strategy utilized common search queries used when attempting either to identify the signs and symptoms of a stroke or to perform stroke education. The search logic was as follows: (stroke signs + stroke symptoms + mini stroke − heat) from January 1, 2005, to December 31, 2010.

The relative number of searches performed (the interest level) for this search logic was established for all 50 states and the District of Columbia. A Pearson product-moment correlation coefficient was calculated from the statespecific stroke prevalence data previously reported.

Results

Web search engine interest level was available for all 50 states and the District of Columbia over the time period for January 1, 2005–December 31, 2010. The interest level was highest in Alabama and Tennessee (100 and 96, respectively) and lowest in California and Virginia (58 and 53, respectively). The Pearson correlation coefficient (r) was calculated to be 0.47 (p = 0.0005, 2-tailed).

Conclusions

Search engine query data analysis allows for the determination of relative stroke prevalence. Further investigation will reveal the reliability of this metric to determine temporal pattern analysis and prevalence in this and other symptomatic diseases.

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Manuel Ferreira Jr., Brian P. Walcott, Brian V. Nahed and Laligam N. Sekhar

Object

Hemifacial spasm (HFS) is caused by arterial or venous compression of cranial nerve VII at its root exit zone. Traditionally, microvascular decompression of the facial nerve has been an effective treatment for posterior inferior and anterior inferior cerebellar artery as well as venous compression. The traditional technique involves Teflon felt or another construct to cushion the offending vessel from the facial nerve, or cautery and division of the offending vein. However, using this technique for severe vertebral artery (VA) compression can be ineffective and fraught with complications. The authors report the use of a new technique of VA pexy to the petrous or clival dura mater in patients with HFS attributed to a severely ectatic and tortuous VA, and detail the results in a series of patients.

Methods

Six patients with HFS due to VA compression underwent a retrosigmoid craniotomy, combined with a far-lateral approach in some patients. On identification of the site of VA compression, the vessel was mobilized adequately for the decompression. Great care was taken to avoid kinking the perforating vessels arising from the VA. Two 8-0 nylon sutures were passed through to the wall of the VA and then through the clival or petrous dura, and then tied to alleviate compression on cranial nerve VII.

Results

Patients were followed for at least 1 year postoperatively (mean 2.7 years, range 1–4 years). All 6 patients had complete resolution of their HFS. Facial function was tested postoperatively, and was stable when compared with the preoperative baseline. Two of the 3 patients with preoperative tinnitus had resolution of this symptom after the procedure. Postoperative imaging demonstrated VA decompression of the facial nerve and no evidence of stroke in all patients. One patient suffered from hearing loss, another developed a postoperative transient unilateral vocal cord paralysis, and a third patient developed a pseudomeningocele that resolved with the placement of a lumbar drain.

Conclusions

Hemifacial spasm and other neurovascular syndromes are effectively treated by repositioning the compressing artery. Careful study of the preoperative MR images may identify a select group of patients with HFS due to an ectatic VA. Rather than traditional decompression with only pledget placement, these patients may benefit from a VA pexy to provide an effective, safe, and durable resolution of their symptoms while minimizing surgical complications.

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Benjamin D. Schanker, Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Yan Michael Li and Jean-Valery C. E. Coumans

Chiari malformations (Types I–IV) are abnormalities of the posterior fossa that affect the cerebellum, brainstem, and the spinal cord with prevalence rates of 0.1%–0.5%. Case reports of familial aggregation of Chiari malformation, twin studies, cosegregation of Chiari malformation with known genetic conditions, and recent gene and genome-wide association studies provide strong evidence of the genetic underpinnings of familial Chiari malformation. The authors report on a series of 3 family pairs with Chiari malformation Type I: 2 mother-daughter pairs and 1 father-daughter pair. The specific genetic causes of familial Chiari malformation have yet to be fully elucidated. The authors review the literature and discuss several candidate genes. Recent advances in the understanding of the genetic influences and pathogenesis of familial Chiari malformation are expected to improve management of affected patients and monitoring of at-risk family members.

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Jean-Valery Coumans, Brian P. Walcott, William E. Butler, Brian V. Nahed and Kristopher T. Kahle

Object

Resolution of syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of syringomyelia resolution.

Methods

A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the syringomyelia. They plotted the syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R 2 (unadjusted and adjusted) were calculated to describe the proportion of variability in each individual data set accounted for by the statistical model.

Results

Two patients were identified as meeting inclusion criteria. Plots of the least-squares best fit were identified as 4.01459e −0.0180804 x and 13.2556e −0.00615859 x. Decay of the syringomyelia followed an exponential model in both patients (R2 = 0.989582 and 0.948864).

Conclusions

Three-dimensional analysis of syringomyelia resolution over time enables the kinetics to be estimated. This technique is yet to be validated in a large cohort. Because syringomyelia is the final common pathway for a number of different pathological processes, it is possible that this exponential only applies to syringomyelia related to treatment of Chiari malformation Type I.

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Brian P. Walcott, Jean-Valery C. E. Coumans and Kristopher T. Kahle

Disorders of the spine are common in clinical medicine, and spine surgery is being performed with increasing frequency in the US. Although many patients with an established diagnosis of a true surgically treatable lesion are referred to a neurosurgeon, the evaluation of patients with spinal disorders can be complex and fraught with diagnostic pitfalls. While “common conditions are common,” astute clinical acumen and vigilance are necessary to identify lesions that masquerade as surgically treatable spine disease that can lead to erroneous diagnosis and treatment. In this review, the authors discuss musculoskeletal, peripheral nerve, metabolic, infectious, inflammatory, and vascular conditions that mimic the syndromes produced by surgical lesions. It is possible that nonsurgical and surgical conditions coexist at times, complicating treatment plans and natural histories. Awareness of these diagnoses can help reduce diagnostic error, thereby avoiding the morbidity and expense associated with an unnecessary operation.

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Jean-Valery C. E. Coumans and Brian P. Walcott

Incidental vertebral lesions on imaging of the spine are commonly encountered in clinical practice. Contributing factors include the aging population, the increasing prevalence of back pain, and increased usage of MR imaging. Additionally, refinements in CT and MR imaging have increased the number of demonstrable lesions. The management of incidental findings varies among practitioners and commonly depends more on practice style than on data or guidelines. In this article we review incidental findings within the vertebral column and review management of these lesions, based on available Class III data.

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Brian P. Walcott, Brian V. Nahed, Kristopher T. Kahle, Ann-Christine Duhaime, Nutan Sharma and Emad N. Eskandar

Generalized dystonic syndromes may escalate into persistent episodes of generalized dystonia known as status dystonicus that can be life-threatening due to dystonia-induced rhabdomyolysis and/or respiratory compromise. Treatment of these conditions usually entails parenteral infusion of antispasmodic agents and sedatives and occasionally necessitates a medically induced coma for symptom control. The authors report a series of 3 children who presented with medically intractable, life-threatening status dystonicus and were successfully treated with bilateral pallidal deep brain stimulation. Bilateral globus pallidus internus stimulation appears to be effective in the urgent treatment of medically refractory and life-threatening movement disorders.

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Brandyn Castro, Brian P. Walcott, Navid Redjal, Jean-Valery Coumans and Brian V. Nahed

Frontal sinus fractures are heterogeneous, and management of these fractures is often modified based on injury pattern and institutional experience. The optimal initial treatment of frontal sinus fractures is controversial. Treatment strategies are aimed at correcting cosmetic deformity, as well as at preventing delayed complications, including CSF fistulas, mucocele formation, and infection. Existing treatment options include observation, reconstruction, obliteration, cranialization, or a combination thereof. Modalities for treatment encompass both open surgical approaches and endoscopic techniques. In the absence of Class I data, the authors review the existing literature related to treatment strategies of frontal sinus fractures, particularly as they relate to CSF fistulas, to provide recommendations based on the best available evidence.