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Brian J. Dlouhy, Nader S. Dahdaleh and Jeremy D. W. Greenlee

Improvement in fiber optics and imaging paved the way for tremendous advancements in neuroendoscopy. These advancements have led to increasingly widespread use of the endoscope in neurosurgical procedures, which in turn incited a technological revolution leading to new approaches, instruments, techniques, and a diverse armamentarium for the treatment of a variety of neurosurgical disorders. Soft-tissue removal is often a rate-limiting aspect to endoscopic procedures, especially when the soft tissue is dense or fibrous. The authors review a series of cases involving patients treated between August 2009 and October 2010 with a new device (the NICO Myriad), a non–heat-generating, oscillating, cutting, and tissue removal instrument that can be used through the working channel of the endoscope as well as in open neurosurgical procedures. They used this device in 14 purely endoscopic intracranial procedures and 1 endoscope-assisted keyhole craniotomy. They report that the device was easy to use and found that tissue resection was more efficient than with other available endoscopic instruments, especially in the resection of fibrotic tissue. There were no observed device-related complications. The authors discuss the technical aspects of using this device in endoscopic resection of pituitary tumors, craniopharyngiomas, and colloid cysts. They also demonstrate its use in hydrocephalus and intraventricular clot removal and discuss its potential use in other neurosurgical disorders.

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Jeremy Greenlee, P. Charles Garell, Nicholas Stence and Arnold H. Menezes

Chiari malformation is a developmental disorder that is often associated with other abnormalities of the cerebrospinal axis. Despite widespread recognition of this association, there is relatively little information on the treatment of these coexisting disorders in the setting of cerebellar tonsillar ectopia. In an effort to improve the care provided to pediatric patients with Chiari malformations the authors reviewed their management practices over the last 20 years. Specifically, they recorded presenting symptoms, radiological studies, comorbidities, and management (surgical and nonsurgical) of 112 patients (all < 20 years of age) with Chiari malformation without myelodysplasia.

They found an associated syrinx in 29% of patients, basilar invagination in 17%, and scoliosis in 14%. The basal angle varied from 120 to 190° and Boogard's angle varied from 120 to 220°; both angles were larger than those measured in normal controls. The vertical height of the posterior fossa was shortened and the volume decreased as compared with normal controls.

The surgical management of this group of patients included posterior decompressive (44%), combined transoral and posterior decompressive (31%), combined posterior decompressive and posterior fusion (8%), and multiple posterior decompressive procedures in the same patient (5%).

The authors conclude that pediatric patients with a Chiari malformation should be specifically examined for evidence of additional craniovertebral malformations so that procedures are directed at correcting both the comorbidities and the herniation of the cerebellar tonsils through the foramen magnum.

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Jeremy D. W. Greenlee, Abdi Ghodsi, Gary L. Baumbach and John C. VanGilder

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Arnold H. Menezes, Jeremy D. W. Greenlee and Brian J. Dlouhy

OBJECTIVE

Syringobulbia (SB) is a rare entity, with few cases associated with Chiari malformation type I (CM-I) in the pediatric population. The authors reviewed all pediatric cases of CM-I–associated SB managed at their institution in order to better understand the presentation, treatment, and surgical outcomes of this condition.

METHODS

A prospectively maintained institutional database of craniovertebral junction abnormalities was analyzed to identify all cases of CM-I and SB from the MRI era (i.e., after 1984). The authors recorded presenting symptoms, physical examination findings, radiological findings, surgical treatment strategy, intraoperative findings, and outcomes. SB cases associated with tumors, infections, or type II Chiari malformations were excluded.

RESULTS

The authors identified 326 pediatric patients with CM-I who were surgically treated. SB was identified in 13 (4%) of these 326 patients. Headache and neck pain were noted in all 13 cases. Cranial nerve abnormalities were common: vagus and glossopharyngeal nerve dysfunction was the most frequent observation. Other cranial nerves affected included the trigeminal, abducens, and hypoglossal nerves. Several patients exhibited multiple cranial nerve palsies at presentation. Central sleep apnea was present in 6 patients.

Syringomyelia (SM) was present in all 13 patients. SB involved the medulla in all cases, and extended rostrally into the pons and midbrain in 2 patients; in 1 of these 2 cases the cavity extended further rostrally to the cerebrum (syringocephaly). SB communicated with the fourth ventricle in 7 of the 13 cases.

All 13 patients were treated with posterior fossa decompression with intradural exploration to ensure CSF egress out of the fourth ventricle and through the foramen magnum. The foramen of Magendie was found to be occluded by an arachnoid veil in 9 cases. Follow-up evaluation revealed that SB improved before SM. Cranial nerve palsies regressed in 11 of the 13 patients, and SB improved in all 13.

CONCLUSIONS

The incidence of SB in our surgical series of pediatric patients with CM-I was 4%, and all of these patients had accompanying SM. The SB cavity involved the medulla in all cases and was found to communicate with the fourth ventricle in 54% of cases. Posterior fossa decompression with intradural exploration and duraplasty is an effective treatment for these patients.

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Hans E. Bakken, Hiroto Kawasaki, Hiroyuki Oya, Jeremy D. W. Greenlee and Matthew A. Howard III

✓ Neurosurgeons use invasive mapping methods during surgery to understand the functional neuroanatomy of patients. Electrical stimulation methods are used routinely for the temporary disruption of focal regions of cerebral cortex so that the surgeon may infer the functional role of the brain site being stimulated. Although it is an efficient and useful method, modes of electrical stimulation mapping have significant limitations. Neuroscientists use focal cooling to effect a more controlled disruption of cortical functions in experimental animals, and in this report, the authors describe their experience using a device to achieve this same objective in patients undergoing neurosurgery. The cooling probe consists of a stainless steel chamber with thermocouples and electroencephalography (EEG) recording contacts. Active cooling is achieved by infusing chilled saline into the chamber when the cooling probe is positioned on the pial surface. Experiments were performed in 18 patients. Temperature gradient measurements indicate that the entire thickness of gray matter under the probe is cooled to temperatures that disrupt local synaptic activity. Statistically significant changes in spontaneous and stimulusevoked EEG activity were consistently observed during cooling, providing clear evidence of reversible disruption of physiological functions. Preliminary findings during functional mapping of the Broca area demonstrated qualitative differences between the temporary neurological deficits induced by cooling and those caused by electrical stimulation. These findings indicate the safety and utility of the cooling probe as a neurosurgical research tool. Additional rigorously designed studies should be undertaken to correlate the effects of cooling, electrical stimulation, and focal lesioning.

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Brian J. Dlouhy, Ana W. Capuano, Karthik Madhavan, James C. Torner and Jeremy D. W. Greenlee

Object

Patients with hydrocephalus often present with both intraventricular obstructive and communicating components, and determination of the predominant component is difficult. Other investigators have observed that third ventricular floor deformation, or “bowing” of the third ventricular floor, is a good indicator of intraventricular obstructive hydrocephalus, resulting in higher success rates with endoscopic third ventriculostomy (ETV). However, additional third ventricular bowing assessment and statistical evidence demonstrating a difference in ETV outcome with third ventricular bowing is needed. The authors hypothesized that patients with preoperative bowing of the third ventricle would exhibit greater long-term success rates after ETV and that lack of bowing would result in increased failure rates after ETV.

Methods

The authors determined success and failure for 59 ETVs performed in 56 patients, and recorded patient age, time to failure, and preoperative third ventricular anatomy, as well as history of infection, intraventricular hemorrhage, and previous shunt. Third ventricular anatomy was assessed on MR imaging for bowing, which was classified as any of the following: depression of the third ventricular floor, enlargement of the supraoptic recess, anterior curvature of the lamina terminalis, dilation of the proximal aqueduct to a greater extent than the distal aqueduct, and blunting or posterior bowing of the suprapineal recess. Univariate and multivariate analyses of ETV failure and the time to failure were performed using logistic regression and the Cox proportional hazards model, respectively.

Results

After adjusting for patient age and history of infection, there was a significant association between lack of anterior third ventricular preoperative bowing (either lamina terminalis, supraoptic recess, or third ventricular floor) and ETV failure (adjusted HR 2.79, 95% CI 1.08–7.20). Of the patients with bowing, 70.5% experienced success with ETV, as did 33.3% of the patients without bowing. Among the individual structures, absence of bowing in the anterior aspect of the third ventricular floor was significantly associated with censored time to ETV failure (multivariate HR 2.59, 95% CI 1.01–6.66; final model including age and history of infection).

Conclusions

The presence of preoperative third ventricular bowing is predictive of ETV success, with nearly a 3-fold likelihood of success compared with patients treated with ETV in the absence of such bowing. Although bowing is predictive, 33% of patients without bowing were also treated successfully with ETV.

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John P. Ney and David N. van der Goes

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Vincent C. Traynelis, Kingsley O. Abode-Iyamah, Katie M. Leick, Sarah M. Bender and Jeremy D. W. Greenlee

Object

The primary goal of this study was to review the immediate postoperative neurological function in patients surgically treated for symptomatic cervical spine disease without intraoperative neurophysiological monitoring. The secondary goal was to assess the economic impact of intraoperative monitoring (IOM) in this patient population.

Methods

This study is a retrospective review of 720 consecutively treated patients who underwent cervical spine procedures. The patients were identified and the data were collected by individuals who were not involved in their care.

Results

A total of 1534 cervical spine levels were treated in 720 patients using anterior, posterior, and combined (360°) approaches. Myelopathy was present preoperatively in 308 patients. There were 185 patients with increased signal intensity within the spinal cord on preoperative T2-weighted MR images, of whom 43 patients had no clinical evidence of myelopathy. Three patients (0.4%) exhibited a new neurological deficit postoperatively. Of these patients, 1 had a preoperative diagnosis of radiculopathy, while the other 2 were treated for myelopathy. The new postoperative deficits completely resolved in all 3 patients and did not require additional treatment. The Current Procedural Terminology (CPT) codes for IOM during cervical decompression include 95925 and 95926 for somatosensory evoked potential monitoring of the upper and lower extremities, respectively, as well as 95928 and 95929 for motor evoked potential monitoring of the upper and lower extremities. In addition to the charge for the baseline [monitoring] study, patients are charged hourly for ongoing electrophysiology testing and monitoring using the CPT code 95920. Based on these codes and assuming an average of 4 hours of monitoring time per surgical case, the savings realized in this group of patients was estimated to be $1,024,754.

Conclusions

With the continuing increase in health care costs, it is our responsibility as providers to minimize expenses when possible. This should be accomplished without compromising the quality of care to patients. This study demonstrates that decompression and reconstruction for symptomatic cervical spine disease without IOM may reduce the cost of treatment without adversely impacting patient safety.

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Arnold H. Menezes, Jeremy D. W. Greenlee, Reid A. Longmuir, Daniel R. Hansen and Kingsley Abode-Iyamah

The authors present the case of a 14-year-old boy with holocord syringohydromyelia extending into the brainstem, cerebral peduncle, internal capsule, and cerebral cortex. At the posterior fossa exploration, an opaque thickened arachnoid with occlusion of the foramen of Magendie was encountered. Careful documentation of postoperative regression of the syringocephaly, syringobulbia, and syringohydromyelia was made. The pathophysiology is discussed.

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Taylor J. Abel, Brian D. Dalm, Andrew J. Grossbach, Adam W. Jackson, Teri Thomsen and Jeremy D. W. Greenlee

Lesch-Nyhan disease (LND) is an X-linked hereditary disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase. This syndrome is characterized by hyperuricemia, self-mutilation, cognitive impairment, and movement disorders such as spasticity and dystonia. The authors describe the case of a 15-year-old boy who underwent bilateral placement of globus pallidus internus (GPi) deep brain stimulation (DBS) electrodes for the treatment of generalized dystonia. His self-mutilating behavior gradually disappeared several weeks after the start of GPi stimulation. The dystonia and self-mutilating behavior returned on the left side only after a right lead fracture. This case is the first reported instance of LND treated with DBS in which the stimulation was interrupted and the self-mutilation returned in a lateralized fashion. The findings indicate that the neurobehavioral aspect of LND is lateralized and that contralateral GPi stimulation is responsible for lateralized improvement in self-injurious behavior.