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Objective: Arteriovenous malformations (AVMs) are the most common cause of spontaneous intracranial hemorrhage (ICH) in children, often leading to devastating complications. The current literature from the adult AVM population suggests that both younger age and associated aneurysms carry an increased risk of hemorrhagic presentation. However, detailed analysis of pediatric AVM-associated aneurysms and their link to ICH is relatively unknown, with the literature largely consisting of case reports. This study aimed to determine whether AVM-associated aneurysms predispose pediatric patients to ICH., Methods: A retrospective cohort study of 238 pediatric patients with AVMs who presented to the Children's Hospital of Pittsburgh from 1988 to 2023 was performed. Hospital records, patient charts, and radiographic imaging studies were reviewed for patient demographic characteristics, presentation status, and AVM architecture., Results: Of the 238 total patients, 44 (18.5%) children with AVM had associated aneurysms. There were 19 (38.8%) feeding artery aneurysms, 8 (16.3%) intranidal aneurysms, 21 (42.9%) postnidal aneurysms, and 1 (2.0%) unrelated aneurysm of 49 aneurysms. Five patients had venous varices. One hundred forty (58.8%) children presented with hemorrhage. Twenty-one of 44 (47.7%) patients with an AVM-associated aneurysm presented with hemorrhage, whereas 119 of 194 (61.3%) with a solitary AVM presented with hemorrhage (p = 0.10). On multivariate analysis, postnidal aneurysm (OR 0.36, p = 0.037) and an increased number of draining veins (OR 0.66, p = 0.049) were significantly associated with a decreased likelihood of hemorrhagic presentation. Deep venous drainage was associated with an increase in hemorrhagic presentation (OR 2.25, p = 0.0045) on multivariate analysis., Conclusions: Approximately one-fifth of children with AVMs in this study had accompanying aneurysms, and in this patient population, those with postnidal aneurysms and increased number of draining veins had a decreased incidence of hemorrhage on presentation. Feeding artery and intranidal aneurysms were not associated with an elevated risk of hemorrhagic presentation.

Objective: When the peritoneal cavity cannot serve as the distal shunt terminus, nonperitoneal shunts, typically terminating in the atrium or pleural space, are used. The comparative effectiveness of these two terminus options has not been evaluated. The authors directly compared shunt survival and complication rates for ventriculoatrial (VA) and ventriculopleural (VPl) shunts in a pediatric cohort., Methods: The Hydrocephalus Clinical Research Network Core Data Project was used to identify children ≤ 18 years of age who underwent either VA or VPl shunt insertion. The primary outcome was time to shunt failure. Secondary outcomes included distal site complications and frequency of shunt failure at 6, 12, and 24 months., Results: The search criteria yielded 416 children from 14 centers with either a VA (n = 318) or VPl (n = 98) shunt, including those converted from ventriculoperitoneal shunts. Children with VA shunts had a lower median age at insertion (6.1 years vs 12.4 years, p < 0.001). Among those children with VA shunts, a hydrocephalus etiology of intraventricular hemorrhage (IVH) secondary to prematurity comprised a higher proportion (47.0% vs 31.2%) and myelomeningocele comprised a lower proportion (17.8% vs 27.3%) (p = 0.024) compared with those with VPl shunts. At 24 months, there was a higher cumulative number of revisions for VA shunts (48.6% vs 38.9%, p = 0.038). When stratified by patient age at shunt insertion, VA shunts in children < 6 years had the lowest shunt survival rate (p < 0.001, log-rank test). After controlling for age and etiology, multivariable analysis did not find that shunt type (VA vs VPl) was predictive of time to shunt failure. No differences were found in the cumulative frequency of complications (VA 6.0% vs VPl 9.2%, p = 0.257), but there was a higher rate of pneumothorax in the VPl cohort (3.1% vs 0%, p = 0.013)., Conclusions: Shunt survival was similar between VA and VPl shunts, although VA shunts are used more often, particularly in younger patients. Children < 6 years with VA shunts appeared to have the shortest shunt survival, which may be a result of the VA group having more cases of IVH secondary to prematurity; however, when age and etiology were included in a multivariable model, shunt location (atrium vs pleural space) was not associated with time to failure. The baseline differences between children treated with a VA versus a VPl shunt likely explain current practice patterns.

Objective: Although keyhole transorbital approaches are gaining traction, their indications have not been adequately studied comparatively. In this study the authors have defined them also as transwing approaches-meaning that they use the different facies of the sphenoid wing for cranial entry-and sought to compare the four major ones: 1) lateral orbitocraniotomy through a lateral canthal incision (LatOrb); 2) modified orbitozygomatic approach through a palpebral incision (ModOzPalp); 3) modified orbitozygomatic approach through an eyebrow incision (ModOzEyB); and 4) supraorbital craniotomy through an eyebrow incision (SupraOrb), coupled with its expanded version (SupraTransOrb)., Methods: Cadaveric dissections were performed at the neuroanatomy lab. To delineate the skull base exposure, four formalin-fixed heads were used, with two sides dedicated to each approach. The outer limits were assessed via image guidance and were mapped and illustrated accordingly. A fifth head was dissected purely endoscopically, just to facilitate an overview of the transwing concept. Qualitative features were also rigorously examined., Results: The LatOrb proves to be more versatile in the middle cranial fossa (MCF), whereas the anterior cranial fossa (ACF) exposure is limited to a small area above the sphenoid ridge. An anterior clinoidectomy is possible; however, the exposure of the roof of the optic canal is suboptimal. The ModOzPalp adequately exposes both the ACF and MCF. Its lateral trajectory allows the inferior to superior view, yet there is restricted access to the medial anterior skull base (olfactory groove). The ModOzEyB also provides extensive exposure of the ACF and MCF, but has a more superior to inferior trajectory compared to the ModOzPalp, making it more appropriate for pathology reaching the medial anterior skull base or even the contralateral side. The anterior clinoidectomy is performed with improved visualization of the optic canal. The SupraOrb provides mainly anterior cranial base exposure, with minimal middle fossa. An anterior clinoidectomy can be performed, but without any direct observation of the superior orbital fissure. Some MCF access can be accomplished if the lateral sphenoid wing is drilled inferiorly, leading to its highly versatile variant, the SupraTransOrb., Conclusions: All the aforementioned approaches use the sphenoid wing as skull base corridor from a specific orientation point; hence these are designated as transwing approaches. Their peculiarities mandate careful case selection for the effective and safe completion of the surgical goals.

Objective: Multimodality treatment has been shown to be the optimal management strategy for pediatric arteriovenous malformations (AVMs). Deep AVMs represent a subset of AVMs for which optimal management may be achieved with a combination of radiosurgery and highly selective embolization, in the absence of compelling features requiring operative intervention. The objective of this study was to identify predictors of good functional outcomes in pediatric patients with deep AVMs., Methods: A retrospective cohort study of the outcomes of 79 patients with deep AVMs from January 1988 through December 2021 was performed. Deep AVMs were defined as those with the majority of the nidus centered in the basal ganglia, thalamus, or brainstem. Collected data included patient demographics and presenting symptoms, presenting modified Rankin Scale (mRS) score, radiographic findings and outcomes, management strategy, complications, and clinical outcomes as indicated by follow-up mRS score. A good outcome was defined as a follow-up mRS score ≤ 2, while a poor outcome was defined as a follow-up mRS score ≥ 3. Statistical analysis was performed to identify factors associated with functional outcomes., Results: With a mean follow-up duration of 85.6 months, there was a 72.2% angiographic obliteration rate, with 75.9% of patients having a good clinical outcome (mRS score ≤ 2). Presenting symptoms and radiographic characteristics were not significantly associated with long-term functional outcomes. There was a significantly higher rate of posttreatment hemorrhage in patients with a poor versus good outcome (11.8% vs 0%, p = 0.010). On multivariate logistic regression analysis, poor long-term functional outcome was only associated with poor presenting mRS score (p = 0.002)., Conclusions: Satisfactory angiographic obliteration rates and good long-term functional outcomes can be achieved for deep AVMs, with stereotactic radiosurgery as the cornerstone of multimodality treatment.

Mesial temporal lobe epilepsy (mTLE) is an important cause of drug-resistant epilepsy (DRE) in adults and children. Traditionally, the surgical option of choice for mTLE includes a frontotemporal craniotomy and open resection of the anterior temporal cortex and mesial temporal structures. Although this technique is effective and durable, the neuropsychological morbidity resulting from temporal neocortical resections has resulted in the investigation of alternative approaches to resect the mesial temporal structures to achieve seizure freedom while minimizing postoperative cognitive deficits. Outcomes supporting the use of selective temporal resections have resulted in alternative approaches to directly access the mesial temporal structures via endoscopic approaches whose direct trajectory to the epileptogenic zone minimizes retraction, resection, and manipulation of surrounding cortex. The authors reviewed the utility of the endoscopic transmaxillary, endoscopic endonasal, endoscopic transorbital, and endoscopic supracerebellar transtentorial approaches for the treatment of drug-resistant mesial temporal lobe epilepsy. First, a review of the literature demonstrated the anatomical feasibility of each approach, including the limits of exposure provided by each trajectory. Next, clinical data assessing the safety and effectiveness of these techniques in the treatment of DRE were analyzed. An outline of the surgical techniques is provided to highlight the technical nuances of each approach. The direct access to mesial temporal structures and avoidance of lateral temporal manipulation makes endoscopic approaches promising alternatives to traditional methods for the treatment of DRE arising from the temporal pole and mesial temporal lobe. A dearth of literature outlining clinical outcomes, a need for qualified cosurgeons, and a lack of experience with endoscopic approaches remain major barriers to widespread application of the aforementioned techniques. Future studies are warranted to define the utility of these approaches moving forward.

Objective: Reports published during the severe acute respiratory syndrome coronavirus 2 (Sars-CoV-2) pandemic suggest that hospitals potentially experienced an increased incidence in the presentation of abusive head trauma (AHT) in children; however, it remains unknown if the pandemic influenced the severity or need for neurosurgical intervention during this time., Methods: This study is a post hoc analysis of a prospectively collected database of pediatric patients who sustained traumatic head injuries from 2018 to 2021 and were treated at the Children's Hospital of Pittsburgh that was screened for concern of AHT at the time of presentation. Pairwise univariate analysis of AHT prevalence, Glasgow Coma Scale (GCS) score, intracranial pathology, and neurosurgical interventions was performed to investigate differences before, during, and after the initial lockdown in Pennsylvania, which was defined as March 23, 2020, to August 26, 2020., Results: Of 2181 pediatric patients who presented with head trauma, 263 (12.1%) with AHT were identified. Prevalence of AHT did not differ during (12.4% before vs 10.0% during, p = 0.31) or following (12.2% after, p = 0.92) lockdown. Need for neurosurgery after AHT remained unchanged during lockdown (10.7% before vs 8.3% during, p = 0.72) and after (10.5% after, p = 0.97). Patients did not differ in terms of sex, age, or race between periods. Average GCS score was lower after lockdown (13.9 before vs 11.9 after, p = 0.008) but not during (12.3, p = 0.062). In this cohort, mortality associated with AHT was 4.8 times higher during lockdown (4.3% before vs 20.8% during, p = 0.002) and returned to pre-lockdown rates thereafter (7.8%, p = 0.27). The primary contributor to mortality was ischemic brain injury (5% before vs 20.8% during, p = 0.005). Patients were 5.5 times more likely to undergo decompressive hemicraniectomy in the months after lockdown compared with prior (1.2% vs 6.6%, p = 0.035)., Conclusions: The authors have presented the findings of the first study to examine the prevalence and neurosurgical management of AHT during the Sars-Cov-2 lockdown in Pennsylvania. The overall prevalence of AHT was not affected by lockdown; however, patients were more likely to experience mortality or traumatic ischemia during lockdown. The GCS score of AHT patients was significantly lower, and these patients were more likely to require decompressive hemicraniectomy after the initial lockdown period.

Objective: Intracranial pressure (ICP) is an important therapeutic target in many critical neuropathologies. The current tools for ICP measurements are invasive; hence, these are only selectively applied in critical cases where the benefits surpass the risks. To address the need for low-risk ICP monitoring, the authors developed a noninvasive alternative., Methods: The authors recently demonstrated noninvasive quantification of ICP in an animal model by using morphological analysis of microvascular cerebral blood flow (CBF) measured with diffuse correlation spectroscopy (DCS). The current prospective observational study expanded on this preclinical study by translating the method to pediatric patients. Here, the CBF features, along with mean arterial pressure (MAP) and heart rate (HR) data, were used to build a random decision forest, machine learning model for estimation of ICP; the results of this model were compared with those of invasive monitoring., Results: Fifteen patients (mean age ± SD [range] 9.8 ± 5.1 [0.3-17.5] years; median age [interquartile range] 11 [7.4] years; 10 males and 5 females) who underwent invasive neuromonitoring for any purpose were enrolled. Estimated ICP (ICPest) very closely matched invasive ICP (ICPinv), with a root mean square error (RMSE) of 1.01 mm Hg and 95% limit of agreement of ≤ 1.99 mm Hg for ICPinv 0.01-41.25 mm Hg. When the ICP range (ICPinv 0.01-29.05 mm Hg) was narrowed on the basis of the sample population, both RMSE and limit of agreement improved to 0.81 mm Hg and ≤ 1.6 mm Hg, respectively. In addition, 0.3% of the test samples for ICPinv ≤ 20 mm Hg and 5.4% of the test samples for ICPinv > 20 mm Hg had a limit of agreement > 5 mm Hg, which may be considered the acceptable limit of agreement for clinical validity of ICP sensing. For the narrower case, 0.1% of test samples for ICPinv ≤ 20 mm Hg and 1.1% of the test samples for ICPinv > 20 mm Hg had a limit of agreement > 5 mm Hg. Although the CBF features were crucial, the best prediction accuracy was achieved when these features were combined with MAP and HR data. Lastly, preliminary leave-one-out analysis showed model accuracy with an RMSE of 6 mm Hg and limit of agreement of ≤ 7 mm Hg., Conclusions: The authors have shown that DCS may enable ICP monitoring with additional clinical validation. The lower risk of such monitoring would allow ICP to be estimated for a wide spectrum of indications, thereby both reducing the use of invasive monitors and increasing the types of patients who may benefit from ICP-directed therapies.

Objective: In mesial temporal lobe epilepsy (MTLE), the ideal surgical approach to achieve seizure freedom and minimize morbidity is an unsolved question. Selective approaches to mesial temporal structures often result in suboptimal seizure outcomes. The authors report the results of a pilot study intended to evaluate the clinical feasibility of using an endoscopic anterior transmaxillary (eATM) approach for minimally invasive management of MTLEs., Methods: The study is a prospectively collected case series of four consecutive patients who underwent the eATM approach for the treatment of MTLE and were followed for a minimum of 12 months. All participants underwent an epilepsy workup and surgical care at a tertiary referral comprehensive epilepsy center and had medically refractory epilepsy. The noninvasive evaluations and intracranial recordings of these patients confirmed the presence of anatomically restricted epileptogenic zones located in the mesial temporal structures. Data on seizure freedom at 1 year, neuropsychological outcomes, diffusion tractography, and adverse events were collected and analyzed., Results: By applying the eATM technique and approaching the far anterior temporal lobe regions, mesial-basal resections of the temporal polar areas and mesial temporal structures were successfully achieved in all patients (2 with left-sided approaches, 2 with right-sided approaches). No neurological complications or neuropsychological declines were observed. All 4 patients achieved Engel class Ia outcome up to the end of the follow-up period (19, 15, 14, and 12 months). One patient developed hypoesthesia in the left V2 distribution but there were no other adverse events. The low degree of white matter injury from the eATM approach was analyzed using high-definition fiber tractography in 1 patient as a putative mechanism for preserving neuropsychological function., Conclusions: The described series demonstrates the feasibility and potential safety profile of a novel approach for medically refractory MTLE. The study affirms the feasibility of performing efficacious mesial temporal lobe resections through an eATM approach.

Objective: Congenital aqueductal stenosis (CAS) is a common etiology of hydrocephalus that occurs in a subset of infants and may be linked to an increased incidence of ophthalmological abnormalities and delayed developmental milestones. Although hydrocephalus is common and widely studied, sparse literature exists on patients with isolated (no identifiable genetic link) CAS along with analysis of ophthalmological manifestations. In this study, the authors sought to describe the ophthalmological abnormalities and delayed developmental milestones of patients with isolated CAS., Methods: Data of patients with CAS were prospectively entered and monitored in a surgical database maintained by the Department of Neurological Surgery at Children's Hospital of Pittsburgh from January 2005 to October 2016. Patients with a family history of congenital hydrocephalus, positive testing for genetic forms of aqueductal stenosis, other congenital abnormalities suggesting an underlying genetic syndrome, and stenosis/obstruction due to secondary causes were excluded from this study. Prenatal and perinatal history, CSF diversion history, and a variety of outcomes, including ophthalmological deficits and developmental milestones, were collected and analyzed., Results: A total of 41 patients with isolated CAS were identified, with a mean follow-up duration of 6 years. Among that cohort, 26 patients (63.4%) developed neuroophthalmological complications, which were further stratified. Fourteen patients (34.1%) developed strabismus and 11 (26.8%) developed astigmatism, and 1 patient (2.4%) with papilledema was recorded. Among patients with ophthalmological abnormalities, 76.9% had delayed developmental milestones (p = 0.045)., Conclusions: Patients with CAS were found to have increased risk of ophthalmological abnormalities requiring correction, along with an increased risk of delayed developmental milestones. Importantly, there was a significant correlation between the development of ophthalmological abnormalities and delayed developmental milestones that was independent of CSF diversion history. Larger patient cohort studies are required to explore whether earlier development of hydrocephalus, as is the case in CAS, causes elevated rates of neurological and ophthalmological complications, and if earlier CSF diversion correlates with improved outcomes.

Endoscopic suturectomy combined with supplementary techniques such as spring-assisted expansion and cranial molding helmets for the correction of craniosynostosis is growing in popularity due to the reduced scar burdened, decreased morbidity, and reduced overall cost. The authors present their technique for the correction of isolated coronal craniosynostosis. The use of dedicated endoscopic tools and lit endoscopes permits enhanced visualization and technical ability, particularly at the distal portions of the suturectomy, and may reduce operative time and cerebrospinal fluid leak risk. The video can be found here: https://vimeo.com/515401366., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2021, The Authors.)

Objective: Myelomeningocele (MMC) is frequently complicated by symptomatic hydrocephalus, necessitating early permanent CSF diversion and revision surgeries. Shunt infections are a common cause of shunt malfunction. This study aims to characterize long-term shunt-related outcomes of patients undergoing MMC closure., Methods: A total of 170 patients undergoing MMC closure between the years of 1995 and 2017 were identified from a retrospective review of a prospectively populated surgical database at the Children's Hospital of Pittsburgh. Patients who underwent MMC closure and required ventriculoperitoneal (VP) shunt insertion met criteria and were included in the primary study analysis. Analysis with a Fisher exact test was performed for categorical variables, and Mann-Whitney U-tests were utilized for numerical data., Results: Of the 158 total patients undergoing MMC closure and meeting inclusion criteria, 137 (87%) required VP shunt insertion. These 137 patients demonstrated a shunt revision rate of 21.1% per person-year and a shunt infection rate of 2.1% per person-year over a mean follow-up of 10.8 years. Patients had a mean of 3.4 ± 0.6 shunt surgeries prior to their first infection. Patients undergoing immediate shunt removal, external ventricular drain placement, or shunt replacement after clearing the infection had lower rates of subsequent infections than patients who initially were managed with shunt externalization (p < 0.001). Placement of a shunt at the time of MMC closure was not found to be a risk factor for infection. Of patients with initial shunt placement after the implementation of the Hydrocephalus Clinical Research Network protocol in 2011, the authors' institution has had a shunt infection rate of 4.2% per person-year and a revision rate of 35.7% per person-year., Conclusions: This study describes long-term outcomes of shunted MMC patients and factors associated with shunt infections. Most patients underwent multiple revisions prior to the first shunt infection. Shunt externalization may be ineffective at clearing the infection and should be avoided in favor of early shunt removal and external ventricular drainage, followed by shunt replacement once infection is demonstrated to have cleared.

Objective: During the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, endoscopic endonasal surgery (EES) is feared to be a high-risk procedure for the transmission of coronavirus disease 2019 (COVID-19). Nonetheless, data are lacking regarding the management of EES during the pandemic. The object of this study was to understand current worldwide practices pertaining to EES for skull base/pituitary tumors during the SARS-CoV-2 pandemic and provide a basis for the formulation of guidelines., Methods: The authors conducted a web-based survey of skull base surgeons worldwide. Different practices by geographic region and COVID-19 prevalence were analyzed., Results: One hundred thirty-five unique responses were collected. Regarding the use of personal protective equipment (PPE), North America reported using more powered air-purifying respirators (PAPRs), and Asia and Europe reported using more standard precautions. North America and Europe resorted more to reverse transcriptase-polymerase chain reaction (RT-PCR) for screening asymptomatic patients. High-prevalence countries showed a higher use of PAPRs. The medium-prevalence group reported lower RT-PCR testing for symptomatic cases, and the high-prevalence group used it significantly more in asymptomatic cases.Nineteen respondents reported transmission of COVID-19 to healthcare personnel during EES, with a higher rate of transmission among countries classified as having a medium prevalence of COVID-19. These specific respondents (medium prevalence) also reported a lower use of airborne PPE. In the cases of healthcare transmission, the patient was reportedly asymptomatic 32% of the time., Conclusions: This survey gives an overview of EES practices during the SARS-CoV-2 pandemic. Intensified preoperative screening, even in asymptomatic patients, RT-PCR for all symptomatic cases, and an increased use of airborne PPE is associated with decreased reports of COVID-19 transmission during EES.

Objective: Pediatric skull base chordoma is a rare entity that is traditionally considered to display aggressive behavior with an increased risk of recurrence. There is an absence of literature examining outcomes in the pediatric population in general and using the endoscopic endonasal approach (EEA)., Methods: The authors retrospectively reviewed all patients with skull base chordomas presenting by the age of 18 years to the Children's Hospital of Pittsburgh or the University of Pittsburgh Medical Center from 2004 to 2019. Clinical outcomes, the number and location of recurrences, and progression-free survival time were determined., Results: Twenty patients met the study criteria. The most common presenting complaints were diplopia (n = 7), headache (n = 6), and swallowing difficulty (n = 4). Three cases were incidentally discovered. Twelve patients underwent single-stage EEA alone, 2 patients had two-stage EEA, and 6 patients had combined EEA with open far-lateral or extreme-lateral approaches. Fourteen patients underwent gross-total resection (GTR), and 6 patients had near-total resection. Larger tumors were more likely to require staging or a combined approach (86% vs 7%) and were less likely to receive GTR (33% vs 86%) but had comparable recurrence and mortality rates. Five patients developed CSF leaks requiring reoperation, 2 patients developed a permanent abducens nerve palsy, 1 patient suffered an internal carotid artery injury, 1 patient developed an epidural hematoma, and 1 patient developed a subdural empyema. Four (20%) patients had recurrence during follow-up (mean radiographic follow-up 59 months and mean time to local recurrence 19 months). Two patients with recurrence underwent further resection, and 1 patient elected to stop treatment. Both patients who underwent repeat resection experienced a second recurrence, one of whom elected to stop treatment. Both patients who died had an elevated Ki-67 (p = 0.039), one of whom developed de-differentiated histology. A third patient died of progressive spinal metastases without local recurrence and is one of 2 patients who developed postoperative spinal metastases. Both patients whose tumors became de-differentiated progressed from tumors with an initial Ki-67 of 15 or greater (p = 0.035) and received prior radiotherapy to the bulk tumor (p = 0.03)., Conclusions: The majority of pediatric skull base chordomas, when managed at a specialized center with a goal of GTR, may have a better outcome than traditionally believed. Elevated Ki-67 rates may predict poor outcome and progression to de-differentiation.

Objective: The study of pediatric arteriovenous malformations (pAVMs) is complicated by the rarity of the entity. Treatment choice has often been affected by the availability of different modalities and the experience of the providers present. The University of Pittsburgh experience of multimodality treatment of pAVMs is presented., Methods: The authors conducted a retrospective cohort study examining 212 patients with pAVM presenting to the University of Pittsburgh between 1988 and 2018, during which patients had access to surgical, endovascular, and radiosurgical options. Univariate analysis was performed comparing good and poor outcomes. A poor outcome was defined as a modified Rankin Scale (mRS) score of ≥ 3. Multivariate analysis via logistic regression was performed on appropriate variables with a p value of ≤ 0.2. Seventy-five percent of the cohort had at least 3 years of follow-up., Results: Five patients (2.4%) did not receive any intervention, 131 (61.8%) had GKRS alone, 14 (6.6%) had craniotomies alone, and 2 (0.9%) had embolization alone. Twenty-two (10.4%) had embolization and Gamma Knife radiosurgery (GKRS); 20 (9.4%) had craniotomies and GKRS; 8 (3.8%) had embolization and craniotomies; and 10 (4.7%) had embolization, craniotomies, and GKRS. Thirty-one patients (14.6%) were found to have poor outcome on follow-up. The multivariate analysis performed in patients with poor outcomes was notable for associations with no treatment (OR 18.9, p = 0.02), hemorrhage requiring craniotomy for decompression alone (OR 6, p = 0.03), preoperative mRS score (OR 2.1, p = 0.004), and Spetzler-Martin score (OR 1.8, p = 0.0005). The mean follow-up was 79.7 ± 62.1 months. The confirmed radiographic obliteration rate was 79.4% and there were 5 recurrences found on average 9.5 years after treatment., Conclusions: High rates of long-term functional independence (mRS score of ≤ 2) can be achieved with comprehensive multimodality treatment of pAVMs. At this center there was no difference in outcome based on treatment choice when accounting for factors such as Spetzler-Martin grade and presenting morbidity. Recurrences are rare but frequently occur years after treatment, emphasizing the need for long-term screening after obliteration.

This is the case of a 76-year-old woman presenting with progressive right vision loss consisting of a right eye temporal field cut and severe visual acuity loss. An MRI was performed showing a suprasellar mass for which she had been referred to our center for an endoscopic endonasal approach. The tumor was found to be densely adherent to adjacent structures, and an ophthalmic artery and A1-A2 junction injury were sustained during resection. The management of intraoperative vascular injuries is described. The video can be found here: https://youtu.be/JJY6nYKTCSg., Competing Interests: Disclosures The author reports no conflict of interest concerning the materials or methods used in this study or the findings specified in this article., (© 2020, The Authors.)

Awake craniotomies are a crucial tool for identifying eloquent cortex, but significant limitations frequently related to patient tolerance have limited their applicability in pediatric cases. The authors describe a comprehensive, longitudinal protocol developed in collaboration with a certified child life specialist (CCLS) in order to enhance patient experiences and develop resiliency related to the intraoperative portion of cases. This protocol includes preoperative conditioning, intraoperative support, and postoperative positive reinforcement and debriefing. A unique coping plan is developed for each prospective patient. With appropriate support, awake craniotomy may be applicable in a wider array of preadolescent and adolescent patients than has previously been possible. Future prospective studies are needed to validate this approach.

Objective: The authors conducted a study to identify clinical features of cranial base chondrosarcomas that will predict tumor progression after resection., Methods: The authors performed a retrospective study at a tertiary referral cranial base center. Patients who underwent resection of cranial base chondrosarcomas between January 2004 and December 2018 were followed longitudinally. The main outcome measure was progression-free survival (PFS)., Results: A total of 41 patients were treated for histopathologically proven "conventional" cranial base chondrosarcomas during the study period, and the median PFS was 123 months for the cohort. Univariate analysis was performed on clinical, anatomical, and radiographic parameters collected for each patient. Features that were statistically significant were fed into a multivariate regression model, which revealed two independent predictors of PFS: patient age and encasement of 3-4 major arteries (> 25% of the vessel wall surrounded by tumor). Using these two variables of age and multiple arterial vessel encasement, the authors generated a risk stratification model using a simple point system to predict PFS in patients with cranial base chondrosarcomas. Based on these two factors known preoperatively, this model could stratify patients into high-risk (10% of patients), intermediate-risk (68% of patients), and low-risk (22% of patients) subgroups corresponding to dramatically distinct median PFS (1.8 years, 10.2 years, and no progression, respectively)., Conclusions: In patients with cranial base chondrosarcomas, age and artery encasement are variables known preoperatively that can powerfully predict tumor progression, define operative goals, and aid in selecting postoperative adjuvant therapy.

Craniocervical instability due to chronic atlantoaxial dissociation presents the challenge of providing adequate decompression, reduction, and fixation to promote long-term stability while avoiding iatrogenic vertebral artery dissection or entrapment. The authors present one patient with chronic atlantoaxial dissociation and basilar invagination treated via Goel's technique and with bilateral vertebral artery mobilization. There was substantial decompression and reduction postoperatively and the patient was discharged with a stable examination. Vertebral artery mobilization at the C1-2 junction can be safely performed via a standard midline suboccipital incision and dissection without vertebral artery injury. The video can be found here: https://youtu.be/VS1Mt1dBLO4., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this publication., (© 2020, Michael M. McDowell, Andrew Venteicher, Ezequiel Goldschmidt, Maximiliano Nuñez, David O. Okonkwo, and Paul A. Gardner.)

Aneurysmal rupture can result in devastating neurological consequences and can be complicated by comorbid disease processes. Patients with autosomal recessive polycystic kidney disease (ARPKD) have a low rate of reported aneurysms, but this may be due to the relative high rate of end-stage illnesses early in childhood. Authors here report the case of a 10-year-old boy with ARPKD who presented with a Hunt and Hess grade V subarachnoid hemorrhage requiring emergency ventriculostomy, embolization, and decompressive craniectomy. Despite initial improvements in his neurological status, the patient succumbed to hepatic failure. Given the catastrophic outcomes of subarachnoid hemorrhage in young patients, early radiographic screening in those with ARPKD may be warranted.

OBJECTIVE Chiari malformation type II (CM-II) in myelomeningocele is associated with a significant rate of mortality and poor outcome. Death is frequently heralded by the onset or progression of neurological symptoms. The authors sought to identify predictors of poor outcome and mortality within the myelomeningocele population at Children's Hospital of Pittsburgh. METHODS A retrospective chart and radiology review was performed on all infants who underwent primary closure of a myelomeningocele defect at Children's Hospital of Pittsburgh between the years of 1995 and 2015. Preoperative symptoms and signs leading to CM-II decompression, as well as operative details and postoperative changes in these symptoms and signs, were investigated in detail and correlated to outcome. Poor outcome was defined as death, stridor, or ventilator dependence. Deceased patients were separately assessed within this subgroup. RESULTS Thirty-two (21%) of 153 patients were found to have symptomatic CM-II. Of the 32 patients meeting inclusion criteria, 12 (38%) had poor outcomes. Eight patients (25%) died since initial presentation; 5 of these patients (16% of the overall cohort) died within the 1st year of life and 3 (9%) died during adolescence. Seven (88%) of the 8 patients who died had central apnea on presentation (p = 0.001) and 7 (44%) of the 16 patients who developed symptoms in the first 3 months of life died, compared with 1 (6.3%) of 16 who developed symptoms later in childhood (p = 0.04). The median Apgar score at 1 minute was 4.5 for patients who died and 8 for surviving patients (p = 0.006). The median diameter of the myelomeningocele defect was 5.75 cm for patients who died and 5 for those who survived (p = 0.01). The anatomical level of defect trended toward higher levels in patients who died, with 4 patients in that group having an anatomical level at L-2 or higher compared with 5 of the surviving patients (p = 0.001). The median initial head circumference for the 5 patients dying in the 1st year of life was 41.5 cm, versus 34 cm for all other patients (p = 0.01). CONCLUSIONS CM-II in spina bifida is associated with a significant mortality rate even when surgical intervention is performed. Death is more frequent in symptomatic patients presenting prior to 1 year of age. Late deaths are associated with symptom progression despite aggressive surgical and medical intervention. In this patient cohort, death was more likely in patients with symptomatic presentation during the first 3 months of life, low Apgar scores, large myelomeningocele defects, early central apnea, and large head circumference at birth.

Objective: Ventricular shunt (VS) durability has been well studied in the pediatric population and in patients with normal pressure hydrocephalus; however, further evaluation in a more heterogeneous adult population is needed. This study aims to evaluate the effect of diagnosis and valve type-fixed versus programmable-on shunt durability and cost for placement of shunts in adult patients., Methods: The authors retrospectively reviewed the medical records of all patients who underwent implantation of a VS for hydrocephalus at their institution over a 3-year period between August 2013 and October 2016 with a minimum postoperative follow-up of 6 months. The primary outcome was shunt revision, which was defined as reoperation for any indication after the initial procedure. Supply costs, shunt durability, and hydrocephalus etiologies were compared between fixed and programmable valves., Results: A total of 417 patients underwent shunt placement during the index time frame, consisting of 62 fixed shunts (15%) and 355 programmable shunts (85%). The mean follow-up was 30 ± 12 (SD) months. The shunt revision rate was 22% for programmable pressure valves and 21% for fixed pressure valves (HR 1.1 [95% CI 0.6-1.8]). Shunt complications, such as valve failure, infection, and overdrainage, occurred with similar frequency across valve types. Kaplan-Meier survival curve analysis showed no difference in durability between fixed (mean 39 months) and programmable (mean 40 months) shunts (p = 0.980, log-rank test). The median shunt supply cost per index case and accounting for subsequent revisions was $3438 (interquartile range $2938-$3876) and $1504 (interquartile range $753-$1584) for programmable and fixed shunts, respectively (p < 0.001, Wilcoxon rank-sum test). Of all hydrocephalus etiologies, pseudotumor cerebri (HR 1.9 [95% CI 1.2-3.1]) and previous shunt malfunction (HR 1.8 [95% CI 1.2-2.7]) were found to significantly increase the risk of shunt revision. Within each diagnosis, there were no significant differences in revision rates between shunts with a fixed valve and shunts with a programmable valve., Conclusions: Long-term shunt revision rates are similar for fixed and programmable shunt pressure valves in adult patients. Hydrocephalus etiology may play a significant role in predicting shunt revision, although programmable valves incur higher supply costs regardless of initial diagnosis. Utilization of fixed pressure valves versus programmable pressure valves may reduce supply costs while maintaining similar revision rates. Given the importance of developing cost-effective management protocols, this study highlights the critical need for large-scale prospective observational studies and randomized clinical trials of ventricular shunt valve revisions and additional patient-centered outcomes.

OBJECTIVE Pathophysiological differences that underlie the development and subsequent growth of multiple aneurysms may exist. In this study, the authors assessed the factors associated with the occurrence of multiple aneurysms in patients presenting with aneurysmal subarachnoid hemorrhage (SAH). METHODS Consecutive patients presenting with aneurysmal SAH between 1996 and 2012 were prospectively enrolled in the Subarachnoid Hemorrhage Outcome Project. Patients harboring 1, 2, or 3 or more aneurysms were stratified into groups, and the clinical and radiological characteristics of each group were compared using multivariate logistic regression. RESULTS Of 1277 patients with ruptured intracranial aneurysms, 890 had 1 aneurysm, 267 had 2 aneurysms, and 120 had 3 or more aneurysms. On multinomial regression using the single-aneurysm cohort as base case, risk factors for patients presenting with 2 aneurysms were female sex (relative risk ratio [RRR] 1.80, p < 0.001), higher body mass index (BMI) (RRR 1.02, p = 0.003), more years of smoking (RRR = 1.01, p = 0.004), and black race (RRR 1.83, p = 0.001). The risk factors for patients presenting with 3 or more aneurysms were female sex (RRR 3.10, p < 0.001), higher BMI (RRR 1.03, p < 0.001), aneurysm in the posterior circulation (RRR 2.59, p < 0.001), and black race (RRR 2.15, p = 0.001). Female sex, longer smoking history, aneurysms in the posterior circulation, BMI, and black race were independently associated with the development of multiple aneurysms in our adjusted multivariate multinomial model. CONCLUSIONS Significant demographic and clinical differences are found between patients presenting with single and multiple aneurysms in the setting of aneurysmal SAH. These predictors of multiple aneurysms likely reflect a predisposition toward inflammation and endothelial injury.

OBJECTIVE Lateral lumbar interbody fusion (LLIF) is a less invasive surgical option commonly used for a variety of spinal conditions, including in high-risk patient populations. LLIF is often performed as a stand-alone procedure, and may be complicated by graft subsidence, the clinical ramifications of which remain unclear. The aim of this study was to characterize further the sequelae of graft subsidence following stand-alone LLIF. METHODS A retrospective review of prospectively collected data was conducted on consecutive patients who underwent stand-alone LLIF between July 2008 and June 2015; 297 patients (623 levels) met inclusion criteria. Imaging studies were examined to grade graft subsidence according to Marchi criteria, and compared between those who required revision surgery and those who did not. Additional variables recorded included levels fused, DEXA (dual-energy x-ray absorptiometry) T-score, body mass index, and routine demographic information. The data were analyzed using the Student t-test, chi-square analysis, and logistic regression analysis to identify potential confounding factors. RESULTS Of 297 patients, 34 (11.4%) had radiographic evidence of subsidence and 18 (6.1%) required revision surgery. The median subsidence grade for patients requiring revision surgery was 2.5, compared with 1 for those who did not. Chi-square analysis revealed a significantly higher incidence of revision surgery in patients with high-grade subsidence compared with those with low-grade subsidence. Seven of 18 patients (38.9%) requiring revision surgery suffered a vertebral body fracture. High-grade subsidence was a significant predictor of the need for revision surgery (p < 0.05; OR 12, 95% CI 1.29-13.6), whereas age, body mass index, T-score, and number of levels fused were not. This relationship remained significant despite adjustment for the other variables (OR 14.4; 95% CI 1.30-15.9). CONCLUSIONS In this series, more than half of the patients who developed graft subsidence following stand-alone LLIF required revision surgery. When evaluating patients for LLIF, supplemental instrumentation should be considered during the index surgery in patients with a significant risk of graft subsidence.

Nivolumab is an immune checkpoint inhibitor (ICI) currently undergoing Phase III clinical trials for the treatment of glioblastoma. The authors present the case of a 10-year-old girl with glioblastoma treated with nivolumab under compassionate-use guidelines. After the first dose of nivolumab the patient developed hemiparesis, cerebral edema, and significant midline shift due to severe tumor necrosis. She was managed using intravenous dexamethasone and discharged on a dexamethasone taper. The patient's condition rapidly deteriorated after the second dose of nivolumab, demonstrating hemiplegia, seizures, and eventually unresponsiveness with a fixed and dilated left pupil. Computed tomography of her brain revealed malignant cerebral edema requiring emergency decompressive hemicraniectomy. Repeat imaging demonstrated increased size of the lesion, reflecting immune-mediated inflammation and tumor necrosis. The patient remained densely hemiplegic, but became progressively more interactive and was ultimately extubated. She resumed nivolumab several weeks later, but again her condition deteriorated with headache, vomiting, swelling at the craniectomy site, and limited right-sided facial movement following the sixth dose. MRI demonstrated severe midline shift and uncal herniation despite her craniectomy. Her condition gradually declined, and she died several days later under "do not resuscitate/do not intubate" orders. To the authors' knowledge, this represents the first case of malignant cerebral edema requiring operative intervention following nivolumab treatment for glioblastoma in a pediatric patient.

OBJECTIVE Increased survival time after diagnosis of neoplastic disease has resulted in a gradual increase in spine tumor incidence. Radiosurgery is frequently a viable alternative to operative management in a population with severe medical comorbidities. The authors sought to assess the histopathological consequences of radiosurgery in the subset of patients progressing to operative intervention. METHODS Eighteen patients who underwent radiosurgery for spine tumors between 2008 and 2014 subsequently progressed to surgical treatment. A histopathological examination of these cases was performed. Indications for surgery included symptomatic compression fractures, radiographic instability, and symptoms of cord or cauda equina compression. Biopsy samples were obtained from the tumor within the radiosurgical zone in all cases and were permanently fixated. Viable tumor samples were stained for Ki 67. RESULTS Fifteen patients had metastatic lesions and 3 patients had neurofibromas. The mean patient age was 57 years. The operative indication was symptomatic compression in 10 cases (67%). The most frequent metastatic lesions were breast cancer (4 cases), renal cell carcinoma (3), prostate cancer (2), and endometrial cancer (2). In 9 (60%) of the 15 metastatic cases, histological examination of the lesions showed minimal evidence of inflammation. Viable tumor at the margins of the radiosurgery was seen in 9 (60%) of the metastatic cases. Necrosis in the tumor bed was frequent, as was fibrotic bone marrow. Vascular ectasia was seen in 2 of 15 metastatic cases, but sclerosis with ectasia was frequent. No evidence of malignant conversion was seen in the periphery of the lesions in the 3 neurofibroma cases. In 1 case of neurofibroma, the lesion demonstrated some small areas of remnant tumor in the radiosurgical target zone. CONCLUSIONS This case series demonstrates important histopathological characteristics of spinal lesions treated by SRS. Regions with the highest exposure to radiation appear to be densely necrotic and show little evidence of tumor growth, whereas peripheral regions distant from the radiation dosage are more likely to demonstrate viable tumor in malignant and benign neoplasms. Physiological tissue appears to be similarly affected. With additional investigation, a more homogenized field of hypofractionated radiation exposure may allow for tumor obliteration with relative preservation of critical anatomical structures.

OBJECT The long-term consequences of atlantoaxial (AA) and occipitocervical (OC) fusion and instrumentation in young children are unknown. Anecdotal reports have raised concerns regarding altered growth and alignment of the cervical spine after surgical intervention. The purpose of this study was to determine the long-term effects of these surgeries on the growth and alignment of the maturing spine. METHODS A multiinstitutional retrospective chart review was conducted for patients less than or equal to 6 years of age who underwent OC or AA fusion with rigid instrumentation at 9 participating centers. All patients had at least 3 years of clinical and radiographic follow-up data and radiographically confirmed fusion. Preoperative, immediate postoperative, and most recent follow-up radiographs and/or CT scans were evaluated to assess changes in spinal growth and alignment. RESULTS Forty children (9 who underwent AA fusion and 31 who underwent OC fusion) were included in the study (mean follow-up duration 56 months). The mean vertical growth over the fused levels in the AA fusion patients represented 30% of the growth of the cervical spine (range 10%-50%). Three different vertical growth patterns of the fusion construct developed among the 31 OC fusion patients during the follow-up period: 1) 16 patients had substantial growth (13%-46% of the total growth of the cervical spine); 2) 9 patients had no meaningful growth; and 3) 6 patients, most of whom presented with a distracted atlantooccipital dislocation, had a decrease in the height of the fused levels (range 7-23 mm). Regarding spinal alignment, 85% (34/40) of the patients had good alignment at follow-up, with straight or mildly lordotic cervical curvatures. In 1 AA fusion patient (11%) and 5 OC fusion patients (16%), we observed new hyperlordosis (range 43°-62°). There were no cases of new kyphosis or swan-neck deformity, evidence of subaxial instability, or unintended subaxial fusion. No preoperative predictors of these growth patterns or alignment were evident. CONCLUSIONS These results demonstrate that most young children undergoing AA and OC fusion with rigid internal fixation continue to have good cervical alignment and continued growth within the fused levels during a prolonged follow-up period. However, some variability in vertical growth and alignment exists, highlighting the need to continue close long-term follow-up.

OBJECT Chiari malformation Type I (CM-I) is associated with a syrinx in 25%-85% of patients. Although posterior fossa decompression (PFD) without dural opening is an accepted treatment option for children with symptomatic CM-I, many surgeons prefer to open the dura if a syrinx exists. The purpose of this study was to investigate the frequency and timing of syrinx resolution in children undergoing PFD without dural opening for CM-I. METHODS A retrospective review of 68 consecutive pediatric patients with CM-I and syringomyelia who underwent PFD without dural opening was conducted. Patient demographics, presenting symptoms and signs, radiographic findings, and intraoperative ultrasound and neuromonitoring findings were studied as well as the patients' clinical and radiographic follow-up. RESULTS During the mean radiographic follow-up period of 32 months, 70% of the syringes improved. Syrinx improvement occurred at a mean of 31 months postoperatively. All patients experienced symptom improvement within the 1st year, despite only 26% of patients showing radiographic improvement during that period. Patients presenting with sensory symptoms or motor weakness had a higher likelihood of having radiographic syrinx improvement postoperatively. CONCLUSIONS In children with CM-I and a syrinx undergoing PFD without dural opening, syrinx resolution occurs in approximately 70% of patients. Radiographic improvement of the syrinx is delayed, but this does not correlate temporally with symptom improvement. Sensory symptoms or motor weakness on presentation are associated with syrinx resolution after surgery.

OBJECT Symptomatic pediatric Chiari malformation Type I (CM-I) is most often treated with posterior fossa decompression (PFD), but controversy exists over whether the dura needs to be opened during PFD. While dural opening as a part of PFD has been suggested to result in a higher rate of resolution of CM symptoms, it has also been shown to lead to more frequent complications. In this paper, the authors present the largest reported series of outcomes after PFD without dural opening surgery, as well as identify risk factors for recurrence. METHODS The authors performed a retrospective review of 156 consecutive pediatric patients in whom the senior authors performed PFD without dural opening from 2003 to 2013. Patient demographics, clinical symptoms and signs, radiographic findings, intraoperative ultrasound results, and neuromonitoring findings were reviewed. Univariate and multivariate regression analyses were performed to determine risk factors for recurrence of symptoms and the need for reoperation. RESULTS Over 90% of patients had a good clinical outcome, with improvement or resolution of their symptoms at last follow-up (mean 32 months). There were no major complications. The mean length of hospital stay was 2.0 days. In a multivariate regression model, partial C-2 laminectomy was an independent risk factor associated with reoperation (p = 0.037). Motor weakness on presentation was also associated with reoperation but only with trend-level significance (p = 0.075). No patient with < 8 mm of tonsillar herniation required reoperation. CONCLUSIONS The vast majority (> 90%) of children with symptomatic CM-I will have improvement or resolution of symptoms after a PFD without dural opening. A non-dural opening approach avoids major complications. While no patient with tonsillar herniation < 8 mm required reoperation, children with tonsillar herniation at or below C-2 have a higher risk for failure when this approach is used.

Object: The significance of draining vein anatomy is poorly defined in pediatric arteriovenous malformations (AVMs). In adult cohorts, the presence of fewer veins has been shown to lead to an increased rate of hemorrhage, but this phenomenon has not yet been studied in pediatric AVMs. This report analyzes the impact of draining vein anatomy on presentation and outcome in a large series of pediatric AVMs., Methods: Eighty-five pediatric patients with AVMs were treated at the Columbia University Medical Center between 1991 and 2012. Charts were retrospectively reviewed for patient characteristics, clinical course, neurological outcome, and AVM angioarchitectural features identified on the angiogram performed at presentation. Univariate analyses were performed using chi-square test and ANOVA when appropriate; multivariate analysis was performed using logistic regression., Results: Four patients were excluded due to incomplete records. Twenty-seven patients had 2 or 3 draining veins; 12 (44.4%) of these patients suffered from hemorrhage prior to surgery. Fifty-four patients had 1 draining vein; 39 (72.2%) of these 54 suffered from hemorrhage. Independent predictors of hemorrhage included the presence of a single draining vein (p = 0.04) and deep venous drainage (p = 0.02). Good outcome (modified Rankin Scale [mRS] score < 3) on discharge was found to be associated with higher admission Glasgow Coma Scale (GCS) scores (p = 0.0001, OR 0.638, 95% CI 0.40-0.93). Poor outcome (mRS score > 2) on discharge was found to be associated with deep venous drainage (p = 0.04, OR 4.68, 95% CI 1.1-19.98). A higher admission GCS score was associated with a lower discharge mRS score (p = 0.0003, OR 0.6, 95% CI 0.46-0.79), and the presence of a single draining vein was associated with a lower mRS score on long-term follow-up (p = 0.04, OR 0.18, 95% CI 0.032-0.99)., Conclusions: The authors' data suggest that the presence of a single draining vein or deep venous drainage plays a role in hemorrhage risk and ultimate outcome in pediatric AVMs. Small AVMs with a single or deep draining vein may have the highest risk of hemorrhage.

Object: Approximately 25% of patients exhibit cognitive dysfunction 24 hours after carotid endarterectomy (CEA). One of the purported mechanisms of early cognitive dysfunction (eCD) is hypoperfusion due to inadequate collateral circulation during cross-clamping of the carotid artery. The authors assessed whether poor collateral circulation within the circle of Willis, as determined by preoperative CT angiography (CTA) or MR angiography (MRA), could predict eCD., Methods: Patients who underwent CEA after preoperative MRA or CTA imaging and full neuropsychometric evaluation were included in this study (n = 42); 4 patients were excluded due to intraoperative electroencephalographic changes and subsequent shunt placement. Thirty-eight patients were included in the statistical analyses. Patients were stratified according to posterior communicating artery (PCoA) status (radiographic visualization of at least 1 PCoA vs of no PCoAs). Variables with p < 0.20 in univariate analyses were included in a stepwise multivariate logistic regression model to identify predictors of eCD after CEA., Results: Overall, 23.7% of patients exhibited eCD. In the final multivariate logistic regression model, radiographic absence of both PCoAs was the only independent predictor of eCD (OR 9.64, 95% CI 1.43-64.92, p = 0.02)., Conclusions: The absence of both PCoAs on preoperative radiographic imaging is predictive of eCD after CEA. This finding supports the evidence for an underlying ischemic etiology of eCD. Larger studies are justified to verify the findings of this study. Clinical trial registration no.: NCT00597883 ( http://www.clinicaltrials.gov ).

The authors report on the surgical management of an extensive lesion of the upper cervical spine that required an uncommon transmandibular approach to facilitate exposure, resection, and stabilization in a pediatric patient. A 6-year-old boy with a large aneurysmal bone cyst of the C-2 vertebra presented with progressive weakness and right-sided neck pain. The lesion extended laterally into the soft tissue of the neck, inferiorly to C-4, and posteriorly around the spinal cord. A transmandibular osteotomy was performed to provide adequate exposure for complete resection of the mass and anterior C1-3 instrumentation and fusion. Subsequently, the patient underwent occiput to C-4 posterior instrumentation and fusion. The patient tolerated the operation well and had regained all function at 3 and 11 months' follow-up. No neurological complications or problems of speech, swallowing, or respiration occurred. Even in pediatric patients, the transmandibular approach for the treatment of upper cervical spine lesions is an effective method of maximizing exposure for complex lesions requiring resection and stabilization.

Object: Ventriculostomy--the placement of an external ventricular drain (EVD)--is a common procedure performed in patients with acute neurological injury. Although generally considered a low-risk intervention, recent studies have cited higher rates of hemorrhagic complications than those previously reported. The authors sought to determine the rate of postventriculostomy hemorrhage in a cohort of patients with intracerebral hemorrhage (ICH) and to identify predictors of hemorrhagic complications of EVD placement., Methods: Patients with ICH who underwent EVD placement and had both pre- and postprocedural imaging available for analysis were included in this study. Relevant data were prospectively collected for each patient who satisfied inclusion criteria. Variables with a p < 0.20 on univariate analyses were included in a stepwise logistic regression model to identify predictors of postventriculostomy hemorrhage., Results: Sixty-nine patients were eligible for this analysis. Postventriculostomy hemorrhage occurred in 31.9% of patients. Among all patients with intraparenchymal hemorrhage, the mean hemorrhage volume was 0.66 ± 1.06 cm(3). Stratified according to ventricular catheter diameter, patients treated with smaller-diameter catheters had a significantly greater mean hemorrhage volume than patients treated with larger-diameter catheters (0.84 ± 1.2 cm(3) vs 0.14 ± 0.12 cm(3), p = 0.049). Postventriculostomy hemorrhage was clinically significant in only 1 patient (1.4%). Overall, postventriculostomy hemorrhage was not associated with functional outcome or mortality at either discharge or 90 days. In the multivariate model, an age > 75 years was the only independent predictor of EVD-associated hemorrhage., Conclusions: Advanced age is predictive of EVD-related hemorrhage in patients with ICH. While postventriculostomy hemorrhage is common, it appears to be of minor clinical significance in the majority of patients.

Object: Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature., Methods: The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes., Results: The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003)., Conclusions: Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.

Object: Randomized clinical trials have established that lumbar selective dorsal rhizotomy (SDR) reduces lower-extremity tone and improves functional outcome in children with spastic cerebral palsy. Significant data exist to support a secondary effect on upper-extremity function in patients with upper-extremity spasticity. The effects of SDR on upper-extremity tone, however, are not well characterized. In this report, the authors sought to assess changes in upper-extremity tone in individual muscle groups after SDR and tried to determine if these changes could be predicted preoperatively., Methods: The authors retrospectively reviewed 42 children who underwent SDR at Columbia University Medical Center/Morgan Stanley Children's Hospital of NewYork-Presbyterian between 2005 and 2011. Twenty-five had upper-extremity spasticity. All underwent pre- and postoperative examination for measuring tone (Modified Ashworth Scale) and assessing functional outcome. Follow-up examinations with therapists were performed at least once at a minimum of 2 months postoperatively (mean 15 months)., Results: In the upper extremities, 23 (92%) of 25 patients had improvements of at least 1 Ashworth point in 2 or more independent motor groups on the Modified Ashworth Scale, and 12 (71%) of 17 families surveyed reported increases in motor control or spontaneous movement. The mean Modified Ashworth Scale scores for all upper-extremity muscle groups demonstrated an improvement from 1.34 to 1.22 (p < 0.001). Patients with a mean preoperative upper-extremity tone of 1.25-1.75 were most likely to benefit from reduction in tone (p = 0.0019). Proximal and pronator muscle groups were most likely to demonstrate reduced tone., Conclusions: In addition to improvements in lower-extremity tone and function, SDR has demonstrable effects on upper extremities. Greater than 90% of our patients with elevated upper-extremity tone demonstrated reduction in tone in at least 2 muscle groups postoperatively. Patients with a mean Modified Ashworth Scale upper-extremity score of 1.25-1.75 may encounter the greatest reduction in upper-extremity tone.

The endoscopic transnasal approach to the rostral pediatric spine and craniovertebral junction is a relatively new technique that provides an alternative to the traditional transoral approach to the anterior pediatric spine. In this case series, the authors provide 2 additional examples of patients undergoing endoscopic transnasal odontoidectomies for ventral decompression of the spinal cord. Both patients would have required transection of the palate to undergo an effective transoral operation, which can be a cause of significant morbidity. In one case, transnasal decompression was initially incomplete, and decompression was successfully achieved via a second endoscopic transnasal operation. Both cases resulted in significant neurological recovery and stable long-term spinal alignment. The transnasal approach benefits from entering into the posterior pharynx at an angle that often reduces the length of postoperative intubation and may speed a patient's return to oral intake. Higher reoperation rates are a concern for many endoscopic approaches, but there are insufficient data to conclude if this is the case for this procedure. Further experience with this technique will provide a better understanding of the indications for which it is most effective. Transcervical and transoral endoscopic approaches have also been reported and provide additional options for pediatric anterior cervical spine surgery.

Intracerebral hemorrhage (ICH) is the most deadly and least treatable subtype of stroke, and at the present time there are no evidence-based therapeutic interventions for patients with this disease. Secondary injury mechanisms are known to cause substantial rates of morbidity and mortality following ICH, and the inflammatory cascade is a major contributor to this post-ICH secondary injury. The alpha-7 nicotinic acetylcholine receptor (α7-nAChR) agonists have a well-established antiinflammatory effect and have been shown to attenuate perihematomal edema volume and to improve functional outcome in experimental ICH. The authors evaluate the current evidence for the use of an α7-nAChR agonist as a novel therapeutic agent in patients with ICH.

Object: Large intracerebral hemorrhage (ICH), compounded by perihematomal edema, can produce severe elevations of intracranial pressure (ICP). Decompressive hemicraniectomy (DHC) with or without clot evacuation has been considered a part of the armamentarium of treatment options for these patients. The authors sought to assess the preliminary utility of DHC without evacuation for ICH in patients with supratentorial, dominant-sided lesions., Methods: From September 2009 to May 2012, patients with ICH who were admitted to the neurological ICU at Columbia University Medical Center were prospectively enrolled in that institution's ICH Outcomes Project (ICHOP). Five patients with spontaneous supratentorial dominant-sided ICH underwent DHC without clot evacuation for recalcitrant elevated ICP. Data pertaining to the patients' characteristics and outcomes of treatment were prospectively collected., Results: The patients' median age was 43 years (range 30-55 years) and the ICH etiology was hypertension in 4 of 5 patients, and systemic lupus erythematosus vasculitis in 1 patient. On admission, the median Glasgow Coma Scale (GCS) score was 7 (range 5-9). The median ICH volume was 53 cm(3) (range 28-79 cm(3)), and the median midline shift was 7.6 mm (range 3.0-11.3 mm). One day after surgery, the median decrease in midline shift was 2.7 mm (range 1.5-4.6 mm), and the median change in GCS score was +1 (range -3 to +5). At discharge, all patients were still alive, and the median GCS score was 10 (range 9-11), the median modified Rankin Scale (mRS) score was 5 (range 5-5), and the median NIHSS (National Institutes of Health Stroke Scale) score was 22 (range 17-27). Six months after hemorrhage, 1 patient had died, 2 were functionally dependent (mRS Score 4-5), and 2 were functionally independent (mRS Score 0-3). Outcomes for the patients treated with DHC were good compared with 1) outcomes for all patients with spontaneous supratentorial ICH admitted during the same period (n = 144) and 2) outcomes for matched patients (dominant ICH, GCS Score 5-9, ICH volume 28-79 cm(3), age < 60 years) whose cases were managed nonoperatively (n = 5)., Conclusions: Decompressive hemicraniectomy without clot evacuation appears feasible in patients with large ICH and deserves further investigation, preferably in a randomized controlled setting.

In this report, the authors sought to summarize existing literature to provide an overview of the currently available techniques and to critically assess the evidence for or against their application in intracerebral hemorrhage (ICH) for management, prognostication, and research. Functional imaging in ICH represents a potential major step forward in the ability of physicians to assess patients suffering from this devastating illness due to the advantages over standing imaging modalities focused on general tissue structure alone, but its use is highly controversial due to the relative paucity of literature and the lack of consolidation of the predominantly small data sets that are currently in existence. Current data support that diffusion tensor imaging and tractography, diffusion-perfusion weighted MRI techniques, and functional MRI all possess major potential in the areas of highlighting motor deficits, motor recovery, and network reorganization. Novel clinical studies designed to objectively assess the value of each of these modalities on a wider scale in conjunction with other methods of investigation and management will allow for their rapid incorporation into standard practice.

Object: Conventional cerebral angiography and treatment for ruptured arteriovenous malformations (AVMs) in children are often performed in a delayed fashion. In adults, current literature suggests that AVM-associated aneurysms may be more likely to hemorrhage than isolated AVMs, which often leads to earlier angiography and endovascular treatment of associated aneurysms. The nature of AVM-associated aneurysms in the pediatric population is virtually unknown. In this report, the authors investigate the relationship of associated aneurysms in a large group of children with AVMs., Methods: Seventy-seven pediatric patients (≤ 21 years old) with AVMs were treated at the Columbia University Medical Center between 1991 and 2010. Medical records and imaging studies were retrospectively reviewed, and associated aneurysms were classified as arterial, intranidal, or venous in location. Clinical presentation and outcome variables were compared between children with and without AVM-associated aneurysms., Results: A total of 30 AVM-associated aneurysms were found in 22 children (29% incidence). Eleven were arterial, 9 intranidal, and 10 were venous in location. There was no significant difference in the rate of hemorrhage (p = 0.91) between children with isolated AVMs (35 of 55 [64%]) and children with AVM-associated aneurysms (13 of 22 [59%]). However, of the 11 children with AVM-associated aneurysms in an arterial location, 10 presented with hemorrhage (91%). An association with hemorrhage was significant in univariate analysis (p = 0.045) but not in multivariate analysis (p = 0.37)., Conclusions: Associated aneurysms are present in nearly a third of children with AVMs, and when arterially located, are more likely to present with hemorrhage. These data suggest that early angiography with endovascular treatment of arterial-based aneurysms in children with AVMs may be indicated.

Traumatic brain injury (TBI) is the current leading cause of death in children over 1 year of age. Adequate management and care of pediatric patients is critical to ensure the best functional outcome in this population. In their controversial trial, Cooper et al. concluded that decompressive craniectomy following TBI did not improve clinical outcome of the analyzed adult population. While the study did not target pediatric populations, the results do raise important and timely clinical questions regarding the effectiveness of decompressive surgery in pediatric patients. There is still a paucity of evidence regarding the effectiveness of this therapy in a pediatric population, and there is an especially noticeable knowledge gap surrounding age-stratified interventions in pediatric trauma. The purposes of this review are to first explore the anatomical variations between pediatric and adult populations in the setting of TBI. Second, the authors assess how these differences between adult and pediatric populations could translate into differences in the impact of decompressive surgery following TBI.