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Post-operative hematoma following anterior cervical discectomy and fusion (ACDF) is an uncommon but feared complication. Typically, these complications present in the immediate post-operative period. We present a case of a 51 year-old woman who underwent a C4-5 ACDF for left sided radicular pain. Her immediate post-operative course was uncomplicated, but she presented 6 weeks subsequently to the emergency department with neck swelling, difficulty swallowing, cough, and shortness of breath. She was found to have a 4.5 cm anterior neck hematoma with settling of the instrumentation and a new C4 vertebral fragment protruding anteriorly. She underwent evacuation of hematoma without clear evidence of a bleeding source. After several days of observation, she was discharged home and ultimately had resolution of her presenting symptoms. Most hematomas resulting in airway compromise appear in the immediate post-operative period, but a high index of suspicion must remain high in any patient with a prior anterior cervical surgery presenting with symptoms of pre-vertebral compression or respiratory compromise., (Copyright © 2018. Published by Elsevier Ltd.)

Translocations between EWSR1 and members of the CREB family of transcription factors (CREB1, ATF1, and CREM) are rare genetic findings occurring in various sarcomas. Of these, the EWSR1-CREM translocation is the most rarely reported. We present the case of a 9-year-old boy who presented with a year of fatigue, weight loss, and abulia. A brain MRI revealed a frontal interhemispheric tumor arising from the falx. After resection, pathology demonstrated a myxoid mesenchymal tumor with an EWSR1-CREM translocation. A series of recent reports of similar tumors has generated ongoing debate in the literature over the classification of these tumors either as intracranial angiomatoid fibrous histiocytomas, which also harbor EWSR1-CREB family translocations, or as a novel diagnostic entity. The present case provides another example of the rare EWSR1-CREM fusion in an intracranial myxoid mesenchymal tumor that recurred in just 6 months despite gross total resection. The findings are discussed in the context of the existing literature and the ongoing effort to appropriately classify this type of tumor., (© 2019 S. Karger AG, Basel.)

Schimmelpenning syndrome is a rare, well-defined constellation of clinical phenotypes associated with the presence of nevus sebaceous and multisystem abnormalities most commonly manifested as cerebral, ocular, and skeletal defects [1]. A single nucleotide mutation in the HRAS or KRAS genes resulting in genetic mosaicism is responsible for the clinical manifestations of this syndrome in the majority of cases. We report a case of an adolescent boy with Schimmelpenning syndrome with a multifocal pilocytic astrocytoma. No HRAS or KRAS gene mutations were noted in the tumor on genetic sequencing. However, glial tumors have been associated with genetic mutations of RAS upregulation, which may imply a common pathway., (© 2019 S. Karger AG, Basel.)

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.

Introduction: The purpose of this study is to identify the national trends of exposure to pediatric procedures during neurosurgical residency and to subsequently evaluate how neurosurgery residents' experiences correlate with the minimum requirements set forth by the American College of Graduate Medical Education (ACGME)., Methods: ACGME resident case logs from residents graduating between 2013 and 2017 were retrospectively reviewed. These reports were analyzed to determine trends in resident operative experience in pediatric procedures. The number of cases performed by residents was compared to the required minimums set by the ACGME within each pediatric surgical category. A linear regression analysis and t tests were utilized to analyze the change in cases performed over the study period., Results: A mean of 98.8 procedures were performed for each of the 877 residents graduating between 2013 and 2017. The total number of pediatric procedures declined at a rate of 1.7 cases/year (r2 = 0.77, p = 0.05). Spine and cerebrospinal fluid diversion procedures showed decreasing trends at rates of 1.9 (r2 = 0.70, p = 0.08) and 1.2 (r2 = 0.70, p = 0.08) cases/year, respectively. The number of trauma and brain tumor cases were shown to have increasing rates at 1.0 (r2 = 0.86, p = 0.02) and 0.3 (r2 = 0.69, p = 0.08) cases/year, respectively, with trauma cases showing significant increases. There was also a trend of increasing cases logged as the lead resident surgeon by 12.9 cases/year (r2 = 0.99, p < 0.001). The number of cases performed by the average graduating resident was also significantly higher than the minimums required by the ACGME; residents, on average, performed 3 times the required minimum number of pediatric cases., Conclusion: Neurosurgical residents graduating from 2013 to 2017 reported significantly higher volumes of pediatric neurosurgery cases than the standards set for by the ACGME. During this time, there was also a significant trend of increasing cases logged as the lead resident surgeon, suggesting more involvement in the critical portions of pediatric cases. There was also a significant, but not clinically impactful, decrease in pediatric case volumes during this time. However, the overall data indicate that residents are continuing to gain valuable pediatric experience during residency training., (© 2019 S. Karger AG, Basel.)

Background: There is extensive debate on the role of fixed pressure shunts in the adult population. Most studies assessing fixed pressure valves do not consider the potential for changes in technique and management of shunts. We sought to examine the natural history of fixed pressure., Methods: We conducted a retrospective chart review of 169 patients undergoing shunt placement by the senior author Daniel Wecht (DW). The etiology of shunt placement, shunt failure rates, and outcome data was assessed for each patient., Results: Overall, 126 patients underwent initial shunt placement. Thirty-three (26.2%) patients required at least one shunt revision during follow-up. The most common cause of first time revision was mechanical shunt malfunction (13, 39.4%), followed by infection (7, 21.2%), and shunt migration (6, 18.2%). Three patients (9.1%) required revision due to misplaced catheters. Underdrainage or overdrainage of shunts each resulted in revisions for 2 (6.1%) patients. The mean follow-up length was 28.1 ± 6.1 months., Conclusion: Fixed pressure shunts failed primarily because of shunt malfunction and occurred most commonly in patients developing hydrocephalus as a result of hemorrhage or normal pressure hydrocephalus (NPH). The overall failure rate between these two groups was proportionally equivalent. Both overdrainage or underdrainage were found to be rare indications for revision.

Background: Expansile duraplasty is frequently performed during Chiari I decompression. Aseptic and bacterial meningitis are possible complications of this procedure. We sought to compare the rates of meningitis and subsequent need for cerebrospinal fluid (CSF) diversion with duraplasty using bovine pericardial (BPC) xenograft and allograft., Methods: We conducted a retrospective review of 112 patients who underwent Chiari I decompression. All patients underwent duraplasty with either allograft or BPC. Occurrence of postoperative lumbar punctures and associated data were recorded to determine rates of meningitis. Rates of subsequent shunting were also recorded., Results: Overall, 112 patients were included in the study: 30 underwent duraplasty with allograft (27%) and 82 received BPC (73%). A total of 26 patients developed postoperative meningitis (23 cases of chemical meningitis and 3 of bacterial meningitis). Rates of meningitis were higher in patients who received BPC than patients who received allograft (28% vs. 10%, respectively; P = 0.047). The rate of shunting was greater in patients with meningitis compared with patients without meningitis (56.5% vs. 5.75%, respectively; P < 0.0001). Moreover, 13 of the 15 patients (87%) in the BPC cohort who were shunted were found to have meningitis compared with 0 of the 3 shunted patients (0%) in the allograft cohort (P = 0.044)., Conclusions: We found that rates of total meningitis were greater in patients who underwent Chiari I decompression and duraplasty with BPC compared with an allograft. The rate of shunting was significantly higher for patients who developed meningitis after decompression compared with patients without meningitis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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.

Background: Ventriculoatrial shunts are common alternatives for patients who cannot tolerate ventriculoperitoneal shunts. The majority of ventriculoatrial shunt malfunctions are related to mechanical problems. We report an interesting case of ventriculoatrial shunt malfunction due to elevated central venous pressure from new-onset atrial fibrillation., Methods: After the patient was confirmed to have ventriculomegaly, he was taken to the operating room for exploration of his ventriculoatrial shunt; there were no obstructions. Subsequently, the patient was cardioverted to normal sinus rhythm for his new onset atrial fibrillation., Results: The clinical syndrome and ventriculomegaly both resolved after the patient's atrial fibrillation was corrected with chemical cardioversion., Conclusions: The cause of this patient's VA shunt malfunction was likely associated with his new onset atrial fibrillation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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.

Introduction: In Chiari I patients, postoperative pain and discomfort frequently slow the transition back to the home setting., Objective: We sought to determine the effect of standardized ketamine infusion protocols on hospital length of stay (LOS)., Methods: This retrospective cohort study reviewed 100 consecutive adult patients undergoing Chiari I decompression. Fifty-nine patients were placed on a 2-3 mg/hr ketamine drip until postoperative day 1. This group was compared with a group who received 2-3 mg/hr of ketamine until postoperative day 2 (19 patients) and patients who did not receive ketamine at all (22 patients). Clinical characteristics, opioid use, LOS, and relative hospitalization costs were assessed. All narcotic amounts were converted into milligram equivalents of morphine., Results: LOS of the short-ketamine group was 46.5 hours when compared with the long-ketamine group (66.8 hours) and no-ketamine group (56.9 hours). There was a statistically significant difference when comparing the short-ketamine group with the long-ketamine group and no-ketamine group together (P < 0.001), as well as when compared individually (P = 0.001 and 0.004). The mean cost of hospitalization was 20% less when a short-ketamine protocol was used (P < 0.001). Mean morphine milligram equivalents used postoperatively were 148 mg in the short-ketamine group, 196 mg in the long-ketamine group, and 187 mg in the no-ketamine group (P = 0.65). No adverse events from ketamine were noted., Conclusions: Ketamine at subanesthetic levels may be an effective tool to facilitate early return home postoperatively and may significantly reduce medical costs., (Copyright © 2017 Elsevier Inc. All rights reserved.)

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.

Introduction: Myelomeningocele (MMC) is an open neural tube defect routinely surgically closed within 48 h of birth to prevent secondary infection. Up to 18% of patients experience wound complications, and 85% require shunting for hydrocephalus. We hypothesized that wound complications could be reduced by cerebrospinal fluid (CSF) diversion at the time of closure., Methods: Institutional review board approval was obtained to review records of the 88 patients who underwent MMC closure between January 2005 and June 2016 at the Children's Hospital of Pittsburgh. Twenty-three patients (26%) had an external ventricular drain (EVD) placed at the time of MMC closure and underwent 7-11 days of CSF drainage. Fourteen patients (16%) had a shunt placed at the time of MMC closure, and 51 (58%) had no form of CSF diversion at the time of MMC closure., Results: Patients with an EVD or shunt placed at the time of closure had no wound complications. In contrast, 8 patients (16%) without CSF diversion at closure developed wound complications (p = 0.048). Seven of the 8 wound complications occurred in the 71 patients with evidence of hydrocephalus at birth (p = 0.98). Of patients with evidence of hydrocephalus at the time of MMC closure, wound complications had a higher rate of occurrence among patients who did not receive a shunt or EVD at closure (p = 0.01). When comparing only patients with evidence of hydrocephalus at birth, the EVD group alone had a lower rate of wound complications than patients who did not receive CSF diversion at the time of closure (p = 0.031)., Conclusions: These results suggest that addressing hydrocephalus at the time of MMC closure significantly reduces the likelihood of wound complications and may justify temporary CSF diversion at birth, at least in those patients manifesting hydrocephalus., (© 2018 S. Karger AG, Basel.)

Background: Sagittal balance in adult spinal deformity is a major predictor of quality of life. A temporary loss of paraspinal muscle force and somatic pain following spine surgery may limit a patient's ability to maintain posture., Objective: To assess the evolution of sagittal balance and clinical outcomes during recovery from adult spinal deformity surgery., Methods: Retrospective review of a prospective observational database identified a consecutive series of patients with sagittal vertical axis (SVA) > 40 mm undergoing adult deformity surgery. Radiographic parameters and clinical outcomes were measured out to 2 yr after surgery., Results: A total of 113 consecutive patients met inclusion criteria. Mean preoperative SVA was 90.3 mm, increased to 104.6 mm in the first week, then gradually reduced at each follow-up interval to 59.2 mm at 6 wk, 45.0 mm at 3 mo, 38.6 mm at 6 mo, and 34.1 mm at 1 yr (all P < .05). SVA did not change between 1 and 2 yr. Pelvic incidence-lumbar lordosis (PI-LL) corrected immediately from 25.3° to 8.5° (16.8° change; P < .01) and a decreased pelvic tilt from 27.6° to 17.6° (10° change; P < .01). No further change was noted in PI-LL. Pelvic tilt increased to 20.2° ( P = .01) at 6 wk and held steady through 2 yr. Mean Visual Analog Scale, Oswestry Disability Index, and Short Form-36 scores all improved; pain rapidly improved, whereas disability measures improved as SVA improved., Conclusion: Radiographic assessment of global sagittal alignment did not fully reflect surgical correction of sagittal balance until 6 mo after adult deformity surgery. Sagittal balance initially worsened then steadily improved at each interval over the first year postoperatively. At 1 yr, all clinical and radiographic measures outcomes were significantly improved., (Copyright © 2017 by the Congress of Neurological Surgeons)

Objectives: The objective of the present study is to report a case of campomelic dysplasia illustrating the absence of cervical and thoracic pedicles. This report reiterates the importance of this clinical peculiarity in the setting of spine instrumentation., Materials and Methods: A 10-year-old female patient with campomelic dysplasia presented with progressive kyphoscoliosis and signs of neural compromise. Imaging studies confirmed thoracic level stenosis and demonstrated absence of multiple pedicles in cervical and thoracic spine. The patient underwent decompression and instrumentation/fusion for her spinal deformity., Results: The patient was instrumented between C2 and L4 with pedicle screws and sublaminar cables. However, pedicle fixation was not possible for the lower cervical and upper-mid thoracic spine. Also, floating posterior elements precluded the use of laminar fixation in the lower cervical spine. Cervicothoracic lumbosacral orthosis (CTLSO) was used for external immobilization to supplement the tenuous fixation in the cervicothoracic area. The patient improved neurologically with no signs of implant failure at the 2-year follow-up., Conclusions: Absence of pedicles and floating posterior elements present a challenge during spine surgery in campomelic dysplasia. Surgeons should prepare for alternative fixation methods and external immobilization when planning on spinal instrumentation in affected patients., Level of Evidence: Level IV Case Report.

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.

Introduction: Facial spasms represent a complicated array of neurological motor disorders with unique diagnostic and treatment algorithms. Due to the rarity of many of these disorders in the pediatric population, special care must be taken in identifying subtle differences in presentation of these disorders., Methods: We present a case of a 3-year-old boy diagnosed with a brainstem ganglioglioma, Chiari 1 malformation, and a 2-year history of left-sided facial spasms. Stereotyped facial contractions and subtle eye deviation occurred every 10 s, with downward movement rather than upward elevation of the eyebrow., Results: MRI revealed absence of a clear compressive vessel of the centrally-myelinized portion of the facial nerve, and EMG of the left facial nerve demonstrated no abnormal motor response or evidence of "lateral spread." Given these findings, a diagnosis of hemifacial seizures was made. Microvascular decompression was not recommended, and botulinum toxin injection was not pursued; however, the patient has remained refractory to antiepileptic drugs, possibly due to biochemical alteration by his ganglioglioma. He may eventually require surgical debulking should his symptoms progress., Conclusion: Hemifacial spasm is a well-recognized disorder, but similar conditions can, at times, imitate its appearance. While our patient presented with facial spasms, his clinical history, examination, and radiographic and electrophysiological findings were more consistent with hemifacial seizures secondary to a brainstem lesion, rather than hemifacial spasms. It is important to distinguish the two entities, as misdiagnosis and inappropriate diagnostic or therapeutic measures may be taken inadvertently.

Background: Intrathecal disc herniation is a rare but serious condition that has the potential to generate devastating neurologic deficits. We present a case of a 56-year-old man who developed cauda equina syndrome after several episodes of severe Valsalva maneuver., Case Description: The patient was found to have developed subacute urinary retention and leg weakness. Magnetic resonance imaging findings were concerning for an unusual-appearing lesion extending cranially at L2-3. Urgent decompression via an L2 laminectomy, exploration, and subsequent discectomy was performed. The patient recovered exceptionally well, regaining bladder function and ultimately being able to ambulate without assistance., Conclusions: Cranially extending intrathecal disc herniations are a rare phenomenon and exceptionally uncommon above L3. The clinician should have a high level of suspicion for herniation when looking at the clinical and historical information consistent with such a diagnosis even in the presence of ambiguous imaging findings., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.

Introduction: Projectile embolization to the cerebral vasculature and is almost exclusively seen in the anterior circulation due to the greater diameter and flow of the internal carotid arteries. In children, this phenomenon is ever rarer., Methods: We present a case of a 9-year-old boy who suffered from a shotgun blast to the thorax and abdomen. He was subsequently found to have a pellet that had presumably traveled from either the left ventricle or directly via the subclavian artery to the vertebrobasilar system to become lodged in the P3 segment of his posterior cerebral artery., Results: The patient developed a small occipital infarct with a corresponding right superior quadrantanopsia. He was managed as an inpatient non-operatively with a heparin drip and was placed on long-term low-dose aspirin on discharge. The patient recovered well from his injury and remains neurologically stable 2 years after the initial injury. Interval imaging demonstrated that the pellet remains stable in its position., Discussion: To our knowledge, this represents the first non-fatal missile embolus to the posterior cerebral artery in a pediatric patient. Patients with minimal symptoms may benefit from conservative management given the inherent risks of embolectomy.

Purpose: Vestibular schwannomas (VS) are increasingly diagnosed in patients with normal hearing because of advances in magnetic resonance imaging. We sought to evaluate whether stereotactic radiosurgery (SRS) performed earlier after diagnosis improved long-term hearing preservation in this population., Methods and Materials: We queried our quality assessment registry and found the records of 1134 acoustic neuroma patients who underwent SRS during a 15-year period (1997-2011). We identified 88 patients who had VS but normal hearing with no subjective hearing loss at the time of diagnosis. All patients were Gardner-Robertson (GR) class I at the time of SRS. Fifty-seven patients underwent early (≤2 years from diagnosis) SRS and 31 patients underwent late (>2 years after diagnosis) SRS. At a median follow-up time of 75 months, we evaluated patient outcomes., Results: Tumor control rates (decreased or stable in size) were similar in the early (95%) and late (90%) treatment groups (P=.73). Patients in the early treatment group retained serviceable (GR class I/II) hearing and normal (GR class I) hearing longer than did patients in the late treatment group (serviceable hearing, P=.006; normal hearing, P<.0001, respectively). At 5 years after SRS, an estimated 88% of the early treatment group retained serviceable hearing and 77% retained normal hearing, compared with 55% with serviceable hearing and 33% with normal hearing in the late treatment group., Conclusions: SRS within 2 years after diagnosis of VS in normal hearing patients resulted in improved retention of all hearing measures compared with later SRS., (Copyright © 2016 Elsevier Inc. All rights reserved.)

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.

The ability to differentially alter specific brain functions via deep brain stimulation (DBS) represents a monumental advance in clinical neuroscience, as well as within medicine as a whole. Despite the efficacy of DBS in the treatment of movement disorders, for which it is often the gold-standard therapy when medical management becomes inadequate, the mechanisms through which DBS in various brain targets produces therapeutic effects is still not well understood. This limited knowledge is a barrier to improving efficacy and reducing side effects in clinical brain stimulation. A field of study related to assessing the network effects of DBS is gradually emerging that promises to reveal aspects of the underlying pathophysiology of various brain disorders and their response to DBS that will be critical to advancing the field. This review summarizes the nascent literature related to network effects of DBS measured by cerebral blood flow and metabolic imaging, functional imaging, and electrophysiology (scalp and intracranial electroencephalography and magnetoencephalography) in order to establish a framework for future studies., (Copyright © 2015 the American Physiological Society.)

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.

The development of de novo intracranial aneurysms following stereotactic radiosurgery for intracranial pathology is a rare complication secondary to vascular injury. Typically, these aneurysms develop within the first few years after radiation surgery. We present the first case of an aneurysm developing 10 years after radiosurgery for a cerebellopontine angle meningioma. This case highlights the importance of careful long-term follow-up of patients who undergo radiosurgery for lesions abutting major vessels and/or who suffer post-radiation complications., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.)

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.

Background: The use of antiplatelet medications has greatly expanded and this has been associated with an increased rate of complications after aneurysmal subarachnoid hemorrhage (SAH). The influence of antiplatelet medications on outcomes after non-aneurysmal SAH is unknown., Objective: To analyze the frequency and impact on outcome of antiplatelet medication use among patients with angiogram-negative SAH., Methods: An analysis of patients within the Columbia University SAH Outcomes Project database was performed. All patients who underwent catheter cerebral angiography after presenting with nontraumatic SAH between 1996 and 2013 were included. Outcomes were assessed by using the modified Rankin Scale., Results: A total of 1351 patients underwent catheter angiography for evaluation of SAH. Of these, 173 (13%) were designated angiogram-negative. The fraction of patients presenting with angiogram-negative SAH as well as the frequency of antiplatelet use among these patients significantly increased during the study period. Antiplatelet use was more commonly associated with angiogram-negative SAH than with angiogram-positive SAH (27% vs 14%, P = .001). At 14 days after presentation, poor outcome was significantly more frequent among patients who took antiplatelet agents than among those who did not (38% vs 20%, P = .017). This effect was also seen after multivariate analysis (odds ratio, 2.58; P = .034), although no difference was observed by 12 months (P > .05)., Conclusion: Antiplatelet medication use is associated with poor early, but not late, outcomes after angiogram-negative SAH. Corresponding increased rates of antiplatelet medication use and angiogram-negative SAH may be related. Additional studies are needed to confirm this association.

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.

The development of de novo intracranial aneurysms following stereotactic radiosurgery for intracranial pathology is a rare complication secondary to vascular injury. Typically, these aneurysms develop within the first few years after radiation surgery. We present the first case of an aneurysm developing 10 years after radiosurgery for a cerebellopontine angle meningioma. This case highlights the importance of careful long-term follow-up of patients who undergo radiosurgery for lesions abutting major vessels and/or who suffer post-radiation complications., (2014 BMJ Publishing Group Ltd.)

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.

Introduction: Admission at 'off times' has been suggested to result in increased risk of poor outcome. The utilization of high volume centers may be a potential remedy to this variability in care., Objective: To assess the ability of a high volume center to mitigate variability in care due to timing of admission in a post hoc analysis of an observational study., Methods: The medical records of 200 hypertensive intracerebral hemorrhage (ICH) patients admitted to the Neurological Intensive Care Unit (NICU) from 12 January 2009 to 4 April 2013 were identified and examined for variable outcome based on admission timing using the modified Rankin Scale (mRS). Multiple logistic regression was used to assess predictors of poor outcome, correcting severity of admission., Results: Seventy-five admissions were recorded to have occurred on the weekend. The 3-month follow-up mRS of surviving patients was 3·78 in weekend admissions and 3·63 in weekday admissions (P  =  0·62). One hundred and seven night admissions occurred. The average mRS at 3 months of surviving patients was 3·56 in night admissions and 3·84 in daytime admissions (P  =  0·36). Thirteen patients were admitted in July. The 3-month mRS of surviving patients was 3·71 for July admissions and 3·38 for non-July admissions (P  =  0·58). Only ICH score was found to be a predictor of outcome on multivariate analysis (P < 0·001)., Conclusions: No significant difference in the outcome of patients was identified regardless of time of admission. High volume centers may be less prone to temporal variability in care, though the existence of temporal variability in care at low volume centers is controversial.

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.

Key Findings: Data from the National Health and Nutrition Examination Surveys. The percentage of infants who were ever breastfed increased from 60% among infants who were born in 1993-1994 to 77% among infants who were born in 2005-2006. Breastfeeding rates increased significantly among non-Hispanic black women from 36% in 1993-1994 to 65% in 2005-2006. Breastfeeding rates in 1999-2006 were significantly higher among those with higher income (74%) compared with those who had lower income (57%). Breastfeeding rates among mothers 30 years and older were significantly higher than those of younger mothers. There was no significant change in the rate of breast-feeding at 6 months of age for infants born between 1993 and 2004. Human milk is the ideal food for most infants. Breastfeeding benefits infants and their mothers. Breastfed infants receive anti-bodies from breast milk, which protect against infection in the early postpartum period, and breastfeeding is less expensive than formula feeding. This report summarizes information on breastfeeding rates in the United States based on data from the 1999-2006 National Health and Nutrition Examination Surveys (NHANES). Results are reported for the total U.S. population and three race-ethnic groups by birth year cohort., (All material appearing in this report is in the public domain and may be reproduced or copied without permission; citation as to source, however, is appreciated.)

Current doctrine states that phosphorus is incorporated into cells in the pentavalent(V) oxidation state as orthophosphate. However, recent studies show that microorganisms contain enzymes used to metabolize reduced forms of phosphorous, including phosphite(III) and hypophosphite(I), which suggests that there is a natural source for these chemical species. This paper will discuss suppressed conductivity ion chromatography methods developed to detect hypophosphite, phosphite, and orthophosphate in a geothermal water matrix containing fluoride, chloride, bromide, nitrate, hydrogen carbonate and sulfate. All peaks were clearly resolved, and calibrations were linear with estimated 3sigma detection limits of 0.83, 0.39, and 0.35 microM for hypophosphite, phosphite, and orthophosphate, respectively.