Rotational vertebrobasilar insufficiency, or bow hunter's syndrome, is a rare cause of posterior circulation ischemia, which, following rotation of the head, results in episodic vertigo, dizziness, nystagmus, or syncope. While typically caused by dynamic occlusion of the vertebral artery in its V2 and V3 segments, the authors here describe a patient with dynamic occlusion of the vertebral artery secondary to a persistent first intersegmental artery, a rare variant course of the vertebral artery. In this case the vertebral artery coursed under rather than over the posterior arch of the C-1. This patient was also found to have incomplete development of the posterior arch of C-1, as is often seen with this variant. The patient underwent dynamic digital subtraction angiography, which demonstrated occlusion at the variant vertebral artery with head turning. He was then taken for decompression of the vertebral artery through removal of the incomplete arch of C-1 that was causing the dynamic compression. After surgery the patient had a complete resolution of symptoms. In this report, the authors present the details of this case, describe the anatomical variants involved, and provide a discussion regarding the need for atlantoaxial fusion in these patients.
Vivek P. Buch, Peter J. Madsen, Kerry A. Vaughan, Paul F. Koch, David K. Kung and Ali K. Ozturk
Frederick L. Hitti, Ashwin G. Ramayya, Brendan J. McShane, Andrew I. Yang, Kerry A. Vaughan and Gordon H. Baltuch
Deep brain stimulation (DBS) is an effective treatment for several movement disorders, including Parkinson’s disease (PD). While this treatment has been available for decades, studies on long-term patient outcomes have been limited. Here, the authors examined survival and long-term outcomes of PD patients treated with DBS.
The authors conducted a retrospective analysis using medical records of their patients to identify the first 400 consecutive patients who underwent DBS implantation at their institution from 1999 to 2007. The medical record was used to obtain baseline demographics and neurological status. The authors performed survival analyses using Kaplan-Meier estimation and multivariate regression using Cox proportional hazards modeling. Telephone surveys were used to determine long-term outcomes.
Demographics for the cohort of patients with PD (n = 320) were as follows: mean age of 61 years, 70% male, 27% of patients had at least 1 medical comorbidity (coronary artery disease, congestive heart failure, diabetes mellitus, atrial fibrillation, or deep vein thrombosis). Kaplan-Meier survival analysis on a subset of patients with at least 10 years of follow-up (n = 200) revealed a survival probability of 51% (mean age at death 73 years). Using multivariate regression, the authors found that age at implantation (HR 1.02, p = 0.01) and male sex (HR 1.42, p = 0.02) were predictive of reduced survival. Number of medical comorbidities was not significantly associated with survival (p > 0.5). Telephone surveys were completed by 40 surviving patients (mean age 55.1 ± 6.4 years, 72.5% male, 95% subthalamic nucleus DBS, mean follow-up 13.0 ± 1.7 years). Tremor responded best to DBS (72.5% of patients improved), while other motor symptoms remained stable. Ability to conduct activities of daily living (ADLs) remained stable (dressing, 78% of patients; running errands, 52.5% of patients) or worsened (preparing meals, 50% of patients). Patient satisfaction, however, remained high (92.5% happy with DBS, 95% would recommend DBS, and 75% felt it provided symptom control).
DBS for PD is associated with a 10-year survival rate of 51%. Survey data suggest that while DBS does not halt disease progression in PD, it provides durable symptomatic relief and allows many individuals to maintain ADLs over long-term follow-up greater than 10 years. Furthermore, patient satisfaction with DBS remains high at long-term follow-up.
Frederick L. Hitti, Kerry A. Vaughan, Ashwin G. Ramayya, Brendan J. McShane and Gordon H. Baltuch
Deep brain stimulation (DBS) has revolutionized the treatment of neurological disease, but its therapeutic efficacy is limited by the lifetime of the implantable pulse generator (IPG) batteries. At the end of the battery life, IPG replacement surgery is required. New IPGs with rechargeable batteries (RC-IPGs) have recently been introduced and allow for decreased reoperation rates for IPG replacements. The authors aimed to examine the merits and limitations of these devices.
The authors reviewed the medical records of patients who underwent DBS implantation at their institution. RC-IPGs were placed either during initial DBS implantation or during an IPG change. A cost analysis was performed that compared RC-IPGs with standard IPGs, and telephone patient surveys were conducted to assess patient satisfaction.
The authors identified 206 consecutive patients from 2011 to 2016 who underwent RC-IPG placement (mean age 61 years; 67 women, 33%). Parkinson’s disease was the most common indication for DBS (n = 144, 70%), followed by essential tremor (n = 41, 20%), dystonia (n = 13, 6%), depression (n = 5, 2%), multiple sclerosis tremor (n = 2, 1%), and epilepsy (n = 1, 0.5%). DBS leads were typically placed bilaterally (n = 192, 93%) and targeted the subthalamic nucleus (n = 136, 66%), ventral intermediate nucleus of the thalamus (n = 43, 21%), internal globus pallidus (n = 21, 10%), ventral striatum (n = 5, 2%), or anterior nucleus of the thalamus (n = 1, 0.5%). RC-IPGs were inserted at initial DBS implantation in 123 patients (60%), while 83 patients (40%) were converted to RC-IPGs during an IPG replacement surgery. The authors found that RC-IPG implantation resulted in $60,900 of cost savings over the course of 9 years. Furthermore, patient satisfaction was high with RC-IPG implantation. Overall, 87.3% of patients who responded to the survey were satisfied with their device, and only 6.7% found the rechargeable component difficult to use. In patients who were switched from a standard IPG to RC-IPG, the majority who responded (70.3%) preferred the rechargeable IPG.
RC-IPGs can provide DBS patients with long-term therapeutic benefit while minimizing the need for battery replacement surgery. The authors have implanted rechargeable stimulators in 206 patients undergoing DBS surgery, and here they demonstrate the cost-effectiveness and high patient satisfaction associated with this procedure.
Gail Rosseau, Walter D. Johnson, Kee B. Park, Miguel Arráez Sánchez, Franco Servadei and Kerry A. Vaughan
Since the creation of the World Health Organization (WHO) in 1948, the annual World Health Assembly (WHA) has been the major forum for discussion, debate, and approval of the global health agenda. As such, it informs the framework for the policies and budgets of many of its Member States. For most of its history, a significant portion of the attention of health ministers and Member States has been given to issues of clean water, vaccination, and communicable diseases. For neurosurgeons, the adoption of WHA Resolution 68.15 changed the global health landscape because the importance of surgical care for universal health coverage was highlighted in the document. This resolution was adopted in 2015, shortly after the publication of The Lancet Commission on Global Surgery Report titled “Global Surgery 2030: evidence and solutions for achieving health, welfare and economic development.” Mandating global strengthening of emergency and essential surgical care and anesthesia, this resolution has led to the formation of surgical and anesthesia collaborations that center on WHO and can be facilitated via the WHA. Participation by neurosurgeons has grown dramatically, in part due to the official relations between WHO and the World Federation of Neurosurgical Societies, with the result that global neurosurgery is gaining momentum.
Brian Y. Hwang, Samuel S. Bruce, Geoffrey Appelboom, Matthew A. Piazza, Amanda M. Carpenter, Paul R. Gigante, Christopher P. Kellner, Andrew F. Ducruet, Michael A. Kellner, Rajeev Deb-Sen, Kerry A. Vaughan, Philip M. Meyers and E. Sander Connolly Jr.
Intraventricular hemorrhage (IVH) associated with intracerebral hemorrhage (ICH) is an independent predictor of poor outcome. Clinical methods for evaluating IVH, however, are not well established. This study sought to determine the best IVH grading scale by evaluating the predictive accuracies of IVH, Graeb, and LeRoux scores in an independent cohort of ICH patients with IVH. Subacute IVH dynamics as well as the impact of external ventricular drain (EVD) placement on IVH and outcome were also investigated.
A consecutive cohort of 142 primary ICH patients with IVH was admitted to Columbia University Medical Center between February 2009 and February 2011. Baseline demographics, clinical presentation, and hospital course were prospectively recorded. Admission CT scans performed within 24 hours of onset were reviewed for ICH location, hematoma volume, and presence of IVH. Intraventricular hemorrhage was categorized according to IVH, Graeb, and LeRoux scores. For each patient, the last scan performed within 6 days of ictus was similarly evaluated. Outcomes at discharge were assessed using the modified Rankin Scale (mRS). Receiver operating characteristic analysis was used to determine the predictive accuracies of the grading scales for poor outcome (mRS score ≥ 3).
Seventy-three primary ICH patients (51%) had IVH. Median admission IVH, Graeb, and LeRoux scores were 13, 6, and 8, respectively. Median IVH, Graeb and LeRoux scores decreased to 9 (p = 0.005), 4 (p = 0.002), and 4 (p = 0.003), respectively, within 6 days of ictus. Poor outcome was noted in 55 patients (75%). Areas under the receiver operating characteristic curve were similar among the IVH, Graeb, and LeRoux scores (0.745, 0.743, and 0.744, respectively) and within 6 days postictus (0.765, 0.722, 0.723, respectively). Moreover, the IVH, Graeb, and LeRoux scores had similar maximum Youden Indices both at admission (0.515 vs 0.477 vs 0.440, respectively) and within 6 days postictus (0.515 vs 0.339 vs 0.365, respectively). Patients who received EVDs had higher mean IVH volumes (23 ± 26 ml vs 9 ± 11 ml, p = 0.003) and increased incidence of Glasgow Coma Scale scores < 8 (67% vs 38%, p = 0.015) and hydrocephalus (82% vs 50%, p = 0.004) at admission but had similar outcome as those who did not receive an EVD.
The IVH, Graeb, and LeRoux scores predict outcome well with similarly good accuracy in ICH patients with IVH when assessed at admission and within 6 days after hemorrhage. Therefore, any of one of the scores would be equally useful for assessing IVH severity and risk-stratifying ICH patients with regard to outcome. These results suggest that EVD placement may be beneficial for patients with severe IVH, who have particularly poor prognosis at admission, but a randomized clinical trial is needed to conclusively demonstrate its therapeutic value.
Geoffrey Appelboom, Stephen D. Zoller, Matthew A. Piazza, Caroline Szpalski, Samuel S. Bruce, Michael M. McDowell, Kerry A. Vaughan, Brad E. Zacharia, Zachary Hickman, Anthony D'Ambrosio, Neil A. Feldstein and Richard C. E. Anderson
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.
Brad E. Zacharia, Kerry A. Vaughan, Zachary L. Hickman, Samuel S. Bruce, Amanda M. Carpenter, Nils H. Petersen, Stacie Deiner, Neeraj Badjatia and E. Sander Connolly Jr
Intracerebral hemorrhage (ICH) is frequently complicated by acute hydrocephalus, necessitating emergency CSF diversion with a subset of patients, ultimately requiring long-term treatment via placement of permanent ventricular shunts. It is unclear what factors may predict the need for ventricular shunt placement in this patient population.
The authors performed a retrospective analysis of a prospective database (ICH Outcomes Project) containing patients with nontraumatic ICH admitted to the neurological ICU at Columbia University Medical Center between January 2009 and September 2011. A multiple logistic regression model was developed to identify independent predictors of shunt-dependent hydrocephalus after ICH. The following variables were included: patient age, admission Glasgow Coma Scale score, temporal horn diameter on admission CT imaging, bicaudate index, admission ICH volume and location, intraventricular hemorrhage volume, Graeb score, LeRoux score, third or fourth ventricle hemorrhage, and intracranial pressure (ICP) and ventriculitis during hospital stay.
Of 210 patients prospectively enrolled in the ICH Outcomes Project, 64 required emergency CSF diversion via placement of an external ventricular drain and were included in the final cohort. Thirteen of these patients underwent permanent ventricular CSF shunting prior to discharge. In univariate analysis, only thalamic hemorrhage and elevated ICP were significantly associated with the requirement for permanent CSF diversion, with p values of 0.008 and 0.033, respectively. Each remained significant in a multiple logistic regression model in which both variables were present.
Of patients with ICH requiring emergency CSF diversion, those with persistently elevated ICP and thalamic location of their hemorrhage are at increased odds of developing persistent hydrocephalus, necessitating permanent ventricular shunt placement. These factors may assist in predicting which patients will require permanent CSF diversion and could ultimately lead to improvements in the management of this disorder and the outcome in patients with ICH.
Kerry A. Vaughan, Christian Lopez Ramos, Vivek P. Buch, Rania A. Mekary, Julia R. Amundson, Meghal Shah, Abbas Rattani, Michael C. Dewan and Kee B. Park
Epilepsy is one of the most common neurological disorders, yet its global surgical burden has yet to be characterized. The authors sought to compile the most current epidemiological data to quantify global prevalence and incidence, and estimate global surgically treatable epilepsy. Understanding regional and global epilepsy trends and potential surgical volume is crucial for future policy efforts and resource allocation.
The authors performed a systematic literature review and meta-analysis to determine the global incidence, lifetime prevalence, and active prevalence of epilepsy; to estimate surgically treatable epilepsy volume; and to evaluate regional trends by WHO regions and World Bank income levels. Data were extracted from all population-based studies with prespecified methodological quality across all countries and demographics, performed between 1990 and 2016 and indexed on PubMed, EMBASE, and Cochrane. The current and annual new case volumes for surgically treatable epilepsy were derived from global epilepsy prevalence and incidence.
This systematic review yielded 167 articles, across all WHO regions and income levels. Meta-analysis showed a raw global prevalence of lifetime epilepsy of 1099 per 100,000 people, whereas active epilepsy prevalence is slightly lower at 690 per 100,000 people. Global incidence was found to be 62 cases per 100,000 person-years. The meta-analysis predicted 4.6 million new cases of epilepsy annually worldwide, a prevalence of 51.7 million active epilepsy cases, and 82.3 million people with any lifetime epilepsy diagnosis. Differences across WHO regions and country incomes were significant. The authors estimate that currently 10.1 million patients with epilepsy may be surgical treatment candidates, and 1.4 million new surgically treatable epilepsy cases arise annually. The highest prevalences are found in Africa and Latin America, although the highest incidences are reported in the Middle East and Latin America. These regions are primarily low- and middle-income countries; as expected, the highest disease burden falls disproportionately on regions with the fewest healthcare resources.
Understanding of the global epilepsy burden has evolved as more regions have been studied. This up-to-date worldwide analysis provides the first estimate of surgical epilepsy volume and an updated comprehensive overview of current epidemiological trends. The disproportionate burden of epilepsy on low- and middle-income countries will require targeted diagnostic and treatment efforts to reduce the global disparities in care and cost. Quantifying global epilepsy provides the first step toward restructuring the allocation of healthcare resources as part of global healthcare system strengthening.
Edith Mbabazi-Kabachelor, Meghal Shah, Kerry A. Vaughan, John Mugamba, Peter Ssenyonga, Justin Onen, Esther Nalule, Kush Kapur and Benjamin C. Warf
Clinical and economic repercussions of ventricular shunt infections are magnified in low-resource countries. The efficacy of antibiotic-impregnated shunts in this setting is unclear. A previous retrospective cohort study comparing the Bactiseal Universal Shunt (BUS) and the Chhabra shunt provided clinical equipoise; thus, the authors conducted this larger randomized controlled trial in Ugandan children requiring shunt placement for hydrocephalus to determine whether there was, in fact, any advantage of one shunt over the other.
Between April 2013 and September 2016, the authors randomly assigned children younger than 16 years of age without evidence of ventriculitis to either BUS or Chhabra shunt implantation in this single-blind randomized controlled trial. The primary outcome was shunt infection, and secondary outcomes included reoperation and death. The minimum follow-up was 6 months. Time to outcome was assessed using the Kaplan-Meier method. The significance of differences was tested using Wilcoxon rank-sum, chi-square, Fisher’s exact, and t-tests.
Of the 248 patients randomized, the BUS was implanted in 124 and the Chhabra shunt in 124. There were no differences between the groups in terms of age, sex, or hydrocephalus etiology. Within 6 months of follow-up, there were 14 infections (5.6%): 6 BUS (4.8%) and 8 Chhabra (6.5%; p = 0.58). There were 14 deaths (5.6%; 5 BUS [4.0%] vs 9 Chhabra [7.3%], p = 0.27) and 30 reoperations (12.1%; 15 BUS vs 15 Chhabra, p = 1.00). There were no significant differences in the time to primary or secondary outcomes at 6 months’ follow-up (p = 0.29 and 0.17, respectively, Wilcoxon rank-sum test).
Among Ugandan infants, BUS implantation did not result in a lower incidence of shunt infection or other complications. Any recommendation for a more costly standard of care in low-resource countries must have contextually relevant, evidence-based support.
Clinical trial registration no.: PACTR201804003240177 (http://www.pactr.org/)