Until recently, cerebral arteriopathy due to heterozygous mutations of the ACTA2 gene was considered a variant of moyamoya disease. However, radiographic analysis of patients with these mutations reveals a distinctive angiographic appearance from that seen in moyamoya disease. Several heterozygous missense ACTA2 mutations have been implicated in the development of this distinct cerebrovascular entity; however, the penetrance and systemic manifestations of these mutations vary based on the location of the amino acid replacement within the α–smooth muscle actin protein. The severity of the phenotype may also differ among patients within a single mutation type. There is limited literature on the safety and efficacy of revascularization procedures for ACTA2 arteriopathy, which have been limited to those patients with known Arg179His mutations. The authors provide a review of the breadth of mutations within the ACTA2 literature and report a case of two siblings with de novo ACTA2 Arg258Cys mutations with differing clinical courses, highlighting the utility of indirect revascularization with 8-year follow-up data. This case highlights the importance of early recognition of the angiographic appearance of ACTA2 cerebral arteriopathy and performance of genetic testing, as the location of the mutation impacts clinical presentation and outcomes.
Christine Tschoe, Teddy E. Kim, Kyle M. Fargen, and Stacey Q. Wolfe
E. Sander Connolly Jr.
Kyle M. Fargen and Brian L. Hoh
Kyle M. Fargen, J. Bridger Cox, and Daniel J. Hoh
Ossification of the posterior longitudinal ligament (OPLL) is a disease process characterized by progressive growth and calcification resulting in spinal canal compromise and serious neurological sequelae in advanced cases. Historically, OPLL has most commonly been treated with posterior surgical decompression. Although this procedure indirectly decompresses the spinal cord, it does not address the offending pathological entity, and further growth of the lesion may result in delayed neurological deterioration. This fact is particularly relevant because a number of long-term studies have revealed both longitudinal and transverse disease progression in individuals treated both surgically and conservatively. Despite these high rates of radiographically documented progression, however, the rate of neurological decline in patients undergoing posterior surgery with laminoplasty is low. In this article, the authors review the pathophysiology of OPLL, evidence of disease progression, and outcome data addressing conservative and surgical treatments.
Kyle M. Fargen and Brian L. Hoh
Kyle M. Fargen, Dan Neal, Spiros L. Blackburn, Brian L. Hoh, and Maryam Rahman
The Agency for Healthcare Research and Quality patient safety indicators (PSIs) and the Centers for Medicare and Medicaid Services hospital-acquired conditions (HACs) are publicly reported quality metrics linked directly to reimbursement. The occurrence of PSIs and HACs is associated with increased mortality and hospital costs after stroke. The relationship between insurance status and PSI and HAC rates in hospitalized patients treated for acute ischemic stroke was determined using the Nationwide Inpatient Sample (NIS) database.
The NIS was queried for all hospitalizations involving acute ischemic stroke between 2002 and 2011. The rate of each PSI and HAC was determined by searching the hospital records for ICD-9 codes. The SAS statistical software package was used to calculate rates and perform multivariable analyses to determine the effects of patient variables on the probability of developing each indicator.
The NIS query revealed 1,507,336 separate patient admissions that had information on both primary payer and hospital teaching status. There were 227,676 PSIs (15.1% of admissions) and 42,841 HACs reported (2.8%). Patient safety indicators occurred more frequently in Medicaid/self-pay/no-charge patients (19.1%) and Medicare patients (15.0%) than in those with private insurance (13.6%; p < 0.0001). In a multivariable analysis, Medicaid, self-pay, or nocharge patients had significantly longer hospital stays, higher mortality, and worse outcomes than those with private insurance (p < 0.0001).
Insurance status is an independent predictor of patient safety events after stroke. Private insurance is associated with lower mortality, shorter lengths of stay, and improved clinical outcomes.
Kyle M. Fargen, Dan Neal, Maryam Rahman, and Brian L. Hoh
The Agency for Healthcare Research and Quality (AHRQ) patient safety indicators (PSIs) and the Centers for Medicare and Medicaid Services hospital-acquired conditions (HACs) are publicly reported metrics used to gauge the quality of health care provided by health care institutions. To better understand the prevalence of these events in hospitalized patients treated for ruptured cerebral aneurysms, the authors determined the incidence rates of PSIs and HACs among patients with a diagnosis of subarachnoid hemorrhage and procedure codes for either coiling or clipping in the Nationwide Inpatient Sample database.
The authors queried the Nationwide Inpatient Sample database, part of the AHRQ's Healthcare Cost and Utilization Project, for all hospitalizations between 2002 and 2010 involving coiling or clipping of ruptured cerebral aneurysms. The incidence rate of each PSI and HAC was determined by searching the hospital records for ICD-9 codes. The authors used the SAS statistical software package to calculate incidence rates and perform multivariate analyses to determine the effects of patient variables on the probability of developing each indicator.
There were 62,972 patient admissions with a diagnosis code of subarachnoid hemorrhage between the years 2002 and 2010; 10,274 (16.3%) underwent clipping and 8248 (13.1%) underwent endovascular coiling. A total of 6547 PSI and HAC events occurred within the 10,274 patients treated with clipping; at least 1 PSI or HAC occurred in 47.9% of these patients. There were 5623 total PSI and HAC events among the 8248 patients treated with coils; at least 1 PSI or HAC occurred in 51.0% of coil-treated patients. Age, sex, comorbidities, hospital size, and hospital type had statistically significant associations with indicator occurrence. Compared with patients without events, those treated by either clipping or coiling and had at least 1 PSI during their hospitalization had significantly longer lengths of stay (p < 0.001), higher hospital costs (p < 0.001), and higher in-hospital mortality rates (p < 0.001).
These results estimate baseline national rates of PSIs and HACs in patients treated for ruptured cerebral aneurysms. These data may be used to gauge individual institutional quality of care and patient safety metrics in comparison with national data.
Kyle M. Fargen, Maryam Rahman, Dan Neal, and Brian L. Hoh
The Agency for Healthcare Research and Quality (AHRQ) patient safety indicators (PSIs) and the Centers for Medicare and Medicaid Services hospital-acquired conditions (HACs) are metrics used to gauge the quality of health care provided by health care institutions. The PSIs and HACs are publicly reported metrics and are directly linked to reimbursement for services. To better understand the prevalence of these adverse events in hospitalized patients treated for unruptured cerebral aneurysms, the authors determined the incidence rates of PSIs and HACs among patients with a diagnosis of unruptured aneurysm in the Nationwide Inpatient Sample (NIS) database.
The NIS, part of the AHRQ's Healthcare Cost and Utilization Project, was queried for all hospitalizations between 2002 and 2010 involving coiling or clipping of unruptured cerebral aneurysms. The incidence rate for each PSI and HAC was determined by searching the hospital records for ICD-9 codes. The SAS statistical software package was used to calculate incidences and perform multivariate analyses to determine the effects of patient variables on the probability of each indicator developing.
There were 54,589 hospitalizations involving unruptured cerebral aneurysms in the NIS database for the years 2002–2010; 8314 patients (15.2%) underwent surgical clipping and 9916 (18.2%) were treated with endovascular coiling. One thousand four hundred ninety-two PSI and HAC events occurred among the 8314 patients treated with clipping; at least 1 PSI or HAC occurred in 14.6% of these patients. There were 1353 PSI and HAC events among the 9916 patients treated with coiling; at least 1 PSI or HAC occurred in 10.9% of these patients. Age, sex, and comorbidities had statistically significant associations with an adverse event. Compared with the patients having no adverse event, those having at least 1 PSI during their hospitalizations had significantly longer hospital stays (p < 0.0001), higher hospital costs (p < 0.0001), and higher mortality rates (p < 0.0001).
These results estimate baseline national rates of PSIs and HACs in patients with unruptured cerebral aneurysms. These data may be used to gauge individual institutional quality of care and patient safety metrics in comparison with national data.
James L. West, Kyle M. Fargen, Wesley Hsu, Charles L. Branch Jr., and Daniel E. Couture
Global access to neurosurgical care is still a work in progress, with many patients in low-income countries not able to access potentially lifesaving neurosurgical procedures. “Big Data” is an increasingly popular data collection and analytical technique predicated on collecting large amounts of data across multiple data sources and types for future analysis. The potential applications of Big Data to global outreach neurosurgery are myriad: from assessing the overall burden of neurosurgical disease to planning cost-effective improvements in access to neurosurgical care, and collecting data on conditions which are rare in developed countries. Although some global neurosurgical outreach programs have intelligently implemented Big Data principles in their global neurosurgery initiatives already, there is still significant progress that remains to be made. Big Data has the potential to drive the efficient improvement of access to neurosurgical care across low- and medium-income countries.
Kyle M. Fargen, Brian L. Hoh, Dan Neal, Timothy O’connor, Marie Rivera-Zengotita, and Gregory J. A. Murad
Ventriculostomy occlusion is a known complication after external ventricular drain (EVD) placement. There have been no prospective published series that primarily evaluate the incidence of and risk factors for EVD occlusion. These phenomena are investigated using a prospective database.
An ongoing prospective study of all patients undergoing frontal EVD placement in the Neurosurgery Intensive Care Unit at the University of Florida was accessed for the purposes of this analysis. Demographic, procedural, and radiographic data were recorded prospectively and retrospectively. The need for catheter irrigation or replacement was meticulously documented. Univariate and multivariate regression analyses were performed.
Ninety-eight of 101 total enrolled patients had accessible data, amounting to 131 total catheters and 1076 total catheter days. Nineteen percent of patients required at least 1 replacement. Forty-one percent of catheters developed at least 1 temporary occlusion, with an average of 2.4 irrigations per patient. Intracranial hemorrhage occurred in 28% of patients after the first EVD placement (2% resulting in new neurological deficit) and in 62% of patients after 1 replacement. The cost of occlusion is estimated at $615 per enrolled patient. Therapeutic anticoagulation and use of small EVD catheters were statistically significant predictors of permanent occlusion (p = 0.01 and 0.04, respectively).
EVD occlusion is frequent and imparts a significant burden in terms of patient morbidity, physician upkeep, and cost. This study suggests that developing strategies or devices to prevent EVD occlusion, such as the preferential use of larger diameter catheters, may be beneficial in reducing the burden associated with ventriculostomy malfunction.