✓ The incidence of chronic hydrocephalus requiring shunting after aneurysmal subarachnoid hemorrhage (SAH) is not precisely known. The authors investigated whether the need for ventriculoperitoneal (VP) shunting can be predicted by initial Hunt and Hess grade or Fisher computerized tomography score. One hundred eight patients who presented with SAH and underwent 116 surgical procedures for aneurysm clipping were evaluated retrospectively to determine the incidence of chronic hydrocephalus. Chronic hydrocephalus was defined as clinically and radiographically demonstrated hydrocephalus that lasted 2 weeks or longer after the original hemorrhage and that required shunting. All SAH patients were managed in a similar fashion with induced hypervolemia, relative hemodilution, and hypertension complemented by a course of calcium channel blockers. The majority of patients underwent perioperative extracranial ventricular drainage to allow intraoperative brain relaxation and to assist intracranial pressure management. The overall mortality rate of the study group was 17%. Of the surviving patients, 20% underwent VP shunt placement secondary to chronic hydrocephalus. There were no statistically significant relationships between chronic hydrocephalus and patient age or gender, aneurysm type and size, or use of a perioperative drain. There was a high clinical correlation between chronic hydrocephalus and admission Hunt and Hess grades and Fisher grades (p < 0.05). All of the patients who survived a second bleeding episode and almost 46% of the patients who presented with intraventricular hemorrhage required placement of a VP shunt. The authors present predictive tables of chronic hydrocephalus based on the patient's admission Hunt and Hess grade and Fisher classification.
Fernando L. Vale, Edwin L. Bradley and Winfield S. Fisher III
Fernando L. Vale, Winfield S. Fisher III, William D. Jordan Jr., Cheryl A. Palmer and Jiri Vitek
✓ Carotid endarterectomy (CEA) is the treatment of choice for asymptomatic and symptomatic disease causing greater than 60% internal carotid artery (ICA) stenosis. Recently, percutaneous transluminal angioplasty (PTA) with stent placement has been investigated as a therapeutic option for the treatment of ICA stenosis. In this report the authors document CEA performed after PTA with stent placement and describe the pathological findings.
A standard CEA was performed. The surgical intervention was more difficult secondary to the following variables: the length of the exposure necessary to dissect out the metallic stent, the difficulty with opening and cutting the artery, and the care required to remove the stent to avoid vessel wall perforation. Pathological examination of the specimen demonstrated classic atherosclerotic changes revealing persistence of native disease. The metallic stent was embedded within the plaque. Many questions remain unanswered regarding the physiological and biological changes that occur in the carotid vessel wall after PTA with stent placement. It is concluded that CEA of a stent-containing carotid artery is feasible and should be considered as an alternative when recurrent stenosis occurs after PTA.
Lauren E. Rotman, James R. Hackney, Benjamin M. McGrew, Winfield S. Fisher III and Curtis J. Rozzelle
Cardiofaciocutaneous syndrome (CFCS) is a rare developmental disorder that is phenotypically similar to Noonan syndrome and is associated with mutations in BRAF, MEK1, MEK2, and KRAS. The relationship between malignancy risk and CFCS is unclear with few cases published in the literature. The purpose of this paper is to describe the case of a patient with CFCS presenting in extremis as a result of a large intracerebral hemorrhage arising from a temporal bone mass with histopathology most consistent with chondroblastoma and secondary aneurysmal bone cyst. This is the first case to document an association between CFCS and chondroblastoma.
Benjamin J. Ditty, Nidal B. Omar, Paul M. Foreman, Joseph H. Miller, Kimberly P. Kicielinski, Winfield S. Fisher III and Mark R. Harrigan
Patients with cerebral arteriovenous malformations (AVMs) commonly present with seizure. Seizure outcomes in patients treated with stereotactic radiosurgery (SRS) are poorly defined. A case series of patients with cerebral AVMs treated with SRS is presented to evaluate long-term seizure outcome.
A retrospective review of the medical record was performed, identifying 204 consecutive patients with AVMs treated with SRS between January 1991 and June 2012. Clinical and radiographic data were evaluated. Seizure outcome was measured using the Engel Epilepsy Surgery Outcome Scale. Mean duration of follow-up was 37.1 months (SD 38.3 months) with a minimum follow-up period of 1 month.
Of the 204 patients with cerebral AVMs treated with SRS, 78 patients (38.2%) presented with seizures and 49 of those patients were treated with antiepileptic drugs (AEDs). Following SRS, 63 (80.8%) of the 78 patients who had had seizures prior to SRS were seizure-free at a mean follow-up time of 37.2 months (SD 41.3 months). Of the 49 patients who had been treated with AEDs, 17 were still taking AEDs at last follow-up. Of the 126 patients who did not present with seizures prior to treatment with SRS, only 5 patients (4.0%) had seizures in the post-SRS period. There was no significant correlation between post-SRS seizure status and patient demographic features, comorbidities, AVM characteristics, history of operative intervention, pre- or posttreatment hemorrhage, or radiographic degree of AVM resolution.
Stereotactic radiosurgery for treatment of cerebral AVMs is effective at providing long-term control of seizures. A substantial number of patients who were treated with SRS were not only seizure free at their last follow-up, but had been successfully weaned from antiepileptic medications.
Paul M. Foreman, Michelle H. Chua, Mark R. Harrigan, Winfield S. Fisher III, R. Shane Tubbs, Mohammadali M. Shoja and Christoph J. Griessenauer
Delayed cerebral ischemia (DCI) following aneurysmal subarachnoid hemorrhage (aSAH) occurs in approximately 30% of patients. The Practical Risk Chart was developed to predict DCI based on admission characteristics; the authors seek to externally validate and critically appraise this prediction tool.
A prospective cohort of aSAH patients was used to externally validate the previously published Practical Risk Chart. The model consists of 4 variables: clinical condition on admission, amount of cisternal and intraventricular blood on CT, and age. External validity was assessed using logistic regression. Model discrimination was evaluated using the area under the receiver operating characteristic curve (AUC).
In a cohort of 125 patients with aSAH, the Practical Risk Chart adequately predicted DCI, with an AUC of 0.66 (95% CI 0.55–0.77). Clinical grade on admission and amount of intracranial blood on CT were the strongest predictors of DCI and clinical vasospasm. The best-fit model used a combination of the Hunt and Hess grade and the modified Fisher scale to yield an AUC of 0.76 (95% CI 0.675–0.85) and 0.70 (95% CI 0.602–0.8) for the prediction of DCI and clinical vasospasm, respectively.
The Practical Risk Chart adequately predicts the risk of DCI following aSAH. However, the best-fit model represents a simpler stratification scheme, using only the Hunt and Hess grade and the modified Fisher scale, and produces a comparable AUC.
Christoph J. Griessenauer, Joseph H. Miller, Bonita S. Agee, Winfield S. Fisher III, Joel K. Curé, Philip R. Chapman, Paul M. Foreman, Wilson A. M. Fisher, Adam C. Witcher and Beverly C. Walters
The aim of this study was to examine observer reliability of frequently used arteriovenous malformation (AVM) grading scales, including the 5-tier Spetzler-Martin scale, the 3-tier Spetzler-Ponce scale, and the Pollock-Flickinger radiosurgery-based scale, using current imaging modalities in a setting closely resembling routine clinical practice.
Five experienced raters, including 1 vascular neurosurgeon, 2 neuroradiologists, and 2 senior neurosurgical residents independently reviewed 15 MRI studies, 15 CT angiograms, and 15 digital subtraction angiograms obtained at the time of initial diagnosis. Assessments of 5 scans of each imaging modality were repeated for measurement of intrarater reliability. Three months after the initial assessment, raters reassessed those scans where there was disagreement. In this second assessment, raters were asked to justify their rating with comments and illustrations. Generalized kappa (κ) analysis for multiple raters, Kendall's coefficient of concordance (W), and interclass correlation coefficient (ICC) were applied to determine interrater reliability. For intrarater reliability analysis, Cohen's kappa (κ), Kendall's correlation coefficient (tau-b), and ICC were used to assess repeat measurement agreement for each rater.
Interrater reliability for the overall 5-tier Spetzler-Martin scale was fair to good (ICC = 0.69) to extremely strong (Kendall's W = 0.73) on initial assessment and improved on reassessment. Assessment of CT angiograms resulted in the highest agreement, followed by MRI and digital subtraction angiography. Agreement for the overall 3-tier Spetzler-Ponce grade was fair to good (ICC = 0.68) to strong (Kendall's W = 0.70) on initial assessment, improved on reassessment, and was comparable to agreement for the 5-tier Spetzler-Martin scale. Agreement for the overall Pollock-Flickinger radiosurgery-based grade was excellent (ICC = 0.89) to extremely strong (Kendall's W = 0.81). Intrarater reliability for the overall 5-tier Spetzler-Martin grade was excellent (ICC > 0.75) in 3 of the 5 raters and fair to good (ICC > 0.40) in the other 2 raters.
The 5-tier Spetzler-Martin scale, the 3-tier Spetzler-Ponce scale, and the Pollock-Flickinger radiosurgery-based scale all showed a high level of agreement. The improved reliability on reassessment was explained by a training effect from the initial assessment and the requirement to defend the rating, which outlines a potential downside for grades determined as part of routine clinical practice to be used for scientific purposes.
Elizabeth N. Alford, Lauren E. Rotman, Matthew S. Erwood, Robert A. Oster, Matthew C. Davis, H. Bruce C. Pittman, H. Evan Zeiger and Winfield S. Fisher III
The purpose of this study was to describe the development of a novel prognostic score, the Subdural Hematoma in the Elderly (SHE) score. The SHE score is intended to predict 30-day mortality in elderly patients (those > 65 years of age) with an acute, chronic, or mixed-density subdural hematoma (SDH) after minor, or no, prior trauma.
The authors used the Prognosis Research Strategy group methods to develop the clinical prediction model. The training data set included patients with acute, chronic, and mixed-density SDH. Based on multivariate analyses from a large data set, in addition to review of the extant literature, 3 components to the score were selected: age, admission Glasgow Coma Scale (GCS) score, and SDH volume. Patients are given 1 point if they are over 80 years old, 1 point for an admission GCS score of 5–12, 2 points for an admission GCS score of 3–4, and 1 point for SDH volume > 50 ml. The sum of points across all categories determines the SHE score.
The 30-day mortality rate steadily increased as the SHE score increased for all SDH acuities. For patients with an acute SDH, the 30-day mortality rate was 3.2% for SHE score of 0, and the rate increased to 13.1%, 32.7%, 95.7%, and 100% for SHE scores of 1, 2, 3, and 4, respectively. The model was most accurate for acute SDH (area under the curve [AUC] = 0.94), although it still performed well for chronic (AUC = 0.80) and mixed-density (AUC = 0.87) SDH.
The SHE score is a simple clinical grading scale that accurately stratifies patients’ risk of mortality based on age, admission GCS score, and SDH volume. Use of the SHE score could improve counseling of patients and their families, allow for standardization of clinical treatment protocols, and facilitate clinical research studies in SDH.
Christoph J. Griessenauer, Robert M. Starke, Paul M. Foreman, Philipp Hendrix, Mark R. Harrigan, Winfield S. Fisher III, Nilesh A. Vyas, Robert H. Lipsky, Mingkuan Lin, Beverly C. Walters, Jean-Francois Pittet and Mali Mathru
Endothelin-1, a potent vasoconstrictor, and its receptors may be involved in the pathogenesis of aneurysmal subarachnoid hemorrhage (aSAH), clinical vasospasm, delayed cerebral ischemia (DCI), and functional outcome following aSAH. In the present study, common endothelin single nucleotide polymorphisms (SNPs) and their relation to aSAH were evaluated.
Blood samples from all patients enrolled in the Cerebral Aneurysm Renin Angiotensin System (CARAS) study were used for genetic evaluation. The CARAS study prospectively enrolled patients with aSAH at 2 academic institutions in the US from 2012 to 2015. Common endothelin SNPs were detected using 5′ exonnuclease (TaqMan) genotyping assays. Analysis of associations between endothelin SNPs and aSAH and its clinical sequelae was performed.
Samples from 149 patients with aSAH and 50 controls were available for analysis. In multivariate logistic regression analysis, the TG (odds ratio [OR] 2.102, 95% confidence interval [CI] 1.048–4.218, p = 0.036) and TT genotypes (OR 7.884, 95% CI 1.003–61.995, p = 0.05) of the endothelin-1 T/G SNP (rs1800541) were significantly associated with aSAH. There was a dominant effect of the G allele (CG/GG genotypes; OR 4.617, 95% CI 1.311–16.262, p = 0.017) of the endothelin receptor A G/C SNP (rs5335) on clinical vasospasm. Endothelin SNPs were not associated with DCI or functional outcome.
Common endothelin SNPs were found to be associated with presentation with aSAH and clinical vasospasm. Further studies are required to elucidate the relevant pathophysiology and its potential implications in the treatment of patients with aSAH.
Paul M. Foreman, Michelle Chua, Mark R. Harrigan, Winfield S. Fisher III, Nilesh A. Vyas, Robert H. Lipsky, Beverly C. Walters, R. Shane Tubbs, Mohammadali M. Shoja and Christoph J. Griessenauer
Delayed cerebral ischemia (DCI) is a recognized complication of aneurysmal subarachnoid hemorrhage (aSAH) that contributes to poor outcome. This study seeks to determine the effect of nosocomial infection on the incidence of DCI and patient outcome.
An exploratory analysis was performed on 156 patients with aSAH enrolled in the Cerebral Aneurysm Renin Angiotensin System study. Clinical and radiographic data were analyzed with univariate analysis to detect risk factors for the development of DCI and poor outcome. Multivariate logistic regression was performed to identify independent predictors of DCI.
One hundred fifty-three patients with aSAH were included. DCI was identified in 32 patients (20.9%). Nosocomial infection (odds ratio [OR] 3.5, 95% confidence interval [CI] 1.09–11.2, p = 0.04), ventriculitis (OR 25.3, 95% CI 1.39–458.7, p = 0.03), aneurysm re-rupture (OR 7.55, 95% CI 1.02–55.7, p = 0.05), and clinical vasospasm (OR 43.4, 95% CI 13.1–143.4, p < 0.01) were independently associated with the development of DCI. Diagnosis of nosocomial infection preceded the diagnosis of DCI in 15 (71.4%) of 21 patients. Patients diagnosed with nosocomial infection experienced significantly worse outcomes as measured by the modified Rankin Scale score at discharge and 1 year (p < 0.01 and p = 0.03, respectively).
Nosocomial infection is independently associated with DCI. This association is hypothesized to be partly causative through the exacerbation of systemic inflammation leading to thrombosis and subsequent ischemia.
Christoph J. Griessenauer, Jeffrey D. Lebensburger, Michelle H. Chua, Winfield S. Fisher III, Lee Hilliard, Christina J. Bemrich-Stolz, Thomas H. Howard and James M. Johnston
Pediatric patients with sickle cell disease (SCD) and moyamoya syndrome (MMS) are at significant risk for cerebrovascular accidents despite chronic transfusion therapy. Encephaloduroarteriosynangiosis (EDAS) and encephalomyoarteriosynangiosis (EMAS) are additional therapeutic options for these patients. To date, the incidence of complications after and efficacy of EDAS and EMAS in stroke prevention in this population have been described in several institutional case series reports, but no randomized prospective trials have been reported.
The authors retrospectively reviewed the cases of all pediatric patients at the University of Alabama at Birmingham with a history of homozygous hemoglobin S (HbS) and sickle cell/β-thalassemia (SB0 thalassemia) and on chronic transfusion therapy, including 14 patients with MMS who underwent EDAS or EMAS.
Sixty-two patients with SCD and on chronic transfusion therapy were identified. After exclusion of patients on chronic transfusion therapy for indications other than stroke prevention, 48 patients (77.4%) remained. Of those patients, 14 (29.1%) underwent EDAS or EMAS. Nine (18.8%) and 25 (52.1%) patients were on chronic transfusion therapy for primary or secondary stroke prevention, respectively, but did not undergo EDAS or EMAS. The 14 patients with SCD and radiological evidence of MMS and on chronic transfusion therapy for primary or secondary stroke prevention underwent 21 EDAS or EMAS procedures for progressive vascular disease (92.9% of patients), stroke (71.4%), and/or seizure (7.1%). The mean (± SD) time from initiation of chronic transfusion therapy to EDAS or EMAS was 76.8 ± 58.8 months. Complications included 1 perioperative stroke, 1 symptomatic subdural hygroma, 1 postoperative seizure, and 1 case of intraoperative cerebral edema that required subsequent cranioplasty. Before EDAS or EMAS, the stroke rate was calculated to be 1 stroke per 7.8 patient-years. One additional stroke occurred during the follow-up period (mean follow-up time 33.7 ± 19.6 months), resulting in a post-EDAS/EMAS stroke rate of 1 stroke per 39.3 patient-years, a 5-fold reduction compared with that in the pre-EDAS/EMAS period. The patients’ mean pre-EDAS/EMAS HbS level of 29.5% ± 6.4% was comparable to the mean post-EDAS/EMAS HbS level of 25.5% ± 6.1% (p = 0.104).
The results of this retrospective case series in a large cohort of pediatric patients with SCD and MMS suggest that EDAS/EMAS provides a stroke-prevention benefit with an acceptably low morbidity rate. Given the combined experience with EDAS and EMAS for this indication at this and other institutions, a prospective clinical trial to assess their efficacy compared with that of chronic transfusion therapy alone is warranted.