Christopher S. Ogilvy

Guidelines for the Management of Aneurysmal Subarachnoid Hemorrhage: A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association

Purpose— The aim of this guideline is to present current and comprehensive recommendations for the diagnosis and treatment of aneurysmal subarachnoid hemorrhage (aSAH). Methods— A formal literature search of MEDLINE (November 1, 2006, through May 1, 2010) was performed. Data were synthesized with the use of evidence tables. Writing group members met by teleconference to discuss data-derived recommendations. The American Heart Association Stroke Councils Levels of Evidence grading algorithm was used to grade each recommendation. The guideline draft was reviewed by 7 expert peer reviewers and by the members of the Stroke Council Leadership and Manuscript Oversight Committees. It is intended that this guideline be fully updated every 3 years. Results— Evidence-based guidelines are presented for the care of patients presenting with aSAH. The focus of the guideline was subdivided into incidence, risk factors, prevention, natural history and outcome, diagnosis, prevention of rebleeding, surgical and endovascular repair of ruptured aneurysms, systems of care, anesthetic management during repair, management of vasospasm and delayed cerebral ischemia, management of hydrocephalus, management of seizures, and management of medical complications. Conclusions— aSAH is a serious medical condition in which outcome can be dramatically impacted by early, aggressive, expert care. The guidelines offer a framework for goal-directed treatment of the patient with aSAH.

Recommendations for the Management of Intracranial Arteriovenous Malformations A Statement for Healthcare Professionals From a Special Writing Group of the Stroke Council, American Stroke Association

Intracranial arteriovenous malformations (AVMs) are relatively uncommon but increasingly recognized lesions that can cause serious neurological symptoms or death. Although AVMs can present with hemorrhage or seizure, since the advent of contemporary brain imaging techniques, an increasing number are detected before rupture. Over the last decade, there have been significant developments in the management of intracranial AVMs. There has been an evolution of microsurgical as well as endovascular and radiosurgical techniques to treat these lesions. As the management options have evolved, individual and combined modality treatment protocols have been developed in different institutions for the management of AVMs. A writing group was formed by the Stroke Council of the American Stroke Association to review published data for intracranial AVMs to develop practice recommendations regarding epidemiology, natural history, potential treatment strategies, and outcomes. The reports reviewed for this synthesis were selected on the basis of study design, sample size, and relevance to a particular topic. Each report was graded according to previously defined criteria.1 2 After review of the available literature, recommendations for current practice standards have been made according to 3 separate grades (Table 1⇓). View this table: Table 1. Levels of Evidence in Grading of Recommendations for Treatment of Patients With Subarachnoid Hemorrhage By the design of this type of review, the recommendations in this report represent an overview of existing treatment protocols that may vary considerably. These guidelines were developed to serve as a basis for the development of treatment strategies for AVMs, which overall represent a fairly heterogeneous group of cerebrovascular lesions and which may demonstrate different natural histories. In addition, for brain AVMs, no level I or II data are available in the literature. Because of the heterogeneity of these lesions and their relatively infrequent occurrence, strictly defined subcategories for comparison of the efficacy of various treatment modalities …

One-year outcome after decompressive surgery for massive nondominant hemispheric infarction.

OBJECTIVE Massive cerebral infarction is often accompanied by early death secondary to transtentorial herniation. We have tested the hypothesis that decompressive hemicraniectomy for massive nondominant cerebral infarction is lifesaving in a series of 14 patients presenting with right hemispheric infarction and clinical signs of uncal herniation and impending death. We have further analyzed, in prospective follow-up examinations, the levels of physical, psychiatric, and social disabilities in these patients. METHODS The methods used included retrospective analysis to determine rates of immediate mortality and morbidity after surgical intervention. Prospective follow-up data were obtained to determine the level of recovery in surviving patients after 1 year. Standardized measures of outcome to assess physical, psychiatric, and social recovery included the Barthel Index, Zung Depression Scale, and Reintegration to Normal Living Index. RESULTS With decompressive hemicraniectomy, we were able to prevent death secondary to transtentorial herniation in all cases; 11 patients experienced long-term survival after the procedure, and three deaths were related to non-neurological causes. We observed that 8 of the 11 surviving patients were at home, were functioning with minimal to moderate assistance, and had Barthel scores greater than 60. The remaining three patients were severely disabled. Seven of the 11 survivors were able to walk at 1 year after undergoing the procedure. Depression and failure to reintegrate socially were experienced by most patients. CONCLUSION This series confirms the lifesaving nature of hemicraniectomy in patients deteriorating because of cerebral edema after infarction. In patients younger than 50 years, recovery to a state of near-independence is possible.

Fever in subarachnoid hemorrhage: Relationship to vasospasm and outcome

Objective: To investigate the causes of fever in subarachnoid hemorrhage (SAH) and examine its relationship to outcome. Background: Fever adversely affects outcome in stroke. Patients with SAH are at risk for cerebral ischemia due to vasospasm (VSP). In these patients, fever may be both caused by, and potentiate, VSP-mediated brain injury. Methods: The authors prospectively studied patients admitted to a neurologic intensive care unit with nontraumatic SAH, documenting Hunt–Hess grade, Fisher group, Glasgow Coma Score, bacterial culture data, daily transcranial Doppler mean velocities, and maximum daily temperatures. Patients were classified as febrile (temperature above 38.3 °C for at least 2 consecutive days) or afebrile (no fever or isolated episodes of temperature above 38.3 °C). VSP was verified by either transcranial Doppler or angiographic criteria. Rankin scale scores on discharge were dichotomized into good (0 to 2) or poor (3 to 6) outcomes. Results: Ninety-two consecutive patients were studied. Thirty-eight patients were classified as febrile. No source for infection was found in 10 of 38 (26%) patients. In a multivariate analysis, three variables independently predicted fever occurrence: ventriculostomy (OR, 8.5 [CI, 2.4 to 29.7]), symptomatic VSP (OR, 5.0 [CI, 1.03 to 24.5]), and older age (OR, 1.75 per 10 years [CI, 1.02 to 3.0]). Poor outcome was related to fever (OR, 1.4 per each day febrile [CI, 1.1 to 1.88]), older age (OR, 1.64 per 10 years [CI, 1.04 to 2.58]), and intubation (OR, 21.8 [CI, 5.6 to 84.5]). Conclusion: Fever in SAH is associated with vasospasm and poor outcome independently of hemorrhage severity or presence of infection.

Results of a prospective protocol of computed tomographic angiography in place of catheter angiography as the only diagnostic and pretreatment planning study for cerebral aneurysms by a combined neurovascular team.

OBJECTIVE:At many centers, patients undergo both computed tomographic angiography (CTA) and digital subtraction angiography (DSA). This practice negates most of the advantages of CTA, and it renders the risks and disadvantages of the two techniques additive. Previous reports in the literature have assessed the sensitivity and specificity of CTA compared with DSA; however, these investigations have not analyzed the clinical implications of a protocol that replaces DSA with CTA as the only diagnostic and pretreatment planning study for patients with cerebral aneurysms. METHODS:Since late 2001/early 2002, the combined neurovascular unit of the Massachusetts General Hospital has adopted a prospective protocol of CTA in place of DSA as the only diagnostic and pretreatment planning study for patients with cerebral aneurysms (ruptured and unruptured). We report the results obtained during the 12-month period from January 2002 to January 2003. RESULTS:During the study period, 223 patients with cerebral aneurysms underwent initial diagnostic evaluation for cerebral aneurysm by the combined neurovascular team of Massachusetts General Hospital. Of the 223 patients, 109 patients had confirmed subarachnoid hemorrhage (Group A) and 114 patients did not have SAH (Group B). All of these patients were included in the prospective CTA protocol. Cerebral aneurysm treatment was initiated on the basis of CTA alone in 93 Group A patients (86%), in 89 Group B patients (78%), and in 182 patients (82%) overall. Treatment consisted of surgical clipping in 152 patients (68%), endovascular coiling in 56 patients (25%), endovascular parent artery balloon occlusion in 4 patients (2%), and external carotid artery to internal carotid artery bypass and carotid artery surgical occlusion in 2 patients (1%). Nine patients (4%) did not undergo treatment. The cerebral aneurysm detection rate by CTA was 100% for the presenting aneurysm (ruptured aneurysm in Group A or symptomatic/presenting aneurysm in Group B) in both groups. The detection rate by CTA for total cerebral aneurysms, including incidental multiple aneurysms, was 95.3% in Group A, 98.3% in Group B, and 97% overall. The overall morbidity associated with DSA (pretreatment or as intraoperative or postoperative clip evaluation) was one patient (1.3%) with a minor nonneurological complication, one patient (1.3%) with a minor neurological complication, and no patients (0%) with a major neurological complication. CONCLUSION:We have demonstrated promising results with a prospective protocol of CTA in place of DSA as the only diagnostic and pretreatment planning study for patients with ruptured and unruptured cerebral aneurysms. It seems safe and effective to make decisions regarding treatment on the basis of CTA, without performing DSA, in the majority of patients with ruptured and unruptured cerebral aneurysms.

Lessons learned from whole exome sequencing in multiplex families affected by a complex genetic disorder, intracranial aneurysm

Genetic risk factors for intracranial aneurysm (IA) are not yet fully understood. Genomewide association studies have been successful at identifying common variants; however, the role of rare variation in IA susceptibility has not been fully explored. In this study, we report the use of whole exome sequencing (WES) in seven densely-affected families (45 individuals) recruited as part of the Familial Intracranial Aneurysm study. WES variants were prioritized by functional prediction, frequency, predicted pathogenicity, and segregation within families. Using these criteria, 68 variants in 68 genes were prioritized across the seven families. Of the genes that were expressed in IA tissue, one gene (TMEM132B) was differentially expressed in aneurysmal samples (n=44) as compared to control samples (n=16) (false discovery rate adjusted p-value=0.023). We demonstrate that sequencing of densely affected families permits exploration of the role of rare variants in a relatively common disease such as IA, although there are important study design considerations for applying sequencing to complex disorders. In this study, we explore methods of WES variant prioritization, including the incorporation of unaffected individuals, multipoint linkage analysis, biological pathway information, and transcriptome profiling. Further studies are needed to validate and characterize the set of variants and genes identified in this study.

Guidelines for the Management of Patients With Unruptured Intracranial Aneurysms A Guideline for Healthcare Professionals From the American Heart Association/American Stroke Association

Purpose— The aim of this updated statement is to provide comprehensive and evidence-based recommendations for management of patients with unruptured intracranial aneurysms. Methods— Writing group members used systematic literature reviews from January 1977 up to June 2014. They also reviewed contemporary published evidence-based guidelines, personal files, and published expert opinion to summarize existing evidence, indicate gaps in current knowledge, and when appropriate, formulated recommendations using standard American Heart Association criteria. The guideline underwent extensive peer review, including review by the Stroke Council Leadership and Stroke Scientific Statement Oversight Committees, before consideration and approval by the American Heart Association Science Advisory and Coordinating Committee. Results— Evidence-based guidelines are presented for the care of patients presenting with unruptured intracranial aneurysms. The guidelines address presentation, natural history, epidemiology, risk factors, screening, diagnosis, imaging and outcomes from surgical and endovascular treatment.

In-hospital mortality and morbidity after surgical treatment of unruptured intracranial aneurysms in the United States, 1996-2000: The effect of hospital and surgeon volume

OBJECTIVE We sought to determine the risk of adverse outcome after contemporary surgical treatment of patients with unruptured intracranial aneurysms in the United States. Patient, surgeon, and hospital characteristics were tested as potential outcome predictors, with particular attention to the surgeons and hospitals volume of care. METHODS We performed a retrospective cohort study with the Nationwide Inpatient Sample, 1996 to 2000. Multivariate logistic and ordinal regression analyses were performed with endpoints of mortality, discharge other than to home, length of stay, and total hospital charges. RESULTS We identified 3498 patients who were treated at 463 hospitals, and we identified 585 surgeons in the database. Of all patients, 2.1% died, 3.3% were discharged to skilled-nursing facilities, and 12.8% were discharged to other facilities. The analysis adjusted for age, sex, race, primary payer, four variables measuring acuity of treatment and medical comorbidity, and five variables indicating symptoms and signs. The statistics for median annual number of unruptured aneurysms treated were eight per hospital and three per surgeon. High-volume hospitals had fewer adverse outcomes than hospitals that handled comparatively fewer unruptured aneurysms: discharge other than to home occurred after 15.6% of operations at high-volume hospitals (20 or more cases/yr) compared with 23.8% at low-volume hospitals (fewer than 4 cases/yr) (P = 0.002). High surgeon volume had a similar effect (15.3 versus 20.6%, P = 0.004). Mortality was lower at high-volume hospitals (1.6 versus 2.2%) than at hospitals that handled comparatively fewer unruptured aneurysms, but not significantly so. Patients treated by high-volume surgeons had fewer postoperative neurological complications (P = 0.04). Length of stay was not related to hospital volume. Charges were slightly higher at high-volume hospitals, partly because arteriography was performed more frequently than at hospitals that handled comparatively fewer unruptured aneurysms. CONCLUSION For patients with unruptured aneurysms who were treated in the United States between 1996 and 2000, surgery performed at high-volume institutions or by high-volume surgeons was associated with significantly lower morbidity and modestly lower mortality.

Intramedullary cavernous angiomas of the spinal cord: clinical presentation, pathological features, and surgical management.

Cavernous angiomas of the spinal cord are rare lesions that can cause severe neurological symptoms. We add a series of 6 patients with intramedullary cavernous angiomas of the spinal cord to 30 patients with 31 histologically verified lesions already described in the literature. Four types of clinical presentation were seen in the 36 patients: 1) discrete episodes of neurological deterioration with varying degrees of recovery between episodes (13 patients); 2) slow progression of neurological decline (12 patients); 3) acute onset of symptoms with rapid decline (8 patients); and 4) acute onset of mild symptoms with subsequent gradual decline lasting weeks to months (3 patients). Of the 36 patients, 25 (69%) were women and 11 (31%) were men. The peak age of presentation was in the fourth decade. The thoracic spinal cord was affected in more than half the patients, with the cervical cord being the next most common location. Histological examination demonstrated the closely apposed vascular channels characteristic of cavernous angiomas. While most vascular channels were thickened and hyalinized, three angiomas had foci of small vessels resembling a capillary angioma. The available data suggest that surgical management of intramedullary angiomas should attempt complete extirpation. The lesions are often well circumscribed with a glial plane between the lesion and normal cord. However, spinal cord angiomas, unlike cranial lesions, have little room for enlargement before devastating symptoms occur. We achieved complete excision in all of our patients, who are all ambulatory and have improved symptoms. Little, if any, increase in neurological deficit was caused by myelotomy and lesion excision. These spinal lesions should be considered for surgery early, before repeated hemorrhage or enlargement can occur.

Proposed Use of Prophylactic Decompressive Craniectomy in Poor-grade Aneurysmal Subarachnoid Hemorrhage Patients Presenting with Associated Large Sylvian Hematomas

OBJECTIVE As a group, patients who present in poor neurological grade after aneurysmal subarachnoid hemorrhage (SAH) often have poor outcomes. There may be subgroups of these patients, however, in which one pathological process predominates and for which the initiation of specific therapeutic interventions that target the predominant pathological process may result in improved outcome. We report the use of prophylactic decompressive craniectomy in patients presenting in poor neurological condition after SAH from middle cerebral artery aneurysms with associated large sylvian fissure hematomas. Craniectomy allowed significant parenchymal swelling in the posthemorrhagic period without increased intracranial pressure (ICP) or herniation syndrome. METHODS Eight patients (mean age, 56.5 yr; age range, 42–66 yr) presented comatose with SAH (five Hunt and Hess Grade IV, three Hunt and Hess Grade V). Radiographic evaluations demonstrated middle cerebral artery aneurysm and associated large sylvian fissure hematoma (mean clot volume, 121 ml; range, 30–175 ml). Patients were brought emergently to the operating room and treated with a modification of the pterional craniotomy and aneurysm clipping that included a planned craniectomy and duraplasty. A large, reverse question mark scalp flap was created, followed by bone removal with the following margins: anterior, frontal to the midpupillary line; posterior at least 2 cm behind the external auditory meatus; superior up to 2 cm lateral to the superior sagittal sinus; and inferior to the floor of the middle cranial fossa. Generous duraplasty was performed using either pericranium or suitable, commercially available dural substitutes. RESULTS All of the eight patients tolerated the craniectomy without operative complications. Postoperatively, all patients experienced immediate decreases in ICP to levels at or below 20 mm Hg (presentation mean ICP, 31.6 mm Hg; postoperative mean ICP, 13.1 mm Hg). ICP control was sustained in seven of eight patients, with the one exception being due to a massive hemispheric infarction secondary to refractory vasospasm. Follow-up (≥1 yr, except for one patient who died during the hospital stay) demonstrated that the craniectomy patients had a remarkably high number of good or excellent outcomes. The outcomes in the hemicraniectomy group were five good or excellent, one fair, and two poor or dead. CONCLUSION The data gathered in this study demonstrate that decompressive craniectomy can be performed safely as part of initial management for a subcategory of patients with SAH who present with large sylvian fissure hematomas. In addition, the performance of decompressive craniectomy in the patients described in this article seemed to be associated with rapid and sustained control of ICP. Although the number of patients in this study is small, the data lend support to the hypothesis that decompressive craniectomy may be associated with good or excellent outcome in a carefully selected subset of patients with SAH.