Plasmapheresis for Management of Antiphospholipid Syndrome in the Neurosurgical Patient.

BACKGROUND AND IMPORTANCE: Antiphospholipid syndrome (APS) is an autoimmune disorder associated with a hypercoagulable state and increased risk of intraoperative and postoperative thrombosis. Few neurosurgical studies have examined the management of these patients, though the standard of care in most other disciplines involves the use of anticoagulation therapy. However, this is associated with risks such as hemorrhage, thrombosis due to warfarin withdrawal, and is not compatible with operative intervention. CLINICAL PRESENTATION: We report the cases of 2 antiphospholipid positive patients who were on anticoagulant therapy and underwent surgical bypasses and received perioperative management with plasmapheresis. The first was a 44-yr-old woman who presented with worsening vision, recurring headaches, and a known left internal carotid artery aneurysm that was unsuccessfully treated twice via extracranial to intracranial (ECIC) bypass at another institution. Preoperative tests at our institution revealed elevated beta 2 glycoprotein 1 IgA autoantibodies. The second case was a 24-yr-old woman with previously diagnosed APS, who presented for surgical evaluation of moyamoya disease after sustaining recurrent left hemispheric strokes. Both cases were managed with perioperative plasmapheresis to avoid the need for anticoagulation during the perioperative period, and both underwent successful ECIC bypass procedures without perioperative ischemic or hemorrhagic complications. CONCLUSION: Management of neurosurgical patients with APS can be a precarious proposition. We describe the successful use of plasmapheresis and antiplatelet therapy to better manage patients undergoing neurosurgical procedures, specifically ECIC bypass, and feel this approach can be considered in future cases.

Predictors of 30-day readmission after aneurysmal subarachnoid hemorrhage: a case-control study.

OBJECTIVE Despite persisting questions regarding its appropriateness, 30-day readmission is an increasingly common quality metric used to influence hospital compensation in the United States. However, there is currently insufficient evidence to identify which patients are at highest risk for readmission after aneurysmal subarachnoid hemorrhage (SAH). The objective of this study was to identify predictors of 30-day readmission after SAH, to focus preventative efforts, and to provide guidance to funding agencies seeking to risk-adjust comparisons among hospitals. METHODS The authors performed a case-control study of 30-day readmission among aneurysmal SAH patients treated at a single center between 2003 and 2013. To control for geographic distance from the hospital and year of treatment, the authors randomly matched each case (30-day readmission) with approximately 2 SAH controls (no readmission) based on home ZIP code and treatment year. They evaluated variables related to patient demographics, socioeconomic characteristics, comorbidities, presentation severity (e.g., Hunt and Hess grade), and clinical course (e.g., need for gastrostomy or tracheostomy, length of stay). Conditional logistic regression was used to identify significant predictors, accounting for the matched design of the study. RESULTS Among 82 SAH patients with unplanned 30-day readmission, the authors matched 78 patients with 153 nonreadmitted controls. Age, demographics, and socioeconomic factors were not associated with readmission. In univariate analysis, multiple variables were significantly associated with readmission, including Hunt and Hess grade (OR 3.0 for Grade IV/V vs I/II), need for gastrostomy placement (OR 2.0), length of hospital stay (OR 1.03 per day), discharge disposition (OR 3.2 for skilled nursing vs other disposition), and Charlson Comorbidity Index (OR 2.3 for score ≥ 2 vs 0). However, the only significant predictor in the multivariate analysis was discharge to a skilled nursing facility (OR 3.2), and the final model was sensitive to criteria used to enter and retain variables. Furthermore, despite the significant association between discharge disposition and readmission, less than 25% of readmitted patients were discharged to a skilled nursing facility. CONCLUSIONS Although discharge disposition remained significant in multivariate analysis, most routinely collected variables appeared to be weak independent predictors of 30-day readmission after SAH. Consequently, hospitals interested in decreasing readmission rates may consider multifaceted, cost-efficient interventions that can be broadly applied to most if not all SAH patients.

Self-injection of household cleaning detergents into a ventriculoperitoneal shunt reservoir during a suicide attempt: a case report and literature review.

Self-injection of household cleaning detergents (more specifically, commercial toilet bowl cleaner) into the reservoir of a ventriculoperitoneal shunt (VPS) has never been reported in the neurosurgical literature. A right-handed 41-year-old female with a past medical history significant for bipolar depression (with multiple prior hospital admissions for suicide attempts) and pseudotumor cerebri (status-post VPS placement from a right frontal approach) successfully injected ∼5 ml of toilet bowl cleaner into her ventricular shunt reservoir during a suicide attempt. She was found unresponsive by a family member 48 h after this event and presented to our hospital in moribund neurological condition (bilaterally fixed and dilated pupils with decerebrate posturing). Head computed tomography (CT) demonstrated marked ventriculomegaly. She was taken emergently to the operating room for placement of a left frontal ventriculostomy. Cerebrospinal fluid (CSF) sampled intraoperatively showed numerous Gram-positive cocci (later determined to be Staphylococcus epidermidis). For this reason, her right-sided shunt system was also removed in its entirety. She was treated with broad-spectrum intravenous and intraventricular antibiotics for her bacterial ventriculitis and her CSF was aggressively drained to treat her hydrocephalus. Once her infection had resolved, the shunt was replaced (using a right parietal approach) and she went on to make an excellent neurological recovery. Here, the authors present the case of a patient who self-injected household cleaning detergents into her VPS reservoir-and, likely, the ventricular system-during a suicide attempt and subsequently developed hydrocephalus and ventriculitis. Following this infrequent clinical scenario, consideration should be given to temporary ventriculostomy placement and shunt removal. Moreover, in patients with a known history of psychiatric co-morbidities-and particularly those patients with prior suicide attempts-the neurosurgeon should give serious consideration to placing the shunt system in an anatomical region which is difficult for the patient to self-access based upon their handedness.

A Phase I proof-of-concept and safety trial of sildenafil to treat cerebral vasospasm following subarachnoid hemorrhage.

OBJECTIVE: Studies show that phosphodiesterase-V (PDE-V) inhibition reduces cerebral vasospasm (CVS) and improves outcomes after experimental subarachnoid hemorrhage (SAH). This study was performed to investigate the safety and effect of sildenafil (an FDA-approved PDE-V inhibitor) on angiographic CVS in SAH patients. METHODS: A2-phase, prospective, nonrandomized, human trial was implemented. Subarachnoid hemorrhage patients underwent angiography on Day 7 to assess for CVS. Those with CVS were given 10 mg of intravenous sildenafil in the first phase of the study and 30 mg in the second phase. In both, angiography was repeated 30 minutes after infusion. Safety was assessed by monitoring neurological examination findings and vital signs and for the development of adverse reactions. For angiographic assessment, in a blinded fashion, pre- and post-sildenafil images were graded as "improvement" or "no improvement" in CVS. Unblinded measurements were made between pre- and post-sildenafil angiograms. RESULTS: Twelve patients received sildenafil; 5 patients received 10 mg and 7 received 30 mg. There were no adverse reactions. There was no adverse effect on heart rate or intracranial pressure. Sildenafil resulted in a transient decline in mean arterial pressure, an average of 17% with a return to baseline in an average of 18 minutes. Eight patients (67%) were found to have a positive angiographic response to sildenafil, 3 (60%) in the low-dose group and 5 (71%) in the high-dose group. The largest degree of vessel dilation was an average of 0.8 mm (range 0-2.1 mm). This corresponded to an average percentage increase in vessel diameter of 62% (range 0%-200%). CONCLUSIONS: The results from this Phase I safety and proof-of-concept trial assessing the use of intravenous sildenafil in patients with CVS show that sildenafil is safe and well tolerated in the setting of SAH. Furthermore, the angiographic data suggest that sildenafil has a positive impact on human CVS.

Intraparenchymal meningioma within the basal ganglia of a child: A case report.

Intraparenchymal meningiomas are rare. To date, no such lesion has been reported within the basal ganglia of a paediatric patient. Here, we describe the case of a 15-year-old-boy who presented with symptoms referable to a cystic, calcified, left basal ganglia intraparenchymal meningioma and discuss the surgical management of this lesion.

Utility of Screening for Cerebral Vasospasm Using Digital Subtraction Angiography.

BACKGROUND AND PURPOSE: Cerebral arterial vasospasm (CVS) is a common complication of aneurysmal subarachnoid hemorrhage strongly associated with neurological deterioration and delayed cerebral ischemia (DCI). The utility of screening for CVS as a surrogate for early detection of DCI, especially in patients without clinical signs of DCI, remains uncertain. METHODS: We performed a retrospective analysis of 116 aneurysmal subarachnoid hemorrhage patients who underwent screening digital subtraction angiography to determine the association of significant CVS and subsequent development of DCI. Patients were stratified into 3 groups: (1) no symptoms of DCI before screening, (2) ≥1 episodes of suspected DCI symptoms before screening, and (3) unable to detect symptoms because of poor examination. RESULTS: Patients asymptomatic before screening had significantly lower rates of CVS (18%) compared with those with transient symptoms of DCI (60%; P<0.0001). None of the 79 asymptomatic patients developed DCI after screening, regardless of digital subtraction angiography findings, compared with 56% of those with symptoms (P<0.0001). Presence of CVS was significantly associated with DCI in those with transient symptoms and in those whose examinations did not permit clear assessment (odds ratio 16.0, 95% confidence interval 2.2-118.3, P=0.003). CONCLUSIONS: Patients asymptomatic before screening have low rates of CVS and seem to be at negligible risk of developing DCI. Routine screening of asymptomatic patients seems to have little utility. Screening may still be considered in patients with possible symptoms of DCI or those with examinations too poor to clinically detect symptoms because finding CVS may be useful for risk stratification and guiding management.

Acute rupture of a feeding artery aneurysm after embolization of a brain arteriovenous malformation.

BACKGROUND: Staged endovascular embolization of large arteriovenous malformations (AVMs) is frequently performed to gradually reduce flow and prevent abrupt hemodynamic changes. While feeding artery aneurysms have been associated with increased risk of hemorrhage in the setting of AVMs, decisions regarding if and when to treat these aneurysms vary. Acute, fatal rupture of a feeding artery aneurysm following embolization of a large, unruptured AVM has been infrequently reported in the literature. CASE DESCRIPTION: A 69-year-old female presented with headache and mild left hemiparesis referable to a 5 cm right fronto-parieto-temporal AVM with surrounding vasogenic edema. The AVM was associated with numerous bilateral feeding artery aneurysms (the largest was a 2 cm right middle cerebral artery (MCA) bifurcation aneurysm). There was also a large, partially thrombosed venous varix. Staged embolization of the AVM was performed. Several hours after the third stage of her embolization, she became obtunded, with a fixed and dilated right pupil. Head computed tomography (CT) showed a large intraparenchymal hemorrhage with midline shift in the right sylvian fissure, remote from the AVM nidus. She was taken to surgery for a decompressive craniectomy and hematoma evacuation. The MCA aneurysm was confirmed to be the source of hemorrhage and it was clipped. Despite aggressive medical and surgical treatments, the patient died. CONCLUSIONS: An increase in AVM feeding artery pressure following endovascular embolization may contribute to the rupture of a feeding artery aneurysm. For this reason, treatment of large arterial aneurysms on feeding pedicles should be considered prior to embolization of the AVM nidus.

Surgical Revascularization in North American Adults with Moyamoya Phenomenon: Long-Term Angiographic Follow-up.

BACKGROUND: North American and Asian forms of moyamoya have distinct clinical characteristics. Asian adults with moyamoya are known to respond better to direct versus indirect revascularization. It is unclear whether North American adults with moyamoya have a similar long-term angiographic response to direct versus indirect bypass. METHODS: A retrospective review of surgical revascularization for adult moyamoya phenomenon was performed. Preoperative and postoperative cerebral angiograms underwent consensus review, with degree of revascularization quantified as extent of new middle cerebral artery (MCA) territory filling. RESULTS: Late angiographic follow-up was available in 15 symptomatic patients who underwent 20 surgical revascularization procedures. In 10 hemispheres treated solely with indirect arterial bypass, 3 had 2/3 revascularization, 4 had 1/3 revascularization, and 3 had no revascularization of the MCA territory. In the 10 hemispheres treated with direct arterial bypass (8 as a stand-alone procedure and 2 in combination with an indirect procedure), 2 had complete revascularization, 7 had 2/3 revascularization, and 1 had 1/3 revascularization. Direct bypass provided a higher rate of "good" angiographic outcome (complete or 2/3 revascularization) when compared with indirect techniques (P = .0198). CONCLUSIONS: Direct bypass provides a statistically significant, more consistent, and complete cerebral revascularization than indirect techniques in this patient population. This is similar to that reported in the Asian literature, which suggests that the manner of presentation (ischemia in North American adults versus hemorrhage in Asian adults) is likely not a contributor to the extent of revascularization achieved after surgical intervention.

High flow EC-IC bypass for a giant left MCA aneurysm.

Giant cerebral aneurysms may be treated through a variety of options, including aneurysm trapping with concurrent bypass. This video describes the case of a large, recurrent, left middle cerebral artery aneurysm that was treated using a high flow, radial artery bypass graft, from the external carotid artery to the left temporal M2 branch. A step-by-step operative description, with emphasis on proper microsurgical technique, is included. The video can be found here: http://youtu.be/9xTMC6InivQ .

Carotid cavernous sinus fistulas without superior ophthalmic vein enlargement.

PURPOSE: Diagnosis of carotid cavernous fistula (CCF) relies on clinical findings, such as proptosis, chemosis, and pulsatile tinnitus, plus imaging features including enlargement of the superior ophthalmic vein (SOV). This study reviewed patients with CCF, with a focus on those who were clinically symptomatic but had a normal-appearing SOV on routine scans. METHODS: Retrospective review was conducted on the clinical records of patients with CCF seen by ophthalmology or interventional neuroradiology, with attention to clinical and imaging features, angiography findings, management, and outcomes. RESULTS: Forty patients presented with CCF. History of head trauma was present in 13 (average age 43.8 years; all direct or complex), while the remainder occurred spontaneously (average 66 years; 85% indirect). The most common presenting ophthalmologic signs or symptoms were proptosis (65%), binocular diplopia (60%), redness (57.5%), and chemosis (47.5%). After diagnosis, 36 underwent endovascular treatment, with successful occlusion achieved in 90% of cases for whom follow-up data was available (n = 21). Notably, 3 patients with CCF did not have SOV enlargement on any imaging modality including catheter angiography. CONCLUSIONS: In this series of patients with clinical signs of CCF, there was no radiologic evidence of enlarged SOV in 26% of patients on noninvasive imaging and in 8% on catheter angiography. To avoid inappropriate interventions or delays in diagnosis and care, it is important to recognize that CCF can exist without SOV enlargement. Patients with clinical features suspicious for CCF should undergo catheter angiography if treatment is being considered. Endovascular treatment can produce clinical improvement or resolution.

Timing and nature of in-house postoperative events following uncomplicated elective endovascular aneurysm treatment.

OBJECT: Most patients with asymptomatic intracranial aneurysms treated with endovascular methods are closely observed overnight in an intensive care unit setting for complications, including ischemic and hemorrhagic stroke, cardiac dysfunction, and groin access complications. The purpose of this study was to analyze the timing, nature, and rate of in-house postoperative events. METHODS: Patients who underwent endovascular treatment or retreatment of unruptured cerebral aneurysms from March 2002 to June 2012 were identified from a prospective case log and their medical records were reviewed. The presentation, patient characteristics, aneurysm size and location, and method of endovascular treatment of each cerebral aneurysm were recorded. Patients with adverse intraprocedural events including perforation and thromboembolism were excluded from this analysis. Overnight postprocedural monitoring was performed in a neurological intensive care unit or postanesthesia care unit for all patients, with discharge planned for postoperative Day 1. Postprocedural events occurring during hospitalization were categorized as intracranial hemorrhage, ischemic stroke, groin hematoma resulting in additional treatment or prolonged hospital stay, retroperitoneal hematoma, and cardiac events. The time from the completion of the procedure to event discovery was recorded. RESULTS: A total of 687 endovascular treatments of unruptured cerebral aneurysms were performed. Nine treatments were excluded from our analysis due to intraprocedural events. Endovascular procedures included coiling alone, stent-assisted coiling, balloon-assisted coiling, balloon-assisted embolization with a liquid embolic agent, and placement of a flow diversion device with or without coiling. Twenty-seven treatments (4.0%) resulted in postprocedural complications: 3 intracranial hemorrhages, 6 ischemic strokes, 4 cardiac events, 5 retroperitoneal hematomas, and 9 groin hematomas. The majority (20 [74.0%]) of these 27 complications were detected within 4 hours from the procedure. These included 1 hemorrhage, 4 ischemic strokes, 4 cardiac events, 2 retroperitoneal hematomas, and 9 groin hematomas. All cardiac events and groin hematomas were detected within 4 hours. Four (14%) of the 27 complications were detected between 4 and 12 hours, 1 (3.7%) between 12 and 24 hours, and 2 (7.4%) more than 24 hours after the procedure. The complications detected more than 4 hours from the conclusion of the procedure included 2 minor intracranial hemorrhages causing headache and resulting in no permanent deficits, 2 mild ischemic strokes, and 3 asymptomatic retroperitoneal hematomas identified by falling hematocrit levels that required no further intervention or treatment. CONCLUSIONS: The large majority of significant postprocedural events after uncomplicated endovascular aneurysm intervention occur within the first 4 hours; these events become less frequent with increasing time. Transfer to a floor bed after 4-12 hours for further observation is reasonable to consider in some patients.

Advances and surgical considerations in the treatment of moyamoya disease.

Moyamoya is a rare disorder that involves steno-occlusive arterial changes of the anterior circulation, along with proliferative development of basal arterial collaterals. It is either idiopathic (called moyamoya disease) or the result of a specific underlying condition such as atherosclerosis, radiation therapy, or sickle cell disease (called moyamoya syndrome or phenomenon). In recent years, numerous insights into and advances in the understanding, evaluation, and management of moyamoya patients have occurred. This article briefly reviews the spectrum of moyamoya conditions and then provides a synopsis of numerous recent investigations that shed light on various aspects of the disease, including its clinical characteristics, natural history, underlying pathology, imaging, surgical techniques, and long-term patient outcome.

Genetic determinants of cerebral vasospasm, delayed cerebral ischemia, and outcome after aneurysmal subarachnoid hemorrhage.

Despite extensive effort to elucidate the cellular and molecular bases for delayed cerebral injury after aneurysmal subarachnoid hemorrhage (aSAH), the pathophysiology of these events remains poorly understood. Recently, much work has focused on evaluating the genetic underpinnings of various diseases in an effort to delineate the contribution of specific molecular pathways as well as to uncover novel mechanisms. The majority of subarachnoid hemorrhage genetic research has focused on gene expression and linkage studies of these markers as they relate to the development of intracranial aneurysms and their subsequent rupture. Far less work has centered on the genetic determinants of cerebral vasospasm, the predisposition to delayed cerebral injury, and the determinants of ensuing functional outcome after aSAH. The suspected genes are diverse and encompass multiple functional systems including fibrinolysis, inflammation, vascular reactivity, and neuronal repair. To this end, we present a systematic review of 21 studies suggesting a genetic basis for clinical outcome after aSAH, with a special emphasis on the pathogenesis of cerebral vasospasm and delayed cerebral ischemia. In addition, we highlight potential pitfalls in the interpretation of genetic association studies, and call for uniformity of design of larger multicenter studies in the future.

The complement cascade: new avenues in stroke therapy.

Recent evidence has shown that after the initial occlusion, a large portion of stroke patients achieve some degree of reperfusion either through collateral circulation or clot dissolution. However, it appears that this reperfusion may lead to increased inflammation-induced damage. Even though the exact mechanism of this secondary injury is unclear, several experimental studies have indicated an intimate connection between complement and this secondary form of damage. We review the available literature and attempt to identify promising clinical therapeutic targets.