Eric J. Arias

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.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.

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.

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.

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.

Acute Rupture of a Previously Unruptured, Untreated Intracerebral Aneurysm during Induced Hypertension for Vasospasm in SubarachnoidHemorrhage

Acute Rupture of a Previously Unruptured, Untreated Intracerebral Aneurysm during Induced Hypertension for Vasospasm in Subarachnoid Hemorrhage Patients with aneurysmal subarachnoid hemorrhage (SAH) often have other unruptured, untreated intracerebral aneurysms following treatment of the ruptured aneurysm. If symptomatic vasospasm then occurs, vasopressor-induced hypertension (VIH) can be initiated to prevent delayed ischemic neurological deficits (DIND) resulting from vasospasm-induced cerebral ischemia. While induced arterial hypertension is commonly utilized in this clinical scenario, the risk of rupturing a previously unruptured aneurysm during VIH therapy is unknown.

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.

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.

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

Abstract 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.