Arteriovenous malformation presenting with epilepsy: a multimodal approach to diagnosis and treatment.

Arteriovenous malformation (AVM) presenting with epilepsy significantly impacts patient quality of life, and it should be considered very much a seizure disorder. Although hemorrhage prevention is the primary treatment aim of AVM surgery, seizure control should also be at the forefront of therapeutic management. Several hemodynamic and morphological characteristics of AVM have been identified to be associated with seizure presentation. This includes increased AVM flow, presence of long pial draining vein, venous outflow obstruction, and frontotemporal location, among other aspects. With the advent of high-throughput image processing and quantification methods, new radiographic attributes of AVM-related epilepsy have been identified. With respect to therapy, several treatment approaches are available, including conservative management or interventional modalities; this includes microsurgery, radiosurgery, and embolization or a combination thereof. Many studies, especially in the domain of microsurgery and radiosurgery, evaluate both techniques with respect to seizure outcomes. The advantage of microsurgery lies in superior AVM obliteration rates and swift seizure response. In addition, by incorporating electrophysiological monitoring during AVM resection, adjacent or even remote epileptogenic foci can be identified, leading to extended lesionectomy and improved seizure control. Radiosurgery, despite resulting in reduced AVM obliteration and prolonged time to seizure freedom, avoids the risks of surgery altogether and may provide seizure control through various antiepileptic mechanisms. Embolization continues to be used as an adjuvant for both microsurgery and radiosurgery. In this study, the authors review the latest imaging techniques in characterizing AVM-related epilepsy, in addition to reviewing each treatment modality.

The role of contrast-enhanced ultrasound in neurosurgical disease.

Contrast-enhanced ultrasound (CEUS) is a relatively new imaging modality in the realm of neurosurgical disease. CEUS permits the examination of blood flow through arteries, veins, and capillaries via intravascular contrast agents and allows vascular architectural mapping with extreme sensitivity and specificity. While it has established utility in other organ systems such as the liver and kidneys, CEUS has not been studied extensively in the brain. This report presents a review of the literature on the neurosurgical applications of CEUS and provides an outline of the imaging modality's role in the diagnosis, evaluation, and treatment of neurosurgical disease.

Cerebral revascularization in cerebral proliferative angiopathy: a systematic review.

OBJECTIVECerebral proliferative angiopathy (CPA) is considered a discrete vascular malformation of the brain separate from classical brain arteriovenous malformations (AVMs). It has unique angiographic characteristics and has been hypothesized to result from chronic cortical ischemia and perinidal oligemia. Treatment with cerebral revascularization has been proposed in an attempt to disrupt regional hypoperfusion and interrupt the angiogenesis that defines CPA. A systematic review of the literature pertaining to the role of cerebral revascularization may highlight a treatment paradigm for this rare disease.METHODSA systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. MEDLINE and Embase were searched from inception for papers relating to CPA. Included articles were categorized according to methodology (case series or imaging study) and treatment modality (conservative, radiation, endovascular, or revascularization). A synthesis was compiled summarizing the current evidence regarding cerebral revascularization in CPA.RESULTSThe initial search revealed 43 articles, of which 28 studies met the inclusion criteria. Nine studies were identified that described imaging findings, which suggested hemodynamic dysregulation and perinidal impairments in the cerebrovascular reserve could be identified compared to unaffected hemispheres and classical brain AVMs. Six studies including 7 patients undergoing indirect forms of cerebral revascularization were identified. Clinical and radiological outcomes following revascularization were favorable in all but one study.CONCLUSIONSA small body of radiological and clinical studies has emerged, suggesting that CPA is a response to perinidal oligemia. While the long-term clinical efficacy of revascularization remains unclear, early results suggest that this may be a novel treatment paradigm for patients with CPA.

Role of pregnancy and female sex steroids on aneurysm formation, growth, and rupture: a systematic review of the literature.

OBJECTIVE: Women have been shown to have a higher risk of cerebral aneurysm formation, growth, and rupture than men. The authors present a review of the recently published neurosurgical literature that studies the role of pregnancy and female sex steroids, to provide a conceptual framework with which to understand the various risk factors associated with cerebral aneurysms in women at different stages in their lives. METHODS: The PubMed database was searched for "("intracranial" OR "cerebral") AND "aneurysm" AND ("pregnancy" OR "estrogen" OR "progesterone")" between January 1980 and February 2019. A total of 392 articles were initially identified, and after applying inclusion and exclusion criteria, 20 papers were selected for review and analysis. These papers were then divided into two categories: 1) epidemiological studies about the formation, growth, rupture, and management of cerebral aneurysms in pregnancy; and 2) investigations on female sex steroids and cerebral aneurysms (animal studies and epidemiological studies). RESULTS: The 20 articles presented in this study include 7 epidemiological articles on pregnancy and cerebral aneurysms, 3 articles reporting case series of cerebral aneurysms treated by endovascular therapies in pregnancy, 3 epidemiological articles reporting the relationship between female sex steroids and cerebral aneurysms through retrospective case-control studies, and 7 experimental studies using animal and/or cell models to understand the relationship between female sex steroids and cerebral aneurysms. The studies in this review report similar risk of aneurysm rupture in pregnant women compared to the general population. Most ruptured aneurysms in pregnancy occur during the 3rd trimester, and most pregnant women who present with cerebral aneurysm have caesarean section deliveries. Endovascular treatment of cerebral aneurysms in pregnancy is shown to provide a new and safe form of therapy for these cases. Epidemiological studies of postmenopausal women show that estrogen hormone therapy and later age at menopause are associated with a lower risk of cerebral aneurysm than in matched controls. Experimental studies in animal models corroborate this epidemiological finding; estrogen deficiency causes endothelial dysfunction and inflammation, which may predispose to the formation and rupture of cerebral aneurysms, while exogenous estrogen treatment in this population may lower this risk. CONCLUSIONS: The aim of this work is to equip the neurosurgical and obstetrical/gynecological readership with the tools to better understand, critique, and apply findings from research on sex differences in cerebral aneurysms.

The use of contrast-enhanced, time-resolved magnetic resonance angiography in cerebrovascular pathology.

Digital subtraction angiography (DSA) has long been the imaging gold standard in the evaluation, treatment, and follow-up of cerebro- and spinovascular disorders. However, DSA has the disadvantages of invasiveness, contrast allergy or nephropathy, the impracticality of procedural preparation and recovery, and expense. Contrast-enhanced (CE), time-resolved (TR) magnetic resonance angiography (CE TR-MRA) is a sophisticated, relatively novel imaging modality that provides multiphasic contrast-enhanced visualization of the neurovasculature. Given the crucial role of angiography in all aspects of care for patients with complex neurovascular disorders, it is incumbent on those who care for these patients to understand the usefulness and pitfalls of novel imaging in this arena to ensure best practices, and to deliver cutting edge care to these patients in a way that minimizes cost, but does not compromise quality. CE TR-MRA has the potential to play an expanded role in the workup and follow-up across the spectrum of neurovascular disease, and this review is aimed to help neurosurgeons better understand how CE TR-MRA can be used to better manage patients in this cohort.

Dynamic assessment of venous anatomy and function in neurosurgery with real-time intraoperative multimodal ultrasound: technical note.

The relevance of the cerebral venous system is often underestimated during neurosurgical procedures. Damage to this draining system can have catastrophic implications for the patient. Surgical decision-making and planning must consider each component of the venous compartment, from the medullary draining vein to the dural sinuses and extracranial veins. Intraoperative ultrasound (ioUS) permits the real-time study of venous compartments using different modalities, thus allowing complete characterization of their anatomical and functional features. The B-mode (brightness mode) offers a high-resolution anatomical representation of veins and their relationships with lesions. Doppler modalities (color, power, spectral) allow the study of blood flow and identification of vessels to distinguish their functional characteristics. Contrast-enhanced US allows one to perform real-time angiosonography showing both the functional and the anatomical aspects of vessels. In this technical report, the authors demonstrate the different applications of multimodal ioUS in neurosurgery for identifying the anatomical and functional characteristics of the venous compartment. They discuss the general principles and technical nuances of ioUS and analyze their potential implications for the study of various venous districts during neurosurgical procedures.

Use of ICG videoangiography and FLOW 800 analysis to identify the patient-specific venous circulation and predict the effect of venous sacrifice: a retrospective study of 172 patients.

OBJECTIVE The best management of veins encountered during the neurosurgical approach is still a matter of debate. Even if venous sacrifice were to lead to devastating consequences, under certain circumstances, it might prove to be desirable, enlarging the surgical field or increasing the extent of resection in tumor surgery. In this study, the authors present a large series of patients with vascular or oncological entities, in which they used indocyanine green videoangiography (ICG-VA) with FLOW 800 analysis to study the patient-specific venous flow characteristics and the management workflow in cases in which a venous sacrifice was necessary. METHODS Between May 2011 and December 2017, 1972 patients were admitted to the authors' division for tumor and/or neurovascular surgery. They retrospectively reviewed all cases in which ICG-VA and FLOW 800 were used intraoperatively with a specific target in the venous angiographic phase or for the management of venous sacrifice, and whose surgical videos and FLOW 800 analysis were available. RESULTS A total of 296 ICG-VA and FLOW 800 studies were performed intraoperatively. In all cases, the venous structures were clearly identifiable and were described according to the flow direction and speed. The authors therefore defined different patterns of presentation: arterialized veins, thrombosed veins, fast-draining veins with anterograde flow, slow-draining veins with anterograde flow, and slow-draining veins with retrograde flow. In 16 cases we also performed a temporary clipping test to predict the effect of the venous sacrifice by the identification of potential collateral circulation. CONCLUSIONS ICG-VA and FLOW 800 analysis can provide complete and real-time intraoperative information regarding patient-specific venous drainage pattern and can guide the decision-making process regarding venous sacrifice, with a possible impact on reduction of surgical complications.

Transvenous embolization of brain arteriovenous malformations: a review of techniques, indications, and outcomes.

Endovascular embolization of brain arteriovenous malformations (AVMs) is conventionally performed from a transarterial approach. Transarterial AVM embolization can be a standalone treatment or, more commonly, used as a neoadjuvant therapy prior to microsurgery or stereotactic radiosurgery. In contrast to the transarterial approach, curative embolization of AVMs may be more readily achieved from a transvenous approach. Transvenous embolization is considered a salvage therapy in contemporary AVM management. Proposed indications for this approach include a small (diameter < 3 cm) and compact AVM nidus, deep AVM location, hemorrhagic presentation, single draining vein, lack of an accessible arterial pedicle, exclusive arterial supply by perforators, and en passage feeding arteries. Available studies of transvenous AVM embolization in the literature have reported high complete obliteration rates, with reasonably low complication rates. However, evaluating the efficacy and safety of this approach is challenging due to the limited number of published cases. In this review the authors describe the technical considerations, indications, and outcomes of transvenous AVM embolization.

Preoperative embolization of skull base meningiomas: current indications, techniques, and pearls for complication avoidance.

Skull base meningiomas are technically challenging tumors to treat because of their deep vascular supply that can preclude early devascularization during resection. Preoperative embolization of these arterial feeders is thought to decrease blood loss and facilitate resection; however, given the complex and varied anatomy of these skull base lesions, preoperative embolization is not without risk. It is essential for both endovascular and skull base neurosurgeons to understand these risks in light of the potential benefits. The authors review the vascular anatomy of skull base meningiomas, indications for preoperative devascularization, endovascular techniques, and published results regarding embolization of these lesions.

A review of extraaxial developmental venous anomalies of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, venous varices or aneurysmal malformations, and enlarged emissary veins.

This paper is a narrative review of extraaxial developmental venous anomalies (eDVAs) of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, enlarged emissary veins, and venous varices or aneurysmal malformations. The article highlights the natural history, anatomy, embryology, imaging, clinical implications, and neurosurgical significance of these lesions, which the authors believe represent a continuum, with different entities characterized by distinct embryopathologic features. The indications and surgical management options are discussed for these individual intracranial pathologies with relevant illustrations, and a novel classification is proposed for persistent falcine sinus (PFS). The role of neurointervention and/or microsurgery in specific cases such as sinus pericranii and enlarged emissary veins of the skull is highlighted. A better understanding of the pathophysiology and developmental anatomy of these lesions can reduce treatment morbidity and mortality. Some patients, including those with vein of Galen malformations (VOGMs), can present with the added systemic morbidity of a high-output cardiac failure. Although VOGM is the most studied and classified of the above-mentioned eDVAs, the authors believe that grouping the former with the other venous anomalies/abnormalities listed above would enable the clinician to convey the exact morphophysiological configuration of these lesions, predict their natural history with respect to evolving venous hypertension or stroke, and extrapolate invaluable insights from VOGM treatment to the treatment of other eDVAs. In recent years, many of these symptomatic venous malformations have been treated with endovascular interventions, although these techniques are still being refined. The authors highlight the broad concept of eDVAs and hope that this work will serve as a basis for future studies investigating the role of evolving focal venous hypertension/global intracranial hypertension and possibilities of fetal surgical intervention in these cases.

Novel use of flow diversion for the treatment of aneurysms associated with arteriovenous malformations.

The use of flow-diverting stents for intracranial aneurysms has become more prevalent, and flow diverters are now routinely used beyond their initial scope of approval at the proximal internal carotid artery. Although flow diversion for the treatment of cerebral aneurysms is becoming more commonplace, there have been no reports of its use to treat flow-related cerebral aneurysms associated with arteriovenous malformations (AVMs). The authors report the cases of 2 patients whose AVM-associated aneurysms were managed with flow diversion. A 40-year-old woman presented with a history of headaches that led to the identification of an unruptured Spetzler-Martin Grade V, right parietooccipital AVM associated with 3 aneurysms of the ipsilateral internal carotid artery. Initial attempts at balloon-assisted coil embolization of the aneurysms were unsuccessful. The patient underwent placement of a flow-diverting stent across the diseased vessel; a 6-month follow-up angiogram demonstrated complete occlusion of the aneurysms. In the second case, a 57-year-old man presented with new-onset seizures, and an unruptured Spetzler-Martin Grade V, right frontal AVM associated with an irregular, wide-necked anterior communicating artery aneurysm was identified. The patient underwent placement of a flow-diverting stent, and complete occlusion of the aneurysm was observed on a 7-month follow-up angiogram. These 2 cases illustrate the potential for use of flow diversion as a treatment strategy for feeding artery aneurysms associated with AVMs. Because of the need for dual antiplatelet medications after flow diversion in this patient population, however, this strategy should be used judiciously.

Initial experience with a robotically operated video optical telescopic-microscope in cranial neurosurgery: feasibility, safety, and clinical applications.

OBJECTIVE The move toward better, more effective optical visualization in the field of neurosurgery has been a focus of technological innovation. In this study, the authors' objectives are to describe the feasibility and safety of a new robotic optical platform, namely, the robotically operated video optical telescopic-microscope (ROVOT-m), in cranial microsurgical applications. METHODS A prospective database comprising patients who underwent a cranial procedure between April 2015 and September 2016 was queried, and the first 200 patients who met the inclusion criteria were selected as the cohort for a retrospective chart review. Only adults who underwent microsurgical procedures in which the ROVOT-m was used were considered for the study. Preoperative, intraoperative, and postoperative data were retrieved from electronic medical records. The authors address the feasibility and safety of the ROVOT-m by studying various intraoperative variables and by reporting perioperative morbidity and mortality, respectively. To assess the learning curve, cranial procedures were categorized into 6 progressively increasing complexity groups. The main categories of pathology were I) intracerebral hemorrhages (ICHs); II) intraaxial tumors involving noneloquent regions or noncomplex extraaxial tumors; III) intraaxial tumors involving eloquent regions; IV) skull base pathologies; V) intraventricular lesions; and VI) cerebrovascular lesions. In addition, the entire cohort was evenly divided into early and late cohorts. RESULTS The patient cohort comprised 104 female (52%) and 96 male (48%) patients with a mean age of 56.7 years. The most common pathological entities encountered were neoplastic lesions (153, 76.5%), followed by ICH (20, 10%). The distribution of cases by complexity categories was 11.5%, 36.5%, 22%, 20%, 3.5%, and 6.5% for Categories I, II, II, IV, V, and VI, respectively. In all 200 cases, the surgical goal was achieved without the need for intraoperative conversion. Overall, the authors encountered 3 (1.5%) major neurological morbidities and 6 (3%) 30-day mortalities. Four of the 6 deaths were in the ICH group, resulting in a 1% mortality rate for the remainder of the cohort when excluding these patients. None of the intraoperative complications were considered to be attributable to the visualization provided by the ROVOT-m. When comparing the early and late cohorts, the authors noticed an increase in the proportion of higher-complexity surgeries (Categories IV-VI), from 23% in the early cohort, to 37% in the late cohort (p = 0.030). In addition, a significant reduction in operating room setup time was demonstrated (p < 0.01). CONCLUSIONS The feasibility and safety of the ROVOT-m was demonstrated in a wide range of cranial microsurgical applications. The authors report a gradual increase in case complexity over time, representing an incremental acquisition of experience with this technology. A learning curve of both setup and execution phases should be anticipated by new adopters of the robot system. Further prospective studies are required to address the efficacy of ROVOT-m. This system may play a role in neurosurgery as an integrated platform that is applicable to a variety of cranial procedures.

Establishing reconstructive neurosurgery as a subspecialty.

Neurosurgery is experiencing the emergence of a new subspecialty focused on function restoration. New, evolving, and reappraised surgical procedures have provided an opportunity to restore function to many patients with previously undertreated disorders. Candidates for reconstruction were previously limited to those with peripheral nerve and brachial plexus injuries, but this has been expanded to include stroke, spinal cord injury, and a host of other paralyzing disorders affecting both upper and lower motor neurons. Similar to the recent evolution of the well-established subdisciplines of spinal and vascular neurosurgery, reconstructive neurosurgery requires the adaptation of techniques and skills that were not traditionally a part of neurosurgical training. Neurosurgeons-as the specialists who already manage this patient population and possess the requisite surgical skills to master the required techniques-have a unique opportunity to lead the development of this field. The full development of this subspecialty will lay the foundation for the subsequent addition of emerging treatments, such as neuroprosthetics and stem cell-based interventions. As such, reconstructive neurosurgery represents an important aspect of neurosurgical training that can ameliorate many of the deficits encountered in the traditional practice of neurosurgery.

Intramedullary hemangioblastomas: surgical results in 16 patients.

OBJECT Hemangioblastomas are rare, benign, highly vascularized tumors that can be found throughout the neuraxis but are mainly located in the cerebellum and in the spinal cord. Spinal hemangioblastomas can present with motor and sensory deficits, whose severity varies according to the size and location of the tumor. Resection is the best treatment option to avoid neurological deterioration. The authors report surgical results in the treatment of intramedullary hemangioblastomas and discuss the technical nuances important to achieving total resection without adding new deficits. METHODS A consecutive series of patients with intramedullary hemangioblastomas operated on between 2000 and 2014 by the senior author (H.T.) is presented. The functional scale proposed by McCormick was used to evaluate the patients' neurological status before and after surgery. RESULTS Sixteen patients were included in the study and underwent 17 surgeries. Follow-up was at least 6 months. Age at presentation varied from 13 to 58 years (mean 33.8 years). Ten patients (62.5%) were males and 6 patients (37.5%) were females. Seven (43.75%) of the 16 patients had associated von Hippel-Lindau syndrome, with hemangioblastomas also presenting in other locations. Three patients had multiple tumors in the same segment in the spinal cord, and 10 patients (62.5%) presented with cysts. According to the site of presentation, 11 tumors (68.75%) were localized at the cervical region (including the cervicomedullary junction) and 5 tumors (31.25%) at the thoracic level. Total resection was achieved in all cases, evidenced by postoperative MRI. Four patients had some functional worsening immediately after surgery. After 6 months, 1 patient had functional worsening compared with preoperative status, and 2 patients had clinical improvement. The majority of the patients remained clinically stable postoperatively. CONCLUSIONS Adequate knowledge of anatomy and the correct use of microsurgical techniques allowed total resection of these tumors, with minimal morbidity and maximum functional recovery. Outcome seems to be directly related to the neurological status before surgery.

Stereotactic radiosurgery with and without embolization for intracranial arteriovenous malformations: a systematic review and meta-analysis.

OBJECT: The effectiveness and risk of stereotactic radiosurgery (SRS) in the management of partially embolized intracranial arteriovenous malformations (AVMs) remain controversial. The aim of this analysis was to assess current evidence regarding the efficiency and safety of SRS for AVM patients with and without prior embolization. METHODS: To compare SRS in patients with and without embolization, the authors conducted a meta-analysis of studies by searching the literature via PubMed and EMBASE for the period between January 2000 and December 2013, complemented by a hand search. Primary outcome was the rate of AVM obliteration on a 3-year follow-up angiogram. Secondary outcome was the rate of hemorrhage at 3 years after SRS. Tertiary outcome was permanent neurological deficits related to radiation-induced changes. RESULTS: Ten studies eligible for analysis included 1988 patients: 593 had undergone embolization followed by SRS and 1395 had undergone SRS alone. The AVM obliteration rate was significantly lower in patients who had undergone embolization followed by SRS than in those who had undergone SRS alone (41.0% vs 59%, OR 0.46, 95% CI 0.37-0.56, p < 0.00001). However, the rates of hemorrhage (7.3% vs 5.6%, OR 1.17, 95% CI 0.74-1.83, p = 0.50) and permanent neurological deficits related to radiation-induced changes (3.3% vs 3.4%, OR 1.41, 95% CI 0.64-3.11, p = 0.39) were not significantly different between the two groups. CONCLUSIONS: Embolization before SRS significantly decreases the AVM obliteration rate. However, there is no significant difference in the risk of hemorrhage and permanent neurological deficits after SRS alone and following embolization. Further validation by well-designed prospective or randomized cohort studies is still needed.

Microsurgery for cerebral arteriovenous malformations: postoperative outcomes and predictors of complications in 264 cases.

OBJECT: The authors conducted a study to assess the safety and efficacy of microsurgical resection of arteriovenous malformations (AVMs) and determine predictors of complications. METHODS: A total of 264 patients with cerebral AVMs were treated with microsurgical resection between 1994 and 2010 at the Jefferson Hospital for Neuroscience. A review of patient data was performed, including initial hemorrhage, clinical presentation, Spetzler-Martin (SM) grade, treatment modalities, clinical outcomes, and obliteration rates. Univariate and multivariate analyses were used to determine predictors of operative complications. RESULTS: Of the 264 patients treated with microsurgery, 120 (45%) patients initially presented with hemorrhage. There were 27 SM Grade I lesions (10.2%), 101 Grade II lesions (38.3%), 96 Grade III lesions (36.4%), 31 Grade IV lesions (11.7%), and 9 Grade V lesions (3.4%). Among these patients, 102 (38.6%) had undergone prior endovascular embolization. In all patients, resection resulted in complete obliteration of the AVM. Complications occurred in 19 (7.2%) patients and resulted in permanent neurological deficits in 5 (1.9%). In multivariate analysis, predictors of complications were increasing AVM size (OR 3.2, 95% CI 1.5-6.6; p = 0.001), increasing number of embolizations (OR 1.6, 95% CI 1.1-2.2; p = 0.01), and unruptured AVMs (OR 2.7, 95% CI 1-7.2; p = 0.05). CONCLUSIONS: Microsurgical resection of AVMs is highly efficient and can be undertaken with low rates of morbidity at high-volume neurovascular centers. Unruptured and larger AVMs were associated with higher complication rates.

Intraoperative arteriovenous malformation rupture: causes, management techniques, outcomes, and the effect of neurosurgeon experience.

OBJECT: Intraoperative rupture can transform an arteriovenous malformation (AVM) resection. Blood suffuses the field and visualization is lost; suction must clear the field and the hand holding the suction device is immobilized; the resection stalls while hemostasis is being reestablished; the cause and site of the bleeding may be unclear; bleeding may force technical errors and morbidity from chasing the source into eloquent white matter; and AVM bleeding can be so brisk that it overwhelms the neurosurgeon. The authors reviewed their experience with this dangerous complication to examine its causes, management, and outcomes. METHODS: From a cohort of 591 patients with AVMs treated surgically during a 15-year period, 32 patients (5%) experienced intraoperative AVM rupture. Their prospective data and medical records were reviewed. RESULTS: Intraoperative AVM rupture was not correlated with presenting hemorrhage, but had a slightly higher incidence infratentorially (7%) than supratentorially (5%). Rupture was due to arterial bleeding in 18 patients (56%), premature occlusion of a major draining vein in 10 (31%), and nidal penetration in 4 (13%). In 14 cases (44%), bleeding control was abandoned and the AVM was removed immediately ("commando resection"). The incidence of intraoperative rupture was highest during the initial 5-year period (9%) and dropped to 3% and 4% in the second and third 5-year periods, respectively. Ruptures due to premature venous occlusion and nidal penetration diminished with experience, whereas those due to arterial bleeding remained steady. Despite intraoperative rupture, 90% of AVMs were completely resected initially and all of them ultimately. Intraoperative rupture negatively impacted outcome, with significantly higher final modified Rankin Scale scores (mean 2.8) than in the overall cohort (mean 1.5; p < 0.001). CONCLUSIONS: Intraoperative AVM rupture is an uncommon complication caused by pathological arterial anatomy and by technical mistakes in judging the dissection distance from the AVM margin and in mishandling or misinterpreting the draining veins. The decrease in intraoperative rupture rate over time suggests the existence of a learning curve. In contrast, intraoperative rupture due to arterial bleeding reflects the difficulty with dysplastic feeding vessels and deep perforator anatomy rather than neurosurgeon experience. The results demonstrate that intraoperative AVM rupture negatively impacts patient outcome, and that skills in managing this catastrophe are critical.

Stereotactic radiosurgery of intracranial arteriovenous malformations and the use of the K index in determining treatment dose.

OBJECT: The appropriate dose during stereotactic radiosurgery (SRS) of cerebral arteriovenous malformations (AVMs) remains a matter of debate. In the present study, the authors retrospectively evaluated the association of using a prescribed dose calculated utilizing the K index with the obliteration rate of cerebral AVMs after SRS. METHODS: The authors performed a retrospective analysis of the Cleveland Clinic SRS database. All patients undergoing Gamma Knife radiosurgery for cerebral AVMs from 1997 to 2010 were selected. Regression techniques and Kaplan-Meier analyses were used to investigate the effect of divergence from the optimal K index dose on the rate of AVM obliteration. RESULTS: In the study period 152 patients (mean age 43.6 years; 53.9% of treatments were performed in females) underwent 165 Gamma Knife radiosurgery treatments for AVMs. In a univariate analysis Spetzler-Martin grade (OR 0.63 [95% CI 0.42-0.93]), higher AVM score (OR 0.43 [95% CI 0.27-0.70]), larger AVM volume (OR 0.88 [95% CI 0.82-0.94]), and higher maximum diameter (OR 0.56 [95% CI 0.41-0.77]) were associated with a lower rate of AVM obliteration. Higher margin dose (OR 1.16 [95% CI 1.08-1.24]) and higher maximum dose (OR 1.08 [95% CI 1.04-1.13]) were associated with a higher obliteration rate. To further examine the effect of prescribed dose divergence from the calculated K index dose, cases were classified to groups depending on the AVM volume and dose variance from the ideal K index dose. Contingency tables and Kaplan-Meier curves were then created, and no significant differences in rates of obliteration were noted among the different groups. CONCLUSIONS: Gamma Knife radiosurgery for cerebral AVMs remains an effective and safe treatment modality. Smaller AVMs may receive doses less than the calculated K index dose without an apparent effect on obliteration rates.

Smoking is a negative predictor of arteriovenous malformation posttreatment obliteration: analysis of vascular risk factors in 774 patients.

OBJECT: Cigarette smoking has been well established as a risk factor in vascular pathology, such as cerebral aneurysms. However, tobacco's implications for patients with cerebral arteriovenous malformations (AVMs) are controversial. The object of this study was to identify predictors of AVM obliteration and risk factors for complications. METHODS: The authors conducted a retrospective analysis of a prospectively maintained database for all patients with AVMs treated using surgical excision, staged endovascular embolization (with N-butyl-cyanoacrylate or Onyx), stereotactic radiosurgery (Gamma Knife or Linear Accelerator), or a combination thereof between 1994 and 2010. Medical risk factors, such as smoking, abuse of alcohol or intravenous recreational drugs, hypercholesterolemia, diabetes mellitus, hypertension, and coronary artery disease, were documented. A multivariate logistic regression analysis was conducted to detect predictors of periprocedural complications, obliteration, and posttreatment hemorrhage. RESULTS: Of 774 patients treated at a single tertiary care cerebrovascular center, 35% initially presented with symptomatic hemorrhage and 57.6% achieved complete obliteration according to digital subtraction angiography (DSA) or MRI. In a multivariate analysis a negative smoking history (OR 1.9, p = 0.006) was a strong independent predictor of AVM obliteration. Of the patients with obliterated AVMs, 31.9% were smokers, whereas 45% were not (p = 0.05). Multivariate analysis of obliteration, after controlling for AVM size and location (eloquent vs noneloquent tissue), revealed that nonsmokers were more likely (0.082) to have obliterated AVMs through radiosurgery. Smoking was not predictive of treatment complications or posttreatment hemorrhage. Abuse of alcohol or intravenous recreational drugs, hypercholesterolemia, diabetes mellitus, and coronary artery disease had no discernible effect on AVM obliteration, periprocedural complications, or posttreatment hemorrhage. CONCLUSIONS: Cerebral AVM patients with a history of smoking are significantly less likely than those without a smoking history to have complete AVM obliteration on follow-up DSA or MRI. Therefore, patients with AVMs should be strongly advised to quit smoking.

Association between posterior fossa arteriovenous malformations and prenidal aneurysm rupture: potential impact on management.

OBJECT: In patients with posterior fossa arteriovenous malformations (AVMs) who present with hemorrhage, feeding artery aneurysms are often the source of bleeding. The aim of this study was to evaluate the relative proportions of cases of infra- and supratentorial AVMs in which patients presented with prenidal aneurysm rupture. The management and outcome of 9 cases of posterior fossa AVMs associated with prenidal aneurysm rupture are presented. METHODS: The authors retrospectively reviewed 233 consecutive AVM cases involving patients treated at their institution between April 2001 and August 2012. Patients with a prenidal aneurysm as the cause of the hemorrhage were identified. The frequencies of prenidal aneurysm-related ruptures were compared in cases of supra- and infratentorial AVMs. Management and clinical outcome (modified Rankin Scale [mRS] score) of patients with posterior fossa AVMs treated for ruptured prenidal aneurysms were recorded. RESULTS: Of 233 AVMs, 25 (11%) were in the posterior fossa, and in 22 (88%) of these cases, the patients presented with hemorrhage, including 9 patients (41%) who presented with hemorrhage due to prenidal aneurysm rupture. Of 208 patients with supratentorial AVMs, 107 (51%) presented with hemorrhage, including 5 patients (4.7%) in whom the hemorrhage was associated with a prenidal aneurysm (p < 0.01). All 9 patients with posterior fossa AVMs and prenidal aneurysm rupture were treated with early embolization of the offending aneurysm. There was no early rebleeding or clinical complication related to this approach. At the end of follow-up (mean 46.8 months), 2 patients had an mRS score of 0, 1 had a score of 1, 3 had a score of 2, 2 patients were dead (mRS score of 6), and 1 patient was lost to follow-up. CONCLUSIONS: Posterior fossa AVM hemorrhages are frequently associated with prenidal arterial aneurysms. Urgent endovascular treatment of the aneurysm was effective in this case series.