Splenic Artery Embolization in a Patient With Intracranial Hemorrhage Due to Refractory Persistent Immune Thrombocytopenia.

BACKGROUND: Managing intracranial bleeding in patients with refractory immune thrombocytopenia is difficult. OBSERVATION: A 16-year-old female refractory to prednisolone, intravenous immunoglobulin, eltrombopag, and cyclosporin exhibited heavy menstrual bleeding requiring packed red blood cell transfusions. Autoimmune antibodies were detected, indicating of lupus, and hydroxychloroquine sulfate was administered. In month 6 following the diagnosis, the patient presented with intracranial hemorrhage. Splenic artery embolization promptly increased platelets, and the patient was discharged without any neurological sequela. In month 5 of embolization, the patient's platelet count continued to exceed 300,000/µL without any medical treatment. CONCLUSIONS: Splenic artery embolization is a life-saving procedure in refractory immune thrombocytopenia.

Use of intravenous cangrelor in the treatment of ruptured and unruptured cerebral aneurysms: an updated single-center analysis and pooled analysis of current studies.

BACKGROUND: Intracranial stent placement for the treatment of cerebral aneurysms is increasingly utilized in both ruptured and unruptured scenarios. Intravenous (IV) cangrelor is a relatively new antiplatelet agent that was initially approved for coronary interventions. In addition to our institution, five other centers have published their results using IV cangrelor in neurointerventional procedures. This article combines the aneurysm treatment data from all prior studies to provide insight into the safety and efficacy of cangrelor for intracranial aneurysm treatment. METHODS: A prospectively maintained database was reviewed to identify all cases of IV cangrelor administration during aneurysm embolization. 20 additional patients were identified who had not been previously published. In addition, a literature search was performed to identify prior publications regarding cangrelor in neurointervention. The data from these were combined with our institutional results in a pooled-analysis. RESULTS: Overall, 85 patients who received IV cangrelor during aneurysm embolization were identified, including 46 ruptured and 39 unruptured cases. The asymptomatic and symptomatic intracranial hemorrhage rates were 4% (2/46) for ruptured cases and 2.6% (1/39) for unruptured cases. The rate of retroperitoneal hematoma and gastrointestinal bleeding was 0%. There were no incidents of intraprocedural thromboembolic complication or intraprocedural in-stent thrombosis in either cohort. One subject suffered an ischemic stroke at 24 hours secondary to in-stent thrombosis in a ruptured case. CONCLUSIONS: IV cangrelor during aneurysm embolization appears to be safe, with a symptomatic intracranial hemorrhage rate of 4% in ruptured cases and 2.6% in unruptured cases. More research is needed to determine the ideal dosing regimen.

Natural history, angiographic presentation and outcomes of anterior cranial fossa dural arteriovenous fistulas.

BACKGROUND: Anterior cranial fossa dural arteriovenous fistulas (ACF-dAVFs) are aggressive vascular lesions. The pattern of venous drainage is the most important determinant of symptoms. Due to the absence of a venous sinus in the anterior cranial fossa, most ACF-dAVFs have some degree of drainage through small cortical veins. We describe the natural history, angiographic presentation and outcomes of the largest cohort of ACF-dAVFs. METHODS: The CONDOR consortium includes data from 12 international centers. Patients included in the study were diagnosed with an arteriovenous fistula between 1990-2017. ACF-dAVFs were selected from a cohort of 1077 arteriovenous fistulas. The presentation, angioarchitecture and treatment outcomes of ACF-dAVF were extracted and analyzed. RESULTS: 60 ACF-dAVFs were included in the analysis. Most ACF-dAVFs were symptomatic (38/60, 63%). The most common symptomatic presentation was intracranial hemorrhage (22/38, 57%). Most ACF-dAVFs drained through cortical veins (85%, 51/60), which in most instances drained into the superior sagittal sinus (63%, 32/51). The presence of cortical venous drainage predicted symptomatic presentation (OR 9.4, CI 1.98 to 69.1, p=0.01). Microsurgery was the most effective modality of treatment. 56% (19/34) of symptomatic patients who were treated had complete resolution of symptoms. Improvement of symptoms was not observed in untreated symptomatic ACF-dAVFs. CONCLUSION: Most ACF-dAVFs have a symptomatic presentation. Drainage through cortical veins is a key angiographic feature of ACF-dAVFs that accounts for their malignant course. Microsurgery is the most effective treatment. Due to the high risk of bleeding, closure of ACF-dAVFs is indicated regardless of presentation.

Clinical Characteristics and Multimodality Therapy Outcomes in 304 Pediatric Patients with Cerebral Arteriovenous Malformations.

BACKGROUND: Clinical follow-up data of pediatric patients with cerebral arteriovenous malformations (AVMs) are limited. This study investigated the characteristics of AVMs in children and analyzed the clinical outcomes of multimodality therapy in pediatric patients with AVMs at a single center. METHODS: This retrospective study included consecutive patients diagnosed with AVMs at our institution between August 2008 and June 2018. Data on demographic characteristics, AVM features, and clinical outcomes were collected. Patients aged <18 years at admission were defined as children. RESULTS: Overall, 1009 patients with AVMs were included, with 304 (30.1%) patients aged <18 years. AVMs in pediatric patients were more likely to present with intracranial hemorrhage, mostly located in deep areas of the brain. A small nidus, exclusively deep drainage, and deep AVM location were associated with hemorrhage in children; Kaplan-Meier analysis revealed that patients with ruptured AVMs had a higher risk of developing a follow-up hemorrhage than those with unruptured AVMs. Among 290 children who were followed up, the multivariate regression analysis showed that a higher pretreatment modified Rankin Scale score, deep AVM location, and conservative treatment were significantly associated with unfavorable outcomes. CONCLUSIONS: In pediatric patients, AVMs were more likely to present with intracranial hemorrhage than that in adults. Hemorrhagic presentation in children was associated with a small nidus, exclusively deep drainage, and deep AVM location. Pediatric patients with ruptured AVMs had significantly higher risks of follow-up hemorrhage than those with unruptured AVMs. Our clinical results suggest that nonconservative treatment is better for pediatric patients with AVMs.

Sphenoparietal Sinus Dural Arteriovenous Fistulas: A Series of 10 Patients.

BACKGROUND: Dural arteriovenous fistulas (DAVFs) of the sphenoparietal sinus or sphenoid wing region are uncommon lesions with unique and interesting angioarchitecture. Understanding appropriate anatomy and recognizing patterns provide important treatment implications. OBJECTIVE: To describe a single surgeon's experience with open surgical treatment of sphenoparietal sinus DAVFs, the surgical indications for this uncommon lesion, and the microsurgical techniques related to its treatment and to review the literature on its surgical treatment. METHODS: Consecutive cases of sphenoparietal sinus DAVF treatment conducted by a single surgeon over 24 years (1997-2020) were retrospectively reviewed. Published reports of similar cases were reviewed. RESULTS: Of 202 surgically treated DAVFs, 10 lesions in 10 patients were sphenoparietal sinus DAVFs. Four patients presented with intracranial hemorrhage, 3 with headache, and 2 with pulsatile tinnitus; 1 patient was incidentally identified as having a DAVF during treatment for a ruptured aneurysm. Most patients (7 of 10) had undergone endovascular embolization previously. Nine patients had Borden type III DAVFs and one had a Borden type II fistula. Surgery in all 10 patients resulted in angiographically confirmed fistula obliteration. Clinical outcomes at the last follow-up, measured by a modified Rankin Scale (mRS) score, were excellent in 6 patients (mRS ≤ 2) and poor in 1 patient (mRS ≥ 3); late outcomes were not available for 3 patients. CONCLUSION: Sphenoparietal sinus DAVFs are an uncommon anatomic subtype. Careful attention to angiographic detail leads to identification of the site of venous interruption and results in a high rate of surgical cure with excellent clinical outcomes.

Evaluation of theTherapeutic Effect of the Hybrid Operation on Patients with Aneurysms Associated with Cerebral Arteriovenous Malformations with different Redekop Classifications.

BACKGROUND: Intracranial aneurysms associated with cerebral arteriovenous malformations (AVMs) are a rare condition in the clinic, and treatment is very difficult due to their particular anatomical features. We present our experience in the treatment of intracranial aneurysms with AVMs and evaluate the effectiveness and safety of endovascular treatment combined with microsurgical resection (the hybrid operation). METHODS: This was a single-center retrospective study in our neurosurgical department from January 2015 to January 2021. We collected clinical data from 48 patients with intracranial aneurysms associated with AVMs and categorized them according to Redekop classifications according to the results of cerebral imaging examination to compare the therapeutic effects of endovascular embolization and the hybrid operation. RESULTS: Compared to nonaneurysmal AVMs, intracranial aneurysms with AVMs more often presented with intracranial hemorrhage (P<0.05). Massive hematoma and severe neurological impairment were more often found in patients with intracranial aneurysms with AVMs (P<0.05). For flow-related aneurysms, the hybrid surgery had a higher one-stage cure rate than endovascular embolization alone (P<0.05). Both treatment methods had similar effects on intranidal aneurysms (P>0.05). There were no significant differences in prognostic indicators between the two treatments. However, the recurrence rate of AVMs with proximal flow-related aneurysms was lower in patients who underwent the hybrid operation (P<0.05). CONCLUSION: The hybrid operation was safe and effective for patients with intracranial aneurysms associated with AVMs. For flow-related aneurysms, the one-stage cure rate was higher and the recurrence rate was lower with the hybrid operation than with endovascular embolization alone.

A proposed algorithm for evaluation and management of pediatric hemophilia patients who present to the emergency department with head trauma.

Hemophilia is the deficiency of plasma clotting factor VIII (hemophilia A) or IX (hemophilia B) where management focuses on the prevention and treatment of acute bleeding symptoms and their sequelae. The most concerning risk is for life-threatening bleeding, including intracranial hemorrhage (ICH), which is caused by head trauma. Guidelines exist for the evaluation and management of pediatric head trauma, including the Pediatric Emergency Care Applied Research Network (PECARN) protocol, but limited evidence exists for when hemophilia patients present to the emergency department (ED), specifically with head trauma. Literature is limited regarding ICH and hemophilia, which further supports the culture of uncertainty among providers. The objective of this study is to conduct a retrospective chart review to determine the prevalence and clinical characteristics of ICH, and to describe computed tomography (CT) scan use in hemophilia patients who present to Phoenix Children's Hospital (PCH) ED with head trauma from January 1, 2007 to June 1, 2019. A total of 89 ED visits and 43 patients met inclusion criteria, and prevalence of ICH was determined to be 4% with the patients presenting with varied clinical characteristics and few commonalities. Using these data, we propose a new algorithm to aid clinicians in determining the need for CT scan in pediatric hemophilia patients who present to the ED with head trauma.

Dose-dependent benefits of iron-magnetic nanoparticle-coated human umbilical-derived mesenchymal stem cell treatment in rat intracranial hemorrhage model.

BACKGROUND: This study tested whether two doses of human umbilical-derived mesenchymal stem cells (hUC-MSCs) were superior to one dose for protecting the brain against intracranial hemorrhage (ICH) induced by intracranial injection collagenase and the capacity of ironic-magnetic-nanoparticles (Ir-MNa) coated hUC-MSCs tracked by MRI. METHODS AND RESULTS: Adult male SD rats (n = 40) were equally categorized into group 1 (sham-operated-control), group 2 (ICH), group 3 [ICH + Ir-MNa-coated hUC-MSCs/1.2 × 106 cells with an extracorporeal magnet over rat head (eCMag)/administered by left internal carotid artery (LICA) at post-3 h ICH], and group 4 (ICH + Ir-MNa-coated hUC-MSCs/1.2 × 106 cells with an eCMag/administered post-3 h ICH by LICA and 24 h by IV) and euthanized by day 28. The result showed that by day 28 after ICH induction the neurological function was severely impaired in group 2 than in group 1 that was significantly improved in group 3 and further significantly improved in group 4, whereas ICH volume exhibited an opposite pattern of neurological impairment among the groups (all p < 0.0001). Brain MRI demonstrated that by 4 h after ICH, Ir-MNa-coated hUC-MSCs were abundantly identified in ischemic area in group 4. The protein expressions of inflammatory (TNF-α/MMP-9/IL-1ß/iNOS)/oxidative-stress (NOX-1/NOX-2/oxidized protein)/apoptotic (caspase-3/mitochondrial Bax/PARP)/fibrotic (Smad3/TGF-ß)/mitochondrial-damaged (cytosolic-cytochrome-C) biomarkers displayed an identical pattern of neurological impairment among the groups (all p < 0.0001). The cellular expressions of inflammation (CD68+/CD11b+)/brain edema (AQP4+) biomarkers exhibited an identical pattern, whereas the neuronal-myelin (Doublecortin+/NeuN/nestin) biomarkers displayed an opposite pattern of neurological impairment (all p < 0.0001). CONCLUSION: Two doses of hUC-MSCs were superior to just one dose for protecting the brain against ICH-induced damage and Ir-MNa-coated hUC-MSCs offered a well adopted method for tracking hUC-MSCs homing into the brain.

End-of-Life Decisions of Intracranial Hemorrhage Patients Successfully Weaned From Prolonged Mechanical Ventilation.

Factors related to the end-of-life decisions of patients with intracranial hemorrhage who were successfully weaned from prolonged mechanical ventilation remain unclear. This study aimed to evaluate factors that influence the end-of-life decisions of these patients. METHODS: This retrospective study examined patients with intracranial hemorrhage successfully weaned from prolonged mechanical ventilation between January 2012 and December 2017. The following data was collected and analyzed: age, gender, comorbidities, Glasgow Coma Scale scores, receipt or non-receipt of intracranial hemorrhage surgery, discharge status, and end-of-life decisions. RESULTS: In total, 91 patients with intracranial hemorrhage were successfully weaned from prolonged mechanical ventilation. The families of 62 (68.1%) patients signed the do-not-resuscitate order. A Glasgow Coma Scale score of ≥10 at discharge from the respiratory care center and zero comorbidities were the influencing factors between patients whose do-not-resuscitate orders were signed and those whose orders were not signed. Patients with intracranial hemorrhage successfully weaned from prolonged mechanical ventilation had chronic kidney disease comorbidity and Glasgow Coma Scale score of <7 on admission to respiratory care center with a general ward mortality rate of 83.3%. CONCLUSIONS: The families of intracranial hemorrhage patients with multiple comorbidities and higher neurologic impairment after successful weaning from the ventilator believed that palliative therapy would provide a greater benefit. Patients with intracranial hemorrhage successfully weaned from prolonged mechanical ventilation with chronic kidney disease comorbidity and Glasgow Coma Scale score of <7 on admission to respiratory care center are candidates for the consideration of hospice care with ventilator withdrawal.

Institutional trends over a decade in catheter-directed interventions for pulmonary embolism.

OBJECTIVE: Catheter-directed interventions (CDIs) are commonly performed for acute pulmonary embolism (PE). The evolving catheter types and treatment algorithms impact the use and outcomes of these interventions. This study aimed to investigate the changes in CDI practice and their impact on outcomes. METHODS: Patients who underwent CDIs for PE between 2010 and 2019 at a single institution were identified from a prospectively maintained database. A PE team was launched in 2012, and in 2014 was established as an official Pulmonary Embolism Response Team. CDI annual use trends and clinical failures were recorded. Clinical success was defined as physiologic improvement in the absence of major bleeding, perioperative stroke or other procedure-related adverse event, decompensation for submassive or persistent shock for massive PE, the need for surgical thromboembolectomy, or death. Major bleeding was defined as requiring a blood transfusion, a surgical intervention, or suffering from an intracranial hemorrhage. RESULTS: There were 372 patients who underwent a CDI for acute PE during the study period with a mean age of 58.9 ± 15.4 years; there were males 187 (50.3%) and 340 patients has a submassive PE (91.4%). CDI showed a steep increase in the early Pulmonary Embolism Response Team years, peaking in 2016 with a subsequent decrease. Ultrasound-assisted thrombolysis was the predominant CDI technique peaking at 84% of all CDI in 2014. Suction thrombectomy use peaked at 15.2% of CDI in 2019. The mean alteplase dose with catheter thrombolysis techniques decreased from 26.8 ± 12.5 mg in 2013 to 13.9 ± 7.5 mg in 2019 (P < .001). The mean lysis time decreased from 17.2 ± 8.3 hours in 2013 to 11.3 ± 8.2 hours in 2019 (P < .001). Clinical success for the massive and the submassive PE cohorts was 58.1% and 91.2%, respectively; the major bleed rates were 25.0% and 5.3%. There were two major clinical success peaks, one in 2015 mirroring our technical learning curve and one in 2019 mirroring our patient selection learning curve. The clinical success decrease in 2018 was primarily derived from blood transfusions owing to acute blood loss during suction thrombectomy. CONCLUSIONS: CDIs for acute PE have rapidly evolved with high success rates. Multidisciplinary approaches among centers with appropriate expertise are advisable for the safe and successful implementation of catheter interventions.

Review of treatment and therapeutic targets in brain arteriovenous malformation.

Brain arteriovenous malformations (bAVM) are an important cause of intracranial hemorrhage (ICH), especially in younger patients. The pathogenesis of bAVM are largely unknown. Current understanding of bAVM etiology is based on studying genetic syndromes, animal models, and surgically resected specimens from patients. The identification of activating somatic mutations in the Kirsten rat sarcoma viral oncogene homologue (KRAS) gene and other mitogen-activated protein kinase (MAPK) pathway genes has opened up new avenues for bAVM study, leading to a paradigm shift to search for somatic, de novo mutations in sporadic bAVMs instead of focusing on inherited genetic mutations. Through the development of new models and understanding of pathways involved in maintaining normal vascular structure and functions, promising therapeutic targets have been identified and safety and efficacy studies are underway in animal models and in patients. The goal of this paper is to provide a thorough review or current diagnostic and treatment tools, known genes and key pathways involved in bAVM pathogenesis to summarize current treatment options and potential therapeutic targets uncovered by recent discoveries.

Intracranial Hemorrhage Associated With T-Cell Acute Lymphoblastic Leukemia With Hyperleukocytosis: A Case Report.

Acute leukemia in children may present with hyperleukocytosis. Symptomatic hyperleukocytosis is a medical emergency that necessitates rapid stabilization of the patient and prompt lowering of the leukocyte count. We report on a patient with intracranial hemorrhage associated with T-cell acute lymphoblastic leukemia with hyperleukocytosis, which is a rare occurrence. A 16-year-old boy with hyperleukocytosis (total white cell count; 398×103/µL) underwent repeated leukapheresis and received supportive treatment until a definite diagnosis of T-cell acute lymphoblastic leukemia was made and chemotherapy was started at 10% of the usual dose. On day 2 of treatment, he had headache, vomiting, and was agitated. Brain magnetic resonance imaging showed bilateral extensive hemispheric and cerebellar punctate areas of hemorrhage and perilesional edema. Chemotherapy intensified to a maximum dose on day 3. If supportive care for tumor lysis syndrome can be promptly provided, initial chemotherapy regimen can immediately be begun at an optimal dose.

Clinical management and outcome of adult patients with extracorporeal life support device-associated intracerebral hemorrhage-a neurocritical perspective and grading.

Intracerebral hemorrhage (ICH) is a devastating complication in patients treated with extracorporeal membrane oxygenation (ECMO) due to respiratory or cardiac issues. Neurosurgical evaluation and management of such cases has only insufficiently been studied. We conducted a retrospective, cohort study of adult patients treated with ECMO between January 2007 and January 2017 in a tertiary healthcare center. Demographics, clinical data, coagulation status, ICH characteristics, and treatment modalities were analyzed. The primary outcome parameter was defined as mortality caused by ICH during ECMO. 525 patients with ECMO therapy were eligible for analysis. An overall incidence for any type of intracranial bleeding of 12.3% was found. Small hemorrhages accounted for 6.4% and acute subdural and epidural hematoma for 1.2%. Twenty-four (4.6%) patients developed ICH, and 11 patients (46%) died due to the ICH. Mortality was significantly higher in patients with larger ICH volumes (86.8 ± 34.8 ml vs 9.9 ± 20.3 ml, p < 0.001), intraventricular hemorrhage (83% vs 8%, p = 0.01), and a fluid level inside the ICH (75% vs 31%, p = 0.04). All patients were classified according to the bleeding pattern on the initial CT scan into 3 types. Patients with type 1 bleeding were statistically more likely to die (p < 0.001). In 15 out of 24 patients (63%), correction of the coagulation status was possible within 12 h after ICH onset. Seven out of 9 patients (78%) without early coagulation correction died compared to 2 out of 15 patients (13%), in whom early coagulation correction was successful (p = 0.01). This is the first study evaluating the course and management of patients experiencing an ICH under ECMO therapy and establishing an ICH classification based on the bleeding patterns. Early correction of the coagulation is of paramount importance in the treatment of these patients.

Pediatric Cerebral Cavernous Malformations.

Cerebral cavernous malformations are the second most common vascular malformations in the central nervous system, and over one-third are found in children. Lesions may be solitary or multiple, be discovered incidentally, be sporadic, or be secondary to familial cavernomatosis or radiation therapy. Children may present with focal seizures, intracranial hemorrhage, or focal neurological deficits without radiological evidence of recent hemorrhage. We present several children with cerebral cavernous malformations and explore the challenges of their diagnosis in children, their key imaging features, the role of follow-up imaging, and their subsequent management including stereotactic radiosurgery and microsurgical resection. Individual patient risk stratification is advocated for all affected children and their families.

Impact of Intracranial Pressure Monitor-Guided Therapy on Neurologic Outcome After Spontaneous Nontraumatic Intracranial Hemorrhage.

OBJECTIVES: Intracranial pressure (ICP) monitors have been used in some patients with spontaneous intracranial hemorrhage (ICH) to provide information to guide treatment without clear evidence for its use in this population. We assessed the impact of ICP monitor placement, including external ventricular drains and intraparenchymal monitors, on neurologic outcome in this population. MATERIALS AND METHODS: In this secondary analysis of the Minimally Invasive Surgery Plus Alteplase for Intracerebral Hemorrhage Evacuation III trial, the primary outcome was poor outcome (modified Rankin Scale score 4-6) and the secondary outcome was death, at 1 year from onset. We compared outcomes in patients with or without an ICP monitor using unadjusted and adjusted logistic regression models. The analyses were repeated in a balanced cohort created with propensity score matching. RESULTS: Seventy patients underwent ICP monitor placement and 424 did not. Poor outcome was seen in 77.1% of patients in the ICP-monitor subgroup compared with 53.8% in the no-monitor subgroup (p<0.001). Of patients in the ICP-monitor subgroup, 31.4% died, compared with 21.0% in the no-monitor subgroup (p=0.053). In multivariate models, ICP monitor placement was associated with a >2-fold greater risk of poor outcome (odds ratio 2.76, 95% CI 1.30-5.85, p=0.008), but not with death (p=0.652). Our findings remained consistent in the propensity score-matched cohort. CONCLUSION: These results question whether ICP monitor-guided therapy in patients with spontaneous nontraumatic ICH improves outcome. Further work is required to define the causal pathway and improve identification of patients that might benefit from invasive ICP monitoring.

Management of Intracranial Hemorrhage in Patients with a Left Ventricular Assist Device: A Systematic Review and Meta-Analysis.

BACKGROUND: Intracranial hemorrhage (ICH) has been reported to occur in up to 23% of patients with left ventricular assist devices (LVADs). Currently, limited data exists to guide neurosurgical management strategies to optimize outcomes in patients with an LVAD who develop ICH. METHODS: A systematic review and meta-analysis of the literature was performed to evaluate the mortality rate in these patients following medical and/or surgical management and to evaluate antithrombotic reversal and resumption strategies after hemorrhage. RESULTS: 17 studies reporting on 3869 LVAD patients and 545 intracranial hemorrhages spanning investigative periods from 1996 to 2019 were included. The rate of ICH in LVAD patients was 10.6% (411/3869) with 58.6% (231/394) being intraparenchymal hemorrhage (IPH), 23.6% (93/394) subarachnoid hemorrhage (SAH), and 15.5% (61/394) subdural hemorrhage (SDH). Total mortality rates for surgical management 65.6% (40/61) differed from medical management at 45.2% (109/241). There was an increased relative risk of mortality (RR=1.45, 95% CI: 1.10-1.91, p = 0.01) for ICH patients undergoing surgical intervention. The hemorrhage subtype most frequently managed with anticoagulation reversal was IPH 81.8% (63/77), followed by SDH 52.2% (12/23), and SAH 39.1% (18/46). Mean number of days until antithrombotic resumption ranged from 6 to 10.5 days. CONCLUSION: Outcomes remain poor, specifically for those undergoing surgery. As experience with this population increases, prospective studies are warranted to contribute to management and prognostication .

High Prevalence of Moyamoya Syndrome in Appalachia.

INTRODUCTION: Moyamoya is a chronic cerebrovascular condition of unclear etiology characterized by progressive occlusion of 1 or both internal carotid arteries with neovascular collateral formation. With both an idiopathic form (moya-moya disease) and congenital condition-associated form (moyamoya syndrome), it can cause ischemic and hemorrhagic stroke. Recent findings in Kentucky have challenged traditional estimates of its incidence in US populations. Using the Kentucky Appalachian Stroke Registry (KApSR), our aim was to further characterize its incidence as a cause of stroke and to understand the patient population in Appalachia. METHODS: A retrospective review of moyamoya patients was performed using the KApSR database. Data collected included demographics, county location, risk factors, comorbidities, and health-care encounters from January 1, 2012, to December 31, 2016. RESULTS: Sixty-seven patients were identified; 36 (53.7%) resided in Appalachian counties. The cohort accounted for 125 of 6,305 stroke admissions, representing an incidence of 1,983 per 100,000 stroke admissions. Patients presented with ischemic strokes rather than hemorrhagic strokes (odds ratio 5.50, 95% CI: 2.74-11.04, p < 0.01). Eleven patients (16.4%) exhibited autoimmune disorders. Compared to the general population with autoimmune disorder prevalence of 4.5%, the presence of autoimmunity within the cohort was significantly higher (p < 0.01). Compared to non-Appalachian patients, Appalachian patients tended to present with lower frequencies of tobacco use (p = 0.08), diabetes mellitus (p = 0.13), and hypertension (p = 0.16). CONCLUSIONS: Moyamoya accounts for a substantial number of stroke admissions in Kentucky; these patients were more likely to develop an ischemic stroke rather than a hemorrhagic stroke. Autoimmune disorders were more prevalent in moyamoya patients than in the general population. The reduced frequency of traditional stroke risk factors within the Appalachian group suggests an etiology distinct to the population.

Any Instrument in a Surgeon's Hand can be Fatal: Unusual Iliac Artery Injury in Lumbar Spinal Deformity Surgery.

BACKGROUND: Vascular complications, which we rarely encounter during lumbosacral stabilization surgeries, can be life-threatening if they are not treated quickly. These arterial injuries occur during screw insertion. Our presentation with the common iliac artery injury during the decortication process in transverse processes with the "pedicle awl" will be the first case in the literature to our knowledge. CASE DESCRIPTION: Lumbosacral decompression and stabilization surgery was performed in a 57-year-old patient with L1-S1 spinal stenosis and scoliosis. After the stabilization process was completed, while decorticating the transverse processes with the pedicle awl, the tool fell to the paravertebral region, and then active arterial hemorrhage was observed at the surgical site. Hemostasis was achieved in the surgical field, but a rapid progressive drop was observed in the patient's blood pressure. The surgery was quickly terminated, and the patient was turned to the supine position. Vascular surgeons opened the abdomen with a midline laparotomy, and approximately 2600 mL hematoma was evacuated from the retroperitoneum. The 5-mm defect in the left common iliac artery was repaired by primary suturing. The patient had no problem in postoperative follow-up and was discharged on the 10th postoperative day. CONCLUSIONS: In these complications that we rarely encounter in lumbosacral stabilization surgeries, perioperative findings should be well evaluated, and rapid intervention should be made in cases in which vascular injury is considered. One must remember that every tool used during surgery can be dangerous even in an experienced hand.

Galenic Pial Arteriovenous Fistulas in Adults.

BACKGROUND: Vein of Galen aneurysmal malformations (VOGMs) are pial arteriovenous fistulas possessing Galenic venous drainage most commonly presenting during the neonatal period and infancy, with initial discovery during adulthood quite rare. OBJECTIVES AND METHODS: We conducted a literature survey of the PubMed database in order to identify Galenic pial arteriovenous fistulas (GPAVFs) with major manifestation or initial presentation during adulthood. Inclusionary criteria included pial AVFs with Galenic drainage with major manifestation or initial presentation at, or older than, 18 years. Exclusionary criteria included exclusive pediatric onset of symptomatology attributable to GPAVFs without a new onset major presentation during adulthood, exclusive or major dural arterial supply, arteriovenous malformations with Galenic drainage, developmental venous anomalies with Galenic drainage, isolated varices or anomalies of the vein of Galen, and any lesions with uncertainty regarding true GPAVF nature. RESULTS: Our search generated 1589 articles. Excluding duplicates, 26 cases met criteria for evaluation. Mean age was 34.1 +/- 2.53 years. Clinical presentations of GPAVFs among adults included headache, intracranial hemorrhage, seizures, and focal neurologic deficits. Management strategies included observation (n = 5), emergent ventriculostomy or Torkildsen shunt (n = 3), cerebrospinal fluid diversion via ventriculoperitoneal shunting (n = 4), microsurgical obliteration or thrombectomy (n = 4), transarterial and/or transvenous embolotherapeutic obliteration (n = 7), and concurrent embolotherapy and radiosurgical irradiation (n = 1). CONCLUSIONS: GPAVFs in adults often present with symptomatology of mild severity and may be effectively managed conservatively, though occasionally present catastrophically or may be treated via cerebrospinal fluid diversion, microsurgical obliteration, or endovascular embolization. Severity sufficient to require emergent intervention portended a poor outcome.

Acute Hemorrhage After Intracerebral Biopsy in COVID-19 Patients: Report of 3 Cases.

BACKGROUND: When Belgium's coronavirus disease 2019 (COVID-19) outbreak began in March 2020, our neurosurgical department followed the protocol of most surgical departments in the world and postponed elective surgery. However, patients with tumor-like brain lesions requiring urgent surgery still received treatment as usual, in order to ensure ongoing neurooncologic care. From a series of 31 patients admitted for brain surgery, 3 were confirmed as infected by the novel severe acute respiratory syndrome coronavirus 2. CASE DESCRIPTION: We present the clinical outcomes of these 3 COVID-19 patients, who underwent an intracerebral biopsy in our department during April 2020. All suffered from a diffuse intraparenchymal hemorrhage postoperatively. Unfortunately, we were not able to identify a clear etiology of these postoperative complications. It could be hypothesized that an active COVID-19 infection status may be related to a higher bleeding risk. The remaining 28 neurooncologic non-COVID-19 patients underwent uneventful surgery during the same period. CONCLUSIONS: This case series reports the previously unreported and unexpected outcomes of COVID-19 patients suffering from acute hemorrhage after intracerebral biopsy procedures. Although no direct relation can yet be established, we recommend the neurosurgical community be cautious in such cases.