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Myeloid immune cells are abundant in both ruptured and unruptured brain arteriovenous malformations (bAVMs). The role of central nervous system (CNS) resident and circulating monocyte-derived macrophages in bAVM pathogenesis has not been fully understood. We hypothesize that CNS resident macrophages enhance bAVM development and hemorrhage. RNA sequencing using cultured endothelial cells (ECs) and mouse bAVM samples revealed that downregulation of two bAVM causative genes, activin-like kinase 1 (ALK1) or endoglin, increased inflammation and innate immune signaling. To understand the role of CNS resident macrophages in bAVM development and hemorrhage, we administrated a colony-stimulating factor 1 receptor inhibitor to bAVM mice with brain focal Alk1 deletion. Transient depletion of CNS resident macrophages at an early stage of bAVM development mitigated the phenotype severity of bAVM, including a prolonged inhibition of angiogenesis, dysplastic vasculature formation, and infiltration of CNS resident and circulating monocyte-derived macrophages during bAVM development. Transient depletion of CNS resident macrophages increased EC tight junction protein expression, reduced the number of dysplasia vessels and severe hemorrhage in established bAVMs. Thus, EC AVM causative gene mutation can activate CNS resident macrophages promoting bAVM progression. CNS resident macrophage could be a therapeutic target to mitigate the development and severity of bAVMs.

ABSTRACT 5q14.3 microdeletion syndrome is a rare condition involving multiple genes such as MEF2C and RASA1 and is potentially classified as a neurocutaneous syndrome. Deletion of the MEF2C gene accounts for the majority of clinical manifestations, including global developmental delay, intellectual disability, seizures, and behavioral disorders. RASA1 deletion is linked to capillary malformations with arteriovenous malformations (CM‐AVM). Until now, only 17 cases have been described with deletions of both genes. We present the first case described in Spain with the microdeletion in the 5q14.3 cytoband simultaneously affecting both MEF2C and RASA1, exhibiting the typical manifestations of this entity, and review the published cases to date. [ABSTRACT FROM AUTHOR]

Purpose: This study aims to explore the impact of ultra-early neurological deterioration (U-END) on the outcome (mortality and poor neurological status) following a brain arteriovenous malformation (BAVM) rupture and identify determinants of U-END. Methods: Patients with BAVM ruptures admitted to a single tertiary care center were retrospectively reviewed. U-END was defined as a worsening by two or more points on the Glasgow Coma Scale (GCS). U-END was tested as a potential predictor of in-hospital mortality and poor outcomes. Univariate and multivariate analyses were performed to identify determinants of U-END. Patients with U-END were also matched and compared with BAVM rupture controls presenting with a GCS close or equal to either their initial or their lowest GCS. Results: A total of 248 patients with BAVM ruptures met the inclusion criteria, with 39 (15.7%) patients presenting with U-END. U-END was not associated with and was not an independent predictor of in-hospital mortality (12.8 vs. 10.5% in the rest of the study population; p = 0.67) or poor outcomes (39.5 vs. 36.9%; p = 0.77). The only independent determinants of U-END were hydrocephalus (OR 2.6 [95%CI, 1.1-6.4]; p = 0.03) and intraventricular hemorrhage (IVH; OR 3.5 [95%CI, 1.1-11.7]; p = 0.04). When compared to the initial GCS control group, U-END patients more often presented with IVH (89.5 vs. 64.1%; p = 0.009) and hydrocephalus (73 vs. 38.5%; p = 0.003). When compared to the lowest GCS control group, U-END patients had lower early S100B serum levels (0.35 ± 0.37 vs. 0.83 ± 1; p = 0.009) and a lower rate of poor outcome (39.5 vs. 64.9%; p = 0.03). Conclusion: Ultra-early neurological deterioration in ruptured BAVMs did not result in increased mortality or poor outcomes and was most often related to IVH and hydrocephalus. [ABSTRACT FROM AUTHOR]

Background: Brain arteriovenous malformations (AVMs) are challenging vascular lesions. Extensive follow-up studies are necessary to refine the therapeutic algorithm, and to improve long-term survival in these patients. The aim of the study was to assess surgical outcomes, and to evaluate overall long-term mortality in patients treated for brain AVMs. Methods: This retrospective single-center study included 191 patients with brain AVMs, admitted between 2012 and 2022. Clinical and angiographical particularities have been analyzed, to identify factors that might influence early outcome and overall long-term mortality. Results: Out of 79 patients undergoing surgery, 51 had ruptured AVMs with total resection achieved in 68 cases (86.1%). Deep venous drainage was associated with incomplete resection. Female sex, admission modified Rankin Scale (mRS) > 2, and eloquent location were independent predictors of poor outcomes. Multiple venous drainage was associated with a higher risk of worsened early outcome. Eloquent brain region involvement, conservative treatment, increasing age, admission mRS > 2, and comorbidities significantly decrease survival in brain AVM patients. Patients treated with interventional treatments had significantly better survival than the conservatively managed ones, when adjusting for age and admission mRS. Conclusion: The study identified female sex, poor neurologic status on admission and eloquence as independent prognostic factors for a negative outcome after surgery. Patients who received interventional treatment had significantly better survival than patients managed conservatively. We recommend employing tailored, proactive management strategies as they significantly enhance longterm survival in brain AVM patients. [ABSTRACT FROM AUTHOR]

Purpose: Recent innovations in radiological imaging have enabled the detection of micro-remnant niduses of arteriovenous malformations (AVMs) after gamma knife radiosurgery (GKS), which have not been previously perceptible. Herein, we focus on the difficulty of evaluating micro-remnant AVMs after GKS that are hardly perceptible on conventional examinations and propose integrating follow-up three-dimensional rotational angiography (3D-RA) in the previous gamma plan as a solution. Methods: We retrospectively searched NTT Medical Center Tokyo hospital database for patients with AVMs who underwent both two-dimensional digital subtraction angiography (2D-DSA) and 3D-RA as follow-up for GKS from February 2021 to January 2024. Patients with suspected nidus occlusion on the latest non-contrast-enhanced magnetic resonance angiography (NC-MRA) were included, and contrast-enhanced magnetic resonance angiography (CE-MRA), 2D-DSA, and 3D-RA were evaluated. Results: Twelve patients with 13 AVM sites were defined as having complete nidus occlusion on upfront NC-MRA. On 2D-DSA, seven AVM sites showed the presence of slight remaining AVMs based on the detection of remnant drainage veins, however the nidus was not detected in three cases. Nevertheless, 3D-RA detected micro-remnant niduses in all seven AVM sites, and four patients underwent re-GKS. Nine patients with ten AVM sites also underwent CE-MRA, and six AVM sites were diagnosed with radiation-induced parenchymal injury. Conclusion: Importing the 3D-RA image into the treatment planning has the potential to be more helpful than NC-MRA or CE-MRA to detect micro-remnant AVMs and evaluate the true remnant volume, and may contribute to a more detailed treatment planning, thereby improving the results of GKS retreatment. [ABSTRACT FROM AUTHOR]

Objective: Advancements in AVM surgical techniques for cerebral arteriovenous malformation (AVM) underscore its efficacy. Our research aims to showcase the positive outcomes of treating low-grade AVMs surgically, focusing on safety and effectiveness. Methods : We retrospectively reviewed 55 patients (36 males and 19 females; average age 37.4 years) with Spetzler-Martin (S-M) grade 1 and 2 AVMs who underwent surgical resection between January 2009 and December 2022. Results : In our study, 55 patients with S-M grade 1 and 2 AVMs underwent surgical resection, evenly divided between grades 1 (50.9%) and 2 (49.1%). Intracranial hemorrhage was the primary symptom in 74.5% of cases. Pre-operative Glasgow coma scale (GCS) scores revealed 69.1% of patients scored above 13, with 18% below 8. Successful resection was achieved in 87.3%. Postoperatively, 95.5% of ruptured and 90.9% of unruptured AVM patients showed lower or same modified Rankin scale scores. Poorer outcomes were significantly linked to lower GCS scores and intranidal/flow-related aneurysms through multivariate logistic regression. Postoperative seizures noted in nine patients, were exclusive to the ruptured AVM group. Conclusion : Our findings indicate surgical resection as a beneficial treatment for low-grade AVMs, yielding high cure rates and positive functional outcomes in both ruptured and unruptured cases. Preoperative GCS scores and the presence of associated aneurysms are predictive of postoperative functional status. Additionally, managing postoperative seizures effectively is key to enhancing prognosis. [ABSTRACT FROM AUTHOR]

Double lumen balloon catheters (DLBCs) have emerged as a potential alternative to single lumen balloon catheters for endovascular embolization of arteriovenous malformations (AVMs) and dural arteriovenous fistulas (dAVFs). This study describes our preliminary experience with the Eclipse 2L DLBC in treating AVMs and dAVFs. Patients who underwent embolization of cranial dAVFs or AVMs at our institution from August 2021 to March 2024 were included. Spinal vascular malformations were excluded. Descriptive statistics were used to analyze procedural outcomes, technical nuances, and postoperative outcomes on follow-up. Twenty-five patients who underwent 38 embolization procedures (15 AVMs and 23 dAVFs) met criteria for inclusion in this study. The mean age of the cohort was 52.44 (standard deviation = 17.26), and 48% of the overall cohort (n = 13) was female. The average procedure times for AVMs and dAVFs were 80.4 minutes and 96.73 minutes, respectively. There was 1 instance of catheter entrapment. Two patients in the AVM cohort experienced mortality, and 1 experienced postoperative rupture. Our preliminary experience using the Eclipse 2L balloon catheter for Onyx embolization reported procedural outcomes comparable to other DLBCs despite relatively higher procedure times and radiation doses. Further long-term studies on its efficacy as primary modality in treating AVMs and dAVFs are encouraged. [ABSTRACT FROM AUTHOR]

Radiation induced changes (RIC) are the most common complications observed post Gamma Knife radiosurgery (GKRS) and may be observed within 6–18 months post procedure. It has been observed that almost one-third of RICs are symptomatic and half of them are persistent. There is no way to predict which patients will develop these changes and to what extent. This was a prospective analytical pilot study with the aim of understanding the role of serum vascular endothelial growth factor (VEGF) and endostatin as predictive factors for clinically symptomatic RIC in intracranial arteriovenous malformations (AVMs) of Spetzler Martin (SM) grade 3 being managed with primary GKRS. A total of 15 patients were analyzed; 60% of them had a history of bleed. The median volume of AVM nidus was 4.36 mL. One-third of the patients had no imaging changes suggestive of RIC at 1 year follow-up and 2 of the patients had symptomatic RIC needing intervention. Before GKRS, the median values of serum concentration of endostatin and VEGF were 34.98 ng/mL and 168.37 pg/mL, respectively. The serum values of VEGF at 1 month post GKRS was much lower than the pre-GKRS values but not found to be predictive of RIC. No correlation could be observed with the levels of serum endostatin and RIC. Some patients may develop resistant edema and necrosis post GKRS for intracranial AVMs, which may warrant medical and surgical intervention. Serum biomarkers like VEGF and endostatin may vary in the post GKRS period and can be used to identify at-risk cases, however more studies are needed to decide on appropriate time of sampling and identify clinically relevant predictive factors. [ABSTRACT FROM AUTHOR]

Endovascular treatment of complex vascular pathologies in the pediatric population is often performed by nonpediatric subspecialists with adaptation of equipment and techniques developed for adult patients. We aimed to report our center's experience with safety and outcomes of endovascular treatments for pediatric vascular pathologies. We performed a retrospective review of our endovascular database. All patients ≤18 years who underwent endovascular treatment between January 1, 2004 and December 1, 2022 were included. During the study time frame, 118 cerebral angiograms were performed for interventional purposes in 55 patients. Of these patients, 8(14.5%) had intracranial aneurysms, 21(38.2%) had intracranial arteriovenous malformations, 6(10.9%) had tumors, 5(9.1%) had arterial occlusions (n = 3) or dissections (n = 2), 8(14.5%) had vein of Galen malformations, and 7(12.7%) had other cerebrovascular conditions. Of the total 118 procedures, access-site complications occurred in 2(1.7%), intraprocedural complications occurred in 3(2.5%), and transient neurological deficits were observed after 2(1.7%). Treatment-related mortality occurred in 1(1.8%) patient. Neurointervention in pediatric patients was safe and effective in our experience. [ABSTRACT FROM AUTHOR]

Acute rupture and hemorrhage of pediatric brain arteriovenous malformations (AVMs) may lead to cerebral herniation or intractable intracranial hypertension, necessitating emerging surgical interventions to alleviate intracranial pressure. However, there is still controversy regarding the timing of treatment for ruptured AVMs. This study aimed to assess the feasibility of utilizing three-pillar expansive craniotomy (3PEC) at different times during the treatment of pediatric ruptured supratentorial AVMs. A retrospective analysis was conducted on all consecutive cases of acute rupture in supratentorial AVM children who underwent 3PEC at a single institution from 2020 to 2022. General information, clinical characteristics, radiological data, and prognosis were reviewed and analyzed. Thirteen children were included in the analysis. The intracranial pressure of all patients decreased to below 15 mmHg within 10 days. The expansion volume of the cranial cavity of the patients increased by 18.3 cm3 (95% confidence interval, 10.2–26.3; P < 0.001) compared to the hematoma volume. None of the patients required decompressive craniectomy due to intractable intracranial hypertension caused by cerebral swelling. The median waiting period for patients with delayed AVMs treatment was 8 days, during which no rebleeding occurred. Emergency intervention with 3PEC in children experiencing acutely ruptured supratentorial AVMs appears to be feasible. For children requiring delayed management of the AVMs, 3PEC may diminish the risk of rebleeding during the waiting period and shorten the waiting period. [ABSTRACT FROM AUTHOR]

One of the pressing constraints in the treatment of arteriovenous malformations (AVM) is the potential development of new neurologic deficits, mainly when the AVM is in an eloquent area. The risk of ischemia when an en passage arterial supply is present is not negligible. In this regard, awake surgery holds promise in increasing the safety of low-grade AVM resection. We conducted a pilot trial on 3 patients with low-grade AVMs affecting speech areas to evaluate the safety of awake craniotomy using Conscious Sedation. Each feeder was temporarily clipped before the section. Also, we performed a systematic review to analyze the existing data about the impact of awake surgery in eloquent AVM resection. None of the 3 patients presented with neurologic deficits after the procedure. Awake craniotomy was useful in 1 case, as it allowed the detection of speech arrest during the temporal clipping of 1 of the feeders. This vessel was identified as an en passage vessel, closer to the nidus. The second attempt revealed the feeder of the AVM, which was sectioned. Systematic review yielded 7 studies meeting our inclusion criteria. Twenty-six of 33 patients included in these studies presented with AVM affecting speech area. Only 2 studies included the motor evoked potentials. Six studies used direct cortical and subcortical stimulation. In all studies the asleep-awake-asleep technique was used. Awake craniotomies are safe procedures and may be helpful in avoiding ischemic complications in low-grade AVMs, either affecting eloquent areas and/or when en passage feeders are present. [ABSTRACT FROM AUTHOR]

Nontraumatic intracranial hemorrhage occurs most commonly due to hypertension and is treated nonoperatively. Iatrogenic pseudoaneurysm from prior neurosurgical therapy represents a rarely described etiology for intracranial hemorrhage that may require emergent surgical therapy. An elderly female patient was brought to the emergency department with fatigue but no recent trauma. Subsequent computed tomography of the brain revealed a right-sided intraparenchymal hematoma. Her history included burr hole drainage of a subdural hematoma near the site, so additional imaging was performed and revealed an arteriovenous malformation, later discovered on operative findings to be a pseudoaneurysm, as the cause of the current bleeding episode. Why Should an Emergency Physician Be Aware of This? Awareness of prior neurosurgical treatment, even including minor procedures, in patients with apparent spontaneous intracranial bleeding should prompt angiographic evaluation for arteriovenous malformation. If found, these lesions are more likely to benefit from surgical treatment. [ABSTRACT FROM AUTHOR]

Background Flow-related aneurysms (FRAs) associated with cerebral arteriovenous malformations (AVMs) pose a significant therapeutic challenge. Both their natural history and management strategy are still unclear and underreported. FRAs generally increase the risk of brain hemorrhage. However, following AVM obliteration these vascular lesions are expected to disappear or remain stable. Methods We present two cases where growth of FRAs was detected following complete obliteration of an unruptured AVM. Results The first patient presented with proximal middle cerebral artery (MCA) aneurysm growth after spontaneous and asymptomatic thrombosis of the AVM. In our second case, a very small aneurysmal-like dilation located at the basilar apex enlarged to a saccular aneurysm following complete endovascular and radiosurgical obliteration of the AVM. Conclusion The natural history of flow-related aneurysms is unpredictable. In the cases where these lesions are not managed first, there should be close follow-up. When aneurysm growth is evident, active management strategy seems mandatory. [ABSTRACT FROM AUTHOR]

Brain arteriovenous malformation (AVM) is a rare congenital disorder affecting young adults with an incidence of 0.94 per 100,000 population. Intracranial digital subtraction angiography has to be done in all patients and grading of AVM is done as per Spetzler-Martin grading. We report a rare case of left basal ganglia large AVM treated by endovascular embolization. Our experience with endovascular embolization using Onyx is successful in the treatment of large brain AVM. Endovascular embolization with Onyx is safe and feasible in deeply located large AVMs of the brain. Our patient has postoperatively recovered completely without any neurological deficit. [ABSTRACT FROM AUTHOR]

Background and Purpose: The prognosis of untreated spinal arteriovenous malformations (SAVMs) is poor. Embolization plays an important role in the management of intramedullary SAVMs. Delayed aggravation due to spinal venous thrombosis following successful embolization has been reported; however, perioperative management strategies to prevent thrombosis have not been explored. We present our single-center experience of SAVM embolization and perioperative management, including anticoagulation. Material and Methods: We retrospectively evaluated 18 patients with SAVMs who underwent transarterial embolization. Perioperative anticoagulation therapy was administered to selected patients. We compared the characteristics of the patients, including perioperative management procedures, between those with and without postoperative worsening following embolization. Results: Acute postoperative worsening within 1 week occurred in 4 (22.2%) patients. Of these, immediate worsening was observed in one patient as a procedure-related complication. Delayed worsening after 24 h was observed in 3 patients, caused by delayed venous thrombosis with severe back pain. Rescue anticoagulation for delayed worsening improved symptoms in two patients. A comparison between patients with and without acute postoperative worsening revealed significant differences in age (median 46.5 vs. 26.5 years, p = 0.009) and the presence of postoperative back pain (75.0% vs. 0%, p = 0.005); however, there was no significant difference in use of selective anticoagulation (p = 0.274). Conclusion: The results of this study suggest that SAVM embolization can cause acute worsening due to postoperative venous thrombosis with severe back pain, which may be reversed by anticoagulation therapy. Back pain is an important finding that suggests venous thrombosis, and anticoagulation should be urgently administered. [ABSTRACT FROM AUTHOR]

Diagnostic accuracy of arteriovenous malformations (AVMs) is imperative for delineating management. The current standard is digital subtraction angiography (DSA). Arterial spin labeling (ASL) is an understudied noninvasive, non-contrast technique that allows angioarchitecture visualization and additionally quantifies cortical and AVM cerebral blood flow and hemodynamics. This meta-analysis aims to compare ASL and DSA imaging in detecting and characterizing cerebral AVMs. EMBASE, Medline, Scopus, and Cochrane databases were queried from inception to July 2022 for reports of AVMs evaluated by DSA and ASL imaging. Fourteen studies with 278 patients evaluated using DSA and ASL imaging prior to intervention were included; pCASL in 11 studies (n = 239, 85.37%) and PASL in three studies (n = 41, 14.64%). The overall AVM detection rate on ASL was 99% (CI 97–100%); subgroup analysis revealed no difference between pCASL vs. PASL (99%; CI 96–100% vs. 100%; CI 95–100% respectively, p = 0.42). The correlation value comparing ASL and DSA nidus size was 0.99. DSA and ASL intermodality agreement Cohen's k factor for Spetzler Martin Grading (SMG) was reported at a median of 0.98 (IQR 0.73–0.1), with a 1.0 agreement on SMG classification. A median of 25 arteries were detected by DSA (IQR 14.5–27), vs. 25 by ASL (IQR 14.5–27.5) at a median 0.92 k factor. ASL provides angioarchitectural visualization noninferior to DSA and additionally quantifies CBF. Our study suggests that ASL should be considered in the detection of AVMs, especially in patients with contrast contraindications or apprehension towards an invasive assessment. [ABSTRACT FROM AUTHOR]

Purpose: Spontaneous spinal hematoma (SSH), a rare neurological disorder, demands immediate diagnostic evaluation and intervention to prevent lasting deficits. This case series analyzes instances, particularly highlighting cases where vascular causes were identified despite inconclusive initial imaging. Methods: In a retrospective study of 20 patients treated for SSH at a Level I spine center from 01/01/2017 to 11/15/2023, we examined demographics, clinical presentation, imaging, and treatment details. Excluding traumatic cases, we present 4 instances of SSH associated with diverse vascular pathologies. Results: Patient ages ranged from 39 to 85 years, with a median age of 66 years. 45% were male, and 55% were female. Among 20 cases, 14 were epidural hematomas, 4 subdural, 1 combined epidural and subdural, and 1 subarachnoid hemorrhage. 85% presented with neurological deficits, while 3 solely had pain-related symptoms. 55% were under anticoagulant medication, and vascular anomalies were found in 25% of cases. The cause of SSH remained unclear in 20% of cases. MRI was performed for all patients, and DSA was conducted in 25% of cases. The 4 highlighted cases involved individuals with distinct vascular pathologies managed surgically. Conclusion: Urgent attention is crucial for SSH due to possible lasting neurological consequences. The study emphasizes comprehensive diagnostics and surgical exploration, especially in cases with unclear etiology, to identify and address vascular causes, preventing hematoma progression or recurrence. Despite their rarity, vascular malformations contributing to spinal hematomas warrant particular attention. [ABSTRACT FROM AUTHOR]

Background: Currently, the treatment outcomes for complex congenital arteriovenous malformations (AVMs) remain unsatisfactory. This article reports on the utilization of an abdominal aortic stent graft, in conjunction with embolization techniques, for managing acute heart failure triggered by complex congenital arteriovenous malformations in the lower limb. Case Presentation: We present a case involving a patient with congenital AVMs in the lower limb, who had suffered from prolonged swelling in the left lower limb and recently developed symptoms of heart failure. At the age of 67, the patient was definitively diagnosed with a complex congenital AVMs in the lower limb. This article delves into the practical experiences and limitations encountered in employing an abdominal aortic stent graft, coupled with embolization, to address acute heart failure caused by complex congenital AVMs in the lower limb. Conclusions: Our article presents the initial report on the challenges and limitations encountered in treating acute heart failure triggered by complex congenital AVMs in the lower limb, utilizing a combination of abdominal aortic stent graft placement and embolization techniques. [ABSTRACT FROM AUTHOR]

Background: Intraosseous vascular anomalies in the facial skeleton present significant diagnostic and therapeutic challenges due to complex anatomy. These anomalies represent about 0.5–1% of bony neoplastic and tumor-like lesions, usually presenting as a firm, painless mass. Most described intraosseous vascular malformations are venous malformations (VMs) and, more rarely, arteriovenous malformations. Objectives: The objectives of this work are to show our experience, protocol and the applications of computer planning, virtual surgery, CAD-CAM design, surgical navigation, and computer-assisted navigated piezoelectric surgery in the treatment of facial intraosseous vascular anomalies and to evaluate the advantages and disadvantages. Methods: Three females and one male with periorbital intraosseous vascular anomalies were treated using en-block resection and immediate reconstruction with a custom-made PEEK prosthesis. One lesion was in the supraorbital rim and orbital roof, one in the frontal bone and orbital roof, and two in the zygomatic region. We accomplished the resection and reconstruction of the lesion using virtual planning, CAD-CAM design, surgical navigation and piezoelectric device navigation. Results: There were no complications related to the surgery assisted with navigation. With an accuracy of less than 1 mm, the procedure may be carried out in accordance with the surgical plan. The surgeon's degree of uncertainty during deep osteotomies and in locations with low visibility was decreased by the use of the navigated piezoelectric device. Conclusions: Resection and reconstruction of facial intraosseous vascular anomalies benefit from this new surgical strategy using CAD-CAM technologies, computer-assisted navigated piezoelectric surgery, and surgical navigation. [ABSTRACT FROM AUTHOR]

Intraosseous occurrence of a spinal AVM is anecdotal, with only four such cases reported previously. This is the first report of a spinal intraosseous AVM in the cervical vertebrae. A 44-year-old male patient presented with a 2-month history of progressive quadriparesis and bladder dysfunction. Magnetic resonance imaging showed multiple flow voids within the C4 and C5 vertebral bodies, and an extradural component causing cord compression. CT showed extensive bony destruction at both levels. The diagnosis of an intraosseous AVM was confirmed with spinal angiography. The AVM was noted to be fed by branches from the ascending cervical arteries and the vertebral artery. The nidus was draining into the vertebral venous plexus and thence into the jugular vein through the marginal sinus. The patient underwent partial embolization of the AVM. Surgical resection was attempted but found to be unfeasible due to torrential bleeding. A 360-degree stabilization along with decompressive laminectomies was performed, resulting in clinical improvement and disease stabilization at one year follow-up. The case and its management dilemmas are discussed in light of a brief literature review. [ABSTRACT FROM AUTHOR]

Introduction and importance: Spontaneous hemoperitoneum (SH) is a rare, life-threatening condition characterized by nontraumatic and non-iatrogenic intraperitoneal bleeding. This article explores three unique cases of SH, shedding light on unusual causes and emphasizing the critical role of diagnostic imaging and exploratory laparotomy in management. Methods: The study was a retrospective single-center non-consecutive case series. Results: We report three distinct cases of SH, each originating from uncommon sources: rupture of greater omentum arterio-venous malformation, a branch of the left gastric artery, and pathological splenic rupture. Clinical evaluation, diagnostic imaging, and surgical interventions are detailed for each case. Conclusion: These rare cases underscore the diverse etiologies of SH, including idiopathic omental bleeding, gastric intramural hematoma, and atraumatic splenic rupture. Enhanced CT imaging plays a crucial role in diagnosis, enabling the characterization of underlying pathologies. Exploratory laparotomy proves to be an essential treatment option for unstable patients with suspected or confirmed diagnoses of SH. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) causes arteriovenous malformations (AVMs) in several organs. This report is the first to document and image a thyroid AVM complication in HHT. A 72-year-old woman with HHT was referred for thyroid nodule evaluation. Ultrasonography showed a hypervascularized nodule in the right thyroid lobe which was initially suspected to be malignant. However, 3-dimensional computed tomography angiography demonstrated a thyroid AVM with abnormal anastomosis of the superior thyroid artery and the inferior thyroid vein. In the formation of thyroid AVM, here, chronic thyroiditis and hypothyroidism complications may have been a second hit, due to the predisposing first-hit germline mutation. This report sheds light on overlooked thyroid lesions in HHT and advocates a noninvasive imaging approach in diagnosing thyroid AVMs. Furthermore, this case suggests a potential mechanism of AVM formation in human HHT, possibly supporting the second-hit hypothesis. [ABSTRACT FROM AUTHOR]

Arteriovenous malformations (AVMs) can be treated with observation, surgery, embolization, stereotactic radiosurgery (SRS), or a combination of therapies. SRS has been used for AVMs that pose a high risk of surgery, such as in deep or eloquent anatomic locations. Smaller AVMs, <3 cm, have been shown to have higher rates of complete obliteration after SRS. For AVMs that are a larger size, embolization prior to SRS has been used to reduce the size of the AVM nidus. In this study we analyzed embolization prior to SRS to reduce nidal volume and describe imaging techniques to target for SRS post embolization. We retrospectively reviewed all patients at a single academic institution treated with embolization prior to SRS for treatment of AVMs. We then used contrast enhanced magnetic resonance imaging (MRI) to contour AVM volumes based on pre-embolization imaging and compared to post-embolization imaging. Planned AVM volume prior to embolization was then compared to actual treated AVM volume. We identified 11 patients treated with embolization prior to SRS from 2011–2023. Median AVM nidal volume prior to embolization was 7.69 mL and post embolization was 3.61 ML (P < 0.01). There was a 45.5% obliteration rate at follow up in our series, with 2 minor complications related to radiosurgery. In our cohort, embolization prior to SRS resulted in a statistically significant reduction in AVM nidal volume. Therefore, embolization prior to SRS can result in dose reduction at time of SRS treatment allowing for decreased risk of SRS complications without higher embolization complication rates. [ABSTRACT FROM AUTHOR]

Pediatric intracranial arteriovenous malformation (AVM) patients are commonly admitted to the emergency room (ER). Increasing patient utilization of the ER has been associated with healthcare disparities and a trend of decreased efficiency. The aim of this study was to evaluate the trends of pediatric AVM ER admissions over recent years and identify factors associated with health care resource utilization and outcomes. The 2016–2019 National Inpatient Sample was queried for patients under the age of 18 admitted with AVM. Cases of admission through the ER were identified. Demographic and severity factors associated with ER admission were explored using comparative and regression statistics. Of 3875 pediatric patients with AVM admitted between 2016 and 2019, 1280 (33.0%) were admitted via the ER. Patients admitted via the ER were more likely to be in the lowest median income category (P < 0.001), on Medicaid insurance (P = 0.008), or in the South (P < 0.001) than patients admitted otherwise. There was increased severity and increased rates of intracranial hemorrhage (ICH) in patients admitted via the ER (P < 0.001). Finally, there were increasing trends in ER admissions and ICH throughout the years. ER admission of pediatric AVM patients with ICH is increasing and is associated with a distinct socioeconomic profile and increased healthcare resource utilization. These findings may reflect decreased access to more advanced diagnostic modalities, primary care, and other important resources. Identifying populations with barriers to care is likely an important component of policy aimed at decreasing the risk of severe disease presentation. [ABSTRACT FROM AUTHOR]

Background: External ventricular drain (EVD) is used for monitoring intracranial pressure or diverting cerebrospinal fluid. However, confirmation of an infection is not immediate and requires obtaining culture results, often leading to the excessive use of antibiotics. This study aimed to compare noninfectious ventriculitis and EVD infection in terms of the risk factors, predictors, prognosis, and effectiveness of care bundle interventions. Methods: This retrospective study was conducted at a medical center with 1,006 beds in northern Taiwan between January 2018 and July 2022. Standard EVD insertion protocols and care bundles have been implemented since 2018, along with the initiation of chlorhexidine. Results: In total, 742 EVD cases were identified. Noninfectious ventriculitis typically presents with fever approximately 8 days following EVD placement, whereas EVD infection typically manifests as fever after 20 days. Aneurysmal subarachnoid hemorrhage was strongly associated with the development of noninfectious ventriculitis (adjusted odds ratio [OR] 2.6, 95% confidence interval [CI] 1.5–4.4). Alcoholism (adjusted OR 3.5, 95% CI 1.1–12.3) and arteriovenous malformation (adjusted OR 13.1, 95% CI 2.9–58.2) significantly increased the risk of EVD infection. The EVD infection rate significantly decreased from 3.6% (14 of 446) to 1.0% (3 of 219) (p = 0.03) after the implementation of chlorhexidine gluconate bathing. Conclusions: Aneurysmal subarachnoid hemorrhage or fever with neuroinflammation within 2 weeks of EVD placement is indicative of a higher likelihood of noninfectious ventriculitis. Conversely, patients with arteriovenous malformation, alcoholism, or fever with neuroinflammation occurring after more than 3 weeks of EVD placement are more likely to necessitate antibiotic treatment for EVD infection. Chlorhexidine gluconate bathing decreases EVD infection. [ABSTRACT FROM AUTHOR]

This study investigated the influence of periprocedural hemorrhage and clinical outcomes with an endovascular therapeutic strategy for cerebellar arteriovenous malformations (cAVMs). From December 2006 to January 2018, 125 cAVMs were classified as types I–IV and received endovascular embolization via Onyx or Glubran 2. The risk factors of hemorrhage were analyzed using univariate and multivariate logistic analyses. A modified Rankin Scale (mRS) score was used to evaluate the neurological function before and 1 year after the operation. Results Of 125 patients, 63 had type I cAVMs, 2 type II cAVMs, 48 type III cAVMs, and 12 type IV cAVMs. A total of 88 (70.4%) patients had clinical manifestations of intracranial hemorrhage. Multivariate logistic regression analysis showed that age (OR, 2.276; 95% CI, 1.132 − 5.663), flow-related aneurysm (OR, 2.845; 95% CI, 1.265 − 6.248), lesion size (OR, 3.005; 95% CI, 1.119 − 5.936), and the number of feeding arteries (OR, 0.105; 95% CI, 0.081 − 0.312) were still the significant independent risk factors of intracranial hemorrhage. During a 1-year follow-up, 109 patients (87.2%) had good outcomes (mRS 0 − 2), 12 patients (9.6%) had poor outcomes (mRS 3 − 4), 4 patients (3.2%) died, and 3 patients had intracranial hemorrhage due to the incomplete embolization of cAVMs. Endovascular embolization is a feasible treatment for cAVMs. Age, flow-related aneurysm, lesion size, and the number of feeding arteries are the significant risk factors of periprocedural hemorrhage. Moreover, the lesion characteristics must be given full consideration when using Onyx or Glubran 2 before cAVMs embolization. [ABSTRACT FROM AUTHOR]

Introduction: Brain arteriovenous malformations (bAVMs) present complex challenges in neurosurgery, requiring precise pre-surgical planning. In this context, 3D printing technology has emerged as a promising tool to aid in understanding bAVM morphology and enhance surgical outcomes, particularly in pediatric patients. This study aims to assess the feasibility and effectiveness of using 3D AVM models in pediatric bAVM surgery. Methodology: The study was conducted at Great Ormond Street Hospital, and cases were selected sequentially between October 2021 and February 2023. Eight pediatric bAVM cases with 3D models were compared to eight cases treated before the introduction of 3D printing models. The 3D modelling fidelity and clinical outcomes were assessed and compared between the two cohorts. Results: The study demonstrated excellent fidelity between 3D models and actual operative anatomy, with a median difference of only 0.31 mm. There was no statistically significant difference in angiographic cure rates or complications between the 3D model group and the non-3D model group. Surgical time showed a non-significant increase in cases involving 3D models. Furthermore, the 3D model cohort included higher-grade bAVMs, indicating increased surgical confidence. Conclusion: This study demonstrates the feasibility and efficacy of utilizing 3D AVM models in pediatric bAVM surgery. The high fidelity between the models and actual operative anatomy suggests that 3D modelling can enhance pre-surgical planning and intraoperative guidance without significantly increasing surgical times or complications. Further research with larger cohorts is warranted to confirm and refine the application of 3D modelling in clinical practice. [ABSTRACT FROM AUTHOR]

Background: CT angiography (CTA), a minimally invasive diagnostic technique, has become popular for looking into acute gastrointestinal hemorrhages. This study aimed to evaluate the validity of Computed tomography angiography (CTA) for diagnosis of the cause of acute GIB and its influence on treatment. Methods: This study was carried out in Radiodiagnosis Department, Faculty of Medicine, Zagazig University on 24 cases with acute GIB during the period from June 2023 to December 2023 on 24 patients who were acute GIB. Age > 18 years. Both male and female were included as a comprehensive sample. Results: The most frequent diagnoses detected by CT-angiography among patients were esophageal gastric varices which was detected among (75%) of the patients, while the least frequent diagnoses detected was pancreatic arteriovenous malformations in 25% of patients. There was no significant difference between bleeding type and age or gender (P>0.05). sensitivity of CT angiography in diagnoses bleeding compared to endoscopy is (92.3%), positive predictive value (PPV) is (63.2%), negative predictive value (NPV) is (80.0%), accuracy of Ct angiography in diagnoses bleeding compared to endoscopy is (66.7%). Conclusions: CT angiography is a valid, noninvasive method that is conveniently accessible. CT angiography has a high sensitivity in identifying the source of current bleeding. The results of CT angiography are useful in guiding the best course of treatment (directed endoscopic intervention, angiographic embolization, or surgery), as well as in determining when these treatments should be carried out [ABSTRACT FROM AUTHOR]

Background: Hemodynamic changes are seen in the feeding arteries of arteriovenous malformations (AVMs). Phase-contrast MRI (PC-MRI) enables the acquisition of hemodynamic information from blood vessels. There is insufficient knowledge on which flow or velocity parameter best discriminates AVMs from healthy subjects. Purpose: To evaluate PC-MRI-measured flow and velocity in feeding arteries of AVMs before and, when possible, also after treatment and to compare these measurements to corresponding measurements in healthy controls. Materials and Methods: Highest flow (HF), lowest flow (LF), mean flow (MF), peak systolic velocity (PSV), end-diastolic velocity (EDV), and mean velocity (MV) were measured in feeding arteries in patients with intracranial AVMs using 2D PC-MRI at 3 T. Measurements were compared to previously reported values in healthy individuals. Values in patients above the 95th percentile in the healthy cohort were categorized as pathological. Nidus volume was measured using 3D time-of-flight MR angiography. Results: Ten patients with diagnosed AVMs were examined with PC-MRI. Among these, three patients also underwent follow-up PC-MRI after treatment. Pathological velocities (PSV, EDV, and MV) were seen in all five subjects with a nidus larger or equal to 5.7 cm3, whereas pathological flow values were not seen in all, that is, pathologic HF in three, pathologic LF in two, and pathologic MF in two. After treatment, there was a decrease in flow and velocity (all measured parameters). After treatment, velocities (PSV, EDV, and MV) were no longer abnormal compared to healthy controls. Conclusion: Patients with a large AVM nidus show pathological velocities, but less consistent flow increases. Following treatment, velocities normalize. [ABSTRACT FROM AUTHOR]

Purpose of Review: The purpose of this review is to provide an updated approach to the evaluation and management of pulsatile tinnitus (PT), an uncommon but often treatable subtype of tinnitus. Recent Findings: Secondary PT can be due to either vascular or non-vascular etiologies, including, but not limited to: neoplasm, arteriovenous malformation or fistula, idiopathic intracranial hypertension, dural venous sinus stenosis, otoacoustic etiologies (e.g., otosclerosis, patulous eustachian tube) and bony defects (e.g., superior semicircular canal dehiscence). Computed tomography (CT) and magnetic resonance imaging (MRI) imaging have comparable diagnostic yield, though each may be more sensitive to specific etiologies. If initial vascular imaging is negative and a vascular etiology is strongly suspected, digital subtraction angiography (DSA) may further aid in the diagnosis. Many vascular etiologies of PT can be managed endovascularly, often leading to PT improvement or resolution. Notably, venous sinus stenting is an emerging therapy for PT secondary to idiopathic intracranial hypertension with venous sinus stenosis. Summary: Careful history and physical exam can help establish the differential diagnosis for PT and guide subsequent evaluation and management. Additional studies on the efficacy and long-term outcome of venous sinus stenting for venous stenosis are warranted. [ABSTRACT FROM AUTHOR]

Background: Mandibular arteriovenous malformation (AVM) is rare. Our work aims to introduce the ethanol embolization of a patient suffering from acute oral hemorrhage induced by mandibular AVM. Methods: A 35‐year‐old woman without coagulopathy underwent tooth extraction, and the acute oral bleeding occurred intraoperatively. Imaging examinations indicated the enhancement of vascular mass with bone destruction inside the mandible. Angiography finally confirmed the high blood flow nature and the diagnosis of AVM. Results: During the interventional procedure, the coils were first applied into the dilated outflowing vein to slow down the blood flow rate of mandibular AVM. Absolute ethanol was injected in a multi‐bolus modality to destroy the nidus of AVM. Her mandibular lesion had been stable in the 12‐month re‐examined angiography, no further bleeding occurred during the period. Conclusions: Ethanol embolotherapy was a less invasive, more precise, and quick‐action approach managing AVM of the jaw and related emergency medicine. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the angiographic recanalization rate of patients who underwent embolization juxta-proximal to the sac with AMPLATZER Vascular Plug type IV (AVP IV) for a simple pulmonary arteriovenous malformation (PAVM). Material and Methods: Ten patients (7 females and 3 males; median age, 47 years [range 28–83 years]) with 19 simple-type PAVMs who underwent embolization using an AVP IV between May 2015 and November 2021 were included in this retrospective study. The median feeding artery diameter on computed tomography was 4.0 mm (range 3–5.9 mm), and the median ratio of AVP IV size to feeding artery diameter on computed tomography was 1.5 (range 1.3–2.1). Technical success was defined by AVP IV placement at the junction between the pulmonary artery and the sac, or the pulmonary artery within 1 cm from the junction and beyond the last normal branch. The primary endpoint was the PAVM recanalization rate in selective or segmental pulmonary angiography performed 1 year post-embolization. Results: The technical success rate of embolization juxta-proximal to the sac for simple-type PAVMs was 100%. None of the 19 lesions showed recanalization in pulmonary angiography performed 1 year after embolization. One patient experienced hemoptysis and pneumonia. Conclusion: Embolization of simple-type PAVMs' feeding vessel using AVP IV is safe and effective, with a high technical success rate and no recanalization on pulmonary angiography performed at 1 year post-embolization. Level of Evidence: 4 [ABSTRACT FROM AUTHOR]

Purpose: To investigate the safety and efficacy of microballoon-occluded transcatheter embolization using n-butyl-2-cyanoacrylate (NBCA) in patients with a single pulmonary arteriovenous malformation (PAVM). Methods: From November 2017 to November 2020, this retrospective study included 38 previously untreated patients with a single PAVM who underwent microballoon-occluded transcatheter embolization using NBCA. All 38 patients had follow-up that included simple chest radiography and contrast-enhanced chest computed tomography (CT). Results: A microballoon was successfully placed in a feeding artery of the PAVM to control the delivery of the NBCA cast in all 38 patients, with complete embolization of sacs and the feeding artery achieved in all cases. The mean diameters of the feeding artery, sac, and draining vein were 3.9 ± 0.9 mm, 7.5 ± 2.6 mm, and 4.6 ± 1.3 mm, respectively. A fixed 1:2 NBCA/Lipiodol ratio was used, and the mean amount of embolic mixture per patient was 1.4 mL (range 0.6–2.2 mL). There were no complications related to microballoon adhesion and non-target embolization of the systemic circulation. Follow-up CT in all 38 patients with a mean delay of 34.5 ± 8.8 months (range 20.7–56.5 months) showed no continued perfusion of the PAVM. Conclusion: In our hands, microballoon-occluded n-butyl-2-cyanoacrylate embolization seemed to be safe and appeared to be clinically effective in patients with simple and complex types of single PAVM. Therefore, the present technique has the potential to become a standard treatment for a single PAVM. [ABSTRACT FROM AUTHOR]

Brain arteriovenous malformations (bAVMs) substantially increase the risk for intracerebral hemorrhage (ICH), which is associated with significant morbidity and mortality. However, the treatment options for bAVMs are severely limited, primarily relying on invasive methods that carry their own risks for intraoperative hemorrhage or even death. Currently, there are no pharmaceutical agents shown to treat this condition, primarily due to a poor understanding of bAVM pathophysiology. For the last decade, bAVM research has made significant advances, including the identification of novel genetic mutations and relevant signaling in bAVM development. However, bAVM pathophysiology is still largely unclear. Further investigation is required to understand the detailed cellular and molecular mechanisms involved, which will enable the development of safer and more effective treatment options. Endothelial cells (ECs), the cells that line the vascular lumen, are integral to the pathogenesis of bAVMs. Understanding the fundamental role of ECs in pathological conditions is crucial to unraveling bAVM pathophysiology. This review focuses on the current knowledge of bAVM-relevant signaling pathways and dysfunctions in ECs, particularly the endothelial-to-mesenchymal transition (EndMT). [ABSTRACT FROM AUTHOR]

Endoglin (ENG) mutation causes type 1 hereditary hemorrhagic telangiectasia (HHT1). HHT1 patients have arteriovenous malformations (AVMs) in multiple organs, including the brain. In mice, Eng deletion induced by R26RCreER or SM22αCre leads to AVM development in the brain and other organs. We hypothesized that an increase in Eng- negative ECs will enhance AVM severity. To increase EC Eng deletion, we used a codon-improved cre (icre), which is more potent in recombination of the floxed alleles than the wild-type (WT) cre. R26RCreER;Engf/f mice that have a Rosa promoter driving and tamoxifen (TM)-inducible WT cre expression globally, and PdgfbiCreER;Engf/f mice that have a Pdgfb promoter driving and TM-inducible icre expression in ECs were treated with three intra-peritoneal injections of TM (2.5 mg/25 g of body weight) to delete Eng globally or in the ECs. AAV-VEGF was stereotactically injected into the brain to induce brain focal angiogenesis and brain AVM. We found that icre caused more Eng deletion in the brain, indicated by a lower level of Eng proteins (p < 0.001) and fewer Eng-positive ECs (p = 0.01) than mice with WT cre. Mice with icre-mediated Eng deletion have more abnormal vessels (p = 0.02), CD68+ macrophages (p = 0.002), and hemorrhage (p = 0.04) and less vascular pericyte and smooth muscle coverage than mice with WT cre. In addition, arteriovenous shunts were detected in the intestines of icre mice, a phenotype that has not been detected in WT cre mice before. RNA-seq analysis showed that 8 out of the 10 top upregulated pathways identified by gene ontology (GO) analysis are related to inflammation. Therefore, the increase in Eng deletion in ECs exacerbates AVM severity, which is associated with enhanced inflammation. Strategies that can reduce Eng-negative ECs could be used to develop new therapies to reduce AVM severity for HHT1 patients. [ABSTRACT FROM AUTHOR]

SOLAMEN syndrome is a rare, recently recognized congenital syndrome that is characterized by progressive and hypertrophic diseases involving multiple systems, including segmental overgrowth, lipomatosis, arteriovenous malformation (AVM) and epidermal nevus. According to literatures, SOLAMEN syndrome is caused by heterozygous PTEN mutation. Phenotypic overlap complicates the clinical identification of diseases associated with PTEN heterozygous mutations, making the diagnosis of SOLAMEN more challenging. In addition, SOLAMEN often presents with segmental tissue overgrowth and vascular malformations, increasing the possibility of misdiagnosis as klipple-trenaunay syndrome or Parks-Weber syndrome. Here, we present a case of a child presenting with macrocephaly, patchy lymphatic malformation on the right chest, marked subcutaneous varicosities and capillaries involving the whole body, overgrowth of the left lower limb, a liner epidermal nevus on the middle of the right lower limb, and a large AVM on the right cranial thoracic entrance. Based on the typical phenotypes, the child was diagnosed as SOLAMEN syndrome. detailed clinical, imaging and genetic diagnoses of SOLAMEN syndrome was rendered. Next-generation sequencing (NGS) data revealed that except for a germline PTEN mutation, a PDGFRB variant was also identified. A subsequent echocardiographic examination detected potential cardiac defects. We suggested that given the progressive nature of AVM and the potential severity of cardiac damage, regular echocardiographic evaluation, imaging follow-up and appropriate interventional therapy for AVM are recommended. [ABSTRACT FROM AUTHOR]

Telangiectases and arteriovenous malformations (AVMs) are the characteristic lesions of Hereditary Hemorrhagic Telangiectasia (HHT). Somatic second-hit loss-of-function variations in the HHT causative genes, ENG and ACVRL1, have been described in dermal telangiectasias. It is unclear if somatic second-hit mutations also cause the formation of AVMs and nasal telangiectasias in HHT. To investigate the genetic mechanism of AVM formation in HHT, we evaluated multiple affected tissues from fourteen individuals. DNA was extracted from fresh/frozen tissue of 15 nasal telangiectasia, 4 dermal telangiectasia, and 9 normal control tissue biopsies, from nine unrelated individuals with HHT. DNA from six formalin-fixed paraffin-embedded (FFPE) AVM tissues (brain, lung, liver, and gallbladder) from five individuals was evaluated. A 736 vascular malformation and cancer gene next-generation sequencing (NGS) panel was used to evaluate these tissues down to 1% somatic mosaicism. Somatic second-hit mutations were identified in three in four AVM biopsies (75%) or half of the FFPE (50%) samples, including the loss of heterozygosity in ENG in one brain AVM sample, in which the germline mutation occurred in a different allele than a nearby somatic mutation (both are loss-of-function mutations). Eight of nine (88.9%) patients in whom telangiectasia tissues were evaluated had a somatic mutation ranging from 0.68 to 1.96% in the same gene with the germline mutation. Six of fifteen (40%) nasal and two of four (50%) dermal telangiectasia had a detectable somatic second hit. Additional low-level somatic mutations in other genes were identified in several telangiectasias. This is the first report that nasal telangiectasias and solid organ AVMs in HHT are caused by very-low-level somatic biallelic second-hit mutations. [ABSTRACT FROM AUTHOR]

Background: Pulmonary arteriovenous malformations are a relatively uncommon medical condition, affecting roughly 1 in every 2500 individuals. Of those suffering from pulmonary arteriovenous malformations, 80% have an underlying genetic condition: hereditary hemorrhagic telangiectasia. Case presentation: We present the case of a 20-year-old Pakistani male with a history of persistent slower-onset frontal headaches that increased in severity within the course of the day. His hemoglobin was 18 g/dl, indicating polycythemia, for which he had undergone seven venesections in a month previously. His physical examination was unremarkable. His computed tomography scan depicted multiple dilated tortuous vessels with branching linear opacities in the right lower lobe of the lungs. The multiple feeding arteries were supplied by the right main pulmonary artery, and the large draining veins led to the right inferior pulmonary vein. This was identified as a diffuse pulmonary arteriovenous malformation. He was recommended for a right pulmonary artery angiogram. It showed multiple tortuous vessels with a nidus and large draining veins—features of a diffuse arteriovenous malformation in the right lower lobe of the lung consistent with the computed tomography scan. Embolization of two of these vessels feeding the arteriovenous malformation was conducted, using Amplatzer Vascular plug 2, whereas multiple pushable coils (five coils) were used for embolizing the third feeding vessel. This achieved 70–80% successful embolization of right pulmonary AVM; however, some residual flow was still seen in the arteriovenous malformation given the complexity of the lesion. Immediately after, his oxygen saturation improved from 78% to 96%. Conclusion: Diffuse pulmonary arteriovenous malformations, as seen in this patient, are rare, accounting for less than 5% of total pulmonary arteriovenous malformations diagnosed. The patient presented with a complaint of progressive frontal headaches, which can be attributed to low oxygen saturation or the presence of a cerebral arteriovenous malformation. There was no history of hereditary hemorrhagic telangiectasia in the patient's family. Furthermore, although most patients with hereditary hemorrhagic telangiectasia and hence pulmonary arteriovenous malformation have complaints of iron-deficiency anemia, our patient in contrast was suffering from polycythemia. This can be explained as a compensatory mechanism in hypoxemic conditions. Moreover, the patient had no complaint of hemoptysis or epistaxis, giving a varied presentation in comparison with a typical pulmonary arteriovenous malformation. [ABSTRACT FROM AUTHOR]

Infected aneurysms, though rare, present significant challenges in contemporary medical practice. Coined by Sir William Osler in 1885, the term "mycotic aneurysm" encompasses infections of native arteries and preexisting aneurysms, posing life-threatening risks of arterial rupture and recurrence. We discuss a case involving a 51-yearold woman with a giant infected aneurysm, highlighting the complexities in diagnosis. The patient presented with bleeding from her left ear, a fluctuating neck mass, and decreased hearing. Contrast-enhanced computed tomography (CT) played a crucial role in revealing the infected giant aneurysm in the left internal carotid artery, associated with arteriovenous malformation and fistula. Additionally, incidental findings included aneurysms in the right internal carotid and bilateral vertebral arteries. The case underscores the importance of a high index of suspicion and advanced diagnostic tools for timely and accurate diagnosis. The rarity of experienced centers in managing such cases, coupled with a lack of consensus in diagnostic criteria, poses challenges for treatment improvements. [ABSTRACT FROM AUTHOR]

Background Brain arteriovenous malformations (bAVMs) are abnormal vascular connections with direct arteriovenous shunts, generally symptomatic in the adult life. However, a small number of bAVMs may manifest in pediatric patients, with higher bleeding risk and mortality rates when compared to adults. The purpose of this study is to review our experience with endovascular treatment of bAVMs in pediatric patients. Methods This is a retrospective analysis of all bAVMs in pediatric patients (0–18 years) who underwent diagnostic digital subtraction angiography (DSA) at our institution from January 2010 to June 2021. Results Twenty-six patients met the inclusion criteria, of which 12 underwent endovascular treatment. Treated patients had a mean age of 10.25 years and 58% were females. Complete angiographic exclusion was achieved in five (42%) patients with endovascular treatment. Five patients with residual bAVM after embolization needed adjuvant therapy with surgery (n = 3) or stereotactic radiosurgery (SRS; n = 2). Two patients are still undergoing embolization sessions. Procedure-related complications occurred in two patients (17%) and included small vessel perforation and an occipital ischemic stroke. Two patients showed bAVM recurrence on follow-up (17%) and subsequently underwent SRS (n = 1) or surgery (n = 1), both resulting in complete bAVM exclusion. All patients had a modified Rankin scale (mRS) score of 0 to 2 on follow-up. Conclusion Our experience supports the effectiveness and safety of endovascular treatment of bAVM in selected pediatric patients. A multidisciplinary approach combining surgery and SRS is warranted to achieve higher complete bAVM obliteration rates. Long-term follow-up is important as these lesions may show recurrence over time, especially in the pediatric population. [ABSTRACT FROM AUTHOR]

Objective: Gamma Knife radiosurgery (GKRS) is an effective and noninvasive treatment for high-risk arteriovenous malformations (AVMs). Since differences in GKRS outcomes by nidus type are unknown, this study evaluated GKRS feasibility and safety in patients with brain AVMs. Methods: This single-center retrospective study included patients with AVM who underwent GKRS between 2008 and 2021. Patients were divided into compact- and diffuse-type groups according to nidus characteristics. We excluded patients who performed GKRS and did not follow-up evaluation with magnetic resonance imaging or digital subtraction angiography within 36 months from the study. We used univariate and multivariate analyses to characterize associations of nidus type with obliteration rate and GKRS-related complications. Results: We enrolled 154 patients (mean age, 32.14±17.17 years; mean post-GKRS follow-up, 52.10±33.67 months) of whom 131 (85.1%) had compact- and 23 (14.9%) diffuse-type nidus AVMs. Of all AVMs, 89 (57.8%) were unruptured, and 65 (42.2%) had ruptured. The mean Spetzler-Martin AVM grades were 2.03±0.95 and 3.39±1.23 for the compact- and diffuse-type groups, respectively (p<0.001). During the follow-up period, AVM-related hemorrhages occurred in four individuals (2.6%), three of whom had compact nidi. Substantial radiation-induced changes and cyst formation were observed in 21 (13.6%) and one patient (0.6%), respectively. The AVM complete obliteration rate was 46.1% across both groups. Post-GKRS complication and complete obliteration rates were not significantly different between nidus types. For diffuse-type nidus AVMs, larger AVM size and volume (p<0.001), lower radiation dose (p<0.001), eloquent area location (p=0.015), and higher Spetzler-Martin grade (p<0.001) were observed. Conclusion: GKRS is a safe and feasible treatment for brain AVMs characterized by both diffuse- and compact-type nidi. [ABSTRACT FROM AUTHOR]

The popularity of trans-radial access (TRA) for cerebral angiography is growing. Potential benefits of TRA over traditional trans-femoral access (TFA) are multitude. This study aimed to evaluate discharge outcomes and patient opinion of TRA compared to TFA in patients undergoing cerebral angiography prior to stereotactic radiosurgery (SRS) treatment for cerebral arteriovenous malformations. Consecutive patients treated at the National Centre for Stereotactic Radiosurgery (Sheffield, United Kingdom) over a 22-month period were included. All patients underwent cerebral angiography with either TRA or TFA as part of treatment planning prior to SRS. TRA patients who had previously undergone TFA in other centres were surveyed for their experience of cerebral angiography using a questionnaire. SRS staff at our centre was approached for their opinion. 492 patients were included (median age = 43 years, 57.5% male, median lesions treated = 1). More patients underwent angiography with TFA (75.2%) than TRA (24.8%). No difference was found in accumulated dose for angiography between the groups (p>0.05). There was 17.6% reduction in overnight stay between TRA and TRF, the proportion of patients requiring overnight admission was higher for the TFA (35.2%) than TRA (17.6%, p<0.05). 101 patients were surveyed, with a response rate of 47%. Most respondents (79%) indicated preference for TRA over TFA. Use of TRA in pre-SRS cerebral angiography is feasible and improves both patient and staff experience. The adoption of TRA could have important implications for department resources and costs by reducing the proportion of overnight admissions. • Trans-radial cerebral angiography for radiosurgery of arteriovenous malformations. • Patient and staff preference for trans-radial angiography. • Improved service efficiency and reduced stay with trans-radial angiography. [ABSTRACT FROM AUTHOR]

Introduction: Gastrointestinal (GI) bleeding events (BEs) in von Willebrand disease (VWD) are difficult to diagnose and often recurrent. Limited data from clinical trials has led to lack of consensus on treatment options. Aim: Describe current treatments and outcomes for GI BEs in people with VWD. Methods: This retrospective, observational, multicentre chart review study was conducted from January 2018 through December 2019 and included patients with inherited VWD with ≥1 GI BE in the preceding 5 years. Baseline characteristics, number and aetiology of BEs, associated GI‐specific morbidities/lesions, treatment and outcomes were analysed descriptively. Results: Sixty bleeds were reported in 20 patients with type 1 (20%), type 2 (50%) and type 3 (30%) VWD. During the 5‐year study period, 31 (52%) BEs had one identified or suspected cause; multiple causes were reported in 11 (18%). Most GI BEs (72%) were treated with a combination of von Willebrand factor (VWF), antifibrinolytics and/or other haemostatic or non‐haemostatic treatments. Time to resolution did not differ by VWF treatment use; however, BEs treated with non‐VWF treatments tended to resolve later. In patients with GI‐specific morbidities/lesions, 84% resolved with first‐line treatment; time to resolution tended to be longer than in patients without such morbidities/lesions. Thirteen BEs occurred in patients receiving prophylaxis and 47 in patients receiving on‐demand treatment; 18 BEs resulted in a switch to prophylaxis after bleed resolution. Conclusions: This study confirms the unmet need for the management of recurrent GI BEs in people with VWD and the need for prospective data, especially on prophylaxis. [ABSTRACT FROM AUTHOR]

Key Clinical Message: A 17‐year‐old girl with a congenital, painless right forehead swelling obstructing her visual field was diagnosed with sinus pericranii. Radiological imaging confirmed extracalvarial arteriovenous malformation with serpentine vessels and bony erosion. Endovascular surgery was chosen for management, highlighting the necessity of considering SP in differential diagnosis for scalp swellings. Despite its rarity, awareness of it is essential to prevent complications from injury, misdiagnosis, or invasive procedures. [ABSTRACT FROM AUTHOR]

Vascular malformations (VMs) are clinically diverse with regard to the vessel type, anatomical location, tissue involvement and size. Consequently, symptoms and disease impact differ significantly. Diverse causative mutations in more and more genes are discovered and play a major role in the development of VMs. However, the relationship between the underlying causative mutations and the highly variable phenotype of VMs is not yet fully understood. In this systematic review, we aimed to provide an overview of known causative mutations in genes in VMs and discuss associations between the causative mutations and clinical phenotypes. PubMed and EMBASE libraries were systematically searched on November 9th, 2022 for randomized controlled trials and observational studies reporting causative mutations in at least five patients with peripheral venous, lymphatic, arteriovenous and combined malformations. Study quality was assessed with the Newcastle‐Ottawa Scale. Data were extracted on patient and VM characteristics, molecular sequencing method and results of molecular analysis. In total, 5667 articles were found of which 69 studies were included, reporting molecular analysis in a total of 4261 patients and 1686 (40%) patients with peripheral VMs a causative mutation was detected. In conclusion, this systematic review provides a comprehensive overview of causative germline and somatic mutations in various genes and associated phenotypes in peripheral VMs. With these findings, we attempt to better understand how the underlying causative mutations in various genes contribute to the highly variable clinical characteristics of VMs. Our study shows that some causative mutations lead to a uniform phenotype, while other causal variants lead to more varying phenotypes. By contrast, distinct causative mutations may lead to similar phenotypes and result in almost indistinguishable VMs. VMs are currently classified based on clinical and histopathology features, however, the findings of this systematic review suggest a larger role for genotype in current diagnostics and classification. [ABSTRACT FROM AUTHOR]

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Background Patients with vein of Galen malformations (VOGMs) can develop fine angiogenic networks with fistulous connections to the precursor of the vein of Galen. In these cases, transarterial embolization (TAE) with liquid embolic agents (LEAs) is challenging due to reflux in the pedicle leading to the network, causing poor penetration. Transvenous approaches carry a risk of hemorrhage from pathologic vasculature. Dual-lumen balloon microcatheters like the Scepter Mini (Microvention, Aliso Viejo, CA) improve distal pedicle access, preventing reflux. Objective Here, we report on the use of the Scepter Mini for TAE of angiogenic VOGM. Methods A single-institution retrospective chart review identified all VOGMs treated with Scepter Mini microcatheters. Clinical data, angioarchitecture, and technical parameters were reviewed. Results 17 Scepter Mini catheters were used in 12 embolization procedures of 7 patients with VOGM at a median age of 2.1 years. Patients presented with hydrocephalus (100%) and gross motor and speech delays (57.1%). Networks developed extra-axially into the subependymal zone fed by posterior choroidal, posterior cerebral, and thalamoperforator arteries. Posterior choroidal branches (n=7/17, 41.2%) were most frequently catheterized to achieve distal access to the network. Embolization with Onyx-18 and significant network penetration occurred in 17/17 uses. Near tip entrapment with LEA cast displacement occurred in 1/17 uses. Another patient experienced postprocedural intraventricular hemorrhage requiring a third ventriculostomy without permanent neurologic deficit. Conclusion The Scepter Mini provided excellent distal access with penetration to the fistula and extra-axial network reduction with few complications. The Scepter Mini provides a means for successful treatment of technically challenging angiogenic VOGM. [ABSTRACT FROM AUTHOR]

Purpose: Proton stereotactic radiosurgery (PSRS) has emerged as an innovative proton therapy modality aimed at achieving precise dose delivery with minimal impact on healthy tissues. This study explores the dosimetric outcomes of PSRS in comparison to traditional intensity‐modulated proton therapy (IMPT) by focusing on cases with small target volumes. A custom‐made aperture system designed for proton therapy, specifically tailored to small target volumes, was developed and implemented for this investigation. Methods: A prerequisite mechanical validation through an isocentricity test precedes dosimetric assessments, ensuring the seamless integration of mechanical and dosimetry analyses. Five patients were enrolled in the study, including two with choroid melanoma and three with arteriovenous malformations (AVM). Two treatment plans were meticulously executed for each patient, one utilizing a collimated aperture and the other without. Both plans were subjected to robust optimization, maintaining identical beam arrangements and consistent optimization parameters to account for setup errors of 2 mm and range uncertainties of 3.5%. Plan evaluation metrics encompassing the Heterogeneity Index (HI), Paddick Conformity Index (CIPaddick), Gradient Index (GI), and the R50% index to evaluate alterations in low‐dose volume distribution. Results: The comparative analysis between PSRS and traditional PBS treatment revealed no significant differences in plan outcomes, with both modalities demonstrating comparable target coverage. However, collimated apertures resulted in discernible improvements in dose conformity, dose fall‐off, and reduced low‐dose volume. Conclusions: This study underscores the advantageous impact of the aperture system on proton therapy, particularly in cases involving small target volumes. [ABSTRACT FROM AUTHOR]

Gross hematuria is one of the most common complications in postrenal transplant patients, accounting for 12% of all renal recipients. The management plan in these cases varies depending on different entities, including infection, renal cell carcinoma, chronic graft rejection, kidney calculus, or recurrence of primary disease. On the other hand, vascular malformation like arteriovenous malformation was less likely to be mentioned due to a lack of consensus in the natural history, pathogenesis, and current management. In this article, we report a 62-year-old man presenting with spontaneous hematuria for a week and 2 days of anuria after 3 years of renal transplantation. Abdominal ultrasound and abdominopelvic computed tomography noted an obstruction of the renal pelvis due to blood clots without signs of vascular injuries. An emergency operation was performed to remove blood clots in the renal pelvis, but after that, hematuria was still recurrence. A digital renal graft subtraction angiography (DSA) revealed an arteriovenous malformation (AVM)in the kidney allograft. This lesion was then successfully selective embolized with glue. Given the high accuracy of DSA, our case highlights the potential role of this imaging modality in diagnosing and treating AVM after failure with other modalities.

Pulmonary arteriovenous malformations, previously considered a rare condition, have been increasingly identified in asymptomatic patients over the past 2 decades. Usually congenital and associated with hereditary hemorrhagic telangiectasia, these fistulae result in right-to-left shunting of blood by abnormal communication of pulmonary arteries and veins lacking capillary beds. Clinical findings of right-to-left shunting in the presence of feeding and draining vessels identified on imaging confirm the diagnosis, for which the first-line therapy is embolization. This report highlights the presentation and management of a large asymptomatic PAVM detected incidentally in a patient who was lost to follow-up for 10 years and represented with acute hypoxic respiratory failure secondary to a viral infection with an interval increase of PAVM size.