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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.

Background: The increase in the collagen I (COL I)/COL III ratio enhances vessel wall stiffness and renders vessels less resistant to blood flow and pressure changes. Activated microglia enhance inflammation-induced fibrosis. Hypotheses: The COL I/COL III ratio in human and mouse brain arteriovenous malformations (bAVMs) is associated with bAVM hemorrhage, and the depletion of microglia decreases the COL I/COL III ratio and hemorrhage. Method: COL I, COL III, and hemorrhages were analyzed in 12 human bAVMs and 6 control brains, and mouse bAVMs induced in three mouse lines with activin receptor-like kinase 1 (n = 7) or endoglin (n = 7) deleted in the endothelial cells or brain focally (n = 5). The controls for the mouse study were no-gene-deleted litter mates. Mouse bAVMs were used to test the relationships between the Col I/Col III ratio and hemorrhage and whether the transient depletion of microglia reduces the Col I/Col III ratio and hemorrhage. Results: The COL I/COL III ratio was higher in the human and mouse bAVMs than in controls. The microhemorrhage in mouse bAVMs was positively correlated with the Col I/Col III ratio. Transient depletion of microglia reduced the Col I/Col III ratio and microhemorrhage. Conclusions: The COL I/COL III ratio in the bAVMs was associated with bAVM hemorrhage. The depletion of microglia reduced the bAVM Col I/Col III ratio and hemorrhage.

Background: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant inherited vascular disorder that can involve multiple organs, thus can be associated with so many clinical departments that proper screening and diagnosis of HHT are needed for providing better management of both patients and their family members. Case presentation: We present a 58-year-old female patient with recurrent paradoxical brain embolism due to HHT. She received aspirin therapy and underwent pulmonary arteriovenous malformation embolization, recovering well and discharged 3 days postoperatively. Though ischemic stroke caused by HHT-induced vascular disorders has been reported, our patient presented with both recurrent paradoxical brain embolisms and radiologic findings of bilateral globus pallidus manganese deposition at the same time, a combination rarely reported. We also review the literature on the clinical features and management of HHT for prompt diagnosis of this genetic disease behind paradoxical embolism. Conclusions: When patients with ischemic stroke, especially recurrent ischemic stroke, have combined arteriovenous malformations (AVMs) in single or multiple organs, or clues for AVMs like manganese deposition in globus pallidus, genetic diseases such as HHT may be the reason for ischemic stroke and shouldn't be missed in the evaluation of embolic sources. [ABSTRACT FROM AUTHOR]

Extra Nasopharyngeal Angiofibromas are rare benign vascular tumors typically affecting adolescent males. They have variable sites of origin. Only five cases of extra nasopharyngeal angiofibroma originating from the middle turbinate have been reported in the literature, here we present the sixth such case. A 44-year-old female presented with a history of left-sided nasal obstruction and spontaneous, profuse and recurrent epistaxis. A contrast-enhanced computed tomography (CT) scan showed a hyper vascular mass arising from the left middle turbinate. A provisional diagnosis of extra nasopharyngeal angiofibroma was made and it was successfully surgically excised using a midfacial degloving approach under general anaesthesia. The diagnosis was confirmed with post-operative histopathological analysis. Due to the extremely rare occurrence of Extra Nasopharyngeal Angiofibromas originating from the middle turbinate and their infrequent presentation in female patients, misdiagnosis poses a greater risk of complications. The surgeon must exercise caution before conducting misguided biopsies, which are contraindicated. [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]

Arteriovenous malformations (AVMs) are rare vascular anomalies that present complex diagnostic and therapeutic challenges, particularly in uncommon locations such as the lower extremities. A 72-year-old female with chronic atrial fibrillation, hypertension, and peripheral vascular disease presented with severe lower extremity edema due to multiple AVMs below the knee. This case underscores the importance of a multidisciplinary, individualized approach in managing complex AVMs and highlights the need for advanced imaging and diverse interventional techniques to ensure effective treatment and long-term outcomes.

Abstract Background Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant inherited vascular disorder that can involve multiple organs, thus can be associated with so many clinical departments that proper screening and diagnosis of HHT are needed for providing better management of both patients and their family members. Case presentation We present a 58-year-old female patient with recurrent paradoxical brain embolism due to HHT. She received aspirin therapy and underwent pulmonary arteriovenous malformation embolization, recovering well and discharged 3 days postoperatively. Though ischemic stroke caused by HHT-induced vascular disorders has been reported, our patient presented with both recurrent paradoxical brain embolisms and radiologic findings of bilateral globus pallidus manganese deposition at the same time, a combination rarely reported. We also review the literature on the clinical features and management of HHT for prompt diagnosis of this genetic disease behind paradoxical embolism. Conclusions When patients with ischemic stroke, especially recurrent ischemic stroke, have combined arteriovenous malformations (AVMs) in single or multiple organs, or clues for AVMs like manganese deposition in globus pallidus, genetic diseases such as HHT may be the reason for ischemic stroke and shouldn’t be missed in the evaluation of embolic sources.

Arteriovenous malformations (AVM) are vascular abnormalities, most often presenting significant risk of massive bleeding and being lethal for the patient. Thus, the knowledge of these lesions must be adequate for both the clinical and the oral radiologists. In this paper we describe the medical history of a young woman with a massive arteriovenous malformation localized at the left side of the mandible.

Brain arteriovenous malformations (AVM) present complex treatment decisions, particularly for low-grade AVM where surgical resection is often considered the standard. This case report emphasizes the importance of patient preferences and cultural considerations in selecting endovascular embolization over traditional surgical approaches for Spetzler-Martin Grade I AVM management, highlighting the evolving practice of patient-centered care in neurointervention. A 30-year-old male presented with recurrent seizures, characterized by a sudden onset of headache followed by speech arrest, without any preceding medical history of neurological deficits. Initial physical examination revealed no focal neurological deficits. Non-contrast computed tomography, magnetic resonance imaging, and magnetic resonance angiography suggested an AVM involving the cortical-subcortical regions of the left frontal lobe, measuring approximately 1.7 × 2.6 × 1.5 cm, fed by the left middle cerebral artery M3 segment, and draining into the superior sagittal sinus. Spetzler-Martin Grade I classification was confirmed via digital subtraction angiography. Given the patient's strong preference against invasive procedures, driven by personal and cultural beliefs, endovascular embolization was selected as the treatment strategy. Post-embolization, the patient showed marked symptomatic improvement with no evidence of residual AVM on follow-up imaging, and no postprocedure complications were reported. This case highlights the importance of considering patient preferences in AVM treatment planning, illustrating that endovascular embolization can be an effective and less invasive alternative to surgery in selected patients, reinforcing the need for personalized, patient-centered approaches in neurointerventional care.

Background: Cerebral arteriovenous malformations (AVMs) are complex, heterogeneous, uncommon lesions that carry a high risk of intracranial hemorrhage with subsequent disability. Aim: To evaluate the outcome of patients with cerebral AVMs treated in our center, King Faisal Specialized Hospital and Research Center (KFSH&RC) Saudi Arabia with SRS using the CyberKnife. Methods: Medical records of patients treated in the above-mentioned way in the period between 2009-2021 were retrospectively evaluated. Results: A total of 51 patients were included in this study. The median age at diagnosis was 31 (3-77) years. Neurological alteration and intracranial hemorrhage were the most common presentations in 24 (47.1%) patients. The mean maximum AVM nidus diameter and volume were 2.9 cm and 5.9 cc respectively. All the patients received SRS using CyberKnife with a median marginal dose of 20 (14-21) Gy over a single fraction. Volume staged treatment was applied in 4 (7.8%) patients. After a median follow-up of 37 (2-170) months, all patients were alive with successful obliteration of the AVM nidus occurring in 31 (60.8%) patients after a median latency period of 27 (4-68.9) months in those who achieved complete obliteration. No patients developed post-SRS hemorrhage and 11 (21.6%) patients developed post-SRS radiological radiation-induced changes of which only 3 (27.3%) patients were symptomatic (in the form of uncontrolled seizures). only SRS dose > 19 Gy was significantly correlated with the obliteration rate, as 79.3% of patients who received SRS dose > 19 Gy achieved obliteration vs 36.4 % only in those who received dose Conclusion: SRS treatment using CyberKnife is effective management for patients with cerebral AVMs without an increase in the incidence of post-SRS hemorrhage, stroke, or death.

We present a case of successful embolization of an anterior chest wall arteriovenous malformation (AVM) in a 24-year-old male patient. This report aims to highlight the efficacy and safety of using the liquid embolic agent (onyx) as well as the combined approaches (trans-femoral and trans-radial) in managing rare complex chest wall AVMs.

Arteriovenous malformations (AVMs) are complex vascular lesions most commonly found in the brain and infrequently found in the head and neck. AVMs are characterized by a tangle of blood vessels called a nidus, which shunts blood from an artery directly to a draining vein. Various treatments are available, including surgical resection and endovascular embolization. Here, we report the case of a 32-year-old male patient who complained of painful pulsating left neck swelling with dysphagia for 1 year, which turned out to be an AVM alongside the left thyroid gland. The AVM was treated by embolization using Onyx in 2 sessions. The patient has been free of symptoms since the treatment.

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]

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]

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Background: Cerebral arteriovenous malformations (AVMs) are complex, heterogeneous, uncommon lesions that carry a high risk of intracranial hemorrhage with subsequent disability. Aim: To evaluate the outcome of patients with cerebral AVMs treated in our center, King Faisal Specialized Hospital and Research Center (KFSH&RC) Saudi Arabia with SRS using the CyberKnife. Methods: Medical records of patients treated in the above-mentioned way in the period between 2009-2021 were retrospectively evaluated. Results: A total of 51 patients were included in this study. The median age at diagnosis was 31 (3-77) years. Neurological alteration and intracranial hemorrhage were the most common presentations in 24 (47.1%) patients. The mean maximum AVM nidus diameter and volume were 2.9 cm and 5.9 cc respectively. All the patients received SRS using CyberKnife with a median marginal dose of 20 (14-21) Gy over a single fraction. Volume staged treatment was applied in 4 (7.8%) patients. After a median follow-up of 37 (2-170) months, all patients were alive with successful obliteration of the AVM nidus occurring in 31 (60.8%) patients after a median latency period of 27 (4-68.9) months in those who achieved complete obliteration. No patients developed post-SRS hemorrhage and 11 (21.6%) patients developed post-SRS radiological radiation-induced changes of which only 3 (27.3%) patients were symptomatic (in the form of uncontrolled seizures). only SRS dose > 19 Gy was significantly correlated with the obliteration rate, as 79.3% of patients who received SRS dose > 19 Gy achieved obliteration vs 36.4 % only in those who received dose <19 Gy, p value 0.002. Conclusion: SRS treatment using CyberKnife is effective management for patients with cerebral AVMs without an increase in the incidence of post-SRS hemorrhage, stroke, or death. [ABSTRACT FROM AUTHOR]

Background: AV malformations are aberrations in the development of vessels with varied presentations that may lead to aesthetic, limb and life-threatening complications. Identification and treatment of the vascular malformations is a challenging task for surgeons. Aims & Objectives: To study clinical presentation/ diagnostic modalities/ treatment strategies, complications and recurrence rates of surgical and other interventional strategies. Material and Methods: A retrospective study of 150 patients who were treated over a period of 3 years from a tertiary centre with regular follow-ups was done to study the best diagnostic and treatment plans for initial and recurrent lesions. Results: The study showed an age group of

Abstract Background Clinically, arteriovenous malformations in the buttocks (bAVMs) are extremely rare. Our study aimed to evaluate the efficacy and safety of ethanol embolotherapy in managing bAVMs. Results A total of 32 patients with bAVMs (14 females and 18 males) from 2012 to 2021 were included in this study. All patients underwent complete clinical and imaging examinations. Further, the AVMs lesions were analyzed according to Schöbinger staging and Yakes classification. Each patient had undergone a multistage ethanol embolization. The amelioration of clinical symptoms and devascularization on angiography were evaluated at regular follow-ups. In the present cohort, the 11–20 age group had the most patients (15/32; 46.88%). A total of 124 embolization procedures were performed (average 3.88 procedures per patient), and the average dose of absolute ethanol was 18.96 mL per procedure. Thirteen patients with dominant draining veins underwent additional coil deployment before ethanol embolization (13/32; 40.63%). During follow-ups, clinical improvement was found in 23 of 27 who presented with a pulsating mass (85.19%), 17 of 20 with abnormal local skin temperature (85%), 5 of 6 with bleeding (83.33%), and 5 of 5 patients treated for pain (100%). More than 75% angiographic devascularization was achieved in 18 patients (18/32; 56.25%). Finally, 12 out of 13 patients (92.31%) reduced from Schöbinger Stage III to a lower grade, and ten patients exhibited a complete response (10/32; 31.23%). There was a single serious complication of local necrosis, while neither paranesthesia nor infection was observed postoperatively. Conclusions Ethanol embolization assisted with coils can treat bAVMs effectively and safely. The Yakes classification contributed to the optimal ethanol embolotherapy of bAVMs.

Abstract Background Osler-Weber-Rendu syndrome (OWRS) is an autosomal dominant disease with recurrent epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations. Its clinical presentation ranges from simple skin lesions to life-threatening complications. When the diagnosis of the disease is delayed, it can be mortal and have high morbidity. This case is presented because OWRS is rare, and although she has all the features of the disease, she was diagnosed in the geriatric age group. Case presentation A 72-year-old female patient who applied to the emergency department with the complaint of melena was diagnosed with upper gastrointestinal system bleeding. Gastroscopy revealed angiodysplasia in the bulb. Colonoscopy was normal. She had telangiectasias on her face, maxilla, nose, tongue and lips. She also had recurrent epistaxis and a family history. Thorax CT showed aneurysmatic vascular malformations in the lung. Abdominal MRI revealed vascular malformations in the liver. Brain MRI was normal. The patient was diagnosed with OWRS according to the Curaçao diagnostic criteria because of telangiectasia in the mouth, nose, and face, angiodysplasia in the gastric bulb, aneurysmatic vascular dilatation in the lung, vascular pathologies in the liver, and a history of epistaxis and telangiectasia in the patient’s family members. Conclusion This patient is presented because OWRS is rare, and although she has all the features of the disease, she was diagnosed in the geriatric age group. Clinicians should be aware of this rare disease. Especially in the elderly, when angiodysplasia causing gastrointestinal bleeding is detected, it should be considered in the differential diagnosis that this may be an OWSR case.