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Objective: This case study aims to present the Foreign Accent Syndrome (FAS) in a patient with Cerebral Arteriovenous Malformation (cAVM), considering neuropsychological, radiological and microsurgical aspects. Methods: The patient underwent preoperative neuropsychological assessment and MRI and Tractography were performed to identify fibers close to the lesion site. In the surgical procedure, a craniotomy was performed for excision of the cAVM. After surgery and 6 months after the surgical procedure, the patient underwent further and neuropsychological evaluations. Results: The presence of AVM located in the posterior part of the medial surface of the left superior frontal gyrus was identified and the neuropsychological evaluation found cognitive deficits and symptoms characteristic of FAS, which disappeared after resection. Conclusion: This report presented a case of cAVM in which symptoms were found even without rupture, which was no longer observed after the surgical procedure, demonstrating the relationship of cAVM with the symptoms and neuroanatomical bases of FAS. [ABSTRACT FROM AUTHOR]

Introduction: Cerebral arteriovenous malformations (CAVMs) are rare, with an estimated prevalence of 0.01%. Symptoms typically present in adults under 40, often beginning with hemorrhage in 61% of cases. The annual risk of hemorrhage is between 2–4%, with a mortality rate of 10%, and 50% of survivors may experience permanent neurological deficits. Embolization can induce changes in the angioarchitecture of the affected vessels. Our case uniquely demonstrates a morphological alteration in the carotid artery (CA) and its primary branches, associated with the presence of a CAVM. Detailed Case Presentation: A 52-year-old patient presented to the Emergency Department with weakness, shortness of breath, dizziness, nausea, and vomiting that began earlier that morning. Clinical and radiological evaluations suggested acute bleeding from a ruptured CAVM. The patient was admitted for digital subtraction angiography (DSA) and potential embolization. DSA confirmed the presence of a CAVM, supplied by the middle meningeal and occipital arteries, with distortion of the left CA angioarchitecture. Selective catheterization and embolization using Onyx 18 were successfully performed. After receiving supportive treatment, the patient was discharged in good condition. A follow-up CT scan three months later showed complete resolution of the brain pathologies. A repeat angiogram revealed no recanalization of the CAVM, with normalization of the CA and its branches. The patient is currently asymptomatic. Conclusions: Detection of pathological changes in the head and neck arteries linked to CAVM can predict rupture risk and complicate endovascular access. Identifying these changes early warrants consideration of interventions to prevent hemorrhage, though alternative access routes or strategies may be needed for safe, effective treatment. [ABSTRACT FROM AUTHOR]

AbstractBackgroundMethodResultsConclusionAngiogenesis and nascent blood vessel formation is thought to be important in cerebral arteriovenous malformation (cAVM) development and maintenance, of which little is known. Digital subtraction angiogram (DSA) features of angiogenesis in cAVMs are poorly described and the aim of this study was to describe the frequency of angiogenesis in patients who had a DSA showing a cAVM. We also sought to evaluate the intra- and inter-observer agreement of a diagnosis of angiogenesis and explore which angioarchitectural features were associated with angiogenesis.Patients that underwent a DSA were identified from the database of referred cAVM patients at the Manchester Centre for Clinical Neurosciences. Data were collected from 100 patients (102 cAVMs). cAVM angioarchitecture, including the presence of angiogenesis, was described after reviewing cAVM patient angiograms. The association of angioarchitectural features with angiogenesis was determined using univariate analysis. Ten cases were distributed amongst two other observers for reporting (inter-observer agreement). Twenty cases (including the previous 10) were reported twice by the first author, after a six-month interval (intra-observer agreement).Angiogenesis was observed in 39 cAVMs (38.2%), with 12 having a complete border (11.8%). Most intra-observer agreement was strong (ranging from κ = 1 to 0.2), but inter-observer agreement was moderate (κ = 1 to −0.316). There was a significant association between angiogenesis and venous reflux (OR 2.52 [95% CI = 1.08–5.88]), venous congestion (OR 4.47 [95% CI = .671–2.52]), arterial ectasia (OR 16.6 [95% CI = 4.65–59.6]), and artery: vein ratio (4.28 [95% CI = .956–19.15]).We have demonstrated perinidal angiogenesis can be visualised on angiograms with moderate reliability, and that it may be related to angioarchitectural characteristics associated with venous hypertension. [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]

Objective: Cerebral arteriovenous malformation during pregnancy is rare but lethal disease that usually present with new-onset seizures and headaches mimicking eclampsia. We report a rare case of cerebral arteriovenous malformation with abrupt seizures in the third trimester. Case Report: A 28-year-old primipara was brought to our emergency department at 32 6/7 weeks of gestation with new-onset acute seizures and hypertension. Owing to neurological deterioration, the patient underwent emergency cesarean delivery. However, 24 h after cesarean delivery and eclampsia treatment, the seizures worsened. Computed tomography and magnetic resonance imaging showed unruptured arteriovenous malformation of the right frontal lobe. Subsequently, intraarterial embolization was performed. The patient was discharged 5 days after surgery without neurological sequelae or obstetric complications. Conclusion: This case report highlights the differential diagnoses of sudden new-onset seizures in late pregnancy for obstetricians and emergency medicine physicians. Lethal cerebral diseases, apart from eclampsia, should be considered during pregnancy.

A cerebral arteriovenous malformation (cAVM) is an abnormal tangle of cerebral blood vessels. The consensus document by the Joint Writing Group (JWG) highlighted which cAVM features should be recorded. Subsequent publications have reported cAVM angioarchitecture, but it is unknown if all followed the JWG recommendations. The aim of this systematic review was to describe use of the JWG guidelines. A database search, using the PRISMA checklist, was performed. We describe the proportion of publications that used JWG reporting standards, which standards were used, whether the definitions used differed from the JWG, or if any additional angiographic features were reported. Out of 4306 articles identified, 105 were selected, and a further 114 from other sources. Thirty-three studies (33/219; 15%) specifically referred to using JWG standards. Since the JWG publication, few studies have used their standards to report cAVMs. This implies that the angioarchitecture of cAVMs are not routinely fully described.

A cerebral arteriovenous malformation (cAVM) is an abnormal tangle of cerebral blood vessels. The consensus document by the Joint Writing Group (JWG) highlighted which cAVM features should be recorded. Subsequent publications have reported cAVM angioarchitecture, but it is unknown if all followed the JWG recommendations. The aim of this systematic review was to describe use of the JWG guidelines. A database search, using the PRISMA checklist, was performed. We describe the proportion of publications that used JWG reporting standards, which standards were used, whether the definitions used differed from the JWG, or if any additional angiographic features were reported. Out of 4306 articles identified, 105 were selected, and a further 114 from other sources. Thirty-three studies (33/219; 15%) specifically referred to using JWG standards. Since the JWG publication, few studies have used their standards to report cAVMs. This implies that the angioarchitecture of cAVMs are not routinely fully described.

Background: The disruption of intracranial fluid dynamics due to large unruptured cerebral arteriovenous malformation (AVM) commonly triggers a domino effect within the central nervous system. This phenomenon is frequently overlooked in prior clinic and may lead to catastrophic misdiagnoses. Our team has documented the world's first case of so-called AVM Pentalogy (AVMP) induced by a AVM. Clinical presentation and result: A 30-year-old female was first seen 9years ago with an occasional fainting, at which time a huge unruptured AVM was discovered. Subsequently, due to progressive symptoms, she sought consultations from several prestigious neurosurgical departments in China, where all consulting neurosurgeons opted for conservation treatment due to perceived surgical risks. During the follow-up period, the patient gradually presented with hydrocephalus, empty sella, secondary Chiari malformation, syringomyelia, and scoliosis (we called as AVMP). When treated in our department, she already displayed numerous symptoms, including severe intracranial hypertension. Our team deduced that the hydrocephalus was the primary driver of her AVMP symptoms, representing the most favorable risk profile for intervention. As expected, a ventriculoperitoneal shunt successfully mitigated all symptoms of AVMP at 21-months post-surgical review. Conclusion: During the monitoring of unruptured AVM, it is crucial to remain vigilant for the development or progression of AVMP. When any component of AVMP is identified, thorough etiological studies and analysis of cascade reactions are imperative to avert misdiagnosis. When direct AVM intervention is not viable, strategically addressing hydrocephalus as part of the AVMP may serve as the critical therapeutic focus. [ABSTRACT FROM AUTHOR]

Purpose: Cerebral arteriovenous malformations (AVM) represent focal abnormal areas of low resistance circulation which render the peri-nidal neuronal tissue susceptible to ischemia. The post-excision cerebral perfusion surge can result in hyperaemic complications.We hypothesised that Near Infrared Spectroscopy (NIRS)-guided perioperative management can aid in the prediction and prevention of perioperative complications in patients presenting for surgical excision of cerebral AVMs. We also intended to identify a threshold value of regional cerebral oxygen saturation (rScO2) to predict the incidence of perioperative complications. Methods: This was a prospective observational study involving patients undergoing elective supratentorial AVM resection surgeries. Intraoperative rScO2 and hemodynamic monitoring were done and continued for postoperatively for 12 h. Any drift in rScO2 by > 12% from baseline was managed as per study protocol and perioperative adverse events were recorded and analyzed. Post surgery,for analytical purpose patients were categorized into two groups, Group A - patients without complications and Group B - patients who had complications postoperatively. Results: Twenty-five patients presenting for surgical excision of cerebral AVM were recruited for this study of which 9 patients had postoperative adverse events and were allocated to group B. The ipsilateral mean rScO2 at the time of complication (Pc) was significantly lower in Group B than in group A [62.08 ± 9.33 vs.70.52 ± 7.17; p = 0.04]. The mean ipsilateral rScO2 drift from N2- N5 (i.e., post excision) was significantly higher in Group B than in Group A [12.01 ± 2.63% vs. 4.98 ± 5.7%;p = 0.02]. Mean ipsilateral rScO2 Drift ratio (N5 :N2) was significantly higher in group B as compared to group A [1.32 ± 0.01 vs. 1.01 ± 0.06;p < 0.001]. In the immediate post excision phase, the ipsilateral mean rScO2 was significantly higher in Group B at the post excision time point compared to Group A [ 83.03 ± 6.08 vs. 73.52 ± 7.07;p < 0.01)]. The mean ipsilateral rScO2 drift from N1-N6 (i.e., postoperatively) was significantly higher in Group B as compared to Group A [14.96 ± 0.080% vs. 6.88 ± 4.5% ; p < 0.01]. Similarly, the Mean Ipsilateral rScO2 Drift ratio (N6:N1) was significantly lower in group B as compared to group A [2.17 ± 0.02 vs. 1.05 ± 0.03 ;p < 0.0001]. Conclusions: In patients undergoing cerebral AVM resection, a post-resection ipsilateral rScO2 increase by > 12% with a drift ratio of > 1.3 could signify cerebral hyperemia. A postoperative ipsilateral rScO2 drift > 14.5% with a drift ratio of 2.1 from the baseline is associated with postoperative complications in our study. Further multi-centric randomized control trials are needed to support our research findings. [ABSTRACT FROM AUTHOR]

A cerebral arteriovenous malformation (cAVM) is an abnormal tangle of cerebral blood vessels. The consensus document by the Joint Writing Group (JWG) highlighted which cAVM features should be recorded. Subsequent publications have reported cAVM angioarchitecture, but it is unknown if all followed the JWG recommendations. The aim of this systematic review was to describe use of the JWG guidelines. A database search, using the PRISMA checklist, was performed. We describe the proportion of publications that used JWG reporting standards, which standards were used, whether the definitions used differed from the JWG, or if any additional angiographic features were reported. Out of 4306 articles identified, 105 were selected, and a further 114 from other sources. Thirty-three studies (33/219; 15%) specifically referred to using JWG standards. Since the JWG publication, few studies have used their standards to report cAVMs. This implies that the angioarchitecture of cAVMs are not routinely fully described.

Arteriovenous malformations (AVMs) are a tangle of blood vessels that connects arteries and veins in which the feeding arteries are directly connected to the venous channels. Cerebral AVM needs special consideration due to high risk of bleeding and neurological deficit associated with morbidity and mortality. In pediatric population, prevalence rate of cerebral AVM is 0.02%. The systemic hemodynamic changes are seen more frequently with peripheral AVM, whereas its association with cerebral AVM is rare. We report a rare case of cerebral AVM in a 12-year-old child with severe systemic hypertension and electrocardiogram changes of biventricular hypertrophy planned for surgical resection of AVM in view of intracerebral bleed. This case illustrates the importance of detailed cardiac, renal, and endocrine evaluation in children with cerebral AVM with systemic hypertension and left ventricular hypertrophy to rule out other causes of systemic hypertension prior to surgery.

Pai syndrome is a rare entity, initially described as a frontonasal dysplasia that associates the presence of skin polyp on the nasal bridge, midline cleft of the upper lip and lipoma of the corpus callosum, due to the wide phenotypic variability. Recently the diagnostic criteria were updated to include the presence of a congenital midfrontal or nasal skin mass or polyp in the alveolar process associated with mid-upper lip fissure and/or pericallosal lipoma. We present the case of a 10-month-old infant with Pai syndrome associated with cerebral arteriovenous malformation, in our review such association was not found in the literature reviewed. The patient received multidisciplinary care that included surgeries by the specialties of otorhinolaryngology and plastic surgery with a favorable outcome. [ABSTRACT FROM AUTHOR]

Arteriovenous malformations (AVMs) are a tangle of blood vessels that connects arteries and veins in which the feeding arteries are directly connected to the venous channels. Cerebral AVM needs special consideration due to high risk of bleeding and neurological deficit associated with morbidity and mortality. In pediatric population, prevalence rate of cerebral AVM is 0.02%. The systemic hemodynamic changes are seen more frequently with peripheral AVM, whereas its association with cerebral AVM is rare. We report a rare case of cerebral AVM in a 12-year-old child with severe systemic hypertension and electrocardiogram changes of biventricular hypertrophy planned for surgical resection of AVM in view of intracerebral bleed. This case illustrates the importance of detailed cardiac, renal, and endocrine evaluation in children with cerebral AVM with systemic hypertension and left ventricular hypertrophy to rule out other causes of systemic hypertension prior to surgery. [ABSTRACT FROM AUTHOR]

Introduction: Cerebral arteriovenous malformations (CAVMs) are rare, with an estimated prevalence of 0.01%. Symptoms typically present in adults under 40, often beginning with hemorrhage in 61% of cases. The annual risk of hemorrhage is between 2–4%, with a mortality rate of 10%, and 50% of survivors may experience permanent neurological deficits. Embolization can induce changes in the angioarchitecture of the affected vessels. Our case uniquely demonstrates a morphological alteration in the carotid artery (CA) and its primary branches, associated with the presence of a CAVM. Detailed Case Presentation: A 52-year-old patient presented to the Emergency Department with weakness, shortness of breath, dizziness, nausea, and vomiting that began earlier that morning. Clinical and radiological evaluations suggested acute bleeding from a ruptured CAVM. The patient was admitted for digital subtraction angiography (DSA) and potential embolization. DSA confirmed the presence of a CAVM, supplied by the middle meningeal and occipital arteries, with distortion of the left CA angioarchitecture. Selective catheterization and embolization using Onyx 18 were successfully performed. After receiving supportive treatment, the patient was discharged in good condition. A follow-up CT scan three months later showed complete resolution of the brain pathologies. A repeat angiogram revealed no recanalization of the CAVM, with normalization of the CA and its branches. The patient is currently asymptomatic. Conclusions: Detection of pathological changes in the head and neck arteries linked to CAVM can predict rupture risk and complicate endovascular access. Identifying these changes early warrants consideration of interventions to prevent hemorrhage, though alternative access routes or strategies may be needed for safe, effective treatment.

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We aimed to determine whether the hemorrhage risks of cerebral arteriovenous malformation (AVM), evaluated through digital subtraction angiography (DSA) using a neural network, were superior to those assessed through angioarchitecture. We conducted a retrospective review of patients with cerebral AVM who underwent DSA from 2011 to 2017. Angioarchitecture parameters, age, and sex were analyzed using univariate and multivariate logistic regression. Additionally, a neural network was trained using a combination of convolutional neural network (CNN) and recurrent neural network (RNN) architectures. The training dataset consisted of 118 samples, while 29 samples were reserved for testing. After adjusting for age at diagnosis and sex, single venous drainage (odds ratio [OR] = 2.48, p = 0.017), exclusive deep venous drainage (OR = 3.19, p = 0.005), and venous sac (OR = 0.43, p = 0.044) were identified as independent risk factors for hemorrhage. The angioarchitecture-based hemorrhagic prediction model achieved 69% accuracy with an AUC (area under the ROC curve) of 0.757, while the CNN–RNN-based model achieved 76% accuracy with an AUC of 0.748. We present a diagnostic performance for hemorrhagic risk assessment of AVMs that is comparable to the angioarchitectural analysis. By leveraging larger datasets, there is significant potential to enhance prediction accuracy further. The CNN–RNN algorithm not only can potentially streamline workflow within the angio-suite but also serves as a complementary approach to optimize diagnostic accuracy and treatment strategies. [ABSTRACT FROM AUTHOR]

Complex cerebral arteriovenous malformations (AVMs) require a combined therapy of endovascular embolization and microsurgical resection to eliminate the lesion and maximize neurological protection, while a deliberate time interval might contribute to optimal clinical outcomes. The present study aimed to explore the feasibility of this paradigm. All patients who underwent deliberately planned presurgery embolization and microsurgery resection between 2015 and 2023 were reviewed, with baseline data, postoperative complications, and follow-up outcomes recorded. The modified Rankin scale (mRS) was used to evaluate clinical outcomes, with mRS 0–2 defined as good. A total of 30 patients were included in the study (15 were ruptured AVMs). The median Spetzler–Martin grade of baseline AVMs was 3 (interquartile range: 2–3). The median interval between the last embolization and microsurgery was 5 days (interquartile range: 2.25–7). The complete removal rate was 100%, and the overall permanent complication rate was 16.67%. At the last follow-up, 26 patients achieved mRS 0–2, while 28 had improved or unaltered mRS. The last follow-up mRS significantly improved from baseline and discharge (P = 0.0006 and P = 0.006). The last follow-up mRS decreased by 0.65 for each additional day of time interval before the 4.4-day inflection point (β = −0.65, P = 0.02) in the AVM ruptured cohort. The deliberately staged combined procedure of embolization and microsurgery might be a safe and efficacious strategy for Spetzler–Martin grade 2–5 AVMs, 4–5 days might be an appropriate staged time interval for ruptured AVMs, although further studies are needed to substantiate these findings. [ABSTRACT FROM AUTHOR]

A cerebral arteriovenous malformation (cAVM) is an abnormal tangle of cerebral blood vessels. The consensus document by the Joint Writing Group (JWG) highlighted which cAVM features should be recorded. Subsequent publications have reported cAVM angioarchitecture, but it is unknown if all followed the JWG recommendations. The aim of this systematic review was to describe use of the JWG guidelines. A database search, using the PRISMA checklist, was performed. We describe the proportion of publications that used JWG reporting standards, which standards were used, whether the definitions used differed from the JWG, or if any additional angiographic features were reported. Out of 4306 articles identified, 105 were selected, and a further 114 from other sources. Thirty-three studies (33/219; 15%) specifically referred to using JWG standards. Since the JWG publication, few studies have used their standards to report cAVMs. This implies that the angioarchitecture of cAVMs are not routinely fully described.

It is difficult to accurately understand the angioarchitecture of cerebral arteriovenous malformations (CAVMs) before surgery using existing imaging methods. This study aimed to evaluate the ability of the stereoscopic virtual reality display system (SVRDS) to display the angioarchitecture of CAVMs by comparing its accuracy with that of the conventional computed tomography workstation (CCTW). Nineteen patients with CAVM confirmed on digital subtraction angiography (DSA) or during surgery were studied. Computed tomography angiography images in the SVRDS and CCTW were retrospectively analyzed by two experienced neuroradiologists using a double-blind method. Angioarchitectural parameters, such as the location and size of the nidus, type and number of the arterial feeders and draining veins, and draining pattern of the vessels, were recorded and compared. The diameter of the nidus ranged from 1.1 to 9 cm. Both CCTW and SVRDS correctly diagnosed the location of the nidus in 19 patients with CAVM. Among the 19 patients, 35 arterial feeders and 25 draining veins were confirmed on DSA and during surgery. With the DSA and intraoperative results as the gold standard bases, the CCTW misjudged one arterial feeder and one draining vein and missed three arterial feeders and two draining veins; meanwhile, the SVRDS missed only two arterial feeders. SVRDS had some advantages in displaying nidus, arterial branches, and draining veins of the CAVM compared with CCTW, as well as SVRDS could more intuitively display the overall angio-architectural spatial picture of CAVM. [ABSTRACT FROM AUTHOR]

Spontaneous occlusion of arteriovenous malformations (AVM), characterized by complete disappearance of the AVM nidus and early venous drainage, and is a rare. The frequency of this phenomenon varied from 0.1 to 1.3 %. We analyzed two cases of spontaneous occlusion of cerebral AVMs with a non-hemorrhagic debut after 3 and 13 years in women aged 28 and 40 years, respectively. In both cases AVM was diagnosed during routine neuroimaging because of headache. Both patients had superficial small malformations. After discussing the risks of surgical treatment, both patients refused to perform any intervention. Scheduled angiography was performed 3 years after the diagnosis to follow-up the course of the disease and revealed complete disappearance of the AVM. Another patient noted persistent regression of headache after 3 years. Follow-up angiography was performed 13 years after diagnosis and confirmed spontaneous occlusion of the AVM. Given the data on the recurrence of the disease after spontaneous occlusion, such patients require long-term follow-up. When an AVM ruptures, hemodynamic changes may explain the thrombosis of the malformation, but the mechanisms of spontaneous occlusion in non-ruptured AVM remain unclear.

This paper summarized the nursing management for a patient with intracerebral hemorrhage due to ruptured cerebral arteriovenous malformation and neurogenic pulmonary edema. Based on the monitoring of postoperative vital signs of the patient, efforts were made in the following aspects: Early identification and intervention on normal perfusion pressure breakthrough syndrome; Ensuring the effective cerebral perfusion pressure during control of blood pressure and intracranial pressure; Early identification and nursing of neurogenic pulmonary edema; Enhancing the postoperative airway, fluid and volume management; Providing lung protective ventilation strategy; Carrying out awakenings and awareness recovery for the patient with chronic disorders of consciousness. (本文总结1例脑动静脉畸形破裂出血并发神经源性肺水肿患者的护理体会。术后在严密监测神志、瞳孔、生命体征变化基础上, 对正常灌注压突破综合征进行早期识别及干预, 在控制性降压及颅内压的同时保证有效的脑灌注压; 做好神经源性肺水肿的早期识别及护理, 在扩容的同时预防肺水肿进一步加重; 做好患者的呼吸道管理及容量管理, 给予正确的肺保护通气策略, 细化出入量; 对慢性意识障碍进行促醒康复, 促进患者早日康复。)

目的 探究散发性脑动静脉畸形组织中Kirsten大鼠肉瘤病毒癌基因同源物（Kirsten rat sarcoma viral oncogene homolog，KRAS）突变与临床特征之间的关系。 方法 回顾性收集散发性动静脉畸形（brain arteriovenous malformation，BAVM）术后病变组织的福尔马林固定石蜡包埋（formali n-fixation and paraffin-embedding，FFPE）样本和对应患者的临床信息，从FFPE样本中提取DNA，通过PCR扩增KRAS的指定区域（以chr12：25398284为靶点）并进行超深扩增子测序。根据是否存在KRAS突变分为突变组和无突变组，比较2组的临床特征差异。 结果 本研究共纳入145例BAVM患者的FFPE样本。其中63例（43.4%）存在KRAS 体细胞突变。其中41例（28.3%）中检测到c.35G→A（G12D）突变，14例（9.7%）中检测到c.35G→T（G12V）突变。此外，还发现了几种罕见报道的KRAS突变，包括c.34G→T（G12C）突变8例（5.5%）、c.34G→A（G12S）突变7例（4.8%）、c.35G→C（G12A）突变2例（1.4%）、c.34G→C（G12R）突变1例（0.7%）。KRAS基因突变组与无突变组的临床特征差异无统计学意义。 结论 本研究发现43.4%的BAVM患者存在KRAS基因突变，其中G12D是最常见的KRAS突变类型。此外，还发现了一些罕见报道的突变类型，包括G12C和G12A等。未发现KRAS突变与临床特征之间的关系. Abstract: Objective To investigate the relationship between Kirsten rat sarcoma viral oncogene (KRAS) mutation in brain arteriovenous malformation (BAVM) and clinical features. Methods The sporadic BAVM formalin-fixation and paraffin-embedding (FFPE) samples and the corresponding clinical features were collected. DNA was extracted from the BAVM FFPE samples, the specified region of KRAS (targeting chr12:25398284) was amplified by PCR and ultra-deep amplicon sequencing was performed. According to having KRAS mutation or not, the patients were divided into mutation group and no mutation group, the clinical features of the two groups were compared. Results A total of 145 BAVM FFPE samples were collected, with 63 (43.4%) samples with KRAS somatic mutation. Of these KRAS mutations, c.35G→A(G12D) were detected in 41 samples (28.3%), and c.35G→T(G12V) in 14 samples (9.7%). In addition, several infrequent KRAS variants rarely reported were identified, including 8 cases (5.5%) with c.34G→T(G12C), 7 cases (4.8%) with c.34G→A(G12S), 2 cases (1.4%) with c.35G→C(G12A) and 1 cases (0.7%) with c.34G→C(G12R). There were no statistical differences in clinical characteristics between the KRAS mutation group and no-mutation group. Conclusions In this study, KRAS gene mutation was found in 43.4% of BAVM patients, of which G12D was the most common mutation type of KRAS. In addition, several infrequent mutant types such as G12C and G12A were found. No relationship between KRAS mutations and clinical features were found.

A 13-year-old boy, with a history of intermittent headache and transient diplopia, was found to have non-hemorrhagic cerebral arteriovenous malformation in the midbrain tegmental region associated with hydrocephalus. Gamma knife radiosurgery was performed at 16 Gy with 75% marginal dose. Posttreatment course was uneventful. Follow-up MR imaging at one year after the treatment revealed complete disappearance of the abnormal vascular flow voids. The size of each ventricle at the treatment and at one year after treatment were as follows; 60.2 cc and 20.9 cc in the lateral ventricles, 3.7 cc and 2.7 cc in the third ventricle. The hydrocephalus might be caused by obstructive mechanism but mostly by high venous pressure due to the shunt blood flow. The goal of treatment for hydrocephalus should be nidus obstruction and normalizing the vascular flow.

The current treatment paradigm for intracranial arteriovenous malformations (AVMs) focuses on reducing the risk of intracranial hemorrhage using various therapeutic means including embolization, stereotactic radiosurgery (SRS), and microsurgical resection. To improve AVM obliteration rates with SRS, pre-radiosurgical embolization has been trialed in a number of studies to reduce the volume of the AVM nidus prior to radiosurgery. This study aimed to review the efficacy of pre-radiosurgical embolization in the pre-Onyx era compared to the current Onyx era. A systematic review was performed using PubMed to identify studies with 20 or more AVM patients, embolization material, and obliteration rates for both embolization + stereotactic radiosurgery (E+SRS) and SRS-only groups. Seventeen articles consisting of 1133 eligible patients were included in this study. A total of 914 (80.7%) patients underwent embolization prior to SRS. Onyx was used as the embolysate in 340 (37.2%) patients in the E+SRS cohorts. Mean obliteration rate for the embolized cohort was 46.9% versus 46.5% in the SRS-only cohort. When comparing obliteration rates based on embolysate material, obliteration rate was 42.1% with Onyx+SRS and 50.0% in the non-Onyx embolysate + SRS cohort. Onyx (ethylene vinyl-alcohol copolymer dissolved in dimethyl sulfoxide and suspended in micronized tantalum powder) has been increasingly used for the embolization of intracranial AVMs with increased success regarding its ease of use from a technical standpoint and performs similarly to other embolysate materials. [ABSTRACT FROM AUTHOR]

Background: As essential techniques, intraoperative indocyanine green video angiography (ICG-VA) and FLOW 800 have been widely used in microsurgery for arteriovenous malformations (AVMs). In the present report, we introduced a supplementary technical trick for judging the degree of lesion resection when there were superficial drainage veins. FLOW 800 analysis is used to verify our conjecture. Methods: A retrospective analysis of a 33 case cohort treated surgically from June 2020 to September 2022 was conducted and their lesions were removed by superficial drainage veins as a supplementary technical trick and analyzed with FLOW800. Results: In our 33 AVMs, the feeding artery was visualized earlier than the draining vein. Intraoperatively, the T1/2 peak and slope of the draining vein were significantly higher than that of the lesion. However, the maximum fluorescence intensity (MFI) of the draining vein decreased as the procedure progressed (p < 0.001). After reducing the blood flow to the nidus by progressive dissection of the feeding artery, the arteriovenous transit time (AVTT) decreased from 0.64 ± 0.47 s, was prolonged to 2.38 ± 0.52 (p < 0.001), and the MFI and slope of the nidus decreased from the pre-resection 435.42 ± 43.90 AI and 139.77 ± 27.55 AI/s, and decreased to 386.70 ± 48.17 AI and 116.12 ± 17.46 AI/s (p < 0.001). After resection of the nidus, the T1/2 peak of the draining vein increased from 21.42 ± 4.70 s, prolonged to after dissection of the blood feeding artery, 23.07 ± 5.29 s (p = 0.424), and after resection of the lesion, 25.13 ± 5.46 s (p = 0.016), with a slope from 135.79 ± 28.17 AI/s increased to 210.86 ± 59.67 AI/s (p < 0.001). Conclusions: ICG-VA integrated with FLOW 800 is an available method for determining the velocity of superficial drainage veins. Whether the color of the superficial drainage veins on the cortical surface returns to normal can determine whether the lesion is completely resected and can reduce the possibility of residual postoperative lesions. [ABSTRACT FROM AUTHOR]

Objective To explore the diagnosis and treatment of complex cerebral arteriovenous malformations by microsurgery. Methods The clinical data of 38 patients with complex cerebral arteriovenous malformations treated by simple microsurgery and combined surgery in the neurosurgery department of the First Affiliated Hospital of Soochow University from January 2011 to January 2021 were analyzed retrospectively, with 24 cases of grade Ⅲ, 9 cases of grade Ⅳ, and 5 cases of grade Ⅴ, according to Spetzler-Martin classification. The mean size of the malformed vascular mass was 5.9 cm (3.5-12.3 cm), located in the functional area in 27 cases and the non-functional area in 11 cases. CTA, DSA, and MRI were performed preoperatively, and CT, CTA or DSA were performed postoperatively. The neurological deficits were evaluated by the Glasgow prognostic score (GOS) at regular follow-up. Results In all cases, 28 underwent combined surgical treatment, and 10 underwent simple microsurgical resection. Postoperative imaging showed total resection in 35 (92.1%) and a significant reduction from a preoperative level in 3 (7.9%) . Postoperative follow-up was 6-79 months, with good recovery in 34 (GOS=4~5 points), moderate disability in 2 (GOS=3 points), severe disability in 1 (GOS=2 points), and death in 1 (GOS=1 point) . Conclusions Microsurgical resection is one of the effective methods for the treatment of complex cerebral arteriovenous malformations. The combination of preoperative imaging, preoperative supply artery embolization, intraoperative navigation, electrophysiological monitoring, and fine intraoperative microscopic manipulation is the key to successful surgery. [ABSTRACT FROM AUTHOR]

An understanding of the complex morphology of an arteriovenous malformation (AVM) is important for successful resection. We have previously reported the utility of intra-arterial indocyanine green (ICG) videoangiography for this purpose, but that method cannot detect the angioarchitecture covered by brain tissue. 3-dimensional (3D) multimodal fusion imaging is reportedly useful for this same purpose, but cannot always visualize the exact angioarchitecture due to poor source images and processing techniques. This study examined the results of utilizing both techniques in patients with AVMs. Both techniques were applied in 12 patients with AVMs. Both images were compared with surgical views and evaluated by surgeons. Although evaluations for identifying superficial feeders by ICG videoangiography were high in all cases, the more complicated the AVM, the lower the evaluation by 3D multimodal fusion imaging. Conversely, evaluation of the estimated range of the nidus was high in all cases by 3D multimodal fusion imaging, but low in all but one case by ICG videoangiography. Nidus flow reduction was recognized by Flow 800 analysis obtained after ICG videoangiography. These results showed that utilizing both techniques together was more useful than each modality alone in AVM surgery. This was particularly effective in identifying superficial feeders and estimating the range of the nidus. This technique is expected to offer an optimal tool for AVM surgery. [ABSTRACT FROM AUTHOR]

Background and Purpose: The performance of a novel prototype four‐dimensional (4D) digital subtraction angiography (DSA) for cerebral arteriovenous malformation (AVM) diagnosis was evaluated and compared with that of two‐dimensional (2D) and three‐dimensional (3D) DSA. Methods: In this retrospective study, 37 consecutive cerebral AVM patients were included. The standard diagnostic results were concluded from the 2D and 3D DSA. Two 4D DSA volumes were reconstructed for each patient by a commercial and a prototype software, then evaluated by two independent experienced neurosurgeons, who were blinded to the diagnosis and treatment process. The evaluation results were compared with the diagnostic results on Spetzler‐Martin (SM) Grading Scale, number of feeding arteries, number of draining veins, and intranidal aneurysms. Results: Complete agreement was achieved between 4D DSA and 2D and 3D DSA in SM Grading Scale and intracranial aneurysm identification (agreement coefficient: 1) for both reviewers. The agreement coefficients were.888 and.917 for both reviewers in feeding artery number determination using 4D DSA product and 4D DSA prototype, respectively. The agreement coefficients in draining vein number determination were all larger than.94 for both reviewers using both 4D DSA volumes. Conclusions: The performance of this prototype 4D DSA in cerebral AVMs diagnosis was largely equivalent to that of 2D and 3D DSA combination. Four‐dimensional DSA can be regarded as a very good complement for 2D DSA in cerebral AVM diagnosis. [ABSTRACT FROM AUTHOR]

Background: Visualization, analysis and characterization of the angioarchitecture of a brain arteriovenous malformation (bAVM) present crucial steps for understanding and management of these complex lesions. Three-dimensional (3D) segmentation and 3D visualization of bAVMs play hereby a significant role. We performed a systematic review regarding currently available 3D segmentation and visualization techniques for bAVMs. Methods: PubMed, Embase and Google Scholar were searched to identify studies reporting 3D segmentation techniques applied to bAVM characterization. Category of input scan, segmentation (automatic, semiautomatic, manual), time needed for segmentation and 3D visualization techniques were noted. Results: Thirty-three studies were included. Thirteen (39%) used MRI as baseline imaging modality, 9 used DSA (27%), and 7 used CT (21%). Segmentation through automatic algorithms was used in 20 (61%), semiautomatic segmentation in 6 (18%), and manual segmentation in 7 (21%) studies. Median automatic segmentation time was 10 min (IQR 33), semiautomatic 25 min (IQR 73). Manual segmentation time was reported in only one study, with the mean of 5–10 min. Thirty-two (97%) studies used screens to visualize the 3D segmentations outcomes and 1 (3%) study utilized a heads-up display (HUD). Integration with mixed reality was used in 4 studies (12%). Conclusions: A golden standard for 3D visualization of bAVMs does not exist. This review describes a tendency over time to base segmentation on algorithms trained with machine learning. Unsupervised fuzzy-based algorithms thereby stand out as potential preferred strategy. Continued efforts will be necessary to improve algorithms, integrate complete hemodynamic assessment and find innovative tools for tridimensional visualization. [ABSTRACT FROM AUTHOR]

Cases of low-grade cerebral arteriovenous malformations (cAVMs) showing dynamic changes and large areas of brain edema on short-term MRI follow-up have rarely been reported. This report describes an incidentally discovered and initially misdiagnosed cAVM in a patient with malignancies. The presence of abnormal signals surrounded by large areas of brain edema combined with tortuous or dilated vessels indicates the possibility of an AVM, especially in young people.

Endovascular embolization is a critical component in the treatment of cerebral arteriovenous malformations. It can be used as an independent treatment modality or as an adjunct to microurgery or radiosurgery. The published literature in the PubMed database until September 2021 was reviewed with reference to the results of cerebral arteriovenous malformations embolization using liquid embolic agents. More scientific data reporting about total embolization of the cerebral arteriovenous malformations with a final cure. Although complications and mortality after arteriovenous malformations embolization have decreased significantly, but they still exist, and decisions about it usage should be weighed against its benefits during the planning phase. Treatment of arteriovenous malformations of the brain requires a multidisciplinary approach involving vascular neurosurgeons, endovascular interventionists and radiation oncologists, with a deep understanding of the natural course and combination of risks of multimodal treatment. Only such approach can increase the likelihood of a positive outcome of the cerebral arteriovenous malformations treatment.

Objective: Intracranial haemorrhage (ICH) is associated with permanent neurological disability resulting in deterioration of the quality of life (QoL). Our study assesses QoL in patients with ruptured arteriovenous malformation (AVM) in long-term follow-up at least five years after ICH and compares their QoL with the QoL of patient with non-ruptured AVM. Methods: Using the Quality of Life Scale (QOLS), the Patient Health Questionnaire (PHQ-9) for depressive symptoms, and the socioeconomic status (SES), a prospective assessment was performed. The modified Rankin Scale (mRS) was assessed for outcome. Results: Of 73 patients, 42 (57.5%) had ruptured (group 1) and 31 (42.5%) a non-ruptured AVM (group 2). Mean follow-up time was 8.6 ± 3.9 years (8.5 ± 4.2 years in group 1 and 8.9 ± 3.7 years in group 2). Favourable outcome (mRS 0–1) was assessed in 60 (83.3%) and unfavourable in 12 (16.7%) patients. Thirty-one of 42 patients (73.8%) in group 1 and 29 of 30 patients in group 2 (96.7%) had favourable outcomes. Mean QOLS was 85.6 ± 14.1 (group 1 86.1 ± 15.9, group 2 84.9 ± 11.4). Patients in group 1 did not show a significant difference in QoL compared to patients in group 2 (p = 0.23). Additional analyses in group 2 (rho = − 0.73; p < 0.01) and in untreated AVM patients (rho = − 0.81; p < 0.01) showed a strong correlation between QOLS and PHQ-9. Conclusion: Long-term follow-up showed no difference in the QoL between patients with and without ICH caused by brain AVM. Outcome- and QoL-scores were high in both groups. Further studies are necessary to evaluate depression and anxiety symptoms in patients with AVM. [ABSTRACT FROM AUTHOR]

The primitive trigeminal artery (PTA), an abnormal carotid-basilar anastomosis, forms the vascular anomaly connection between the internal carotid artery and vertebrobasilar system. Rarely, PTA can be complicated by several other cerebrovascular disease, including arteriovenous malformations (AVMs), intracranial aneurysms, moyamoya disease, and carotid-cavernous malformations. Herein, we reported a rare case of PTA combined with an AVM in a male patient. The patient was a 28-year-old male with epileptic seizures at the onset of symptoms. Magnetic resonance imaging showed abnormal signal foci and localized softening foci formation with gliosis in the right parietal temporal lobe. Furthermore, using a digital subtraction angiogram (DSA), it was found that an abnormal carotid-basilar anastomosis had developed through a PTA originating from the cavernous portion of the right internal carotid artery (ICA) and a large AVM on the surface of the right carotid artery. The lesion of AVM tightly developed and draining into superior sagittal sinus. A hybrid operating room was used for the surgery. The main feeding arteries of the AVM originating from three major arteries, including the right middle cerebral artery, the right anterior cerebral artery, and the right posterior cerebral artery, were clipped and subsequently, then the AVM was thoroughly removed. The intraoperative DSA showed that the AVM had been resected completely. Postoperative pathological examination of the resected specimen indicated the presence of an AVM. The patient recovered well after surgery and has been symptom-free for more than 3 months. In summary, the pathogenesis of the coexistence of PTA and AVM remains unknown. As highlighted in this case report, hybrid surgery can be used to remove AVMs and can improve the patients' prognosis. To our best knowledge, this is the first case in the literature of successful AVM treatment using hybrid surgery.

Abstract Background Cerebral arteriovenous malformation (AVM) is a serious life-threatening congenital cerebrovascular disease. Specific anatomical features, such as nidus size, location, and venous drainage, have been validated to affect treatment outcomes. Until recently, molecular biomarkers and corresponding molecular mechanism related to anatomical features and treatment outcomes remain unknown. Methods RNA N6-methyladenosine (m6A) Methyltransferase METTL3 was identified as a differentially expressed gene in groups with different lesion sizes by analyzing the transcriptome sequencing (RNA-seq) data. Tube formation and wound healing assays were performed to investigate the effect of METTL3 on angiogenesis. In addition, Methylated RNA Immunoprecipitation Sequencing technology (MeRIP-seq) was performed to screen downstream targets of METTL3 in endothelial cells and to fully clarify the specific underlying molecular mechanisms affecting the phenotype of cerebral AVM. Results In the current study, we found that the expression level of METTL3 was reduced in the larger pathological tissues of cerebral AVMs. Moreover, knockdown of METTL3 significantly affected angiogenesis of the human endothelial cells. Mechanistically, down-regulation of METTL3 reduced the level of heterodimeric Notch E3 ubiquitin ligase formed by DTX1 and DTX3L, thereby continuously activating the Notch signaling pathway. Ultimately, the up-regulated downstream genes of Notch signaling pathway dramatically affected the angiogenesis of endothelial cells. In addition, we demonstrated that blocking Notch pathway with DAPT could restore the phenotype of METTL3 deficient endothelial cells. Conclusions Our findings revealed the mechanism by which m6A modification regulated the angiogenesis and might provide potential biomarkers to predict the outcome of treatment, as well as provide suitable pharmacological targets for preventing the formation and progression of cerebral AVM.

Yu-Chen Jia,1,* Jia-Yue Fu,2,* Ping Huang,3 Zhan-Pu Zhang,3 Bo Chao,3 Jie Bai3 1Inner Mongolia Key Laboratory of Molecular Biology, School of Basic Medical Sciences, Inner Mongolia Medical University, Hohhot, People’s Republic of China; 2Inner Mongolia Medical University, Hohhot, People’s Republic of China; 3Department of Neurosurgery, Affiliated Hospital, Inner Mongolia Medical University, Hohhot, People’s Republic of China*These authors contributed equally to this workCorrespondence: Jie BaiDepartment of Neurosurgery, Affiliated Hospital, Inner Mongolia Medical University, No. 1 Tongdao North Road, Hohhot City, Inner Mongolia 010000, People’s Republic of ChinaTel +86-471-6351352Fax +86-471-6351184Email baijie0611@126.comIntroduction: Cerebral arteriovenous malformation (cAVM) is a disease characterized by the angiogenesis and remodeling of veins. However, whether vascular endothelial cells (ECs) exhibit morphological and functional changes during cAVM remains unclear. This study aimed to investigate the role of ECs in the pathogenesis of cAVM.Methods: Rat model of cAVM was established by anastomosing the common carotid artery with the external jugular vein. The digital subtraction angiography (DSA), HE, Masson and immunohistochemical staining were performed to evaluate the model. ECs were isolated from AVM rat model or control rats, and characterized by MTT, cell scratch, and tube formation assays. The secretion of vascular endothelial growth factor (VEGF) was detected by ELISA.Results: AVM rat model showed typical pathological characteristics of cAVM. In addition, the proliferation, migration and tube formation abilities of ECs of arterialized vein (AV-ECs) were significantly better than those of ECs of normal vein (NV-ECs). Moreover, the levels of secreted VEGF were significantly higher in AV-ECs than in NV-ECs.Conclusion: AV-ECs isolated from AVM rat model showed increased proliferation, migration and angiogenesis and may be potential target for the treatment of cAVM.Keywords: cerebral arteriovenous malformation, endothelial cell, angiogenesis, VEGF

Background: Cerebral arteriovenous malformations (CAVMs) are either clinically silent or symptomatic. The most common presentation in more than half of all CAVMs presenting patients is hemorrhage which is accompanied by long-standing neurological morbidity and mortality. This report presents a case of an atypical large, slow-flow paramedian AVM with a dilated venous varix managed with surgery. The impact of the intraoperative findings on the diagnosis and the operative technique will be discussed. Case Description: In otherwise, healthy 26-year-old male complained of repeated episodes of generalized seizures and loss of consciousness. Brain magnetic resonance imaging (MRI) revealed a right parietal paramedian arteriovenous malformation (AVM) with signs of an old hemorrhagic cavity beneath it. Digital subtraction angiography demonstrated a slow-filling AVM with dilated venous varix drains into the superior sagittal sinus. However, the exact point of drainage cannot be appreciated. The filling of the AVM occurred precisely with the beginning of the venous phase. Intraoperatively, we noticed a whitish spherical mass, thick hemosiderin tissue, and a large cavity below the nidus; then, a complication-free complete microsurgical resection of this high-grade AVM was performed. Postoperatively, the patient suffered two attacks of seizures in the first few hours after the surgery, for which he received antiepileptics. MRI was clear during follow-up, and the patient was seizure-free and neurologically intact. Conclusion: Parietal convexity AVMs are challenging lesions to tackle. However, the chronicity and the slowfilling of the AVM, in this case, can render the surgical pathway more direct and accessible. [ABSTRACT FROM AUTHOR]

Cerebral arteriovenous malformations (cAVMs) represent tangles of abnormal vasculature without intervening capillaries. High-pressure vascular channels due to abnormal arterial and venous shunts can lead to rupture. Multiple pathways are involved in the pathobiology of cAVMs including inflammation and genetic factors such as KRAS mutations. Neutrophil release of nuclear chromatin, known as neutrophil extracellular traps (NETs), plays a multifunctional role in infection, inflammation, thrombosis, intracranial aneurysms, and tumor progression. However, the relationship between NETs and the pathobiology of cAVMs remains unknown. We tested whether NETs play a role in the pathobiology of cAVMs. We analyzed samples from patients who had undergone surgery for cAVM and immunohistochemically investigated expression of citrullinated histone H3 (CitH3) as a marker of NETs. CitH3 expression was compared among samples from cAVM patients, epilepsy patients, and normal human brain tissue. Expressions of thrombotic and inflammatory markers were also examined immunohistochemically in samples from cAVM patients. Expression of CitH3 derived from neutrophils was observed intravascularly in all cAVM samples but not other samples. Nidi of AVMs showed migration of many Iba-I-positive cells adjacent to the endothelium and endothelial COX2 expression, accompanied by expression of IL-6 and IL-8 in the endothelium and intravascular neutrophils. Unexpectedly, expression of CitH3 was not necessarily localized to the vascular wall and thrombus. Our results offer the first evidence of intravascular expression of NETs, which might be associated with vascular inflammation in cAVMs. [ABSTRACT FROM AUTHOR]

Background: Cerebral arteriovenous malformations (AVMs) are pathologic communications between veins and arteries of the brain vasculature. Its spontaneous regression is rare, and many factors have been described in the effort to explain this phenomenon, including a hypercoagulable state. Case Description: We present the case of a spontaneous unruptured AVM regression in a patient where thrombosis of the malformation was found, probably due to a prothrombotic state associated with multiple myeloma (MM). Conclusion: We aim to contribute to the study of this rare phenomenon, presenting the relationship between a hypercoagulable state caused by MM and the spontaneous AVM regression that has not been previously reported. [ABSTRACT FROM AUTHOR]

Acute hemorrhage caused by cerebral arteriovenous malformation (cAVM) during pregnancy is uncommon but life-threatening for both mother and fetus and presents a great challenge to clinical management. However, there is still no consensus on the treatment strategy and the treatment timing of acute hemorrhage from cAVM during pregnancy. The aim of this study was to amalgamate reported case series and our cases regarding the clinical management of pregnant patients under this special condition. We report a case series of 3 pregnant patients with acute hemorrhage caused by cAVM in our hospital. A systematic PubMed search of the English-language literature published between 1970 and 2020 was carried out. Clinical information including patients' age, gestational age, imaging studies, treatment strategy, treatment timing, delivery mode, and outcomes were collected and analyzed. The rebleed rate is about 7.1% and the mortality from rebleeding is up to 25%. Treatment modalities included radical surgery, endovascular embolization, radiosurgery/stereotactic radiosurgery, palliative surgery, and conservative treatment. There were no maternal deaths in either the intrapartum intervention group and the postpartum intervention subgroup of gestational age <34 weeks. A high rebleed rate and high mortality from rebleeding indicate that the intervention of ruptured cAVM should not be delayed. Intervention of ruptured cAVM within 2 weeks after initial hemorrhage is advisable in patients at gestational age <34 weeks, whereas termination of pregnancy as soon as possible followed by timely intervention of ruptured cAVM is practicable in patients at gestational age ≥34 weeks. [ABSTRACT FROM AUTHOR]

Objective To explore the clinical effect and experience of the interventional embolotherapy of cerebral arteriovenous malformations with Apollo detachable tip microcatheter.Methods The clinical data of 22 patients with cerebral arteriovenous malformation diagnosed by DSA were analyzed retrospectively. All the patients were treated with Apollo detachable tip microcatheter alone or with other microcatheter. Results Among the 22 patients,12 were cured,4 were assisted embolization before microsurgery,2 were targeted embolization of accompanying aneurysm with partial arteriovenous malformation embolization,and 4 were partial embolization. A total of 25 Apollo detachable tip microcatheters were used and extubated successfully. Tip detachment occurred in 17 Apollo detachable tip microcatheters,and 5 catheters were completely pulled out. There were no complications related to Apollo catheter during the operation. 17 cases were followed up clinically and all of them had favorable prognosis（ GOS score 4-5）,of which 12 cases were followed up by imaging（ DSA）, no recurrence was found in the cured or postembolization resection cases. Conclusions Apollo detachable tip microcatheter can solve the problem of catheter retain caused by the excessive reverse flow of liquid embolization materials,and reduce the risk of extubation. Use of Apollo detachable tip microcatheter can significantly shorten the operation time,and improve the cure rate of cerebral arteriovenous malformation embolization. [ABSTRACT FROM AUTHOR]

Background: Spontaneous regression of arteriovenous malformations (AVMs) is usually associated with bleeding, but sometimes asymptomatic regression occurs. Case description: We describe a case of spontaneous regression of AVM without evidence of hemorrhagic event. A 60-year-old otherwise healthy woman came to our hospital with complaints of headache. Digital subtraction angiography (DSA) showed an AVM with a 1.4 × 1.0 × 0.9 cm nidus in the right temporal lobe. The patient decided on observation without treatment rather than stereotactic radiation therapy or direct surgery. Seven years later, DSA revealed partial regression of the AVM. Venous ectasia and the nidus had decreased in size, and the nidus was obscure, especially around the feeding artery. No obvious features of hemorrhage were detected. Conclusions: Spontaneous regression of AVM is the result of multiple interacting factors. Structural changes to the AVM and histopathological changes may be important.

Background: Sporadic brain arteriovenous malformations (BAVM) are a major cause of hemorrhagic stroke in younger persons. Prior studies have reported contradictory results regarding the risk of hemorrhage during pregnancy, and there are no standard guidelines for the management of pregnant women who present with BAVM rupture. The purpose of this study is to describe maternal and fetal outcomes and treatment strategies in patients with BAVM hemorrhage during pregnancy. Methods: We performed a retrospective review of the University of California, San Francisco Brain AVM Project database for female patients who were pregnant at the time of BAVM hemorrhage between 2000 and 2017. Clinical and angiographic characteristics at presentation, BAVM treatment, and maternal outcomes using modified Rankin scale (mRS) score at presentation and 2-year follow-up were recorded. Fetal outcomes were abstracted from medical records and maternal reports. Results: Sixteen patients presented with BAVM hemorrhage during pregnancy, 81% (n = 13) of whom were in their second or third trimester. Three patients (19%) who were in their first trimester terminated or miscarried pregnancy prior to BAVM intervention. Of the remaining 13 patients, 77% (n = 10) received emergent BAVM treatment at time of hemorrhage prior to delivery, and 85% of patients achieved BAVM obliteration and good maternal outcomes (mRS 0–2) at 2-year follow-up. All patients had uncomplicated deliveries (69% cesarean and 23% vaginal) with no reports of postnatal cognitive or developmental delays in infants at 2-year follow-up. Conclusions: Our study shows good long-term maternal and fetal outcomes in ruptured BAVM patients presenting during pregnancy, the majority who received BAVM interventional treatment prior to delivery. [ABSTRACT FROM AUTHOR]

Background: Management after cerebral arteriovenous malformation (AVM) rupture aims toward preventing hemorrhagic expansion while maintaining cerebral perfusion to avoid secondary injury. We investigated associations of model-based indices of cerebral autoregulation (CA) and autonomic function (AF) with outcomes after pediatric cerebral AVM rupture. Methods: Multimodal neurologic monitoring data from the initial 3 days after cerebral AVM rupture were retrospectively analyzed in children (< 18 years). AF indices included standard deviation of heart rate (HRsd), root-mean-square of successive differences in heart rate (HRrmssd), low–high frequency ratio (LHF), and baroreflex sensitivity (BRS). CA indices include pressure reactivity index (PRx), wavelet pressure reactivity indices (wPRx and wPRx-thr), pulse amplitude index (PAx), and correlation coefficient between intracranial pressure pulse amplitude and cerebral perfusion pressure (RAC). Percent time of cerebral perfusion pressure (CPP) below lower limits of autoregulation (LLA) was also computed for each CA index. Primary outcomes were determined using Pediatric Glasgow Outcome Score Extended-Pediatrics (GOSE-PEDs) at 12 months and acquired epilepsy. Association of biomarkers with outcomes was investigated using linear regression, Wilcoxon signed-rank, or Chi-square. Results: Fourteen children were analyzed. Lower AF indices were associated with poor outcomes (BRS [p = 0.04], HRsd [p = 0.04], and HRrmssd [p = 0.00]; and acquired epilepsy (LHF [p = 0.027]). Higher CA indices were associated with poor outcomes (PRx [p = 0.00], wPRx [p = 0.00], and wPRx-thr [p = 0.01]), and acquired epilepsy (PRx [p = 0.02] and wPRx [p = 0.00]). Increased time below LLA was associated with poor outcome (percent time below LLA based on PRx [p = 0.00], PAx [p = 0.04], wPRx-thr [p = 0.03], and RAC [p = 0.01]; and acquired epilepsy (PRx [p = 0.00], PAx [p = 0.00], wPRx-thr [p = 0.03], and RAC [p = 0.01]). Conclusions: After pediatric cerebral AVM rupture, poor outcomes are associated with AF and CA when applying various neurophysiologic model-based indices. Prospective work is needed to assess these indices of CA and AF in clinical decision support. [ABSTRACT FROM AUTHOR]

Background: We proposed an algorithm to automate the components within the identification of components within the nidus of cerebral arteriovenous malformations (AVMs) which may be used to analyze the relationship between its diffuseness and treatment outcomes following stereotactic radiosurgery (SRS). Objectives: to determine the impact of the diffuseness of the AVM nidus on SRS outcomes. Methods: This study conducted regular follow-ups of 209 patients with unruptured AVMs who underwent SRS. The diffuseness of the AVM nidus was estimated by quantifying the proportions of vascular nidal component, brain parenchyma, and cerebrospinal fluid in T2-weighted MRIs. We used Cox regression analysis to characterize the association between nidal diffuseness and treatment outcomes in terms of obliteration rate and radiation-induced change (RICs) rate following SRS. Results: The median AVM volume was 20.7 cm3. The median duration of imaging follow-up was 51 months after SRS. The overall AVM obliteration rate was 68.4%. RICs were identified in 156 of the 209 patients (74.6%). The median proportions of the nidus of AVM and brain parenchyma components within the prescription isodose range were 30.2 and 52.2%, respectively. Cox regression multivariate analysis revealed that the only factor associated with AVM obliteration rate after SRS was AVM volume. However, a larger AVM volume (>20 mL) and a larger proportion of brain parenchyma (>50%) within the prescription isodose range were both correlated with a higher RIC rate following SRS. Conclusions: The diffuseness of the nidus indeed appears to affect the RIC rate following SRS in patients with unruptured AVMs. [ABSTRACT FROM AUTHOR]

Although carcinoma metastasis to primary intracranial neoplasms has occasionally been reported, metastasis to a cerebral arteriovenous malformation (AVM) has been exceedingly rare, with only 5 cases reported to date. In the present study, we have reported a case of lung carcinoma that had metastasized to a cerebral AVM. To the best of our knowledge, the present report is the first case in which the pathological examination detected the bleeding mechanism of this rare condition, showing destruction of the feeders by the metastatic tumor. A 61-year-old man who had had a tumor shadow in the right middle lung field identified at a medical examination 5 weeks previously had suddenly experienced a disturbance of consciousness. Head computed tomography and computed tomography angiography revealed a right occipital subcortical hemorrhage with abnormal vessels, suggesting a ruptured AVM. Magnetic resonance imaging with gadolinium-based contrast agents did not show any other lesions. Cerebral angiography revealed a Spetzler-Martin grade III AVM in the right occipital lobe. Endovascular feeder embolization and subsequent removal of the AVM were performed. Histopathological examination of the resected mass showed a small cell carcinoma that had metastasized to the AVM. The tumor cells had infiltrated to the vessel walls of the feeders, which might have elicited the bleeding. Although rare, clinicians should recognize that undifferentiated carcinomas can metastasize to AVMs and cause bleeding. Because the preoperative diagnosis can be difficult, even using the latest imaging modalities, careful examination of the resected specimen is required to reveal such pathological conditions. [ABSTRACT FROM AUTHOR]