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Purpose: The differentiation between conditions such as uterine arteriovenous malformation, pseudoaneurysm, gestational trophoblastic disease, and retained trophoblastic tissue can be challenging. Ultrasound imaging and Doppler interrogation are the primary diagnostic tools to assess cases of enhanced myometrial vascularity and differentiate intrauterine vascular anomalies. However, some cases remain of difficult differentiation. This study aims to analyze suspected cases and describe their diagnostic management and outcomes., Methods: We reviewed post-abortion cases that underwent pelvic transvaginal U/S imaging and Doppler examinations due to suspected uterine vascular anomalies. CT scans were performed in cases in which ultrasound did not reach a diagnosis. Simple follow-up, medical or surgical therapy, or embolization of uterine arteries were performed according to the final diagnosis., Results: From 2015 to 2022, we retrieved from electronic ultrasound records 22 cases of suspected vascular malformations. In eight cases, first-line U/S at admission excluded the suspected anomaly. In Five of the remaining 14 patients, uterine vascular anomalies were excluded upon a second-level  U/S based on angio-Doppler imaging and Doppler peak velocity interrogation. Nine cases underwent CT scan, and a digital angiography and embolization were performed in eight of these cases, of whom only two had a documented uterine arteriovenous malformation., Conclusion: Our triage proved that only two out of 22 suspected cases had a uterine arteriovenous malformation. This diagnosis is frequently misused in clinical practice. Our data confirm that enhanced myometrial vascularity should be used to encompass the spectrum of possible differential diagnosis. A precise step-by-step diagnostic method is of paramount importance to prevent unnecessary interventions., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Arteriovenous Malformations are complex and challenging entities, and their treatment is often tailored on the patient. Interstitial sclerosing treatment with bleomycin is promising especially in cases where the aesthetical and/or functional burden of surgical intervention would be impactful as in the S3 AVM according to the SECg classification. We treated 15 patients presenting small (<10cm3) S3 arterio-venous malformations with 3 + 3 sessions of 15.000 IU of interstitial bleomycin. 9 patients presented moderate to good response to the first 3 sessions and thus surgical re-shaping was performed between the two cycles. US examination was done pre-treatment, before each session and every 3 months after the end of the protocol to assess the arterio-venous malformations. Fifteen of the seventeen patients concluded the protocol. 9 patients underwent surgical re-shaping while 6 skipped it because of their excellent response to the first 3 bleomycin injections. Major improvement in terms of appearance and symptoms (pulsations, ulcerations, bleeding or pain) were always achieved. There was a 20% of minor complications. The presented data are encouraging and seem to suggest that this may be a promising protocol to extend the use of bleomycin in arterio-venous malformations and reduce the impact of surgery., (Copyright © 2024 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Background: Pulmonary arteriovenous malformation (PAVM) is abnormal arteriovenous shunts between pulmonary artery (PA) and pulmonary vein, and rarely has congenital direct communications with systemic arteries., Case Presentation: A 33-year-old male presented to our hospital with intermittent bloody sputum with no evidence of pulmonary infection, trauma or surgery. Chest computed tomography angiography (CTA) indicated the congenital inferior phrenic artery (IPA)-to-PAVM surrounded by diffuse alveolar hemorrhage located in the lower lobe of right lung. Both the afferent PA and IPA were successfully embolized with coils. Recurrent hemoptysis did not occur during one-year follow up., Conclusions: The congenital communication between IPA and PAVM is rare, and the abnormal direct shunt would induce hemodynamically unstable condition within PAVM. Endovascular embolization of the afferent PA and IPA is a safe and effective method for this abnormal congenital shunt in lung., (© 2024. The Author(s).)

Both arteriovenous malformation (AVM) and trisomy 21 are rare diseases. Studies have shown that individuals with trisomy 21 may have potential vascular malformations, with the main site of onset being the portal system. This case involved an adult patient with trisomy 21 who presented with pelvic iliac arteriovenous malformations. The patient underwent interventional treatment, and the prognosis was favorable. It is important to enhance the screening for vascular malformations in patients with trisomy 21 syndrome. At the same time, interventional treatment for AVMs is effective and increasingly important for managing AVMs., (© 2024. The Author(s).)

Objectives: To summarize the clinical characteristics and investigate the efficacy of ethanol embolotherapy in the treatment of chest well arteriovenous malformation (AVM). Treatment-associated complications were also explored., Materials and Methods: Between March 2017 and August 2021, 32 consecutive patients (mean age, 23.7 years; age range, 5-54 years) who underwent ethanol embolotherapy for chest well AVMs under general anesthesia were included in this study. Embolization was performed through a direct puncture, transarterial catheterization, or a combination of the 2 procedures. The mean follow-up duration after the last treatment was 18.0 months (range, 3-42 months). The degree of devascularization on follow-up (assessed using angiography or computed tomography), and the clinical signs and symptoms of AVMs were evaluated as the therapeutic outcomes. The major and minor complications associated with the procedures were recorded., Results: A total of 103 embolization procedures (mean, 3.2; range, 2-7) comprising 101 ethanol embolization and 2 coil embolizations were performed on 32 patients with chest wall AVMs. The AVM nidus was accessed through the transarterial approach alone in 4 patients, by direct puncture in 11, and a combined procedure in 17 patients. Overall, more than 80% of the procedures were performed using the combined approach. Complete AVM devascularization was achieved in 12 (37.5%) patients. Moreover, 76% to 99% AVM was achieved in 18 patients (56.3%), and 50% to 75% in 2 patients (6.3%). Bleeding, pain, heart failure, and cosmetic deformities were the indications for treatment. For 3 patients (3/32, 9.4%) who had bleeding, the treatment stopped the hemorrhage. Complete pain relief was reported in 8 patients (8/32, 25.0%), whereas complete relief from congestive heart failure post-embolization was observed in 5 of the 6 patients with congestive heart failure (5/6, 83.3%). Complete correction of cosmesis deformities after embolization was achieved in 10 patients (10/32, 31.3%). Two patients who underwent surgery to correct persistent deformity after embolization only showed insignificant improvement. In addition, 6 (18.8%) patients developed 13 complications including blister, necrosis, hemothorax, transient hemoglobinuria, and transient pulmonary artery hypertension., Conclusions: Ethanol embolotherapy is a safe and effective procedure for chest well AVMs. Surgery is required for some patients with residual cosmesis deformity., Clinical Impact: Currently, there is no standard treatment for chest well AVMs due to their rarity and high heterogeneity. The present study shows that thanol embolotherapy is a safe and clinically effective treatment procedure for the chest well AVMs. Transarterial embolization in combination with direct puncture embolization can reach the AVM nidus. Ethanol embolotherapy can achieve complete obliteration of the AVM nidus in the majority of patients. Surgery may still be needed to correct cosmetic deformity after embolization. The present study provides valuable evidence to inform clinical decision-making., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To evaluate effectiveness of pulmonary arteriovenous malformation (PAVM) embolization using dual-energy computed tomography (CT) and spectral curve analysis by characterizing contrast enhancement and vascular perfusion as a surrogate of the degree of vascular occlusion after embolotherapy., Materials and Methods: Nine consecutive adult patients underwent embolization for 21 PAVMs (size range, 0.4-2.0 cm; 15/21 simple angioarchitecture) and subsequent postembolization chest dual-energy CT angiography. Twelve PAVMs were treated with vascular plugs with or without coils, whereas 9 PAVMs were treated with coils alone. Virtual spectral curves were generated using dual-energy image postprocessing in order to measure embolization effectiveness., Results: Complete occlusion of target PAVM was achieved in all cases on digital subtraction angiography (DSA) at the end of the embolization procedure. With a median follow-up of 12.7 months, the vascular plug group demonstrated significantly less vascular opacification compared with the coils-only group, as measured by opacification between upstream feeding artery and different downstream vasculature locations (Δslope1: median 79.1 vs 28.6; P = .003; Δslope2: 76.4 vs 28.6; P = .0197; Δslope3: 78.9 vs 28.6; P = .004). Persistence occurred in 3 PAVMs based on size criteria, which demonstrated higher vascular opacification by dual-energy CT (Δslope1: 72 vs 28.6; P = .253; Δslope2: 65.1 vs 32.7; P = .326; Δslope3: 72.9 vs 53.5; P = .733), although statistical significance was not reached., Conclusions: Similar to emerging literature, dual-energy CT showed improved occlusion in PAVMs treated with vascular plugs compared with those treated with coils alone., (Copyright © 2024 SIR. Published by Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to compare ultra-low dose (ULD) and standard low-dose (SLD) chest computed tomography (CT) in terms of radiation exposure, image quality and diagnostic value for diagnosing pulmonary arteriovenous malformation (AVM) in patients with hereditary hemorrhagic telangiectasia (HHT)., Materials and Methods: In this prospective board-approved study consecutive patients with HHT referred to a reference center for screening and/or follow-up chest CT examination were prospectively included from December 2020 to January 2022. Patients underwent two consecutive non-contrast chest CTs without dose modulation (i.e., one ULD protocol [80 kVp or 100 kVp, CTDIvol of 0.3 mGy or 0.6 mGy] and one SLD protocol [140 kVp, CTDIvol of 1.3 mGy]). Objective image noises measured at the level of tracheal carina were compared between the two protocols. Overall image quality and diagnostic confidence were scored on a 4-point Likert scale (1 = insufficient to 4 = excellent). Sensitivity, specificity, positive predictive value and negative predictive value of ULD CT for diagnosing pulmonary AVM with a feeding artery of over 2 mm in diameter were calculated along with their 95% confidence intervals (CI) using SLD images as the standard of reference., Results: A total of 44 consecutive patients with HHT (31 women; mean age, 42 ± 16 [standard deviation (SD)] years; body mass index, 23.2 ± 4.5 [SD] kg/m 2 ) were included. Thirty-four pulmonary AVMs with a feeding artery of over 2 mm in diameter were found with SLD images versus 35 with ULD images. Sensitivity, specificity, predictive positive value, and predictive negative value of ULD CT for the diagnosis of PAVM were 100% (34/34; 95% CI: 90-100), 96% (18/19; 95% CI: 74-100), 97% (34/35; 95% CI: 85-100) and 100% (18/18; 95% CI: 81-100), respectively. A significant difference in diagnostic confidence scores was found between ULD (3.8 ± 0.4 [SD]) and SLD (3.9 ± 0.1 [SD]) CT images (P = 0.03). No differences in overall image quality scores were found between ULD CT examinations (3.9 ± 0.2 [SD]) and SLD (4 ± 0 [SD]) CT examinations (P = 0.77). Effective radiation dose decreased significantly by 78.8% with ULD protocol, with no significant differences in noise values between ULD CT images (16.7 ± 5.0 [SD] HU) and SLD images (17.7 ± 6.6 [SD] HU) (P = 0.07)., Conclusion: ULD chest CT provides 100% sensitivity and 96% specificity for the diagnosis of treatable pulmonary AVM with a feeding artery of over 2 mm in diameter, leading to a 78.8% dose-saving compared with a standard low-dose protocol., Competing Interests: Declaration of competing interest Hugo Lacombe is an employee of Philips Healthcare. The other authors have no competing interests to disclose in relation with this article., (Copyright © 2024 The Author(s). Published by Elsevier Masson SAS.. All rights reserved.)

Purpose: Patients with arteriovenous malformations (AVMs) have a lower health-related quality of life (QoL) than the general population. QoL assessment of patients with peripheral AVMs after endovascular treatment is scarce in the literature. Radiologic and clinical outcomes are not always correlated in vascular malformation treatment. This study aimed to investigate the relationship between clinical outcomes, QoL, and angiographic outcomes., Materials and Methods: Patients with peripheral AVM that underwent endovascular treatment between January 2009 and December 2021 in a single center were retrospectively evaluated. Patients' characteristics (age, sex), AVM characteristics (Schobinger classification, location, angiographic architecture), previous treatment, treatment characteristics (type of endovascular approach, embolizing agent and number of sessions), percentages of angiographic response, complications, and recurrence were evaluated. The angiographic architecture was evaluated according to the Yakes classification. The questionnaire was applied for evaluation of clinical response and QoL. Patients older than 12 years and those who can be contacted were included in clinical and QoL analysis. Clinical response was defined as improvement in the patient's most important pretreatment symptom. Treatment response was defined as clinical response plus >50% angiographic response., Results: Eighty-six patients (41 males [47.7%], 45 females [52.3%]) were included in angiographic analysis. The mean age was 28.44±12.99 years (range=5-61). Forty-three patients (50%) had previous treatment. The median number of sessions was 2 (range 1-15, InterQuartile Range [IOR]=2). Sixty-one patients (30 males [49.2%], 31 females [50.8%]) were included in clinical analysis. The clinical response rate was 73.8%, 95% confidence interval (CI) [0.60, 0.84]. The treatment response rate was 45.9%, 95% CI [0.33, 0.59]. The complication rate was 8.2%. Before treatment, 48 patients (78.7%) reported a negative impact on their QoL. Thirty-three of 48 patients (68.8%) reported improvement on their QoL after treatment. Higher Schobinger stages were related to a negative impact on QoL before treatment (p<0.01). Yakes types were not related to QoL (p=0.065). Clinical response was related to improvement on QoL after treatment (p<0.01). Angiographic and treatment responses were not related to improved QoL after treatment (p=0.52 and p=0.055, respectively)., Conclusion: Angiographic architecture and outcomes were not always reflected in QoL after endovascular treatment., Clinical Impact: This study's findings will help clinicians with what to focus on in AVM treatment and how to monitor patients with peripheral AVM after endovascular treatment. Rather than relying too much on the angiographic response, patients should be checked for symptoms and quality of life improvement. No clear data in the literature regarding the applicability of the Yakes Classification in patients with previous treatment. This study questioned the applicability of the Yakes Classification in patients with previous treatments. In this study, type 4 AVMs were more common in patients with previous treatment., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Right-to-left shunt (RLS) caused by patent foramen ovale (PFO) and pulmonary arteriovenous malformations (PAVM) have been associated with a variety of diseases, and reliable techniques for detecting RLS are essential for diagnosis. This study aimed to compare the diagnostic accuracy of multimodal ultrasound imaging, including transthoracic echocardiography (TTE) plus contrast transthoracic echocardiography (CTTE) and transesophageal echocardiography (TEE) plus contrast transesophageal echocardiography (CTEE) for PFO and PAVM in patients with cryptogenic stroke or migraine. This prospective study enrolled patients with cryptogenic stroke or migraine admitted to First Hospital of Shanxi Medical University between July 2018 and April 2023. The TTE + CTTE + TEE + CTEE multimodal ultrasound imaging was defined as the gold standard. A total of 230 patients with cryptogenic stroke (108) or migraine (122) were enrolled. The TEE + CTEE generated a better area under the receiver operator characteristic (ROC) curves (AUC) than TTE + CTTE [0.995 (0.988-1.000) vs 0.975 (0.767-0.984), P < .001], indicating better identification of PFO and PAVM. The sensitivity and specificity of the TTE + CTTE were 89.4% and 85.7%, respectively, whereas the sensitivity and specificity of TEE + CTEE were 99.1% and 100%, respectively. The missed diagnosis rate of TTE + CTTE and TEE + CTEE was 65.7 % and 12.5%, respectively. The combination of TEE + CTEE may be a more reliable and sensitive tool to detect PFO and PAVM than TTE + CTTE in patients with cryptogenic stroke or migraine., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

First described by Virchow in the 19th century, intracranial arteriovenous malformations (AVMs) are complex, tangle-shaped vascular lesions with a number of associated neuroparenchymal, hemodynamic, and angio-architectural changes. However, the clinical description of extracranial AVMs dates back to the Ebers Papyrus (c. 1500 BC), with a still unknown definitive underlying etiology thus far. AVMs are rare lesions, with approximately 0.15% incidence and 0.001-0.5% prevalence, but of high importance as they tend to affect young patients who are frequently otherwise healthy. In the majority of cases, AVMs present as sudden intracranial hemorrhages that require immediate intervention, but incidentally-detected unruptured AVMs are only found in ∼15% of cases, leaving a confusing dilemma regarding the appropriate next step, particularly given the several therapeutic interventions available and clinical trials that were vulnerable to follow-up criticism. Herein, we present a case of an incidentally detected asymptomatic AVM in a 15-year-old boy via advanced imaging techniques that was initially misinterpreted as a post-traumatic subarachnoid hemorrhage on routine imaging studies. In providing a comprehensive overview of pathological classification schemes and the currently available diagnostic options for these silent dilemmatic AVMs, we highlight three management techniques: microsurgical resection, endovascular embolization, and stereotactic surgery, with the best option depends mostly on addressing lesion resection properly with minimal associated mortality and morbidity., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Background: Interventional embolization schedules based on absolute ethanol are usually used for peripheral arteriovenous malformations (PAVMs), and clinicians often choose the scheme according to the classification., Aim: To evaluate different interventional embolization schedules based on absolute ethanol for PAVMs., Methods: A retrospective study was performed of 165 patients with PAVMs treated with interventional embolization based on absolute ethanol in Henan Provincial People's Hospital from January 2018 to May 2021. PAVMs were classified as type II ( n  = 67), type III ( n  = 81) and type IV ( n  = 17) according to the Yakes classification system, including 123 maxillofacial, 13 trunk and 29 limbs. Effectiveness of embolization was based on PAVM devascularization on angiography: 100% (total), 90%~99% (near-total), 70%~90% (substantial), 30%~70% (partial) and 0%~30% (failure)., Results: PAVMs were classified as type II ( n  = 67), type III ( n  = 81) and type IV ( n  = 17) according to the Yakes classification system, including 123 maxillofacial (74.55%), 13 trunk (7.88%) and 29 limbs (17.58%). There are statistical differences in the angiographic outcomes among different Yakes classification and between different methods ( P  < 0.05), and there was a statistical difference in the failure rates among different Yakes classification ( P  < 0.05)., Conclusions: PAVMs occur maxillofacial usually, and Type II can achieve better effect by spring coil and absolute ethanol, while Type III and Type IV have no ideal effect by Pingyangmycin + iodized oil + PVA + absolute ethanol and spring coil + absolute ethanol, respectively. Both the two happen to be complications, and wound accounts the highest.

Arteriovenous malformations (AVMs) in mesenteric vessels are exceptionally rare. These congenital vascular anomalies lead to direct vascular flow between the highly pressured arterial system and the low-pressure venous system. We describe the case of a patient with prior left colectomy for splenic flexure colonic adenocarcinoma presenting with persistent abdominal pain after developing multiple mesenteric thromboses. CT and colonoscopy showed left hemicolon congestion, anastomotic stenosis and mucosal oedema. Mesenteric angiogram revealed AVMs in the right colic and left colic arteries. Embolisation of the left colic AVM led to symptom resolution without recurrence at interval follow-up., Competing Interests: oCompeting interests: JCA has no actual or potential conflicts to disclose. ACB, research funding to the institution for clinical trials in coeliac disease from Chugai Pharmaceutical and Kanyos Bio (Anokion) unrelated to this case. BJG has no actual or potential conflicts to disclose., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

A boy in his mid-teens presented with progressively increasing bleeding from the right eye and nostril intermittently over a period of 6 weeks. A complete ophthalmic examination revealed nothing significant. His otorhinological examination and haematological investigations were within normal limits. The patient came a month later with frank bleeding from the right eye. Ophthalmic examination revealed hyperaemia and maceration of the right lower palpebral conjunctiva. A histopathological examination of conjunctival scrapings from the site showed abnormal dilated blood vessels suggestive of a vascular malformation. Digital subtraction angiography confirmed the presence of a conjunctival micro arteriovenous malformation supplied by the external carotid and ophthalmic artery branches. He underwent successful transarterial Onyx embolisation resulting in complete resolution of the haemolacria., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

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., (© 2024. The Author(s).)

Competing Interests: Competing interests: None declared.

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., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Purpose: To evaluate the correlation between pulmonary hypertension (PH) and recurrence of pulmonary arteriovenous malformation (PAVM) after embolization., Materials and Methods: With institutional review board (IRB) approval, the records of 377 patients with PAVMs evaluated at a single hereditary hemorrhagic telangiectasia (HHT) center of excellence between January 1, 2013, and September 10, 2023, were retrospectively reviewed. PAVMs embolized during this time period were evaluated for recurrence. Patients and PAVMs not treated during this time period were excluded. Growth of previously untreated PAVMs was not considered recurrence. Patients without chest computed tomography (CT) follow-up were excluded. General demographics, HHT status as defined by genetic testing or Curacao criteria, presence of PH, history of smoking, anemia, and hepatic arteriovenous malformations (AVMs) were documented. Odds ratio (OR) was calculated and stratified analysis was performed to assay the correlation between PAVM recurrence, PH, and possible confounders., Results: A total of 151 patients with PAVMs were treated during the study period, including 438 PAVMs, for which follow-up was available. This included 106 patients with definite, 31 with doubtful, and 14 with possible HHT. The presence of PH was significantly associated with PAVM recurrence both by patient (OR, 8.13; 95% CI, 3.50-19.67) and by lesion (OR, 4.07; 95% CI, 2.14-7.91). Multivariate analysis demonstrated that this correlation was independent of several variables including HHT status, smoking history, presence of hepatic AVMs, and anemia., Conclusions: There is a high correlation between PH and PAVM recurrence, suspected to be due to high pulmonary artery pressures causing recanalization. PH may suggest the need for shorter surveillance intervals., (Copyright © 2024 SIR. Published by Elsevier Inc. All rights reserved.)

A 42-year-old man was referred to our hospital because of anemia. The patient underwent gastroscopy and colonoscopy, but no bleeding site was detected. Abdominal contrast-enhanced computed tomography (CT) showed vascular dilatation along the wall of the small intestine. Small bowel capsule endoscopy and antegrade double-balloon endoscopy (DBE) were performed, and the patient was diagnosed with a small intestinal arteriovenous malformation (AVM). The AVM was clipped using DBE. After clipping, abdominal contrast-enhanced CT and small bowel angiography revealed the disappearance of the AVM. DBE may be a viable therapeutic option, helping avoid surgery and its associated risks.

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., (© 2024. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

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., (© 2024. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

KRAS gain-of-function mutations are frequently observed in sporadic arteriovenous malformations. The mechanisms underlying the progression of such KRAS -driven malformations are still incompletely understood, and no treatments for the condition are approved. Here, we show the effectiveness of sotorasib, a specific KRAS G12C inhibitor, in reducing the volume of vascular malformations and improving survival in two mouse models carrying a mosaic Kras G12C mutation. We then administered sotorasib to two adult patients with severe KRAS G12C-related arteriovenous malformations. Both patients had rapid reductions in symptoms and arteriovenous malformation size. Targeting KRAS G12C appears to be a promising therapeutic approach for patients with KRAS G12C-related vascular malformations. (Funded by the European Research Council and others.)., (Copyright © 2024 Massachusetts Medical Society.)

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., (© 2024. The Author(s).)

Hereditary haemorrhagic telangiectasia is an inherited disorder characterised by vascular dysplasia that leads to the development of arteriovenous malformations. Pulmonary arteriovenous malformations occur in approximately 30% of patients with haemorrhagic telangiectasia. Given the complex characteristics of haemorrhagic telangiectasia lesions, the application of three-dimensional fusion imaging holds significant promise for procedural guidance and decrease in contrast and radiation dosing. We reviewed all patients who underwent transcatheter approach for pulmonary arteriovenous malformation occlusion with fusion image guidance from June 2018 to September 2023 from a single centre. A total of nine cases with haemorrhagic telangiectasia and transcatheter occlusion of pulmonary arteriovenous malformations using fusion imaging were identified. Five (56%) were male, mean age at procedure was 15.7 years (10-28 years) and mean number of pulmonary arteriovenous malformations intervened was three per patient (1-7). Two of the cases were complex repeat embolisations. The mean fluoroscopy time was 40.6 min (10.7-68.8 min), with mean contrast dose of 28.8 mL (11-60 mL; mean of 0.51 mL/kg) and mean radiation dose of 66.3 mGy (25.6-140 mGy; mean of 40.5 mGy/m 2 ). There were no complications reported during the procedures, with no additional interventions necessary. Fusion imaging in pulmonary arteriovenous malformations embolisation for patients with haemorrhagic telangiectasia is feasible and has the potential to reduce contrast and radiation doses. To our knowledge, we describe the lowest radiation and contrast doses per patient using fusion imaging technology reported in the literature to date.

Purpose: To assess the different adjunctive catheter techniques required to achieve complete occlusion of renal arteriovenous malformations (rAVMs) of different angioarchitectural types., Materials and Methods: Overall, data on 18 patients with rAVM (Type 1, n = 7; Type 2, n = 2; Type 3, n = 9; mean age, 53.8 years) who underwent 25 procedures between 2011 and 2022 were reviewed. The clinical presentations, endovascular techniques, arteriovenous malformation (AVM) occlusion rate, adverse events (including the incidence of renal infarction), and clinical symptoms and outcomes (including recurrence/increase of AVM) were analyzed according to the Cho-Do angioarchitectural classification. Posttreatment renal infarction was classified as no infarction, small infarction (<12.5%), medium infarction (12.5%-25%), and large infarction (>25%) using contrast-enhanced computed tomography or magnetic resonance imaging., Results: Hematuria and heart failure were presenting symptoms in 10 and 2 patients, respectively. The embolic materials used were as follows: Type 1 rAVM, coils alone or with n-butyl-2-cyanoacrylate (nBCA); Type 2 rAVM, nBCA alone or with coils; and Type 3 rAVMs, nBCA alone. Fourteen patients underwent adjunctive catheter techniques, including flow control with a balloon catheter and multiple microcatheter placement, alone or in combination. Immediate postprocedural angiography revealed complete occlusion in 15 patients (83%) and marked regression of rAVM in 3 (17%). Small asymptomatic renal infarctions were observed in 6 patients with Type 3 rAVMs without any decrease in renal function. No major adverse events were observed. All symptomatic patients experienced symptom resolution. Recurrence/increase of rAVM was not observed during the mean 32-month follow-up period (range, 2-120 months)., Conclusions: Transarterial embolization using adjunctive catheter techniques according to angioarchitectural types can be an effective treatment for rAVMs., (Copyright © 2024 SIR. Published by Elsevier Inc. All rights reserved.)

Arteriovenous malformation (AVM) is an abnormal connection of vasculature resulting in capillary bed bypassing and leading to neurological deterioration and high risk of bleeding. Intramedullary AVMs in the cervical spinal cord are rare and require precise diagnostics and treatment. We present a clinical case of recurrent AVMs in a 28-year-old Caucasian female with sudden and severe neck pain and variable neurological symptoms along with current diagnostic and treatment modalities. Conservative treatment was partially effective. MRI and DSA confirmed AVMs at C4 level with subsequent several endovascular treatment sessions at the age of 15 and 24 with mild neurological improvement. Afterwards the patient underwent rehabilitation with minor neurological improvement. This case highlights the clinical progression and treatment of AVMs along with showcasing current pathophysiology, classification, and imaging.

Hereditary Hemorrhagic Telangiectasia (HHT), commonly known as Osler-Weber-Rendu disease, is an autosomal dominant multisystemic vascular disease associated with approximately 70% of cases of pulmonary arteriovenous malformations (PAVMs). Prenatal cases of PAVMs typically present with pulmonary vein dilatation on ultrasonography. This study presents a prenatal diagnosis of PAVMs with enlarged right pulmonary vein, cardiomegaly, cystic-appearing areas in the right lung and subsequent confirmation of Osler-Weber-Rendu syndrome using autopsy and whole exom sequencing., (© 2024 Wiley Periodicals LLC.)

Pulmonary arteriovenous malformations (PAVMs) occur in 30% to 50% of patients with hereditary hemorrhagic telangiectasia. Clinical presentations vary from asymptomatic disease to complications resulting from the right to left shunting of blood through the PAVM such as paradoxical stroke, brain abscesses, hypoxemia, and cardiac failure. Radiology plays an important role both in the diagnosis and treatment of PAVM. Based on different clinical scenarios, the appropriate imaging study has been reviewed and is presented in this document. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation., (Copyright © 2024 American College of Radiology. Published by Elsevier Inc. All rights reserved.)

Purpose: To evaluate by meta-analysis the safety and efficacy of venous sac embolization (VSE) with or without feeding artery embolization versus feeding artery embolization (FAE) alone in the management of pulmonary arteriovenous malformations (PAVMs)., Methods: This systematic review and meta-analysis was performed and reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A systematic literature search was performed in MEDLINE, Embase, and Scopus till August 31, 2021 to identify studies comparing the safety and efficacy of VSE with or without FAE versus FAE alone in PAVMs. The success of treatment was assessed by comparing the number of PAVMs with ≥70% decrease in the size of draining vein/sac between the 2 groups. The pooled odds ratio (OR) and 95% confidence interval (95% CI) were calculated using the random-effects inverse-variance model and were used to compare pooled therapeutic efficacy between the groups., Results: Three studies were found eligible for the meta-analysis. The included studies had a total of 169 patients (119 females and 50 males). The total number of PAVMs was 298, and hereditary hemorrhagic telangiectasia was present in 119 patients. The success of treatment was higher with VSE as compared to FAE (OR=3.54, 95% CI=1.66-7.56). The minor complications were similar in both groups, and one major complication occurred in the FAE group. More coils were used in the VSE group., Conclusions: The present systematic review and meta-analysis has consolidated the evidence that VSE with or without FAE is more efficacious than FAE alone in the management of PAVMs without any increase in the associated risk of sac rupture or coil migration., Clinical Impact: Until high-quality data from a prospective, multicentric, randomized controlled trial becomes available, the evidence consolidated by the present systematic review and meta-analysis showing the efficacy of venous sac embolization (with or without feeding artery embolization) over feeding artery embolization alone, can be used for clinical decision-making in the management of pulmonary arterio-venous malformations., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To evaluate the efficacy of the combined approach of preoperative endovascular embolization (EE) and surgical excision (SE) for scalp arteriovenous malformation (AVM) and present an illustrative case report., Methods: A systematic review was conducted using online databases (PubMed/Medline, Cochrane, and Embase) on February 15, 2023. The inclusion criteria were any type of study of patients with scalp AVMs who were diagnosed and confirmed through angiography and treated with combined preoperative EE and SE. All the articles that met the inclusion criteria were included in this study., Results: A total of 49 articles (91 patients) were included. The patients' age ranged from 10 days to 70 years at the time of presentation. The most common symptoms were a pulsatile mass in 51 patients (56.04%), progressively growing mass in 31 patients (34.06%), and bruits and/or thrills in 22 patients (24.17%). Complications of preoperative EE and SE were observed in only 5 patients; 3 patients (3.29%) had harvested skin graft marginal necrosis, 1 patient (1.09%) had skin necrosis, and 1 patient (1.09%) had a wound infection. Only 2 patients (2.19%) reported a recurrent or residual mass during a median follow-up period of 12 months., Conclusions: The management of scalp AVMs can be challenging; therefore, focused, and accurate identification of the complexity of the vascular anatomy is required. The combined method of preoperative EE and SE showed satisfactory outcomes with low rates of complications and recurrence; thus, we recommend this approach for the management of scalp AVMs., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Arteriovenous malformations of the head and neck are rare and inborn errors of vascular morphogenesis. They pose therapeutic challenges owing to their bleeding tendency and a high chance of recurrence. Treatment modalities include digital subtraction angiography with embolisation as a primary treatment or can be done preoperatively followed by surgical excision. We present a case of recurrent arteriovenous malformation of the postauricular region in an adolescent male who was taken up for upfront surgical excision with the help of a tumescent solution, thereby eliminating the need for embolisation and drastically reduced intraoperative blood loss., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

A previously healthy adult male presented with a slowly enlarging orbital mass associated with 5 mm of non-pulsatile proptosis. On imaging, a soft tissue lesion with avid contrast enhancement and associated bony hyperostosis was noted. The lesion and hyperostotic bone were surgically debulked, and significant arterial bleeding was noted intraoperatively consistent with an arteriovenous malformation. Histopathologic analysis revealed a vascular malformation with enhanced microvasculature infiltrating the periosteum. While vascular lesions elsewhere in the body can be associated with skeletal changes, bony hyperostosis is a rare feature of orbital vascular malformations.

Objective: Conus medullaris arteriovenous malformation (AVM) is rare and challenging to treat. To better define the presentation, prognosis, and optimal treatment of these lesions, the authors present their treatment experiences for conus medullaris AVM., Methods: Eleven patients with AVM of the conus medullaris were identified between March 2013 and December 2021. Among these patients, 7 who underwent microsurgical treatment were included. Patient data, including age, sex, symptoms at presentation, neurological status, radiological findings, nidus depth (mainly pial lesion vs intramedullary lesion), type of treatment, and recurrence at follow-up, were collected. Postoperative angiography was performed in all patients. Spinal cord function was evaluated using the Frankel grade at the time of admission and 1 year after surgery., Results: All 7 patients presenting with myeloradiculopathy were treated surgically. Four patients (57.1%) underwent endovascular embolization, followed by resection. The other 3 patients underwent microsurgery only. Complete occlusion was confirmed with postoperative angiography in all patients. Of the 3 patients who were nonambulatory before surgery (Frankel grade C), 2 were able to walk after surgery (Frankel grade D) and 1 remained nonambulatory (Frankel grade C) at 1-year follow-up., Conclusions: Based on the authors' clinical experiences, the results of multimodal treatment for conus medullaris AVM are good, with microsurgical treatment playing an important role. The microsurgical strategy can differ depending on the location of the nidus, and when possible, good results can be expected through microsurgical resection.

Purpose: To evaluate the safety, technical success and short-term effectiveness of polyurethane-covered stent (PK Papyrus, BioTronik, Berlin) in the treatment of pulmonary arteriovenous malformations (PAVMs) that are not amenable to embolotherapy., Materials and Methods: In this IRB-approved, retrospective study, data from patients who received polyurethane-covered stents for exclusion of PAVMs were analyzed. The study included 5 patients (all women) with a median age of 40 years (range 25-60). Patients presented with hypoxemia, TIAs, and/or epistaxis; 4 were confirmed to have HHT. All had multiple PAVMs diagnosed on chest CT and underwent embolization with other devices in addition to the polyurethane-covered stent. The indication for stent placement in all cases was a short and/or tortuous feeding artery. Safety was assessed by immediate or short-term complications, e.g., migration, stent thrombosis, and fracture. Technical success was defined as the ability to accurately place the stent at the intended location. Effectiveness was defined as successful exclusion of PAVM with no perfusion across the AVM., Results: Technical success of stent placement was 100%. AVM exclusion rate was 80% after single stent deployment; in the case of incomplete exclusion, success was achieved using an overlapping stent to completely cover a second feeding artery. During the median follow-up period of 5 months (range 2-10), all stents remained patent, and AVMs were excluded without other complications., Conclusion: Exclusion of PAVMs with polyurethane-covered stents is technically feasible, safe, and shows short-term effectiveness for PAVMs with a short/tortuous feeding artery when traditional embolization techniques are not possible., (© 2024. Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE).)

Purpose: To compare the safety, effectiveness, and persistence rates of 0.018-inch coils with those of Amplatzer vascular plugs (AVPs; Abbott Vascular, Abbott Park, Illinois) for the treatment of pulmonary arteriovenous malformations (PAVMs) in response to a growing concern that 0.018-inch coil embolization would increase the long-term persistence rate., Materials and Methods: This is a retrospective, single-center study of a database (2002-2020) of 633 PAVM embolizations. Complex PAVMs and those not embolized with 0.018-inch coils or plugs were excluded. PAVM embolization material was classified into 4 groups: (a) 0.018-inch nonfibered coils (NFCs), (b) 0.018-inch fibered coils (FCs), (c) NFCs and FCs, or (d) plugs. Persistence was defined as flow through the PAVM on digital subtraction angiography (DSA) or as <30% diameter reduction of the aneurysmal sac on unenhanced computed tomography (CT). Kaplan-Meier analysis and Cox regression were used to assess PAVM's persistence-free survival., Results: A total of 312 PAVM embolizations with NFCs (43 PAVMs), FCs (127 PAVMs), NFCs and FCs (12 PAVMs), or plugs (130 PAVMs) in 109 patients (28% men; mean age = 49 years) were included. All PAVM embolizations were technically successful without any major adverse events. PAVM persistence-free survival rates at 10 years' follow-up were 40.8% versus 44.7% in the NFC and FC groups (P = .22) and 47.3% versus 81.0% in the 0.018-inch coil (NFC or FC) and plug groups (P < .0001), respectively. There were 0.43 (79/182) and 0.08 (10/130) re-embolization procedures per PAVM in the 0.018-inch coil and plug groups, respectively (P < .001)., Conclusions: PAVM embolization with 0.018-inch coils was safe, but persistence rate with PAVM embolization was significantly higher than that with plugs, with no significant differences between FCs and NFCs., (Copyright © 2023 SIR. Published by Elsevier Inc. All rights reserved.)

Abstract: We obtained breath-hold zero TE (ZTE) magnetic resonance imaging for the evaluation of pulmonary arteriovenous malformations before and after embolotherapy. To the best of our knowledge, there have been no reports of ZTE for the entire lung imaging in single breath-hold scan time such as 20 seconds. Breath-hold ZTE magnetic resonance imaging can be a useful technique for magnetic resonance-based follow-up of vascular lung diseases without using contrast media, reducing the undesired artifacts from metallic devices., Competing Interests: Conflicts of Interest and Source of Funding: The authors have no conflicts of interest to declare that are relevant to the content of this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)