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Objective Intra-procedural WEB device migration is a scarcely reported complication that necessitates prompt intervention. Case Presentation Endovascular treatment of two broad necked flow-related aneurysms was planned aided by WEB-SL (Woven EndoBridge-single layer) devices in a 71-year-old female with known left temporal arteriovenous malformation. Inadvertent distal migration occurred while performing a control angiogram with an automated iodine injector. Immediate retrieval was successfully performed using a Solitaire stent-retriever. Conclusion To our knowledge, we report for the first time the successful retrieval of a distally migrated WEB using a stent-retriever device.

Background: A variety of emerging medical imaging technologies based on artificial intelligence have been widely applied in many diseases, but they are still limitedly used in the cerebrovascular field even though the diseases can lead to catastrophic consequences. Objective: This work aims to discuss the current challenges and future directions of artificial intelligence technology in cerebrovascular diseases through reviewing the existing literature related to applications in terms of computer-aided detection, prediction and treatment of cerebrovascular diseases. Methods: Based on artificial intelligence applications in four representative cerebrovascular diseases including intracranial aneurysm, arteriovenous malformation, arteriosclerosis and moyamoya disease, this paper systematically reviews studies published between 2006 and 2021 in five databases: National Center for Biotechnology Information, Elsevier Science Direct, IEEE Xplore Digital Library, Web of Science and Springer Link. And three refinement steps were further conducted after identifying relevant literature from these databases. Results: For the popular research topic, most of the included publications involved computer-aided detection and prediction of aneurysms, while studies about arteriovenous malformation, arteriosclerosis and moyamoya disease showed an upward trend in recent years. Both conventional machine learning and deep learning algorithms were utilized in these publications, but machine learning techniques accounted for a larger proportion. Conclusion: Algorithms related to artificial intelligence, especially deep learning, are promising tools for medical imaging analysis and will enhance the performance of computer-aided detection, prediction and treatment of cerebrovascular diseases.

Background: Language dysfunction is rarely seen in patients with unruptured brain arteriovenous malformation (AVM) albeit the AVM nidus involving language areas, which provides a unique disease model to study language reorganization. The objective of this study was to investigate the impairment and reorganization patterns and characteristics of language-related white matter in AVMs located at different brain areas. Methods: Thirty-three patients with AVMs involving language areas were prospectively enrolled. Patients were categorized into 3 groups according to the lesion locations: the frontal (14 patients), temporal (15 patients), and parietal groups (4 patients). Thirty age- and sex-matched healthy controls were enrolled as comparison. All participants underwent diffusion tensor imaging scans, and automated fiber quantification method was applied to quantitatively study the difference of segmented language-related white matter connectivity between 3 AVM groups and control group. Results: Language functions were normal in all subjects according to Western Aphasia Battery test. In the frontal group, fractional anisotropy (FA) value decreased in the left arcuate fascicle and increased in left superior longitudinal fasciculus and uncinate fascicle; in the temporal group, FA values decreased in left inferior fronto-occipital fascicle and inferior longitudinal fascicle and increased in right anterior thalamic radiation and uncinate fascicle; in the parietal group, FA values decreased in left arcuate fascicle and inferior longitudinal fascicle and increased in bilateral anterior thalamic radiations and uncinate fascicles and right inferior fronto-occipital fascicle. In fascicles with decreased FA values, the increase of radial diffusivity was common, and fascicles with increased FA values usually presented along with increased axial diffusivity values. Conclusions: Remodeling of language-related white matter occurs when traditional language areas are involved by AVM nidus, and its reorganization patterns vary with locations of AVM nidus. Fascicle impairment is mainly caused by the myelin deficits, and its plasticity may be dominated by the axon remodeling procedure.

Maxillomandibular or dental arcade arteriovenous malformations (AVMs) are uncommon lesions with potential for life-threatening bleeding. We report three cases of vascular malformations and a case of pyogenic granuloma and propose a contrast-enhanced computed tomography (CT)-based workup of these lesions for definitive treatment. Four cases of orodental bleeding were diagnosed based on CT angiography (CTA) and treated using endovascular means. Three of them were subsequently operated and confirmed by histopathology. Two were low-flow AVMs, the third one a case of pyogenic granuloma, and one high-flow AVM. The first three lesions were embolized with polyvinyl alcohol particles and subsequently operated and the fourth high-flow one embolized using liquid embolic agent n-butyl cyanoacrylate (Glue). CTA can be used to show separate lesions of arterial vascularity and those of delayed/ venous enhancing lesions. Arterial phase-enhancing lesions are again subdivided in digital subtraction angiography into those of low-to-moderate vascularity and those with high flow, which are treated using particle embolization and liquid glue/onyx (± coil) embolization, respectively. The delayed venous phase enhancing lesions may mimic neoplasms and after tissue biopsy may be managed by sclerotherapy.

Cobb Syndrome (Spinal Arteriovenous Metameric Syndrome 1-31 (SAMS 1-31)) is a rare, non-hereditary disorder. Approximately 100 cases of CS have been described to date. The actual incidence may be much higher since only symptomatic patients were documented. In particular, post mortem studies suggest a possibly higher incidence of this syndrome. The main clinical manifestations of this syndrome include skin stains of vascular nature on the torso, in combination with spinal vascular malformations localized in one and the same metameric or spinal segment. A rare diagnosis of this syndrome in patients over 18 is probably related to the fact that the disease may be asymptomatic throughout a long period of time, while patients may tend to disregard the skin lesions. As a result, most publications on this pathology are based on separate case reports. : Significant variability of clinical manifestations as well as prolonged progress of the disease often cause errors in diagnosis. What follows is a case report of a young patient with Cobb Syndrome, who was admitted to a regional vascular centre with a misdiagnosis of stroke. : 20 patients of young age (from 20 to 35 years old), with a diagnosis of stroke, who were admitted to a University Clinic (of the Russian National Research Medical University Named After Pirogov N.I., Moscow). Among this group of patients, a patient with Cobb syndrome was identified. Patient P., of 22 years, presented with acute, intensive cervical spinal pain, predominantly on the right, numbness and weakness in the arms and legs. About 3 weeks before admission to the hospital, the patient had ARVI with a fever of up to 37.5°C: two weeks before the onset of symptoms, he had undergone extirpation of 2 teeth, for which reason he spent over 2 hours in a forced position with his head thrown back (prolonged overextension in the cervical spine). Multiple skin angiomas on the chest spreading to the shoulder and scapula region. Tetraparesis up to 4 points: tetraparesis in hands with low muscle tone, low reflexes, tetraparesis in legs with high muscle tone, high reflexes, foot clonus when causing Achilles reflexes, tremor in the extremities and no plantar reflex pathology were detected, sensitivity disorders in the hands “the high gloves” and no pelvic disorders were detected. Given the presence and exacerbation of neurological symptoms and cutaneous angiomas, MRI with a contrast agent of the cervical spine was recommended. MR-image of an advanced arteriovenous malformation (AVM) of the cervical spinal cord with signs of gliosis and spinal cord oedema at the C2-C7 level. Endovascular embolization of the AVM in the cervical spinal cord was performed. The treatment led to the complete reversal of neurological symptoms. In the presence of skin lesions, the diagnosis of CS does not present particular difficulties, so in children and young patients with skin angiomatosis, it is advisable to conduct a comprehensive examination using selective spinal angiography or MR angiography to exclude arteriovenous malformations in the spinal cord.

Objective To analyze a case of heavy uterine bleeding in adolescence caused by an arteriovenous malformation (AVM) in Hanoi Obstetrics and Gynecology Hospital, Vietnam. Methods We observed a case of a 14-year-old girl, without vaginal sexual intercourse experience, having heavy uterine bleeding caused by AVM. She underwent a laparoscopic operation at the hospital for vascular lesions in the uterine anterior wall, which caused an internal hemorrhage of 1500 ml blood loss into the peritoneal cavity. Three years later, this patient was hospitalized twice for massive vaginal bleeding. Results Results of ultrasound and magnetic resonance imaging indicated a uterine intramural mass with enlarged vessels connecting to the endometrial cavity. A pelvic digital subtraction angiography was performed and showed profuse bleeding from a ruptured branch of the left uterine artery. This artery was embolized at Bach Mai hospital and the bleeding was stopped. The patient had stable health and normal menstrual periods after 4 months of follow up. Conclusion Abnormal mass with dilated vessels in the myometrium in a patient experiencing heavy uterine bleeding is an exclusive sign of uterine vascular malformation and can be treated by angioembolization.

Hereditary haemorrhagic telangiectasia is a rare, genetic disorder that can present at any age. It is characterised by epistaxis, mucocutaneous telangiectasia and visceral arteriovenous malformations, which can affect multiple organs. Early diagnosis and management reduces the morbidity and mortality associated with the disease.There is a well-established hereditary haemorrhagic telangiectasia clinic in London, and excellent links across Europe via the European Reference Network. However, local coordinated care for patients with hereditary haemorrhagic telangiectasia across the UK can be variable and often absent for children and young people. Some patients travel long distances to receive care in London, while others are referred to local clinicians or lost to follow up entirely.This article presents the experience to date from two regional UK centres (Liverpool and Dundee) where care for patients with hereditary haemorrhagic telangiectasia is being coordinated and streamlined. While there is still a lot to learn, this article highlights some of the successes and challenges identified so far, with suggestions for how these could be addressed.Collaborative regional networks such as these can facilitate the sharing of best practice and ensure that all patients with hereditary haemorrhagic telangiectasia are able to access safe, high-quality care.

Spinal epidural arteriovenous fistulas (SEDAVFs) are rare spinal vascular malformations that are difficult to diagnose and treat. SEDAVFs can be asymptomatic; however, symptoms can arise from the compression of adjacent nerve roots by dilated vein and perimedullary venous reflux, caused by shunting into the epidural venous plexus. A 31-year-old male presented to our institution with a 2-year history of progressively worsening low-back pain, radiating thigh pain, and sensory changes in his lower extremities. MRI and CT angiography demonstrated dilated epidural vascular lesion compressing the nerve root. The SEDAVF was embolized with multiple coils, which alleviated the nerve root compression from the engorged venous varix and improved the patient’s radiculopathy. Our experience from this case shows that endovascular coil embolization using the transarterial approach can be an effective treatment for SEDAVF and an alternative to surgical ligations.

Rationale: Brain arteriovenous malformations (bAVMs) are abnormal entanglement of blood vessels in brain, with direct connections from arteries to veins, lacking functional capillary bed. Although several somatic mutations were reported, the molecular mechanism and genetic disposition of bAVM remain poorly understood. Objective: We aim to identify transcriptional anomalies and critical functional pathways in bAVM lesions and explore their association with key de novo germline and somatic variants in bAVM patients. Methods and Results: We established a comprehensive bAVM dataset from 269 patients, by performing single-cell sequencing of 17 bAVM lesions, whole-exome sequencing of germline DNA from 60 case-unaffected-parental trios, and genomic/transcriptomic sequencing of 231 bAVM lesions. We found abnormal expression of endothelial and mesenchymal markers in bAVM at both bulk and single-cell level, which was validated by flow cytometric analysis and immunofluorescence staining, suggesting an involvement of endothelial-to-mesenchymal transition (EndMT) process in AVM (arteriovenous-malformation). Using data from the 60 trios, we identified nonsynonymous de novo germline mutations affecting 46 genes, including EXPH5 (detected in 2 independent cases), and vessel-related genes, such as EPAS1 and ENG . Interestingly, knockdown of epas1 in zebrafish embryo showed AVM-like phenotype exclusively in brain. Subsequent computational and experimental analyses demonstrated that expression of genes affected by de novo germline mutations was enriched in vascular cell types and was involved in EndMT-relevant behaviors including cell migration, angiogenesis, and cell marker transition. Moreover, we detected somatic KRAS mutations in 129 of 179 (72%) cases and showed that KRAS mutations were associated with bleeding as the first symptom ( P =0.0072). Following experimental studies demonstrated that KRAS mutations independently regulated EndMT features, consolidating the involvement of EndMT in this disease. Lastly, we showed that lovastatin reversed EndMT features in vitro and ex vivo. Conclusions: Our results suggest the convergent role of de novo germline mutations and somatic mutations in regulating EndMT in bAVM and provided a potential therapeutic option.

Sciatic nerve symptomatology may arise from both intra- and extra-neural pathology, at any point along descent from the sacral plexus to its bifurcation. The potential aetiology is broad, ranging from degenerative spinal disease to muscle, bony and vascular pathology. We present an extremely unusual case of position and exercise-induced nerve compression secondary to arteriovenous malformation and review the potential extraspinal causes, many of which may be ameliorated by surgical excision or decompression. We further discuss the usefulness of diagnostic imaging, specific clinical tests and histopathological tools that may aid in management.

Background The aim of the present study is to evaluate the short-term efficacy and feasibility of radiofrequency ablation in the treatment of complex diffuse arteriovenous (AV) malformations. Methods The data of 18 patients (8 male and 10 female) with complex AV malformations treated between December 2014 and June 2019 were analyzed retrospectively. The lesion area was 10 × 7 cm ~ 28 × 30 cm. Under duplex ultrasound guidance, the site with the most abundant blood flow signals in the lesion was percutaneously punctured with the radiofrequency ablation needle (electrode). The impedance automatic adjustment mode was adopted, and ablation was monitored usingduplex ultrasoundduring the entire process. Results Of the included patients, 1 had a high fever after two rounds of treatment, 2 had transient hemoglobinuria, and 1 had tissue necrosis in the original ruptured tumor area as well as a penetrating defect in the cheek, which was repaired with a pedicled trapezius myocutaneous flap. In 9 patients who experienced bleeding, the bleeding stopped after one round of treatment. During the follow-up period of 1–5 years, there were 0 grade I (poor) cases, 0 grade II (medium) cases, 7 grade III (good) cases, and 11 grade IV (excellent) cases. Conclusion The “high power and continuous” radiofrequency ablation technique conducted under real-time duplex ultrasoundmonitoring can completely destroy the deep core lesions of AV malformations and effectively control life-threatening massive hemorrhage; it is an effective alternative treatment method for complex diffuse AV malformations in which interventional embolization, sclerotherapy, and surgery are ineffective.

BACKGROUND: It is crucial to identify a shunt point for spinal arteriovenous malformation (AVM) treatment. For this purpose, some intraoperative supports have been reported & mdash;intravenous injection of indocyanine green (ICG), selective arterial injection of ICG, and selective arterial injection of saline with a high frame rate digital camera. However, there are difficulties in accurately identifying the shunt point, especially if the lesion has multiple feeders. The aim of this technical note was to report a novel method, selective arterial injection of saline to subtract signals of ICG, to precisely identify perimedullary arteriovenous fistula shunt points having multiple feeding arteries. METHODS: After exposing the lesion, a 4-F catheter was cannulated into the origins of the segmental artery. ICG was injected intravenously as a first step, and then heparinized saline solution was flushed from the catheter. RESULTS: Compared with other methods, this method could identify the exact shunt point and was effective for certain shunt point obliterations. CONCLUSIONS: Despite having similar invasiveness, selective arterial injection of saline to subtract signals of ICG is superior to previously described techniques, such as selective arterial injection of ICG. Therefore, it will be useful in spinal arteriovenous malformation surgical treatment.

Objectives To evaluate whether the signal intensity ratio (rSI) of the draining vein on silent MR angiography is correlated with arteriovenous (A–V) transit time on digital subtraction angiography (DSA), thereby identifying high-flow A–V shunt in brain arteriovenous malformation (BAVM), and to analyze whether the rSI and the characteristic of draining veins on silent MRA are associated with hemorrhage presentation. Methods Eighty-one draining veins of 46 participants with BAVM (mean age 33.2 ± 16.9 years) who underwent silent MRA and DSA were evaluated retrospectively. The correlation between the rSI of the draining vein on silent MRA and A–V transit time on DSA was examined. The AUC-ROC was obtained to evaluate the performance of the rSI in determining the presence of high-flow A–V shunt. The characteristics of draining veins with the maximum rSI (rSImax) were further compared between the hemorrhagic and non-hemorrhagic untreated BAVM. Results The rSI of each draining vein on silent MRA was significantly correlated with A–V transit time from DSA (r = −0.81, p < .001). The AUC-ROC was 0.89 for using the rSI to determine the presence of high-flow A–V shunt. A cut-off rSI value of 1.09 yielded a sensitivity of 82.4% and a specificity of 82.8%. The draining vein with rSImax and no ectasia was significantly more observed in the hemorrhagic group (p = 0.045). Conclusions The rSI of the draining vein on silent MRA is significantly correlated with A–V transit time on DSA, and it can be used as an indicator of high-flow A–V shunt in BAVM. Key Points • The signal intensity ratio (rSI) of the draining vein on silent MRA significantly correlated with arteriovenous (A–V) transit time of brain arteriovenous malformation (BAVM) on digital subtraction angiography (DSA). • The area under the receiver operating characteristic curve (AUC) was 0.89 for using the rSI of draining veins to determine high-flow A–V shunt. • Draining veins with maximum rSI and no ectasia were significantly more observed in the hemorrhagic group of BAVM (p = 0.045).

Head and neck arteriovenous malformations are the commonest extracranial vascular malformations but demonstrate a unique challenge in the limited available surgical options secondary to their intimate association to vital structures. We present a case of middle-aged female patient who presented with threatened upper-airway obstruction and bleeding secondary to a slowly enlarging parapharyngeal arteriovenous malformations. She was treated with an endovascular-only approach with the proximal arteriole branches selectively undergoing embolo-sclerotherapy with an optimal radiological and clinical outcome. We also demonstrate the utility of elective tracheostomy prior to intervention.

The purpose of this review is to raise the index of suspicion for paradoxical embolism among generalists. The review is based solely on anecdotal reports compiled from EMBASE, MEDLINE, Googlescholar and Pubmed. Search terms were ‘paradoxical embolism’, ‘pulmonary embolism’ and ‘pulmonary arteriovenous malformations’. What emerged was that right-to-left paradoxical embolism could occur with or without concurrent pulmonary embolism, and also with and without proof of the presence of an ‘embolus-in-transit’. Potential sites of single or multiple systemic involvement included the central nervous system, the coronary circulation, renal arterial circulation, splenic circulation, the mesenteric circulation and the limbs. In many cases, the deep veins of the lower limbs were the source of thromboembolism. In other cases, thrombi originated from an atrial septal aneurysm, from a central venous line, from a haemodialysis-related arterio-venous shunt, from a popliteal vein aneurysm, internal jugular vein, superior vena cava, from a pulmonary arteriovenous malformation, from tricuspid valve endocarditis (with and without pulmonary embolism) and from the right atrium, respectively. Stroke was by far the commonest systemic manifestation of paradoxical embolism. Some strokes were attributable to pulmonary arteriovenous malformations with or without coexistence of intracardiac shunts. Clinicians should have a high index of suspicion for paradoxical embolism because of its time-sensitive dimension when it occurs in the context of involvement of the intracranial circulation, coronary circulation, mesenteric circulation, and peripheral limb circulation.

Arteriovenous malformation (AVM) results from development of abnormal connections between veins and arteries. This study reported anAVM case suffering from dysgraphia and dysprosody. According to the results after the trauma, the patient's handwriting was identified as macrographic and illegible, and written letters and verbs were neglected in free writing or dictation. Moreover, prosody of the patient's utterances was changed. Finally, an intervention was conducted to improve the writing impairments whereby they eventually enhanced. AVM can adversely affect communication opportunities and working life due to these impairments. Thus referring the patient to speech and language pathologists seems sensible and necessary.

Objective: The purpose of this study is to assess demographic, clinical, and morphological characteristics of patients with brain arteriovenous malformations (bAVMs). The relation of outcome using modified Ranklin Scale (mRS) at time of discharge, early and last follow ups with respect to various factors. Materials and Methods: Demographic data, arteriovenous malformation characteristics, and treatment outcomes were evaluated in 43 bAVMs treated with microsurgery between 2009 and 2019. For this series, 43 patients were retrospectively reviewed. A subgroup analysis for Spetzler-Martin grades (SMG) I/II, III, IV/V and III–V were performed. The mRS was used to assess functional outcomes. Results: Overall, mean age at diagnosis was 33 years (standard deviation = 19). Transient deficit, mRS deterioration and impaired functional outcome occurred less frequently in SMG I–II patients compared with Grade III–V patients combined (29% vs. 32% respectively, P = 0.00). All patients with SMG Grade I, Supplemented SMG Grade 2, 3, 4 and 6 had a mRS score of 2 or less at the last follow-up. Age was the only significant predictor of overall outcome after bAVM surgery on Chi-square test (P = 0.046), i.e: all patients

Vascular arteriovenous malformations originate during the early stages of embryonic development and generally grow progressively, especially during adolescence and pregnancy. Limb salvage using microsurgery is presented, in a patient with an arteriovenous malformation who was initially a candidate for limb amputation. En bloc resection of the arteriovenous malformation of all segments with extended brachial approach and the cutaneous component was performed, with an anterolateral thigh free flap for the lateral reconstruction of the hand.

Background We wanted to understand how patients with different modified Rankin Scale (mRS) grades differ regarding their health-related quality of life (HRQoL) and how this affects the interpretation and dichotomization of the grade. Methods In 2016, all adult patients in our brain arteriovenous malformation (AVM) database (n = 432) were asked to fill in mailed letters including a questionnaire about self-sufficiency and lifestyle and the 15D HRQoL questionnaire. The follow-up mRS was defined in 2016 using the electronic patient registry and the questionnaire data. The 15D profiles of each mRS grade were compared to those of the general population and to each other, using ANCOVA with age and sex standardization. Results Patients in mRS 0 (mean 15D score = 0.954 ± 0.060) had significantly better HRQoL than the general population (mean = 0.927 ± 0.028), p p Conclusions Using 15D, we were able to find significant differences in the HRQoL between mRS 0 and mRS 1 AVM patients, against the recent findings on stroke patients using EQ-5D in their analyses. Although the dichotomization cut point is commonly set between mRS 1 and 2, patients in these grades had a similar HRQoL and a decreased ability to continue their premorbid lifestyle, in contrast to patients in mRS 0.

The pressure cooker technique was originally ideated to obtain wedge-flow conditions during arteriovenous malformation or arteriovenous fistula embolisation. The anti-reflux plug created with coils or glue around the tip of a detachable microcatheter enables a continuous injection with a more in-depth penetration. Here we describe two illustrative cases performed with a variation of the technique that we describe as the hand-compression pressure cooker technique.

Complete hydatidiform mole (CHM) is a rare type of pregnancy, in which 15 to 20% of the cases may develop into gestational trophoblastic neoplasia (GTN). The diagnostic of GTN must be done as early as possible through weekly surveillance of serum hCG after uterine evacuation. We report the case of 23-year-old primigravida, with CHM but without surveillance of hCG after uterine evacuation. Two months later, the patient presented to the emergency with vaginal bleeding and was referred to the Centro de Doenças Trofoblásticas do Hospital São Paulo. She was diagnosed with high risk GTN stage/score III:7 as per The International Federation of Gynecology and Obstetrics/World Health Organization (FIGO/WHO). The sonographic examination revealed enlarged uterus with a heterogeneous mass constituted of multiple large vessels invading and causing disarrangement of the myometrium. The patient evolved with progressive worsening of vaginal bleeding after chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO) regimen. She underwent blood transfusion and embolization of uterine arteries due to severe vaginal hemorrhage episodes, with complete control of bleeding. The hCG reached a negative value after the third cycle, and there was a complete regression of the anomalous vascularization of the uterus as well as full recovery of the uterine anatomy. The treatment in a reference center was essential for the appropriate management, especially regarding the uterine arteries embolization trough percutaneous femoral artery puncture, which was crucial to avoid the hysterectomy and allow GTN cure and maintenance of reproductive life.Mola hidatiforme completa (MHC) é um tipo raro de gravidez, na qual 15 a 20% dos casos podem desenvolver neoplasia trofoblástica gestacional (NTG). O diagnóstico de NTG deve ser feito o mais cedo possível, pelo monitoramento semanal do hCG sérico após esvaziamento uterino. Relatamos o caso de uma paciente primigesta, de 23 anos de idade, com MHC, sem vigilância de hCG após esvaziamento uterino. Dois meses depois, a paciente compareceu na emergência com sangramento vaginal, sendo encaminhada ao Centro de Doenças Trofoblásticas do Hospital São Paulo, onde foi diagnosticada com NTG de alto risco, estádio e score de risco III:7 de acordo com a

Objective. We aimed to investigate the risk factors associated with hemorrhage and clarify the relation of homocysteine (Hcy) with brain arteriovenous malformations (bAVMs). Method. We retrospectively reviewed bAVM patients from Beijing Tiantan Hospital between January 2019 and December 2019. Clinical and laboratory variables were analyzed in enrolled patients with bAVMs. Potential predictors associated with hemorrhage were evaluated by logistic regression analysis. Results. A total of 143 bAVM patients were identified in the study, including 69 unruptured and 74 ruptured cases. Patients with hemorrhage were less likely to have hyperhomocysteinemia ( P = 0.023 ). Logistic regression analysis showed that increased maximum diameter of bAVM lesions (odds ratio (OR) 0.634, 95% confidence intervals (CI) 0.479-0.839; P = 0.001 ) and serum Hcy level (OR 0.956, 95% CI 0.920-0.993; P = 0.021 ) were associated with lower risk of hemorrhage in bAVMs. Conclusion. The present study provided evidence regarding the association between serum Hcy and hemorrhage in patients with bAVMs. Higher Hcy level was correlated with a lower risk of rupture. Detection of factors for subsequent hemorrhage is necessary to develop therapeutic strategies for bAVMs preferably.

Background:Pulmonary arteriovenous malformations in single ventricle congenital heart disease are poorly understood. Previous studies investigating pulmonary arteriovenous malformations predominantly focus on patients with heterotaxy syndrome and interrupted inferior caval vein. It is unknown if development and resolution of pulmonary arteriovenous malformations are similar for patients with and without heterotaxy syndrome.Methods:In this retrospective single-institution study, we identified patients with a history of single ventricle congenital heart disease and Fontan palliation. We then matched patients with heterotaxy syndrome (intact and interrupted inferior caval vein) and non-heterotaxy hypoplastic left heart syndrome. To compare development of pulmonary arteriovenous malformations, we identified the frequency of positive diagnoses pre-Fontan. To compare resolution of pulmonary arteriovenous malformations, we recorded oxygen saturation changes for 12 months following Fontan.Results:A total of 124 patients were included. Patients with heterotaxy and interrupted inferior caval vein were more likely to have a pre-Fontan contrast echocardiogram performed (p < 0.01) and more likely to be diagnosed with pulmonary arteriovenous malformations pre-Fontan (p < 0.01). There was no difference in oxygen saturation prior to Fontan, yet all patient groups had increased their oxygen saturations in the first year after Fontan discharge.Conclusions:Pulmonary arteriovenous malformations are variably diagnosed prior to Fontan palliation; however, all study groups had increased oxygen saturations after Fontan discharge, potentially indicating resolution of pulmonary arteriovenous malformations in all groups. The prevalence of pulmonary arteriovenous malformations pre-Fontan is likely underestimated. A quantitative, systematic approach to diagnosis and follow-up of pulmonary arteriovenous malformations is needed to better understand susceptibility and pathophysiology.

Background Vascular endothelial cells (ECs) are subject to continuous shear stress due to blood circulation. Mechanical stress due to high shear flow can also cause arteriovenous malformation (AVM) when ECs respond hyper-sensitively to shear flow. This study was conducted to test the hypothesis that angiogenesis could be promoted in response to mechanical stress via regulation of pro-angiogenic factors in AVM cells. Methods ECs were extracted from the tissue samples from six AVM patients and six normal patients. Shear stress at 7 dynes/cm2 were applied for 24 h. Before and after application of shear stress to each group, RT-PCR was performed to access the expression levels of angiopoietin2(AGP2), aquaporin1(AQP1) and TGFβR1. Immunofluorescences was also performed to evaluate the level of protein expressions. Results In both normal and AVM tissues, AGP2 and TGFβR1 under the shear stress showed increased expression in the ECs compared to the non-sheared samples. When AVMs and normal arterial vasculature were compared, the expression levels of both AGP2 and TGFβR1 in AVMs were higher when compared to normal arterial vasculature with or without shear stress. Immunofluorescence-based protein analysis also confirmed shear-induced AGP2 and TGFβR1 in both samples of normal and AVM patients. Conclusions AVMs exhibited higher sensitivity to shear stress by producing higher expressions of some marked genes and proteins that regulate the endothelial functions upon exposure to shear stress. While the physiological mechanism for AVMs remain elusive, our study shows the plausibility of physical stress imposed by the shearing flow can cause the occurrence of AVMs.

Uterine arteriovenous malformations are rare but may represent a life-threatening cause of vaginal bleeding. The typical patient affected is a multiparous woman during her thirties. The origin can be congenital or acquired, with the latter being more common after uterine surgery and presenting mainly as arteriovenous fistulous connections into the myometrium supplied by uterine arteries. The correct diagnosis of uterine arteriovenous malformations requires imaging findings of tubular and tortuous structures with mixed signal from arterial and venous flows; transvaginal color-Doppler ultrasound is the initial technique applied, then integrated with contrast-enhanced magnetic resonance or computed tomography. Multiple treatment approaches are available, including conservative-medical, endovascular embolization and surgery. Transarterial embolization represents the most applied, preserving childbearing capacity with negligible procedural complications; clinical and technical success rates are elevated, up to 90%. The goal of embolization is to occlude the point of fistula or the nidus and the application of multiple embolizing agents has been reported: despite there is no clear superiority of one over the others, liquids, especially those related to the dymethil-sulfoxide family, present relevant technical advantages. Surgery is nowadays to be considered when the endovascular approach fails and in these cases hysterectomy remains the common recommendation.

Intraorbital arteriovenous malformations (AVMs) are extremely rare, and their actual prevalence is unknown. There is no consensus regarding the best treatment options, and the treatment is usually challenging, involving endovascular and other surgical procedures. Herein, we report the case of a patient diagnosed with an intraorbital AVM, presenting with thrombosis and hemorrhage, with rapidly progressive proptosis, chemosis, ophthalmoparesis, and vision loss. Treatment was performed with a transorbital puncture targeting a venous aneurysm of the superior ophthalmic vein, and closure of the AVM was possible with the use of coils and Onyx. We obtained anatomical occlusion of the lesion, and the patient showed progressive improvement of chemosis, vision acuity, and ophthalmoparesis. At six-month’ follow-up, only mild proptosis was noted, and a control digital subtraction angiography confirmed complete closure of the AVM, with no residual lesion. To date, this is the first case of an intraorbital AVM treated with transorbital direct puncture.

Aim To present the endodontic management of a patient diagnosed with a high-flow arteriovenous malformation (AVM) requiring root canal treatment and to discuss the relevant endodontic literature for this rare but significant problem. Summary AVM is a complex communication of an artery and a vein in which the oxygenated blood is forced away from the intended tissue. The incidence of its occurrence in the face and neck is rare, and when present, the most common sign is gingival bleeding. Arteriovenous malformation is both a diagnostic and therapeutic challenge for dentists. Also, there is ambiguity regarding the precautions to be taken whilst doing endodontic procedures in such cases. Hence, there is a need to understand the disease process and its implications in order to prevent life-threatening complications during treatment. This case report highlights the successful endodontic management of a cariously exposed right maxillary second premolar in a patient diagnosed with high-flow AVM. On an orthopantomogram, the AVM was spreading in the left submandibular space involving the left ramus area. The clinical findings of limited mouth opening with inter-appointment swelling and trismus during treatment made the case unusual and challenging. This paper addresses the current understanding of the classification, diagnosis, clinical features and endodontic management, together with specific guidelines and recommendations whilst performing endodontic procedures in AVM cases. Key learning points The classification and oral manifestations of AVM must be known and understood. Practitioners should be aware of the radiographic appearance of AVM. Recommended precautions should be taken when carrying out restorative and endodontic procedures in a patient with AVM. Antibiotic prophylaxis may be considered before endodontic treatment in a patient with AVM. Multidisciplinary treatment planning may be required.

A 57-year-old man was admitted to our hospital because of frequent hematochezia. Colonoscopy exhibited a submucosal tumor-like lesion in the lower rectum. Abdominal contrast-enhanced computed tomography showed a rectal arteriovenous malformation (AVM) on the right side wall of the lower rectum. The feeder was the superior rectal artery, with early venous return. Embolization of the draining vein and feeding artery of the AVM with N-butyl-2-cyanoacrylate under balloon occlusion was planned. Angiography of the superior rectal artery showed the nidus in the rectum with early venous return of contrast material. The portal vein was punctured percutaneously under ultrasound guidance, and a balloon catheter advanced to the distal part of the superior rectal vein. Venography under balloon occlusion showed the outflow vein and nidus. Transvenous and transarterial nidus embolization with N-butyl-2-cyanoacrylate under balloon occlusion was then performed. Since the embolization, there have been no further episodes of bleeding. There is no established treatment for AVMs. Successful treatment requires targeting and eradication of the nidus. We successfully performed embolization therapy for a rectal AVM via a retrograde transvenous approach. This technique may be suitable for completely eradicating the nidus without the risk of embolism.

Background: Arteriovenous malformations (AVMs) of the brain and face present unique challenges for clinicians. Cerebral AVMs may induce hemorrhage or form aneurysms, while facial AVMs can cause significant disfigurement and pain. Moreover, facial AVMs often draw blood supply from arteries providing critical blood flow to other important structures of the head which may make them impossible to treat curatively. Medical adjuvants may be an important consideration in the management of these patients. Summary: We conducted a systematic review of the literature to identify other instances of molecular target of rapamycin (mTOR) inhibitors used as medical adjuvants for the treatment of cranial and facial AVMs. We also present 2 cases from our own institution where patients were treated with partial embolization, followed by adjuvant therapy with rapamycin. After screening a total of 75 articles, 7 were identified which described use of rapamycin in the treatment of inoperable cranial or facial AVM. In total, 21 cases were reviewed. The median treatment duration was 12 months (3–24.5 months), and the highest recorded dose was 3.5 mg/m2. 76.2% of patients demonstrated at least a partial response to rapamycin therapy. In 2 patients treated at our institution, symptomatic and radiographic improvement were noted 6 months after initiation of therapy. Key Messages: Early results have been encouraging in a small number of patients with inoperable AVM of the head and face treated with mTOR inhibitors. Further study of medical adjuvants such as rapamycin may be worthwhile.

Objective: Intraosseous Arteriovenous malformation (AVM) is a vascular hamartoma with almost 50% of cases occurring in the head and neck. These lesions are of great importance as they may cause massive bleeding during tooth extraction and surgeries. Case report: A 33 year old female complained of swelling and gingival bleeding on the right side of the maxilla and hard palate. The swelling was noticed during pregnancy and the patient underwent a surgical excision 6 months after labor. The specimen was submitted for histological examination. An intraosseous AVM presenting in maxilla was diagnosed. Conclusion: Although rare, intraosseous AVM may occur. The role of pregnancy and hormonal changes is still controversial. Clinical, radiographic and histological characteristics of AVM should be thoroughly assessed before surgical excision.

Mesenteric arteriovenous vasculopathy (MAVD/V) is an extremely rare and poorly understood disease and its incidence is probably underestimated. It is an uncommon, non-inflammatory and non-atherosclerotic form of mesenteric vascular injury, first reported in 2016, with characteristic histopathologic evidence of fibromuscular dysplasia-like vascular changes. We present the case of a chronically ill 84-year-old female with a 5 year history of recurrent small bowel obstruction, who underwent segmental resection of the small bowel. Intraoperative examination showed bowel stricture with fibrosis, intraluminal pill fragments and creeping mesenteric adipose tissue clinically compatible with Crohn's disease. Histological examination showed acute and chronic mucosal injury characterized by crypt distortion, ulcerations with granulation tissue, pseudo-pyloric metaplasia, areas of fibrosis and serosal adhesions. Multiple blood vessels (including both veins and arteries) demonstrated wall hyalinization, elastic degeneration and non-atherosclerotic luminal occlusion. The pattern of the mucosal injury is, in this case, potentially a consequence of acute and chronic ischemic processes secondary to mesenteric arteriovenous vasculopathy.

Background Resection of tumors adjacent to motor pathways carries risks of both postoperative motor deficit and incomplete resection. Our aim was to assess usefulness and limitations of a multimodal strategy that combines intraoperative ultrasound (iUS) guided resection with intraoperative neurophysiology. Methodology This is a prospective study of 25 patients with brain lesions adjacent to motor areas who underwent intracranial surgery with assistance of the iUS guidance system and intraoperative neurophysiological monitoring and mapping. Pathologies treated included 19 gliomas, 3 metastases, 1 anaplastic meningioma, 1 arteriovenous malformation (AVM), and 1 ependymoma. The iUS-guided lesion removal accuracy and the extent of resection were estimated and compared with a 30-day postoperative brain MRI. The results were assessed considering the extent of resection related to 6-month motor function outcome. Results iUS was accurate in checking the extent of resection in 17 patients, whereas in 8 cases the decline of the iUS images quality did not allow a valuable assessment. Positive mapping was obtained in 16 patients. Gross total resection was achieved in 16 patients. In five of nine cases with subtotal resection, surgery was stopped because a functional area was reached. In four patients, tumor removal was limited due to the difficulty of identifying neoplastic tissue. Motor function worsening was transient in six patients and permanent in two. Conclusions The integrated use of intraoperative neuromonitoring to identify motor areas and iUS to identify tumor–tissue interface could help increase the rate of radical resection respecting the eloquent areas.

Contains fulltext : 229345.pdf (Publisher’s version ) (Closed access) PURPOSE: To evaluate treatment outcomes of embolization for peripheral arteriovenous malformations (AVMs) in a tertiary referral center where ethanol is the primary agent of choice. METHODS: A retrospective study was performed of 93 patients (median age, 31 years; range, 2-66 years) with peripheral AVMs treated with embolization (n = 442; median, 2 per patient; range, 1-82) between January 2010 and July 2016. Ethanol was used in most cases (n = 428; 97%). AVMs were classified as type I (n = 3), type II (n = 57), type IIIa (n = 5), type IIIb (n = 15), and type IV (n = 13) according to the Yakes classification system. Effectiveness of embolization was based on AVM devascularization on angiography: 100% (total), 90%-99% (near-total), 70%-90% (substantial), 30%-70% (partial), and 0%-30% (failure). Complications were graded according to the Society of Interventional Radiology classification. RESULTS: In 69% of patients, 70%-100% devascularization was achieved. Total and near-total occlusion of the nidus were more often achieved in AVMs of types I and IIIa (both 100%) than in AVMs of types II, IIIb, and IV (56%, 67%, and 39%, respectively; P = .019). A total of 109 complications were identified: 101 minor (22.9%) and 8 major (1.8%). Major complications included wounds (n = 5), false aneurysm (n = 1), finger contracture (n = 1), and severe pain (n = 1) requiring therapy. The patient complication risk was significantly affected by the number of procedures (relative risk = 2.0; P < .001). Age, AVM location, and angioarchitecture type did not significantly affect complication risk. CONCLUSIONS: AVM embolization resulted in 70%-100% devascularization in 69% of patients, with few major complications. This study indicates that the type of AVM angioarchitecture affects the number of procedures needed and the achievability of AVM devascularization.

Background Multiple high-flow arteriovenous malformations (AVMs) are the hallmark of Parkes-Weber syndrome (PWS). Surgical resection has historically shown poor outcomes with PWS. The aim of this study was to assess the management of PWS in the current era. Methods A retrospective review was performed from 2004 to 2017 on all patients presenting to a single institution for the management of PWS. Presentation, techniques, complications, and outcomes were reviewed. Results Fourteen patients (50% male) with PWS were seen at our institution, and mean age was 19.9 years (range, 4.7–68.8). The lower extremity was affected in 12 (86%) and the upper extremity in 2 (14%) patients. All patients presented with pain and swelling in the affected limb. Seven (50%) patients presented with ulcers, of which 3 (43%) had extensive wounds. Five (36%) patients had echocardiographic evidence of high-output cardiac failure. All patients underwent angiography with the intention to treat. Three (21%) patients were found to have diffuse arteriovenous communication with no discrete AVM nidus and thus did not undergo intervention. The remaining 11 (79%) patients underwent transcatheter embolization of the AVM's arterial inflow. Six (55%) patients required multiple or staged inflow embolization procedures, with a mean of 3.3 (range, 1–10) interventions. Thirty-two arterial embolization procedures were performed in total. n-Butyl-2-cyanoacrylate (nBCA) adhesive was used in 22 (69%), microspheres in 8 (25%), and a combination of coils and nBCA adhesive in 2 (6%) cases. Technical angiographic success was seen in all patients. Six (55%) patients also had interventions to treat the venous component of the malformation, either concomitantly or during a separate procedure. This included radiofrequency ablation in 1 (17%), coil embolization in 1 (17%), sodium tetradecyl sulfate (STS) sclerotherapy in 2 (33%), and a combination of STS, coil embolization, and vein stripping in 2 (33%) patients. Ten (91%) patients experienced a partial response and 1 (9%) patient experienced no response to treatment. No patients had a complete response, as expected with the diffuse nature of this disease. There were no periprocedural complications. Two of 3 patients with complex wounds required major amputations for gangrene, including one above-knee and one below-knee amputation at 128 months and 66 months after the index procedure, respectively. Conclusions AVMs in PWS can be successfully treated by a transcatheter approach. Multiple interventions are usually required. Patients with extensive wounds remain at risk for loss of limb.

BACKGROUND Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a rare syndrome with characteristic skin lesions that are associated with fast-flow vascular malformations (FFVMs) in one-third of patients. Few case series have been described, and none in Spain. AIM To identify the prevalence of dermatological parameters, FFVMs and associated features in a large series of patients with CM-AVM. METHODS We conducted an observational study of patients with CM-AVM syndrome diagnosed in 15 Spanish hospitals over 3 years. The main clinical, radiological, genetic findings and associated diseases were analysed. RESULTS In total, 64 patients were assessed. In 26.5% of cases, the diagnosis was incidental. In 75% of patients, there was one significantly larger macule, which we termed the 'herald patch'. FFVMs were detected in 34% of the patients, with 30% located on the skin, 7.8% in the brain and in 1.5% in the spine. There was a positive family history in 65% of the 64 patients. Genetic analysis was performed for RASA1 mutations in 57 patients, of whom 42 (73%) had a positive result. All 4 patients tested for EPHB4 mutations had a positive result. No tumour lesions were detected in the series, except for five infantile haemangiomas. CONCLUSIONS Our data on clinical lesions, associated FFVM, family history and genetics are similar to those previously published in the literature. An extensive data analysis failed to demonstrate any statistically significant association between the presence of an FFVM and any clinical, familial or genetic parameter that could predict its onset, although a link between the presence of a herald patch on the midline face and the presence of a brain FFVM was observed. We did not detect any genotype-phenotype correlation.

Objective: This paper presents a case of a bronchial Dieulafoy’s lesion in a pediatric patient with recurrent hemoptysis. Case report: A 11-year old female presented multiple times with dry cough and hemoptysis to an outside hospital, each time leading to a diagnosis of epistaxis and subsequent discharge. When she arrived to our tertiary center with heavy hemoptysis and no evidence of epistaxis, the patient was urgently taken to the operating room by both the otolaryngology and pediatric pulmonology services. Active bleeding from a Dieulafoy’s lesion on the right lower bronchus was found and selective embolization of two tortuous arteries was subsequently performed. The patient was discharged in stable condition without recurrence of hemoptysis over the last two months. Conclusion: While rare, especially in pediatric patients, bronchial Dieulafoy’s lesions may cause severe hemoptysis and should be considered in the differential diagnosis when the etiology for hemoptysis is unclear.

Arteriovenous malformation (AVM) in the pancreas rarely causes acute pancreatitis. However, even when it does cause pancreatitis, the pathogenesis is unclear. A 61-year-old man was admitted to our hospital for acute pancreatitis. The findings of computed abdominal tomography, magnetic resonance imaging, and endoscopic ultrasonography revealed pancreatic AVM and hematoma in the tail of the pancreas. These lesions were suspected to be associated with pancreatitis. Although endoscopic retrograde pancreatography could not confirm hemosuccus pancreaticus, distal pancreatectomy was performed because of repeated pancreatitis. The histopathological findings of the resected specimen revealed rupture of the AVM vessels into the main pancreatic ducts. Finally, we considered that intermittent bleeding due to AVM rupture and hematoma formation in the main pancreatic duct caused the repeated pancreatitis.

Vascular anomalies are classified as vascular tumors (VT) and vascular malformations (VM). VTs are divided according to their clinical behavior, while VMs are classified according to their flow characteristics, histopathologic features, and associations with other anomalies. Sonography emerges as the diagnostic imaging method of first choice for assessing abdominal wall disorders and masses, thus representing a valuable tool for the diagnosis and follow-up of abdominal wall VMs. In this review, we report a case of abdominal wall arteriovenous malformation (AVM) in a 17-year old and briefly discuss the cases of abdominal wall VMs.

Background Spinal dural arteriovenous fistulas (DAVFs) and spinal arteriovenous malformations (AVMs) are relatively rare. Moreover, multiple spinal cord vascular lesions in an individual, such as double DAVFs and DAVF with AVM, are extremely rare. To date, there are only several reported cases of concomitant spinal DAVFs and AVMs. Case Description A 25-year-old woman presented with pain of the right lower limb persisting for 1 month. Spinal magnetic resonance imaging showed prominent signal flow voids in the lumbosacral canal. Spinal digital subtraction angiography (DSA) showed a sacral DAVF fed by the right lateral sacral artery and a spinal intradural AVM at the conus medullaris fed by the anterior spinal artery from the left T11 intercostal artery. The drainage veins in these 2 lesions were respective and without common anastomosis. The diagnosis of concomitant sacral DAVF and conus AVM was made. Microsurgery was performed to excise these lesions successfully in the same operation. The patient's symptoms gradually improved after surgery. Postoperative spinal DSA confirmed the complete disappearance of the 2 lesions. Conclusions The concomitant presence of sacral DAVF and conus AVM is extremely rare. Nonetheless, clinicians should be aware of the possibility of the coexistence of multiple types of spinal vascular lesions in a single patient.

Background Radiation-induced spinal cord cavernous malformations (CMs) are rare pathologies compared with radiation-induced cerebral CMs. We present a case of a radiation-induced spinal cord CM developed 31 years after radiation therapy for medulloblastoma. Case Description A 37-year-old man developed a symptomatic spinal hemorrhagic lesion 31 years after radiation therapy for medulloblastoma. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the C7 area. Surgery was performed leading to an unclear diagnosis. Two years later, the patient had a relapse and underwent a second operation, allowing a definitive diagnosis of radiation-induced spinal cord CM. This is believed to be the second case of de novo intramedullary CM formation following spinal radiation therapy for medulloblastoma. Conclusions Radiation-induced spinal cord CMs should be recognized as a possible late adverse effect in patients treated with radiation therapy for medulloblastoma.

Background When a flow-related aneurysm originates from an anterior inferior cerebellar artery (AICA) supplying the cerebellar arteriovenous malformation (AVM), the management becomes very complicated. Endovascular treatment (EVT) was an option, but no consensus has been achieved. Methods and materials: A retrospective investigation was performed for patients with flow-related aneurysm originating from an AICA supplying the cerebellar AVM. Results Ten patients, harboring 13 aneurysms, were identified. Of the 5 a1 aneurysms, 2 underwent stent assisted coiling, 2 underwent parent artery occlusion (PAO), and 1 was intact. Of the 8 a2 aneurysms, 3 underwent coiling with preservation of the AICA, 3 underwent PAO with Onyx, 1 underwent PAO with coils, and 1 was intact. Seven patients underwent partial embolization of the cerebellar AVM, 3 were intact. One patient died 6 hours postoperatively for cerebellar AVM rebleeding. During a follow-up from 6 months to 6 years, 9 patients had favorable recovery. Conclusion For the flow-related aneurysm originating from an AICA supplying the cerebellar AVM, the EVT depends on the specific circumstances. When the aneurysm is located at the a1 segment, coiling of the aneurysm with preservation of the parent AICA should be performed. PAO is the last resort. When the a2 aneurysm is proximal to the internal auditory artery, coiling of the aneurysm with preservation of the AICA is preferred. When the aneurysm is distal to the internal auditory artery, PAO can be safely performed.

Neonates with vein of Galen aneurysmal malformation (VGAM) presenting with severe cardiac failure and pulmonary hypertension represent a challenge for endovascular therapy. When early treatment is required, the small femoral arteries in this population are usually difficult to cannulate. Alternatively, the umbilical vessels offer a natural pathway to reach the lesion. Therefore, prenatal diagnosis of VGAM allows for delivery planning, perinatal management, and embolization through umbilical approach, thus leading to better outcomes.

Undiagnosed significant hydrocephalus is an uncommon finding at forensic autopsy as many cases present in life with complex neurological symptoms. We present a case of a 46-year-old man with no neurological deficits or history of head trauma that was incidentally found to have a massive hydrocephalus at autopsy. This was found to be associated with an unruptured arteriovenous malformation completely confined to the choroid plexus as well as complete agenesis of the corpus callosum. The arteriovenous malformation was found to form a calcified obstruction at the foramen of Monro analogous to a mass lesion, such as a colloid cyst of the third ventricle. The association of this malformation and agenesis of the corpus callosum has never been described. Histologic examination of the brain confirmed significant loss of white matter tracts and thinning of the cortical ribbon due to pressure atrophy of the ependymal lining without significant gliosis, cortical dysplasia, or evidence of other developmental malformations. Autopsy is a vital tool in the evaluation of such rare cases, enhances epidemiologic data, and increases the understanding of these pathophysiological associations.

Gastrointestinal bleeding (GIB) is a common cause of morbidity among patients supported by left ventricular assist devices (LVADs). The aim of this study was to identify if pre-LVAD right ventricular (RV) dysfunction is associated with risk of GIB after LVAD implantation. Of 398 patients implanted with LVADs between July 2008 and July 2016, 130 (33%) developed GIB at a median of 2.6 months following LVAD implantation. Arteriovenous malformations (AVMs) were found in 42 (34%) GIB patients. Patients with GIB were older and more likely to have hypertension, diabetes, and ischemic cardiomyopathy. On pre-LVAD echocardiography, GIB patients had increased RV diastolic dimension (4.7 ± 0.8 vs. 4.4 ± 0.9 cm, p = 0.02), a higher rate of greater than mild tricuspid valve (TV) regurgitation (73 [60%] vs. 120 [47%], p = 0.006), and underwent TV repair more often (38 [30%] vs. 43 [16%], p = 0.0006) during LVAD implantation. After multivariable adjustment, preoperative greater than mild RV enlargement (hazard ratio [HR] 2.32, 95% CI 1.12-5.03; p = 0.03), TV regurgitation (HR 1.83, CI 1.02-3.44; p = 0.01), and TV repair (HR 3.76, confidence interval [CI] 1.02-4.44; p = 0.01) remained associated with risk of GIB. This finding was driven by the AVM-GIB subgroup. Preoperative RV enlargement and TV regurgitation are associated with post-LVAD AVM-related GIB.

Objective The objectives of this study were to document the results of using fibrin glue (FG) combined with pingyangmycin (PYM) for the embolism and sclerotherapy of maxillofacial arteriovenous malformations (AVMs). Study Design We reviewed the associated clinical data from December 2012 to June 2017 for 25 patients with maxillofacial AVMs. The major treatment method was direct percutaneous puncture and injection of FG combined with PYM. Treatment outcomes were assessed through physical examination, Doppler ultrasonography, computed tomography, and 3-dimensional computed tomography angiography scans. Follow-up time ranged from 12 months to 3 years after the last treatment (mean 21 months). Results Of the 25 lesions, 80% showed greater than 90% reduction, 12% showed greater than 75% reduction, and 8% showed greater than 50% reduction. Superficial skin necrosis or mucous ulcer occurred in 3 patients and healed without intervention. Regrowth was observed in 3 patients with extensive lesions involving multiple anatomic regions. Conclusions These data suggest that embolization and sclerotherapy with the use of FG combined with PYM are safe and effective for the treatment of small- to medium-sized, locally dilated maxillofacial AVMs. For AVMs involving multiple anatomic regions, combined application of this approach with other options should be considered.

Background: Cerebrovascular malformations are encountered frequently in clinical practice, but not much is known about the pattern of publication in the Middle East and North Africa (MENA) countries. We aim to evaluate the status and pattern of publications of cerebral arteriovenous malformations (AVM) and cavernomas in the MENA. Materials and Methods: PubMed database was searched for publications on cerebral AVM and cavernomas in the MENA between 2009 and 2019. Results: We found only 94 publications in the MENA region out of 31,333 publications pertaining to AVMs (0.3%). The highest publishing country was Turkey, with 50 (53.1%) studies. The case report was the study design used most by authors with 59 (62.7%) studies. The majority of publications were by neurosurgeons with 42 of 94 (42.4%) papers. European journals ranked first in the number of published articles with 42 of 94 (46.1%) articles. Conclusions: We found a limited number of publications on cerebral AVMs and cavernomas by MENA countries in the past decade. Research support and national/regional registries are important factors to improve the academic output on AVMs and cavernomas in the MENA region.

Arteriovenous malformations (AVMs) are congenital anomalies of the cerebrovascular system. AVM harbors 2.2% annual hemorrhage risk in unruptured cases and 4.5% annual hemorrhage risk of previously ruptured cases. Stereotactic radiosurgery (SRS) have been shown excellent treatment outcomes for patients with small- to moderated sized AVM which can be achieved in 80-90% complete obliteration rate with a 2-3 years latency period. The most important factors are associated with obliteration after SRS is the radiation dose to the AVM. In our institutional clinical practice, now 22 Gy (50% isodose line) dose of radiation has been used for treatment of cerebral AVM in single-session radiosurgery. However, dose-volume relationship can be unfavorable for large AVMs when treated in a single-session radiosurgery, resulting high complication rates for effective dose. Thus, various strategies should be considered to treat large AVM. The role of pre-SRS embolization is permanent volume reduction of the nidus and treat high-risk lesion such as AVM-related aneurysm and high-flow arteriovenous shunt. Various staging technique of radiosurgery including volume-staged radiosurgery, hypofractionated radiotherapy and dose-staged radiosurgery are possible option for large AVM. The incidence of post-radiosurgery complication is varied, the incidence rate of radiological post-radiosurgical complication has been reported 30-40% and symptomatic complication rate was reported from 8.1% to 11.8%. In the future, novel therapy which incorporate endovascular treatment using liquid embolic material and new radiosurgical technique such as gene or cytokine-targeted radio-sensitization should be needed.