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BACKGROUND: Endovascular embolization is frequently used for vascular lesions of the head and neck. Newer agents may help to enhance visualization and improve treatment outcomes. METHODS: The CLARIDAD clinical trial was a prospective, single center, first-in-man investigation of neurovascular embolization using the novel embolic agent ihtObtura for a broad indication, covering the need for a liquid embolic agent in head and neck procedures. The primary outcomes assessed were therapeutic efficacy to deliver ihtObtura to embolize the catheterized pedicle and associated angiographic vascularity, and subsequent loss of radiopacity. Safety endpoints included procedural adverse events, modified Rankin Scale (mRS) score, morbidity, and mortality. Radiologic and clinical follow-up evaluations were conducted at 30, 90, 180 days, and 1 year post-treatment. RESULTS: 65 consecutive patients (mean age 37.8 years, 50.8% women) were treated over 129 sessions. A total of 42 brain arteriovenous malformations (AVMs; 90% grades III and IV), 8 dural arteriovenous fistulas (DAVFs), and 15 hypervascular tumors were treated with ihtObtura using an average of 3.9 mL per session and 7.7 mL per patient. We achieved therapeutic effectiveness in 99% of catheterizations. Radiopacity loss was complete after 74.3% of the sessions at 30 days, 95.6% at 90 days, and 100% at the 1 year follow-up. Serious adverse events (mRS score >2) occurred in two patients (3.1%) with previously ruptured high grade AVMs leading to one death and one permanent disabling morbidity. CONCLUSIONS: The study showed that ihtObtura was a novel, safe, and effective liquid embolic agent for the treatment of AVMs, DAVFs, and hypervascular tumors. Its key property of significant radiopacity loss contributes to improve anatomical understanding, particularly in staged procedures, as well as reduction in post-procedural imaging artifact. There may be additional benefits of eliminating tantalum from the embolic mixture in terms of lesion penetration.

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We present a case of a symptomatic, giant, left upper back arteriovenous malformation that was treated through a staged endovascular and open approach. Through a series of embolizations, followed by resection, we were able to preserve the limb and upper back neurovascular supply, demonstrating an approach to preserve sensation and function and improving quality of life.

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Resumen La embolia paradojal debido a una malformación arteriovenosa pulmonar (MAVP) aislada es una causa in frecuente de accidente cerebrovascular (ACV) isquémico. Las MAVP son conductos anómalos de alta circulación entre arterias y venas pulmonares, desviando sangre desoxigenada hacia la circulación sistémica y represen tan una fuente menos común de embolias paradojales, especialmente en personas jóvenes. La embolización endovascular es el tratamiento preferido para MAVP clínicamente significativas. Presentamos el caso de una mujer de 34 años con ACV isquémico talámico izquierdo. Se detectó pasaje de burbujas "en cortina" en arterias cerebrales mediante Doppler transcraneal. En ecografía intracardíaca no se encontró foramen oval permeable, motivo por el cual se avanzó con realización de angiotomografía pulmonar, la cual confirmó la presencia de MAVP. La paciente recibió tratamiento endovascular exitoso. Es esencial considerar la MAVP en el diagnóstico etio lógico del ACV isquémico, especialmente en pacientes jóvenes con signos de comunicación anormal de derecha a izquierda. Se recomienda un seguimiento periódico mediante imágenes para evaluar la posible recurrencia o cambios en la MAVP, resaltando la importancia del manejo adecuado de estas malformaciones.

Abstract Paradoxical embolism due to an isolated pulmonary arteriovenous malformation (PAVM) is a rare cause of ischemic stroke. PAVMs are abnormal high-flow connec tions between pulmonary arteries and veins, diverting deoxygenated blood into the systemic circulation and they represent a less common source of paradoxical embolisms, especially in young individuals. Endovascular embolization is the preferred treatment for clinically significant PAVMs. We present the case of a 34-year-old woman with a left thalamic ischemic stroke. Severe contrast passage was detected in cerebral arteries through transcranial Doppler. Intracardiac ultrasound did not reveal a patent foramen ovale, prompting further investigation with pulmonary CT angiography, confirming the presence of PAVM. The patient underwent successful endovascular treatment. It is essential to consider PAVM in the etiological di agnosis of ischemic stroke, especially in young patients with signs of abnormal right-to-left communication. Periodic follow-up imaging is recommended to assess potential recurrence or changes in PAVM, emphasizing the importance of appropriate management of these malformations.

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BACKGROUND: Cerebral arteriovenous malformations (AVMs) are complex lesions that can cause hemorrhagic stroke and significant neurological disability. Adenosine induces cardiac standstill and hypotension, which are thought to be useful during cerebral AVM embolization. Herein, we conducted a systematic review and meta-analysis of the technique's safety. METHODS: Following PRISMA guidelines, four databases were queried for studies describing the use of adenosine-assisted embolization of cerebral AVMs. Adenosine-related intraoperative complications, permanent neurological outcomes, morbidity, and mortality assessed the technique's safety. Single proportion analysis under a random-effects model was performed. Heterogeneity was assessed using I² statistics, and publication bias was evaluated through funnel plot analysis and Egger's regression test. RESULTS: Ten studies were included, involving 79 patients (55.7% male) with 79 AVMs (54.4% unruptured and 70.9% Spetzler-Martin grade III-V) who underwent 123 embolizations (80.4% and 5.9% under transarterial and transvenous approaches, respectively) with n-butyl cyanoacrylate (80.4%), ethylene vinyl alcohol (14.4%), or both (5.2%). The incidence of transient adenosine-related intraoperative complications was 0% (95% CI 0% to 3%, I2=24%). Besides, the incidence of adenosine-related morbidity, mortality, and permanent outcomes was 0% (95% CI 0% to 3%, I2=0%). During follow-up, good functional outcomes were reported for 64 patients (81%). CONCLUSIONS: Adenosine's effects on blood flow control can facilitate embolization and mitigate the risk of AVM rupture and embolic agent migration. Although current evidence stems from observational studies, the results of this meta-analysis suggest a safe drug profile due to minimal associated morbidity and mortality. Further research from larger randomized and controlled studies is warranted to attain a higher level of evidence. PROSPERO REGISTRY NUMBER: CRD42023494116.

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RASopathies encompass a diverse set of disorders affecting genes that encode proteins within the RAS-MAPK pathway. RASA1 mutations are the cause of an autosomal dominant disorder called capillary malformation-arteriovenous malformation type 1 (CM-AVM1). Unlike other RASopathies, facial dysmorphism has not been described in these patients. We phenotypically delineated a large family of individuals with multifocal fast-flow capillary malformations, severe lymphatic anomalies of perinatal onset, and dysmorphic features not previously described. Sequencing studies were performed on probands and related family members, confirming the segregation of dysmorphic features in affected members of a novel heterozygous variant in RASA1 (NM_002890.3:c.2366G>A, p.(Arg789Gln)). In this work, we broaden the phenotypic spectrum of CM-AVM type 1 and propose a new RASA1 variant as likely pathogenic.

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Se presenta un caso poco frecuente de hemoptisis en un paciente adolescente con cirugía de Fontan, causada por el sangrado de una malformación arteriovenosa pulmonar (MAVP), tras la práctica de un instrumento de viento (saxofón). El paciente se estudió con angiografía y se realizó cierre percutáneo, consiguiendo posteriormente mejoría clínica y evitando la aparición de nuevos episodios de hemoptisis. Actualmente existe escasa literatura que reporte casos de hemoptisis en pacientes con cirugía de Fontan y ningún caso publicado en relación a gatillantes relacionados a las maniobras de valsalva; como la generada al practicar instrumentos de viento. Por esta razón, se decide publicar este caso clínico en pro de generar mayores conocimientos en este grupo de pacientes con cardiopatías congénitas operadas con procedimientos paliativos como es la cirugía de Fontan.

A rare case of hemoptysis is presented in an adolescent patient with Fontan surgery, caused by bleeding from a pulmonary arteriovenous malformation (PAVM), after playing a wind instrument (saxophone). The patient was studied with angiography and percutaneous closure was performed, subsequently achieving clinical improvement and preventing the appearance of new episodes of hemoptysis. Currently, there is little literature on hemoptysis in patients with Fontan surgery and no case published in relation to triggers related to valsalva maneuvers; like that generated when practicing wind instruments. For this reason, it was decided to publish this clinical case in order to generate greater knowledge in this group of patients with congenital heart disease operated on with palliative procedures such as Fontan surgery.

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The November 2013 online publication of ARUBA, the first multi-institutional randomized controlled trial for unruptured brain arteriovenous malformations (AVMs), has sparked over 100 publications in protracted debates METHODS: This study sought to examine inpatient management patterns of brain AVMs from 2009 to 2016 and observe if changes in U.S. inpatient management were attributable to the ARUBA publication using interrupted time series of brain AVM studies from the National Inpatient Sample data 2009-2016. Outcomes of interest were use of embolization, surgery, combined embolization and microsurgery, radiotherapy, and observation during that admission. An interrupted time series design compared management trends before and after ARUBA. Segmented linear regression analysis tested for immediate and long-term impacts of ARUBA on management. RESULTS: Elective and asymptomatic patient admissions declined 2009-2016. In keeping with the ARUBA findings, observation for unruptured brain AVMs increased and microsurgery decreased. However, embolization, radiosurgery, and combined embolization and microsurgery also increased. For ruptured brain AVMs, treatment modality trends remained positive with even greater rates of observation, embolization, and combined embolization and microsurgery occurring after ARUBA (data on radiosurgery were scarce). None of the estimates for the change in trends were statistically significant. CONCLUSIONS: The publication of ARUBA was associated with a decrease in microsurgery and increase in observation for unruptured brain AVMs in the US. However, inpatient radiotherapy, embolization, and combined embolization and surgery also increased, suggesting trends moved counter to ARUBA's conclusions. This analysis suggested that ARUBA had a small impact as clinicians rejected ARUBA's findings in managing unruptured brain AVMs.

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Brain Arteriovenous Malformations (bAVMs) are rare but high-risk developmental anomalies of the vascular system. Microsurgery through craniotomy is believed to be the mainstay standard treatment for many grades of bAVMs. However, a significant challenge emerges in the existing body of clinical studies on open surgery for bAVMs: the lack of reproducibility and comparability. This study aims to assess the quality of studies reporting clinical and surgical outcomes for bAVMs treated by open surgery and develop a reporting guideline checklist focusing on essential elements to ensure comparability and reproducibility. This is a systematic literature review that followed the PRISMA guidelines with the search in Medline, Embase, and Web of Science databases, for studies published between January 1, 2018, and December 1, 2023. Included studies were scrutinized focusing on seven domains: (1) Assessment of How Studies Reported on the Baseline Characteristics of the Patient Sample; (2) Assessment and reporting on bAVMs grading, anatomical characteristics, and radiological aspects; (3) Angioarchitecture Assessment and Reporting; (4) Reporting on Pivotal Concepts Definitions; (5) Reporting on Neurosurgeon(s) and Staff Characteristics; (6) Reporting on Surgical Details; (7) Assessing and Reporting Clinical and Surgical Outcomes and AEs. A total of 47 studies comprising 5,884 patients were included. The scrutiny of the studies identified that the current literature in bAVM open surgery is deficient in many aspects, ranging from fundamental pieces of information of methodology to baseline characteristics of included patients and data reporting. Included studies demonstrated a lack of reproducibility that hinders building cumulative evidence. A bAVM Open Surgery Reporting Guideline with 65 items distributed across eight domains was developed and is proposed in this study aiming to address these shortcomings. This systematic review identified that the available literature regarding microsurgery for bAVM treatment, particularly in studies reporting clinical and surgical outcomes, lacks rigorous scientific methodology and quality in reporting. The proposed bAVM Open Surgery Reporting Guideline covers all essential aspects and is a potential solution to address these shortcomings and increase transparency, comparability, and reproducibility in this scenario. This proposal aims to advance the level of evidence and enhance knowledge regarding the Open Surgery treatment for bAVMs.

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Paradoxical embolism due to an isolated pulmonary arteriovenous malformation (PAVM) is a rare cause of ischemic stroke. PAVMs are abnormal high-flow connections between pulmonary arteries and veins, diverting deoxygenated blood into the systemic circulation and they represent a less common source of paradoxical embolisms, especially in young individuals. Endovascular embolization is the preferred treatment for clinically significant PAVMs. We present the case of a 34-year-old woman with a left thalamic ischemic stroke. Severe contrast passage was detected in cerebral arteries through transcranial Doppler. Intracardiac ultrasound did not reveal a patent foramen ovale, prompting further investigation with pulmonary CT angiography, confirming the presence of PAVM. The patient underwent successful endovascular treatment. It is essential to consider PAVM in the etiological diagnosis of ischemic stroke, especially in young patients with signs of abnormal right-to-left communication. Periodic follow-up imaging is recommended to assess potential recurrence or changes in PAVM, emphasizing the importance of appropriate management of these malformations.

La embolia paradojal debido a una malformación arteriovenosa pulmonar (MAVP) aislada es una causa infrecuente de accidente cerebrovascular (ACV) isquémico. Las MAVP son conductos anómalos de alta circulación entre arterias y venas pulmonares, desviando sangre desoxigenada hacia la circulación sistémica y representan una fuente menos común de embolias paradojales, especialmente en personas jóvenes. La embolización endovascular es el tratamiento preferido para MAVP clínicamente significativas. Presentamos el caso de una mujer de 34 años con ACV isquémico talámico izquierdo. Se detectó pasaje de burbujas "en cortina" en arterias cerebrales mediante Doppler transcraneal. En ecografía intracardíaca no se encontró foramen oval permeable, motivo por el cual se avanzó con realización de angiotomografía pulmonar, la cual confirmó la presencia de MAVP. La paciente recibió tratamiento endovascular exitoso. Es esencial considerar la MAVP en el diagnóstico etiológico del ACV isquémico, especialmente en pacientes jóvenes con signos de comunicación anormal de derecha a izquierda. Se recomienda un seguimiento periódico mediante imágenes para evaluar la posible recurrencia o cambios en la MAVP, resaltando la importancia del manejo adecuado de estas malformaciones.

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OBJECTIVE: The brain arteriovenous malformation (BAVM) nidus compactness score (CS), determined on angiography, predicts BAVM recurrence after surgical resection among children with sporadic BAVMs. We measured the angiographic CS for BAVMs among children with hereditary hemorrhagic telangiectasia (HHT) to determine CS characteristics in this population. METHODS: A pediatric interventional neuroradiologist reviewed angiograms to determine the CS of BAVMs in children with HHT recruited to the BVMC. CS is based on overall nidus and perinidal anomalous vessel compactness. CS categories included 1 = diffuse nidus, 2 = intermediate nidus, and 3 = compact nidus. RESULTS: Forty-eight of 78 children (61.5%) with HHT and brain vascular malformations had a conventional angiogram; 47 (97.9%) angiograms were available. Fifty-four BAVMs were identified in 40 of these 47 children (85.1%). Of 54 BAVMs in children with HHT, CS was 1 in 7 (13%), 2 in 29 (53.7%), and 3 in 18 BAVMs (33.3%) compared with CS of 1 in six (26.1%), 2 in 15 (65.2%), and 3 in 2 BAVMs (8.7%) among 23 previously reported children with sporadic BAVMs, p = 0.045 (Fisher's exact). Seven children with HHT had intracranial hemorrhage: 4 had CS = 3, 1 had CS = 2, and 2 had CS = 1. CONCLUSIONS: A range of CSs exists across HHT BAVMs, suggesting it may be an angiographic measure of interest for future studies of BAVM recurrence and hemorrhage risk. Children with HHT may have more compact niduses compared to children with sporadic BAVMs. Additional research should determine whether CS affects hemorrhage risk or post-surgical recurrence risk in HHT-associated BAVMs, which could be used to direct BAVM treatment.

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BACKGROUND: Significant controversy exists about the management of unruptured cerebral arteriovenous malformations (AVMs). Results from A Randomized Trial of Unruptured Brain Arteriovenous Malformations (ARUBA) suggested that intervention increases the risk of stroke/death compared with medical management. However, numerous study limitations raised concerns about the trial's generalizability. OBJECTIVE: To assess the rate of stroke/death and functional outcomes in ARUBA-eligible patients from a multicenter database, the Neurovascular Quality Initiative-Quality Outcomes Database (NVQI-QOD). METHODS: We performed a retrospective analysis of prospectively collected data of ARUBA-eligible patients who underwent intervention at 18 participating centers. The primary endpoint was stroke/death from any cause. Secondary endpoints included neurologic, systemic, radiographic, and functional outcomes. RESULTS: 173 ARUBA-eligible patients underwent intervention with median follow-up of 269 (25-722.5) days. Seventy-five patients received microsurgery±embolization, 37 received radiosurgery, and 61 received embolization. Baseline demographics, risk factors, and general AVM characteristics were similar between groups. A total of 15 (8.7%) patients experienced stroke/death with no significant difference in primary outcome between treatment modalities. Microsurgery±embolization was more likely to achieve AVM obliteration (P<0.001). Kaplan-Meier survival curves demonstrated no difference in overall death/stroke outcomes between the different treatment modalities' 5-year period (P=0.087). Additionally, when compared with the ARUBA interventional arm, our patients were significantly less likely to experience death/stroke (8.7% vs 30.7%; P<0.001) and functional impairment (mRS score ≥2 25.4% vs 46.2%; P<0.01). CONCLUSION: Our results suggest that intervention for unruptured brain AVMs at comprehensive stroke centers across the United States is safe.

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Cobb syndrome, or cutaneomeningospinal angiomatosis, is a rare condition that affects young adults, and its etiology has not been completely elucidated. It is characterized by a cutaneous sign, or stigma, associated with spinal or intracranial malformations. The symptoms are quite diverse, but, in most cases, the disease presents motor deficit and pain. The present study reports the case of a 48-year-old female patient, who initially sought dermatological medical care for a single skin lesion in the posterior cervical region. During the excision, it was noticed that the lesion had contiguous behavior to the deep anatomical planes, thus requiring the evaluation of the neurosurgical team. The purpose of this report is to describe this rare disease, covering more details about diagnosis and therapy.

Síndrome de Cobb, ou angiomatose cutâneo-meningo-espinhal, é uma condição rara que afeta adultos jovens, e sua etiologia não foi completamente elucidada. Caracterizase por um sinal cutâneo, ou estigma, associado a malformações espinhais ou intracranianas. Os sintomas são bastante diversos, mas, na maioria dos casos, a doença apresenta déficit motor e dor. O presente estudo relata o caso de uma paciente do sexo feminino de 48 anos, que inicialmente procurou atendimento médico dermatológico para uma única lesão cutânea na região cervical posterior. Durante a excisão, notou-se que a lesão apresentava comportamento contíguo aos planos anatômicos profundos, exigindo assim a avaliação da equipe neurocirúrgica. O objetivo deste relatório é descrever essa doença rara, abrangendo mais detalhes sobre diagnóstico e terapia.

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BACKGROUND: Transvenous embolization is a potential therapy for brain arteriovenous malformation, involving the use of microcatheters to guide an ethylene vinyl alcohol coil for vessel occlusion. However, the safety and efficacy of transvenous embolization are not fully established. OBJECTIVE: To evaluate the safety and efficacy of transvenous embolization for brain arteriovenous malformation. METHODS: A systematic review of the literature of studies investigating the safety and efficacy of transvenous embolization for brain arteriovenous malformation was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Databases, including PubMed, Cochrane Library, Web of Science, and Embase were queried. RESULTS: In the final analysis of 16 studies involving 368 brain arteriovenous malformation cases who received transvenous embolization, the complete occlusion was achieved in 91% (95% CI: 88%, 94%; I2 = 43%, p = 0.04) of cases. The overall rate of good outcomes after discharge was high at 89% (95% CI: 82%, 95%; I2 = 60%, p < 0.01). Ischemic complications were reported in 1% of cases (95% CI: 0%, 2%; I2 = 0%, p = 0.96), while hemorrhagic complications occurred in 6% of cases (95% CI: 3%, 8%; I2 = 8%, p = 0.37), and technical complications rate of 8% (95% CI: 4%, 11%; I2 = 8%, p = 0.36). Finally, only one death was related to the procedure. CONCLUSION: Transvenous embolization for brain arteriovenous malformation shows promising safety and effectiveness, with low mortality, a considerable rate of positive outcomes, and a relatively low incidence of complications. The majority of patients achieved complete occlusion, indicating transvenous embolization as a potential option, especially for challenging deep-seated lesions.

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INTRODUCTION: Trigeminal neuropathic pain (TNP) is a syndrome of severe, disabling, constant facial pain arising from the trigeminal nerve or ganglion. Arteriovenous malformations (AVM) are a rare cause of TNP. The limited choices of intervention of TNP include peripheral nerve stimulation, trigeminal nucleotomy and motor cortex stimulation. CASE REPORT: We present a 56-year-old man who suffered from trigeminal neuropathic pain secondary to nerve compression due to a giant posterior fossa AVM. The pain was refractory to drug treatment. From all the therapeutic options available we declined the microvascular decompression of the trigeminal nerve due to the presence of the giant AVM, or stereotactic radiosurgery because of the AVM´s diffuse nidus. After a multidisciplinary discussion we proposed a minimally invasive, safe and reversible treatment: Motor Cortical Stimulation (MCS). We placed a 16-pole epidural electrode on the right precentral gyrus. The patient had satisfactory pain control with some supplemental medication. No complications or side effects such as seizures, sensory disturbances or infections were presented. DISCUSSION: The limited choices of intervention of TNP include peripheral nerve stimulation, trigeminal nucleotomy and MCS. Henssen et al performed a systematic review where they investigated the effectiveness of MCS and discovered that this is significantly different among different chronic neuropathic orofacial pain disorders. A visual analogue scale (VAS) measured median pain relief of 66.5% was found. CONCLUSION: MCS should be one more tool to consider in highly selected cases, when other treatments are unfeasible.

Introducción: El dolor neuropático trigeminal (DNT) es un síndrome de dolor facial intenso, incapacitante y constante que surge del nervio o ganglio del trigémino. Las malformaciones arteriovenosas (MAV) son una causa rara de DNT. Las opciones terapéuticas de DNT incluyen la estimulación de los nervios periféricos, la nucleotomía del trigémino y la estimulación cortical motora. Caso clínico: Presentamos el caso de un varón de 56 años con dolor neuropático trigeminal secundario a compresión nerviosa por una MAV gigante de fosa posterior. El dolor era refractario al tratamiento farmacológico. De todas las opciones terapéuticas disponibles, desestimamos la descompresión microvascular del nervio trigémino por la presencia de la MAV gigante, o la radiocirugía estereotáctica, por ser difuso el nido de la MAV. Tras una discusión multidisciplinar propusimos un tratamiento mínimamente invasivo, seguro y reversible: Estimulación cortical motora (ECM). Colocamos un electrodo epidural en el giro precentral derecho. El paciente tuvo un control satisfactorio del dolor con medicación suplementaria. No presentó complicaciones ni efectos secundarios como convulsiones, alteraciones sensoriales o infecciones. Discusión: Las opciones limitadas de intervención de DNT incluyen estimulación nerviosa periférica, nucleotomía trigeminal y ECM. Henssen et al realizaron una revisión sistemática donde investigaron la efectividad de MCS y descubrieron que esto es significativamente diferente entre los diferentes trastornos de dolor orofacial neuropático crónico. Se encontró un promedio de alivio del dolor medida por una escala analógica visual del 66,5%. Conclusión: La ECM debería ser una herramienta más a considerar en casos estrictamente seleccionados donde otros tratamientos no son viables.

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Resumen Las malformaciones arteriovenosas son conexiones aberrantes entre los sistemas arterial y venoso que con frecuencia se manifiestan a través de sangrados, los cuales pueden ser espontáneos o tener un factor detonante. Por su parte, el monóxido de carbono es un gas sin olor ni color que debe sus efectos a su afinidad por proteínas indispensables para el metabolismo humano (hemoglobina, mioglobina, citocromos), así como a la formación de óxido nítrico, de especies reactivas de oxígeno, inducción de apop-tosis, peroxidación lipídica, por mecanismos proinflamatorios y al actuar como molécula de señalización, además de otros posibles mecanismos que aun se desconocen. Presentamos el caso de un paciente con una malformación arteriovenosa quien sufrió un infarto cerebral hemorrágico luego de intoxicarse con monóxido de carbono y posteriormente exponemos los posibles mecanismos por los cuales el monóxido de carbono pudo participar como un factor detonante.

Abstract Arteriovenous malformations are aberrant connections between the arterial and venous systems that often manifest them-selves through bleeding, which can be spontaneous or have a trigger. On the other hand, carbon monoxide is an odorless, colorless gas that owes its effects to its affinity for proteins essential for human metabolism (hemoglobin, myoglobin, cytochromes), as well as the formation of nitric oxide, reactive oxygen species, induction of apoptosis, lipid peroxidation, pro-inflammatory mechanisms and by acting as a signaling molecule, in addition to other possible mechanisms that are still unknown. We present the case of a patient with an arteriovenous malformation who suffered a hemorrhagic cerebral infarction after being intoxicated with carbon monoxide and later we expose the possible mechanisms by which carbon monoxide could trigger the hemorrhage.

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OBJECTIVE: To assess the utility of the Curaçao criteria by age over time in children with hereditary hemorrhagic telangiectasia (HHT). STUDY DESIGN: This was a single-center, retrospective analysis of patients attending the HHT clinic at the Hospital for Sick Children (Toronto, Canada) between 2000 and 2019. The evaluation of the Curaçao criteria was completed during initial and follow-up visits. Screening for pulmonary and brain arteriovenous malformations was completed at 5 yearly intervals. RESULTS: A total of 116 patients with genetic confirmation of HHT were included in the analysis. At initial screening at a median (IQR) age of 8.4 (2.8, 12.9) years, 41% met criteria for a definite clinical diagnosis (≥3 criteria). In children <6 years at presentation, only 23% fulfilled at least 3 criteria initially. In longitudinal follow-up, 63% reached a definite clinical diagnosis, with a median (IQR) follow-up duration of 5.2 (3.2, 7.9) years (P = .005). Specifically, more patients met the epistaxis and telangiectasia criteria at last visit compared with initial (79% vs 60%; P = .006; 47% vs 30%; P = .02) but not for the arteriovenous malformation criterion (59% vs 57%; P = .65). CONCLUSIONS: In the pediatric population, most patients do not meet definite clinical criteria of HHT at initial presentation. Although the number of diagnostic criteria met increased over time, mainly due to new onset of epistaxis and telangiectasia, accuracy remained low during follow-up visits. Relying solely on clinical criteria may lead to underdiagnosis of HHT in children.

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O sangramento uterino anormal é diagnóstico sindrômico comum no consultório do ginecologista e pode comprometer substancialmente a qualidade de vida. O objetivo no diagnóstico de sangramento uterino anormal é distinguir pacientes com causas estruturais (anatômicas), como pólipo, adenomiose, leiomioma, malignidade e hiperplasia, de pacientes que apresentam anatomia normal, nas quais o sangramento pode ser devido a alteração dos mecanismos de coagulação, distúrbios ovulatórios, distúrbios primários do endométrio, iatrogenia, ou ter outra causa não classificada. O diagnóstico se inicia a partir de anamnese detalhada e exame físico geral e ginecológico completos, seguidos da solicitação de exames complementares (laboratoriais e de imagem), conforme indicado. O exame de imagem de primeira linha para identificação das causas estruturais inclui a ultrassonografia pélvica. Histerossonografia, histeroscopia, ressonância magnética e amostragem endometrial para exame de anatomia patológica são opções que podem ser incluídas no diagnóstico a depender da necessidade. O objetivo deste artigo é apresentar a relevância dos exames de imagem na investigação das causas de sangramento uterino anormal.

Abnormal uterine bleeding is one of the commonest presenting complaints encountered in a gynecologist's office and may substantially affect quality of life. The aim in the diagnosis of abnormal uterine bleeding is to distinguish women with anatomic causes such as polyp, adenomyosis, leiomyoma, malignancy and hyperplasia from women with normal anatomy where the cause may be coagulopathy, ovulatory disorders, endometrial, iatrogenic and not otherwise classified. Diagnosis begins with a thorough history and physical examination followed by appropriate laboratory and imaging tests as indicated. The primary imaging test for the identification of anatomic causes include ultrasonography. Saline infusion sonohysterography, magnetic resonance, hysteroscopy, endometrial sampling are options that can be included in the diagnosis depending on the need. The aim of this article is to present the relevance of imaging exams in the investigation of the causes of abnormal uterine bleeding.

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