Alopecia temporária após tratamento endovascular relacionada a uma malformação arteriovenosa em face: relato de caso / Temporary alopecia after endovascular treatment related to an arteriovenous malformation in the face: case report / Alopecia temporal posterior a tratamiento endovascular relacionado con malformación arteriovenosa en la cara: reporte de caso

Objetivo: relatar um caso de alopecia temporária após tratamento endovascular com exposição por fluoroscopia devido a uma malformação arteriovenosa na face. Detalhamento do caso: sexo masculino, 34 anos, com queixa de lesão na asa nasal, lábio superior e lateral da face (direita). O paciente trouxe exame de angioressonância apresentando uma malformação arteriovenosa em face com nutrição pela artéria facial e drenagem pela veia mandibular. Como tratamento foi optado uma arteriografia diagnóstica para melhor avaliação de vascularização da malformação arteriovenosa seguida de embolização com onyx® (mistura de etileno vinil álcool copolímero) que fornece o contraste necessário para a visibilização da mistura sob fluoroscopia. O procedimento foi realizado 14 dias após a 1ª consulta, sem intercorrências indicando sucesso terapêutico. No retorno, terceira semana após o procedimento, apresentou alopecia setorial em região occipitoparietal direita. Não havia manchas em região, bem como outros sintomas associados. Foi realizado como tratamento o uso de Minoxidil tópico e Cilostazol via oral. Após o tratamento houve retorno do crescimento espontâneo em cerca de 2 meses. Considerações finais: a embolização com onyx® mostrou-se uma valiosa opção terapêutica com uma maior conservação das estruturas nobres em malformações arteriovenosas, com baixa taxa de complicações no médio e longo prazo.

Objective: to report a case of temporary alopecia after endovascular treatment with fluoroscopy exposure due to an arteriovenous malformation on the face. Case detail: male, 34 years old, complaining of a lesion on the nasal wing, upper lip and side of the face (right). The patient brought an angioresonance exam showing an arteriovenous malformation in the face with nutrition through the facial artery and drainage through the mandibular vein. As a treatment, a diagnostic arteriography was chosen for a better assessment of the vascularity of the arteriovenous malformation followed by embolization with onyx® (mixture of ethylene vinyl alcohol copolymer), which provides the necessary contrast for visualization of the mixture under fluoroscopy. The procedure was performed 14 days after the 1st consultation, with no intercurrences indicating therapeutic success. On return, third week after the procedure, he presented sectoral alopecia in the right occipitoparietal region. There were no stains in the region, as well as other associated symptoms. The use of topical Minoxidil and oral Cilostazol was carried out as treatment. After treatment there was a return of spontaneous growth in about 2 months. Final considerations: embolization with onyx® proved to be a valuable therapeutic option with greater conservation of noble structures in arteriovenous malformations, with a low rate of complications in the medium and long term.

Objetivo: reportar un caso de alopecia transitoria posterior a tratamiento endovascular con exposición radioscópica debido a una malformación arteriovenosa en la cara. Detalle del caso: varón, 34 años, que se queja de una lesión en el ala nasal, labio superior y lado de la cara (derecha). El paciente trajo un examen de angiorresonancia que mostró una malformación arteriovenosa en la cara con nutrición a través de la arteria facial y drenaje a través de la vena mandibular. Como tratamiento se optó por una arteriografía diagnóstica para una mejor valoración de la vascularización de la malformación arteriovenosa seguida de embolización con onyx® (mezcla de copolímero de etileno alcohol vinílico), que proporciona el contraste necesario para la visualización de la mezcla bajo fluoroscopia. El procedimiento se realizó 14 días después de la 1.ª consulta, sin intercurrencias que indicaran éxito terapéutico. A su regreso, a la tercera semana del procedimiento, presenta alopecia sectorial en región occipitoparietal derecha. No había manchas en la región, así como otros síntomas asociados. Como tratamiento se realizó el uso de Minoxidil tópico y Cilostazol oral. Después del tratamiento hubo un retorno del crecimiento espontáneo en aproximadamente 2 meses. Consideraciones finales: la embolización con onyx® demostró ser una valiosa opción terapéutica con mayor conservación de las estructuras nobles en las malformaciones arteriovenosas, con una baja tasa de complicaciones a medio y largo plazo.

Unusual Arteriovenous Malformation in Total Hip Arthroplasty: Case Report of a Complicated Perioperative Course / Malformação arteriovenosa incomum em artroplastia total do quadril: relato de caso de um perioperatório de curso complicado

Abstract It is not common to encounter arteriovenous malformations (AVMs) during total hip arthroplasty (THA). We report the present case to draw attention to the possibility of an AVM during the direct anterior approach (DAA) for THA, which, if not borne in mind, may lead to the myriad of complications related to excessive bleeding. An 81-year-old female presented to the emergency department with a left femoral neck fracture. She elected to undergo a THA via the DAA. Abnormal appearing blood vessels were present near the ascending circumflex branches, which provided difficulty in achieving hemostasis. Excessive blood loss was noted, and the patient received one unit of packed red blood cells during the operation. Hemoglobin and hematocrit dropped in the days following surgery, requiring several additional transfusions of blood products. When the patient complained of progressive left leg swelling on postoperative day 3, a computed tomography revealed large hematomas within the left adductors and the left iliopsoas muscle. Active extravasation was identified arising from a branch of the left profunda femoral artery, as well as an arteriovenous fistula (AVF) in this area. Bleeding was controlled by selective endovascular coil embolization. As of current knowledge, this is the first reported intraoperative discovery of congenital arteriovenous malformation (AVM) with subsequent development of postoperative arteriovenous fistula and associated symptomatic hematomas in the setting of THA using the DAA. Early recognition and intervention of vascular malformations is essential in preventing potential limb- or life-threatening surgical complication.

Resumo Não é comum encontrar malformações arteriovenosas (MAV) durante a artroplastia total do quadril (ATQ). Relatamos o presente caso para chamar a atenção para a possibilidade de uma MAV durante a abordagem anterior direta (AAD) para ATQ, que se não for considerada, pode levar a uma miríade de complicações relacionadas ao sangramento excessivo. Uma mulher de 81 anos foi apresentada ao pronto-socorro com fratura no pescoço do fêmur esquerdo. Ela optou por se submeter a uma artroplastia total do quadril (ATQ) através da AAD. Vasos sanguíneos aparentemente anormais estavam presentes perto dos ramos circunflexos ascendentes, proporcionando dificuldade em alcançar hemostasia. A perda excessiva de sangue foi notada e a paciente recebeu uma unidade de glóbulos vermelhos embalados durante a operação. Hemoglobina e hematócrito caíram nos dias seguintes à cirurgia, exigindo várias transfusões adicionais de produtos sanguíneos. Quando a paciente reclamou de inchaço progressivo na perna esquerda no terceiro dia pós-operatório, a tomografia computadorizada revelou hematomas grandes dentro dos adutores esquerdos e do músculo iliopsoas esquerdo. A extravasão ativa foi identificada a partir de um ramo da artéria femoral esquerda, bem como de uma fístula arteriovenosa (FAV) nesta área. O sangramento foi controlado por embolização seletiva da bobina endovascular. A partir do conhecimento atual, esta é a primeira descoberta intraoperatória relatada de MAC congênita com desenvolvimento subsequente de FAV pós-operatória e hematomas sintomáticos associados no cenário de ATQ utilizando a AAD. O reconhecimento precoce e a intervenção de malformações vasculares são essenciais para prevenir possíveis complicações cirúrgicas de membros ou de risco de vida.

Efficacy of Embolization in Acquired Uterine Vascular Malformations: An Experience in Tertiary Care Centre in India / Eficácia da embolização em malformações vasculares uterinas adquiridas: uma experiência em centro de atendimento terciário na Índia

Abstract Objective: To determine the efficacy of Uterine Artery Embolization in patients with bleeding acquired uterine arteriovenous malformations (AVMs). Methods: A prospective review of all patients who underwent Uterine Artery Embolization at our institution between July 2015 and April 2022 was performed. 225 patients were diagnosed with a uterine vascular malformation on doppler and corresponding MRI imaging. All patients underwent transcatheter embolization of the uterine arteries. Embolic agents in the 375 procedures included Histoacryl glue only (n = 326), polyvinyl alcohol (PVA) particles and Histoacryl glue (n = 29), PVA particles (n = 5), Gelfoam (n = 5), coils (n = 4), PVA particles and coils (n = 3), Histoacryl glue and Gelfoam (n = 2), and Histoacryl glue and coils (n = 1). Results: A total of 375 embolization procedures were performed in 225 patients. 90 patients required repeat embolization for recurrence of bleeding. The technical success rate of embolization was 100%. The clinical success rate was 92%: bleeding was controlled in 222 of 225 patients and three patients underwent a hysterectomy. 60 of the 225 patients had uneventful intrauterine pregnancies carried to term. The 210 patients who underwent successful embolization had no recurrence of bleeding at a median follow-up of 53 months (range, 5-122 months) after treatment. 15 patients were eventually lost to follow-up. One minor complication (0.4%) of non-flow-limiting dissection of the internal iliac artery occurred. Conclusion: Uterine Artery Embolization is a safe, effective, minimally invasive method to treat uterine AVMs with long-term efficacy, which can provide the preservation of fertility.

Pulmonary arteriovenous malformation and cryptogenic stroke / 国际脑血管病杂志

The relationship between pulmonary arteriovenous malformations (PAVMs) and stroke remains unclear. With the development of imaging technology, studies shows that PAVMs are an important cause of cryptogenic stroke (CS). Most PAVMs are not diagnosed until the onset of stroke. The main pathogenesis of PAVMs-related CS is paradoxical embolism and increased blood viscosity caused by iron deficiency anemia. Antiplatelet therapy and interventional therapy may have a preventive effect on recurrent stroke in such patients. This article summarizes the pathophysiological mechanism, diagnosis and treatment of PAVMs-related CS, hoping to provide new ideas for the diagnosis and treatment of CS.

Embolização percutânea de fístulas arteriovenosas pulmonares / Percutaneous embolization of pulmonary arteriovenous fistulas

Malformações arteriovenosas pulmonares são uma comunicação anormal entre uma artéria e uma veia, causando manifestações clínicas, como hipoxemia crônica e eventos embólicos. As malformações arteriovenosas eram tratadas cirurgicamente, com taxa expressiva de complicações. Na década de 1970, a primeira embolização percutânea por cateter foi realizada com molas. Descrevemos três casos nos quais técnicas de embolização percutânea foram efetivas em prevenir eventos embólicos, hemorrágicos e hipoxêmicos no seguimento dos pacientes.

Pulmonary arteriovenous malformation is an abnormal communication between an artery and a vein, causing clinical manifestations, such as chronic hypoxemia and embolic events. Arteriovenous malformations were treated surgically, with a significant rate of complications. In the 1970 ́s, the first percutaneous catheter embolization was performed with coils. We describe three cases in which percutaneous embolization techniques were effective to prevent embolic, hemorrhagic, and hypoxemic events in the follow-up of patients.

Update on pulmonary arteriovenous malformations / Atualização sobre malformações arteriovenosas pulmonares

ABSTRACT This review aimed to provide an overview of pulmonary arteriovenous malformations, including the major clinical and radiological presentations, investigation, and treatment algorithm of the condition. The primary etiology of pulmonary arteriovenous malformations is hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, with mutations in the ENG gene on chromosome 9 (HHT type 1) or in the ACVRL1/ALK1 complex (HHT type 2). Epistaxis should always be evaluated when repeated, when associated with anemia, and in some cases of hypoxemia. In the investigation, contrast echocardiography and chest CT are essential for evaluating this condition. Embolization is the best treatment choice, especially for correction in cases of hypoxemia or to avoid systemic infections. Finally, disease management was addressed in special conditions such as pregnancy. CT follow-up should be performed every 3-5 years, depending on the size of the afferent and efferent vessels, and antibiotic prophylactic care should always be oriented. Ultimately, knowledge of the disease by health professionals is a crucial point for the early diagnosis of these patients in clinical practice, which can potentially modify the natural course of the disease.

RESUMO Esta revisão teve como objetivo fornecer uma visão geral das malformações arteriovenosas pulmonares, incluindo as principais apresentações clínicas e radiológicas, investigação e algoritmo de tratamento da condição. A principal etiologia das malformações arteriovenosas pulmonares é a telangiectasia hemorrágica hereditária (THH), também conhecida como síndrome de Rendu-Osler-Weber, com mutações no gene ENG no cromossomo 9 (THH tipo 1) ou no complexo ACVRL1/ALK1 (THH tipo 2). A epistaxe sempre deve ser avaliada quando repetida, quando associada à anemia e em alguns casos de hipoxemia. Na investigação, a ecocardiografia e TC de tórax com contraste são essenciais para avaliar essa condição. A embolização é a melhor escolha terapêutica, especialmente para correção em casos de hipoxemia ou para evitar infecções sistêmicas. Por fim, o manejo da doença foi abordado em condições especiais, como a gravidez. O acompanhamento por TC deve ser feito a cada 3-5 anos, dependendo do tamanho dos vasos aferentes e eferentes, e a antibioticoprofilaxia deve sempre ser orientada. Em última análise, o conhecimento da doença pelos profissionais de saúde é um ponto crucial para o diagnóstico precoce desses pacientes na prática clínica, o que pode potencialmente modificar o curso natural da doença.

Malformaciones arteriovenosas pulmonares: presentación de un caso clínico pediátrico y revisión de la literatura / Pulmonary arteriovenous malformations: presentation of a pediatric case and review of the literature

Las malformaciones arteriovenosas pulmonares (MAVP) consisten en comunicaciones directas entre el sistema arterial y el sistema venoso pulmonar, sin paso de la sangre por el lecho capilar, produciéndose un cortocircuito de derecha a izquierda extracardíaco, pueden ser congénitas o adquiridas. Algunos casos pueden ser asintomáticos, en cambio en otros pueden ocasionar diversas manifestaciones clínicas y se pueden asociar a complicaciones severas. En niños se observa una baja incidencia y son más frecuentes las formas congénitas. La MAVP se debe sospechar por las manifestaciones clínicas y las imágenes de la radiografía de tórax (RxTx) y su confirmación se realiza mediante una AngioTomografía Computada (TC) de tórax. La embolización endovascular es actualmente el tratamiento de elección, con excelentes resultados, aunque requiere de un seguimiento posterior y de un operador experimentado. Reportamos el caso de una niña que ingresó con clínica muy sugerente, incluyendo: disnea, acropaquia, cianosis periférica, e hipoxemia refractaria. Sin embargo, inicialmente el cuadro clínico fue confundido con una crisis asmática. La Angio-TC de tórax confirmó el diagnóstico y el tratamiento mediante embolización endovascular resultó exitoso.

Pulmonary arteriovenous malformations (PAVM) are communications between the arterial and the pulmonary venous system, without passage of blood through the capillary bed, causing a left to right extracardiac shunt. Some cases may be asymptomatic, while others may cause various clinical manifestations and may be associated with severe complications. In children a low incidence is observed, and congenital forms are more frequent. PAVM should be suspected by clinical manifestations and chest x-ray imaging and confirmed by chest Computed Tomography Angiography (CTA). Endovascular embolization is currently the treatment of choice, with excellent results, although it requires subsequent follow-up. We report a patient who was admitted with a very suggestive clinical history, including: dyspnea, clubbing, peripheral cyanosis, and severe hypoxemia, refractory to oxygen therapy. However, initially the clinical picture was confounded with an asthmatic crisis. CTA confirmed the diagnosis and treatment by endovascular embolization was successful.

Enfermedad de Rendu-Osler-Weber con presentación inusual / Rendu-Osler-Weber´s disease with unusual presentation

La enfermedad de Rendu-Osler-Weber, también conocido como telangiectasia hemorrágica hereditaria, es una enfermedad genética de herencia autosómica dominante con penetrancia incompleta. Afecta por igual a ambos sexos y los síntomas se inician habitualmente entre los 20 y 40 años, pero se considera que la enfermedad está infradiagnosticada. Típicamente las formas clínicas y el debut de esta enfermedad se asocian a los órganos y tejidos que se afectan con mayor frecuencia: telangiectasias en mucosas y en piel, epistaxis, sangramiento gastrointestinal, pulmonar e intracerebral. En contraste, el caso clínico que se presenta se caracteriza porque las primeras manifestaciones clínicas que motivaron la consulta médica fueron crisis de dolores e inflamación ósea en el miembro superior derecho, lo cual es inusual y se inscribe como un elemento de novedad en la enfermedad. Es el objetivo de esta publicación exponer un caso de telangiectasia hemorrágica hereditaria con una forma de presentación atípica en una adolescente. Al alta hospitalaria, la paciente estaba estable, sin complicaciones. Se recomendó seguimiento hospitalario fundamentalmente por la especialidad de Neumología, por ser los pulmones los órganos más afectados(AU)

Rendu-Osler-Weber´s disease, also known as hereditary hemorrhagic telangiectasia, is a genetic disease of autosomal dominant inheritance with incomplete penetrance. It affects both sexes equally and symptoms usually begin between the ages of 20 and 40, but it is considered that the disease is underdiagnosed. Typically, the clinical forms and the onset of this disease are associated with the organs and tissues that are most frequently affected: mucosal and skin telangiectasias, epistaxis, gastrointestinal, pulmonary and intracerebral bleeding. In contrast, the clinical case that is presented is characterized because the first clinical manifestations that motivated the medical consultation were crises of pain and bone inflammation in the right upper limb, which is unusual and is inscribed as an element of novelty in the disease. The objective of this publication is to present a case of hereditary hemorrhagic telangiectasia with an atypical presentation in a female teenager(AU)

Utilidad de la angiografía intra-operatoria en el tratamiento quirúrgico de malformaciones arteriovenosas y aneurismas intracraneales

Objetivo: Determinar la utilidad de la angiografía intraoperatoria (AIO) para detectar lesiones residuales en casos de resección quirúrgica de malformaciones arteriovenosas (MAV) y aneurismas intracraneales. Material y métodos: Estudio observacional, descriptivo, tipo serie de casos. Entre noviembre de 1993 y abril de 2001, se diagnosticaron 778 pacientes con patología vascular cerebral, de los cuales 477 fueron sometidos a cirugía. Se empleó AIO en 119 casos y se analizaron las variables clínicas y radiológicas. Resultados: Se analizaron 119 casos, 105 (88,2%) con MAV y 14 (11,8%) con aneurisma. La edad promedio fue de 35 años (rango 6 - 69) y el sexo masculino representó el 52% de los casos. La asociación entre MAV y aneurisma se encontró en 17 casos (14,3%). El aneurisma más frecuente fue el paraclinoideo gigante (71,3%), mientras que las MAV supratentoriales y Spetzler-Martin grado 3 representaron el 83,8% y 73,3% de los casos, respectivamente. Se demostró lesión residual en 7 casos, de los cuales 5 fueron nido residual de MAV y 2 casos aneurisma remanente. Las complicaciones relacionadas a la AIO fueron del 3,4% y mortalidad del 2,5%. Conclusiones: La AIO es una técnica útil para detectar lesiones residuales en patología vascular cerebral sometidas a cirugía abierta.

SUMMARY Objective: To determine the utility of intraoperative angiography (IOA) to detect residual lesions after surgical repair of arteriovenous malformations (AVM) and intracranial aneurysms (ICA). Methods: This is a case series including 778 patients from November 1993 to April 2001; of which 477 underwent surgical intervention. IOA was used in 119 cases. Results: A total of 119 cases were analyzed, 105 patients with AVM (88.2%) and 14 with an aneurysm (11.8%). The mean age was 35 years (range 6 - 69) and males represented 52% of the cases. Both AVM and aneurysms occurred in 17 cases (14.3%). Giant paraclinoid aneurysm was the most common aneurysm (71.3%), whereas supratentorial and grade 3 Spetzler-Martin AVM represented 83.3% and 73.3% of the cases, respectively. A residual lesion was was detected in 7 cases, of which 5 were residual nidus of an AVM and 2 remnant aneurysms. IOA-related complications occurred in 3.4% and mortality was 2.5%. Conclusions: IOA is a useful technique to detect residual cerebro-vascular lesions after open surgeries

Passive smoking may be associated with bleeding of cerebral arteriovenous malformation in non-smoking women: a retrospective analysis / O tabagismo passivo pode estar associado com o sangramento de malformação arteriovenosa cerebral em mulheres não fumantes: uma análise retrospectiva

ABSTRACT Background Smoking has been considered to be a risk factor for cardiovascular disease, cancer, depression and other diseases in previous reports, and active smoking is considered to be a risk factor for hemorrhagic stroke. In addition, a retrospective study showed that male smokers were at increased risk of bleeding from arteriovenous malformation (AVM), compared with non-smokers. However, the effect of passive smoking on rupturing of cerebral AVM in non-smoking women has not been addressed. Objective This study aimed to assess the impact of tobacco exposure on AVM bleeding risk in non-smoking women. Methods A total of 393 non-smoking women diagnosed with AVM were included. They were divided into a bleeding group (205 women) and a non-bleeding group (188 women). We conducted univariate and multivariate analysis on these two groups. In univariate analysis, risk factors that might be related to AVM bleeding were analyzed. In multivariate analysis, the relationship between passive smoking and AVM rupture was analyzed by correcting confounding factors. Results Multivariate analysis showed that the proportion of passive smoking was statistically different between the bleeding group and the non-bleeding group (OR = 1.609; CI = 1.031-2.509; p = 0.036). Conclusion Passive smoking may increase the risk of AVM bleeding in non-smoking women. This increased risk may be related to the inflammatory response, vascular wall damage, hemodynamic disorders, changes in atherosclerosis and changes in gene expression caused by passive smoking.

RESUMO Antecedentes O tabagismo tem sido considerado fator de risco para doenças cardiovasculares, câncer, depressão e outras doenças em relatos anteriores, e o tabagismo ativo é considerado fator de risco para acidente vascular cerebral hemorrágico. Além disso, um estudo retrospectivo mostrou que os fumantes do sexo masculino apresentavam risco aumentado de sangramento por malformação arteriovenosa (MAV), em comparação com os não fumantes. No entanto, o efeito do tabagismo passivo na ruptura da MAV cerebral em mulheres não fumantes não foi abordado. Objetivo: Este estudo teve como objetivo avaliar o impacto da exposição ao tabaco no risco de sangramento de MAV em mulheres não fumantes. Métodos Foram incluídas 393 mulheres não fumantes diagnosticadas com MAV. Elas foram divididas em um grupo com sangramento (205 mulheres) e um grupo sem sangramento (188 mulheres). Realizamos análise univariada e multivariada nesses dois grupos. Na análise univariada, foram analisados ​​os fatores de risco que podem estar relacionados ao sangramento de MAV. Na análise multivariada, a relação entre tabagismo passivo e ruptura de MAV foi analisada por meio da correção de fatores de confusão. Resultados A análise multivariada mostrou que a proporção de tabagismo passivo foi estatisticamente diferente entre o grupo com sangramento e o grupo sem sangramento (OR = 1,609; IC = 1,031-2,509; p = 0,036). Conclusão O tabagismo passivo pode aumentar o risco de sangramento de MAV em mulheres não fumantes. Esse risco aumentado pode estar relacionado à resposta inflamatória, danos na parede vascular, distúrbios hemodinâmicos, alterações na aterosclerose e alterações na expressão gênica causadas pelo tabagismo passivo.

Malformación arteriovenosa epicraneal compleja (aneurisma cirsoideo). Presentación de un caso y revisión de la literatura

RESUMEN Las malformaciones arteriovenosas extracraneales son raras lesiones vasculares que consisten en comunicaciones fistulosas anormales, asociadas a venas de drenajes dilatadas, que crecen a través del tiempo, con tendencia a hemorragias masivas y lesiones de la piel. Se presenta el caso de un paciente masculino de 19 años de edad con diagnóstico de malformación arteriovenosa extracraneal consistente en un aneurisma cirsoideo de gran tamaño y alto flujo que afectaba cuero cabelludo, cara y cuello. El mismo fue seguido por el servicio de Neurocirugía del Hospital "Carlos Manuel de Céspedes" de Bayamo desde el año 2015, evidenciándose crecimiento progresivo de la misma y aumento de tamaño, ocasionando ulceración de la piel suprayacente y hemorragias de gran magnitud por lo que en un primer momento y como medida emergente para salvar la vida se le realizó ligadura de la arteria carótida común izquierda, luego de esto se comprobó disminución de las dimensiones y el flujo intranidal, no obstante persistieron las hemorragias, programándose entonces la excéresis total macroscópica de la lesión, proceder que fue exitoso. Seguidamente, fue sometido a tratamiento reconstructivo por parte de Caumatología con injerto autólogo de piel del muslo sin complicaciones. Actualmente a dos años de estos tratamientos no se evidencian recurrencias.

ABSTRACT Extracranial arteriovenous malformations are rare vascular lesions consisting of abnormal fistulous communications, associated with dilated drainage veins, which grow over time, with a tendency to massive bleeding and skin lesions. We present the case of a 19-year-old male patient diagnosed with an extracranial arteriovenous malformation consisting of a large, high-flow cirsoidal aneurysm that affected the scalp, face, and neck. The same was followed by the Neurosurgery service of the "Carlos Manuel de Céspedes" Hospital in Bayamo since 2015, showing progressive growth of the same and increase in size, causing ulceration of the overlying skin and hemorrhages of great magnitude so that in Initially, as an emergency measure to save life, ligation of the left common carotid artery was performed. After this, a decrease in dimensions and intranidal flow was verified. However, the hemorrhages persisted. Total macroscopic excision of the artery was scheduled. injury, proceed that was successful. Subsequently, he underwent reconstructive treatment by Caumatology with an autologous thigh skin graft without complications. Currently, two years after these treatments, there are no evidence of recurrences.

RESUMO As malformações arteriovenosas extracranianas são lesões vasculares raras que consistem em comunicações fistulosas anormais, associadas a veias de drenagem dilatadas, que crescem ao longo do tempo, com tendência a sangramentos maciços e lesões cutâneas. Apresentamos o caso de um paciente do sexo masculino de 19 anos com diagnóstico de malformação arteriovenosa extracraniana constituída por um grande aneurisma cirsoidal de alto fluxo que acometeu couro cabeludo, face e pescoço. O mesmo foi acompanhado pelo serviço de Neurocirurgia do Hospital "Carlos Manuel de Céspedes" em Bayamo desde 2015, apresentando crescimento progressivo do mesmo e aumento de tamanho, causando ulceração da pele sobrejacente e hemorragias de grande magnitude de modo que em Inicialmente, como como medida de emergência para salvar a vida, foi realizada ligadura da artéria carótida comum esquerda. Após isso, verificou-se diminuição das dimensões e fluxo intranidal. No entanto, as hemorragias persistiram. Foi programada a excisão macroscópica total da artéria. lesão, procedimento que foibem sucedido. Posteriormente, foi submetido a tratamento reconstrutivo por Caumatologia com enxerto autólogo de pele na coxa sem intercorrências. Atualmente, dois anos após esses tratamentos, não há evidências de recorrências.

What do Cochrane systematic reviews say about congenital vascular anomalies and hemangiomas? A narrative review

ABSTRACT BACKGROUND: Congenital vascular anomalies and hemangiomas (CVAH) such as infantile hemangiomas, port-wine stains and brain arteriovenous malformations (AVMs) impair patients' lives and may require treatment if complications occur. However, a great variety of treatments for those conditions exist and the best interventions remain under discussion. OBJECTIVE: To summarize Cochrane systematic review (SR) evidence on treatments for CVAH. DESIGN AND SETTING: Review of SRs conducted in the Division of Vascular and Endovascular Surgery of Universidade Federal de São Paulo, Brazil. METHODS: A broad search was conducted on March 9, 2021, in the Cochrane Database of Systematic Reviews to retrieve any Cochrane SRs that assessed treatments for CVAH. The key characteristics and results of all SRs included were summarized and discussed. RESULTS: A total of three SRs fulfilled the inclusion criteria and were presented as a qualitative synthesis. One SR reported a significant clinical reduction of skin redness by at least 20%, with more pain, among 103 participants with port-wine stains. One SR reported that propranolol improved the likelihood of clearance 13 to 16-fold among 312 children with hemangiomas. One SR reported that the relative risk of death or dependence was 2.53 times greater in the intervention arm than with conservative management, among 218 participants with brain AVMs. CONCLUSION: Cochrane reviews suggest that treatment of port-wine stains with pulsed-dye laser improves redness; propranolol remains the best option for infantile hemangiomas; and conservative management seems to be superior to surgical intervention for treating brain AVMs.

Correlation between angioarchitectural characteristics of brain arteriovenous malformations and clinical presentation of 183 patients / Impacto das características angioarquiteturais das malformações arteriovenosas cerebrais na apresentaçáo clínica: análise de 183 pacientes

ABSTRACT Background: The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate. Objective: The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation. Methods: A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental). Results: A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01). Conclusions: Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.

RESUMO Antecedentes: A correlação entre a angioarquitetura e a apresentação clínica da Malformação Arteriovenosa do cérebro (MAVc) permanece um assunto de debate. Objetivos: Correlacionar as características angioarquiteturais das MAVc com a apresentação clínica. Métodos: Estudou-se pacientes consecutivos atendidos no Hospital Beneficência Portuguesa-SP, entre 2006 a 2016. Após análise geral, criaram-se cinco grupos de acordo com a apresentação clínica: 1- Hemorragia; 2 - Epilepsia; 3 - Cefaléia; 4 - Déficit Neurológico Progressivo (DNP) e 5 - Incidental. Características epidemiológicas (clínica e topografia) e angioarquiteturais (Classificação de Spetzler-Martin Modificada - SMM; Fluxo intranidal; Aneurismas arteriais, intranidais e venosos; Ectasia venosa; Congestão venosa; "Roubo" arterial; Vascularização dural; Drenagem Venosa Profunda) foram analisadas. Resultados: 183 pacientes foram incluídos e analisados globalmente. Após essa etapa, foram divididos nos grupos: 1 - 56 casos (30,6%); 2 - 49 casos (26,7%); 3 - 41 casos (22,4%); 4 - 28 casos (15,3%) e 5 - 9 casos (4,9%). Principais achados foram referentes a apresentação hemorrágica, na qual observamos correlação estatística positiva com o sexo feminino (P<0,02), lesões classificadas como SMM 3B (P<0,0015) e baixo fluxo (P<0,04). Relacionado à epilepsia, encontramos significância estatística que possibilitou a correlação com pacientes com idade inferior a 36 anos (P<0,001), sexo masculino (P<0,018), lesões superficiais (P<0,002), presença de ectasia venosa (P<0,003) e "roubo" arterial (P<0,01). Pacientes com DNP se apresentam com idade superior aos demais (P<0,01). Conclusões: Após análise multivariada, foi possível separar as MAV em grupos de acordo com as características angioarquiteturais, comprovando que algumas dessas características estão fortemente relacionadas a determinada manifestação.

Treatment of cerebral arteriovenous malformations in children / 国际脑血管病杂志

Brain arteriovenous malformation (bAVM) is a common cerebrovascular disease in clinical practice. Compared with adults, children with bAVM are more prone to rupture and bleeding, and have a higher mortality and disability. Therefore, it is very important to accurately evaluate the bleeding risk of children with bAVM and reasonably select intervention measures to improve the prognosis. The treatment methods of children with bAVM mainly include conservative treatment, microsurgical treatment, endovascular embolization, stereotactic radiosurgery, and multimodal combined treatment. At present, the treatment indications and specific treatment options for children with bAVM are controversial.

Successful management of brain abscess and paradoxical embolization caused by huge pulmonary arteriovenous malformation: a case report / 中国医师杂志

Objective:The purpose of this case report is to improve the understanding of pulmonary arteriovenous malformation (PAVM) and its related complications by reviewing case data and literature.Methods:A case of huge PAVM complicated with brain abscess and paradoxical embolization of liver, spleen and kidney is reported, and then we summarize its clinical characteristics, diagnosis and treatment, and review the relevant references.Results:Brain abscess and paradoxical embolization are the most common complications of PAVM. The diagnosis and process of this patient was timely and clear. The patient received interventional embolization for PAVM and achieved good results.Conclusions:PAVM can cause brain abscess and paradoxical embolization. We should be vigilant to avoid missed diagnosis and misdiagnosis.

Application of susceptibility-weighted imaging in intracranial dural arteriovenous fistula / 国际脑血管病杂志

Objective:To investigate the application value of susceptibility-weighted imaging (SWI) in the diagnosis of intracranial dural arteriovenous fistula (DAVF).Methods:Patients with DAVF confirmed by digital subtraction angiography (DSA) in Weihai Municipal Hospital from January 2014 to January 2021 were retrospectively included. All patients underwent conventional T 1-weighted imaging (T 1WI), T 2-weighted imaging (T 2WI) and SWI, and some patients also underwent 3D time-of-flight magnetic resonance angiography (3D-TOF-MRA). Results:A total of 36 patients with DAVF were enrolled, 29 of them received 3D-TOF-MRA. The fistula location of 24 patients (24/36, 66.7%) underwent SWI and 26 patients (26/29, 89.7%) underwent 3D-TOF-MRA were correctly judged, and the difference was statistically significant (Fisher's Exact Test, P=0.039). SWI showed that the proportion of patients with thickened supply arteries (7/36, 19.4%) was significantly lower than that on 3D-TOF-MRA (14/29, 48.3%; χ2=6.105, P=0.013). T 2WI, 3D-TOF-MRA and SWI showed no cerebral venous abnormalities in all 7 patients with DAVF without cortical venous reflux; in 29 patients with DAVF with cortical venous reflux revealed by DSA, SWI and T 2WI showed all patients (100%) and 26 patients (89.7%) had superficial venous dilatation respectively, but there was no significant difference ( χ2=0.693, P=0.405). SWI showed medullary vein dilation in 17 patients (47.2%), and only 2 patients (5.6%) had medullary vein thickening on T 2WI, and the difference was statistically significant ( P<0.001). The proportion of patients with venous cerebral infarction on T 2WI was significantly higher than that on SWI (22.2% vs. 0%; Fisher’s Exact Test P=0.005), and the proportion of patients with intracerebral hemorrhage on SWI was significantly higher than that on T 2WI (61.1% vs. 25.0%; χ2=9.574, P=0.004). Conclusion:SWI is helpful to evaluate the abnormal drainage vein of DAVF and the secondary changes in brain, especially intracerebral hemorrhage.

Telangiectasia hemorrágica hereditaria durante el embarazo: Reporte de caso / Hereditary hemorrhagic telangiectasia during pregnancy: Case report

Resumen ANTECEDENTES: La telangiectasia hemorrágica hereditaria, o síndrome de Rendu-Osler-Weber, es una enfermedad vascular, hereditaria y autosómica caracterizada por telangiectasias mucocutáneas y malformaciones arteriovenosas en el pulmón, el cerebro e hígado. La prevalencia estimada es de 1.5 a 2 personas afectadas por cada 10,000 habitantes. El 90% de los casos se debe a una mutación en el gen endoglina y en el de la cinasa 1 similar al receptor de activina (ACVRL1). En la mujer embarazada, la telangiectasia hemorrágica hereditaria es de alto riesgo, sobre todo durante el segundo y tercer trimestre. OBJETIVO: Reportar un caso de telangiectasia hemorrágica hereditaria y exponer las complicaciones que pueden registrarse durante el embarazo. CASO CLÍNICO: Paciente de 23 años, con antecedente heredofamiliar de madre con diagnóstico de telangiectasia hemorrágica hereditaria (síndrome de Osler-Weber-Rendu) que falleció a los 38 años. Antecedente personal patológico de telangiectasia hemorrágica hereditaria, con diagnóstico a los 12 años luego de múltiples episodios de epistaxis. Recibió tratamiento con transfusiones sanguíneas en múltiples ocasiones y 200 mg de sulfato ferroso cada 24 horas. CONCLUSIÓN: La telangiectasia hemorrágica hereditaria condiciona, en la mujer embarazada, la aparición de complicaciones que pueden poner en riesgo la vida de la madre y el feto. Las mujeres con antecedente conocido deben valorarse antes de la concepción con el propósito de conocer el estado de la enfermedad.

Abstract BACKGROUND: Hereditary hemorrhagic telangiectasia, or Rendu-Osler-Weber syndrome, is an autosomal inherited vascular disease characterized by mucocutaneous telangiectasias and arteriovenous malformations in the lung, brain and liver. The estimated prevalence is 1.5 to 2 affected persons per 10,000 population. Ninety percent of cases are due to a mutation in the endoglin gene and in the activin receptor-like kinase 1 gene (ACVRL1). In pregnant women, hereditary hemorrhagic telangiectasia is high risk, especially during the second and third trimester. OBJECTIVE: To report a case of hereditary hemorrhagic telangiectasia and to expose the complications that can occur during pregnancy. CLINICAL CASE: 23-year-old patient, with hereditary family history of mother diagnosed with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) who died at 38 years of age. Personal pathological history of hereditary hemorrhagic telangiectasia, diagnosed at the age of 12 years after multiple episodes of epistaxis. She was treated with multiple blood transfusions and 200 mg of ferrous sulfate every 24 hours. CONCLUSION: Hereditary hemorrhagic telangiectasia conditions, in pregnant women, the appearance of complications that can put the life of the mother and fetus at risk. Women with a known history should be evaluated before conception in order to know the status of the disease.

Pulmonary arteriovenous malformations and ischemic stroke / 国际脑血管病杂志

Pulmonary arteriovenous malformations (PAVMs) can induce cryptogenic stroke and are also an important cause of stroke in young adults. The risk of ischemic stroke increases with age in patients with PAVMs. Contrast echocardiography can be used for early screening of PAVMs, especially in young patients with cryptogenic stroke. After treatment, patients with PAVMs have a reduced risk of ischemic stroke. This article summarizes the etiology, pathogenesis, diagnosis and treatment of PAVMs by retrieving studies on PAVMs and ischemic stroke, in order to improve the understanding of PAVMs and avoid misdiagnosis and missed diagnosis.

Vein of Galen Aneurysmal Malformations in Pediatric Patients Submitted to Endovascular Treatment: Report of 3 Cases

Objective To report three cases of vein of Galen aneurysmalmalformation (VGAM) in pediatric patients treated at the hemodynamics lab of Hospital Santa Isabel (HSI) in Blumenau, state of Santa Catarina, Brazil, from 2006 to 2020. Clinical presentation, endovascular treatment, and postprocedure evolution to date are included. Case description Three children aged 5 to 12 months with cardiac, respiratory, or neurological damage in the neonatal stage, were referred to the neurosurgery service and diagnosed with VGAM. The three patients underwent endovascular embolization of themalformation, with different clinical evolution throughout outpatient follow-up. Conclusion Vein of Galen aneurysmal malformations are uncommon vascular abnormalities that, until the advent of endovascular embolization, were associated with high morbidity and mortality rates. Its prognosis is linked with initial clinic, early diagnosis, and timely surgical correction.

Aprendizaje remoto para procedimientos neuro-endovasculares durante la pandemia COVID-19 / Remote learning for neuro-endovascular procedures during the COVID-19 pandemic

RESUMEN Durante la pandemia COVID-19 se ha incrementado el uso de la telemedicina y de plataformas virtuales en el campo de la medicina, por ello, en nuestra institución contamos con un sistema multicámara que permite la visualización en vivo de procedimientos endovasculares. Se realizaron once casos de aneurismas, malformaciones arteriovenosas y hematomas subdurales crónicos que fueron tratados y transmitidos en vivo sin problemas técnicos a través de la plataforma Zoom®. El tiempo promedio de transmisión y del número de participantes fue de 2.5 horas y 6 participantes, respectivamente. En todos los casos se discutió la técnica empleada y las complicaciones ocurridas. El aprendizaje remoto con plataformas en línea es hoy en día una herramienta importante, pero no un sustituto del aprendizaje práctico para procedimientos endovasculares. Recomendamos su implementación durante la pandemia de COVID-19 como un sustituto temporal, especialmente para los médicos en entrenamiento que no tienen acceso a intervenciones endovasculares avanzadas.

ABSTRACT During the COVID-19 pandemics there has been a substantial increase in the use of telemedicine and virtual platforms in the medical field. For this reason, we have in our institution a multi- camera system that allows us live visualizing endovascular procedures. Eleven cases dealing with aneurysms, arteriovenous malformations and chronic subdural hematomas were treated and broadcasted live with no technical problems using the Zoomâ platform. The average time for transmissions was 2.5 hours, and the average number of participants was 6 persons. The used technique and occurring complications were discussed for all cases. Remote learning using online platforms is nowadays a very important tool, but it is not a substitute for practical learning when performing endovascular procedures. We recommend to implement such techniques during COVID-19 pandemics as a temporary substitute for live learning, particularly for young in-training physicians who may not have access to advanced endovascular interventions.