Resumen Las malformaciones arteriovenosas AVM del cuero cabelludo, comúnmente conocidas como aneurismas cirsoides, son colecciones complejas de arterias y venas que se comunican directamente. Dependiendo de su tamaño y complejidad, se puede utilizar una variedad de estrategias para tratarlos. Se presenta el caso de un paciente del sexo masculino de 53 años de edad, que acudió a valoración por presentar una aumento de volumen de una lesión pulsátil que se extendía desde la región parietotemporal derecha acompañado de cefalea holocraneana y tinnitus, se realizó una angiotomografía cerebral en la que se observó una lesión compuesta de imágenes serpentiformes ectásicas, con una localización extracraneal derecha, posteriormente se realizó una angiografía cerebral diagnóstica que demostró un cortocircuito arteriovenoso plexiforme y fistuloso frontoparietal derecho alimentado por aferencias provenientes de la arteria temporal superficial, auricular posterior, occipital y meníngea media derechas, con drenaje a través de una vena varicosa y ectásica hacia la vena yugular externa. Se realizó por vía femoral una embolización por vía endovascular del cortocircuito arteriovenoso en dos tiempos, logrando una construcción de “olla a presión” para realizar la inyección de líquido embolizante y tener una embolización subtotal del 93%, con esto se pudo realizar una resección microquirúrgica con un abordaje frontoparietotemporal derecho, resecando la parte más profunda, y dejando la piel cabelluda con vasos embolizados sin resecar. Para el cierre de la herida quirúrgica se realizó rotación de colgajo simple para cubrir el defecto de piel derivado de su resección. Se sometió al paciente a panangiografía cerebral postresección, y se identificó la ausencia de cortocircuito arteriovenoso. Dependiendo de la extensión y la complejidad anatómica de la malformación, las manifestaciones clínicas pueden variar. Más del 80% de las MAV estarán presentes al nacer y el resto se pueden desarrollar después de un trauma craneal o intervenciones quirúrgicas. Se necesita una intervención terapéutica adecuada a través de una angiografía selectiva, durante la cual se puede realizar la embolización endovascular de los principales vasos de alimentación antes de la intervención quirúrgica abierta. Las reconstrucciones por tomografía computarizada tridimensionales por volumen se pueden utilizar para una mayor planificación quirúrgica, la reducción del aporte arterial postembolización disminuirá la hemorragia intraoperatoria, facilitando la resección quirúrgica total. La eliminación puede dar lugar a defectos cutáneos grandes y de espesor completo que pueden ser difíciles de reconstruir sin comprometer los resultados funcionales y cosméticos.

Abstract Scalp arteriovenous malformations AVMs, commonly known as cirsoid aneurysms, are complex collections of arteries and veins that communicate directly. It has been postulated that AVMs occur both through the activation of inactive fistulous lesions and through the formation of new fistulous vascular channels through the canalization of injured vessels. Depending on their size and complexity, a variety of strategies can be used to treat them. The case of a 53-year-old male is presented who came for evaluation due to an increase in the volume of a pulsating lesion that extended from the right parietotemporal region accompanied by holocraneal headache and tinnitus. A cerebral angiotomography was performed in which a lesion composed of ectatic serpentine images, with a right extracranial location, subsequently diagnostic cerebral angiography was performed demonstrating a right frontoparietal fistulous and plexiform arteriovenous shunt fed by afferents from the right superficial temporal, posterior auricular, occipital and middle meningeal arteries, with drainage through a varicose and ectatic vein towards the external jugular vein. An endovascular embolization of the arteriovenous shunt was performed via the femoral route in two stages, achieving a “pressure cooker” construction to perform the injection of embolizing fluid and have a subtotal embolization of 93%, with this it was possible to perform a microsurgical resection with a right fronto-parietal-temporal approach resecting the deepest part, leaving the scalp with embolized vessels without resecting. To close the surgical wound, a simple flap rotation was performed to cover the skin defect derived from its resection. The patient underwent post-resection cerebral panangiography, identifying the absence of arteriovenous shunt. Depending on the extent and anatomical complexity of the malformation, clinical manifestations may vary. More than 80% of AVMs will be present at birth and the rest may develop after head trauma or surgical interventions. Appropriate therapeutic intervention is needed through selective angiography, during which endovascular embolization of the major feeding vessels can be performed before open surgical intervention. Volume-based three-dimensional computed tomography reconstructions can be used for further planning. Surgical reduction of post-embolization arterial supply will reduce intraoperative hemorrhage, facilitating total surgical resection. Removal can result in large, full-thickness skin defects that may be difficult to reconstruct without compromising functional and cosmetic results.

Abstract Introduction: Arteriovenous Malformations (AVMs) are high-flow anomalous connections between the arterial and venous systems composed of dysplastic vessels resulting from aberrant angiogenesis. They are congenital and when symptomatic they rarely manifest before adolescence. Depending on the location, size, stage and severity of the symptoms, treatment options vary from conservative management to surgical resection. We report a case of a giant arteriovenous malformation of abdominal wall (tipe IIIb of Yakes Classification) treated with surgical resection after prior attempts of scleroembolization. Clinical case: 54-year-old woman with known history of osteoarticular pathology and dyspepsia presented a mass on the left side of the abdominal wall with hard consistency, warm, slightly pulsating and tenderness to touch with several years of evolution. The mass showed infiltration of the internal and external oblique muscles sparing the transverse muscle. Clinically she presented easy fatigue with efforts. Due to the risk of abdominal wall herniation after excision of the AVM, scleroembolization was considered first-line treatment in this case. This strategy resulted in regression of the mass and symptoms improvement. Four years after the last intervention, the patient presented lesion growth, recurrence and worsening of symptoms with severe interference in the quality of life (QoL). After multidisciplinary discussion, she was proposed for complete resection of the AVM. She was first submitted to scleroembolization with Onyx of identified arterial afferents and sclerosis of the lesion nidus with 2% polidocanol. One month after she underwent successfully total resection of the AVM with the collaboration of General Surgery. Conclusion: No unified agreement exists on the best treatment of these complex high flow lesions and it is difficult to establish a comprehensive strategy given the pathology’s clinical variability, complex stratification and the risk of relapse. A case-by-case approach is needed in managing these types of lesions.

Abstract Background: Vascular malformations are a heterogeneous group of vascular development disorders with historically few treatment options. Recent studies have shown the potential benefit of mTOR inhibitors-such as sirolimus-in their treatment, particularly in low-flow malformations. However, reports on their efficacy in high-flow malformations are scarce and controversial. We report the case of a patient with a combined malformation who benefited from treatment with sirolimus. Report: A three-year-old patient was referred to our center with a diffuse and progressive vascular malformation on her right leg, difficult to classify according to ISSVA, with disabling pain, limb deformity, and walking impairment. The initial MRI showed a diffuse malformation on the deep subcutaneous tissue without muscle or bone involvement, measuring 18cm on the largest axis. A progressive clinical worsening occurred, and after discussion with an international reference center in vascular malformations, it was classified as a diffuse vascular malformation with the involvement of contiguous structures without defined borders, with both a low and high-flow component. The high-flow component now behaves like a type IV Yakes AVM. Nevertheless, long-term therapy with sirolimus was instituted when the patient was nine years old, resulting in complete remission of pain, recovery of walking ability, and partial recovery of knee valgus. No side effects were reported. With 38 months of follow-up, the 13-year-old remains pain-free and has improved quality of life despite no imagological improvement. Conclusion: Although the available evidence for mTOR inhibitors is mainly for low-flow malformations, it may be a safe and effective option for selected high-flow malformations when intervention is not possible or carries significant morbidity risks. Some studies showed a reduction in the size of the malformation with treatment; however, this was not the case in our patient, notwithstanding the clear improvement in quality of life for four years without other intervention.

ABSTRACT It is estimated that lymphatic malformations in children account for 6% of all benign vascular malformations. New medical therapies have been developed for the management of lymphatic orbital disease. The purpose of this article was to describe a clinical case of orbital venolymphatic malformation in a 10-year-old boy, causing proptosis and palpebral edema. The lesion was initially treated with local sclerotherapy. However, the lesion relapsed, and was successfully treated with oral sirolimus. Prospective studies are warranted to determine the appropriate dose and extend the indications of sirolimus in these patients. 6 disease 10yearold yearold 10 year old boy edema sclerotherapy However relapsed patients 1

RESUMO A incidência de malformações linfáticas em crianças é estimada em 6% de todas as malformações vasculares benignas. Têm sido desenvolvidos novos tratamento para doenças linfáticas orbitárias. Nosso objetivo é descrever um caso clínico de malformação venolinfática orbitária em um menino de 10 anos de idade, causando proptose e edema palpebral. A lesão foi tratada inicialmente com escleroterapia local. No entanto, a lesão teve recidiva e foi tratada com sucesso com sirolimo oral. Ainda são necessários estudos prospectivos para estabelecer a dose apropriada e a duração do tratamento com sirolimo nesses pacientes. 6 benignas orbitárias 1 idade palpebral local entanto oral pacientes

Abstract The anomalies of the inferior vena cava (IVC) represent a broad group of entities that are not uncommon, mostly being asymptomatic. Their development has its starting point in embryogenesis, representing a complex sequence of processes that occur during the fourth to eighth week of gestation. This process involves complex anastomoses and regressions of embryonic veins. The IVC is the main venous return structure for the extremities and abdominal organs. Proper identification of the IVC is often crucial, as it has direct clinical and pathological implications. Additionally, some anomalies will require a different approach in the planning of vascular interventions, while recognizing others will be useful to differentiate them from pathological conditions. With current multislice tomography, high-resolution spatial images can be acquired, allowing for high-quality reconstructions and highly detailed representations of the anatomy. There is no definitive classification for IVC anomalies. There are numerous possibilities, including agenesis, duplication, left-sided location, interruption of the intrahepatic portion, and Abernethy malformation. There are also anomalies of the tributary veins. In this article, we will describe the different entities, their embryological development, and their correlation with diagrams and tomographic images.

Resumen Las anomalías de la vena cava inferior (VCI) representan un amplio grupo de entidades que no son infrecuentes y en su mayoría son asintomáticas. Su desarrollo tiene como punto de partida la embriogénesis, con una secuencia compleja de eventos que ocurren durante la cuarta a octava semana de gestación. Dicho proceso involucra anastomosis complejas y regresiones de venas embrionarias. La VCI es la principal estructura de retorno venoso de las extremidades y los órganos abdominales. La correcta identificación de sus anomalías es fundamental, ya que algunas tendrán implicancia clínico-patológica directa, otras determinarán un abordaje diferente en la planificación de intervenciones vasculares y, en algunos casos, será de utilidad para diferenciarlas de condiciones patológicas. Con los actuales tomógrafos multislice se pueden adquirir imágenes con alta resolución espacial, lo que hace posible reconstrucciones de alta calidad y representaciones altamente detalladas de la anatomía. No existe una clasificación definitiva para las anomalías de la VCI. Se han descripto numerosas variantes, entre las que se destacan: agenesia, duplicación, localización izquierda, interrupción de la porción intrahepática y malformación de Abernethy; existen también anomalías de las venas tributarias. En este artículo describiremos las diferentes entidades, su desarrollo embriológico y su correlación con diagramas e imágenes tomográficas.

Thyroid gland vascular proliferations pose a challenge to pathologists, radiologists and surgeons. This is illustrated in the case of a 36-year-old woman who underwent a diagnostic thyroid lobectomy for a suspected follicular neoplasm according to cytology. Histological diagnosis confirmed a follicular adenoma as well as a cavernous haemangioma or venous malformation. CONTRIBUTION: Thyroid vascular lesions are rare. Evolving nomenclature and application of the International Society for the Study of Vascular Anomalies classification are discussed. Pertinent radiological clues are highlighted to pre-empt the diagnosis and avoid potential surgical complications.

ABSTRACT Introduction: Klippel-Trenaunay syndrome is a rare congenital disease characterized by a classic triad consisting of a cutaneous capillary malformation, hypertrophy of soft tissue and/or bone in the limbs, and venous malformations, resulting in varicose veins or other malformations of the deep venous system. Objective: To present sclerotherapy as a therapeutic option in the management of Klippel-Trenaunay syndrome. Methods: It is presented the case of a 27-year-old female patient, with white skin, a history of diagnosis of Klippel-Trenaunay syndrome since she was three years old, which manifested as a nevus from birth, located in the left gluteal region, increased size of the limb and presence of venous dilations since early childhood. Results: Foam sclerotherapy was used as a treatment for varicose veins. Conclusions: Varicose tracts were eliminated, which improved the aesthetics and functionality of the limb.

RESUMEN Introducción: El síndrome de Klippel-Trenaunay es una enfermedad congénita poco frecuente, caracterizada por una tríada clásica, que consiste en una malformación capilar cutánea, hipertrofia del tejido blando y/u óseo en extremidades, y malformaciones venosas, resultantes en venas varicosas u otras malformaciones del sistema venoso profundo. Objetivo: Presentar la escleroterapia como opción terapéutica en el manejo del síndrome de Klippel-Trenaunay. Métodos: Paciente femenina de 27 años, color de piel blanca, con antecedentes de diagnóstico de Síndrome de Klippel-Trenaunay desde los tres años de edad, el cual se manifestó como un nevus desde su nacimiento, localizado en la región glútea izquierda, aumento de tamaño de la extremidad y presencia de dilataciones venosas desde la infancia temprana. Resultados: Se empleó, como tratamiento de las várices, la escleroterapia con espuma. Conclusiones: Se logró eliminar los trayectos varicosos, mejorando la estética y funcionabilidad de la extremidad.

ABSTRACT Introduction: Arteriovenous malformations of the brain are vascular dysplasias that commonly present with intracerebral hemorrhage, seizures and headache, their treatment options are still controversial. The objective of this paper is to present our first experience with the introduction of preoperative embolization followed by microsurgery. Methods: Retrospective descriptive study of 7 patients diagnosed with arteriovenous malformations in the period from January 2015 to December 2021. Results: The average age was 35 years, the hemorrhagic presentation was seen in all cases, with grade II (4 patients) and grade III (3 patients) of Spetzler-Martin, including 4 supratentorial and 3 infratentorial, all received embolization at different stages with 90 to 100% occlusion followed by microsurgery. Conclusions: Preoperative endovascular embolization was associated with good neurological results after microsurgical resection by achieving complete excision of the nidus in the absence of morbidity and mortality.

RESUMEN Introducción: Las malformaciones arteriovenosas del encéfalo son displasias vasculares que comúnmente se presentan con hemorragia intracerebral, convulsiones y cefalea, sus opciones de tratamiento siguen siendo aún controversiales. El objetivo de este trabajo es presentar nuestra primera experiencia con la introducción de la embolización preoperatoria seguida de microcirugía. Métodos: Estudio descriptivo retrospectivo de 7 pacientes con el diagnóstico de malformación arteriovenosa en el periodo comprendido entre enero del 2015 a diciembre 2021. Resultados: La edad promedio fue de 35 años, la forma de presentación hemorrágica fue vista en todos los casos, con grado II (4 pacientes) y grado III (3 pacientes) de Spetzler-Martin, de ellas 4 supratentoriales y 3 infratentoriales, todas recibieron embolización preoperatoria en diferentes etapas con un 90 % a 100 % de oclusión seguidas de microcirugía. Las conclusiones fueron que la embolización endovascular preoperatoria se asoció a buenos resultados neurológicos después de la resección microquirúrgica al alcanzar la excéresis completa del nido en ausencia de morbimortalidad.

Resumen El término "disfagia lusoria" se aplica a los síntomas que surgen debido a una compresión vascular del esófago, que resulta de la localización anómala de una arteria subclavia derecha aberrante. Esta es una malformación congênita poco común que, ocasionalmente, podría condicionar una obstrucción completa o incompleta del esófago, constituyendo un anillo esofágico. El reto diagnóstico se relaciona con la falta de una etiología evidente, asociada a la persistencia y empeoramiento de los síntomas, incluida la disfagia progresiva. El objetivo de este artículo es presentar un caso clínico y revisión de la literatura abordando el caso de una paciente pediátrica con disfagia progresiva y fallo de medro, quien presentaba un anillo vascular incompleto por arteria subclavia derecha aberrante. Es importante realizar una adecuada anamnesis clínica y uso de ayudas diagnósticas para determinar el origen de la sintomatología, ya que cada caso requiere un manejo acorde a la presentación clínica.

Abstract The term "Dysphagia lusoria" is applied to symptoms that arise due to vascular compression of the esophagus, resulting from the abnormal location of an aberrant right subclavian artery. This is a rare congenital malformation that could occasionally cause a complete or incomplete obstruction of the esophagus, constituting an esophageal ring. The diagnostic challenge is related to the lack of an obvious etiology, associated with the persistence and worsening of symptoms, including progressive dysphagia. The objective of this article is to present a clinical case and review of the literature addressing the case of a pediatric patient with progressive dysphagia and failure to gain weight, who presented an incomplete vascular ring due to an aberrant right subclavian artery. It is important to carry out an appropriate clinical history and use diagnostic aids to determine the origin of the symptoms, as each case requires management according to the clinical presentation.

ABSTRACT Arterioportal communications are complex hepatic vascular abnormalities. These are rarely seen in dogs and typically manifest as neurological, gastrointestinal, and developmental changes. This report describes clinical, laboratory and imaging findings associated with hepatic arterioportal malformation in a male Shih-Tzu dog aged 12 months. The diagnosis was achieved using computed tomographic angiography. The therapeutic approach selected consisted of palliative medical management (diuretics) combined with dietary protein restriction (3.6 g/100 kcal) provided by hepatic diet and gut activity modulation using lactulose. Surgical intervention was not recommended due to the complexity of vascular changes and portal hypertension. Despite initial clinical improvement, the patient died of disease-related complications seven months after diagnosis. Computed tomographic angiography was vital for accurate diagnosis and treatment selection, that needs to be more investigated. abnormalities neurological gastrointestinal ShihTzu Shih Tzu 1 diuretics (diuretics 3.6 36 3 6 (3. g100 g 100 g/10 kcal lactulose hypertension improvement diseaserelated disease related selection investigated 3. (3 g10 10 g/1 ( g1 g/

RESUMO As comunicações arterioportais são anormalidades vasculares hepáticas complexas que raramente são vistas em cães. As manifestações clínicas geralmente são alterações neurológicas, gastrointestinais e no desenvolvimento dos filhotes. Este relato descreve os achados clínicos, laboratoriais e de imagem associados à malformação arterioportal hepática em um cão Shih-Tzu macho, com 12 meses de idade. O diagnóstico foi feito por angiotomografia computadorizada. A abordagem terapêutica selecionada consistiu no manejo médico (diuréticos) combinado com restrição proteica dietética (3,6g/100kcal), por meio de alimento coadjuvante indicado para hepatopatias, e modulação da atividade intestinal com uso de lactulose. A intervenção cirúrgica não foi recomendada devido à complexidade das alterações vasculares e à hipertensão portal. Apesar da melhora clínica inicial, o paciente morreu de complicações relacionadas à doença sete meses após o diagnóstico. A angiotomografia computadorizada foi vital para o diagnóstico preciso e a seleção do tratamento, que precisa ser mais estudado. cães neurológicas filhotes clínicos ShihTzu Shih Tzu macho 1 idade diuréticos (diuréticos 3,6g/100kcal, 36g100kcal gkcal 3,6g/100kcal , 3 6g 100kcal g kcal (3,6g/100kcal) hepatopatias lactulose portal inicial tratamento estudado (3,6g/100kcal

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. AVMs (AVMs THA. . (THA) DAA (DAA mind bleeding 81yearold yearold 81 year old fracture branches hemostasis noted operation surgery products 3 muscle artery AVF (AVF area embolization knowledge (AVM limb lifethreatening life threatening complication (THA 8

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. (MAV ATQ. . AAD (AAD considerada excessivo 8 prontosocorro pronto socorro esquerdo (ATQ ascendentes hemostasia operação cirurgia pósoperatório, pósoperatório pós operatório, operatório pós-operatório (FAV área endovascular atual pósoperatória operatória vida

SUMMARY: An aberrant right subclavian artery described by David Bayford, is rare and one of the aortic arch anomalies. It gives usually incidental findings. We present the case of a 57-year-old woman who was admitted to a neurology outpatient clinic due to a headache and no obvious pathology was detected during a physical examination. In consequence of the brain and chest CT angiography, incidental anomalies of the aortic arch branches were found and asymptomatic aberrant right subclavian artery and bicarotid trunk was diagnosed. The presence of this anomaly is becoming increasingly important in avoiding vascular injuries and cerebral complications in patients undergoing endovascular intervention on the aorta aortic arch surgery, thyroidectomy, or cervicothoracic surgery. So, detailed knowledge of variations of the subclavian artery and bicarotid trunk is paramount for radiologists and surgeons operating on the region of the anterior neck.

RESUMEN: Una arteria subclavia derecha aberrante descrita por David Bayford, es rara y es una de las anomalías del arco aórtico. Además, suele ser hallazgos accidentales. Presentamos el caso de una mujer de 57 años que ingresó a una consulta de neurología por dolor de cabeza, sin embargo, no se le detectó patología evidente al examen físico. En el angio-TC de cerebro y tórax, se encontraron anomalías incidentales de las ramas del arco aórtico y se diagnosticó arteria subclavia derecha aberrante asintomática y tronco bicarotídeo. Determinar la presencia de esta anomalía es cada vez más importante, para así evitar lesiones vasculares y complicaciones cerebrales en pacientes sometidos a intervención endovascular de la aorta, cirugía del arco aórtico, tiroidectomía o cirugía cervicotorácica. Por lo tanto, el conocimiento detallado de las variaciones de la arteria subclavia y el tronco bicarotídeo es primordial para los radiólogos y cirujanos que operan en la región anterior del cuello.

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. Objective AVMs. . (AVMs) Methods 201 202 22 imaging arteries 37 n 326, 326 , 326) (PVA 29, 29 29) 5, 5 5) 4, 4 4) 3, 3 3) 2, 2 2) 1. 1 1) Results 9 100 100% 92% 92 hysterectomy 6 term 21 followup follow up range, range (range 5122 122 5-12 treatment followup. up. 0.4% 04 0 (0.4% nonflowlimiting non flow limiting occurred Conclusion safe effective longterm long fertility (AVMs 20 32 10 512 12 5-1 0.4 (0.4 51 5- 0. (0. (0 (

RESUMEN El Síndrome de Klippel-Trenaunay se constituye en una malformación vascular compleja con una incidencia de 2 a 3 casos por cada 100.000 nacidos vivos, clínicamente presenta una triada clásica: manchas cutáneas en vino de Oporto, venas varicosas de localización atípica e hipertrofia ósea y de tejidos blandos. Se presenta el caso de una paciente femenina de 33 años, sin antecedentes patológicos o quirúrgicos de importancia quien acude a consulta por aumento de volumen de la extremidad derecha, mancha violácea ipsilateral, además de dolor y parestesias. Al examen físico se evidencia aumento longitudinal de miembro inferior derecho, nevus hiperpigmentario en cara lateral de pierna derecha y muslo que se extiende al glúteo ipsilateral además de venas varicosas atípicas. Se practica ecografía Doppler venosa con transductor lineal que reporta incompetencia de vena Safena Mayor y perforantes suprageniculares. Se realizó manejo quirúrgico mediante safenectomía, corrección de deformidad y referencia a dermatología para terapia láser por el nevus hiperpigmentario. La paciente mostró evolución clínico - quirúrgica favorable con remisión de la sintomatología que motivó su consulta.

RESUMO A síndrome de Klippel-Trenaunay é uma malformação vascular complexa com uma incidência de 2 a 3 casos por 100.000 nascidos vivos. Clinicamente, apresenta uma tríade clássica: manchas vinho do porto, veias varicosas de localização atípica e hipertrofia óssea e de tecidos moles. Apresentamos o caso de uma paciente do sexo feminino, 33 anos, sem histórico patológico ou cirúrgico de importância, que consultou por aumento de volume do membro direito, mancha violácea ipsilateral, além de dor e parestesia. O exame físico revelou aumento longitudinal do membro inferior direito, nevo hiperpigmentado na face lateral da perna e coxa direitas, estendendo-se até a nádega ipsilateral e veias varicosas atípicas. Foi realizado ultrassom Doppler venoso com transdutor linear, que relatou incompetência da veia safena magna e das perfurantes suprageniculares. O tratamento cirúrgico foi realizado por meio de safenectomia, correção da deformidade e encaminhamento à dermatologia para terapia a laser para nevo hiperpigmentado. A paciente apresentou uma evolução clínico-cirúrgica favorável com remissão dos sintomas que a levaram à consulta.

ABSTRACT Klippel-Trenaunay syndrome is a complex vascular malformation with an incidence of 2 to 3 cases per 100,000 live births. Clinically, it presents a classic triad: port-wine stains, varicose veins of atypical location and bone and soft tissue hypertrophy. We present the case of a 33-year-old female patient, with no pathologic or surgical history of importance, who comes to the clinic for an increase in volume of the right extremity, ipsilateral violaceous spot, in addition to pain and paresthesia. Physical examination revealed longitudinal enlargement of the right lower limb, hyperpigmented nevus on the lateral aspect of the right leg and thigh extending to the ipsilateral buttock and atypical varicose veins. Venous Doppler ultrasound with linear transducer reported incompetence of the greater saphenous vein and supragenicular perforators. Surgical management was performed by saphenectomy, deformity correction and referral to dermatology for laser therapy for hyperpigmented nevus. The patient showed favorable clinical-surgical evolution with remission of the symptomatology that motivated her consultation.

Resumen Introducción: Las malformaciones vasculares (MVa) son un grupo heterogéneo de trastornos resultantes de la dismorfogénesis de los vasos sanguíneos. A pesar de que la correcta clasificación es relevante para brindar un adecuado tratamiento de acuerdo con la medicina basada en la evidencia, la terminología diagnóstica podría resultar confusa o utilizarse de manera inapropiada. Métodos: En este estudio retrospectivo se midieron el acuerdo y la concordancia entre los diagnósticos de referencia (o derivación) y los diagnósticos finales confirmados de 435 pacientes pediátricos con MVa recién remitidos a la Clínica de anomalías vasculares (CAV) multidisciplinaria, mediante el análisis de concordancia kappa de Fleiss (κ). Resultados: Se encontró una buena concordancia entre los diagnósticos de referencia (o derivación) y los diagnósticos confirmados de MVa (κ 0.306, p < 0.001). Las malformaciones linfáticas (LM) y las MVa asociadas con otras anomalías presentaron concordancias diagnósticas moderadas (κ 0.593, p < 0.001 y κ 0.469, p < 0.001, respectivamente). Conclusiones: Se requiere de estrategias de educación médica continua para mejorar el conocimiento de los médicos y la precisión diagnóstica de los pacientes con MVa.

Abstract Background: Vascular malformations (VaM) are a heterogeneous group of disorders resulting from the dysmorphogenesis of blood vessels. Although correct classification is relevant to providing adequate treatment according to evidence-based medicine, diagnostic terminology may be misused or need clarification. Methods: We conducted a retrospective study to measure agreement and concordance between referral and final confirmed diagnoses of 435 pediatric patients with VaM newly referred to the multidisciplinary Vascular Anomalies Clinic (VAC) using Fleiss kappa (κ) concordance analysis. Results: We found fair concordance between referral and confirmed diagnoses of VaM (κ 0.306, p < 0.001). Lymphatic malformations (LM) and VaM associated with other anomalies showed moderate diagnostic concordance (κ 0.593, p < 0.001 and κ 0.469, p < 0.001, respectively). Conclusions: Continuing medical education strategies are required to improve physician knowledge and diagnostic accuracy in patients with VaM.