RESUMO

Las malformaciones vasculares congénitas afectan con mayor frecuencia al sistema tegumentario y se hacen evidentes al nacer o en las primeras semanas de vida con una prevalencia estimada del 4,5%. Las anomalías linfáticas, suelen tener una presentación variable, y en la mayoría de ocasiones su manejo se convierte en un desafío. Se estima que su incidencia global oscila entre 1 en 2.000 y 1 en 16.0004,5 casos. Objetivo: Determinar la importancia de un adecuado manejo por cirugía vascular en el contexto de un paciente pediátrico con Linfangioma en miembro inferior tomando como metodología la presentación de un caso clínico. Descripción del caso: Paciente de 7 meses de edad sin antecedentes clínicos o quirúrgicos de interés, quien es traído por su madre a consulta de cirugía vascular por presencia de masa en miembro inferior derecho que progresivamente ha mostrado crecimiento, cuadro que se acompaña de tumefacción en partes blandas y roce o dolor a la distensión en la zona de la malformación, de acuerdo a la clasificación de Mulliken y Glowacki se concluye que se trata de un Linfangioma, diagnóstico que se corroboró por ultrasonografía Doppler, Angiotac y flebografía ascendente. Para su manejo se realizó drenaje percutáneo ecoguiado y escleroterapia mediante espuma de polidocanol al 1% para sellar la malformación vascular, obteniendo mejoría clínica con resultados estéticos y funcionales satisfactorios. Conclusión: El Linfangioma es una malformación vascular poco frecuente que no se ha documentado localmente, por lo tanto, la presentación de este caso pretendió proporcionar información científica actualizada sobre el tratamiento de la patología vascular y abogar por un manejo basado en la evidencia médica existente, que contribuya a resultados favorables para los pacientes pediátricos.

Congenital vascular malformations most frequently affect the integumentary system and become evident at birth or in the first weeks of life with an estimated prevalence of 4.5%. Lymphatic anomalies usually have a variable presentation, and in most cases their management becomes a challenge. Its global incidence is estimated to range between 1 in 2,000 and 1 in 16,0004.5 cases. Objective: Determine the importance of adequate management by vascular surgery in the context of a pediatric patient with Lymphangioma in the lower limb using the presentation of a clinical case as a methodology. Description of the case: A 7-month-old patient with no clinical or surgical history of interest, who was brought by his mother to a vascular surgery consultation due to the presence of a mass in the right lower limb that has progressively shown growth, a condition that is accompanied by swelling in the soft tissues and friction or pain upon distension in the area of the malformation, according to the classification of Mulliken and Glowacki, it is concluded that it is a Lymphangioma, a diagnosis that was confirmed by Doppler ultrasonography, Angiotac and ascending phlebography. For its management, ultrasound-guided percutaneous drainage and sclerotherapy using 1% polidocanol foam was performed to seal the vascular malformation, obtaining clinical improvement with satisfactory aesthetic and functional results. Conclusion: Lymphangioma is a rare vascular malformation that has not been documented locally, therefore, the presentation of this case aimed to provide updated scientific information on the treatment of vascular pathology and advocate management based on existing medical evidence. that contributes to favorable outcomes for pediatric patients.

As malformações vasculares congênitas afetam mais frequentemente o sistema tegumentar e tornam-se evidentes ao nascimento ou nas primeiras semanas de vida, com prevalência estimada em 4,5%. As anomalias linfáticas costumam ter apresentação variável e na maioria dos casos seu manejo torna-se um desafio. Estima-se que sua incidência global varie entre 1 em 2.000 e 1 em 16.0004,5 casos. Objetivo: Determinar a importância do manejo adequado por cirurgia vascular no contexto de um paciente pediátrico com Linfangioma em membro inferior utilizando como metodologia a apresentação de um caso clínico. Descrição do caso: Paciente de 7 meses, sem antecedentes clínicos ou cirúrgicos de interesse, que foi trazido pela mãe à consulta de cirurgia vascular devido à presença de uma massa no membro inferior direito que apresentava crescimento progressivo, quadro que vem acompanhado de inchaço nos tecidos moles e fricção ou dor à distensão na área da malformação, segundo a classificação de Mulliken e Glowacki, conclui-se que se trata de um Linfangioma, diagnóstico que foi confirmado por Ultrassonografia Doppler, Angiotac e flebografia ascendente. Para seu manejo foi realizada drenagem percutânea guiada por ultrassom e escleroterapia com espuma de polidocanol a 1% para selar a malformação vascular, obtendo melhora clínica com resultados estéticos e funcionais satisfatórios. Conclusão: O linfangioma é uma malformação vascular rara e não documentada localmente, portanto, a apresentação deste caso teve como objetivo fornecer informação científica atualizada sobre o tratamento da patologia vascular e defender uma gestão baseada na evidência médica existente que contribua para resultados favoráveis para a pediatria. pacientes.

RESUMO

Las malformaciones vasculares congénitas afectan con mayor frecuencia al sistema tegumentario y se hacen evidentes al nacer o en las primeras semanas de vida con una prevalencia estimada del 4,5%. Las anomalías linfáticas, suelen tener una presentación variable, y en la mayoría de ocasiones su manejo se convierte en un desafío. Se estima que su incidencia global oscila entre 1 en 2.000 y 1 en 16.0004,5 casos. Objetivo: Determinar la importancia de un adecuado manejo por cirugía vascular en el contexto de un paciente pediátrico con Linfangioma en miembro inferior tomando como metodología la presentación de un caso clínico. Descripción del caso: Paciente de 7 meses de edad sin antecedentes clínicos o quirúrgicos de interés, quien es traído por su madre a consulta de cirugía vascular por presencia de masa en miembro inferior derecho que progresivamente ha mostrado crecimiento, cuadro que se acompaña de tumefacción en partes blandas y roce o dolor a la distensión en la zona de la malformación, de acuerdo a la clasificación de Mulliken y Glowacki se concluye que se trata de un Linfangioma, diagnóstico que se corroboró por ultrasonografía Doppler, Angiotac y flebografía ascendente. Para su manejo se realizó drenaje percutáneo ecoguiado y escleroterapia mediante espuma de polidocanol al 1% para sellar la malformación vascular, obteniendo mejoría clínica con resultados estéticos y funcionales satisfactorios. Conclusión: El Linfangioma es una malformación vascular poco frecuente que no se ha documentado localmente, por lo tanto, la presentación de este caso pretendió proporcionar información científica actualizada sobre el tratamiento de la patología vascular y abogar por un manejo basado en la evidencia médica existente, que contribuya a resultados favorables para los pacientes pediátricos

Congenital vascular malformations most frequently affect the integumentary system and become evident at birth or in the first weeks of life with an estimated prevalence of 4.5%. Lymphatic anomalies usually have a variable presentation, and in most cases their management becomes a challenge. Its global incidence is estimated to range between 1 in 2,000 and 1 in 16,0004.5 cases. Objective: Determine the importance of adequate management by vascular surgery in the context of a pediatric patient with Lymphangioma in the lower limb using the presentation of a clinical case as a methodology. Description of the case: A 7-monthold patient with no clinical or surgical history of interest, who was brought by his mother to a vascular surgery consultation due to the presence of a mass in the right lower limb that has progressively shown growth, a condition that is accompanied by swelling in the soft tissues and friction or pain upon distension in the area of the malformation, according to the classification of Mulliken and Glowacki, it is concluded that it is a Lymphangioma, a diagnosis that was confirmed by Doppler ultrasonography, Angiotac and ascending phlebography. For its management, ultrasound-guided percutaneous drainage and sclerotherapy using 1% polidocanol foam was performed to seal the vascular malformation, obtaining clinical improvement with satisfactory aesthetic and functional results. Conclusion: Lymphangioma is a rare vascular malformation that has not been documented locally, therefore, the presentation of this case aimed to provide updated scientific information on the treatment of vascular pathology and advocate management based on existing medical evidence. that contributes to favorable outcomes for pediatric patients

As malformações vasculares congênitas afetam mais frequentemente o sistema tegumentar e tornam-se evidentes ao nascimento ou nas primeiras semanas de vida, com prevalência estimada em 4,5%. As anomalias linfáticas costumam ter apresentação variável e na maioria dos casos seu manejo torna-se um desafio. Estima-se que sua incidência global varie entre 1 em 2.000 e 1 em 16.0004,5 casos. Objetivo: Determinar a importância do manejo adequado por cirurgia vascular no contexto de um paciente pediátrico com Linfangioma em membro inferior utilizando como metodologia a apresentação de um caso clínico. Descrição do caso: Paciente de 7 meses, sem antecedentes clínicos ou cirúrgicos de interesse, que foi trazido pela mãe à consulta de cirurgia vascular devido à presença de uma massa no membro inferior direito que apresentava crescimento progressivo, quadro que vem acompanhado de inchaço nos tecidos moles e fricção ou dor à distensão na área da malformação, segundo a classificação de Mulliken e Glowacki, conclui-se que se trata de um Linfangioma, diagnóstico que foi confirmado por Ultrassonografia Doppler, Angiotac e flebografia ascendente. Para seu manejo foi realizada drenagem percutânea guiada por ultrassom e escleroterapia com espuma de polidocanol a 1% para selar a malformação vascular, obtendo melhora clínica com resultados estéticos e funcionais satisfatórios. Conclusão: O linfangioma é uma malformação vascular rara e não documentada localmente, portanto, a apresentação deste caso teve como objetivo fornecer informação científica atualizada sobre o tratamento da patologia vascular e defender uma gestão baseada na evidência médica existente que contribua para resultados favoráveis para a pediatria. pacientes

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RESUMO

Phleboliths are reported as calcifications that occur in vascular malformations, associated with changes in blood flow dynamics, thrombus formation and subsequent calcifications. Radiological examination, such as cone beam computed tomography (CBCT) could help in demonstrating the presence of a calcifiied mass. A 45-year-old male was referred to our service with an asymptomatic nodular purplish lesion located on the ventrolateral tongue. Within the lesion, a stony mass was also evident on palpation. A digital dental radiograph demonstrated two circumscribed radiopaque structures. Phleboliths associated with vascular malformation was the main diagnostic hypothesis. The patient underwent a sclerotherapy protocol allowing surgical accessibility to the area. Phlebolyts were surgically removed using electrocoagulation. Histopathological examination revealed phleboliths in the context of a vascular malformation with intense fibrosis.

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PIK3CA-related overgrowth spectrum (PROS) encompasses different clinical entities caused by somatic activating mutations in PIK3CA. Among PROS, CLOVES syndrome represents a severe phenotype with poor survival rate. We present the case of a 4-month-old girl with CLOVES syndrome successfully treated with alpelisib, a PIKC3A inhibitor.

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Pediatric intracranial arteriovenous shunts are rare vascular malformations that can be diagnosed prenatally or postnatally, as an incidental finding or due to complications. We propose a review of cerebral vascular malformations in newborns and infants with special emphasis on neurosonography and Doppler ultrasound as the first diagnostic method. Sonography can thus contribute in the planning of further studies that are always necessary, and in post-therapy follow-up.

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ABSTRACT Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

RESUMO

ABSTRACT BACKGROUND: Embolization is a promising treatment strategy for cerebral arteriovenous malformations (AVMs). However, consensus regarding the main complications or long-term outcomes of embolization in AVMs remains lacking. OBJECTIVE: To characterize the most prevalent complications and long-term outcomes in patients with AVM undergoing therapeutic embolization. DESIGN AND SETTING: This systematic review was conducted at the Federal University of Alagoas, Arapiraca, Brazil. METHODS: This systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria. Data were obtained from MEDLINE, PubMed, LILACS, and SciELO databases, which included the epidemiological profile of the population, characteristics of the proposed therapy, complications (hemorrhagic events and neurological deficits), and long-term outcomes (modified Rankin scale pre- and post-treatment, AVM recanalization, complete obliteration, and deaths). RESULTS: Overall, the analysis included 34 articles (2,799 patients). Grade III Spetzler-Martin AVMs were observed in 34.2% of cases. Notably, 39.3% of patients underwent embolization combined with radiosurgery. The most frequently reported long-term complication was hemorrhage, which occurred in 8.7% of patients at a mean follow-up period of 58.6 months. Further, 6.3% of patients exhibited neurological deficits after an average of 34.7 months. Complete obliteration was achieved in 51.4% of the cases after a mean period of 36 months. Recanalization of AVMs was observed in 3.5% of patients. Long-term death occurred in 4.0% of patients. CONCLUSION: Embolization of AVMs is an increasingly safe strategy with low long-term complications and satisfactory outcomes, especially in patients who have undergone combination therapies. SYSTEMATIC REVIEW REGISTRATION: https://www.crd.york.ac.uk/prospero/ Registration number CRD42020204867.

RESUMO

We present the case of a patient with venous thromboembolic disease and contraindication to anticoagulation, where the incidental finding of a duplication of the inferior vena cava was made. This observation determined the need to implant two embolic protection filters. Although this vascular anomaly is rarely present, it is important to keep this possibility in mind to ensure that proper protection is provided when inferior vena cava filters are implanted.

Se presenta el caso de un paciente con enfermedad tromboembólica venosa y contraindicación de anticoagulación en el cual se halló incidentalmente una duplicación de vena cava inferior, situación que determinó la necesidad del implante de dos filtros de protección embólica. Si bien esta anomalía vascular es de escasa presentación, es importante tener presente esta posibilidad para asegurarse de brindar una correcta protección a la hora del implante de filtros de vena cava inferior.

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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.

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Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

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El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

Assuntos

Assuntos

RESUMO

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Assuntos

RESUMO

Congenital portosystemic shunt is a venous vascular abnormality that connects portal and systemic circulation, resulting in diversion of the blood flow, bypassing the hepatic passage. It is a rare malformation; its incidence varies from 1:30 000 to 1:50 000 newborns. It may be asymptomatic or present with complications in the pediatric age or, less frequently, in the neonatal age. Upon diagnosis, the need for a surgical or an intravascular intervention for closure should be defined. This decision depends on the malformation anatomical characteristics, clinical manifestations, and complications. We present the case of a 1-month-old patient referred to our center for the study of neonatal cholestatic hepatitis, with a diagnosis of extrahepatic portosystemic shunt. Intravascular closure of the defect was performed with significant subsequent improvement.

El shunt portosistémico congénito es una anomalía vascular venosa que comunica circulación portal y sistémica, por la que se deriva el flujo sanguíneo, salteando el paso hepático. Es una entidad poco frecuente, cuya incidencia varía entre 1/30 000 y 1/50 000 recién nacidos. Puede cursar de forma asintomática o presentarse con complicaciones en la edad pediátrica o, menos frecuente, en la edad neonatal. Ante el diagnóstico, se deberá definir la necesidad de intervención quirúrgica o intravascular para el cierre. Esta decisión depende de las características anatómicas de la malformación, de las manifestaciones clínicas y complicaciones presentes. Se presenta el caso de un paciente de un mes de vida derivado a nuestro centro para estudio de hepatitis colestásica neonatal, con diagnóstico de shunt portosistémico extrahepático. Se realizó cierre intravascular de la lesión con mejoría significativa posterior.

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RESUMO

El hemangiolinfangioma es un tipo muy raro de malformación del sistema vascular, caracterizado histológicamente por la presencia de vasos venosos y linfáticos dilatados quísticamente, cuyas células endoteliales de revestimiento son positivas para marcadores de inmunohistoquímica como CD31, CD34 y D2-40. El compromiso extenso retroperitoneal y del tracto gastrointestinal es infrecuente. Se presenta el caso de una paciente femenina de 24 años con antecedente de dolor pélvico crónico, con exacerbación de síntomas. El diagnóstico imagenológico mostró una masa retroperitoneal multiquística. Se hizo hemicolectomía derecha y resección de la masa, encontrándose que dicha lesión estaba íntimamente adherida al mesenterio con compromiso extenso del tracto gastrointestinal, y cuyo estudio histopatológico reveló un hemangiolinfangioma, con mejoría clínica posterior a la resección quirúrgica. Aportamos a la literatura mundial, la caracterización de los hallazgos clínicos, imagenológicos e histopatológicos de este tipo de malformaciones

Hemangiolymphangioma is a very rare type of malformation of the vascular system, characterized histologically by the presence of cystically dilated venous and lymphatic vessels, whose lining endothelial cells are positive for immunohistochemical markers such as CD31, CD34 and D2-40. Extensive retroperitoneal and gastrointestinal tract involvement is uncommon. We present the case of a 24-yearold female patient with a history of chronic pelvic pain with exacerbation of symptoms. The imaging diagnosis revealed a multicystic retroperitoneal mass. A right hemicolectomy and resection of the mass was performed, finding that the lesion was intimately adherent to the mesentery with extensive involvement of the gastrointestinal tract, and whose histopathological study revealed a hemangiolymphangioma, with clinical improvement after surgical resection. We contribute to the world literature with the characterization of the clinical, imaging and histopathological findings of this type of malformations

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RESUMO

Vascular malformations are frequent in the head and neck region, affecting the nervous system. The wide range of therapeutic approaches demand the correct anatomical, morphological, and functional characterization of these lesions supported by imaging. Using a systematic search protocol in PubMed, Google Scholar, Ebsco, Redalyc, and SciELO, the authors extracted clinical studies, review articles, book chapters, and case reports that provided information about vascular cerebral malformations, in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 385,614 articles were grouped; using the inclusion and exclusion criteria, three of the authors independently selected 51 articles about five vascular cerebral malformations: venous malformation, brain capillary telangiectasia, brain cavernous angiomas, arteriovenous malformation, and leptomeningeal angiomatosis as part of Sturge-Weber syndrome. We described the next topics-"definition", "etiology", "pathophysiology", and "treatment"-with a focus on the relationship with the imaging approach. We concluded that the correct anatomical, morphological, and functional characterization of cerebral vascular malformations by means of various imaging studies is highly relevant in determining the therapeutic approach, and that new lines of therapeutic approaches continue to depend on the imaging evaluation of these lesions.

RESUMO

Spinal arteriovenous shunts (sAVSs) are an uncommon disease, constituting 3 to 4% of intradural lesions; 70% of these lesions are spinal arteriovenous fistulas (sAVFs), whereas spinal arteriovenous malformations (sAVMs) are rarer. Both share the problem of their classification due to the heterogeneity of their angioarchitecture. The aim of this study is to report a series of sAVSs treated in the neurosurgery department of the Hospital Nacional Guillermo Almenara during the 2018-2020 period and to present an overview of the current literature on sAVS classification. We reviewed all medical records of patients diagnosed with sAVFs and sAVMs during the 2018-2020 period, and then we analyzed images with Horos v4.0.0, illustrated some cases with Clip Studio Paint v1.10.5, and performed a descriptive statistical analysis with SPSS v25. Twelve patients were included in this study, eight of which were women (67%) and four of which were men (33%); the age range was from 3 to 74 years. Eight sAVSs were sAVFs, whereas four were sAVMs. The most frequent clinical manifestation was chronic myelopathy in seven patients (58%). Of those treated only by embolization, seven (70%) resulted in complete occlusion (five sAVFs and two sAVMs), while three (30%) remained with a residual lesion. At last follow-up, five patients (42%) improved clinically, and the seven remaining (58%) maintained the same neurological state. sAVSs require a detailed study of their angioarchitecture for proper management. The endovascular treatment is safe with acceptable cure rates. The surgical option should not be set aside.

RESUMO

RESUMEN Los estudios imagenológicos permiten evaluar de forma global la cavidad abdominal. Las malformaciones vasculares intraabdominales son entidades poco frecuentes, la mayoría cursa de forma asintomática. Se expone el uso de los medios diagnóstico de imágenes en la detección de una malformación arterio-venosa asintomática de páncreas, en paciente con otra sintomatología. Paciente de 31años, kenyana, que acude a la emergencia del Hospital Cubano de Catar, aquejando dolor en bajo vientre y fosa iliaca derecha (FID), reacción peritoneal, leucograma negativo y proteína C reactiva elevada. Se descartaron patologías ginecológicas, gastrointestinales y urinarias. Se indicó ultrasonido y posteriormente TAC con contraste endovenoso (EV) abdomino-pélvico, que demostró mioma uterino sub-seroso e imagen tumoral hiper-vascularizada en proceso uncinado de páncreas, compatible con malformación arterio-venosa. Fue remitida a las especialidades de ginecología, cirugía y gastroenterología. Sugerimos tener siempre presente enfermedades poco frecuentes en el curso de patologías comunes. Publicar imágenes de patologías raras, ofrece patrón de comparación para futuros diagnósticos.

ABSTRACT Imaging studies allow a global assessment of the abdominal cavity. Intra-abdominal vascular malformations are rare entities, most of which are asymptomatic. The use of diagnostic imaging technique in the detection of an asymptomatic arterio-venous malformation of the pancreas in a patient with other symptoms is exposed. 31-year-old Kenyan patient who came to the emergency department at the Cuban Hospital in Qatar with pain in the lower abdomen and right iliac fossa (RIF), peritoneal reaction, negative leukogram and elevated C-reactive protein. Gynecological, gastrointestinal and urinary pathologies were ruled out. An ultrasound and later CT abdominal-pelvic with intravenous contrast (IV) were indicated, which demonstrated subserous uterine myoma and hyper-vascularized tumor image in the uncinate process of pancreas, compatible with arterio-venous malformation. She was referred to the specialties of gynecology, surgery and gastroenterology. We suggest always keeping in mind rare diseases during common pathologies. Publish images of rare pathologies, offers a comparison pattern for future diagnoses.

RESUMO Estudos de imagem permitem a avaliação geral da cavidade abdominal. Malformações vasculares intra-abdominais são raras, a maioria das quais ocorrem assintomáticamente. O uso de imagens de diagnóstico significa na detecção de uma malformação arterio-venosa assintomática do pâncreas em pacientes com outros sintomas. Um paciente queniano de 31 anos, que foiao pronto-socorro do Hospital Cubano do Catar, sofrendo de dorna barriga inferior e fossa ilínica direita (FID), reação peritoneal, leucograma negativo e proteína C-reativa elevada. Foram descartadas patologias ginecológicas, gastrointestinais e urinárias. Foram indicadas ultrassonografia e tomografia computadorizada com contraste intravenoso abdomino-pélvico (EV), que demonstrou imagem de tumor uterino sub-seroso e hiper-vascularizada em processonãocinado do pâncreas, compatível com malformação arterio-venosa. Ela foi encaminhada para as especialidades de ginecologia, cirurgia e gastroenterologia. Sugerimos sempre ter em mente doenças raras no curso de patologias comuns. A publicação de imagens de patologias raras oferece um padrão de comparação para diagnósticos futuros.