Manejo de Linfangioma en la edad pediátrica: presentación de caso

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.

Manejo de Linfangioma en la edad pediátrica: presentación de caso / Management of Lymphangioma in pediatric age: case presentation / Manejo do Linfangioma em idade pediátrica: apresentação de caso

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

Prenatal ultrasound diagnosis of isolated persistent vitelline artery: a case report and literature review / 中华围产医学杂志

Objective:To summarize the ultrasonographic features and prognosis of fetal persistent vitelline artery.Methods:The prenatal ultrasound features, genetic testing results, and prognosis of a fetus with an isolated persistent vitelline artery that was diagnosed in our hospital in December 2021 were retrospectively analyzed. Relevant articles were retrieved from CNKI, VIP, Wanfang, Yiigle, PubMed, Embase, and UpToDate databases using the terms "persistent vitelline artery", "type Ⅱ single umbilical artery", and "prenatal ultrasound" in both Chinese and English. Prenatal ultrasound features and prognosis of the persistent vitelline artery in fetuses were summarized using descriptive statistical analysis.Results:(1) Case report: In this case, ultrasound at 23 gestational weeks showed that an abnormally large blood vessel deriving from the celiac artery near the superior mesenteric artery entered the placenta through the umbilical opening in parallel with the umbilical vein. Color Doppler showed a blood flow spectrum like that in the umbilical artery. The transverse section image showed that bilateral umbilical arteries were not observed in the bladder and the free segment of the umbilical cord was in the shape of the Chinese character "Lyu". No obvious other structural abnormalities and a negative result of genetic testing were observed in the fetus. Followed up to one year old, the patient showed normal growth and development. (2) Literature review: A total of five articles involving four cases were retrieved (three in English and two in Chinese). Among the five cases, including the present case, one was terminated due to left renal agenesis and abnormal heart arteries ratio revealed by prenatal ultrasound, and the remaining four cases without obvious structural abnormalities in the prenatal ultrasound were born and developed well. Histopathological examination of the umbilical cord was performed in three cases, of which two with persistent vitelline artery had a distinct internal elastic lamina, and one with remained vitelline duct.Conclusions:The prenatal ultrasound of fetal persistent vitelline artery is typically characterized by an abnormal vessel that is derived from the abdominal aorta or superior mesenteric artery and plays the function of the umbilical artery. The prognosis of the isolated persistent vitelline artery is good, but a better understanding of such embryonic abnormalities is needed as there are few relevant reports at home and abroad.

Research progresses of clinical and imaging evaluation on efficacy of sclerotherapy for venous malformations / 中国介入影像与治疗学

Venous malformation(VM)is the most common congenital vascular malformation.Clinical symptoms of VM,including pain,swelling,activity limitation and bleeding,mainly depend on the extent and location of VM.The treatment methods of VM included sclerotherapy,surgery and laser therapy,and sclerotherapy was regarded as the first-line plan.Up till now,no unified method or standard had been established for evaluating the efficacy of sclerotherapy for VM.The research progresses of clinical and imaging evaluation on efficacy of sclerotherapy for VM were reviewed in this article.

Long-term complications and outcomes of therapeutic embolization of cerebral arteriovenous malformations: a systematic review

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.

Biological identity of orbital cavernous venous malformations

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.

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.

Histological analysis of different types of port-wine stains to guide clinical decision making: A retrospective study

Background and objectives: Port-wine stains are defined as congenital benign vascular lesions. The treatment of port-wine stains remains a challenge, worldwide. This study aimed to analyze the histological characteristics in different types of port-wine stains and provide guidance for clinical decision-making. Methods and materials: Biopsies were from the hospital from 2015 to 2021. H&E staining, Immunofluorescence staining, Masson’s trichrome staining and Weigert staining were performed on the tissues. Results: A total of 35 port-wine stains patients were included in the study of four distinct types, namely red port-wine stains (11 cases), purple port-wine stains (seven cases), hypertrophic port-wine stains (nine cases) and nodular port-wine stains (eight cases). The mean vessel diameter of the different types was 38.7 ± 5.9 ?m, 93.5 ± 9.7 ?m, 155.6 ± 21.8 ?m and 155.6 ± 29.54 ?m, respectively. Mean vessel depth was 396.4 ± 31 ?m, 944.2 ± 105.4 ?m, 2,971 ± 161.3 ?m and 3,594 ± 364.6 ?m, respectively. The vessels in red port-wine stains, purple port-wine stains and hypertrophic port-wine stains were mainly composed of capillary and venous malformations, whereas those in nodular port-wine stains were venous or arteriovenous malformations. Limitation: The main limitation of the current study was the small number of patients. Conclusion: As the disease progresses, vessel diameters become larger, the vessel wall becomes thicker and vessels were found in a greater depth. A treatment plan should be scientifically formulated keeping in mind the histological characteristics of port-wine stains.

Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies 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.

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.

Shunt portosistémico, una causa poco frecuente de hepatitis colestásica en neonatología. A propósito de un caso / Portosystemic shunt, a rare cause of neonatal cholestatic hepatitis. A case report

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.

Prenatal diagnosis and pregnancy outcome of anomalous origin of one pulmonary artery branch from the aorta: analysis of 14 cases / 中华围产医学杂志

Objective:To summarize the prenatal diagnosis features, classification and pregnancy outcome of anomalous origin of one pulmonary artery branch from the aorta (AOPA).Methods:This study involved 14 cases who were prenatally diagnosed with AOPA in Guangzhou Women and Children's Medical Center between June 2016 and August 2022. Prenatal and postnatal echocardiographic features, postpartum diagnosis, surgical treatment and pregnancy outcome in these cases were summarized and analyzed by descriptive analysis.Results:Out of the 14 fetuses, there were seven fetuses with proximal-type AOPA (including three isolated AOPA, three Berry syndrome and one with interruption of the aortic arch, aorticopulmonary septal defect and ventricular septal defect) and another seven with isolated distal-type of AOPA. Among the seven cases of proximal-type AOPA, two were terminated and five were born alive. The postpartum diagnosis was consistent with the prenatal diagnosis in the five babies who later underwent surgical treatment with good outcomes. Among the seven cases of distal-type AOPA, one was terminated; two were initially diagnosed as AOPA in the neonatal period but then as unilateral absence of pulmonary artery (UAPA) due to tapering or closure of the ductus arteriosus during follow-up; the other four were confirmed with UAPA after delivery. All of the six neonates underwent surgical treatment with good outcomes.Conclusions:Prenatal diagnosis and classification of AOPA should be as accurate as possible. It is recommended that the distal-type of AOPA could be diagnosed as UAPA after delivery and treated according to UAPA. Both kinds of patients should be treated with surgery timely after delivery to ensure a good prognosis.

Medial tentorial dural arteriovenous fistula with venous sinus thrombosis leading to thalamic dementia: a challenging diagnosis of 4 cases / 国际脑血管病杂志

Objective:To investigate the imaging characteristics and diagnosis of thalamic dementia caused by medial tentorial dural arteriovenous fistula (TDAVF).Methods:The clinical and imaging data of 4 patients with medial TDAVF diagnosed at Nanjing Brain Hospital from August 2022 to September 2023 were retrospectively collected, summarized and analyzed.Results:Four patients were all males, with an average age of 67 years. The duration of cognitive impairment varies from 13 days to 2 months, with an average of about 1 month. The average score on the Mini-Mental State Examination (MMSE) was 13, while the average score on the Montreal Cognitive Assessment (MoCA) was 15. One patient was unable to complete these tests. MRI showed bilateral thalamic swelling, with scattered microbleeding lesions on diffusion-weighted imaging and susceptibility-weighted imaging ( n=3). Enhanced scans showed patchy or clumpy enhancement of the bilateral thalamus. Magnetic resonance angiography showed abnormal blood vessels along the straight sinus area ( n=4), while magnetic resonance venography showed no straight sinus development ( n=4). Magnetic resonance spectroscopy showed neuronal swelling and damage ( n=3), with no increase in choline peak. Arterial spin labeling perfusion imaging showed decreased perfusion in the lesions. Digital subtraction angiography confirmed TDAVF with straight sinus thrombosis. Two patients underwent embolization, with significant improvement in postoperative memory impairment. One patient underwent surgical clipping, while the other was transferred to other hospital for treatment. Conclusions:Patients with medial TDAVF often present with thalamic dementia. Imaging examinations show typical bilateral thalamic swelling with microbleeds, and early visualization of vascular shadows, without development of straight sinus. These features would be beneficial for the early diagnosis of TDAVF.

Research progress on targeted drugs for vascular malformations / 口腔疾病防治

@#Vascular malformations, which mainly occur in the head and neck region, are a group of congenital disorders that cannot involute and dilate gradually as patients grow. Traditional therapeutic strategies for vascular malformations include laser therapy, sclerotherapy, interventional embolization, surgical resection, etc. However, for some cases with a relatively larger range of lesions, traditional therapeutic strategies might fall short of the goals. With the development of molecular genetics, gene mutations are currently recognized as the root cause of the occurrence of vascular malformations. The progression of vascular malformation lesions is further promoted by the activation of related pathways. Low-flow vascular malformations mainly involve activation of the phosphatidylinositol 3-kinase (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway, whereas high-flow vascular malformations mainly involve activation of the rat sarcoma (RAS)/rapidly accelerated fibrosarcoma (RAF)/mitogen-activated protein kinase kinase (MAPKK)/extracellular-signal regulated protein kinase (ERK) pathway. Targeted drugs against relevant gene mutations and signaling pathways have also been applied in the treatment of vascular malformations, and previous studies have shown that the mTOR inhibitor rapamycin is effective and now widely used in the treatment of low-flow vascular malformations. The PI3K inhibitor alpelisib is also promising in the treatment of venous malformations, and the MAPKK inhibitor trametinib has shown good results in the treatment of arteriovenous malformations. Therefore, traditional therapies supplemented by targeted drugs may bring new breakthroughs to the treatment of vascular malformations.

Approach to clinically significant vascular anomalies in children

Vascular anomalies consist of tumours or malformations made up of abnormal growth or collections of blood vessels that can result in functional or cosmetic problems. While many vascular anomalies are present at birth, some do not appear until later in life, making diagnosis more challenging. Although many vascular anomalies are benign, some are associated with serious complications and may involve multiple organ systems. This article highlights the important features of clinically significant vascular anomalies to help physicians promptly identify and refer these cases to a specialised multidisciplinary team for evaluation and management. The discussion includes the various presenting complaints of vascular anomalies in children, namely, rapidly growing birthmarks, painful lesions, seizures/neurological manifestations, bleeding diathesis, cardiac/airway abnormalities and part of an overgrowth syndrome.

Therapeutic effect of endovascular embolization for perimedullary arteriovenous fistula / 中国介入影像与治疗学

Objective To observe the therapeutic effect of endovascular embolization for perimedullary arteriovenous fistula(PMAVF).Methods Data of 8 PMAVF patients who underwent endovascular embolization were retrospectively analyzed.Findings of digital subtraction angiography(DSA)immediately and 6 months after treatment were reviewed,and the modified Aminoff-Logue score(mALS)was compared before and 6 months after treatment.Results Among 8 cases of PMAVF,there were 2 cases of type Ⅰ,5 cases of type Ⅱand 1 case of type Ⅲ,among them 3 were treated with Onyx glue while 5 with Glubrane glue.The immediate DSA results after treatment showed complete embolization of the fistula in all 8 cases,while those 6 months after treatment showed no recurrence.One case recovered and the other 7 cases improved,and mALS(2.25±2.12)6 months after treatment was lower than that(5.50±2.39)before treatment(P＜0.05).Conclusion Endovascular embolization could safely and effectively treat PMAVF.

Hemangiolinfangioma mesentérico: reporte de un caso / Mesenteric hemangiolymphangioma: case report

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

Diagnóstico Incidental de Malformación Vascular Pancreática en paciente con dolor abdominal. Caso Clínico-Radiológico / Incidental Diagnosis of Pancreatic Vascular Malformation in patients with abdominal pain. Clinical-Radiological Case / Diagnóstico Incidental de Malformação Vascular Pancreática em pacientes com dor abdominal. Caso Clínico-Radiológico

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.

Escleroterapia no tratamento de lesões vasculares orais / Escleroterapia en el tratamiento de las lesiones vasculares orales / Sclerotherapy in the treatment of oral vascular injuries

Objetivo: apresentar um caso de lesão vascular em paciente idoso tratado pela técnica da escleroterapia. Relato do caso: paciente masculino, 67 anos, vítima de acidente vascular cerebral, buscou atendimento odontológico por apresentar raízes residuais. Durante o exame físico foi identificada lesão exofítica, de coloração violácea, base séssil, com aproximadamente dois centímetros, localizada em comissura labial esquerda. Para confirmar a origem da alteração foi realizada manobra semiotécnica (diascopia) que revelou tratar-se de lesão vascular. Por ser o paciente idoso, hipertenso, com histórico de acidente vascular cerebral, foi feita a opção por tratamento conservador, sendo realizada a escleroterapia com oleato de monoetanolamina 5%, numa única sessão. No retorno de sete dias, foi observada regressão parcial da lesão e com 30 dias a região se mostrou completamente cicatrizada, sem vestígios da alteração. O oleato de monoetanolamina provoca uma reação inflamatória estéril, aguda, dose-dependente, no endotélio vascular e nos tecidos extravasculares que resulta em fibrose e obliteração dos vasos sanguíneos, induzindo a regressão das lesões. Conclusão: Com base no caso apresentado e nos registros da literatura é possível afirmar que a escleroterapia é uma alternativa terapêutica minimamente invasiva, eficaz, de baixo custo e com resultado estético favorável no tratamento de lesões vasculares orais... (AU)

Objective: to present a case of vascular injury in an elderly patient treated by the sclerotherapy technique. Case report: male patient, 67 years old, victim of a stroke, sought dental care due to residual dental roots. During the physical examination, an exophytic lesion, violet in color, sessile base, approximately two centimeters, located in the left labial commissure, was identified. Diascopy was performed to confirm the origin of the alteration, which revealed that it was a vascular le sion. As the patient was elderly, hypertensive, with a history of stroke, conservative treatment was chosen, with sclerotherapy with 5% mon oethanolamine oleate in a single session. On return after seven days, partial regression of the lesion was observed and, after 30 days, the region was completely healed, with no traces of the alteration. Mon oethanolamine oleate causes a sterile, acute, dose-dependent inflam matory reaction in the vascular endothelium and extravascular tissues that results in fibrosis and obliteration of blood vessels, inducing re gression of the lesions. Conclusion: Based on the case presented and on the literature records, it is possible to affirm that sclerotherapy is a minimally invasive, effective, low-cost therapeutic alternative with a favorable aesthetic result in the treatment of oral vascular lesions... (AU)

Objetivo: presentar un caso de lesión vascular en un paciente de edad avanzada, tratado mediante la técnica de escleroterapia. Reporte de caso: paciente masculino, 67 años, víctima de un derrame cerebral, buscó atención odontológica por raíces dentarias residuales. Durante el examen físico se identificó una lesión exofítica, de color violeta, de base sésil, de aproximadamente dos centímetros, ubicada en la comisura labial izquierda. Se realizó diascopia para confirmar el origen de la alteración, que reveló que se trataba de una lesión vascular. Como el paciente era anciano, hipertenso, con antecedentes de ictus, se optó por tratamiento conservador, con escleroterapia con oleato de monoetanolamina al 5% en una sola sesión. Al regreso a los siete días se observó una regresión parcial de la lesión y, a los 30 días, la región estaba completamente curada, sin rastros de la alteración. El oleato de monoetanolamina provoca una reacción inflamatoria estéril, aguda y dependiente de la dosis en el endotelio vascular y los tejidos extravasculares que produce fibrosis y obliteración de los vasos sanguíneos, lo que induce la regresión de las lesiones. Conclusión: Con base en el caso presentado y en los registros de la literatura, es posible afirmar que la escleroterapia es una alternativa terapéutica mínimamente invasiva, efectiva, de bajo costo y con resultado estético favorable en el tratamiento de las lesiones vasculares orales... (AU)

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.