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1.
Rev. baiana saúde pública ; 45(Supl. Especial 2): 118-129, 2021/12/28.
Article in Portuguese | LILACS | ID: biblio-1352341

ABSTRACT

A síndrome de Rendu-Osler-Weber, ou telangiectasia hemorrágica hereditária (THH), é uma doença autossômica dominante de penetrância variável que altera a camada muscular e elástica da parede dos vasos sanguíneos, causando neles grande fragilidade e dilatação e tornando-os suscetíveis a sangramentos secundários, a traumatismos e malformações arteriovenosas (MAV) ­ sendo elas as mais comuns no cérebro, nos pulmões e no trato gastrointestinal e hepático. Este relato de caso tem como objetivo realizar uma revisão sobre essa síndrome, bem como a respeito da melhor terapia e do melhor manejo para sangramentos gastrointestinais por angiectasias. Trata-se de uma paciente de 62 anos de idade, com episódio de hematêmese e melena recorrentes, que foi admitida instável hemodinamicamente e com queda importante dos níveis de hemoglobina, necessitando de múltiplas transfusões sanguíneas. Após entrevista clínica, que chamou a atenção para o histórico familiar de quadros similares, epistaxes recorrentes associados ao exame físico e angiectasias gástricas à endoscopia digestiva alta, foi atribuído o diagnóstico de síndrome de Rendu-Osler-Weber. Angiectasias foram erradicadas com terapia endoscópica usando plasma de argônio. A THH é uma doença subdiagnosticada que merece atenção especial por conta do seu risco de causar hemorragias graves com expressão fenotípica mais aflorada com o avançar da idade. Por seu cunho hereditário, faz-se necessário um acompanhamento médico linear com a família para evitar complicações secundárias da doença, bem como para o manejo adequado de suas apresentações.


Osler-Weber-Rendu syndrome or hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that changes the muscular and elastic layer of the blood vessel wall, making them fragile and more susceptible to bleeding secondary to trauma and arteriovenous malformations (AVM) ­ a condition that commonly affects the brain, lungs, gastrointestinal tract, and liver. This study aims to perform a literature review on this syndrome, as well as on the best treatment and management for gastrointestinal bleeding from angioctasia. To this end, it reports the case of a 62-year-old female patient with recurrent episodes of hematemesis and melena, who was admitted to hospital with hemodynamic instability and low hemoglobin levels, requiring multiple blood transfusions. After clinical interview, which indicated family history of recurrent epistaxis, physical examination, and upper digestive endoscopy showing gastric angiectasias, the patient was diagnosed with Osler-Weber-Rendu syndrome. Angiectasias were treated with endoscopic argon plasma. Considering the risk of severe hemorrhages and its more pronounced phenotypic expression with advancing age, HHT is an underdiagnosed disease that deserves special attention. Due to its hereditary nature, a linear medical follow-up with the family is necessary to avoid secondary complications of the disease, as well as the proper management of its presentations.


El síndrome de Rendu-Osler-Weber o telangiectasia hemorrágica hereditaria (THH) es una enfermedad autosómica dominante de penetrancia variable, que modifica la capa muscular y elástica de la pared de los vasos sanguíneos, provocando una gran fragilidad y dilatación de estas, haciéndolas susceptibles a hemorragias secundarias a trauma y malformaciones arteriovenosas (MAV), que son las más comunes en el cerebro, pulmones, tracto gastrointestinal e hígado. Este estudio tiene como objetivo realizar una revisión de este síndrome, así como la mejor terapia y manejo del sangrado gastrointestinal por angioctasia en esta patología. Se trata de un paciente de 62 años, con episodio de hematemesis recurrente y melena, que ingresa hemodinámicamente inestable y con descenso significativo de los niveles de hemoglobina, requiriendo múltiples transfusiones sanguíneas. Tras entrevista clínica, destacando los antecedentes familiares de patologías similares, epistaxis recurrente, exploración física y angiectasias gástricas a endoscopia digestiva alta, se asignó el diagnóstico de síndrome de Rendu-Osler-Weber. Las angiectasias se erradicaron con terapia endoscópica con plasma de argón. La HHT es una enfermedad infradiagnosticada que merece una atención especial por su riesgo de hemorragias severas, con una expresión fenotípica más pronunciada con el avance de la edad. Además de su carácter hereditario, es necesario un seguimiento médico lineal con la familia para evitar complicaciones secundarias de la enfermedad, así como el adecuado manejo de sus presentaciones.


Subject(s)
Humans , Arteriovenous Malformations , Telangiectasia, Hereditary Hemorrhagic , Blood Vessels , Gastrointestinal Tract
3.
Arch. argent. pediatr ; 119(3): 152-161, Junio 2021. tab, ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1222429

ABSTRACT

Introducción. Las malformaciones vasculares cerebrales de alto flujo son poco comunes en la edad pediátrica. El objetivo del trabajo es diferenciar y agrupar estas enfermedades según edad de debut, manifestaciones clínicas y angioarquitectura.Población y método. Se realizó un estudio retrospectivo y observacional. Se analizaron las historias clínicas, los estudios por imágenes y los protocolos de procedimientos de pacientes del Hospital J. P. Garrahan con diagnóstico de malformaciones vasculares cerebrales desde enero de 2010 hasta enero de 2020.Resultados. Ciento ochenta y tres pacientes cumplieron los criterios de inclusión. Se identificaron 131 pacientes con malformaciones arteriovenosas con nido (MAV) y 52 con fístulas directas (sin nido), entre los que se hallaron 19 malformaciones aneurismáticas de vena de Galeno, 23 fístulas piales y 10 fístulas durales. La edad promedio fue de 105 meses para las MAV, 1,7 meses para las malformaciones aneurismáticas de vena de Galeno, 60,5 meses para fístulas piales y 41 meses para fístulas durales.Conclusión. Según su angioarquitectura, las malformaciones vasculares cerebrales de alto flujo tuvieron nido (MAV) o fueron fístulas directas (malformaciones aneurismáticas de vena de Galeno, fístulas piales y fístulas durales). Las MAV se manifestaron a partir de la primera infancia, sobre todo, por hemorragia intracraneana. Las fístulas directas se expresaron en la primera etapa de la vida, frecuentemente, con insuficiencia cardíaca.


Introduction. High-flow vascular malformations of the brain are uncommon in pediatrics. The objective of this study is to establish the differences among these pathologies and group them by age at onset, clinical manifestations, and angioarchitecture.Population and method. This was a retrospective and observational study. The medical records, imaging studies, and procedure protocols of patients seen at Hospital J. P. Garrahan diagnosed with vascular malformations of the brain between January 2010 and January 2020 were analyzed.Results. A total of 183 patients met the inclusion criteria. It was possible to identify 131 patients with arteriovenous malformations with a nidus (AVMs) and 52 with direct fistulas (without a nidus), including 19 vein of Galen aneurysmal malformations, 23 pial fistulas, and 10 dural fistulas. The average age of patients was 105 months for AVMs, 1.7 months for vein of Galen aneurysmal malformations, 60.5 months for pial fistulas, and 41 months for dural fistulas.Conclusion. Based on their angioarchitecture, high-flow vascular malformations of the brain presented a nidus (AVMs) or direct fistulas (vein of Galen aneurysmal malformations, pial fistulas, and dural fistulas). AVMs were observed in early childhood, especially due to intracranial hemorrhage. Direct fistulas occurred in the first stage of life, commonly with heart failure.


Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Arteriovenous Malformations/therapy , Arteriovenous Malformations/diagnostic imaging , Retrospective Studies , Arteriovenous Fistula/therapy , Arteriovenous Fistula/diagnostic imaging , Intracranial Hemorrhages , Vein of Galen Malformations/therapy , Vein of Galen Malformations/diagnostic imaging , Heart Failure
4.
Rev. bras. ginecol. obstet ; 43(4): 323-328, Apr. 2021. tab, graf
Article in English | LILACS | ID: biblio-1280047

ABSTRACT

Abstract Complete hydatidiform mole (CHM) is a rare type of pregnancy, in which 15 to 20% of the cases may develop into gestational trophoblastic neoplasia (GTN). The diagnostic of GTN must be done as early as possible through weekly surveillance of serum hCG after uterine evacuation.We report the case of 23-year-old primigravida, with CHM but without surveillance of hCG after uterine evacuation. Two months later, the patient presented to the emergency with vaginal bleeding and was referred to the Centro de Doenças Trofoblásticas do Hospital São Paulo. She was diagnosed with high risk GTN stage/score III:7 as per The International Federation of Gynecology and Obstetrics/World Health Organization (FIGO/WHO). The sonographic examination revealed enlarged uterus with a heterogeneous mass constituted of multiple large vessels invading and causing disarrangement of the myometrium. The patient evolved with progressive worsening of vaginal bleeding after chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO) regimen. She underwent blood transfusion and embolization of uterine arteries due to severe vaginal hemorrhage episodes, with complete control of bleeding. The hCG reached a negative value after the third cycle, and there was a complete regression of the anomalous vascularization of the uterus as well as full recovery of the uterine anatomy. The treatment in a reference center was essential for the appropriate management, especially regarding the uterine arteries embolization trough percutaneous femoral


Resumo Mola hidatiforme completa (MHC) é um tipo raro de gravidez, na qual 15 a 20% dos casos podem desenvolver neoplasia trofoblástica gestacional (NTG). O diagnóstico de NTG deve ser feito o mais cedo possível, pelo monitoramento semanal do hCG sérico após esvaziamento uterino. Relatamos o caso de uma paciente primigesta, de 23 anos de idade, com MHC, sem vigilância de hCG após esvaziamento uterino. Dois meses depois, a paciente compareceu na emergência com sangramento vaginal, sendo encaminhada ao Centro de Doenças Trofoblásticas do Hospital São Paulo, onde foi diagnosticada com NTG de alto risco, estádio e score de risco III:7 de acordo com a The International Federation of Gynecology and Obstetrics/Organização Mundial de Saúde (FIGO/OMS). O exame ultrassonográfico revelou útero aumentado com uma massa heterogênea constituída pormúltiplos vasos volumosos invadindo e desestruturando o miométrio. A paciente evoluiu com piora progressiva do sangramento vaginal após quimioterapia com o regime etoposide, methotrexate, actinomycin D, cyclophosphamide and vincristine (EMA-CO). Ela foi submetida a transfusão de sangue e embolização das artérias uterinas devido aos episódios graves de hemorragia vaginal, com completo controle do sangramento. O hCG atingiu valor negativo após o terceiro ciclo, havendo regressão completa da vascularização uterina anômala, assim como recuperação da anatomia uterina. O tratamento em um centro de referência permitiu o manejo adequado, principalmente no que se refere à embolização das artérias uterinas através da punção percutânea da artéria femoral, que foi crucial para evitar a histerectomia, permitindo a cura da NTG e a manutenção da vida reprodutiva.


Subject(s)
Humans , Female , Pregnancy , Young Adult , Arteriovenous Malformations/complications , Uterine Hemorrhage/therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Gestational Trophoblastic Disease/complications , Gestational Trophoblastic Disease/drug therapy , Embolization, Therapeutic , Uterine Hemorrhage/etiology , Uterine Hemorrhage/diagnostic imaging , Vincristine/therapeutic use , Methotrexate/therapeutic use , Ultrasonography, Prenatal , Pregnancy, High-Risk , Cyclophosphamide/therapeutic use , Dactinomycin/therapeutic use , Gestational Trophoblastic Disease/diagnostic imaging , Etoposide/therapeutic use , Uterine Artery
5.
Article in Chinese | WPRIM | ID: wpr-879848

ABSTRACT

A girl, aged 15 years, was admitted due to sudden convulsion once and multiple pulmonary nodules on lung CT. Acrocyanosis or acropachy/toe deformity was not observed. Laboratory examinations showed an increase in hemoglobin (162 g/L) and a reduction in arterial partial pressure of oxygen (61.5 mm Hg). Lung CT showed irregular slightly high-density nodules in the middle lobe of the right lung, and contrast-enhanced CT scan showed obvious enhancement with thick vascular shadow locally. An investigation of medical history revealed that the girl's mother had a history of epistaxis and resection of pulmonary mass and the girl presented with tongue telangiectasia. The girl was diagnosed with hereditary hemorrhagic telangiectasia and pulmonary arteriovenous malformation. she was given interventional embolization therapy. Transcutaneous oxygen saturation reached 98% without oxygen inhalation on the day after surgery. Pulmonary angiography at 3 months after surgery showed the recurrence of pulmonary vascular malformation, and embolization of pulmonary arterial fistula was not performed since the guide wire could not enter the branch artery. There was still a need for long-term follow-up.


Subject(s)
Adolescent , Arteriovenous Fistula , Arteriovenous Malformations , Female , Humans , Multiple Pulmonary Nodules , Neoplasm Recurrence, Local , Pulmonary Artery/diagnostic imaging , Seizures
6.
Autops. Case Rep ; 10(3): e2020144, 2020. graf
Article in English | LILACS | ID: biblio-1131832

ABSTRACT

Arteriovenous malformation (AVM) is a rare lesion in the uterus, which can lead to abnormal uterine bleeding. While AVM has been described in other organs in the literature, there is a paucity of pathology reports of the AVM in uterus. On gross examination, the uterus was markedly enlarged and partly distorted with a pedunculated solid mass, which on the cut surface showed multiple well-circumscribed hemorrhagic cysts ranging from 0.1 to 4.0 cm in size. Microscopically, they were malformed dilated vascular structures containing organized thrombi. We present this case of uterine AVM with gross and microscopic findings, which can serve as a crucial reminder for pathologists to keep in the differential diagnoses as a potential cause of abnormal uterine bleeding.


Subject(s)
Humans , Female , Pregnancy , Middle Aged , Arteriovenous Malformations/pathology , Uterine Hemorrhage/etiology , Uterus/injuries , Cysts/etiology , Diagnosis, Differential
7.
West Afr. j. radiol ; 27(2): 81-88, 2020. ilus
Article in English | AIM, AIM | ID: biblio-1273556

ABSTRACT

Background: Lately, there has been an increased utilization of computed tomography angiography (CTA) as the preferred first-line modality for the evaluation and diagnosis of most cerebral vascular lesions.Objective: The objective of this study was to evaluate suspected intracranial vascular cases, using CTA at a major referral tertiary hospital in South West Nigeria.Materials and Methods: This was a hospital-based retrospective study of suspected intracranial vascular cases in all ages and both sexes that had CTA from January 2011 to December 2018. Data were analyzed with IBM SPSS version 23.0, and P < 0.005 was considered statistically significant.Results: A total of 128 patients were studied, the mean age was 44.1 ± 17.7 years, and male: female ratio was 1:1.06. The leading clinical diagnoses were as follows: intracranial aneurysms (34/128), subarachnoid hemorrhage (27/128), intracranial vascular tumors (26/128), brain hemorrhage from vascular abnormality (19/128), and arteriovenous malformations (AVMs) (10/128). At CTA, 61 patients had vascular abnormalities: intracranial aneurysm was seen in 63.9% with a peak age range of 41­60 years, and the leading location of aneurysms was posterior cerebral artery (18.8%), followed by posterior communicating artery (16.7%) and the cavernous segment of the internal carotid artery (16.7%). AVMs were more common in patients aged 40 years and below (91.7%) in males (66.7%) and in the parietal lobe. Intracranial aneurysms were 3.25 times as common as brain AVMs.Conclusion: Intracranial aneurysms are the predominant vascular lesions, occurring mostly in the older age group. AVMs occurred mostly in younger people, more in males, and predominantly in the parietal lobes. The hospital incidence of aneurysms to AVMs was 3.25:1


Subject(s)
Aneurysm , Arteriovenous Malformations , Nigeria , Subarachnoid Hemorrhage , Wounds and Injuries
8.
Article in English | WPRIM | ID: wpr-876630

ABSTRACT

@#Arteriovenous malformations (AVM) are vascular disorders with a mixture of arterial, venous and small capillary-like channels with fistulous connections. Uterine arteriovenous malformations are rare cause of abnormal uterine bleeding with only a few reported cases. They may arise from pregnancy, miscarriage, previous cesarean section or other uterine surgery and gestational trophoblastic disease. Diagnosis can be made through angiography or doppler ultrasonography. Traditionally, uterine AVMs are treated with hysterectomy but with the advances in technology, minimally invasive conservative approaches such as radiologic arterial embolization or laparoscopic uterine artery ligation have become available. We present a case of a 29-year-old, G2P1 (1011) who had a three- month history of heavy, intermittent vaginal bleeding from uterine arteriovenous malformation after a miscarriage. Laparoscopic bilateral uterine artery occlusion, offered a minimally invasive treatment with high symptomatic effectiveness.


Subject(s)
Uterine Artery , Arteriovenous Malformations , Uterine Diseases , Ligation , Uterine Hemorrhage
10.
Rev. argent. neurocir ; 33(1): 26-38, mar. 2019. ilus, tab
Article in Spanish | LILACS, BINACIS | ID: biblio-1177888

ABSTRACT

Introducción: Las malformaciones arteriovenosas (MAVs) cerebrales comprenden una compleja patología responsable de hasta el 38% de las hemorragias en pacientes de entre 15-45 años, acarreando cada episodio de sangrado un 25-50% de morbilidad y un 10-20% de mortalidad. La decisión terapéutica en un paciente con una MAV debe tener en cuenta la comparación entre los riesgos propios de la intervención y los de la historia natural de esta enfermedad. Objetivo: Evaluar la utilidad de predecir riesgo quirúrgico de diferentes escalas de gradación de MAV cerebrales según nuestra experiencia en una serie de casos. Material y Métodos: Se realizó un análisis bibliográfico de escalas de gradación de riesgo quirúrgico de MAV cerebrales utilizando como motor de búsqueda Pubmed incluyendo como palabras clave "malformación arteriovenosa cerebral" y "escala de gradación" (brain arteriovenous and malformation grading scale). Se analizaron de forma retrospectiva aquellos pacientes intervenidos quirúrgicamente por MAV en este hospital público, se las clasificó acorde a las escalas analizadas y se compararon los resultados obtenidos con los previstos en ellas. Resultados: Se analizaron 90 pacientes intervenidos quirúrgicamente por MAV, sin tratamiento coadyuvante. De forma retrospectiva se los agrupó acorde a las escalas de Spetzler Martin (SM), Spetzler-Ponce (SP) y suplementaria de Lawton. Las MAV grado 3 se subclasificaron según las escalas de Lawton y de de Oliveira. Considerando buenos resultados aquellos con Rankin modificado (mRs) igual o menor a 2. Con un rango de seguimiento de 12 a 48 meses, encontramos buenos resultados en el 100% de MAV SM grado 1, 91.7% de las grado 2, 80% en grado 3 y 42.9% en grado 4. Utilizando la escala SP, 93.7% de buenos resultados en tipo A, 80% en tipo B y 42.9% en tipo C. Subclasifican-do las MAV SM 3 acorde a las escalas de de Oliveira y Lawton, 84% de buenos resultados en el tipo 3A, 71.3% en las 3B, 92% en MAV tipo 3-, 72.1% en el tipo 3+, 60% en tipo 3. Utilizando la escala suplementaria de Lawton combinada con SM, buen resultado en 100% grados II y III, 85,7% grado IV, 87,6 grado V, 80% grado VI, 75% grado VII y 66,6% grado VIII. Conclusión: Reafirmamos en esta serie, la utilidad de estimar riesgo quirúrgico con las escalas SM, SP, y la subclasificación de las MAV grado 3 propuesta por Lawton. Y principalmente el utilizar la escala suplementaria de Lawton-Young al considerar el tratamiento quirúrgico de los pacientes con MAV que sangraron.


Introduction: Brain arteriovenous malformations (AVM) are a complex disease responsible for up to 38% of hemorrhages in patients between 15-45 years old, carrying every bleeding episode a 25-50% risk of morbidity and a 10-20% of mortality. The therapeutic decision in a patient with an AVM needs to consider both the risks of the intervention and the risks of the natural evolution of the disease. Objective: To assess the effectiveness of different AVM grading scales in predicting surgical risks according to our experience in a case series. Material and Method: a literature review of the AVM grading scales was made, through Pubmed including as key words "brain arteriovenous malformations" and "grading scale". A retrospective analysis was made of patients with AVM who were operated in our institution, they were classified according to the scales and their results were compared. Results: 90 patients were operated in our institution with AVM. Retrospectively, they were classified according to the Spetzler-Martin (SM), Spetzler-Ponce (SP), Lawton supplementary, and the sub-classifications in AVM grade 3, from Lawton and de Oliveira. Good outcome were considered when modified Rankin Scale (mRs) was equal or less than 2. The follow-up ranged from 12-48 months, having good outcome in 100% of AVM SM grade I, 91,7% grade II, 80% in grade III and 42,9% in grade IV. Using the SP scale, 93,7% of good outcome in grade A, 80% in grade B and 42,9% in grade C. In the sub-classification of AVM SM 3, we found 84% of good outcome in type 3A de Oliveira and 71,3% in type 3B. According to the Lawton scale, good outcome were found in 92% in type 3-, 72,1% in type 3+ and 60% in type 3. Using Lawton supplementary scale combined with SM, there were 100% of good outcome in grades II and III, 85,7% in grade IV, 87,6% in grade V, 80% in grade VI, 75% in grade VII, 66,6% in grade VIII. Conclusion: In our series, we reaffirm the effectiveness to predict surgical risk of the following scales: SM, SP and the Lawton's sub-classification of AVM grade 3. Specially, the use of the supplementary Lawton-Young scale in the surgical treatment of bleeding AVMs.


Subject(s)
Arteriovenous Malformations , Therapeutics , Brain , Morbidity , Mortality , Hemorrhage
11.
J. bras. pneumol ; 45(4): e20180137, 2019. tab, graf
Article in English | LILACS | ID: biblio-1012569

ABSTRACT

ABSTRACT Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.


RESUMO Objetivo: Apresentar uma série de casos de malformações arteriovenosas pulmonares (MAVP) e descrever os principais achados clínicos, a quantidade e localização das MAVP, as complicações clínicas e os tratamentos realizados. Métodos: Estudo retrospectivo observacional que avaliou pacientes com MAVP divididos em dois grupos: telangiectasia hemorrágica hereditária (THH) e MAVP idiopática (MAVPi). Resultados: Foram avaliados 41 pacientes, sendo 33 pacientes portadores de MAVP. Após a avaliação clínica, 27 e 6 foram diagnosticados com THH e MAVPi, respectivamente. No grupo THH a média de idade foi de 49,6 anos e 88,9% eram do sexo feminino. Desses pacientes, 4 tinham SpO2 < 90% e o achado clínico mais frequente era epistaxe. No grupo MAVPi a média de idade foi de 48,1 anos, sendo que 83,3% eram do sexo feminino. Desses, 3 tinham SpO2 < 90%. Após a realização de angiotomografia de tórax observou-se que a maior parte das MAVP se situava nos lobos inferiores, totalizando 56,4% e 85,7% nos grupos THH e MAVPi, respectivamente. O tratamento por embolização foi realizado em 23 pacientes nos dois grupos, enquanto 10 aguardavam o procedimento até o momento da escrita deste estudo. Um paciente submetido à embolização foi encaminhado para ressecção pulmonar. Conclusões: Em ambos os grupos de pacientes com MAVP observou-se uma predominância de mulheres e de fístulas localizadas nos lobos inferiores. A maioria era assintomático respiratório com SpO2 > 90%. O tratamento de escolha para todos foi a embolização percutânea por cateter.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Young Adult , Arteriovenous Malformations/etiology , Arteriovenous Malformations/therapy , Arteriovenous Malformations/diagnostic imaging , Pulmonary Artery/abnormalities , Pulmonary Artery/diagnostic imaging , Telangiectasia, Hereditary Hemorrhagic/complications , Catheterization/methods , Angiography/methods , Echocardiography , Radiography, Thoracic/methods , Retrospective Studies , Treatment Outcome , Embolization, Therapeutic/methods , Septal Occluder Device , Computed Tomography Angiography
12.
Article in English | WPRIM | ID: wpr-763509

ABSTRACT

Vascular anomalies comprise a heterogeneous group of disorders characterized by abnormal growth or development of blood vessels. Diagnosis of vascular anomalies is often challenging due to the large variety of conditions, which exhibit phenotypic heterogeneity and a wide range of symptomology and severity. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Recent interdisciplinary collaboration has led to collaborative studies and their outcomes are being prospectively evaluated. While there is still a role for surgical intervention in various vascular anomalies, discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options. This paper focuses on vascular anomaly issues often seen by the pediatricians and reviews the clinical pearls on infantile hemangiomas, lymphatic malformations, venous malformations, and arteriovenous malformations.


Subject(s)
Arteriovenous Malformations , Blood Vessels , Cooperative Behavior , Diagnosis , Hemangioma , Population Characteristics , Prospective Studies , Specialization
13.
Article in Korean | WPRIM | ID: wpr-762261

ABSTRACT

With the field of neuroradiological procedures consistently expanding, breaking the border between the medical and surgical treatment, anesthetic involvement in those procedures is also increasing, which underscores the importance of related anesthesia management. The objective of this study is to review the closing or open endovascular procedures for intracranial aneurysm, arteriovenous malformation, acute ischemic stroke, and carotid stenosis and related anesthetic implications.


Subject(s)
Anesthesia , Arteriovenous Malformations , Carotid Stenosis , Endovascular Procedures , Intracranial Aneurysm , Stroke
14.
Article in English | WPRIM | ID: wpr-762023

ABSTRACT

A 58-year-old male patient with severe claudication due to thrombosis of the left ilio-femoro-popliteal artery aneurysm. He also had a venous stasis ulcer with a history of multiple embolotherapy of arteriovenous malformation. Duplex sonography revealed reflux and varicose veins of the left great saphenous vein (GSV). A sequential bypass surgery was performed that consisted of excision of the left external iliac and common femoral artery aneurysm, external iliac to deep femoral interposition with an expanded polytetrafluoroethylene graft, and femoro-posterior tibial artery bypass with the reversed left GSV. Symptoms of claudication were alleviated and the chronic ulcer was healed in time. To our knowledge, this is the first report of successful bypass in a patient with arterial aneurysm, arteriovenous malformation, and venous insufficiency that can be diagnosed as an atypical case of Parkes Weber syndrome. Long-term follow-up is needed to define the fate of aneurysms and varicose vein graft.


Subject(s)
Aneurysm , Arteries , Arteriovenous Fistula , Arteriovenous Malformations , Embolization, Therapeutic , Femoral Artery , Follow-Up Studies , Humans , Male , Middle Aged , Polytetrafluoroethylene , Popliteal Artery , Saphenous Vein , Sturge-Weber Syndrome , Thrombosis , Tibial Arteries , Transplants , Ulcer , Varicose Ulcer , Varicose Veins , Venous Insufficiency
15.
Article in English | WPRIM | ID: wpr-765322

ABSTRACT

OBJECTIVE: To describe our experiences with a fully equipped high-end digital subtraction angiography (DSA) system within a hybrid operating room (OR). METHODS: A single-plane DSA system with 3-dimensional rotational angiography, cone-beam computed tomography (CBCT), and real-time navigation software was used in our hybrid OR. Between April 2014 and January 2018, 191 sessions of cerebrovascular procedures were performed in our hybrid OR. After the retrospective review of all cases, the procedures were categorized into three subcategorical procedures : combined endovascular and surgical procedure, complementary rescue procedure during intervention and surgery, and frameless stereotaxic operation. RESULTS: Forty-nine of 191 procedures were performed using hybrid techniques. Four cases of blood blister aneurysms and a ruptured posterior inferior cerebellar artery aneurysm were treated using bypass surgery and endovascular trapping. Eight cases of ruptured aneurysm with intracranial hemorrhage (ICH) were treated by partial embolization and surgical clipping. Six cases of ruptured arteriovenous malformation with ICH were treated by Onyx embolization of nidus and subsequent surgical removal of nidus and ICH. Two (5.4%) of the 37 cases of pre-mature rupture during clipping were secured by endovascular coil embolization. In one (0.8%) complicated case of 103 intra-arterial thrombectomy procedures, emergency surgical embolectomy with bypass surgery was performed. In 27 cases of ICH, frameless stereotaxic hematoma aspiration was performed using XperGuide® system (Philips Medical Systems, Best, the Netherlands). All procedures were performed in single sessions without any procedural complications. CONCLUSION: Hybrid OR with a fully equipped DSA system could provide precise and safe treatment strategies for cerebrovascular diseases. Especially, we could suggest a strategy to cope flexibly in complex lesions or unexpected situations in hybrid OR. CBCT with real-time navigation software could augment the usefulness of hybrid OR.


Subject(s)
Aneurysm , Aneurysm, Ruptured , Angiography , Angiography, Digital Subtraction , Arteries , Arteriovenous Malformations , Blister , Cerebrovascular Disorders , Cone-Beam Computed Tomography , Embolectomy , Embolization, Therapeutic , Emergencies , Hematoma , Intracranial Hemorrhages , Operating Rooms , Retrospective Studies , Rupture , Surgical Instruments , Thrombectomy
16.
Article in English | WPRIM | ID: wpr-765072

ABSTRACT

BACKGROUND: A randomized trial of unruptured brain arteriovenous malformations (ARUBA) reported superior outcomes in conservative management compared to interventional treatment. There were numerous limitations to the study. This study aimed to investigate the efficacy of gamma knife radiosurgery (GKS) for patients with brain arteriovenous malformations (AVMs) by comparing its outcomes to those of the ARUBA study. METHODS: We retrospectively reviewed ARUBA-eligible patients treated with GKS from June 2002 to September 2017 and compared against those in the ARUBA study. AVM obliteration and hemorrhage rates, and clinical outcomes following GKS were also evaluated. RESULTS: The ARUBA-eligible cohort comprised 264 patients. The Spetzler-Martin grade was Grade I to II in 52.7% and III to IV in 47.3% of the patients. The mean AVM nidus volume, marginal dose, and follow-up period were 4.8 cm³, 20.8 Gy, and 55.5 months, respectively. AVM obliteration was achieved in 62.1%. The annual hemorrhage rate after GKS was 3.4%. A stroke or death occurred in 14.0%. The overall stroke or death rate of the ARUBA-eligible cohort was significantly lower than that of the interventional arm of the ARUBA study (P < 0.001) and did not significantly differ from that of the medical arm in the ARUBA study (P = 0.601). CONCLUSION: GKS was shown to achieve a favorable outcome with low procedure-related morbidity in majority of the ARUBA-eligible patients. The outcome after GKS in our patients was not inferior to that of medical care alone in the ARUBA study. It is suggested that GKS is rather superior to medical care considering the short follow-up duration of the ARUBA study.


Subject(s)
Arm , Arteriovenous Malformations , Brain , Cohort Studies , Follow-Up Studies , Hemorrhage , Humans , Intracranial Hemorrhages , Mortality , Netherlands , Radiosurgery , Retrospective Studies , Stroke
17.
Neurointervention ; : 91-98, 2019.
Article in English | WPRIM | ID: wpr-760598

ABSTRACT

PURPOSE: Hereditary hemorrhagic telangiectasia (HHT), a rare genetic vascular disorder, has been rarely reported in South Korea. We investigated the current prevalence and presenting patterns of genetically confirmed HHT in South Korea. MATERIALS AND METHODS: We defined HHT patients as those with proven mutations on known HHT-related genes (ENG, ACVRL1, SMAD4, and GDF2) or those fulfilling 3 or 4 of the Curaçao criteria. A computerized systematic search was performed in PubMed and KoreaMed using the following search term: (“hereditary hemorrhagic telangiectasia” AND “Korea”) OR (“Osler-Weber-Rendu” AND “Korea”). We also collected government health insurance data. HHT genetic testing results were collected from three tertiary hospitals in which the genetic tests were performed. We integrated patient data by analyzing each case to obtain the prevalence and presenting pattern of HHT in South Korea. RESULTS: We extracted 90 cases from 52 relevant articles from PubMed and KoreaMed. An additional 22 cases were identified from the three Korean tertiary hospitals after excluding seven cases that overlapped with those in the published articles. Finally, 112 HHT patients were identified (41 males and 71 females, aged 4–82 years [mean±standard deviation, 45.3±20.6 years]). The prevalence of HHT in South Korea is about 1 in 500,000, with an almost equal prevalence among men and women. Forty-nine patients underwent genetic testing, of whom 28 had HHT1 (ENG mutation) and 19 had HHT2 (ACVRL1 mutation); the other two patients were negative for ENG, ACVRL1, and SMAD4 mutations. CONCLUSION: The prevalence of HHT is underestimated in Korea. The rate of phenotypic presentation seems to be similar to that found worldwide. Korean health insurance coverage is limited to representative genetic analysis to detect ENG and ACVRL1 mutations. Further genetic analyses to detect HHT3, HHT4, and other forms of HHT should be implemented.


Subject(s)
Arteriovenous Fistula , Arteriovenous Malformations , Diagnosis , Epistaxis , Female , Genetic Testing , Hemorrhage , Humans , Insurance, Health , Korea , Male , Prevalence , Telangiectasia, Hereditary Hemorrhagic , Tertiary Care Centers
18.
Korean Journal of Radiology ; : 1285-1292, 2019.
Article in English | WPRIM | ID: wpr-760295

ABSTRACT

OBJECTIVE: To evaluate the safety and efficacy of the coil-protected technique for liquid embolization in neurovascular malformations. MATERIALS AND METHODS: Twenty-two patients who underwent coil-protected liquid embolization for symptomatic cranial (n = 13) and spinal (n = 9) arteriovenous fistula (AVF) or arteriovenous malformations (AVMs) were identified. A total of 36 target feeder vessels were embolized with N-butyl cyanoacrylate and/or Onyx (Medtronic). This technique was used to promote delivery of a sufficient amount of liquid embolic agent into the target shunt or nidus in cases where tortuous feeding arteries preclude a microcatheter wedging techniqu and/or to prevent reflux of the liquid embolic agent in cases with a short safety margin. The procedure was considered technically successful if the target lesion was sufficiently filled with liquid embolic agent without unintentional reflux. Angiographic and clinical outcomes were retrospectively evaluated. RESULTS: Technical success was achieved for all 36 target feeders. Post-embolization angiographies revealed complete occlusion in 16 patients and near-complete and partial occlusion in three patients each. There were no treatment-related complications. Of the six patients who showed near-complete or partial occlusion, five received additional treatments: two received stereotactic radiosurgery for cerebral AVM, two underwent surgical removal of cerebral AVM, and one underwent additional embolization by direct puncture for a mandibular AVM. Finally, all patients showed complete (n = 19) or near-complete (n = 3) occlusion of the target AVF or AVM on follow-up angiographies. The presenting neurological symptoms improved completely in 15 patients (68.2%) and partially in seven patients (31.8%). CONCLUSION: The coil-protected technique is a safe and effective method for liquid embolization, especially in patients with various neurovascular shunts or malformations who could not be successfully treated with conventional techniques.


Subject(s)
Angiography , Arteries , Arteriovenous Fistula , Arteriovenous Malformations , Cyanoacrylates , Follow-Up Studies , Humans , Methods , Punctures , Radiosurgery , Retrospective Studies
19.
Article in English | WPRIM | ID: wpr-741738

ABSTRACT

Uterine arteriovenous vascular malformation (UAVM) is a disease that causes excessive bleeding. The symptoms do not subside without proper treatment and this can lead to life-threatening situations. The correct diagnosis of UAVM can be complicated if the patient's uterus did not completely discharge everything during abortion (in broader terms, retaining remnants of the products of conception). In this case, Doppler ultrasonography and computed tomography angiography with 3-dimensional rendering were used to analyze the cause of bleeding and provide proper treatment of this patient. Then, uterine artery embolization, dilatation, and curettage were performed safely and successfully. The patient no longer had symptoms of vaginal spotting during the planned follow up care. UAVM is uncommon; however, if reproductive-age women show repeated abnormal vaginal bleeding after dilatation and curettage, a diagnosis of UAVM must be considered based on the medical history and examination.


Subject(s)
Angiography , Arteriovenous Malformations , Curettage , Diagnosis , Dilatation and Curettage , Dilatation , Female , Follow-Up Studies , Hemorrhage , Humans , Metrorrhagia , Ultrasonography , Ultrasonography, Doppler , Uterine Artery , Uterine Artery Embolization , Uterine Hemorrhage , Uterus , Vascular Malformations
20.
Article in English | WPRIM | ID: wpr-785931

ABSTRACT

Stereotactic radiosurgery has become excellent alternative treatment for cerebral arteriovenous malformations (AVM). This technique has expanded to treatment of larger AVM which is not amenable to surgical management. However, its variable adverse effects should be also taken into considerations sincerely because of radiobiological characteristics such as delayed onset and progressive neurological deteriorations. Herein, we report a case in which progressively expanding hemorrhagic cyst with repeated bleedings so called chronic encapsulated expanding hematoma was developed on several years after radiosurgery treatment. Neurological and radiological findings were improved by surgical removal.


Subject(s)
Arteriovenous Malformations , Hematoma , Intracranial Arteriovenous Malformations , Radiosurgery
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