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Neurofibromatosis Type 1 (NF1) is a rare cause of ischemic stroke (IS) in the general population. We report a case of a young patient with NF1 in whom IS was caused by fibromuscular dysplasia. An angiographic study demonstrated occlusion in the right internal carotid artery (ICA), just after its origin, and the left ICA, just before the intracranial portion, and brain magnetic resonance imaging showed the limits of an area of brain infarction in the right frontoparietal region. Despite these concomitant neuroimaging findings, this association is rare, and it is difficult to establish the contribution to the outcome made by each of these diseases, which treatment is the best to implement, or what prognosis is.
A neurofibromatose tipo 1 (NF1) é uma causa rara de acidente vascular cerebral isquêmico (AVCi) na população geral. Neste estudo, relatamos o caso de um paciente jovem com AVCi, com diagnóstico de NF1 associada a displasia fibromuscular. O estudo angiográfico demonstrou oclusão da carótida interna direita, logo após sua origem, e esquerda, antes da porção intracraniana. A ressonância magnética do encéfalo mostrou delimitação de um infarto na região frontoparietal direita. Apesar desses achados concomitantes na neuroimagem, essa associação é rara, sendo difícil de estabelecer a contribuição de cada uma dessas doenças no desfecho, tampouco qual o melhor tratamento a ser implementado e qual o prognóstico.
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Abstract Neurofibromatosis Type 1 (NF1) is a rare cause of ischemic stroke (IS) in the general population. We report a case of a young patient with NF1 in whom IS was caused by fibromuscular dysplasia. An angiographic study demonstrated occlusion in the right internal carotid artery (ICA), just after its origin, and the left ICA, just before the intracranial portion, and brain magnetic resonance imaging showed the limits of an area of brain infarction in the right frontoparietal region. Despite these concomitant neuroimaging findings, this association is rare, and it is difficult to establish the contribution to the outcome made by each of these diseases, which treatment is the best to implement, or what prognosis is.
Resumo A neurofibromatose tipo 1 (NF1) é uma causa rara de acidente vascular cerebral isquêmico (AVCi) na população geral. Neste estudo, relatamos o caso de um paciente jovem com AVCi, com diagnóstico de NF1 associada a displasia fibromuscular. O estudo angiográfico demonstrou oclusão da carótida interna direita, logo após sua origem, e esquerda, antes da porção intracraniana. A ressonância magnética do encéfalo mostrou delimitação de um infarto na região frontoparietal direita. Apesar desses achados concomitantes na neuroimagem, essa associação é rara, sendo difícil de estabelecer a contribuição de cada uma dessas doenças no desfecho, tampouco qual o melhor tratamento a ser implementado e qual o prognóstico.
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Isolated dissection of the internal carotid artery (ICA) is rare in young patients and is a cause for strong suspicion of fibromuscular dysplasia (FMD), especially when associated with artery elongation and tortuosity. The natural history of cerebrovascular FMD is unknown and management of symptomatic patients can be challenging. We report the case of a 44-year-old female patient with a history of transient ischemic attack in the absence of cardiovascular risk factors, associated with an isolated left ICA dissection and kinking. Carotid duplex ultrasound confirmed the diagnosis of dissection and demonstrated severe stenosis of the left ICA. The patient underwent surgical repair and histopathological evaluation confirmed the diagnosis of FMD with dissection. An autogenous great saphenous vein bypass was performed and the patient had an uneventful recovery. Cervical carotid artery dissection can be related to underlying arterial pathologies such as FMD, and the presence of ICA tortuosity highlights certain peculiarities for optimal management, which might be surgical.
A dissecção isolada da artéria carótida interna em pacientes jovens é rara, e a displasia fibromuscular deve ser altamente suspeitada principalmente quando estiver associada a alongamento e tortuosidade da artéria. A história natural da displasia fibromuscular cerebrovascular é desconhecida, e o manejo de pacientes sintomáticos pode ser desafiador. Apresentamos o caso de uma paciente de 44 anos com histórico de ataque isquêmico transitório sem fatores de risco cardiovasculares, associado a dissecção e acotovelamento isolados da artéria carótida interna esquerda. O ultrassom duplo das carótidas confirmou o diagnóstico de dissecção e demonstrou estenose grave na artéria carótida interna esquerda. A paciente foi submetida a reparo cirúrgico, e a avaliação histopatológica confirmou o diagnóstico de displasia fibromuscular com dissecção. Foi realizada cirurgia de ressecção do segmento e reconstrução com veia safena magna autógena, e a paciente se recuperou sem complicações. A dissecção da artéria carótida cervical pode estar relacionada a doenças arteriais subjacentes, como a displasia fibromuscular, e a presença da tortuosidade da artéria carótida interna destaca algumas particularidades no manejo ideal, o qual pode ser cirúrgico.
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Renovascular hypertension (RVH) is defined as an elevated blood pressure caused by kidney hypoperfusion, generally as a result of anatomic stenosis of the renal artery with consequent activation of the Renin Angiotensin-Aldosterone System. The main causes include genetic and inflammatory disorders, extrinsic compression, and idiopathic alterations. RVH is often asymptomatic and should be suspected in any child with refractory hypertension, especially if other suggestive findings are present, including those with severe hypertension, abdominal bruit, and abrupt fall of glomerular filtration rate after administration of angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers. There is a consensus that digital subtraction angiography is the gold standard method for the diagnosis of RVH. Nevertheless, the role of non-invasive imaging studies such as Doppler ultrasound, magnetic resonance angiography, or computed tomographic angiography remains controversial, especially due to limited pediatric evidence. The therapeutic approach should be individualized, and management options include non-surgical pharmacological therapy and revascularization with percutaneous transluminal renal angioplasty (PTRA) or surgery. The prognosis is related to the procedure performed, and PTRA has a higher restenosis rate compared to surgery, although a decreased risk of complications. This review summarizes the causes, physiopathology, diagnosis, treatment, and prognosis of RVH in pediatric patients. Further studies are required to define the best approach for RVH in children.
Assuntos
Hipertensão Renovascular , Obstrução da Artéria Renal , Angioplastia com Balão , Criança , Humanos , Hipertensão Renovascular/diagnóstico , Hipertensão Renovascular/etiologia , Hipertensão Renovascular/terapia , Artéria Renal/patologia , Obstrução da Artéria Renal/diagnóstico , Obstrução da Artéria Renal/diagnóstico por imagemRESUMO
AIMS: Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. METHODS AND RESULTS: All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. CONCLUSIONS: The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD.
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Dissecção Aórtica/epidemiologia , Displasia Fibromuscular/epidemiologia , Adulto , Fatores Etários , Idoso , Dissecção Aórtica/diagnóstico por imagem , Argentina/epidemiologia , Ásia/epidemiologia , Angiografia por Tomografia Computadorizada , Europa (Continente)/epidemiologia , Feminino , Displasia Fibromuscular/diagnóstico por imagem , Humanos , Incidência , Angiografia por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Fenótipo , Valor Preditivo dos Testes , Prevalência , Prognóstico , Sistema de Registros , Medição de Risco , Fatores de Risco , Fatores Sexuais , Tunísia/epidemiologiaRESUMO
Resistant hypertension is common in the chronic kidney disease population and conveys increased risk for adverse cardiovascular outcomes and the development of kidney failure. Recently, the American College of Cardiology and American Heart Association published a revised scientific statement on the definition and management of resistant hypertension, which codified the long-debated differences between pseudoresistant hypertension and true resistant hypertension. We review this distinction and its importance to nephrologists, who frequently encounter patients for whom antihypertensive therapy fails due to difficulty adhering to complex multidrug regimens. Second, we discuss the evaluation of patients with resistant hypertension, including appropriate screening and diagnostic testing for causes of secondary hypertension. Third, we examine the management of established resistant hypertension, including medication optimization, recent clinical trials supporting lifestyle modifications, and the evidence behind the routine use of mineralocorticoid receptor antagonists. Special attention is given to the vital role of diuretics in the treatment of patients with chronic kidney disease. We propose an algorithm for the diagnosis and management of these cases. Finally, we briefly discuss the current state of antihypertensive device therapies, including kidney denervation and baroreceptor-directed therapies.
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Anti-Hipertensivos/farmacologia , Hipertensão , Insuficiência Renal Crônica , Resistência a Medicamentos , Humanos , Hipertensão/complicações , Hipertensão/terapia , Conduta do Tratamento Medicamentoso , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/fisiopatologiaRESUMO
Fibromuscular dysplasia (FMD) is a known cause of pulsatile tinnitus that can, on rare occasion, evolve into an incapacitating condition. It is a noninflammatory and nonatherosclerotic arteriopathy of unknown cause that affects medium-sized vessels, such as the carotid and renal arteries, occurring mainly in women. We describe a 72-year-old woman suffering from pulsatile tinnitus refractory to medical treatment who was successfully treated with Casper stent in the carotid artery. The different treatment strategies published in the literature were reviewed.
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Abstract Isolated dissection of the internal carotid artery (ICA) is rare in young patients and is a cause for strong suspicion of fibromuscular dysplasia (FMD), especially when associated with artery elongation and tortuosity. The natural history of cerebrovascular FMD is unknown and management of symptomatic patients can be challenging. We report the case of a 44-year-old female patient with a history of transient ischemic attack in the absence of cardiovascular risk factors, associated with an isolated left ICA dissection and kinking. Carotid duplex ultrasound confirmed the diagnosis of dissection and demonstrated severe stenosis of the left ICA. The patient underwent surgical repair and histopathological evaluation confirmed the diagnosis of FMD with dissection. An autogenous great saphenous vein bypass was performed and the patient had an uneventful recovery. Cervical carotid artery dissection can be related to underlying arterial pathologies such as FMD, and the presence of ICA tortuosity highlights certain peculiarities for optimal management, which might be surgical.
Resumo A dissecção isolada da artéria carótida interna em pacientes jovens é rara, e a displasia fibromuscular deve ser altamente suspeitada principalmente quando estiver associada a alongamento e tortuosidade da artéria. A história natural da displasia fibromuscular cerebrovascular é desconhecida, e o manejo de pacientes sintomáticos pode ser desafiador. Apresentamos o caso de uma paciente de 44 anos com histórico de ataque isquêmico transitório sem fatores de risco cardiovasculares, associado a dissecção e acotovelamento isolados da artéria carótida interna esquerda. O ultrassom duplo das carótidas confirmou o diagnóstico de dissecção e demonstrou estenose grave na artéria carótida interna esquerda. A paciente foi submetida a reparo cirúrgico, e a avaliação histopatológica confirmou o diagnóstico de displasia fibromuscular com dissecção. Foi realizada cirurgia de ressecção do segmento e reconstrução com veia safena magna autógena, e a paciente se recuperou sem complicações. A dissecção da artéria carótida cervical pode estar relacionada a doenças arteriais subjacentes, como a displasia fibromuscular, e a presença da tortuosidade da artéria carótida interna destaca algumas particularidades no manejo ideal, o qual pode ser cirúrgico.
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Humanos , Feminino , Adulto , Artéria Carótida Interna/patologia , Dissecação da Artéria Carótida Interna/complicações , Displasia Fibromuscular/complicações , Constrição Patológica , Dissecação da Artéria Carótida Interna/cirurgia , Dissecação da Artéria Carótida Interna/diagnóstico por imagemRESUMO
RESUMEN Carotid web es un término usado para describir en imágenes una anomalía vascular del desarrollo del sistema braquiocefálico. Se trata de una causa poco reconocida de accidente cerebrovascular, especialmente en pacientes jóvenes. Su frecuencia en la población general no se ha determinado y se asocia con eventos cerebrovasculares isquémicos de tipo embólico. En este reporte describimos dos casos de web carotídeo sintomático, uno que se manifiesta como un ataque isquémico transitorio (AIT) y el otro como infarto cerebral. Hasta el momento, no existe un método diagnóstico establecido como óptimo; la angiografía por resonancia magnética y por tomo-grafía se presentan como una opción confiable y alternativa a la angiografía convencional. Una vez identificado el carotid web, la evidencia es escasa con respecto a su manejo; algunas opciones con las que contamos son antiagregación, anticoagulación y manejo invasivo con endarterectomía y colocación de stent. Se dispone de poca literatura en español sobre web carotídeo. A efectos de una mejor comprensión proponemos el término de septo o banda carotídea para denominar a esta anomalía vascular.
SUMMARY Carotid web is a term used to describe by images a vascular abnormality of development of the brachiocephalic system. It is an under-recognized cause of stroke, especially in young patients. Its frequency in the general population has not been determined and is associated with ischemic cerebrovascular events of embolic type. Two cases of symptomatic carotid web are reported, one manifested as a transient ischemic attack (TIA) and the other as a cerebral infarction. There is not an established optimal diagnostic method, MRI and CT angiography are presented as a good option and alternative to conventional angiography. Once the carotid web has been identified, the evidence is scarce regarding its management, there are some options such as antiplatelets, anticoagulation and invasive management with endarterectomy and stent placement, there is little information in Spanish literature on carotid web so we propose the term septo o banda carotidea to name this vascular abnormality.
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Mobilidade UrbanaRESUMO
BACKGROUND: Fibromuscular dysplasia affects generally renal artery, causing renovascular hypertension. The most classical angiographic pattern, string-of-beads, can be found in cervical and more rarely in other arteries. With the advance of endovascular procedures techniques, the number of open surgeries is decreasing, and complications related to the selective catheterization of diseased vessels are increasing. CASE REPORT: A 37-year-old man presenting with subarachnoid hemorrhage was submitted to angioplasty for dissecting aneurysms of vertebral artery with a good outcome. Several arteries were angiographically diagnosed with the dysplasia (renal, carotid, femoral), and some complications like stenosis, dissection, arteriovenous fistula, and dissecting aneurysm occurred in sequence. CONCLUSIONS: FMD of cervical arteries is usually asymptomatic. There are no guidelines or protocols to cervical FMD treatment, being indicated only for the complications. Because of the vessels fragility, a several spontaneous or post endovascular procedure complications can be disastrous.
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Dissecção Aórtica , Displasia Fibromuscular , Hemorragia Subaracnóidea , Adulto , Artérias Carótidas , Displasia Fibromuscular/complicações , Displasia Fibromuscular/diagnóstico por imagem , Humanos , Masculino , Artéria VertebralRESUMO
Resumen La displasia fibromuscular es una enfermedad no inflamatoria y no aterosclerótica, que puede afectar cualquier lecho arterial; sin embargo, las arterias renales y la carótida interna son las más comprometidas. Se presenta el caso de un paciente que cursó con dolor abdominal y cefalea hemicraneana posterior, con síndrome de Horner incompleto. En arteriografía renal y panangiografía cerebral realizada por Cardiología intervencionista se observó infarto renal derecho y disección de la carótida interna derecha con formación de pseudoaneurisma. Se hizo terapia endovascular con angioplastia y se dio de alta con antiagregación dual. Es importante conocer este tipo de presentación clínica ya que el diagnóstico podría confundirse con otras enfermedades que generan signos y síntomas similares; por consiguiente, es ideal tener sospecha clínica alta para evitar retrasos en el manejo.
Abstract Fibromuscular dysplasia is a non-inflammatory and non-atherosclerotic disease that can affect any arterial bed, with the renal and the internal carotid arteries being the most compromised. A case is presented on a patient that had abdominal pain and pain in the back of the head, and an incomplete Horner syndrome. In the renal arteriography and cerebral pan-angiography carried out by interventionist Cardiology, a right renal infarction and dissection of the right internal carotid was observed with a pseudo-aneurysm formation. Intravenous treatment was performed with angioplasty, and the patient was discharged with dual antiplatelet therapy. It is important to be aware of this type of clinical presentation, since the diagnosis can be confused with other diseases that produce similar signs and symptoms. It would be ideal to have a high clinical suspicion in order to avoid delays in the management.
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Humanos , Feminino , Pessoa de Meia-Idade , Acidente Vascular Cerebral , Dissecação da Artéria Carótida Interna , Displasia Fibromuscular , InfartoRESUMO
En la Argentina no existen datos epidemiológicos sobre displasia fibromuscular. La realización de un registro nacional puede aportar información que conduzca a una actualización de los consensos y recomendaciones para un correcto diagnóstico, evaluación y tratamiento. El Registro Argentino de Displasia Fibromuscular (SAHARA-DF) inició su actividad de recopilación de datos en octubre de 2015. Al año 2019 se confirmaron 49 pacientes (44 mujeres, 38 hipertensos, edad 45,3 ± 17,2 años, 12 con presentación neurológica). Veintidós pacientes tuvieron lesiones vasculares en más de un sitio, a pesar del sesgo diagnóstico por falta de estudios complementarios en casi la mitad de los casos. El sitio afectado más frecuente fue el renovascular, seguido por el carotídeo y el ilíaco, y las lesiones multifocales fueron más frecuentes que las unifocales (35 versus 14, respectivamente). Se constató la presencia de aneurismas asociados en 13 casos y disección arterial en 4 casos. De las 22 angioplastias renales realizadas, 14 fueron con colocación de stent (endoprótesis). En este estudio preliminar de una población argentina se evidencia el carácter sistémico de la enfermedad y se plantea un llamado a actuar en cuanto a la necesidad de debatir el algoritmo diagnóstico y el método de tratamiento. (AU)
In Argentina there are no epidemiological data regarding fibromuscular dysplasia. Building a National Registry may provide information leading to updated consensus and recommendations for a correct diagnosis, assessment and treatment. Data gathering for the Argentine Registry of Fibromuscular Dysplasia (SAHARA-DF) was initiated in October 2015. By 2019, 49 patients were confirmed (44 women, 38 hypertensives, age 45.3 ± 17.2 years, 12 with a neurological presentation). Twenty-two patients had multi-site vascular lesions, in spite of a diagnosis bias due to lack of supporting studies in almost half of the cases. The renovascular site was the most affected, followed by the carotid and iliac sites, and multifocal lesions were more frequent than unifocal (35 versus 14, respectively). Associated aneurysms were found in 13 cases, and arterial dissection in 4. Twenty-two renal angioplasties were performed, 14 with stent placement. In this preliminary study of an Argentinian population, the systemic nature of the disease is evidenced, and a call for action arises regarding the need for discussing the diagnostic algorithm and treatment method. (AU)
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Adulto Jovem , Registros/estatística & dados numéricos , Displasia Fibromuscular/diagnóstico , Argentina/epidemiologia , Algoritmos , Viés , Fatores Sexuais , Estudos Transversais , Fatores de Risco , Fatores Etários , Angioplastia/métodos , Fatores Culturais , Lesões do Sistema Vascular/diagnóstico por imagem , Displasia Fibromuscular/classificação , Displasia Fibromuscular/etiologia , Displasia Fibromuscular/terapia , Displasia Fibromuscular/epidemiologia , Hipertensão/epidemiologia , Dissecção Aórtica/diagnóstico por imagemRESUMO
Background Fibromuscular dysplasia (FMD) affects predominantly the cervical and renal arteries and may cause the classical angiographic pattern of string-of-beads. The diagnosis is increasing with the advances of imaging techniques. Case Report A 37-year-old man presenting with subarachnoid hemorrhage due to a dissecting aneurysm of the vertebral artery was treated by angioplasty with stent, with good outcome. All of the cervical and renal arteries were diseased and showed dysplasia and/or ectasias. Conclusions There are no guidelines or protocols to treat patients with FMD.
Assuntos
Humanos , Masculino , Adulto , Hemorragia Subaracnóidea/complicações , Estenose das Carótidas/etiologia , Procedimentos Endovasculares/reabilitação , Displasia Fibromuscular/complicações , Dissecção Aórtica/diagnóstico por imagem , Angioplastia/métodos , Malformações Vasculares/complicaçõesRESUMO
BACKGROUND: We present a patient with history of sinus venous thrombosis and hypertension during the last year. Her blood pressure was not controlled despite drugs, diet, and exercise. She denied symptoms. She does not smoke nor drink alcohol. Her body mass index was 20 kg/m2, NYHA Class I/IV. CASE SUMMARY: A 40-year-old Latin-American female patient, concerned because despite taking verapamil 160 mg/day, losartan 100 mg/day, and hydrochlorothiazide 25 mg/day her diastolic blood pressure was over 100 mmHg. Routine serum and urine lab tests and a transthoracic echocardiogram were done and were normal. The treatment was increased over the next consultations but without control of her blood pressure. She developed resistant hypertension, and she was taking four antihypertensive drugs and two diuretics. A first renal artery Doppler was normal. Because of a high clinical suspicion a renal angio-CT was performed showing bilateral fibromuscular dysplasia. The patient underwent a renal artery angioplasty with balloon with excellent results and better control of her blood pressure after the procedure. Over the next month, her doctors were able to decrease her treatment to two drugs at intermediate doses. Currently, she is doing well and asymptomatic. DISCUSSION: Renal artery fibromuscular dysplasia (FMD) could be a challenging disease to be diagnosed. Patients with this condition may suffer from symptomatic and resistant hypertension. Many patients do not have abnormalities on their physical exam or in the routine lab tests. Treatment includes renal artery angioplasty if patient is symptomatic and blood pressure is resistant.
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Resumen La estenosis de la arteria renal es una afección clínica que puede ser asintomática o causar hipertensión arterial sistémica, así como deterioro de la función renal de uno o ambos riñones. La estenosis puede ser causada por aterosclerosis en 90% de los casos y por displasia fibromuscular en 10%, la estenosis de la arteria renal es la principal causa de hipertensión secundaria reversible o modificable si ésta es diagnosticada y tratada antes de cursar con el daño renal. La displasia fibromuscular es indicación de angioplastia percutánea logrando el control de la presión arterial en, incluso, 63% sin antihipertensivos. El objetivo de este artículo es mostrar la complejidad del proceso diagnóstico y la importancia que tiene la sospecha clínica para la corrección oportuna de este tipo de hipertensión arterial reversible.
Abstract Renal artery stenosis (RAS) is a clinical entity that can be asymptomatic or cause systemic arterial hypertension likewise spoil the renal function of one or both kidneys. The stenosis can be caused by atherosclerosis in 90% of the cases and by fibromuscular dysplasia in 10%; the stenosis of the renal artery is the main cause of reversible or modifiable secondary hypertension if it is diagnosed and treated before the renal damage. Fibromuscular dysplasia is an indication for percutaneous angioplasty achieving blood pressure control in up to 63% without antihypertensive drugs. The objective of this paper is to show the complexity of the diagnostic process and the importance of the clinical suspicion for the well-timed correction of this type of arterial hypertension.
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Background There have been studies trying to characterize Fibromuscular Dysplasia (FMD); however, most of them are based in mainly non-Hispanic sample. The objective of this study is to better understand the epidemiology and clinical characteristics of craniocervical FMD in the Hispanic population. Methods We retrospectively reviewed the cerebral angiograms performed in our center in a period of 3.5 years under any indication looking for angiographic patterns of FMD. Exclusion criteria consisted of cases in which the first angiogram was done when the patients were younger than 18 years. Patients were subdivided based on those with FMD and those without it for baseline characteristics and were looked for any associations. We further compared the same baseline characteristics among Hispanic FMD and non-Hispanic FMD population. A chart review was conducted looking for clinical features and vascular events. Results We analyzed 448 angiograms among patients younger than 18 years. We identified 47 patients with evidence of FMD involving the cervical arteries and 401 patients without FMD. Of the 47 patients with FMD in our neuroendovascular registry, we found that 76.6% were women and 57.4% were Hispanics. There was no statistical significant difference when comparing the variables across ethnicities, except history of cigarette smoking and dyslipidemia. The most common associated supra-aortic arterial lesions seen in the FMD group were intracranial aneurysm and arterial dissections. We then used same variables to compare Hispanic FMD with non-Hispanic FMD. We have found that there has been a positive association of cigarette smoking and dyslipidemia with FMD ( p ≤ 0.05). Conclusion Our study suggests that FMD affecting the carotid and vertebral arteries has similar demographic pattern across ethnicities in the United States. In Hispanics, the disease appears to have a predilection for women and history of cigarette smoking. Intracranial aneurysm and arterial dissection were the most commonly associated arterial lesions.
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Spontaneous hemoperitoneum due to vascular injury is a life-threatening condition mostly associated with aortic or splanchnic arterial disease, which stems from atherosclerotic, inflammatory, or infectious origin. However, in 1976, Slavin and Gonzales described a nonatherosclerotic arterial disease that may render aneurysmal formation predominantly in the splanchnic arterial bed. The clinical presentation is diverse, but abdominal pain and shock prevail. We report the case of a middle-aged man who presented a hemoperitoneum due to a middle colic artery aneurysm rupture and died after undergoing a surgical treatment attempt. The preoperative imaging study revealed the presence of a huge hematoma in the epiplon retrocavity, and abdominal free liquid as well as extensive arterial disease with multiple aneurysms. The autopsy findings included hemoperitoneum, hematoma in the upper left abdominal quadrant, the surgical ligature of the middle colic artery, and histologic features consistent with segmental arterial mediolysis. The authors call attention to this rare entity and highlight the autopsy as a fundamental examination to accurately reach this diagnosis.
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A fibrodisplasia muscular renal é de etiologia desconhecida, de origem não inflamatória e não aterosclerótica, estando associada ao desenvolvimento de estenoses e de aneurismas. Relatamos um caso de fibrodisplasia muscular renal bilateral associada a aneurisma volumoso de artéria renal tratado com angioplastia com balão e stent multicamadas.
Renal artery fibromuscular dysplasia is a condition of unknown etiology, with non-inflammatory, nonatheroscleroticorigin, associated to the development of stenosis and aneurysms. The authors report a case of bilateral renal artery fibromuscular dysplasia associated with a large renal artery aneurysm, treatedwith balloon angioplasty and a multilayer stent.
Assuntos
Humanos , Feminino , Adulto , Artéria Renal , Displasia Fibromuscular/etiologia , Displasia Fibromuscular/terapia , Obstrução da Artéria Renal/complicações , Procedimentos Endovasculares/métodos , Vasos Coronários , Angioplastia/métodos , Cefuroxima/administração & dosagem , Hipertensão , Stents , Ultrassonografia Doppler/métodosRESUMO
Se presenta el caso clínico de una paciente que debutó con hipertensión arterial (HTA) grado II realizándose los estudios sistematizados correspondientes para descartar patologías secundarias. Dentro de los estudios de primera línea en la valoración merece destacarse la ecografía Doppler renal por la información que ella aporta, orientado al diagnóstico de estenosis de la arteria renal. La identificación de una HTA de origen renovascular supone el hallazgo de una de las causas reversibles de hipertensión. Dentro de esta etiología la más frecuente es la displasia fibromuscular (DFM) que mejora con la corrección de la estenosis mediante técnicas quirúrgicas o percutáneas con angioplastia con balón o stent. El objetivo es presentar un caso clínico para mostrar la complejidad del proceso diagnóstico y la importancia que tiene la sospecha clínica para la corrección oportuna de este tipo de HTA reversible...