Rol de las imágenes en la patología arterial no ateroesclerótica poco frecuente en el abdomen / Role of images in rare vascular pathology in the abdomen

Resumen La patología arterial no ateromatosa constituye un grupo de patologías poco frecuentes, pero de gran importancia debido a su morbilidad y mortalidad asociadas. La presentación clínica de estas entidades es inespecífica, por lo que el estudio inicial con imágenes es fundamental. Debido a esto, es muy importante reconocer los signos radiológicos característicos de cada una en las diversas modalidades imagenológicas para poder sospechar y orientar el diagnóstico.

Abstract Non-atheromatous arterial pathology constitutes a group of infrequent pathologies but of great importance due to their associated morbidity and mortality. In general, the clinical presentation of these pathologies is quite nonspecific, so the clinician decides to perform imaging studies thinking of more common entities. It is for this reason that it is very important to recognize characteristic radiological signs in the various imaging modalities to be able to suspect and orient a pathology of this kind.

Symptomatic internal carotid artery dissection and kinking in a patient with fibromuscular dysplasia / Dissecção e acotovelamento sintomáticos da artéria carótida interna em paciente com displasia fibromuscular

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.

Infarto renal y disección carotídea espontánea: complicaciones de la displasia fibromuscular / Spontaneous renal infarction and carotid dissection: complications of fibromuscular dysplasia

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.

Occurrence and risk factors of renal artery stenosis in patients undergoing abdominal computed tomography angiography, a one-year study in a tertiary hospital in Manila

@#Renal artery stenosis (RAS) is one of the most common primary diseases of the renal arteries. It is associated with hypertension as well as ischemic renal disease. Its concomitant presence constitutes a high cardiovascular risk, thus timely diagnosis is important. This study aims to determine the occurrence of RAS in patients undergoing abdominal CT angiography and determines other factors that predict the highest-risk subset who have signifi cant renal artery narrowing. A retrospective review of records of 470 patients who underwent CT angiography of the whole abdomen was done. RAS occurs in about 9.57% of patients who had a CT scan of the whole abdomen. Older patients, patients with heart disease and patients with more atherosclerotic vessels in the abdomen increases the risk for signifi cant RAS (p values of 0.005, 0.008 and <0.001, respectively). Diabetes, hypertension as well as other imaging fi ndings such as the presence of aneurysm elsewhere seen in the abdominal scans and renal artery duplication do not statistically correlate with the increased risk for RAS in this study

Displasia fibromuscular en la Argentina. Registro del grupo de trabajo de Hipertensión Secundaria de la Sociedad Argentina de Hipertensión Arterial (SAHARA-DF): proyecto de protocolo y resultados preliminares / Fibromuscular dysplasia in Argentina. A registry from the Secondary Hypertension working group of the Argentine Society of Hypertension (SAHARA-DF): project protocol and preliminary results

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)

Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia

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.

Incomplete Oculomotor Nerve Palsy Caused by Internal Carotid Artery Giant Aneurysm with Fibromuscular Dysplasia

No abstract available.

Aneurisma de arteria renal y carcinoma de células claras metastásico / Renal Artery Aneurysm and Metastatic Clear Cell Renal Carcinoma

Introducción: Los aneurismas de arteria renal son poco frecuentes, la mayoría asintomáticos. Se pueden asociar a la hipertensión arterial, fibrodisplasia, arteriosclerosis sistémica y aneurismas en otras localizaciones. Son más frecuentes en mujeres. El cáncer renal representa el 2%­3% de todos los tumores con una incidencia creciente en las últimas décadas. Predomina en los varones y es conocida su gran capacidad para metastatizar. Se presenta un caso clínico en el que de forma poco habitual, coexisten ambas entidades.n Caso Clínico: Mujer de 52 años con antecedente de autotrasplante de riñón derecho tras cirugía de aneurisma de arteria renal. Durante el seguimiento, se objetiva masa renal en dicha unidad que precisa cirugía con resultado de carcinoma de células claras. Cuatro años más tarde progresa con metástasis a nivel de suprarrenal izquierda realizándose adrenalectomía izquierda laparoscópica. Discusión: La indicación de tratamiento de los aneurismas de arteria renal radica en evitar sus complicaciones, principalmente la rotura por su capacidad letal. Entre las técnicas aún tiene su papel la cirugía exvivo y el autotrasplante. El tratamiento del cáncer renal se basa en conseguir la radicalidad en estadios localizados; por otro lado, en enfermedad avanzada, la cirugía puede estar indicada en pacientes seleccionados. En ese caso, el seguimiento del aneurisma de arteria renal permite realizar un diagnóstico y tratamiento temprano del cáncer.

Introduction: Renal artery aneurysms are rare and most of them are asymptomatic. They could be linked to arterial hypertension, fibromuscular dysplasia, systemic arteriosclerosis and other visceral aneurysms. They appear more frequently in women. Renal cancer represents 2­3% among all the malignancies. In the last years, the incidence is increasing and it is slightly more common in men. It is Known its widely capacity to develop metastasis. We report the rare coexistence of both entities in the same patient. Clinical Case: Fifty-two year-old-woman, right kidney auto transplantation operated because of renal artery aneurysm surgery. In a scheduled examination, she presents a mass in that kidney, which turns out to be a clear cell carcinoma. Four years later, a left adrenal metastasis is detected and a laparoscopic adrenalectomy is performed. Discussion: Aneurysms treatment has the purpose of avoid its complications, mainly its rupture because it can cause death. Among the several techniques described, exvivo surgery and auto transplantation is one of them. Renal cancer treatment aims to be radical in localized stages. On the other hand, in some selected advanced renal cancer patients, surgery has an indication too. In the present case, the renal aneurism follow-up allow us to perform an early diagnosis and treatment of the neoplasm.

Sudden Cardiac Death due to Coronary Fibromuscular Dysplasia: Case Report and Literature Review / 대한법의학회지

Fibromuscular dysplasia (FMD) of the coronary artery is a rare cause of sudden cardiac death; however, its prevalence and fatality may have been overlooked so far. A 47-year-old man complained of pain in his back and shoulder and became unconscious. Despite resuscitation, he died 3 hours after symptom onset. The heart weight was in the normal range; however, all three major coronary arteries showed intimal thickening without atherosclerosis or inflammatory cell infiltration. Fragmentations and duplications of the internal elastic lamina which are histologic features of intimal fibroplasia, a focal-type FMD, were observed. The prevalence of coronary FMD remains unknown, although it may be related to spontaneous coronary artery dissection and sudden death. The histopathologic confirmation of coronary FMD and exclusion of other possible coronary diseases through autopsy are essential to reveal the nature of the disease and therefore apply the information in dealing with legal problems after death.

Spontaneous coronary artery dissection: an under-recognized cause of acute coronary syndromes

Spontaneous coronary artery dissection [SCAD] is a rare condition that is often underdiagnosed given limitations of conventional cineangiography

In addition to the diagnostic challenge, the condition poses a major therapeutic dilemma given paucity of literature to guide management. We report the case of a 55-year-old woman, who presented with acute coronary syndrome

Coronary angiography at the time of the index hospitalization revealed type 2 SCAD. She was managed conservatively. Repeat coronary angiography three months later showed complete resolution of the previously noted dissection. Because of the high association between SCAD and fibromuscular dysplasia [FMD], a cross-sectional imaging was performed in this case, which ruled out underlying FMD. The patient has been followed longitudinally since her index event and has had no reported recurrences

Enfermedad aneurismática difusa e hipertensión arterial: displasia fibromuscular / Diffuse aneurysmal disease and arterial hypertension: fibromuscular dysplasia

Resumen La displasia fibromuscular es una enfermedad vascular no arteriosclerótica, que causa la hipertensión arterial secundaria y cursa con aneurismas en las arterias de pequeño y mediano calibre. El compromiso de las arterias renales en la displasia fibromuscular es una de las causas más comunes de la hipertensión secundaria en niños y en adultos jóvenes. Se describe el caso de una paciente que se presentó como una emergencia hipertensiva, en la cual, con base en los estudios imagenológicos se le documenta la enfermedad aneurismática y más adelante la displasia fibromuscular. Se discute en el presente reporte la presentación clínica de la enfermedad, la clasificación patológica y los diagnósticos diferenciales.

Abstract Fibromuscular dysplasia is a nonatherosclerotic vascular disease that causes secondary arterial hypertension associated with aneurysms in small and medium sized arteries. Involvement of renal arteries in fibromuscular dysplasia is one of the most common causes of secondary hypertension in children and young adults. The case of a female patient is described where she presented a hypertensive emergency and, based on imaging results, the aneurysmal disease is documented and further on fibromuscular dysplasia. This report discusses the clinical presentation of the disease, its pathological classification and differential diagnoses.

Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis / 소아과

Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS.

Spontaneous renal artery dissection in Ehlers-Danlos syndrome / 영남의대학술지

Primary dissection of the renal artery is rare. Spontaneous renal artery dissection can be associated with diseases such as medial degeneration, neurofibromatosis, syphilitic arteritis, tuberculosis, polyarteritis nodosa, Marfan syndrome, fibromuscular dysplasia, or Ehlers-Danlos syndrome (EDS). Among these causes, EDS related renal artery dissection is very rare worldwide and has not been previously reported in Korea. EDS are a group of heritable connective tissue disorders characterized by fragility of the skin and hypermobility of the joints. We describe the case history of a young man who presented with left side flank pain, hypermobility of the hand joints and showed left renal artery dissection on computed tomography and angiography that turned out to be the first complication of vascular type EDS.

Abordagem endovascular de paciente com fibrodisplasia de artéria renal bilateral associada a volumoso aneurisma renal / Endovascular approach of a patient with bilateral renal artery fibrodysplasia associated with a massive renal aneurysm

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.

Fibromuscular dysplasia: a cause of secondary hypertension

No abstract available.

Hipertensión arterial secundaria a displasia fibromuscular de la arteria renal. A propósito de un caso / Hypertension secondary to fibromuscular dysplasia of the renal artery. Report of a case

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...