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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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Objective:To analyze the clinical characteristics and treatment of patients with renovascular hypertension (RVH) caused by renal arterial fibromuscular dysplasia (FMD).Methods:Clinical data and treatment result of 38 patients with renal arterial FMD and RVH admitted to our hospital from Jan 2014 to Dec 2020 were reviewed.Results:A total of 38 patients were enrolled in this study. Renal artery CTA showed that 40 renal arteries were involved, among these 6 branches had multifocal stenosis, and 34 branches had focal stenosis. Thity-three patients received surgical treatment, of which 32 patients underwent percutaneous transluminal renal angioplasty (PTRA), and 1 patient with renal aneurysm underwent renal artery stent implantation combined with aneurysm coil embolization. Postoperative blood pressure was significantly lower than that before the operation [(129.79±17.63) mmHg vs. (178.52±28.63) mmHg, t=-11.42, P<0.001]. The mean follow-up time was 35.5 months. Renal artery restenosis occurred in 4 patients and underwent reintervention. Conclusion:For patients with renal arterial FMD and RVH, PTRA is safe and effective, especially for patients with focal lesions, with fair short and mid-term prognosis.
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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.
الموضوعات
Humans , Female , Adult , Carotid Artery, Internal/pathology , Carotid Artery, Internal, Dissection/complications , Fibromuscular Dysplasia/complications , Constriction, Pathologic , Carotid Artery, Internal, Dissection/surgery , Carotid Artery, Internal, Dissection/diagnostic imagingالملخص
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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Transit-Oriented Developmentالملخص
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.
الموضوعات
Humans , Female , Middle Aged , Stroke , Carotid Artery, Internal, Dissection , Fibromuscular Dysplasia , Infarctionالملخص
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)
الموضوعات
Humans , Male , Female , Adolescent , Adult , Middle Aged , Young Adult , Records/statistics & numerical data , Fibromuscular Dysplasia/diagnosis , Argentina/epidemiology , Algorithms , Bias , Sex Factors , Cross-Sectional Studies , Risk Factors , Age Factors , Angioplasty/methods , Cultural Factors , Vascular System Injuries/diagnostic imaging , Fibromuscular Dysplasia/classification , Fibromuscular Dysplasia/etiology , Fibromuscular Dysplasia/therapy , Fibromuscular Dysplasia/epidemiology , Hypertension/epidemiology , Aortic Dissection/diagnostic imagingالملخص
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.
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Humans , Male , Adult , Subarachnoid Hemorrhage/complications , Carotid Stenosis/etiology , Endovascular Procedures/rehabilitation , Fibromuscular Dysplasia/complications , Aortic Dissection/diagnostic imaging , Angioplasty/methods , Vascular Malformations/complicationsالملخص
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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Objective To explore the clinical characteristics, diagnosis and treatment of refractory hypertension caused by renal artery fibromuscular dysplasia (FMD) in children. Method The clinical data of a child with refractory hypertension caused by FMD were retrospectively analyzed. Results A boy, with onset at age of 4 years, had significantly increased blood pressure and decreased serum potassium. His renin and angiotensin levels were significantly elevated. The boy was diagnosed with renovascular hypertension by color Doppler ultrasonography, enhanced thoracoabdominal CT and vascular reconstruction. Oral administration of a variety of antihypertensive drugs is not effective. The child was finally diagnosed of refractory hypertension caused by FMD using renal angiography. The child was not suitable for surgery. After adjusting the antihypertensive drugs, the blood pressure of the child tended to be stable. The boy received continuous follow-up for 8 years. The antihypertensive regimen was adjusted annually and blood pressure fluctuations and target organ damage were dynamically assessed. Conclusion Children with FMD caused renal vascular hypertension may have no specific manifestations in the early stage. The drug treatment is preferred, and surgical treatment may be considered.
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Carotid web is a rare risk factor of ischemic stroke.A total of 32 (0.54％) patients with carotid web were finally diagnosed in 5 943 patients who underwent carotid computerized tomography angiography (CTA) in two hospitals.Only one patient received carotid endarterectomy that pathological findings were fibrous tissue hyperplasia of vascular wall with mucinous degeneration.Stent implantation was administrated in two cases.Among 13 asymptomatic patients,the observational follow-up period was (20.9± 12.4) months without strokes.Carotid web is a rare aberration.Asymptomatic patients with carotid web are usually silent.Large sized cohort and long-term follow-up are further needed.
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Objective To investigate the efficacy and safty of percutaneous transluminal angioplasty (PTA) of renal artery stenosis resulting from fibromuscular dysplasia.Methods A total of 32 patients with fibromuscular dysplasia complicated with hypertension in level 2 to 3 or refractory hypertension were enrolled,who underwent PTA.The blood pressure,types of medication,restenosis,survival rates and complications were observed after treatment.Results Technical success rate of PTA was 93.94％ (31/33).The blood pressure decreased from (189.6±26.0)mmHg/(121.6± 21.7)mmHg before operation to (136.6±8.0)mmHg/(81.1±11.5)mmHg after operation (t＝9.117,7.745,both P＜ 0.001).No renal artery rupture,interlayer,branch blockage,thrombosis and other related complications and postoperative adverse events occurred.All patients were followed up for 5 months to 100 months,with an average of (40.4±26.1) months.Survival rate was 100％ (32/32).Conclusion PTA is safe and effective for treating renal artery stenosis from fibromuscular dysplasia.
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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.
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Humans , Middle Aged , Atherosclerosis , Autopsy , Cause of Death , Coronary Disease , Coronary Vessels , Death, Sudden , Death, Sudden, Cardiac , Fibromuscular Dysplasia , Heart , Prevalence , Reference Values , Resuscitation , Shoulder , Tunica Intimaالملخص
Objective To explore the lesion types of renal branches and evaluate the clinical results of endovascular treatment for renal artery branch lesions associated with fibromuscular dysplasia (FMD).Methods Eight cases with renal artery branch lesions due to FMD from June 2014 to December 2015 in Department of Vascular and Endovascnlar Surgery,Henan Provincial People's Hospital were retrospectively reviewed.The computed tomography angiography was routinely performed to ascertain the property of renal artery lesion,and digital-subtraction angiography was further performed to confirm FMD diagnosis and analyze lesion characteristics.The renal artery branch lesion types,blood pressure (BP),renin activity,glomerular filtration rate (GFR) and restenosis after operation were observed,and the outcomes were recorded.Results In 8 patients,6 patients were found with the second grade renal branch lesions and 2 patients were found with tertiary branch lesions,involving 2 and more branch arteries for 4 cases and 1 branch for 4 cases.Five cases with branch-type cystic dilatation or aneurysms with coexisting stenosis including 1 case with dissecting aneurysm and renal local infarction,and 1 case with aneurysm located in the bifurcations of branches,and 3 cases with only branch stenosis were found.The angiography showed the small branch lesions of renal artery indicating FMD-mediated focal (5 cases) and multiple stenosis (3 cases).The branch stenosis was not operated in 1 case with dissecting aneurysm and renal local infarction,as BP could be controlled by antihypertensive treatment.Other 7 cases underwent endovascular angioplasties and the technical successful rate was 100％ without complications.After 1 week of operation,BP was improved,renal function remained normal,plasma renin activity was reduced and GFR was recovered for all patients.During the follow-up period (3 to 12 months),BP improvement was observed in 3 cases,and cure were found in 4 cases,and patient's GFR and plasma renin activity were improved in all patients.There were 2 cases with re-intervention during the follow-up period due to restenosis,and the involved branches were unobstructed without obvious stenosis after 1-year follow-up.Conclusions FMD can affect the renal branch artery with different lesions types and endovascular treatment is the first choice for the renal branch lesion of FMD.
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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.
الموضوعات
Humans , Female , Adult , Renal Artery , Fibromuscular Dysplasia/etiology , Fibromuscular Dysplasia/therapy , Renal Artery Obstruction/complications , Endovascular Procedures/methods , Coronary Vessels , Angioplasty/methods , Cefuroxime/administration & dosage , Hypertension , Stents , Ultrasonography, Doppler/methodsالملخص
Objective To analyze the basal conditions of 5 patients with cerebrovascular fibromuscular dysplasia (FMD),and explore the clinical presentation,imaging features,therapies and outcomes of cerebrovascular FMD.Methods Five patients with cerebrovascular FMD,admitted to our hospital from January 2012 to April 2013,were chosen in our study; their medical history and imaging features were collected,and their clinical presentation,imaging features,therapies and outcomes were retrospectively analyzed when combining with literature review.Results Five patients all presented with stroke,including four having hemiplegia as the initial symptom,and one having headache with nausea and vomiting as the main performance.One was diagnosed as having subarachnoid hemorrhage by CT scan,and four had different infarction lesions in lobes and basal ganglia on MRI.Artery dissection was discovered in all patients by cerebral digital substraction angiography,including two with right internal carotid artery dissection,one with right vertebral artery dissection combined with aneurysm in anterior communicating artery associated with "string-of-beads" appearance on C1 segment of right internal carotid artery and left vertebral artery,one with bilateral vertebral artery dissection,and the last one with bilateral internal carotid artery dissection and moyamoya disease-like vessels.No abnormality was discovered in two patients by renal artery angiography.The patient with anterior communicating artery aneurysm was treated with endovascular aneurysm embolization,having poor prognosis.In the remaining 4 patients with cerebral infarction,two were treated with carotid artery stenting,and the other 2 were only given antiplatelet therapy.No patient suffered cerebrovascular accidents during the 12-month follow-up.Conclusions Cerebrovascular FMD is a rare cause of young stroke,and patients with cerebrovascular FMD often manifest headache,neck bruit,carotid artery dissection and stroke.It is particularly important to make definitive diagnosis by performing cerebrovascular imaging examinations and give treatment accordingly.The long term outcome of FMD is not clear now.
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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...
الموضوعات
Humans , Adolescent , Female , Fibromuscular Dysplasia/complications , Fibromuscular Dysplasia/diagnosis , Fibromuscular Dysplasia/therapy , Hypertension, Renovascular/etiology , Renal Artery Obstruction/therapy , Echocardiography, Doppler , Physical Examinationالملخص
A displasia fibromuscular (DFM) envolve artérias de pequeno e médio calibre e é uma causa bem conhecida de hipertensão em mulheres jovens caucasianas, quando envolve as artérias renais. A etiologia da DFM permanece desconhecida, a despeito de inúmeras teorias. Há suspeita de um componente genético, já que a doença atinge primariamente caucasianos. Também é descrita associação entre DFM e antígeno de histocompatibilidade HlA-DRw6. Os principais sítios acometidos são as artérias renais, cerebrais, carótidas, viscerais, ilíacas, subclávias, braquiais e poplíteas. As manifestações clínicas correlacionam-se com o sítio acometido, e a hipertensão arterial sistêmica é um sintoma frequente pelo acometimento das artérias renais em 60 por cento-75 por cento dos casos. O diagnóstico da DFM é feito por meio de exame histopatológico e/ou angiográfico. A DFM pode apresentar-se como doença vascular sistêmica, mimetizando vasculites. Essa compreensão é importante porque tanto a vasculite quanto a DFM podem ter curso clínico grave, e exigem tratamentos diferentes. O diagnóstico diferencial pode ser difícil diante de uma apresentação clínica atípica ou da incapacidade de obter a confirmação histopatológica. Há relatos isolados de DFM mimetizando poliarterite nodosa, síndrome de Ehlers-Danlos, síndrome de Alport, feocromocitoma, síndrome de Marfan e arterite de Takayasu. Os reumatologistas devem estar atentos para fazer esse diagnóstico diferencial. O tratamento da DFM é recomendado apenas em casos sintomáticos, e consiste em revascularização (cirúrgica ou por angioplastia percutânea transluminal). Na DFM, efeitos da corticoterapia podem ser direta e rapidamente deletérios para a parede vascular, levando à piora das lesões.
Fibromuscular dysplasia (FMD) involves small- and medium-sized arteries, being a well-known cause of hypertension in young Caucasian women, when renal arteries are involved. The etiology of FMD remains unknown, despite many theories. A genetic component is suspected to exist, because the pathology affects primarily Caucasians. Association between FMD and the HLA-DRw6 histocompatibility antigen has also been described. The major sites affected are renal, cerebral, carotid, visceral, iliac, subclavian, brachial and popliteal arteries. Clinical manifestations correlate with the affected site, arterial hypertension being a frequent symptom, resulting from the involvement of the renal arteries in 60 percent-75 percent of the cases. The diagnosis of FMD is made by histopathology and/or angiography. FMD can manifest as a systemic vascular disease, mimicking vasculitis. This understanding is important because vasculitis and FMD can both have a severe clinical course, but require distinct treatments. The differential diagnosis can be difficult in face of an atypical clinical presentation or lack of histopathologic confirmation. Isolated cases of FMD have been reported mimicking the following conditions: polyarteritis nodosa, Ehlers-Danlos's syndrome, Alport's syndrome, pheochromocytoma, Marfan's syndrome, and Takayasu's arteritis. Rheumatologists should be aware of this differential diagnosis. Treatment of FMD is recommended only in symptomatic cases, and consists in revascularization, which may be either surgical or via percutaneous transluminal angioplasty. In FMD, the effects of corticotherapy can directly and rapidly harm the vascular wall, aggravating the lesions.
الموضوعات
Female , Humans , Fibromuscular Dysplasia/diagnosis , Vasculitis/diagnosis , Diagnosis, Differentialالملخص
We found a case of hypoplasia of vertebral artery with fibromuscular dysplasia in an 82-yr-old Korean female cadaver during a routine dissection course. In the present case, intracranial hypoplasia in left vertebral artery and bilateral origin of posterior inferior cerebellar artery at the vertebrobasilar junction were recognized. Histopathologically, left vertebral artery showed intimal type of fibromuscular dysplasia both in its extracranial and intracranial courses. These results indicate that the association of fibromuscular dysplasia and hypoplasia does exist in the vertebral artery, although the etiologies are not verified yet.
الموضوعات
Aged, 80 and over , Female , Humans , Fibromuscular Dysplasia/pathology , Heart Ventricles/abnormalities , Republic of Korea , Vertebral Artery/pathologyالملخص
Objective To evaluate the clinical characteristics and surgical treatment of renal stenosis resulting from fibromuscular dysplasia ( FMD ). Methods 16 cases from 1998 to 2011 were reviewed,the average age was (28 ± 13 ) years old. Among them, all patients suffered refractory hypertension and only one presented renal dysfunction. Aorto-renal bypass was performed in 2 cases.Resection and reconstruction was performed in 2 cases,balloon angioplasty in 10 and stent implantation in 3.Results There was 1 death peri-operatively.13 out of 15 cases were followed-up with no death.Significant decline of blood pressure [ (134 ± 14 )/( 83 ± 8 ) mm Hg vs.( 151 ± 17 )/( 96 ± 16 ) mm H g ( P ＜ 0.01 ) ]and 100％ effective rate were observed.The creatine level were preserved after surgical treatment [ (61 ±22) μmol/L vs.(69 ± 22) μmol/L,(P ＞ 0.05 ) ] with 4 cases improved and the other 9 stable.12 patients received ultrasound surveillance with only one case of 20％ restenosis. Conclusions Renal stenosis due to FMD are most common in children and the youth with hypertension as major presentation. Balloon angioplasty is the first choice of surgical treatment with positive efficacy.