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.

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.

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...

Percutaneous Transluminal Angioplasty of Renal Artery Fibromuscular Dysplasia: Mid-term Results

OBJECTIVE: To evaluate mid-term imaging, clinical follow-up, and restenosis rates from patients that had undergone percutaneous transluminal renal artery angioplasty (PTRA) for symptomatic renal artery fibromuscular dysplasia (FMD). MATERIALS AND METHODS: Between March 1999 and July 2006, 16 consecutive renal artery FMD patients underwent PTRA for poorly controlled hypertension. The patients were enrolled into this retrospective study after receiving 19 primary and four secondary PTRAs in 19 renal artery segments. Follow-up monitoring of blood pressure, use of antihypertensive medication, and the serum creatinine level after PTRA were assessed at 1, 3, 6, 9, 12 months, and each following year. The degree of restenosis was evaluated with computed tomographic angiography (CTA) after PTRA at 6, 12 months, and every year if possible. Technical and clinical success rates for the treatment of FMD, and restenosis rates for the renal artery were evaluated. RESULTS: The technical success rate for primary PTRA was 79% (15/19) and the complication rate was 16% (3/19). Hypertension improved in 80% (12/15) of the patients after four weeks follow-up, and was finally cured or improved in 93% (14/15) during the mean follow-up period of 23.6 months. There was a cumulative 22% (4/18) restenosis rate during the follow-up period. All of the patients were treated with a second PTRA without complications and all of the patients were cured of hypertension after the second PTRA. CONCLUSION: Percutaneous transluminal renal artery angioplasty for clinically symptomatic renal FMD is technically and clinically successful and safe to perform. For all patients with restenosis, there was a good response after undergoing a second PTRA.

Isolated intracranial fibromuscular dysplasia presents as stroke in a 19-year-old female

Displasia fibromuscular intracraniana isolada é rara e pode se apresentar como acidente vascular cerebral (AVC). Ela deve ser considerada como etiologia do AVC em paciente jovem saudável. Embora o seu diagnóstico possa ser um desafio, características morfológicas específicas podem ser reveladas através da angiografia através de ressonância nuclear magnética e por de catéter. A displasia fibromuscular envolvendo a circulação cérvico-cefálica afeta tipicamente a artéria carótida interna extracraniana (95%). O presente caso relata uma rara ocorrência de displasia fibromuscular intracraniana em uma jovem de 19 anos com infarto do núcleo caudado e joelho da cápsula interna à esquerda.

Displasia fibromuscular atípica: relato de caso / Atypical fibromuscular dysplasia: case report

É relatada uma forma atípica de displasia fibromuscular da artéria carótida interna cuja denominação é causa de grande confusão na literatura devido a sua raridade. Há menos de 20 casos descritos. Sua aparência em forma de septo é bem singular. A lesão foi causa de isquemia cerebral embólica transitória nuam paciente de 49 anos de idade. A paciente foi submetida a arteriotomia e a membrana rígida, que se originava na parede da artéria carótida interna projetando-se para luz arterial, foi removida. O aspecto histopatológico é a presentado e as controvérsia na nomenclatura dessa lesão é discutida.

Displasia fibromuscular sistémica: compromiso de ramas supraaórticas y renales / Systemic fibromuscular dysplasia with supra aortic and renal artery involvement: report of 1 case

We report a 39 years old male presenting with an intense hemicrania pain in the tract of the carotid artery and Claude Bernard Horner syndrome. The patient had also a high blood pressure. Magnetic resonance imaging demonstrated a left carotid artery fibrodysplastic disease. Conventional angiography of 4 cerebral vessels showed a dissection of the left carotid artery and fibromuscular dysplasia of the left vertebral artery. Renal angiography showed a stenosis and occlusion of renal artery with radiological exclusion of right kidney. On the left side there was a stenosis and occlusion of segmental branches. The diagnostic importance of Claude Bernard Horner syndrome and the need to study renal artery involvement when carotid dissection is accompanied with high blood pressure, is highlighted

Long term follow-up of percutaneous transluminal renal angioplasty with special reference to aorto-arteritis.

Percutaneous transluminal renal angioplasty (PTRA) was attempted in 96 patients of renovascular hypertension (RVHT) admitted during the period 1986 to 1992. The patients' age ranged from 14-70 (mean: 38.7 +/- 18.8) years. There were 42 (43.8%) males and 54 (56.2%) females. The cause of renal artery stenosis (RAS) was aorto-arteritis in 44 (45.8%), atherosclerosis in 28 (29.2%) and fibromuscular dysplasia in 24 (25%). Bilateral RAS was found in 16 (16.7%). PTRA was angiographically successful in 92 (95.8%) patients. The mean pressure gradient decreased from 82.6 +/- 8.2 to 11.2 +/- 3.6 mm Hg in aorto-arteritis, 75.2 +/- 13.2 to 9.6 +/- 6.4 mm Hg in atherosclerosis and from 86.4 +/- 10.6 to 13.2 +/- 8.2 mm Hg in fibromuscular dysplasia respectively. The patients were followed up for 43.2 +/- 24.1 (range: 6-77) months. Remission or satisfactory lowering of blood pressure was achieved in 80 (86.9%) patients. Clinical success rate (remission or satisfactory lowering of BP) at the end of follow-up period was 75.3 percent. Randomly selected repeat angiography was done in 45 out of 92 (49.1%) patients at the end of 24 months of follow-up. Restenosis was detected in 12 (26.7%) patients and was commonest in the atherosclerotic group (42.8%), followed by fibromuscular dysplasia (14.3%) and least common in aorto-arteritis (11.8%). Repeat angioplasty was done successfully in 10 (83.3%) patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Tratamento cirúrgico da hipertensäo / Surgical treatment of Hypertension

Relata-se um caso de Hipertensäo Renovascular devido a displasia fibromuscular da artéria renal em uma mulher de 45 anos com hipertensäo há 24 anos. Verificou-se um sopro sistólico em regiäo abdominal superior direita. A investigaçäo constou de exames laboratoriais, ultra-sonografia abdominal, urografia excretora, cintilografia renal e arteriografia renal. Optou-se pelo tratamento cirúrgico, obtendo-se a normalizaçäo dos níveis pressóricos, com boa evoluçäo clínica. Comenta-se a rotina de investigaçäo, prognóstico e técnica cirúrgica

Renal angioplasty in a case of renovascular hypertension presenting with stroke.

A young female patient with renovascular hypertension due to fibromuscular dysplasia of the right renal artery presenting with stroke is described. The patient was treated by renal angioplasty.

Primary intimal fibroplasia of the renal artery

Two cases of primary intimal fibroplasia of the renal artery with renovascular hypertension are described. Case 1 was 27 year old female who was incidentally found to have blood pressure of 210/130 mmHg on routine physical examination. Renal arteriogram revealed tubular narrowing of the mid protion of the left renal artery. Both patients showed lateralization in renin activity at the involved side renal vein. Case 2 was a 10 year old girl who was first noted to have a hypertension of 180/120mmHg after a sudden attack of seizure, vomiting and altered consciousness. Renal arteriogram showed concentric narrowing of the proximal half of the right renal artery. Histopathologic examination of the affected arterial segments from both cases showed essentially same findings, i,e., diffuse fibrous thickening of the intima occluding the lumen, focal fragmentation, duplication and disappearance of the internal elastic membranes. There were no deposit of lipid and inflammatory cells. The media and adventitia remained intact. The blood pressure of both patients became normal, after the surgery and the patients are in good health up to this time.

Hipertensión renovascular secundaria a estenosis de la arteria renal izquierda, por enfermedad fibromuscular: un caso operado en el Hospital del Niño / Renovascular hypertension secondary to stenosis of renal left artery by fibromuscular disease: an operated case in the Hospital del Niño

Se reporta un caso de Hipertensión Renovascular secundaria a Estenosis de la Arteria Renal izquierda debido a enfermedad fibromuscular y probable Endarteritis Sistémica en adolescente de 13 años. Hallazgo de Hipertensión casual en consulta por resfriado. Se realizaron estudios pertinentes: Arteriografía Renal, Pruebas Inmunológicas, P.I.V., pruebas de Funcionamiento Renal y se somete a cirugía con puente Aorta-Renal con éxito. Actualmente normotensa y controlada en la Consulta Externa por Urología y Cirugía Cardiovascular. Al momento de confeccionar el material la paciente tenía un mes de operada