Reversible Cerebral Vasoconstriction Syndrome And Fibromuscular Dysplasia: An Epiphenomenon Or A Causal Relationship?

Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic arterial disease that primarily affects middle-aged Caucasian women. Carotid web (CW) is a variant of FMD characterized by a nonatheromatous, membrane-like tissue protrusion into the carotid bulb. Reversible cerebral vasoconstriction syndrome (RCVS) is defined by severe headaches and reversible narrowing of cerebral arteries, which typically resolves within three months. While most RCVS cases have identifiable triggers, a significant portion occurs without known causes. Recent studies have reported a high prevalence of neurovascular abnormalities in RCVS patients. We present a case of a thirty-year-old woman with a sudden-onset severe headache, diagnosed with RCVS associated with carotid web. The patient had no ischemic involvement and responded well to flunarizine treatment. Follow-up imaging showed no stenosis. This case highlights a potential association between carotid web and RCVS, suggesting that FMD may contribute to vascular hyperreactivity and presents as a risk factor for RCVS. Further investigations are needed to understand the underlying mechanisms connecting these two vascular disorders. Keywords: reversible vasoconstriction syndrome; fibromuscular dysplasia; carotid web; structural abnormalities; vascular hyperreactivity.

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.

Complications of Endovascular Treatment in Fibromuscular Dysplasia.

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.

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.

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...

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.

Systemic hypertension and transient ischemic attack in a 6-year-old girl with fibromuscular dysplasia treated with percutaneous angioplasty.

We describe a 6-year-old girl with arterial hypertension secondary to fibromuscular dysplasia with stenoses of both renal arteries and transient ischemic attack due to extracranial right internal carotid artery subtotal occlusion as well as left internal carotid artery stenosis. She was treated with percutaneous angioplasty of both renal and both carotid arteries.

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

Isolated intracranial fibromuscular dysplasia is rare and may present with cerebrovascular events. It should be considered as etiology of stroke in otherwise healthy young patients. Though diagnosis is often challenging, characteristic morphologies may be revealed on magnetic resonance and catheter angiography. Cephalocervical fibromuscular dysplasia typically involves the extracranial portion of the internal carotid artery (nearly 95%). This rare case demonstrates isolated intracranial fibromuscular dysplasia in a 19-year-old female with left caudate and genu of internal capsule stroke.

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.

[Systemic fibromuscular dysplasia. Supra aortic and renal artery involvement]. / Displasia fibromuscular sistémica. Compromiso de ramas supraaórticas y renales.

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.

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

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

[Postangioplasty renal artery thrombosis treated intraluminally with thrombolysis. A case report]. / Trombosis arterial renal postangioplastía intraluminal tratada con trombólisis. Informe de un caso.

The authors present the clinical case of a 31 year female with systemic vascular hypertension secondary to a 65 percent unilateral fibromuscular renal artery stenosis. The centellographic study demonstrated hypoperfusion on the right kidney and serious functional damage; the patient was subject to intraluminal renal angioplasty resulting in right renal arterial thrombosis, which was treated successfully with intra-arterial and intravenous streptokinase infusion. Following the thrombotic therapy, recanalization of the thrombosed artery and normal renal excretion of the contrast substance was observed.