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Cleveland Clinic Journal of Medicine Nov 2016Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries... (Review)
Review
Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries that affects mainly women. While the true prevalence of this disease remains unknown, studies suggest that more people may be affected than previously reported. Better understanding of the clinical manifestations and natural history of FMD and advances in diagnostic imaging have altered the clinical approach to managing patients with this uncommon vascular disease. Although there are a multitude of unanswered questions regarding FMD, this review highlights recent insights and how this information has modified clinical care for those affected.
Topics: Fibromuscular Dysplasia; Humans
PubMed: 27861117
DOI: 10.3949/ccjm.83.s2.06 -
Tidsskrift For Den Norske Laegeforening... Feb 2019Fibromuscular dysplasia affects the muscles of small and medium-sized arteries. The aetiology of the condition is unknown; it is most frequently seen in middle-aged... (Review)
Review
Fibromuscular dysplasia affects the muscles of small and medium-sized arteries. The aetiology of the condition is unknown; it is most frequently seen in middle-aged women, but can affect both sexes at any age. Hypertension is the most common clinical manifestation when the renal arteries are affected. The diagnosis is made based on clinical suspicion and specific angiographic findings. The treatment is aimed at normalisation of blood pressure with the aid of drugs or through revacularisation.
Topics: Angiography; Fibromuscular Dysplasia; Humans; Hypertension; Magnetic Resonance Angiography; Renal Artery
PubMed: 30808101
DOI: 10.4045/tidsskr.18.0226 -
The Neuroradiology Journal Dec 2021Carotid web is thought to be a focal intimal variant of fibromuscular dysplasia, which comprises a high risk of stroke because of blood stasis and subsequent coagulative...
Carotid web is thought to be a focal intimal variant of fibromuscular dysplasia, which comprises a high risk of stroke because of blood stasis and subsequent coagulative reactions that occur distal to the web. These lesions generally involve the posterolateral wall of the carotid and their developmental pathogenesis is controversial. This case report describes a 51-year-old woman who presented to the hospital with sudden onset aphasia, right hemi-sensory loss, and right visual field cut. Magnetic resonance imaging (MRI) of the brain demonstrated a left middle cerebral artery (MCA) distribution embolic ischemic infarct with shelf-like linear filling defects in the carotid bulb bilaterally on a computed tomography angiography (CTA) of the head and neck consistent with bilateral carotid webs that were confirmed by catheter angiography. The carotid webs were projecting on the left posteriorly and on the right anteriorly into the inferior aspects of the bilateral proximal internal carotid arteries. The patient was started on clopidogrel and a high-intensity statin and remained on Plavix monotherapy for a 10-month follow up without a recurrent ischemic event.
Topics: Brain Ischemia; Carotid Arteries; Carotid Artery, Internal; Female; Fibromuscular Dysplasia; Humans; Middle Aged; Stroke
PubMed: 34000900
DOI: 10.1177/19714009211017783 -
Surgical Neurology International 2022Fibromuscular dysplasia (FMD) is a noninflammatory and nonatherosclerotic arteriopathy that is characterized by irregular cellular proliferation and deformed... (Review)
Review
BACKGROUND
Fibromuscular dysplasia (FMD) is a noninflammatory and nonatherosclerotic arteriopathy that is characterized by irregular cellular proliferation and deformed construction of the arterial wall that causes segmentation, constriction, or aneurysm in the intermediate-sized arteries. The incidence of FMD is 0.42-3.4%, and the unilateral occurrence is even rarer. Herein, we report a rare case of a localized extracranial carotid unilateral FMD associated with recurrent transient ischemic attacks (TIAs) treated by extracranial-intracranial bypass for indirect revascularization. The specific localization of the disease rendered our case unique.
METHODS
We conducted a review of the PubMed Medline database search using the following combined formula: ((FMD [Title/Abstract]) AND ((isolated [Title/Abstract]) OR (localized [Title/Abstract]))) AND Internal carotid artery (ICA) (Title/Abstract). Additional resources were included by screening the reference list of the selected papers.
RESULTS
A total of six cases were found, and all accounted for localized FMD affecting the ICA. The age range was between 19 and 52, the male-to-female ratio was (2:4), and all of the cases consisted of unilateral carotid FMD, mainly on the left side with a left-to-right ratio of 5:1. The management and outcome of these cases varied according to the case and associated complications.
CONCLUSION
Extracranial localized FMD of the ICA is a rare subtype of FMD that has little documentation in the literature. In our case, it was a localized extracranial carotid unilateral FMD associated with recurrent TIAs. The appropriate treatment was using the intracranial-extracranial bypass.
PubMed: 36447869
DOI: 10.25259/SNI_937_2022 -
World Journal of Gastroenterology Aug 2018The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are... (Review)
Review
The vascular diseases including aneurysm, occlusion, and thromboses in the mesenteric lesions could cause severe symptoms and appropriate diagnosis and treatment are essential for managing patients. With the development and improvement of imaging modalities, diagnostic frequency of these vascular diseases in abdominal lesions is increasing even with the small changes in the vasculatures. Among various vascular diseases, fibromuscular dysplasia (FMD) and segmental arterial mediolysis (SAM) are noninflammatory, nonatherosclerotic arterial diseases which need to be diagnosed urgently because these diseases could affect various organs and be lethal if the appropriate management is not provided. However, because FMD and SAM are rare, the cause, prevalence, clinical characteristics including the symptoms, findings in the imaging studies, pathological findings, management, and prognoses have not been systematically summarized. Therefore, there have been neither standard diagnostic criteria nor therapeutic methodologies established, to date. To systematically summarize the information and to compare these disease entities, we have summarized the characteristics of FMD and SAM in the gastroenterological regions by reviewing the cases reported thus far. The information summarized will be helpful for physicians treating these patients in an emergency care unit and for the differential diagnosis of other diseases showing severe abdominal pain.
Topics: Abdominal Pain; Aneurysm; Angiography; Arteries; Cardiovascular Agents; Diagnosis, Differential; Endovascular Procedures; Fibromuscular Dysplasia; Gastroenterology; Gastrointestinal Hemorrhage; Gastrointestinal Tract; Humans; Prognosis; Treatment Outcome; Tunica Media
PubMed: 30166859
DOI: 10.3748/wjg.v24.i32.3637 -
Presse Medicale (Paris, France : 1983) Jun 2007Fibromuscular dysplasia is an idiopathic, segmental, nonatherosclerotic and noninflammatory disease of the muscle layer of arterial walls that leads to stenosis of... (Review)
Review
Fibromuscular dysplasia is an idiopathic, segmental, nonatherosclerotic and noninflammatory disease of the muscle layer of arterial walls that leads to stenosis of small- and medium-sized arteries. Fibromuscular dysplasia preferentially affects young women. Although it can affect every arterial tree, it most often touches the renal and internal carotid arteries. Renal fibromuscular dysplasia can cause hypertension by stenosis of the renal artery, most often seen on angiography as resembling a "pearl necklace". Cerebrovascular fibromuscular dysplasia becomes symptomatic when the arterial stenosis is tight and causes hypoperfusion, embolism, or thrombosis or when arterial dissection or rupture of the associated aneurysm occurs.
Topics: Carotid Artery, Internal; Diagnosis, Differential; Fibromuscular Dysplasia; Humans; Renal Artery
PubMed: 17442534
DOI: 10.1016/j.lpm.2007.02.027 -
Kidney International Feb 2009
Topics: Black or African American; Angiography, Digital Subtraction; Creatinine; Diagnosis, Differential; Fibromuscular Dysplasia; Follow-Up Studies; Humans; Hypertension; Male; Middle Aged; Polyarteritis Nodosa; Renal Artery; Renal Artery Obstruction; Thrombosis; Ultrasonography
PubMed: 18528320
DOI: 10.1038/ki.2008.232 -
Ochsner Journal 2019Carotid webs are luminal, shelf-like protrusions at the carotid bulb. Considered to be a variant of fibromuscular dysplasia, carotid webs have been observed principally...
Carotid webs are luminal, shelf-like protrusions at the carotid bulb. Considered to be a variant of fibromuscular dysplasia, carotid webs have been observed principally in African American females. The association between carotid webs and recurrent ischemic strokes continues to be established as patients without traditional stroke risk factors are found to have carotid webs as the only possible cause of symptomatic deficits. The majority of patients undergo endarterectomy; few stent placements are reported. We present the case of an African American female who underwent stent placement for treatment and secondary prevention of stroke attributed to a carotid web. A 33-year-old African American female presented with acute onset left hemiparesis and left facial droop. Alteplase was administered; multiphase head and neck computed tomography angiography revealed an occlusion of the right middle cerebral artery with a web at the carotid bulb. Aspiration catheter thrombectomy achieved a Thrombolysis in Cerebral Infarction (TICI) score of 3. She was placed on 2 antiplatelet agents, and 3 weeks later she underwent stent placement. Follow-up digital subtraction angiography at 3 months showed obliteration of the right carotid artery web and satisfactory integration of the stent into the vessel wall. She was reassessed at 1 year and reported no neurologic symptoms. Although carotid webs are most commonly treated with endarterectomy, they may be amenable to stent therapy with favorable clinical outcomes as shown with this patient.
PubMed: 30983905
DOI: 10.31486/toj.18.0143 -
Cardiovascular Research Jan 2022Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The majority of FMD patients... (Review)
Review
Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The majority of FMD patients are women. Although a variety of genetic, mechanical, and hormonal factors play a role in the pathogenesis of FMD, overall, its cause remains poorly understood. It is probable that the pathogenesis of FMD is linked to a combination of genetic and environmental factors. Extensive studies have correlated the arterial lesions of FMD to histopathological findings of arterial fibrosis, cellular hyperplasia, and distortion of the abnormal architecture of the arterial wall. More recently, the vascular phenotype of lesions associated with FMD has been expanded to include arterial aneurysms, dissections, and tortuosity. However, in the absence of a string-of-beads or focal stenosis, these lesions do not suffice to establish the diagnosis. While FMD most commonly involves renal and cerebrovascular arteries, involvement of most arteries throughout the body has been reported. Increasing evidence highlights that FMD is a systemic arterial disease and that subclinical alterations can be found in non-affected arterial segments. Recent significant progress in FMD-related research has led to improve our understanding of the disease's clinical manifestations, natural history, epidemiology, and genetics. Ongoing work continues to focus on FMD genetics and proteomics, physiological effects of FMD on cardiovascular structure and function, and novel imaging modalities and blood-based biomarkers that can be used to identify subclinical FMD. It is also hoped that the next decade will bring the development of multi-centred and potentially international clinical trials to provide comparative effectiveness data to inform the optimal management of patients with FMD.
Topics: Animals; Arteries; Biomedical Research; Fibromuscular Dysplasia; Gene Expression Profiling; Genetic Predisposition to Disease; Hemodynamics; Humans; Molecular Diagnostic Techniques; Phenotype; Predictive Value of Tests; Prognosis; Proteomics; Risk Assessment; Risk Factors; Vascular Remodeling
PubMed: 33739371
DOI: 10.1093/cvr/cvab086 -
Clinical Science (London, England :... Aug 2022Artery stenosis is a common cause of hypertension and stroke and can be due to atherosclerosis accumulation in the majority of cases and in a small fraction of patients... (Review)
Review
Artery stenosis is a common cause of hypertension and stroke and can be due to atherosclerosis accumulation in the majority of cases and in a small fraction of patients to arterial fibromuscular dysplasia (FMD). Artery stenosis due to atherosclerosis is widely studied with known risk factors (e.g. increasing age, male gender, and dyslipidemia) to influence its etiology, including genetic factors. However, the causes of noninflammatory and nonatherosclerotic stenosis in FMD are less understood. FMD occurs predominantly in early middle-age women, a fraction of the population where cardiovascular risk is different and understudied. FMD arteriopathies are often diagnosed in the context of hypertension and stroke and co-occur mainly with spontaneous coronary artery dissection, an atypical cause of acute myocardial infarction. In this review, we provide a comprehensive overview of the recent advances in the understanding of molecular origins of FMD. Data were obtained from genetic studies using complementary methodological approaches applied to familial, syndromic, and sporadic forms of this intriguing arteriopathy. Rare variation analyses point toward mechanisms related to impaired prostacyclin signaling and defaults in fibrillar collagens. The study of common variation, mainly through a recent genome-wide association study, describes a shared genetic link with blood pressure, in addition to point at potential risk genes involved in actin cytoskeleton and intracellular calcium homeostasis supporting impaired vascular contraction as a key mechanism. We conclude this review with future strategies and approaches needed to fully understand the genetic and molecular mechanisms related to FMD.
Topics: Atherosclerosis; Cardiovascular Diseases; Constriction, Pathologic; Female; Fibromuscular Dysplasia; Genome-Wide Association Study; Humans; Hypertension; Male; Middle Aged; Risk Factors; Stroke
PubMed: 36043395
DOI: 10.1042/CS20210990