Fibromuscular dysplasia

INTRODUCTION: Cervicocephalic arterial dissections (CADs) occur in 3 cases per 100,000 individuals across all ages. Multiple simultaneous CADs are found in 13 to 22% of cases, and three or more dissections occur in approximately 2%. CADs might result from multifactorial intrinsic deficiencies of vessel wall integrity and extrinsic factors, e.g., minor trauma. CASE PRESENTATION: A young gentleman presented to the emergency department with a sudden onset of a spinning sensation of surrounding, left side arm weakness, blurring of vision, and an NIHSS score of 4...

Mesenteric arteriovenous dysplasia/vasculopathy (MAVD/V) is an exceedingly rare noninflammatory vascular disorder affecting small-calibre mesenteric arteries and veins. This report details a case of a 51-year-old male diagnosed with MAVD/V following abdominal pain and vomiting. Surgical exploration revealed distinctive smooth muscle collarette around subserosal arteries and veins. The rarity of this condition, with only 13 cases reported globally, underscores the importance of recognizing this rare entity to prevent misdiagnosis...

Intrinsic pathologies of the vertebral arteries, such as atherosclerosis, dissection, fibromuscular dysplasia, radionecrosis and vasculitis, are important causes of vertebrobasilar insufficiency and cerebrovascular events. This review focuses on non-aneurysmal intrinsic stenosing and occlusive pathologies, covering their epidemiology, diagnosis, and treatment options. It also provides a detailed summary of key clinical presentations and syndromes, including an in-depth examination of lateral medullary syndrome, historically known as Wallenberg's syndrome, which is arguably the most emblematic condition resulting from vertebral artery involvement and is depicted in an illustrative cartoon...

A carotid web (CaW) is an atypical form of fibromuscular dysplasia characterized by a fibrous, shelf-like intimal flap originating from the posterior wall of the internal carotid bulb projecting into the arterial lumen. CaWs disturb normal blood flow and create stasis between the intimal reflection and the carotid wall, thereby promoting thrombogenesis and increasing the risk of downstream embolic strokes. Observational data have suggested that CaWs are associated with strokes with otherwise unknown etiology, particularly in young patients without other stroke factors, and stroke recurrence rates of symptomatic CaWs have been reported to be as high as 20% over 2 years...

No abstract text is available yet for this article.

Fibromuscular dysplasia of the coronary is an uncommon coronary defect with a range of pathological alterations and unpredictable clinical description that can cause sudden death. We present an autopsy case of sudden cardiac death due to a rupture of a coronary artery aneurysm in a 59-year-old woman. Postmortem autopsy revealed two huge saccular aneurysms located at the right coronary artery, one of which was ruptured leading to a fatal hemopericardium. Histopathological examination revealed coronary artery fibromuscular dysplasia with fibromyxoid dissociation of the media causing saccular aneurysms...

Fibromuscular dysplasia (FMD) is a rare vascular disease with broad, potentially severe complications. We present three cases of FMD covering the spectrum of clinical presentations involving the abdominal and visceral vasculature, and highlight the potential role of high-risk genotype detection in assisting with the determination of which patients may benefit from a more aggressive surveillance strategy.

Fibromuscular dysplasia is a vascular disorder characterized by nonatherosclerotic, noninflammatory arterial abnormalities, primarily affecting renal arteries. While extrarenal involvement is rare, it poses diagnostic challenges due to diverse clinical presentations. We present the case of a middle-aged female who presented with sudden-onset severe abdominal pain and hypertension. Physical examination revealed tenderness, guarding, and hypertensive urgency. Diagnostic workup, including laboratory investigations and computed tomography, identified multifocal arterial stenosis in both renal and mesenteric arteries, consistent with fibromuscular dysplasia...

No abstract text is available yet for this article.

BACKGROUND: Spontaneous coronary artery dissection is a rare and important cause of myocardial infarction, especially in young women without other coronary artery disease. This arterial dissection can occur within or between any of the 3 layers. Its predisposing factors include connective tissue diseases (Marfone syndrome, Ehlers-Danlos syndrome), vasculitis (polyarteritis nodosa, systemic lupus erythematosus, and Kawasaki disease), atherosclerosis and fibromuscular dysplasia. Clinical presentations of spontaneous coronary artery dissection are wide spectrum from asymptomatic to acute coronary disease, sustained ventricular arrhythmia and sudden cardiac death...

No abstract text is available yet for this article.

Spontaneous coronary artery dissection (SCAD) is a rare clinical entity, often presenting similar to atherosclerotic acute coronary syndrome (ACS), although it is a non-atherosclerotic, non-traumatic, and non-iatrogenic coronary artery pathology. We report the case of a 36-year-old woman who presented with substernal, pressure-like chest pain without aggravating, alleviating, or associated symptoms. Initial evaluation revealed elevated troponin levels which peaked at 8.71 ng/mL. Electrocardiogram showed borderline J point elevation in the lateral leads...

Spontaneous coronary artery dissection (SCAD), although in the majority of cases presents as an acute coronary syndrome (ACS), has different pathophysiology from atherosclerosis that influences specific angiography findings and enables most patients to be solved by optimal medical therapy rather than percutaneous coronary intervention (PCI). Therefore, accurate diagnosis is essential for adequate treatment of each patient as management of SCAD differs from that of ACS of atherosclerotic aetiology. So far, invasive coronary angiography remains the most important diagnostic tool in suspected SCAD...

INTRODUCTION: Although renal stenting is the standard revascularization method for atherosclerotic renal artery stenosis (RAS) (FMD-RAS), stenting in fibromuscular dysplasia (FMD) RAS is usually limited to periprocedural complications of angioplasty and primary arterial dissection. The main aim of the study was to retrospectively analyze the immediate and long-term results of renal stenting versus angioplasty in patients with FMD. METHODS: Of 343 patients in the ARCADIA-POL registry, 58 patients underwent percutaneous treatment due to FMD-RAS (in 70 arteries)...

We present a rare case of fibromuscular dysplasia (FMD) manifesting in the mid segment of right renal artery, which led to the development of refractory hypertension. The patient received balloon angioplasty to a severe lesion on the middle of right renal artery and subsequently had normalisation of blood pressures. Fractional flow reserve (FFR) detection of the renal artery before and after balloon dilatation was 0.71 and 0.98, respectively. The patient showed renal artery stenosis (RAS) with distal tumour-like dilatation, and multiple tortuosity and stenosis in carotid artery and coronary artery...

AIM: The first expert consensus documents on management of patients with spontaneous coronary artery dissection (SCAD) were published in 2018. Worldwide quality of care, as measured by adherence to these recommendations, has not been systematically reviewed. We aim to review the proportion of patients with SCAD receiving consensus recommendations globally, regionally and, determine differences in practice before and after 2018. METHODS AND RESULTS: A systematic review was performed by searching four main databases (Medline, Embase, SCOPUS, CINAHL) from their inception to 16 June 2022...

Fibromuscular dysplasia (FMD) is a congenital vascular anomaly resulting in arterial stenosis and weakening of typically medium-sized arteries. It is a noninflammatory, nonatherosclerotic arterial disease that affects most commonly the renal and internal carotid arteries, but intracranial FMD in the pediatric population is very rare. We report a young age-onset ischemic stroke patient with FMD affecting the middle cerebral artery (MCA). A 14-year-old boy was admitted with left-side weakness during physical education at school...

BACKGROUND: Carotid web (CaW) is a subtype of fibromuscular dysplasia that predominantly involves the intimal layer of the arterial wall and is commonly overlooked as a separate causative entity for recurrent strokes. CaW is defined as a shelf-like lesion at the carotid bulb, although different morphological features have been reported. Optical coherence tomography (OCT) has been described in the literature as a useful microscopic and cross-sectional tomographic imaging tool. This study aimed to evaluate the potential utility of OCT in characterizing the wall structure features of patients with suspected CaW...

Rare vascular conditions frequently pose a diagnostic and therapeutic dilemma for health care providers. Several of these conditions have distinct relevance to females populations but, due to their infrequency, there has been little reported on the outcomes of rare vascular conditions specifically in females populations. We performed a literature review of a selection of three rare vascular conditions known to either disproportionately affect females (median arcuate ligament syndrome and fibromuscular dysplasia) or have unique manifestations in females populations (vascular Ehlers-Danlos syndrome)...

Spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic separation of the coronary artery wall with subsequent intramural hematoma (IMH) formation in the false lumen. It can be associated with or without an intimal tear. It is clinically divided into three types based on its angiographic appearance. Most SCAD cases are seen in young or middle-aged women, especially in a peripartum state. Additionally, SCAD patients usually have fewer cardiovascular risk factors and more commonly have predisposing conditions like fibromuscular dysplasia (FMD)...