OBJECTIVE: To highlight the clinical and diagnostic importance of correctly identifying cervical internal carotid artery fenestration (fcICA), an extremely rare vascular anomaly, and to present a case where fcICA was initially misdiagnosed as a dissection in a patient with fibromuscular dysplasia (FMD).
METHODS: A 47-year-old woman with pulsatile tinnitus underwent computed tomography angiography (CTA) and digital subtraction angiography (DSA) to differentiate between fenestration and dissection of the internal carotid artery.
RESULTS: CTA revealed a fusiform dilatation of the distal C1 segment of the right internal carotid artery (ICA) with a linear filling defect, suggesting either fenestration or dissection. DSA confirmed the presence of a fenestrated right ICA segment composed of two symmetrical, smooth-walled limbs without a dissection flap, along with signs of FMD in the proximal vessel. The patient\'s symptoms were attributed to local flow perturbations induced by fcICA and FMD.
CONCLUSIONS: This case illustrates that fcICA can be a true anatomical variant rather than a result of dissection, emphasizing the need for accurate imaging and diagnosis to avoid unnecessary treatments. The coexistence of fcICA with FMD increases the risk of dissection, necessitating careful monitoring. The distinction between fenestration and pseudofenestration remains challenging, requiring comprehensive imaging and close collaboration between radiologists and vascular neurologists.

Fibromuscular dysplasia (FMD) is a disease of the musculature of arterial walls leading to stenoses, aneurysms, and dissections. The purpose of this report was to summarize the evidence for (1) one-time routine imaging from brain-to-pelvis and (2) lifelong antiplatelet therapy, for example, aspirin, for patients diagnosed with FMD as suggested by an international consensus report from 2019. PubMed was systematically searched, and the evidence providing a basis for the current consensus points, as well as articles published since, were reviewed. In four registries evaluating patients with FMD, the prevalence of multivessel involvement, aneurysms, and dissections was reported to be 43.5%-66.3%, 21.6%-30.6%, and 5.6%-28.1%, respectively. Any antiplatelet drug was used in 72.9% of patients, and aspirin was prescribed in up to 70.2% of patients. Based on the high prevalence of vascular manifestations, their associated morbidity, and the potential for endovascular or surgical intervention, the suggestion of one-time brain-to-pelvis screening with computed tomography angiography or magnetic resonance angiography is well supported. Contrarily, the evidence to support the consensus statement of lifelong antiplatelet therapy to all patients in the absence of contraindications is more uncertain since a beneficial effect has not been demonstrated specifically in patients with fibromuscular dysplasia. Therefore, until the efficacy and safety of primary thromboprophylaxis have been demonstrated in this patient group specifically, it may be equally appropriate to only use antiplatelet agents in patients with a clear indication after individual evaluation according to risk factors for thrombotic and thromboembolic complications.

UNASSIGNED: Spontaneous coronary artery dissection (SCAD) is a nonatherosclerotic cause of myocardial infarction. Migraine headache has been reported to be common among patients with SCAD, but the degree of migraine-related disability has not been quantified.
UNASSIGNED: Clinical data and headache variables were obtained from the baseline assessment of the prospective, multicenter iSCAD Registry. Migraine-related disability was quantified using the self-reported Migraine Disability Assessment (MIDAS). Demographic, clinical, psychosocial, and medical characteristics from data entry forms were compared between patients with and without migraine.
UNASSIGNED: Of the 773 patients with available data, 46% reported previous or current migraines. Those with migraines were more likely to be women (96.9% vs 90.3%, p = 0.0003). The presence of underlying carotid fibromuscular dysplasia was associated with migraine (35% vs 27%, p = 0.0175). There was not a significant association with carotid artery dissection and migraine. Current migraine frequency was less than monthly (58%), monthly (24%), weekly (16%), and daily (3%). Triptan use was reported in 32.5% of patients, and 17.5% used daily migraine prophylactic medications. Using the MIDAS to quantify disability related to migraine, 60.2% reported little or no disability, 14.4% mild, 12.7% moderate, and 12.7% severe. The mean MIDAS score was 9.9 (mild to moderate disability). Patients with SCAD had higher rates of depression and anxiety (28.2% vs 17.7% [p = 0.0004] and 35.3% vs 26.7% [p = 0.0099], respectively).
UNASSIGNED: Migraines are common, frequent, and a source of disability in patients with SCAD. The association between female sex, anxiety, and depression may provide some insight for potential treatment modalities.

Systemic vascular involvement in children with cerebral arteriopathies is increasingly recognized and often highly morbid. Fibromuscular dysplasia (FMD) represents a cerebral arteriopathy with systemic involvement, commonly affecting the renal and carotid arteries. In adults, FMD diagnosis and classification typically relies on angiographic features, like the \'string-of-beads\' appearance, following exclusion of other diseases. Pediatric FMD (pFMD) is considered equivalent to adult FMD although robust evidence for similarities is lacking. We conducted a comprehensive literature review on pFMD and revealed inherent differences between pediatric and adult-onset FMD across various domains including epidemiology, natural history, histopathophysiology, clinical, and radiological features. Although focal arterial lesions are often described in children with FMD, the radiological appearance of \'string-of-beads\' is highly nonspecific in children. Furthermore, children predominantly exhibit intimal-type fibroplasia, common in other childhood monogenic arteriopathies. Our findings lend support to the notion that pFMD broadly reflects an undefined heterogenous group of monogenic systemic medium-or-large vessel steno-occlusive arteriopathies rather than a single entity. Recognizing the challenges in categorizing complex morphologies of cerebral arteriopathy using current classifications, we propose a novel term for describing children with cerebral and systemic vascular involvement: \'cerebral and systemic arteriopathy of childhood\' (CSA-c). This term aims to streamline patient categorization and, when coupled with advanced vascular imaging and high-throughput genomics, will enhance our comprehension of etiology, and accelerate mechanism-targeted therapeutic developments. Lastly, in light of the high morbidity in children with cerebral and systemic arteriopathies, we suggest that investigating for systemic vascular involvement is important in children with cerebral arteriopathies.

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BACKGROUND: 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.
METHODS: 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. An urgent CT scan of the head and intracranial angiogram showed bilateral severe stenosis of the distal cervical segment of internal carotid arteries (ICAs) and right vertebral artery moderate stenosis at the V2 segment. He had been given IV TPA (Alteplase) within the 4.5-hour window. After 4 hours, the patient\'s GCS dropped from 15 to 10, and the NIHSS score increased from 4 to 24, followed by witnessed a generalized tonic-clonic seizure. Repeat urgent CT head showed no evidence of intracerebral hemorrhage (ICH). The patient was arranged for cerebral angiographic catheterization that showed bilateral flame-shaped occlusion of cervical ICA dissection. There is a mild focal narrowing of the right cervical vertebral artery, likely dissection. Routine laboratory blood workup for vasculitis was negative. During MICU admission, he had witnessed the right arm hemichorea-ballism spectrum abnormal movement. After the 6th-month follow-up, intracranial CT angiogram showed reduced caliber of the bilateral distal cervical course of the internal carotid arteries seen with residual dissection and focal outpouching of the right ICA representing pseudoaneurysm.
CONCLUSIONS: The occurrence of multiple CADs suggests the presence of an underlying intrinsic arteriopathy, such as FMD, the presence of pseudoaneurysm, environmental triggers, cervical manipulation, and remote history of head or neck surgery. A study of the most extensive case series of patients with cervical artery dissection showed 15.2% of patients with multiple CAD. In most patients with multiple cervical artery dissections, antithrombotic treatment is effective, complete recanalization, and the outcome is favorable. Outside the window period of acute ischemic stroke, either anticoagulation or antiplatelet therapy is a recognized treatment for secondary ischemic stroke prevention due to extracranial artery dissection. For acute stroke or TIA patients caused by intracranial artery dissection, experts recommend antiplatelet therapy rather than anticoagulation.
CONCLUSIONS: Simultaneous triple-vessel cervicocephalic arterial dissections are rarely reported condition. Multiple CADs are associated with underlying vasculopathy and environmental triggers, and a majority are recanalized with antithrombotic treatment with favorable outcomes. Antithrombotic treatment is effective in most patients with multiple CADs, and most expect complete recanalization. This case report guides physicians in the treatment and outcome of acute stroke due to multiple CAD.

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.
Of 343 patients in the ARCADIA-POL registry, 58 patients underwent percutaneous treatment due to FMD-RAS (in 70 arteries). Percutaneous transluminal renal angioplasty (PTRA) was performed as an initial treatment in 61 arteries (PTRA-group), whereas primary stenting was undertaken in nine arteries (stent-group). Stent-related complications were defined as: in-stent restenosis > 50% (ISR); stent fracture; under-expansion; or migration.
In the PTRA-group, the initial restenosis rate was 50.8%. A second procedure was then performed in 22 arteries: re-PTRA (12 arteries) or stenting (10 arteries). The incidence of recurrent restenosis after re-PTRA was 41.7%. Complications occurred in seven of 10 (70%) arteries secondarily treated by stenting: two with under-expansion and five with ISR. In the stent-group, stent under-expansion occurred in one case (11.1%) and ISR in three of nine stents (33.3%). In combined analysis of stented arteries, either primarily or secondarily, stent-related complications occurred in 11/19 stenting procedures (57.9%): three due to under-expansion and eight due to ISRs. Finally, despite several revascularization attempts, four of 19 (21%) stented arteries were totally occluded and one was significantly stenosed at follow-up imaging.
Our study indicates that renal stenting in FMD-RAS may carry a high risk of late complications, including stent occlusion. Further observational data from large-scale registries are required.

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Renovascular hypertension (RVH) is a secondary form of high blood pressure resulting from impaired blood flow to the kidneys with subsequent activation of the renin-angiotensin-aldosterone system. Often, this occurs due to abnormally small, narrowed, or blocked blood vessels supplying one or both kidneys (ie: renal artery occlusive disease) and is correctable. Juxtaglomerular cells release renin in response to decreased pressure, which in turn catalyzes the cleavage of circulating angiotensinogen synthesized by the liver to the decapeptide angiotensin I. Angiotensin-converting enzyme then cleaves angiotensin I to form the octapeptide angiotensin II, a potent vasopressor and the primary effector of renin-induced hypertension. The effects of angiotensin II are mediated by signaling downstream of its receptors. Angiotensin receptor type 1 is a G-protein-coupled receptor that activates vasoconstrictor and mitogenic signaling pathways resulting in peripheral arteriolar vasoconstriction and increased renal tubular reabsorption of sodium and water which promotes intravascular volume expansion. Angiotensin II stimulates the adrenal cortical release of aldosterone, which promotes renal tubular sodium reabsorption, resulting in volume expansion. Angiotensin II acts on glial cells and regions of the brain responsible for blood pressure regulation increasing renal sympathetic activation. Angiotensin II simulates the release of vasopressin from the pituitary which stimulates thirst and water reabsorption from the kidney to expand the intravascular volume and cause peripheral vasoconstriction (increased sympathetic tone). All of these mechanisms coalesce to increase arterial pressure by way of arteriolar constriction, enhanced cardiac output, and the retention of sodium and water.

Spontaneous coronary artery dissection (SCAD) is an increasingly recognized cause of acute coronary syndrome. Guidance regarding the optimal management of patients with SCAD has been published over the past 10 years, but the impact on clinical practice has not been evaluated. The present study aims to examine if approaches to invasive management, medical therapy, and vascular imaging have changed over time.
This is a retrospective cohort study of 157 patients treated for SCAD between 2005 and 2019 at an academic health system in Philadelphia, Pennsylvania. We aimed to examine change in management over time, including rates of coronary revascularization, discharge medications, and vascular imaging.
Conservative management of SCAD increased over time from 35% before 2013 to 89% in 2019, p < 0.001. Revascularization was associated with younger age, pregnancy-associated SCAD, and lesions of the left main artery, left anterior descending artery, and multiple vessels, p < 0.05 for all. Partial imaging for extracoronary vascular abnormalities ranged from 33% before 2013 to 71% in 2018, p = 0.146. The rate of comprehensive vascular imaging (cross-sectional head to pelvis imaging) remained low in all time categories (10-18%) and did not change over time. Patients who underwent comprehensive imaging were more likely to be diagnosed with fibromuscular dysplasia (FMD) compared to those with partial imaging (63% vs 15%, p < 0.001).
Management of spontaneous coronary artery dissection has changed over time. More patients are being managed conservatively and undergo screening for extracoronary vascular abnormalities such as FMD. Future efforts should focus on improving rates of comprehensive vascular screening.