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Fibromuscular dysplasia (FMD) affecting the carotid artery is an idiopathic, non-inflammatory, and non-atherosclerotic condition characterized by segmental arterial vessel wall abnormalities. Predominantly observed in small and medium-sized arteries, FMD can present with stenosis, aneurysm, dissection, occlusion, and arterial tortuosity, particularly in the renal and carotid arteries. Women show a higher prevalence of FMD. The outdated histopathological classification has been replaced by angiographic evaluation, categorizing arterial lesions as focal or multifocal. Despite ongoing research, the precise mechanism behind FMD development, involving genetic, mechanical, tobacco use, and hormonal factors, remains elusive. Symptoms vary based on the affected arterial segment, with carotid FMD often being asymptomatic but may include headache, pulsatile tinnitus, dizziness, and cerebrovascular accidents. Vascular imaging plays a crucial role in diagnosis, and conservative treatments involve antiplatelet therapy and hypertension control. Surgical or endovascular interventions are considered for persistent cerebrovascular ischemia. Due to its chronic nature, long-term follow-up with periodic imaging is crucial. Future research should focus on genetic factors, establish tissue banks, and develop innovative treatments. In conclusion, Fibromuscular Dysplasia of the Carotid Artery requires a multidisciplinary approach for effective management.
Faculty of Medicine, Department of Cardiovascular Surgery, Hitit University, Turkey
*Address all correspondence to: ufukturkmen@hitit.edu.tr
1. Introduction
1.1 Definition
Following the initial use of the term “fibromuscular hyperplasia” in hypertension and renal artery stenosis, Hunt introduced this term due to the observation of dysplasia rather than hyperplasia alone. In the same year, Connett reported the first Fibromuscular Dysplasia (FMD) in the internal carotid artery [1, 2, 3].
FMD is a disease characterized by idiopathic, segmental, non-inflammatory, and non-atherosclerotic abnormal cell proliferation of the muscular system of the arterial walls. It can be observed in all small and medium-sized arteries in the body, most commonly affecting the renal and carotid arteries. It may present with stenosis, aneurysm, dissection, occlusion, and arterial tortuosity. The clinical spectrum varies from a symptomatic picture to a multisystem disorder, depending on the arterial segment affected, the severity of stenosis, and the type of FMD [4].
Fibromuscular Dysplasia of the Carotid Artery (CaFMD) is most commonly observed in the C1-C2 segments of the internal carotid artery. While FMD may be present in up to 4% of the general population, carotid artery involvement is reported in 25–70% of all FMD cases. It frequently manifests bilaterally, with approximately two-thirds of cases exhibiting FMD in an additional arterial bed [5, 6].
Although FMD primarily affects the renal, extracranial carotid, and vertebral arteries, almost all arterial beds can be affected, and multivessel involvement is common. Approximately 80–90% of patients with FMD are women. FMD is also seen in men, but it may have a more aggressive course with more common occurrences of aneurysms and dissections [5].
The presentation and natural course of the disease in the pediatric population are quite different from those in adults, and the prevalence and manifestation of FMD in children are currently unknown [4].
1.2 Classification
Histopathological and angiographic classification systems have been used in the classification of FMD disease. Although histopathological classification of FMD was used in the past, it has lost its validity in current clinical practice.
1.2.1 Histopathological classification
Histopathological classification was primarily divided into three categories: Intimal, Medial, and Adventitial [7].
Intimal FMD: The second most common form, accounting for 5–10% of cases with accumulation of circumferential collagen in the intima, leading to the breakdown of the internal elastic lamina. Angiographically, it appears as concentric smooth stenosis or constriction.
Medial FMD: The most common type, observed in 85% of cases. This type exhibits alternating collagen protrusions and loss of the elastic membrane, while the internal elastic lamina remains intact. The classic “string of beads” appearance on angiography arises from varying areas of constriction and dilation in the artery. Unlike atherosclerotic carotid artery stenosis, which affects the proximal and ostial regions of the affected artery, CaFMD rarely affects the proximal or ostial portion of the internal carotid artery.
Adventitial FMD: The least common, occurring in less than 1% of cases. Dense collagen accumulation is present in the adventitia, resulting in a smooth constriction appearance on angiography.
1.2.2 Angiographic variants classification
Another classification, proposed by Kincaid et al., suggests four different angiographic variants of the disease [8]:
Multifocal type: Multiple stenoses typically associated with medial FMD, presenting a string of beads appearance.
Tubular type: Large, concentric constriction exceeding 1 cm.
Focal type: Solitary constriction with a length of less than 1 cm.
Mixed type.
1.2.3 The American heart association classification
As per the AHA, arterial lesions are evaluated angiographically and classified as focal FMD disease or multifocal FMD disease. FMD on angiography leads to two types of appearances [9]:
Focal FMD: which can be seen anywhere in the artery.
Multifocal FMD: a characteristic lesion termed “string of beads”, typically observed in the middle and distal portions of the artery, consisting of stenotic and expansive areas (Figure 1). These morphological patterns are most commonly observed in the renal and carotid arteries.
Figure 1.
The characteristic “string of beads” appearance in fibromuscular dysplasia of the carotid artery.
Histopathologically, the appearance of focal Fibromuscular Dysplasia (FMD) typically involves the accumulation of fibrous tissue in the intima, often with a cellular component. In multifocal FMD, there is accumulation in areas of degenerate elastic fibrils within a loose collagen environment. This results in alternating arterial constriction and subsequent arterial dilation in regions of smooth muscle loss, giving rise to the angiographic appearance of “string of beads”, where these beads are larger than the main artery’s caliber.
The etiology of FMD remains unclear despite extensive research. While genetic, mechanical, and hormonal factors have been proposed in the development of FMD, it is likely associated with a combination of genetic and environmental factors [10]. FMD may manifest sporadically in some individuals, while others suggest a role for autosomal dominant inheritance; however, some studies indicate that only a small percentage of patients have affected family members [11]. A genome-wide association study identified a prevalent genetic risk variant, a single nucleotide polymorphism (SNP) rs9349379-A, located in the PHACTR1 locus (6p24), with an odds ratio (OR) of ~1.4 for FMD [12]. Understanding the genes responsible for FMD will be crucial for developing targeted treatment approaches. Additionally, knowledge of which genes are involved in FMD could be clinically beneficial in predicting the risk of FMD in individuals, especially within affected families and symptomatic patients. Further genetic studies are needed to better understand the role of genetics in the pathogenesis of FMD.
Tobacco use has been identified as a potential pathogenic factor associated with FMD [13]. Despite the association of endogenous and exogenous female hormones with FMD, a definitive relationship has not been established, despite the disease being much more prevalent in women than in men [5].
In a study, an abnormal balance between estrogen and progesterone receptors was observed in arterial samples of patients undergoing surgery for FMD, suggesting that progesterone may play a role in the pathogenesis of FMD [14].
Mechanical factors may contribute to the formation of FMD in the middle and distal internal carotid arteries, although specific mechanisms remain unknown. Recent studies have indicated that transforming growth factor (TGF)-β1 and TGF-β2 produced by dermal fibroblast cells and lysophosphatidylcholine (lisoPC), a pro-inflammatory and pro-apoptotic lipid mediator, could potentially be associated with FMD pathogenesis [15, 16].
Patients may be symptomatic or asymptomatic, and clinical presentations can vary based on the affected arterial segment (Table 1). The clinical manifestation of Fibromuscular Dysplasia (FMD) primarily depends on the vascular territory involved, with a positive family history often indicating stroke and hypertension at a young age [5, 17].
Primary symptoms or signs
Severe and/or chronic migraine headaches, especially in the presence of other symptoms or signs.
Pulsatile tinnitus.
Neck pain.
Stroke, Transient Ischemic Attack (TIA), or Amorosis Fugax.
Unilateral head/neck pain or focal neurological findings suggestive of cervical arterial dissection.
Possible symptoms
Headaches (non-migraine or non-migraine type).
Tinnitus (non-pulsatile).
Dizziness/lightheadedness.
Table 1.
Clinical symptoms of carotid artery FMD disease [4].
In patients with Carotid Artery Fibromuscular Dysplasia (CaFMD), focal neurological findings may emerge in association with or independently of neck, facial, or head pain. Arterial dissection, particularly in the carotid or vertebral arteries, can be observed in CaFMD patients [18].
Symptoms related to CaFMD are nonspecific, and approximately 50% of patients may experience migraine-type headaches, pulsatile tinnitus, dizziness, or lightheadedness. In some cases, FMD can be incidentally detected during imaging for various reasons without any apparent symptoms [11].
When major symptoms or signs indicative of CaFMD are present, consideration should be given to the diagnosis of FMD, and imaging modalities should be employed for further evaluation.
Vascular imaging remains the primary method for diagnosing Carotid Artery Fibromuscular Dysplasia (CaFMD). There is insufficient data to prefer one imaging method over another for CaFMD diagnosis. Catheter-based angiography continues to be the diagnostic gold standard. Contrast-enhanced CTA or contrast-enhanced MRA is commonly used as the initial imaging method due to its non-invasiveness and simultaneous evaluation of other tissues [19, 20].
4.1.1 Doppler ultrasonography
Doppler USG continues to be the first-line screening test for CaFMD. It reveals turbulence, tortuosity, high velocities, and resistant indices in the carotid arteries. However, Doppler USG is operator-dependent and inadequate for assessing the intracranial carotid artery. In centers experienced in vascular Doppler USG, starting with carotid artery Doppler examination is logical for evaluating CaFMD. To date, there is no confirmed criterion for CaFMD diagnosis using Doppler USG. Carotid Doppler USG may be useful for the intermittent monitoring and surveillance of patients with CaFMD [9].
4.1.2 Computed tomography angiography
Computed tomographic angiography (CTA) is one of the best methods for diagnosing CaFMD. It is non-invasive, accurate, reliable, and provides high-resolution images. Classic CTA can easily visualize focal and multifocal CaFMD images. It allows the assessment of the carotid artery’s intracranial segments along with surrounding tissues and intracranial structures. However, consideration should be given to the risk of radiation exposure and contrast nephropathy [9].
4.1.3 Magnetic resonance angiography
Magnetic Resonance Angiography (MRA), a non-invasive examination, has comparable sensitivity and specificity to CTA. Unlike CTA, MRA has no risk of radiation exposure and contrast nephropathy. An unruptured intracranial aneurysm is a major sign of CaFMD disease. In patients confirmed to have CaFMD at any location, particularly in the carotid and vertebral arteries, brain imaging with CTA or MRA should be performed to assess intracranial aneurysms [9].
4.1.4 Arteriography
Traditional angiography remains the gold standard for diagnosing CaFMD. It also allows the measurement of pressure gradients over stenotic lesions. Although catheter-based angiography is invasive, it also allows the possibility of endovascular treatment during imaging. New diagnostic methods such as intravascular ultrasound may further strengthen angiography and help assess restenosis after angioplasty [9].
4.2 Differential diagnosis
Distinguishing CaFMD from other carotid artery diseases is crucial. The differential diagnosis of CaFMD is extensive and relies on imaging, based on the presence of associated arterial findings (dissection, aneurysm, and tortuosity) in addition to typical focal or multifocal CaFMD lesions.
4.2.1 Systemic arterial mediolysis (SAM)
SAM is a non-inflammatory, non-atherosclerotic disease that typically manifests with spontaneous arterial dissection, rupture, occlusion, or aneurysm, primarily in abdominal visceral arteries. Histopathological examination demonstrating vacuolar degeneration of the arterial media is required for a definitive diagnosis [21].
4.2.2 Standing waves due to arterial spasm
Radiological findings of benign, transient physiological changes caused by ergotamine derivatives, sympathomimetic drugs, or catheter-related vasospasm [22].
4.2.3 Atherosclerosis
In some patients, CaFMD can be differentiated from atherosclerotic disease by its occurrence at a younger age and the absence of traditional atherosclerotic risk factors. Additionally, CaFMD occurs in the middle or distal parts of the carotid arteries, while atherosclerosis occurs in the ostium or proximal part of these arteries. Atherosclerotic patients typically have cardiovascular risk factors such as advanced age, hypertension, hyperlipidemia, tobacco use, obesity, diabetes, etc.
4.2.4 Large vessel vasculitis
Although FMD is a non-inflammatory process, vasculitides exhibit significant inflammation. Vasculitides are characterized by fever, weight loss, pain along the affected artery trace, elevated inflammatory markers, anemia, and thrombocytopenia. Acute-phase reactant measurements are generally within normal values in CaFMD disease.
4.2.5 Neurofibromatosis type 1
An autosomal dominant disorder with multiple system involvement and typical café-au-lait spots, capable of causing intracranial arterial stenosis [23].
4.2.6 Alagille syndrome
An autosomal dominant disorder with multiple system involvement and a typical facial appearance, capable of being confused with CaFMD due to intracranial arterial aneurysms [24].
4.2.7 Loeys-Dietz syndrome
A connective tissue disorder characterized by tortuosity, aneurysm, and dissection in the carotid arteries [25].
4.2.8 Ehlers-Danlos syndrome, type IV, or vascular type
In families with a history of sudden death, there may be dissection, aneurysm, or carotid-cavernous fistula development in the carotid arteries [26].
Rare conditions such as Marfan syndrome, tuberous sclerosis, Alport syndrome, alpha-1 antitrypsin deficiency, moyamoya, and Williams syndrome can be confused with CaFMD, but they can be distinguished by their phenotypic features and genetics.
While there is no study evaluating the benefit of medical treatment in CaFMD, antiplatelet therapy should be applied, without contraindications, in both symptomatic and asymptomatic cases due to the potential for thrombotic and thromboembolic events in CaFMD. According to the latest consensus, daily use of 75–100 mg of aspirin is recommended for FMD [4].
5.1.2 Hypertension management
The use of antihypertensive drugs is common in CaFMD patients, particularly in those with hypertension associated with renal artery involvement. Controlling hypertension and the use of beta-blockers are especially necessary for CaFMD patients with intracranial aneurysms [27].
5.1.3 Treatment of headache and pulsatile tinnitus
Although migraine is the most common type of headache in CaFMD patients, headaches can also be associated with uncontrolled hypertension or CaFMD. Pulsatile tinnitus, a common symptom in 32% of all patients, is a widespread sign of cerebrovascular FMD. Consulting with audiology and otolaryngology specialists may be useful in evaluating other causes of pulsatile tinnitus [28].
5.1.4 Additional considerations
All CaFMD patients who continue to smoke should be encouraged to quit. Smokers with CaFMD have shown higher rates of arterial aneurysms, increased need for vascular procedures, and more adverse events [29].
Given that FMD is more prevalent in women, there are theoretical concerns regarding exogenous hormone therapies. However, there is no data supporting the safety or harm of hormones in this regard.
Patients who have had a stroke related to CaFMD should be adequately educated. Further education on CaFMD should help patients recognize other symptoms that may indicate disease progression or complications. Education should cover the benefits of avoiding smoking, healthy eating, and understanding other risk factors for stroke, including good glycemic control. Patients should be informed about the risk of dissection and advised to avoid neck trauma and vigorous neck manipulations.
5.2 Surgical intervention and endovascular procedures
Management of the carotid arteries in CaFMD patients is similar to those without CaFMD. In cases of acute stroke due to CaFMD, both intravenous thrombolysis and mechanical thrombectomy can be used in appropriate patients. Endovascular treatment (placing a stent in the carotid artery) is limited to cases with persistent cerebrovascular ischemia despite optimal medical treatment. The risk of ischemic events or rupture is low in dissections leading to pseudoaneurysms, and endovascular treatment is rarely required. If endovascular intervention is indicated, care should be taken to avoid iatrogenic femoral artery injury during femoral access. However, it is unknown whether the presence of FMD increases the risk of iatrogenic dissection or pseudoaneurysm development post-stent [30, 31, 32, 33].
6. Prognosis, follow-up, and complications of the disease
CaFMD is a chronic vascular disease requiring long-term care focused on both periodic imaging of affected vascular beds and medical treatment. After detecting CaFMD, patients should be followed up at least annually, with more frequent monitoring for those with severe symptoms or the disease (Figure 2). Clinical evaluation should include assessing migraine headaches, neck pain, pulsatile tinnitus, claudication, mesenteric angina, exertional angina, and blood pressure control.
Figure 2.
The lesions seen with CTA in the left internal carotid artery (LICA) and left external carotid artery (LECA) of a 36-year-old female patient under follow-up for FMD due to headaches.
Possible Adverse Reactions to antiplatelet agents and antihypertensive drugs should be evaluated. In patients with renal artery involvement, periodic urine analysis for screening albuminuria, and blood biochemistry for kidney function and electrolytes, should be assessed at least annually.
Education should include reviewing potential warning signs and symptoms of TIA, stroke, and arterial dissection. CaFMD patients should be informed about the risks of dissection and warned to avoid neck trauma and vigorous neck manipulations.
Specific algorithms for follow-up imaging methods for CaFMD patients are currently insufficient. However, in experienced centers, non-invasive monitoring with Doppler ultrasound can be performed frequently in appropriate patients.
CaFMD should be managed by an experienced team consisting of a cardiologist, vascular surgeon, interventional radiologist, and internist.
In individuals desiring pregnancy with CaFMD, arterial aneurysms need careful and close monitoring. The rupture of an aneurysm in this population can have adverse consequences for both the mother and the fetus. Monitoring the development of hypertension during pregnancy and managing pregnancy-related hypertension require close monitoring in each trimester and the immediate postpartum period [4].
6.1 Possible complications
CaFMD can lead to various complications in approximately 10% of cases. These may include carotid artery dissection leading to rupture, arterial lumen involvement causing decreased brain perfusion, and thrombus formation that may lead to distal embolization. Additionally, atherosclerotic occlusion of the carotid artery bifurcation, which complicates the determination of the cause of cerebral symptoms, extracranial carotid aneurysms, and FMD of the vertebral arteries, can present simultaneous issues that complicate the management of the current condition [6].
Studies are needed to increase the number of genes associated with FMD, examine familial factors, and apply different genetic methods in screenings. More work and understanding are required to gain further insights into the underlying molecular and pathological aspects. A tissue bank comprising arterial tissue samples from FMD patients should be established. Determining factors associated with disease progression is crucial. In terms of treatment, new pharmacological studies should be conducted to assess the effectiveness of antiplatelet therapy or new drugs. Creating awareness about CaFMD, a disease not well-recognized or understood by the medical community, is critically important.
The examined data indicate that Fibromuscular Dysplasia (FMD) of the Carotid Artery is a vascular disorder resulting from the complex interactions of genetic, environmental, and hormonal factors. The information provided on various imaging methods, treatment options, and prognosis used for the diagnosis and monitoring of the disease guides clinicians in better understanding and managing the condition.
In the context of future research methods and innovations, more studies are needed to better understand the genetic foundations of the disease and develop targeted approaches for treatment. Additionally, it is important to establish standardized protocols for the long-term follow-up of CaFMD patients and the assessment of treatment response.
This text aims to fill the knowledge gaps regarding this rare disease in both clinical practice and research. A deeper understanding of Fibromuscular Dysplasia of the Carotid Artery holds the potential to provide patients with more effective and personalized treatment.
The author declares no conflict of interest regarding the publication of this article.
References
1.McCormack LJ, Hazard JB, Poutasse EF. Obstructive lesions of the renal artery associated with remediable hypertension. American Journal of Pathology. 1958;34(2):582
2.Hunt J, Harrison EG, Sheps SG, et al. Hypertension caused by fibromuscular dyplasia of renal arteries. Postgraduate Medicine. 1965;38:53-63
3.Connett MC, Lansche JM. Fibromuscular hyperplasia of the internal carotid artery: Report of a case. Annals of Surgery. 1965;162(1):59-62
4.Gornik HL, Persu A, Adlam D, et al. First international consensus on the diagnosis and management of fibromuscular dysplasia. Vascular Medicine (London, England). 2019;24(2):164-189
5.Kim ESH, Olin JW, Froehlich JB, et al. Clinical manifestations of fibromuscular dysplasia vary by patient sex: A report of the United States Registry for Fibromuscular Dysplasia. Journal of the American College of Cardiology. 2013;62(21):2026-2028
6.Olin JW, Sealove BA. Diagnosis, management, and future developments of fibromuscular dysplasia. Journal of Vascular Surgery. 2011;53(3):826-836
7.Stanley JC, Gewertz BL, Bove EL, et al. Arterial fibrodysplasia. Histopathologic character and current etiologic concepts. Archives of Surgery. 1975;110(5):561-566
8.Kincaid OW, Davis GD, Hallermann FJ, et al. Fibromuscular dysplasia of the renal arteries. Arteriographic features, classification, and observations on natural history of the disease. The American Journal of Roentgenology, Radium Therapy, and Nuclear Medicine. 1968;104(2):271-282
9.Olin JW, Gornik HL, Bacharach JM, et al. Fibromuscular dysplasia: State of the science and critical unanswered questions: A scientific statement from the American Heart Association. Circulation. 2014;129(9):1048-1078
10.Perdu J, Boutouyrie P, Bourgain C, et al. Inheritance of arterial lesions in renal fibromuscular dysplasia. Journal of Human Hypertension. 2007;21(5):393-400
11.Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia: Results in the first 447 patients. Circulation. 2012;125(25):3182-3190
12.Kiando SR, Tucker NR, Castro-Vega LJ, et al. PHACTR1 is a genetic susceptibility locus for fibromuscular dysplasia supporting its complex genetic pattern of inheritance. PLOS Genetics. 2016;12(10):e1006367
13.Savard S, Azarine A, Jeunemaitre X, et al. Association of smoking with phenotype at diagnosis and vascular interventions in patients with renal artery fibromuscular dysplasia. Hypertension. 2013;61(6):1227-1232
14.Silhol F, Sarlon-Bartoli G, Daniel L, et al. Intranuclear expression of progesterone receptors in smooth muscle cells of renovascular fibromuscular dysplasia: A pilot study. Annals of Vascular Surgery. 2015;29(4):830-835
15.Shimabukuro M. A new plausible link between lysophosphatidylcholine, TGF-β, and fibromuscular dysplasia. Journal of Atherosclerosis and Thrombosis. 2016;23(6):665-667
16.Ganesh SK, Morissette R, Xu Z, et al. Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-β expression and connective tissue features. FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology. 2014;28(8):3313-3324
17.McInnis CP, Haynor DR, Francis CE. Horner syndrome in fibromuscular dysplasia without carotid dissection. Canadian Journal of Ophthalmology. 2016;51(2):53-55
18.Kadian-Dodov D, Gornik HL, Gu X, et al. Dissection and aneurysm in patients with fibromuscular dysplasia: Findings from the U.S. registry for FMD. Journal of the American College of Cardiology. 2016;68(2):176-185
19.Kong W, Hu Z. Unique imaging findings in fibromuscular dysplasia of renal arteries: A case report. Medicine (Baltimore). 2018;97(46):e12815
20.Ko M, Kamimura K, Ogawa K, et al. Diagnosis and management of fibromuscular dysplasia and segmental arterial mediolysis in gastroenterology field: A mini-review. World Journal of Gastroenterology. 2018;24(32):3637-3649
21.Slavin RE, Saeki K, Bhagavan B, et al. Segmental arterial mediolysis: A precursor to fibromuscular dysplasia? Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology. 1995;8(3):287-294
22.Sharma AM, Gornik HL. Standing arterial waves is NOT fibromuscular dysplasia. Circulation. Cardiovascular Interventions. 2012;5(1):e9-e11
23.Gutmann DH, Ferner RE, Listernick RH, et al. Neurofibromatosis type 1. Nature Reviews. Disease Primers. 2017;3:17004
24.Kamath BM, Spinner NB, Emerick KM, et al. Vascular anomalies in Alagille syndrome: A significant cause of morbidity and mortality. Circulation. 2004;109(11):1354-1358
25.MacCarrick G, Black JH 3rd, Bowdin S, et al. Loeys-Dietz syndrome: A primer for diagnosis and management. Genetics in Medicine: Official Journal of the American College of Medical Genetics. 2014;16(8):576-587
26.Shalhub S, Black JH 3rd, Cecchi AC, et al. Molecular diagnosis in vascular Ehlers-Danlos syndrome predicts pattern of arterial involvement and outcomes. Journal of Vascular Surgery. 2014;60(1):160-169
27.Weinberg I, Gu X, Giri J, et al. Anti-platelet and antihypertension medication use in patients with fibromuscular dysplasia: Results from the United States Registry for Fibromuscular Dysplasia. Vascular Medicine. 2015;20(5):447-453
28.Mahmood RZ, Olin J, Gu X, et al. Unraveling pulsatile tinnitus in FMD: A report of the United States Registry for Fibromuscular Dysplasia. Journal of the American College of Cardiology. 2014;63:A2060
29.O’Connor S, Gornik HL, Froehlich JB, et al. Smoking and adverse outcomes in fibromuscular dysplasia: U.S. Registry report. Journal of the American College of Cardiology. 2016;67(14):1750-1751
30.Markus HS, Hayter E, Levi C, et al. Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): A randomised trial. The Lancet. Neurology. 2015;14(4):361-367
31.Lin J, Sun Y, Zhao S, et al. Safety and efficacy of thrombolysis in cervical artery dissection-related ischemic stroke: A meta-analysis of observational studies. Cerebrovascular Diseases. 2016;42(3-4):272-279
32.Schirmer CM, Atalay B, Malek AM. Endovascular recanalization of symptomatic flow-limiting cervical carotid dissection in an isolated hemisphere. Neurosurgical Focus. 2011;30(6):E16
33.Larsson SC, King A, Madigan J, et al. Prognosis of carotid dissecting aneurysms: Results from CADISS and a systematic review. Neurology. 2017;88(7):646-652
Written By
Ufuk Türkmen
Submitted: 16 January 2024Reviewed: 18 June 2024Published: 03 August 2024
Open access peer-reviewed chapter - ONLINE FIRST
