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Carotid Artery Fibromuscular Dysplasia in Neck
Philip R. Chapman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Fibromuscular dysplasia (FMD)
            • Definitions

              • Arterial disease of unknown etiology affecting medium-sized arteries
                • Noninflammatory, nonatherosclerotic arteriopathy with dysplastic arterial wall with overgrowth of smooth muscle and fibrous tissue
                • While FMD most commonly affects renal arteries, term cervicocranial FMD relates to involvement of cervical internal carotid arteries (ICAs) and vertebral arteries and is associated with intracranial aneurysms

            IMAGING

            • General Features

              • Angiographic Findings

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Abozeed M et al: Screening CT angiography in patients with suspected fibromuscular dysplasia: improved patient care with single-session skull vertex to pelvis coverage. Cardiovasc Diagn Ther. 10(2):201-7, 2020
                                        2. Warchol-Celinska E et al: Systematic and multidisciplinary evaluation of fibromuscular dysplasia patients reveals high prevalence of previously undetected fibromuscular dysplasia lesions and affects clinical decisions: the ARCADIA-POL study. Hypertension. 75(4):1102-9, 2020
                                        3. Gornik HL et al: First international consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 24(2):164-89, 2019
                                        4. Harriott A: Idiopathic non-atherosclerotic carotid artery disease. Curr Treat Options Cardiovasc Med. 21(11):64, 2019
                                        5. Kim SJ et al: Carotid webs in cryptogenic ischemic strokes: a matched case-control study. J Stroke Cerebrovasc Dis. 28(12):104402, 2019
                                        6. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                        7. Kadian-Dodov D et al: Natural history of cervical artery fibromuscular dysplasia and associated neurovascular events. Cerebrovasc Dis. 46(1-2):33-9, 2018
                                        8. Persu A et al: European consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens. 32(7):1367-78, 2014
                                        9. Olin JW et al: The United States registry for fibromuscular dysplasia: results in the first 447 patients. Circulation. 125(25):3182-90, 2012
                                        10. Cohen JE et al: Petrous carotid artery pseudoaneurysm in bilateral carotid fibromuscular dysplasia: treatment by means of self-expanding covered stent. Surg Neurol. 68(2):216-20; discussion 220, 2007
                                        11. de Bray JM et al: Fibromuscular dysplasia may herald symptomatic recurrence of cervical artery dissection. Cerebrovasc Dis. 23(5-6):448-52, 2007
                                        12. Perdu J et al: Inheritance of arterial lesions in renal fibromuscular dysplasia. J Hum Hypertens. 21(5):393-400, 2007
                                        13. Beregi JP et al: Fibromuscular dysplasia of the renal arteries: comparison of helical CT angiography and arteriography. AJR Am J Roentgenol. 172(1):27-34, 1999
                                        14. Van Damme H et al: Fibromuscular dysplasia of the internal carotid artery. Personal experience with 13 cases and literature review. Acta Chir Belg. 99(4):163-8, 1999
                                        15. Wong CY et al: Cerebral perfusion and vascular reserve in fibromuscular dysplasia of the internal carotid artery. J Stroke Cerebrovasc Dis. 7(5):364-6, 1998
                                        16. Stewart MT et al: The natural history of carotid fibromuscular dysplasia. J Vasc Surg. 3(2):305-10, 1986
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  TERMINOLOGY

                                                  • Abbreviations

                                                    • Fibromuscular dysplasia (FMD)
                                                  • Definitions

                                                    • Arterial disease of unknown etiology affecting medium-sized arteries
                                                      • Noninflammatory, nonatherosclerotic arteriopathy with dysplastic arterial wall with overgrowth of smooth muscle and fibrous tissue
                                                      • While FMD most commonly affects renal arteries, term cervicocranial FMD relates to involvement of cervical internal carotid arteries (ICAs) and vertebral arteries and is associated with intracranial aneurysms

                                                  IMAGING

                                                  • General Features

                                                    • Angiographic Findings

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Abozeed M et al: Screening CT angiography in patients with suspected fibromuscular dysplasia: improved patient care with single-session skull vertex to pelvis coverage. Cardiovasc Diagn Ther. 10(2):201-7, 2020
                                                                              2. Warchol-Celinska E et al: Systematic and multidisciplinary evaluation of fibromuscular dysplasia patients reveals high prevalence of previously undetected fibromuscular dysplasia lesions and affects clinical decisions: the ARCADIA-POL study. Hypertension. 75(4):1102-9, 2020
                                                                              3. Gornik HL et al: First international consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 24(2):164-89, 2019
                                                                              4. Harriott A: Idiopathic non-atherosclerotic carotid artery disease. Curr Treat Options Cardiovasc Med. 21(11):64, 2019
                                                                              5. Kim SJ et al: Carotid webs in cryptogenic ischemic strokes: a matched case-control study. J Stroke Cerebrovasc Dis. 28(12):104402, 2019
                                                                              6. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                                                              7. Kadian-Dodov D et al: Natural history of cervical artery fibromuscular dysplasia and associated neurovascular events. Cerebrovasc Dis. 46(1-2):33-9, 2018
                                                                              8. Persu A et al: European consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens. 32(7):1367-78, 2014
                                                                              9. Olin JW et al: The United States registry for fibromuscular dysplasia: results in the first 447 patients. Circulation. 125(25):3182-90, 2012
                                                                              10. Cohen JE et al: Petrous carotid artery pseudoaneurysm in bilateral carotid fibromuscular dysplasia: treatment by means of self-expanding covered stent. Surg Neurol. 68(2):216-20; discussion 220, 2007
                                                                              11. de Bray JM et al: Fibromuscular dysplasia may herald symptomatic recurrence of cervical artery dissection. Cerebrovasc Dis. 23(5-6):448-52, 2007
                                                                              12. Perdu J et al: Inheritance of arterial lesions in renal fibromuscular dysplasia. J Hum Hypertens. 21(5):393-400, 2007
                                                                              13. Beregi JP et al: Fibromuscular dysplasia of the renal arteries: comparison of helical CT angiography and arteriography. AJR Am J Roentgenol. 172(1):27-34, 1999
                                                                              14. Van Damme H et al: Fibromuscular dysplasia of the internal carotid artery. Personal experience with 13 cases and literature review. Acta Chir Belg. 99(4):163-8, 1999
                                                                              15. Wong CY et al: Cerebral perfusion and vascular reserve in fibromuscular dysplasia of the internal carotid artery. J Stroke Cerebrovasc Dis. 7(5):364-6, 1998
                                                                              16. Stewart MT et al: The natural history of carotid fibromuscular dysplasia. J Vasc Surg. 3(2):305-10, 1986