Advertisement

Fibromuscular Dysplasia, Carotid Kinks, and Other Rare Lesions

  • Ahmed Kayssi
  • Dipankar Mukherjee
Chapter

Abstract

While most extracranial cerebrovascular pathologies are secondary to atherosclerosis, several hereditary and acquired conditions may also lead to devastating complications and should be familiar to the vascular specialist. Such conditions include fibromuscular dysplasia, carotid kinks and coils, and other rarer lesions such as Takayasu’s arteritis, giant-cell arteritis, Marfan’s syndrome, and vascular-type Ehlers-Danlos syndrome. Fibromuscular dysplasia is a non-atherosclerotic, noninflammatory arteriopathy that affects middle-sized vessels such as the renal and carotid arteries. It is a chronic, non-curable condition that should be managed medically, unless patients present with complications or are refractory to medical management. Elongated carotid artery abnormalities, including carotid tortuosity, coiling, or kinking, may lead to dissections, aneurysmal degenerations, and stroke and complicate attempts at endovascular repair of the carotid artery. Finally, the management of inflammatory arteriopathies such as Takayasu’s and giant-cell arteritis is primarily medical, but there are no consensus guidelines for these lesions because of their rarity. In this chapter, we will review the medical and operative management of these conditions and provide the reader with an approach to these conditions.

Keywords

Cerebrovascular Carotid artery Fibromuscular dysplasia Carotid kinks Rare lesions 

References

  1. 1.
    Luscher TF, Lie JT, Stanson AW, Houser OW, Hollier LH, Sheps SG. Arterial fibromuscular dysplasia. Mayo Clin Proc. 1987;62(10):931–52.CrossRefGoogle Scholar
  2. 2.
    Leadbetter WF, Burkland CE. Hypertension in unilateral renal disease. J Urol. 1938;39:611–26.CrossRefGoogle Scholar
  3. 3.
    McCormack LJ, Hazard JB, Poutasse EF. Obstructive lesions of the renal artery associated with remediable hypertension. Am J Pathol. 1938;34:582.Google Scholar
  4. 4.
    Connett MC, Lansche JM. Fibromuscular hyperplasia of the internal carotid artery: report of a case. Ann Surg. 1965;162:59–62.CrossRefGoogle Scholar
  5. 5.
    Begelman SM, Olin JW. Fibromuscular dysplasia. Curr Opin Rheumatol. 2000;12(1):41–7.CrossRefGoogle Scholar
  6. 6.
    So EL, Toole JF, Dalal P, Moody DM. Cephalic fibromuscular dysplasia in 32 patients: clinical findings and radiologic features. Arch Neurol. 1981;38(10):619–22.CrossRefGoogle Scholar
  7. 7.
    Brinza EK, Gornik HL. Fibromuscular dysplasia: advances in understanding and management. Cleve Clin J Med. 2016;83(11 Suppl 2):S45–51.CrossRefGoogle Scholar
  8. 8.
    Shivapour DM, Erwin P, Kim E. Epidemiology of fibromuscular dysplasia: a review of the literature. Vasc Med. 2016;21(4):376–81.CrossRefGoogle Scholar
  9. 9.
    Touze E, Oppenheim C, Trystram D, Nokam G, Pasquini M, Alamowitch S, et al. Fibromuscular dysplasia of cervical and intracranial arteries. Int J Stroke. 2010;5(4):296–305.CrossRefGoogle Scholar
  10. 10.
    Olin JW, Froehlich J, Gu X, Bacharach JM, Eagle K, Gray BH, et al. The United States registry for fibromuscular dysplasia: results in the first 447 patients. Circulation. 2012;125(25):3182–90.CrossRefGoogle Scholar
  11. 11.
    Olin JW, Pierce M. Contemporary management of fibromuscular dysplasia. Curr Opin Cardiol. 2008;23(6):527–36.CrossRefGoogle Scholar
  12. 12.
    Olin JW, Sealove BA. Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg. 2011;53(3):826–36.e1.CrossRefGoogle Scholar
  13. 13.
    Rushton AR. The genetics of fibromuscular dysplasia. Arch Intern Med. 1980;140(2):233–6.CrossRefGoogle Scholar
  14. 14.
    Mettinger KL, Ericson K. Fibromuscular dysplasia and the brain. I. Observations on angiographic, clinical and genetic characteristics. Stroke. 1982;13(1):46–52.CrossRefGoogle Scholar
  15. 15.
    Perdu J, Boutouyrie P, Bourgain C, Stern N, Laloux B, Bozec E, et al. Inheritance of arterial lesions in renal fibromuscular dysplasia. J Hum Hypertens. 2007;21(5):393–400.CrossRefGoogle Scholar
  16. 16.
    Harrison EG Jr, McCormack LJ. Pathologic classification of renal arterial disease in renovascular hypertension. Mayo Clin Proc. 1971;46(3):161–7.PubMedGoogle Scholar
  17. 17.
    Poloskey SL, Olin JW, Mace P, Gornik HL. Fibromuscular dysplasia. Circulation. 2012;125(18):e636–9.CrossRefGoogle Scholar
  18. 18.
    Stanley JC, Gewertz BL, Bove EL, Sottiurai V, Fry WJ. Arterial fibrodysplasia. Histopathologic character and current etiologic concepts. Arch Surg. 1975;110(5):561–6.CrossRefGoogle Scholar
  19. 19.
    Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med. 2004;350(18):1862–71.CrossRefGoogle Scholar
  20. 20.
    Aqel R, Gupta R, Zoghbi G. Coexistent fibromuscular dysplasia and atherosclerotic renal artery stenosis. J Invasive Cardiol. 2005;17(10):572–3.PubMedGoogle Scholar
  21. 21.
    Aqel R, AlJaroudi WW, Hage FG, Nanda NC. Renal artery fibromuscular dysplasia is a cause of refractory hypertension in the elderly. Echocardiography. 2009;26(1):109–10.CrossRefGoogle Scholar
  22. 22.
    Jayawardene S, Reidy J, Scoble J. Clinical picture: ipsilateral atherosclerotic and fibromuscular renal artery stenosis. Lancet. 2000;356(9248):2138.CrossRefGoogle Scholar
  23. 23.
    Schievink WI, Bjornsson J, Piepgras DG. Coexistence of fibromuscular dysplasia and cystic medial necrosis in a patient with Marfan’s syndrome and bilateral carotid artery dissections. Stroke. 1994;25(12):2492–6.CrossRefGoogle Scholar
  24. 24.
    Schievink WI, Limburg M. Angiographic abnormalities mimicking fibromuscular dysplasia in a patient with Ehlers-Danlos syndrome, type IV. Neurosurgery. 1989;25(3):482–3.CrossRefGoogle Scholar
  25. 25.
    Slavin RE. Segmental arterial mediolysis: course, sequelae, prognosis, and pathologic-radiologic correlation. Cardiovasc Pathol. 2009;18(6):352–60.CrossRefGoogle Scholar
  26. 26.
    Bol A, Missault L, Dewilde W. Renovascular hypertension associated with neurofibromatosis: a case report. Acta Clin Belg. 2007;62(1):61–3.CrossRefGoogle Scholar
  27. 27.
    Janzen J, Vuong PN, Rothenberger-Janzen K. Takayasu’s arteritis and fibromuscular dysplasia as causes of acquired atypical coarctation of the aorta: retrospective analysis of seven cases. Heart Vessels. 1999;14(6):277–82.CrossRefGoogle Scholar
  28. 28.
    Sandok BA. Fibromuscular dysplasia of the internal carotid artery. Neurol Clin. 1983;1(1):17–26.CrossRefGoogle Scholar
  29. 29.
    Corrin LS, Sandok BA, Houser OW. Cerebral ischemic events in patients with carotid artery fibromuscular dysplasia. Arch Neurol. 1981;38(10):616–8.CrossRefGoogle Scholar
  30. 30.
    Abdul-Rahman AM, Abu S, Brun A, Kin H, Ljunggren B, Mizukami M, et al. Fibromuscular dysplasia of the cervico-cephalic arteries. Surg Neurol. 1978;9(4):217–22.PubMedGoogle Scholar
  31. 31.
    Hart RG, Easton JD. Dissections of cervical and cerebral arteries. Neurol Clin. 1983;1(1):155–82.CrossRefGoogle Scholar
  32. 32.
    Grotta JC, Ward RE, Flynn TC, Cullen ML. Spontaneous internal carotid artery dissection associated with fibromuscular dysplasia. J Cardiovasc Surg. 1982;23(6):512–4.Google Scholar
  33. 33.
    Cloft HJ, Kallmes DF, Kallmes MH, Goldstein JH, Jensen ME, Dion JE. Prevalence of cerebral aneurysms in patients with fibromuscular dysplasia: a reassessment. J Neurosurg. 1998;88(3):436–40.CrossRefGoogle Scholar
  34. 34.
    van de Nes JA, Bajanowski T, Trubner K. Fibromuscular dysplasia of the basilar artery: an unusual case with medico-legal implications. Forensic Sci Int. 2007;173(2–3):188–92.PubMedGoogle Scholar
  35. 35.
    Zimmerman R, Leeds NE, Naidich TP. Carotid-cavernous fistula associated with intracranial fibromuscular dysplasia. Radiology. 1977;122(3):725–6.CrossRefGoogle Scholar
  36. 36.
    Reddy SV, Karnes WE, Earnest F, Sundt TM Jr. Spontaneous extracranial vertebral arteriovenous fistula with fibromuscular dysplasia. Case report. J Neurosurg. 1981;54(3):399–402.CrossRefGoogle Scholar
  37. 37.
    Varennes L, Tahon F, Kastler A, Grand S, Thony F, Baguet JP, et al. Fibromuscular dysplasia: what the radiologist should know: a pictorial review. Insights Imaging. 2015;6(3):295–307.CrossRefGoogle Scholar
  38. 38.
    Osborn AG, Anderson RE. Angiographic spectrum of cervical and intracranial fibromuscular dysplasia. Stroke. 1977;8(5):617–26.CrossRefGoogle Scholar
  39. 39.
    Leung DA, Hoffmann U, Pfammatter T, Hany TF, Rainoni L, Hilfiker P, et al. Magnetic resonance angiography versus duplex sonography for diagnosing renovascular disease. Hypertension. 1999;33(2):726–31.CrossRefGoogle Scholar
  40. 40.
    Carman TL, Olin JW, Czum J. Noninvasive imaging of the renal arteries. Urol Clin North Am. 2001;28(4):815–26.CrossRefGoogle Scholar
  41. 41.
    Furie DM, Tien RD. Fibromuscular dysplasia of arteries of the head and neck: imaging findings. AJR Am J Roentgenol. 1994;162(5):1205–9.CrossRefGoogle Scholar
  42. 42.
    Jahnlova D, Veselka J. Fibromuscular dysplasia of renal and carotid arteries. Int J Angiol. 2015;24(3):241–3.CrossRefGoogle Scholar
  43. 43.
    Kadian-Dodov D, Gornik HL, Gu X, Froehlich J, Bacharach JM, Chi YW, et al. Dissection and aneurysm in patients with fibromuscular dysplasia: findings from the U.S. registry for FMD. J Am Coll Cardiol. 2016;68(2):176–85.CrossRefGoogle Scholar
  44. 44.
    Schievink WI. Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med. 2001;344(12):898–906.CrossRefGoogle Scholar
  45. 45.
    Edgell RC, Abou-Chebl A, Yadav JS. Endovascular management of spontaneous carotid artery dissection. J Vasc Surg. 2005;42(5):854–60. Discussion 60.CrossRefGoogle Scholar
  46. 46.
    Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X. Fibromuscular dysplasia. Orphanet J Rare Dis. 2007;2:28.CrossRefGoogle Scholar
  47. 47.
    Stewart MT, Moritz MW, Smith RB 3rd, Fulenwider JT, Perdue GD. The natural history of carotid fibromuscular dysplasia. J Vasc Surg. 1986;3(2):305–10.CrossRefGoogle Scholar
  48. 48.
    Edington GH. Tortuosity of both internal carotid arteries. Br Med J. 1901;2(2134):1526–7.CrossRefGoogle Scholar
  49. 49.
    Mukherjee D, Inahara T. Management of the tortuous internal carotid artery. Am J Surg. 1985;149(5):651–5.CrossRefGoogle Scholar
  50. 50.
    Milic DJ, Jovanovic MM, Zivic SS, Jankovic RJ. Coiling of the left common carotid artery as a cause of transient ischemic attacks. J Vasc Surg. 2007;45(2):411–3.CrossRefGoogle Scholar
  51. 51.
    Stilo F, Catanese V, Casale M, Bernardini S, Montelione N, Spinelli F. Carotid-carotid bypass graft for internal carotid artery kinking causing dysphagia. Ann Vasc Surg. 2017;43:310.e5–7.CrossRefGoogle Scholar
  52. 52.
    Leipzig TJ, Dohrmann GJ. The tortuous or kinked carotid artery: pathogenesis and clinical considerations. A historical review. Surg Neurol. 1986;25(5):478–86.CrossRefGoogle Scholar
  53. 53.
    Cioffi FA, Meduri M, Tomasello F, Bonavita V, Conforti P. Kinking and coiling of the internal carotid artery: clinical-statistical observations and surgical perspectives. J Neurosurg Sci. 1975;19(1–2):15–22.PubMedGoogle Scholar
  54. 54.
    Poulias GE, Skoutas B, Doundoulakis N, Haddad H, Karkanias G, Lyberiadis D. Kinking and coiling of internal carotid artery with and without associated stenosis. Surgical considerations and long-term follow-up. Panminerva Med. 1996;38(1):22–7.PubMedGoogle Scholar
  55. 55.
    Del Corso L, Moruzzo D, Conte B, Agelli M, Romanelli AM, Pastine F, et al. Tortuosity, kinking, and coiling of the carotid artery: expression of atherosclerosis or aging? Angiology. 1998;49(5):361–71.CrossRefGoogle Scholar
  56. 56.
    Weibel J, Fields WS. Tortuosity, coiling, and kinking of the internal carotid artery. I. Etiology and radiographic anatomy. Neurology. 1965;15:7–18.CrossRefGoogle Scholar
  57. 57.
    Rowlands RP, Swan RH. Tortuosity of both internal carotid arteries. Br Med J. 1902;1(2141):76.CrossRefGoogle Scholar
  58. 58.
    Vannix RS, Joergenson EJ, Carter R. Kinking of the internal carotid artery. Clinical significance and surgical management. Am J Surg. 1977;134(1):82–9.CrossRefGoogle Scholar
  59. 59.
    Ballotta E, Thiene G, Baracchini C, Ermani M, Militello C, Da Giau G, et al. Surgical vs medical treatment for isolated internal carotid artery elongation with coiling or kinking in symptomatic patients: a prospective randomized clinical study. J Vasc Surg. 2005;42(5):838–46. Discussion 46.CrossRefGoogle Scholar
  60. 60.
    Metz H, Murray-Leslie RM, Bannister RG, Bull JW, Marshall J. Kinking of the internal carotid artery. Lancet. 1961;1(7174):424–6.CrossRefGoogle Scholar
  61. 61.
    Scotti G, Melancon D, Olivier A. Hypoglossal paralysis due to compression by a tortuous internal carotid artery in the neck. Neuroradiology. 1978;14(5):263–5.CrossRefGoogle Scholar
  62. 62.
    Geraud JR, Ducoudray J, Ribaut L. Long internal carotid artery with vertigo syndrome. Rev Neurol (Paris). 1951;85(2):145–7.Google Scholar
  63. 63.
    Grego F, Lepidi S, Cognolato D, Frigatti P, Morelli I, Deriu GP. Rationale of the surgical treatment of carotid kinking. J Cardiovasc Surg. 2003;44(1):79–85.Google Scholar
  64. 64.
    Alibaz-Oner F, Direskeneli H. Update on Takayasu’s arteritis. Presse Med. 2015;44(6 Pt 2):e259–65.CrossRefGoogle Scholar
  65. 65.
    Hao R, Zhang J, Ma Z, Xiao M, Zhou L, Kang N, et al. Takayasu’s arteritis presenting with common carotid artery dissection: a rare case report. Exp Ther Med. 2016;12(6):4061–3.CrossRefGoogle Scholar
  66. 66.
    Park SH, Chung JW, Lee JW, Han MH, Park JH. Carotid artery involvement in Takayasu’s arteritis: evaluation of the activity by ultrasonography. J Ultrasound Med. 2001;20(4):371–8.CrossRefGoogle Scholar
  67. 67.
    Park JH, Chung JW, Im JG, Kim SK, Park YB, Han MC. Takayasu arteritis: evaluation of mural changes in the aorta and pulmonary artery with CT angiography. Radiology. 1995;196(1):89–93.CrossRefGoogle Scholar
  68. 68.
    Tabata M, Kitagawa T, Saito T, Uozaki H, Oshiro H, Miyata T, et al. Extracranial carotid aneurysm in Takayasu’s arteritis. J Vasc Surg. 2001;34(4):739–42.CrossRefGoogle Scholar
  69. 69.
    Takagi A, Tada Y, Sato O, Miyata T. Surgical treatment for Takayasu’s arteritis. A long-term follow-up study. J Cardiovasc Surg. 1989;30(4):553–8.Google Scholar
  70. 70.
    Matsumura K, Hirano T, Takeda K, Matsuda A, Nakagawa T, Yamaguchi N, et al. Incidence of aneurysms in Takayasu’s arteritis. Angiology. 1991;42(4):308–15.CrossRefGoogle Scholar
  71. 71.
    Kumar S, Subramanyan R, Mandalam KR, Rao VR, Gupta AK, Joseph S, et al. Aneurysmal form of aortoarteritis (Takayasu’s disease): analysis of thirty cases. Clin Radiol. 1990;42(5):342–7.CrossRefGoogle Scholar
  72. 72.
    Gonzalez-Gay MA, Barros S, Lopez-Diaz MJ, Garcia-Porrua C, Sanchez-Andrade A, Llorca J. Giant cell arteritis: disease patterns of clinical presentation in a series of 240 patients. Medicine (Baltimore). 2005;84(5):269–76.CrossRefGoogle Scholar
  73. 73.
    Nesher G. The diagnosis and classification of giant cell arteritis. J Autoimmun. 2014;48-49:73–5.CrossRefGoogle Scholar
  74. 74.
    Ly KH, Regent A, Tamby MC, Mouthon L. Pathogenesis of giant cell arteritis: more than just an inflammatory condition? Autoimmun Rev. 2010;9(10):635–45.CrossRefGoogle Scholar
  75. 75.
    Shikino K, Yamashita S, Ikusaka M. Giant cell arteritis with carotidynia. J Gen Intern Med. 2017;32:1403.CrossRefGoogle Scholar
  76. 76.
    Watelet B, Samson M, de Boysson H, Bienvenu B. Treatment of giant-cell arteritis, a literature review. Mod Rheumatol. 2017:1–8.Google Scholar
  77. 77.
    Canadas V, Vilacosta I, Bruna I, Fuster V. Marfan syndrome. Part 1: pathophysiology and diagnosis. Nat Rev Cardiol. 2010;7(5):256–65.CrossRefGoogle Scholar
  78. 78.
    Canadas V, Vilacosta I, Bruna I, Fuster V. Marfan syndrome. Part 2: treatment and management of patients. Nat Rev Cardiol. 2010;7(5):266–76.CrossRefGoogle Scholar
  79. 79.
    Alurkar A, Karanam LS, Oak S, Sorte S. Carotid dissection in Marfan’s syndrome. Neurol India. 2013;61(2):206–7.CrossRefGoogle Scholar
  80. 80.
    Ohyama T, Ohara S, Momma F. Aneurysm of the cervical internal carotid artery associated with Marfan’s syndrome—case report. Neurol Med Chir (Tokyo). 1992;32(13):965–8.CrossRefGoogle Scholar
  81. 81.
    Latter DA, Ricci MA, Forbes RD, Graham AM. Internal carotid artery aneurysm and Marfan’s syndrome. Can J Surg. 1989;32(6):463–6.PubMedGoogle Scholar
  82. 82.
    Sztajzel R, Hefft S, Girardet C. Marfan’s syndrome and multiple extracranial aneurysms. Cerebrovasc Dis. 2001;11(4):346–9.CrossRefGoogle Scholar
  83. 83.
    Eagleton MJ. Arterial complications of vascular Ehlers-Danlos syndrome. J Vasc Surg. 2016;64(6):1869–80.CrossRefGoogle Scholar
  84. 84.
    Pepin M, Schwarze U, Superti-Furga A, Byers PH. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342(10):673–80.CrossRefGoogle Scholar
  85. 85.
    De Paepe A, Malfait F. The Ehlers-Danlos syndrome, a disorder with many faces. Clin Genet. 2012;82(1):1–11.CrossRefGoogle Scholar
  86. 86.
    Debette S, Germain DP. Neurologic manifestations of inherited disorders of connective tissue. Handb Clin Neurol. 2014;119:565–76.CrossRefGoogle Scholar

Copyright information

© The Author(s) 2018

Authors and Affiliations

  • Ahmed Kayssi
    • 1
  • Dipankar Mukherjee
    • 2
  1. 1.Vascular SurgeryUniversity of TorontoTorontoCanada
  2. 2.Vascular SurgeryInova Fairfax HospitalFalls ChurchUSA

Personalised recommendations