Skip to main content

Advertisement

SpringerLink
Log in

Multimodality imaging of fibromuscular dysplasia

  • Pictorial Essay
  • Published:
Abdominal Radiology Aims and scope Submit manuscript

Abstract

Objective

Fibromuscular dysplasia (FMD) is an uncommon non-inflammatory and non-atherosclerotic cause of arterial disease that may result in stenosis, tortuosity, aneurysm, or dissection. The clinical presentation depends on the vascular bed involved and ranges from asymptomatic to multisystem disease and end organ ischemia. The purpose of this article is to review the role of imaging in patients with FMD with an emphasis on renal FMD. The relevant epidemiology, histopathology, imaging techniques, and interpretation of images will be discussed.

Conclusion

Renal artery FMD requires a high index of suspicion for accurate and prompt diagnosis and implementation of appropriate therapy. The treatment will vary based on clinical presentation and distribution of involvement. Noninvasive imaging with duplex ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI) are reasonable alternatives for the depiction of FMD in comparison to catheter-directed angiography (CA). Patients with FMD are often treated by multispecialty practice including the interventional radiologist.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11

Similar content being viewed by others

References

  1. Olin JW, Sealove BA (2011) Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg 53(3):826–836 e821

    Article  PubMed  Google Scholar 

  2. Persu A, Touze E, Mousseaux E, et al. (2012) Diagnosis and management of fibromuscular dysplasia: an expert consensus. Eur J Clin Invest 42(3):338–347

    Article  PubMed  Google Scholar 

  3. Plouin PF, Perdu J, La Batide-Alanore A, et al. (2007) Fibromuscular dysplasia. Orphanet J Rare Dis 2:28

    Article  PubMed  PubMed Central  Google Scholar 

  4. Olin JW, Froehlich J, Gu X, et al. (2012) The United States Registry for fibromuscular dysplasia: results in the first 447 patients. Circulation 125(25):3182–3190

    Article  PubMed  Google Scholar 

  5. Cragg AH, Smith TP, Thompson BH, et al. (1989) Incidental fibromuscular dysplasia in potential renal donors: long-term clinical follow-up. Radiology 172(1):145–147

    Article  CAS  PubMed  Google Scholar 

  6. Blondin D, Lanzman R, Schellhammer F, et al. (2010) Fibromuscular dysplasia in living renal donors: still a challenge to computed tomographic angiography. Eur J Radiol 75(1):67–71

    Article  CAS  PubMed  Google Scholar 

  7. Neymark E, LaBerge JM, Hirose R, et al. (2000) Arteriographic detection of renovascular disease in potential renal donors: incidence and effect on donor surgery. Radiology 214(3):755–760

    Article  CAS  PubMed  Google Scholar 

  8. Lorenz EC, Vrtiska TJ, Lieske JC, et al. (2010) Prevalence of renal artery and kidney abnormalities by computed tomography among healthy adults. Clin J Am Soc Nephrol 5(3):431–438

    Article  PubMed  PubMed Central  Google Scholar 

  9. Hendricks NJ, Matsumoto AH, Angle JF, et al. (2014) Is fibromuscular dysplasia underdiagnosed? A comparison of the prevalence of FMD seen in CORAL trial participants versus a single institution population of renal donor candidates. Vasc Med 19(5):363–367

    Article  PubMed  Google Scholar 

  10. Slovut DP, Olin JW (2004) Fibromuscular dysplasia. N Engl J Med 350(18):1862–1871

    Article  CAS  PubMed  Google Scholar 

  11. Savard S, Steichen O, Azarine A, et al. (2012) Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics. Circulation 126(25):3062–3069

    Article  PubMed  Google Scholar 

  12. Tegtmeyer CJ, Selby JB, Hartwell GD, Ayers C, Tegtmeyer V (1991) Results and complications of angioplasty in fibromuscular disease. Circulation 83(2 Suppl):I155–I161

    CAS  PubMed  Google Scholar 

  13. Alhadad A, Mattiasson I, Ivancev K, Gottsater A, Lindblad B (2005) Revascularisation of renal artery stenosis caused by fibromuscular dysplasia: effects on blood pressure during 7-year follow-up are influenced by duration of hypertension and branch artery stenosis. J Hum Hypertens 19(10):761–767

    Article  CAS  PubMed  Google Scholar 

  14. Davies MG, Saad WE, Peden EK, et al. (2008) The long-term outcomes of percutaneous therapy for renal artery fibromuscular dysplasia. J Vasc Surg 48(4):865–871

    Article  PubMed  Google Scholar 

  15. Trinquart L, Mounier-Vehier C, Sapoval M, Gagnon N, Plouin PF (2010) Efficacy of revascularization for renal artery stenosis caused by fibromuscular dysplasia: a systematic review and meta-analysis. Hypertension 56(3):525–532

    Article  CAS  PubMed  Google Scholar 

  16. Olin JWGH, Bacharach MJ, Biller J, Fine LJ, et al. (2014) Fibromuscular dysplasia: state of the science and critical unanswered questions. Circulation 129:1048–1078

    Article  PubMed  Google Scholar 

  17. Saw J, Ricci D, Starovoytov A, Fox R, Buller CE (2013) Spontaneous coronary artery dissection: prevalence of predisposing conditions including fibromuscular dysplasia in a tertiary center cohort. JACC Cardiovasc Interv 6(1):44–52

    Article  PubMed  Google Scholar 

  18. Olin JW, Gornik HL, Bacharach JM, et al. (2014) Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation 129(9):1048–1078

    Article  PubMed  Google Scholar 

  19. Savard S, Azarine A, Jeunemaitre X, et al. (2013) Association of smoking with phenotype at diagnosis and vascular interventions in patients with renal artery fibromuscular dysplasia. Hypertension 61(6):1227–1232

    Article  CAS  PubMed  Google Scholar 

  20. Perdu J, Boutouyrie P, Bourgain C, et al. (2007) Inheritance of arterial lesions in renal fibromuscular dysplasia. J Hum Hypertens 21(5):393–400

    CAS  PubMed  Google Scholar 

  21. Kiando SR, Tucker TN, Katz A, et al. (2015) Genetic study identifies common variation in PHACTR1 to associate with fibromuscular dysplasia. Circulation 132:A15370

    Google Scholar 

  22. Ganesh SK, Morissette R, Xu Z, et al. (2014) Clinical and biochemical profiles suggest fibromuscular dysplasia is a systemic disease with altered TGF-beta expression and connective tissue features. FASEB J 28(8):3313–3324

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Poloskey SL, Kim E, Sanghani R, et al. (2012) Low yield of genetic testing for known vascular connective tissue disorders in patients with fibromuscular dysplasia. Vasc Med 17(6):371–378

    Article  PubMed  Google Scholar 

  24. Harrison EGML (1971) Pathologic classification of renal arterial disease in renovascular hypertension. Mayo Clin Proc 46:161–167

    PubMed  Google Scholar 

  25. Kadian-Dodov D, Gornik H, Gu X, et al. (2014) Aneurysm and dissection in fibromuscular dysplasia: findings from the United States registry for FMD. J Am Coll Cardiol 63(12_S):A2030

    Article  Google Scholar 

  26. Meuse MA, Turba UC, Sabri SS, et al. (2010) Treatment of renal artery fibromuscular dysplasia. Tech Vasc Interv Radiol 13(2):126–133

    Article  PubMed  Google Scholar 

  27. Pannier-Moreau I, Grimbert P, Fiquet-Kempf B, et al. (1997) Possible familial origin of multifocal renal artery fibromuscular dysplasia. J Hypertens 15(12 Pt 2):1797–1801

    Article  CAS  PubMed  Google Scholar 

  28. McKenzie GA, Oderich GS, Kawashima A, Misra S (2013) Renal artery fibromuscular dysplasia in 2,640 renal donor subjects: a CT angiography analysis. J Vasc Interv Radiol 24(10):1477–1480

    Article  PubMed  PubMed Central  Google Scholar 

  29. Lassiter FD (1998) The string-of-beads sign. Radiology 206(2):437–438

    Article  CAS  PubMed  Google Scholar 

  30. Gowda MS, Loeb AL, Crouse LJ, Kramer PH (2003) Complementary roles of color-flow duplex imaging and intravascular ultrasound in the diagnosis of renal artery fibromuscular dysplasia: should renal arteriography serve as the “gold standard”? J Am Coll Cardiol 41(8):1305–1311

    Article  PubMed  Google Scholar 

  31. Prasad A, Zafar N, Mahmud E (2009) Assessment of renal artery fibromuscular dysplasia: angiography, intravascular ultrasound (with virtual histology), and pressure wire measurements. Catheter Cardiovasc Interv 74(2):260–264

    PubMed  Google Scholar 

  32. Lau JF (2012) Clinical evaluation of renal artery disease. In: Creager Mark A, Loscalzo Joseph (eds) Vascular medicine: a companion to Braunwald’s heart disease, 2nd edn. Philedelphia: Elsevier Saunders, Inc., pp 296–306

    Google Scholar 

  33. Zierler REOK (2010) Renal duplex scanning. In: Zierler RE (ed) Strandness’s duplex scanning in vascular disorders, 4th edn. Philadelphia: Lippincott Williams & Wilkins, pp 283–310

    Google Scholar 

  34. Das CJ, Neyaz Z, Thapa P, Sharma S, Vashist S (2007) Fibromuscular dysplasia of the renal arteries: a radiological review. Int Urol Nephrol 39(1):233–238

    Article  PubMed  Google Scholar 

  35. Sharma AM, Gornik HL (2012) Standing arterial waves is NOT fibromuscular dysplasia. Circ Cardiovasc Interv 5(1):e9–e11

    Article  PubMed  Google Scholar 

  36. Lehrer H (1967) The physiology of angiographic arterial waves. Radiology 89(1):11–19

    Article  CAS  PubMed  Google Scholar 

  37. Jacobsen JC, Beierholm U, Mikkelsen R, et al. (2002) “Sausage-string” appearance of arteries and arterioles can be caused by an instability of the blood vessel wall. Am J Physiol 283(5):R1118–R1130

    Google Scholar 

  38. Olin JWPM, Young JR, DeAnna S, Grubb M, Childs MB (1995) The utility of duplex ultrasound scanning of the renal arteries for diagnosing significant renal artery stenosis. Ann Intern Med 11(122):833–838

    Article  Google Scholar 

  39. Jw O (1992) Role of duplex ultrasonography in screening for significant renal artery disease. Urol Clin North Am 2(21):215–226

    Google Scholar 

  40. Mousa AY, Campbell JE, Stone PA, et al. (2012) Short- and long-term outcomes of percutaneous transluminal angioplasty/stenting of renal fibromuscular dysplasia over a ten-year period. J Vasc Surg 55(2):421–427

    Article  PubMed  Google Scholar 

  41. Edwards JM, Zaccardi MJ, Strandness DE Jr (1992) A preliminary study of the role of duplex scanning in defining the adequacy of treatment of patients with renal artery fibromuscular dysplasia. J Vasc Surg 15(4):604–609 (discussion 609-611)

    Article  CAS  PubMed  Google Scholar 

  42. Li JC, Yuan Y, Qin W, et al. (2007) Evaluation of the tardus-parvus pattern in patients with atherosclerotic and nonatherosclerotic renal artery stenosis. J Ultrasound Med 26(4):419–426

    PubMed  Google Scholar 

  43. Kim TS, Chung JW, Park JH, et al. (1998) Renal artery evaluation: comparison of spiral CT angiography to intra-arterial DSA. J Vasc Interv Radiol 9(4):553–559

    Article  CAS  PubMed  Google Scholar 

  44. Olbricht CJ, Paul K, Prokop M, et al. (1995) Minimally invasive diagnosis of renal artery stenosis by spiral computed tomography angiography. Kidney Int 48(4):1332–1337

    Article  CAS  PubMed  Google Scholar 

  45. Sabharwal R, Vladica P, Coleman P (2007) Multidetector spiral CT renal angiography in the diagnosis of renal artery fibromuscular dysplasia. Eur J Radiol 61(3):520–527

    Article  PubMed  Google Scholar 

  46. Beregi JP, Louvegny S, Gautier C, et al. (1999) Fibromuscular dysplasia of the renal arteries: comparison of helical CT angiography and arteriography. AJR Am J Roentgenol 172(1):27–34

    Article  CAS  PubMed  Google Scholar 

  47. Vasbinder GB, Nelemans PJ, Kessels AG, et al. (2004) Accuracy of computed tomographic angiography and magnetic resonance angiography for diagnosing renal artery stenosis. Ann Intern Med 141(9):674–682 (discussion 682)

    Article  PubMed  Google Scholar 

  48. Galanski M, Prokop M, Chavan A, et al. (1993) Renal arterial stenoses: spiral CT angiography. Radiology 189(1):185–192

    Article  CAS  PubMed  Google Scholar 

  49. Rubin GD, Dake MD, Napel S, et al. (1994) Spiral CT of renal artery stenosis: comparison of three-dimensional rendering techniques. Radiology 190(1):181–189

    Article  CAS  PubMed  Google Scholar 

  50. Leiner T, Michaely H (2008) Advances in contrast-enhanced MR angiography of the renal arteries. Magn Reson Imaging Clin N Am. 16(4):561–572, vii

    Article  PubMed  Google Scholar 

  51. Loubeyre P, Trolliet P, Cahen R, et al. (1996) MR angiography of renal artery stenosis: value of the combination of three-dimensional time-of-flight and three-dimensional phase-contrast MR angiography sequences. AJR Am J Roentgenol 167(2):489–494

    Article  CAS  PubMed  Google Scholar 

  52. Silverman JM, Friedman ML, Van Allan RJ (1996) Detection of main renal artery stenosis using phase-contrast cine MR angiography. AJR Am J Roentgenol 166(5):1131–1137

    Article  CAS  PubMed  Google Scholar 

  53. Michaely HJ, Schoenberg SO, Ittrich C, et al. (2004) Renal disease: value of functional magnetic resonance imaging with flow and perfusion measurements. Invest Radiol 39(11):698–705

    Article  PubMed  Google Scholar 

  54. Potthast S, Maki JH (2008) Non-contrast-enhanced MR imaging of the renal arteries. Magn Reson Imaging Clin N Am 16(4):573–584, vii

    Article  PubMed  Google Scholar 

  55. Glockner JF, Takahashi N, Kawashima A, et al. (2010) Non-contrast renal artery MRA using an inflow inversion recovery steady state free precession technique (Inhance): comparison with 3D contrast-enhanced MRA. J Magn Reson Imaging 31(6):1411–1418

    Article  PubMed  Google Scholar 

  56. Furie DM, Tien RD (1994) Fibromuscular dysplasia of arteries of the head and neck: imaging findings. AJR Am J Roentgenol 162(5):1205–1209

    Article  CAS  PubMed  Google Scholar 

  57. Korst MB, Joosten FB, Postma CT, et al. (2000) Accuracy of normal-dose contrast-enhanced MR angiography in assessing renal artery stenosis and accessory renal arteries. AJR Am J Roentgenol 174(3):629–634

    Article  CAS  PubMed  Google Scholar 

  58. Lee VS, Rofsky NM, Krinsky GA, Stemerman DH, Weinreb JC (1999) Single-dose breath-hold gadolinium-enhanced three-dimensional MR angiography of the renal arteries. Radiology 211(1):69–78

    Article  CAS  PubMed  Google Scholar 

  59. Gilfeather M, Yoon HC, Siegelman ES, et al. (1999) Renal artery stenosis: evaluation with conventional angiography versus gadolinium-enhanced MR angiography. Radiology 210(2):367–372

    Article  CAS  PubMed  Google Scholar 

  60. Willoteaux S, Faivre-Pierret M, Moranne O, et al. (2006) Fibromuscular dysplasia of the main renal arteries: comparison of contrast-enhanced MR angiography with digital subtraction angiography. Radiology 241(3):922–929

    Article  PubMed  Google Scholar 

  61. Rountas C, Vlychou M, Vassiou K, et al. (2007) Imaging modalities for renal artery stenosis in suspected renovascular hypertension: prospective intraindividual comparison of color Doppler US, CT angiography, GD-enhanced MR angiography, and digital substraction angiography. Ren Fail 29(3):295–302

    Article  CAS  PubMed  Google Scholar 

  62. Hirsch AT, Haskal ZJ, Hertzer NR, et al. (2006) ACC/AHA 2005 Practice Guidelines for the management of patients with peripheral arterial disease (lower extremity, renal, mesenteric, and abdominal aortic): a collaborative report from the American Association for Vascular Surgery/Society for Vascular Surgery, Society for Cardiovascular Angiography and Interventions, Society for Vascular Medicine and Biology, Society of Interventional Radiology, and the ACC/AHA Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Patients With Peripheral Arterial Disease): endorsed by the American Association of Cardiovascular and Pulmonary Rehabilitation; National Heart, Lung, and Blood Institute; Society for Vascular Nursing; TransAtlantic Inter-Society Consensus; and Vascular Disease Foundation. Circulation 113(11):e463–e654

    Article  PubMed  Google Scholar 

  63. Sos TA, Pickering TG, Sniderman K, et al. (1983) Percutaneous transluminal renal angioplasty in renovascular hypertension due to atheroma or fibromuscular dysplasia. N Engl J Med 309(5):274–279

    Article  CAS  PubMed  Google Scholar 

  64. Kim HJ, Do YS, Shin SW, et al. (2008) Percutaneous transluminal angioplasty of renal artery fibromuscular dysplasia: mid-term results. Korean J Radiol 9(1):38–44

    Article  PubMed  PubMed Central  Google Scholar 

  65. Begelman SM, Olin JW (2000) Fibromuscular dysplasia. Curr Opin Rheumatol 12(1):41–47

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Body Imaging Section, Department of Radiology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, New York, NY, 10029, USA

    Sara Lewis & A. Bansal

  2. Division of Cardiology, Department of Medicine, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, New York, NY, 10029, USA

    Daniella Kadian-Dodov

  3. Division of Interventional Radiology, Department of Radiology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, New York, NY, 10029, USA

    R. A. Lookstein

Authors
  1. Sara Lewis
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniella Kadian-Dodov
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. Bansal
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R. A. Lookstein
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sara Lewis.

Ethics declarations

Funding

This study did not receive funding.

Conflict of Interest

Dr. Lewis declares that she has no conflict of interest. Dr. Kadian-Dodov declares that she has no conflict of interest. Dr. Bansal declares that she has no conflict of interest. Dr. Lookstein is a consultant for Johnson and Johnson.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Lewis, S., Kadian-Dodov, D., Bansal, A. et al. Multimodality imaging of fibromuscular dysplasia. Abdom Radiol 41, 2048–2060 (2016). https://doi.org/10.1007/s00261-016-0778-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00261-016-0778-8

Keywords

Search

Navigation