eMedicine Specialties > Neurology > Neuro-vascular Diseases

Fibromuscular Dysplasia: Follow-up

Author: James A Wilson, MD, MSc, FRCPC, BSc(H), Neurologist and Clinical Neurophysiologist, Oconee Neurology Services
Coauthor(s): Richard L Hughes, MD, Associate Professor, Department of Neurology, University of Colorado School of Medicine; Director, University of Colorado Affiliated Hospitals Stroke Project; Chief, Division of Neurology, Denver Health Medical Center
Contributor Information and Disclosures

Updated: Aug 30, 2007

Follow-up

Further Inpatient Care

  • Physical and occupational therapy and speech therapy may be important aspects of patient care should neurologic deficits exist.

Further Outpatient Care

  • Neurorehabilitation generally helps to recover function if any residual neurologic deficits are present.

Inpatient & Outpatient Medications

  • As mentioned above, life-long antiplatelet agents are generally suggested if a stroke occurred because of their benefit in reducing stroke and their relatively low morbidity.
  • Given the potential pro-coagulant effects of estrogen, especially in smokers, oral contraceptives and hormonal replacement therapy is often avoided. No data exists to support or refute this strategy.

Deterrence/Prevention

  • Lifestyle changes that minimize the risk of vascular disease should be stressed.
  • These changes include quitting smoking, weight control, doing exercise, and eating a healthy diet.

Complications

  • The complications of FMD can be diverse, notably because it is a systemic vascular disease. Fortunately, most patients have relatively isolated disease, and invasive measures during follow-up are not required.
  • Blood pressure should be monitored carefully throughout the patient's lifetime because of the possibility of renovascular hypertension.
  • Any symptoms compatible with ischemia and angiographic findings should prompt consideration of FMD as the underlying etiology. Symptoms include neurologic deficits, angina pectoris, limb claudication, and abdominal pain.

Prognosis

  • Statistics on the natural course of cerebrovascular FMD are not available, especially because most cases are symptomatic and relatively benign.
  • Case series tend to present a relatively favorable picture of long-term stroke-free survival, both in medically and in surgically managed patients. For example, in a 1981 report, Collins et al monitored 18 patients after surgical dilatation for a mean of just over 4 years, and none had strokes.32 They monitored 5 patients with global symptoms (eg, hypoperfusion and not embolic events) and conservative therapy for a mean of 42 months, and none had strokes.
  • Saccular aneurysm rupture has such a high mortality that autopsy series may be biased in their detection of FMD. The presence of saccular aneurysms likely poses the greatest morbidity and mortality threat, especially if blood pressure is not controlled.
  • In a prospective series of patients with carotid artery dissection observed for an average of 4 years, of those with recurrence (5 of 103), 80% had FMD.20

Patient Education

  • Patients with FMD who have experienced strokes should receive proper education in this regard. Further education regarding FMD should help patients recognize further symptoms that may indicate disease progression or complications.
  • Education regarding other risk factors for stroke should be provided where applicable. Topics should include abstinence from smoking, the benefits of a healthy diet, and good glycemic control.
  • Patients should be informed about the risk of dissection and cautioned to avoid neck trauma and rigorous neck manipulations.
  • For excellent patient education resources, visit eMedicine's Brain and Nervous System. Also, see eMedicine's patient education articles Aneurysm, Brain and Stroke.

Miscellaneous

Medicolegal Pitfalls

  • The medicolegal issues in FMD stem from the fact that this disease entity is rare and that large prospective studies are lacking to guide management.
  • Some authors suggest more aggressive management such as early surgery, while others favor a more conservative approach.
  • Retrospective studies suggest a favorable outcome in both treatment pathways, but unexpected bad outcomes occasionally occur.
  • Therefore, educating the patient regarding what is known about FMD and what treatment strategies are available is especially important. This allows them to make an informed decision based on the risks and benefits involved in each strategy.

Special Concerns

  • FMD can progress to cause a variety of systemic ischemic problems.
  • An individual with known FMD of any artery should be monitored for symptoms and signs of ischemic events in all vascular distributions (see the Clinical section above).
 


More on Fibromuscular Dysplasia

Overview: Fibromuscular Dysplasia
Differential Diagnoses & Workup: Fibromuscular Dysplasia
Treatment & Medication: Fibromuscular Dysplasia
Follow-up: Fibromuscular Dysplasia
Multimedia: Fibromuscular Dysplasia
References
[ CLOSE WINDOW ]

References

  1. Luscher TF, Lie JT, Stanson AW, et al. Arterial fibromuscular dysplasia. Mayo Clin Proc. Oct 1987;62(10):931-52. [Medline].

  2. Gray GH, Young JR, Olin JW. Miscellaneous arterial diseases. In: Young JR, Olin JW, Bartholomew J, eds. Peripheral Vascular Diseases. 2nd ed. St Louis: Mosby-Yearbook; 1996:425-40.

  3. James TN. Morphologic characteristics and functional significance of focal fibromuscular dysplasia of small coronary arteries. Am J Cardiol. Apr 3 1990;65(14):12G-22G. [Medline].

  4. Campman SC, Holmes JF, Sokolove PE, et al. Pulmonary arterial fibromuscular dysplasia: a rare cause of fulminant lunghemorrhage. Am J Forensic Med Pathol. Mar 2000;21(1):69-73. [Medline].

  5. Maresi E, Becchina G, Ottoveggio G, et al. Arrhythmic sudden cardiac death in a 3-year-old child with intimal fibroplasia of coronary arteries, aorta, and its branches. Cardiovasc Pathol. Jan-Feb 2001;10(1):43-8. [Medline].

  6. Luscher TF, Keller HM, Imhof HG, et al. Fibromuscular hyperplasia: extension of the disease and therapeutic outcome. Results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia. Nephron. 1986;44 Suppl 1:109-14. [Medline].

  7. Hill LD, Antonius JI. Arterial dysplasia: an important surgical lesion. Arch Surg. Apr 1965;90:585-95. [Medline].

  8. Heffelfinger MJ, Holley KE, Havrison EG. Arterial fibromuscular dysplasia studied at autopsy [abstract]. Am J Clin Pathol. 1970;54:274.

  9. Schievink WI, Bjornsson J. Fibromuscular dysplasia of the internal carotid artery: a clinicopathological study. Clin Neuropathol. Jan-Feb 1996;15(1):2-6. [Medline].

  10. Mettinger KL, Ericson K. Fibromuscular dysplasia and the brain. I. Observations on angiographic, clinical and genetic characteristics. Stroke. Jan-Feb 1982;13(1):46-52. [Medline].

  11. Cloft HJ, Kallmes DF, Kallmes MH, et al. Prevalence of cerebral aneurysms in patients with fibromuscular dysplasia:a reassessment. J Neurosurg. Mar 1998;88(3):436-40. [Medline].

  12. Stanley JC, Gewertz BL, Bove EL, et al. Arterial fibrodysplasia. Histopathologic character and current etiologic concepts. Arch Surg. May 1975;110(5):561-6. [Medline].

  13. Arunodaya GR, Vani S, Shankar SK, et al. Fibromuscular dysplasia with dissection of basilar artery presenting as "locked-in-syndrome". Neurology. Jun 1997;48(6):1605-8. [Medline].

  14. Eachempati SR, Sebastian MW, Reed RL 2nd. Posttraumatic bilateral carotid artery and right vertebral artery dissections in a patient with fibromuscular dysplasia: case report and review of the literature. J Trauma. Feb 1998;44(2):406-9. [Medline].

  15. Rushton AR. The genetics of fibromuscular dysplasia. Arch Intern Med. Feb 1980;140(2):233-6. [Medline].

  16. Bigazzi R, Bianchi S, Quilici N, et al. Bilateral fibromuscular dysplasia in identical twins. Am J Kidney Dis. Dec 1998;32(6):E4. [Medline].

  17. Tromp G, Wu Y, Prockop DJ, et al. Sequencing of cDNA from 50 unrelated patients reveals that mutations inthe triple-helical domain of type III procollagen are an infrequent causeof aortic aneurysms. J Clin Invest. Jun 1993;91(6):2539-45. [Medline].

  18. McKusick VA. Heritable Disorders of Connective Tissue. 4th ed. St Louis: Mosby-Yearbook; 1972:382-6.

  19. Schievink WI, Meyer FB, Parisi JE, Wijdicks EF. Fibromuscular dysplasia of the internal carotid artery associated with alpha1-antitrypsin deficiency. Neurosurgery. Aug 1998;43(2):229-33; discussion 233-4. [Medline].

  20. de Bray JM, Marc G, Pautot V, Vielle B, Pasco A, Lhoste P. Fibromuscular dysplasia may herald symptomatic recurrence of cervical artery dissection. Cerebrovasc Dis. 2007;23(5-6):448-52. [Medline].

  21. Lee EK, Hecht ST, Lie JT. Multiple intracranial and systemic aneurysms associated withinfantile-onset arterial fibromuscular dysplasia. Neurology. Mar 1998;50(3):828-9. [Medline].

  22. Mettinger KL. Fibromuscular dysplasia and the brain. II. Current concept of the disease. Stroke. Jan-Feb 1982;13(1):53-8. [Medline].

  23. de Monyé C, Dippel DW, Dijkshoorn ML, Tanghe HL, van der Lugt A. MDCT detection of fibromuscular dysplasia of the internal carotid artery. AJR Am J Roentgenol. April 2007;188(4):W367-9. [Medline].

  24. Sabharwal R, Vladica P, Coleman P. Multidetector spiral CT renal angiography in the diagnosis of renal artery fibromuscular dysplasia. Eur J Radiol. March 2007;61(3):520-7. [Medline].

  25. Willoteaux S, Faivre-Pierret M, Moranne O, Lions C, Bruzzi J, Finot M, et al. Fibromuscular dysplasia of the main renal arteries: comparison of contrast-enhanced MR angiography with digital subtraction angiography. Radiology. December 2006;241(3):922-9. [Medline].

  26. Bragin MA, Cherkasov AP. [Morphogenesis of fibromuscular dysplasia of the renal arteries (anultrastructural study)]. Arkh Patol. 1979;41(2):46-52. [Medline].

  27. Harrison EG Jr, McCormack LJ. Pathologic classification of renal arterial disease in renovascular hypertension. Mayo Clin Proc. Mar 1971;46(3):161-7. [Medline].

  28. Begelman SM, Olin JW. Fibromuscular dysplasia. Curr Opin Rheumatol. Jan 2000;12(1):41-7. [Medline].

  29. Reiher L, Pfeiffer T, Sandmann W. Long-term results after surgical reconstruction for renal artery fibromuscular dysplasia. Eur J Vasc Endovasc Surg. Dec 2000;20(6):556-9. [Medline].

  30. Van Damme H, Sakalihasan N, Limet R. Fibromuscular dysplasia of the internal carotid artery. Personal experience with 13 cases and literature review. Acta Chir Belg. Aug 1999;99(4):163-8. [Medline].

  31. Chiche L, Bahnini A, Koskas F, Kieffer E. Occlusive fibromuscular disease of arteries supplying the brain: results of surgical treatment. Ann Vasc Surg. Sep 1997;11(5):496-504. [Medline].

  32. Collins GJ Jr, Rich NM, Clagett GP, et al. Fibromuscular dysplasia of the internal carotid arteries. Clinical experience and follow-up. Ann Surg. Jul 1981;194(1):89-96. [Medline].

  33. Finsterer J, Strassegger J, Haymerle A, Hagmüller G. Bilateral stenting of symptomatic and asymptomatic internal carotid artery stenosis due to fibromuscular dysplasia. J Neurol Neurosurg Psychiatry. Nov 2000;69(5):683-6. [Medline].

  34. Leadbetter WF, Burkland CD. Hypertension in unilateral renal disease. J Urol. 1938;39:611-26.

  35. Leary MC, Finley A, Caplan LR. Cerebrovascular Complications of Fibromuscular Dysplasia. Curr Treat Options Cardiovasc Med. Jun 2004;6(3):237-248. [Medline].

  36. Palubinskas AJ, Ripley HR. Fibromuscular hyperplasia in extrarenal arteries. Radiology. 1946;82:451-55.

  37. Vuong PN, Desoutter P, Mickley V. Fibromuscular dysplasia of the renal artery responsible for renovascular hypertension: a histological presentation based on a series of 102 patients. Vasa. Feb 2004;33(1):13-8. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

FMD, fibromuscular hyperplasia, medial hyperplasia, arterial fibrodysplasia, angiopathy, renal artery disease, stroke

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

James A Wilson, MD, MSc, FRCPC, BSc(H), Neurologist and Clinical Neurophysiologist, Oconee Neurology Services
James A Wilson, MD, MSc, FRCPC, BSc(H) is a member of the following medical societies: American Academy of Neurology and Ontario Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Richard L Hughes, MD, Associate Professor, Department of Neurology, University of Colorado School of Medicine; Director, University of Colorado Affiliated Hospitals Stroke Project; Chief, Division of Neurology, Denver Health Medical Center
Richard L Hughes, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Medical Association, and North American Neuro-Ophthalmology Society
Disclosure: Nothing to disclose.

Medical Editor

Jeffrey L Saver, MD, Director, Stroke Center, Professor, Department of Neurology, University of California at Los Angeles Medical Center
Jeffrey L Saver, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, American Neurological Association, and National Stroke Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Howard S Kirshner, MD, Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association
Disclosure: Boehringer Ingelheim Honoraria Speaking and teaching; BMS/Sanofi Honoraria Speaking and teaching; Pfizer Honoraria Speaking and teaching; Novartis Consulting fee Review panel membership

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Helmi L Lutsep, MD, Associate Professor, Department of Neurology, Oregon Health and Science University; Associate Director, Oregon Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology and American Stroke Association
Disclosure: Co-Axia Consulting fee Review panel membership; Talecris Consulting fee Review panel membership; AGA Medical Consulting fee Review panel membership; Boehringer Ingelheim Honoraria Speaking and teaching; Boston Scientific Honoraria Speaking and teaching

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.