Fibromuscular Dysplasia in Children and Adolescents

Figure 1. Conventional angiography. Panel A: Tight narrowing with a small aneurysm of 2 mm at the trifurcation of the left renal artery. Post balloon dilatation dimensions are shown in Panel B.
Figure 2. Conventional angiography. Panel A. In children, bilateral renal artery FMD is as common as unilateral FMD. Panel B. FMD rarely affects the carotid vessels in children, but these should be screened by CTA/MRA or at the time of conventional angiog
Figure 3. 3D CT angiography. Panel A. Left renal artery shows beading typical of FMD (arrow). Panel B. Renal artery with a long segment of narrowing with aneurysmal dilatation at the major bifurcation (arrow).
Figure 4. Conventional angiography. Tight stenosis of the right renal artery with marked post stenotic dilatation. Right nephrectomy was required after failed backbench repair to the thin-walled aneurysm.
Author(s): 

Kevin E. C. Meyers, MD1,2 and Neha Sharma BA IV1,2;
The Children's Hospital of Philadelphia1 and the University of Pennsylvania
Philadelphia, Pennsylvania

While no prospective studies have evaluated the surgical treatment of renovascular FMD in children and young adults, it is reassuring to know that in selected patients less than 25 years of age, blood pressure is well-controlled and the majority of patients do not require anti-hypertensive medication post vascular repair. 26

The causes, natural history, management and long-term outcomes of FMD in children requires further research and evaluation.

The authors can be contacted at:
Kevin Meyers, MD
Pediatric Nephrologist

Assistant Professor of Pediatrics
Second Floor Main Building
The Children’s Hospital of Philadelphia Cnr
34th Street and Civic Center Boulevard
Philadelphia, PA 19104
Telephone: (215) 590-4155
Fax: (215) 590-3705
Email: [email protected]

Note: This article underwent double-blind peer review by members of the Cath Lab Digest Editorial Board.

References: 

1. Olin JW. Recognizing and managing Fibromuscular dysplasia. Cleveland Clinic Journal of Medicine 2007;74(4): 277-282.

2. Cluzel P, Raynaud A, Beyssen B, et al. Stenoses of renal branch arteries in fibromuscular dysplasia: results of percutaneous transluminal angioplasty. Radiology 1994; 193(1): 227-232.

3. Bofinger A, Hawley C, Fisher P, et al. Alpha-1-antitrypsin phenotypes in patients with renal arterial fibromuscular dysplasia. Journal of Human Hypertension 2000; 14(2): 91-94.

4. Rushton AR. The genetics of fibromuscular dysplasia. Arch Internal Medicine 1980;140(2): 233-236.

5. Safian RD, Textor SC. Renal-artery Stenosis. N Engl J Med 2001;344(6):431-442.

6. Bofinger A, Hawley C, Fisher P, et al. Increased severity of multifocal renal arterial fibromuscular dysplasia in smokers. Journal of Human Hypertension 1999; 13(8):517-520.

7. McDonnell NB, Yang J, Chen W, et al. A cohort of patients with generalized Fibromuscular Dysplasia and features of Ehlers-Danlos Syndrome: A new phenotype. Abstract. Presented at the American Society of Human Genetics Annual Meeting. October 2006.

8. Feig D, Croix B. Symptoms in hypertensive children before and after antihypertensive treatment. Abstract. Presented at the American Society of Nephrology annual meeting in Philadelphia, 2005.

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

10. Plouin PF, Perdu J, La Batide-Alanore A, et al. Fibromuscular dysplasia. Orphanet Journal of Rare Diseases 2007;2(1):1-28.

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

12. National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents. The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 2004;114(2):555-576.

13. McLaren CA, Roebuck DJ. Interventional radiology for renovascular hypertension in children. Technical Vascular Interventional Radiology 2003; 6(4):150-157.

14. Towbin RB, Pelchovitz DJ, Baskin KM, et al. Cutting balloon angioplasty in children with resistant renal artery stenosis. J Vasc Interv Radiol 2007;18(5):663-669.

15. Klow NE, Paulsen D, Vatne K, et al. Percutaneous transluminal renal artery angioplasty using the coaxial technique. Ten years of experience from 591 procedures in 419 patients. Acta Radiology 1998;39(6):594-603.

16. Hansen KJ, Starr SM, Sands RE, et al. Contemporary surgical management of renovascular disease. J Vasc Surg 1992; 16(3):319-331.

17. O’Neill JA Jr. Long-term outcome with surgical treatment of renovascular hypertension. J Pediatr Surg 1998;33(1): 106-111.

18. Robitaille P, Lord H, Dubois J, et al. A large unilateral renal artery aneurysm in a young child. Pediatric Radiology 2004;34(3):253-255.

19. GoncharenkoV, Gerlock AJ Jr., Shaff MI, et al. Progression of renal artery fibromuscular dysplasia in 42 patients as seen on angiography. Radiology 1981;139(1):45-51.

20. Schreiber MJ, Pohl MA, Novick AC. The natural history of atherosclerotic and fibrous renal artery disease. Urol Clin North Am 1984;11(3):383-392.

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

22. Davidson RA, Barri Y, Wilcox CS. Predictors of cure of hypertension in fibromuscular renovascular disease. American Journal of Kidney Disease 1996;28(3): 334-338.

23. Smith JM, Stablein DM, Munoz R, et al. Contributions of the Transplant Registry: The 2006 Annual Report of the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS). Pediatric Transplantation 2007;11(4):366-373.

24. Benavides CA, Csapo Z, Timmins K, et al. Fibromuscular dysplasia recurrence after kidney transplantation: case report. Clinical Nephrology 2006;66(1):67-70.

25. Sevastos J, Lonergan M. Fibromuscular hyperplasia as a cause of transplant renal artery stenosis. Nephrology (Carlton) 2003;8(2):98-100.

26. Chalmers RT, Dhadwal A, Deal JE, et al. The surgical management of renovascular hypertension in children and young adults. Eur J Vasc Endovasc Surg 2000;19(4): 400-405.


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