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Iliac Artery Fibromuscular Dysplasia Treated With Stent Deployment
Abstract
Fibromuscular dysplasia (FMD) is defined as non-inflammatory, non-atherosclerotic, degenerative vascular disease that may clinically remain asymptomatic or may have serious vascular consequences such arterial stenosis, aneurysmal rupture, or dissection, if left untreated. The exact etiology and prevalence of FMD remain uncertain, although any vascular bed can be affected by FMD with a higher predilection for renal and carotid arteries. Iliac artery involvement is rare and seen only in <10% of patients with diagnosis of FMD. Antiplatelet therapy is the suggested choice for medical management, and vascular intervention and surgery are reserved for symptomatic cases, which may manifest with complications. Balloon angioplasty has been solely recommended in treatment of stenotic arterial segments; however, a clear indication for stent placement remains uncertain. Our report describes arterial recoiling as an indication for stent placement in a case of symptomatic FMD affecting the right external iliac artery.
VASCULAR DISEASE MANAGEMENT 2019;16(4):E47-E51.
Key words: fibromuscular dysplasia, stenosis, external iliac artery, stent
Fibromuscular dysplasia is defined as non-inflammatory, non-atherosclerotic, degenerative vascular disease pointing to an anomaly with regard to the development of the arteries, and more rarely of the veins. It is characterized by severe hyperplasia of fibrous or muscular parietal components, which includes the structure of the vessels, causing focal and irregular stricture of the lumen and formation of aneurysms. It was initially described as fibromuscular hyperplasia in patients with renal stenosis; however, subsequent pathological classifications used the term fibromuscular dysplasia (FMD).1,2 Although prevalence of FMD is more than previously estimated,3 little progress has been made in understanding the epidemiology, pathogenesis, and natural history of disease since its first description in 1938 by Leadbetter and Burkland.4
The multifactorial etiology for FMD is suspected to affect connective tissue, resulting in pathological arterial wall weakness and formation of intraluminal webs causing stenosis, beading, and vessel dilation.5 FMD is mostly seen in young, hypertensive women and commonly affects renal, extracranial carotid, vertebral arteries, and carotid arteries.6,7 Although reports may vary about the most involved anatomic site, it is reported that renal arteries can be affected in up to 75% of cases, followed by the carotid arteries, in up to 20% to 70% of cases.8
Reported FMD of the iliac artery is rare and is usually diagnosed when the patient undergoes investigation for another reason, mainly renovascular hypertension.9 An incidence of 5% has been reported in patients with existing renal and or carotid artery FMD. 2 Wylie and colleagues reviewed the arteriograms of 70 patients with renal FMD and found that 4 (5.7%) demonstrated involvement of the external iliac arteries.10 Symptoms associated with iliac FMD may occasionally result in claudication and far less often in critical limb ischemia and distal embolism and dissection.11
Diagnostic advancement in vascular ultrasonography and the recent generation of noninvasive imaging such as magnetic resonance angiography (MRA) or multislice computed tomography angiography (MSCT) has eliminated the initial need for diagnostic conventional angiography in the FMD population. Yet early diagnosis seems to be crucial, considering the significant clinical impact of FMD, especially its impact on the cerebrovascular system, which can lead to unwanted outcomes such as ischemic or hemorrhagic stroke. 12
Case Report
A 50-year-old athletic woman presented with complaints of intermittent claudication and discomfort in her right thigh while jogging. She was initially treated by her provider with non-steroidal anti-inflammatory drugs (NSAID) without resolution of her symptoms.
Her medical history was remarkable for colonic polyp removal and cholecystectomy. Upon presentation, her blood pressure was 154/84 mm Hg, and there were no pertinent findings from a cardiopulmonary examination. Ankle pulses were symmetrical and palpable at rest.
A Pulse Volume Recording (PVR) test revealed resting ankle-brachial indices (ABIs) of 1.10 on the right and 1.08 on the left lower leg with a post-exercise reduction in the right ABI to 0.2 (Figure 1). Arterial ultrasonography revealed a peak systolic velocity (PSV) of 89 cm/s in the right and 110 cm/s in the left external iliac artery without abnormal alteration in Doppler waveforms. A significant drop was noted in right ankle pressure during walking exercise (Figure 2).
CT angiography of the aorta and extremity with runoff showed mild irregularity in the right iliac system without significant stenosis in iliac or lower extremity arteries (Figure 3).
The patient was taken for selective abdominal aortography with bilateral runoff. After establishing access, a catheter was advanced to the distal superficial femoral artery and a significant pressure gradient (100 mm Hg) at the right external iliac artery was detected during pullback maneuver (Figure 4). A focused angiography revealed a focal high grade stenosis of 90% inside a vessel segment with a beaded appearance. A percutaneous transluminal angioplasty (PTA) was then performed on the right external iliac artery lesion with several inflations of a 7 mm × 2 mm balloon. Despite multiple inflations, there was consistent and substantial vessel recoiling. A 7 mm × 4 mmself-expanding nitinol S.M.A.R.T. stent (Cordis) was deployed in the area and then post-dilated with the 7 mm × 2 mm balloon in 2 inflations. There was an excellent angiographic result demonstrating complete resolution of obstructive disease (Figure 5).
The patient tolerated the procedure well and was continued on antiplatelet therapy. There was a significant improvement in the patient’s claudication symptoms and normalization of ankle pressures after exercise (Figure 6A-B). Non-invasive vascular study revealed significant improvement in post-exercise ABI value, improving from 0.21 to 1.08 after angioplasty and stent deployment.
DISCUSSION AND LITERATURE REVIEW
The treatment of asymptomatic FMD consists of antiplatelet therapy, especially in the case of cerebrovascular FMD,1 percutaneous transluminal angioplasty with or without stenting, and open vascular surgery. Early surgical intervention by performing peripheral bypass with a prosthetic graft or autogenous vein is recommended in patients with more profound complications such as artery dissection, stenosis, or rupture due to dissection.13 Vascular surgery is invasive, with morbidity and mortality risks, and is reserved as the last treatment option.14
A successful procedure should ideally establish flow to the distal vessel segment by eliminating the lumen stenosis, resulting in clinical and imaging improvement of the stenotic area and normalization of the pressure gradient by selective angiography. Angioplasty is reported to be solely effective in most obstructive FMD lesions, and has been shown to normalize the aortoiliac pressure gradient and the intramural appearance on intravascular ultrasound (IVUS), yielding remarkable results. Stenting is usually recommended to disrupt intraluminal webs when they exist,1, 5, 8 or after failed balloon angioplasty with flow-limiting dissection.1,14-17
Our report describes a patient with symptomatic, isolated FMD in the right external iliac artery who underwent a stent placement after multiple, unsuccessful angioplasty attempts due to recoiling of the vessel segment.
Several definitions for elastic recoil have been described in the literature. It has been delineated as the differences between the maximal diameter of the inflated balloon and the minimal luminal diameter of the dilated segment soon after the angioplasty dilatation20 and quantitatively has been defined as nearly 50% loss of achievable cross-sectional area after balloon angioplasty, because of the elastic property of the artery.19,20
Table 1 provides limited list of published articles on selective cases of FMD treated with stent placement. There are significant dissimilarities in diagnostic method, indication for stent deployment, means, and duration of follow-up in the intervened arterial segment.
Conclusion
We present a multimodality approach for the diagnosis and endovascular treatment of a rare case of external iliac artery FMD with a successful stent placement after arterial recoil, despite significant shortcomings in the current medical literature on indication and evaluation methods for patency of treated arterial segments with stenting. Thus, we believe arterial recoil may be considered as an indication for the use of stenting in the FMD population beside previously described indications.
As we seek to better understand intervention strategies in FMD, generating further evidence-based knowledge through a multicenter registry would help elucidate optimal methods of treatment and intervention.
Disclosure: The authors report no financial relationships or conflicts of interest regarding the content herein.The study was supported by research fund from Vascular Medicine Department at Cleveland Clinic Florida.
Manuscript submitted February 27, 2019; accepted on March 8, 2019.
Address for correspondence: Cleveland Clinic Florida, Heart and Vascular Institute, Department of Vascular Medicine, 2950 Cleveland Clinic Boulevard Weston FL 33331. Email: carabal2@ccf.org
REFERENCES
1. Olin JW, Sealove BA. Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg. 2011;53(3):826-836.e1.
2. Zurin AA, Houkin K, Asano T, Ishikawa T, Abe H. Childhood ischemic stroke caused by fibromuscular dysplasia of the intracranial artery: case report. Neurol Med Chir (Tokyo). 1997;37(7): 542-545.
3. O'Connor SC, Gornik HG. Recent developments in the understanding and management of fibromuscular dysplasia. J Am Heart Assoc. 2014;3(6):e001259.
4. Leadbetter WF, Burkland CE. Hypertension in unilateral renal disease. J Urol.1938;5. 611-626.
5. Harrison Jr, E. G. 1971. Pathologic classification of renal arterial disease in renovascular hypertension. Mayo Clin Proc. 1971;46(3):161-167.
6. Touzé E, Oppenheim C, Trystram D, et al. Fibromuscular dysplasia of cervical and intracranial arteries.Int J Stroke. 2010;5(4):296-305.
7. Van den Driessche A, Van Hul E, Ichiche M, Verpooten GA, Bosmans JL. Fibromuscular dysplasia presenting as a renal infarction: a case report. J Med Case Rep. 2010;4:199.
8. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med. 2004;350(18):1862-1871.
9. Fujihara M, Akihiro H, Yoshiaki Y. Iliac artery fibromuscular dysplasia successfully treated by balloon angioplasty guided by intravascular ultrasound and pressure wire measurements: a case report. J Cardiol Cases. 2014;9(5):203-207.
10. Wylie EJ, Binkley FM, Palubinskas AJ. Extrarenal fibromuscular hyperplasia. Am J Surg.112(2):149-155.
11. Sauer Loie, Reilly LM, Goldstone J, Ehrenfeld WK, Hutton JE, Stoney RJ. Clinical spectrum of symptomatic external iliac fibromuscular dysplasia. J Vasc Surg. 1990;12(4):488-496.
12. Rinaldi I, Harris WO Jr, Koop KE, Legier J. Intracranial fibromuscular dysplasia: report of two cases, one with autopsy verification. Stroke. 1976;7(5):511-516.
13. Wychulis AR, Kincaid OW, Wallace RB. Primary dissecting aneurysms of peripheral arteries. Mayo Clin Proc. 1969;44(11):804-810.
14. Soga, Y, Lida O, Kawasaki D, et al. Contemporary outcomes after endovascular treatment for aorto-iliac artery disease. Circulation J. 2012;76(11): 2697-2704.
14. Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation. 2012; 125(25):3182-3190.
15. Savard S, Steichen O, Azarine A, Azizi M, Jeunemaitre X, Plouin PF. Association between 2 angiographic subtypes of renal artery fibromuscular dysplasia and clinical characteristics. Circulation. 2012;126(25):3062-3069.
16. Ketha SS, Bjarnason H, Oderich GS, Misra S. Clinical features and endovascular management of iliac artery fibromuscular dysplasia. J Vasc Inter Radiol. 2014;25(6):949-953.
17. Honjo O, Yamada Y, Kuroko Y, Kushida Y, Une D, Hioki K. Spontaneous dissection and rupture of common iliac artery in a patient with fibromuscular dysplasia: a case report and review of the literature on iliac artery dissections secondary to fibromuscular dysplasia. J Vasc Surg. 2004;40(5):1032-1036.
19. Waller BF. The eccentric coronary atherosclerotic plaque: morphologic observations and clinical relevance. Clin Cardiol. 1989;12(1):14-20.
20. Rensing BJ, Hermans WR, Strauss BH, Serruys PW. Regional differences in elastic recoil after percutaneous transluminal coronary angioplasty; a quantitative angiographic study. J Am Coll Cardiol. 1991;17(6 Suppl B):34B-38B.
21. Wang L, Scott DJ, Clemens MS, Hislop SJ, Arthurs ZM. Mechanism of stent failure in a patient with fibromuscular dysplasia following renal artery stenting. Ann Vasc Surg. 2015;29(1):123-e19-e21.
22. Edem E, Aksoy MN, Pabuccu MT, Tatli E. Endovascular treatment of renal artery stenosis due to fibromuscular dysplasia-Is stent implantation underused in this circumstance. Heart Views. 2016;17(2):69-71.
23. Poulter R, Ricci D, Saw J. Perforation during stenting of a coronary artery with morphologic changes of fibromuscular dysplasia: an unrecognized risk with percutaneous intervention. Can J Cardiol. 2013;29(4): 519.e1-e3.
24. Liu F, Xu J, Li Y, Wang B. Renal fibromuscular dysplasia with malignant hypertension cured by balloon angioplasty with stenting. Blood Press. 2014;23(6):381-383.
25. Portugaller R, Mache C, Tiesenhausen K, Hebel P, Deutschsmann H. Fibromuscular dysplasia–related aneurysms: one-year follow- up after treatment with a flow-diverting stent. J Vasc Interv Radiol. 2013;24(6):915-918.
26. Cohen JE, Itshayek E, Keigler G, Eichel R, Leker RR. Endovascular thrombectomy and stenting in the management of carotid fibromuscular dysplasia presenting with major ischemic stroke. J Clin Neurosci. 2014;21(11): 2021-2023.
27. Buso R, Rattazzi M, Leoni M, Puato M, Paola FD, Pauletto P. An unusual case of fibromuscular dysplasia with bilateral renal macroaneurysms: three-year outcome after endovascular treatment. Open Cardiovasc Med J. 2013;7:50-53.
28. Raju MG, Bajzer CT, Clair DG, Kim ES, Gornik HL. Renal artery stent fracture in patients with fibromuscular dysplasia: a cautionary tale. Circ Cardiovasc Interv. 2013;6(3):e30-e31.
