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Table of Contents    
Year : 2020  |  Volume : 68  |  Issue : 5  |  Page : 1220-1223

Complications of Endovascular Treatment in Fibromuscular Dysplasia

1 Neurosurgeon and Interventional Neuroradiologist of Cajuru University Hospital of Pontifical Catholic University of Paraná (HUC-PUC PR), Curitiba – Parana, Brazil
2 Resident of Neurosurgery in HUC-PUC PR, Curitiba – Parana, Brazil
3 Head of Department of Interventional Neuroradiology of HUC-PUC PR, Curitiba – Parana, Brazil

Date of Web Publication27-Oct-2020

Correspondence Address:
Dr. Luana Antunes Maranha Gatto
Av. São Jose, 300, Cristo Rei. CEP - 80050-350, Curitiba – Parana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.299152

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 » Abstract 

Background: Fibromuscular dysplasia affects generally renal artery, causing renovascular hypertension. The most classical angiographic pattern, string-of-beads, can be found in cervical and more rarely in other arteries. With the advance of endovascular procedures techniques, the number of open surgeries is decreasing, and complications related to the selective catheterization of diseased vessels are increasing.
Case Report: A 37-year-old man presenting with subarachnoid hemorrhage was submitted to angioplasty for dissecting aneurysms of vertebral artery with a good outcome. Several arteries were angiographically diagnosed with the dysplasia (renal, carotid, femoral), and some complications like stenosis, dissection, arteriovenous fistula, and dissecting aneurysm occurred in sequence.
Conclusions: FMD of cervical arteries is usually asymptomatic. There are no guidelines or protocols to cervical FMD treatment, being indicated only for the complications. Because of the vessels fragility, a several spontaneous or post endovascular procedure complications can be disastrous.

Keywords: Angioplasty, arteriovenous fistula, carotid stenosis, dissecting aneurysm, dissection, endovascular procedure, fibromuscular dysplasia, iatrogenic disease, postoperative complications, renovascular hypertension
Key Message: The vascular fragility of fibromuscular dysplasia predisposes to ischemic and hemorrhagic complications secondary, mainly, to dissections, saccular and dissecting aneurysms. Endovascular treatment is restricted in its indication, since major complications can occur iatrogenically.

How to cite this article:
Maranha Gatto LA, Rodrigues Seabra Dd, Koppe GL, Demartini Z. Complications of Endovascular Treatment in Fibromuscular Dysplasia. Neurol India 2020;68:1220-3

How to cite this URL:
Maranha Gatto LA, Rodrigues Seabra Dd, Koppe GL, Demartini Z. Complications of Endovascular Treatment in Fibromuscular Dysplasia. Neurol India [serial online] 2020 [cited 2021 Jul 28];68:1220-3. Available from:

Fibromuscular dysplasia (FMD) is a group of idiopathic, non-atherosclerotic, and non-inflammatory diseases of the arterial walls that mainly affect women, leading to small and medium-sized artery stenosis.[1],[2]

FMD can occur in any artery, although it is more prevalent in the renal and cervical arteries.[3] The clinical presentation varies, depending on the location of the arterial lesions.[3] The most common manifestation is renovascular hypertension secondary to involvement of the renal artery. The FMD of the cervical artery, which mainly affects the internal carotid artery, is rarely symptomatic.[4] In addition, FMD is associated with a substantial risk of formation and rupture of aneurysms, as well as arterial dissection and occlusion,[3] causing a great diversity of signs and symptoms.

The gold standard for diagnosis is digital subtraction angiography (DSA), but this invasive procedure is recommended only for patients who require revascularization during the same procedure.[5]

 » Case Report Top

A 37-year-old male using Amlodipine due to high blood pressure (HBP) for 12 years, presented with sudden severe headache and vomiting. He was admitted 7 days after ictus in the Emergency Room. In his medical history, he denied head trauma, addictions, family history of cerebrovascular event and that there was nothing in history to suggest the central nervous system. Head computed tomography (CT) showed subarachnoid hemorrhage (SAH) in skull base cisterns and4t h ventricle (Fisher IV). Digital subtraction angiography (DSA) in another institution showed multiple tortuosities and ectasias in cervical vessels, and he was referred to our service. He was admitted with Glasgow Come Scale 15, mild stiff neck and no neurological deficit on physical examination (Hunt-Hess I/WFNS I). Magnetic resonance (MR) image rules out other abnormalities. A new DSA (the first one held in the other institution had poor quality) demonstrated two dissecting aneurysms in V3–V4 transition of left vertebral artery (VA), another one in right petrous carotid portion, and a “string-of-beads” pattern with stenosis followed by dilatations in both internal carotid arteries (ICA) (worst in the right side) and in both V2 segments. Similar findings in the right renal artery concluded the diagnosis of fibromuscular dysplasia. All intracranial vessels were normal. Shortly after this second DSA, there was a significant clinical worsening and the patient required intensive care and mechanical ventilation due to urinary focus sepsis, non-biliary pancreatitis, and acute renal failure (both contrast-related), bilateral hydronephrosis grade II and transient thrombocytopenia due to organ dysfunction. On admission, creatinine and urea were 0.9 and 43, respectively. After arteriography, they increased to 3.54 and 142. After clinical improvement without the need for dialysis, on the 37th-day post-ictus a transluminal percutaneous angioplasty (TPA) of dissecting aneurysms was performed without intercurrences [Figure 1]. All our procedures are made under full intravenous heparinization and general anesthesia, preferably via right femoral artery (FA). Two coronary stents were telescoped to cover the aneurysms. The patient was discharged with no neurological deficit, using acetylsalicylic acid (ASA) and Clopidogrel. A control DSA 8 months after showed exclusion of one left VA aneurysm and decrease of the other one, but worsening dilatations and dissecting aneurysm of the right petrous ICA. In addition, a giant dissecting aneurysm in the right subclavian artery was visualized, which did not exist in the first examinations. A puncture hematoma of the right FA following this control DSA was treated conservatively. TPA of right ICA was performed 1 month thereafter, in which was seen a volumous dilatation post dissection of right FA. Two long self-expanding stents covered the petrous aneurysm and other dilatations until the cervical segment, rectifying the diseased arterial segment, but causing a distal kinking with severe stenosis [Figure 2]. After 12 days, a new attempt to right ICA TPA via the left FA was performed unsuccessfully. The plan was to correct the kinking with a telescoped stent, but we could not overcome the stenosis with a new stent. The patient had a new inguinal hematoma, this time on the left side, also without need for local intervention. Both femoral arteries showed typical angiographic changes post complication of dissection [Figure 3]. Regarding the giant subclavian aneurysm, the Vascular Surgery team proposed cervico-thoracotomy and extracorporeal circulation. Faced with the risks, the patient preferred a new endovascular procedure. Two months after the attempted ICA TPA, we performed a TPA of the right subclavian artery via the ipsilateral brachial artery, with good aneurysm coverage by a self-expanding stent [Figure 4]. Ten days after, the patient was admitted to the Emergency Room for brachiobasilic arteriovenous fistula, at the site of the last puncture, which was surgically corrected by the Vascular Surgery team. Control cervical AngioCT 8 months later showed endoleak in the subclavian artery [Figure 5]. In the face of so many post-procedure complications, in addition to risks due to vascular fragility in both endovascular and open surgery interventions, and the patient being completely asymptomatic, we chose not to perform any treatment thereafter, unless he had a cerebrovascular event life-threatening. Follow-up AngioCT performed 5-months after showed no increase of the giant endoleak of right subclavian artery and exclusion of circulation of the aneurysms of left VA and right ICA. The patient keeps asymptomatic in outpatient follow-up, with double antiaggregation.
Figure 1: A and B = Axial T1 head CT with SAH Fisher IV. C (perfil view) and D (anteroposterior view) – Left vertebral artery DSA showing two dissecting aneurysms (arrows). E – TPA (1 open stent and 1 closed stent). F – Final control DSA

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Figure 2: DSA of right internal arotid artery showing dilatations and stenosis. A = Normal intracranial vessels. B = Anteroposterior view. C = Right anterior oblique view. D = Perfil view. E = TPA with two telescoped stents and balloon insuflation.

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Figure 3: Other vessels in DSA. A and B = right (affected) and left renal arteries. C, D and E = Left internal carotid artery. C = Normal intracranial vessels. D = Anteroposterior view. E = Perfil view. F and G = Common femoral arteries (right and left, respectively)

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Figure 4: DSA of right subclavian artery (rSA). A = Normal rSA and ectasic V1 segment of vertebral artery. B = Second DSA shows vertebral artery dissection. C = After 8 months, the rSA has a giant dissecting aneurysm. D = Brachial puncture for angioplasty. D = Final angiography shows good coverage of the aneurysm with two stents

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Figure 5: Angiotomography showing endoleak of the giant subclavian aneurysm on second day post-TPA

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 » Discussion Top

McCormack, in 1958, reported a pathological description of fibromuscular hyperplasia in four patients with renovascular hypertension.[6] In 1965, Hunt observed that the disease was heterogeneous and not necessarily associated with hyperplasia, and introduced the term FMD.[7]

Since the earliest studies, FMD has always been considered a more prevalent disease in females. Recent studies have further corroborated this assertion: Plouin and Kadian-Dodov, in 2016, with a prevalence of 84%[5] and 93.5%,[1] respectively; and Lather, in 2017, with 94.1%.[3]

Three main types of renal FMD were identified according to the arterial wall layer that is mainly affected: Intimal (about 10% of cases of renal artery FMD), medial (80–90%), and adventitia (<5%). However, these categories are not mutually exclusive, since the involvement of more than one layer in the same diseased artery is not uncommon.[8] Cervico-cephalic FMD is classified angiographically into four subtypes: Multifocal, unifocal, tubular, and atypical.[8] Multifocal, with a typical string-of-beads pattern, is the most common angiographic presentation and histologically corresponds to medial FMD.[5] Our patient fits in angiographic classification of multifocal FMD, but we don × t have the histopathological analysis, since no open surgery for the affected vessels was carried out.

FMD can affect several arterial sites, the most common being cervical and renal. There are several reports of involvement of the mesenteric, axillary, iliac, hepatic, intracranial and, in some cases, coronary arteries. In a case series review conducted by Persu, the prevalence of involvement of two or more arterial sites varied from 16 to 28%. For this reason, it is recommended the investigation of secondary lesions in patients with FMD, mainly in renal and cervical arteries.[8] The involvement of so many arteries affected by the FMD of our patient makes this a rare and unprecedented case.

The clinical presentation is variable, not only by the multiple sites of involvement, but also by the large number of complications. Kadian-Dodov carried out a study with 921 patients with FMD, which 384 of them (41.7%) had aneurysms (21.7% of cases) and arterial dissections (25.7%), in addition to 5.8% with simultaneous involvement.[1] In this same study, the most commonly identified aneurysm sites reflected the most frequently affected arterial beds by the FMD: The renal (34%) and extracranial carotid arteries (31%). Intracranial circulation was the third most common site, occurring in 21.5% of all patients with aneurysms and 4.7% of the 921 patients in the study.[1]

Our patient had no intracranial arteries affected by the disease, but the initial diagnosis was secondary to subarachnoid hemorrhage. Although the dissecting aneurysms of the lfVA were extracranial, just they could account for bleeding in the 4th ventricle and base cisterns. The diagnosis of renal FMD, however, could have been made up to 12 years earlier, if underwent to investigation of HBP in young.

Lather, in 2017, underwent an angiographic review of 669 women diagnosed with FMD in the USA, showing a 12.9% prevalence of intracranial aneurysms. There was no difference in the comparison between the different FMD sites: Renal involvement with prevalence of 11.9%, cervical with 13.7% and mixed with 13.2%.[3] If we compare with the prevalence in the general population, with rates of 3.2%,[9] it becomes clear the strong relation of the development of intracranial aneurysms with all forms of FMD.

Spontaneous cervical artery dissections are a common cause of stroke in young and middle-aged adults and are associated with FMD in about 15% of cases,[10] and the most frequent dissections are in the extracranial carotid (63.7%) and vertebral arteries (20.7%).[1]

The management of FMD patients with drug therapy is well established, either with antiplatelet aggregators or anticoagulants. Surgical/endovascular treatment is indicated in patients with symptomatic or critical FMD, and may be directed to complications. In renal FMD, options are available in the presence of renovascular hypertension, renal ischemic atrophy or presence of lesions in other sites. In cervical FMD, the indications are more individualized, because of the low possibility of disease progression and, generally, they are directed to the complications. Among the endovascular possibilities are the use of coils for aneurysms and TPA and stents for dissections or critical FMD.[11]

Finally, we recognize a structural flaw in this clinical case. Problems of the Brazilian public health system made the patient submit to more endovascular procedures than recommended. There is a limit of exams, procedures and materials per month and per hospitalization for each patient.

Because FMD is a disease of unknown cause, it remains a therapeutic challenge because it does not have specific treatment. Currently, the therapy is directed to complications: Aneurysms and arterial dissections. Although endovascular/surgical treatment is well established for symptomatic complications or those that cause a serious risk to the patient, more studies are needed regarding the treatment of asymptomatic lesions. The follow-up of these patients with DSA, Doppler or AngioCT/MR is indissoluble, due to the great possibility of progression of the disease and its complications.

 » Conclusions Top

The vascular fragility of patients with fibromuscular dysplasia of cervical arteries predisposes to dissection, stenosis and occlusion, in addition to being related to intracranial aneurysms. All these vascular malformations may present spontaneous complications, with cerebral hemorrhage or stroke, or secondary to iatrogenesis. Endovascular procedures should be well indicated, and reserved only for treatment of symptomatic lesions.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

Kadian-Dodov D, Gornik HL, Gu X, Froehlich J, Bacharach JM, Chi YW, et al. Dissection and aneurysm in patients with fibromuscular dysplasia. J Am Coll Cardiol 2016;68:176-85.  Back to cited text no. 1
Slovut D, Olin J. Fibromuscular dysplasia. N Engl J Med 2004;350:1862-71.  Back to cited text no. 2
Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie A, Gimenez-Roqueplo AP, Jeunemaitre X. Fibromuscular dysplasia. Orphanet J Rare Dis 2007;2:28.  Back to cited text no. 3
Plouin PF, Baguet JP, Thony F, Ormezzano O, Azarine A, Silhol F, et al. High prevalence of multiple arterial bed lesions in patients with fibromuscular dysplasia. Hypertension 2017;70:652-8.  Back to cited text no. 4
Lather DH, Gornik HL, Olin OW, Gu X, Heidt ST, Kim ESH, et al. Prevalence of intracranial aneurysm in women with fibromuscular dysplasia. JAMA Neurol 2017;74:1081-7.  Back to cited text no. 5
McCormack LJ, Hazard JB, Poutasse EF. Obstructive lesions of renal artery associated with remediable hypertension. Am J Pathol 1958;34:582.  Back to cited text no. 6
Hunt J, Harrison EG, Sheps SG, Bernatz PE, Davis GD. Hypertension caused by fibromuscular dyplasia of renal arteries. Postgrad Med 1965;38:53-63.  Back to cited text no. 7
Persu A, Touzé E, Mousseaux E, Barral X, Joffre F, Plouin PF. Diagnosis and management of fibromuscular dysplasia: An expert consensus. Eur J Clin Invest 2012;42:338-47.  Back to cited text no. 8
Vlak MHM, Algra A, Brandenburg R, Rinke IGJE. Prevalence of unruptured intracrania aneurysms, with emphasis on sex, age, comorbidity, country, and time period: A systematic review and meta-analysis. Lancet Neurol 2011;10:626-36.  Back to cited text no. 9
Schievink WI. Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med 2001;344:898-906.  Back to cited text no. 10
De Groote M, Van der Niepen P, Hemelsoet D, Callewaert B, Vermassen F, Billiouw JM, et al. Fibromuscular dysplasia – results of a multicentre study in Flanders. Vasa 2017;46:211-8.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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