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http://dx.doi.org/10.1016/j.jocn.2014.04.013

Endovascular thrombectomy and stenting in the management of carotid
fibromuscular dysplasia presenting with major ischemic stroke
José E. Cohen a,b,⇑, Eyal Itshayek a, Galina Keigler c, Roni Eichel c, Ronen R. Leker c
a
b
c

Department of Neurosurgery, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem 91120, Israel
Department of Endovascular Neurosurgery and Interventional Neuroradiology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
Department of Neurology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

a r t i c l e

i n f o

Article history:
Received 24 July 2014
Accepted 28 July 2014

Keywords:
Carotid artery
Fibromuscular dysplasia
Ischemic stroke
Stent
Thrombectomy

a b s t r a c t
Fibromuscular dysplasia (FMD) affecting the internal carotid artery (ICA) is considered a benign condition; however, retinal and cerebral ischemic events may occur. We present a patient with carotid FMD
presenting with a major ischemic stroke due to major embolic occlusion of the ICA in conjunction with
a hemodynamic component caused by narrowing and beading of the lumen associated with FMD. The
patient was successfully treated with intracranial stent-assisted thrombectomy followed by cervical
ICA stenting that aimed to reconstruct and angioplasty the FMD-affected arterial segment. Recently,
stent-based thrombectomy has emerged as the most effective endovascular option for the rapid
revascularization of major intracranial occlusions; however, to our knowledge, its use in a rare case of
FMD-associated major stroke has not yet been reported.
Ó 2014 Elsevier Ltd. All rights reserved.

1. Introduction
Fibromuscular dysplasia (FMD) affecting the internal carotid
artery (ICA) is considered a benign condition, and is usually
detected incidentally [1,2]. However, it is an infrequent cause of
retinal and cerebral ischemic events and, in rare cases, of major
ischemic stroke. It most commonly occurs in middle-aged women
[1–4]. We present a patient with carotid FMD presenting with
a major ischemic stroke, successfully treated by intracranial
stent-assisted thrombectomy followed by cervical ICA stenting.
2. Case presentation
A 47-year-old woman with an unremarkable medical history
except for hypertension and chronic headaches was admitted
through the emergency room 3 hours after presenting with an episode of sudden onset speech alterations and right-side hemiparesis. Upon admission, gaze deviation, global aphasia and dense
right hemiparesis were evident (National Institutes of Health
Stroke Scale 24). Urgent noncontrast head CT scan ruled out intracranial hemorrhage but showed incipient signs of insular cortex

⇑ Corresponding author. Tel.: +972 2 677 7092; fax: +972 2 641 6281.
E-mail address: jcohenns@yahoo.com (J.E. Cohen).

grey-white matter effacement. CT angiogram confirmed occlusion
of the distal left ICA.
The patient was urgently brought to the neurointerventional
suite and general anesthesia was induced. Diagnostic angiogram
confirmed complete ‘‘T’’ occlusion of the left ICA and showed the
‘‘string of beads’’ pattern preceded by a tapered luminal stenosis
that is characteristic of FMD, but there were no signs of dissection
(Fig. 1).
The distal ICA was rapidly recanalized by way of stent-based
thrombectomy, as described in a previous technical note [5].
Briefly, an 8 French balloon-tipped guiding catheter was placed
at the proximal ICA. Coaxially, a 0.02 inch microcatheter was navigated across the occluded arterial segment and a self-expandable
microstent (Solitaire, Covidien/eV3, Plymouth, MN, USA) was
deployed across the embolus and retrieved under proximal occlusion and aspiration. After complete ICA recanalization was confirmed (Fig. 2), 300 mg of aspirin and 600 mg of clopidogrel were
administered per nasogastric tube and the ICA segment affected
by FMD was covered by a single carotid stent to achieve angiographic reconstruction of the artery (Fig. 3).
Coagulation tests performed at admission and cardiovascular
studies at admission and on follow-up were unremarkable. No cardioembolic sources were detected on prolonged Holter, echocardiographic and tomographic cardiovascular studies. The renal
arteries were not found to be affected by FMD. The patient was

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Case Reports / Journal of Clinical Neuroscience 21 (2014) 2021–2023

Fig. 1. Selective angiogram of the left common carotid artery showing complete ‘‘T’’
occlusion of the distal left internal carotid artery and demonstrating the characteristic ‘‘string of beads’’ pattern, preceded by a tapered luminal stenosis, associated
with fibromuscular dysplasia. There are no signs of an associated dissection.

continued on aspirin and clopidogrel therapy, and made a rapid
and uneventful neurological recovery. She was discharged home
after 7 days with only mild residual right arm paresis. At 90 days,
the patient achieved complete neurological recovery (modified
Rankin Scale score 0).

3. Discussion
FMD is a systemic arteriopathy with a predilection for the renal
arteries, followed by the cervical vessels. FMD of the extracranial
ICA is an infrequent and generally incidental finding that is usually
asymptomatic [1,2]. The prevalence of FMD is 0.5–0.7% by angiography and in the vast majority of patients it presents a benign natural history. It usually occurs in middle-aged women and is
secondary to medial fibrodysplasia or, less commonly, intimal
fibrodysplasia. On vascular studies in patients with FMD, the ICA
may be elongated or kinked, and may show the characteristic
‘‘string of beads’’ luminal narrowing, in some cases with associated
aneurysms. Antiplatelet agents represent the cornerstone of therapy in these patients. Rarely, ICA FMD can present with thromboembolic or hemodynamic ischemic events, spontaneous or
posttraumatic dissection, aneurysmal degeneration, or intracranial
hemorrhage [6]. Transient ischemic attack or stroke are uncommon, but may occur due to low flow or embolization of platelet
aggregates [3,4]. Major ischemic stroke may occur after a flowlimiting complicating dissection or luminal narrowing associated

Fig. 2. The distal internal carotid artery was rapidly recanalized with a standard
stent-based thrombectomy procedure. Immediately afterwards, angioplasty was
performed in the fibromuscular dysplasia segment before stent implantation.

with FMD, emboli secondary to luminal irregularities or complicating
dissection, or after a combination of these mechanisms.
The presented patient debuted with a major embolic occlusion
of the ICA in conjunction with a hemodynamic component caused
by narrowing and beading of the lumen associated with FMD. The
therapeutic strategy consisted of intracranial stent-assisted thrombectomy using a technique comparable to the approach we have
described in more typical embolic occlusions of the intracranial
circulation, followed by cervical ICA stenting that aimed to reconstruct and angioplasty the FMD-affected arterial segment. Percutaneous angioplasty has emerged as the preferred treatment for
symptomatic ischemic cerebrovascular FMD without intracranial
occlusions [7], but surgical and hybrid procedures are still practiced by different groups with acceptable results [8]. Recently,
stent-based thrombectomy has emerged as the most effective
endovascular option for the rapid revascularization of major intracranial occlusions [9]; however, to our knowledge, its use in a rare
case of FMD-associated major stroke has not yet been reported.
The endovascular repair sequence chosen for this patient (that
is, intracranial thrombectomy followed by ICA stenting) is analogous to the procedural sequence already proposed for the revascularization of tandem middle cerebral artery and ICA occlusions
[10]. In patients with tandem occlusions, extracranial stenting is
performed before intracranial stent-based thrombectomy, and we
recommend the use of closed-cells stents (such as Wallstent,
Boston Scientific, Marlborough, MA, USA). Alternatively, the
guiding catheter should be advanced beyond the implanted stent
to reduce the risk of trapping the thrombectomy device in the
implanted carotid stent.

Case Reports / Journal of Clinical Neuroscience 21 (2014) 2023–2025

2023

stroke using a two-step procedure, namely revascularization of
the intracranial occlusion with immediate subsequent correction
of the causative condition.
Conflicts of Interest/Disclosures
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
Acknowledgements
The authors wish to thank Shifra Fraifeld, a research associate
and senior medical writer in the Department of Neurosurgery at
the Hadassah-Hebrew University Medical Center, for her editorial
contribution during the preparation of the manuscript.
References

Fig. 3. Angiogram obtained immediately after carotid stent implantation shows
reconstruction of the fibromuscular dysplasia-affected internal carotid artery
segment.

The current patient contributes to the rapidly growing body of
evidence on the endovascular management of major ischemic

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http://dx.doi.org/10.1016/j.jocn.2014.07.008

Pediatric cerebral ganglioneuroblastoma
Sean P. Steenberge, Richard A. Prayson ⇑
Cleveland Clinic Lerner College of Medicine and Department of Pathology, L25 9500 Euclid Avenue, Cleveland, OH 44195, USA

a r t i c l e

i n f o

Article history:
Received 15 July 2014
Accepted 28 July 2014

Keywords:
Brain tumor
Ganglioneuroblastoma
Pediatric brain tumor
Primitive neuroectodermal tumor

a b s t r a c t
We describe a 4-year-old girl with acute onset headaches and transient blindness who underwent surgical intervention, chemotherapy, and radiotherapy for an intracranial mass. This mass was pathologically
confirmed as a primary intracranial ganglioneuroblastoma, a rare finding in the pediatric population. The
literature on pediatric primary intracranial ganglioneuroblastoma is reviewed.
Ó 2014 Elsevier Ltd. All rights reserved.

⇑ Corresponding author. Tel.: +1 216 444 8805; fax: +1 216 445 6967.
E-mail address: praysor@ccf.org (R.A. Prayson).