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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 206-207

Carotid dissection in Marfan's syndrome

Department of Neurointervention, King Edward Memorial Hospital, Pune - 411 011, India

Date of Submission13-Mar-2013
Date of Decision13-Mar-2013
Date of Acceptance17-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Lakshmi Sudha Prasanna Karanam
Department of Neurointervention, King Edward Memorial Hospital, Pune - 411 011
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.111164

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How to cite this article:
Alurkar A, Karanam LP, Oak S, Sorte S. Carotid dissection in Marfan's syndrome. Neurol India 2013;61:206-7

How to cite this URL:
Alurkar A, Karanam LP, Oak S, Sorte S. Carotid dissection in Marfan's syndrome. Neurol India [serial online] 2013 [cited 2020 Dec 3];61:206-7. Available from:


Marfan's syndrome is an autosomal dominant disorder of the connective tissues affecting the cardiovascular, ocular, and skeletal systems. Defects in fibrillin in medium-sized and small arteries predispose to the formation of aneurysm and arterial dissection which can present with ischemic events. The extension of proximal aortic dissection into the brachiocephalic or spinal arteries is a potential mechanism of stroke. Isolated dissections are relatively uncommon. We report the case of a young male with Marfan's syndrome who presented with recurrent transient ischemic attacks due to isolated proximal and distal carotid dissection.

A 32-year-old male, a known case of Marfan's syndrome previously treated for aortic dissection and aortic regurgitation with aortic graft and aortic valve replacement, presented with right-sided neck pain and recurrent right-sided transient ischemic attacks of a duration of three weeks. Computed tomography (CT) of the brain did not reveal any acute infarcts. CT angiogram showed dissection in the innominate and right common carotid artery extending upto the bifurcation. He was kept on injection enoxaparin (0.6cc twice a day) and Ecosprin 150 mg/day for a period of one week, despite which he had recurrent transient ischemic attacks. Digital subtraction angiogram (DAS) was done which confirmed the CT-angiogram findings [Figure 1]a-c. A staged procedure was planned. In the first stage, a PRECISE (10 × 40 mm) stent (Cordis) was placed with a distal protection device (Spider ev3) across the proximal lesion involving the innominate and proximal right common carotid artery [Figure 1]d and e. The second procedure was done two weeks later. The distal common carotid artery lesion was first treated with a tapered stent (8 × 6 × 40 mm Protege ev3); later, another self-expanding stent (10 × 40 mm PRECISE Cordis) was placed in the mid common carotid overlapping the first stent [Figure 1]f. The Spider protection device (ev3) was placed in the internal carotid artery (ICA) during the placement of the stent. The procedure was uneventful and patient was discharged on Ecosprin 150 mg and warfarin. At a clinical follow-up after one month, his neurological examination was normal.
Figure 1: (a) Aortogram showing the patent graft placed earlier for dissection, (b) digital subtraction angiography showing the dissection in the innominate and the proximal right carotid artery with luminal narrowing, (c) dissection in the mid and distal common carotid artery extending upto the bifurcation with significant stenosis, (d) post stenting angiogram showing the patent precise stent in the innominate and proximal common carotid with good flow across the lesion, (e) angiogram showing good flow across the lesion in the patent stents in the proximal, mid, and distal common carotid arteries, (f) flouroscopy image showing three stents (as shown by the arrows) which are overlapped

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Spontaneous cerebral arterial dissections are relatively rare in Marfan's syndrome. Cystic medial necrosis with disruption of the elastic fibres is the most characteristic lesion in Marfan's [1] and predominantly found in the aortic annulus and proximal aorta. It is seen less extensively in peripheral vasculature. Carotid artery dissection is due to the extension of the aortic dissection. Isolated extracranial carotid dissection as in our present case is uncommon. [2],[3],[4],[5] Incomplete expression of the syndrome with pathological evidence of cystic medial necrosis associated with internal carotid dissection has been reported. [6] Our patient is unique because of the presence of isolated dissection in the right carotid and was treated successfully with endovascular stenting. To our best of knowledge, this is probably the first report of carotid dissection in Marfan's treated with carotid artery stenting.

  References Top

1.Trotter SE, Olsen EG. Marfan's disease and Erdheim's cystic medionecrosis: A study of their pathology. Eur Heart J 1991;12:83-7.  Back to cited text no. 1
2.Schievink WI, Michels VV, Piepgras DG. Neurovascular manifestations of heritable connective tissue disorders: A review. Stroke 1994;25:889-903.  Back to cited text no. 2
3.Schievink WI. Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med 2001;344:898-906.  Back to cited text no. 3
4.Youl BD, Coutellier A, Dubois B, Jeger JM, Bousser MG. Three cases of spontaneous extracranial vertebral artery dissection. Stroke 1990;21:618-25.  Back to cited text no. 4
5.Godfrey M. The Marfan syndrome. In: Beighton P, editor. McKusick's Heritable Disorders of Connective Tissue. 5 th ed. St Louis, Mo: CV Mosby Co; 1993. p. 51-135.  Back to cited text no. 5
6.Mokri B, Okazaki H. Cystic medial necrosis and internal carotid artery dissection in a Marfan sibling: Partial expression of Marfan syndrome. J Stroke Cerebrovasc Dis 1992;2:100-5.  Back to cited text no. 6


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