Indian Journal of Radiology Indian Journal of Radiology  

   Login   | Users online: 1263

Home Bookmark this page Print this page Email this page Small font sizeDefault font size Increase font size     


NEURORADIOLOGY Table of Contents   
Year : 2009  |  Volume : 19  |  Issue : 4  |  Page : 308-310
Case Report: Carotid-cavernous fistula due to aneurysmal rupture in a case of aortoaortitis with bilateral giant internal carotid artery aneurysms

Department of Neuroradiology, AIIMS, Ansari Nagar, New Delhi - 110 029, India

Click here for correspondence address and email

Date of Web Publication24-Oct-2009


Takayasu aortoarteritis (TA) rarely affects the nervous system, but when it does, it usually manifests as cerebral ischemia or stroke. These strokes have mainly been attributed to stenotic extracranial vessels. Stenoses of intracranial vessels, although rare in TA, can occur due to either embolization into the vessel or because of the vasculitic process itself. Intracranial aneurysms are very rare in patients with TA. Bilateral cavernous internal carotid artery (ICA) aneurysms are rarer. They have been reported following radiation therapy and in association with fibromuscular dysplasia and juvenile Paget disease. Bilateral mycotic intracavernous aneurysms also occur. Bilateral giant cavernous ICA aneurysms with carotid-cavernous fistula (CCF) consequent to rupture into the cavernous sinus in a case of TA are extremely unusual. We report a case that fulfilled both American College of Rheumatology and European League against Rheumatology criteria for TA. The patient had bilateral cavernous sinus giant aneurysms and CCF because the right-sided aneurysm had ruptured and was leaking into the cavernous sinus.

Keywords: Bilateral giant ICA aneurysms, carotid cavernous fistula, Takayasu arteritis

How to cite this article:
Sharma S, Kumar S, Nanda A, Moses E. Case Report: Carotid-cavernous fistula due to aneurysmal rupture in a case of aortoaortitis with bilateral giant internal carotid artery aneurysms. Indian J Radiol Imaging 2009;19:308-10

How to cite this URL:
Sharma S, Kumar S, Nanda A, Moses E. Case Report: Carotid-cavernous fistula due to aneurysmal rupture in a case of aortoaortitis with bilateral giant internal carotid artery aneurysms. Indian J Radiol Imaging [serial online] 2009 [cited 2020 Nov 24];19:308-10. Available from:

   Introduction Top

Takayasu aortoarteritis (TA) is a large-vessel disease that causes aortic stenosis and/or stenoses of its major branches. A relatively rare, aneurysmal form of the disease is also known to occur. [1],[2]

Carotid aneurysms are rare, but when they occur, they are predominantly extracranial. [1],[3] Intracranial aneurysms are very rare. They usually occur in the vertebrobasilar system, associated with ipsilateral carotid stenosis, suggesting that hemodynamic stress may be the cause. [4] Bilateral cavernous internal carotid artery (ICA) aneurysms leading to carotid-cavernous fistula (CCF) have never been described in TA, to the best of our knowledge.

We present a case of TA in which bilateral giant intracavernous ICA aneurysms were present with rupture of the right aneurysm, leading to CCF. Other vessels were also involved in the disease process: bilateral renal artery stenosis, right mid-subclavian artery stenosis, right vertebral artery origin stenosis, right common iliac artery occlusion, and arch of aorta calcification.

   Case Report Top

A 17-year-old girl presented with a sudden onset of proptosis, pain, and chemosis of both eyes, the right eye being more severely affected. She also had subjective and objective tinnitus involving both ears. On examination, she had an elevated blood pressure, asymmetry of pulses, and an elevated erythrocyte sedimentation rate (ESR). USG of the abdomen revealed bilateral small kidneys. A color Doppler study revealed bilateral renal artery stenoses. A CT scan of the brain revealed enlarged, bulging cavernous sinuses and a large superior ophthalmic vein on the right side [Figure 1]A, B. Subtracted CT angiography (CTA) [Figure 1]C, D and [Figure 2] was performed using a dual-source scanner (Definition, Siemens, Erlangen). Digital subtraction angiography (DSA) [Figure 3] and [Figure 4] showed bilateral giant cavernous ICA aneurysms. The right aneurysm [Figure 4] was shown to be leaking into the cavernous sinus, leading to early drainage through both superior ophthalmic veins and the petrous sinuses into the jugular veins. The patient's symptoms regressed over the next few days and spontaneous closure of the fistula was suspected. However, this could not be confirmed as the patient refused further investigations or treatment and was lost to follow-up.

   Discussion Top

According to the American College of Rheumatology (ACR), the presence of three of the following six features is diagnostic of TA [5] :

  1. Age of onset before 40 years
  2. Claudication
  3. Decreased brachial artery pulse
  4. Blood pressure difference of >10 mm Hg between the arms
  5. Bruit over the subclavian arteries or aorta
  6. Arteriogram abnormality

According to the more recent European League Against Rheumatism (EULAR)/Pediatric Rheumatology European Society consensus criteria, the diagnosis of TA in children requires the presence of angiographic abnormalities, on conventional, CT, or MRI angiography of the aorta or one of its major branches plus one or more of the following [6] :

  1. Claudication or decreased peripheral artery pulses
  2. Blood pressure difference >10 mm Hg between the arms
  3. Bruits over the aorta or its major branches
  4. Hypertension

On imaging, our patient had aortic arch calcification, calcification, and stenosis of the right subclavian artery also involving the vertebral artery origin, bilateral renal artery stenoses, and occlusion of the right common iliac artery, which was filling via collaterals. Clinically, she had asymmetry of pulses, hypertension, and an elevated ESR in addition to the cerebral symptoms. Thus, our patient fulfilled both ACR and EULAR criteria for the diagnosis of TA.

Cerebral ischemia or stroke is the usual central nervous system manifestation of TA, affecting about 20% of TA patients. [7],[8] These strokes have mainly been attributed to stenotic extracranial vessels, but have increasingly also been recognized to be due to involvement of the intracranial vessels. [9],[10]

Other intracranial manifestations of TA include the development of aneurysms. The development of cerebral aneurysms in patients with TA is often attributed to hemodynamic stress caused by increased flow in the affected arteries. [4] However, the intracranial arteries may be directly affected by TA and our case report shows a situation where the aneurysms symmetrically involved the cavernous carotid arteries. [9],[11] There are about 20 reports of intracranial aneurysms in TA, in the literature. [12] These aneurysms have been reported in the paraclinoid, anterior communicating artery, middle cerebral artery M1 segment, anterior cerebral artery A1 segment, and the vertebro-basilar regions. [4],[12] No case of intracavernous aneurysm has been reported in patients with TA till date. [4],[12]

   References Top

1.Regina G, Fullone M, Testini M. Aneurysms of the supra-aortic trunks in Takayasu's disease: Report of two cases. J Cardiovascular Surg 1998;39:757-60.  Back to cited text no. 1      
2.Sueyoshi E, Sakamoto I, Hayashi K. Aortic aneurysms in patients with Takayasu's arteritis: CT evaluation. AJR 2000;175:1727-33.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.Tabata M, Kitagawa T, Saito T, Uozaki H, Oshiro H, Miyata T, et al. Extracranial carotid aneurysm in Takayasu's arteritis. J Vasc Surg 2001;34:739-42.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]  
4.Kanda M, Shinoda S, Masuzawa T. Ruptured vertebral artery-posterior inferior cerebellar artery aneurysm associated with pulseless disease--case report. Neurol Med Chir (Tokyo) 2004;44:363-7.  Back to cited text no. 4      
5.Arsend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990;33:1129-34.  Back to cited text no. 5      
6.Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006;65:936-41.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
7.Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, et al. Takayasu arteritis. Ann Intern Med 1994;120:919-29.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]  
8.Takano K, Sadoshima S, Ibayashi S, Ichiya Y, Fujishima M. Altered cerebral hemodynamics and metabolism in Takayasu's arteritis with neurological deficits. Stroke 1993;24:1501-6.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Klos K, Flemming KD, Petty GW, Luthra HS. Takayasu's arteritis with arteriographic evidence of intracranial vessel involvement. Neurology 2003;60:1550-1.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Kumral E, Evyapan D, Aksu K, Keser G, Kabasakal Y, Balkir K Microembolus detection in patients with Takayasu's arteritis. Stroke 2002;33:712-6.  Back to cited text no. 10      
11.Molnar P, Hegedus K. Direct involvement of intracerebral arteries in Takayasu's arteritis. Acta Neuropathol (Berl) 1984;63:83-6.  Back to cited text no. 11      
12.Weiss PF, Corao DA, Pollock AV, Finkel TH, Smith SE. Takayasu arteritis presenting as cerebral aneurysms in an 18 month old: A case report. Pediatr Rheumatol 2008;6:4.  Back to cited text no. 12      

Correspondence Address:
Sandeep Sharma
Department of Neuroradiology, AIIMS, Ansari Nagar, New Delhi - 110 029
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.57216

Rights and Permissions


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


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  

    Case Report
    Article Figures

 Article Access Statistics
    PDF Downloaded620    
    Comments [Add]    

Recommend this journal