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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 1  |  Page : 119-121

Endovascular management of carotid-cavernous fistula in Ehlers-Danlos syndrome Type IV

1 Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Medical Genetics, Christian Medical College, Vellore, Tamil Nadu, India
3 Department of Ophthalmology, Christian Medical College, Vellore, Tamil Nadu, India
4 Department of Neurosurgery Unit 1, Christian Medical College, Vellore, Tamil Nadu, India

Date of Submission27-Oct-2011
Date of Decision22-Nov-2011
Date of Acceptance05-Dec-2011
Date of Web Publication7-Mar-2012

Correspondence Address:
Suraj Mammen
Department of Radiology, Christian Medical College, Vellore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.93616

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How to cite this article:
Mammen S, Keshava SN, Danda S, Raju R, Chacko AG. Endovascular management of carotid-cavernous fistula in Ehlers-Danlos syndrome Type IV. Neurol India 2012;60:119-21

How to cite this URL:
Mammen S, Keshava SN, Danda S, Raju R, Chacko AG. Endovascular management of carotid-cavernous fistula in Ehlers-Danlos syndrome Type IV. Neurol India [serial online] 2012 [cited 2022 Dec 10];60:119-21. Available from: https://www.neurologyindia.com/text.asp?2012/60/1/119/93616


A 27-year-old lady presented with progressive proptosis of right eye of three months duration. On examination, she was short-statured, had curly wispy hair, pinched nose, hyper-pigmented extremities, swollen joints without hypermobility, wrinkled, dry, thin skin without hyper-elasticity with visible underlying veins. She had proptosis of right eye with chemosis and normal visual acuity and visual fields. Both pupils were equal in size with no relative afferent pupillary defect. Fundoscopy revealed blurring of right disc margin. Movements of the right eye were minimally restricted in all directions. Magnetic resonance imaging of orbits and cerebral computed tomography-angiogram (CTA) revealed a dilated right superior ophthalmic vein (SOV) and dilated cavernous varix [Figure 1]. With a diagnosis of a caroticocavernous fistula (CCF) in a patient with  Ehlers-Danlos syndrome More Details (EDS), we planned a combined diagnostic angiogram and intervention to minimize vascular trauma. During vascular access, she had intimal dissection of right common femoral artery, which was managed conservatively. A right direct CCF (Type A) was confirmed [Figure 2] and [Figure 3]. She was heparinised with 5000 U of heparin intravenously. A 6F guiding catheter (Neuron catheter (Penumbra)) was placed and a 0.008-in microcatheter (MAGIC 1, 2 FM 180, BALT) was advanced across the defect in the internal carotid artery (ICA) and into the cavernous sinus. A detachable Goldbal balloon (9 mm × 14 mm, BALT) was used to occlude the fistula. A final check arteriogram showed non-opacification of the SOV, with minimal residual filling of the inferior petrosal sinus [Figure 4]. Post procedure patient remained on heparin (1000 U per h) for 12 h. She was kept in the intensive care unit (ICU) for 24 h. Her proptosis disappeared over a period of one week. She is completely asymptomatic during follow-up after nine months.
Figure 1: (a) Pre-embolization right eye proptosis with conjunctival chemosis and ectropion, (b) post procedure on one-year follow-up

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Figure 2: CT angiogram axial demonstrating dilation of the superior ophthalmic vein (arrow), dilated cavernous sinus and adjacent varix (arrowhead)

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Figure 3: Pre-embolization right internal carotid artery selective angiogram showed a direct (Type A) CCF (lateral projection), with a dilated superior ophthalmic vein (arrow), dilated cavernous sinus with adjacent varix (arrowhead) and drainage into the Internal jugular vein through the inferior petrosal sinus (curved arrow)

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Figure 4: (a, b) Post-embolization angiogram demonstrates the detachable balloon inflated with iso-osmolar non-ionic contrast and deployed at the site of the fistulous rend (arrowhead), lateral projection, and non-opacification of the superior ophthalmic vein and delayed opacification of the cavernous sinus and inferior petrosal sinus (arrow)

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In the 1960s, the vascular component of EDS was recognized, and EDS 4, the vascular type, was identified by Andras Barbaras. [1] This subcategory remained the most malignant form of EDS because of the high probability of developing spontaneous blood vessel rupture from aneurysms, dissections, and transmural tears. [1],[2] They account for less than 4% of all EDS patients. [3]

Our patient had a direct cavernous fistula, characterized by carotid arterial blood shunting directly into the venous cavernous sinus. The treatment options for direct CCF include transarterial coiling via the ICA, transvenous coiling through the superior or inferior petrosal sinuses or an ophthalmic approach via the ophthalmic vein. [4] Despite taking precautions with gentle catheter manipulation, our patient had a dissection of the femoral artery at the puncture site. Attempts at balloon embolization of CCFs by transarterial and transvenous routes have both been complicated by lethal hemorrhages. [5] Arteriography is indicated only if coil embolization is considered. [6],[7] We had excluded pre-existing arterial dissections in the abdominal aorta, iliac arteries by CTA and in the carotid arteries by ultrasonography. Thus diagnostic angiography and endovascular intervention through balloon occlusion of the CCF were performed together to minimize vascular trauma. We believe that the use of a glide diagnostic catheter, a high-profile soft guiding catheter like the Neuron catheter (Penumbra), as were used, could further minimize traumatic events during the procedure.

 » References Top

1.Horowitz MB, Purdy PD, James Valentine JR, Morrill K. Remote Vascular Catastrophes after Neurovascular Interventional Therapy for Type 4 Ehlers-Danlos Syndrome. AJNR Am J Neuroradiol 2000;21:974-6.  Back to cited text no. 1
2.Holodny AI, Deck M, Petito CK. Induction and Subsequent Rupture of Aneurysms of the Circle of Willis after Radiation Therapy in Ehlers-Danlos Syndrome: A Plausible Hypothesis. AJNR Am J Neuroradiol 1996;17:226-32.  Back to cited text no. 2
3.Zilocchi M, Macedo TA, Oderich GS, Vrtiska TJ, Biondetti PR, Stanson AW. Vascular Ehlers-Danlos Syndrome: Imaging Findings. AJR Am J Roentgenol 2007;189:712-9.  Back to cited text no. 3
4.Ruff IM, Strozyk D, Rahman C, Szeder V, Spellman JP, Marshall RS. Clinical Reasoning: A 21-year-old woman with right eye swelling and bruising. Neurology 2010;75;2039.  Back to cited text no. 4
5.Schievink WI, Limburg M, Oorthuys JW, Fleury P, Pope FM. Cerebrovascuiar Disease in Ehiers-Danlos Syndrome Type IV. Stroke 1990;21:626-32.  Back to cited text no. 5
6.Oderich GS, Panneton JM, Bower TC, Lindor NM, Cherry KJ, Noel AA, et al. The spectrum, management and clinical outcome of Ehlers-Danlos syndrome type IV: A 30-year experience. J Vasc Surg 2005;42:98-106.  Back to cited text no. 6
7.Slingenberg EJ. Complications during intravascular diagnostic manipulations in the Ehlers-Danlos syndrome. Neth J Surg 1980;32:56-8.  Back to cited text no. 7


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

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