Case Report Treatment of Barrow type ‘B’ carotid cavernous fistulas with flow diverter stent (Pipeline) The Neuroradiology Journal 0(00) 1–8 ! The Author(s) 2017 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/1971400917695319 journals.sagepub.com/home/neu Carlos Castaño1, Sebastián Remollo1, Rosa Garcı́a-Sort1, Carlos Domı́nguez2 and Mikel Terceño1 Abstract Background: Carotid cavernous fistulas (CCFs) Barrow type ‘B’ are dural shunts between the meningeal branches of the internal carotid artery and the cavernous sinus. The symptoms include vision deterioration, ophthalmoplegia with diplopia, exophthalmos, conjunctival injection, chemosis, ocular bruit, seizures, or neurological deficit. Endovascular treatment remains the gold standard for treatment through the transvenous or transarterial routes. The transvenous approaches have been proved to be the first option. Endovascular access through the superior ophthalmic vein (SOV) or inferior petrosal sinus have been widely used. The problem arises when there is no vascular access. For these cases, different approaches have been described, such as: direct access to the SOV; combining direct access to the SOV along with blind probing of the proximal occluded SOV; and a direct puncture of the cavernous sinus. But these techniques are very aggressive and can cause serious complications. As a result of the above, we describe a new alternative technique, which is effective and less invasive for the treatment of these special cases. Case reports: We report two cases of Barrow type ‘B’ CCFs that did not have vascular access (neither arterial nor venous) to embolise fistulas with coils or glue, and which were successfully resolved with a flow diverter (Pipeline) stent in the internal carotid artery. To our knowledge, this treatment has not previously been described for this pathology. Conclusions: The placement of a flow diverter stent in the internal carotid artery is an effective alternative technique in those cases of Barrow type ‘B’ CCFs that have no vascular access (neither venous nor arterial). Keywords Carotid cavernous fistula, flow diverter, Pipeline, interventional neuroradiology Introduction Carotid cavernous fistulas (CCFs) Barrow type ‘B’ are dural shunts between the meningeal branches of the internal carotid artery (ICA) and the cavernous sinus.1,2 The symptoms include vision deterioration, ophthalmoplegia with diplopia, exophthalmos, conjunctival injection, chemosis, ocular bruit, seizures, or neurological deficit.3 Endovascular treatment remains the gold standard. The treatment options are endovascular embolisation with coils, stents, or liquid embolic agents. The procedure can be performed from either an arterial or venous approach. Use of these materials and techniques can yield a high cure rate with minimal complications.4–7 In the modern era, the transvenous approaches have been proved to be the first option, and endovascular access through the superior ophthalmic vein (SOV) or inferior petrosal sinus (IPS) has been widely used.8 The problem arises when there is no vascular access. For these cases, different approaches to treat a symptomatic CCF have been described, such as: direct access to the SOV (the vein is surgically exposed and then catheterised);9 an approach of combining direct access to the SOV along with blind probing of the proximal occluded SOV;10 and a direct puncture of the cavernous sinus.11,12 But these techniques are very aggressive and can cause serious complications, such as perforation during advancement of the sharp microwire, intra or 1 Interventional Neuroradiology Unit, Hospital Universitario Germans Trias i Pujol, Spain 2 Neurosurgery Department, Hospital Universitario Germans Trias i Pujol, Spain Corresponding author: Carlos Castaño, Interventional Neuroradiology Unit, Department of Neurosciences, Hospital Universitario Germans Trias i Pujol, Universidad Autónoma de Barcelona, Ctra Canyet s/n, CP 08916 Badalona, Barcelona, Spain. Email: ccastanod@gmail.com 2 retro-orbital haematoma, optic nerve injury, ICA injury, or infections. As a result of the above, we describe a new alternative technique, which is effective and less invasive for the treatment of these special cases. Case reports Case 1 A 65-year-old woman, without toxic habits, allergies, or previous pathologies of interest. She was living a normal life (mRS of 0). In January 2015 she experienced binocular diplopia, to discover the reason she consulted another hospital, where she was studied (including cerebral magnetic resonance imaging) without any pathology being found. After 3 weeks the symptoms were self-limiting and she became asymptomatic. In April 2015, she experienced chemosis, conjunctival injection and mild exophthalmus in both eyes, so she consulted the ophthalmology department of our hospital, which diagnosed glaucoma and started treatment. In February 2016, due to poor response to medical treatment, she was referred to the neurosurgery and interventional neuroradiology departments, who suspected a CCF. Because the ophthalmological examination showed bilateral intraocular pressure (IOP) of 38 mmHg, it was decided to perform a four-vessel cerebral arteriography (Figure 1). On 12 February 2016, cerebral arteriography was performed, showing a left CCF (Barrow type ‘B’), which was fed by the meningohypophyseal trunk of the left ICA and drained in both SOVs, conditioning congestion of both orbits. It should be noted that IPSs, the facial veins and the anterior part of the right SOV were not observed to be present (Figure 2). Figure 1. Case 1: Note the chemosis, conjunctival injection and mild exophthalmos in both eyes. The Neuroradiology Journal 0(00) On 15 February 2016, endovascular treatment of CCF was attempted by the arterial route, without being able to catheterise the branch that fed the fistula securely (Figure 3). On 26 February 2016 the CCF was treated by placing a single flow diverter stent (Pipeline flex embolisation device with shield technology (Medtronic, Irvine, CA, USA)) in the left ICA, with a complete closure of the fistula without complications (Figure 4). Prior to stent placement, the patient was treated with aspirin 300 mg/day and clopidogrel 75 mg/day, and an aggregometry study was performed with a Multiplate analyser that showed correct activity of the two drugs. The patient had a very good evolution, with a complete recovery of all signs and symptoms of both eyes (Figure 5). On 1 June 2016 a control arteriography was performed, which showed a complete closure of the fistula with no signs of complications (Figure 6). Case 2 A 77-year-old woman with no known toxic habits or allergies, in medical treatment for hypertension, diabetes and dyslipidaemia. She was living a normal life (mRS of 0). At the end of August 2016, she began to have progressive alterations in the right eye, with conjunctival injection, chemosis and palpebral ptosis; her visual acuity subsequently decreased. She came to our hospital on 22 September 2016 where ophthalmoplegia, exophthalmus, conjunctival injection, chemosis, an IOP of 40 mm Hg and a visual acuity of 0.1, in the right eye, were observed (Figure 7). On 23 September 2016, a cerebral arteriography was performed, which showed the presence of a CCF that compromised the intercavernous sinus and the right cavernous sinus, receiving contributions from small branches of the left (contralateral) ICA, and which drained into the right SOV, which was dilated. It should be noted that the IPS, the facial veins and the anterior part of the right SOV were observed not to be present (Figure 8). On 29 September 2016, endovascular treatment was performed by placing a single flow diverter stent (Pipeline flex embolisation device with shield technology (Medtronic)) in the left ICA, covering the small arterial contributions of the fistula (Figure 9). Prior to stent placement, the patient was treated with aspirin 300 mg/day and clopidogrel 75 mg/day and an aggregometry study was performed with a Multiplate analyser that showed correct activity of the two drugs. The patient had a very good evolution, with complete recovery of all signs and symptoms of the right eye (Figure 10). Castaño et al. 3 Figure 2. Case 1: This arteriography (post-anterior projection (a and b), lateral projection (c and d)) show a left carotid cavernous fistula (Barrow type ‘B’). In the enlarged photos of the post-anterior (e) and oblique (f and g) projections, it is appreciated that it was filled by the meningohypophyseal trunk of the left internal carotid artery (f and g) and drained in both superior ophthalmic veins (e), conditioning congestion of both orbits. It should be noted that the inferior petrosal sinuses, the facial veins and the anterior part of the right superior ophthalmic vein are not observed. 4 The Neuroradiology Journal 0(00) Figure 3. Case 1: (a and b) Endovascular treatment of the carotid cavernous fistula was attempted by the arterial route, without being able to catheterise the branch feeder that fed it securely. Figure 4. Case 1: (a and b) The carotid cavernous fistula was treated by placing a single flow diverter stent (Pipeline flex embolisation device with shield technology (Medtronic)) in the left internal carotid artery, with a complete closure of the fistula without complications. Figure 5. Case 1: This photo shows the normal appearance of both eyes of the patient. On 17 October 2016, an angiographic study was performed that showed a complete closure of the fistula, with no signs of complications (Figure 11). Discussion Traditional therapies for the treatment of CCFs include manual compression of the common carotid artery and jugular vein, and external ocular compression of the angular vein with or without carotid compression. The rate of complete occlusion with this techniques is around 30% of cases.8,13 Endovascular treatment remains the gold standard through the transvenous or transarterial routes.4–8 In the past, the transarterial approach was commonly used; however, in patients with tortuous or arteriosclerotic arteries, reaching the fistula site may be challenging, and the transarterial approach may be accompanied by other complicating factors, including migration of the embolic agents to the cerebral vasculature4 and revascularisation of the fistula after initial success.9 A paper reported that all patients treated by the transarterial route remained symptomatic.14 The transvenous approaches have now been proved to be the first option for patients with CCFs.10 The transvenous approach has a low risk and 90% success rate in all CCF cases, with a higher clinical and anatomical cure rate.7,8 However, it is necessary to discriminate between clinical and angiographic success. In a recent study15 the authors described an angiographic success rate of 74% and clinical success of 91%, and in the references they used success rates were 71–89% and 77–96%, respectively. The transvenous approach via the IPS was the first established route and is still the most commonly used Castaño et al. 5 Figure 6. Case 1: (a–c) Control arteriography, at one month, shows a complete closure of the fistula without signs of complications. Figure 7. Case 2: (a and b) Note the ophthalmoplegia, exophthalmus, chemosis and conjunctival injection in the right eye. Figure 8. Case 2: A cerebral arteriography (post-anterior projection (a and b), lateral projection (c)) show the presence of a carotid cavernous fistula that compromised the intercavernous sinus and the right cavernous sinus, receiving contributions from small branches of the left (contralateral) internal carotid artery ((f) oblique projection), and which drains into the right superior ophthalmic vein, which is dilated (d and e). It should be noted that the inferior petrosal sinuses, the facial veins and the anterior part of the right superior ophthalmic vein are not observed. 6 The Neuroradiology Journal 0(00) Figure 9. Case 2: (a–d) Endovascular treatment was performed by placing a single flow diverter stent (Pipeline flex embolisation device with shield technology (Medtronic)) in the left internal carotid artery, covering the small arterial feeders of the fistula, with a complete closure of the fistula without complications. Figure 10. Case 2: This photo shows the normal appearance of the patient’s right eye. method,16,17 even when this sinus is partially or completely thrombosed.16 When the IPS cannot be visualised filling early through the fistula, or in the delayed venous phase, blind cannulation of the IPS has been used.18 In about 31% of patients, the IPS cannot be catheterised due to plexiform architecture.18 When access to the IPS is restricted due to thrombosis or no communication between the IPS and fistula site, other routes may be considered; an anterior approach via a facial vein and SOV, or via the SOV, which was surgically exposed, may also be used.9,19–23 An approach of combining direct access to the SOV along with blind probing of the proximal occluded SOV has also been described.10 The potential complications of blind catheterisation of a thrombosed vein include perforation during advancement of the sharp microwire.10 The superior petrosal sinus and pterygoid plexus may serve as alternative routes when it receives significant venous drainage from CCFs.24,25 The direct exposure of the SOV approach (surgical exposure with anterior orbitotomy) and retrograde catheterisation is used if the facial vein cannot be visualised or is seen to be too tortuous, and if the SOV was sufficiently dilated and if there was significant venous drainage to the SOV. If the SOV is not dilated or if it is located deep in the orbit, transorbital venous access may not be possible.26,27 A direct puncture of the cavernous sinus has also been described.11,12 The potential complications of surgical exposure and direct puncture of the cavernous sinus include intra or retro-orbital haematoma, optic nerve injury, ICA injury, or infections. As a result of the risks and possible complications of the treatments of patients with Barrow type ‘B’ CCFs with inaccessible vascular access to the cavernous sinus, we present another new treatment, which is the placement of a flow diverter stent in the ICA, as we show in these two cases. Castaño et al. 7 Figure 11. Case 2: (a–c) Control arteriography, at one month, shows a complete closure of the fistula without signs of complications. This treatment has previously been published for the treatment of direct CCFs (Barrow type ‘A’),28–30 but has not been described for the treatment of Barrow type ‘B’ CCFs, and involves the rates of complications with the use of flow diverters in other contexts such as the treatment of aneurysms. Although flow diverter stents are designed to preserve the permeability of arteries originating in the wall covered by the stent, the complications described include side branch and perforator vessel occlusion.31 In this new situation of closing the ‘B’ type Barrow CCFs, we intend to close these side branches and perforator vessels with the stent. We believe that in order to improve the effectiveness of this treatment, a few key technical aspects must be taken into account, such as adjusting the stent diameter to the artery diameter as much as possible, choosing a stent whose diameter is the same as the artery or discretely inferior, which would allow us to compress the mesh of the stent, and compressing the stent mesh as much as possible in all sites where the origins of fistula feeders are located, with the aim of closing the mesh of the stent as much as possible, so that it acts as a covered stent. Another aspect to consider is the fact that Barrow ‘B’ type fistulas are low flow fistulas, which facilitates the closure of side branches and perforators. This therapy should be used in very well selected cases, and more studies are required in order to gain more experience. Conclusions The placement of a flow diverter stent in the ICA is an effective alternative technique in those cases of Barrow type ‘B’ CCFs that have no vascular access (neither venous nor arterial). Acknowledgements The authors acknowledge the work and cooperation of vascular neurologists, residents, anaesthetists and neurointerventional nurses in the management of these patients. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Conflict of interest The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. All the co-authors have reviewed and approved the final version of the manuscript. All co-authors had full access to all data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. References 1. Barrow DL, Spector RH, Braun IF, et al. 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