Carotid-Cavernous Fistulas (CCF s): Imaging Features and Endovascular Treatment

Transcription

1 Carotid-Cavernous Fistulas (CCF s): Imaging Features and Endovascular Treatment Poster No.: C-2141 Congress: ECR 2014 Type: Educational Exhibit Authors: D. Rodriguez, L. Aja Rodriguez, P. Mora Montoya, L. VALOYES GUERRERO, M. Á. De Miquel Miquel, A. Camins ; El Vendrell/ ES, Barcelona/ES, L'Hospitalet de Llobregat/ES, L Hospitalet, 5 Es/ES, Hospitalet de Llobregat (Barcelona)/ES Keywords: Fistula, Technical aspects, Embolisation, Diagnostic procedure, MR, CT-Angiography, Catheter arteriography, Vascular, Interventional vascular, CNS DOI: /ecr2014/C-2141 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 21

2 Page 2 of 21

3 Learning objectives To describe the classification, imaging features (CT, MRI, DSA), etiology, clinical presentation and the approach to endovascular treatment of CCF s. Background Introduction: CCF is an abnormal communication between carotid arterial system (Internal Carotid Artery -ICA- or External Carotid Artery -ECA-) and cavernous sinus (CS) (Fig. 1). Increased pressure within the CS seems to be the mainstay of pathophysiology, although arterial steal and increased flow plays an important role. Classification and etiology: There are several classifications of CCF s, according to its etiology (traumatic or spontaneous), flow dynamics (high or low flow) and anatomy (direct or indirect). Barrow et al [1] made a classification based on their arterial supply (Fig. 2). Type A fistulas are direct connections between the intracavernous ICA and CS, usually associated with high flow rates, and are mostly secondary to trauma (facial and skull base fractures) [2]. Type B, C and D are low flow indirect fistulae: Type B fistulas have dural branches of ICA to CS, and are relatively uncommon. Type C fistulas have arterial supply from dural branches of ECA. Type D fistulas have dural ICA and ECA branches to the CS; is the most prevalent indirect fistula. Spontaneous CCF s may fall into any of the previous types. The most frequent location of direct CCF is at the proximal horizontal intracavernous segment of the ICA near the inferolateral trunk. Traumatic disruption of the vessel wall is the most common cause for direct CCF s (trauma, iatrogenic), although 20% of cases Page 3 of 21

4 are regarded as spontaneous (conditions that predisposes weakness of the ICA wall, e.g. Ehlers-Danlos syndrome) [3]. Indirect CCF s are usually spontaneous and occur frequently in post-menopausal women. Predisposing factors include hypertension, diabetes, pregnancy, collagen vascular disease, among others. Sometimes it can resolve spontaneously without treatment [4, 5]. Clinical Features: Clinical presentation is consequence of the elevated intracavernous pressure. Moreover, other factors like dominant pattern of venous drainage (Fig. 3), the size and location of CCF and the presence of collateral vessels (arterial or venous) are important in this setting. For example, principal drainage into superior and inferior ophthalmic veins present with orbital symptoms [4]. In direct, high-flow CCF s, symptoms appear suddenly. The Dandy s triad (pulsatile exophthalmos, bruit and chemosis) is not completely observed in all patients, and is accompanied by other clinical features (diplopia, pain, cephalic bruit, ophtalmoplegia, visual loss). Visual loss is one of the most feared complications and requires immediate treatment [6, 7]. Other less common clinical manifestations includes intracranial haemorrhage (secondary to drainage into sphenoparietal sinus and deep middle cerebral vein) and external haemorrhage (otorrhagia, epistaxis) [3, 8]. Regarding indirect CCF s, the onset of symptoms is not as sudden as direct CCF and progess insidiously, showing progressive glaucoma, proptosis or red eye. Differential diagnosis: Includes a wide spectrum of pathologies (Table 1), so patients may be carefully evaluated before the presence of vascular pathology is recognized. Vascular Marginal sinus fistulas Anomalous intracranial venous drainage CS thrombosis Inflamatory, allergic, infectious Page 4 of 21

5 Conjunctivitis Endocrine Thyroid ophtalmopathy Other Frontal sinus mucocele Osteoma Ocular neoplasms Hemangioma Fibrous dysplasia DIFFERENTIAL DIAGNOSIS OF CCF Table 1. Images for this section: Fig. 1: Carotid-cavernous fistula Page 5 of 21

6 Fig. 2: Barrow s Classification of CCF s. Page 6 of 21

7 Fig. 3: Anatomy of the venous vasculature of the skull base (superior view). 1. Superior ophthalmic vein; 2. Anterior intercavernous sinus; 3. Inferior ophthalmic vein; 4. Pterygoid plexus; 5. Middle meningeal vein; 6. Superior petrosal sinus; 7. Inferior petrosal sinus; 8. Basilar venous plexus; 9. Transverse sinus; 10. Posterior intercavernous sinus; 11. Cavernous sinus; 12. Sphenoparietal sinus. Page 7 of 21

8 Findings and procedure details CT and MRI are the preferred non-invasive imaging techniques in diagnostic approach of CCF. CT scan can depict proptosis, enlargement of the extraocular muscles, dilatation and tortuosity of ophthalmic veins (mainly superior ophthalmic vein -SOV-), and enlargement of the ipsilateral cavernous sinus, with bulging of its lateral wall. A noncontrast cranial CT scan must be done in order to search skull fractures or intracranial haemorrhages [3]. MR images show similar findings to those seen on CT; also depicts orbital edema, enlarged pituitary gland (due to venous congestion) and abnormal flow voids in CS that correspond to shunted blood. Furthermore, is useful in screening and follow-up. In the setting of a high-flow fistula and retrograde cortical venous reflux, MR or CT studies may reveal dilatation of leptomeningeal and cortical veins [7, 9, 10]. However, CT and MRI cannot exclude totally the diagnosis, and therefore cerebral angiography is required to confirm CT/MRI findings prior to treatment. Cerebral angiography is the gold standard for diagnosis, classification (depicting the anatomy and estimating shunt dynamics) and treatment in CCF [3, 7, 9, 10]. Digital substraction angiography (DSA) is used to obtain the following information: Size and location of the fistula. Characterize them as direct or indirect. To identify associated cavernous carotid aneurysms. Presence of complete or partial steal phenomena. Identification and confirmation of patency of outflow pathways of the CS. Assessment of cortical arterial circulation and collateral flow through circle of Willis. Identification of high-risk features (cortical venous drainage, pseudoaneurysm, CS varix). To depict venous drainage patterns, therapeutic route, associated vascular injuries and evaluation of carotid bifurcation before compression therapy. Endovascular treatment options In the last years, it has been developed several therapeutic options in management of CCF. The endovascular approach has become the first-line option in clinical emergencies, being used in symptomatic direct CCF s and indirect fistulas with progressive symptoms (visual loss, dangerous drainage pathways) [11]. The treatment is chosen according to the type and anatomy of the fistula, size of arterial defect and operator/institution preferences. Page 8 of 21

9 The goal of treatment in direct CCF is to occlude the tear betweenicaand CS, preserving the patency of ICA, whereas in indirect CCF the goal is to interrupt fistulous communications and reduce CS pressure [3]. Balloon Occlusion: This procedure requires that the CS must be large enough to put the balloon for embolization and the size of fistula must be smaller than the inflated balloon, but large enough to allow a deflated balloon. The balloon has the advantage of being able to be flow-directed through the fistula and CS, and must be inflated to a volume larger than the fistula orifice to prevent its retrograde migration into ICA [3]. Later, angiography is repeated to ensure closure of the fistula and patency of the ICA. Transarterial embolization: In the last years it has become the mainstay of treatment in high-flow direct CCF s [12, 13]. Also, it's an alternative when residual AV shunt remains in dural CCF. Embolization can be made with detachable platinum coils and liquid embolic agents (n-butyl cyanoacrylate, ethylene-vinyl alcohol copolymer); the first are preferred because of their reliable and controlled deployment into CS. Complications of this procedure includes thromboembolus and ICA dissection [7]. Covered stent graft placement: Can be useful to obliterate direct CCF s, while preservates ICA patency. Actually their use is limited. Transvenous embolization: Is the current method of choice in treatment of indirect CCF s. the goal of this technique is to catheterize the abnormal CS superselectively and occlude the fistula without rerouting venous drainage to cortical structures [7, 9, 10]. This can be made in several routes; the most used is through inferior petrosal sinus (IPS) [9, 10, 14]. There are other alternative routes, including facial vein and SOV, trans-contralateral CS, superficial middle cerebral vein and sphenoparietal sinus, pterygoid plexus and direct transorbital puncture of CS via the superior orbital fissure. Images for this section: Page 9 of 21

10 Fig. 4: 73 year-old female with an indirect type D CCF. Lateral digital substraction angiogram with right ICA (left) and right ECA (right) injection. Fistulous point located at left CS, with ICA supply by meningo-hipofisary trunks (red arrow) and ECA supply by middle meningeal artery (blue arrow)and clivus branches from ascendent pharyngeal artery. Venous drainage to superior ophtalmic vein (yellow arrow) and to inferior petrous sinus. Page 10 of 21

11 Fig. 5: Coronal (left) and lateral (right) digital substraction angiogram of the previous patient. Coil embolization of the fistula (red arrows) was performed, from the middle meningeal artery supply. Further, onyx was used to embolize from the middle meningeal artery to CS. Fig. 6: Coronal (left) and lateral (right) digital substraction angiogram of the previous patient. After embolization (red arrows), practical fistula occlusion is achieved. Ocular symptoms improved significantly. Fig. 7: 34 year-old male with a traumatic, direct, high-flow CCF (red arrow). Digital substraction angiogram with left internal carotid artery injection. Coronal (left) and lateral Page 11 of 21

12 (right) views. Venous drainage to superior ophtamlmic vein, left superficial sylvian vein, inferior petrosal sinus, left pterygoid plexus, and right CS. Fig. 8: Digital substraction angiogram of the previous patient. Coronal view. Right ICA injection during left ICA manual compression. Note the delayed opacification of right hemisphere. Page 12 of 21

13 Fig. 9: Digital substraction angiogram of the previous patient. Lateral view. Coil embolization by simultaneus arterial and venous approach was performed, without complete occlusion of the fistula (red arrow), but keeping permeability of the ICA. Few days later, because of symptoms persistance, complete occlusion of the ICA was performed. Page 13 of 21

14 Fig. 10: 64 year-old male with a infiltrative, destructive skull base lesion (mucormicosis) with a massive epistaxis. Contrast-enhanced CT images in axial and sagital plane depict direct CCF with contrast extravasation in arterial phase (A and C) and contrast accumulation in retarded phase (B). Also, note the bone erosion asdociated (yellow arrow). Carotid occlusion was chosen as the only possible treatment. Because of the emergency no carotid occlusion test was performed Page 14 of 21

15 Fig. 11: Digital substraction angiogram of the previous patient. Left common carotid artery injection at the end of the procedure. Coronal (A) and lateral (B) view. Complete occlusion of the ICA and filling of the distal ICA and its branches, including ophtalmic artery (red arrow) was observed. Non-substracted image (C) shows embolization material (coils -blue arrow- and Amplatzer device -yellow arrow- proximally). Page 15 of 21

16 Fig. 12: Bleeding stopped but patient blood pressure remained low next hours. 24 hour control TC showed extense left middle cerebral artery infarct Page 16 of 21

17 Page 17 of 21

18 Fig. 13: 78 year-old male with a indirect CCF. Axial (up) and coronal (down) CT images show bilateral engorgement of superior ophtalmic veins (red arrows). Page 18 of 21

19 Fig. 14: Coronal T2-weighted MR of the previous patient. Observe the flow void located at the left CS (red arrow), secondary to CCF. Fig. 15: Coronal (left) and lateral (right) digital substraction angiogram of the previous patient. Indirect type D CCF with ICA and ECA supply by sphenopalatine artery, middle meningeal artery and ascendent pharyngeal artery. Venous drainage to superior ophtalmic vein and to CS. Embolization with PVA particles of the ECA supply was performed successfully nd ocular symptoms improved. Page 19 of 21

20 Conclusion CCF is an uncommon pathology that must be suspected by the radiologist in setting of trauma or in the appropriate clinic context. Careful examination of the CT/MRI can yield an early diagnosis and prompt treatment. DSA remains the gold standard for diagnosis and treatment. Low-flow indirect fistulas (types B, C and D) can be conservatively managed and those with dangerous drainage patterns and high-flow direct (type A) fistulas require adequate treatment. With recent advances, endovascular therapy has become the most used therapy in management of CCF s, being effective and with good clinical outcomes in most of cases. Personal information Daniel Rodriguez Bejarano MD, Radiology Resident, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); daniel.rodriguez@bellvitgehospital.cat Lucia Aja Rodriguez MD, Department of Neuroradiology, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); lucia.aja@bellvitgehospital.cat Paloma Mora Montoya MD, Department of Neuroradiology, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); palomamora@bellvitgehospital.cat Lisbeth Valoyes Guerrero MD, Radiology Resident, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); lisbeth.valoyes@bellvitgehospital.cat Maria Angeles de Miquel Miquel MD, Department of Neuroradiology, Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); mademiquel@bellvitgehospital.cat Angels Camins Simon MD, Radiologist. Institut de Diagnostic per la imatge (IDI), Hospital Universitari de Bellvitge, L Hospitalet de Llobregat (Barcelona); acamins@bellvitgehospital.cat References Page 20 of 21

21 Barrow DL, Spector RH, Braun IF el al. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg.1985; 62(2): Liang W, Xiaofeng Y, Weiguo L, Wusi Q, Gang S, Xuesheng Z. Traumatic carotid cavernous fistula accompanying basilar skull fracture: a study on the incidence of traumatic carotid cavernous fistula in the patients with basilar skull fracture and the prognostic analysis about traumatic carotid cavernous fistula. J Trauma. 2007; 63(5): Korkmazer B, Kocak B, Tureci E, Islak C, Kocer N, Kizilkilic O. Endovascular treatment of carotid cavernous sinus fistula: A systematic review. World J Radiol 2013 April 28; 5(4): Kim MS, Han DH, Kwon OK, Oh CW, Han MH. Clinical characteristics of dural arteriovenous fistula. J Clin Neurosci 2002; 9: Sasaki H, Nukui H, Kaneko M, et al. Long-term observations in cases with spontaneous carotid-cavernous fistulas. Acta Neurochir (Wien) 1988; 90: Yoshida K, Melake M, Oishi H et al. Transvenous Embolization of Dural Carotid-Cavernous Fistulas: A Series of 44 Consecutive Patients. Am J Neuroradiol 2009; /ajnr.A1882. Ringer AJ, Salud L, Tomsick TA. Carotid cavernous fistulas: anatomy, classification, and treatment. Neurosurg Clin N Am 2005; 16: Halbach VV, Hieshima GB, Higashida RT, Reicher M. Carotid cavernous fistulae: indications for urgent treatment. AJR Am J Roentgenol 1987; 149: Tjoumakaris SI, Jabbour PM, Rosenwasser RH. Neuroendovascular management of carotid cavernous fistulae. Neurosurg Clin N Am 2009; 20: Gemmete JJ, Ansari SA, Gandhi DM. Endovascular techniques for treatment of carotid-cavernous fistula. J Neuroophthalmol 2009; 29: Kiyosue H, Hori Y, Okahara M et al. Treatment of Intracranial Dural Arteriovenous Fistulas: Current Strategies Based on Location and Hemodynamics, and Alternative Techniques of Transcatheter Embolization. Radiographics : Perase P. Morris, Balloon Reconstructive Technique for the Treatment of Carotid Cavernous Fistula. American Journal of Neuroradiology 1999; 20: Charbel Mounayer, Michel Piotin, Laurent Spelle and Jacques Moret, Superior Petrosal Sinus Catheterization for Transvenous Embolization of a Dural Carotid Cavernous Sinus Fistula American Journal of Neuroradiology 2002; 23: Meyers PM, Halbach VV, Dowd CF, Lempert TE, Malek AM, Phatouros CC, Lefler JE, Higashida RT. Dural carotid cavernous fistula: definitive endovascular management and long-term follow-up. Am J Ophthalmol 2002; 134: Page 21 of 21