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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Interventional Case Record
Interventional Case Record
Contributed by Abhishek Bairy
Contributed by Abhishek Bairy
Transvenous embolization in a case of indirect carotid-cavernous fistula by anterior approach
Transvenous embolization in a case of indirect carotid-cavernous fistula by anterior approach
Introduction:
Introduction:
Carotid cavernous fistula (CCF), a special type of dural arterio-venous malformation, is an abnormal communication between the carotid arterial system and the cavernous sinus. CCF can be classified as direct or indirect fistula based on anatomical features, and traumatic or spontaneous on the basis of etiological features. The most common etiology of direct fistula is a head trauma, as a result of which the internal carotid artery is damaged and a fistula is formed between the lacerated artery and the cavernous sinus. CCF uncommonly occurs spontaneously as well. Patients of CCF usually present with chemosis, proptosis and ophthalmoplegia. The definitive treatment of CCF using endovascular techniques have surpassed the surgical approach to management. Transarterial is the most common approach to manage direct CCF, whereas indirect CCF can be embolized using transarterial or transvenous approach. We present a case of indirect CCF embolized using anterior transvenous approach in a 62 year old lady presenting with proptosis.
Carotid cavernous fistula (CCF), a special type of dural arterio-venous malformation, is an abnormal communication between the carotid arterial system and the cavernous sinus. CCF can be classified as direct or indirect fistula based on anatomical features, and traumatic or spontaneous on the basis of etiological features. The most common etiology of direct fistula is a head trauma, as a result of which the internal carotid artery is damaged and a fistula is formed between the lacerated artery and the cavernous sinus. CCF uncommonly occurs spontaneously as well. Patients of CCF usually present with chemosis, proptosis and ophthalmoplegia. The definitive treatment of CCF using endovascular techniques have surpassed the surgical approach to management. Transarterial is the most common approach to manage direct CCF, whereas indirect CCF can be embolized using transarterial or transvenous approach. We present a case of indirect CCF embolized using anterior transvenous approach in a 62 year old lady presenting with proptosis.
Case presentation:
Case presentation:
A 62 year old lady presented with 1redness of left eye, blurring of vision and headache since 4 months. She also had proptosis of the left eye (Figure 1).
A 62 year old lady presented with 1redness of left eye, blurring of vision and headache since 4 months. She also had proptosis of the left eye (Figure 1).
Fig. 1 Clinical photograph of the patient showing swollen, red left eye with proptosis
There was no history of trauma. She was a known diabetic since 15 years. She had Haemoglobin of 12.2gm%, Total leucocyte count of 10,300/cu.mm, platelet count of 3,00,000/cu.mm and serum creatinine of 0.6mg/dl and PT-INR was 1.35. On examination, she had proptosis in left eye with finger counting at 3 feet, visual acuity in right eye was 6/12.
There was no history of trauma. She was a known diabetic since 15 years. She had Haemoglobin of 12.2gm%, Total leucocyte count of 10,300/cu.mm, platelet count of 3,00,000/cu.mm and serum creatinine of 0.6mg/dl and PT-INR was 1.35. On examination, she had proptosis in left eye with finger counting at 3 feet, visual acuity in right eye was 6/12.
Ultrasound B-scan with doppler of the left eye showed dilated superior ophthalmic vein (Figure 2). Contrast-enhanced computed tomography of the head showed proptosis of the left eye with a dilated left superior ophthalmic vein (Figure 3).
Ultrasound B-scan with doppler of the left eye showed dilated superior ophthalmic vein (Figure 2). Contrast-enhanced computed tomography of the head showed proptosis of the left eye with a dilated left superior ophthalmic vein (Figure 3).
Fig. 2 Doppler ultrasound of the left eye shows a prominent superior ophthalmic vein
Fig. 3 Contrast enhanced CT of the brain shows left eye proptosis, preseptal thickening with multiple collaterals and dilated superior ophthalmic vein
4-vessel Digital subtraction angiography (DSA) showed multiple small feeders arising from the internal maxillary branch of left external carotid artery with drainage across the cavernous sinus into the ophthalmic veins suggestive of type C CCF(Figure 4A, B). This was done using a 4F arterial access through the right femoral artery. Using a 4F Head Hunter (Cordis) and single length guide wire (Terumo), selective angiograms of the left external carotid artery was obtained. However, the there was no direct fistula demonstrated. Hence, arterial access route for embolization of CCF could not be performed. Therefore, a transvenous approach to embolization was decided. The inferior petrosal sinus was not well seen, and the right facial and superior ophthalmic vein were prominent and well delineated on late phase of ECA Angiogram (Figure 5A, B). The right facial vein was observed to be more prominent as compared to the left facial vein even on left ECA angiogram (Figure 6). Hence, an anterior approach across right facial vein for CCF embolization was decided.
4-vessel Digital subtraction angiography (DSA) showed multiple small feeders arising from the internal maxillary branch of left external carotid artery with drainage across the cavernous sinus into the ophthalmic veins suggestive of type C CCF(Figure 4A, B). This was done using a 4F arterial access through the right femoral artery. Using a 4F Head Hunter (Cordis) and single length guide wire (Terumo), selective angiograms of the left external carotid artery was obtained. However, the there was no direct fistula demonstrated. Hence, arterial access route for embolization of CCF could not be performed. Therefore, a transvenous approach to embolization was decided. The inferior petrosal sinus was not well seen, and the right facial and superior ophthalmic vein were prominent and well delineated on late phase of ECA Angiogram (Figure 5A, B). The right facial vein was observed to be more prominent as compared to the left facial vein even on left ECA angiogram (Figure 6). Hence, an anterior approach across right facial vein for CCF embolization was decided.
Fig. 4A
Fig. 4B
Lateral view(A) and Oblique view(B) of left ECA Angiogram shows multiple collaterals from internal maxillary artery both the cavernous sinus with early drainage into right superior ophthalmic vein and angular vein.
Fig. 5A
Fig. 5B
Fig 5: Lateral view(A) and Oblique view(B) of delayed phase of ECA Angiogram show prominent right facial, angular and superior ophthalmic vein.
Fig. 6 Venous phase of left ECA angiogram shows a dilated right facial vein more prominent as compared to the left facial vein
Therefore, a transvenous access through the right femoral vein was obtained. The catheter was navigated across the IVC and SVC into the internal jugular vein, where a 7F long sheath was placed. Using the venous phase of ECA angiogram as a roadmap, a microcatheter (Headway) and microwire (Synchro) system was negotiated through the venous route into the right facial vein (Figure 7A, B). This system was then negotiated into the right angular vein (Figure 8A, B, C), and then across the right superior ophthalmic vein (Figure 9A, B, C) into the right cavernous sinus (Figure 10 A, B).
Therefore, a transvenous access through the right femoral vein was obtained. The catheter was navigated across the IVC and SVC into the internal jugular vein, where a 7F long sheath was placed. Using the venous phase of ECA angiogram as a roadmap, a microcatheter (Headway) and microwire (Synchro) system was negotiated through the venous route into the right facial vein (Figure 7A, B). This system was then negotiated into the right angular vein (Figure 8A, B, C), and then across the right superior ophthalmic vein (Figure 9A, B, C) into the right cavernous sinus (Figure 10 A, B).
Fig. 7A
Fig. 7B
igs. 7A,B Lateral view(A) and Oblique view(B) of delayed phase of ECA Angiogram shows the microcatheter in the right facial vein (black arrowhead)
Fig. 8A
Fig. 8B
Fig. 8C
Fig. 8 AP view(A), Lateral view(B) and magnified lateral view(C) of delayed phase of ECA angiogram showing microcatheter (black arrowhead) in the angular vein.
Fig. 9A
Fig. 9B
Fig. 9C
Fig 9: Serial fluoroscopic images (A,B,C) show negotiation of the microwire and microcatheter from the right angular vein into the superior ophthalmic vein.
Fig. 10A
Fig. 10B
Fig 10: Fluoroscopic image(A) and delayed phase of ECA Angiogram(B) shows microcatheter-microwire system across the superior ophthalmic vein reaching the cavernous sinus
The system was directed across the intercavernous communication into the left cavernous sinus (Figure 11A, B, C). Two detachable soft coils (Target) 6mm X 30cm and 10mm X 40cm were deployed into the left cavernous sinus (Figure 12 A, B) and also into the intercavernous communication (Figure 13 A, B).
The system was directed across the intercavernous communication into the left cavernous sinus (Figure 11A, B, C). Two detachable soft coils (Target) 6mm X 30cm and 10mm X 40cm were deployed into the left cavernous sinus (Figure 12 A, B) and also into the intercavernous communication (Figure 13 A, B).
Figs. 11A,B, C
Fig 11: Serial fluoroscopic images (A.B.C) show negotiation of the microwire from the right cavernous sinus, across the intercavernous communication into the left cavernous sinus.
Fig. 12,A,B
Fig 12: AP(A) and lateral view(B) of the orbits showing deployed coils with microcatheter.
Figs. 13A,B
Fig 13: AP(A) and lateral view(B) of the orbits showing coils in the left cavernous sinus and intercavernous communication
Check angiogram obtained immediately post procedure showed complete occlusion of the CCF (Figure 14A, B).
Check angiogram obtained immediately post procedure showed complete occlusion of the CCF (Figure 14A, B).
Figs. 14 A,B
Fig 14: AP(A) and lateral view(B) of check CCA angiogram confirm obliteration of the fistula.
Clinical picture of patient after procedure showed significant improvement in left eye swelling with clinical improvement of vision (Figure 15).
Clinical picture of patient after procedure showed significant improvement in left eye swelling with clinical improvement of vision (Figure 15).
Figs. 15 Photograph of patient 8 months after the procedure showing complete clinical improvement
Discussion:
Discussion:
Barrow classified the CCF into direct (type A) and indirect (types B–D) types, as follows (1):
Barrow classified the CCF into direct (type A) and indirect (types B–D) types, as follows (1):
· Type A: Direct communication between the intracavernous internal carotid artery (ICA) and cavernous sinus, usually traumatic (AI) or due to a ruptured aneurysm (AII).
· Type A: Direct communication between the intracavernous internal carotid artery (ICA) and cavernous sinus, usually traumatic (AI) or due to a ruptured aneurysm (AII).
· Type B: Dural shunt between intracavernous branches of the ICA and cavernous sinus
· Type B: Dural shunt between intracavernous branches of the ICA and cavernous sinus
· Type C: Dural shunts between meningeal branches of the external carotid artery (ECA) and cavernous sinus
· Type C: Dural shunts between meningeal branches of the external carotid artery (ECA) and cavernous sinus
· Type D: Dural shunts between meningeal branches of both the ICA and the ECA and the cavernous sinus.
· Type D: Dural shunts between meningeal branches of both the ICA and the ECA and the cavernous sinus.
Type B-D are usually spontaneous in origin.
Type B-D are usually spontaneous in origin.
Direct CCFs are usually a result of trauma, commonly seen in the young male patients. They present with acute symptoms as opposed to indirect CCFs, that have an insidious course of disease, commonly seen in postmenopausal females. They are usually unilateral and bilateral spontaneous indirect CCF is are rare and usually associated with an underlying etiology or a known cause like Ehlers–Danlos syndrome or diabetes mellitus (2).
Direct CCFs are usually a result of trauma, commonly seen in the young male patients. They present with acute symptoms as opposed to indirect CCFs, that have an insidious course of disease, commonly seen in postmenopausal females. They are usually unilateral and bilateral spontaneous indirect CCF is are rare and usually associated with an underlying etiology or a known cause like Ehlers–Danlos syndrome or diabetes mellitus (2).
The clinical features of a CCF can be attributed to increased venous pressure due to high flow across the fistula, causing increased pressures in the cavernous sinus and its tributaries with altered flow dynamics. Hence most patients present with a red, swollen eye with proptosis, diplopia and ophthalmoplegia. They can also present with pulsatile tinnitus and headache. Signs of the disease include dilated episcleral veins, chemosis, proptosis, diminished visual acuity, elevated intra-ocular pressure, and palsies of cranial nerves III and VI. Anteriorly draining fistulas are more likely to cause ocular symptoms. Patients with posteriorly draining fistulas may develop neurologic symptoms, such as confusion and expressive aphasia, as well as diplopia from isolated ocular motor nerve pareses (3).
The clinical features of a CCF can be attributed to increased venous pressure due to high flow across the fistula, causing increased pressures in the cavernous sinus and its tributaries with altered flow dynamics. Hence most patients present with a red, swollen eye with proptosis, diplopia and ophthalmoplegia. They can also present with pulsatile tinnitus and headache. Signs of the disease include dilated episcleral veins, chemosis, proptosis, diminished visual acuity, elevated intra-ocular pressure, and palsies of cranial nerves III and VI. Anteriorly draining fistulas are more likely to cause ocular symptoms. Patients with posteriorly draining fistulas may develop neurologic symptoms, such as confusion and expressive aphasia, as well as diplopia from isolated ocular motor nerve pareses (3).
Ultrasound of the eye is an easily available modality that can show a dilated tortuous superior ophthalmic vein. On spectral doppler analysis, low resistance arterialized flow can be seen in the vein(4). Cross-sectional imaging including CT & MRI can show dilated tortuous superior ophthalmic vein, with orbital congestion, with enlargement of the extraocular muscles and periorbital fat, and convexity of the lateral wall of the cavernous sinus. However, as these findings are not specific for a CCF, patients in whom a CCF is suspected still may require DSA, which remains the gold standard for classification and diagnosis of CCF and can be both diagnostic and therapeutic. Angiographically, high-flow fistulas show rapid filling of the cavernous sinus through the fistula with minimal or no filling of the intracranial vasculature, whereas low-flow fistulas demonstrate slower filling of the cerebral venous system through the fistula, with preserved filling of the intracranial arteries. In addition, DSA characterizes the drainage pattern of the fistula.
Ultrasound of the eye is an easily available modality that can show a dilated tortuous superior ophthalmic vein. On spectral doppler analysis, low resistance arterialized flow can be seen in the vein(4). Cross-sectional imaging including CT & MRI can show dilated tortuous superior ophthalmic vein, with orbital congestion, with enlargement of the extraocular muscles and periorbital fat, and convexity of the lateral wall of the cavernous sinus. However, as these findings are not specific for a CCF, patients in whom a CCF is suspected still may require DSA, which remains the gold standard for classification and diagnosis of CCF and can be both diagnostic and therapeutic. Angiographically, high-flow fistulas show rapid filling of the cavernous sinus through the fistula with minimal or no filling of the intracranial vasculature, whereas low-flow fistulas demonstrate slower filling of the cerebral venous system through the fistula, with preserved filling of the intracranial arteries. In addition, DSA characterizes the drainage pattern of the fistula.
Treatment of CCF is mainly interventional in nature. Previously, the treatment options for direct CCFs were limited to observation or treatment consisting of trapping of the fistula by ligating the cervical ICA proximal to the fistula and the intracranial ICA distal to the fistula or occlusion of the common carotid artery or ICA, either of which could result in a cerebral ischaemic event due to an induced low-flow state or an embolic event. With the development of endovascular interventional techniques, open surgical procedures are no longer preferred, the range of potential therapies has broadened, and the ICA almost always can be preserved. Endovascular treatment is less invasive and carries a lower risk of cerebral infarction, compared with ICA sacrifice. The ideal treatment approach depends on the arterial supply, the venous drainage, the speed of blood flow through the fistula, and the patency of the circle of Willis. Overall, endovascular intervention offers a 90–100% cure rate with a low rate of complications (4). A transarterial approach across the ICA is commonly used. A guiding catheter is placed in the ipsilateral femoral artery and advanced up to the ICA, followed by introduction of a microcatheter into the cavernous ICA, then through the fistula into the cavernous sinus. The embolic material of choice, including detachable balloons, coils, n-butyl cyanoacrylate, or ethylene vinyl alcohol copolymer (Onyx Liquid Embolic System) is then injected into the cavernous sinus through the microcatheter.
Treatment of CCF is mainly interventional in nature. Previously, the treatment options for direct CCFs were limited to observation or treatment consisting of trapping of the fistula by ligating the cervical ICA proximal to the fistula and the intracranial ICA distal to the fistula or occlusion of the common carotid artery or ICA, either of which could result in a cerebral ischaemic event due to an induced low-flow state or an embolic event. With the development of endovascular interventional techniques, open surgical procedures are no longer preferred, the range of potential therapies has broadened, and the ICA almost always can be preserved. Endovascular treatment is less invasive and carries a lower risk of cerebral infarction, compared with ICA sacrifice. The ideal treatment approach depends on the arterial supply, the venous drainage, the speed of blood flow through the fistula, and the patency of the circle of Willis. Overall, endovascular intervention offers a 90–100% cure rate with a low rate of complications (4). A transarterial approach across the ICA is commonly used. A guiding catheter is placed in the ipsilateral femoral artery and advanced up to the ICA, followed by introduction of a microcatheter into the cavernous ICA, then through the fistula into the cavernous sinus. The embolic material of choice, including detachable balloons, coils, n-butyl cyanoacrylate, or ethylene vinyl alcohol copolymer (Onyx Liquid Embolic System) is then injected into the cavernous sinus through the microcatheter.
Type A fistulas very rarely resolve spontaneously; however, type B, C, and D fistulas have a higher incidence of spontaneous resolution (5). Therefore, some cases of indirect and low-flow fistulas can initially be observed only or managed conservatively for ocular symptoms with medical management or manual carotid compression. Endovascular treatment is first line and may be performed transarterially or transvenously. Similar to embolization of direct CCFs, embolization of indirect CCFs may be achieved using coils, acrylic glue, or Onyx, which can be used individually or in combination. Flow-diverting stents also may be used alone or in combination with coils (6). Advantages of coils include their radio-opacity and ability to be re-deployed or removed if initial placement is not ideal. Despite increasing success of transarterial procedures, a transvenous approach via the Inferior petrosal sinus(IPS), superior petrosal sinus, basilar plexus, pterygoid plexus, superior, or inferior ophthalmic vein, still is preferred for most dural CCFs that require treatment (7). Intervention across the petrosal vein or SOV routes is preferred because neither contain valves. To access the IPS, a posterior approach via the internal jugular vein is used. When the IPS approach is not possible due to anatomic variations or thrombosis, an SOV approach may be used across the facial vein. Use of 3D rotational angiography, an emerging imaging technique, permits identification of the fistula and its venous drainage When transvenous approaches are not feasible due to vessel tortuosity, or venous sinus thrombosis or occlusion, a direct orbital approach to the cavernous sinus with fluoroscopic guidance may be considered. The reported complete cure rate for indirect CCFs is 70% to 90% with a complication rate of 2.3% to 5% (8).
Type A fistulas very rarely resolve spontaneously; however, type B, C, and D fistulas have a higher incidence of spontaneous resolution (5). Therefore, some cases of indirect and low-flow fistulas can initially be observed only or managed conservatively for ocular symptoms with medical management or manual carotid compression. Endovascular treatment is first line and may be performed transarterially or transvenously. Similar to embolization of direct CCFs, embolization of indirect CCFs may be achieved using coils, acrylic glue, or Onyx, which can be used individually or in combination. Flow-diverting stents also may be used alone or in combination with coils (6). Advantages of coils include their radio-opacity and ability to be re-deployed or removed if initial placement is not ideal. Despite increasing success of transarterial procedures, a transvenous approach via the Inferior petrosal sinus(IPS), superior petrosal sinus, basilar plexus, pterygoid plexus, superior, or inferior ophthalmic vein, still is preferred for most dural CCFs that require treatment (7). Intervention across the petrosal vein or SOV routes is preferred because neither contain valves. To access the IPS, a posterior approach via the internal jugular vein is used. When the IPS approach is not possible due to anatomic variations or thrombosis, an SOV approach may be used across the facial vein. Use of 3D rotational angiography, an emerging imaging technique, permits identification of the fistula and its venous drainage When transvenous approaches are not feasible due to vessel tortuosity, or venous sinus thrombosis or occlusion, a direct orbital approach to the cavernous sinus with fluoroscopic guidance may be considered. The reported complete cure rate for indirect CCFs is 70% to 90% with a complication rate of 2.3% to 5% (8).
Conclusion:
Conclusion:
CCF is special type of arteriovenous fistula, diagnosed clinically in patients presenting with proptosis, red eye, and orbital symptoms. Diagnostic angiography is the gold standard investigation for diagnosis and endovascular approach is the mainstay for treatment. Transarterial route is preferred for management of direct CCF, whereas transvenous coil occlusion of the superior and inferior ophthalmic veins and the cavernous sinus of the symptomatic eye is efficient and safe treatment in dural carotid–cavernous sinus fistulas.
CCF is special type of arteriovenous fistula, diagnosed clinically in patients presenting with proptosis, red eye, and orbital symptoms. Diagnostic angiography is the gold standard investigation for diagnosis and endovascular approach is the mainstay for treatment. Transarterial route is preferred for management of direct CCF, whereas transvenous coil occlusion of the superior and inferior ophthalmic veins and the cavernous sinus of the symptomatic eye is efficient and safe treatment in dural carotid–cavernous sinus fistulas.
References:
References:
1. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. Journal of neurosurgery. 1985 Feb 1;62(2):248-56.
1. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. Journal of neurosurgery. 1985 Feb 1;62(2):248-56.
2. Khan S, Gibbon C, Johns S. A rare case of bilateral spontaneous indirect caroticocavernous fistula treated previously as a case of conjunctivitis. Therapeutic Advances in Ophthalmology. 2018 Jul 16;10:2515841418788303.
2. Khan S, Gibbon C, Johns S. A rare case of bilateral spontaneous indirect caroticocavernous fistula treated previously as a case of conjunctivitis. Therapeutic Advances in Ophthalmology. 2018 Jul 16;10:2515841418788303.
3. Henderson AD, Miller NR. Carotid-cavernous fistula: current concepts in aetiology, investigation, and management. Eye. 2018 Feb;32(2):164-72.
3. Henderson AD, Miller NR. Carotid-cavernous fistula: current concepts in aetiology, investigation, and management. Eye. 2018 Feb;32(2):164-72.
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7. Gemmete JJ, Ansari SA, Gandhi D . Endovascular treatment of carotid cavernous fistulas. Neuroimaging Clin N Am 2009; 19 (2): 241–255.
7. Gemmete JJ, Ansari SA, Gandhi D . Endovascular treatment of carotid cavernous fistulas. Neuroimaging Clin N Am 2009; 19 (2): 241–255.
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