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Successful repair of spontaneous indirect bilateral carotid-cavernous fistula with coil embolization

Successful repair of spontaneous indirect bilateral carotid-cavernous fistula with coil embolization Bilateral carotid-cavernous fistula (CCF) is a rare disease process, which portends poor visual outcome with delayed diagnosis and treatment. An 82-year-old woman presented with sudden onset of proptosis and decreased vision. A complete ophthalmologic examination along with magnetic resonance (MR) imaging of the brain and orbits, and MR angiography and venography of the brain confirmed the diagnosis of bilateral CCF. Diagnostic cerebral angiogram with concurrent coil embolization of the right cavernous sinus via left superior ophthalmic vein approach was performed. Bilateral indirect CCFs (type D CCF on the right and a type B CCF on the left) regressed completely after unilateral coil embolization. Visual acuity and limitation in extraocular movements significantly improved with complete resolution. In summary, we describe successful management of bilateral concurrent CCF with image-guided embolization and immediate recovery of vision and resolution of ophthalmological symptoms including proptosis and diplopia. the result of fistulization between both the ICA and ECA and INTRODUCTION cavernous sinus [1]. A carotid-cavernous fistula (CCF) is the result of an abnormal Bilateral CCFs are rare and seen in 1–2% of patients with CCFs vascular communication between the carotid arterial system [1]. Spontaneous indirect bilateral CCFs of different types are [the internal carotid artery (ICA) and/or external carotid artery rarer. We report a case of bilateral concurrent (types D and B) (ECA)], and the venous channels of the cavernous sinus. They non-traumatic indirect CCFs successfully treated with endovas- may be direct or indirect based on angiographic architecture, cular coil embolization. traumatic or spontaneous based on its etiology and stable or progressive based on hemodynamics [4, 5, 7]. Indirect CCFs are rare (30% of all CCFs) and typically result CASE REPORT from ICA aneurysm rupture. Hypertension, collagen disorder, An 82-year-old female with history of macular degeneration female gender and older age are known risk factors [1, 6]. The and left frontotemporal craniotomy for meningioma resection Barrow classification system for CCFs sub-divides them into (5 years earlier) presented with sudden onset of worsening right direct (type A; direct connection between the ICA and cavernous eye pain, proptosis, diplopia, decreased vision, and associated sinus) and indirect (types B, C, D) CCFs. Type B CCF is a dural periorbital edema and erythema (Fig. 1). shunt between meningeal branches of the ICA and cavernous Ocular examination revealed significant periorbital edema sinus (1%). Type C CCF is a dural shunt between meningeal and erythema, moderate proptosis of the right eye. branches of the ECA and cavernous sinus, and type D CCF is Received: February 16, 2021. Accepted: March 23, 2021 Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2021. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. 1 Downloaded from https://academic.oup.com/jscr/article/2021/4/rjab140/6246283 by guest on 27 April 2021 2 S.A. Gasparian and K.V. Chalam Figure 1: External photos of the patient’s eye before and after management of carotid-cavernous fistulas. (A) External photo of patient’s right eye upon presentation, prior to diagnosis of CCF. (B) External photos of patient’s eyes 6 months after resolution of CCFs. (C) Diagrammatic illustration of type D CCF on the right and type B CCF on the left as demonstrated by abnormal, yellow vasculature. Type D CCF on the right illustrates dural shunts between meningeal branches of both the internal and external carotid arteries and the cavernous sinus. Type B CCF illustrates dural shunts between meningeal branches of the internal carotid artery and cavernous sinus. Figure 2: Diagnostic cerebral angiogram demonstrative of right indirect CCF. (A) Right ICA angiogram revealed indirect fistulization between the ECA branches and cavernous sinus (type D CCF) (B) with subsequent successful endovascular coil embolization. Best-corrected visual acuity (BCVA) was 20/200 in the right used to access the right cavernous sinus via the left cavernous eye and 20/40 in the left eye. Intraocular pressure (IOP) was sinus through the intercavernous sinus. Right indirect CCF with 33 mmHg in the right eye and 21 mmHg in the left eye. Pupillary arterial feeders arising from both the internal and external examination revealed a sluggish, poorly reactive 3 mm pupil carotid arteries with outflow through the intercavernous sinus on the right and a reactive 3 mm pupil on the left. Extraocular was confirmed. Embolization of the right cavernous sinus movements were full in the left eye; however, there was complete and intercavernous sinus was performed with 11 detachable ophthalmoplegia of the right eye. Anterior segment examination coils and resolution of the type D carotid-cavernous flow was revealed conjunctival vascular congestion, significant chemosis immediately noted (Fig. 2B); spontaneous resolution of the left and corkscrew vessels in the right eye (Fig. 1A); the left eye type B CCF was noted (Fig. 3B) over a 24-h period of time. demonstrated mild chemosis with conjunctival corkscrew Over the course of 2 months, the patient’s vision improved vessels prominent medially. There was no optic disk edema to 20/20 in the right eye with normalization of IOP to 11 mmHg or choroidal folds on fundoscopy. and recovery of extraocular movements. Proptosis and signs of A computerized tomography (CT) scan of the orbits demon- conjunctival vascular congestion completely resolved in a week strated right periorbital tissue swelling and superior ophthalmic (Fig. 1B). vein thrombosis (SOVT). Magnetic resonance venography/mag- netic resonance angiography (MRA) of the brain demonstrated prominent superior ophthalmic veins bilaterally with flow void DISCUSSION extending from the septal region to the posterior orbit on the right consistent with thrombosis. Although various classifications of CCFs have been cited in the Diagnostic cerebral angiogram (DCA) revealed bilateral literature, reports of bilateral, spontaneous, combined Barrow indirect CCFs (type D on the right, type B on the left) with type D and B type CCFs remain limited. We present the case significantly higher flow on the right compared to the left of an older woman without history of head trauma, albeit with (Figs 1C, 2A, 3A). A day later, through micro puncture of the a remote history of meningioma resection, presenting with a right common femoral artery, coil was advanced (under real- unique variant of bilateral indirect CCFs successfully treated time sonographic guidance and DCA). A microcatheter was with unilateral endovascular therapy. Downloaded from https://academic.oup.com/jscr/article/2021/4/rjab140/6246283 by guest on 27 April 2021 Bilateral concurrent CCF 3 Figure 3: Diagnostic cerebral angiogram demonstrative of left indirect CCF. (A) Left ICA angiogram revealed a type B CCF supplied by branches of the meningohypophyseal trunk with inferior venous drainage (B) with evidence of subsequent spontaneous resolution. Ophthalmic complications secondary to CCFs occur as a embolization with spontaneous resolution of visual symptoms result of congestion from impaired venous drainage of the orbit and immediate recovery of vision. to the cavernous sinus. Clinical presentation is variable due to differences in fistula size, location, rate of flow and location CONFLICT OF INTEREST STATEMENT in the cavernous sinus [4]. Patients with direct CCFs classi- None declared. cally present with the acute-onset clinical triad of chemosis (55–100%), proptosis (72%) and an ocular bruit (80%) [1, 4]. Other findings include eyelid edema, conjunctival arterialization, FUNDING elevated IOP, ocular misalignment, diplopia and decrease in None. vision [1–3]. Diagnosis of CCF suspected clinically, is confirmed with contrast-enhanced CT angiography (CTA), MRA of the brain or diagnostic cerebral angiogram (the gold standard). REFERENCES Indirect or dural, low-flow CCFs are uncommon clinical enti- 1. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall ties, typically occur spontaneously, and are insidious in onset. SC, Tindall GT. Classification and treatment of sponta- Bilateral, spontaneous indirect CCFs are rare, with a total of neous carotid-cavernous sinus fistulas. J Neurosurg 1985;62: 35 reported cases in the literature [8]. There have been only 248–56. two reported cases of bilateral CCFs involving a combined Bar- 2. Miller NR. Diagnosis and management of dural carotid- row type D and B classification [9, 10]. Unlike the patient pre- cavernous sinus fistulas. Neurosurg Focus 2007;23:E13. sented in this study, these patients either underwent a staged, 3. Williams. Carotid-cavernous fistulae. Int Ophthalmol Clin bilateral-surgical arterial embolization or were managed conser- 2018;58:271–94. vatively with complete resolution of symptoms. Unlike direct 4. Ellis JA, Goldstein H, Connolly ES, Meyers PM. Carotid- CCFs, which are rarely asymptomatic and are treated urgently, cavernous fistulas. Neurosurg Focus 2012;32:E9. indirect CCFs may resolve spontaneously due to thrombosis in 5. Ono K, Oishi H, Tanoue S, Hasegawa H, Yoshida K, up to 60% of cases [1, 4]. Over all, conventional endovascular ther- Yamamoto M, et al. Direct carotid-cavernous fistulas occur- apy remains the mainstay of treatment. About 80% of patients ring during neurointerventional procedures. Interv Neurora- who undergo endovascular repair experience resolution of CCF diol 2016;22:91–6. [4]. Interestingly, as seen in our patient, certain cases of bilateral 6. Chuman H, Trobe JD, Petty EM. Spontaneous direct carotid- CCFs may be sufficiently treated with unilateral endovascular cavernous fistula in Ehlers-Danlos syndrome type IV: two treatment to obliterate both fistulas. About 20–60% of patients case reports and a review of the literature. J Neuroophthalmol with indirect CCFs have spontaneous fistula closure [1]. 2002;22:75–81. Ocular involvement in CCFs may denote devastating vision- 7. Debrun GM, Viñuela F, Fox AJ, Davis KR, Ahn HS. Indications threatening complications with a delay in proper diagnosis and for treatment and classification of 132 carotid-cavernous treatment. Venous congestion from raised venous pressure and fistulas. Neurosurgery 1988;22:285–9. resultant secondary glaucoma may disrupt ocular perfusion and 8. Khan S, Gibbon C, Johns S. A rare case of bilateral spon- result in irreversible vision loss. Both direct and indirect CCFs taneous indirect caroticocavernous fistula treated previ- carry a favorable visual prognosis unless there is evidence of ously as a case of conjunctivitis. Ther Adv Ophthalmol retinal or optic nerve ischemia prior to treatment [3]. Prompt 2018;10:2515841418788303. diagnosis and treatment of CCFs can result in favorable visual 9. Al-Mufti F, Amuluru K, El-Ghanem M. Spontaneous bilat- outcomes. We therefore recommend considering the diagnosis eral carotid-cavernous fistulas secondary to cavernous sinus of CCF when there is concern for possible orbital cellulitis, thrombosis. Neurosurgery 2017;80:646–54. diplopia, an ICA aneurysm, or SOVT. 10. Albert P, Polaina M, Trujillo F. Direct carotid sinus approach In summary, we describe a rare case of bilateral concurrent, to treatment of bilateral carotid-cavernous fistulas. Case non-traumatic indirect CCF (type B and type D confirmed on report. J Neurosurg 1988;69:942–4. cerebral angiogram) successfully treated with endovascular coil http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Successful repair of spontaneous indirect bilateral carotid-cavernous fistula with coil embolization

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Oxford University Press
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Copyright © 2021 Oxford University Press and JSCR Publishing Ltd
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2042-8812
DOI
10.1093/jscr/rjab140
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Abstract

Bilateral carotid-cavernous fistula (CCF) is a rare disease process, which portends poor visual outcome with delayed diagnosis and treatment. An 82-year-old woman presented with sudden onset of proptosis and decreased vision. A complete ophthalmologic examination along with magnetic resonance (MR) imaging of the brain and orbits, and MR angiography and venography of the brain confirmed the diagnosis of bilateral CCF. Diagnostic cerebral angiogram with concurrent coil embolization of the right cavernous sinus via left superior ophthalmic vein approach was performed. Bilateral indirect CCFs (type D CCF on the right and a type B CCF on the left) regressed completely after unilateral coil embolization. Visual acuity and limitation in extraocular movements significantly improved with complete resolution. In summary, we describe successful management of bilateral concurrent CCF with image-guided embolization and immediate recovery of vision and resolution of ophthalmological symptoms including proptosis and diplopia. the result of fistulization between both the ICA and ECA and INTRODUCTION cavernous sinus [1]. A carotid-cavernous fistula (CCF) is the result of an abnormal Bilateral CCFs are rare and seen in 1–2% of patients with CCFs vascular communication between the carotid arterial system [1]. Spontaneous indirect bilateral CCFs of different types are [the internal carotid artery (ICA) and/or external carotid artery rarer. We report a case of bilateral concurrent (types D and B) (ECA)], and the venous channels of the cavernous sinus. They non-traumatic indirect CCFs successfully treated with endovas- may be direct or indirect based on angiographic architecture, cular coil embolization. traumatic or spontaneous based on its etiology and stable or progressive based on hemodynamics [4, 5, 7]. Indirect CCFs are rare (30% of all CCFs) and typically result CASE REPORT from ICA aneurysm rupture. Hypertension, collagen disorder, An 82-year-old female with history of macular degeneration female gender and older age are known risk factors [1, 6]. The and left frontotemporal craniotomy for meningioma resection Barrow classification system for CCFs sub-divides them into (5 years earlier) presented with sudden onset of worsening right direct (type A; direct connection between the ICA and cavernous eye pain, proptosis, diplopia, decreased vision, and associated sinus) and indirect (types B, C, D) CCFs. Type B CCF is a dural periorbital edema and erythema (Fig. 1). shunt between meningeal branches of the ICA and cavernous Ocular examination revealed significant periorbital edema sinus (1%). Type C CCF is a dural shunt between meningeal and erythema, moderate proptosis of the right eye. branches of the ECA and cavernous sinus, and type D CCF is Received: February 16, 2021. Accepted: March 23, 2021 Published by Oxford University Press and JSCR Publishing Ltd. © The Author(s) 2021. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. 1 Downloaded from https://academic.oup.com/jscr/article/2021/4/rjab140/6246283 by guest on 27 April 2021 2 S.A. Gasparian and K.V. Chalam Figure 1: External photos of the patient’s eye before and after management of carotid-cavernous fistulas. (A) External photo of patient’s right eye upon presentation, prior to diagnosis of CCF. (B) External photos of patient’s eyes 6 months after resolution of CCFs. (C) Diagrammatic illustration of type D CCF on the right and type B CCF on the left as demonstrated by abnormal, yellow vasculature. Type D CCF on the right illustrates dural shunts between meningeal branches of both the internal and external carotid arteries and the cavernous sinus. Type B CCF illustrates dural shunts between meningeal branches of the internal carotid artery and cavernous sinus. Figure 2: Diagnostic cerebral angiogram demonstrative of right indirect CCF. (A) Right ICA angiogram revealed indirect fistulization between the ECA branches and cavernous sinus (type D CCF) (B) with subsequent successful endovascular coil embolization. Best-corrected visual acuity (BCVA) was 20/200 in the right used to access the right cavernous sinus via the left cavernous eye and 20/40 in the left eye. Intraocular pressure (IOP) was sinus through the intercavernous sinus. Right indirect CCF with 33 mmHg in the right eye and 21 mmHg in the left eye. Pupillary arterial feeders arising from both the internal and external examination revealed a sluggish, poorly reactive 3 mm pupil carotid arteries with outflow through the intercavernous sinus on the right and a reactive 3 mm pupil on the left. Extraocular was confirmed. Embolization of the right cavernous sinus movements were full in the left eye; however, there was complete and intercavernous sinus was performed with 11 detachable ophthalmoplegia of the right eye. Anterior segment examination coils and resolution of the type D carotid-cavernous flow was revealed conjunctival vascular congestion, significant chemosis immediately noted (Fig. 2B); spontaneous resolution of the left and corkscrew vessels in the right eye (Fig. 1A); the left eye type B CCF was noted (Fig. 3B) over a 24-h period of time. demonstrated mild chemosis with conjunctival corkscrew Over the course of 2 months, the patient’s vision improved vessels prominent medially. There was no optic disk edema to 20/20 in the right eye with normalization of IOP to 11 mmHg or choroidal folds on fundoscopy. and recovery of extraocular movements. Proptosis and signs of A computerized tomography (CT) scan of the orbits demon- conjunctival vascular congestion completely resolved in a week strated right periorbital tissue swelling and superior ophthalmic (Fig. 1B). vein thrombosis (SOVT). Magnetic resonance venography/mag- netic resonance angiography (MRA) of the brain demonstrated prominent superior ophthalmic veins bilaterally with flow void DISCUSSION extending from the septal region to the posterior orbit on the right consistent with thrombosis. Although various classifications of CCFs have been cited in the Diagnostic cerebral angiogram (DCA) revealed bilateral literature, reports of bilateral, spontaneous, combined Barrow indirect CCFs (type D on the right, type B on the left) with type D and B type CCFs remain limited. We present the case significantly higher flow on the right compared to the left of an older woman without history of head trauma, albeit with (Figs 1C, 2A, 3A). A day later, through micro puncture of the a remote history of meningioma resection, presenting with a right common femoral artery, coil was advanced (under real- unique variant of bilateral indirect CCFs successfully treated time sonographic guidance and DCA). A microcatheter was with unilateral endovascular therapy. Downloaded from https://academic.oup.com/jscr/article/2021/4/rjab140/6246283 by guest on 27 April 2021 Bilateral concurrent CCF 3 Figure 3: Diagnostic cerebral angiogram demonstrative of left indirect CCF. (A) Left ICA angiogram revealed a type B CCF supplied by branches of the meningohypophyseal trunk with inferior venous drainage (B) with evidence of subsequent spontaneous resolution. Ophthalmic complications secondary to CCFs occur as a embolization with spontaneous resolution of visual symptoms result of congestion from impaired venous drainage of the orbit and immediate recovery of vision. to the cavernous sinus. Clinical presentation is variable due to differences in fistula size, location, rate of flow and location CONFLICT OF INTEREST STATEMENT in the cavernous sinus [4]. Patients with direct CCFs classi- None declared. cally present with the acute-onset clinical triad of chemosis (55–100%), proptosis (72%) and an ocular bruit (80%) [1, 4]. Other findings include eyelid edema, conjunctival arterialization, FUNDING elevated IOP, ocular misalignment, diplopia and decrease in None. vision [1–3]. Diagnosis of CCF suspected clinically, is confirmed with contrast-enhanced CT angiography (CTA), MRA of the brain or diagnostic cerebral angiogram (the gold standard). REFERENCES Indirect or dural, low-flow CCFs are uncommon clinical enti- 1. Barrow DL, Spector RH, Braun IF, Landman JA, Tindall ties, typically occur spontaneously, and are insidious in onset. SC, Tindall GT. Classification and treatment of sponta- Bilateral, spontaneous indirect CCFs are rare, with a total of neous carotid-cavernous sinus fistulas. J Neurosurg 1985;62: 35 reported cases in the literature [8]. There have been only 248–56. two reported cases of bilateral CCFs involving a combined Bar- 2. Miller NR. Diagnosis and management of dural carotid- row type D and B classification [9, 10]. Unlike the patient pre- cavernous sinus fistulas. Neurosurg Focus 2007;23:E13. sented in this study, these patients either underwent a staged, 3. Williams. Carotid-cavernous fistulae. Int Ophthalmol Clin bilateral-surgical arterial embolization or were managed conser- 2018;58:271–94. vatively with complete resolution of symptoms. Unlike direct 4. Ellis JA, Goldstein H, Connolly ES, Meyers PM. Carotid- CCFs, which are rarely asymptomatic and are treated urgently, cavernous fistulas. Neurosurg Focus 2012;32:E9. indirect CCFs may resolve spontaneously due to thrombosis in 5. Ono K, Oishi H, Tanoue S, Hasegawa H, Yoshida K, up to 60% of cases [1, 4]. Over all, conventional endovascular ther- Yamamoto M, et al. Direct carotid-cavernous fistulas occur- apy remains the mainstay of treatment. About 80% of patients ring during neurointerventional procedures. Interv Neurora- who undergo endovascular repair experience resolution of CCF diol 2016;22:91–6. [4]. Interestingly, as seen in our patient, certain cases of bilateral 6. Chuman H, Trobe JD, Petty EM. Spontaneous direct carotid- CCFs may be sufficiently treated with unilateral endovascular cavernous fistula in Ehlers-Danlos syndrome type IV: two treatment to obliterate both fistulas. About 20–60% of patients case reports and a review of the literature. J Neuroophthalmol with indirect CCFs have spontaneous fistula closure [1]. 2002;22:75–81. Ocular involvement in CCFs may denote devastating vision- 7. Debrun GM, Viñuela F, Fox AJ, Davis KR, Ahn HS. Indications threatening complications with a delay in proper diagnosis and for treatment and classification of 132 carotid-cavernous treatment. Venous congestion from raised venous pressure and fistulas. Neurosurgery 1988;22:285–9. resultant secondary glaucoma may disrupt ocular perfusion and 8. Khan S, Gibbon C, Johns S. A rare case of bilateral spon- result in irreversible vision loss. Both direct and indirect CCFs taneous indirect caroticocavernous fistula treated previ- carry a favorable visual prognosis unless there is evidence of ously as a case of conjunctivitis. Ther Adv Ophthalmol retinal or optic nerve ischemia prior to treatment [3]. Prompt 2018;10:2515841418788303. diagnosis and treatment of CCFs can result in favorable visual 9. Al-Mufti F, Amuluru K, El-Ghanem M. Spontaneous bilat- outcomes. We therefore recommend considering the diagnosis eral carotid-cavernous fistulas secondary to cavernous sinus of CCF when there is concern for possible orbital cellulitis, thrombosis. Neurosurgery 2017;80:646–54. diplopia, an ICA aneurysm, or SOVT. 10. Albert P, Polaina M, Trujillo F. Direct carotid sinus approach In summary, we describe a rare case of bilateral concurrent, to treatment of bilateral carotid-cavernous fistulas. Case non-traumatic indirect CCF (type B and type D confirmed on report. J Neurosurg 1988;69:942–4. cerebral angiogram) successfully treated with endovascular coil

Journal

Journal of Surgical Case ReportsOxford University Press

Published: Apr 1, 2021

Keywords: angiogram; carotid-cavernous sinus fistula; cavernous sinus; exophthalmos; embolization; vision; brain; diagnosis; diplopia; orbit; eye movement; visual impairment; visual acuity; ophthalmic examination and evaluation; superior ophthalmic vein; magnetic resonance angiography; nuclear magnetic resonance; venography; delayed diagnosis; rare diseases; diagnostic imaging

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