The Journal of Emergency Medicine, Vol. 46, No. 4, pp. 475–478, 2014 Copyright Ó 2014 Elsevier Inc. Printed in the USA. All rights reserved 0736-4679/$ - see front matter
http://dx.doi.org/10.1016/j.jemermed.2013.09.015
Clinical Communications: Adults A 32-YEAR-OLD MAN WITH DELAYED ONSET POST-TRAUMATIC PROPTOSIS AND DIPLOPIA Benjamin P. Erickson, MD and Thomas E. Johnson, MD Department of Ophthalmology, Bascom Palmer Eye Institute, Miami, Florida Reprint Address: Benjamin P. Erickson, MD, Department of Ophthalmology, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136
, Abstract—Background: Proptosis and motility deficits are common findings in the setting of craniofacial trauma, but can indicate the presence of vision and even lifethreatening pathology. Objective: Our aim was to identify presentations consistent with traumatic carotid cavernous fistula (CCF) and to review the appropriate initial workup and management. Case Report: A 32-year-old man came to our emergency department with proptosis, ocular motility deficits, and decreased vision 1 month after a restrained motor vehicle accident. An orbital bruit was auscultated and four-vessel angiography revealed a CCF. Covered stents and an embolic agent were used to abolish the arteriovenous communication and the patient rapidly returned to his premorbid baseline. Conclusions: CCF is a relatively rare but important consequence of craniofacial trauma that must be recognized promptly in order to minimize the likelihood of serious sequelae. It should be suspected in patients with antecedent trauma presenting with exophthalmos, arterialized conjunctival vessels, and orbital bruit. Ó 2014 Elsevier Inc.
chemosis, and mild proptosis is frequently observed, even in the absence of serious ocular or orbital pathology. Clinically significant fractures and retrobulbar hemorrhage must be identified and treated appropriately. Management of these conditions has been addressed extensively in the trauma literature. Traumatic carotid cavernous fistula (CCF) is a less common and often neglected cause of post-traumatic proptosis and motility deficits that results from the formation of an arteriovenous communication between the internal carotid artery (ICA) and cavernous sinus (CS). It must be recognized due to the potential for visionthreatening and even life-threatening sequelae. CASE REPORT A 32-year-old African-American man reported to our emergency department with a 1-month history of double vision and redness of the right eye after a high-speed automobile accident. He lost consciousness after hitting his head on the dashboard, but was not ejected from the vehicle. Per the patient, he spent several days in a trauma intensive care unit before discharge. Uncorrected visual acuity at the time of presentation was 20/50 in right eye and 20/20 in the left. Intraocular pressures were 27 and 17 mm Hg, respectively. In primary gaze, he had a slight right exotropia (out-turning of the eye), along with moderate motility deficits in all cardinal gaze directions. Pupils were 3 mm with brisk
, Keywords—craniofacial trauma; carotid cavernous fistula; angiography; proptosis; vision loss
INTRODUCTION Proptosis, ocular motility deficits, and transient reductions in vision are very common in the setting of craniofacial trauma. Some degree of periocular edema,
RECEIVED: 3 November 2012; FINAL SUBMISSION RECEIVED: 21 July 2013; ACCEPTED: 17 September 2013 475
476
B. P. Erickson and T. E. Johnson
light reactivity and absence of an afferent pupillary defect. Visual fields by confrontation were grossly normal. Apart from dilated conjunctival blood vessels and 3 mm of proptosis in the right eye, the remainder of his ocular examination was unremarkable (Figure 1). Despite his motility deficits, the absence of eyelid ptosis and pupillary changes was not consistent with a traumatic or compressive third cranial nerve palsy. Orbital cellulitis and inflammatory conditions, such as orbital pseudotumor or scleritis, were not thought likely based on the absence of pain, chemosis, and periocular softtissue changes. Given the combination of proptosis and unilateral arterialization of the conjunctival blood vessels, a vascular process of the orbit was suspected. Subtle pulsatility of the right eye was observed. A right orbital bruit was auscultated, and he was referred to a neurointerventionalist with the tentative clinical diagnosis of traumatic CCF. Four-vessel digital subtraction angiography revealed profound dilatation of the right superior ophthalmic vein (Figure 2). A covered stent was deployed in the intracavernous ICA via a femoral approach after ethylene vinyl alcohol copolymer embolic agent was introduced into the ipsilateral CS with a microcatheter. Post-procedure angiography confirmed successful ablation of the abnormal arteriovenous connection, as well as preservation of distal arterial flow (Figure 3). On repeat examination, the patient’s visual acuity improved to 20/20 and his intraocular pressures were normal and symmetric. His motility deficits and double vision also resolved rapidly, as did his proptosis. DISCUSSION CCF is important to consider in the differential for posttraumatic proptosis. It is the result of an acquired or recanalized congenital arteriovenous communication between the ICA and the CS. The classic clinical triad of high-flow lesions consists of exophthalmos, arterialization of conjunctival blood vessels (due to ocular and orbital congestion), and a cranial bruit that diminishes with compression of the ipsilateral carotid artery.
Figure 1. Facial photograph demonstrating right-sided proptosis, moderate restriction in downgaze, and unilateral arterialization of conjunctival vessels.
Figure 2. Digital subtraction angiography demonstrating dilatation and abnormal filling of the right superior ophthalmic vein during the arterial phase.
Common associated findings include elevated intraocular pressure, visible or palpable ocular pulsations, diplopia, and reduced vision (1). Lesions are categorized according to the Barrow Classification, which is based on angiographic features. Barrow type A lesions are direct, high-flow lesions resulting from a wall defect between the ICA and CS. Barrow type B, C, and D lesions are indirect, lower-flow lesions resulting from abnormal communication between branches of the ICA or external carotid artery and the CS (2). Type A lesions account for 70% to 90% of all CCF, and occur most commonly in younger males (3). Eighty percent are seen in the context of craniofacial trauma, with an overall estimated incidence of 0.2% (3). Onset is highly variable, ranging from hours to months after trauma. They are highly associated with skull-base fractures and penetrating injuries. The remaining 20% are generally the result of spontaneous rupture of occult intracavernous ICA aneurysms or weakened atherosclerotic arteries. Direct lesions rarely close spontaneously, unlike the indirect lesions that generally represent the spontaneous recanalization of small carotid cavernous connections in the context of hypertension and atherosclerosis, childbirth, or collagen vascular disease. Natural variability of venous sinus anatomy results in several important variations in clinical presentation. Patients with widely patent superior or inferior petrosal sinuses might shunt arterial blood to the internal jugular vein, resulting in few or no ocular sequelae (‘‘white eye shunt’’). Those with limited petrosal sinus patency can
Post-Traumatic Proptosis and Diplopia
Figure 3. Preserved internal carotid artery flow after deployment of a covered stent and occlusion of the ipsilateral cavernous sinus with an embolic agent.
shunt blood to the cortical and nasal veins as well as to orbital vessels. This accounts for the 3% 5% risk of significant cerebral edema and hemorrhagic cerebrovascular accident, as well as for the 1% 2% risk of profound, potentially fatal, epistaxis (1). Orbital congestion results in increased episcleral venous pressure, accounting for increased intraocular pressure, and in mechanical restriction and ischemia of the intraocular muscles. Prolonged disease can lead to blinding glaucoma. Direct cranial nerve impairment can also occur due to compression, vascular thrombosis, or steal phenomena. The abducens nerve is the most commonly affected due to its central and unshielded position in the CS (4). Imaging can reveal proptosis, engorgement of the extraocular muscles or superior ophthalmic vein, CS enlargement, and dilatation of the leptomeningeal and cortical veins. Magnetic resonance imaging is particularly useful for identifying orbital and cerebral edema and abnormal flow voids in the CS (5). Color Doppler ultrasound can often demonstrate pulsatile flow and flow reversal within the superior ophthalmic artery (6). Digital subtraction angiography is the diagnostic gold standard and an integral component of current therapeutic modalities (5). The goal of therapy is to abolish abnormal communication between the ICA and CS, while preventing ischemia in the distribution of the distal vessels. Endovascular approaches may be arterial (via the common femoral), venous (via the femoral, jugular, common facial, or superior ophthalmic), or combined (7).
477
This therapeutic goal can be accomplished by mechanically occluding the sinus using a detachable balloon catheter, inducing thrombosis with platinum coils or embolic agents, or redirecting flow with covered stents. Balloon catheters have largely fallen out of favor due to the risk of deflation, migration, and rupture by osseous fragments (5,8). They are also difficult to pass through small ruptures. Embolic agents such as N-butyl cyanoacrylate and ethylene vinyl alcohol copolymer (OnyxÒ, Micro Therapeutics Inc., Irvine, CA) are the current therapeutic staples, but stents are gaining popularity, despite risks of thrombosis, endoleak, and the need for prolonged antiplatelet therapy (9 11). Surgical ligation of the ICA is reserved for endovascular failures due to high risk of morbidity, and requires trial balloon occlusion to assess the adequacy of collateral flow. Treatment risks include cerebral infarction, ocular ischemia, and a temporary ‘‘paradoxical effect,’’ or worsening of proptosis and ophthalmoplegia due to propagation of thrombus in the CS (12). Despite this, treatments are generally very effective and well tolerated, although motility problems are often slower to resolve than congestive symptoms. CONCLUSIONS CCF is a relatively rare but important consequence of craniofacial trauma that may manifest immediately or in a delayed fashion, and should be recognized in the urgent care context due to the risk of serious and potentially fatal sequelae if untreated. Emergency physicians should suspect this entity when confronted with a patient with unilateral exophthalmos, arterialized conjunctival vessels, and an orbital bruit. Eye movements and vision may also be compromised. When available, Doppler ultrasound is an excellent first study and permits detection of abnormal flow within the superior ophthalmic vein. Magnetic resonance imaging/magnetic resonance angiography of the brain and orbits is a lower-risk study than computed tomography angiography, although the latter is necessary for definitive therapeutic interventions.
REFERENCES 1. Miller NR. Diagnosis and management of dural carotid-cavernous sinus fistulas. Neurosurg Focus 2007;23(5):E13. 2. Barrow DL, Spector RH, Braun IF, et al. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985;62:248–56. 3. Ellis JA, Goldstein H, Connolly ES Jr, et al. Carotid-cavernous fistulas. Neurosurg Focus 2012;32(5):E9. 4. Grumann AJ, Boivin-Faure L, Chapot R, et al. Ophthalmologic outcome of direct and indirect carotid cavernous fistulas. Int Ophthalmol 2012;32:153–9.
478 5. Gemmete JJ, Ansari SA, Gandhi DM. Endovascular techniques for treatment of carotid-cavernous fistula. J Neuroophthalmol 2009;29: 62–71. 6. Duan Y, Liu X, Zhou X, et al. Diagnosis and follow-up study of carotid cavernous fistulas with color Doppler ultrasonography: analysis of 33 cases. J Ultrasound Med 2005;24:739–45. 7. Wang W, Li YD, Li MH, et al. Endovascular treatment of posttraumatic direct carotid-cavernous fistulas: a single-center experience. J Clin Neurosci 2011;18:24–8. 8. Serbinenko FA. Balloon catheterization and occlusion of major cerebral vessels. J Neurosurg 1974;41:125–45.
B. P. Erickson and T. E. Johnson 9. Luo CB, Teng MMH, Chang FC, et al. Transarterial balloonassisted n-butyl-2-cyanoacrylate embolization of direct carotid cavernous fistulas. AJNR Am J Neuroradiol 2006;27:1535–40. 10. Halbach VV, Higashida RT, Barnwell SL, et al. Transarterial platinum coil embolization of carotid-cavernous fistulas. AJNR Am J Neuroradiol 1991;12:429–33. 11. Madan A, Mujic A, Daniels K, et al. Traumatic carotid-cavernous sinus fistula treated with a covered stent: report of two cases. J Neurosurg 2006;104:969–73. 12. Oishi A, Miyamoto K, Yoshimura N. Etiology of carotid cavernous fistula in Japanese. Jpn J Ophthalmol 2009;53:40–3.
http://dx.doi.org/10.1016/j.jemermed.2013.09.015
Clinical Communications: Adults A 32-YEAR-OLD MAN WITH DELAYED ONSET POST-TRAUMATIC PROPTOSIS AND DIPLOPIA Benjamin P. Erickson, MD and Thomas E. Johnson, MD Department of Ophthalmology, Bascom Palmer Eye Institute, Miami, Florida Reprint Address: Benjamin P. Erickson, MD, Department of Ophthalmology, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136
, Abstract—Background: Proptosis and motility deficits are common findings in the setting of craniofacial trauma, but can indicate the presence of vision and even lifethreatening pathology. Objective: Our aim was to identify presentations consistent with traumatic carotid cavernous fistula (CCF) and to review the appropriate initial workup and management. Case Report: A 32-year-old man came to our emergency department with proptosis, ocular motility deficits, and decreased vision 1 month after a restrained motor vehicle accident. An orbital bruit was auscultated and four-vessel angiography revealed a CCF. Covered stents and an embolic agent were used to abolish the arteriovenous communication and the patient rapidly returned to his premorbid baseline. Conclusions: CCF is a relatively rare but important consequence of craniofacial trauma that must be recognized promptly in order to minimize the likelihood of serious sequelae. It should be suspected in patients with antecedent trauma presenting with exophthalmos, arterialized conjunctival vessels, and orbital bruit. Ó 2014 Elsevier Inc.
chemosis, and mild proptosis is frequently observed, even in the absence of serious ocular or orbital pathology. Clinically significant fractures and retrobulbar hemorrhage must be identified and treated appropriately. Management of these conditions has been addressed extensively in the trauma literature. Traumatic carotid cavernous fistula (CCF) is a less common and often neglected cause of post-traumatic proptosis and motility deficits that results from the formation of an arteriovenous communication between the internal carotid artery (ICA) and cavernous sinus (CS). It must be recognized due to the potential for visionthreatening and even life-threatening sequelae. CASE REPORT A 32-year-old African-American man reported to our emergency department with a 1-month history of double vision and redness of the right eye after a high-speed automobile accident. He lost consciousness after hitting his head on the dashboard, but was not ejected from the vehicle. Per the patient, he spent several days in a trauma intensive care unit before discharge. Uncorrected visual acuity at the time of presentation was 20/50 in right eye and 20/20 in the left. Intraocular pressures were 27 and 17 mm Hg, respectively. In primary gaze, he had a slight right exotropia (out-turning of the eye), along with moderate motility deficits in all cardinal gaze directions. Pupils were 3 mm with brisk
, Keywords—craniofacial trauma; carotid cavernous fistula; angiography; proptosis; vision loss
INTRODUCTION Proptosis, ocular motility deficits, and transient reductions in vision are very common in the setting of craniofacial trauma. Some degree of periocular edema,
RECEIVED: 3 November 2012; FINAL SUBMISSION RECEIVED: 21 July 2013; ACCEPTED: 17 September 2013 475
476
B. P. Erickson and T. E. Johnson
light reactivity and absence of an afferent pupillary defect. Visual fields by confrontation were grossly normal. Apart from dilated conjunctival blood vessels and 3 mm of proptosis in the right eye, the remainder of his ocular examination was unremarkable (Figure 1). Despite his motility deficits, the absence of eyelid ptosis and pupillary changes was not consistent with a traumatic or compressive third cranial nerve palsy. Orbital cellulitis and inflammatory conditions, such as orbital pseudotumor or scleritis, were not thought likely based on the absence of pain, chemosis, and periocular softtissue changes. Given the combination of proptosis and unilateral arterialization of the conjunctival blood vessels, a vascular process of the orbit was suspected. Subtle pulsatility of the right eye was observed. A right orbital bruit was auscultated, and he was referred to a neurointerventionalist with the tentative clinical diagnosis of traumatic CCF. Four-vessel digital subtraction angiography revealed profound dilatation of the right superior ophthalmic vein (Figure 2). A covered stent was deployed in the intracavernous ICA via a femoral approach after ethylene vinyl alcohol copolymer embolic agent was introduced into the ipsilateral CS with a microcatheter. Post-procedure angiography confirmed successful ablation of the abnormal arteriovenous connection, as well as preservation of distal arterial flow (Figure 3). On repeat examination, the patient’s visual acuity improved to 20/20 and his intraocular pressures were normal and symmetric. His motility deficits and double vision also resolved rapidly, as did his proptosis. DISCUSSION CCF is important to consider in the differential for posttraumatic proptosis. It is the result of an acquired or recanalized congenital arteriovenous communication between the ICA and the CS. The classic clinical triad of high-flow lesions consists of exophthalmos, arterialization of conjunctival blood vessels (due to ocular and orbital congestion), and a cranial bruit that diminishes with compression of the ipsilateral carotid artery.
Figure 1. Facial photograph demonstrating right-sided proptosis, moderate restriction in downgaze, and unilateral arterialization of conjunctival vessels.
Figure 2. Digital subtraction angiography demonstrating dilatation and abnormal filling of the right superior ophthalmic vein during the arterial phase.
Common associated findings include elevated intraocular pressure, visible or palpable ocular pulsations, diplopia, and reduced vision (1). Lesions are categorized according to the Barrow Classification, which is based on angiographic features. Barrow type A lesions are direct, high-flow lesions resulting from a wall defect between the ICA and CS. Barrow type B, C, and D lesions are indirect, lower-flow lesions resulting from abnormal communication between branches of the ICA or external carotid artery and the CS (2). Type A lesions account for 70% to 90% of all CCF, and occur most commonly in younger males (3). Eighty percent are seen in the context of craniofacial trauma, with an overall estimated incidence of 0.2% (3). Onset is highly variable, ranging from hours to months after trauma. They are highly associated with skull-base fractures and penetrating injuries. The remaining 20% are generally the result of spontaneous rupture of occult intracavernous ICA aneurysms or weakened atherosclerotic arteries. Direct lesions rarely close spontaneously, unlike the indirect lesions that generally represent the spontaneous recanalization of small carotid cavernous connections in the context of hypertension and atherosclerosis, childbirth, or collagen vascular disease. Natural variability of venous sinus anatomy results in several important variations in clinical presentation. Patients with widely patent superior or inferior petrosal sinuses might shunt arterial blood to the internal jugular vein, resulting in few or no ocular sequelae (‘‘white eye shunt’’). Those with limited petrosal sinus patency can
Post-Traumatic Proptosis and Diplopia
Figure 3. Preserved internal carotid artery flow after deployment of a covered stent and occlusion of the ipsilateral cavernous sinus with an embolic agent.
shunt blood to the cortical and nasal veins as well as to orbital vessels. This accounts for the 3% 5% risk of significant cerebral edema and hemorrhagic cerebrovascular accident, as well as for the 1% 2% risk of profound, potentially fatal, epistaxis (1). Orbital congestion results in increased episcleral venous pressure, accounting for increased intraocular pressure, and in mechanical restriction and ischemia of the intraocular muscles. Prolonged disease can lead to blinding glaucoma. Direct cranial nerve impairment can also occur due to compression, vascular thrombosis, or steal phenomena. The abducens nerve is the most commonly affected due to its central and unshielded position in the CS (4). Imaging can reveal proptosis, engorgement of the extraocular muscles or superior ophthalmic vein, CS enlargement, and dilatation of the leptomeningeal and cortical veins. Magnetic resonance imaging is particularly useful for identifying orbital and cerebral edema and abnormal flow voids in the CS (5). Color Doppler ultrasound can often demonstrate pulsatile flow and flow reversal within the superior ophthalmic artery (6). Digital subtraction angiography is the diagnostic gold standard and an integral component of current therapeutic modalities (5). The goal of therapy is to abolish abnormal communication between the ICA and CS, while preventing ischemia in the distribution of the distal vessels. Endovascular approaches may be arterial (via the common femoral), venous (via the femoral, jugular, common facial, or superior ophthalmic), or combined (7).
477
This therapeutic goal can be accomplished by mechanically occluding the sinus using a detachable balloon catheter, inducing thrombosis with platinum coils or embolic agents, or redirecting flow with covered stents. Balloon catheters have largely fallen out of favor due to the risk of deflation, migration, and rupture by osseous fragments (5,8). They are also difficult to pass through small ruptures. Embolic agents such as N-butyl cyanoacrylate and ethylene vinyl alcohol copolymer (OnyxÒ, Micro Therapeutics Inc., Irvine, CA) are the current therapeutic staples, but stents are gaining popularity, despite risks of thrombosis, endoleak, and the need for prolonged antiplatelet therapy (9 11). Surgical ligation of the ICA is reserved for endovascular failures due to high risk of morbidity, and requires trial balloon occlusion to assess the adequacy of collateral flow. Treatment risks include cerebral infarction, ocular ischemia, and a temporary ‘‘paradoxical effect,’’ or worsening of proptosis and ophthalmoplegia due to propagation of thrombus in the CS (12). Despite this, treatments are generally very effective and well tolerated, although motility problems are often slower to resolve than congestive symptoms. CONCLUSIONS CCF is a relatively rare but important consequence of craniofacial trauma that may manifest immediately or in a delayed fashion, and should be recognized in the urgent care context due to the risk of serious and potentially fatal sequelae if untreated. Emergency physicians should suspect this entity when confronted with a patient with unilateral exophthalmos, arterialized conjunctival vessels, and an orbital bruit. Eye movements and vision may also be compromised. When available, Doppler ultrasound is an excellent first study and permits detection of abnormal flow within the superior ophthalmic vein. Magnetic resonance imaging/magnetic resonance angiography of the brain and orbits is a lower-risk study than computed tomography angiography, although the latter is necessary for definitive therapeutic interventions.
REFERENCES 1. Miller NR. Diagnosis and management of dural carotid-cavernous sinus fistulas. Neurosurg Focus 2007;23(5):E13. 2. Barrow DL, Spector RH, Braun IF, et al. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg 1985;62:248–56. 3. Ellis JA, Goldstein H, Connolly ES Jr, et al. Carotid-cavernous fistulas. Neurosurg Focus 2012;32(5):E9. 4. Grumann AJ, Boivin-Faure L, Chapot R, et al. Ophthalmologic outcome of direct and indirect carotid cavernous fistulas. Int Ophthalmol 2012;32:153–9.
478 5. Gemmete JJ, Ansari SA, Gandhi DM. Endovascular techniques for treatment of carotid-cavernous fistula. J Neuroophthalmol 2009;29: 62–71. 6. Duan Y, Liu X, Zhou X, et al. Diagnosis and follow-up study of carotid cavernous fistulas with color Doppler ultrasonography: analysis of 33 cases. J Ultrasound Med 2005;24:739–45. 7. Wang W, Li YD, Li MH, et al. Endovascular treatment of posttraumatic direct carotid-cavernous fistulas: a single-center experience. J Clin Neurosci 2011;18:24–8. 8. Serbinenko FA. Balloon catheterization and occlusion of major cerebral vessels. J Neurosurg 1974;41:125–45.
B. P. Erickson and T. E. Johnson 9. Luo CB, Teng MMH, Chang FC, et al. Transarterial balloonassisted n-butyl-2-cyanoacrylate embolization of direct carotid cavernous fistulas. AJNR Am J Neuroradiol 2006;27:1535–40. 10. Halbach VV, Higashida RT, Barnwell SL, et al. Transarterial platinum coil embolization of carotid-cavernous fistulas. AJNR Am J Neuroradiol 1991;12:429–33. 11. Madan A, Mujic A, Daniels K, et al. Traumatic carotid-cavernous sinus fistula treated with a covered stent: report of two cases. J Neurosurg 2006;104:969–73. 12. Oishi A, Miyamoto K, Yoshimura N. Etiology of carotid cavernous fistula in Japanese. Jpn J Ophthalmol 2009;53:40–3.
