CENTRAL RETINAL ARTERY OCCLUSION OCCURRING INTRAOPERATIVELY DURING ANGIOGRAPHY FOR SUBARACHNOID HEMORRHAGE: A CASE REPORT Michael D. Greenwood, MD, Shawn C. Wilker, MD
Purpose: To report a case of central retinal artery occlusion that occurred during cerebral angiography for the treatment of vasospasm secondary to subarachnoid hemorrhage. Results: Examination was notable for 20/Hand Motion vision and an afferent pupillary defect. Fundus examination at the time of the event revealed a cherry red spot typical of central retinal artery occlusion with surrounding edema of the retina and severe attenuation of the arterioles. There were no emboli noted. Review of the angiography confirmed flow to the ophthalmic artery and retina at the start of the procedure, with severely decreased flow noted at the end of the procedure. Five days after presentation, there were notable areas of potential choroidal infarction, indicating that the ophthalmic artery was also involved to some extent. The patient refused fluorescein angiography, which could have provided more insight to the extent of ischemia. Optical coherence tomography also showed diffuse retinal edema. She was monitored for complications resulting from this event with visual acuity remaining largely unchanged. Conclusion: Ophthalmic and central retinal artery occlusions are devastating events that result in severe vision loss. Most patients have some underlying risk factors precluding the initial event. Undergoing angiography, or other invasive vascular procedure, may put the patient at risk for distal vascular occlusions. Our case is unique in that it shows intraoperative imaging of the acute event occurrence. RETINAL CASES & BRIEF REPORTS 8:345–347, 2014
is a risk factor for CRAO. It is also a major risk factor for complications during angiography.2 We present a case of sudden vision loss after angiography.
From the Department of Ophthalmology and Visual Sciences, Case Western Reserve University School of Medicine, University Hospitals Case Medical Center, Cleveland, Ohio.
I
n 1859, von Graefes1 described central retinal artery occlusion (CRAO) as an embolic event to the central retina artery in a patient with endocarditis. This was one of the first descriptions of CRAO. Atherosclerosis
Case Report A 44-year-old African American woman who is otherwise healthy presented to her primary care provider with sudden onset headache. She was quickly diagnosed with a left sided subarachnoid hemorrhage (SAH) and admitted for further management. Subsequent imaging showed the SAH arising from a middle cerebral aneurysm and also a right internal carotid artery (ICA) aneurysm. The right ICA was 100% patent. The left ICA was 60% patent with atherosclerotic disease that extended to the supraclinoid, ophthalmic, and communicating segments. This was thought to be not hemodynamically significant. On hospital day 5, she underwent clipping of the aneurysm and postoperatively
None of the authors have any financial/conflicting interests to disclose. Reprint requests: Michael D. Greenwood, MD, Department of Ophthalmology and Visual Sciences, University Hospital of Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106-5068; e-mail: [email protected]
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developed right-sided weakness and aphasia. On hospital day 6, she underwent angiography for diagnosis and treatment of vasospasm resulting from the SAH, with some improvement in her symptoms. On hospital day 8 she again underwent angiography for vasospasm. When she returned from the procedure she noted severe vision loss in her left eye. Examination showed visual acuity 20/20 in the right eye and 20/ Hand Motion in the left eye. There was an afferent papillary defect in the left eye. The intraocular pressure was 18 in both eyes. Examination in the right eye was unremarkable including dilated fundus exam (DFE). External and portable slit-lamp exam in the left eye showed mild ptosis. Dilated fundus exam in the left eye showed a pale posterior pole with attenuated arterioles and a “cherry-red spot.” At this time, the diagnosis of CRAO was made. Fundus photographs taken 5 days after the inciting event are seen in Figure 1, with findings similar to what was seen at presentation, stated above. The angiography images were reviewed and are shown in Figure 2, A and B. The image in Figure 2A shows angiography of the left ICA and its tributaries before administration of verapamil. Note the perfusion of the ophthalmic artery and outline of the posterior globe. There is diffuse vasospasm present throughout the cerebrum. Figure 2B shows angiography after administration of the verapamil, approximately 20 minutes later. Note that the cerebral vasospasm has improved and the cerebrum is now perfused. The ophthalmic artery is now minimally perfused, and the outline of the posterior globe is difficult to identify. The patient was treated with intraocular pressure–lowering drops and ocular-digital massage. Additional workup including echocardiogram and electrocardiogram were unremarkable. The patient refused fluorescein angiography on subsequent follow-up visits.
Discussion Vasospasm is a serious complication of SAH that can cause ischemic brain injury. Approximately one third of patients with SAH will develop vasospasm and up to half of them will have permanent damage as a result.3 Current treatment includes using oral calcium channel blockers
Fig. 1. Fundus photography of the left eye taken 5 days after the initial event. Note the opaque edematous retinal tissue with the “cherry red spot.” There are also areas of possible choroidal infarction throughout the posterior pole.
for prevention. If these fail to prevent or relieve vasospasm, angiography with administration of verapamil can be used.4 Angiography has its own risks, including neurologic damage from microemboli. This risk is increased in patients with known atherosclerotic disease.2 Central retinal artery occlusion (CRAO) is an ocular emergency and is analogous to an acute stroke. The incidence is estimated to be 1 in 100,000 people.5 Most patients with CRAO undergo profound monocular visual acuity loss, 20/400 or worse.6 There are many underlying conditions that can place patients at risk for CRAO, but atherosclerotic disease is a frequent contributor. Other common causes of emboli induced CRAO include atrial fibrillation, endocarditis, and hypercoagulable states. Giant cell arteritis can also cause a CRAO and must be included in the differential diagnosis.7 Our case has intraoperative imaging that shows decreased perfusion to the ophthalmic artery and the posterior pole. As stated above, Figure 2A shows angiography of the left ICA and its tributaries before the administration of verapamil. There is good perfusion of the ophthalmic artery, whereas diffuse vasospasm is present throughout the cerebrum. Verapamil was given, and then the attention was turned to the right ICA. After 20 minutes, the attention returned to the left side to see the effects of the verapamil. As one would expect, the vasospasm is decreased and the cerebrum is now better perfused (Figure 2B). The ophthalmic artery, however, is now minimally perfused and the outline of the posterior globe is barely identifiable. This decrease in perfusion of the ophthalmic artery was captured throughout the entire angiogram sequence (approximately 2 minutes), whereas the cerebral perfusion continued to be patent. These findings likely suggest thromboembolic disease, rather than localized vasospasm of the ophthalmic artery because the effects of verapamil can be seen throughout the rest of the cerebrum. Unfortunately, the effects of the verapamil were not able to completely overcome the blockage of blood flow caused by the probable embolus. The fundus photographs in Figure 1 are typical of a CRAO, showing the classic “cherry-red spot” against a pale posterior pole and attenuated arterioles. No emboli were ever identified on our exam. Although emboli can be present anywhere along the pathway, it has been hypothesized that the narrowest part of the CRA lumen is where it pierces the dural sheath and that this is the site where many CRAOs occur.8 Once the occlusion has occurred, the ability of the retina to recover depends on whether the blockage has been removed and how quickly. It is thought that removal of the embolus within 90 minutes gives the best chance at recovery, but irreversible damage occurs after 4 hours or possibly sooner.9
347
CRAO DURING ANGIOGRAPHY FOR SAH
Fig. 2. A. Angiography of the left internal carotid artery and associated circulation. Note the prominent flow through the ophthalmic artery (black arrow) and distally providing good choroidal flow and outlining the posterior globe (white arrow). There is also significant vasospasm present throughout the angiogram. B. Angiography of the left internal carotid artery and associated circulation. This imaging was taken 20 minutes later, after verapamil treatment and imaging the right internal carotid artery circulation. Note the decreased flow in the ophthalmic artery (short white arrow) and loss of the posterior globe circulation and outline. There is increased flow throughout the rest of the angiogram.
Management of CRAO includes prompt diagnosis and is divided into 3 categories: acute, subacute, and long-term. Acute management focuses on restoring ocular perfusion to the CRA. Subacute is targeted at preventing secondary neovascular complications to the eye. The long-term management includes preventing other vascular ischemic events to the eye and other end organs.7 There have been many suggestions for acute treatment of a CRAO. Treatment measures include increasing blood oxygen content and dilation of retinal arteries, ocular massage, reducing intraocular pressure through medications and anterior chamber paracentesis, and the use of thrombolytics. As of now, there is no consensus on the most effective treatment approach but many physicians will combine many of the above treatments to give the best opportunity for recovering vision.10 The subacute management requires regular follow-up to detect neovascularization. The prevalence ranges from 2.5 to 31.6%, with the average presentation at 8 weeks after the initial incident. Patients with an ophthalmic artery occlusion have a greater risk of neovascularization because of the greater ischemic insult that takes place. Long-term management needs to address the systemic atherosclerotic risk factors and identify any sources of emboli. A proper workup includes appropriate imaging and laboratory work should be undertaken, with modification of any risks factors including diet. The patient in this case had known atherosclerotic disease. This is a risk factor for embolus production during angiography and a risk factor for CRAO. The imaging that is provided in this case gives a unique look at occlusive disease affecting the retina in real time. Conclusion Atherosclerosis is a relatively common disease among our population. It is also a risk factor for
CRAO and complications from angiography. Our case demonstrates angiographic evidence of occlusive disease likely from an embolic source that occurred during the procedure. Timely diagnosis and treatment is essential to give the best chance at vision recovery and prevention of future events. Key words: angiography, central retinal artery occlusion, complications. References 1. von Graefes A. Ueber Embolie der Arteria centralis retinae als Ursache plotzlicher Erblindung. Arch Ophthalmol 1859;5:136–157. 2. Davies KN, Humphrey PR. Complications of cerebral angiography in patients with symptomatic carotid territory ischaemia screened by carotid ultrasound. J Neurol Neurosurg Psychiatry 1993;56:967–972. 3. Dorhout Mees SM, Rinkel GJ, Feigin VL, et al. Calcium antagonists for aneurysmal subarachnoid haemorrhage. In: Rinkel GJE, ed. Cochrane Database of Systematic Reviews (Online). Vol 3:CD000277; 2007. 4. Suarez JI, Tarr RW, Selman WR. Aneurysmal subarachnoid hemorrhage. New Engl J Med 2006;354:387–396. 5. Leavitt JA, Larson TA, Hodge DO, Gullerud RE. The incidence of central retinal artery occlusion in Olmsted County, Minnesota. Am J Ophthalmol 2011;152:820–823. 6. Hayreh SS, Zimmerman MB. Central retinal artery occlusion: visual outcome. Am J Ophthalmol 2005;140:376–391. 7. Varma DD, Cugati S, Lee AW, Chen CS. A review of central retinal artery occlusion. Eye (Lond) 2013;27:688–697. 8. Hayreh SS. Acute retinal arterial occlusive disorders. Prog Retin Eye Res 2011;30:359–394. 9. Hayreh SS, Kolder HE, Weingeist TA. Central retinal artery occlusion and retinal tolerance time. Ophthalmology 1980;87:75–78. 10. Cugati S, Varma DD, Chen CS, Lee AW. Treatment options for central retinal artery occlusion. Curr Treat Options Neurol 2013;15:63–77.
Purpose: To report a case of central retinal artery occlusion that occurred during cerebral angiography for the treatment of vasospasm secondary to subarachnoid hemorrhage. Results: Examination was notable for 20/Hand Motion vision and an afferent pupillary defect. Fundus examination at the time of the event revealed a cherry red spot typical of central retinal artery occlusion with surrounding edema of the retina and severe attenuation of the arterioles. There were no emboli noted. Review of the angiography confirmed flow to the ophthalmic artery and retina at the start of the procedure, with severely decreased flow noted at the end of the procedure. Five days after presentation, there were notable areas of potential choroidal infarction, indicating that the ophthalmic artery was also involved to some extent. The patient refused fluorescein angiography, which could have provided more insight to the extent of ischemia. Optical coherence tomography also showed diffuse retinal edema. She was monitored for complications resulting from this event with visual acuity remaining largely unchanged. Conclusion: Ophthalmic and central retinal artery occlusions are devastating events that result in severe vision loss. Most patients have some underlying risk factors precluding the initial event. Undergoing angiography, or other invasive vascular procedure, may put the patient at risk for distal vascular occlusions. Our case is unique in that it shows intraoperative imaging of the acute event occurrence. RETINAL CASES & BRIEF REPORTS 8:345–347, 2014
is a risk factor for CRAO. It is also a major risk factor for complications during angiography.2 We present a case of sudden vision loss after angiography.
From the Department of Ophthalmology and Visual Sciences, Case Western Reserve University School of Medicine, University Hospitals Case Medical Center, Cleveland, Ohio.
I
n 1859, von Graefes1 described central retinal artery occlusion (CRAO) as an embolic event to the central retina artery in a patient with endocarditis. This was one of the first descriptions of CRAO. Atherosclerosis
Case Report A 44-year-old African American woman who is otherwise healthy presented to her primary care provider with sudden onset headache. She was quickly diagnosed with a left sided subarachnoid hemorrhage (SAH) and admitted for further management. Subsequent imaging showed the SAH arising from a middle cerebral aneurysm and also a right internal carotid artery (ICA) aneurysm. The right ICA was 100% patent. The left ICA was 60% patent with atherosclerotic disease that extended to the supraclinoid, ophthalmic, and communicating segments. This was thought to be not hemodynamically significant. On hospital day 5, she underwent clipping of the aneurysm and postoperatively
None of the authors have any financial/conflicting interests to disclose. Reprint requests: Michael D. Greenwood, MD, Department of Ophthalmology and Visual Sciences, University Hospital of Case Western Reserve University, 11100 Euclid Avenue, Cleveland, OH 44106-5068; e-mail: [email protected]
345
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RETINAL CASES & BRIEF REPORTS´ 2014 VOLUME 8 NUMBER 4
developed right-sided weakness and aphasia. On hospital day 6, she underwent angiography for diagnosis and treatment of vasospasm resulting from the SAH, with some improvement in her symptoms. On hospital day 8 she again underwent angiography for vasospasm. When she returned from the procedure she noted severe vision loss in her left eye. Examination showed visual acuity 20/20 in the right eye and 20/ Hand Motion in the left eye. There was an afferent papillary defect in the left eye. The intraocular pressure was 18 in both eyes. Examination in the right eye was unremarkable including dilated fundus exam (DFE). External and portable slit-lamp exam in the left eye showed mild ptosis. Dilated fundus exam in the left eye showed a pale posterior pole with attenuated arterioles and a “cherry-red spot.” At this time, the diagnosis of CRAO was made. Fundus photographs taken 5 days after the inciting event are seen in Figure 1, with findings similar to what was seen at presentation, stated above. The angiography images were reviewed and are shown in Figure 2, A and B. The image in Figure 2A shows angiography of the left ICA and its tributaries before administration of verapamil. Note the perfusion of the ophthalmic artery and outline of the posterior globe. There is diffuse vasospasm present throughout the cerebrum. Figure 2B shows angiography after administration of the verapamil, approximately 20 minutes later. Note that the cerebral vasospasm has improved and the cerebrum is now perfused. The ophthalmic artery is now minimally perfused, and the outline of the posterior globe is difficult to identify. The patient was treated with intraocular pressure–lowering drops and ocular-digital massage. Additional workup including echocardiogram and electrocardiogram were unremarkable. The patient refused fluorescein angiography on subsequent follow-up visits.
Discussion Vasospasm is a serious complication of SAH that can cause ischemic brain injury. Approximately one third of patients with SAH will develop vasospasm and up to half of them will have permanent damage as a result.3 Current treatment includes using oral calcium channel blockers
Fig. 1. Fundus photography of the left eye taken 5 days after the initial event. Note the opaque edematous retinal tissue with the “cherry red spot.” There are also areas of possible choroidal infarction throughout the posterior pole.
for prevention. If these fail to prevent or relieve vasospasm, angiography with administration of verapamil can be used.4 Angiography has its own risks, including neurologic damage from microemboli. This risk is increased in patients with known atherosclerotic disease.2 Central retinal artery occlusion (CRAO) is an ocular emergency and is analogous to an acute stroke. The incidence is estimated to be 1 in 100,000 people.5 Most patients with CRAO undergo profound monocular visual acuity loss, 20/400 or worse.6 There are many underlying conditions that can place patients at risk for CRAO, but atherosclerotic disease is a frequent contributor. Other common causes of emboli induced CRAO include atrial fibrillation, endocarditis, and hypercoagulable states. Giant cell arteritis can also cause a CRAO and must be included in the differential diagnosis.7 Our case has intraoperative imaging that shows decreased perfusion to the ophthalmic artery and the posterior pole. As stated above, Figure 2A shows angiography of the left ICA and its tributaries before the administration of verapamil. There is good perfusion of the ophthalmic artery, whereas diffuse vasospasm is present throughout the cerebrum. Verapamil was given, and then the attention was turned to the right ICA. After 20 minutes, the attention returned to the left side to see the effects of the verapamil. As one would expect, the vasospasm is decreased and the cerebrum is now better perfused (Figure 2B). The ophthalmic artery, however, is now minimally perfused and the outline of the posterior globe is barely identifiable. This decrease in perfusion of the ophthalmic artery was captured throughout the entire angiogram sequence (approximately 2 minutes), whereas the cerebral perfusion continued to be patent. These findings likely suggest thromboembolic disease, rather than localized vasospasm of the ophthalmic artery because the effects of verapamil can be seen throughout the rest of the cerebrum. Unfortunately, the effects of the verapamil were not able to completely overcome the blockage of blood flow caused by the probable embolus. The fundus photographs in Figure 1 are typical of a CRAO, showing the classic “cherry-red spot” against a pale posterior pole and attenuated arterioles. No emboli were ever identified on our exam. Although emboli can be present anywhere along the pathway, it has been hypothesized that the narrowest part of the CRA lumen is where it pierces the dural sheath and that this is the site where many CRAOs occur.8 Once the occlusion has occurred, the ability of the retina to recover depends on whether the blockage has been removed and how quickly. It is thought that removal of the embolus within 90 minutes gives the best chance at recovery, but irreversible damage occurs after 4 hours or possibly sooner.9
347
CRAO DURING ANGIOGRAPHY FOR SAH
Fig. 2. A. Angiography of the left internal carotid artery and associated circulation. Note the prominent flow through the ophthalmic artery (black arrow) and distally providing good choroidal flow and outlining the posterior globe (white arrow). There is also significant vasospasm present throughout the angiogram. B. Angiography of the left internal carotid artery and associated circulation. This imaging was taken 20 minutes later, after verapamil treatment and imaging the right internal carotid artery circulation. Note the decreased flow in the ophthalmic artery (short white arrow) and loss of the posterior globe circulation and outline. There is increased flow throughout the rest of the angiogram.
Management of CRAO includes prompt diagnosis and is divided into 3 categories: acute, subacute, and long-term. Acute management focuses on restoring ocular perfusion to the CRA. Subacute is targeted at preventing secondary neovascular complications to the eye. The long-term management includes preventing other vascular ischemic events to the eye and other end organs.7 There have been many suggestions for acute treatment of a CRAO. Treatment measures include increasing blood oxygen content and dilation of retinal arteries, ocular massage, reducing intraocular pressure through medications and anterior chamber paracentesis, and the use of thrombolytics. As of now, there is no consensus on the most effective treatment approach but many physicians will combine many of the above treatments to give the best opportunity for recovering vision.10 The subacute management requires regular follow-up to detect neovascularization. The prevalence ranges from 2.5 to 31.6%, with the average presentation at 8 weeks after the initial incident. Patients with an ophthalmic artery occlusion have a greater risk of neovascularization because of the greater ischemic insult that takes place. Long-term management needs to address the systemic atherosclerotic risk factors and identify any sources of emboli. A proper workup includes appropriate imaging and laboratory work should be undertaken, with modification of any risks factors including diet. The patient in this case had known atherosclerotic disease. This is a risk factor for embolus production during angiography and a risk factor for CRAO. The imaging that is provided in this case gives a unique look at occlusive disease affecting the retina in real time. Conclusion Atherosclerosis is a relatively common disease among our population. It is also a risk factor for
CRAO and complications from angiography. Our case demonstrates angiographic evidence of occlusive disease likely from an embolic source that occurred during the procedure. Timely diagnosis and treatment is essential to give the best chance at vision recovery and prevention of future events. Key words: angiography, central retinal artery occlusion, complications. References 1. von Graefes A. Ueber Embolie der Arteria centralis retinae als Ursache plotzlicher Erblindung. Arch Ophthalmol 1859;5:136–157. 2. Davies KN, Humphrey PR. Complications of cerebral angiography in patients with symptomatic carotid territory ischaemia screened by carotid ultrasound. J Neurol Neurosurg Psychiatry 1993;56:967–972. 3. Dorhout Mees SM, Rinkel GJ, Feigin VL, et al. Calcium antagonists for aneurysmal subarachnoid haemorrhage. In: Rinkel GJE, ed. Cochrane Database of Systematic Reviews (Online). Vol 3:CD000277; 2007. 4. Suarez JI, Tarr RW, Selman WR. Aneurysmal subarachnoid hemorrhage. New Engl J Med 2006;354:387–396. 5. Leavitt JA, Larson TA, Hodge DO, Gullerud RE. The incidence of central retinal artery occlusion in Olmsted County, Minnesota. Am J Ophthalmol 2011;152:820–823. 6. Hayreh SS, Zimmerman MB. Central retinal artery occlusion: visual outcome. Am J Ophthalmol 2005;140:376–391. 7. Varma DD, Cugati S, Lee AW, Chen CS. A review of central retinal artery occlusion. Eye (Lond) 2013;27:688–697. 8. Hayreh SS. Acute retinal arterial occlusive disorders. Prog Retin Eye Res 2011;30:359–394. 9. Hayreh SS, Kolder HE, Weingeist TA. Central retinal artery occlusion and retinal tolerance time. Ophthalmology 1980;87:75–78. 10. Cugati S, Varma DD, Chen CS, Lee AW. Treatment options for central retinal artery occlusion. Curr Treat Options Neurol 2013;15:63–77.
