Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
Acute Postoperative Visual Loss Daniel S. Churgin, MD; Norman J. Schatz, MD; Daniel L. Chao, MD, PhD
A
B
Figure 1. Case presentation. A, Fundus photograph of right eye showing diffuse retinal whitening with a cherry-red spot. B, Immediate postoperative magnetic resonance imaging showing enlarged extraocular muscles on the right side (arrowheads).
A 50-year-old healthy woman underwent an uncomplicated elective clipping of a right middle cerebral artery aneurysm via pterional craniotomy. Ophthalmology was consulted urgently in the recovery room because of complaints of vision loss and ophthalmoplegia in the right eye. On examination, visual acuity was no light perception (NLP) OD and 20/20 OS. Pupils were equal and round, with a 3+ relative afferent pupillary defect in the right eye. Intraocular pressure was within normal limits in both eyes. There was Quiz at jamaophthalmology.com complete ophthalmoplegia in the right eye with no signs of restriction on forced ductions and normal motility in the left eye. Visual field was full to confrontation in the left eye. The right eye also demonstrated mild fullness of the orbit, right cheek skin erythema, trace proptosis, and trace conjunctival chemosis. Posterior examination was significant for diffuse retinal whitening with a cherry-red spot in the macula in the right eye (Figure 1A). There were no other neurologic deficits identified on examination. A computed tomography scan without contrast did not show an acute intracranial hemorrhage and demonstrated diffuse enlargement of the right extraocular muscles. Magnetic resonance imaging of the brain and orbits with contrast again showed enlargement and abnormal signal of the extraocular muscles on the right (Figure 1B); there was no evidence of cavernous sinus pathology.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Immediate surgical re-exploration B. Emergent angiography C. Immediate anticoagulation D. Lateral canthotomy and cantholysis
JAMA Ophthalmology November 2014 Volume 132, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 06/01/2015
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Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Orbital infarction syndrome
What To Do Next? B. Emergent angiography In the setting of sudden painless vision loss with ophthalmoplegia and extraocular muscle enlargement immediately after craniotomy, one would be concerned about acute occlusion of the ophthalmic artery and its branches. Although computed tomography and magnetic resonance imaging did not show any gross hemorrhage, further neuroimaging with angiography to evaluate the status of the aneurysm clipping, as well as the state of the ophthalmic artery circulation, would be the most appropriate next step. As the intraocular pressure was within normal limits and the patient had negative forced ductions, there was no suggestion of an orbital compartment syndrome that would necessitate a lateral canthotomy and cantholysis at this time. Surgical exploration would not be warranted because of a lack of clear etiology. There was no indication for anticoagulation at this time.
Discussion The triad of sudden painless severe visual loss, complete ophthalmoplegia, and a fundus examination consistent with a central retinal artery occlusion (CRAO) is highly suggestive of orbital infarction syndrome.1 Orbital infarction results in retinal infarction, choroidal ischemia, and ophthalmoplegia. Ophthalmic artery occlusion alone cannot cause these symptoms because of the extensive anastomoses between the ophthalmic artery and external carotid artery. A
Orbital infarction syndrome has been reported in the setting of acute hypoperfusion, systemic vasculitides such as giant cell arteritis, and extensive thrombosis secondary to mucormycosis.1 All cases had acharacteristicconstellationofsymptomsincludingseverevisionloss— oftenNLP—completeophthalmoplegia,proptosisororbitalcongestion, andafundusexaminationconsistentwithaCRAO.Orbitalinfarctionsyndrome has been reported postoperatively after intracranial aneurysm clipping and spinal surgery as well as due to orbital cellulitis after transcanalicular dacryocystorhinostomy.2-4 The mechanism for postsurgical orbital infarction syndrome is unclear. A myocutaneous flap is raised in these surgeries for the craniotomy, which may cause indirect or direct pressure on the orbits, resulting in hypoperfusion.2 Other factors speculatedtoplayaroleareincreasedintracranialpressureandsystemic hypotension, which may cause relative hypoperfusion to the orbit.
Patient Outcome Orbital infarction syndrome was likely due to a combination of these factors. Angiography showed intact clipping of the aneurysm as well as patent flow through the ophthalmic artery and its branches (Figure 2A). This suggests the ischemic event must have been transient, though sustained long enough to cause permanent damage. Patients with orbital infarction syndrome can also develop a delayed orbital compartment syndrome. Twelve hours after surgery, our patient had increased proptosis and intraocular ocular pressure of 34 mm Hg, and a lateral canthotomy and cantholysis were performed. On follow-up at 2 weeks, the ophthalmoplegia and orbital congestion had resolved but vision remained NLP. Fundus examination demonstrated a pale nerve, attenuated vessels, and diffuse retinal pigment epithelium clumping (Figure 2B).
B
Figure 2. Postoperative imaging of orbital infarction syndrome. A, Postoperative angiography of patient. Note successful clipping of middle cerebral artery aneurysm (white arrowhead) as well as patent flow through the ophthalmic artery (black arrowhead). B, Fundus photograph 2 weeks after orbital infarction syndrome. Note pale disc, attenuated vessels, and diffuse retinal pigment clumping.
ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology, University of Miami, Bascom Palmer Eye Institute, Miami, Florida. Corresponding Author: Daniel L. Chao, MD, PhD, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17 St, Miami, FL 33136 ([email protected]). Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
Funding/Support: This work was supported in part by Florida Lions Eye Bank, National Institutes of Health Center Core Grant P30EY014801, a Research to Prevent Blindness unrestricted grant, and Department of Defense grant W81XWH-09-1-0675. Role of the Funder/Sponsor: The funding agencies had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. REFERENCES
2. Zimmerman CF, Van Patten PD, Golnik KC, Kopitnik TA Jr, Anand R. Orbital infarction syndrome after surgery for intracranial aneurysms. Ophthalmology. 1995;102(4):594-598. 3. Morofuji Y, Tsutsumi K, Takahata H, et al. Radiological findings of orbital infarction syndrome following intracranial aneurysm surgery. Clin Neurol Neurosurg. 2013;115(8):1546-1548. 4. Yeniad B, Tuncer S, Kir N, Bilgin LK. Orbital infarction syndrome after transcanalicular dacryocystorhinostomy with diode laser. Ophthalmic Surg Lasers Imaging. 2012;43 Online:e107-e109.
1. Borruat FX, Bogousslavsky J, Uffer S, Klainguti G, Schatz NJ. Orbital infarction syndrome. Ophthalmology. 1993;100(4):562-568.
1366
JAMA Ophthalmology November 2014 Volume 132, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 06/01/2015
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
Acute Postoperative Visual Loss Daniel S. Churgin, MD; Norman J. Schatz, MD; Daniel L. Chao, MD, PhD
A
B
Figure 1. Case presentation. A, Fundus photograph of right eye showing diffuse retinal whitening with a cherry-red spot. B, Immediate postoperative magnetic resonance imaging showing enlarged extraocular muscles on the right side (arrowheads).
A 50-year-old healthy woman underwent an uncomplicated elective clipping of a right middle cerebral artery aneurysm via pterional craniotomy. Ophthalmology was consulted urgently in the recovery room because of complaints of vision loss and ophthalmoplegia in the right eye. On examination, visual acuity was no light perception (NLP) OD and 20/20 OS. Pupils were equal and round, with a 3+ relative afferent pupillary defect in the right eye. Intraocular pressure was within normal limits in both eyes. There was Quiz at jamaophthalmology.com complete ophthalmoplegia in the right eye with no signs of restriction on forced ductions and normal motility in the left eye. Visual field was full to confrontation in the left eye. The right eye also demonstrated mild fullness of the orbit, right cheek skin erythema, trace proptosis, and trace conjunctival chemosis. Posterior examination was significant for diffuse retinal whitening with a cherry-red spot in the macula in the right eye (Figure 1A). There were no other neurologic deficits identified on examination. A computed tomography scan without contrast did not show an acute intracranial hemorrhage and demonstrated diffuse enlargement of the right extraocular muscles. Magnetic resonance imaging of the brain and orbits with contrast again showed enlargement and abnormal signal of the extraocular muscles on the right (Figure 1B); there was no evidence of cavernous sinus pathology.
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Immediate surgical re-exploration B. Emergent angiography C. Immediate anticoagulation D. Lateral canthotomy and cantholysis
JAMA Ophthalmology November 2014 Volume 132, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 06/01/2015
1365
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Orbital infarction syndrome
What To Do Next? B. Emergent angiography In the setting of sudden painless vision loss with ophthalmoplegia and extraocular muscle enlargement immediately after craniotomy, one would be concerned about acute occlusion of the ophthalmic artery and its branches. Although computed tomography and magnetic resonance imaging did not show any gross hemorrhage, further neuroimaging with angiography to evaluate the status of the aneurysm clipping, as well as the state of the ophthalmic artery circulation, would be the most appropriate next step. As the intraocular pressure was within normal limits and the patient had negative forced ductions, there was no suggestion of an orbital compartment syndrome that would necessitate a lateral canthotomy and cantholysis at this time. Surgical exploration would not be warranted because of a lack of clear etiology. There was no indication for anticoagulation at this time.
Discussion The triad of sudden painless severe visual loss, complete ophthalmoplegia, and a fundus examination consistent with a central retinal artery occlusion (CRAO) is highly suggestive of orbital infarction syndrome.1 Orbital infarction results in retinal infarction, choroidal ischemia, and ophthalmoplegia. Ophthalmic artery occlusion alone cannot cause these symptoms because of the extensive anastomoses between the ophthalmic artery and external carotid artery. A
Orbital infarction syndrome has been reported in the setting of acute hypoperfusion, systemic vasculitides such as giant cell arteritis, and extensive thrombosis secondary to mucormycosis.1 All cases had acharacteristicconstellationofsymptomsincludingseverevisionloss— oftenNLP—completeophthalmoplegia,proptosisororbitalcongestion, andafundusexaminationconsistentwithaCRAO.Orbitalinfarctionsyndrome has been reported postoperatively after intracranial aneurysm clipping and spinal surgery as well as due to orbital cellulitis after transcanalicular dacryocystorhinostomy.2-4 The mechanism for postsurgical orbital infarction syndrome is unclear. A myocutaneous flap is raised in these surgeries for the craniotomy, which may cause indirect or direct pressure on the orbits, resulting in hypoperfusion.2 Other factors speculatedtoplayaroleareincreasedintracranialpressureandsystemic hypotension, which may cause relative hypoperfusion to the orbit.
Patient Outcome Orbital infarction syndrome was likely due to a combination of these factors. Angiography showed intact clipping of the aneurysm as well as patent flow through the ophthalmic artery and its branches (Figure 2A). This suggests the ischemic event must have been transient, though sustained long enough to cause permanent damage. Patients with orbital infarction syndrome can also develop a delayed orbital compartment syndrome. Twelve hours after surgery, our patient had increased proptosis and intraocular ocular pressure of 34 mm Hg, and a lateral canthotomy and cantholysis were performed. On follow-up at 2 weeks, the ophthalmoplegia and orbital congestion had resolved but vision remained NLP. Fundus examination demonstrated a pale nerve, attenuated vessels, and diffuse retinal pigment epithelium clumping (Figure 2B).
B
Figure 2. Postoperative imaging of orbital infarction syndrome. A, Postoperative angiography of patient. Note successful clipping of middle cerebral artery aneurysm (white arrowhead) as well as patent flow through the ophthalmic artery (black arrowhead). B, Fundus photograph 2 weeks after orbital infarction syndrome. Note pale disc, attenuated vessels, and diffuse retinal pigment clumping.
ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology, University of Miami, Bascom Palmer Eye Institute, Miami, Florida. Corresponding Author: Daniel L. Chao, MD, PhD, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17 St, Miami, FL 33136 ([email protected]). Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.
Funding/Support: This work was supported in part by Florida Lions Eye Bank, National Institutes of Health Center Core Grant P30EY014801, a Research to Prevent Blindness unrestricted grant, and Department of Defense grant W81XWH-09-1-0675. Role of the Funder/Sponsor: The funding agencies had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. REFERENCES
2. Zimmerman CF, Van Patten PD, Golnik KC, Kopitnik TA Jr, Anand R. Orbital infarction syndrome after surgery for intracranial aneurysms. Ophthalmology. 1995;102(4):594-598. 3. Morofuji Y, Tsutsumi K, Takahata H, et al. Radiological findings of orbital infarction syndrome following intracranial aneurysm surgery. Clin Neurol Neurosurg. 2013;115(8):1546-1548. 4. Yeniad B, Tuncer S, Kir N, Bilgin LK. Orbital infarction syndrome after transcanalicular dacryocystorhinostomy with diode laser. Ophthalmic Surg Lasers Imaging. 2012;43 Online:e107-e109.
1. Borruat FX, Bogousslavsky J, Uffer S, Klainguti G, Schatz NJ. Orbital infarction syndrome. Ophthalmology. 1993;100(4):562-568.
1366
JAMA Ophthalmology November 2014 Volume 132, Number 11
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of California - San Diego User on 06/01/2015
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