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Acute Bilateral Vision Loss

Acute Bilateral Vision Loss Case A man in his 60s with a history of untreated, indolent B-cell lymphoma diagnosed 8 years ago noted acute-onset blurred vision in both eyes. His vision progressively worsened over 2 months before he sought care. In addition to his visual symptoms, he reported an episode of shortness of breath several weeks prior to presentation, ongoing fatigue, and headaches. He had no relevant ophthalmic history. His uncorrected visual acuity was 20/80 OD and 20/50 OS without pinhole improvement. Pupils, intraocular pressure, motility, confrontation visual fields, and slitlamp examination of the anterior segment were unremarkable. Dilated examination of both eyes revealed the findings seen in Figure 1. Figure 1. View LargeDownload Fundus photographs of the right eye (A) and the left eye (B) at initial presentation with vascular tortuosity, venous dilation, and diffuse intraretinal hemorrhages in all quadrants in both eyes. Box Section Ref ID What Would You Do Next? Hypercoagulability workup (including levels of advanced protein C, antithrombin III, protein C and S, homocysteine, and anticardiolipin antibodies) Complete blood cell count, serum immunoglobulin level, serum protein electrophoresis, and serum viscosity Brain magnetic resonance imaging Fasting lipid levels, hemoglobin A1c level, and communication with primary care professional Read the Discussion Discussion Diagnosis Serum hyperviscosity syndrome What To Do Next B. Complete blood cell count, serum immunoglobulin level, serum protein electrophoresis, and serum viscosity Discussion The fundus findings were consistent with simultaneous bilateral central retinal vein occlusions, which was highly suggestive of serum hyperviscosity syndrome. Fluorescein angiogram revealed dramatically delayed arteriovenous transit with petaloid leakage in the late frames of both eyes (not shown). Optical coherence tomograph demonstrated bilateral macular edema with serous foveal detachments, worse in the right eye (Figure 2). The patient was sent urgently for laboratory studies, which revealed anemia (hemoglobin level 7.9 g/dL [to convert to grams per liter, multiply by 10]), thrombocytopenia (platelet count 87 × 103 cells/µL [to convert to × 109 cells/L, multiply by 1]), serum viscosity greater than 4.6 (normal 1.4-1.8), monoclonal IgM spike on serum protein electrophoresis with elevated kappa to lambda ratio of 19:1 (normal 3:1), and IgM level of 7467 mg/dL (normal, 56-352 mg/dL [to convert to milligrams per liter, multiply by 10]). Given his eye findings, shortness of breath, fatigue, and headaches, he was admitted for initiation of plasmapheresis for symptomatic serum hyperviscosity. A bone marrow biopsy revealed increased plasma cells, which in combination with the monoclonal IgM hyperviscosity syndrome was most consistent with lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. He started a chemotherapy regimen consisting of bortezomib, rituximab, and dexamethasone, and he received intravitreal bevacizumab in both eyes. Figure 2. View LargeDownload Optical coherence tomography of the right eye reveals macular edema and serous detachments of the fovea. Central retinal vein occlusion (CRVO) is a common cause of vision loss with a prevalence of 0.8 per 1000. Risk factors include hypertension, hyperlipidemia, diabetes, and glaucoma, and less commonly vasculitis, hypercoagulable states, and hyperviscosity.1,2 Simultaneous bilateral CRVO, however, is a very rare presentation that is associated almost exclusively with serum hyperviscosity. Serum hyperviscosity syndrome was initially described as a triad of bleeding, ocular disorders, and neurologic signs,3 and the limited differential diagnosis, including blood dyscrasias and dysproteinemias, should prompt immediate workup. Despite the low incidence of Waldenstrom macroglobulinemia, with 3.8 cases per 1 million persons per year, it is the most common cause of serum hyperviscosity syndrome owing to the large 925-kDa pentameric IgM paraprotein,4 and several cases of simultaneous bilateral CRVO have been reported.5 Multiple myeloma and blood dyscrasias, including acute myeloid leukemia and polycythemia, have also been reported to cause bilateral CRVO in the setting of elevated serum viscosity.6-8 In each of these conditions, prompt diagnosis is essential, as plasmapheresis may be indicated immediately to prevent life-threatening complications.9 As the serum viscosity decreases, the venous outflow obstruction is relieved, allowing for resolution of macular edema and improvement in vision. While the role for anti–vascular endothelial growth factor medications is well established for treating macular edema and neovascular complications of retinal vein occlusions, their efficacy in serum hyperviscosity syndrome is less clear. Nearly all of the reported cases of simultaneous CRVOs resolved with treatment of the underlying disorder, but Ratanam and colleagues10 suggest that intravitreal anti–vascular endothelial growth factor agents may be a helpful adjunctive therapy in addition to plasmapheresis and chemotherapy in refractory cases. Patient Outcome After discussion with the patient, he elected to proceed with bilateral intravitreal injections of bevacizumab despite the unclear benefit. For his serum hyperviscosity syndrome, he received a total of 4 plasmapheresis treatments in addition to chemotherapy with bortezomib, rituximab, and dexamethasone with improvement in respiratory symptoms and slow improvement in visual acuity to 20/50 OD and 20/30 OS over several months. Back to top Article Information Corresponding Author: Christopher M. Aderman, MD, University of California, San Francisco, 10 Koret Way, San Francisco, CA 94143 (christopher.aderman@ucsf.edu). Published Online: December 30, 2015. doi:10.1001/jamaophthalmol.2015.3572. Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. References 1. Kolar P. Risk factors for central and branch retinal vein occlusion. J Ophthalmol. 2014;2014:724780. doi: 10.1155/2014/724780.Google Scholar 2. Hayreh SS, Zimmerman MB, Podhajsky P. Incidence of various types of retinal vein occlusion and their recurrence and demographic characteristics. Am J Ophthalmol. 1994;117(4):429-441.PubMedGoogle ScholarCrossref 3. Fahey JL, Barth WF, Solomon A. Serum hyperviscosity syndrome. JAMA. 1965;192:464-467.PubMedGoogle ScholarCrossref 4. Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003;29(5):467-471.PubMedGoogle ScholarCrossref 5. Alexander P, Flanagan D, Rege K, Foss A, Hingorani M. Bilateral simultaneous central retinal vein occlusion secondary to hyperviscosity in Waldenstrom’s macroglobulinaemia. Eye (Lond). 2008;22(8):1089-1092.PubMedGoogle ScholarCrossref 6. Aggio FB, Cariello AJ, Almeida MS, et al. Bilateral central retinal vein occlusion associated with multiple myeloma. Ophthalmologica. 2004;218(4):283-287.PubMedGoogle ScholarCrossref 7. Tseng MY, Chen YC, Lin YY, Chu SJ, Tsai SH. Simultaneous bilateral central retinal vein occlusion as the initial presentation of acute myeloid leukemia. Am J Med Sci. 2010;339(4):387-389.PubMedGoogle ScholarCrossref 8. Govetto A, Domínguez R, Rojas L, Pereiro M, Lorente R. Bilateral and simultaneous central retinal vein occlusion in a patient with obstructive sleep apnea syndrome. Case Rep Ophthalmol. 2014;5(2):150-156.PubMedGoogle ScholarCrossref 9. Stone MJ, Bogen SA. Role of plasmapheresis in Waldenström’s macroglobulinemia. Clin Lymphoma Myeloma Leuk. 2013;13(2):238-240.PubMedGoogle ScholarCrossref 10. Ratanam M, Ngim YS, Khalidin N, Subrayan V. Intravitreal bevacizumab. Br J Haematol. 2015;170(3):431-434.PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Ophthalmology American Medical Association

Acute Bilateral Vision Loss

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Publisher
American Medical Association
Copyright
Copyright © 2016 American Medical Association. All Rights Reserved.
ISSN
2168-6165
eISSN
2168-6173
DOI
10.1001/jamaophthalmol.2015.3572
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Abstract

Case A man in his 60s with a history of untreated, indolent B-cell lymphoma diagnosed 8 years ago noted acute-onset blurred vision in both eyes. His vision progressively worsened over 2 months before he sought care. In addition to his visual symptoms, he reported an episode of shortness of breath several weeks prior to presentation, ongoing fatigue, and headaches. He had no relevant ophthalmic history. His uncorrected visual acuity was 20/80 OD and 20/50 OS without pinhole improvement. Pupils, intraocular pressure, motility, confrontation visual fields, and slitlamp examination of the anterior segment were unremarkable. Dilated examination of both eyes revealed the findings seen in Figure 1. Figure 1. View LargeDownload Fundus photographs of the right eye (A) and the left eye (B) at initial presentation with vascular tortuosity, venous dilation, and diffuse intraretinal hemorrhages in all quadrants in both eyes. Box Section Ref ID What Would You Do Next? Hypercoagulability workup (including levels of advanced protein C, antithrombin III, protein C and S, homocysteine, and anticardiolipin antibodies) Complete blood cell count, serum immunoglobulin level, serum protein electrophoresis, and serum viscosity Brain magnetic resonance imaging Fasting lipid levels, hemoglobin A1c level, and communication with primary care professional Read the Discussion Discussion Diagnosis Serum hyperviscosity syndrome What To Do Next B. Complete blood cell count, serum immunoglobulin level, serum protein electrophoresis, and serum viscosity Discussion The fundus findings were consistent with simultaneous bilateral central retinal vein occlusions, which was highly suggestive of serum hyperviscosity syndrome. Fluorescein angiogram revealed dramatically delayed arteriovenous transit with petaloid leakage in the late frames of both eyes (not shown). Optical coherence tomograph demonstrated bilateral macular edema with serous foveal detachments, worse in the right eye (Figure 2). The patient was sent urgently for laboratory studies, which revealed anemia (hemoglobin level 7.9 g/dL [to convert to grams per liter, multiply by 10]), thrombocytopenia (platelet count 87 × 103 cells/µL [to convert to × 109 cells/L, multiply by 1]), serum viscosity greater than 4.6 (normal 1.4-1.8), monoclonal IgM spike on serum protein electrophoresis with elevated kappa to lambda ratio of 19:1 (normal 3:1), and IgM level of 7467 mg/dL (normal, 56-352 mg/dL [to convert to milligrams per liter, multiply by 10]). Given his eye findings, shortness of breath, fatigue, and headaches, he was admitted for initiation of plasmapheresis for symptomatic serum hyperviscosity. A bone marrow biopsy revealed increased plasma cells, which in combination with the monoclonal IgM hyperviscosity syndrome was most consistent with lymphoplasmacytic lymphoma or Waldenstrom macroglobulinemia. He started a chemotherapy regimen consisting of bortezomib, rituximab, and dexamethasone, and he received intravitreal bevacizumab in both eyes. Figure 2. View LargeDownload Optical coherence tomography of the right eye reveals macular edema and serous detachments of the fovea. Central retinal vein occlusion (CRVO) is a common cause of vision loss with a prevalence of 0.8 per 1000. Risk factors include hypertension, hyperlipidemia, diabetes, and glaucoma, and less commonly vasculitis, hypercoagulable states, and hyperviscosity.1,2 Simultaneous bilateral CRVO, however, is a very rare presentation that is associated almost exclusively with serum hyperviscosity. Serum hyperviscosity syndrome was initially described as a triad of bleeding, ocular disorders, and neurologic signs,3 and the limited differential diagnosis, including blood dyscrasias and dysproteinemias, should prompt immediate workup. Despite the low incidence of Waldenstrom macroglobulinemia, with 3.8 cases per 1 million persons per year, it is the most common cause of serum hyperviscosity syndrome owing to the large 925-kDa pentameric IgM paraprotein,4 and several cases of simultaneous bilateral CRVO have been reported.5 Multiple myeloma and blood dyscrasias, including acute myeloid leukemia and polycythemia, have also been reported to cause bilateral CRVO in the setting of elevated serum viscosity.6-8 In each of these conditions, prompt diagnosis is essential, as plasmapheresis may be indicated immediately to prevent life-threatening complications.9 As the serum viscosity decreases, the venous outflow obstruction is relieved, allowing for resolution of macular edema and improvement in vision. While the role for anti–vascular endothelial growth factor medications is well established for treating macular edema and neovascular complications of retinal vein occlusions, their efficacy in serum hyperviscosity syndrome is less clear. Nearly all of the reported cases of simultaneous CRVOs resolved with treatment of the underlying disorder, but Ratanam and colleagues10 suggest that intravitreal anti–vascular endothelial growth factor agents may be a helpful adjunctive therapy in addition to plasmapheresis and chemotherapy in refractory cases. Patient Outcome After discussion with the patient, he elected to proceed with bilateral intravitreal injections of bevacizumab despite the unclear benefit. For his serum hyperviscosity syndrome, he received a total of 4 plasmapheresis treatments in addition to chemotherapy with bortezomib, rituximab, and dexamethasone with improvement in respiratory symptoms and slow improvement in visual acuity to 20/50 OD and 20/30 OS over several months. Back to top Article Information Corresponding Author: Christopher M. Aderman, MD, University of California, San Francisco, 10 Koret Way, San Francisco, CA 94143 (christopher.aderman@ucsf.edu). Published Online: December 30, 2015. doi:10.1001/jamaophthalmol.2015.3572. Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. References 1. Kolar P. Risk factors for central and branch retinal vein occlusion. J Ophthalmol. 2014;2014:724780. doi: 10.1155/2014/724780.Google Scholar 2. Hayreh SS, Zimmerman MB, Podhajsky P. Incidence of various types of retinal vein occlusion and their recurrence and demographic characteristics. Am J Ophthalmol. 1994;117(4):429-441.PubMedGoogle ScholarCrossref 3. Fahey JL, Barth WF, Solomon A. Serum hyperviscosity syndrome. JAMA. 1965;192:464-467.PubMedGoogle ScholarCrossref 4. Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003;29(5):467-471.PubMedGoogle ScholarCrossref 5. Alexander P, Flanagan D, Rege K, Foss A, Hingorani M. Bilateral simultaneous central retinal vein occlusion secondary to hyperviscosity in Waldenstrom’s macroglobulinaemia. Eye (Lond). 2008;22(8):1089-1092.PubMedGoogle ScholarCrossref 6. Aggio FB, Cariello AJ, Almeida MS, et al. Bilateral central retinal vein occlusion associated with multiple myeloma. Ophthalmologica. 2004;218(4):283-287.PubMedGoogle ScholarCrossref 7. Tseng MY, Chen YC, Lin YY, Chu SJ, Tsai SH. Simultaneous bilateral central retinal vein occlusion as the initial presentation of acute myeloid leukemia. Am J Med Sci. 2010;339(4):387-389.PubMedGoogle ScholarCrossref 8. Govetto A, Domínguez R, Rojas L, Pereiro M, Lorente R. Bilateral and simultaneous central retinal vein occlusion in a patient with obstructive sleep apnea syndrome. Case Rep Ophthalmol. 2014;5(2):150-156.PubMedGoogle ScholarCrossref 9. Stone MJ, Bogen SA. Role of plasmapheresis in Waldenström’s macroglobulinemia. Clin Lymphoma Myeloma Leuk. 2013;13(2):238-240.PubMedGoogle ScholarCrossref 10. Ratanam M, Ngim YS, Khalidin N, Subrayan V. Intravitreal bevacizumab. Br J Haematol. 2015;170(3):431-434.PubMedGoogle ScholarCrossref

Journal

JAMA Ophthalmology – American Medical Association

Published: Feb 1, 2016

Keywords: diagnostic techniques, ophthalmological,hyperviscosity syndrome,lymphoma,plasmapheresis,retinal vein occlusion,b-lymphocytes,blood tests,fundus photography,optical coherence tomography,eye,blurry vision

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