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Blurry Vision in a Patient With Leukemia

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 57-year-old man with chronic myelogenous leukemia (CML) refractory to multiple tyrosine kinase inhibitors (ie, imatinib, nilotinib, and bosutinib) presented for subacute-onset blurry vision in both eyes. He had been admitted for an upcoming bone marrow transplant. The patient had floaters without flashes and denied any eye pain. A review of symptoms found chronic fatigue and a new, mild headache beginning a few days prior. On examination, his visual acuity was 20/50 OU; intraocular pressures were 15 mm Hg OD and 13 mm Hg OS; and pupils were equal, round, and reactive, without a relative afferent pupillary defect. Extraocular movements, confrontational visual fields, and Ishihara color plates were full. An anterior segment examination had normal results. A fundus examination revealed clinically significant peripapillary intraretinal and preretinal hemorrhages obscuring the optic disc margins of both eyes (Figure 1). A peripheral retinal examination of both eyes had normal results.

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Terson syndrome in a patient with CML with central nervous system involvement

C. Magnetic resonance imaging of the brain and orbits

This patient, who had refractory CML and blurry vision, presented with bilateral peripapillary hemorrhages and whitening suggestive of possible optic nerve edema. His presentation was concerning for leukemic optic nerve infiltration. Also considered were hypertensive papillopathy, an intracranial mass with intracranial hypertension, an intracranial hemorrhage with Terson syndrome, and inflammatory or infectious sources of bilateral atypical optic neuritis. Urgent magnetic resonance imaging of the brain and orbits was ordered (choice C), and high-dose intravenous methylprednisolone was started. Magnetic resonance imaging revealed subdural and subarachnoid hemorrhage without optic nerve enlargement or enhancement, findings that did not suggest leukemic optic nerve infiltration.1 A lumbar puncture demonstrated red blood cells, a mildly elevated opening pressure of 31 cm H2O, and cytology findings of malignant cells consistent with CML with central nervous system (CNS) involvement. Given the intracranial hemorrhage with both intraretinal and preretinal hemorrhages, a diagnosis of Terson syndrome was made.

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Article Information

Corresponding Author: Danny A. Mammo, MD, Cole Eye Institute, Cleveland Clinic, 2022 E 105th St, I Building, Cleveland, OH 44106 (danny.mammo24@gmail.com).

Published Online: October 21, 2021. doi:10.1001/jamaophthalmol.2021.1456

Conflict of Interest Disclosures: Dr Rachitskaya reported personal fees from Alcon, Allergan, Regeneron, Genentech, Novartis, Samsara, and Zeiss outside the submitted work. Dr Singh reported having stock options in Aura Biosciences and consultancy relationships with IsoAid LLC, Immunocore, Isoaid, and Eckert and Zeigler outside the submitted work. Dr Mammo is supported by the Heed Ophthalmic Foundation outside the submitted work.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Horton  JC , Garcia  EC , Becker  EK .  Magnetic resonance imaging of leukemic invasion of the optic nerve.   Arch Ophthalmol. 1992;110(9):1207-1208. doi:10.1001/archopht.1992.01080210025010 PubMedGoogle ScholarCrossref
2.
Czorlich  P , Skevas  C , Knospe  V ,  et al.  Terson syndrome in subarachnoid hemorrhage, intracerebral hemorrhage, and traumatic brain injury.   Neurosurg Rev. 2015;38(1):129-136. doi:10.1007/s10143-014-0564-4 PubMedGoogle ScholarCrossref
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Tarlan  B , Kiratli  H .  Intraocular Manifestations of Hematopoietic Disorders. Springer Nature; 2019. doi:10.1007/978-3-030-17879-6_25
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Damato  BE , Singh  AD , eds.  Clinical Ophthalmic Oncology: Uveal Tumors. Vol 25. Springer; 2019:365-378. doi:10.1007/978-3-030-17879-6
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Myers  KA , Nikolic  A , Romanchuk  K ,  et al.  Optic neuropathy in the context of leukemia or lymphoma: diagnostic approach to a neuro-oncologic emergency.   Neurooncol Pract. 2017;4(1):60-66. doi:10.1093/nop/npw006 PubMedGoogle Scholar
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Gnanaraj  L , Tyagi  AK , Cottrell  DG ,  et al.  Referral delay and ocular surgical outcome in Terson syndrome.   Retina. 2000;20(4):374-377. doi:10.1097/00006982-200007000-00009 PubMedGoogle ScholarCrossref
7.
Narayanan  R , Taylor  SC , Nayaka  A ,  et al.  Visual outcomes after vitrectomy for Terson syndrome secondary to traumatic brain injury.   Ophthalmology. 2017;124(1):118-122. doi:10.1016/j.ophtha.2016.09.009 PubMedGoogle ScholarCrossref
8.
Chen  C-Y , Tai  C-H , Cheng  A ,  et al.  Intracranial hemorrhage in adult patients with hematological malignancies.   BMC Med. 2012;10(1):97. doi:10.1186/1741-7015-10-97 PubMedGoogle ScholarCrossref
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Lorenzi  U , Buschini  E , Fea  A , Machetta  F , Grignolo  FM .  Terson syndrome and leukemia: a case report.   Clin Ophthalmol. 2014;8:681-683.PubMedGoogle Scholar
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