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Neuro-oncology

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

JAMA Ophthalmology

Published Online: October 21, 2021

JAMA Ophthalmology Clinical Challenge

Article Information and Disclosures

Danny A. Mammo, MD¹;

Aleksandra R. Rachitskaya, MD¹;

Arun D. Singh, MD¹

Author Affiliations

¹Cole Eye Institute, Cleveland Clinic, Cleveland, Ohio

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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

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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 PubMed Google Scholar Crossref

Tarlan B , Kiratli H . Intraocular Manifestations of Hematopoietic Disorders. Springer Nature; 2019. doi:10.1007/978-3-030-17879-6_25

Damato BE , Singh AD , eds. Clinical Ophthalmic Oncology: Uveal Tumors. Vol 25. Springer; 2019:365-378. doi:10.1007/978-3-030-17879-6

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 PubMed Google Scholar

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 PubMed Google Scholar Crossref

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 PubMed Google Scholar Crossref

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 PubMed Google Scholar Crossref

Lorenzi U , Buschini E , Fea A , Machetta F , Grignolo FM . Terson syndrome and leukemia: a case report. Clin Ophthalmol. 2014;8:681-683.PubMed Google Scholar

AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.