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Severe Progressive Bilateral Vision Loss With Headaches

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

An African American woman in her 20s presented to an outside hospital with progressive vision loss in both eyes, photophobia, and nausea, which had developed over weeks. Her home medications were hydrocodone and ibuprofen. Bilateral disc edema was noted on examination, and neuroimaging was obtained. Noncontrast head computed tomography (CT) and CT angiogram were remarkable only for bilateral optic nerve head abnormalities. Magnetic resonance imaging was concerning for prominent optic nerves (Figure, A). There was no evidence of intracranial masses, hydrocephalus, or bleed. Lumbar puncture revealed cerebrospinal fluid opening pressure of 40 cm of water. Cerebrospinal fluid protein, glucose, IgG, and oligoclonal band levels were within normal limits. Renal function was normal. The patient started receiving oral acetazolamide, 500 mg, twice daily for presumed idiopathic intracranial hypertension (IIH) and transferred to our institution.

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Bilateral dural venous sinus thrombosis

C. Immediate optic nerve sheath fenestration (ONSF)

The key in choosing the first step is recognizing the severity of the patient’s vision loss and papilledema on examination. For acutely threatened vision, ONSF is recommended (choice C).14 Typically, intravenous anticoagulation (choice A) is started with symptomatic improvement in most cases.1 However, this is not recommended urgently in cases of severe intracranial hypertension owing to DVST because treatment guidelines recommend immediate pressure reduction by lumbar puncture or neurosurgical shunt prior to anticoagulation. Serial lumbar punctures (choice B) are not recommended because their effect is only transient. Lumbar drain placement (choice D) can be considered for severe intracranial hypertension, but ONSF should be performed first to rapidly halt cases of severe progressive vision loss.1

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Article Information

Corresponding Author: Catherine Y. Liu, MD, PhD, Shiley Eye Institute, 9415 Campus Point Dr, La Jolla, CA 92093 (yul107@ucsd.edu).

Published Online: March 19, 2020. doi:10.1001/jamaophthalmol.2020.0362

Conflict of Interest Disclosures: None reported.

Funding/Support: Bell Charitable Foundation, Rancho Santa Fe, California and Research to Prevent Blindness, New York, New York.

Role of the Funder/Sponsor: The funding sources 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.

Additional Contributions: We thank Amy Maduram, MD, Department of Radiology, University of California, San Diego, for her expertise in radiology. No compensation was received from a funding sponsor for her contributions. We thank the patient for granting permission to publish this information.

References
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Silvis  SM , de Sousa  DA , Ferro  JM , Coutinho  JM .  Cerebral venous thrombosis.   Nat Rev Neurol. 2017;13(9):555-565. doi:10.1038/nrneurol.2017.104PubMedGoogle ScholarCrossref
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Einhäupl  K , Stam  J , Bousser  M-G ,  et al; European Federation of Neurological Societies.  EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients.   Eur J Neurol. 2010;17(10):1229-1235. doi:10.1111/j.1468-1331.2010.03011.xPubMedGoogle ScholarCrossref
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Galgano  MA , Deshaies  EM .  An update on the management of pseudotumor cerebri.   Clin Neurol Neurosurg. 2013;115(3):252-259. doi:10.1016/j.clineuro.2012.11.018PubMedGoogle ScholarCrossref
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Murdock  J , Tzu  JH , Schatz  NJ , Lee  WW .  Optic nerve sheath fenestration for the treatment of papilledema secondary to cerebral venous thrombosis.   J Neuroophthalmol. 2014;34(1):67-69. doi:10.1097/WNO.0000000000000087PubMedGoogle ScholarCrossref
9.
Horton  JC , Seiff  SR , Pitts  LH , Weinstein  PR , Rosenblum  ML , Hoyt  WF .  Decompression of the optic nerve sheath for vision-threatening papilledema caused by dural sinus occlusion.   Neurosurgery. 1992;31(2):203-211. doi:10.1227/00006123-199208000-00005PubMedGoogle ScholarCrossref
10.
Miranda  B , Aaron  S , Arauz  A ,  et al.  The benefit of EXtending oral antiCOAgulation treatment (EXCOA) after acute cerebral vein thrombosis (CVT): EXCOA-CVT cluster randomized trial protocol.   Int J Stroke. 2018;13(7):771-774. doi:10.1177/1747493018778137PubMedGoogle ScholarCrossref
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