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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).1- 4 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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Corresponding Author: Catherine Y. Liu, MD, PhD, Shiley Eye Institute, 9415 Campus Point Dr, La Jolla, CA 92093 (email@example.com).
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.
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