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JAMA Ophthalmology Clinical Challenge
March 19, 2020

Severe Progressive Bilateral Vision Loss With Headaches

Author Affiliations
  • 1Shiley Eye Institute, Division of Oculofacial Plastic and Reconstructive Surgery, The Viterbi Family Department of Ophthalmology, University of California, San Diego, La Jolla
JAMA Ophthalmol. Published online March 19, 2020. doi:10.1001/jamaophthalmol.2020.0362

Case

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.

Figure.  
Neuroimaging at presentation.

Neuroimaging at presentation.

On admission, she was noted to have near-complete vision loss in both eyes, severe postural headaches, and nausea with vomiting. Visual acuity was hand motion OD and no light perception OS. Fundoscopic examination revealed severe papilledema (Frisen grade 4-5 OD; grade 5 OS). A magnetic resonance venography was performed and revealed bilateral filling defect of the transverse sinuses, suggestive of bilateral nonocclusive dural venous sinus thromboses (DVST) (Figure, B). The patient continued receiving acetazolamide, 500 mg, twice daily, which was dose escalated to 2000 mg twice daily over 24 hours.

Box Section Ref ID

What Would You Do Next?

  1. Begin intravenous anticoagulation

  2. Serial lumbar punctures

  3. Immediate optic nerve sheath fenestration (ONSF)

  4. Consult neurosurgery for consideration of drain or shunt placement

Discussion

Diagnosis

Bilateral dural venous sinus thrombosis

What to Do Next

C. Immediate optic nerve sheath fenestration (ONSF)

Discussion

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

Given this patient’s degree of vision loss, a decision was made for immediate ONSF to decompress the optic nerves. According to American Heart and Stroke Association treatment guidelines, low-molecular-weight heparin was started postoperatively to minimize neurologic complications from thrombosis while taking perioperative bleeding risks into consideration.1,3

Although previously thought to be rare, DVST is identified in as many as 11% of patients previously diagnosed as having IIH.5 The disease is 3 times more common in women, perhaps owing to prothrombotic state associated with pregnancy, puerperium, and oral contraceptive use.4 The most common presenting symptoms of DVST include headache, seizures, and focal motor deficits.2 However, approximately 40% of patients present solely with symptoms of IIH including headache, papilledema, and visual disturbances.6 Without proper workup, these patients may appear to meet the diagnostic criteria for IIH.6 Magnetic resonance venography is the most sensitive imaging method to identify DVST and must be ordered prior to establishing a case of IIH, which is a diagnosis of exclusion.1

Once the diagnosis of DVST is made, treatment decisions are highly dependent on the clinical picture. As mentioned previously, immediate first-line therapy is anticoagulation with subcutaneous LMWH or intravenous heparin.1,4 However, clinicians should be aware of signs and symptoms of significantly elevated intracranial pressure including headaches, vision loss, or papilledema. Persistence of symptoms following acetazolamide therapy or therapeutic lumbar puncture is an indication to consider further shunting procedures including ventriculoperitoneal shunt or ONSF.1,3 Deciding between these 2 procedures is largely determined by the clinical picture and most prominent symptoms at the time of presentation. Traditionally ventriculoperitoneal shunt is indicated for severe headaches, while ONSF has been effective in preventing further vision loss in cases of DVST with refractory symptoms or cases complicated by severe vision loss.7-9 Optimal duration of anticoagulation is under investigation.10 Follow-up magnetic resonance venography is recommended at 3 to 6 months to assess for recanalization of the occluded sinus.1 This case serves as a strong reminder of the significance of recognizing symptom severity in consideration of treatment options for elevated intracranial pressure secondary to DVST to optimize long-term visual prognosis.

Patient Outcome

Given the severity of vision loss and papilledema, the patient underwent bilateral ONSF with improvement in visual acuity noted on postoperative day 1. A subsequent lumbar puncture revealed an opening pressure of 60 mm Hg, after which a temporary lumbar drain was placed with improvement in headache symptoms. The patient subsequently started receiving intravenous heparin for 24 hours and was converted to warfarin (goal international normalized ratio, 2-3). The lumbar drain was ultimately transitioned to a lumboperitoneal shunt. At 4 weeks’ follow-up, visual acuity was 20/30 OD and 20/40 OS with central islands on Humphrey visual fields (MD, −32.21 OD and −32.58 OS; pattern SD, 6.24 OD and 6.79 OS).

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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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Coutinho  JM, Stam  J.  How to treat cerebral venous and sinus thrombosis.  J Thromb Haemost. 2010;8(5):877-883. doi:10.1111/j.1538-7836.2010.03799.xPubMedGoogle Scholar
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Biousse  V, Ameri  A, Bousser  M-G.  Isolated intracranial hypertension as the only sign of cerebral venous thrombosis.  Neurology. 1999;53(7):1537-1542. doi:10.1212/WNL.53.7.1537PubMedGoogle 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
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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
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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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