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JAMA Ophthalmology Clinical Challenge
October 2016

Severe Headache and Blurred Vision

Author Affiliations
  • 1Department of Ophthalmology, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania
  • 2Sidney Kimmel Medical College at Thomas Jefferson University, Philadelphia, Pennsylvania
  • 3Neuro-Ophthalmology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania
JAMA Ophthalmol. 2016;134(10):1191-1192. doi:10.1001/jamaophthalmol.2016.1295
Case

A woman in her 30s presented 2 weeks after cesarean delivery complaining of bilateral blurred vision, photophobia, headache, and nausea. Her postoperative course had initially been complicated by the development of a transverse sinus thrombosis on postoperative day 4, for which she was treated with anticoagulation.

Over the next several days, she experienced marked worsening of her headaches, nausea, and blurred vision. She also noticed subjective fevers, weakness, and spasticity in her left upper and lower limbs, along with right ear pain and decreased hearing. Repeated magnetic resonance imaging and venography of the head and neck demonstrated worsening of the cerebral venous sinus thrombosis, now extending into the right sigmoid sinus and jugular vein. Additionally, opacification of the mastoid air cells on the right side was noted, consistent with mastoiditis. Physical examination revealed otitis media on the right side.

Ophthalmologic examination showed a visual acuity of 20/30 OD and 20/20 OS; color plates were 8 of 8 bilaterally, and there was no afferent pupillary defect. Motility and confrontational visual fields were without abnormality. Dilated fundus examination showed edema of the optic nerves bilaterally. Spectralis MultiColor fundus images were obtained (Figure, A), as well as traditional spectral-domain optical coherence tomography images (Figure, B).

Figure.
Optical coherence tomography (OCT) of the patient’s optic nerves prior to lumbar puncture.

Optical coherence tomography (OCT) of the patient’s optic nerves prior to lumbar puncture.

Box Section Ref ID

What Would You Do Next?

  1. Continue anticoagulation and monitor closely in an outpatient clinical setting

  2. Continue anticoagulation, prescribe oral antibiotics, and monitor closely in an outpatient clinical setting

  3. Perform an immediate lumbar puncture

  4. Perform an urgent radical mastoidectomy

Discussion
Diagnosis

Lateral venous sinus thrombosis

What to Do Next

C. Perform an immediate lumbar puncture

Dural sinus thrombosis may result in grossly elevated intracranial pressure. In the setting of bilateral papilledema, headaches, and nausea, it is important to quantify the elevation of intracranial pressure to aid in determining whether to continue medical therapy or to move to surgical intervention. Lumbar punctures also may be therapeutic on a temporary basis until decisions can be made about a more permanent cerebrospinal fluid diversion procedure.

Ipsilateral mastoiditis suggests that the patient may be developing a septic venous sinus thrombosis, requiring aggressive intravenous antibiotic therapy (oral antibiotics are insufficient), as well as anticoagulation and intracranial pressure–lowering surgery. Outpatient observation is inappropriate because often these individuals are very systemically ill and require close monitoring. If the patient fails to defervesce within 24 hours, urgent radical mastoidectomy should be considered; however, this is not a first-line treatment.

Cerebral venous thromboses (CVTs) are characterized by elevated intracranial pressure, focal neurological deficits, seizures, and encephalopathy.1 Neuro-ophthalmologic manifestations may include papilledema (with retinal nerve fiber layer edema), visual field defects, decreased central visual acuity, and diplopia.2

Septic lateral sinus thrombosis is a condition associated with mastoiditis and causes a clinical presentation of classic CVT with a systemic inflammatory response syndrome, including fever, tachycardia, tachypnea, and/or leukocytosis.3 Diagnosis requires imaging, most often magnetic resonance imaging and venography.

Treatment of CVT begins with anticoagulation to prevent propagation of clots and to allow recanalization of thrombosed vessels. In otogenic septic lateral sinus thrombosis, control of the infection and antibiotics are critical to early treatment.4 In the antibiotic era, death due to septic lateral sinus thrombosis is rare; however, neurologic sequelae can result. Elevated intracranial pressure from CVT can be treated medically, with cerebrospinal fluid shunting, or in severe cases with decompressive craniectomy.5

Severe papilledema occurs with CVT secondary to substantially elevated intracranial pressure. Elevated intracranial pressure can result in permanent optic nerve dysfunction if left untreated, so the ophthalmologist must work closely with the neurosurgical team to guide the timing of intervention. Some authors have published good results for visual recovery following optic nerve sheath fenestrations.6,7

Using the different depths of penetration due to the different wavelengths of blue, red, and green light, multicolor optical coherence tomography is a new technology for evaluation of the fundus and optic disc by providing topographic maps of the inner, mid, and deep retinal layers. Subtle changes in nerve head architecture may be seen that are not at all visible with fundus examination or photography with monochromatic light, and may be a useful tool in monitoring response to therapies.

Patient Outcome

A lumbar puncture revealed an opening pressure of 430 mm of water with an otherwise normal fluid analysis. During the lumbar puncture, a lumbar drain was placed. The patient’s symptoms improved for 2 days. The lumbar drain was then clamped, and symptoms quickly returned. Mechanical cerebrospinal fluid diversion dependence prompted neurosurgery to implant a permanent ventriculoperitoneal shunt, which was tolerated well.

Oral anticoagulation was continued and the patient was treated with intravenous vancomycin and ciprofloxacin for mastoiditis, because of the concern for septic lateral sinus thrombosis. Otolaryngology was consulted and performed a myringotomy.

The patient’s visual symptoms improved and she had an uneventful postoperative course. She was discharged home with a prescription of oral anticoagulation and has had no return of symptoms.

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Article Information

Corresponding Author: Brenton D. Finklea, MD, Wills Eye Hospital Medical Education, Attn: Brenton Finklea, 840 Walnut St, Ste 800, Philadelphia, PA 19107 (brent.finklea@gmail.com).

Published Online: August 11, 2016. doi:10.1001/jamaophthalmol.2016.1295

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Dr Sergott reports personal fees and other from Heidelberg Engineering, during the conduct of the study. No other disclosures are reported.

References
1.
Saposnik  G, Barinagarrementeria  F, Brown  RD  Jr,  et al; American Heart Association Stroke Council and the Council on Epidemiology and Prevention.  Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association.  Stroke. 2011;42(4):1158-1192.PubMedGoogle ScholarCrossref
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Purvin  VA, Trobe  JD, Kosmorsky  G.  Neuro-ophthalmic features of cerebral venous obstruction.  Arch Neurol. 1995;52(9):880-885.PubMedGoogle ScholarCrossref
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Southwick  FS, Richardson  EP  Jr, Swartz  MN.  Septic thrombosis of the dural venous sinuses.  Medicine (Baltimore). 1986;65(2):82-106.PubMedGoogle ScholarCrossref
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Seven  H, Ozbal  AE, Turgut  S.  Management of otogenic lateral sinus thrombosis.  Am J Otolaryngol. 2004;25(5):329-333.PubMedGoogle ScholarCrossref
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Ferro  JM, Crassard  I, Coutinho  JM,  et al; Second International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT 2) Investigators.  Decompressive surgery in cerebrovenous thrombosis: a multicenter registry and a systematic review of individual patient data.  Stroke. 2011;42(10):2825-2831.PubMedGoogle ScholarCrossref
6.
Acheson  JF.  Optic nerve disorders: role of canal and nerve sheath decompression surgery.  Eye (Lond). 2004;18(11):1169-1174.PubMedGoogle ScholarCrossref
7.
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.PubMedGoogle ScholarCrossref
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