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Man presents with 1-month history of bilateral intermittent blurred vision
Both eyes featured optic disc swelling with vessel obscuration and peripapillary flame hemorrhages, cotton wool spots and venous tortuosity.
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A 57-year-old man presented to the Lahey Medical Center ophthalmology clinic with a 1-month history of transient blurred vision in both eyes. His medical history was significant for hypertension, diabetes type 2, osteoarthritis, chronic back pain and a cholesteatoma, which was surgically treated with right tympanomastoidectomy 6 weeks before presentation. His ocular history was significant only for mild myopia, for which he was already wearing glasses.
On evaluation in the eye clinic, he endorsed a month-long history of intermittent blurred vision. He noted recurrent episodes of vision loss in both eyes that lasted only a few seconds. The patient noticed the vision would get very blurry and dim. Between these episodes, the vision returned to normal. He was unsure if the vision loss was positional. He was experiencing more frequent episodes, which prompted him to schedule an eye exam. He denied any headaches, nausea or vomiting. He did not endorse any pulsatile tinnitus or increased intracranial noises. He felt systemically well and had no recent sick contacts. He denied any scalp tenderness, jaw claudication, fevers, weight loss, fatigue or myalgias. He had no recent travel.
Examination
Initial exam in the ophthalmology clinic showed a best corrected visual acuity of 20/30 in the right eye and 20/40 in the left eye. Both pupils were briskly reactive, and there was no afferent pupillary defect in either eye. Color vision, evaluated by HRR pseudoisochromatic plates, was full in both eyes. Extraocular muscle movements were normal, and the patient was orthophoric in primary gaze at distance and near. Anterior segment exam was normal in both eyes. There was a mild nuclear sclerotic cataract in both eyes. No anterior or posterior cell was appreciated.
Images: Tirpack A, Athappilly G
Dilated funduscopic examination showed bilateral optic disc swelling with 360° of vessel obscuration and peripapillary flame hemorrhages, cotton wool spots and venous tortuosity (Figure 1). OCT of the optic nerves confirmed severe bilateral optic nerve swelling with an average retinal nerve fiber layer thickness of 542 µm in the right eye and 524 µm in the left eye (Figure 2). Humphrey visual fields showed inferior field defects in both eyes with enlargement of the blind spot in the right eye (Figure 3). OCT of the macula showed nasal intraretinal fluid tracking from the swollen optic nerves (Figure 4).
What is your diagnosis?
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Bilateral optic disc edema
The differential diagnosis of severe bilateral optic disc edema with flame hemorrhages includes malignant hypertension, elevated intracranial pressure (papilledema), and vascular or infectious etiologies.
All patients presenting with bilateral optic disc edema should have their blood pressure measured in the office. Malignant hypertension can lead to optic nerve ischemia with subsequent swelling and disc hemorrhages. Given the morbidity and mortality associated with severely elevated blood pressure, this must be kept high in the differential of bilateral optic disc edema. Our patient’s blood pressure in the office was 136/78, making this a less likely diagnosis. Additionally, the patient’s posterior pole did not show any evidence of hypertensive retinopathy, such as retinal hemorrhages or retinal cotton wool spots.
Given the patient’s preserved central visual acuity and color vision, papilledema was high on the differential. Chronic elevated intracranial pressure can lead to severe optic nerve swelling with flame hemorrhages and cotton wool spots. The patient’s visual field findings of enlarged blind spot and inferior arcuate changes are characteristic of papilledema. Additionally, his complaint of temporary visual loss lasting seconds is characteristic of the transient visual obscurations seen in cases of elevated intracranial pressure.
Other less likely causes of bilateral optic nerve swelling in this patient include vascular or infectious causes. Sequential non-arteritic anterior ischemic optic neuropathy could present with bilateral optic disc edema. Typically, however, this is associated with a more marked decline in central visual acuity and altitudinal visual field defects, which are not seen in our patient. Additionally, arteritic anterior ischemic optic neuropathy, secondary to giant cell arteritis, can lead to bilateral optic nerve swelling but is usually accompanied by severe vision loss, pallid edema and systemic symptoms not seen in our patient. Infectious causes of optic nerve swelling include Bartonella, Lyme and syphilis. These infectious causes are typically associated with vitreous inflammation and retinal exudates not present in our patient.
Ultimately, we were concerned that elevated intracranial pressure with subsequent papilledema was the cause of our patient’s optic disc swelling. On further discussion with the patient, we learned his recent tympanomastoidectomy was complicated by intraoperative bleeding from the sigmoid sinus. Neurosurgery was consulted during the operation, and a CT of the head following surgery was negative for thrombus or intracranial bleed. Given this history, we were concerned for a sigmoid sinus thrombosis causing increased intracranial pressure.
Diagnosis and management
The patient was urgently transferred to the emergency room for further workup and management. MRI and MRV of the head showed stenotic flow through the right sigmoid and transverse sinus (Figure 5). The patient was diagnosed with a right cerebral venous sinus thrombosis and admitted to the neurology service. Given the evidence of recanalization on MRV, it was decided to proceed with anticoagulation rather than pursue primary surgical intervention. A lumbar puncture was deferred given the clinical evidence of elevated intracranial pressure and desire to expedite anticoagulation. He was started on low molecular weight heparin while bridging to a therapeutic dose of warfarin. He was also started on 2 g acetazolamide daily by mouth. Two days after initiation of anticoagulation and acetazolamide, he noted subjective improvement in his vision. The intermittent episodes of vision loss had resolved.
He remained anticoagulated with warfarin for 3 months. Repeat MRV imaging showed improved flow through the right sigmoid sinus. On the most recent examination, 8 months after initial presentation, his vision remained stable at 20/30 in the right eye and 20/40 in the left eye. His papilledema had improved to the 200 µm range in both eyes. He continues on 1.5 g acetazolamide daily.
Discussion
Papilledema must be considered in the differential diagnosis of any patient presenting with bilateral optic disc edema. Symptoms of papilledema characteristically include transient visual obscurations, headache and pulsatile tinnitus. Patients can also present with new onset binocular horizontal diplopia in the setting of a sixth nerve palsy. Central visual acuity and color vision are typically preserved. Patients with clinical evidence of papilledema require neuroimaging. An MRI and MRV of the head are recommended to rule out any mass lesion or cerebral venous sinus thrombosis. In the absence of a structural mass lesion, a lumbar puncture can then be pursued to confirm elevated intracranial pressure.
It is important obtain an MRV in all patients with suspected elevated intracranial pressure. Our patient did not fit the typical demographic of idiopathic intracranial hypertension (obese woman of childbearing age), and therefore our suspicion for mass lesion or venous obstruction was quite high. Even in patients with clinically suspected idiopathic intracranial hypertension, an MRV is indicated because up to 9% of these patients have cerebral venous sinus thrombosis on MRV. MRI alone without venogram is inadequate to evaluate the cerebral sinuses and can therefore lead to false negatives.
The incidence of cerebral sinus thrombosis is 1.3 per 100,000 people. It is most common among 30- to 40-year-olds and more frequently seen in women. Symptoms can be quite variable, including blurred vision, headache, seizures and hemiplegia. Interestingly, not all patients present with headache (including our patient), and therefore a high index of suspicion must be maintained in patients presenting with other signs or symptoms.
Risk factors for development of cerebral sinus thrombosis include a hypercoagulable state, head trauma, dehydration and infection. The anatomical proximity of the middle ear structures to the cerebral venous sinuses can lead to thrombosis. Acute and chronic ear infections can lead to lateral cerebral sinus thromboses. Our patient underwent surgery for a right cholesteatoma, which is an abnormal skin growth of the middle ear. The surgical procedure, a tympanomastoidectomy, involves removal of the mastoid bone and repair of the eardrum, which is in close relation to the sigmoid sinus. As seen in our patient’s case, trauma to the cerebral venous sinuses can lead to subsequent thrombosis.
Once the diagnosis of cerebral sinus thrombosis has been confirmed by neuroimaging, the patient requires immediate anticoagulation. The patient can then be bridged to an oral anticoagulant with a goal INR of 2 to 3. Oral acetazolamide can also be initiated in cases of elevated intracranial pressure. Patients need close follow-up and monitoring. Sequential neuroimaging is required to establish improved flow through the sinus. Surgical intervention may be considered in cases refractory to anticoagulation. When papilledema is present, close monitoring with serial fundus exams, OCT and visual fields are needed to guide management and acetazolamide dosing.
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- Dubey SP, et al. Am J Otolaryngol. 2010;doi:10.1016/j.amjoto.2008.10.001.
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- For more information:
- Aubrey Tirpack, MD, and Geetha Athappilly, MD, can be reached at New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.
- Edited by Jessica Moon, MD, and Emily C. Wright, MD. They can be reached at the New England Eye Center, Tufts University School of Medicine, 750 Washington St., Box 450, Boston, MA 02111; website: www.neec.com.