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August 18, 2021
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Woman presents with blurred peripheral vision, pulsatile tinnitus and headaches

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A 40-year-old white woman was referred to the Tufts Medical Center neuro-ophthalmology service for 3 weeks of blurred peripheral vision, pulse-synchronous tinnitus and headaches.

Her medical history included myopia and obesity. She denied taking any daily medications.

Allison V. Coombs
Allison V. Coombs
Nisha S. Dhawlikar
Nisha S. Dhawlikar

On thorough review of systems, she endorsed her heartbeat creating pulsations in her vision. She had two migraines in the past 6 months that resulted in emesis, but she denied transient visual obscurations, daily headaches, diplopia or pain with extraocular motility. Upon further questioning, she reported taking 800 mg ibuprofen multiple times daily each week to prevent headaches.

Examination

Best corrected visual acuity was 20/30 and 20/50 in the right and left eyes, respectively. Confrontation visual fields were full, and color vision using the Hardy-Rand-Rittler standard pseudoisochromatic color plates was full. Pupillary examination was unremarkable, with no evidence of a relative afferent pupillary defect in either eye. Extraocular motility was full, and the patient was orthophoric. The anterior segment examination was unremarkable. The posterior segment examination was pertinent for bilateral optic disc edema, intraretinal hemorrhages, macular exudates, subretinal fluid and dilated, tortuous vasculature (Figure 1). Humphrey visual fields 30-2 SITA Fast noted enlarged blind spots bilaterally (Figure 2). The patient appeared fatigued and anxious.

bilateral optic disc edema
Figure 1. Color fundus photos of the right (a) and left (b) eyes demonstrating bilateral optic disc edema, dilated, tortuous vasculature, decreased foveal reflex and macular exudates. Magnified view of the posterior pole of the right (c) and left (d) eyes.

Source: Allison V. Coombs, DO, MS, and Yosbelkys Martin Paez, MD
enlarged blind spot bilaterally
Figure 2. Humphrey visual fields 30-2 demonstrating an enlarged blind spot bilaterally and lid artifact superiorly in the left eye.

What is your diagnosis?

See answer below.

Bilateral optic disc edema

The differential diagnosis for bilateral optic disc edema, intraretinal hemorrhages, dilated, tortuous vasculature and macular exudates includes pathologies resulting from elevated intracranial pressure, such as space-occupying lesions, dural venous sinus thrombosis, venous stenosis and idiopathic intracranial hypertension. Bilateral disc edema can also occur without elevated intracranial pressure from hypertensive emergency, diabetic papillitis, inflammatory or autoimmune optic neuritis, or bilateral infiltrative optic neuropathies. Finally, bilateral optic disc edema with resultant hemorrhages and macular exudate (neuroretinitis) can result from infectious etiologies, such as Bartonella henselae, tuberculosis, syphilis or Lyme disease, or from bilateral infiltrative optic neuropathies or inflammatory/autoimmune optic neuritis.

Workup and management

OCT through the optic nerves and macula was obtained in clinic, which further characterized the optic disc edema, intraretinal and subretinal fluid, and exudates (Figure 3). The patient’s blood pressure was checked in the clinic and found to be 230/130. She was transported immediately to the emergency room. The patient was found to have poor kidney function (creatinine 4.78, glomerular filtration rate 11, chronic kidney disease stage 5) and an elevated troponin (0.45 ng/mL). Imaging included a CT of the head without contrast that revealed hyperdensity in the temporal lobe, highly suspicious for intraparenchymal hemorrhage. MRI, MRA and MRV of the head did not reveal any additional abnormalities and were negative for a mass, venous sinus stenosis or thrombosis, or vascular structural abnormality. A lumbar puncture was performed and revealed an opening pressure of greater than 55 cm H2O, with normal cerebrospinal fluid (CSF). Renal biopsy was performed due to poor kidney function, which ultimately revealed IgA nephropathy as well as interstitial nephritis suspected to be from NSAID abuse. This patient’s ophthalmic presentation was thought to be most consistent with malignant hypertension secondary to IgA nephropathy and interstitial nephritis from NSAID abuse as well as idiopathic intracranial hypertension.

optic disc edema
Figure 3. OCT through the optic nerves and macula of the right (a) and left (b) eyes, demonstrating optic disc edema, intraretinal and subretinal fluid, and exudates within Henle’s layer.

The patient was admitted to the ICU for further management. Blood pressure was slowly titrated to goal. Idiopathic intracranial hypertension was treated with a low dose of acetazolamide, dose adjusted for the poor renal function. Systemic steroids were concurrently administered to attempt to reverse any possible interstitial nephritis caused by NSAID overuse. Blood pressure normalized over the course of 4 weeks.

Discussion

Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is an elevation in the intracranial pressure (ICP) resulting in optic disc edema. It is diagnosed using the Modified Dandy Criteria published by Friedman and colleagues. Patients meet the criteria for IIH if they have signs and symptoms of increased ICP, such as episodic headaches, papilledema and transient visual obscurations, with the following features on clinical examination: no localizing neurologic signs except for unilateral or bilateral sixth cranial nerve palsy, elevated CSF opening pressure greater than 25 cm H2O with normal CSF composition, no evidence of hydrocephalus, mass, structural or vascular lesion (including venous sinus thrombosis) on imaging, and no other cause of increased ICP identified. Our patient met this criterion with a CSF opening pressure of greater than 55 cm H2O with normal CSF composition.

Malignant hypertension was previously defined clinically as diastolic blood pressure greater than 130 mm Hg, with features of hypertensive retinopathy grade III or IV based on the classification by Keith and colleagues, including retinal vascular hemorrhages, dilated tortuous vasculature, exudates and, rarely, papilledema. Malignant hypertension is now more broadly defined to include end-organ damage of the kidney and/or heart, encephalopathy and microangiopathy. Malignant hypertension carries a poor prognosis. There is no known association between malignant hypertension and elevated cerebrospinal fluid opening pressure, hence our inference that our patient has two concurrent pathologies: both IIH and malignant hypertension.

Both diagnoses should be considered in patients with optic disc swelling in whom cerebral imaging does not suggest an alternative cause, especially if they meet the Dandy criteria and have elevated blood pressure. A few studies are investigating the overlap between IIH and malignant hypertension; however, in patients with fulminant IIH, more than 25% are known to have concurrent uncontrolled systemic hypertension. When IIH and malignant hypertension are concurrent, visual outcomes are known to be poor. The pathophysiology is presumed to be that elevated ICP creates increased pressure around the optic nerve sheath, possibly impinging the posterior ciliary artery perfusion of the optic nerve. It is theorized that when systemic blood pressure is dropped, the continued elevated pressure exerted from IIH can cause ischemia to the optic disc head. Abbasi and colleagues reported a case of malignant hypertension with initially unidentified and therefore untreated IIH, with resultant fulminant vision loss. In that case, the poor visual outcome was hypothesized to be due to an intentional drop in systemic blood pressure to treat malignant hypertension, with subsequent unintended ischemia of the optic nerve heads due to concurrent IIH.

Our patient likely had malignant hypertension from her underlying IgA nephropathy; however, she also had elevated intracranial pressure, which is not a known feature of malignant hypertension. Checking the opening pressure by lumbar puncture and concurrently treating elevated ICP with acetazolamide may have preserved optic nerve head perfusion and viability while systemic blood pressure was lowered.

Ongoing studies are necessary to further elucidate the associations of malignant hypertension and IIH. However, ophthalmologists should be aware that these diseases can present concurrently, and patients with bilateral optic disc edema without an identified central process, such as an intracranial mass or venous sinus thrombosis, should be worked up for IIH and systemic hypertension. Both IIH and malignant hypertension must be treated and managed appropriately by multidisciplinary teams to preserve vision.

Clinical course continued

Unfortunately, although the patient’s blood pressure stabilized, the kidney function has not significantly improved. Eight weeks later, the visual acuity has remained stable at 20/30 and 20/60 (pinhole 20/40) in the right and left eyes, respectively. Blood pressure has been stable, and her symptoms have significantly improved. She continues to follow with nephrology, neurology and neuro-ophthalmology.