|Year : | Volume
| Issue : | Page :
Fulminant idiopathic intracranial hypertension
Rimpi Rana, Siddharth Patel, Raghavendra R Mareguddi, Devesh Kumawat
Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
|Date of Submission||27-Nov-2020|
|Date of Acceptance||09-Jul-2021|
|Date of Web Publication||16-Sep-2022|
Department of Ophthalmology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Keywords: Disc edema, idiopathic intracranial hypertension, papilledema
| Introduction|| |
A 42-year-old female with morbid obesity (body mass index - 44.2 kg/m2) presented with gradual, painless, and progressive diminution of vision for the past 2 months along with headache with nausea since the last few months. Past medical history was unremarkable. Her best-corrected distance visual acuity was 20/200 in both eyes with normal color perception. The anterior segment was normal and fundus showed severe disc edema in both eyes [Figure 1]a and [Figure 1]b. Systemic blood pressure was found to be normal. Visual field analysis revealed severe restriction of peripheral fields with center spared (tunnel vision) in both eyes. The diagnostic tests showed normal magnetic resonance imaging (MRI) of the brain, bilateral optic nerve tortuosity and posterior globe flattening on MRI orbit, raised cerebrospinal fluid (CSF) opening pressure (28 cm of H2O) and normal CSF composition on lumbar puncture, normal MR venogram of the head, and a normal coagulopathy screen. She was diagnosed with idiopathic intracranial hypertension (IIH) and started on oral acetazolamide, and later, ventriculoperitoneal shunt surgery was successfully performed. The headache subsided postsurgery. At 8-week follow-up, papilledema subsided [Figure 2]a and [Figure 2]b and the subhyaloid hemorrhage and macular hard exudates had reduced from baseline. The distance best-corrected visual acuity improved to 20/60 in both eyes.
|Figure 1: Standard color fundus photograph of the right (a) and left eye (b) shows severe disc edema (Frisen Scale Grade V), tortuous retinal veins, peripapillary retinal splinter and subhyaloid hemorrhages and hard exudates at the macula. Incomplete macular fan could be seen in the left eye|
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|Figure 2: Montage of color fundus photograph of the right (a) and left eye (b) at 2 months following ventriculoperitoneal shunt surgery shows resolved disc edema and peripapillary splinter hemorrhages, neuroretinal rim pallor, and reduced hard exudates at the macula|
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| Comment|| |
Benign intracranial hypertension is a disorder of increased intracranial pressure without a cause identified on imaging of the brain. The criteria for diagnosis predominantly include the presence of headache, elevated CSF opening pressure with normal composition, absence of pathology on MRI brain and MR venogram, with or without the presence of papilledema. It commonly affects young obese women. Visual dysfunction, in terms of blurring of vision and visual field constriction, occurs if pressure in the subarachnoid space continues to remain high for long duration due to disruption and ischemia of axon of ganglion cells in the optic nerve. It occurs in 6%–14% of patients with IIH, with 50% suffering some degree of vision loss. In MR venography, bilateral transverse sinus stenosis of 50% degree is seen in 93% of patients with IIH, being the most sensitive imaging characteristic of this condition. However, till the specific diagnosis of IIH has been made in advance, and the imaging sequences have been protocoled; accordingly, it is very likely that in a substantial proportion of cases of IIH, the diagnosis is likely to be missed. Visual function measurement using standard automated perimetry is a primary basis for papilledema management decisions because it assesses the clinical outcome of interest. Other subjective test for visual acuity using Snellen chart can be done along with the objective tests such as electroretinogram, visual evoked potential, and pupillometry for the follow-up. Treatment aims at reducing the intracranial pressure with medical or surgical therapy. A timely diagnosis along with adequate and timely control of CSF pressure may prevent permanent visual loss. Patient's counseling on being alert to changes in the vision or visual field, weight control, and compliance to regular follow-up is important for long-term good prognosis because of the chances of recurrence.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]