Select Patients with High-Risk IIH May Be Managed with Medical Therapy Alone
Jeffrey Gluckstein1, Joseph Rizzo2, Amalie Chen3
1Neurology and Ophthalmology, Keck Medicine of USC, 2Mass Eye & Ear Infirmary, 3Mass General Brigham
Objective:
Measure visual outcomes in high-risk idiopathic intracranial hypertension (IIH), with particular attention to medical or surgical treatment course and disease recurrence.
Background:
In IIH, presentations with BMI >40, high-grade papilledema, worse visual field mean deviation (VFMD), and worse visual acuity are associated with worse visual outcomes. In the absence of randomized data on high-risk patients, surgery is often recommended, but risks complications, infections, or surgical revision.
Design/Methods:
We retrospectively reviewed the charts of all IIH patients who presented to 3 providers between April 2019 and April 2022 with grade IV or V papilledema. Patients were treated at the discretion of their neuro-ophthalmologist. Outcomes were stratified by presenting VFMD.
Results:
11 patients were evaluated. Average age was 25.8 years, 10/11 were female, and average BMI was 38.3 kg/m2. On presentation, VA was 20/30 or worse in 9/11 patients, but improved to 20/20 or better in 9/11 patients. Average presenting VFMD was -13.8 dB, but improved by an average of 7.1 dB in those with initial VFMD worse than -7.0 dB and 2.1 dB in those with initial VFMD better than -7.0 dB. 2 patients met published criteria for “fulminant” IIH, but both improved to 20/20 acuity OU with significant VFMD improvements on medical therapy alone. All patients received acetazolamide with rapid dose escalation. Average maximum dose was 2.75 g per day. 2 also received topiramate. 1 patient received a ventriculoperitoneal shunt 3 days after presentation. 1 patient received an optic nerve sheath fenestration 15 days after presentation. 1 patient on medical therapy had recurrent disease over an average of 352 days of follow up.
Conclusions:
Select patients with high-risk or “fulminant” IIH may be successfully managed without surgery. These patients required close monitoring and aggressive medical therapy. Reduced visual acuity on presentation recovered well in most patients. Larger cohorts with longer follow-up are needed.