Longitudinal Visual Outcomes and Risk of Papilledema Recurrence in Fulminant Idiopathic Intracranial Hypertension.

Abstract

Background: Fulminant idiopathic intracranial hypertension (IIH) is a rare presentation of IIH characterized by rapid visual loss. Little is known regarding the cause of fulminant IIH and how to treat this condition. We conducted an analytical study aimed at characterizing disease, treatment, and visual outcomes while evaluating the time and frequency of recurrence of papilledema.

Methods: This retrospective cohort study used records at a single tertiary institution screened between June 1, 2012, and September 31, 2023, for having an international classification of diseases (ICD) code of IIH or papilledema. After meeting the revised Dandy diagnostic criteria and excluding secondary causes of IIH using clinical review, fulminant IIH was determined by rapid visual loss occurring within 1 month of symptom onset. Demographics, treatment, and visual outcomes data were collected with final visual outcomes reported between 3 months and 1 year after diagnosis. Surgical intervention and relapse of papilledema were noted. All analyses were performed in R Studio and Excel, with a P value <0.05 being significant.

Results: Of the 731 IIH patients who were evaluated during this interval, 3.2% had fulminant IIH (n = 24). Patients had a mean age of 27.6 years and presented with an average visual field mean deviation of -19.55 dB. Overall, 95.8% were overweight/obese, 87.5% female, and 50% were Black. In total, 62.5% of patients with fulminant IIH received optic nerve sheath fenestrations. At follow-up, 41.7% of patients were legally blind (with 20/400 vision or worse), including 13% having no light perception. Within our cohort, 42% had a significant visual deficit (visual field mean deviation worse than -7 dB or best-corrected visual acuity between 20/80 and 20/200). Although not statistically significant, patients who underwent cerebral venous sinus stenting had the smallest visual deficit. On average, patients recovered from their initial papilledema after 7.90 months. One quarter (25%) of patients had a recurrence at 4.5 months (SD ± 4.1) after the initial resolution of papilledema.

Conclusions: Fulminant IIH is a rare and blinding variant of IIH. Owing to the recurrence time after initial papilledema resolution, we recommend all such patients be monitored until 8 months after initial papilledema resolution. Future studies should evaluate optimal surgical interventions for preserving vision.