Abstract
Purpose :
Idiopathic intracranial hypertension (IIH) has an incidence of 0.9 per 100,000 in the U.S., causing vision loss, headache, and negative impact on quality of life, with limited data available on the disease. Even scarcer is research on fulminant IIH (FIIH), which involves rapid visual deterioration within 4 weeks of onset. In a disease where earlier intervention is critical, more active research in this field is paramount for improving patient outcomes. The objective of this study was to provide an updated analysis on prevalence, visual outcomes, and potential risk factors for FIIH.
Methods :
Patients who met the Dandy Criteria and received an ICD code for IIH or papilledema at the Cleveland Clinic from June 1, 2012 to September 1, 2023 were selected. Data were collected from time of diagnosis up to 5 years.
Results :
Among patients with IIH, 32 patients (4.2%) were diagnosed with FIIH. Mean age at diagnosis was 27.98 (SD 8.84) with mean BMI of 40.0 (SD 7.94). Twenty-three (71.9%) were treated with surgical and/or interventional procedure within 3 months of diagnosis (mean of 13.9 days). Visual field (VF) mean deviations (MD) were -16.61 OD, -14.03 OS at baseline and -14.93 OD, -15.70 OS at follow-up 3 months to 1 year after diagnosis. FIIH patients treated with ventriculoperitoneal shunt or optic nerve sheath fenestration (ONSF) had the worst VF MD. However, of these two surgical modalities, greater VF MD improvement was seen with ONSF. Potential risk factors found for FIIH include history of anemia (2.67, CI 95%: 1.26, 5.66) and being a current smoker (2.31, CI 95%: 1.09, 4.89). Additionally, Black racial identity compared to white racial identity was identified as a potential risk factor (2.42 CI 95%: 1.26, 4.64).
Conclusions :
We report an FIIH prevalence of 4.2% of the IIH cohort, with patients diagnosed with FIIH being predominantly female. Most patients with FIIH were treated with surgery soon after diagnosis. ONSF was more effective in improving visual outcomes compared to ventriculoperitoneal shunt. History of anemia, smoking, and Black racial identity are possible risk factors for FIIH. Although this is the largest FIIH cohort reported to our knowledge, small sample size is a limitation of this study. Further analysis on longitudinal data from this cohort is needed to evaluate association of identified potential risk factors and visual outcomes in FIIH patients.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.