Fulminant Idiopathic Intracranial Hypertension-Clinical Characteristics and Continuous Drainage as a Novel Treatment Approach.

BACKGROUND

Fulminant idiopathic intracranial hypertension (fIIH) is a severe condition which causes rapid visual loss. Conventional fIIH management involves surgical interventions, but their effectiveness and safety remain uncertain. The use of temporary continuous CSF drainage (CD) for fIIH has not been fully explored in the adult population. We present the results of CD treatment in severe IIH cases.

METHOD

IIH Patients treated with CD were compared to control groups of extreme CSF opening pressure (> 45 cm HO) and random IIH patients (> 25 cm HO).

RESULTS

Eighteen female patients underwent CD treatment. Indications for CD treatment were acute visual deterioration or development of a VI nerve palsy and severe papilledema (despite acetazolamide treatment). All patients stabilized after CD treatment. Four patients had acute complications that fully resolved. Visual acuity (VA) in fIIH patients was significantly reduced prior to treatment (mean 6/15 ± 6/19 vs. 6/7.5 ± 6/30, p < 0.01). CD treatment resulted in marked long-term improvement in VA (mean 6/7.5 ± 6/19 vs. 6/7 ± 6/38, p = 0.12 and 6/7 ± 6/48, p = 0.05). Compared to the extreme CSF opening pressure group, female sex, obesity, and TVO's were more common in the CD group. Compared to the general IIH group, female sex, obesity, endocrinopathy, and CSF opening pressure were higher in the CD group.

CONCLUSION

IIH patients with very high CSF opening pressure and the presence of obesity and endocrinopathy may be at higher risk to develop fIIH. CD treatment is an extremely effective and safe treatment option for severe cases of IIH.