Fulminant idiopathic intracranial hypertension (IIH) is a rare form of IIH with rapid and ag- gressive vision loss. Urgent management is required to prevent blindness. We present the case of a 28-year-old woman with new symptoms of transient visual obscurations, headache and pulsatile tinnitus with findings of severe bilateral optic disc edema that developed devastating visual loss. Blood tests, brain imaging and cerebral spinal fluid analysis excluded secondary causes. A lumbar puncture revealed intracranial hypertension and magnetic resonance imaging findings supported it. A ventriculoperitoneal shunt was placed, but due to continued vision deterioration, an urgent bilateral optic nerve sheath fenestration was performed with slight vision improvement. This case highlights the relevance of early recognition of this entity and the demand for prompt considera- tion of surgical management to prevent further visual loss and blindness. It also illustrates the importance of multidisciplinary work and low vision rehabilitation for a young, visually impaired adult.

Revista Sociedade Portuguesa de Oftalmologia, Vol. 47 N.º 2 (2023)