This is a classic presentation of idiopathic intracranial hypertension (IIH).
It is mainly diagnosed in young obese women; predisposing factors include oral contraceptives, anabolic steroids, tetracycline, and vitamin A. The pathophysiology is not known, but may be due to an imbalance of CSF production and absorption. CSF pressure is greater than 200–250 mm H20.
Papilledema is present, and visual field defects and blindness can occur with progression of the disease. Neuroimaging is required to rule out other causes of intracranial hypertension including intracranial masses, obstruction of CSF flow, and venous sinus thrombosis. A negative D-Dimer can help evaluate for venous sinus thrombosis in low-risk patients, although venous phase imaging with MRI is more sensitive and specific.
Evidence-based treatment of IIH is lacking. Acetazolamide has been used to decrease CSF production, but emergent referral for ventricular shunting or optic nerve sheath fenestration is indicated if visual loss is present. The retinal image was taken at the bedside using a Panoptic™ attached to a smartphone camera.
Take Home Points
- Papilledema is a sign of intracranial hypertension and may be caused by intracranial masses, obstruction of CSF flow, or idiopathic intracranial hypertension.
- Idiopathic intracranial hypertension is typically seen in young, obese women.
- Predisposing factors include oral contraceptives, anabolic steroids, tetracycline, and vitamin A.