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A 25 year old woman is found to have these abnormalities on routine optometric examination. She is without visual symptoms but reports moderate continuous headache for the past six months. She is markedly overweight, but denies any medical problems and takes no medications. Visual acuities are normal; visual fields show very small nerve fiber bundle defects with mean deviations of -2.00 decibels. Brain MRI and contrast magnetic resonance venography (MRV) are normal. Lumbar puncture opening pressure is 30cm water with normal constituents. She reports unbearable side effects from a 4-week trial of acetazolamide. Trials of other intracranial pressure-lowering agents have not altered the optic disc appearance, optical coherence tomography results, or visual field measurements over several months.

  • Review Topic

    What should you do?

    Correct! The diagnosis is not in doubt here. If all studies were correctly interpreted, she has idiopathic intracranial hypertension (IIH, pseudotumor cerebri). It has caused only minimal damage to visual function. Yet the optic discs remain moderately elevated, so the question is whether she is at risk for eventual optic nerve damage if left untreated. We do not know the answer to that important question, and no tests can reliably help us. What we do know is that the surgical options entail considerable risks. Lumbo-peritoneal shunts have given way to ventriculo-peritoneal shunts because of technical problems. Ventriculo-peritoneal shunts are not difficult to perform, but they have a 20% chance of complications, some serious. Venous sinus stenting is an option, but it too has risks and experience is limited. Bariatric surgery, not one of the answer choices here, might solve the intracranial pressure problem, but has its own adverse consequences. In the end, “watchful waiting” is probably the most prudent approach here. One fact favors this approach: if visual function in IIH is to deteriorate, it usually does so early in the course of management.