History

A female patient in her 30s was referred to me for evaluation of papilledema.

Presentation and examination

At her first neuro-ophthalmology appointment, the patient reported the following symptoms:

• Headaches that felt like a squeezing sensation around her head; her headaches were also worse when she lay flat and stood up too quickly.
• She reported vision changes that included constant blurry vision and occasional transient tunnel vision.
• The patient also reported an occasional whooshing sound in both ears.

Objective findings showed the following:

• The patient had significant visual field deficits in both of her eyes; her right eye was worse than her left eye.
• She had 2+ bilateral optic disc edema.
• An MRI of her brain was normal but also demonstrated partially empty sella.
• An MR venogram of the patient’s head showed stenosis of the bilateral transverse sinuses.
• An elevated opening pressure of 40 mmHg with lumbar puncture that decreased to an opening pressure of 29 mmHg on repeat lumbar puncture after starting Diamox.

I diagnosed the patient with papilledema, and her presentation and exam findings were consistent with idiopathic intracranial hypertension. I hypothesized that the patient may have a component of chronic migraine as well.

Treatment

The initial approach to treat the patient was through medication management.

Idiopathic intracranial hypertension

Consistent with the standard of care, I treated the patient with acetazolamide to lower the intracranial pressure and discussed the role that weight loss can play in the management of the disease. Unfortunately, acetazolamide caused unpleasant side effects with minimal improvement of her symptoms. I added Lasix at bedtime, which produced no improvement of her side effects or symptoms. I then changed her medication to methazolamide, which I had hoped she would tolerate better.

Chronic migraine

I started her with nightly nortriptyline for headache prevention, but she had unpleasant side effects with minimal improvement of symptoms; I had to change her medication to gabapentin. I then added sumatriptan for abortive treatment and Zofran for severe nausea. This was helpful for most of her headaches, but she continued to have some positional headaches that I hypothesized were from increased intracranial pressure.

After approximately four months of close monitoring, the patient’s papilledema was mildly decreased, but her visual field also worsened, which was concerning for optic nerve damage despite maximal medical treatment for this patient. I recommended that she see Webster Crowley, MD, a neurosurgeon at RUSH, for venous sinus stenting due to her medication intolerance and intractable papilledema with progressive vision loss. The stenting was performed in June 2023.

Outcome

Two months following the stenting, I found that the patient’s papilledema improved in both eyes and her visual field deficits were also found to be improving in both eyes via optical coherence tomography (OCT) and Humphrey visual field testing.

At this visit, she reported that she continued to have daily headaches, and I made some adjustments to her headache medications, which have been very helpful as she is now only experiencing minimal symptoms.

Analysis

For most patients, IIH can be cured through a combination of weight loss and medication management. It is critical to closely monitor visual fields with formal Humphrey visual field testing and to monitor nerve edema with examination and OCT testing to evaluate the efficacy of the medications we are using. Ultimately, despite optimal medical management, more invasive treatments may be needed. Fortunately, through close monitoring of this patient, we were able to determine the point at which our treatment regimen needed to be altered and the patient had to be referred for more definitive treatment. As a result, these efforts produced an excellent outcome for this patient.