Case of the Month I July 2019

Case of the Month
July 25, 2019

The Case

The patient was a 33 year old obese woman referred for bilateral optic disc swelling. She had experienced episodes of transient visual loss lasting 15-30 seconds for about six months. These episodes had been occurring with increased frequency, and there were many each day at the time of initial presentation. She was six weeks pregnant when she first presented to us. She had occasional mild headaches. She was otherwise in good health.

The visual acuity was 20/25 OU and the eye pressures were 10 OD and 8 OS.  The anterior and posterior segment examinations were remarkable only for bilateral disc swelling (see pictures). What was the most likely diagnosis? What work-up and treatment would you recommend?

The patient had bilateral, chronic disc edema. In acute disc edema, the smaller blood vessels are obscured by swollen axons. In chronic disc edema, the smaller blood vessels can become more visible and the optic disc can become pale. This patient's demographics of being young, female, and obese, as well as the symptoms of transient visual obscurations, are consistent with benign intracranial hypertension (BIH), also known as pseudotumor cerebri. Work-up involved a CT of the head to rule out an intracranial mass and then a lumbar puncture, which confirmed an elevated intracranial pressure. Acetazolamide (Diamox) is often effective for BIH. Though it has not been associated with birth defects in humans, animal studies using a much higher dose than typical human exposure did reveal some birth defects (see www.ncbi.nlm.nih.gov.pubmed/22635167). This patient was unable to tolerate 1000 mg acetazolamide daily but she was able to take 500 mg and her dose was later increased to 750 mg daily.  There was gradual improvement in her disc edema and symptoms of transient visual loss.

Medical therapy and weight loss are often sufficient to manage BIH. In more severe or recalcitrant cases, particularly when there is visual loss, optic nerve sheath decompression is often effective. Unilateral treatment is often sufficient. In patients who have headache as the main symptom, ventriculoperitoneal or lumboperitoneal shunt may be done.

Case Photos

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The patient had bilateral, chronic disc edema. In acute disc edema, the smaller blood vessels are obscured by swollen axons. In chronic disc edema, the smaller blood vessels can become more visible and the optic disc can become pale. This patient's demographics of being young, female, and obese, as well as the symptoms of transient visual obscurations, are consistent with benign intracranial hypertension (BIH), also known as pseudotumor cerebri. Work-up involved a CT of the head to rule out an intracranial mass and then a lumbar puncture, which confirmed an elevated intracranial pressure. Acetazolamide (Diamox) is often effective for BIH. Though it has not been associated with birth defects in humans, animal studies using a much higher dose than typical human exposure did reveal some birth defects (see www.ncbi.nlm.nih.gov.pubmed/22635167). This patient was unable to tolerate 1000 mg acetazolamide daily but she was able to take 500 mg and her dose was later increased to 750 mg daily.  There was gradual improvement in her disc edema and symptoms of transient visual loss.

Medical therapy and weight loss are often sufficient to manage BIH. In more severe or recalcitrant cases, particularly when there is visual loss, optic nerve sheath decompression is often effective. Unilateral treatment is often sufficient. In patients who have headache as the main symptom, ventriculoperitoneal or lumboperitoneal shunt may be done.

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