Case of the Month I August 2020

Case of the Month
August 24, 2020

The Case

The patient was a 66-year-old White woman who presented in early 2018 with decreased vision in the right eye for several weeks. Her past medical history included interstitial cystitis and Hashimoto’s thyroiditis. Her past ophthalmic history was remarkable for cataract surgery in both eyes. Her medications included Synthroid (levothyroxine – for thyroid insufficiency), Elmiron (pentosan – for interstitial nephritis), Coreg (carvedilol – a beta-blocker), Cymbalta (duloxetine – for anxiety), Amerge (naratriptan – for migraine headaches), Levsin-SL (hyoscyamine – for pain), and Nexium (esomeprazole – for GERD). Her initial visual acuity was 20/400 OD and 20/20 OS. She was treated with Avastin and the deep swelling in the right eye quickly resolved. The visual acuity in the right eye was 20/40 at the next visit and eventually improved to 20/25. Subsequently, the subjective vision has been stable. Could her entire clinical picture be explained by macular degeneration, or was there another condition contributing to her clinical picture?

The patient initially had a choroidal neovascular membrane that responded well to Avastin treatment. The distribution of RPE changes and the autofluorescence findings are typical of pentosan maculopathy. Pentosan polysulfate is a mainstay for the treatment of interstitial cystitis, which causes chronic pain in the bladder and pelvic region. It affects more than 1 million people in the United States, mostly women. The maculopathy can be associated with severe atrophic changes and significant visual loss. A recent oral presentation at the American Society of Retina Specialists showed that the maculopathy can progress after cessation of the drug. Our patient has been stable after discontinuing pentosan polysulfate.

Reference

Pearce W, Chen R, Jain N. Pigmentary maculopathy associated with chronic exposure to pentosan polysulfate sodium. Ophthalmology 2018;125(11):1793-1802.

Case Photos

Click the Images below to enlarge
Color OD
Color OS
OCT OD January 2018
OCT OD June 2020
OCT OS June 2020
Autofluorescence OD
Autofluorescence OS

The patient initially had a choroidal neovascular membrane that responded well to Avastin treatment. The distribution of RPE changes and the autofluorescence findings are typical of pentosan maculopathy. Pentosan polysulfate is a mainstay for the treatment of interstitial cystitis, which causes chronic pain in the bladder and pelvic region. It affects more than 1 million people in the United States, mostly women. The maculopathy can be associated with severe atrophic changes and significant visual loss. A recent oral presentation at the American Society of Retina Specialists showed that the maculopathy can progress after cessation of the drug. Our patient has been stable after discontinuing pentosan polysulfate.

Reference

Pearce W, Chen R, Jain N. Pigmentary maculopathy associated with chronic exposure to pentosan polysulfate sodium. Ophthalmology 2018;125(11):1793-1802.

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