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Retina

Case 78

Contributors: Korolos Sawires MD (C) and Dr. R. Rishi Gupta MD, FRCSC, DABO, FASRS

Patient presentation: A 65-year-old woman was referred to a tertiary care retina clinic for a second opinion of a bilateral maculopathy. The patient reported bilateral progressive blurred vision, flashes, floaters, and metamorphopsia. She had prior laser refractive surgery and bilateral cataract extraction with intraocular lens (IOL) implantation. Medical conditions included interstitial cystitis treated with pentosan polysulfate (Elmiron), and hypothyroidism treated with levothyroxine. Her family history was significant for paternal glaucoma and age-related macular degeneration (AMD).

Best corrected visual acuity (BCVA) was 20/100 OD and 20/50-1 OS with no improvement with pinhole correction. Her IOP was 8 mmHg OD, and 7 mmHg OS. There was no RAPD. Ultrawide field fundus, fundus autofluorescence, fluorescein angiography, and near infrared imaging photographs of both eyes are found below:

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Question 1: How would you describe the photographs above?

SD-OCT macula images of both eyes were taken and are shown below:

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Question 2: What abnormality is not seen on the OCT scans above?

Question 3: How can you tell which eye is being imaged based solely on the OCT?

Question 4: Based on the patient’s history and images above, what is the most likely diagnosis?

Question 5: How would you describe the patient’s choroid; what is notable about it?

References:

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  1. Pinto AM, Jain N, Gupta RR. Pentosan Polysulfate Maculopathy With 13 Years of Follow-up Imaging. J Vitreoretin Dis. May-Jun 2024;8(3):325-333. doi:10.1177/24741264241228375

  2. Lindeke-Myers A, Hanif AM, Jain N. Pentosan polysulfate maculopathy. Surv Ophthalmol. Jan-Feb 2022;67(1):83-96. doi:10.1016/j.survophthal.2021.05.005

  3. Pearce WA, Chen R, Jain N. Pigmentary Maculopathy Associated with Chronic Exposure to Pentosan Polysulfate Sodium. Ophthalmology. Nov 2018;125(11):1793-1802. doi:10.1016/j.ophtha.2018.04.026

  4. Dolz-Marco R, Glover JP, Gal-Or O, et al. Choroidal and Sub-Retinal Pigment Epithelium Caverns: Multimodal Imaging and Correspondence with Friedman Lipid Globules. Ophthalmology. Aug 2018;125(8):1287-1301. doi:10.1016/j.ophtha.2018.02.036

  5. Friedman E, Smith TR. Clinical and pathological study of choroidal lipid globules. Arch Ophthalmol. Mar 1966;75(3):334-6. doi:10.1001/archopht.1966.00970050336006

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Learning Objectives:

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  1. To identify OCT features of pentosan polysulfate maculopathy.

  2. Review layers of the retina and how to use the RNFL to determine which eye is being scanned on OCT.

  3. To identify choroidal hyporeflective caverns on OCT.

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