Patient Presentation: A 23-year-old obese female was diagnosed with idiopathic intracranial hypertension (IIH) and referred to neurosurgery for ventriculoperitoneal shunt. A baseline ocular examination was performed prior to the procedure.
On examination, vision was 20/200 in the right eye, and 20/40 in the left eye. There was a right relative afferent pupillary defect. Slit lamp examination was normal.
A dilated fundus examination was performed demonstrating the following:
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Retina
Case 79
Patient presentation: A 61-year-old woman of Asian descent, with a 12-year history of systemic lupus erythematosus (SLE) treated with a daily dose of 5.83mg/kg hydroxychloroquine, was referred to a comprehensive ophthalmologist for a retinal examination. She did not endorse any ocular symptoms. On examination, best-corrected distance visual acuity was 20/20 OD and 20/20 OS. IOP was 17 mmHg OD and 16 mmHg OS. Colour fundus photos and macular OCT imaging are shown below:
Question 2: What will typically not produce parafoveal ellipsoid zone loss?
Fundus autofluorescence and 24-2/10-2 Visual Fields were performed and are shown below:
Question 3: Based on the patient’s history and images above, what is the most likely diagnosis?
Question 4: What other disease entities can lead to a Bull’s Eye Maculopathy?
Question 5: Which of the following correctly conveys incidence of hydroxychloroquine toxicity at a dose higher than recommended by AAO guidelines (>5mg/kg/day)?
References:
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Yusuf IH, Sharma S, Luqmani R, Downes SM. Hydroxychloroquine retinopathy. Eye (Lond). 2017;31(6):828-845.
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Schmitz-Valckenberg S, Holz FG, Bird AC, Spaide RF. Fundus autofluorescence imaging: review and perspectives. Retina. 2008;28(3):385-409.
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Marmor MF, Kellner U, Lai TYY, et al. Recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology. 2016;123(6):1386-1394.
Learning Objectives:
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Hydroxychloroquine toxicity causes disruption of the outer retinal layers with ‘saucer sign’ indicating sparing of the subfoveal region with predominantly parafoveal disruption.
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Fundus autofluorescence (FAF) and automatic perimetry are necessary to screen for plaquenil toxicity and monitor progression.
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The risk of hydroxychloroquine toxicity is determined based on real weight and duration of therapy.