Acute retinal pigment epitheliitis using adaptive optics imaging: a case report
BMC Ophthalmology, vol. 24, pp. 507
Abstract
BACKGROUND: Acute Retinal Pigment Epitheliitis (ARPE, Krill's disease) is a rare inflammatory retinal disorder commonly affecting young adults. It often presents unilaterally with central vision disruption, and typically resolves with vision restoration within 6 to 12 weeks. The pathogenesis of ARPE remains a subject of ongoing debate. Adaptive Optics Flood Illumination Ophthalmoscopy (AO-FIO) imaging has emerged as a valuable tool capable of detecting early cone photoreceptor changes and recovery. This case study presents two patients with ARPE, with longitudinal follow-up using multimodal imaging, including optical coherence tomography (OCT) and AO-FIO. CASE PRESENTATIONS: A 30-year-old male presented with sudden vision loss in both eyes. The best corrected visual acuity (BCVA) was 20/33 and 20/40 Snellen in the right and left eye, respectively. OCT showed interruption of the ellipsoid zone (EZ) band and outer nuclear layer (ONL) in both eyes; AO-FIO imaging revealed a foveal lesion and diminished parafoveal cone density in both eyes compared to two age-matched controls. After 6 months, BCVA was restored to 20/20, and OCT showed recovery of the ONL and EZ. On AO-FIO, the foveal lesion was still present and the parafoveal cone density increased but remained reduced even up to 15 months after onset when compared to the controls. The second patient, a 30-year-old woman, presented with a unilateral drop in vision to 20/63 Snellen. OCT showed discontinuation of the EZ and hyperreflectivity within the ONL and retinal pigment epithelium in the affected eye. The unaffected eye showed no abnormalities. After 3 months, the BCVA improved to 20/16 Snellen and OCT showed recovery of the EZ. AO-FIO was conducted 9 months after onset and revealed reduced parafoveal cone density in the affected and non-affected eye compared to the controls while OCT still showed recovery of all retinal layers. CONCLUSIONS: ARPE is a self-limiting disease with recovery of BCVA and OCT retinal layers within 6 months. However, our 2 cases showed that parafoveal cone density recovered during follow-up but did not reach levels observed in controls. AO-FIO is an imaging modality that enhances sensitivity in measurements and can therefore be used as a complementary tool for follow-up.
Authors 8
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Affiliation as printed
Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
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Leiden University · Erasmus MC
Affiliation as printed
Eye Image Analysis Group Rotterdam, Department of Radiology & Nuclear Medicine, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Leiden Institute of Advanced Computer Science, Leiden University, Leiden, The Netherlands
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Affiliation as printed
Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
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Erasmus MC · Rotterdam Eye Hospital
Affiliation as printed
Eye Image Analysis Group Rotterdam, Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Eye Image Analysis Group Rotterdam, Department of Radiology & Nuclear Medicine, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Eye Image Analysis Group Rotterdam, Rotterdam Ophthalmic Institute, Rotterdam Eye Hospital, Rotterdam, The Netherlands
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University of Basel · Erasmus MC · Radboud University Medical Center · Institute of Molecular and Clinical Ophthalmology Basel
Affiliation as printed
Department of Epidemiology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Department of Ophthalmology, Radboud University Medical Center, Nijmegen, The Netherlands
Institute of Molecular and Clinical Ophthalmology, University of Basel, Basel, Switzerland
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Affiliation as printed
Department of Clinical Genetics, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
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Erasmus MC · Rotterdam Eye Hospital
Affiliation as printed
Eye Image Analysis Group Rotterdam, Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Eye Image Analysis Group Rotterdam, Department of Radiology & Nuclear Medicine, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
Eye Image Analysis Group Rotterdam, Rotterdam Ophthalmic Institute, Rotterdam Eye Hospital, Rotterdam, The Netherlands
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Affiliation as printed
Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands. a.thiadens@erasmusmc.nl
Department of Ophthalmology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands
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