Acute retinal pigment epitheliitis using adaptive optics imaging: a case report
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...
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2024-11-01
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| author | P.A.T. Heutinck S. Wooning K. Liman M. Durand L. Sanchez Brea C.C.W. Klaver V.J.M. Verhoeven D. Andrade De Jesus A.A.H.J. Thiadens |
| author_facet | P.A.T. Heutinck S. Wooning K. Liman M. Durand L. Sanchez Brea C.C.W. Klaver V.J.M. Verhoeven D. Andrade De Jesus A.A.H.J. Thiadens |
| author_sort | P.A.T. Heutinck |
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| description | 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. |
| format | Article |
| id | doaj-art-5a7096fe8b224b999a1e9286b337e5e9 |
| institution | Kabale University |
| issn | 1471-2415 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | BMC |
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| series | BMC Ophthalmology |
| spelling | doaj-art-5a7096fe8b224b999a1e9286b337e5e92024-12-01T12:14:56ZengBMCBMC Ophthalmology1471-24152024-11-0124111110.1186/s12886-024-03768-0Acute retinal pigment epitheliitis using adaptive optics imaging: a case reportP.A.T. Heutinck0S. Wooning1K. Liman2M. DurandL. Sanchez Brea3C.C.W. Klaver4V.J.M. Verhoeven5D. Andrade De Jesus6A.A.H.J. Thiadens7Department of Ophthalmology, Erasmus MC, University Medical CenterEye Image Analysis Group Rotterdam, Department of Radiology & Nuclear Medicine, Erasmus MC, University Medical CenterDepartment of Ophthalmology, Erasmus MC, University Medical CenterEye Image Analysis Group Rotterdam, Department of Radiology & Nuclear Medicine, Erasmus MC, University Medical CenterDepartment of Ophthalmology, Erasmus MC, University Medical CenterDepartment of Ophthalmology, Erasmus MC, University Medical CenterEye Image Analysis Group Rotterdam, Department of Radiology & Nuclear Medicine, Erasmus MC, University Medical CenterDepartment of Ophthalmology, Erasmus MC, University Medical CenterAbstract 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.https://doi.org/10.1186/s12886-024-03768-0Adaptive optics flood illumination ophthalmoscopyAcute retinal pigment epitheliitisKrill’s diseaseCone photoreceptors |
| spellingShingle | P.A.T. Heutinck S. Wooning K. Liman M. Durand L. Sanchez Brea C.C.W. Klaver V.J.M. Verhoeven D. Andrade De Jesus A.A.H.J. Thiadens Acute retinal pigment epitheliitis using adaptive optics imaging: a case report BMC Ophthalmology Adaptive optics flood illumination ophthalmoscopy Acute retinal pigment epitheliitis Krill’s disease Cone photoreceptors |
| title | Acute retinal pigment epitheliitis using adaptive optics imaging: a case report |
| title_full | Acute retinal pigment epitheliitis using adaptive optics imaging: a case report |
| title_fullStr | Acute retinal pigment epitheliitis using adaptive optics imaging: a case report |
| title_full_unstemmed | Acute retinal pigment epitheliitis using adaptive optics imaging: a case report |
| title_short | Acute retinal pigment epitheliitis using adaptive optics imaging: a case report |
| title_sort | acute retinal pigment epitheliitis using adaptive optics imaging a case report |
| topic | Adaptive optics flood illumination ophthalmoscopy Acute retinal pigment epitheliitis Krill’s disease Cone photoreceptors |
| url | https://doi.org/10.1186/s12886-024-03768-0 |
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