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.
BMC Ophthalmol. 2024 Nov 25;24(1):507. doi: 10.1186/s12886-024-03768-0.
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.
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.
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.
急性视网膜色素上皮炎（ARPE，又名 Krill 病）是一种罕见的炎症性视网膜疾病，常见于年轻人。它常单侧发生，伴有中心视力障碍，通常在 6 至 12 周内视力恢复。ARPE 的发病机制仍存在争议。自适应光学眼底血管造影（AO-FIO）成像已成为一种有价值的工具，能够检测早期的视锥细胞变化和恢复情况。本病例研究报告了两例 ARPE 患者，通过多模态成像（包括光学相干断层扫描（OCT）和 AO-FIO）进行纵向随访。
一名 30 岁男性因双眼突发性视力丧失就诊。右眼最佳矫正视力（BCVA）为 20/33，左眼为 20/40。OCT 显示双眼的椭圆体带（EZ）和外核层（ONL）中断；AO-FIO 成像显示双眼与两个年龄匹配的对照组相比，中心凹病变和旁中心凹视锥细胞密度降低。6 个月后，BCVA 恢复至 20/20，OCT 显示 ONL 和 EZ 恢复。AO-FIO 显示中心凹病变仍然存在，旁中心凹视锥细胞密度增加，但与对照组相比，即使在发病后 15 个月仍降低。第二位患者，一名 30 岁女性，因单侧视力下降至 20/63 Snellen 就诊。OCT 显示受影响眼的 EZ 中断，ONL 内高反射性和视网膜色素上皮内高反射性。未受影响的眼未见异常。3 个月后，BCVA 提高至 20/16 Snellen，OCT 显示 EZ 恢复。发病后 9 个月进行 AO-FIO，结果显示受影响眼和未受影响眼的旁中心凹视锥细胞密度与对照组相比降低，而 OCT 仍显示所有视网膜层的恢复。
ARPE 是一种自限性疾病，BCVA 和 OCT 视网膜层在 6 个月内恢复。然而，我们的 2 个病例显示，旁中心凹视锥细胞密度在随访中恢复，但未达到对照组的水平。AO-FIO 是一种增强测量灵敏度的成像方式，因此可作为随访的补充工具。
