case_images: PMC11324243_01
case_images: Clinical course of pachydrusen, as illustrated by color fundus photography and optical coherence tomography (OCT). The patient presented with subfoveal yellowish lesions, with a halo of retinal pigment epithelium (RPE) alteration in the left eye (OS). Corresponding OCT showed a poorly defined RPE layer with sub-RPE hyperreflective material that extended to the ellipsoidal zone. a Enlarged choroidal hyporeflective lumens (pachyvessels) were observed in the Haller's layer underneath the fovea (asterisk). A decrease in lesion size with partial resolution of drusenoid appearance was observed at 3 (b) and 8 (c) months, after initiation of evolocumab cholesterol-lowering treatment. d A noticeable deposit of newly assembled material in the RPE basal extracellular space was detected 8 months after drug discontinuation. All OCT images are not provided with their concurrent infrared scans since the 21-HD scan mode was utilized to acquire and export them.
case_images: cop-2024-0015-0001-540014_F01.jpg
case_images: PMC11324243_01_cop-2024-0015-0001-540014_F01.jpg
case_images: F1
case_images: The optical coherence tomography (OCT; CIRRUS  5000, ZEISS, Dublin, CA, USA) revealed a sub-RPE hyperreflective lesion extending to the photoreceptor layers, concurrent with choroidal pachyvessels (Fig. 1a) and having the largest horizontal dimension of 768 mum. Despite a subfoveal choroidal thickness of 259 mum (axial length, 24.10 mm), the diagnosis was changed from adult-onset foveomacular vitelliform dystrophy to pachydrusen because of the unilateral involvement and material predominantly located within the sub-RPE space, coinciding with choroidal pachyvessels underneath the lesion (Fig. 1a). Three months later (March 2019), her vision improved to 20/32-2, and the lesion's dimension shrank to 684 mum (Fig. 1b). A corresponding OCT image revealed the lesion only remaining in the sub-RPE pocket of the foveolar regions and partial restoration of ellipsoid and interdigitation zones without pigmentary migration (BCVA, 20/20-2 OS) (Fig. 1c). Eight months after drug discontinuation, her vision deteriorated to 20/40, consistent with her baseline visual acuity, and the OCT scans indicated a recurrence of subretinal deposits extending to the myoid zone (Fig. 1d).
case_images: PPE and the patient's clinical responses during a second course of cholesterol therapy. a The patient later developed serous pigment epithelial detachment (white arrowhead) inferonasal to the fovea, corresponding with pachyvessels and overlying collapsed choriocapillaris in the right eye. The recurrent lesion in the left eye (b) was reabsorbed again, starting from the subretinal space, and progressing outward, at 2 (c) and 7 (d) months after restarting evolocumab.
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case_images: PMC11324243_01_cop-2024-0015-0001-540014_F02.jpg
case_images: F2
case_images: OD; 20/50 OS). Ocular examination revealed perifoveal pachychoroid pigment epitheliopathy inferonasal to the fovea (Fig. 2a) with significant cataract (OD) and non-ischemic branch retinal vein occlusion affecting the inferonasal branch (OS). Increased subfoveal deposits with overlying disintegrated photoreceptor layers were observed (OS) (Fig. 2b). After five injections (February 2023), the material was substantially reabsorbed, beginning from the subretinal space (Fig. 2c), but the photoreceptor layers remained disintegrated, and OCT angiography revealed no macular neovascularization (LDL-C, 70 mmol/L) (Fig. 2d).