Ophthalmology Department, IRCCS San Raffaele Hospital, San Raffaele Vita- Salute University, Milan, Italy.
Division of Endocrinology, IRCCS San Raffaele Hospital, San Raffaele Vita- Salute University, Milan, Italy; and.
Retin Cases Brief Rep. 2022 Sep 1;16(5):543-546. doi: 10.1097/ICB.0000000000001061. Epub 2020 Nov 6.
To describe a case of paracentral acute middle maculopathy associated with central retinal vein occlusion in a patient affected by amyloid light-chain amyloidosis.
One patient with confirmed diagnosis of amyloid light-chain amyloidosis, displaying paracentral acute middle maculopathy and central retinal vein occlusion, was recruited. The patient underwent complete ophthalmologic examination and multimodal imaging, including: fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography.
Fundus autofluorescence showed a ferning pattern, corresponding to linear hypofluorescence in late-phase indocyanine green angiography and delayed venous filling, detected by fluorescein angiography. Structural optical coherence tomography revealed a hyper-reflective line located in the outer plexiform layer, corresponding to the prominent middle limiting membrane, along with several placoid lesions. Optical coherence tomography angiography found that the superficial capillary plexus was preserved, whereas vessel density was reduced in both the deep capillary plexus and the choriocapillaris. After 1 year of follow-up, the patient achieved an almost complete morphological recovery.
Multimodal imaging described in depth the morphological features of a case of combined paracentral acute middle maculopathy and central retinal vein occlusion in a patient affected by amyloid light-chain amyloidosis.
描述 1 例淀粉样轻链淀粉样变性患者合并中心性视网膜静脉阻塞的旁中心急性中黄斑病变。
招募了 1 例确诊为淀粉样轻链淀粉样变性、表现为旁中心急性中黄斑病变和中心性视网膜静脉阻塞的患者。该患者接受了全面的眼科检查和多模态成像，包括：眼底自发荧光、荧光素血管造影、吲哚青绿血管造影、谱域光相干断层扫描和光相干断层扫描血管造影。
眼底自发荧光显示出扇形模式，对应于吲哚青绿血管造影晚期的线性低荧光和荧光素血管造影检测到的静脉充盈延迟。结构光相干断层扫描显示在外丛状层有一条高反射线，对应于突出的中层限膜，并伴有几个斑块状病变。光相干断层扫描血管造影发现浅层毛细血管丛保留，而深层毛细血管丛和脉络膜毛细血管的血管密度降低。经过 1 年的随访，患者获得了几乎完全的形态学恢复。
多模态成像详细描述了 1 例淀粉样轻链淀粉样变性患者合并中心性视网膜静脉阻塞的旁中心急性中黄斑病变的形态特征。
