Acute Macular Neuroretinopathy Associated with COVID-19 Pandemic: A Real-world Observation Study.

To evaluate the clinical and retinal imaging features of Chinese patients with acute macular neuroretinopathy (AMN) associated with COVID-19. A prospective observational study. Retinal imaging, including color fundus photography, near-infrared imaging (NIR), swept-source optical coherence tomography (SS-OCT), optical coherence tomography angiography (OCTA), and Humphrey perimetry, were conducted for each case. All cases were included within the first three months following the pandemic outbreak. A total of 12 male patients (36.36 %) and 21 female patients (63.64 %) were prospectively recruited, and 29 cases (87.88 %) were bilaterally affected. The median interval between the onset of fever and the appearance of ocular symptoms was two days (range, 0.5-5.0 days). Apart from the outer retinal changes typical of AMN, changes in the inner retinal layers were observed, including intraretinal hemorrhage (8.06 %), cotton wool spots (9.68 %), and paracentral acute middle maculopathy (PAMM) (8.06 %). Smaller retinal inner nuclear layer hyperreflective speckles (RIHS) (41.94 %) were identified as a distinguishing feature from typical PAMM. Voids of vessel signals were found in the superficial (11.54 %), intermediate (82.69 %), and deep capillary plexus (98.08 %), and in the choriocapillaris (19.23 %) on OCTA. Humphrey perimetry illustrated central, paracentral, and peripheral scotomas. The occult lesions associated with AMN, PAMM, and some of the RIHS illustrated by OCT were visualized topographically and further confirmed by OCTA as perfusion defects. An increase in AMN cases correlated with the SARS-CoV-2 outbreak. Additional features, including widespread inner retinal perfusion deficits, were observed and may serve as potential biomarkers for systemic microcirculation dysregulation in COVID-19.

Song X ，Yu Y ，Zhou H ，Zhang Y ，Mao Y ，Wang H ，Cao X ，Zhu X ，Li Z ，Li L ，Liu J ，Peng X ，Li Q ... -  《Asia-Pacific Journal of Ophthalmology》

The clinical features and perfusion density in paracentral acute middle maculopathy by optical coherence tomography angiography study.

To observe the clinical features of the paracentral acute middle maculopathy (PAMM) and to investigate the changes in perfusion density of superficial capillary plexus (SCP) and deep capillary plexus (DCP) in PAMM by optical coherence tomography angiography (OCTA). Twelve patients (12 eyes) diagnosed with PAMM and 13 healthy participants (13 eyes) were enrolled. The clinical features of 12 PAMM patients were recorded. The perfusion density of SCP and DCP in the parafovea and perifovea (divided into Temporal, Superior, Nasal, and Inferior) was measured by OCTA in PAMM and healthy eyes. Automated macular maps, including SCP and DCP, were also performed. The mean age of patients was 48.08 (range 14-72) years. The best corrected visual acuity (BCVA) was from finger count (FC) to 1.0. Risk factors included hypertension, diabetes mellitus, COVID-19, etc. The patients had signs of retinal vascular disease except for one patient with isolated PAMM. A spectral-domain OCT image of the PAMM eye showed hyperreflectivity in the inner nuclear layer (INL). Compared with healthy eyes, SCP and DCP in PAMM eyes were significantly reduced in all sectors (all p < 0.05) in the parafovea and perifovea. The average perfusion density of SCP in parafovea and perifovea in PAMM eyes were 46.94±4.34% vs 52.21±3.98 % (p = 0.002), 44.01±3.95% vs 50.51±3.14 (p = 0.000) in healthy eyes. The average perfusion density of DCP in parafovea and perifovea in PAMM eyes were 51.22±4.16% vs 57.15±3.87 % (p = 0.002), 47.375±6.23% vs 54.27±5.33 % (p = 0.002) in healthy eyes. PAMM often accompanies concurrent ocular and systemic diseases. OCTA results confirm that the perfusion density of SCP and DCP in the parafovea and perifovea significantly reduced in PAMM patients.

Liu Z ，Pan X ，Wang D ，Zou Y ，Liu P ，Wang Y ，Bi H ，Xie X ... -  《-》

Case report: Acute macular neuroretinopathy post-COVID-19 infection.

Acute macular neuroretinopathy (AMN) is a rare disease that causes transient or permanent visual disturbance. The exact etiology remains unknown, but vascular compromise of the deep retinal capillary plexus was postulated as the main mechanism. Retinal vascular event post-coronavirus disease 2019 (COVID-19) infection is recently highlighted during the pandemic, which includes AMN. To report a case of AMN post-COVID-19 infection. A 24-year-old Indian woman presented with acute-onset painless bilateral central scotoma for a day. The symptom was preceded by a history of COVID-19 infection 3 weeks prior. Ocular examination revealed a near-normal visual acuity for both eyes. Fundus examination showed bilateral dull foveal reflex with mild scattered cotton wool spot and vascular tortuosity. Optical coherence tomography macula revealed a distinct short hyperreflective band involving the outer plexiform and outer nuclear layers nasal to the fovea. The Bjerrum perimetry test revealed central scotoma temporal to the fixation. Optical coherence tomography lesions and scotomas are identical and congruous in both eyes. Serial fundus photographs are captured showing the evolving changes of near-normal macula to pigmented wedge-shaped petaloid lesions around the fovea. The patient was diagnosed as bilateral AMN and treated with oral prednisolone. On subsequent follow-up, the central scotoma improved. This case illustrates a clear temporal and possible causal relationship of COVID-19 infection with AMN. Further studies and data are required to justify its association, but the rising cases of AMN shall be anticipated as COVID-19 infections have become endemic worldwide.

Mohamad NA ，Yong MH ，Mohd Khialdin S ，Bastion MC ... -  《-》

Optical coherence tomography angiography suggests choriocapillaris perfusion deficit as etiology of acute macular neuroretinopathy.

Acute macular neuroretinopathy (AMN) can cause sudden-onset and permanent scotoma in healthy young patients. Analysis of optical coherence tomography (OCT) and OCT angiography (OCTA) of AMN patients may provide insights into disease mechanism. We conducted a retrospective study of consecutive SARS-Cov-2-related AMN patients that presented in our clinic between Jan 1st, 2022, and April 30th, 2023, within 30 days of symptom onset. Retinal vessel area density (VAD) of AMN lesions in OCTA was quantified and compared to an adjacent tissue control (ATC). This quantification was performed for the superficial vascular plexus (SVP), the intermediate capillary plexus (ICP), the deep capillary plexus (DCP), the choriocapillaris (CC), and choroid. Furthermore, en face OCT images were analyzed. Nine AMN patients were identified, 6 of these (4 female, 2 male, average age 25 years) fulfilled the inclusion criteria and were included into this study. Average time from symptom onset to OCTA was 14.3 days. No VAD differences between AMN and adjacent tissue were found in either retinal layer (SVP, ICP, DCP). In contrast, VAD in CC was reduced by 27% against the ATC (p = 0.007) and choroidal VAD was reduced by 41% (p = 0.017). Further analysis of en face OCT could show that the pathognomonic infrared hyporeflectivity in AMN is caused by photoreceptor alterations rather than changes in the inner retinal layers. Our data suggests that a perfusion deficit in the choroidal layers is responsible for AMN rather than in the DCP, which is the predominant hypothesis in current literature.

von der Burchard C ，Gruben A ，Roider J 《-》

Association of Acute Macular Neuroretinopathy or Paracentral Acute Middle Maculopathy with Sickle Cell Disease.

To investigate the structural and microvascular changes in the retina and choriocapillaris in patients with sickle cell disease (SCD) demonstrating acute vision loss and acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) using multimodal imaging including OCT and OCT angiography (OCTA). Retrospective case series. Four hemoglobin SS (HbSS) or hemoglobin SC (HbSC) patients who demonstrated vision loss attributed to AMN (n = 2) or PAMM (n = 2). Clinical characteristics including best-corrected visual acuity and multimodal imaging features from fundus photography, fluorescein angiography, OCT, and OCTA were analyzed. Longitudinal changes in the structure and microvasculature of the retina and choriocapillaris were examined. In 2 patients with AMN, characteristic hyperreflective changes were observed in the outer nuclear layer with involvement of the ellipsoid and interdigitation zones. In 1 patient, the lesion was located in the foveal avascular zone, and only flow deficits in the choriocapillaris were noted. In the second patient, no flow loss was observed in the superficial capillary plexus (SCP) corresponding to the lesion, but flow loss was seen in the deep capillary plexus (DCP) and choriocapillaris. At the respective 1-year and 4-month follow-up examinations, the hyperreflectivities improved with no ensuing retinal atrophy. Deep capillary plexus and choriocapillaris flow also improved. Of the 2 patients with PAMM, 1 had PAMM in association with branch retinal arteriolar occlusions. In both patients, a hyperreflective band involving the inner plexiform, inner nuclear, and outer plexiform layers was seen in or adjacent to areas of prior macular thinning. These areas corresponded to flow deficits in both the SCP and DCP. At the respective 1-month and 1-year follow-up examinations, the hyperreflective band improved with ensuing atrophy of the middle retinal layers. OCT angiography demonstrated worsening of flow deficits in the corresponding SCP and DCP. To the best of our knowledge, this is the first report of AMN and the largest case series of AMN or PAMM in SCD to date. Paracentral acute middle maculopathy may precede macular thinning commonly observed in SCD. OCT angiography is a useful tool to demonstrate associated microvascular changes in AMN and PAMM in SCD.

Ong SS ，Ahmed I ，Scott AW 《-》