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BackgroundPurtscher-like retinopathy (PLR) is a rare retinal microangiopathy with unclear pathogenesis. Paracentral acute middle maculopathy (PAMM) is an optical coherence tomography (OCT) sign proposed in recent years, which is characterized by infarction of the middle layer of the retina. This article reported a rare case of PRL and PAMM probably related to improper antihypertensive drug use in a middle-aged male.Case presentationA 49-year-old man presented with a complaint of sudden-onset vision loss and paracentral scotomas in the right eye for approximately 1 week. At presentation, the best-corrected visual acuity (BCVA) was 20/63 OD and 20/20 OS. Fundus examination showed multiple cotton-wool spots and Purtscher flecken in the posterior segment of the right eye. OCT revealed hyper-reflectivities in the inner nuclear layer (INL), consistent with PAMM. En face OCT showed PAMM’s characteristic “fern-like” perivenular changes. Fluorescein angiography demonstrated prolonged arm-to-retina time, delayed artery and venous filling, and hypofluorescence corresponding to cotton-wool spots. Examinations of the left eye were unremarkable. Many imaging and laboratory tests were performed to detect the possible cause of PLR and PAMM, but no possible explanation was found except improper antihypertensive drug use. The patient was recommended to stop his antihypertensive medication, and prescribed other systemic medicines, including oral prednisolone (40 mg q.d. with gradual tapering), oral cobalamin (0.5 mg t.i.d.), and subcutaneous injections of compound anisodine (2.0 mL q.d.) beside the superficial temporal artery. Two weeks after onset, his BCVA improved to 20/25 in the right eye. During follow-ups, his BCVA recovered to 20/20, accompanied by the regression of fundus lesions. The patient reported no treatment-related adverse effects.ConclusionThis is the first reported case of PLR and PAMM following improper antihypertensive drug use. Our report expands our understanding of the etiology and pathophysiology of PLR and PAMM. We also stress the importance of proper application of medications in clinical practice.

Purpose: To describe and evaluate the multimodal imaging findings in retinal microvascular ischemia associated with COVID-19 infection. Methods: Patients with COVID-19 associated retinal microvascular ischemia and visiting the outpatient Department of Ophthalmology, Shanghai General Hospital from December 2022, to February 2023, were documented and their multimodal images were retrospectively reviewed. Retinal microvascular ischemia was defined as the presence of isolated or multiple focal retinal whitening(s) on color fundus images. Patients with retinal vessel occlusion or retinopathies secondary to systematic disorders diagnosed before infection were excluded. Results: A total of 32 eyes from 21 patients were included, 24 (75.00 %) eyes with multiple retinal whitenings, while 8 (25.00 %) eyes with isolated lesions. When divided by the types of ischemia, 9 (28.13 %) eyes had only inner retinal involvement (known as cotton wool spot, CWS), 4 (12.50 %) eyes had only middle retinal involvement (known as paracentral acute middle maculopathy, PAMM), and 19 (59.38 %) eyes had both. In addition, 4 (12.50 %) eyes had coincident angular sign of Henle fiber layer hyperreflectivity (ASHH). Patients with hypertension tended to have multiple lesions rather than isolated lesion of retinal microvascular ischemia (P = 0.008). Transient uncontrolled high blood pressure or acute kidney injury was simultaneously detected in some cases. Conclusions: Ocular manifestation of COVID-19 associated microvascular ischemia can be variable, including CWS, PAMM and ASHH. Multimodal fundus imaging technologies are useful tools to reveal involved retinal layers, extent, and severity. Moreover, ocular manifestations may serve as a window of COVID-19 related microcirculation in other systems throughout the body.

Purpose: To describe a representative case and review the literature on paracentral acute middle maculopathy (PAMM) and giant cell arteritis (GCA). Methods: A review of the English-language ophthalmic literature was performed using the search terms of PAMM, giant cell arteritis, and temporal arteritis. Results: A 72-year-old woman with PAMM as the presenting ophthalmic manifestation of GCA was described with a review of the prior cases from the literature. It was found that there were 26 cases of PAMM in GCA. In 19 of 26 cases, PAMM was associated with no other fundus abnormalities and was only seen on multimodal imaging including optical coherence tomography. Conclusion: PAMM can cause acute paracentral visual loss, and GCA should be suspected in all cases of PAMM of the elderly patients, even when isolated and not associated with constitutional symptoms of GCA. [ABSTRACT FROM AUTHOR]

Background: To evaluate the incidence rate of small paracentral acute middle maculopathy (PAMM) lesions in healthy fellow eyes of patients with retinal vein occlusions (RVO). Methods: Patients with unilateral branch RVO or central RVO who were followed up for at least one year were included. Fellow healthy eyes were examined with 6 mm optical coherence tomography angiography scans at baseline and at the end of the follow-up. Small resolved PAMM lesions were displayed using structural en face projections of the slab between two segmentation lines of the outer plexiform layer with 0 µm and −9 µm offset. New lesions were identified by overlaying baseline and final structural en face projections. Results: A total of 41 eyes of 41 patients (12 females and 29 males, mean age 63.5 ± 10.1 years) were included. The mean follow-up was 23.6 ± 11.4 months. Among all RVO patients, small resolved PAMM lesions in healthy fellow eyes were found in 39 patients (95.1%). In the whole cohort, the median number of PAMM lesions was 5.5 (95% CI 3.0 to 8.0) (mean 6.5 ± 5.7) at baseline, which increased statistically significantly to 5.5 (95% CI 3.8 to 8.2) (mean 6.7 ± 5.7) at the end of the follow-up period (p = 0.03). Eight (19.5%) patients demonstrated new lesions, with a median number of new lesions of 1 (ranged 1 to 2). Conclusions: Small PAMM lesions seem to be a slowly progressing retinal vascular biomarker.

Purpose: To report a case of paracentral acute middle maculopathy in a pediatric patient with sickle cell trait. Methods: The patient was evaluated with a comprehensive ophthalmic examination, optical coherence tomography, optical coherence tomography angiography, and fluorescein angiography. Results: Acute loss of vision occurred immediately after an uneventful scleral buckling procedure for retinal detachment in a 16-year-old female patient. Retinal imaging studies confirmed the occurrence of paracentral acute middle maculopathy, an ischemic condition characterized by infarction of the inner nuclear layer of the retina caused by hypoperfusion of the intermediate and deep capillary plexuses. Laboratory evaluation was only remarkable for sickle cell trait. Over a course of 3 months after the loss of vision, visual acuity improved from 20/1,000 to 20/20, and optical coherence tomography lesion resolved to a parafoveal area of inner and middle retinal layer thinning. Paracentral scotoma corresponding to the atrophic area persisted. Conclusion: Dehydration because of the presurgical nil-per-os status and transient increase in intraocular pressure during the buckling surgery may have predisposed this patient with sickle cell trait to a sickling event that caused localized ischemia in the middle retina's end-capillaries. Avoiding long nil-per-os status, being mindful of transient intraocular pressure elevation during scleral buckling procedures, and presurgical hemoglobin electrophoresis in at-risk populations are recommended to prevent sickling attacks during retinal surgeries in individuals with sickle cell trait. [ABSTRACT FROM AUTHOR]

Paracentral acute middle maculopathy (PAMM) is identified by the occurrence of an inner nuclear layer (INL) hyperreflective band on optical coherence tomography (OCT), which was subsequently accompanied by INL atrophy. A multitude of systemic diseases and vasculopathy risk factors have been linked to PAMM. PAMM, which has been associated with infection with Coronavirus disease 2019(COVID 19), may indicate post-infectious complications. By influencing vascular endothelial cells, COVID-19 infection can induce an inflammatory and procoagulatory state, resulting in vascular thromboembolic complications, although the precise mechanisms underlying these complications remain unknown. Thus, the present review explores and discusses about paracentral acute middle maculopathy, its diagnosis and treatment and adds a note on its implications after the outbreak of COVID 19. [ABSTRACT FROM AUTHOR]

Purpose: To report a case of bilateral paracentral acute middle maculopathy lesions on spectral domain-optical coherence tomography(OCT) secondary to severe Plasmodium falciparum malaria. Methods: Retrospective case report. Spectral domain-OCT, ultra-widefield fluorescein angiography, and OCT angiography were performed and analyzed. Results: A 54-year-old healthy man presented with acute vision loss in both eyes few days after being diagnosed with severe Plasmodium falciparum malaria. Ophthalmoscopic examination was unremarkable, but near-infrared reflectance imaging showed patchy hyporeflective areas located at the terminal tips of the venous branches. Corresponding spectral-domain OCT demonstrated alternating bands of hyperreflectivity involving the inner nuclear layer, consistent with skip paracentral acute middle maculopathy lesions. Optical coherance tomography angiography illustrated corresponding flow signal loss at the level of the deep capillary plexus. Ultra-widefield fluorescein angiography showed peripheral retinal vein staining and capillary nonperfusion. Conclusion: Paracentral acute middle maculopathy may be an OCT manifestation of malarial retinopathy associated with severe Plasmodium falciparum infection. [ABSTRACT FROM AUTHOR]

Abstract Background To report a rare case of pulmonary and ocular complications with visual loss due to bilateral Purtscher-like retinopathy and paracentral acute middle maculopathy (PAMM) following a hyaluronic acid (HA) filler injection to the breast. Systemic and visual recovery was attained following corticosteroid therapy. Case presentation A 27-year-old lady presented with painless blurring of vision in both eyes for 2 weeks following hyaluronic acid breast filler injections by a non-medical practitioner. She was initially admitted to the medical ward for diffuse alveolar haemorrhage and altered sensorium. The presenting visual acuity was counting fingers in both eyes. Bilateral dilated fundus examination showed hyperaemic discs, concentric rim of retinal whitening around macula with patches of polygonal-shaped retinal whitening, generalised cotton-wool spots, tortuous veins, and flame-shaped haemorrhages. Spectral-domain optical coherence tomography (SD-OCT) macula revealed hyper-reflective bands at the inner nuclear layer (INL). Fluorescein angiography demonstrated hot discs, delayed arm-to-retina time, arterial filling, and arterio-venous transit time with staining of the vessels at the posterior pole. She was managed with a tapering dose of systemic corticosteroids. The visual acuity improved to 6/12 over 8 weeks with significant anatomical and functional improvement. Dilated fundus examination showed resolution of initial funduscopy findings. The hyper-reflective bands on the OCT had resolved with subsequent thinning of the INL and disorganisation of retinal inner layers. Conclusion Filler injections are in increasing demand and are frequently being performed by non-medical practitioners. Visual loss from non-facial HA fillers is rare. Inadvertent entry of HA into a blood vessel may potentially cause systemic and sight-threatening ocular complications. Good anatomical knowledge and proper injection technique are vital in preventing this unfortunate sequela. There are limited reports on successful visual recovery following various treatment approaches and we hope this case provides valuable insights.

Hui Xiao,&ast; Ni Guo,&ast; Junyi Li, Ling Jin, Yuan Liu, Shufen Lin, Lei Fang, Xing Liu, Chengguo Zuo State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science, Guangdong Provincial Clinical Research Center for Ocular Diseases, Guangzhou, 510060, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Xing Liu; Chengguo Zuo, Zhongshan Ophthalmic Center, Sun Yat-sen University, 7 Jinsui Road, Guangzhou, 510000, People’s Republic of China, Email liuxing@mail.sysu.edu.cn; zuochengguo@mail.sysu.edu.cnPurpose: To report the prevalence, clinical characteristics and risk factors for paracentral acute middle maculopathy (PAMM) following acute primary angle closure (APAC) and acute primary angle closure glaucoma (APACG).Methods: This retrospective study consecutively recruited patients diagnosed with APAC or APACG. Based on the spectral domain optical coherence tomography characteristics, PAMM eyes were divided into three stages. Characteristics of different stages such as the time from symptoms to treatment (TST), retinal thickness and BCVA improvement were analyzed. The risk factors of PAMM were evaluated by binary logistic regression models.Results: A total of 781 eyes of 781 APAC or APACG patients were included, and PAMM was found in 22 (2.9%) of them. Stage III eyes had a significantly longer TST than stage I eyes (P = 0.008) while exhibiting significantly thinner retinal thicknesses (P < 0.0001). The BCVA improvement was significantly worse in the eyes treated in stage III than in those treated in stage I (P = 0.008). Older age, longer axial length and without type 2 diabetes were associated with a lower risk of incident PAMM (OR = 0.95, P = 0.028; OR = 0.52, P = 0.019; OR = 3.92, P = 0.022).Conclusion: PAMM can be secondary to APAC or APACG at a rate of 2.9%. Different visual outcomes were observed in patients who received the intervention at different stages of PAMM. Younger patients with a shorter axial length and type 2 diabetes were found to be more susceptible to PAMM.Keywords: paracentral acute middle maculopathy, acute primary angle closure, acute primary angle closure glaucoma, risk factors, spectral domain optical coherence tomography

Paracentral acute middle maculopathy (PAMM) has recently been described following episodes of migraine. In this report, we present a case of PAMM and describe the role of en face optical coherence tomography (OCT). A 75-year-old woman presented with subjective vision loss over a 2-week period in the right eye. She was known for migraines with aura that presented with progressive spreading of positive and negative visual phenomena which usually resolved in under an hour. Her recent migraine episode was “atypical,” as it lasted 3 days. She also experienced a monocular central scotoma with “black spots and jagged, zig-zag edges.” The positive auras resolved spontaneously, whereas the central scotoma persisted. Spectral domain OCT showed an area of perifoveal hyperreflectivity from the inner plexiform to the outer plexiform layers consistent with PAMM. The mid-retina en face OCT and OCT angiography demonstrated an ovoid focal patch of hyperreflectivity with flow interruption, characteristic of globular PAMM. We diagnosed her with migraines with aura and presumed retinal vasospasm, complicated by retinal ischemia in the form of globular PAMM. Acute retinal ischemia, which may require urgent neurovascular workup and giant cell arteritis evaluation, must be considered in patients with migraines alongside persistent visual changes. Diagnosing PAMM requires a high level of suspicion since it can present without significant changes in visual acuity, visual fields, and fundus photographs. With the inclusion of en face OCT in the clinicians’ diagnostic armamentarium, the slightest signs of retinal ischemic changes, such as PAMM, become evident.

Abstract Purpose The association between paracentral acute middle maculopathy (PAMM) and visual acuity in patients with central retinal artery occlusion (CRAO) is still unclear. The present study investigated the visual acuity of CRAO patients with and without PAMM. Methods CRAO patients with PAMM or without PAMM were included. Optical coherence tomography angiography (OCT-A) was used to record the macular retinal thickness and density of shallow and deep vessels. The Best-corrected visual acuity (BCVA) was converted to a logarithm of the minimum angle of resolution (LogMAR) for statistical analysis. Results There were 34 CRAO patients with PAMM (43.13%), other 30 CRAO patients without PAMM (46.87%). Compared with the no-PAMM group, PAMM group had better LogMAR BCVA (1.48 (0.49, 1.85) Vs. 1.85 (1.70, 1.96), P

Purpose: To present a case of paracentral acute middle maculopathy (PAMM) associated with transient loss of vision from eosinophilic granulomatosis with polyangiitis (EGPA)-associated ischemic vasculitis. Methods: Case report and review of the literature. Results: A 52-year-old man presented with recurrent episodes of amaurosis of the right eye. A diagnosis of EGPA was established with the combination of late-onset asthma, peripheral eosinophilia, patchy parenchymal consolidation, and diffuse pansinusitis. Fundus examination displayed cotton wool spots in the right eye. Optical coherence tomography (OCT) of the macula showed features of paracentral focal inner nuclear layer/outer plexiform layer hyperreflectivity consistent with PAMM. He was treated with corticosteroids and cyclophosphamide. At 2 months following the treatment, his EGPA was in remission; the patient reported no recurrence of visual symptoms. Dilated fundoscopy revealed resolution of the cotton wool spots. A repeat OCT of the macula showed resolution of PAMM. Conclusion: This case supports sublethal hypoxia of the middle retinal layers as the underlying mechanism for PAMM. Ophthalmologists should be cognizant to look for PAMM on OCT in patients presenting with transient visual loss and be aware of EGPA-associated ischemic vasculitis as a rare cause of amaurosis. [ABSTRACT FROM AUTHOR]

Ocular toxoplasmosis has a known, rare association with acute retinal artery occlusion (RAO). We describe a 21-year-old male who presented with acute focal toxoplasmosis chorioretinitis in the right eye treated with intravitreal clindamycin, intravitreal dexamethasone, and adjunct oral therapy for vision-threatening retinitis with subsequent quiescence. Nine months from his initial presentation, the patient presented with a branch RAO adjacent to an inactive retinal scar in the right eye. Widefield en face structural swept-source optical coherence tomography (SS-OCT) centered on the middle retina showed paracentral acute middle maculopathy (PAMM) in an arteriolar distribution. The patient was started on 81 mg of aspirin daily. Six months later, the en face structural SS-OCT and corresponding B-scans showed resolution of PAMM. Along with a review of the literature on toxoplasmosis-related RAOs, we present the first case of delayed-onset RAO in ocular toxoplasmosis.

Abstract Purpose To investigate the association between retinal vein occlusion (RVO) and evidence of previous, unnoticed inner nuclear layer (INL) infarction in the fellow eye. Methods This prospective case-control study compared the prevalence of INL lesions in the fellow eye of consecutive people with hypertension (PwHTN) with unilateral RVO versus a randomly chosen eye of an age-matched control group of PwHTN without RVO. En face slabs above the outer plexiform layer (OPL) were generated from 6 × 6 fovea-centered optical coherence tomography scans. Cross-sectional scans and en face slabs were surveyed for evidence of active/resolved ischemic INL lesions. Results 69 PwHTN were included and assigned to two groups, i.e., the RVO group (n = 37; 22 BRVO and 15 CRVO) and the control group (n = 32). There was no inter-group difference regarding age, gender distribution, and background diseases. Resolved INL lesions were more frequent in the RVO group (n = 26) than in the control group (n = 4) (70.3% vs. 12.5%, p

Background: To report a rare case of pulmonary and ocular complications with visual loss due to bilateral Purtscher-like retinopathy and paracentral acute middle maculopathy (PAMM) following a hyaluronic acid (HA) filler injection to the breast. Systemic and visual recovery was attained following corticosteroid therapy. Case presentation: A 27-year-old lady presented with painless blurring of vision in both eyes for 2 weeks following hyaluronic acid breast filler injections by a non-medical practitioner. She was initially admitted to the medical ward for diffuse alveolar haemorrhage and altered sensorium. The presenting visual acuity was counting fingers in both eyes. Bilateral dilated fundus examination showed hyperaemic discs, concentric rim of retinal whitening around macula with patches of polygonal-shaped retinal whitening, generalised cotton-wool spots, tortuous veins, and flame-shaped haemorrhages. Spectral-domain optical coherence tomography (SD-OCT) macula revealed hyper-reflective bands at the inner nuclear layer (INL). Fluorescein angiography demonstrated hot discs, delayed arm-to-retina time, arterial filling, and arterio-venous transit time with staining of the vessels at the posterior pole. She was managed with a tapering dose of systemic corticosteroids. The visual acuity improved to 6/12 over 8 weeks with significant anatomical and functional improvement. Dilated fundus examination showed resolution of initial funduscopy findings. The hyper-reflective bands on the OCT had resolved with subsequent thinning of the INL and disorganisation of retinal inner layers. Conclusion: Filler injections are in increasing demand and are frequently being performed by non-medical practitioners. Visual loss from non-facial HA fillers is rare. Inadvertent entry of HA into a blood vessel may potentially cause systemic and sight-threatening ocular complications. Good anatomical knowledge and proper injection technique are vital in preventing this unfortunate sequela. There are limited reports on successful visual recovery following various treatment approaches and we hope this case provides valuable insights. [ABSTRACT FROM AUTHOR]

Purpose: To report the case and multimodal imaging findings of a healthy young woman who developed paracentral acute middle maculopathy 9 weeks after COVID-19. Methods: Case report. Ultra-widefield fundus photography, macular spectral domain optical coherence tomography, fluorescein angiography, and optical coherence tomography angiography were performed. Results: A 36-year-old woman went to the emergency department with sudden, painless, left eye vision loss. The only relevant medical history was COVID-19 9 weeks before. The best-corrected visual acuity was 20/200, a left eye relative afferent pupillary defect was present, and superficial hemorrhages throughout the macular area and peripheral retina were found. Nearly 4 hours after admission, the left eye best-corrected visual acuity recovered to 20/20 without relative afferent pupillary defect. Five days after presentation in the emergency department, the patient returned with recurrent left eye vision loss, with spontaneous recovery within 12 hours. Macular spectral domain optical coherence tomography revealed hyperreflectivity of the inner plexiform and inner nuclear layers, and the diagnosis of paracentral acute middle maculopathy was established. The patient started oral acetylsalicylic acid and oral prednisolone. The patient did not report any new episodes of vision loss, and there was a progressive resolution of abnormal fundus findings. Conclusion: SARS-CoV-2 infection increases the risk of vascular thrombotic events with possible involvement of the retinal circulation, and paracentral acute middle maculopathy may present as a possible complication. Ophthalmologists should be able to recognize it promptly through multimodal imaging findings. [ABSTRACT FROM AUTHOR]

Background/Purpose: To report an atypical case of ocular bartonellosis evaluated longitudinally with multimodal imaging and review the literature on the less common posterior segment manifestations of bartonella infection. Methods: Case report and review of the literature. Results: A 26-year-old man presented with focal choroiditis in the right eye and focal retinitis, a branch retinal artery occlusion, and paracentral acute middle maculopathy in the left eye after a systemic prodrome including headache and lymphadenopathy. Serologic testing revealed positive bartonella immunoglobulin G with a titer of 1:512. The patient was diagnosed with ocular bartonellosis infection and started on systemic doxycycline. The posterior segment findings resolved over the follow-up period, and their resolution was documented with optical coherence tomography. Conclusion: Neuroretinitis is the classic ocular finding in cat scratch disease, but less common presenting findings may include focal choroiditis, retinitis, and branch retinal artery occlusion. To the best of our knowledge, this is the first report of paracentral acute middle maculopathy secondary to a retinitis-associated branch retinal artery occlusion as a presenting sign in ocular bartonellosis. [ABSTRACT FROM AUTHOR]

Purpose: The association between paracentral acute middle maculopathy (PAMM) and visual acuity in patients with central retinal artery occlusion (CRAO) is still unclear. The present study investigated the visual acuity of CRAO patients with and without PAMM. Methods: CRAO patients with PAMM or without PAMM were included. Optical coherence tomography angiography (OCT-A) was used to record the macular retinal thickness and density of shallow and deep vessels. The Best-corrected visual acuity (BCVA) was converted to a logarithm of the minimum angle of resolution (LogMAR) for statistical analysis. Results: There were 34 CRAO patients with PAMM (43.13%), other 30 CRAO patients without PAMM (46.87%). Compared with the no-PAMM group, PAMM group had better LogMAR BCVA (1.48 (0.49, 1.85) Vs. 1.85 (1.70, 1.96), P < 0.01). There was also a significant difference in retinal thickness of the central macular sulcus (328.00 (304.50–332.25) Vs. 352.50 (311.75–420.50), P = 0.01). A significant correlation between LogMAR BCVA and macular retinal thickness was found (r = 0.42; P < 0.01). Conclusion: CRAO patients with PAMM had significantly better visual acuity and less macular edema. OCT-A can be used to distinguish different levels of damage due to CRAO. [ABSTRACT FROM AUTHOR]

We present a 65-year-old woman who developed sudden and severe vision loss in her left eye one day after the administration of the second dose of coronavirus disease-19 vaccine. The best corrected visual acuity in this eye was 1/10. Diffuse paracentral acute middle maculopathy was detected on spectral domain optical coherence tomography (OCT). OCT angiography images revealed concurrent vascular flow defects consistent with acute macular neuroretinopathy in the deep retinal capillary plexus and choriocapillaris layers. At the end of the six-month follow-up, there was no improvement in visual acuity, and atrophy and thinning developed in all layers of the retina.

Abstract Background To report a case of paracentral acute middle maculopathy (PAMM) that progressed to central retinal artery occlusion (CRAO) on spectral domain-optical coherence tomography (SD-OCT). Case Presentation A 63-year-old male presented with a paracentral scotoma that began several days ago. His past medical history consisted of third-degree atrioventricular heart block requiring a pacemaker. Giant cell arteritis was unlikely given the patient’s labs, demographics and review of systems. SD-OCT revealed a characteristic hyperreflective band in the inner nuclear layer consistent with PAMM in his left eye. Fluorescein angiography was obtained and was unremarkable. Five days later, the patient developed no light perception in the left eye. SD-OCT showed a diffuse inner retinal hyperreflectivity consistent with CRAO. Conclusion PAMM can be a harbinger event for complete CRAO. Complete stroke evaluation should be performed to prevent a cerebrovascular event or progression to complete blindness in the involved eye.

AIM:To investigate the changes in the macular microvasculature in eyes with central retinal artery occlusion(CRAO)and paracentral acute middle maculopathy(PAMM).METHODS: Retrospective study. A total of 27 cases(27 eyes)who diagnosed with CRAO-PAMM and 29 patients(29 eyes)diagnosed as CRAO but with no PAMM were hospitalized in our hospital from January 2020 to December 2021. There were 33 normal people(33 eyes)who underwent physical examination in our hospital selected as control group. Optical coherence tomography angiography(OCTA)was used to measure retinal blood flow and thickness parameters in the 3 mm×3 mm area of the macula. The correlation among macular retinal blood flow density, retinal thickness, foveal avascular zone(FAZ)area, FAZ perimeter, acircularity index(AI), flow density in a 300-μm-wide region around the FAZ(FD-300)and lesion area, best corrected visual acuity(BCVA)in the CRAO-PAMM group was analyzed.RESULTS: Among the three groups, there were significant differences in the overall and parafoveal blood flow density of superficial capillary layer(SCP)and deep capillary layer(DCP), foveal thickness, FAZ area, FAZ perimeter, AI and FD-300(all P

An ophthalmology consultation was requested for a 29-year-old woman complaining of visual field defects. The patient had presented to the emergency department with cough and high fever one day earlier. Chest computed tomography demonstrated pneumonia and two severe acute respiratory syndrome coronavirus 2 polymerase chain reaction tests were positive. The patient had undergone renal transplantation 11 years ago due to glomerulonephritis. Best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/30 in the left eye. Fluorescein angiography showed macular hypoperfusion, and optical coherence tomography (OCT) showed hyperreflectivity in the inner nuclear, outer plexiform, and outer nuclear layers, as well as discontinuity of the ellipsoid zone. Perimetry confirmed bilateral central scotoma. Levels of D-dimer and fibrinogen were 0.86 g/mL and 435.6 g/mL, respectively. The patient was diagnosed as having concurrent acute macular neuroretinopathy and paracentral acute middle maculopathy and was given low-molecular-weight heparin treatment for one month. Her BCVA improved to 20/20 in both eyes, and regression was observed in the retinal findings, hyperreflectivity and ellipsoid zone disruption on OCT, and scotoma in perimetry. Inflammation, thrombosis, and glial involvement may play a role in the pathogenesis of retinal microvascular impairment in COVID-19.

Ocular toxoplasmosis has a known, rare association with acute retinal artery occlusion (RAO). We describe a 21-year-old male who presented with acute focal toxoplasmosis chorioretinitis in the right eye treated with intravitreal clindamycin, intravitreal dexamethasone, and adjunct oral therapy for vision-threatening retinitis with subsequent quiescence. Nine months from his initial presentation, the patient presented with a branch RAO adjacent to an inactive retinal scar in the right eye. Widefield en face structural swept-source optical coherence tomography (SS-OCT) centered on the middle retina showed paracentral acute middle maculopathy (PAMM) in an arteriolar distribution. The patient was started on 81 mg of aspirin daily. Six months later, the en face structural SS-OCT and corresponding B-scans showed resolution of PAMM. Along with a review of the literature on toxoplasmosis-related RAOs, we present the first case of delayed-onset RAO in ocular toxoplasmosis. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the association between retinal vein occlusion (RVO) and evidence of previous, unnoticed inner nuclear layer (INL) infarction in the fellow eye. Methods: This prospective case-control study compared the prevalence of INL lesions in the fellow eye of consecutive people with hypertension (PwHTN) with unilateral RVO versus a randomly chosen eye of an age-matched control group of PwHTN without RVO. En face slabs above the outer plexiform layer (OPL) were generated from 6 × 6 fovea-centered optical coherence tomography scans. Cross-sectional scans and en face slabs were surveyed for evidence of active/resolved ischemic INL lesions. Results: 69 PwHTN were included and assigned to two groups, i.e., the RVO group (n = 37; 22 BRVO and 15 CRVO) and the control group (n = 32). There was no inter-group difference regarding age, gender distribution, and background diseases. Resolved INL lesions were more frequent in the RVO group (n = 26) than in the control group (n = 4) (70.3% vs. 12.5%, p < 0.001). BRVO and CRVO cases had similar prevalence of INL lesions in their fellow eyes. Unlike diabetes, ischemic heart disease, and gender, INL lesions were associated with RVO (in the fellow eye) with an odds ratio of 15.7 (95%CI: 4.17–76.73, p < 0.001). Conclusion: We identified a substantially higher prevalence of INL lesions in PwHTN with RVO compared to PwHTN without RVO. The atrophic appearance of lesions suggests they may serve as early markers of increased RVO risk in individuals with systemic or cardiovascular predisposing factors. [ABSTRACT FROM AUTHOR]

Purpose: To analyze the imaging characteristics and the clinical course of patients showing concomitant paracentral acute middle maculopathy (PAMM) and acute macular neuroretinopathy (AMN) post-blunt trauma. Methods: PAMM and AMN lesions post-blunt trauma diagnosed on enhanced depth imaging optical coherence tomography (EDI-OCT) were recruited for the study. Results: Thirteen eyes of 13 individuals with a history of blunt trauma were included in the study, of whom 11 (85%) were males. Mean age of the patients was 33.62 (range 16-67) years. Mean visual acuity at presentation and the last visit was 1.67 log of minimum angle of resolution (logMAR) and 0.82 logMAR, respectively. Mean interval between trauma and imaging was 5.08 (range 1-15) days. All patients had unilateral involvement, with the right eye being involved in 10 patients (77%). All patients had concomitant PAMM and AMN lesions. Conclusion: Presence of coincident PAMM and AMN suggests a common pathophysiologic etiology, but the description of concomitant PAMM and AMN in the setting of blunt trauma to eye is hitherto unreported. Identifying AMN in a setting of PAMM requires meticulous examination of the OCT and OCTA images. It can be a cause of suboptimal visual recovery in such eyes. [ABSTRACT FROM AUTHOR]

Paracentral acute middle maculopathy (PAMM) is an optical coherence tomography (OCT) finding secondary to retinal ischemia. In this presentation, we describe the diagnosis of hereditary thrombophilia caused by multiple gene mutation, based on the PAMM finding on OCT, in a patient with no known disease who presented with unilateral scotoma. Hematological evaluation has an important role in young PAMM cases without systemic and ocular disease. Patients with thrombophilic mutations may be first recognized with ocular symptoms by ophthalmologists. It may be life-saving to reveal the risks of patients such as cardiovascular disease and thromboembolism at an early age. [ABSTRACT FROM AUTHOR]

COVID-19 is a disease accompanied by severe coagulopathy secondary to inflammation and multi-organ involvement. The frequency of thromboembolic complications in COVID-19 and its relationship with the severity of the disease has still not been determined. In this article, we present a case of cilioretinal artery occlusion associated with impending central retinal vein occlusion after severe COVID-19 infection. [ABSTRACT FROM AUTHOR]

Posterior eye segment involvement in COVID-19 has varied manifestations: vascular, inflammatory, and neuronal. All of them are triggered by SARS-CoV-2 virus but they cannot be viewed as exclusively specific to COVID-19. According to the literature, the mean age of the patients varies from 17 to 75 with the median of 50 years. The median duration between the appearance of ophthalmic symptoms and the detection of COVID-19 was 12 days. The disease affects both men and women equally. Direct exposure to the virus, immune-mediated tissue damage, activation of the coagulation system, the prothrombotic state caused by a viral infection, concomitant diseases and medications used in the treatment contribute to the development of eye pathologies. Ophthalmologists should be aware of the possible relations of posterior eye segment pathologies, orbit and neuro-ophthalmic disorders with SARS-CoV-2, as well as the possible exacerbation of chronic forms of inflammatory eye diseases and autoimmune disorders due to anti-COVID-19 vaccination.

Purpose: To analyze the imaging characteristics and the clinical course of patients showing concomitant paracentral acute middle maculopathy (PAMM) and acute macular neuroretinopathy (AMN) post-blunt trauma. Methods: PAMM and AMN lesions post-blunt trauma diagnosed on enhanced depth imaging optical coherence tomography (EDI-OCT) were recruited for the study. Results: Thirteen eyes of 13 individuals with a history of blunt trauma were included in the study, of whom 11 (85%) were males. Mean age of the patients was 33.62 (range 16–67) years. Mean visual acuity at presentation and the last visit was 1.67 log of minimum angle of resolution (logMAR) and 0.82 logMAR, respectively. Mean interval between trauma and imaging was 5.08 (range 1–15) days. All patients had unilateral involvement, with the right eye being involved in 10 patients (77%). All patients had concomitant PAMM and AMN lesions. Conclusion: Presence of coincident PAMM and AMN suggests a common pathophysiologic etiology, but the description of concomitant PAMM and AMN in the setting of blunt trauma to eye is hitherto unreported. Identifying AMN in a setting of PAMM requires meticulous examination of the OCT and OCTA images. It can be a cause of suboptimal visual recovery in such eyes.

This report describes a case of a newly diagnosed 49-year-old HIV patient, who presented with decreased visual acuity and retinal lesions characterized by ischemia at the level of the deep retinal capillary plexus, documented with optical coherence tomography (OCT), OCT angiography, fluorescein angiography, and visual fields testing. These lesions closely resembled the morphologic and clinical characteristics of late paracentral acute middle maculopathy. The presence of these lesions suggests that HIV microangiopathy can potentially affect both superficial and deep retinal capillary plexuses.

Background: To report a case of paracentral acute middle maculopathy (PAMM) that progressed to central retinal artery occlusion (CRAO) on spectral domain-optical coherence tomography (SD-OCT). Case Presentation: A 63-year-old male presented with a paracentral scotoma that began several days ago. His past medical history consisted of third-degree atrioventricular heart block requiring a pacemaker. Giant cell arteritis was unlikely given the patient's labs, demographics and review of systems. SD-OCT revealed a characteristic hyperreflective band in the inner nuclear layer consistent with PAMM in his left eye. Fluorescein angiography was obtained and was unremarkable. Five days later, the patient developed no light perception in the left eye. SD-OCT showed a diffuse inner retinal hyperreflectivity consistent with CRAO. Conclusion: PAMM can be a harbinger event for complete CRAO. Complete stroke evaluation should be performed to prevent a cerebrovascular event or progression to complete blindness in the involved eye. [ABSTRACT FROM AUTHOR]

Retinal complications in patients with inflammatory optic neuritis (ON) are generally related to post-infectious neuroretinitis and are considered uncommon in autoimmune/demyelinating ON, whether isolated or caused by multiple sclerosis (MS) or neuromyelitis optica spectrum disorder (NMOSD). More recently, however, cases with retinal complications have been reported in subjects positive for myelin oligodendrocyte glycoprotein (MOG) antibodies. We report a 53-year-old woman presenting with severe bilateral ON associated with a focal area of paracentral acute middle maculopathy (PAMM) in one eye. Visual loss recovered remarkably after high-dose intravenous corticosteroid treatment and plasmapheresis, but the PAMM lesion remained visible on both optical coherence tomography and angiography as an ischaemic lesion affecting the middle layers of the retina. The report emphasises the possible occurrence of retinal vascular complications in MOG–related optic neuritis, an important addition to the diagnosis of, and possible differentiation from, MS–related or NMOSD–related ON. [ABSTRACT FROM AUTHOR]

We present a 65-year-old woman who developed sudden and severe vision loss in her left eye one day after the administration of the second dose of COVID vaccine. The best corrected visual acuity in this eye was 1/10. Diffuse paracentral acute middle maculopathy was detected on spectral domain optical coherence tomography (OCT). OCT angiography images revealed concurrent vascular flow defects consistent with acute macular neuroretinopathy in the deep retinal capillary plexus and choriocapillaris layers. At the end of the six-month follow-up, there was no improvement in visual acuity, and atrophy and thinning developed in all layers of the retina. [ABSTRACT FROM AUTHOR]

Background: Growing evidence suggests an association between the infection from the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) and eye disorders. The aim of this review was to analyze the clinical presentation and diagnostic features of acute macular neuroretinopathy (AMN) and paracentral acute middle maculopathy (PAMM) associated with COVID-19 infection. The features are then compared with previous reports regarding these retinal disorders, to recognize possible specific characteristics and to assess the role of multimodal ophthalmic imaging. Summary: A literature search was performed by consulting PubMed, Scopus, and Embase. The following terms were searched: "(COVID-19 OR SARS-CoV-2 OR coronavirus) AND ([acute macular neuroretinopathy] OR [paracentral acute middle maculopathy])." Inclusion criteria were as follows: (1) publication date from January 31, 2020 to January 31, 2022; (2) English language; (3) original research or case report; (4) free full-text availability.Optical coherence tomography (OCT) findings in AMN patients were hyper-reflectivity (HR) of the outer plexiform layer, of the outer nuclear layer, and ellipsoid or interdigitation zones (EZ and IZ, respectively) disruption. In most cases, the presence of HR and EZ/IZ abnormalities resulted combined. When performed, OCT angiography (OCTA) identified attenuation of signal of the deep capillary plexus (DCP). The most common OCT finding in PAMM was an alteration of the inner nuclear layer, associated with other areas of HR, while no signs of EZ/IZ disruption were detected. When performed, OCTA showed the attenuation of signal of both the DCP and the superficial capillary plexus. Key Messages: In this review, we reported a case series of AMN and PAMM in patients with a previous or concomitant infection from SARS-CoV-2. The microvascular changes in these cases are highlighted by the OCTA scans. Even if we are far from the determination of a direct link between COVID-19 and these retinal disorders, we could hypothesize that the vascular alterations associated with SARS-CoV-2 infection could be a possible risk factor for both AMN and PAMM. [ABSTRACT FROM AUTHOR]

Retinal ischemia gives rise to a complex spectrum in which the cumulative profile of ischemia of the middle and inner retina can be highly variable. We reviewed the current knowledge on paracentral acute middle maculopathy (PAMM) pathophysiology and accompanying risk factors, the middle and inner retinal vasculature and blood flow, and the vulnerability of the middle retina in vaso-occlusive disorders. The inner nuclear layer (INL) is easily affected by slight degrees of retinal hypoperfusion and ischemia. INL infarction starts at perivenular sites, manifesting as skip PAMM lesions and a fern-like appearance in cross-sectional and en face views, respectively. With horizontal progression, INL infarction may develop into diffuse globular PAMM. If vertical progression occurs, the entire middle and inner portions of the retina can be affected. Transmural infarction of the middle and inner retina would be at the end of this spectrum. This gradient of ischemic progression resembles an ischemic cascade. We review the evidence supporting the term "retinal ischemic cascade," which encompasses a broad continuum of manifestations with roots in middle retinal infarction. With this terminology, variations in spatial and temporal progression and resolution of ischemia can also be delivered; it further enables addressing the possible associations between the middle and inner retinal ischemic patterns. [ABSTRACT FROM AUTHOR]

We report a case of paracentral acute middle maculopathy (PAMM) in association with coronavirus disease 2019 (COVID-19) vaccination. A 67-year-old male patient presented with a complaint of decreased vision in his right eye (OD) three days after receiving the Pfizer-BioNTech COVID-19 vaccine. Dilated fundus examination showed a white retinal lesion in the superior parafoveal region and optic disc edema in OD, and fundus autofluorescence (FAF) revealed hypoautofluorescence at the corresponding area. Spectral domain optical coherence tomography (SD-OCT) demonstrated the presence of a hyperreflective band along the inner nuclear layers (INL) and extending from the outer plexiform layer (OPL) to inner plexiform layer (IPL) in OD, SD-OCT was normal in his left eye (OS). Fundus fluorescein angiography (FA) showed hypofluorescence in the superior parafoveal area with late disc leakage in OD and FA was normal in OS. Current case highlights the recent severe acute respiratory syndrome coronavirus - 2 (SARS-CoV-2) vaccine as a possible cause for PAMM as a form of a rare adverse effect. However, further studies should be done to describe any association between COVID-19 vaccination and post-vaccination related retinopathies. [ABSTRACT FROM AUTHOR]

To describe a case of interferon-beta retinopathy associated with paracentral acute middle maculopathy A 15-year-old girl with Epstein–Barr virus-positive advanced nasopharyngeal carcinoma WAS REFERRED with reduced visual acuity. Multimodal imaging findings, including optical coherence tomography angiography, at presentation and evolution following cessation of interferon therapy are presented. The presentation of paracentral acute middle maculopathy in this patient supports the presumed ischaemic pathogenesis in interferon retinopathy. The imaging findings provide evidence of deep capillary plexus involvement in interferon retinopathy with evolution to permanent structural damage within the inner nuclear layer. [ABSTRACT FROM AUTHOR]

Abstract Purpose To describe chorioretinal signs in a case series of Giant Cell Arteritis (GCA). Methods This is a multicenter retrospective observational case series with GCA that presented with a headache and an abrupt, unilateral loss in vision. Workup included temporal artery biopsies, intravenous fluorescein angiography, optical coherence tomography (OCT), optical coherence tomography angiography (OCTA), blood levels of erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). Results There are a total of 8 GCA instances presented. Average age was 74.5. (Range 68–83 years). The patients reported that one eye's visual loss had suddenly started, along with a fresh headache and other systemic symptoms. Eight patients exhibited choroidal ischemia, five paracentral acute middle maculopathy (PAMM) lesions, five cotton wool spots, four anterior ischemic optic neuropathy, and one central retinal arterial occlusion at the time of presentation. The average ESR at presentation was 68 mm/hr (range 4–110), and 4/6 individuals had a significant increase. The mean CRP level was 6.2 mg/dL (range 2.0–15.4), and the level was always over the normal range. All patients' temporal artery biopsies were positive. Conclusion Alongside PAMM lesions, cotton wool spots, anterior ischemic optic neuropathy, and central retinal artery occlusion, choroidal ischemia is a key angiographic indicator in the diagnosis of GCA. It may be crucial to recognize these typical ischemic chorioretinal signs while diagnosing GCA.

An ophthalmology consultation was requested for a 29-year-old woman complaining of visual field defects. The patient had presented to the emergency department with cough and high fever one day earlier. Chest computed tomography demonstrated pneumonia and two severe acute respiratory syndrome coronavirus 2 polymerase chain reaction tests were positive. The patient had undergone renal transplantation 11 years ago due to glomerulonephritis. Best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/30 in the left eye. Fluorescein angiography showed macular hypoperfusion, and optical coherence tomography (OCT) showed hyperreflectivity in the inner nuclear, outer plexiform, and outer nuclear layers, as well as discontinuity of the ellipsoid zone. Perimetry confirmed bilateral central scotoma. Levels of D-dimer and fibrinogen were 0.86 g/mL and 435.6 g/mL, respectively. The patient was diagnosed as having concurrent acute macular neuroretinopathy and paracentral acute middle maculopathy and was given low-molecular-weight heparin treatment for one month. Her BCVA improved to 20/20 in both eyes, and regression was observed in the retinal findings, hyperreflectivity and ellipsoid zone disruption on OCT, and scotoma in perimetry. Inflammation, thrombosis, and glial involvement may play a role in the pathogenesis of retinal microvascular impairment in COVID-19. [ABSTRACT FROM AUTHOR]

Purpose: To review the demographic and clinical profile of patients developing acute macular neuroretinopathy (AMN) or paracentral acute middle maculopathy (PAMM) after receiving coronavirus disease-2019 (COVID-19) vaccination or infection. Methods: In this review article, the published literature was searched to determine cases developing either AMN or PAMM after COVID-19 vaccinations or infections. Data, including demographic profile, presenting features, symptoms, diagnosis, and clinical outcomes, were extracted from the selected publications. These parameters were compared between the two groups, i.e., patients developing AMN/PAMM either after vaccination or infection. Results: After the literature review, 57 patients developing either AMN (n = 40), PAMM (n = 14), or both (n = 3) after COVID-19 infection (n = 29) or vaccination (n = 28) were included (mean age: 34.9 ± 14.4 years; n = 38; 66.7% females). In 24.6% patients, the diagnosis of COVID-19 infection was preceded by the development of ocular disease. There were no significant differences in the age or gender between the patients developing AMN or PAMM after vaccination or infection (p > 0.13). Among the vaccination group, the highest number of patients developing AMN/PAMM were after the Oxford-AstraZeneca (n = 12; 42.9%). Patients with vaccination had a significantly early onset of AMN/PAMM compared to those with infection (11.5 ± 17.6 days versus 37.8 ± 43.6 days; p = 0.001). Conclusions: Both AMN and PAMM are reported to be associated with COVID-19 infections and in persons receiving vaccination against COVID-19. While COVID-19 infections and vaccinations may have a contributory role, other risk factors such as oral contraceptive pills may also play a role in the development of the disease. [ABSTRACT FROM AUTHOR]

This report describes a case of a newly diagnosed 49-year-old HIV patient, who presented with decreased visual acuity and retinal lesions characterized by ischemia at the level of the deep retinal capillary plexus, documented with optical coherence tomography (OCT), OCT angiography, fluorescein angiography, and visual fields testing. These lesions closely resembled the morphologic and clinical characteristics of late paracentral acute middle maculopathy. The presence of these lesions suggests that HIV microangiopathy can potentially affect both superficial and deep retinal capillary plexuses. [ABSTRACT FROM AUTHOR]

A 75-year-old Caucasian woman presented with sudden-onset multifocal scotomas in her right eye's central vision for 1 day. There were subtle white intraretinal foveal lesions that correlated with patchy inner retinal hyperreflectivity on optical coherence tomography, suggestive of paracentral acute middle maculopathy. Initial cerebrovascular work-up was negative. Review of systems was positive for lethargy and jaw claudication. The sedimentation rate and c-reactive protein were elevated, but platelet count was normal. The patient was started on 60 mg oral prednisone daily and underwent bilateral temporal artery that confirmed the diagnosis of giant cell arteritis. [ABSTRACT FROM AUTHOR]

We report a case of a healthy multiparous woman in her second trimester of pregnancy presenting with a paracentral scotoma found to have a paracentral acute middle maculopathy lesion, resulting in a new diagnosis of a possible hypercoagulable condition with elevated Factor VIII. Purpose: To report a case of paracentral acute middle maculopathy in an otherwise healthy young, multiparous woman in her second trimester of pregnancy. Methods: A case report. Results: A 38-year-old woman in her twentieth week of pregnancy presented with a four-day history of an acute paracentral scotoma in her left eye. Fundoscopic examination of the left eye was significant for a white–gray lesion inferonasal to the fovea which corresponded with spectral-domain optical coherence tomography hyperreflectivity at the outer plexiform layer–inner nuclear layer junction and optical coherence tomography angiography nonperfusion. A diagnosis of paracentral acute middle maculopathy was made. The patient was sent for a hypercoagulability workup that revealed elevated Factor VIII activity, which has been associated with increased risk of complications during pregnancy. Conclusion: Paracentral acute middle maculopathy in pregnancy may be secondary to an underlying hypercoagulable condition. We recommend systemic evaluation and referral to a high-risk pregnancy specialist if paracentral acute middle maculopathy is diagnosed during pregnancy. In addition, optical coherence tomography angiography in paracentral acute middle maculopathy may demonstrate reperfusion of the affected vessels. [ABSTRACT FROM AUTHOR]

Abstract Background Retinal arterial occlusive events in young patients are rare. However, because of physiological multifactorial adaptations during pregnancy, retinal vascular occlusive disease may occur spontaneously. In addition, a patent foramen ovale is a risk factor for an ischemic thromboembolic event. Since fluorescein angiography, a central tool in the evaluation of these occlusions, should be avoided during pregnancy, optical coherence tomography angiography, a novel technique, offers a good opportunity for visualizing vascular perfusion of retinal tissue. Case presentation Here we present a case series of three patients (Caucasian, nonsmoker) who visited our clinic owing to acute visual impairment and central scotoma. Using regular optical coherence tomography and optical coherence tomography angiography, retinal vascular occlusions were detected, thus initiating the evaluation of systemic risk factors. We report two patients (30 and 32 years old) who developed cilioretinal artery occlusion but whose etiology differed: one was of thromboembolic origin associated with patent foramen ovale, while the other was caused by hemodynamic blockade secondary to central retinal vein occlusion. In both cases, optical coherence tomography angiography revealed reperfusion of the cilioretinal artery occlusion. However, transient ischemia led to retinal atrophy after a few weeks. In the third patient (32 years old), 8 weeks after onset of scotoma, optical coherence tomography angiography revealed atrophy of the middle layers and impaired perfusion in the deep capillary plexus, and thus a paracentral acute middle maculopathy was diagnosed. All patients regained normal visual acuity and had otherwise uncomplicated pregnancies, and laboratory blood tests did not reveal any defects or alterations. Conclusions As shown here, optical coherence tomography angiography enables risk-free imaging of retinal vessel perfusion during pregnancy. Together with regular optical coherence tomography, it allows one to predict functional outcome according to the existing retinal occlusion-related atrophy.

Koki Ishibashi,1,2 Hiroyuki Yatsuka,3 Masatoshi Haruta,1 Kenichi Kimoto,3 Shigeo Yoshida,1 Toshiaki Kubota3 1Department of Ophthalmology, Kurume University School of Medicine, Kurume, Japan; 2Department of Ophthalmology, Yame General Hospital, Yame, Japan; 3Department of Ophthalmology, Faculty of Medicine, Oita University, Yufu, JapanCorrespondence: Masatoshi Haruta, Department of Ophthalmology, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka, 830-0011, Japan, Tel +81 942 31 7574, Fax +81 942 37 0324, Email haruta_masatoshi@med.kurume-u.ac.jpPurpose: Potential retinal adverse events after COVID-19 vaccinations reported previously include paracentral acute middle maculopathy (PAMM), acute macular neuroretinopathy (AMN), and central serous chorioretinopathy. We report four cases of branch retinal artery occlusion (BRAO), one case of PAMM, and one case of AMN that occurred after administration of the Pfizer-BioNTech COVID-19 vaccine.Patients and Methods: We retrospectively reviewed the medical records of six patients who presented to Yame General Hospital or Oita University Hospital from July through October 2021.Results: Four patients (2 males) presented with visual field defects associated with BRAO, one male patient with PAMM, and one female patient with AMN after receiving the Pfizer-BioNTech COVID-19 vaccine. The mean age was 59.3 years; the mean best-corrected visual acuity was 20/21. The mean time from the last vaccination to the onset of visual field defect was 22.8 days. Five patients had received two doses of the vaccine and one patient one dose. Patients’ medical history included diabetes mellitus in case 2, hypertension in cases 2, 3 and 6, and Alport syndrome and end-stage renal disease in case 6 for which the patient was undergoing regular hemodialysis.Conclusion: Although rare, retinal adverse events may occur after COVID-19 vaccinations. Further studies with a larger sample size should determine whether these retinal abnormalities are causally associated with COVID-19 vaccinations or just coincidental. Potential risks of BRAO/PAMM/AMN after COVID-19 vaccinations must be carefully weighed against the substantial benefit of COVID-19 vaccinations.Keywords: acute macular neuroretinopathy, branch retinal artery occlusion, paracentral acute middle maculopathy, Pfizer-BioNTech COVID-19 vaccine, retinal adverse events

Paracentral acute middle maculopathy can cause acute paracentral scotomas because of capillary ischemia. We report the first case of paracentral acute middle maculopathy secondary to Waldenström macroglobulinemia, a hyperviscosity syndrome. This case highlights the importance of initiating a work-up for unexplained paracentral acute middle maculopathy-associated visual field defects, especially when associated with arteriolar tortuosity. Purpose: To report a case of paracentral acute middle maculopathy as the earliest sign of an undiagnosed, life-threatening hyperviscosity syndrome. Methods: A 78-year-old man with an acute paracentral scotoma and examination findings of bilateral arteriolar tortuosity and unilateral paracentral acute middle maculopathy. Results: Work-up revealed anemia and elevated serum viscosity. Protein electrophoresis demonstrated an immunoglobulin M kappa monoclonal protein spike, and bone marrow biopsy confirmed an immunoglobulin M gammopathy consistent with Waldenström macroglobulinemia. Systemic chemotherapy was initiated. Conclusion: This case demonstrates typical optical coherence tomography findings of paracentral acute middle maculopathy, which led to the diagnosis of a rare lymphoproliferative disorder. This highlights the importance of a prompt work-up for paracentral acute middle maculopathy to detect underlying systemic diseases, including hyperviscosity syndromes. [ABSTRACT FROM AUTHOR]

Background: Primary antiphospholipid syndrome (PAPS) is characterized by the presence of antiphospholipid antibodies (aPL), repetitive fetal loss, and arterial/venous thrombosis and no association with other autoimmune rheumatic disease. Ocular involvement can also occur including retinal vascular thrombosis and neuro-ophthalmological manifestations, such as optic neuropathy and amaurosis fugax. Early detection of ocular changes is crucial to minimize functional loss. Purpose: To perform a multimodal evaluation, including the use of Optical Coherence Angiotomography (OCTA), in patients with PAPS without ocular complaints and compare with healthy individuals. Methods: We performed a complete structural and functional ophthalmological evaluation using OCTA and microperimetry exam in patients with PAPS, followed at a tertiary Rheumatology outpatient clinic. Results: We included 104 eyes of 52 subjects [PAPS without ocular complaints (N = 26) and healthy individuals (N = 26)]. Among PAPS patients, 21 were female (80.8%) and 21 (80.8%) were Caucasians. PAPS manifestations were venous (65.4%), arterial thrombosis (34.6%), and obstetrical (34.6%) and all of them had lupus anticoagulant. Ophthalmologic findings were more frequent in PAPS compared to healthy individuals (19.2% vs. 0%, p = 0.05). The most common retinal change was paracentral acute middle maculopathy (PAMM) (3 patients, 5 eyes), followed by drusen (1 patient, 2 eyes) and pachychoroid pigment epitheliopathy (PPE) (1 patient, 1 eye). Hypertension and hyperlipidemia were present in 100% of the PAPS patients with PAMM, while only six patients (26.1%) with PAPS without PAMM presented these two risk factors together (p = 0.03). Conclusions: We provide novel evidence that approximately 20% of our asymptomatic PAPS patients without ocular symptoms have ophthalmologic findings that require early identification and careful surveillance focusing on minimizing systemic and vascular risk factors. [ABSTRACT FROM AUTHOR]

This report describes a case with paracentral acute middle maculopathy revealing a giant aortic aneurysm. A 41-year-old man presented with unilaterally unexplained sudden vision loss in the left eye. Fundoscopy revealed a slightly pale r -26etinal reflex in the superior retinal hemifield. SDOCT revealed blurred boundaries between retinal segments and prominent edema in the middle retinal layers. OCTA revealed decreased vascular density in the deep capillary plexus. FA showed low arterial circulation mainly in the superior retinal hemifield. Humphrey VFT revealed an inferior altitudinal defect in the left eye, implying a related circulatory disturbance in the corresponding region. A thorough systemic work-up, including a cardiovascular etiological investigation, revealed giant aortic aneurysm in the ascending aorta, necessitating emergency surgery. This report highlights the importance of a systemic etiological investigation in patients with PAMM to rule out any potential cardiovascular issues. [ABSTRACT FROM AUTHOR]