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Purpose: To report a case of tuberculosis-related serpiginous-like choroiditis (TB-SLC) in Denmark in a patient with few risk factors., Methods: Single case report., Results: A 54-year-old Caucasian male with no relevant travel history presented with unilateral light placoid confluent elements in the macula of the right eye with a best-corrected visual acuity of 0.2 Snellen. The left eye was normal. Wide-field Fluorescein and Indocyanine green-angiography were performed, and findings were consistent with acute posterior multifocal placoid pigment epitheliopathy. Since the condition was considered sight-threatening, and the patient had no recognizable risk factors for tuberculosis (TB), he was prescribed 50 mg of oral prednisolone. Blood tests and an X-ray were ordered to exclude infectious causes. The first interferon-ỿ release assay (IGRA) test was inconclusive and a new test was ordered. Over the following weeks new white dots appeared in the retina. After the patient had been treated for seven weeks with prednisolone, the second IGRA came back positive, and he was diagnosed with TB-SLC. Upon repeated questioning two months after baseline, the patient remembered that ten years ago he had been in a workplace with 50 different nationalities, and seven years ago he had been in contact with a friend who was treated for latent TB, thus supporting relevant exposure., Conclusion: TB-SLC may occur even in a patient with few recognizable risk factors and in a setting that is not TB endemic. It is imperative to continuously reassess differential diagnoses and initiate or repeat paraclinical testing in cases with atypical features.

In this study, we report paracentral acute middle maculopathy (PAMM) and cotton wool spots (CWS) in a patient with ocular migraine. A 74-year-old man presented with persistent paracentral scotoma in the right eye that began a week prior. His visual acuity was 20/25 in the right eye and 20/40 in the left. Dilated fundoscopy revealed CWS in the right eye. Spectral-domain optical coherence tomography (SD-OCT) showed hyper-reflective bands in the inner nuclear layer corresponding to parafoveal lesions seen on near-infrared imaging in the right eye consistent with the diagnosis of PAMM. Further laboratory studies were unremarkable, and a transthoracic echocardiogram and a carotid ultrasound were unrevealing. The patient was started on brimonidine three times daily in both eyes. The patient reported subjective improvement in the paracentral scotoma and the absence of ocular migraine symptoms at two-month follow-up. We conclude from this case that PAMM and CWS can occur simultaneously in ocular migraine, and we suggest that retinal vascular changes associated with ocular migraine may contribute to ischemia underlying both entities. Additionally, we suggest a potential therapy in brimonidine due to its proposed beneficial effects on retinal vasculature and neuroprotection., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To assess the effect of corticosteroids (CS) on choroidal neovascularization (CNV) occurrence and recurrence of activity over 2 years in patients with punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC)., Methods: Retrospective longitudinal study. Previous use of CS was analyzed between group without CNV and group with CNV occurrence and recurrence., Results: Thirty-six patients were included. Patients with CNV were less likely to have received CS in the 6 months following PIC or MFC diagnosis (17% versus 65%, p-value = 0.01). Patients with CNV who had a recurrence of neovascular activity were less likely to have received a previous CS therapy (20% versus 78%; odds ratio = 0.08, p-value = 0.005)., Conclusions: This study suggests that patients with PIC and MFC should be treated by CS to prevent CNV development and decrease CNV recurrences.

Purpose: To assess the efficacy of treatment on acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and relentless placoid chorioretinopathy (RPC)., Methods: Cases were identified from three UK uveitis centers. Retrospective analysis of visual acuity recovery; OCT structural outcomes; and retinal lesion quantification in observed and treated cases of APMPPE/RPC., Results: There were nine APMPPE and three RPC cases. Out of 12 patients, six were female. Median age: 26.5 years (range, 20-57 years). Four cases (six eyes) were observed, and eight cases (15 eyes) received corticosteroids ± immunosuppression. 4/4 observed and 6/10 treated foveal involving eyes regained 0.00 LogMAR vision. Observed lesions achieved more favorable anatomical outcomes. New lesions post-presentation developed in 1/6 (16%) observed eye versus 10/15 (66%) treated eyes. In three cases, a delayed, rebound lesion occurrence was observed post-high-dose corticosteroids., Conclusions: While subject to potential treatment bias, in this small case series, natural history alone appears non-inferior to corticosteroid treatment.

Purpose: To present the process from acute Posner-Schlossman syndrome (PSS) relapse to remission under 2% ganciclovir (GCV), corticosteroids and anti-glaucoma agents on 323 patients., Methods: A retrospective study enrolling 323 PSS patients. Demographics and ophthalmic examination results were generated. Patients were treated with GCV, corticosteroids and anti-glaucoma agents andfollowed up every 2-6 weeks., Results: Patients were divided into the GCV monotherapy ( N  = 65, 20.12%), GCV and corticosteroids (G+C, N  = 106) and GCV, corticosteroid and IOP-lowering drugs (G+C+L, N  = 152) group. The G+C+L group had the highest intraocular pressure (IOP, 26.33 ± 10.26 mmHg, P  < 0.001) and largest cup-to-disc ratio (0.58 ± 0.19, P  < 0.05). After treatment, IOP of three groups dropped to similar level. Ninety-nine (30.65%) patients were corticosteroid-dependent whose daily corticosteroid consumption decreased after using GCV (from 2.23 ± 1.02 to 0.97 ± 0.98 drops/day)., Conclusion: 2% GCV solutions worked effectively on PSS relapse with corticosteroids and anti-glaucoma agents. In patients suspected of CMV infection, proper GCV could reduce the chance of corticosteroid dependence.

Choroidal neovascularization (CNV) affects 64-75% of eyes with punctate inner choroidopathy (PIC). Although anti-VEGF agents are considered first-line therapy, there is controversy regarding other modalities, such as immunosuppression. We performed a systematic review of individual participant data (IPD) and generated a dataset of 278 eyes with PIC-related CNV from 45 studies. Forty-two percent presented with moderate visual loss (MVL) or worse. Four different treatment modalities (anti-VEGF, photodynamic therapy, local immunosuppression, and systemic immunosuppression) and most combinations among them were represented. Anti-VEGF injections decreased the likelihood of MVL (Odds Ratio 0.3, p = .027), an effect moderated by presenting visual acuity and patient age. Eyes receiving more than 3 injections were more likely to receive additional therapeutic modalities. Increasing number of modalities was associated with longer follow-up time and did not improve vision. The beneficial effect of anti-VEGF injections persisted when controlling for presenting visual acuity and follow-up time.

Purpose: To evaluate the clinical course of idiopathic multifocal choroiditis (MFC) and punctate inner choroidopathy (PIC) and the efficacy and safety of treatment options during pregnancy., Methods: Patients with MFC or PIC and a pregnancy in 2011-2019 from two academic centres were enrolled. For the most recent pregnancy, data on best-corrected visual acuity (BCVA) before and after pregnancy, relapse rate in pregnancy and postpartum period and obstetric, maternal and neonatal outcomes were collected. Treatment regimens consisted of a wait-and-see regime and an immunosuppressive treatment regime with systemic corticosteroids and/or azathioprine, both combined with intravitreal antivascular endothelial growth factor injections when indicated., Results: Sixteen women (26 affected eyes) were included. Median Snellen BCVA was 20/19 before pregnancy and 20/18 after delivery. In seven pregnancies a wait-and-see regime and in nine pregnancies an immunosuppressive treatment regime was carried out. Fourteen intravitreal anti-VEGF injections were given in six pregnancies. The relapse rate during pregnancy was 44% and in the postpartum period 31%. Maternal/obstetrical and fetal complications occurred in 31% and 13% of the pregnancies, respectively. Fifteen healthy children were born and one pregnancy ended in a stillbirth in a patient with a complicated obstetrical history. One patient treated with azathioprine developed intrahepatic cholestasis of pregnancy (ICP)., Conclusions: Among women with MFC and PIC BCVA remained stable during pregnancy despite a relapse rate of 44% in pregnancy. No major maternal, obstetric and fetal complications occurred in pregnant patients treated with systemic corticosteroids, azathioprine or intravitreal anti-VEGF injections, though one patient developed ICP while treated with azathioprine., (© 2021 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose : To report two cases of syphilitic outer retinopathy that showed diffuse phlebitis on fluorescein angiography (FA). Methods : Retrospective chart review. Results : The two patients had presentation similar to acute zonal occult outer retinopathy (AZOOR), including hyperautofluorescence on fundus autofluorescence and ellipsoid zone disruption on optical coherence tomography. The main difference from AZOOR was the finding of diffuse phlebitis on FA. Both patients recovered well after antibiotic and steroid treatment. Conclusion : FA is especially important for differentiating syphilitic outer retinopathy from AZOOR. Timely diagnosis and treatment with penicillin and cautious use of steroid usually lead to favorable prognosis.

Purpose: To describe a clinical picture resembling classic multiple evanescent white dot syndrome (MEWDS) potentially triggered by previous or concurrent, apparently unrelated, ocular events and to provide a literature review of similar presentations., Design: Retrospective chart series and literature review., Participants: Consecutive patients diagnosed with MEWDS at the Feinberg School of Medicine, Northwestern University, Chicago, Illinois, and the IRCCS San Raffaele Scientific Institute, Milan, Italy, between July 2019 and June 2020., Methods: Charts of patients were reviewed. Ophthalmic history, best-corrected visual acuity, spectral-domain OCT results, OCT angiography results, fundus autofluorescence results, ultra-widefield fluorescein angiography results, and indocyanine green angiography results were collected. A PubMed-based search was carried out for similar presentations using the terms MEWDS and white spot syndromes., Main Outcome Measures: An ocular history positive for previous or concurrent ocular events in patients with MEWDS was sought in our cohort and the existing literature., Results: Five eyes of 4 patients (2 females; age range, 16-81 years) were included. The first eye had a history of bilateral Best vitelliform dystrophy and unilateral choroidal neovascularization. The second eye had angioid streaks complicated by choroidal neovascularization and underwent prior thermal laser photocoagulation. The third eye had a history of high myopia and a scleral buckle procedure for retinal detachment. The fourth patient had bilateral idiopathic retinochoroiditis. We identified 16 case reports from 5 previous publications that support a MEWDS-like reaction to previous ocular insults., Conclusions: We suggest a MEWDS-like reaction may be elicited by ocular events in a subset of susceptible patients. We hypothesize that damage to the outer retina may play a role in triggering the local inflammatory response., (Copyright © 2020 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Purpose : Placoid-pigment-epitheliopathies like Acute-Posterior-Multifocal-Placoid-Pigment Epitheliopathy (APMPPE) and Relentless-Placoid-Choroidopathy (RPC) usually show bilateral involvement. This retrospective case series describes the clinical features with unilateral disease onset. Methods : 21 patients from 9 tertiary referring institutions were collected and evaluated. Results : Seventeen patients were included: 11 (65%) had unilateral placoid-pigment-epitheliopathy consistent with APMPPE, 6 (35%) with RPC (mean age: 28.82 ± 9.1 years). Mean follow-up time was 45 ± 48 months. Forty-one percent fellow-eyes had initial inflammatory signs including vitreous-cells, vascular-sheathing, and ONH swelling. Thirty-five percent developed chorioretinal lesions during follow-up after 19.5 ± 16.9 months. Initial inflammation in fellow-eye was not associated with fellow-eye involvement ( p = .5). However, subfoveal choroidal-thickness (SCT) at baseline was significantly thicker in fellow-eyes who developed chorioretinal lesion compared to fellow-eyes who did not (400 ± 70 µm versus 283 ± 127 µm, p = .047). Conclusion : Unaffected fellow-eyes may have inflammatory signs at initial disease-onset and may develop lesions over time. They should be carefully monitored, particularly if they present with an increased SCT.

Purpose: To present an unusual case of recurrent multiple evanescent white dot syndrome (MEWDS) following the coronavirus 2019 (Covid-19) vaccination., Methods: Review of the clinical, laboratory, photographic, and angiographic records of a patient with MEWDS., Results: A 49-year-old female who had MEWDS nine years ago presented to our clinic with blurred vision in her left eye. These symptoms occurred 2 days following Covid-19 vaccination. Fundus examinations and diagnostic testing were consistent with recurrent MEWDS., Conclusion: While rare, MEWDS can reoccur following Covid-19 vaccination.

Purpose: To report the long-term prognosis of punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC) and associated zonal outer retinopathy (ZOR). Method: Retrospective study in patients with PIC/MFC and ZOR with a clinical follow-up of 4 years or longer. Results: There were 14 patients in this study (M: F = 11:3). All patients received systemic steroid therapy. The initial and final median logarithm of minimal angle of resolution of BCVA were 1.00 and 0.22 ( p = .002). Ellipsoid zone recovery was noted in all patients. The median visual field loss improved from -6.38 dB to -3.41 dB ( p = .035). The median of total area of PIC/MFC lesions enlarged from 6.82 mm 2 to 8.77 mm 2 ( p = .005). Recurrent disease was noted in 4 eyes and maintenance steroid was needed in 3 eyes. Conclusion: With steroid therapy, most patients with PIC/MFC and ZOR had good visual prognosis.

Purpose : To describe the changes seen on optical coherence tomography angiography [OCTA] in patients with PIC following immunosuppressive therapy. Methods : We reviewed serial OCTA scans from five consecutive PIC patients (5 eyes) with at least 3 months of follow-up, who underwent imaging before and after immunosuppressive therapy. Using ImageJ, superficial and deep retinal vasculature were analyzed for vessel area and foveal avascular zone. Choriocapillaris layer was analyzed for flow signal loss. Results : Five out of five patients received an orbital floor triamcinolone acetonide injection as the initial treatment for periods of activity. Mean choriocapillaris (CC) flow void area obtained after immunosuppressive therapy was significantly lower than the mean CC flow void area obtained prior to treatment (Pre-treatment: 0.270 vs Post-treatment: 0.144; p = .0068). In 2 out of 2 patients with longitudinal visual field testing, CC flow voids were spatially associated with visual field defects, and immunosuppressive therapy was associated with reduced CC flow void area and improved visual function. Conclusion : OCTA can detect alterations in choriocapillaris flow. Longitudinal follow-up demonstrates a centripetal restoration of choriocapillaris flow in response to immunosuppressive therapy. OCTA may be a useful adjunct for monitoring and evaluating treatment of PIC.

Purpose: To describe the differential response of two distinct inflammatory signs occurring in eyes with punctate inner choroidopathy., Methods: Retrospective, observational case series using multimodal imaging., Results: Four eyes of 4 myopic female patients (mean age of 35 years, range 31-42 years) presenting with retinal manifestations of punctate inner choroidopathy. All study eyes had 2 distinct signs of active disease: 1) acute focal hyperreflective lesions splitting the retinal pigment epithelium/Bruch membrane complex on optical coherence tomography which appeared hypoautofluorescent on fundus autofluorescence and 2) more diffuse areas of outer retinal disruption limited to the ellipsoid zone and interdigitation zone on optical coherence tomography and corresponding to hyperautofluorescence on fundus autofluorescence. All patients were treated with oral prednisone and demonstrated prompt regression of the retinal pigment epithelium/Bruch membrane complex lesions with a concurrent, paradoxical centrifugal expansion of outer retinal disruption. The outer retinal disruption eventually resolved in all eyes (mean time of 6 weeks, range 4-10 weeks)., Conclusion: In patients with punctate inner choroidopathy, two distinct inflammatory signs observed with multimodal imaging display a differential response to systemic corticosteroids. Although focal inflammatory lesions splitting the retinal pigment epithelium/Bruch membrane complex seem to respond rapidly, the more diffuse, transient outer retinal disruption shows little response. This difference in treatment response may reflect different immunological phenomena with independent natural history.

Background: This paper reports the case of a young man who presented with syphilis masquerading as multiple evanescent white dots syndrome (MEWDS), which turned out to be an acute syphilitic posterior placoid chorioretinopathy (ASPPC) during follow-up., Case Presentation: A 59-year-old healthy male consulted for a three days' history of visual impairment in both eyes. On multimodal imaging, he was diagnosed as MEWDS. Fundus fluorescein angiography (FFA) showed early peripheral bilateral granular hyperfluorescence that correlated with the yellow-white dots found on fundus exam. Indocyanine green angiography (ICGA) depicted hypofluorescent dots on late phase. Spectral-domain optical coherence tomography (SD-OCT) revealed numerous inner retinal highly reflective deposits in the outer nuclear layer and disruption of the ellipsoid zone. After initial improvement, he presented again for a sudden visual loss at 3 weeks. FFA, ICGA and SD-OCT demonstrated the same but more numerous and outer lesions suggesting an ASPPC. A full inflammatory work-up revealed highly positive titers of rapid plasma regain (RPR) and fluorescent treponemal antibody absorption (FTA-Abs), suggesting a syphilis infection. The ophthalmological manifestations dramatically improved after the patient was admitted for high-dose intravenous penicillin G 24 million per day for 2 weeks., Conclusion: This is the first case that reports an ocular syphilitic infection masquerading as MEWDS at presentation and that turns to be an ASPPC. Syphilis serology should be routinely done in every case of atypical MEWDS especially when unusually presented in a young healthy man, with bilateral involvement and a bad clinical evolution.

An atypical case of acute posterior multifocal placoid pigment epitheliopathy with a clear reappearance of the ellipsoid layer of the retina after Ozurdex ® intravitreal implantation is presented. A 51-year-old woman reported a 3-week history of left eye photopsia. On slit-lamp examination, yellowish placoid lesions were found on her left eye fundus. Ancillary tests were performed. The patient was diagnosed as a left eye acute posterior multifocal placoid pigment epitheliopathy, and observational approach was decided. Later, the condition started to progress in an ampiginous manner and a decrease of visual acuity caused by an increase in number and size of the lesions was observed. As the disease was progressing with the conservative, observational approach, and the macula was menaced, an intravitreal dexamethasone implant was injected in the left eye with a consequent improvement of the visual acuity and lesion stabilization. The ellipsoid layer, unidentifiable inside the placoid lesions in previous optical coherence tomography tests, reappeared after the treatment. Intravitreal dexamethasone implants can be used to stabilize acute posterior multifocal placoid pigment epitheliopathy lesions and help resolve the condition. Spectral domain optical coherence tomography can also be useful for monitoring these lesions, as the ellipsoid layer may reappear upon resolution.

Purpose: To report a case of acute macular neuroretinopathy in a patient with Susac syndrome., Methods: Case report., Results: A 39-year-old male patient presented with severe headache, photopsias, and a sudden onset of hearing loss in the right ear. Fluorescein angiography of the right eye revealed multiple branch retinal artery occlusions. Clinical presentation of encephalopathy, hearing loss, and branch retinal artery occlusions, along with characteristic magnetic resonance imaging findings, led to a diagnosis of Susac syndrome. Despite aggressive immunosuppression for four months, the patient later presented with acute macular neuroretinopathy in the left eye., Conclusion: Acute macular neuroretinopathy and Susac is a new association of two well-defined disorders. The concurrence of both disorders supports retinal ischemia as the proximate cause of acute macular neuroretinopathy and inflammation as a potential etiology.

Hemodialysis-related portosystemic encephalopathy (HRPSE) is a clinical phenomenon where portosystemic encephalopathy (PSE) develops without liver dysfunction, usually caused by changes in the portosystemic blood flow related to hemodialysis. We describe the case of a 22-year old patient with a transjugular intrahepatic portosystemic shunt (TIPS) who developed HRPSE several months after initiation of hemodialysis. Despite initial therapy with laxatives and neomycin symptoms recurred. It was only after relocation of the hemodialysis catheter from the superior caval vein to the femoral vein that symptoms completely resolved.

Multiple evanescent white dot syndrome (MEWDS) is an inflammatory eye disease of the outer retina, retinal pigmented epithelium, choroid presenting with photopsia, loss of vision, and temporal scotoma. The patient was a 31-year-old female with a history of vision loss since 11 days ago (left eye). At presentation, best-corrected Snellen visual acuity was 20/140 in the Snellen chart. We decided to treat her with short time corticosteroid therapy (0.75 mg/kg/day prednisolone which was tapered in 3 weeks) for any possible rapid recovery of vision. The visual acuity of the involved eye was improved to 20/25 and 20/20, one week and three weeks after starting treatment respectively. Thus, it seems that short-term oral steroids might be an alternative method of management for patients with MEWDS.

Purpose : To evaluate the long-term outcome of active choroidal neovascularization (CNV) in punctate inner choroidopathy (PIC) or multifocal choroiditis (MFC) after intravitreal bevacizumab treatment. Methods : Retrospective study of consecutive patients of PIC/MFC complicated with active CNV. Outcome measures included best-corrected visual acuity (BCVA), total number of intravitreal injections of bevacizumab and recurrence of CNV. Correlation analysis was performed to find the correlation of various clinical factors and final BCVA. Results : There were 23 eyes in 22 patients with a mean age of 33.22 years included in this study. The mean duration of follow-up was 6.48 years. Improvement of BCVA was noted through the first 3 years and at the final follow-up. BCVA at 1, 6, 12 months and recurrence of CNV were correlated with final BCVA. Conclusion : Most patients of PIC/MFC complicated with CNV managed with intravitreal bevacizumab had improved BCVA over 4 years.

Twenty-six-year-old male patient admitted to the outpatient clinic with a complaint of decreased vision in both eyes. Visual acuity was 2/10 in the right and 7/10 in the left eye. Fundus examination revealed bilateral yellow-white subretinal lesions involving the macula. Fluorescein angiography showed hypofluorescent lesions in early and hyperflourescent lesions in the late phase. Optical coherence tomography angiography performed in the acute phase showed bilateral hypofluorescency at the choriocapillary level. A diagnosis of acute posterior multifocal placoid pigment epitheliopathy was given. After steroid treatment, visual acuity was improved to 10/10 and earlier lesions in optical coherence tomography angiography were found to be reduced. In conclusion, optical coherence tomography angiography is a noninvasive imaging technique that can be used in the diagnosis and follow-up of acute posterior multifocal placoid pigment epitheliopathy.

A 21-year-old man experienced unilateral vision loss associated with multiple atrophic chorioretinal lesions. He was treated for a presumptive diagnosis of acute retinal necrosis, but his vision did not improve with antiviral therapy. Over the course of several weeks, his symptoms progressed to involve both eyes. The fellow eye showed characteristic yellow-white placoid lesions, prompting treatment with oral corticosteroids for acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Despite high-dose therapy with prednisone 80 mg daily, the patient developed the acute onset of mental status changes within the next several days. Neuroimaging revealed multifocal large-vessel strokes associated with cerebral edema; these infarcts led to herniation and death. Postmortem histopathologic examination confirmed granulomatous inflammation in both ocular and cerebral vasculatures. Together with findings from multimodal imaging obtained throughout this patient's clinical course, our findings support the notion that granulomatous choroiditis is the mechanism of the ocular lesions seen in APMPPE. This granulomatous inflammation can also affect cerebral vessels, leading to strokes.

Purpose : To describe with multimodal imaging a case of post-infectious unilateral outer retinopathy with choroiditis. Methods : Retrospective chart review of a case of a 67-year old male who presented following the onset of viral symptoms with an acute onset outer retinopathy, small vessel leakage on fluorescein angiography, and choroidal involvement evident on indocyanine green angiography and near infrared fundus autofluorescence (NIR-AF). Work up for infectious and autoimmune etiologies was negative. Results : Treatment with IV methylprednisolone followed by high dose oral prednisone resulted in improvement in visual acuity, outer retinal reconstitution and resolution of the choroidal changes. Conclusion s: Despite this presentation sharing features with both acute zonal occult outer retinopathy (AZOOR) and multifocal choroiditis (MFC), the case is highly atypical of both entities.