Routine ophthalmologic examination in Klebsiella pneumoniae bacteremia is not necessary: incidence of and risk factors for ocular involvement.

Klebsiella pneumoniae bacteremia is known to present a virulent clinical course, including multiple metastatic infections, which is not uncommon in Asia. However, there are limited data on the incidence and risk factors for ocular involvement in K. pneumoniae bacteremia. We retrospectively reviewed the medical records of all patients with K. pneumoniae bacteremia who underwent ophthalmologic examination in a tertiary center in Seoul, Korea, from February 2012 to December 2020. Two retinal specialists reviewed the findings of the ophthalmologic examinations and classified them as endophthalmitis, chorioretinitis, and no ocular involvement. Of 689 patients, 56 [8.1%; 95% confidence interval (CI) 6.2-10.4] had ocular involvement, and 9 (1.3%; 95% CI 0.6-2.5) were diagnosed with endophthalmitis. Of 47 patients with chorioretinitis, 45 (95.7%) improved with systemic antibiotic therapy alone. Community-onset bacteremia (100% vs 62.1% vs 57.4%, P = 0.04), cryptogenic liver abscess (55.6% vs 11.8% vs 8.5%, P = 0.003), and metastatic infection (66.7% vs 5.8% vs 10.6%, P < 0.001) were more common in endophthalmitis than in no ocular involvement or chorioretinitis. In the multivariable analysis, cryptogenic liver abscess [adjusted odds ratio (aOR), 6.63; 95% CI 1.44-35.20] and metastatic infection (aOR, 17.52; 95% CI 3.69-96.93) were independent risk factors for endophthalmitis. Endophthalmitis was not associated with 30-day mortality. Endophthalmitis is rare in Asian patients with K. pneumoniae bacteremia. Targeted ophthalmologic examination in those with cryptogenic liver abscess, metastatic infection, or ocular symptoms may be more appropriate than routine examination of all patients.

Incidental multiple myeloma in a patient with neuroretinitis: a case report.

BACKGROUND: Neuroretinitis is classically defined as a clinical triad of unilateral, painless vision loss, accompanied by optic disc edema and characteristic macular star formation. The causes of neuroretinitis can be categorized as infectious, non-infectious, and idiopathic, therefore differential diagnosis and careful evaluation are required, owing to the various etiologies and masqueraders. CASE PRESENTATION: A 54-year-old woman presented to the clinic with blurred vision in both eyes. A complete ophthalmic examination revealed optic disc edema with blurred margins and macular exudates, intraretinal edema in the temporal peripapillary area, and subretinal fluid with neurosensory retinal detachment in the macular area. Systemic laboratory investigations showed no signs of infection or inflammation. However, bone marrow suppression was suspected based on the results of the complete blood count test, and the patient was diagnosed with multiple myeloma. CONCLUSION: Although neuroretinitis is rarely accompanied by hematological malignancy, it is important to be mindful of the latter because ophthalmic manifestations are a common feature of hematological malignancies and lesions occur in nearly every ocular structure.

BILATERAL SERPIGINOUS-LIKE CHORIORETINITIS ASSOCIATED WITH CILIOCHOROIDAL MELANOMA: A Clinicopathologic Correlation.

PURPOSE: To report the clinicopathologic correlation of a case of bilateral serpiginous-like chorioretinitis (SLC) associated with unilateral ciliochoroidal melanoma. METHODS: A 71-year-old white woman was diagnosed with progressive SLC in both eyes associated with ciliochoroidal melanoma in the right eye. Clinical findings and imaging before and after enucleation in the right eye were correlated to histologic and immunohistochemistry sections. RESULTS: Examination and imaging identified a peripheral bilobed amelanotic lesion with low reflectivity on B-scan ultrasound with an associated exudative detachment in the right eye. Additionally, multiple areas of new SLC lesions in the macula and peripapillary region in the right eye and along the inferior arcade in the left eye were observed. Oncologic evaluation confirmed a Class 2, ciliochoroidal melanoma, and the eye was enucleated. Autoimmune and infectious laboratory evaluations for the etiology of the SLC lesions were negative. Histopathology of the enucleated eye confirmed the diagnosis of uveal melanoma with lymphocytic inflammation at the edges of the tumor itself and in the areas of discrete SLC lesions. Immunohistochemistry identified similar predominantly CD3 and CD8 T cells and fewer CD20 B cells in both regions. CONCLUSION: Serpiginous-like chorioretinitis may present as a paraneoplastic, predominantly T-lymphocyte inflammation associated with intraocular tumor such as uveal melanoma.

Optic Disc Edema in Syphilis.

BACKGROUND: Syphilis is an uncommon cause of optic nerve head edema; however, differentiating syphilis from other etiologies of optic nerve head swelling may be challenging. We describe 4 cases of ocular syphilis presenting with swollen optic nerve head(s) without overt signs of intraocular inflammation to better define the phenotypic presentation of this condition to allow its early recognition and treatment and discuss potential pathophysiological mechanisms of syphilitic optic neuropathy. METHODS: Retrospective case series of patients presenting to a tertiary neuro-ophthalmology practice with a swollen optic nerve head(s) but no overt signs of intraocular inflammation, which was eventually determined to be secondary to syphilis. RESULTS: Four patients were included in the study. The mean age was 43 years, 2 were women and 2 had bilateral involvement. Two patients had a recent history of skin rash, and one patient was investigated for abdominal pain and elevated liver enzymes. Two patients presented with photopsias and preserved visual function, whereas 2 presented with vision loss. Although chorioretinitis was present in all cases, it was very subtle in all and was only appreciated on fundus autofluorescence (FA) in 3 of 4 cases. Three patients demonstrated evidence of optic perineuritis on neuro-imaging. All patients were treated with a course of intravenous penicillin with a variable degree of visual recovery. CONCLUSIONS: Systemic symptoms are common in patients with syphilic optic neuropathy. Optic disc edema as a manifestation of syphilis is usually accompanied by subtle chorioretinitis, which is best appreciated on FA. Optic perineuritis is common in patients with syphilitic optic neuropathy, with its pathophysiology likely similar to meningitis seen in neurosyphilis.

Concurrent tuberculous chorioretinitis with choroidal neovascularization and tuberculous meningitis: a case report.

BACKGROUND: Tuberculosis (TB) remains a severe health burden worldwide. The manifestation of concurrent tuberculous cerebral and ocular involvements associated with TB is uncommon. CASE PRESENTATION: We report a 17-year-old girl with concurrent tuberculous cerebral and ocular involvements and visual impairment due to choroidal neovascularization. This study emphasizes the definite diagnosis with the combination of ophthalmological examination, multimodal imaging and routine tuberculosis testing, and the proper management with intravitreal anti-VEGF injection accompanied by systemic anti-tuberculosis therapy. CONCLUSION: Combined applications of routine TB tests, fundus multimodal imaging and diagnostic therapy greatly help the clinician to establish a precise diagnosis and in monitoring the therapeutic response.

Adalimumab in refractory cystoid macular edema associated with birdshot chorioretinopathy.

PURPOSE: To report the clinical outcomes of adalimumab therapy in cases of birdshot chorioretinitis (BCR) with cystoid macular edema (CME) refractory to conventional immunotherapy. METHODS: This is a retrospective case series of three BCR patients treated with adalimumab for refractory CME. The main outcome measure was central subfield thickness (CST) on optical coherence tomography. Any patients treated with local steroids and/or receiving systemic steroids higher than 40 mg prednisolone daily during adalimumab therapy were excluded. RESULTS: At baseline, all patients were receiving systemic corticosteroids and two second-line immunosuppressive agents. The mean duration of treatment with adalimumab was 31.2 months (range 17.2-52). The mean CST was 327 ± 112.7 µm (mean ± SD) at baseline and 256.2 ± 39.7 µm at 6 months and 235.5 ± 32.5 µm at 12 months. Adalimumab permitted cessation or reduction in the daily dose of oral prednisolone plus withdrawal of a second-line agent in all patients. CONCLUSIONS: In these patients, adalimumab was effective in the treatment of refractory CME.

[The clinical manifestations and imaging characteristics of acute syphilitic posterior placoid chorioretinitis].

Objective: To investigate the clinical manifestations and imaging characteristics of acute syphilitic posterior placoid chorioretinitis (ASPPC). Methods: Retrospective study of 10 patients diagnosed ASPPC in the Department of Ophthalmology, Beijing Tongren Hospital from 2011 to 2016, including 6 males (10 eyes involved) and 4 females (8 eyes involved) with an age of (43.4±11.6) years (range, 26-60 years). Their clinical manifestations and imaging characteristics were summarized. Results: All 10 ASPPC patients complained about the reduction of visual acuity, and the majority of them had both eyes involved. The initial visual acuity ranged from finger count to 0.8, with an average of 0.4 and a median visual acuity of 0.3. The intraocular pressure was in the normal range. The inflammation of anterior chamber occurred in only one patient (1/10). The lesions were located at the posterior pole as shown on fundus photographs. There are six placoid lesions, four yellowish massive lesions and seven mixed lesions (placoid and yellowish massive lesions). Partial or whole disappearance, abnormality and opacity of the ellipsoid layer, retinal pigment epithelial (RPE) nodules, and cells in the vitreous body were observed by optical coherence tomography. There were partial detachment between the neuronal retina and RPE layer with fine-sand like hypereflective dots. The lesion size and morphology on autofluorescence (AF), fundus fluorescence angiography (FFA) and indocyanine green angiography (ICG) were almost consistent with those on fundus photography. The lesions were hyperautofluorescent in a placoid or massive shape. The lesions on FFA showed slight hyperfluorescence with unclear edges from the venous phase, and the fluorescence increased with the time and leaked in the late phase. Vascular walls showed blood staining in the late phase. The lesions on ICG showed hypofluorescence with hyperfluorescence and hypofluorescence dots like fine needles in the middle and late phases. Conclusion: ASPPC mainly occurs among the young adults without gender difference. The characteristic is the contradiction between sever clinical symptoms and slight signs on the fundus. The lesion area shown on fundus photography, FFA, ICG and AF is consistent, as well as injuries of the ellipsoid layer, RPE nodules and cells in the vitreous body. The above manifestations and imaging characteristics of ASPPC are significant for the diagnosis and differential diagnosis. (Chin J Ophthalmol, 2017, 53:352-357).

Spectral-domain optical coherence tomographic features of choroidal neovascular membranes in multifocal choroiditis and punctate inner choroidopathy.

PURPOSE: To describe the spectral-domain optical coherence tomographic (SD-OCT) features of inflammatory choroidal neovascular membranes (iCNV) in multifocal choroiditis and punctate inner choroidopathy, and to compare them to those of the acute inflammatory lesions in the same underlying diseases. This is a retrospective, consecutive, observational case series. METHODS: Each patient underwent a comprehensive eye examination, fundus photography, and fluorescein angiography (FA) on the initial visit. SD-OCT features of iCNV were reviewed at presentation and 4 weeks later, and were compared to SD-OCT features of the inflammatory lesions. There were ten eyes with iCNV and eight eyes with the acute lesions of chorioretinitis. RESULTS: All iCNV had a sub-retinal pigment epithelium (sub-RPE) component and a subretinal or retinal component that infiltrated the outer retinal layers to different extents. All iCNV had associated fluid exudation, and all showed RPE and inner segment/outer segment junction layer (IS/OS) disruption. On the other hand, approximately half of the inflammatory lesions were confined between Bruch's membrane and RPE; the rest showed infiltration into the outer retinal layers in a pattern similar to iCNV, with no fluid exudation but with associated choroidal hyperreflectivity. In most of them, disruption of RPE and IS/OS was also noted. CONCLUSIONS: The acute lesions of chorioretinitis can be difficult to distinguish from iCNV based on clinical examination and FA. However, iCNV demonstrate characteristic SD-OCT features not seen with the inflammatory lesions. These findings may help to differentiate these two entities that typically require different treatments.

Choroidal degeneration in birdshot chorioretinopathy.

PURPOSE: The purpose of the study was to determine if progressive choroidal changes occur in birdshot chorioretinopathy (BSCR). METHODS: Retrospective chart review of all patients with BSCR who were seen over a 3-year period. Controls consisted of healthy age-matched and gender-matched patients. Choroidal thickness at baseline and final follow-up visit was measured with the use of optical coherence tomography. Results were analyzed using univariate and multivariable statistical models. RESULTS: A total of 11 patients (22 eyes) with BSCR were identified. The majority of BSCR eyes (86%) had clinically inactive disease. Follow-up ranged from 2 months to 27 months. Mean age was 55 years. Patients with BSCR had significantly thinner choroid compared with controls (P < 0.001). Furthermore, the rate of choroid thinning for patients diagnosed with BSCR (2.68 µm per month) was significantly higher than that of controls (0.27 µm per month) (P = 0.003). There was no statistically significant difference in the rate of choroidal thinning between the two eyes of patients with BSCR (P = 0.859), indicating that the choroidal thinning was symmetrical. CONCLUSION: Despite having clinically inactive uveitis, eyes with BSCR can develop progressive choroidal thinning. The clinical relevance of this choroidal thinning, or degeneration, remains to be fully elucidated.

Focal choroidal excavation associated with focal retinochoroiditis.

PURPOSE: To describe detailed spectral-domain optical coherence tomography (OCT) findings for two patients with focal choroidal excavation (FCE) associated with focal retinochoroiditis. CASE REPORT: Three eyes from two patients with FCE associated with focal retinochoroiditis were evaluated by funduscopy, fluorescence angiography, indocyanine green angiography, and spectral-domain OCT during follow-up. Both patients with focal retinochoroiditis developed new FCE after oral steroid treatment and two eyes showed regression of the FCE during the follow-up. Both eyes from one patient transformed from the conforming to the nonconforming type and neither of the eyes were stable during the follow-up. Ultimately, all eyes exhibited the conforming-type FCE. CONCLUSIONS: Focal choroidal excavation can be seen as a tomographic phenotype after the treatment of focal retinochoroiditis. Spectral-domain OCT was useful for detecting the development of FCE after the treatment and for observing FCE regression.

[Ocular manifestations of syphilitic uveoretinitis in patients co-infected with human immunodeficiency virus].

OBJECTIVE: To investigate the clinical manifestations of syphilitic uveoretinitis in patients co-infected with human immunodeficiency virus (HIV). METHODS: Uveoretinitis patients presenting between January 2008 and December 2014 at Eye and ENT Hospital of Fudan University were collected. Patients were selected with positive serologic tests, including rapid plasma regain titer (RPR) > 1:8, treponema pallidum particle agglutination assay (TPPA) and human immunodeficiency virus (HIV). Other causes of uveoretinitis were excluded. Each patient underwent complete ophthalmologic examination including best-corrected visual acuity (BCVA), slit-lamp biomicroscopy, ophthalmoscopy, applanation tonometry and B-scan ultrasonography. Fundus fluorescein angiography were obtained in each case except for patients whose fundus was blurred with dense vitritis. Optical coherence tomography (OCT) was performed in selected patients. Thorough physical examination was performed simultaneously. RESULTS: Twenty six patients were included in this study including 24 male (92.3%) and 2 female (7.7%). The mean age at presentation was 39.3 ± 13.2 (range 20 to 63 years). RPR titres ranged from 1:32 to 1:512. There were 49 eyes altogether and ocular involvement was bilateral in 23 patients (88.5%) and unilateral in 3 patients (11.5%). Panuveitis was the most common ophthalmic presentation (n = 39, 79.6%). Only two eyes were anterior uveitis (4.1%) and 8 eyes were posterior uveitis (16.3%). Anterior chamber cells+++ and mild to severe vitreous opacities were observed. Sixteen eyes presented with neuroretinitis (32.7%), 27 eyes had vitritis (55.1%), 5 eyes had retinochoroiditis (10.2%), 5 eyes had optic neuritis (10.2%), 5 eyes had retinitis (10.2%) including 2 eyes with necrotizing retinitis (4.1%), 4 eyes had retinal vasculitis (8.2%). Two eyes (11.5%) presented with posterior placoid chorioretinitis (4.1%) and multifocal choroiditis (4.1%). Systemic manifestations were detected. Five patients had a history of skin rash (19.2%), five (19.2%) had genital ulcers, three (11.5%) had chancre, two (7.7%) suffered from headache, two (7.7%) had a fever, one (3.8%) had oral ulcers and one patient (3.8%) lost his hair. CONCLUSIONS: The clinical features are protean and lack of specificity. The most common manifestations was neuroretinitis, ususlly with vitritis.

Long-term Ocular Outcomes in Congenital Toxoplasmosis Treated Perinatally.

BACKGROUND: Congenital toxoplasmosis (CT) can be accompanied by serious organ manifestations, particularly retinochoroiditis, and may occur throughout life. We aimed to monitor long-term ocular prognosis in a large French cohort of patients with CT and its changes over time in the context of mandatory prenatal screening (since 1992) and incidence decrease since 2008. METHODS: Patients with CT diagnosed between 1987 and 2021 were prospectively included and followed for up to 35 years. The effect of the period of conception on the risk of first retinochoroiditis has been tested using a flexible extension of the Cox model. Incidence rates of retinochoroiditis were estimated. RESULTS: A total of 646 infected live born children were followed for a median of 12 years (range, 0.5-35); 187 patients (29%) had at least 1 ocular lesion (first at a median age of 5 years; range, 0-26 years) with peaks at 7 and 12 years. Early maternal infection and the presence of nonocular signs at birth were associated with a higher risk of retinochoroiditis, whereas delayed diagnosis of CT (after birth versus before or at birth) was associated with a lower risk (13% decrease for each additional month after birth; P = .01). A period effect for the risk of developing retinochoroiditis in patients born after 2008 was not detected. CONCLUSIONS: Despite prenatal screening and prolonged perinatal treatment, retinochoroiditis is not a rare event in French patients with CT and can occur well into adulthood, with peak incidences at 7 and 12 years of age. It rarely causes severe damage but warrants regular follow-up into adulthood.

Interleukin-17 production and T helper 17 cells in peripheral blood mononuclear cells in response to ocular lysate in patients with birdshot chorioretinopathy.

PURPOSE: To determine the cytokine response to ocular lysates of peripheral blood mononuclear cells (PBMCs) from patients with birdshot chorioretinopathy (BSCR). METHODS: In the PBMCs of 19 patients with BSCR, T cell cytokine production in response to human retina and choroid lysates was analyzed with flow cytometry and compared to the responses against skin lysates. Five patients had active disease and had not yet been treated (naïve to systemic therapy); 14 patients had either immunomodulatory therapy (IMT) or inactive disease (referred as inactive/IMT). The PBMCs of 11 HLA-A29-positive healthy individuals were used as controls. RESULTS: The levels of interleukin-17 (IL-17) in supernatant of cultures stimulated with retina lysate were higher in patients with active BSCR compared to the HLA-A29 positive controls. The levels of other T cell cytokines (IL-10 and interferon-γ [IFN-γ]) in PBMC cultures did not change significantly after stimulation with ocular lysate. The frequency of CD4(+) IL-17(+) (T helper 17 [Th17]) T cells but not of CD4(+) IFN-γ (Th1) T cells was elevated in the PBMCs of patients with active BSCR stimulated by retina lysates compared to skin lysates. CONCLUSIONS: Our data demonstrate that PBMCs exhibit an IL-17-mediated immune response to retina lysate in patients with active disease naïve to systemic therapy. This is accompanied by the enrichment of IL-17-producing CD4(+) T cells. These findings support the current concept of chronic Th17-cell mediated inflammation and provide evidence that links the Th17 signatures to ocular-specific immune responses in BSCR.

Idiopathic Retinal Vasculitis, Aneurysms and Neuroretinitis: Clinical Improvement with Infliximab.

PURPOSE: Idiopathic retinal vasculitis, aneurysms and neuroretinitis is a rare vision-threatening condition. If untreated vision loss occurs due to complications of progressive retinal ischaemia including retinal neovascularisation, neovascular glaucoma and retinal exudation. Despite the proposed underlying inflammatory aetiology this condition demonstrates poor response to corticosteroid treatment. The aim was to describe two paediatric cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis treated with infliximab. METHODS: Two case reports. RESULTS: Infliximab treatment led to resolution of aneurysmal dilatations and retinal vasculitis, and reversal of some retinal capillary non-perfusion. CONCLUSION: Early infliximab treatment should be considered in cases of idiopathic retinal vasculitis, aneurysms and neuroretinitis.

A Case of Neuroretinitis following Inactivated Virion COVID-19 Vaccination.

PURPOSE: We report a rare presentation of neuroretinitis following vaccination with inactivated virion vaccine (COVAXIN). METHODS: Interventional Case Report. OBSERVATION: A 14-year-old female presented with sudden unilateral vision loss 3 days following COVID-19 vaccination. The clinical and radiological evaluation was consistent with classical neuroretinitis; the serological and immunological workup was negative. The patient responded well to the pulse steroid therapy and regained complete vision. CONCLUSION: The COVID-19 vaccine related adverse ocular events are beginning to emerge slowly and thus warrants close monitoring of all such cases. Also, ophthalmologists should be encouraged to seek vaccination status of patients presenting with inflammatory ocular conditions.

Response to Comments on 'Acute Bilateral Neuroretinitis and Panuveitis in A Patient with Coronavirus Disease 2019: A Case Report'.

PURPOSE: to respond to comments on our case report on COVID associated neuroretititis. METHODS: We gathered up to date statistics about the prevalence and incidence of epidemic retinitis in Iran and specifically our region, north-east of Iran. RESULTS: Our response to Kawal et al comments includes 3 items. First, the PCR result of the vitreous specimen was positive for COVID-19. Secondly, the clinical course of the patient's illness was typically similar to our numerous COVID patients and we were in the middle of the second peak of COVID at the time. Thirdly, although other causes of epidemic retinitis such as west nile river are relatively rare in our region, we had a significant rise in the incidence of epidemic retinitis in the peak of COVID. So, the most probable cause may be COVID-19. CONCLUSION: Although the ocular findings of our patient was similar to epidemic retinitis caused by other well-known organisms, we believe that based on positive vitreous sample PCR for COVID, typical clinical course of systemic illness, being in the peak of COVID pandemic with significant increase of similar patients during this period, our patient had COVID associated neuroretinitis/ epidemic retinitis.

Ocular Toxoplasmosis after Exposure to Wild Game.

PURPOSE: To describe eight patients with toxoplasma retinochoroiditis following exposure to wild game. METHODS: Retrospective, multicenter case series. RESULTS: Eight men, aged 29 to 71 (mean, 56 years), developed toxoplasmic retinochoroiditis after hunting and/or consuming wild game in the United States, including seven deer and one bear. Five patients developed the disease after eating undercooked game meat, while three developed ocular findings after cleaning hunted animals. Seven patients were healthy prior to exposure. LogMAR visual acuity at presentation was 0.697 ± 0.745, improving to 0.256 ± 0.335 by last follow-up. Disease complications developed in five (62.5%) patients, of which recurrence of retinochoroiditis was the most common. CONCLUSIONS: Contact with wild game is a potential source of primary ocular toxoplasmosis in immunocompetent adults. Hunters and consumers of rare game are at risk of serious ocular disease and appropriate contact precautions and cooking may reduce this complication.

Indocyanine Green Angiography Features in Acute Syphilitic Posterior Placoid Chorioretinitis.

PURPOSE: Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a rare clinical manifestation of ocular syphilis. The cause of the placoid lesion is still up for debate but could be caused by an impaired choriocapillaris perfusion. However, less attention has been paid to the hypofluorescence of the plaque on late-phase indocyanine green angiography (ICGA). The aim of this study was to comprehensively analyze multimodal imaging findings in patients with ASPPC and to highlight the value of ICGA for the diagnosis of ASPPC. DESIGN: Retrospective observational case study. METHODS: The medical records of patients with uveitis who consulted our tertiary center between 2012 and December 2015 were reviewed. Patients who were diagnosed with uveitis related to syphilis infection with posterior placoid lesions seen on multimodal imaging were included. We compared the aspect of ASPPC on fundus color photography, blue autofluorescence, fluorescein angiography, optical coherence tomography, and early-, mid- and late-phase ICGA. RESULTS: Fifteen eyes of 12 patients were included in the study. Hypofluorescent plaques were seen on late-phase ICGA in all eyes, corresponding to the placoid lesions visible on blue autofluorescence, while the choriocapillaris filling was normal on fluorescein angiography and ICGA. Within the plaques, optical coherence tomography showed ellipsoid zone disruptions, outer retinal disruptions, and retinal pigment epithelium granulations. CONCLUSION: ASPPC could be caused by retinal pigment epithelium dysfunction secondary to an infectious or inflammatory disorder, characterized by a hypofluorescence visible only on late-phase ICGA, and resulting in photoreceptor disruptions. The RPE impairment was reversible after prompt antibiotic treatment.