CYSTOID MACULAR EDEMA IN BIRDSHOT RETINOCHOROÏDITIS: Long-Term Treatment Study in 142 Patients.

PURPOSE: To assess the long-term efficacy and safety of treatments for cystoid macular edema in birdshot retinochoroïditis. METHODS: Observational retrospective study of 142 HLA-A29-positive patients with cystoid macular edema; the main outcome was the optical coherence tomography intraretinal cysts resolution. RESULTS: During the mean follow-up of 75 months (12-178), 61.3% of patients were successfully treated using 1 to 3 treatment steps, while the others needed more steps. At 6 months, there were no significant effects on ME for anti-TNF (tumor necrosis factor) and IVIg (immunoglobulin) in contrast to antimetabolites (OR 1.98), systemic GCS (glucocorticosteroids), CsA (cyclosporine A) and tocilizumab (odds ratio closed to 2.7), intraocular injected GCS (odds ratio of 4.2), and interferon (odds ratio of 4.4). The percentages of therapeutic success trend to decrease from the initial three treatment steps to the subsequent treatment steps, for systemic GCS (84% to 70%), for anti-TNF (42% to 33%), and for CsA (71% to 33%); the success percentages did not decrease for injected GCS (83% to 89%). Macular edema recurrence occurred with the highest percentage for injected GCS (86.8%, P = 0.01) and the lowest for tocilizumab (10.5%, P = 0.001). Interferons-α and tocilizumab were associated with the lowest prednisone daily doses. CONCLUSION: The classical uveitic cystoid macular edema therapeutic algorithm could be adapted to birdshot retinochoroïditis.

Unilateral Toxoplasma retinochoroiditis with frosted branch angiitis: a multimodal imaging study.

A woman in her late 30s presented with sudden diminution of vision, redness and pain in the right eye (OD) of 10 days' duration. Best corrected visual acuity (BCVA) was 20/160 in OD and 20/20 in the left eye (OS). Anterior segment of OD showed keratic precipitates, flare 3+, cells 2+ and a festooned pupil. Vitreous haze and cells were seen in OD. Frosted branch angiitis (FBA) was seen in all quadrants in OD and old Toxoplasma scar was seen in both eyes. Serum toxoplasma immunoglobulin G (IgG) was positive and IgM negative, and PCR of an aqueous humour sample was negative for Toxoplasma She was diagnosed with toxoplasa retinochoroiditis in OD and treated with intravitreal clindamycin injections, oral anti-Toxoplasma antibiotics and steroids. Three months later, her BCVA in OD was 20/40 with resolving inflammation. She presented 2 months later with a new focus of retinochoroiditis without FBA and an old Toxoplasma scar.

Fractal Changes of the Retinal Microvasculature in Syphilitic Uveitis.

PURPOSE: To quantify chorioretinal microvascular damage and recovery post-treatment in patients with acute syphilitic posterior placoid chorioretinitis (ASPPC) using fractal dimension (FD). METHODS: Retrospective cohort study of patients with serologically confirmed syphilitic uveitis. We obtained optical coherence tomography angiography (OCTA) scans at baseline and follow-up after intravenous penicillin treatment and computed FD of the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) using ImageJ. RESULTS: We enrolled seven patients with ASPPC (11 eyes), and 17 control subjects (34 eyes). Pre-treatment averages of FD-SCP, FD-DCP, and FD-CC were: 1.672 (±0.115), 1.638 (±0.097), and 1.72 (±0.137); post-treatment: 1.760 (±0.071), 1.764 (±0.043), and 1.898 (±0.047). After treatment FD-CC increased in all 11 eyes with an average of 0.163 (p = 0.003); FD-DCP increased in 10 (91%) eyes with an average of 0.126 (p = 0.003); and FD-SCP increased in seven (64%) eyes with an average of 0.089 (p = 0.059). Compared to the post-treatment FD values in the syphilitic group, controls had similar FD-SCP (p = 0.266), FD-DCP (p = 0.078), and FD-CC (p = 0.449). CONCLUSIONS: CC and DCP are mostly affected in ASPPC with minimal changes in the SCP. All vascular layers FD recovered after completing antibiotic treatment.

Endogenous Fungal Endophthalmitis: A Single-Center Retrospective Study and Review of the Literature.

PURPOSE: To evaluate factors that inform systemic antifungal choices in patients with endogenous fungal endophthalmitis (EFE). DESIGN: Single-institution retrospective case series. METHODS: Charts of EFE patients from 2010 to 2023 were reviewed. Patients treated systemically for EFE with a minimum of 14 days of follow-up were included. Outcome measures included time to improvement in vitritis or chorioretinitis, systemic therapy modification, and need for surgical intervention. RESULTS: A total of 20 eyes of 16 patients were included. Candida species were most common (43.8%), followed by culture-negative EFE (37.5%) and Aspergillus species (18.8%). In all, 90% of eyes had vitritis and/or macula-involving chorioretinitis. The majority of Candida infections (60%) or culture-negative EFE (75%) were treated initially with oral antifungals. Patients with a history of immune compromise, positive fungal culture, or positive Fungitell assay were more likely to be treated with early intravenous (IV) antifungal therapy. Two patients required systemic antifungal therapy modification because of worsening chorioretinitis, in 1 case due to voriconazole-resistant Aspergillosis that demonstrated chorioretinal lesion growth despite intravitreal amphotericin B injections and systemic voriconazole, and in the second case due to worsening chorioretinitis from Candida dubliniensis infection that regressed upon switch from oral to IV fluconazole. CONCLUSIONS: Initial systemic treatment decisions in patients with EFE were driven by systemic culture positivity, systemic symptoms, or comorbidities. Intravitreal antifungal therapy may be insufficient to arrest progression of chorioretinal lesions in some cases. Larger studies are needed to determine whether visible end-organ damage in the form of chorioretinitis may be useful for guiding systemic therapy changes.

Ocular toxoplasmosis following anti-tumour necrosis factor-α therapy combined with oral methotrexate therapy: A case report and review of literature.

Purpose: To report a case of ocular toxoplasmosis following long-term treatment with adalimumab and review the literature on ocular toxoplasmosis following anti-Tumour necrosis factor-α therapy. Method: A retrospective chart review of A 21-year-old male who developed retinochoroiditis in his left eye following adalimumab therapy combined with oral methotrexate. Result: A known patient of juvenile idiopathic arthritis (JIA) on adalimumab and oral methotrexate for the last four years presented to us with a blurring of vision for the last 15 days. Fundus examination of the left eye revealed severe vitritis and two patches of retinochoroiditis in the inferior part of the fundus. Subsequent investigations confirmed it to be a case of toxoplasma retinochoroiditis, and he responded to anti-toxoplasma treatment. A review of literature on a similar topic revealed five such cases, and the index case was the first such report in patients with JIA. Conclusion: The index case highlights the importance of early recognition and management of opportunistic infections in patients receiving biologicals.

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.

Effects of Subcutaneous Methotrexate in Patients with Refractory Uveitis and Chorioretinitis.

PURPOSE: Methotrexate (MTX) is an immunosuppressive agent used to treat noninfectious inflammatory eye conditions and is generally administered orally for ocular inflammatory diseases. When used in rheumatological diseases, subcutaneous administration has been reported to show higher efficacy than oral administration. Therefore, this study aimed to evaluate the effect of subcutaneous MTX in patients with refractory uveitis or choroiditis who did not respond to other immunosuppressive agents. METHODS: A retrospective case series study was performed between January and December 2018. Patients with uveitis or chorioretinitis who showed little to no treatment response for 6 months or more with conventional immunosuppressive agents were treated with MTX, administered subcutaneously. After 6 months of treatment, patients were evaluated to determine whether complete suppression of inflammation sustained for ≥28 days was achieved in both eyes and whether improvement can be confirmed by fluorescein angiography (FAG). RESULTS: Subcutaneous MTX treatment was performed on 18 patients: 11 had intermediate uveitis and seven had posterior uveitis. In the intermediate uveitis patient group, five patients (50% of the group excluding one patient who dropped out) showed improvement in FAG and three patients (30%) showed complete suppression of inflammation. In the posterior uveitis group, two out of seven patients (excluding two patients who dropped out) showed an improvement, two patients in the group showed little change, and one patient showed aggravation of FAG findings. CONCLUSIONS: The study confirmed that in patients with uveitis or chorioretinitis who had a refractory response to treatment with other immunosuppressive agents, subcutaneous MTX showed improved treatment efficacy.

Case Report: Multimode Imaging of Chronic Syphilitic Chorioretinitis.

SIGNIFICANCE: The clinical manifestations of ocular syphilis may mimic those of other diseases, which may result in a missed diagnosis and delayed treatment. PURPOSE: We describe multimodal imaging findings and treatment outcomes of a patient with chronic syphilitic chorioretinitis. CASE REPORT: A 40-year-old male patient complained of progressive decreased visual acuity of his left eye for more than 1 year. The best-corrected visual acuity was 20/20 in the right eye and 3/50 in the left eye. Relative afferent pupillary defect and 1+ vitreous cells were detected in the left eye. The authors performed fundus examination, fluorescence angiography, ultrawide-field fundus autofluorescence, structure optical coherence tomography, wide-field montage optical coherence tomography angiography, and visual field. Laboratory tests including a toluidine red unheated serum test (1:32) and the Treponema pallidum antibody (9.01S/CO) showed positive results. Chronic syphilitic chorioretinitis was diagnosed in both eyes. The patient was admitted for administration of intravenous penicillin G for 14 days, followed by intramuscular benzathine penicillin G weekly for three doses. Six months after treatment, the toluidine red unheated serum test ratio had decreased to 1:2 (positive). The best-corrected visual acuity was 20/20 in the right eye and 6/20 in the left eye. The reexamination results showed that the ocular structure and capillaris flow partially recovered. CONCLUSIONS: Chronic syphilitic chorioretinitis profoundly affects the structure of the retina and choroid; however, eyes may partially recover after an effective treatment. Ultrawide-field imaging technology has several advantages, such as broader imaging field and more details provided, in determining syphilis-induced ocular disorders.

TUBERCULOUS CHORIORETINITIS: A CHALLENGING CASE OF AN OPHTHALMIC MIMIC.

PURPOSE: To report a challenging case of tuberculous chorioretinitis. METHODS: Case report of a 51-year-old woman from the Middle East, who was referred from an optometrist with a suspicious retinal lesion in her right eye. RESULTS: Clinical examination showed multifocal, pale, elevated lesions temporal to the right macula with no vasculitis or hemorrhages. Infective and inflammatory workup showed unremarkable results. B-scan ultrasound confirmed an 8 mm × 3 mm × 10 mm right focal chorioretinal thickening. Computed tomography scanning showed calcified lung hilar nodes supporting a prior granulomatous process, along with an enhancing nodule in the right globe. Magnetic resonance imaging of the brain and obits showed retinal thickening of the temporal surface of the right globe with subtle enhancement without retrobulbar extension or evidence for cerebral vasculitis. Subretinal lesion biopsy showed mononuclear inflammatory cells with granulomatous inflammation, including multinucleated giant cells but no neoplastic features. Interferon-gamma release assay testing for tuberculosis showed negative result, but a high index of suspicion lead to tuberculin skin testing and subsequent treatment for tuberculous chorioretinitis. CONCLUSION: Ocular tuberculosis presents in a variety of ways, making it a challenging diagnosis. Herein, we describe such case of tuberculous chorioretinitis.

Atypical Unilateral Birdshot Chorioretinitis in a Hispanic Female.

A 62-year-old female patient was evaluated for gradual vision loss, floaters, and photopsia in her left eye. A left fundus examination revealed vitreous cells, hypopigmented lesions, and retinal vasculitis, and a workup revealed a positive HLA-A29 serology, all of which findings were consistent with birdshot chorioretinopathy. The patient was treated with oral prednisone and mycophenolate mofetil, which resulted in the adequate control of her uveitis. Even though unilateral cases are exceedingly rare and do not meet the established research criteria, this case highlights the importance of not minimizing the significance of birdshot lesions in the differential of patients with unilateral multifocal chorioretinitis.

The incidence, presenting clinical findings and treatment patterns of Birdshot Retinochoroiditis in a high-prevalence region: findings from Northern Ireland, England and Wales.

BACKGROUND: Birdshot Retinochoroiditis (BSRC) is a rare, chronic posterior uveitis that is strongly associated with HLA-A*29.2 positivity. To date, no robust incidence studies of BSRC have been undertaken. We present the first epidemiological study of BSRC in a high-prevalence region. METHODS: In collaboration with the British Ophthalmological Surveillance Unit, all new cases of BSRC between May 2017 and June 2019 were prospectively collected. Presenting demographics, symptoms, signs and treatment modalities were collected. A follow-up questionnaire twelve months later was also sent. RESULTS: Thirty-seven confirmed cases meeting the reporting criteria were identified. Twenty-three cases had both baseline and follow-up data. The total population incidence of BSRC was 0.035 cases per 100,000 person-years [95% CI 0.025-0.048 cases per 100 000 people]. 97.3% were HLA-A*29 positive. The median age was 46 years, with females making up 78% of patients. There were no significant differences in the latitudinal incidence of BSRC. At presentation, floaters were the most common symptom. Optic disc swelling was the most common sign. Mean presenting visual acuity was independent of symptom duration. Combined systemic corticosteroids and immunomodulatory therapy were the most common treatments at baseline and follow-up. Intravitreal steroids were equally popular at follow-up. CONCLUSIONS: This study provides the first nationwide estimate of the incidence of BSRC in a high-prevalence region. Cases were more common in females, with a broad range of presentation ages. No significant latitudinal effect of incidence was identified. Systemic therapy with steroids and IMT remain the most common treatments.

Spectral-domain optical coherence tomography biomarkers in vitreoretinal lymphoma.

BACKGROUND: Although early detection is critical, diagnosing vitreoretinal lymphoma (VRL) remains difficult. We sought to assess the potential diagnostic value of spectral-domain optical coherence tomography (SD-OCT) in VRL. METHODS: We reviewed the clinical records and pre-treatment SD-OCT images of biopsy-confirmed VRL and uveitis patients, with primary involvement of the sub-retinal pigment epithelium (RPE) and the outer retina, including acute syphilitic posterior placoid chorioretinitis (ASPPC), chronic stage sympathetic ophthalmitis (SO), and idiopathic multifocal choroiditis (MFC). RESULTS: We included 45 eyes of 45 VRL patients and 40 eyes of 40 uveitis patients (17 ASPPC eyes, eight chronic SO eyes, and 15 MFC eyes). On SD-OCT, lymphoma cell infiltration was observed in various retinal layers, most commonly in the sub-RPE (80%) and sub-retinal space (62%). Highly sensitive features for VRL as compared to uveitis included vitreous cells (93%), focal hyper-reflective sub-retinal infiltration (51%), and diffuse RPE elevations (56%). The features strongly specific for VRL included preretinal deposits (92.5%), intra-retinal infiltration (except the incomplete vertical hyper-reflective type, 100%), banded hyper-reflective sub-retinal infiltration (90%), and confluent RPE detachments (100%). We identified an approach to VRL diagnosis based on these SD-OCT findings: (1) two highly sensitive features plus one strongly specific feature; or (2) one highly sensitive feature plus two strongly specific features, demonstrated a sensitivity of 80% and specificity of 95% for VRL. CONCLUSIONS: The SD-OCT may enable the detection of detailed lymphoma infiltration characteristics and provide significant supplemental value for VRL diagnosis, particularly when combining highly sensitive and specific VRL-associated SD-OCT features.

Birdshot Chorioretinopathy: Resistant versus Responsive.

PURPOSE: To search findings that can explain the heterogeneity between Resistant and Responsive patients with birdshot chorioretinopathy. PATIENTS AND METHODS: This was a retrospective observational case series on "Responsive" versus "Resistant" birdshot chorioretinopathy. RESULTS: One-hundred-eighty and Ninety-nine patients were included in the Responsive and Resistant groups respectively. Multivariate analysis of paraclinical variables at the first visit demonstrated that mean deviation (p = .04), pattern standard deviation (p < .001), optic nerve head leakage (p = .012), large vessel leakage and staining (p = .01), and macular small vessel leakage (p = .03) were statistically significantly different between the two groups; however, at the visit preceding successful therapy, only macular small vessel leakage (p = .01) was statistically significantly different between the two groups. CONCLUSION: .Small vessel leakage in the macular area and/or optic nerve head leakage at the earliest visit might be risk factors for resistant birdshot chorioretinopathy.

Saprochaete clavata Chorioretinitis in a Post-chemotherapy Immunocompromised 9-Year-Old Child.

PURPOSE: To describe the management of bilateral chorioretinitis with Saprochaete clavata in a post-chemotherapy immunocompromised young patient. METHOD: A retrospective case report. RESULT: A 9-year-old boy treated with chemotherapy for type 2 acute myeloid leukaemia was diagnosed with Saprochaete clavata (formerly called Geotrichum clavatum) fungaemia. Systematic ocular examination revealed chorioretinitis of the left eye becoming bilateral within the next 3 days. Therapy was based on systemic administration of voriconazole, amphotericin B and flucytosine associated with granulocytic stimulation without stabilizing the ophthalmological situation. Bilateral intravitreal injections of amphotericin B were administered. Voriconazole residual blood concentration was monitored to adjust daily dose. Final best corrected visual acuity in the right eye was 20/50 and 20/20 in the left eye. CONCLUSION: This is the first report of chorioretinitis with Saprochaete clavata. Because of its unpredictable pharmacokinetics, especially in pediatric population, therapeutic drug monitoring of voriconazole is essential to control fungal infection.

ATYPICAL FORMS OF EYE TOXOPLASMOSIS IN CHILDHOOD. CASE REPORTS.

AIM: To present an outline of acquired atypical forms of ocular toxoplasmosis (OT) in childhood, with reference to the 100th anniversary of the discovery of this etiology by Professor Janků from Czechoslovakia, who was first to describe the clinical congenital picture of OT characterised by macular scar. MATERIAL AND METHODS: Symptoms of intraocular bilateral neuritis appeared in a 6-year-old girl, with visual acuity (VA) bilaterally 0.1. Toxoplasmic etiology was demonstrated in laboratory tests, and the patient was immunocompetent. Following treatment with macrolide antibiotic and parabulbar application of corticosteroid, the condition was normalised stably at VA 1.0 in both eyes. Bilateral retinal vasculitis was determined in an 8-year-old boy, with VA of 0.25 in the right eye and 0.25 in the left, with a medical history of strabismus detected after suffering from varicella. The examination for toxoplasmosis was negative, but pronounced general hypogammaglobulinaemia classes IgG, IgM and IgA was detected. Immunosuppressive and immunomodulatory therapy did not produce the desired effect, and the condition progressed to retinochoroiditis. Due to blindness and dolorous glaucoma, enucleation of the right eye was performed at the age of 15 years. Histologically toxoplasmic cysts with bradyzoites were detected, a subsequent laboratory test demonstrated toxoplasmic etiology upon a background of persistent regressing hypogammaglobulinaemia. General anti-toxoplasma and subsequent immunosuppressive treatment did not produce the desired effect, and at the age of 22 years the patient lost his sight also in the left eye. CONCLUSION: Atypical form of OT intraocular neuritis in an immunocompetent patient had a favourable course, whereas retinal vasculitis with retinochoroiditis in a temporarily immunocompromised patient ended in bilateral blindness.

Case Report: Choriocapillaris Flow Voids in Acute Syphilitic Posterior Placoid Chorioretinitis.

SIGNIFICANCE: Acute syphilitic posterior placoid chorioretinitis (ASPPC) is a rare clinical presentation of ocular syphilis. The worldwide incidence of syphilis has recently increased. Clinicians should be familiar with the distinct appearance and imaging findings of ASPPC and promptly initiate antibiotic treatment to limit functional vision loss and systemic complications of neurosyphilis. PURPOSE: This case demonstrates the utility of optical coherence tomography angiography (OCT-A) imaging to further understand the pathophysiology and visual prognosis of ASPPC. CASE REPORT: A 75-year-old man presented with 7 days of decreased vision in his left eye. His visual acuity was 20/20 in the right eye and 20/200 in the left eye. Fundus examination revealed placoid lesions in the superior and inferotemporal aspects of the posterior pole in the right eye and a large placoid macular lesion in the left eye. Optical coherence tomography imaging revealed disruption and loss of the ellipsoid zone and external limiting membrane, nodular elevations on the retinal pigment epithelium, and choroidal hyperreflective punctate lesions in the left eye. Optical coherence tomography angiography displayed choriocapillaris perfusion flow voids greater in the left eye than in the right eye. Two months after penicillin treatment, the placoid lesions resolved in both eyes with an improvement in outer retinal structural abnormalities on optical coherence tomography imaging and visual acuity to 20/25 in the left eye. Eleven months after presentation, the OCT-A choriocapillaris flow voids had improved without complete restoration. CONCLUSIONS: The flow voids seen on OCT-A imaging in this case of ASPPC suggest a possible inflammatory process with a primary location in the choriocapillaris.