Multifocal Chorioretinitis with Serous Macular Detachment in Diffuse Unilateral Subacute Neuroretinitis (DUSN): Unique Presentation and a Diagnostic Dilemma.

PURPOSE: To report a case of multifocal chorioretinitis with serous macular detachment in diffuse unilateral subacute neuroretinitis (DUSN) and its diagnostic dilemma. DESIGN: Case report. METHOD: A 43-year-old woman presented with painless diminution of vision of right eye for 1 week. Dilated fundoscopy showed mild disc edema, multiple deep grey-white retinitis lesions in posterior pole with mild vitritis. Optical coherence tomography revealed serous macular detachment. Fundus fluorescein angiography was suggestive of active retinitis. Comprehensive workup was unremarkable. Six days later, a live subretinal worm was noticed in inferotemporal retina. A diagnosis of DUSN was made and treated with laser photocoagulation followed by antihelminthic therapy. One month later, patients vision improved and chorioretinal lesions also healed. CONCLUSION: This report presents a unique scenario of serous macular detachment in DUSN in addition to commonly seen multifocal deep retinitis lesions. Prompt treatment with laser, antihelminthic agent can prevent irreversible vision loss.

Uveomeningeal syndrome in a healthy, young male: an unusual presentation of West Nile virus.

West Nile virus (WNV) is an RNA flavivirus transmitted through a mosquito vector. In 2018 Nebraska reported 242 cases, the highest incidence of WNV since 2003. This included 119 neuroinvasive cases (49%) and 11 deaths (4.5%) (DHHS 2018). Clinical presentation ranges from uncomplicated symptoms including fever, headache, and myalgias to neuroinvasive disease characterized by meningoencephalitis, flaccid paralysis, and other neurologic manifestations. Neuroinvasive WNV usually occurs in elderly and immunocompromised individuals, and ocular involvement is often not detected until later in the disease course. We describe a case of neuroinvasive WNV presenting with uveomeningitis in a young and otherwise healthy patient.

Ultrasonographic characteristics of active ocular toxoplasmosis / Achados ultrassonográficos em toxoplasmose ocular ativa

ABSTRACT Purpose: To evaluate ophthalmic ultrasonographic findings associated with active ocular toxoplasmosis. Methods: Forty-seven eyes with active ocular toxoplasmosis in 47 patients were subjected to ocular ultrasonography using the transpalpebral technique (10-MHz transducer) and fundus photography. Patient medical records were retrospectively reviewed. Results: Ocular ultrasonography revealed vitritis, posterior vitreous detachment, retinal wall thickening, and non-rhegmatogenous retinal detachment in 47 (100%), 36 [76.6%; partial in 12 (25.5%) and total in 23 (48.9%)], 12 (25.5%), and 5 eyes (10.6%). Thirty-five of the 36 eyes with posterior vitreous detachment (97.2%) exhibited posterior hyaloid thickening; moreover, adhesion to the exudative lesion and vitreoschisis were observed in 4 (11.1%) and 12 eyes (25.5%), respectively. Ultrasonography detected the location of the exudative focus in 12 eyes (25.5%). Conclusion: Ultrasonography is helpful for detecting important intraocular findings of acute ocular toxoplasmosis that can be hindered by medial opacity or posterior synechiae.

RESUMO Objetivo: Avaliar os achados da ultrassonografia na toxoplasmose ocular ativa. Métodos: Quarenta e sete olhos com toxoplasmose ocular ativa em 47 pacientes foram submetidos à ultrassonografia ocular pela técnica transpalpebral (transdutor de 10 MHz) e fundo de olho. Os prontuários médicos foram revistos retrospectivamente. Resultados: A ultrassonografia ocular revelou vitreíte, descolamento vítreo posterior, espessamento da parede da retina e descolamento de retina não regmatogênico em 47 (100%), 36 [76,6%; parcial em 12 (25,5%) e total em 23 (48,9%)], 12 (25,5%) e 5 olhos (10,6%). Trinta e cinco dos 36 olhos com descolamento vítreo posterior (97,2%) exibiram espessamento hialoide posterior; além disso, a adesão à lesão exsudativa e vitreosquise foi observada em 4 (11,1%) e 12 (25,5%), respectivamente. A ultrassonografia detectou a localização do foco exsudativo em 12 olhos (25,5%). Conclusão: A ultrassonografia é útil na detecção de importantes achados intra-oculares de toxoplasmose ocular aguda que podem ser prejudicados pela opacidade medial ou sinéquia posterior.

Ultrasonographic characteristics of active ocular toxoplasmosis.

PURPOSE: To evaluate ophthalmic ultrasonographic findings associated with active ocular toxoplasmosis. METHODS: Forty-seven eyes with active ocular toxoplasmosis in 47 patients were subjected to ocular ultrasonography using the transpalpebral technique (10-MHz transducer) and fundus photography. Patient medical records were retrospectively reviewed. RESULTS: Ocular ultrasonography revealed vitritis, posterior vitreous detachment, retinal wall thickening, and non-rhegmatogenous retinal detachment in 47 (100%), 36 [76.6%; partial in 12 (25.5%) and total in 23 (48.9%)], 12 (25.5%), and 5 eyes (10.6%). Thirty-five of the 36 eyes with posterior vitreous detachment (97.2%) exhibited posterior hyaloid thickening; moreover, adhesion to the exudative lesion and vitreoschisis were observed in 4 (11.1%) and 12 eyes (25.5%), respectively. Ultrasonography detected the location of the exudative focus in 12 eyes (25.5%). CONCLUSION: Ultrasonography is helpful for detecting important intraocular findings of acute ocular toxoplasmosis that can be hindered by medial opacity or posterior synechiae.

CLINICAL PATHOLOGIC CORRELATION OF SARCOIDOSIS-ASSOCIATED MULTIFOCAL CHORIORETINITIS IN AN ELDERLY PATIENT.

BACKGROUND/PURPOSE: To describe a case of histopathologic clinical correlation of sarcoidosis-related multifocal chorioretinitis in an elderly individual. METHODS: This is a single case report and review of the pertinent literature. A 72-year-old white woman with a history of asthma was referred with bilateral asymmetric low-grade chronic panuveitis with advancing peripheral punched-out chorioretinal lesions despite systemic corticosteroid treatment. RESULTS: Coalescent chorioretinal punched-out lesions on fundus examination in the more severely affected right eye did not respond to corticosteroid treatment, and the refractory nature resulted in a suspicion of intraocular lymphoma despite a previous inconclusive diagnostic vitrectomy result. A transvitreal chorioretinal biopsy in the vitrectomized eye demonstrated a chorioretinal noncaseating granuloma centered on the choriocapillaris and invading Bruch membrane and the outer retina. This case illustrates an example of the second peak of incidence in sarcoidosis that can occur in ages >50 in Caucasians and Japanese patients, as well as a tendency to develop chronic disease in Caucasians, and to have extrapulmonary sarcoidosis in the elderly. CONCLUSION: This case emphasizes the differences in presentation of ocular sarcoid in older Caucasian women as well as the histopathologic findings in multifocal peripheral chorioretinitis associated with sarcoidosis.

Regional Differences in the Clinical Manifestation of Ocular Toxoplasmosis Between the Center and Northeast of Argentina.

Purpose: To compare the clinical characteristics of patients with active episodes of ocular toxoplasmosis from three provinces, Misiones, Santa Fe, and Buenos Aires, Argentina. Methods: Patients with a diagnosis of ocular toxoplasmosis from three databases of four tertiary referral uveitis centers were reviewed. Collected data included presentation of the retinochoroiditis, location of the active lesions, associated inflammatory ocular signs and complications. Results: Three hundred thirty-four patients were included in this study. Bilateral involvement of the ocular disease occurred in 26 patients in Misiones (35.14%), 21 patients (12.8%) in Santa Fe, and 9 patients in Buenos Aires (9.4%) (p < 0.001). Extensive retinitis was observed in 49 patients (66.2%) in Misiones, 39 patients (23.8%) in Santa Fe, and 12 patients (12.5%) in Buenos Aires (p < 0.001). Conclusion: The results indicate that there are differences in the clinical characteristics of ocular toxoplasmosis in patients from Misiones, Santa Fe, and Buenos Aires.

Congenital Cytomegalovirus Pneumonitis and Treatment Response Evaluation Using Viral Load during Ganciclovir Therapy: a Case Report.

Cytomegalovirus (CMV) is the most common cause of congenital infection. Pneumonitis is considered to be a rare manifestation although congenital CMV infection presents with various non-specific findings. Ganciclovir and valganciclovir are beneficial for improving neurodevelopmental sequelae and hearing outcomes of congenital CMV infection; however, treatment response evaluation is not well reported. We report a female case of congenital CMV infection presenting with pneumonitis, meningoencephalitis, and chorioretinitis. She was treated with intravenous ganciclovir for 6 weeks, and clinical features improved. Measurement of the CMV genome load by real-time polymerase chain reaction assay was performed during treatment. After the administration of ganciclovir, the CMV genome was not detected in the blood and levels decreased gradually in the urine. Physicians should consider the possibility of congenital CMV infection in neonates who present with respiratory distress. Furthermore, measurement of the CMV genome load in blood and urine may be useful for evaluating treatment response.

Contribution of dual fluorescein and indocyanine green angiography to the appraisal of posterior involvement in birdshot retinochoroiditis and Vogt-Koyanagi-Harada disease.

PURPOSE: To assess the levels of retinal and choroidal involvement in initial-onset birdshot retinochoroiditis (BRC) and Vogt-Koyanagi-Harada (VKH) disease, two stromal choroiditis entities. METHODS: This retrospective study included patients diagnosed with BRC and VKH, seen during initial-onset disease at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified, using an established dual fluorescein angiography (FA) and indocyanine green angiography (ICGA) scoring system for uveitis, and the FA/ICGA score ratios were compared between diseases. RESULTS: Among 1793 patients with uveitis seen from 1995 to 2015, 7 newly diagnosed BRC patients and 4 patients with newly diagnosed VKH disease had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA angiographic scores of 16.91 ± 3.42 and 4.06 ± 1.87; mean ICGA angiographic scores of 21.34 ± 3.49 and 25.75 ± 3.88; and mean FA/ICGA ratios of 0.79 ± 0.21 and 0.16 ± 0.09, respectively. CONCLUSION: This study showed the differential involvements of the retina and choroid in BRC and VKH. The choroid was preponderantly involved in both diseases; thus, ICGA is essential for disease assessment and follow-up. However, these diseases also differed substantially. The origin of inflammation was primarily in the choroid in VKH and in both the choroid and retina in BRC. We recommend dual FA and ICGA for evaluating posterior uveitis, when choroiditis is suspected.

Non-invasive method of monitoring retinal vasculitis in patients with birdshot chorioretinopathy using optical coherence tomography.

BACKGROUND/AIMS: To investigate the utility of using montaged optical coherence tomography (OCT) thickness maps to monitor perivascular thickness as a marker of vasculitic activity in patients with large-vessel retinal vasculitis. METHODS: This is a retrospective cohort study of 22 eyes of 11 patients with a history of retinal vasculitis associated with birdshot chorioretinopathy (BCR). Patients had serial spectral domain 6×6 mm cube OCT scans centred on the fovea, optic nerve and proximal branches of the superior and inferior retinal vessels. OCT thickness change maps for each respective region were analysed. Changes in perivascular thickness were confirmed by assessing vasculitic activity on fluorescein angiography (FA), when clinically indicated. RESULTS: In three patients, montaged OCT scans were acquired at diagnosis and serially through initial treatment. In all three patients, montaged OCT demonstrated reduced perivascular thickening with oral prednisone treatment, which was confirmed by FA showing reduced vascular leakage in both eyes. Eight patients had serial montaged OCT scans after diagnosis and initial treatment of BCR. Four of these patients showed fluctuations in perivascular thickness during flares and treatment that were confirmed by either increased or decreased vascular leakage on FA. The other four patients remained quiet on their immunosuppressive treatment regimens, and no changes in perivascular thickness were detected. CONCLUSIONS: Evaluating large-vessel perivascular thickness on OCT scans may be a useful method for non-invasively monitoring posterior pole large-vessel retinal vasculitis.

Safety and Efficacy of AAV5 Vectors Expressing Human or Canine CNGB3 in CNGB3-Mutant Dogs.

Achromatopsia is an inherited retinal disorder of cone photoreceptors characterized by markedly reduced visual acuity, extreme light sensitivity, and absence of color discrimination. Approximately 50% of cases are caused by mutations in the cone photoreceptor-specific cyclic nucleotide gated channel beta subunit (CNGB3) gene. Studies in CNGB3-mutant dogs showed that subretinal injection of an AAV vector expressing human CNGB3, which has 76% amino acid identity with canine CNGB3, driven by a 2.1 kb human red cone opsin promoter (PR2.1) and packaged in AAV5 capsids (AAV5-PR2.1-hCNGB3) rescued cone photoreceptor function, but at high doses was associated with an inflammatory response (focal chorioretinitis) consistent with immune-mediated toxicity. AAV vectors containing the PR2.1 promoter packaged in AAV5 capsids and expressing either the native canine CNGB3 (AAV5-PR2.1-cCNGB3) or the human CNGB3 (AAV5-PR2.1-hCNGB3) were evaluated at different dose levels in CNGB3-mutant dogs. The vector expressing canine CNGB3 achieved somewhat better rescue of cone function but unexpectedly was associated with a greater degree of retinal toxicity than the vector expressing human CNGB3. Very low-level T-cell immune responses to some AAV or CNGB3 peptides were observed in animals that received the higher vector dose. There was a more than twofold increase in serum neutralizing antibodies to AAV in one of three animals in the low-dose group and in two of three animals in the high-dose group. No serum anti-hCNGB3 antibodies were detected in any animal. The results of this study do not support the hypothesis that the focal chorioretinitis seen with high doses of AAV5-PR2.1-hCNGB3 in the initial studies was due to an immune response to human CNGB3.

Zika virus infects cells lining the blood-retinal barrier and causes chorioretinal atrophy in mouse eyes.

Zika virus (ZIKV) is an important pathogen that causes not only neurologic, but also ocular, abnormalities. Thus, it is imperative that models to study ZIKV pathogenesis in the eye are developed to identify potential targets for interventions. Here, we studied ZIKV interactions with human retinal cells and evaluated ZIKV's pathobiology in mouse eyes. We showed that cells lining the blood-retinal barrier (BRB), the retinal endothelium, and retinal pigment epithelium (RPE) were highly permissive and susceptible to ZIKV-induced cell death. Direct inoculation of ZIKV in eyes of adult C57BL/6 and IFN-stimulated gene 15 (ISG15) KO mice caused chorioretinal atrophy with RPE mottling, a common ocular manifestation of congenital ZIKV infection in humans. This response was associated with induced expression of multiple inflammatory and antiviral (IFNs) response genes in the infected mouse retina. Interestingly, ISG15 KO eyes exhibited severe chorioretinitis, which coincided with increased retinal cell death and higher ZIKV replication. Collectively, our study provides the first evidence to our knowledge that ZIKV causes retinal lesions and infects the cells lining the BRB and that ISG15 plays a role in retinal innate defense against ZIKV infection. Our mouse model can be used to study mechanisms underlying ZIKV-induced chorioretinitis and to gauge ocular antiviral therapies.

CLINICOPATHOLOGICAL CORRELATION IN A PATIENT WITH PREVIOUSLY TREATED BIRDSHOT CHORIORETINOPATHY.

PURPOSE: Birdshot chorioretinopathy (BCR) is a bilateral, chronic uveitis primarily involving the posterior segment that often results in progressive vision loss. Histopathology on eyes with BCR has been limited, but we had the rare opportunity to study the eyes of a donor with BCR. We sought to compare immunolabeling in the eyes of this donor who was treated with immunosuppression for over 30 years to age-matched controls. METHODS: From each eye, a macular punch and superotemporal regions were used for cryostat sectioning, and immunohistochemistry was performed on the sections using antibodies directed against CD45, intercellular adhesion molecule-1, IBA1, and GFAP. The vasculature-binding lectin, Ulex europaeus agglutinin-I (UEA-I), was also used to perform lectin histochemistry. RESULTS: At death, her visual acuity was 20/25 right eye, 20/250 left eye with extensive chorioretinal atrophy, vascular attenuation, and disk pallor. Compared with controls, the BCR donor had extensive degeneration of the outer nuclear layer and retinal pigment epithelium as well as choroidal thinning with inner retinal preservation. Loss of UEA-I+ choroidal endothelial cells was extensive, and atypical intercellular adhesion molecule-1 labeling and IBA+ microglia/macrophages were present along with widespread GFAP labeling throughout the retina. CONCLUSION: The BCR may cause progressive chorioretinal and optic atrophy with long-standing increased leukocyte abundance throughout the retina and microglial activation especially at the retina-choroid interface.

[Ocular syphilis: 9 cases]. / Syphilis oculaire : à propos de 9 cas.

Syphilis is a disease with ocular involvement may be opening and cover all ocular structures. The objective of the study was to retrospectively identify and describe all cases of ocular syphilis diagnosed in the ophthalmology department of Besançon University Hospital from March 2004 to April 2014. Between March 2004 and April 2014, we diagnosed and monitored 9 male patients (13 eyes) with ocular syphilis (1 interstitial keratitis, chorioretinitis associated with a 1 hyalite, 3 panuvéites, 1 chorioretinitis after placoid, 3 bilateral papillary edema). Patients had a mean age of 52.6 years (32-77 years) at diagnosis and the inaugural symptoms were in 100% of cases decreased visual acuity. The follow-up period ranged from 1 month to 5 years. The curative treatment included intravenous antibiotics penicillin G for 3 weeks. All patients had an improvement in their AV (AV initial range "counting fingers" and 0.6 versus AV final between 0.5 and 1 in decimal scale). Syphilis is a disease currently on the rise. It must be systematically sought before any inflammatory disease of the eye or papilledema.

Incidence and Risk Factors of Ocular Infection Caused by Staphylococcus aureus Bacteremia.

Staphylococcus aureusbacteremia (SAB) often leads to ocular infections, including endophthalmitis and chorioretinitis. However, the incidence, risk factors, and outcomes of ocular infections complicated by SAB are largely unknown. We retrospectively analyzed the incidence and risk factors of ocular involvement in a prospective cohort of patients with SAB at a tertiary-care hospital. Ophthalmologists reviewed the fundoscopic findings and classified the ocular infections as endophthalmitis or chorioretinitis. During the 5-year study period, 1,109 patients had SAB, and data for 612 (55%) who underwent ophthalmic examinations within 14 days after SAB onset were analyzed. Of those 612 patients, 56 (9% [95% confidence interval [CI], 7 to 12%]) had ocular involvement, including 15 (2.5%) with endophthalmitis and 41 (6.7%) with chorioretinitis. In a multivariate analysis, infective endocarditis (adjusted odds ratio [aOR], 5.74 [95% CI, 2.25 to 14.64]) and metastatic infection (aOR, 2.38 [95% CI, 1.29 to 4.39]) were independent risk factors for ocular involvement. Of the 47 patients with ocular involvement who could communicate, only 17 (36%) had visual disturbances. Two-thirds of the patients with endophthalmitis (10/15 patients) were treated with intravitreal antibiotics combined with parenteral antibiotics, whereas all of the patients with chorioretinitis were treated only with systemic antibiotics. No patients became blind. Among 42 patients for whom follow-up assessments were available, the ocular lesions improved in 29 (69%) but remained the same in the others. Ocular involvement was independently associated with death within 30 days after SAB onset. Ocular involvement is not uncommon among patients with SAB. Routine ophthalmic examinations should be considered for patients with infective endocarditis or metastatic infections caused by SAB.

Visual function and macular architecture in patients with inactive zone 2 and 3 toxoplasmic retinochoroiditis.

PURPOSE: To evaluate the visual function and architecture of the central and peripapillary retina in patients with inactive toxoplasmic retinochoroiditis outside the macular and peripapillary regions (zones 2 and 3). METHODS: Cross-sectional study of 20 eyes (18 patients) with zone 2 and 3 toxoplasmic scars and visual acuity ≥20/25. Patients underwent Humphrey 10-2 perimetry, contrast sensitivity (Mars test), and color vision testing (L'Anthony desaturated D-15). The retinal nerve fiber layer (RNFL) and macular thicknesses were determined by optical coherence tomography. RESULTS: The patients' mean age was 27.4 ± 10.3 years, and the mean duration of remission was 6.15 ± 5.19 months. Abnormal contrast sensitivity and color vision were observed in three (15.0%) and four eyes (20.0%), respectively. Mean deviation (MD) and pattern standard deviation (PSD) fell outside the 95% normal confidence limits of the perimeter's database in 14 (70.0%) and seven eyes (35.0%), respectively. Foveal and mean RNFL thicknesses were within the normal limits in all eyes. Eyes with zone 2 retinochoroiditis had lower foveal sensitivity than eyes with zone 3 lesions (p=0.041). Eyes with a longer duration of remission had a higher MD (r=0.575; p=0.013) and a lower PSD (r=-0.593; p=0.010). CONCLUSIONS: Despite normal central and peripapillary retinal architecture, eyes with inactive zone 2 and 3 toxoplasmic retinochoroiditis can present with abnormal color, contrast, and macular perimetric sensitivity. Zone 2 retinochoroiditis was associated with lower foveal sensitivity, and a longer duration of retinochoroiditis remission was associated with better perimetric parameters (MD and PSD).