Macular and peripapillary spectral domain optical coherence tomography changes in sickle cell retinopathy.

PURPOSE: To assess peripapillary retinal nerve fiber layer, macular ganglion cell complex, and total macular thicknesses using spectral domain optical coherence tomography on sickle cell disease patients with and without sickle retinopathy. METHOD: Nineteen eyes of 11 patients with hemoglobin sickle cell disease, 65 eyes of 36 patients with hemoglobin SS disease, and 48 eyes of 24 healthy subjects underwent spectral domain optical coherence tomography scanning (RTVue). Eyes of patients with sickle cell disease were classified into 3 groups according to posterior segment changes: no retinopathy (n = 64), nonproliferative retinopathy (n = 12), and proliferative retinopathy (n = 8). RESULTS: The central fovea in eyes with proliferative retinopathy was thickened compared with control group, sickle cell disease without retinopathy, and nonproliferative retinopathy (P = 0.004); a difference between proliferative retinopathy and sickle cell disease without retinopathy groups was still present after age adjustment (P = 0.014). Eyes with proliferative changes showed higher ganglion cell complex focal loss of volume compared with control group (P = 0.002), even after age adjustment (P = 0.004). Thinning of the nasal retinal nerve fiber layer quadrant was observed in eyes with proliferative retinopathy (P < 0.001); however, no retinal nerve fiber layer thickness difference was observed after age correction (P > 0.05). CONCLUSION: Peripheral changes secondary to proliferative sickle retinopathy were associated with thinning of macular inner retinal layers and thickening of central fovea.

Uveitis in human immunodeficiency virus-infected persons with CD4+ T-lymphocyte count over 200 cells/mL.

BACKGROUND: Introduction of highly active antiretroviral therapy has altered the course of disease for persons infected with human immunodeficiency virus by elevating CD4+ T-lymphocyte levels. Changes in the spectrum of systemic diseases encountered in human immunodeficiency virus-positive individuals are reported in the general medical literature. DESIGN: Retrospective case series. PARTICIPANTS: Sixty-one individuals infected with human immunodeficiency virus, who presented with uveitis when the peripheral CD4+ T-lymphocyte count was over 200 cells/µL. METHODS: Standardized data collection at seven tertiary-referral inflammatory eye disease clinics. MAIN OUTCOME MEASURES: Standardization of Uveitis Nomenclature anatomic classification and descriptors, cause of uveitis, and visual acuity RESULTS: Peripheral CD4+ T cell counts varied between 207 and 1777 (median = 421) cells/µL at the time of diagnosis of uveitis. Uveitis was classified anatomically as anterior (47.5%), intermediate (6.6%), anterior/intermediate (16.4%), posterior (14.8%) and pan (14.8%). Specific causes of uveitis included infections (34.4%), with syphilis responsible for 16.4% of all cases, and defined immunological disorders (27.0%); no cause for the inflammation was identified in 34.4% of persons. Visual acuity was better than 6/15 in 66.7% and 6/60 or worse in 11.8% of 93 eyes at presentation, and better than 6/15 in 82.4% and 6/60 or worse in 8.8% of 34 eyes at 1 year of follow-up. CONCLUSIONS: Both infectious and non-infectious forms of uveitis occur in individuals who are infected with human immunodeficiency virus and have preserved or restored peripheral CD4+ T cell levels. Individuals who are human immunodeficiency virus-positive and present with uveitis should be evaluated in the same way all patients with uveitis are assessed.

Influence of gender on clinical presentation, management practices and outcomes of ocular syphilis.

Ocular syphilis is a re-emerging inflammatory eye disease with a clear gender imbalance, disproportionately affecting men. We investigated the impact of gender on the presentation, management practices and clinical outcomes of this condition. Data generated from a study of patients consecutively diagnosed with ocular syphilis who attended a subspecialist uveitis service at one of four hospitals in Brazil over a 30-month period were disaggregated for analysis by gender. Two-hundred and fourteen eyes (161 men and 53 women) of 127 patients (96 men and 31 women) were included. Posterior uveitis was the most common presentation in both men and women (80.1% vs. 66.7%, p > 0.05), but men were significantly more likely to have vitritis as a feature of their disease (49.4% versus 28.8%, p = 0.019). Three eyes of women had nodular anterior scleritis (p = 0.015). Men were more likely to undergo a lumbar puncture to assess for neurosyphilis (71.9% vs. 51.6%, p = 0.048), but men and women undergoing a lumbar puncture were equally likely to have a cerebrospinal fluid abnormality (36.2% vs. 25.0%, p = 0.393). All patients were treated with aqueous penicillin G or ceftriaxone, and there was a trend towards more men receiving adjunctive systemic corticosteroid treatment as part of their management (65.2% vs. 46.7%, p = 0.071). There were no significant differences in the age of presentation, bilaterality of disease, anatomical classification of uveitis, initial or final visual acuity, and rates of ocular complications between men and women. Our findings indicate that ocular syphilis has comparable outcomes in men and women, but that there are differences in the type of ocular inflammation and management practices between the genders.

Vision-related quality of life in patients treated for ocular syphilis.

Multiple studies have showed negative impact of non-infectious uveitis on quality of life (QoL). Less is understood regarding life experiences in patients with infectious uveitis. We investigated vision-related QoL in individuals who had recovered from ocular syphilis. 32 adults treated for ocular syphilis at a uveitis service in Brazil completed the 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25), and a comprehensive ophthalmic examination was performed. Medical records were reviewed to confirm resolution of ocular inflammation for 3 months pre-enrolment, and collect clinical data. The NEI VFQ-25 composite score was low overall (75.5 ± 19.8, mean ± standard deviation), and subscale scores varied from relative lows of 59.1 ± 39.6 (driving) and 60.9 ± 24.5 (mental health), to relative highs of 84.8 ± 21.8 (ocular) and 89.1 ± 21.0 (color vision). Adults aged over 40 years and those with a final visual acuity of 20/50 or worse had significantly lower mean composite and subscale scores. Other clinical characteristics-including gender, HIV co-infection, and type of uveitis-did not significantly influence scores. Our findings, taken in context with previous observations that prompt recognition achieves better vision outcomes, suggest early treatment may improve QoL after recovery from ocular syphilis.

Relationship between retinal nerve fiber layer and visual field function in human immunodeficiency virus-infected patients without retinitis.

PURPOSE: To evaluate the spatial association between visual field (VF) sensitivity loss and retinal nerve fiber layer (RNFL) thinning in patients infected by the human immunodeficiency virus. METHODS: Fifty-one eyes of 51 human immunodeficiency virus-infected patients and 22 eyes of 22 control subjects were enrolled. Patients were evaluated using the Fast RNFL scan strategy on Stratus OCT and the 24-2 full-threshold program on the Humphrey Matrix frequency doubling technology (FDT) perimeter. Associations between RNFL thickness and VF sensitivity were evaluated globally, in 12 clock-hour optical coherence tomography sectors and in 21 VF zones; linear and quadratic regression models were used in the statistical analysis. RESULTS: The linear and quadratic regression associations between the FDT Matrix pattern standard deviation and the average RNFL thickness in human immunodeficiency virus-infected patients were r2 = 0.185 and r2 = 0.218 (P < 0.05), respectively. The correlation between the FDT Matrix mean deviation and the average RNFL thickness was not significant (P > 0.05). Stronger associations were found when regional RNFL thinning was compared with locally measured FDT Matrix pattern deviation, especially between nasal RNFL measurements and temporal VF zones, and between superior RNFL measurements and inferior VF zones. CONCLUSION: Retinal nerve fiber layer thinning was related to VF sensitivity loss in human immunodeficiency virus-infected patients and regional associations between optical coherence tomography and FDT Matrix sectors were stronger than the associations between global measurements.

Optical Coherence Tomography Findings in Ocular Syphilis Involving the Posterior Segment of the Eye.

PURPOSE: To analyse posterior segment optical coherence tomography (OCT) findings in ocular syphilis. METHODS: Medical records of 54 patients presenting consecutively with syphilitic uveitis were reviewed. Vitreous, retina and choroid (one eye/patient) were assessed by spectral-domain OCT on presentation (54 eyes) and after treatment (31 eyes). Improvement in signs and associations between presenting signs and final best corrected visual acuity (BCVA) were determined by McNemar's and Fisher's exact tests. RESULTS: Early inner retinal OCT findings included hyperreflective dots (n = 49, 91%), tongue-like projections (n = 44, 81%) and large rounded spots (n = 41, 76%). Common outer retinal findings included thickening, irregularity, elevations and/or detachment of retinal pigment epithelium (n = 46, 85%), and disruption or loss of the ellipsoid zone (n = 33, 61%). Most outer retinal changes resolved with treatment (p < .05), and common presenting signs were not associated with poor final BCVA (p > .05). CONCLUSION: OCT findings have diagnostic value in ocular syphilis, but do not predict prognosis.

PRESUMED TUBERCULOUS MULTIFOCAL RETINITIS IN PATIENTS UNDER TREATMENT WITH BIOLOGIC AGENTS.

PURPOSE: To report unique retinal fundus lesions and treatment outcomes of intraocular tuberculosis in patients under anti-tumor necrosis factor treatment. METHODS: Retrospective review of two patients with laboratorial evidence of tuberculosis who had bilateral ocular signs and symptoms not attributable to other diseases. Multimodal imaging was analyzed at the time of presentation and after the treatment initiation. The study patients underwent standard treatment for tuberculosis. RESULTS: Clinical and laboratory findings were consistent with the diagnosis of presumed tuberculosis. Color fundus photograph revealed the presence of multifocal yellowish retinal spots in the study eyes. On fluorescein angiography, the retinal lesions seen on color fundus photograph showed early hypofluorescence with progressive staining of its edges. Occlusive vasculitis with peripheral nonperfusion was also observed in both cases. Spectral domain optical coherence tomography demonstrated increased reflectivity and thickness on the topography of retinitis lesions. After specific antibiotic treatment for tuberculosis, there was complete disappearance of the retinal lesions in all study eyes. CONCLUSION: We report two unique cases of bilateral presumed intraocular tuberculosis presenting as multifocal retinitis in patients under biologic agent treatment. Anti-tumor necrosis factor agents may be related to unusual fundus manifestations of tuberculosis.

Current ophthalmology practice patterns for syphilitic uveitis.

BACKGROUND: Syphilitic uveitis is re-emerging alongside the systemic infection. In July 2017, an international group of uveitis-specialised ophthalmologists formed the International Ocular Syphilis Study Group to define current practice patterns. METHODS: 103 Study Group members based in 35 countries completed a 25-item questionnaire focused on case load, clinical presentations, use and interpretation of investigations, treatment and clinical indicators of poor prognosis. RESULTS: Members managed a mean of 6.1 patients with syphilitic uveitis in clinics that averaged 707 annual cases of uveitis (0.9%); 53.2% reported increasing numbers over the past decade. Patients presented to more members (40.2%) during secondary syphilis. Uveitis was usually posterior (60.8%) or pan (22.5%); complications included optic neuropathy, macular oedema and posterior synechiae. All members diagnosed syphilitic uveitis using serological tests (simultaneous or sequential testing algorithms), and 97.0% routinely checked for HIV co-infection. Cerebrospinal fluid (CSF) analysis was ordered by 90.2% of members, and 92.7% took uveitis plus Venereal Disease Research Laboratory test (VDRL) or fluorescent treponemal antibody absorption test (FTA-ABS) to indicate neurosyphilis. Patients were commonly co-managed with infectious disease physicians, and treated with penicillin for at least 10-14 days, plus corticosteroid. Features predicting poor outcome included optic neuropathy (86.3%) and initial misdiagnosis (63.7%). Reasons for delayed diagnosis were often practitioner-related. 82.5% of members tested every patient they managed with uveitis for syphilis. CONCLUSION: This comprehensive report by an international group of uveitis-specialised ophthalmologists provides a current approach for the management of syphilitic uveitis.

Visual impairment evaluation in 119 children with congenital Zika syndrome.

PURPOSE: To assess visual impairment in a large sample of infants with congenital Zika syndrome (CZS) and to compare with a control group using the same assessment protocol. METHODS: The study group was composed of infants with confirmed diagnosis of CZS. Controls were healthy infants matched for age, sex, and socioeconomic status. All infants underwent comprehensive ophthalmologic evaluation including visual acuity, visual function assessment, and visual developmental milestones. RESULTS: The CZS group included 119 infants; the control group, 85 infants. At examination, the mean age of the CZS group was 8.5 ± 1.2 months (range, 6-13 months); of the controls, 8.4 ± 1.8 months (range, 5-12 months; P = 0.598). Binocular Teller Acuity Card (TAC) testing was abnormal in 107 CZS infants and in 4 controls (89.9% versus 5% [P < 0.001]). In the study group, abnormal monocular TAC results were more frequent in eyes with funduscopic alterations (P = 0.008); however, 104 of 123 structurally normal eyes (84.6%) also presented abnormal TAC results. Binocular contrast sensitivity was reduced in 87 of 107 CZS infants and in 8 of 80 controls (81.3% versus 10% [P < 0.001]). The visual development milestones were less achieved by infants with CZS compared to controls (P < 0.001). CONCLUSIONS: Infants with CZS present with severe visual impairment. A protocol for assessment of the ocular findings, visual acuity, and visual developmental milestones tested against age-matched controls is suggested.

Author Correction: Clinical Manifestations and Ophthalmic Outcomes of Ocular Syphilis at a Time of Re-Emergence of the Systemic Infection.

A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has not been fixed in the paper.

Clinical Manifestations and Ophthalmic Outcomes of Ocular Syphilis at a Time of Re-Emergence of the Systemic Infection.

Recent reports from different world regions suggest ocular syphilis is re-emerging, in parallel with an increasing incidence of the systemic infection globally. We conducted a large observational study of 127 persons consecutively treated for ocular syphilis at public medical centers in Brazil over a 2.5-year period ending July 2015. Of 104 individuals serologically tested for human immunodeficiency virus (HIV), 34.6% were positive. Ophthalmological evaluations included measurement of Snellen visual acuity and intraocular pressure, and assessment of inflammation by slit lamp examination and dilated posterior eye examination. Involvements in 214 eyes were anterior (6.1%), intermediate (8.4%), posterior (76.2%) and pan- (8.4%) uveitis, and scleritis (0.9%). Multiple anterior and posterior eye complications were observed, including cataract in the anterior eye (incidence rate, 0.18/eye-year) and epiretinal membrane in the posterior eye (incidence rate, 0.09/eye-year); incidence rates of reduction in best-corrected visual acuity to ≤20/50 and ≤20/200 were 0.10 and 0.06/eye-year, respectively. Rates of complications and visual acuity loss did not differ significantly between HIV- positive and negative individuals. In an era of re-emergence, syphilis has ocular complications that may compromise vision, despite treatment with appropriate anti-microbial drugs.

Ocular Involvement Following Postnatally Acquired Toxoplasma gondii Infection in Southern Brazil: A 28-Year Experience.

PURPOSE: To determine the incidence of, and risk factors for, ocular involvement among people known to have postnatally acquired Toxoplasma gondii infection in a region of southern Brazil where there is a high prevalence of endemic disease. DESIGN: Retrospective longitudinal cohort study. METHODS: Records of 302 patients with serologic evidence of recent T gondii infection (a positive anti-T gondii IgM antibody test) from Erechim, Rio Grande do Sul state, Brazil (1974-2002) were analyzed. The incidence of ocular involvement was calculated in terms of person-years (PY) of follow-up. Risk factors for ocular involvement were analyzed using log-rank and Fisher exact tests. RESULTS: At initial ocular examination (baseline), 30 patients (9.9%) had intraocular inflammation only (anterior chamber cells and flare, vitreous inflammatory reactions, retinal whitening), without clinically apparent necrotizing retinochoroiditis. At baseline, men were more likely to have ocular involvement (P = .043) and antiparasitic treatment was associated with less ocular involvement (P = .015). Follow-up examinations were performed on 255 patients (median follow-up, 13.7 months [range 0.4-261.9 months]). Among those without ocular involvement at baseline, the incidence of necrotizing retinochoroiditis was 6.4/100 PY. Patients >40 years of age at first IgM test had a greater risk of incident necrotizing retinochoroiditis (hazard ratio = 4.47, 95% CI = 1.67-11.93, P = .003) than younger patients. The incidence of recurrent necrotizing retinochoroiditis was 10.5/100 PY. CONCLUSION: Isolated intraocular inflammatory reactions can be an initial manifestation of T gondii infection, with necrotizing retinochoroiditis occurring months or years later. Male sex and older age are risk factors for toxoplasmic retinochoroiditis. Antitoxoplasmic treatment may protect against early ocular involvement.

Outcomes of congenital cataract surgery: intraoperative intracameral triamcinolone injection versus postoperative oral prednisolone.

PURPOSE: To compare the outcomes of congenital cataract surgery using intraoperative intracameral triamcinolone versus postoperative oral prednisolone to modulate ocular inflammation. SETTING: Department of Congenital Cataract, Altino Ventura Foundation, Recife, Brazil. DESIGN: Randomized clinical trial. METHODS: Children younger than 2 years were randomly divided into 2 groups. The study group received an intraoperative intracameral injection of 1.2 mg/0.03 mL of triamcinolone acetonide. The control group (29 eyes) received 1 mg/kg per day of prednisolone syrup for 15 days postoperatively, which was then tapered over the following 2 weeks. Intraocular pressure (IOP), central corneal thickness (CCT), cell deposits on the intraocular lens (IOL), posterior synechiae, visual axis obscuration, additional surgical procedures, and IOL centration were assessed 12 months postoperatively. RESULTS: The mean patient age at surgery was 10.45 months±6.22 (SD) in the study group (31 eyes) and 10.0±6.15 months in the control group (29 eyes) (P=.779). In both groups, the mean IOP and CCT did not change significantly postoperatively (study group P=.922 and P=.149, respectively; control group P=.483 and P=.416, respectively). The groups had similar incidences of cell deposits (P=.517) and posterior synechiae (P=.247). No eye developed visual axis obscuration or had additional surgical procedures. All eyes had a clinically centered IOL. CONCLUSION: One year postoperatively, the outcomes were similar with intraoperative intracameral triamcinolone injection and postoperative oral prednisolone for modulating inflammation after congenital cataract surgery. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Association between retinal nerve fiber layer thickness and abnormalities of vision in people with human immunodeficiency virus infection.

PURPOSE: To investigate relationships between contrast sensitivity (CS), color vision, and retinal nerve fiber layer (RNFL) among people with human immunodeficiency virus (HIV) infection; to evaluate the effect of time since diagnosis of HIV infection on RNFL thickness. DESIGN: Noninterventional cross-sectional study. METHODS: We evaluated 102 eyes of 57 HIV-infected individuals without ocular opportunistic infections. Peripapillary RNFL thickness was determined with spectral-domain optical coherence tomography in 4 quadrants. CS was measured with the Pelli-Robson technique (expressed as logCS); color vision was measured with the Lanthony desaturated 15-hue technique (expressed as color confusion index [C-index], with higher scores indicating worse color vision). Correlations between values were assessed using Spearman correlation coefficients. RESULTS: Median RNFL thickness (average of 4 quadrants) was 102.9 µm (range, 75.0-134.7 µm). Median logCS was 1.90 (range, 1.25-1.95). Median C-index was 1.58 (range, 0.96-4.07). Temporal RNFL thickness was correlated with logCS (r=0.295, P=.003) and C-index (r=-0.338, P=.0005). Time since diagnosis of HIV infection was shorter for those with thick average RNFL than for those with thin average RNFL (P=.18). CONCLUSIONS: Both worse CS and worse color vision are correlated with thinning of the temporal RNFL, with possible threshold effects. Increased prevalences of abnormal CS and abnormal color vision in this population are therefore likely attributable to neuroretinal compromise. This pattern of structural and functional losses may reflect preferential damage to small-caliber axons in the maculopapillary bundle, possibly associated with mitochondrial dysfunction, providing a potential disease mechanism for HIV-associated "neuroretinal disorder."

Fundus autofluorescence and spectral domain optical coherence tomography in recurrent serpiginous choroiditis: case report.

PURPOSE: To report fundus autofluorescence (FA) and spectral domain optical coherence tomography (SDOCT) findings in serpiginous choroiditis. DESIGN: Case report. METHODS: SDOCT and FA imaging of a 37-year-old woman with bilateral recurrent serpiginous choroiditis. RESULTS: Active new lesions disclosed hyperautofluorescence, in contrast to hypoautofluorescent scarred lesions. SDOCT showed increased reflectance of the choroid and deeper retinal layers, along with disruption of the photoreceptor inner and outer segment junction in both active and inactive lesions. CONCLUSION: Autofluorescence imaging and SDOCT are useful noninvasive methods for the evaluation of serpiginous choroiditis. Autofluorescence imaging allows identification of recurrences and retinal pigment epithelium involvement in the follow-up of this disease.

Spectral domain optical coherence tomography and angiographic findings in central serous chorioretinopathy complicated by bilateral nonrhegmatogenous retinal detachment associated with systemic corticosteroids.

PURPOSE: To report spectral domain optical coherence tomography (OCT) and angiographic findings in exudative retinal detachment complicating central serous chorioretinopathy. DESIGN: interventional case report. METHODS: 33-year old man with bilateral nonrhegmatogenous retinal detachment, misdiagnosed as uveitis, iatrogenically treated with systemic corticosteroids. RESULTS: Discontinuation of corticotherapy led to anatomic and visual improvement. Fluorescein angiography demonstrated multiple leakage points; indocyanine green angiography disclosed large hyperfluorescent patches in the choroid and OCT demonstrated retinal detachment with dense subretinal deposits. CONCLUSION: The recognition of this atypical presentation with a combination of opthalmoscopic, angiographic and OCT findings may avoid inappropriate diagnosis and treatment with corticosteroids.