Optic Disc Edema Is an Under-Recognized Feature of Birdshot Chorioretinitis.

BACKGROUND: Optic disc edema is a feature of many ophthalmic and neurologic conditions. It remains an underappreciated feature of birdshot chorioretinitis (BSCR), leading to delay in diagnosis and treatment. The purpose of our study was to identify clinical features that are concomitant with optic disc edema and suggest a diagnosis of BSCR. METHODS: Retrospective multicenter case series of 29 patients who were referred to a neuro-ophthalmologist or uveitis specialist for evaluation of disc edema and were ultimately diagnosed with BSCR. RESULTS: Fifty-four eyes of 30 patients, from the practices of 15 uveitis specialists, met the eligibility criteria. In addition to disc edema, concomitant features in all patients included vitritis, chorioretinal lesions, and retinal vasculitis. Visual recovery to 20/40 or better occurred in 26 of 29 patients. Visual acuity remained 20/100 or worse in 2 patients previously diagnosed with idiopathic intracranial hypertension, 1 patient previously diagnosed with optic neuritis, and 1 patient for whom treatment was delayed for years, leading to optic disc atrophy. CONCLUSIONS: Optic disc edema is a presenting feature in some cases of BSCR. A diagnosis of BSCR should be considered when disc edema occurs with vitritis, chorioretinal inflammation, and retinal vasculitis. Patients should be referred to a uveitis specialist for treatment.

Initial Occurrence of Bilateral Anterior Uveitis in a Patient with Juvenile Idiopathic Arthritis following Discontinuation of Topical Steroids after Bilateral Strabismus Surgery.

Post-operative inflammation is a well-known complication of intraocular surgery. This is especially true in patients with a prior history of uveitis or with a predisposition for an inflammatory response. We report the first published case of a patient who developed bilateral anterior uveitis after bilateral strabismus surgery. It was the first episode of uveitis for this patient who had a pre-existing diagnosis of juvenile idiopathic arthritis.

Novel Case of Multifocal Choroiditis following Powassan Virus Infection.

PURPOSE: To present a case study that describes novel ocular manifestations of Powassan virus encephalitis using fundus imaging. STUDY DESIGN: Case Report. RESULTS: In this case study, fundus photographs, fundus autofluorescence, and fluorescein angiography were used to highlight Powassan virus-induced multifocal choroiditis with linear streaks. CONCLUSIONS: Our case study demonstrates novel retinal findings of Powassan virus, a pattern which is commonly described in eyes infected with West Nile Virus, another type of flavivirus.

TREATMENT OF REFRACTORY TUBERCULAR SERPIGINOUS-LIKE CHOROIDITIS WITH INTRAVITREAL METHOTREXATE.

PURPOSE: To describe a case of tubercular serpiginous-like choroiditis that progressed, despite antitubercular medication, corticosteroids, and immunomodulatory treatment, which ultimately quieted after two intravitreal methotrexate injections. METHODS: Case report. RESULTS: A 35-year-old woman reported a shadow in the left eye for 2 weeks. She presented with tubercular serpiginous-like choroiditis in the right eye 2 years prior. At that time, she was started on antituberculosis therapy but was noncompliant and lost to follow-up. On re-presentation, there was a new active left-eye serpiginous lesion, with repeat positive QuantiFERON gold testing. Four antituberculosis drugs were started, followed by corticosteroids and azathioprine, with continued progression despite aggressive treatment. She was finally given 2 intravitreal methotrexate injections (400 µg/0.1 cc) 1 month apart, with final arrest of lesion extension. The uveitis remained quiet for over 24 months, and the patient was able to discontinue all systemic therapy. CONCLUSION: Intravitreal methotrexate injections halted progression of treatment-refractory tubercular serpiginous-like choroiditis.

TUBERCULOUS CHOROIDITIS IN PATIENT WITH VOGT-KOYANAGI-HARADA DISEASE.

PURPOSE: To describe a novel case of intraocular tuberculosis (TB) arising in a patient undergoing treatment for Vogt-Koyanagi-Harada disease, and to highlight the use of spectral domain optical coherence tomography for helping confirm the diagnosis and monitor treatment response. METHODS: Case report of a patient with Vogt-Koyanagi-Harada disease on prednisone, with acute clinical changes suspicious for bilateral tuberculous choroiditis. Spectral optical coherence tomography, fundus photography, and B-scan ultrasonography were all used to capture the acute lesions, and to monitor their responses after initiation of anti-TB therapy. RESULTS: New subretinal lesions arose bilaterally, as characterized by spectral domain optical coherence tomography, and appeared to regress after a first round of anti-TB therapy, thereby helping confirm the presumed diagnosis of intraocular TB. A new peripheral choroidal lesion arose shortly after temporary cessation of antimicrobial treatment, and again regressed once four-drug therapy was instituted, with no recurrent lesions thereafter. CONCLUSION: The use of multimodal imaging was instrumental in the management of a rare case of intraocular TB arising in the setting of underlying Vogt-Koyanagi-Harada disease.

MULTIMODAL IMAGING OF IGG4-RELATED PAPILLITIS AND RETINAL DISEASE.

PURPOSE: To report a case of IgG4-related ophthalmic disease, which presented with papillitis and subretinal deposits. METHODS: Observational case report with multimodal imaging. RESULTS: A 52-year-old man with a history of persistent lymphadenopathy presented with decreased vision in his left eye. Funduscopic examination demonstrated cuticular drusen in both eyes and florid edema of the left optic nerve, along with scattered circumscribed grey-yellow subretinal deposits that were distinct from the cuticular drusen. Swept-source optical coherence tomography demonstrated a hyper-reflective subretinal material corresponding to the grey-yellow subretinal deposits on clinical examination along with diffuse outer retinal disruption. Fundus autofluorescence revealed scattered hypoautofluorescence corresponding to cuticular drusen and also larger patches of hypoautofluorescence corresponding to the grey-yellow subretinal deposits. Fluorescein angiography demonstrated hypofluorescence corresponding to the large subretinal deposits and leakage at the optic nerve. Lymph node biopsy demonstrated IgG4-positive plasma cells and elevated serum IgG4 levels leading to a diagnosis of IgG4-related ophthalmic disease. The patient was treated with oral prednisone with subsequent resolution of the optic nerve edema. CONCLUSION: We describe multimodal imaging of unique retinal and optic nerve findings associated with IgG4-related ophthalmic disease. Our report broadens the spectrum of ocular involvement associated with IgG4-related disease.

Oscillatory potentials in patients with birdshot chorioretinopathy.

PURPOSE: The electroretinogram (ERG) has proven to be useful in the evaluation and monitoring of patients with posterior uveitis. ERG oscillatory potentials (OPs) are sometimes reduced in many uveitic eyes with otherwise grossly normal ERG responses. This study compares ERG parameters, including OPs, between patients with birdshot chorioretinopathy, other posterior uveitis, and controls. METHODS: This was a retrospective case-control study. Sixty-four patients seen at a clinical practice had a total of 93 visits during which ERG was performed on both eyes. ERG data from 93 age-matched controls were also collected. Root-mean-squared (RMS) energy of the OPs was calculated using Fourier analysis for 88 patients and 88 age-matched controls for whom complete data were available. Photopic flicker amplitudes, photopic flicker latencies, scotopic b-wave amplitudes, and OP RMS values were compared between patients and controls. Diagnostic performance was assessed using receiver operating characteristic (ROC) curves. RESULTS: The mean ages of patients and controls were 55.9 ± 10.8 (SD) years and 55.1 ± 11.5, respectively. 83% of the patients had a diagnosis of BCR. The mean OP RMS value was significantly different in patients (15.6 µV ± 9.7 µV) versus control eyes (33.0 µV ± 12.7 µV), p < 0.001. Area under the ROC curves (AUROC) was 0.75 for photopic flicker amplitudes, 0.77 for photopic flicker latencies, 0.72 for scotopic b-wave amplitudes, and 0.88 for OP RMS. AUROC was significantly different between OP RMS and photopic flicker amplitudes (p < 0.001), between OP RMS and flicker latencies (p = 0.0032), and between OP RMS and scotopic b-wave amplitudes (p < 0.0001). CONCLUSION: Analysis of OPs shows greater sensitivity and specificity in the diagnosis and evaluation of patients with birdshot chorioretinopathy than photopic and scotopic ERG amplitudes and photopic flicker latencies.

Trends in State Policy Support for Sexual Minorities and HIV-Related Outcomes Among Men Who Have Sex With Men in the United States, 2008-2014.

BACKGROUND: To examine trends in state-level policy support for sexual minorities and HIV outcomes among men who have sex with men (MSM). METHODS: This longitudinal analysis linked state-level policy support for sexual minorities [N = 94 metropolitan statistical areas (MSAs) in 38 states] to 7 years of data (2008-2014) from the Centers for Disease Control and Prevention on HIV outcomes among MSM. Using latent growth mixture modeling, we combined 11 state-level policies (eg, nondiscrimination laws including sexual orientation as a protected class) from 1999 to 2014, deriving the following 3 latent groups: consistently low policy support, consistently high policy support, and increasing trajectory of policy support. Outcomes were HIV diagnoses per 10,000 MSM, late diagnoses (number of deaths within 12 months of HIV diagnosis and AIDS diagnoses within 3 months of HIV diagnosis) per 10,000 MSM, AIDS diagnoses per 10,000 MSM with HIV, and AIDS-related mortality per 10,000 MSM with AIDS. RESULTS: Compared with MSAs in states with low policy support and increasing policy support for sexual minorities, MSAs in states with the highest level of policy support had lower risks of HIV diagnoses [risk difference (RD) = -37.9, 95% confidence interval (CI): -54.7 to -21.0], late diagnoses (RD = -12.5, 95% CI: -20.4 to -4.7), and AIDS-related mortality (RD = -33.7, 95% CI: -61.2 to -6.2), controlling for time and 7 MSA-level covariates. In low policy support states, 27% of HIV diagnoses, 21% of late diagnoses, and 10% of AIDS deaths among MSM were attributable to the policy climate. CONCLUSION: The state-level policy climate related to sexual minorities was associated with HIV health outcomes among MSM and could be a potential public health tool for HIV prevention and care.

Nab-paclitaxel-induced cystoid macular edema in a patient with pre-existing optic neuropathy.

Paclitaxel is a widely used chemotherapy agent that has rarely been associated with ophthalmic toxicities. Cystoid macular edema is one such rare side effect of paclitaxel therapy. Its pathophysiology remains poorly understood. Here, we report on a 69-year-old woman who developed cystoid macular edema associated with the albumin-bound formulation of paclitaxel after several months of therapy for breast cancer. After 2 months of drug withdrawal, her vision improved and there was a significant improvement in the macular edema by imaging with spectral-domain optical coherence tomography. Oncologists using taxane agents should be aware of this rare adverse outcome for timely patient referral to an ophthalmologist and appropriate treatment to preserve a patient's visual acuity.

EXPANDED CLINICAL SPECTRUM OF MULTIPLE EVANESCENT WHITE DOT SYNDROME WITH MULTIMODAL IMAGING.

PURPOSE: To evaluate and characterize multiple evanescent white dot syndrome abnormalities with modern multimodal imaging modalities. METHODS: This retrospective cohort study evaluated fundus photography, fluorescein angiography, indocyanine green angiography, optical coherence tomography, enhanced depth imaging optical coherence tomography, short-wavelength autofluorescence, and near-infrared autofluorescence. RESULTS: Thirty-four multiple evanescent white dot syndrome patients with mean age of 28.7 years were studied (range, 14-49 years). Twenty-six patients were women, and eight were men. Initial mean visual acuity was 0.41 logMAR. Final mean visual acuity was 0.03 logMAR. Fluorescein angiography shows a variable number of mid retinal early fluorescent dots distributed in a wreathlike pattern, which correlate to fundus photography, fundus autofluorescence, and indocyanine green angiography. Indocyanine green angiography imaging shows the dots and also hypofluorescent, deeper, and larger spots, which are occasionally confluent, demonstrating a large plaque of deep retinal hypofluorescence. Optical coherence tomography imaging shows multifocal debris centered at and around the ellipsoid layer, corresponding to the location of spots seen with photography, indocyanine green angiography, and fluorescein angiography. Protrusions of the hyperreflectant material from the ellipsoid layer toward the outer nuclear layer correspond to the location of dots seen with photography, indocyanine green angiography, and fluorescein angiography. CONCLUSION: Multimodal imaging analysis of the retina in patients with multiple evanescent white dot syndrome shows additional features that may help in the diagnosis of the disease and in further understanding its etiology. Multiple evanescent white dot syndrome is predominantly a disease of the outer retina, centered at the ellipsoid zone, but also involving the interdigitation zone and the outer nuclear layer.

Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules.

PURPOSE: To characterize nodular lesions of the retina and optic nerve with spectral-domain optical coherence tomography (SD-OCT) in patients with sarcoidosis. METHODS: This is a retrospective series of 6 eyes from 5 patients with an established diagnosis of sarcoidosis, with clinically detected nodules of the optic nerve or retina. All lesions were imaged with fundus photography and SD-OCT on presentation, and followed with serial imaging after treatment with corticosteroids and/or immunomodulatory therapy. RESULTS: Spectral OCT through the lesions revealed nodular hyperreflective processes obscuring the retinal layers or optic cup, with local structural changes, including subretinal and intraretinal fluid. After treatment with corticosteroids and/or immunosuppression in 4 followed patients, all lesions regressed with improvement in associated structural changes, but did not entirely disappear. CONCLUSIONS: Spectral OCT can be useful in identifying lesion morphology and location, and in tracking the response to treatment in eyes with posterior-segment nodules, presumably secondary to sarcoidosis.

Satisfaction with known, open-identity, or unknown sperm donors: reports from lesbian mothers of 17-year-old adolescents.

OBJECTIVE: To assess whether lesbian mothers of 17-year-old adolescents conceived through donor insemination are satisfied with their choice of a known, open-identity, or unknown sperm donor and whether the mothers' satisfaction is associated with psychological health problems in the index adolescent offspring. DESIGN: Mixed-method study. SETTING: Not applicable. PATIENT(S): One hundred twenty-nine lesbian mothers and 77 index offspring. INTERVENTION(S): Semistructured interviews with the mothers conducted by telephone and the State-Trait Personality Inventory (STPI) completed online by the adolescent offspring. MAIN OUTCOME MEASURE(S): Satisfaction with the type of sperm donor selected was assessed through multiple choice questions, and adolescent psychological health problems by the STPI. The reasons for the mothers' (dis)satisfaction were evaluated through a thematic analysis of transcribed interviews. RESULT(S): Overall, 77.5% of mothers were satisfied with the type of donor chosen. There were no significant differences between birth mothers and comothers on (dis)satisfaction. In comparing satisfied with dissatisfied birth mothers by donor type, the only significant differences were that those selecting open-identity donors were more satisfied than dissatisfied and that those using unknown donors were more dissatisfied than satisfied; (dis)satisfaction with donor type was unrelated to offspring psychological health problems. Qualitative analyses revealed six themes concerning all mothers' reasons for (dis)satisfaction. CONCLUSION(S): Donor access and custody concerns were the primary themes mentioned by lesbian mothers regarding their (dis)satisfaction with the type of sperm donor they had selected.

New best1 mutations in autosomal recessive bestrophinopathy.

PURPOSE: To report the ocular phenotype in patients with autosomal recessive bestrophinopathy and carriers, and to describe novel BEST1 mutations. METHODS: Patients with clinically suspected and subsequently genetically proven autosomal recessive bestrophinopathy underwent full ophthalmic examination and investigation with fundus autofluorescence imaging, spectral domain optical coherence tomography, electroretinography, and electrooculography. Mutation analysis of the BEST1 gene was performed through direct Sanger sequencing. RESULTS: Five affected patients from four families were identified. Mean age was 16 years (range, 6-42 years). All affected patients presented with reduced visual acuity and bilateral, hyperautofluorescent subretinal yellowish deposits within the posterior pole. Spectral domain optical coherence tomography demonstrated submacular fluid and subretinal vitelliform material in all patients. A cystoid maculopathy was seen in all but one patient. In 1 patient, the location of the vitelliform material was seen to change over a follow-up period of 3 years despite relatively stable vision. Visual acuity and fundus changes were unresponsive to topical and systemic carbonic anhydrase inhibitors and systemic steroids. Carriers had normal ocular examinations including normal fundus autofluorescence. Three novel mutations were detected. CONCLUSION: Three novel BEST1 mutations are described, suggesting that many deleterious variants in BEST1 resulting in haploinsufficiency are still unknown. Mutations causing autosomal recessive bestrophinopathy are mostly located outside of the exons that usually harbor vitelliform macular dystrophy-associated dominant mutations.

Adaptive optics imaging of cone mosaic abnormalities in acute macular neuroretinopathy.

BACKGROUND AND OBJECTIVE: To assess the cone photoreceptor mosaic in acute macular neuroretinopathy (AMN) using adaptive optics (AO) imaging. PATIENTS AND METHODS: Four patients with AMN were evaluated retrospectively by near-infrared reflectance (IR) confocal scanning laser ophthalmoscopy (SLO), spectral-domain optical coherence tomography (SD-OCT), and a flood-illuminated retinal AO camera. Microperimetry was performed in one patient. RESULTS: The cone photoreceptor density was decreased at the level of the AMN lesions. The cone mosaic disruption appeared heterogeneous and more widespread than the lesion detected in the IR-SLO and SD-OCT images. The areas of cone loss correlated with SD-OCT and microperimetry. After resolution of the AMN lesion on IR-SLO, there was incomplete recovery of the cone photoreceptor mosaic. CONCLUSION: Cone photoreceptor damage and reconstitution were documented in vivo at the cellular level in AMN using AO imaging. AO imaging appeared more sensitive than combined IR-SLO and SD-OCT to detect and follow photoreceptor damage in patients with AMN.

Success with single-agent immunosuppression for multifocal choroidopathies.

PURPOSE: To evaluate the success of single-agent immunosuppression for patients with the posterior uveitides, birdshot chorioretinitis, multifocal choroiditis with panuveitis, and punctate inner choroiditis. DESIGN: Retrospective case series. METHODS: setting: Tertiary care uveitis practices. population: Patients initiated on immunomodulatory therapy. intervention: Patients were treated with prednisone 1 mg/kg and mycophenolate 2 g daily. Prednisone was tapered after 1 month. Immunosuppression was escalated to mycophenolate 3 g daily, with addition of a second agent, as needed, to achieve treatment success. outcome measure: Treatment success, defined as no disease activity with prednisone dose ≤10 mg daily, at 6, 12, and 24 months. RESULTS: Twenty-seven patients were followed. Mean presentation and 2-year follow-up acuities were 20/41 and 20/42, respectively. For birdshot chorioretinitis, mean (±standard deviation) quantitative Goldmann visual field scores improved from 761 ± 69 degrees (IV/4 isopter) and 496 ± 115 degrees (I/4 isopter) at presentation to 784 ± 57 degrees and 564 ± 125 degrees, respectively. Prednisone was successfully tapered in 95% of patients; mean prednisone doses at 1 and 2 years were 5.3 ± 4.1 and 5.7 ± 4.8 mg/day, respectively. At 2 years, prednisone was discontinued in 11% of patients. Treatment success was achieved in 74% of patients on 1 immunosuppressant, and in an additional 21% of patients on 2 agents, for an overall 95% success rate at 2 years. CONCLUSIONS: Posterior uveitides can be treated with 1 agent in most patients, but the data suggest a need to escalate therapy to higher mycophenolate doses, and in one fifth of cases to add a second agent to maintain disease suppression with acceptably low prednisone doses.

Epiretinal membranes in uveitic macular edema: effect on vision and response to therapy.

PURPOSE: To evaluate the effects of epiretinal membranes on the response of uveitic macular edema to therapy and on visual acuity outcomes. DESIGN: Retrospective case series. METHODS: One hundred four eyes of 77 patients with uveitic macular edema were identified at a tertiary care center. Epiretinal membranes were diagnosed when identified by 2 investigators' grading of spectral-domain optical coherence tomography and scored for the presence or absence of surface wrinkling. Outcomes included best-corrected visual acuity, central subfield thickness, and rates of macular edema improvement (>20% reduction in central subfield thickness) and resolution (reduction of central subfield thickness to <315 µm) at 3 and 6 months follow-up. RESULTS: Seventy-two eyes of 59 patients had an epiretinal membrane on presentation. Eyes without epiretinal membranes and with epiretinal membranes without surface wrinkling were not significantly different at presentation or at 3 and 6 months follow-up. Conversely, eyes with an epiretinal membrane with retinal surface wrinkling had a greater proportion of eyes with 20/200 or worse visual acuity at presentation, and had worse mean acuities at 3 months (20/94 vs 20/35 for eyes without an epiretinal membrane, P = .002) and at 6 months follow-up (20/110 vs 20/36 for eyes without an epiretinal membrane, P = .02). At 6 months of follow-up the mean central subfield thicknesses were: eyes without an epiretinal membrane, 338 ± 23 µm; and eyes with an epiretinal membrane and surface wrinkling, 405 ± 22 µm (P = .05). CONCLUSIONS: In eyes with epiretinal membranes and retinal surface wrinkling, uveitic macular edema had a poorer visual acuity response to medical therapy and thicker maculae at 6 months.

Paracentral acute middle maculopathy: a new variant of acute macular neuroretinopathy associated with retinal capillary ischemia.

IMPORTANCE: With the advent of more sophisticated imaging systems, such as spectral domain optical coherence tomography (SD-OCT), disruption of the inner segment/outer segment (IS/OS) band, and thinning of the outer nuclear layer (ONL) have been identified in association with acute macular neuroretinopathy (AMN). OBJECTIVES: To characterize a new SD-OCT presentation of AMN as a paracentral acute middle maculopathy and to describe multimodal imaging findings that implicate an underlying pathogenesis related to retinal capillary ischemia. DESIGN, SETTING, AND PARTICIPANTS: Retrospective observational case series (January 1, 2012, to January 1, 2013) reviewing clinical and imaging data from 9 patients (11 eyes) with AMN at 6 tertiary referral centers. Lesions were classified as type 1 or 2 in relation to the SD-OCT location of the lesion above (type 1) or below (type 2) the outer plexiform layer (OPL) at 6 tertiary referral centers. RESULTS: Of the 9 patients, 5 were female and 4 were male (mean age, 47.6 years; range, 21-65 years). All patients presented with an acute paracentral scotoma and demonstrated a classic dark gray paracentral lesion with near-infrared imaging. Visual acuity ranged from 20/15 to 20/30. Six eyes (5 patients) had type 1 SD-OCT lesions, also referred to as paracentral acute middle maculopathy, and 5 eyes (4 patients) had type 2 SD-OCT lesions. Although type 1 lesions lead to inner nuclear layer (INL) thinning, type 2 lesions resulted in ONL thinning. Type 2 lesions were always associated with significant outer macular defects, including disruption of the inner segment/outer segment and outer segment/retinal pigment epithelium bands, whereas type 1 lesions spared the outer macula. CONCLUSIONS AND RELEVANCE: Paracentral acute middle maculopathy may represent a novel variant of AMN that affects the middle layers of the macula above the OPL as diagnosed with SD-OCT imaging. Two types of AMN lesions may be seen with SD-OCT occurring above and below the OPL. Type 1 refers to hyperreflective bands in the OPL/INL region with subsequent INL thinning. Type 2 is hyperreflective bands in the OPL/ONL region with subsequent ONL thinning. Type 2 lesions may be associated with concomitant defects of the inner segment/outer segment layer. We propose that each of these lesions may be explained by occlusion of either the superficial capillary plexus (type 1) or deep capillary plexus (type 2) located in the innermost and outermost portion of the INL, respectively, immediately adjacent to each corresponding lesion type.