JAMA Ophthalmology

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2013.5963

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2013.5964

Han, Dennis P.

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.1405pmid: 25058876

Goldstein, Judith E.; Chun, Melissa W.; Fletcher, Donald C.; Deremeik, James T.; Massof, Robert W.

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.1747pmid: 25073745

ImportanceMost patients with low vision are elderly and have functional limitations from other health problems that could add to the functional limitations caused by their visual impairments. ObjectiveTo identify factors that contribute to visual ability measures in patients who present for outpatient low vision rehabilitation (LVR) services. Design, Setting, and ParticipantsAs part of a prospective, observational study of new patients seeking outpatient LVR, 779 patients from 28 clinical centers in the United States were enrolled in the Low Vision Rehabilitation Outcomes Study (LVROS) from April 25, 2008, through May 2, 2011. The Activity Inventory (AI), an adaptive visual function questionnaire, was administered to measure overall visual ability and visual ability in 4 functional domains (reading, mobility, visual motor function, and visual information processing) at baseline before LVR. The Geriatric Depression Scale, Telephone Interview for Cognitive Status, and Medical Outcomes Study 36-Item Short-Form Health Survey physical functioning questionnaires were also administered to measure patients’ psychological, cognitive, and physical health states, respectively. Main Outcomes and MeasuresPredictors of visual ability and functional domains as measured by the AI. ResultsAmong the 779 patients in the LVROS sample, the mean age was 76.4 years, 33% were male, and the median logMAR visual acuity score was 0.60 (0.40-0.90 interquartile range). Correlations were observed between logMAR visual acuity and baseline visual ability overall (r = −0.42) and for all functional domains. Visual acuity was the strongest predictor of visual ability (P  < .001) and reading ability (P < .001) and had a significant independent effect on the other functional domains. Physical ability was independently associated with (P < .001) overall visual ability as well as mobility and visual motor function. Depression had a consistent independent effect (P < .001) on overall visual ability and on all functional domains, whereas cognition had an effect on only reading and mobility (P < .001). Conclusions and RelevanceVisual ability is a multidimensional construct, with visual acuity, depression, physical ability, and cognition explaining more than one-third of the variance in visual ability as measured by the AI. The significant contributions of the nonvisual factors to visual ability measures and the rehabilitation potential (ie, ceiling) effects they may impose on LVR are important considerations when measuring baseline visual ability and ultimately LVR outcomes in ongoing clinical research.

Gupta, Amit; Agarwal, Aniruddha; Ram, Jagat

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.186pmid: 25124654

Quinn, Graham E.; Ying, Gui-shuang; Daniel, Ebenezer; Hildebrand, P. Lloyd; Ells, Anna; Baumritter, Agnieshka; Kemper, Alex R.; Schron, Eleanor B.; Wade, Kelly

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.1604pmid: 24970095

ImportanceThe present strategy to identify infants needing treatment for retinopathy of prematurity (ROP) requires repeated examinations of at-risk infants by physicians. However, less than 10% ultimately require treatment. Retinal imaging by nonphysicians with remote image interpretation by nonphysicians may provide a more efficient strategy. ObjectiveTo evaluate the validity of a telemedicine system to identify infants who have sufficiently severe ROP to require evaluation by an ophthalmologist. Design, Setting, and ParticipantsAn observational study of premature infants starting at 32 weeks’ postmenstrual age was conducted. This study involved 1257 infants with birth weight less than 1251 g in neonatal intensive care units in 13 North American centers enrolled from May 25, 2011, through October 31, 2013. InterventionsInfants underwent regularly scheduled diagnostic examinations by an ophthalmologist and digital imaging by nonphysician staff using a wide-field digital camera. Ophthalmologists documented findings consistent with referral-warranted (RW) ROP (ie, zone I ROP, stage 3 ROP or worse, or plus disease). A standard 6-image set per eye was sent to a central server and graded by 2 trained, masked, nonphysician readers. A reading supervisor adjudicated disagreements. Main Outcomes and MeasuresThe validity of grading retinal image sets was based on the sensitivity and specificity for detecting RW-ROP compared with the criterion standard diagnostic examination. ResultsA total of 1257 infants (mean birth weight, 864 g; mean gestational age, 27 weeks) underwent a median of 3 sessions of examinations and imaging. Diagnostic examination identified characteristics of RW-ROP in 18.2% of eyes (19.4% of infants). Remote grading of images of an eye at a single session had sensitivity of 81.9% (95% CI, 77.4-85.6) and specificity of 90.1% (95% CI, 87.9-91.8). When both eyes were considered for the presence of RW-ROP, as would routinely be done in a screening, the sensitivity was 90.0% (95% CI, 85.4-93.5), with specificity of 87.0% (95% CI, 84.0-89.5), negative predictive value of 97.3%, and positive predictive value of 62.5% at the observed RW-ROP rate of 19.4%. Conclusions and RelevanceWhen compared with the criterion standard diagnostic examination, these results provide strong support for the validity of remote evaluation by trained nonphysician readers of digital retinal images taken by trained nonphysician imagers from infants at risk for RW-ROP. Trial Registrationclinicaltrials.gov Identifier:NCT01264276

Durlu, Yusuf K.; Köroğlu, Çiğdem; Tolun, Aslihan

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.1658pmid: 24945461

ImportanceA new form of cone-rod dystrophy (CORD) is described and the gene responsible for the disease is identified. ObjectiveTo clinically evaluate 4 patients and 5 control relatives, perform disease gene mapping, and identify the gene defect responsible for CORD. Design, Setting, and ParticipantsProspective observational case series of 13 members of a consanguineous family and 113 unrelated control individuals. InterventionsClinical investigations included eye examination with color fundus and autofluorescent imaging, spectral-domain optical coherence tomography, and electrophysiologic measurements. Linkage mapping was performed using single-nucleotide polymorphism genotype data. Candidate genes were analyzed for mutations via Sanger sequencing. Main Outcomes and MeasuresClinical diagnosis of CORD, disease gene mapping, and mutation identification. ResultsThe onset of CORD occurred in early childhood. The clinical phenotype was typical CORD with photophobia, decreased central vision, and dyschromatopsia. In all patients, a disrupted inner segment/outer segment line and the external limiting membrane were noted as a single blurry line at the central fovea, and the cone outer segment tip line was absent. In the midperipheral retina, the rod inner segment/outer segment line was disrupted and blurry, and the rod outer segment tip line was absent. Cone response was nonrecordable in all patients, whereas rod response was nonrecordable in the eldest patient and subnormal in the others. The Arden Index was abnormal in the youngest patient and flat in the others. The disease gene mapped to a less than 2-megabase recessive locus at 12q21.33 with a logarithm of odds score of 3.92. At the locus, we identified a homozygous missense POC1B p.R106P mutation that was predicted as damaging by online tools. Conclusions and RelevancePOC1B is a novel gene for a new disease typical of CORD except that patients did not report night blindness. The clinical course was slowly progressive. Screening for POC1B mutation could benefit families afflicted with CORD.

Taney, Lauren S.; Baumal, Caroline R.

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.190pmid: 25144247

Monnereau, Claire; Quilendrino, Ruth; Dapena, Isabel; Liarakos, Vasilios S.; Alfonso, Jose F.; Arnalich-Montiel, Francisco; Böhnke, Matthias; Pereira, Nicolas Cesário; Dirisamer, Martin; Parker, John; Droutsas, Konstantinos; Geerling, Gerd; Gerten, Georg; Hashemi, Hassan; Kobayashi, Akira; Naveiras, Miguel; Oganesyan, Oganes; Orduña Domingo, Emeterio; Priglinger, Siegfried; Stodulka, Pavel; Torrano Silva, José; Venzano, Davide; Vetter, Jan Markus; Yiu, Evan; Melles, Gerrit R. J.

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.1710pmid: 24993643

ImportanceSurgeons starting to perform Descemet membrane endothelial keratoplasty (DMEK) should be informed about the learning curve and experience of others. ObjectiveTo document the clinical outcome of standardized “no-touch” DMEK and its complications during the learning curves of experienced surgeons. Design, Setting, and ParticipantsRetrospective multicenter study. A total of 431 eyes from 401 patients with Fuchs endothelial dystrophy (68.2%) and bullous keratopathy (31.8%) underwent DMEK performed by 18 surgeons in 11 countries. ExposuresDescemet membrane endothelial keratoplasty. Main Outcomes and MeasuresBest-corrected visual acuity (BCVA), endothelial cell density, and intraoperative and postoperative complications. ResultsOf 275 eyes available for BCVA pooled analysis, BCVA improved in 258 eyes (93.8%), remained unchanged in 12 (4.4%), and deteriorated in 5 (1.8%). Two hundred seventeen eyes (78.9%) reached a BCVA of at least 20/40 (≥0.5), 117 (42.5%) at least 20/25 (≥0.8), and 61 (22.2%) at least 20/20 (≥1.0). Eyes with at least 6 months of follow-up (n = 176) reached similar BCVA outcomes. Mean (SD) decrease in endothelial cell density at 6 months was 47% (20%) (n = 133 [P = .02]). Intraoperative complications were rare, including difficulties in inserting, unfolding, or positioning of the graft (1.2%) and intraoperative hemorrhage (0.5%). The main postoperative complication was graft detachment (34.6%); 20.4% underwent a single rebubbling procedure, occasionally requiring a second (2.6%) and a third rebubbling (0.7%), and 17.6% underwent a second keratoplasty. Conclusions and RelevanceOur multicenter study showed that the standardized no-touch DMEK technique was feasible in most hands. The main challenges for surgeons starting to perform the procedure may be (1) to decide whether graft preparation is outsourced or performed during surgery, (2) to limit the number of graft detachments and secondary procedures, and (3) to obtain organ cultured donor corneal tissue.

Nika, Melisa; Blachley, Taylor S.; Edwards, Paul; Lee, Paul P.; Stein, Joshua D.

2014 JAMA Ophthalmology

doi: 10.1001/jamaophthalmol.2014.1720pmid: 24970348

ImportanceAccording to evidence-based, expert recommendations, long-term users of chloroquine or hydroxychloroquine sulfate should undergo regular visits to eye care providers and diagnostic testing to check for maculopathy. ObjectiveTo determine whether patients with rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE) taking chloroquine or hydroxychloroquine are regularly visiting eye care providers and being screened for maculopathy. Design, Setting, and ParticipantsPatients with RA or SLE who were continuously enrolled in a particular managed care network for at least 5 years between January 1, 2001, and December 31, 2011, were studied. Patients’ amount of chloroquine or hydroxychloroquine use in the 5 years since the initial RA or SLE diagnosis was calculated, along with their number of eye care visits and diagnostic tests for maculopathy. Those at high risk for maculopathy were identified. Logistic regression was performed to assess potential factors associated with regular eye care visits (annual visits in ≥3 of 5 years) among chloroquine or hydroxychloroquine users, including those at highest risk for maculopathy. Main Outcomes and MeasuresAmong chloroquine or hydroxychloroquine users and those at high risk for toxic maculopathy, the proportions with regular eye care visits and diagnostic testing, as well as the likelihood of regular eye care visits. ResultsAmong 18 051 beneficiaries with RA or SLE, 6339 (35.1%) had at least 1 record of chloroquine or hydroxychloroquine use, and 1409 (7.8%) had used chloroquine or hydroxychloroquine for at least 4 years. Among those at high risk for maculopathy, 27.9% lacked regular eye care visits, 6.1% had no visits to eye care providers, and 34.5% had no diagnostic testing for maculopathy during the 5-year period. Among high-risk patients, each additional month of chloroquine or hydroxychloroquine use was associated with a 2.0% increased likelihood of regular eye care (adjusted odds ratio, 1.02; 95% CI, 1.01-1.03). High-risk patients whose SLE or RA was managed by rheumatologists had a 77.4% increased likelihood of regular eye care (adjusted odds ratio, 1.77; 95% CI, 1.27-2.47) relative to other patients. Conclusions and RelevanceIn this insured population, many patients at high risk for maculopathy associated with the use of chloroquine or hydroxychloroquine are not undergoing routine monitoring for this serious adverse effect. Future studies should explore factors contributing to suboptimal adherence to expert guidelines and the potential effect on patients’ vision-related outcomes.