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2014 JAMA Ophthalmology
doi: 10.1001/jamaophthalmol.2013.5949
Mission Statement: To be the indispensable source of ophthalmic knowledge by promptly publishing innovative, clinically relevant research through consistent and authoritative peer review and;thereby, to be the first choice of authors for their important manuscripts. Editor: Neil M. Bressler, MD Deputy Editors Roy W. Beck, MD, PhD, Tampa, Florida Michael A. Kass, MD, St Louis, Missouri Viewpoint Editor Frederick L. Ferris III, MD, Bethesda, Maryland Web Editor Michael V. Boland, MD, PhD, Baltimore, Maryland CME Editor Jennifer I. Lim, MD, Chicago, Illinois Managing Editor Veronica (Ronnie) Doyle Editorial Office Wilmer Eye Institute Johns Hopkins University School of Medicine Maumenee 752 600 N Wolfe St Baltimore, MD 21287-9227 Phone: (410) 955-6494 Fax: (866) 542-4857 [email protected] Editorial Board Lloyd P. Aiello, MD, PhD, Boston, Massachusetts Adam R. Glassman, MS, Tampa, Florida Dennis P. Han, MD, Milwaukee, Wisconsin Lee M. Jampol, MD, Chicago, Illinois Judy E. Kim, MD, Milwaukee, Wisconsin Andrew G. Lee, MD, Houston, Texas Paul P. Lee, MD, JD, Ann Arbor, Michigan Stephen D. McLeod, MD, San Francisco, California David C. Musch, PhD, MPH, Ann Arbor, Michigan Hendrik P. N. Scholl, MD, MA, Baltimore, Maryland Nasreen A. Syed, MD, Iowa City, Iowa Janey L. Wiggs, MD, PhD, Boston, Massachusetts Contributing Writers Kay Dickersin, PhD, Baltimore, Maryland Malvina B. Eydelman, MD, Rockville, Maryland Marco A. Zarbin, MD, PhD, Newark, New Jersey Advisory Committee Francesco Bandello, MD, Milan, Italy Wiley A. Chambers II, MD, Rockville, Maryland Usha Chakravarthy, MD, Belfast, Ireland Julie Gottlieb, MA, Baltimore, Maryland Tatsuro Ishibashi, MD, PhD, Fukuoka, Japan Douglas A. Jabs, MD, MBA, New York, New York Anne S. Lindblad, PhD, Rockville, Maryland Former Editors Herman Knapp, MD 1869-1911 Arnold Knapp, MD 1911-1950 Francis Heed Adler, MD 1950-1960 David G. Cogan, MD 1960-1966 Henry F. Allen, MD 1966-1976 Frederick C. Blodi, MD 1976-1984 Morton F. Goldberg, MD 1984-1994 Daniel M. Albert, MD, MS 1994-2013 Publication Staff Offices: 330 N Wabash Ave, Chicago, IL 60611 Howard Bauchner, MD Editor in Chief, The JAMA Network Executive Editor Phil B. Fontanarosa, MD, MBA Executive Managing Editor Annette Flanagin Managing Editor Brenda Gregoline Editorial Processing Manuscript Editing Manager Tracy Frey Senior Manuscript Editor Shannon L. Sparenga Manuscript Editors Nicole E. Balin, Miriam Y. Cintron, Paul Ruich Freelance Manuscript Editing Manager Diane L. Cannon Freelance Manuscript Editing Coordinators Connie L. Manno, Fred Marshall Freelance Copy Editors and Copyreaders Laura Adamczyk, Catherine A. Alden, Stephanie Lang Beckmeyer, Jan Clavey, Mary Coerver-Connolly, Roya Khatiblou, Laura King, Juliann Lundell Tarsney, Donna J. 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Hengesbaugh The JAMA Network is a consortium of peer-reviewed print and online medical publications that includes JAMA, JAMA Ophthalmology, and other specialty journals. All articles published, including editorials, letters, and book reviews, represent the opinions of the authors and do not reflect the policy of the American Medical Association, the Editorial Board, or the institution with which the author is affiliated, unless this is clearly specified. ©2014 American Medical Association. All rights reserved. Reproduction without permission is prohibited.
Repka, Michael X.; Kraker, Raymond T.; Holmes, Jonathan M.; Summers, Allison I.; Glaser, Stephen R.; Barnhardt, Carmen N.; Tien, David R.
2014 JAMA Ophthalmology
doi: 10.1001/jamaophthalmol.2014.392pmid: 24789375
ImportanceInitial treatment for amblyopia of the fellow eye with patching and atropine sulfate eyedrops improves visual acuity. Long-term data on the durability of treatment benefit are needed. ObjectiveTo report visual acuity at 15 years of age among patients who were younger than 7 years when enrolled in a treatment trial for moderate amblyopia. Design, Setting, and ParticipantsIn a multicenter clinical trial, 419 children with amblyopia (visual acuity, 20/40 to 20/100) were randomly assigned to patching (minimum of 6 h/d) or atropine sulfate eyedrops, 1% (1 drop daily), for 6 months. Treatment after 6 months was at the discretion of the investigator. Two years after enrollment, an unselected subgroup of 188 children were enrolled into long-term follow-up. InterventionInitial treatment with patching or atropine with subsequent treatment at investigator discretion. Main Outcomes and MeasuresVisual acuity at 15 years of age with the electronic Early Treatment Diabetic Retinopathy Study test in amblyopic and fellow eyes. ResultsMean visual acuity in the amblyopic eye measured in 147 participants at 15 years of age was 0.14 logMAR (approximately 20/25); 59.9% of amblyopic eyes had visual acuity of 20/25 or better and 33.3%, 20/20 or better. Mean interocular acuity difference (IOD) at 15 years of age was 0.21 logMAR (2.1 lines); 48.3% had an IOD of 2 or more lines and 71.4%, 1 or more lines. Treatment (other than spectacles) was prescribed for 9 participants (6.1%) aged 10 to 15 years. Mean IOD was similar at examinations at 10 and 15 years of age (2.0 and 2.1 logMAR lines, respectively; P = .39). Better visual acuity at the 15-year examination was achieved in those who were younger than 5 years at the time of entry into the randomized clinical trial (mean logMAR, 0.09) compared with those aged 5 to 6 years (mean logMAR, 0.18; P < .001). When we compared subgroups based on original treatment with atropine or patching, no significant differences were observed in visual acuity of amblyopic and fellow eyes at 15 years of age (P = .44 and P = .43, respectively). Conclusions and RelevanceAt 15 years of age, most children treated for moderate amblyopia when younger than 7 years have good visual acuity, although mild residual amblyopia is common. The outcome is similar regardless of initial treatment with atropine or patching. The results indicate that improvement occurring with amblyopia treatment is maintained until at least 15 years of age. Trial Registrationclinicaltrials.gov Identifier: NCT00000170
Ooto, Sotaro; Vongkulsiri, Sritatath; Sato, Taku; Suzuki, Mihoko; Curcio, Christine A.; Spaide, Richard F.
2014 JAMA Ophthalmology
doi: 10.1001/jamaophthalmol.2014.1871pmid: 24801396
ImportanceOptical coherence tomography (OCT) abnormalities of age-related macular degeneration (AMD) have not been fully characterized because of the complex morphology and a lack of correlative histologic studies. Expansion of our ability to interpret increasing attributes brings us closer to the goal of in vivo histologic analysis of the eye by OCT. ObjectiveTo describe a new outer retinal finding of AMD using spectral-domain (SD) OCT and suggest histopathologic correlates. Design, Setting, and ParticipantsTwenty-five eyes of 16 patients with AMD with severe atrophy due to either choroidal neovascularization (CNV) or geographic atrophy (GA) and 53 donor eyes of 53 patients with late AMD were included. Imaging studies were conducted at a referral retinal practice and histopathology was done at a university research laboratory. ExposuresFindings in the outer retina were evaluated in SD-OCT images in eyes with atrophy of the retinal pigment epithelium (RPE) and compared with histopathologic findings in eyes with GA or CNV that also showed loss of the RPE. Main Outcomes and MeasuresSpectral-domain OCT and histologic characteristics of the outer retina. ResultsThe mean (SD) age of the 16 patients was 82.7 (7.9) years. Twenty eyes had CNV and 5 eyes had GA. The mean best-corrected visual acuity was 0.800 logMAR (interquartile range, 0.350-1.000 logMAR), a Snellen equivalent of 20/126. A curvilinear hyperreflective density was identified above the Bruch membrane line within the atrophic area in the SD-OCT images. At the internal border, the material was contiguous with the outer portion of the RPE band. Below the material was a relatively hyporeflective space. The material was thrown into folds in cases with atrophy following CNV or was seen as a sheet with numerous bumps in eyes with GA. Review of histopathologic findings of eyes with advanced GA and CNV revealed a rippled layer of basal laminar deposits in an area of RPE atrophy that was located in the same level as the curvilinear line seen in the OCT images. Conclusions and RelevanceWe have described a new entity, termed outer retinal corrugations, which may correspond to histological findings of basal laminar deposits, extracellular deposits that persist in eyes with late AMD. Observation of this undulating band does not necessarily mean there is exudation or leakage; as a consequence, these patients do not need treatment based on this solitary finding.
Jost, Reed M.; Yanni, Susan E.; Beauchamp, Cynthia L.; Stager, David R.; Stager, David; Dao, Lori; Birch, Eileen E.
2014 JAMA Ophthalmology
doi: 10.1001/jamaophthalmol.2014.424pmid: 24875453
ImportanceCommercially available automated vision screening devices assess refractive risk factors, not amblyopia or strabismus, underreferring affected children and overreferring healthy children. Nearly half of affected children are not identified until after age 5 years, when treatment is less effective. ObjectivesTo determine the diagnostic accuracy of the Pediatric Vision Scanner (PVS), a binocular retinal birefringence scanner, to objectively identify strabismus and amblyopia, and to compare retinal birefringence screening with a widely used automated pediatric screening device. Design, Setting, and ParticipantsThree hundred consecutive preschool children (aged 2-6 years) were screened using the PVS and the SureSight Autorefractor at 2 pediatric ophthalmology private practices. A masked comprehensive pediatric ophthalmic examination provided the gold standard for determining sensitivity and specificity for each screening device. Main Outcomes and MeasuresThe primary outcome was sensitivity and specificity of the PVS for detecting the targeted conditions, strabismus and amblyopia, in children aged 2 to 6 years. Secondary outcomes included the positive and negative likelihood ratios of the PVS for identifying the targeted conditions. In addition, sensitivity, specificity, and positive and negative likelihood ratios of the SureSight Autorefractor for the targeted conditions were assessed in the same cohort of children. ResultsOf the 300 patients, 188 had strabismus only, amblyopia only, or both, and 112 had no strabismus or amblyopia. The sensitivity of the PVS to detect strabismus and amblyopia (0.97; 95% CI, 0.94-1.00) was significantly higher than that of the SureSight Autorefractor (0.74; 95% CI, 0.66-0.83). Specificity of the PVS for strabismus and amblyopia (0.87; 95% CI, 0.80-0.95) was significantly higher than that of the SureSight Autorefractor (0.62; 95% CI, 0.50-0.73). Conclusions and RelevanceThe PVS identified children with strabismus and/or amblyopia with high sensitivity, outperforming the SureSight Autorefractor. Accurate, early detection of these conditions could improve long-term vision outcomes of affected preschool children.
Espandar, Ladan;Cummings, Thomas J.;Boehlke, Christopher S.
2014 JAMA Ophthalmology
doi: 10.1001/jamaophthalmol.2013.4079pmid: 24830662
A, A 55-year-old immunocompetent man presented with unilateral, progressive, anterior to midstromal opacification unresponsive to treatment. B, The photograph (green arrow and lines) and an anterior segment module of a spectral-domain optical coherence tomographic scan illustrate the stromal opacification (282-μm thickness). The patient underwent penetrating keratoplasty. Light micrographs (hematoxylin-eosin, original magnification ×40 [C] and ×200 [D]) reveal the innumerable microorganisms in the anterior and midstroma, without inflammation. E, An electron micrograph (original magnification ×37 500) reveals a typical microsporidian spore.
Yang, Paul; Michaels, Keith V.; Courtney, Robert J.; Wen, Yuquan; Greninger, Daniel A.; Reznick, Leah; Karr, Daniel J.; Wilson, Lorri B.; Weleber, Richard G.; Pennesi, Mark E.
2014 JAMA Ophthalmology
doi: 10.1001/jamaophthalmol.2014.685pmid: 24676353
ImportanceWhile older children and adults with achromatopsia have been studied, less is known of young children with achromatopsia. ObjectivesTo characterize the macular and foveal architecture of patients with achromatopsia during early childhood with handheld spectral-domain optical coherence tomographic imaging and to make phenotype-genotype correlations. Design, Setting, and ParticipantsComparative case series of 9 patients with achromatopsia and 9 age-matched control participants at a tertiary ophthalmology referral center. Main Outcomes and MeasuresPatients underwent complete ocular examination, full-field electroretinography, handheld spectral-domain optical coherence tomographic imaging, and screening for genetic mutations. ResultsThe mean (SD) age of the patients with achromatopsia was 4.2 (2.4) years, and the mean (SD) age of the control participants was 4.0 (2.1) years. Cone-driven responses to photopic single-flash or 30-Hz stimuli were nonrecordable in 7 patients and severely attenuated in 2. Rod-driven responses to dim scotopic single-flash stimuli were normal in 7 patients and mildly subnormal in 2. Six patients (67%) had foveal ellipsoid zone disruption, of which 1 had a hyporeflective zone. Four patients (44%) had foveal hypoplasia. The average total retinal thicknesses of the macula and fovea in the patients with achromatopsia were 14% and 17% thinner than in the control participants (P < .001 and P = .001), which was mostly due to the outer retina that was 18% and 26% thinner than in control participants (both P < .001), respectively. Genetic testing revealed a common homozygous mutation in CNGB3 in 5 patients with complete achromatopsia and heterozygous mutations in CNGA3 in 2 patients with incomplete achromatopsia. The youngest and worst-affected patient harbored compound heterozygous mutations in CNGB3 and a single mutation in CNGA3. Conclusions and RelevanceIn early childhood, there is a spectrum of foveal pathology that is milder than reported in older individuals with achromatopsia, which suggests the need for early therapeutic intervention. Neither age alone nor genotype alone predicts the degree of photoreceptor loss or preservation. Thus, in anticipation of future gene therapy trials in humans, we propose that handheld spectral-domain optical coherence tomography is an important tool for the early assessment and stratification of macular architecture in young children with achromatopsia.
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