A 53-year-old Asian woman was treated with hydroxychloroquine and chloroquine for lupus erythematosus. Within a few years, she noticed circle-shaped shadows in her central vision. Upon examination, the patient's visual acuity was 20 / 25 in both eyes. Humphrey visual field (HVF) testing revealed a central visual defect, and fundoscopy showed a ring-shaped area of parafoveal retinal pigment epithelium depigmentation. Fundus autofluorescence imaging showed a hypofluorescent lesion consistent with bull's eye retinopathy. Adaptive optics scanning laser ophthalmoscope (AO-SLO) revealed patch cone mosaic lesions, in which cones were missing or lost. In addition, the remaining cones consisted of asymmetrical shapes and sizes that varied in brightness. Unlike previous studies employing deformable mirrors for wavefront aberration correction, our AO-SLO approach utilized dual liquid crystal on silicon spatial light modulators. Thus, by using AO-SLO, we were able to create a photographic montage consisting of high quality images. Disrupted cone AO-SLO images were matched with visual field test results and functional deficits were associated with a precise location on the montage, which allowed correlation of histological findings with functional changes determined by HVF. We also investigated whether adaptive optics imaging was more sensitive to anatomical changes compared with spectral-domain optical coherence tomography.
Chloroquine/*adverse effects/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Image Enhancement/*methods ; Lupus Erythematosus, Systemic/drug therapy ; Macula Lutea/drug effects/*pathology ; Middle Aged ; Ophthalmoscopy/*methods ; Retinal Diseases/chemically induced/*diagnosis

A 53-year-old Asian woman was treated with hydroxychloroquine and chloroquine for lupus erythematosus. Within a few years, she noticed circle-shaped shadows in her central vision. Upon examination, the patient's visual acuity was 20 / 25 in both eyes. Humphrey visual field (HVF) testing revealed a central visual defect, and fundoscopy showed a ring-shaped area of parafoveal retinal pigment epithelium depigmentation. Fundus autofluorescence imaging showed a hypofluorescent lesion consistent with bull's eye retinopathy. Adaptive optics scanning laser ophthalmoscope (AO-SLO) revealed patch cone mosaic lesions, in which cones were missing or lost. In addition, the remaining cones consisted of asymmetrical shapes and sizes that varied in brightness. Unlike previous studies employing deformable mirrors for wavefront aberration correction, our AO-SLO approach utilized dual liquid crystal on silicon spatial light modulators. Thus, by using AO-SLO, we were able to create a photographic montage consisting of high quality images. Disrupted cone AO-SLO images were matched with visual field test results and functional deficits were associated with a precise location on the montage, which allowed correlation of histological findings with functional changes determined by HVF. We also investigated whether adaptive optics imaging was more sensitive to anatomical changes compared with spectral-domain optical coherence tomography.
Chloroquine/*adverse effects/therapeutic use ; Diagnosis, Differential ; Female ; Humans ; Image Enhancement/*methods ; Lupus Erythematosus, Systemic/drug therapy ; Macula Lutea/drug effects/*pathology ; Middle Aged ; Ophthalmoscopy/*methods ; Retinal Diseases/chemically induced/*diagnosis

INTRODUCTIONThis study describes the pathologic changes in the retina of a group of young Asian subjects with myopia worse than -10 diopters spherical equivalent (SE) refraction.

MATERIALS AND METHODSThe study population consists of 20 male subjects undergoing preemployment screening for public service for a 1-year period from 2009 to 2010. A detailed series of visual tests of function, fundus examination and grading, ocular biometry and posterior segment optical coherence tomography were performed for all eyes.

RESULTSA total of 21 eyes with mean SE of -10.88 diopters, [standard deviation (SD) , 1.28 diopters], and mean age of 21.8 years (SD, 1.3 years) were included. Out of 21 eyes, 17 (81.0%) had beta peripapillary atrophy, 10 (47.6%) had clinically detectable optic disc tilt, 1 (4.8%) had positive T-sign and 18 (85.7%) had retinal tessellation, 4 (19.0%) had posterior vitreous detachment and 14 (66.7%) had peripheral retina degeneration. The mean retinal nerve fibre layer (RNFL) thickness was 92.48 mm (SD, 9.99 mm).

CONCLUSIONNone of the 21 highly myopic eyes had features of myopic retinopathy but most of these young males had clinically visible myopia-associated abnormalities of the optic disc, vitreous and peripheral retina. Generally, these eyes had thinner RNFL. Further longitudinal studies are required to investigate if these eyes will eventually develop complications of pathological myopia.

Adolescent ; Adult ; Age of Onset ; Choroid Diseases ; diagnosis ; Fluorescein Angiography ; Humans ; Male ; Myopia ; classification ; pathology ; Nerve Fibers ; pathology ; Ophthalmoscopy ; Optic Atrophy ; diagnosis ; Optic Disk ; pathology ; Optic Nerve Diseases ; diagnosis ; Posterior Eye Segment ; pathology ; Retina ; pathology ; Retinal Degeneration ; diagnosis ; Retinal Diseases ; diagnosis ; Retinal Vessels ; pathology ; Singapore ; Tomography, Optical Coherence ; methods ; Vision Tests ; Visual Acuity ; Vitreous Detachment ; diagnosis ; Young Adult

PURPOSE: To investigate the morphologic changes in the outer retina of patients with cone dystrophy, using spectral-domain optical coherence tomography (SD-OCT). METHODS: The medical records of 15 cone dystrophy patients examined from January 2007 to January 2012 were reviewed retrospectively. All patients underwent ophthalmic evaluation including best-corrected visual acuity (BCVA), color vision testing, fundus examination, full-field standard electroretinography (ERG), multifocal (mf) ERG, and SD-OCT. Qualitative and quantitative SD-OCT data and ERG responses were analyzed and compared among the patient categories and the normal control group. RESULTS: There were 4 major categories of SD-OCT findings, based on the status of the ellipsoid portion of the photoreceptor inner segment (ISe), outer segment (OS) contact cylinder, and retinal pigment epithelium (RPE) layer. Category 0 showed no structural abnormalities. Category 1 showed foveal ISe loss and obscurity of the border between the ISe band and the external limiting membrane (ELM). Category 2 showed foveal thinning and focal foveal ISe disruption with an intact ELM. Category 3 showed foveal thickening and perifoveal disruption of the ISe layer. Category 1 to 3 showed OS contact cylinder layer absence and RPE thickening. The patients in category 0 tended to be younger (mean, 10.0 years) than those in categories 1 to 3 (mean, 17.6 years), although this difference was not statistically significant. Category 1 to 3 patients exhibited statistically significant thinning of the central retina and outer nuclear layer and thickening of the RPE layer relative to the category 0 and normal control group. There was a significant correlation between the central foveal thickness and BCVA in the patients with cone dystrophy. ERG and mfERG responses did not differ significantly among the different cone dystrophy categories. CONCLUSIONS: The morphologic features of cone dystrophy as revealed by SD-OCT, could be categorized as either normal or 1 of 3 different types of outer retinal changes. The presence of normal retinal structures in young cone dystrophy patients with functional impairment (category 0) indicates that electrophysiologic studies are superior to current imaging modalities for the early diagnosis of cone dystrophy. The characteristic SD-OCT findings in cone dystrophy patients may aid in differential diagnosis and be useful for future research on the pathology of cone dystrophy.
Adolescent ; Adult ; Child ; Child, Preschool ; Electroretinography ; Female ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Male ; Middle Aged ; Ophthalmoscopy ; Reproducibility of Results ; Retinal Cone Photoreceptor Cells/*pathology ; Retinal Dystrophies/*pathology/physiopathology ; Retrospective Studies ; Tomography, Optical Coherence/*methods ; Visual Acuity ; Young Adult

This paper is aimed to fulfill a prototype system used to classify and retrieve retinal image automatically. With the content-based image retrieval (CBIR) technology, a method to represent the retinal characteristics mixing the fundus image color (gray) histogram with bright, dark region features and other local comprehensive information was proposed. The method uses kernel principal component analysis (KPCA) to further extract nonlinear features and dimensionality reduced. It also puts forward a measurement method using support vector machine (SVM) on KPCA weighted distance in similarity measure aspect. Testing 300 samples with this prototype system randomly, we obtained the total image number of wrong retrieved 32, and the retrieval rate 89.33%. It showed that the identification rate of the system for retinal image was high.
Algorithms ; Fundus Oculi ; Humans ; Image Processing, Computer-Assisted ; methods ; Information Storage and Retrieval ; methods ; Numerical Analysis, Computer-Assisted ; Ophthalmoscopy ; standards ; Pattern Recognition, Automated ; methods ; Retina ; pathology ; Retinal Vessels ; pathology

Aicardi syndrome is a rare neurodevelopmental disease characterised by congenital chorioretinal lacunae, corpus callosum dysgenesis, seizures, polymicrogyria, cerebral callosum, chorioretinopathy and electroencephalogram abnormality. We present a case of Aicardi syndrome with callosal hypogenesis in a 4.5-month-old baby who presented with infantile spasms. Ophthalmoscopy revealed chorioretinal lacunae. The clinical and magnetic resonance imaging features were diagnostic of Aicardi syndrome.
Agenesis of Corpus Callosum ; diagnosis ; Aicardi Syndrome ; diagnosis ; Brain ; diagnostic imaging ; pathology ; Choroid ; abnormalities ; Cornea ; physiopathology ; Female ; Humans ; Infant ; Magnetic Resonance Imaging ; methods ; Malformations of Cortical Development ; diagnosis ; Ophthalmoscopy ; methods ; Radiography ; Retina ; abnormalities ; Spasms, Infantile ; diagnosis

PURPOSE: Bell's phenomenon (BP), which may disturb screening examinations for retinopathy of prematurity (ROP), is known to present infrequently in premature babies. Stress associated with the examinations can influence expression of BP. The authors of the present study evaluated BP during examinations for ROP. METHODS: The present study included 102 eyes of 51 premature babies. Expression of BP was assessed at 3 steps of the examination in the following order: after insertion of a speculum, after illumination of an indirect ophthalmoscope and after scleral depression. The relationship between the expression of BP and the gestational age at the examination was analyzed in each step of the examination. RESULTS: The frequency of BP after the speculum insertion and the illumination was 77% to 92% in infants 32 weeks of age or younger, and decreased significantly to 16% to 57% in infants 42 weeks of age or older (p < 0.005). BP after the scleral depression had no significant association with the gestational age. Frequency of BP increased significantly as the steps of the examination proceeded (p < 0.01). CONCLUSIONS: BP was frequent in premature infants during ROP examination in spite of neurological immaturity. The examiner should take BP into consideration, which frequently occurs in younger infants.
Gestational Age ; Humans ; Incidence ; Infant, Newborn ; *Infant, Premature ; Korea/epidemiology ; Mass Screening/adverse effects/*methods/psychology ; Ophthalmoscopy/*psychology ; Retinopathy of Prematurity/*diagnosis/epidemiology ; Retrospective Studies ; Risk Factors ; Stress, Psychological/*epidemiology/etiology ; Vision Screening/adverse effects/*psychology

We investigated the case of a young man with blurred vision in his left eye. His visual acuity was slightly decreased, and ophthalmoscopy disclosed a gray-white lesion in the macula. He had no systemic or ocular history. On the visual field test, the threshold sensitivity was decreased in the corresponding region. Spectral domain optical coherence tomography (OCT) demonstrated a disruption in the photoreceptor inner and outer segment (IS/OS) junction and undulation of the retinal pigment epithelium (RPE) with backscattering. We re-examined the patient after two weeks and after three months without any treatment. Visual acuity and visual field results were gradually normalized, and OCT demonstrated the recovery of continuity in the photoreceptor IS/OS junction, as well as decreased RPE irregularity with minimal backscattering. We used spectral domain OCT instead of time domain OCT (OCT3) so that we could provide better image resolution of the acute retinal pigment epitheliitis (ARPE). Finally, we observed recovery of the functional and anatomical changes in the ARPE patient with a resolution of the condition within three months following the initial examination, using OCT and visual field tests.
Adolescent ; Humans ; Macula Lutea/pathology ; Male ; Ophthalmoscopy ; Recovery of Function ; Retinal Pigment Epithelium/*pathology/physiopathology ; Retinitis/*pathology/physiopathology ; Time Factors ; *Tomography, Optical Coherence/methods ; Visual Acuity ; Visual Fields

PURPOSE: To evaluate the efficacy of primary chemotherapy combined with local therapy in the treatment of retinoblastomas not treatable with a single therapeutic method. METHODS: We performed a retrospective chart review of 227 patients diagnosed with retinoblastoma. Sixty-five eyes in 52 patients had tumors not treatable with a single therapeutic method and received primary chemotherapy combined with local therapy as needed. RESULTS: Tumor control and eye salvage was achieved in 34 of the 65 eyes; the probability of ocular survival was 46.56% using the Kaplan-Meier method. Forty-three of the 65 eyes were group D or E tumors, in which tumor control and eye salvage was achieved in 16 eyes. Twenty eyes were treated with chemotherapy only, while 28 eyes received one additional modality of local therapy, and 17 eyes received two modalities of local therapy. Of the eyes treated with chemotherapy only, tumor control was achieved in 5 eyes. CONCLUSIONS: Primary chemotherapy combined with local therapy can be effective and safe in the treatment of retinoblastomas otherwise untreatable with other therapeutic methods, such as group D and E retinoblastomas. More vigorous treatment with more local therapeutic methods combined may yield even better results.
Antineoplastic Agents/*therapeutic use ; Child, Preschool ; Cryotherapy/*methods ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hyperthermia, Induced/*methods ; Infant ; Infant, Newborn ; Laser Coagulation/*methods ; Male ; Ophthalmoscopy ; Retinal Neoplasms/diagnosis/physiopathology/*therapy ; Retinoblastoma/diagnosis/physiopathology/*therapy ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome ; *Visual Acuity

PURPOSE: This study was designed to evaluate the efficacy of fluid-gas exchange for the treatment of postvitrectomy retinal detachment. METHODS: We retrospectively reviewed the records of 33 consecutive patients (35 eyes) who underwent fluid-gas exchange treatment for postvitrectomy retinal detachment using the two-needle pars plana approach technique. RESULTS: The retinal reattachment rate was 80.0% after complete intravitreal gas disappearance following the fluid-gas exchange; the overall success rate was 65.7%. Visual acuity was improved or stable in 80.0% of cases; a two-line or greater vision improvement or a best-corrected visual acuity of 0.4 or better occurred in 62.9% of cases. The success rates for superior retinal detachments and posterior pole retinal detachments were 76.5% and 85.7%, respectively. CONCLUSIONS: Fluid-gas exchange represents a simple and cost-effective alternative outpatient procedure for retinal reattachment without reoperation for the treatment of superior and posterior pole retinal detachments.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Fluorocarbons/*administration & dosage ; Follow-Up Studies ; Humans ; Injections ; Laser Coagulation/methods ; Male ; Middle Aged ; Ophthalmoscopy ; Postoperative Care/*methods ; Retinal Detachment/diagnosis/etiology/*therapy ; Retrospective Studies ; Sulfur Hexafluoride/*administration & dosage ; Time Factors ; Treatment Outcome ; Visual Acuity ; Vitrectomy/*adverse effects ; Vitreous Body ; Young Adult