Bilateral Best-corrected Visual Acuity Research Articles

Birth prevalence and characteristics of congenital corneal opacities.

To report the birth prevalence and natural history of congenital corneal opacities among a population-based cohort of children. The medical records of patients <5 years diagnosed with a congenital onset corneal opacity while residing in Olmsted County, Minnesota, from January 1, 1977, through December 31, 2016, were retrospectively reviewed. Fourteen patients were diagnosed with a congenital corneal opacity during the 40-year study period for a birth prevalence of 1 in 5188 live births. The mean age at diagnosis was 7.5 months (range 0-48 months) and 9 (64.3%) were males. Four patients had congenital glaucoma, 4 had limbal dermoids, 2 had sclerocornea, and 1 patient each had Descemet's tear from birth trauma, herpes simplex virus type 1 keratitis, corneal leukoma, and an undiagnosed scar. Six (42.8%) patients required treatment for their underlying corneal opacity including the four patients with congenital glaucoma. The other 8 (57.1%) patients had a clear central axis. Four (28.6%) of 14 patients required amblyopia therapy, and 4 (28.6%) developed strabismus. Four (28.6%) patients had associated systemic conditions. During a mean follow up of 5.4 years (range 1.3-27.0 years), the median best corrected visual acuity (BCVA) was logmar 0.16 (20/25) (range 20/20-hand motion) with one patient with unilateral BCVA less than 20/60 and one patient with bilateral BCVA less than 20/60. In this 40-year cohort, congenital corneal opacities were relatively rare and the result of a variety of disorders. Although amblyopia and strabismus occurred commonly, most patients had good visual outcomes.

Validation of a novel iPhone application for evaluating near functional visual acuity

Monitoring dynamic changes in near vision is important for early detection of presbyopia. This study assessed the accuracy and reliability of a new smartphone-based application, the Smart Vision Check (SVC), compared with those of a conventional device (AS-28; Kowa, Aichi, Japan), for measuring near functional visual acuity (NFVA). We enrolled 115 healthy volunteers aged ≥ 20 years with bilateral best-corrected visual acuity of ≥ 20/25. The SVC was designed for use on an Apple iPhone SE2 to measure NFVA by tapping on the orientation icon manually. Conventional FVA was measured using the AS-28 with − 2.50 D added to the best distance correction at baseline. There was no significant difference in NFVA-related measurements between the AS-28 and SVC (P > 0.05). The Spearman correlation coefficients of NFVA measurements between the two devices were over 0.60 (P < 0.001). The Bland–Altman plot indicated minimal bias with limits of agreements of ± 0.34 logMAR for NFVA with habitual correction when comparing the AS-28 and SVC. The intraclass correlation coefficient of the repeated SVC-measured NFVA was 0.915 (95% CI 0.800–0.969). In summary, the SVC has the potential to evaluate NFVA in a relatively easy manner. Applied clinically, the SVC can be useful for presbyopia screening.

Spontaneous functional changes in specific cerebral regions in patients with hypertensive retinopathy: a resting-state functional magnetic resonance imaging study.

This study investigated functional alterations in the cerebral network of patients with hypertensive retinopathy (HR) by resting-state functional magnetic resonance imaging (rs-fMRI) and degree centrality (DC) methods. 31 patients with HR along with 31 healthy controls (HC) closely matched in gender and age were enrolled for the research. All participants were examined by rs-fMRI, and the DC method was applied to evaluate alterations in spontaneous cerebral activity between the 2 groups. We used the independent samples t test to evaluate demographic and general information differences between HR patients and HCs. The 2-sample t test was used to compare the DC values of different cerebral regions between the 2 groups. The accuracy of differential diagnostic HR was analyzed by receiver operating characteristic (ROC) curve method for rs-fMRI DC values changes. Pearson’s correlation coefficient was applied to determine the correlation between differences in DC in specific cerebral areas and clinical manifestation. Results showed that DC values were higher in the left cerebellum posterior lobe (LCPL), left medial occipital gyrus (LMOG), and bilateral precuneus (BP) of HR patients compared to HCs. Mean DC values were lower in the right medial frontal gyrus/bilateral anterior cingulate cortex of HR patients. Anxiety and depression scores were positively correlated with DC values of LMOG and LCPL, respectively. Bilateral best-corrected visual acuity in HR patients was negatively correlated with the DC value of BP. Hence, changes in DC in specific cerebral areas of patients with HR reflect functional alterations that provide insight into the pathophysiologic mechanisms of HR.

Mommy, why can’t I see clearly?

Ectopia lentis or crystalline lens subluxation is one of the major criteria to diagnose Marfan syndrome. It may vary from mild lens subluxation to lens dislocation. Herewith is a case report of a 4-year-old autistic boy who had never been diagnosed with Marfan syndrome. He presented to the clinic after his parents noticed he had difficulty focusing on near objects. His bilateral best-corrected visual acuity was 6/60. On examination, there was bilateral lens subluxation superotemporally and lens equator blocking his visual axis. He was sent to the paediatric team and further Marfan workout showed dilated aortic root. He was then diagnosed with Marfan syndrome. He underwent bilateral lens aspiration, anterior vitrectomy, and iris-claw lens implantation. His postoperative bilateral visual acuity on day 1 was 6/30 and his best-corrected visual acuity 3 months after surgery was 6/9 for both eyes. In conclusion, ophthalmologists play an important role in diagnosing and managing Marfan syndrome. Early diagnosis is important to help preserve vision and improve quality of life.

Macular Telangiectasia Type 2: Visual Acuity, Disease End Stage, and the MacTel Area: MacTel Project Report Number 8

To report the visual acuity measures from the macular telangiectasia type 2 (MacTel) registry and to investigate and describe phenotypic findings in eyes with substantial vision loss resulting from MacTel. Cross-sectional multicenter study. Participants in the MacTel Natural History Observation Registration Study. Best-corrected visual acuity (BCVA) data, retinal imaging data, and clinical data were accessed from the MacTel Study databases in May2019. Frequency distribution of BCVA and its relationship to age; morphologic changes in eyes with very late disease stages, defined by a BCVA of 20/200 or worse; average retinal thickness of macular subfields on OCT; and dimensions of the area affected by MacTel (i.e., the MacTel area). Best-corrected visual acuity was 20/50 or worse in 37.3% and 20/200 or worse in 3.8% of 4449 eyes of 2248 patients; 18.4% and 0.7% of all patients showed bilateral BCVA of 20/50 or worse and 20/200 or worse, respectively. Asymmetry between right and left eyes was present (median BCVA, 71 letters vs. 74 letters), a finding supported by more advanced morphologic changes in right eyes. Participant age correlated with BCVA, but the effect size was small. If a neovascularization or macular hole were present, bilateral occurrence was frequent (33% or 17%, respectively), and BCVA was better than 20/200 (79% or 78%, respectively) or 20/50 or better (26% or 13%, respectively). Eyes with advanced disease (BCVA, ≤20/200) showed the following characteristics: (1) atrophy of the foveal photoreceptor layer with or without associated subretinal fibrosis; (2) an affected area, termed MacTel area, limited to a horizontal diameter not exceeding the distance between the temporal optic disc margin and foveal center, and the vertical diameter not exceeding approximately 0.8 times this distance (exceptions were eyes with large active or inactive neovascular membranes); (3) reduced retinal thickness measures within the MacTel area; and (4) less frequent retinal greying and more frequent hyperpigmentations compared with eyes that have better BCVA. Severe vision loss is rare in MacTel and is related to photoreceptor atrophy in most people. Results indicate disease asymmetry with slightly worse vision and more advanced disease manifestation in right eyes. MacTel-related neurodegeneration does not spread beyond the limits of the MacTel area.

Photoreceptor assessment in focal laser-treated central serous chorioretinopathy using adaptive optics and fundus autofluorescence.

This study analyzed cone density, cone mosaic, and fundus autofluorescence (FAF) images in patients with focal laser-treated central serous chorioretinopathy (CSC).Observational case series.Forty-two eyes of 21 patients with unilateral treated CSC and bilateral best-corrected visual acuity of 1.0 (decimal fraction) were included. FAF and cone mosaic images were obtained in all patients with an adaptive optics fundus camera. Densities were recorded at 20 points throughout the macula, and choroidal thicknesses were measured.Mean choroidal thicknesses were 419.95 ± 110.33 μm in normal eyes, 459.09 ± 90.07 μm in eyes with active CSC, and 438.61 ± 107.57 μm in treated eyes. The highest density of cones in healthy eyes was 38146 cones/mm2, with a 5.66-μm intercellular space (IS), at 700 μm temporal to the center. In eyes with treated CSC, the highest density was 32749 cones/mm2, with a 6.13-μm IS, at 500 μm nasal to the center. In all quadrants, median values of maximum cone density were significantly higher in healthy eyes (P = .02, P = .003, P = .0001, and P = .001). Three types of lesions were identified on FAF and were correlated with those on cone mosaic images. Strong correlations were detected between the presence of hypoautofluorescent lesions on the first FAF image and a greater difference between maximum values of photoreceptor density (r 2 = 0.46, P = .03), as well as between the presence of hypoautofluorescent lesions and the duration of pathology (r 2 = 0.68, P < .001).The presence of hypoautofluorescent lesions and the duration of pathology were negative prognostic factors in CSC. Laser treatment could prevent photoreceptor loss.

The impact of vision screening in preschool children on visual function in the Swedish adult population.

Amblyopia is the most common monocular visual impairment in Scandinavia. A visual screening programme for preschool children was introduced in Sweden in the 1970s to reduce the prevalence of amblyopia. The purpose of this study was to investigate the impact of this visual screening programme in adult men recruited to the armed forces in Sweden. The prevalence of amblyopia was compared in recruits born in 1956-1957 (n=1500), before the introduction of the visual screening programme (unscreened), and those born in 1977-1979 (n=2626), after the visual screening programme was introduced (screened). Amblyopia was defined as bilateral, if the bilateral best corrected visual acuity (BCVA) was <0.5 (20/40), and unilateral if there was a ≥2-line interocular difference and BCVA was <0.625 (20/32) in the worse eye. Subgroup analysis was performed on recruits with hyperopia, myopia and anisometropia. The prevalence of amblyopia was significantly lower after the introduction of the Swedish visual screening programme. Forty-seven (3.3%) of the unscreened and 23 (0.9%) of the screened recruits had unilateral amblyopia (p<0.0001, OR=0.26 (95%CI 0.16-0.43)). The risk for developing amblyopia was markedly reduced by screening in recruits with hyperopia (≥2D) (p<0.0001 and OR 0.034 (95% CI 0.003-0.207)) and anisometropia (≥1.5D interocular difference) (p<0.01 and OR 0.20 (95% CI 0.08-0.66)). Only a few recruits with myopia had amblyopia. The results demonstrate the value of the Swedish visual screening programme for preschool children in preventing amblyopia. Anisometropia or high hyperopic refractive errors can be identified by screening and corrected to prevent amblyopia.

Long-term outcome of bilateral surgery in infant with congenital cataract using different approaches

Objective: To explore the best surgical approach for infant with congenital cataract by comparing long-term follow-up results of bilateral congenital cataract surgery by different approaches conducted during infant age at last time of visit. Methods: This retrospective case control study includes 108 eyes of 54 infants with bilateral congenital cataract who received bilateral cataract triple procedure (vitrector lensectomy, posterior capsulotomy and anterior vitrectomy) performed by a single surgeon, by either anterior or posterior approach, followed by refractive correction by aphakic glasses or intraocular lens (if suitable)during the follow-up periods (at least for 3 years), between January 2002 and February 2013 in Department of Ophthalmology, Peking University People's Hospital. The unilateral and bilateral best corrected visual acuity (BCVA), intraocular pressure, anterior segment and fundus exam of each visit and post-operative complications were evaluated, and data collected at patients' last visit were compared between two approaches (Student's t test for continuous variables, chi-squeare test for constitutional variables). Results: A total of 44 cases (33 males) were included in anterior approach group, median age of primary surgery was 3.0 (0.5-10.0) months. The rest 10 cases (7 males) were included in the posterior approach group, median age of primary surgery was 4.0 (1.0-11.0) months. At last visit, longest follow up period of anterior and posterior group were 3.5 (3.0-8.5) years and 4.0 (3.0-7.0) years, respectively. Neither unilateral nor bilateral BCVA (logarithm of minimum angle of resolution) of both anterior and posterior approach groups at last visit showed any difference(both P>0.05).Long-term post-operation complications of anterior and posterior group include strabismus (8 cases and 2 cases), nystagmus(8 cases and 6 cases), optical opaque (4 cases and 2 cases), and ocular hypertension(2 cases and 1 case). Nystagmus (P<0.001) and optical opaque (P=0.041) was more prevalent in posterior approach group. Conclusion: For bilateral congenital cataract surgery during infant age, anterior approach would produce less post-operation complications as compared to posterior approach, and is recommended for infants without other abnormalities of posterior segment of the eye. (Chin J Ophthalmol, 2019, 55:46-50).

Severe ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review

BackgroundGranulomatosis with polyangiitis (GPA), a necrotizing granulomatous disease, very rarely involves the central nervous system (CNS), particularly the pituitary. Delayed treatment may cause permanent bilateral blindness. We report an isolated case of pituitary GPA that manifested as a progressive bilateral temporal visual field (VF) defect and was diagnosed via pituitary biopsy. Additionally, we review ocular, chiasmal and cranial nerve involvement in pituitary GPA.Case presentationA 20-year-old Chinese man was referred for repeated fever, sudden headache, diplopia with a bilateral best-corrected visual acuity (BCVA) of 10/20, ptosis in both eyes and restricted abduction on the right side. VF tests showed bitemporal hemianopsia. Laboratory tests revealed hypothyroidism and were negative for autoimmune markers. Enhanced magnetic resonance imaging (MRI) showed pituitary enlargement. The diagnosis was lymphocytic pituitaritis. After intravenous (IV) dexamethasone treatment, full recovery occurred within 2 months. Two years later, the patient was readmitted for headache recurrence. With oral prednisone, the visual acuity in his right eye rapidly decreased to hand motion (HM) within one month. Enhanced MRI showed pituitary enlargement and a new, invasive suprasellar CNS lesion. All infection- and autoimmune-related tests were negative. The visual acuity in his right and left eye decreased to no light perception (NLP) after 6 days and 2 weeks, respectively. The biopsy results suggested GPA. After IV methylprednisolone treatment, complete remission of the symptoms occurred and was confirmed by MRI. The 15-month follow-up showed no signs of recurrence.ConclusionGPA typically affects the respiratory tract, lungs and kidneys. To date, 50 cases with pituitary involvement have been reported. Chiasmal and cranial nerve involvement leading to visual acuity impairment are common. We found 2 cases with severe visual loss resembling our case and discuss certain similarities.

Ocular findings in Noonan syndrome: a retrospective cohort study of 105 patients

The aim of this retrospective study is to describe ocular findings in a large Noonan syndrome cohort and to detect associations between ocular features and genetic mutations that were not found in earlier studies. We collected ophthalmological and genetic data of 105 patients (median age, 12 years; range, 0–60 years) clinically diagnosed as Noonan syndrome. The ocular findings were linked to the genotypes. All patients with Noonan syndrome showed multiple abnormalities in the categories of vision and refraction, external ocular features, ocular alignment and motility, anterior ocular segment, and posterior ocular segment. In total, 50 patients have NS due to a mutation in PTPN11. Permanent visual impairment (bilateral best-corrected visual acuity < 0.3) was found in 7 patients, including patients with a mutation in RAF1, SHOC2, and KRAS. Keratoconus was found in 2 PTPN11 positive patients, and prominent corneal nerves were observed in a patient with a SOS1 mutation.Conclusions: This study shows an overview of ocular abnormalities in Noonan syndrome, including permanent visual impairment caused by binocular optic nerve abnormalities and nystagmus. Delay in ophthalmological diagnosis is still present, also in patients with visual impairment. All Noonan syndrome patients should have a complete ophthalmological examination at the time of diagnosis.What is Known:• Although we discover more pathogenic mutations in patients with Noonan syndrome, Noonan syndrome still is a clinical diagnosis• Ocular features of Noonan syndrome are characterized by developmental anomalies of the eyelids and associated with other ocular abnormalities in childhood (including refractive errors, strabismus and amblyopia).What is New:• There seems to be a delay in the ophthalmological diagnosis and awareness of the broad variety ofophthalmological features including refractive errors and visual impairment in Noonan syndrome is needed. All children should have a full ophthalmological examination at the time of diagnosis.• Permanent visual impairment (best-corrected visual acuity < 0.3) is found in patients with mutations in RAF1, SHOC2, and KRAS and the cause is probably a developmental disorder of the optic nerves.

Outcomes of bilateral sequential implantation of the Boston keratoprosthesis type 1

ObjectiveTo evaluate the risks and benefits of sequential bilateral Boston type 1 keratoprosthesis (KPro) implantation for bilateral corneal blindness. DesignComparative retrospective study. ParticipantsPatients who underwent a sequential bilateral KPro surgery. MethodsAll 11 patients who underwent sequential bilateral KPro surgery at the Centre Hospitalier de l’Université de Montréal between October 2008 and October 2011 were recruited. Data from the 22 patient eyes in our study were separated in 2 groups based on the first (group 1) or second eye (group 2) implanted with the Kpro. Both groups were then compared for the best corrected visual acuity (BCVA) and complications. Bilateral BCVA following both surgeries and the overall number of complications that affected each patient after both interventions were also analyzed. ResultsAt 24 months, BCVA was 20/150 in group 1 and 20/200 in group 2 (p = 0.67). Throughout the study, there was no significant difference in BCVA between the 2 groups (p > 0.05), and bilateral BCVA was similar. The complication rate was comparable in the 2 groups. Patients had a mean of 2.45 complications following the first surgery and a mean of 5.27 in both eyes combined after the second intervention (p = 0.03). ConclusionsBecause visual gain observed following the second surgery was the same as that with the first and multiple surgeries increased the number of complications for each patient, we recommend saving bilateral KPro implantation for cases in which the first eye with KPro develops a disease limiting its visual potential.

A Pink Conjunctival Lesion.

Keywords: Conjunctival neoplasms; Granuloma, pyogenic.

Medical and Surgical Treatment in Pediatric Orbital Myositis Associated with Coxsackie Virus.

Purpose. To report a case of orbital myositis associated with Coxsackie virus and its medical and surgical approach. Methods. Complete ophthalmological examination and imaging and analytical investigation were performed. Results. A 6-year-old male presented with subacute painless binocular horizontal diplopia. Examination revealed bilateral best-corrected visual acuity (BCVA) of 20/20 and right eye 45-prism-dioptre (PD) esotropia in near and distance fixations, with no motility restrictions. Serologic screening was positive for Coxsackie virus acute infection and computerized tomography (CT) suggested right eye medial rectus orbital myositis. An oral corticosteroid 1.0 mg/kg/day regimen was started. A new CT after two months showed symmetrical lesions in both medial rectus muscles. Corticosteroids were increased to 1.5 mg/kg/day. After imagiological resolution on the 4th month, alternating 45 PD esotropia persisted. Bilateral 7 mm medial rectus recession was performed after 1 year without spontaneous recovery. At 1-year follow-up, the patient is orthophoric with 200′′ stereopsis and bilateral 20/20 BCVA. Conclusions. To our knowledge, this is the first reported case of orbital myositis associated with Coxsackie virus. This is also the first reported case of isolated strabismus surgery after orbital myositis in pediatric age, highlighting the favourable aesthetic and functional outcomes even in cases of late ocular motility disorders.

Further Genetic and Clinical Insights of Posterior Polymorphous Corneal Dystrophy 3

Posterior polymorphous corneal dystrophy (PPCD) is a very rare disorder characterized by primary changes of the posterior corneal layers. Sequence variants in 3 genes are associated with the development of PPCD, including ZEB1 that is responsible for PPCD3. Evidence suggests at least 1 more gene remains to be identified. To determine the molecular genetic cause of PPCD3. We performed extensive ophthalmological examination, including rotating Scheimpflug imaging technology and specular microscopy, and direct sequencing of the ZEB1 coding region. Comprehensive review of published PPCD3-causing variants was undertaken. Ophthalmology department of a university hospital. Four Czech probands. Results of ophthalmological examination and direct sequencing of the ZEB1 coding region. The following 2 novel frameshift mutations within ZEB1 were identified: c.2617dup in exon 8 in a 22-year-old woman, considered to be most likely de novo in origin, and c.698dup in exon 6 in a 20-year-old man. The first patient had mild changes consistent with PPCD and bilateral best-corrected visual acuity of 1.00. The corneal phenotype of the patient in the second case was more severe, with best-corrected visual acuity of 0.40 OD and 0.05 OS. Corneas of both probands were abnormally steep (keratometry readings, flat ≥ 47.4 diopters [D] and steep ≥ 49.2 D) with increased pachymetry values but no pattern indicative of keratoconus. Specular microscopy in both patients revealed reduced endothelial cell density (range, 1055/mm² to 1655/mm²). Both probands had a history of surgery for inguinal hernia; the male patient also reported hydrocele. Nucleotide changes within the coding region of ZEB1 underlie the pathogenesis of PPCD in 4 of 23 Czech probands (17%). The cumulative de novo ZEB1 mutation rate is at least 14%. Possible involvement of ZEB1 sequence variants not readily identified by direct sequencing of coding regions needs to be further investigated. Our findings also have implications for patient counseling.

Comparative Study of Two Silicone Hydrogel Contact Lenses Used as Bandage Contact Lenses after LASEK

To evaluate two silicone hydrogel contact lens materials as continuous-wear bandage contact lenses after laser-assisted subepithelial keratomileusis (LASEK). The inclusion criteria were myopia of -6.00 diopters (D) or less, astigmatism lower than -1.5 D, bilateral best-corrected visual acuity of 20/20 or better, and candidates for bilateral LASEK. The patients were randomized to be fitted with a Balafilcon A (PureVision) lens in one eye and Galyfilcon A (Acuvue Advance) in the fellow eye. Uncorrected visual acuity, corneal epithelial status, conjunctival and limbal hyperemia, lens movement, contact lens debris, and the responses to a subjective-comfort questionnaire were assessed postoperatively. The parameters were evaluated before and 1 and 5 days after the procedure. A paired Student's t-test and chi-square tests were used when appropriate. We analyzed 44 eyes of 22 consecutive patients who underwent LASEK to correct low-to-moderate myopia. The mean spherical equivalent was -3.25 +/- 2.36 D. There was no difference in conjunctival or limbal hyperemia, lens movement, uncorrected visual acuity, or epithelial healing between the two lenses at any visit. A significant difference was found in lens deposition and discomfort, which were greater with the PureVision lens 5 days postoperatively (p = 0.01 for both comparisons). PureVision and Acuvue Advance contact lenses seem to be useful as continuous wear bandage contact lenses. There is no major clinical difference between the lenses, although patients report greater comfort with Acuvue Advance.