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Sara Pålsson,1,2 Claes Schuborg,2,3 Bertil Sterner,1,2 Marita Andersson Grönlund,1,4 Madeleine Zetterberg1,2 1Department of Clinical Neuroscience/Ophthalmology, Institute of Neuroscience and Physiology, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; 2Region Västra Götaland, Sahlgrenska University Hospital, Department of Ophthalmology, Mölndal, Sweden; 3Guldhedskliniken, Gothenburg, Sweden; 4Department of Ophthalmology, Faculty of Medicine and Health, Örebro University, Örebro, SwedenCorrespondence: Madeleine Zetterberg, Sahlgrenska University Hospital, Department of Ophthalmology, Mölndal, SE 431 80, Sweden, Tel +46 31 33 43 31 50, Fax +46 31 41 29 04, Email madeleine.zetterberg@vgregion.sePurpose: To compare inflammatory response, visual acuity, and complications of two intraocular lenses (IOLs) in patients with and without uveitis.Setting: Tertiary referral centre at Sahlgrenska University Hospital/Mölndal, Sweden.Design: Prospective randomised controlled trial.Patients and Methods: Patients with and without uveitis eligible for cataract surgery were randomised to receive a hydrophobic or a hydrophilic square-edged intraocular lens (IOL). Patients undergoing bilateral surgery received a hydrophobic IOL in one eye and a hydrophilic in the other. Visual acuity, flare, and central foveal thickness were used as outcome measures.Results: In total, 34 (61%) patients (52 eyes) with uveitis and 22 (39%) non-uveitic patients (38 eyes) were included in the study. Comparable corrected distance visual acuity (CDVA) was seen, regardless of IOL material. Flare, six months postoperatively, for those undergoing bilateral surgery, showed no significant difference between eyes receiving a hydrophilic IOL or a hydrophobic IOL; mean difference was − 3.2 (SD ± 20.7) ph/ms between eyes with uveitis (p = 0.53) and − 0.6 SD ± 7.5 ph/ms between eyes without uveitis (p = 0.77). No significant difference in cystoid macular edema (CME) was seen for uveitic patients receiving a hydrophobic IOL (n = 2; 8.0%) and those receiving a hydrophilic IOL (n = 6; 22.2%; p = 0.25).Conclusion: No significant differences in postoperative inflammatory reaction or rate of CME were seen regardless of using a hydrophobic or a hydrophilic IOL. In general, an improvement in CDVA was seen after cataract surgery with both types of IOLs. Thus, the present study did not support either of the IOL materials as advantageous in patients with uveitis.Plain Language Summary: Uveitis, an inflammatory eye condition, often leads to patients needing cataract surgery. The surgery involves removing the eye’s clouded lens and typically replacing it with an artificial lens known as an intraocular lens. Improvement in vision and possible complications vary depending on the type of replacement lens used. This randomised controlled trial compares inflammation, improvement in vision, and possible complications in patients that received two differing types of intraocular lenses. Improvement in vision was seen in both lens types yet neither lens type was superior in terms of inflammation.Keywords: biocompatibility, cataract, complications, implant, inflammation

Purpose: It is well established that children born very preterm are at increased risk for ocular abnormalities including altered foveal morphology. However, little is known about how children born moderate-to-late preterm (MLP), gestational age 32 + 0-36 + 6 weeks, are affected later in life. This study investigates foveal avascular zone (FAZ) area, vascular density (VD), central foveal thickness (CFT) and ganglion cell layer thickness (GCL++) of adolescents born MLP without history of retinopathy of prematurity and compare the results with full-term controls., Methods: In a prospective population-based cohort study, 50 adolescents (26 girls; mean age 16.5 years) born MLP were examined with optical coherence tomography (OCT) and OCT angiography (OCT-A). FAZ and VD were obtained from OCT-A scans and adjusted for ocular magnification. CFT and GCL++ were obtained from the OCT scans. The results from the MLP individuals were compared with the results from 49 healthy full-term controls (29 girls; mean age 16.7 years)., Results: The results showed statistically significant differences in FAZ area (0.22 vs 0.28 mm 2 ; p = 0.0032) and CFT (198.1 vs 187.1 μm; p = 0.0010) between the MLP group and controls. Strong correlations between FAZ area and CFT (r = -0.773, p < 0.0001) and GCL++ (r = -0.924, p < 0.0001) were found in the MLP group. There were no differences in VD., Conclusions: Our results show that adolescents born MLP have a smaller FAZ area and an increased CFT compared with full-term controls. These results indicate that adolescents born MLP have similar parafoveal changes as children born extreme or very preterm., (© 2024 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Aim: To evaluate health- and vision-related quality of life (HR- and VR-QoL) and perceptual visual dysfunction (PVD) in adolescents with hydrocephalus surgically treated in infancy., Methods: In total, 23 adolescents (15 males and 8 females; median age 14.9 years) with hydrocephalus and 31 controls were evaluated using validated instruments to measure HR-QoL and VR-QoL. PVDs were reported by history taking in five areas: recognition, orientation, depth, movement and simultaneous perception., Results: Adolescents with hydrocephalus and the parent proxy reports showed lower mean total Paediatric Quality of Life Inventory 4.0 scores (75.8 and 63.7, respectively) compared with controls (87.6 and 91.5), p = 0.016 and p < 0.0001. Parent-reported scores were lower than self-reported scores (p = 0.001). Adolescents with myelomeningocele (n = 10) showed lower physical health scores (p = 0.001). No VR-QoL difference was found between groups. PVDs were reported in ≥1 area by 14/23 hydrocephalus participants and 2/31 controls (p < 0.0001). Associations were found in the hydrocephalus group between VR-QoL and HR-QoL (r s  = 0.47, p = 0.026) and number of PVD areas (r s  = -0.6, p = 0.003)., Conclusion: Adolescents with hydrocephalus and their parents reported lower HR-QoL and more PVDs. These problems indicate the need for not only ophthalmological follow-ups but also evaluation of QoL and PVDs in individuals with infantile hydrocephalus., (© 2024 The Author(s). Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.)

Purpose: To map the morphology of the retina and optic disc in adolescents with surgically treated hydrocephalus (HC) in infancy and to compare the results with healthy controls., Methods: The study comprised 26 adolescents (16 male, mean age 15 years) with HC and 31 sex- and aged-matched controls. The following optical coherence tomography (OCT) parameters were obtained: macular retinal thickness (MRT) and volume (MRV), thickness of the macular and peripapillary retinal nerve fibre layer (ppRNFL), and area of the optic disc, cup and rim., Results: The MRT was thinner amongst those with HC compared with controls (right eye (RE) 262.2 ± 15.3 and 275.8 ± 15.1 μm; p = 0.0051), and the MRV was smaller (RE 7.37 ± 0.36 and 7.83 ± 0.35 mm 3 ; p = 0.0002). The HC group showed a thinner ppRNFL (RE 88.3 ± 14.9 and 103.5 ± 8.1 μm; p = 0.0002) but a thicker central macular RNFL (RE 11.6 ± 10.4 and 2.07 ± 3.00 μm; p = <0.0001) and foveal minimum (RE 211.1 ± 32.0 and 186.3 ± 15.9 μm; p = 0.0013). Optic disc variables showed no difference between groups. Correlations were found in the HC group between best corrected visual acuity (expressed in logMAR) and ppRNFL (RE r = -0.56, p = 0.018), and disc area (RE r = -0.52, p = 0.033)., Conclusion: Thinner ppRNFL and MRT and smaller MRV were found in adolescents with surgically treated HC in infancy compared with controls. In contrast, the central macular RNFL and foveal minimum were thicker. Further studies are required to evaluate the diagnostic value of OCT to indicate increased intracranial pressure timely and follow-up in individuals with surgically treated HC., (© 2022 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

To review and evaluate the surgical outcomes of the Fells-modified Harada-Ito procedure using a dosage scale approach with long-term follow up in patients with torsional diplopia. The records of patients who underwent the modified Harada-Ito procedure by the same surgeon during 2012-2019 were retrospectively reviewed regarding pre- and post-operative data and individual dose-scale used for the surgery. The modified Harada-Ito procedure involved advancing the anterior half of the superior oblique tendon toward the inferior edge of the lateral rectus muscle by a distance determined using a five-graded scale. A total of 27 patients (mean age 57.6 years, range, 22-81 years; 10 female) were included. Evaluating surgical outcome showed a significant difference in pre- to post-operative cyclodeviation (p = <0.001). Pre-operative mean extorsion was -10.4° and mean torsional correction achieved was 7.7°. The dose-effect relationship showed a wide spread effect, yet yielded a high success rate. All but two patients were symptom free from their torsional diplopia at the last post-operative evaluation, on average 24 months after surgery. Post-operative results and the dose-effect of the modified Harada-Ito corresponded with the aimed-for correction of torsional diplopia.. Fusion evaluation and individually based pre-operative assessments proved essential in determining individual doses for successful surgical outcomes.

Purpose: Congenital anophthalmia (A) and microphthalmia (M) are rare developmental defects, which could be isolated or syndromic. Our objective was to describe a cohort of children and young adults with A/M treated with ocular prosthesis, emphasizing clinical features, diagnosis, treatment, and follow-up., Methods: Eighteen individuals (10 female) with unilateral A (n = 3) and M (n = 15) with a mean age of 9.5 years (range 0.8-31.8) and treated with ocular prosthesis were included. Data on medical history, clinical examinations and management of ocular prosthesis were collected. Genetic screening with microarray and whole-exome sequencing targeting 121 A/M-related genes was performed., Results: A/M appeared isolated (seven cases) or as part of a syndromic condition (11 cases). In 4/16 patients, mutations were detected in TFAP2A, CHD7, FOXE3 and BCOR-genes. In one patient, a possibly causal microdeletion 10q11 was shown. Associated ocular anomalies such as cataract and cysts were found in 16 (89%) of the A/M eyes, and in nine (50%) ophthalmological findings were found in the fellow eyes. The median ages at which the conformer and ocular prosthesis first were initiated were 7.8 months and 1.5 years. 16/17 patients fulfilled satisfactory orbital growth and cosmetic results when treated with ocular prosthesis from an early age., Conclusion: Based upon our findings, a multidisciplinary approach, including genetic assessment, is necessary to cover all aspects of A/M. Imaging, ultrasound and visual evoked potentials should be included. Early management is crucial for the outcome, in terms of non-ocular findings, vision in the fellow eye, and for facial cosmetic development., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate growth, blood pressure and ophthalmological status in preschool children born preterm to mothers with preeclampsia., Methods: In a prospective cohort study, 78 children (34 girls) born preterm without retinopathy of prematurity were examined regarding length/height, weight, head circumference and insulin-like growth factor I (IGF-I) at birth and at 5 years of age. At 5 years, IGF-binding protein 3 and blood pressure were also measured. A detailed ophthalmological examination including ocular dimensions, fundus morphology, visual fields, visual evoked potentials and perceptual visual dysfunction was performed. Children born to preeclamptic mothers (n = 24) were compared to children with non-preeclamptic mothers (n = 54)., Results: Children exposed to preeclampsia had lower weight (p = 0.0002, mean difference -1.46, 95% CI -2.09; -0.83), length (p = 0.013, -1.10, 95% CI -1.92; -0.23) and IGF-I levels (p = 0.0002, -26.0, 95% CI -36.0; -16.1) at birth compared to non-exposed children. At 5 years of age, the preeclamptic group had larger optic cup areas (p = 0.0006, 0.32, 95% CI 0.15; 0.46, in right eye, p = 0.049, 0.18, 95% CI 0.001; 0.35, in left eye). There was no significant difference between the groups regarding other ophthalmological findings or blood pressure. Children with reduced eye motility had lower neonatal IGF-I levels (p = 0.033, 15.5, 95% CI 1.1; 30.3)., Conclusion: Preeclampsia was shown to affect growth and IGF-I levels, confirming previous studies. Children exposed to preeclampsia were shown to have larger optic cup areas. Furthermore, lower neonatal IGF-I levels were seen in preterm children with reduced eye motility at 5 years of age., (© 2020 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To evaluate health-related quality of life (HR-QoL), vision-related (VR-)QoL and perceptual visual dysfunction (PVD) among individuals with anophthalmia (A) and microphthalmia (M) treated with ocular prosthesis., Methods: The study comprised 15 individuals (mean age 6.6 years; range 1.7-14.1) with unilateral A or M. Three validated instruments measuring HR-QoL and VR-QoL were used: The Pediatric QoL Inventory (PedsQL), consisting of physical and psychosocial self-report and parent-proxy report (2-18 years); Children's Visual Function Questionnaire (CVFQ); and Effects of Youngsters' Eyesight on Quality of Life (EYE-Q). Perceptual visual dysfunctions (PVDs) were assessed by history taking according to a specific protocol., Results: A/M children and their parents showed low HR-QoL scores (PedsQL total score: 66.3; 69.6) compared with controls (83.0; 87.61) (p = 0.0035 and <0.0001, respectively, unpaired t-test). No differences were found between A/M children and parents, but parents tended to underestimate their children's emotional state. A/M children with subnormal visual acuity (VA) for age scored lower in physical health compared with A/M children with normal VA (p = 0.03, Mann-Whitney U-test). No significant VR-QoL differences between A/M children and references or between A/M children with subnormal or normal VA for age were found. More A/M children than controls exhibited PVDs in ≥1 area (7/11 versus 4/118; p < 0.0001, Fisher's exact test)., Conclusion: A/M individuals show poor HR-QoL and increased PVDs. No difference in QoL was found between children and parents, though the children tended to score lower in emotional well-being. A/M children with subnormal VA showed lower physical health score. These problems indicate the necessity of a thorough multidisciplinary assessment and follow-up of children with A/M., (© 2020 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To investigate normative subjective cyclotorsion values and cyclofusion ranges in a healthy adult population., Methods: A cross-sectional investigation was performed in 120 healthy, non-strabismic adults, 60 men and 60 women in the age range of 18-69 years. All subjects were assessed for cyclotorsion using the synoptophore and the single Maddox rod (SMR) methods. Cyclofusion was investigated with the synoptophore in 60 of the subjects., Results: All age groups showed low values of subjective torsion, mainly excyclotorsion with mean values of -1 degree for both methods. Reference ranges of cyclotorsion were between -0.7 and -1.5 degrees for the SMR method and between -0.7 and -1.4 degrees using the synoptophore method. There were no significant differences between gender (p = 0.48), but the effect of age was significant for both methods (p = 0.026) demonstrating a slight increase in excyclotorsion with age. Cyclofusion showed a total mean amplitude of 16 degrees, the fusion range was +7 degrees of incyclotorsion to -9 degrees of excyclotorsion., Conclusions: Subjective reference ranges for cyclotorsion and cyclofusion reveal that low values of torsion are to be expected upon clinical investigation in non-strabismic adult individuals. Values outside of the reference range may be indicators of possible binocular abnormalities or physiological variations. The cyclotorsional measurements and prevalence in this adult population group can be regarded as normative data in clinical settings., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To determine the prevalence of strabismus among Danish adults and to find the frequency of history of strabismus and strabismus surgery. Furthermore, to evaluate the prevalence of strabismus-associated amblyopia among participants with strabismus and to relate the results to the current national vision screening programme., Methods: In total, 3785 adults in the Danish Rural Eye Study underwent an interview regarding eye health, visual acuity measurement, Hirschberg test and retinal photography. Participants were categorized into groups based on their birth date in relation to the introduction of the national vision screening programme., Results: In total, the prevalence of strabismus was 1.1% (41/3785; 95% CI: 0.8-1.5); no differences were found in relation to gender or screening status. The prevalence of exotropia (XT) was 0.3% (12/3785; 95% CI: 0.2-0.6) and of esotropia (ET) 0.8% (29/3785; 95% CI: 0.5-1.1), resulting in an XT:ET ratio of 1:2.7. A history of strabismus was present in 4.6% (174/3785; 95% CI: 4.0-5.3), and a history of strabismus surgery was present in 0.8% (32/3785; 95% CI: 0.6-1.2) of the participants. Among participants with manifest strabismus, 24% had strabismus-associated amblyopia., Conclusion: In this first European population-based study of strabismus prevalence in adults, the prevalence was similar to three out of five previous studies using cover test. The use of Hirschberg test may have led to an underestimation of the true prevalence. The prevalence was neither related to screening status nor gender. Our results are the first to display a predominance of ET among Caucasian Scandinavian adults., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To examine visual acuity, refraction and ocular status before, during and after hyperbaric oxygen therapy (HBOT)., Methods: Twenty-nine patients underwent 40 standard protocol HBOT treatments. In all subjects, refraction and best corrected visual acuity were measured at baseline and after ten, 20, 30 and 40 treatments, and at a 12-week follow-up. A subgroup (n = 19) were given additional examinations at baseline, after 40 treatments and at follow-up including measuring central corneal thickness (CCT), corneal curvature, anterior chamber depth, lens thickness, axial length, fundus morphology, blood pressure and intraocular pressure (IOP). Lens colour and opalescence were graded using the Lens Opacities Classification System III (LOCS III)., Results: Myopic shifts [≥0.5 dioptre (D) spherical equivalent (SE)] occurred in 45 (77.6%) eyes. Median refractive changes of -0.75 D SE (right eye; p < 0.001) and -0.66 D SE (left eye; p < 0.001) were observed between pretreatment and treatment end (Wilcoxon signed rank test). Refraction returned to baseline at follow-up, except for a small persisting change towards myopia, median -0.25 D SE (left eye; p = 0.01). Using the LOCS III, median increases in lens nuclear colour, of 0.6 (right eye; p < 0.001) and 0.7 (left eye; p < 0.001), and opalescence of 0.7 (both eyes; p = 0.01) were found at the last examination. Small reductions were noted in CCT of -6.00 μm (right eye; p = 0.03) and -4.00 μm (left eye; p = 0.03), and IOP of -1.50 mmHg (left eye; p = 0.01)., Conclusions: The transient myopic shift may have been due to lenticular refractive index changes. Reduced lens transparency is a potential consequence of HBOT., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To study visual function and ocular fundus morphology in relation to growth, metabolic status, and blood pressure in moderate-to-late preterm (MLP) children at 10 years of age., Design: Prospective cohort study., Methods: In this population-based observational study, nonsyndromic MLP children born in Gothenburg, Sweden, were examined neonatally in the years 2002-2003 concerning length, weight, head circumference, and insulin-like growth factor I (IGF-I). At 10 years of age, 33 children (10 girls) were examined regarding previously mentioned variables, and regarding visual acuity, refraction, fundus morphology, IGF binding protein 3, leptin, adiponectin, and blood pressure. An age- and sex-matched control group consisted of 28 children (9 girls)., Results: Myopia was more commonly found in MLP children than in controls (P = .004, 95% CI 1.8 to 49.8). The MLP group had smaller optic disc area (P = .01, 95% CI -0.5 to -0.1), smaller rim area (P = .001, 95% CI -0.5 to -0.2), fewer branching points (P = .0001, 95% CI -5.7 to -2.1), and higher index of tortuosity of arteries (P = .03, 95% CI 0.002 to 0.03) and veins (P = .02, 95% CI 0.003 to 0.02). Refraction correlated with IGF-I (P = .0005, r s  = 0.60 in right eye, and P = .002, r s  = 0.55 in left eye) at 10 years of age. Tortuosity of arteries at assessment correlated with neonatal IGF-I levels (P = .03, r s  = -0.39). Tortuosity of veins correlated with a leptin/adiponectin ratio at assessment (P = .04, r s  = 0.37)., Conclusion: The findings suggest that being born MLP is associated with myopia, smaller optic disc and rim areas, and abnormal retinal vascularization. Furthermore, metabolic status and growth factors seem to have an impact on ocular development., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To describe clinical features, risk factors and complications in a cohort of Swedish children with juvenile idiopathic arthritis (JIA) screened for uveitis between 2002 and 2011., Methods: Medical records of 299 children with JIA (93 male, 206 female; median age 5.0 years at diagnosis) were retrospectively scrutinized focusing on subtype of JIA, onset of arthritis/uveitis, presence of antinuclear antibodies (ANA) and ophthalmological status., Results: Uveitis was found in 32 (11%) children, 78% bilaterally affected. The median age of arthritis onset in children who developed uveitis was 2.5 years (range 1-10) versus 5.0 years (range 1-15) in those who did not. Sex ratio was 3.5:1 (girl:boy). The most prevalent JIA subtype was oligoarthritis (75%). All but one child with uveitis was found to be ANA (+). The median interval between diagnosis of arthritis and uveitis was 12 months. Only one child developed uveitis between the fourth and fifth years after arthritis onset. Ocular complications were recorded in 45.6% (26/57 affected eyes) at last follow-up. On univariate analysis, both young age at arthritis onset and ANA positivity were possible predictors for developing uveitis, but on multivariate analysis, the latter was the most important predictor (HR 16.25, 95%; CI 2.19-120.44; p = 0.006, Cox regression analysis)., Conclusion: Almost all of the children developing JIA-associated uveitis did so within 4 years after arthritis onset, a fact that accentuates the importance of early initiation of ophthalmological screening and more frequent regular follow-ups during the first 4 years. The most important predictor for developing uveitis was ANA positivity., (© 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate the outcome of cataract surgery in adult patients with uveitis., Materials and Methods: In this retrospective cohort study, medical charts of patients with uveitis and matched control patients without uveitis who underwent cataract surgery at the Eye Clinic, Sahlgrenska University Hospital, Mölndal, between January 2005 and December 2009 were analyzed., Results: The study included 58 eyes with and 283 eyes without uveitis. The most common etiologies were idiopathic anterior uveitis and Fuchs' heterochromic uveitis. Postoperative visual acuity at 4 weeks was ≥0.5 decimal (0.3 logMAR) in 48 eyes with uveitis (87.3%) compared to 180 non-uveitic eyes (86.1%). Four eyes with uveitis (7.1%) and one eye without uveitis (0.5%) developed postoperative intraocular hypertension/glaucoma. Posterior capsule opacification developed in 11 eyes (19.0%) with and 28 eyes (12.4%) without uveitis., Conclusion: With appropriate perioperative anti-inflammatory regimen and surveillance, modern cataract surgery using phacoemulsification and primary intraocular lens implantation can be performed in patients with uveitis without greater risk of complications, yielding similar visual outcome as in patients without uveitis., Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Purpose: To study ocular morphology and visual function in relation to general growth in moderate-to-late preterm (MLP) children., Methods: Visual acuity (VA), refraction, optic disc parameters, biometric values and anthropometric measures were obtained from 50 eight-year-old MLP and 43 full-term children. Macular parameters were examined by optical coherence tomography. Serum insulin-like growth factor I (IGF-I) levels were analysed at birth and at assessment and delta IGF-I was calculated., Results: Total macular volume was significantly less in MLP than in controls (both eyes p < 0.01). Macular volume correlated with head circumference (HCF) at assessment [right eye (RE) p = 0.002, r = 0.67; left eyes (LE) p = 0.01, r = 0.54] and refraction (both eyes p < 0.05, r = 0.4) in the MLP children. Furthermore, central retinal thickness correlated significantly with delta IGF-I (RE p = 0.03, r = -0.51, LE p = 0.006, r = -0.59) and refraction (both eyes p < 0.01, r = 0.5) and optic disc areas correlated with weight and height at assessment (all p < 0.05, r = 0.4). Total axial length correlated with HCF at assessment (both eyes p < 0.01, r = 0.5) and VA logMAR (both eyes p < 0.02, r = -0.4)., Conclusions: Macular volume was significantly less in MLP children than in controls examined at 8 years of age. General growth of children and IGF-I levels seem to be involved in development of ocular growth and morphology., (© 2016 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To establish if cyclotorsion measurements obtained in a patient population using different methods of testing give equivalent results, and if the results from each test are repeatable over time., Methods: Twenty referrals, from the orthoptic waiting list at Sahlgrenska University Hospital, Mölndal, Sweden, with vertical deviation stated as the primary reason for referral, were examined. Subjective cyclotorsion measurements using three different methods were performed. The methods included two standard tests: a synoptophore, with slides A17a/18a, the single Maddox rod test (SMR), and a new test method: the KMScreen method. Measurements were repeated 3 times for fixing either eye, and on two separate test occasions, for repeatability within each of the methods., Results: During the first test measurements, 11 (55%) showed excyclotorsion measured with the synoptophore method, 18 (90%) with the SMR test, and 15 (75%) with the KMScreen method. During the second set of measurements, 11 (55%) showed excyclotorsion with the synoptophore method, 17 (85%) with the SMR test, and 10 (50%) with the KMScreen test. Median perceived torsional measurements at the first visit for the synoptophore, SMR, and KMScreen tests were -1°, -5°, and -1°, respectively. There were significant differences between the synoptophore and SMR (P=0.025), and the SMR and KMScreen (P=0.025), but not between the synoptophore and KMScreen (P=0.90) methods on measuring the affected eye. All three tests showed repeatable values. The 95% limits of agreement (LoAs) between visits, defined as mean torsional difference ± 1.96 SD of difference, for the synoptophore, SMR, and KMScreen methods were -0.5° ± 4.5°, -0.3° ± 5°, and -0.7° ± 3.5°, respectively. P values for differences between visits were P=0.45, P=0.75, and P=0.90, respectively, using an exact permutation test., Conclusion: In this small study group, the three different methods used for testing cyclotorsion showed significantly different results. However, all methods showed significant repeatability (test-retest reliability) over time. This finding is of clinical importance when investigating cyclotorsion in patients. Clinicians need to be aware of the differences between methods when monitoring recovery or changes in patients, and in particular when planning surgical correction for cyclotorsion.

Aim: The aim of this study was to study long-term visual outcome and cataract development in children and adolescents after hematopoietic stem cell transplantation (HSCT) in childhood., Methods: Best corrected visual acuity (BCVA), refraction and lens status were examined in a prospective study of 139 children and adolescents., Results: In total, 139 patients (58 female), median age 6.6 years at HSCT (range 0.4-17.5 years), were followed up for a median of 8.0 years (1-19.4 years). Median BCVA in the better eye was 1.0 decimal. Altogether 19 of 131 patients developed cataract requiring surgery, while 46 developed less prominent lens opacities and 66 had clear lenses at time of latest follow-up. Patients conditioned with total body irradiation had a higher risk of developing lens opacities or cataract (p < 0.0001) as did patients with malignant disease, irrespective of irradiation treatment (p < 0.0001). Cumulative analysis showed that 50% of all patients had developed lens opacities/cataract after 10.2 years. Patients who ultimately needed cataract surgery developed cataract earlier than others (p = 0.006)., Conclusion: Lens opacities or cataract were more common in children or adolescents with malignant disease and after conditioning with irradiation. Regular ophthalmological follow-up is important after HSCT for early intervention to avoid amblyopia., (©2015 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Purpose: To evaluate ophthalmological findings in preschool children born moderate-to- late preterm (MLP) and relate the findings to auxological data at birth and at 5.5 years of age., Methods: Seventy-eight MLP children [gestational age (GA) 32-36 weeks; 34 girls; mean age 5.7 years] were investigated. Gestational age, weight, length and head circumference at birth and at the time of assessment were registered. Visual acuity (VA), refraction, orthoptic evaluation, slit-lamp examination and ophthalmoscopy were conducted, and a history of visuoperceptual problems was recorded. The data were compared with age- and sex-matched controls born full term (n = 35)., Results: Ophthalmological abnormalities were noted in 82% of MLP children and 47% of controls (p = 0.0004). There was a significant difference with regard to impaired motility (p = 0.03), heterophoria at distance (p = 0.006) and refraction expressed as spherical equivalent dioptre (p = 0.01). Amongst auxological data at birth, birthweight (BW) was the strongest predictor to ophthalmological abnormalities (p = 0.0003). In a stepwise logistic regression, GA was the strongest predictor of VA outcome at time of assessment (p = 0.0036). Moderate-to-late preterm birth showed a 2.4-fold increased risk of refractive errors compared with full-term children (RR 2.39: 95% CI 1.10-5.20; p = 0.02)., Conclusion: Based on our findings, MLP birth may be associated with increased ocular morbidity compared with their full-term counterparts. Auxological data at birth, especially BW, seems to be an important factor when conducting an ophthalmological diagnosis in preschool MLP children, and an increased VA was correlated to a higher GA., (© 2015 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Anophthalmia/microphthalmia (A/M) are rare congenital eye malformations. Early intervention with ocular prosthesis can stimulate orbital growth and prevent facial asymmetry. We reviewed medical records from 18 individuals with A/M (0.8-31 years) treated with ocular prosthesis at Sahlgrenska University Hospital between 2000 and 2012. A majority had other ocular findings. Seven had subnormal visual acuity in the fellow eye, one third were in contact with vision support services and half of the group wore glasses. Eleven individuals had extra-ocular findings such as cardiac defect, hearing impairment and neuropsychiatric disorders, possibly indicating syndromic conditions. We suggest that investigation of A/M children should include ultrasound of the eye, optionally visual evoked potential and magnetic resonance imaging of the CNS. The ophthalmologist should initiate treatment with prosthesis, pediatric assessment, hearing tests and genetic counseling, but should also monitor visual development of the fellow eye.

Purpose: To evaluate the outcome of combined cataract surgery with primary intraocular lens (IOL) implantation and pars plana vitrectomy (PPV) in children with uveitis., Methods: Data regarding visual acuity (VA), inflammatory status, medical therapy, and complications was collected from the medical charts of 17 children (21 eyes) with chronic uveitis who underwent combined cataract surgery and PPV at the Eye Clinic, Sahlgrenska/Mölndal, between 2002 and 2011., Results: Seventy-six percent of the children had juvenile idiopathic arthritis. Median preoperative VA was 1.70 logMAR and median VA after 12 months was 0.17 logMAR. Postoperatively, glaucoma developed in 7 eyes, cystoid macular edema in 3 eyes, and visual axis opacification requiring treatment in 5 eyes., Conclusions: Although combined phacoemulsification, primary IOL implantation, and PPV in children with uveitis resulted in favorable visual outcome and stable inflammation in a majority of children, the technique should so far be reserved for uveitic cases with vitreous pathology.

We report a mutation in the anticodon of the tRNA(Arg) gene (m.10437 G>A), resulting in an anticodon swap from GCU to ACU, which is the anticodon of tRNA(Trp), in a boy with mitochondrial encephalomyopathy. Enzyme histochemical analysis of muscle tissue and biochemical analysis of isolated muscle mitochondria demonstrated cytochrome c oxidase (COX) deficiency. Restriction fragment length polymorphism analysis showed that 90% of muscle and 82% of urinary epithelium mtDNA harbored the mutation. The mutation was not identified in blood, fibroblasts, hair roots, or buccal epithelial cells and it was absent in the asymptomatic mother, suggesting that it was a de novo mutation. Single-fiber PCR analysis showed that the proportion of mutated mtDNA correlated with enzyme histochemical COX deficiency. This mutation adds to the three previously described disease-causing mutations in tRNA(Arg), but it is the first mutation occurring in the anticodon of tRNA(Arg).

Objectives: The purposes of this investigation were to determine the frequencies of and associations between different neurodevelopmental disorders and to study the potential lasting effects of alcohol on children adopted from eastern Europe., Methods: In a population-based, prospective, observational, multidisciplinary, cross-sectional, cohort study of 71 children adopted from eastern Europe, children were assessed 5 years after adoption, from pediatric, neuropsychological, and ophthalmologic perspectives., Results: Fetal alcohol spectrum disorders, that is, fetal alcohol syndrome (FAS), partial FAS, and alcohol-related neurodevelopmental disorders, were identified for 52% of children; FAS was found for 30%, partial FAS for 14%, and alcohol-related neurodevelopmental disorders for 9%. Alcohol-related birth defects were found for 11% of children, all of whom also were diagnosed as having FAS. Mental retardation or significant cognitive impairment was found for 23% of children, autism for 9%, attention-deficit/hyperactivity disorder for 51%, and developmental coordination disorder for 34%., Conclusions: Fetal alcohol spectrum disorders and neurodevelopmental disorders were common in this long-term follow-up study of children adopted from orphanages in eastern Europe. Maternal alcohol consumption during pregnancy has long-lasting adverse effects, causing structural, behavioral, and cognitive damage despite a radically improved environment.

Purpose: This study aimed to evaluate and relate visual function, ocular dimensions and neuropaediatric findings in adoptees from Eastern Europe., Methods: We studied 72 of 99 children, born during 1990-95 and adopted from Eastern Europe to western Sweden during 1993-97. The children (mean age 7.5 years, range 4.8-10.5 years; 41 boys, 31 girls) were examined after a mean period of 5 years post-adoption by a multidisciplinary team. Correlations between ophthalmological findings and neuropaediatric data were analysed., Results: Bivariate and regression analyses indicate a significant positive correlation between visual acuity (VA) and perceptual organization (p < 0.001), as well as between strabismus and verbal comprehension (p < 0.02). Fetal alcohol syndrome (FAS) was correlated with low VA (p < 0.02), subnormal stereovision (p < 0.009) and small optic discs (p < 0.02). Small head circumference was related to low VA (p < 0.015) and small optic discs (p < 0.03). Furthermore, small optic discs were related to low birthweight (p < 0.005) and preterm birth (p < 0.01). Large optic cups were correlated with poorer perceptual organization (p < 0.02)., Conclusions: In this group of adoptees from Eastern Europe, ophthalmological findings were correlated to neuropaediatric findings, especially those arising from prenatal adverse events resulting in growth deficiency and central nervous system damage. Therefore, it is important and valuable with an ophthalmological examination in children adopted from Eastern Europe.

Purpose: This study aimed to evaluate visual function in children with attention-deficit/hyperactivity disorder (ADHD), to correlate these data with the morphology of the optic nerve, and to find out if and how psychostimulant medication affects visual functions., Methods: The visual acuity (VA) and visual fields (VFs) of 18 children with ADHD (two girls and 16 boys), aged 6-17 years, were examined before and after treatment with psychostimulants. A control group, consisting of 24 children (nine girls and 15 boys), aged 7-18 years, were examined twice to evaluate the repeatability of the tests and the learning effect. Fundus photographs were analysed by digital planimetry., Results: Visual acuity increased significantly (p = 0.0039) in the ADHD group after treatment. The difference between the two VF examinations was significantly larger in the ADHD group compared with the control group (p = 0.036). Significantly more ADHD subjects had subnormal VF results without stimulants, compared with controls (p = 0.0043), but with stimulants the difference was no longer significant., Conclusions: Children with ADHD showed better VA and VF results with than without psychostimulant medication.

Design: A population-based study of pre-adoption, arrival and post-adoption health., Aim: To report prenatal and postnatal background factors, morbidity, growth and development in adoptees from Eastern Europe., Subjects and Methods: All 99 children born in Eastern Europe between 1990 and 1995 and adopted to western Sweden during 1993-1997 were invited to participate in the study. Altogether, 76 (77%) participated. Medical records from the birth countries, from the examination at arrival and from medical reports made during a mean post-adoption period of 5 years were evaluated., Results: Low birth weight (< or = 2500 g) occurred in 48%. Congenital malformations were found in 22%. The biological mothers of 33% of the children had been considered alcoholics, and 16% of the children's mothers had been diagnosed with a psychiatric disability. A high incidence of infectious diseases, neurodevelopment disorders and growth retardation had been noted during the pre-adoption period. Upon arrival in Sweden 75% were diagnosed with a medical condition, most often an infection. After a 5-year post-adoption period, small head circumference was associated with alcohol exposure during pregnancy and 46% had at least one neurodevelopment or behavioural disorder., Conclusion: Adverse prenatal and perinatal factors, congenital malformations and post-adoption neurodevelopment disorders were common. Adoptees and adopters have complex needs for health support and information.

Background: The aim of the study was to evaluate congenital malformations and functional anomalies in the offspring of Swedish parents with thalidomide embryopathy (TE)., Methods: Sixty-four children (29 girls, 35 boys) with ages ranging from 0-18 years, born to 34 Swedish parents (14 women, 20 men) with TE, were studied. Data on malformations and dysfunction were collected from medical records at maternity and child healthcare units, delivery units, hospitals, outpatient clinics and schools., Results: Five children had both a mother and father with TE, 23 had a mother suffering from TE, and in 36 children the father had TE. One girl had a major malformation consisting of pulmonary stenosis, and single cases of minor physical features and positional deformities were observed. One boy had autism. Four children were born preterm, all to a TE mother. One child died within 24 hr after birth. Seven spontaneous abortions were registered, five of them in TE mothers. The cesarian section rate was 39% among the TE mothers, compared to 14% among the non-TE mothers., Conclusions: Malformations or functional anomalies similar to those typical for TE were not found in this group of children born to Swedish parents with TE. Cesarian sections were more frequently performed in TE mothers, partly because of pelvic and uterine malformations., (Copyright 2002 Wiley-Liss, Inc.)