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  • 1. Ceynowa, Dylan J.
    et al.
    Wickström, Ronny
    Olsson, Monica
    Ek, Ulla
    Stockholms universitet, Samhällsvetenskapliga fakulteten, Specialpedagogiska institutionen.
    Eriksson, Urban
    Kristoffersen Wiberg, Maria
    Tear Fahnehjelm, Kristina
    Morning Glory Disc Anomaly in childhood - a population-based study2015Inngår i: Acta Ophthalmologica, ISSN 1755-375X, E-ISSN 1755-3768, Vol. 93, nr 7, s. 626-634Artikkel i tidsskrift (Fagfellevurdert)
    Abstract [en]

    Purpose: To report prevalence, ocular characteristics and coexisting neurological, behavioural, somatic and neuroradiological abnormalities in children and adolescents with morning glory disc anomaly (MGDA).

    Methods: In a cross-sectional population-based study, 12 patients with MGDA, aged 2-20years, were identified. All 12 agreed to ophthalmological assessments including visual functions, refraction, fundus photography, optical coherence tomography (OCT) and ocular motor score (OMS). Neurological examinations and behavioural/developmental screening were carried out. Data from previous or new neuroradiological investigations were collected.

    Results: The prevalence of MGDA was 2.6/100000. MGDA was unilateral in 11/12 patients with a best-corrected visual acuity (BCVA) in the MGDA eye ranging from hand motion to 0.65 (median 0.06). Severe microphthalmus prevented unilaterality to be determined in one adolescent. All patients had a binocular BCVA of 0.5. OMS showed abnormalities in pupil response, vestibulo-ocular reflex, stereo visual acuity, strabismus and convergence. OCT revealed peripapillary or macular oedema in 5/8 patients and foveal aplasia in 3/8 patients. Three patients had extensive capillary hemangiomas, of which one had PHACES syndrome and one had additional cerebrovascular anomalies and corpus callosum agenesis. Neuroradiology showed craniovascular anomalies in two patients. Neurology was mostly normal. Behavioural/developmental screening showed attention deficit hyperactivity disorder in one patient.

    Conclusions: The prevalence data, previously not reported, of morning glory disc anomaly was 2.6/100 000. Coexisting retinal peripapillary or macular oedema was common, as were cerebral abnormalities and/or cutaneous vascular malformations. The associated findings may not be discovered through routine ophthalmological examination why OCT and neuroimaging are called for.

  • 2. Granstam, Elisabet
    et al.
    Rosenblad, Andreas
    Stockholms universitet, Samhällsvetenskapliga fakulteten, Statistiska institutionen. Uppsala University, Sweden.
    Modher Raghib, Aseel
    Granström, Therese
    Eriksson, Jan W.
    Lindholm Olinder, Anna
    Leksell, Janeth
    Long-term follow-up of antivascular endothelial growth factor treatment for diabetic macular oedema: a four-year real-world study2020Inngår i: Acta Ophthalmologica, ISSN 1755-375X, E-ISSN 1755-3768, Vol. 98, nr 4, s. 360-367Artikkel i tidsskrift (Fagfellevurdert)
    Abstract [en]

    Purpose: To evaluate visual acuity (VA) and central retinal thickness (CRT) on optical coherence tomography during a 4-year period in patients treated for sight-threatening diabetic macular oedema (DMO) at two Swedish county hospitals. Additionally, to compare health-related quality of life and subjective visual functioning before and after 4 years of treatment.

    Methods: Fifty-eight patients with DMO were evaluated after 4 years of antivascular endothelial growth factor (VEGF) treatment. VA, CRT and clinical data were retrospectively reviewed. Health-related quality of life and subjective visual functioning were evaluated with Short Form Health Survey (SF-36) and National Eye Institute Vision Functioning Questionnaire 25 (VFQ-25). Comparisons between independent groups were performed using Pearson's chi(2) test, Fisher's exact test or Mann-Whitney U test. Spearman's rho was used for correlation analyses. Wilcoxon signed-rank test was used for comparison between dependent groups. Logistic regression analysis was applied for analysis of VA and CRT over 4 years.

    Results: Follow-up data were obtained from 37 of 58 (63.8%) patients. Baseline characteristics were similar, regardless of follow-up. VA improvement at 1 year (mean + 4.4, SD 7.5; ETDRS letter score) was maintained over 3 years, then declined. CRT was reduced throughout the study. In the first treatment year, eyes received 5.1 (1.4) anti-VEGF injections, followed by approximately two injections yearly. Additional treatment included laser and dexamethasone implants. SF-36 showed no change at 4 years, compared with baseline. VFQ-25 demonstrated improvement in near vision activities (p = 0.036).

    Conclusion: Significant long-term improvement in visual function was present in patients with anti-VEGF-treated DMO.

  • 3. Skriapa Manta, Athanasia
    et al.
    Olsson, Monica
    Ek, Ulla
    Stockholms universitet, Samhällsvetenskapliga fakulteten, Specialpedagogiska institutionen.
    Wickström, Ronny
    Teär Fahnehjelm, Kristina
    Optic Disc Coloboma in children - prevalence, clinical characteristics and associated morbidity2019Inngår i: Acta Ophthalmologica, ISSN 1755-375X, E-ISSN 1755-3768, Vol. 97, nr 5, s. 478-485Artikkel i tidsskrift (Fagfellevurdert)
    Abstract [en]

    Purpose: The aim of the study was to report prevalence, ocular outcome, neurological characteristics, cognitive and behavioural problems in children with optic disc coloboma (ODC).

    Methods: This was a population-based, cross-sectional study of 31 children between 2 and 18 years of age diagnosed with ODC. The children were part of a larger cohort of 184 children with congenital optic disc malformations. Clinical ophthalmological examinations, neurological assessments, behavioural and developmental screening were performed.

    Results: The prevalence of ODC was 8.9/100 000 children. Of the 31 patients, 18 had unilateral ODC (p = 0.21). The best-corrected visual acuity (BCVA) in the ODC eye ranged from blindness to 1.3 (median 0.3). BCVA was 0.82 in eyes with an isolated ODC (range 0.4-1.3) and 0.15 (range 0-0.5) in eyes with concurrent macular involvement (p < 0.0001). Nystagmus was observed more often in patients with bilateral ODC (9/13 versus 3/17, p = 0.004). Two patients had retinal detachment. Behavioural/psychological screening was performed in 21 patients with severe deficits identified in six cases. Intellectual disability was present in seven patients. Neurological dysfunction was diagnosed in 8/22 cases. All of the above children had already systemic diagnoses before the ODC diagnosis was made.

    Conclusions: ODC was the second most common optic disc malformation in this cohort after optic nerve hypoplasia. The children had a wide range of ocular comorbidity. An isolated ODC without macular involvement was not associated with profound vision loss. The ability of screening in the regular child care centres to diagnose extraocular comorbidities was very good and referral to a paediatrician appears redundant in cases of normal development.

  • 4. Tear Fahnehjelm, Kristina
    et al.
    Dahl, Sara
    Martin, Lene
    Ek, Ulla
    Stockholms universitet, Samhällsvetenskapliga fakulteten, Specialpedagogiska institutionen.
    Optic nerve hypoplasia in children and adolescents; prevalence, ocular characteristics and behavioural problems2014Inngår i: Acta Ophthalmologica, ISSN 1755-375X, E-ISSN 1755-3768, Vol. 92, nr 6, s. 563-570Artikkel i tidsskrift (Fagfellevurdert)
    Abstract [en]

    Purpose: To report prevalence, ocular characteristics and coexisting behavioural problems in children and adolescents with optic nerve hypoplasia (ONH), which is a common cause of visual impairment in children in western countries, often associated with neurological or endocrinological problems and where autism has been reported in severe cases with blindness. Methods: This is a population-based cross-sectional study of patients <20 years of age who had been diagnosed with NH and lived in the county of Stockholm in December 2009. Ophthalmological assessments including fundus photographs with optic disc analyses were made. A questionnaire was used to screen for behaviour and development. Results: The prevalence of ONH in all living children <18 years of age in Stockholm was 17.3/100 000 with a prevalence of visual impairment (<0.3) of 3.9/100 000. In total, 66 patients, median age 9.3 years (0.6-19.4), 36 with bilateral and 30 with unilateral ONH, were included in the current study; 53 were re-examined clinically, group A, and 13 agreed to retrospective analyses of existing medical records, group B. Analyses of the optic discs were made in fundus photographs from 53 patients comparing a semi-automated (Retinal Size Tool) and a manual method (Zeki). There was a strong curvilinear correlation (r(S) = 0.91 p < 0.0001 for both eyes). Behavioural problems were more common (p < 0.05) in bilateral ONII. Conclusion: Optic nerve hypoplasia is a common ocular malformation with a prevalence of 17.3/100 000 children and adolescents <18 years of age in Stockholm. Unilateral ONH seems as common as bilateral.

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