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Purpose: The aim of the study was to describe the temporal changes in causes and prevalence of childhood visual impairment in Denmark based on the National Danish Registry of Children with Visual Impairment (NDRCVI)., Methods: Annual reports on the NDRCVI since its establishment in 1979 were reviewed and data on the number of registered children and the causes for registration with a visual impairment were evaluated., Results: The average annual incidence of childhood visual impairment in Denmark is 2.8 per 1000 live-born children and the prevalence of childhood visual impairment is 1.6 per 1000 children <18 years. Today, fewer children are severely visually impaired (visual acuity ≤6/60) at the time of registration (31.6% since 2010 vs. 51.1% in the 1980s). Cerebral visual impairment and optic nerve atrophy have remained common causes of childhood visual impairment whereas sequelae to retinopathy of prematurity have been almost eliminated as a cause. Systemic comorbidities are more common now in children with visual impairment (seen in 63.9% in the last decades vs. 44.6%in the 1980-ties)., Conclusion: Whereas the prevalence of visual impairment has remained relatively stable over the years, the severity of visual impairment has improved, suggesting that more children will be able to live an active life supported by aids compensating vision loss. However, more children have systemic comorbidities in combination with their visual impairment suggesting that children with visual impairment face a life not only limited by the obstacles of poor vision. This calls for multidisciplinary management and support of affected children and families., (© 2024 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Purpose: To investigate ophthalmic onset manifestations and the impact of diagnostic delay on the prognosis in infants (<1 year) diagnosed with a brain tumour., Methods: A retrospective population-based nationwide study of infants diagnosed with a brain tumour between 2007 and 2017 in Denmark. Data was retrieved from the Danish Childhood Cancer Registry, the National Danish Health registries, and medical files. Primary outcome measures included symptoms, clinical findings, time to diagnosis and survival., Results: Thirty-seven infants were diagnosed with a brain tumour in Denmark between 2007 and 2017. In total, 19/37 infants (51%, 95% CI: 34-68) had ophthalmic manifestations at any time prior to or at diagnosis; and in 6/37 (16%, 95% CI: 6-32) ophthalmic manifestations were the initial symptom. The most common ophthalmic manifestations were strabismus (n = 7), sunset eyes (n = 6), nystagmus (n = 4), reduced pupillary light reflex (n = 4), and/or decreased vision (n = 4). The median number of symptoms per infant at the time of diagnosis was three (range 0-9). The median diagnostic delay was 26 days (range 0-283, IQR: 6;90). 5-year survival rate was 75% (95% CI: 61-90) and all children with diagnostic delay > 100 days (n = 9, 24%) were still alive at the end of follow-up (median 6.3 years, range 2.2-10.2)., Conclusion: We provide an overview of symptoms and clinical signs in a nation-wide series of infants with CNS tumours and demonstrate that ophthalmic manifestations are frequently observed in infants prior to diagnosis, but, often in combination with other clinical signs. The diagnostic delay was substantial for a large part of the infants, but this was not associated with increased mortality., (© 2023 The Authors. Acta Ophthalmologica published by John Wiley & Sons Ltd on behalf of Acta Ophthalmologica Scandinavica Foundation.)

Background: We evaluated health care costs in patients with childhood onset visual impairment caused by inherited retinal dystrophies (IRD)., Methods: The IRD cohort, identified from the Danish Registry of Blind and Partially Sighted Children, was compared to age- and sex-matched controls from the national, Danish population registry. Information on health care expenditures for somatic and psychiatric in- and outpatient services, purchase of prescription medications and paid assistance at home were obtained from national registries for the years 2002-2017., Results: We included 412 in the IRD cohort (6,290 person years) and 1656 (25,088 person years) in the control cohort. Average, annual health care expenditures from age 0-48 years of age were €1,488 (SD 4,711) in the IRD cohort and €1,030 (4,639) in the control cohort. The largest difference was for out-patient eye care (13.26 times greater, 95% confidence interval 12.90-13.64). Psychiatric in-patient expenditures were 1.71 times greater (95% CI 1.66-1.76) in the IRD cohort but psychiatric out-patient health care costs were comparable between groups., Conclusions: Health care costs were approximately 40% greater in the IRD cohort compared to an age- and sex-matched sample from the general Danish population. This is relevant in the current situation with a number of trials aimed at treating IRDs using genetically based therapies. Although eye care expenditures were many times greater, they made up < 10% of the total health care expenditures even in the IRD cohort. The reduced costs related to injuries in the visually impaired cohort was a surprising finding but may reflect a reduced propensity to seek medical care rather than a reduced risk of injuries., (© 2022. The Author(s).)

Purpose: The aim of this study was to provide a population-based estimate on the prevalence of infantile nystagmus and to describe the causes in the Capital Region of Denmark., Methods: Review of medical records of children with infantile nystagmus born in the period 1 January 2010 through 31 December 2017 and living in the Capital Region of Denmark. We used birth registry data from Statistics Denmark and the National Danish Birth Registry to calculate the prevalence of nystagmus in children born at term and prematurely., Results: A total of 103 patients (52 males/51 females) with infantile nystagmus were included. The overall prevalence of infantile nystagmus was 6.1 per 10 000 live births. It was higher in premature children (28.4/10 000 live births) than children born at term (4.4/10 000), p < 0.0001, and highest in children born extremely preterm, (97.3/10 000). The most common cause of infantile nystagmus was ocular disease (44%) followed by idiopathic nystagmus (32%), neurological disorders and genetic syndromes (20%) and prematurity without retinopathy of prematurity as the only cause (4%)., Conclusions: In this study, we provide the prevalence of infantile nystagmus based on national medical records in which all residents are accounted for. Our findings show a prevalence of 6.1 per 10 000 live births but six times higher among children born preterm than born at term. Ocular disease was the leading cause of infantile nystagmus with albinism and ocular malformations as the most frequent. In 1/3 of patients, no cause could be identified., (© 2020 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Purpose: To investigate outcomes of routine vision screening compared to as-indicated ophthalmological investigation of all children born preterm in a Danish region from 1997 to 2014., Methods: All children born preterm (gestation age < 32 weeks or birthweight < 1500 g) screened for retinopathy of prematurity (ROP) were divided into two groups. From 1997 to 2009, only children treated for ROP or referred for visual problems received ophthalmological investigation (as-indicated group). From 2010 to 2014, all ROP-screened infants were offered ophthalmological investigation at 6 months and 3 years of age (screening group)., Results: A total of 560 children were included in the as-indicated period, 41 and 87 were referred for ophthalmological investigation at 6 months and 3 years, respectively. In the screening period, 295 children were included, 251 and 150 of whom underwent vision evaluation at 6 months and 3 years, respectively. Mean visual acuity was 4.1 cycles per degree with Teller acuity cards at 6 months and 0.78 decimal at 3 years. At 3 years, 2.7%(n = 11) in the as-indicated versus 3.5%(n = 10) screening group had visual acuity < 6/18 (p = 0.24). Cerebral palsy (n = 28) and epilepsy (n = 5) were significantly related to vision impairment (p = 0.001/0.006), while treated ROP was not (n = 13). Refractive error was common at 3 years (61%), especially astigmatism (50%). Gestational age, birthweight and ROP were not associated with vision impairment or refractive error., Conclusion: Screening preterm children at 6 months and 3 years did not reveal more visually impaired children compared to examination when indicated., (© 2019 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

In this case study, we investigate a child presenting with patent ductus arteriosus, short philtrum, duck-bill lips, strabismus, a flat nasal bridge, a broad forehead, low-set ears, hypertelorism, up-slanting palpebral fissures, almond-shaped eyes, and hypodontia, all leading to the clinical diagnosis of Char syndrome. Genetic analysis showed heterozygosity for the novel variant c.851T>C, p. Leu284Ser in the TFAP2B gene. Family analysis suggested that at least 20 members, extending six generations back, were affected. All 10 members available for genetic testing were heterozygous for the novel pathogenic variant. Qualitative analysis of the facial dysmorphology in the proband and three of the affected family members using three-dimensional surface scanning showed that the major deviations were observed in the forehead/eyebrow, nose, upper lip, and chin regions with, for example, a flattened nose and reduced height of the upper lip and the face. Furthermore, it is suggested that Char syndrome is associated with disturbances of tooth formation and eruption.

Purpose: Craniopharyngioma often causes visual loss due to the close relation to the anterior visual pathways. This study investigates the incidence and predictors of visual outcomes in patients with craniopharyngioma., Methods: Data from sixty-six patients who underwent surgery for craniopharyngioma from 2009 to 2013 in Denmark were reviewed. Primary outcomes were visual acuity (VA) and visual field (VF) defects from pre-and postoperative visits. Secondary outcomes were optic nerve atrophy (OA) and papilledema., Results: Fifty-eight patients were included. The VA of the patients 1-year after surgery improved by -0.16 log(MAR) (95%CI: -0.30 to -0.02; p = 0.0266). Visual field (VF) defects worsened in 17 eyes (30%), remained stable in 21 eyes (37%) and improved in 19 eyes (33%). The presence of papilledema and the absence of OA were significantly correlated with an improvement in VA postoperatively (p = 0.011 and p = 0.011, respectively). Patients undergoing surgery within a week or less after their first ophthalmological examination had a significant improvement in VA (-0.36; 95%CI: -0.62 to -0.09; p = 0.0099). Patients undergoing surgery using a subfrontal approach also showed improvement in VA (p = 0.048). Tumour recurrence had a significantly worse VA outcome (p = 0.0074)., Conclusion: Patients show a slight improvement in VA 1-year after operation for craniopharyngioma. The presence of papilledema and early surgical intervention is associated with a significant improvement in VA. Early involvement of a dedicated ophthalmologist is recommended to secure an early detection of a visual decline and potential tumour recurrence., (© 2017 Acta Ophthalmologica Scandinavica Foundation. Published by John Wiley & Sons Ltd.)

Objective: The aim of this study was to uncover the most effective and safe criterion to implement for retinopathy of prematurity screening in Denmark., Methods: This retrospective national cohort study is based on data from 3 national registers. These registers provided on infants treated for retinopathy of prematurity, infants in need of treatment but missed by the present screening program, and the candidate neonates for advanced retinopathy of prematurity development A nonlinear logistic regression model was fitted to the data, and various screening criteria were evaluated., Results: During the study period (2002-2006), 116 infants were treated for retinopathy of prematurity, no treatment-demanding retinopathy of prematurity infants were missed by the screening program, and 182 premature infants were candidates for developing treatment-demanding retinopathy of prematurity. Screening criteria combining gestational age at delivery and birth weight limits and new risk-based criteria were compared with regards to their effectiveness. The risk-based criteria were the most effective. Use of the 0.13% risk-based criterion to define the population to be screened resulted in the detection of all treated infants in the study period and 17.4% fewer infants to screen. The model predicted this criterion to result in 1 missed case of treatment-demanding retinopathy of prematurity every 11 years and 1 case of blindness every 18 years in Denmark., Conclusions: Screening criteria based on risk estimates of developing treatment-demanding retinopathy of prematurity are the most effective for retinopathy-of-prematurity screening. The risk-based criterion of 0.13% can safely be implemented for future retinopathy-of-prematurity screening in Denmark.

Introduction: To present our experience with anti-TNFalpha treatment of juvenile idiopatic arthritis (JIA) associated uveitis., Materials and Methods: All 11 children with severe uveitis were monitored between 2001 and 2005. Nine of the children had JIA and a set of twins had a rare hereditary granulomatous disease, Blau's syndrome. The patients were selected and the reason for starting anti-TNFalpha treatment was an insufficient response in the arthritis or uveitis to previous therapy., Results: In all patients the anti-TNFalpha treatment reduced the activity of uveitis. The response to treatment was related to 1) visual acuity and 2) the reduction of systemic immune-suppressing agents. All 11 children are still on anti-TNFalpha treatment., Conclusion: Anti-TNFalpha was effective in treating uveitis in all 11 patients. In patients with active uveitis associated JIA not responding to corticosteroids and methotrexate and with declined vision the trend is to start anti-TNFalpha treatment early in the inflammatory disease to prevent long term complications to the eyes. The results indicate a superiority of infliximab over Eternacept in the treatment of uveitis.

Purpose: To determine the incidence of retinal detachment (RD) after cataract extraction in people 40 years of age or older with axial myopia (i.e., axial length > or = 25.5 mm)., Setting: Fifteen Danish eye clinics., Methods: Two hundred forty-five eyes had cataract extraction performed at 15 eye clinics; 237 eyes had extracapsular cataract extraction (ECCE) and 8 eyes, intracapsular cataract extraction (ICCE). Postoperative data were reported by the practicing ophthalmologists. Mean follow-up was 27 months (range 14 to 32 months)., Results: Five RDs occurred in the 245 eyes (2.0%). Excluding the ICCE cases and the two cases of combined cornea transplantation and ECCE, RD occurred in 4 of the 235 eyes that had ECCE (1.7%). The incidence after ECCE with posterior chamber lens implantation was 1.4%. Complete postoperative status was reported on 158 eyes. Forty-eight eyes (30.4%) had a neodymium:YAG capsulotomy and 3 (6.0%) developed an RD 1, 3.5, and 21 months after the capsulotomy., Conclusion: The RD incidence after ECCE with posterior chamber lens implantation was low but higher than that in unselected populations. The incidence increased after laser capsulotomy.

Purpose: To compare the healing of the cornea and the incidence of infection after traumatic corneal epithelial defect after single treatment with double bandage combined with either Fucithalmic single unit dose eye drops or chloramphenicol eye ointment., Methods: This is a single-centre, randomised, single-blind, parallel-group study of 144 patients with accidental corneal abrasion or corpus alieni cornea who were referred to the Eye Department at Gentofte Hospital. The injured eye was examined with a photo slit-lamp before and 24 hours after treatment. The size of the abrasion was recorded and calculated on a PCX computerized video system and by slit-lamp photography., Results & Conclusion: The Fucithalmic and chloramphenicol ointment treated groups showed no significant difference in corneal healing, local side effects, or signs of local infection.

A prospective Danish multicentre study was conducted to evaluate the incidence of retinal detachment after cataract extraction in myopic eyes (axial length, > or = 25.5 mm). Two hundred and forty-seven cataract extractions in myopic eyes were reported during a period of 13 months. Two hundred and forty-one eyes underwent extracapsular and six eyes intracapsular cataract extraction. The mean follow-up time for 158 eyes was seven months (ranging from 1-30 months). In five cases a retinal detachment was observed, one case was probably present preoperatively, this person had undergone intracapsular cataract extraction. The incidence of retinal detachment was thus 1.62-2.02% in the total material and 1.66% in eyes operated with extracapsular cataract extraction.

The prevalence and the incidence of glaucoma blindness in Denmark was evaluated by examining all registration forms of persons > or = 50 years of age admitted to the Danish Association of the Blind (DAB) between 1955 and 1987 with glaucoma as main cause of blindness. In 1987, 6.7% of DAB-members > or = 50 years suffered from blindness caused at least partially by glaucoma, equivalent to an estimated prevalence of 45 per 100,000 of the Danish population > or = 50 years. The estimated annual incidence of blindness due to glaucoma was seven per 100,000 > or = 65 years, and in an equal number of patients glaucoma was a contributory cause of blindness. The incidence of glaucoma blindness was decreasing in the younger age groups (< 65 years) throughout the study period. Glaucoma blindness seems to occur at a later age now than earlier, leaving the patients blind for a shorter time. The proportion of glaucoma blindness in the glaucoma population was estimated to be 4-5%.

An ophthalmic status is given for 95 consecutive patients referred from other departments of the Central County Hospital 1986-88 due to suspected giant cell arteritis. Eventually, the diagnosis was confirmed in 51/95 (18 had positive biopsy of the temporal artery; in 33 it was on clinical grounds). Nine of the 51 had significant visual loss, in one even as bilateral blindness. Generalized malignancies were found in 2 of the 44 with diagnosis other than giant cell arteritis. Retrospectively, in the same 3-year period a total of 263 cases of giant cell arteritis were registered in the somatic hospitals of the county (population 340,000). One further case with visual loss became known from requests to the primary health sector ophthalmologists. Between 3-4% were thus known to have significant visual loss, a result mainly in keeping with other Nordic clinical studies. Probably, the low figures of visual impairment are related to high diagnostic rates of giant cell arteritis, and to early treatment. Our hospital-based data of giant cell arteritis gave a calculated annual incidence of 1 per 1000 of those older than 50 years in the county, or 27/100,000 including all ages. The true county incidence would be even higher had it been possible to find and include the additional cases of giant cell arteritis who were diagnosed and treated in the primary health sector only.

During the three-year period 1986-1988, 95 patients were referred to the county eye department on account of suspected giant cell arteritis (GCA = temporal arteritis/polymyalgia rheumatica). Among the 51 with GCA, nine had visual loss. Bilateral blindness occurred in one patient. Temporal artery biopsy was positive in 18 patients. Malignancies were found in two of the 44 patients in whom the GCA diagnosis was subsequently rejected. In the somatic hospitals of the county, a total of 263 GCA patients could be collected during the sampling period. Requests to the primary health service ophthalmologists revealed one further case with marked visual loss. A total of ten out of 264 patients visual loss associated with GCA denotes a lower ocular risk than traditionally presumed. The result is in agreement with previous Danish and Nordic investigations. Probably the low visual risk is related to a high diagnostic rate of GCA, given here by an annual hospital based incidence in the county of 1 per 1,000 of age 50+. Considering the number of cases diagnosed and treated outside hospital, the real incidence is even higher. Regarding visual loss, however, we feel that all known cases with serious reduction of sight due to recognized GCA have been included.

In order to evaluate the prevalence and the incidence of glaucoma blindness in Denmark, all registration forms of persons greater than or equal to 50 years of age at admission, admitted to the Danish Association of the Blind (DAB) between 1955 and 1987 with glaucoma as main cause of blindness, were examined. In 1987 6.7% of DAB-members greater than or equal to 50 years of age suffered from blindness caused at least partially by glaucoma, equivalent to an estimated prevalence of 45 per 100,000 of the Danish population greater than or equal to 50 years. The estimated annual incidence of blindness due to glaucoma was 7 per 100,000 greater than or equal to 65 years, and in an equal number of patients glaucoma was a contributory cause of blindness. The incidence of blindness due to glaucoma was decreasing in the young age groups (less than 65 years) throughout the study period. Glaucoma blindness seems to occur at a later age now than earlier, leaving the patients blind for a shorter time. This pattern may be explained by improved health services but could also be due to a change in the spontaneous course of glaucoma. The proportion of glaucoma blindness in the glaucoma population was estimated to be 4-5%.