School performances in children with cataract: results from a population-based cohort study.

OBJECTIVES: Childhood cataract is a chronic condition that may interfere with the child's learning capacities. We aimed to investigate whether childhood cataract influences academic development by comparing school performance in reading and mathematics in children with cataract to a matched control group. DESIGN: Nationwide registry-based cohort study. SETTINGS: Two surgical centres that perform all treatments for childhood cataract in Denmark. PARTICIPANTS: Children born between 2000 and 2009 diagnosed with cataract before 10 years of age (n=275) and an age-matched and sex-matched control group (n=2473). MAIN OUTCOME MEASURES: School performance was assessed as test scores in national tests performed at regular intervals from grade 2 to grade 8 in reading and mathematics. Analyses were corrected for birth origin, child somatic and mental disorder and parental socioeconomic status and mental disorders. RESULTS: Of 275 children, 85 (30.9%) were operated for bilateral cataract, 79 (28.7%) unilateral cataract and 111 (40,4%) were not operated. We found that children with cataract have lower participation rate in the tests (62.5%) compared with the control cohort (77.2%) (p value=0.0001). After adjusting the pooled analyses for birth origin, somatic and mental disease in the child and parental socioeconomic status and mental disorders, we found that the children with cataract scored significantly lower in mathematics compared with those without cataract (mean difference=-4.78, 95% CI: -8.18 to -1.38, p value=0.006), whereas no difference was found regarding scores in reading (p=0.576). The lower score in mathematics was driven by children who had been operated for bilateral cataract (p-value=0.004). CONCLUSION: Children with cataract without somatic or neurodevelopmental comorbidities or psychosocial adversities seem to do well in school, whereas children operated for bilateral cataract have higher frequencies of difficulties in mathematical tasks.

Optic pathway glioma and the sex association in neurofibromatosis type 1: a single-center study.

BACKGROUND: Low-grade optic pathway glioma (OPG) develops in 15-20% of children with neurofibromatosis type 1 (NF1). OPGs are symptomatic in 30-50% and one-third of these require treatment. A few studies have suggested female sex as a risk factor for visual impairment associated with NF1-OPG. This descriptive study investigated the correlation between NF1-OPG growth, sex and visual impairment. METHOD: We based our cross-sectional study on a systematic, retrospective data collection in a NF1 cohort of children and adolescents below 21 years of age followed at Center for Rare Diseases, Aarhus University Hospital, Denmark. For each patient with OPG a medical chart review was performed including demographics, ophthalmological examinations and magnetic resonance imaging (MRI) of OPG. RESULTS: Of 176 patients with NF1 (85 females, 91 males), we identified 21 patients with OPG (11.9%) with a preponderance of females, p = 0.184. Eight females (62%) and one male (13%) had visual impairment at the last ophthalmological evaluation. Five out of 21 children with OPG (24%) underwent diagnostic MRI because of clinical findings at the ophthalmological screening. Nine children (43%) had symptoms suggestive of OPG and seven (33%) experienced no OPG-related symptoms before the diagnostic MRI. Of eight children diagnosed with OPG ≤ two years of age, one had visual impairment. Of 13 children diagnosed > two years of age, eight had visual impairment; in each group, four of the children were treated with chemotherapy. The study suggested no correlation between NF1-OPG growth and sex. CONCLUSION: Our data suggest sex as a risk factor for visual impairment, while an OPG diagnose ≤ two years of age was a protective factor for visual impairment. Females with NF1-OPG had a higher prevalence of visual impairment outcome compared to males. Interestingly, our data also suggest a better response to treatment in children with OPG diagnosed ≤ two years of age compared to older children. The findings in our study suggest sex as a potential prognostic factor for visual impairment.

[Diagnosis, monitoring and treatment of tuberous sclerosis complex].

Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder with highly varying disease manifestations, many of which cause extensive morbidity. There are international consensus criteria for the diagnosis, monitoring and treatment of TSC, and approved medical treatment for some of the most serious disease manifestations. However, organisation of a rational and coordinated care of TSC patients involves many different medical specialities and is only sparsely described. This review describes the interdisciplinary care of TSC patients at Aarhus University Hospital, Denmark.

Children with congenital and childhood cataract require frequent follow-up visits and examinations in general anaesthesia: considerations for the strain on families.

BACKGROUND: Children with cataract require frequent monitoring to detect complications, adjust refractive correction and treat amblyopia. This is time consuming for the families. The aim of the study was to evaluate how often children with cataract are seen as outpatients or under general anaesthesia during the first 7 years of life. METHODS: We performed a retrospective chart review of all children with congenital and childhood cataract born between 2000 primo and 2017 seen at our institution. The cumulated number of outpatient visits and examinations and/or surgeries in general anaesthesia was extracted for age 1, 3, 5 and 7 years. RESULTS: Children who had cataract surgery were seen significantly more often than children without surgery. During the first year of life, children with bilateral surgery had a median of nine outpatient visits, children with unilateral cataract had 11 and children without surgery had five outpatient visits. At 7 years of age, half of the children operated bilaterally before 1 year of age had undergone at least five procedures/examinations in general anaesthesia versus 1/4 of those with unilateral surgery and none of those without surgery. Children were seen less frequently with advancing age. CONCLUSION: The management, treatment and follow-up of children with cataract are demanding, requiring frequent hospital visits and repeated examinations and/or surgical procedures in general anaesthesia over many years, but mainly during the first year of life. Surgical patients are more complex and require closer follow-up. This message is important to convey to the parents at the onset of the disease.

The area of fixation covaries with short-term changes in visual acuity after anti-vascular endothelial growth factor treatment in patients with diabetic macular oedema.

PURPOSE: Diabetic maculopathy can be treated with intravitreal injection of vascular endothelial growth factor (VEGF) inhibitors. However, the therapy is not effective in all patients, and it would be desirable to have parameters for differentiating patients who will benefit from treatment from those who will not. Retinal fixation has been shown to be impaired in patients with low visual acuity (VA) secondary to macular disease, but the changes in fixational eye movements after anti-VEGF treatment for diabetic maculopathy have not been investigated. METHODS: Retinal fixation was studied in 29 patients with diabetic macular oedema before three monthly anti-VEGF injections, and 1 and 4 months after the last injection. The change in VA was correlated with changes in area, frequency, amplitude and total number of fixational saccades. RESULTS: During three monthly injections, best-corrected visual acuity (BCVA) increased from (mean ± SD) 74.0 ± 11.5 Early Treatment Diabetic Retinopathy Study (ETDRS) letters to 78.3 ± 9.8 ETDRS letters, (p = 0.003) and central retinal thickness (CRT) decreased from (mean ± SD) 441.7 ± 107.0 µm to 339.5 ± 74.2 µm, (p = <0.0001), which was followed by the opposite changes after treatment (BCVA reduced nonsignificantly to 77.5 ± 10.4 ETDRS letters (p = 0.06), and CRT increased to 393.0 ± 9.8 µm, p = <0.0001). Both improvement and worsening of BCVA correlated with the fixation area (r2  = 0.28, p = 0.003 and r2  = 0.14, p = 0.045, respectively), but only improvement of BCVA correlated with the frequency (r2  = 0.15, p = 0.037) and total number of saccades (r2  = 0.18, p = 0.02). BCVA showed no correlation with the amplitude and most frequently occurring saccade amplitude. CONCLUSION: Fixational eye movements may be used to monitor short-term effects of anti-VEGF treatment on diabetic macular oedema. Future studies should aim at investigating a possible predictive value of fixational eye movements for visual function in the long term.

Binocular Fixation Reduces Fixational Eye Movements in the Worst Eye of Patients with Center-Involving Diabetic Macular Edema.

PURPOSE: Treatment of diabetic maculopathy with vascular endothelial growth factor inhibitors is in some patients ineffective, and, therefore, parameters predicting visual outcome after treatment should be identified. It has been shown that fixational saccades are increased in patients with reduced visual acuity secondary to diabetic maculopathy, but it is unknown to what extent these saccades in an eye affected by diabetic maculopathy are influenced by the other eye during binocular fixation. METHODS: In 57 eyes from 29 diabetic patients with clinically significant macular edema, fixational eye movements were recorded using the iView X™ video-based eye tracker, and quantitative measures of fixation with the worst eye obtained during monocular and binocular fixation were compared. RESULTS: Fixational saccades during monocular fixation had a significantly higher frequency (p = 0.005), a larger amplitude (p = 0.03), and involved a larger retinal area (p = 0.02) than during binocular fixation. There was a significant negative correlation (r2 = 0.18, p = 0.02) between visual acuity and the area of fixation during monocular but not during binocular fixation (r2 = 0.007, p = 0.68). CONCLUSION: Binocular fixation can reduce the area of fixation and the amplitude of fixational saccades in the worst eye of patients with diabetic maculopathy. Fixational saccades in diabetic maculopathy should be studied during monocular fixation.

The predictive value of subjective symptoms and clinical signs for the presence of treatment-requiring exudative age-related macular degeneration.

PURPOSE: The introduction of vascular endothelial growth factor inhibitors for the treatment of exudative age-related macular degeneration (AMD) has increased the referral rates of AMD patients with visual symptoms to treating centres considerably. However, a large proportion of the referred patients do not qualify for treatment implying that considerable resources could be saved if these patients could be identified on the basis of the clinical data available in the referring nonspecialized setting. METHODS: A prospective observational study of 1682 consecutive patients referred with suspicion of exudative AMD qualifying for intravitreal angiostatic treatment. On the basis of the structured interviewing about symptoms, ophthalmoscopy, optical coherence tomography scanning, and fluorescein angiography, the patients were divided into two groups: one qualifying for and another not qualifying for treatment. Multiple logistic regression was used to identify independent parameters predicting the need for treatment. RESULTS: The presence of metamorphopsia, dyschromatopsia, retinal haemorrhages and exudates, central retinal thickness, and the absence of micropsia were highly significant individual determinants of treatment-requiring AMD. Sudden onset and worsening of symptoms and the presence of a central dark spot covaried with the occurrence of retinal haemorrhages, whereas reduced visual acuity and blurred vision covaried with the presence of both haemorrhages and exudates. CONCLUSION: Patients with treatment-requiring AMD can be reliably identified by questioning about the presence of metamorphopsia and dyschromatopsia and the absence of micropsia, combined with ophthalmoscopical detection of retinal haemorrhages and exudates. This information may improve the triage of patients considered for referral.