Brain MRI findings and their association with visual impairment in young adolescents born very preterm.

PURPOSE: Very preterm birth increases risk for neonatal white matter injury, but there is limited data on to what extent this persists into adolescence and how this relates to ophthalmological outcomes. The aim of this study was to assess brain MRI findings in 12-year-old children born very preterm compared to controls and their association with concurrent ophthalmological outcomes. METHODS: We included 47 children born very preterm and 22 full-term controls (gestational age <32 and >37 weeks, respectively). Brain MRI findings were studied in association with concurrent ophthalmological outcomes at 12-year follow-up. RESULTS: Evans index (0.27 vs 0.25, p<0.001) and a proposed "posterior ventricle index" (0.47 vs 0.45, p=0.018) were increased in children born very preterm. Higher gestational age associated with larger corpus callosum area (ß=10.7, 95%CI 0.59-20.8). Focal white matter lesions were observed in 15 (32%) of very preterm children and in 1 (5%) of full-term controls. Increased posterior ventricle index increased risk for visual acuity ≤1.0 (OR=1.07×1011, 95%CI=7.78-1.48×1021) and contrast sensitivity <0.5 (OR=2.6×1027, 95%CI=1.9×108-3.5×1046). Decreased peritrigonal white matter thickness associated with impaired visual acuity (ß=0.04, 95%CI 0.002-0.07). CONCLUSION: More white matter lesions and evidence of lower white matter volume were found in children born very preterm compared with full-term controls at 12-year follow-up. The association between larger posterior ventricle index and reduced visual acuity and contrast sensitivity suggests disturbances of the posterior visual pathway due to diffuse white matter lesions.

Visual tracking at 4 months in preterm infants predicts 6.5-year cognition and attention.

BACKGROUND: Visual tracking of moving objects requires sustained attention and prediction of the object's trajectory. We tested the hypothesis that measures of eye-head tracking of moving objects are associated to long-term neurodevelopment in very preterm infants. METHODS: Visual tracking performance was assessed at 4 month's corrected age in 57 infants with gestational age <32 weeks. An object moved in front of the infant with sinusoidal or triangular (i.e. abrupt) turns of the direction. Gaze gain, smooth pursuit gain, and timing of gaze to object motion were analyzed. At 6.5 years the Wechsler Intelligence Scale for Children (WISC-IV), the Brown Attention Deficit Disorder (Brown ADD), and visual examination were performed. RESULTS: Gaze gain and smooth pursuit gain at 4 months were strongly related to all WISC-IV parameters at 6.5 years. Gaze gain for the triangular and sinusoidal motion patterns related similarly to the cognitive scores. For the sinusoidal motion pattern, timing related to most Brown ADD parameters. There were no statistically significant differences in associations dependent on motion pattern. Visual function did not influence the results. CONCLUSION: The ability to attend to and smoothly track a moving object in infancy is an early marker of cognition and attention at 6.5 years. IMPACT: Potential long-term implications of infant visual tracking of moving objects for school-age neurodevelopment has not been previously studied in very preterm infants. Early coordination of eye and head movements in gaze gain, smooth pursuit, and timing of gaze to object motion are closely associated with cognition and attention at 6.5 years. As related functions at 6.5 years include perceptual and verbal skills, working memory, processing speed and attention, predictive elements in gaze tracking of moving objects might be a suitable target for future intervention studies.

Antenatal steroids and neurodevelopment in 12-year-old children born extremely preterm.

AIM: To investigate neurodevelopmental outcome in 12-year-old children born very preterm in relation to perinatal, neonatal and socioeconomic variables. To examine whether previously described positive effects of antenatal steroids on cognition persist at 12 years. METHODS: Prospective cohort, 78 children with gestational ages 22.7-31.9 weeks, born in 2004-2007 and examined at 12 years of age with cognitive, motor and visual motor integration tasks and compared to an age-matched control group (n = 50). Two preterm subgroups were studied: very preterm children (28-31 gestational weeks, n = 53) and extremely preterm children (22-27 gestational weeks, n = 25). RESULTS: The preterm children had significantly lower scores on all cognitive, motor and visual motor integration tasks than the controls. Gestational age and maternal education influenced associations differently in the two preterm subgroups. Also, severe retinopathy of prematurity demonstrated strong associations to outcome. In the extremely preterm group, administration of antenatal steroids was associated with better cognition, basic attention, word generation and motor skills. CONCLUSION: At 12 years of age, very preterm children born in the 2000s still have deficits across several neurodevelopmental domains compared to term-born peers. Administration of antenatal steroids has long-lasting associations to cognition and motor skills in extremely preterm-born children.

National cohort of infants born before 24 gestational weeks showed increased survival rates but no improvement in neonatal morbidity.

AIM: To describe survival and neonatal morbidities in infants born before 24 weeks of gestation during a 12-year period. METHODS: Data were retrieved from national registries and validated in medical files of infants born before 24 weeks of gestation 2007-2018 in Sweden. Temporal changes were evaluated. RESULTS: In 2007-2018, 282 live births were recorded at 22 weeks and 460 at 23 weeks of gestation. Survival to discharge from hospital of infants born alive at 22 and 23 weeks increased from 20% to 38% (p = 0.006) and from 45% to 67% (p < 0.001) respectively. Caesarean section increased from 12% to 22% (p = 0.038) for infants born at 22 weeks. Neonatal morbidity rates in infants alive at 40 weeks of postmenstrual age (n = 399) were unchanged except for an increase in necrotising enterocolitis from 0 to 33% (p = 0.017) in infants born at 22 weeks of gestation. Bronchopulmonary dysplasia was more common in boys than girls, 90% versus 82% (p = 0.044). The number of infants surviving to 40 weeks doubled over time. CONCLUSION: Increased survival of infants born before 24 weeks of gestation resulted in increasing numbers of very immature infants with severe neonatal morbidities likely to have a negative impact on long-term outcome.

International Classification of Retinopathy of Prematurity, Third Edition.

PURPOSE: The International Classification of Retinopathy of Prematurity is a consensus statement that creates a standard nomenclature for classification of retinopathy of prematurity (ROP). It was initially published in 1984, expanded in 1987, and revisited in 2005. This article presents a third revision, the International Classification of Retinopathy of Prematurity, Third Edition (ICROP3), which is now required because of challenges such as: (1) concerns about subjectivity in critical elements of disease classification; (2) innovations in ophthalmic imaging; (3) novel pharmacologic therapies (e.g., anti-vascular endothelial growth factor agents) with unique regression and reactivation features after treatment compared with ablative therapies; and (4) recognition that patterns of ROP in some regions of the world do not fit neatly into the current classification system. DESIGN: Review of evidence-based literature, along with expert consensus opinion. PARTICIPANTS: International ROP expert committee assembled in March 2019 representing 17 countries and comprising 14 pediatric ophthalmologists and 20 retinal specialists, as well as 12 women and 22 men. METHODS: The committee was initially divided into 3 subcommittees-acute phase, regression or reactivation, and imaging-each of which used iterative videoconferences and an online message board to identify key challenges and approaches. Subsequently, the entire committee used iterative videoconferences, 2 in-person multiday meetings, and an online message board to develop consensus on classification. MAIN OUTCOME MEASURES: Consensus statement. RESULTS: The ICROP3 retains current definitions such as zone (location of disease), stage (appearance of disease at the avascular-vascular junction), and circumferential extent of disease. Major updates in the ICROP3 include refined classification metrics (e.g., posterior zone II, notch, subcategorization of stage 5, and recognition that a continuous spectrum of vascular abnormality exists from normal to plus disease). Updates also include the definition of aggressive ROP to replace aggressive-posterior ROP because of increasing recognition that aggressive disease may occur in larger preterm infants and beyond the posterior retina, particularly in regions of the world with limited resources. ROP regression and reactivation are described in detail, with additional description of long-term sequelae. CONCLUSIONS: These principles may improve the quality and standardization of ROP care worldwide and may provide a foundation to improve research and clinical care.

Visual-motor functions are affected in young adults who were born premature and screened for retinopathy of prematurity.

AIM: To assess visual-motor integration in young adults previously included in a prospective study on the incidence of retinopathy of prematurity (ROP). METHODS: The study encompassed 59 preterm individuals, born 1988-1990, with a birth weight ≤1500 g, and 44 full-term controls, aged 25-29 years. Ophthalmological examination, including visual acuity and contrast sensitivity, and the Beery Visual-Motor Integration (VMI) with supplemental tests of visual perception and motor coordination, were performed. A short questionnaire was filled in. RESULTS: The preterm individuals had significantly lower scores than the controls in all VMI tests, median values and interquartile ranges: Beery VMI 87 (21) vs 103 (11), visual perception 97 (15) vs 101 (8) and motor coordination 97 (21) vs 102 (15), respectively. Within the preterm group, no correlations were found between the VMI tests and ROP, gestational age, birth weight or visual acuity. Contrast sensitivity was correlated to visual perception. Neurological complication at 2.5 years was a risk factor for lower scores on Beery VMI. The preterm subjects reported six times as many health problems as compared to the controls. CONCLUSION: Being born preterm seemed to have life-long effects. This study shows that visual-motor integration was affected in young adults born preterm.

Average 2.5-year neurodevelopmental test results in children born very preterm did not rule out cognitive deficits at 6.5 years of age.

AIM: The aim of the study was to investigate cognitive outcomes at 6.5 years in children born very preterm, in relation to neonatal characteristics and 2.5-year neurodevelopment. METHODS: A prospective cohort, with gestational age 22.3-31.9 weeks, born 2004-2007, were examined at 2.5 years with the Bayley Scales of Infant and Toddler Development (Bayley-III) (n = 100) and at 6.5 years with the Wechsler Intelligence Scales (n = 91). RESULTS: Neonatal factors independently related to 6.5-year outcome were gestational age, retinopathy of prematurity and treated persistent ductus arteriosus. The Bayley-III cognitive scores explained only 44% of the Full-Scale Intelligence Quotient result at 6.5 years, and 22% of the children had Wechsler index results below -1 SD, indicating cognitive impairment, after average test results at 2.5 years. The relative risk to score below -1 SD on the Full-Scale IQ was 2.83 (95% CI 1.45-5.53) in children with gestational age below 28 weeks and 2.22 (95% CI 1.18-4.17) at gestational age 28-31 weeks. CONCLUSION: Very preterm infants born in the 2000s had increased risks for impaired cognition at 6.5 years, but individual predictions based on neonatal risks and 2.5-year test results were not enough to identify all high-risk children.

Using different definitions affected the reported prevalence of neurodevelopmental impairment in children born very preterm.

AIM: We investigated the impact of varying definitions on the prevalence of neurodevelopmental impairment (NDI) in children born very preterm at 6.5 years of age. METHODS: Cognitive development and neurosensory impairments were assessed in 91 children (40/51 girls/boys) born <32 gestational weeks, in 2004-2007 in Uppsala county, Sweden. The results were compared with data from a reference group of 67 children born full term. The prevalence of NDI in the present cohort was reported according to definitions used by seven contemporary studies of children born very or extremely preterm. RESULTS: The prevalence of severe NDI varied from 2% to 23% depending on the definition used. The prevalence of cognitive impairment varied from 2% (-3 SD according to test norms) to 16% (-2 SD according to control group), the prevalence of cerebral palsy from 0% (severe) to 9% (any) and the prevalence of severe visual impairment from 0% (blindness) to 1% (visual acuity < 0.3). There were no children with severe hearing impairment. CONCLUSION: A high variability in definitions affects the reporting of the prevalence of NDI in long-term follow-up studies of very or extremely preterm born children. There is a need for a better consensus to enable comparisons across studies.

Repeatability, reproducibility and interocular difference in the assessments of optic nerve OCT in children- a Swedish population-based study.

BACKGROUND: The aim was, first, to collect normative data of the optic nerve head and the peripapillary retinal nerve fibre layer (RNFL) thickness assessed with Cirrus SD-OCT, in healthy children in a population-based study; second, using these data, to examine repeatability, reproducibility and the interocular difference. METHODS: One-hundred and ten eyes from 57 children aged 6-15 born at term, were examined. Best-corrected visual acuity and refraction were assessed. Both eyes were examined and the interocular difference was calculated. Repeatability was calculated by one examiner performing three assessments. Thereafter, a second examiner repeated the assessments to calculate reproducibility. RESULTS: Mean RNFL thickness was 99.2 (SD 8.8) µm, mean disc area 1.89 (SD 0.37) mm2 and mean rim area 1.52 (SD 0.26) mm2. No significant correlations with age, gender or refraction were found. Repeatability and reproducibility were good overall. There was interocular symmetry between the eyes. CONCLUSIONS: Normal values for optic nerve head and RNFL thickness assessed with Cirrus SD-OCT were gathered to obtain a normal material in children. High repeatability and reproducibility indicated reliability of assessments performed by different examiners on different occasions. Overall, good correlation between right and left eyes was found.

Impaired cognitive ability at 2.5 years predicts later visual and ophthalmological problems in children born very preterm.

AIM: To identify possible predictive factors for visual problems at 6.5 years in children born very preterm. METHODS: During 2004-2007, all very preterm infants (gestational age [GA] <32 weeks) in Uppsala County, Sweden were screened for retinopathy of prematurity (ROP) neonatally; at four months, visual tracking was tested; at 2.5 years, visuospatial and cognitive tests were carried out. At 6.5 years, 84 preterm children and a reference group of 64 full-term children underwent ophthalmological testing. RESULTS: Mean visual acuity (VA) did not differ between the groups, but subnormal VA (≤0.8) was more common in the preterm group (31% vs 14%; p < 0.05). More often than full-term children, preterm children had impaired contrast sensitivity (<0.5) (36% vs 19%; p < 0.05) and strabismus (8% vs 0%; p < 0.05). Low GA, ROP, intraventricular haemorrhage 3-4/periventricular leukomalacia and cognitive disability at 2.5 years predicted ophthalmological and visual problems at 6.5 years. Visual tracking ability at four months was not predictive of ophthalmological outcome. CONCLUSION: Children born preterm had more ophthalmological problems at 6.5 years of age, including subtle dysfunctions. ROP, early brain injury and impaired cognitive function around 2.5 years predicted later ophthalmological dysfunctions.

Extreme prematurity, treated retinopathy, bronchopulmonary dysplasia and cerebral palsy are significant risk factors for ophthalmological abnormalities at 6.5 years of age.

AIM: This study evaluated the contributions of various prenatal and postnatal predictive factors to a documented high prevalence of ophthalmological abnormalities in children aged 6.5 years who were born extremely preterm. METHODS: We carried out a prospective population-based study of all children born in Sweden at a gestational age of 22 + 0 to 26 + 6 weeks based on the Extremely Preterm Infants in Sweden Study. The main outcome measures were a combined score of visual impairment, refractive errors and strabismus at 6.5 years of age. Models of univariate and multivariable regression were used to analyse potential prenatal and postnatal predictive factors at different clinically relevant time-points from one minute after birth to 30 months. RESULTS: We focused on 399 known extremely preterm survivors and compared them to 300 full-term controls. Significant antecedents for ophthalmological abnormalities included prematurity per se, retinopathy of prematurity that required treatment, severe bronchopulmonary dysplasia and cerebral palsy. Severe intraventricular haemorrhage was no longer a significant risk factor when we adjusted it for the 30-month cognitive and neuromotor development outcomes. CONCLUSION: This time-course risk analysis model showed a changing panorama of significant risk factors for ophthalmological abnormalities in children aged 6.5 years who were born extremely preterm.

CENTRAL MACULAR THICKNESS IN 6.5-YEAR-OLD CHILDREN BORN EXTREMELY PRETERM IS STRONGLY ASSOCIATED WITH GESTATIONAL AGE EVEN WHEN ADJUSTED FOR RISK FACTORS.

PURPOSE: To assess the macular thickness in 6.5-year-old children born extremely preterm (EPT) in comparison with children born at term and to investigate risk factors associated with the macular thickness in the preterm group. METHODS: A population-based study of 6.5-year-old children born before the gestational age of 27 weeks and age-matched control subjects. Macular assessments with optical coherence tomography were performed, and the results were compared with neonatal risk factors and sex. RESULTS: Adequate optical coherence tomography measurements were obtained from 134 children born EPT (mean gestational age of 25 weeks [range 23-26]) and 145 control subjects. The mean (range) of central macula thickness was significantly increased (P < 0.001) in the EPT group (right eyes: 282 µm [238-356], left eyes: 283 µm [229-351]), compared with the control group (right eyes: 249 µm [208-293], left eyes: 248 µm [207-290]). A multiple linear mixed model analysis of the EPT group revealed gestational age, retinopathy of prematurity, and male gender as important risk factors for an increased macular thickness. The macular thickness decreased by 3.9 µm per gestational week, when adjusted for retinopathy of prematurity and sex. CONCLUSION: Extremely preterm birth constitutes a substantial risk factor for a thick central macula, even when adjusted for retinopathy of prematurity and male gender.

Visual tracking in very preterm infants at 4 mo predicts neurodevelopment at 3 y of age.

BACKGROUND: Typically developing infants track moving objects with eye and head movements in a smooth and predictive way at 4 mo of age, but this ability is delayed in very preterm infants. We hypothesized that visual tracking ability in very preterm infants predicts later neurodevelopment. METHOD: In 67 very preterm infants (gestational age<32 wk), eye and head movements were assessed at 4 mo corrected age while the infant tracked a moving object. Gaze gain, smooth pursuit, head movements, and timing of gaze relative the object were analyzed off line. Results of the five subscales included in the Bayley Scales of Infant Development (BSID-III) at 3 y of age were evaluated in relation to the visual tracking data and to perinatal risk factors. RESULTS: Significant correlations were obtained between gaze gain and cognition, receptive and expressive language, and fine motor function, respectively, also after controlling for gestational age, severe brain damage, retinopathy of prematurity, and bronchopulmonary dysplasia. CONCLUSION: This is the first study demonstrating that the basic ability to visually track a moving object at 4 mo robustly predicts neurodevelopment at 3 y of age in children born very preterm.

Macular function in preterm children at school age.

PURPOSE: Macular development is a complex process that starts by mid-gestation and continues several years after birth. A preterm birth could affect this development, causing increased thickness in the central macula, but the effect of the macular function remains uncertain. The aim of this study was to investigate the macular function measured with multifocal electroretinography (mfERG), in former preterm children and compare with healthy controls. A second aim was to correlate central macular function with central macular thickness measured with optical coherent tomography (OCT), in the preterm group. METHODS: Fifteen former preterm children born before 32 weeks of gestation were included in the study. MfERG results from 12 children acted as controls. Visual acuity, refraction in cycloplegia and mfERG were carried out in all children, and optical coherent tomography (OCT) was performed in the preterm children. Main outcomes were P1 amplitudes and implicit times for Rings 1-5 and "sum of groups" of the mfERG, and central macula thickness in area A1 measured with OCT. RESULTS: The P1 amplitudes were reduced in Rings 1-5 and "Sum of groups" in the preterm children compared to controls. There were no significant correlation between P1 amplitude or implicit times in Ring 1 and central macular thickness in the preterm group. CONCLUSIONS: Macular function is reduced in former preterm children compared to children born at term. This suggests that the structural changes with a thicker central retina can have an effect on function and may be one, of probably several, explanations for visual dysfunction in preterm children at school age.

Most patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency develop pathological or subnormal retinal function.

AIM: There have been few studies on long-term electroretinographic findings in patients with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). This study correlated long-term electroretinographic findings with age, metabolic control and clinical symptoms. METHODS: We examined 12 Swedish patients with LCHADD. Visual acuity testing, fundus examinations, optical coherence tomography and electroretinography were performed. The results were correlated to age, the levels of 3-hydroxyacylcarnitine and acylcarnitine and clinical metabolic control. RESULTS: Blindness or moderate visual impairment was found in two patients. Retinal pigmentation, atrophy and fibrosis were present in 11, seven and one of the patients, respectively, and optical coherence tomography showed retinal thinning in three of the six patients examined. Electroretinography was performed on 11 of the 12 patients. It was pathological, with reduced rod and cone responses, in five patients, subnormal in four and was related to poor clinical metabolic control and severe neonatal symptoms. Repeated electroretinographies revealed reduced function with increasing age. CONCLUSION: More than 80% of the LCHADD patients developed pathological or subnormal retinal function. This was more pronounced in patients with neonatal symptoms, but ameliorated by strict dietary treatment. Annual ophthalmological follow-ups, with electroretinography every second or third year, are recommended.

Macular function measured by binocular mfERG and compared with macular structure in healthy children.

PURPOSE: To create normative data in children from binocular multifocal ERG (mfERG) recordings and compare results with the macular thickness. METHODS: Forty-nine 5- to 15-year-old healthy, full-term children were examined with Espion Multifocal System, using DTL electrodes. The stimulus matrix consisted of 37 hexagonal elements. Amplitudes, implicit times and response densities (presented in three rings) of the first-order component P1 were analyzed. Measurements of macular thickness were performed with spectral-domain Cirrus OCT. RESULTS: There were no significant differences between right and left eyes regarding mfERG recordings. Median P1 implicit times of Rings 1-3 of the 46 right eyes were 30.0, 30.0 and 30.8 ms and response densities 20.5, 10.9 and 7.6 nV/deg(2), respectively. Implicit time was longer in boys than in girls (p = 0.009, 0.039, 0.005 in Rings 1-3) and was correlated with age (r s = 0.417, 0.316, 0.274 in Rings 1-3). Implicit time in Ring 1 correlated significantly with the inner circle of the OCT measurements (p = 0.014). CONCLUSION: Binocular mfERG with DTL electrodes is a reliable test of the central macular function in children and correlates with macular structure. As previously not shown, there was a significant difference in implicit time between boys and girls.

Placental pathology in relation to stillbirth and neonatal outcome in an extremely preterm population: a prospective cohort study.

OBJECTIVE: To study associations between placental histopathology and stillbirth as well as neonatal outcome in a population born extremely preterm. DESIGN: Prospective cohort study. SETTING: Stockholm, Sweden. POPULATION: 167 infants born <27 gestational weeks during 2004-2007. METHODS: One senior perinatal pathologist, blinded to outcome data, evaluated all placental slides. MAIN OUTCOME MEASURES: Intrauterine fetal death, small-for-gestational age, major neonatal morbidity (intraventricular hemorrhage ≥grade 3, retinopathy of prematurity ≥grade 3, necrotizing enterocolitis, cystic periventricular leukomalacia or severe bronchopulmonary dysplasia) and neonatal mortality. Additional outcome variables were Apgar score at 5 min, sepsis, and treated patent ductus arteriosus. RESULTS: Accelerated villous maturation was associated with a decreased risk for Apgar score <7 at 5 min (p = 0.041). Fetal thrombosis and low placental weight were associated with an increased risk for both intrauterine fetal death (p < 0.001 and p = 0.011, respectively) and small-for-gestational age (p < 0.001 and p < 0.001, respectively). CONCLUSION: Placental histology may have prognostic value as it appears to be associated with intrauterine fetal death, as well as with being small-for-gestational age and assignment of a low Apgar score at birth.

EXPRESS study shows significant regional differences in 1-year outcome of extremely preterm infants in Sweden.

AIM: The aim of this study was to investigate differences in mortality up to 1 year of age in extremely preterm infants (before 27 weeks) born in seven Swedish healthcare regions. METHODS: National prospective observational study of consecutively born, extremely preterm infants in Sweden 2004-2007. Mortality was compared between regions. Crude and adjusted odds ratios and 95% CI were calculated. RESULTS: Among 844 foetuses alive at mother's admission for delivery, regional differences were identified in perinatal mortality for the total group (22-26 weeks) and in the stillbirth and perinatal and 365-day mortality rates for the subgroup born at 22-24 weeks. Among 707 infants born alive, regional differences were found both in mortality before 12 h and in the 365-day mortality rate for the subgroup (22-24 weeks) and for the total group (22-26 weeks). The mortality rates were consistently lower in two healthcare regions. There were no differences in the 365-day mortality rate for infants alive at 12 h or for infants born at 25 weeks. Neonatal morbidity rates among survivors were not higher in regions with better survival rates. Perinatal practices varied between regions. CONCLUSION: Mortality rates in extremely preterm infants varied considerably between Swedish healthcare regions in the first year after birth, particularly between the most immature infants.

Strabismus and refraction in non-syndromic craniosynostosis - A longitudinal study up to 5 years of age.

PURPOSE: To evaluate the refractive outcome and strabismus at 5 years of age, in children operated for various types of non-syndromic craniosynostosis, and further analyse the refractive and strabismic development over time. METHODS: Eighty-nine children, who had undergone operations for non-syndromic craniosynostosis, were examined at 5 years of age. These children also underwent ophthalmological examination preoperatively and up to 1 year after the operation. An age-matched control group including 32 healthy children was also recruited. Strabismus and eye motility were registered. Refraction was measured in cycloplegia. RESULTS: There was a difference regarding the refractive outcome between the different types of craniosynostosis. Higher values of hypermetropia were found in the metopic craniosynostosis group on both eyes. In the unicoronal craniosynostosis group, high values of hypermetropia and a higher degree of astigmatism were found on the side contralateral to the craniosynostosis. Strabismus was found in 11/88 children of whom 10/11 had unicoronal craniosynostosis. A vertical deviation on the side ipsilateral to the fused suture was highly prevalent (6/10 cases). Ophthalmological dysfunctions were rare in children operated for sagittal craniosynostosis. CONCLUSION: Ocular manifestations such as strabismus, astigmatism and anisometropia were highly prevalent in children operated for unilateral coronal craniosynostosis. Children operated for metopic craniosynostosis had higher rates of hypermetropia. The screening and follow-up protocols need to be tailored with regard to the type of craniosynostosis.