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The final refraction after intraocular lens (IOL) implantation remains a challenge in the management of paediatric cataracts. No consensual guidelines exist for the choice of IOL power. The aim of this study was to validate a method of IOL power calculation by evaluating the final refractive error in all patients with IOL implantation operated at our institution.We retrospectively studied all children under 7 years of age who underwent cataract surgery with IOL implantation at our institution between 2010 and 2015. Intraocular lens (IOL) power was calculated as follows: After B-scan determination of the emmetropic IOL power, a reduction of 40%, 35%, 30%, 25%, 20%, 15%, 10% and 5% was applied to children 0-3, 3-6, 6-12, 12-18, 18-24, 24-30, 30-36, 36-48 months, respectively. The following data were collected: follow-up, age at surgery, uni- or bilaterality, implanted IOL power and final refraction.During this period, 81 children (125 eyes) met the inclusion criteria with a median follow-up of 60 months (36-97). The median age at surgery was 6.61 months (0.76-48). We included 52 children with bilateral cataract (96 eyes) and 29 children with unilateral cataract (29 eyes). The mean implanted IOL power was 23.3 ± 4.6 diopters (D). The mean spherical equivalent at last follow-up was 0.07 ± 3.5 D.Our undercorrection formula for IOL implantation after congenital cataract surgery leads to long-term refractive results globally close to emmetropia.

Botulinum toxin (BT) is used in a variety of therapeutic applications, including the treatment of strabismus. Two injection techniques coexist - transconjunctival injection and open sky injection. The goal of this study was to evaluate the results of BT injections in esotropia in children under 10 years of age and to compare the two techniques.This is a retrospective, monocentric study, including children who received BT injections to treat their strabismus by the aforementioned techniques between 2014 and 2017. The results of these injections were recorded, and subgroup analyses (injection technique, age, degree of deviation, type of strabismus) were performed. The primary endpoint was the optimal success rate defined as residual strabismus less than or equal to 10 Δ.The study included 68 children with a mean age of 28.9 months and a mean deviation angle of 34.7 Δ. Patients received 1.2 BT injections. The success rate was 38% at 6 months, 35% at 12 months, and 35% at 24 months. There was 33% transient ptosis and 5% consecutive exotropia. There was no evidence of significant difference in success rate between the transconjunctival and open sky injection methods, baseline angles, age of injection, or type of strabismus.BT injection is effective and safe in pediatric esotropia, regardless of the injection method.

To evaluate the current French screening guidelines for retinopathy of prematurity (ROP) and to suggest modifications to it.In this multicentric retrospective, noncomparative, interventional case series we included infants with a gestational age (GA) ≤32 weeks who were screened for ROP by fundus examination between 2011 and 2018. Main Outcome Measures were the presence of ROP and the need for treatment.A total of 2246 children with a mean GA of 28.9 ± 2.0 weeks and mean birth weight (BW) of 1141.1 ± 332.0 g were screened. Retinopathy of prematurity (ROP) was found in 683 infants (30.4%), of whom 145 (6.5%) had type 2 ROP and 58 (2.6%) had type 1 ROP. Mean GA of infants with type 1 ROP needing treatment was 25.9 + 1.5 weeks (range: 23.6-30) and mean BW was 774.1 ± 173.7 g (range: 540-1400). Both GA and BW had an impact on the development of type 1 and 2 ROP. None of the infants needing treatment had a GA of 31 weeks or more. None of the children needed treatment before 33 weeks of postmenstrual age (PMA) or 6 weeks of postnatal age (PNA).It seems possible to decrease the screening of premature infants to ≤31 weeks of GA and to start screening at 31 weeks PMA for infants having a GA 26 weeks and at 6 weeks PNA for more mature children.

Disruption of any of the ocular development steps can result in ocular defects such as microphthalmia, coloboma and anterior segment dysgeneses including aniridia and cataract. All of these anomalies can be isolated or seen in association with each other. Except for aniridia (almost exclusively due to PAX6 mutations), most of these congenital ocular malformations are related to a wide genetic heterogeneity, as hundreds of genes are implied in ocular development. Here we describe a patient presenting with bilateral microphthalmia, congenital cataract, corneal dystrophy and iris hypoplasia, associated with extra-ocular features, who underwent an analysis of 119 ocular development related genes. Genetic testing revealed the presence of two truncating variants in the EPHA2 gene. While EPHA2 mutations are mainly known to be responsible for isolated dominant congenital cataract, we report here the first case of complex anterior segment dysgenesis caused by a biallelic EPHA2 mutation. This gene should be screened in case of aniridia with a negative PAX6 testing, as the ocular features of our patient clearly mimic those of PAX6 mutated patients. This observation enlarges the phenotype associated with EPHA2 variations and rise the insight of a possible PAX6-EPHA2 interaction that needs further investigations. Moreover, despite a great variability in ocular and extra-ocular phenotypes, mutations type and inheritance pattern, a possible genotype-phenotype correlation can also be drawn for this gene.

Retinal coloboma is a rare condition which is difficult to diagnose in foetuses. It can cause blindness. It can be isolated or associated with other malformations in various syndromes. Our objective is to describe the different prenatal ultrasound findings and management of coloboma. We describe a case of prenatal ultrasound diagnosis of retinal coloboma at 27.5 weeks of gestation. Our case adds to the 8 previously reported in the prenatal ultrasound literature, which together illustrate that microphthalmia is the main associated sign, present in 66.6% (6/9) of cases followed by retro-orbital cysts (44.4%) (4/9). These two ultrasound findings should alert us to a close examination of the eye to look for a posterior retinal cleft, the main direct sign of a chorioretinal coloboma.

To evaluate the evolution of chronic uveitis in children undergoing cataract surgery with primary intraocular lens (IOL) implantation.Twelve children with chronic uveitis underwent cataract surgery with primary posterior chamber intraocular lens (IOL) implantation.Fourteen eyes were implanted with a foldable hydrophobic acrylic IOL. The mean follow-up was 35.39 months (8.72-69.57). The mean BCDVA before surgery and at the end of follow-up was 1.11 (0.40-2.30; SD: 0.57) and 0.48 (0-3; SD: 0.77; p=0.007) respectively. The mean oral corticosteroids dosage after surgery and at the end of follow-up was 0.80 mg/kg/day (SD: 0.37) and 0.17 mg/kg/day (SD: 0.24; p=0.001) respectively. All patients except one were treated with methotrexate. Four patients (5 eyes) were additionally treated with anti-tumor necrosis factor agent.Cataract surgery with primary posterior chamber hydrophobic IOL implantation is possible and leads to a good visual recovery in cases of pediatric chronic uveitis. This surgery requires aggressive anti-inflammatory management with immunosuppressive drugs to control inflammation and reduce the corticosteroids dosage.

Summary Hereditary vitreoretinopathies (VRPs), traditionally divided into the groups of exudative and degenerative VRPs, encompass a number of diseases affecting the vitreous and the retina. These disorders are the commonest cause of inherited retinal detachment with high risk of vision loss during infancy. We designed a next generation sequencing (NGS) assay including ATOH7, COL2A1, COL9A1, COL9A2, COL9A3, COL11A1, COL18A1, FZD4, KCNJ13, KIF11, LOXL3, LRP5, NDP, TSPAN12, VCAN and ZNF408 allowing an exhaustive genetic screening with the most frequently mutated genes reported to be associated with all inherited VRPs. The diagnostic performance of this new design was evaluated on a cohort of 166 probands with a suspected clinical diagnosis of exudative or degenerative VRPs comprising 47% of children under 15 years old. We identified a large spectrum of causative mutations (single nucleotide changes as well as copy number variants) diagnosing molecular defects for 60% of the patients. Our NGS panel is therefore a promising screening method to be used efficiently in routine practice, able to detect a higher number of molecular defects than Sanger sequencing, improving significantly the molecular diagnosis of inherited VRPs.

International audience; PURPOSE: Comparison of the efficacy of tropicamide eyedrops to the combination of 0.5% tropicamide and 5% phenylephrine eyedrops in order to achieve a proper dilation in premature infants undergoing screening for retinopathy of prematurity. METHODS: A prospective, randomized, double-blind study was conducted to compare the efficacy of two mydriatic regimens: one regimen consisting of three drops of 0.5% tropicamide (TTT regimen), the other regimen consisting of one drop of 5% phenylephrine and two drops of 0.5% tropicamide (PTT regimen). Thirty premature infants were enrolled and received both mydriatic regimens: one regimen in each eye. Outcomes were pupil dilation evaluated by the percentage of pupil diameter over cornea diameter, the percentage of pupil surface over cornea surface and the quality of the eye fundus examination. RESULTS: The percentage of pupil diameter over cornea diameter was 47.3% (±8.7) with the TTT regimen and 65.9% (±8.8) with the PTT regimen (p

La retinopathie du premature (ROP) est une maladie cecitante necessitant un depistage dont l'observance est rarement evaluee. L'objectif etait de determiner la prevalence de l'examen du fond d'oeil (FO) chez les prematures nes avant 32 SA ainsi que les facteurs enfants et centres associes au depistage de la ROP en France. Pour ce faire, les donnees issues d'EPIPAGE-2, une cohorte observationnelle de prematures nes en France en 2011, ont ete analyses a l’aide de modeles mixtes. Au total, 2169/3077 (70,5%) des enfants eligibles au depistage ont subi un examen du FO (de 96% a 24 SA a 50% a 31 SA). Les caracteristiques infantiles associees a l'examen du FO etaient un faible âge gestationnel, un faible poids a la naissance, une dysplasie bronchopulmonaire severe, des lesions neurologiques graves et un transfert vers une autre unite au moment du depistage. La probabilite de depistage etait augmentee chez les nourrissons hospitalises dans des unites utilisant l'imagerie grand angle (OR 2,65 [IC95% 1,17-6,01]). Le niveau de soins, le statut administratif et le volume d'activite des unites n'etaient pas associes au depistage de la ROP. Parmi les enfants depistes, 1641/2169 (75,7%) ont ete depistes a temps. Avoir ete depiste a temps etait associe a un âge gestationnel eleve et a l'utilisation de l'imagerie grand angle (OR 2,20, IC95% [1,06-4,58]); un examen retarde etait associe a un faible poids a la naissance, a une dysplasie bronchopulmonaire severe et a une enterocolite necrosante. Pour conclure, les enfants les plus a risque de ROP etaient les mieux depistes, mais souvent avec un retard. La meilleure compliance des unites neonatales utilisant l'imagerie grand champ plaide pour sa diffusion.

Purpose To describe 3 cases of ectopia lentis (EL) associated with primary congenital glaucoma (PCG) and differentiate between primary and secondary EL regarding pathogenesis. Methods We reviewed the clinical charts of 3 children previously diagnosed with congenital glaucoma who developed secondary EL. The following points were noted: medical and surgical history, refraction, keratometry, corneal diameter, axial length (AL), intraocular pressure, and cup/disc ratio. Results The 3 patients were respectively aged 12, 3, and 4 months. A trabeculectomy was performed in both eyes at 1-week interval in all cases. The EL was noted during follow-up controls. Lensectomy/vitrectomy was performed in 2 patients, in case of high astigmatism (>5.00 D) and/or the lens border visible in the pupillary area. Postoperative refraction was hyperopic. Conclusions Ectopia lentis secondary to PCG should be differentiated from primary EL regarding pathogenesis. Anterior segment distension appears to be the principal mechanism in these cases. Postoperative hyperopia is observed despite the increased AL secondary to PCG. These biometric results are due to global distension of the globe, which also flattens the cornea with consequently lower refractive effect.

Some pseudophakic patients implanted with a monofocal intraocular lens (IOL) have good near visual acuity (VA) with their distance correction. The objective was to evaluate the prevalence of pseudo-accommodation in children after bilateral cataract surgery, without amblyopia, and to define its mechanisms. Observational study that took place in a pediatric ophthalmology department, Paris, France. A total of 68 eyes were included, 40 from 23 children and 28 from 14 adults, with a corrected distance VA above 20/25 and a normal near VA (20/25) with +3 addition. Pseudo-accommodation was defined as a near VA better than 20/50 with the distance correction and without addition. Prevalence of pseudo-accommodation was calculated in each group. In order to determine the possible mechanisms of pseudo-accommodation in children, we compared children with pseudo-accommodation and adults without pseudo-accommodation regarding several parameters: refraction, axial length, corneal topography, aberrometry, pupillary diameter and IOL shift after cyclopentolate instillation. Among the children group, 36 (90 %) had pseudo-accommodation versus 2 (7 %) in the adult group. We found that spherical equivalent, implant power, corneal multifocality and corneal higher-order aberrations (mainly coma and trefoil) were significantly higher in the pseudo-accommodation group, while pupil diameter and implant shift were not significantly different. Pseudo-accommodation has a high prevalence among non-amblyopic pseudophakic children. Several possible mechanisms have been found to explain pseudo-accommodation in children: a high power of the IOL and a small axial length, maximizing the effect of the IOL shift, corneal multifocality and corneal higher-order aberrations.

Bien qu’elle ne contribue qu’à environ 3 % du marché mondial des huiles végétales alimentaires, l’huile d’olive fait l’objet d’un intérêt croissant de la part de nouveaux pays, notamment grâce aux résultats des recherches scientifiques qui confirment les caractéristiques positives de cet «or liquide» et sa place fondamentale dans la diète méditerranéenne. Cet intérêt se traduit par une transformation de la structure de la production et une concurrence plus forte entre les pays producteurs : derrière le géant espagnol et les producteurs traditionnels se trouvent désormais de nouveaux pays, producteurs de dernière génération qui recherchent une rentabilité maximum. L’évolution parallèle des circuits commerciaux d’approvisionnement reflète également un changement profond de la structure du marché mondial. L’offre encore trop atomisée et la pression de la demande extrêmement concentrée de la grande distribution donnent lieu à un niveau de prix particulièrement bas et préoccupant, de nature à remettre en question la rentabilité de cette production agricole dans les zones rurales les moins compétitives où elle joue un rôle si important.

Purpose To detail the highly variable ocular phenotypes of a French family affected with an autosomal dominantly inherited vitreoretinopathy and to identify the disease gene. Methods Sixteen family members with ten affected individuals underwent detailed ophthalmic evaluation. Genetic linkage analysis and gene screening were undertaken for genes known to be involved in degenerative and exudative vitreoretinopathies. Qualitative reverse transcriptase-PCR analysis of the versiscan (VCAN) transcripts was performed after mutation detection in the VCAN gene. Results The first index patient of this French family was referred to us because of a chronic uveitis since infancy; this uveitis was associated with exudative retinal detachment in the context of a severe uncharacterized familial vitreoretinopathy. Genetic linkage was obtained to the VCAN locus, and we further identified a new pathogenic mutation at the highly conserved splice acceptor site in intron 7 of the VCAN gene (c.4004–2A>T), which produced aberrantly spliced VCAN transcripts. Conclusions Extensive molecular investigation allowed us to classify this familial vitreoretinopathy as Wagner syndrome. This study illustrates the need to confirm clinical diagnosis by molecular genetic testing and adds new ocular phenotypes to the Wagner syndrome, such as vascular and inflammatory features.