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PURPOSE: To describe the clinical characteristics, management, and outcomes of toddlers (under the age of 3) diagnosed with familial exudative vitreoretinopathy. METHODS: In this retrospective study, patients diagnosed with familial exudative vitreoretinopathy before the age of 3 were included. Presenting characteristics, genetic testing, management, and outcomes were collected. RESULTS: A total of 54 patients (108 eyes) with a mean age at diagnosis of 10.9 ± 2.6 months were included. Poor visual behavior (33%) and strabismus (26%) were the most common presenting symptoms, whereas screening only represented 11%. About half of included patients had a severe disease (stages 4 and 5). Genetic testing was positive in 40.7% of patients with 24% having a family history of familial exudative vitreoretinopathy. LRP5 was the most prevalent mutation (54.5%).Surgery was performed in 44.4% of eyes and was successful in 69.8% of cases. Failure exclusively occurred in eyes with severe stages. Among eyes evaluated for visual acuity (72 eyes), most (76.4%) had a vision of hand motion or better. CONCLUSION: Familial exudative vitreoretinopathy tended to be worse with earlier age at diagnosis, subsequently affecting the prognosis. Surgical intervention was common and primarily included lens-sparing vitrectomy and combined lensectomy and vitrectomy. Surgical success hinged on the stage of the disease.
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Cristalino , Vitrectomia , Humanos , Lactente , Vitreorretinopatias Exsudativas Familiares , Estudos Retrospectivos , PrognósticoRESUMO
PURPOSE: To describe clinical features, management, and outcomes of posterior persistent fetal vasculature (PFV) and suggest a management algorithm. DESIGN: Retrospective, consecutive case series. PARTICIPANTS: All children diagnosed with posterior PFV and treated or followed at the Rothschild Foundation Hospital in France between June 2011 and September 2021. METHODS: Retrospective analysis of the clinical characteristics of posterior PFV. We reported age, gender, presenting symptoms, intraocular pressure, and visual acuity (VA) at diagnosis. Patients were divided into 4 groups depending on the severity and involvement or not of anterior segment. We reported the vitreoretinal surgical techniques used. MAIN OUTCOME MEASURES: Anatomic results, ocular hypertension, best-corrected distance visual acuity (BCDVA), presence of postoperative adverse events, and additional surgical interventions were recorded at each follow-up visit. RESULTS: Ninety-six patients were included. The median age at diagnosis was 8 months (IQR = 12), mean 18.9 ± 30.9 months) with a mean follow-up of 27 ± 31.2 months. Although PFV is often an isolated disease, it was associated with a systemic disease in 8% of cases. There was anterior involvement in 62 (64%) of eyes. Forty-one eyes (42.7%) were microphthalmic and more frequently associated with severe PFV (53% vs. 25%; P = 0.01). Surgery was performed in 85 patients (89%). Of them, 69 (81%) had a total success, 5 (6%) had a partial success due to persistent limited peripheral retinal detachment (RD), and 11 (13%) had a failure due to persistent total RD after surgery. Postoperative adverse events occured in 38 eyes including ocular hypertension requiring eye drop medication (7.1%), secondary cell proliferation around the intraocular lens (8.2%), intravitreal hemorrhages (7.1%), and persistent tractional RD (10.6%). Second surgery was performed in 18 patients (21%). At last follow-up, VA could be measured in logarithm of the minimum angle of resolution in 43 children (45%), light perception in 21 eyes (22%), and no light perception or impossible to assess in 32 eyes (33%). CONCLUSIONS: In our case series, most patients presenting with posterior PFV received complex vitreoretinal surgery. Goals of the surgery vary and include retinal flattening, reduction of vitreoretinal traction, freeing of visual axis, and aesthetic concerns. We propose a surgical and medical management algorithm for PFV. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.
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Glaucoma , Vítreo Primário Hiperplásico Persistente , Descolamento Retiniano , Criança , Humanos , Lactente , Estudos Retrospectivos , Vitrectomia , Vítreo Primário Hiperplásico Persistente/diagnóstico , Vítreo Primário Hiperplásico Persistente/cirurgia , Vítreo Primário Hiperplásico Persistente/complicações , Resultado do Tratamento , Descolamento Retiniano/cirurgia , Glaucoma/cirurgia , Complicações Pós-Operatórias/cirurgiaRESUMO
PURPOSE: To describe clinical characteristics and outcomes of children with early-onset X-linked retinoschisis. METHODS: In this retrospective consecutive case series, we included children diagnosed with symptomatic X-linked retinoschisis younger than 2 years. Presenting signs, clinical characteristics, treatments, and outcomes were recorded. RESULTS: Seven patients (14 eyes) with a mean age of 17.14 ± 6.28 months were included. Strabismus was the most common presenting symptom (6 of 7 patients, 86%). Clinical signs at the first diagnosis included peripheral retinoschisis in 13 eyes (13/14, 93%), of which 5 (5/13, 38%) were bullous, vitreous hemorrhage in 3 eyes (3/14, 21%), and retinal detachment in 3 eyes (3/14, 21%). The macula was involved in all eyes: It was detached in 2 eyes (2/14, 14%) and involved in the peripheral schisis in 4 eyes (4/14, 29%). In all remaining eyes, optical coherence tomography revealed foveoschisis. Six eyes (6/14, 42%) received surgery. At the last follow-up, visual acuity, when available, ranged from no light perception to 20/40, and no children had persistent retinal detachment. CONCLUSION: Children with early-onset X-linked retinoschisis had severe forms. All children had peripheral retinoschisis which was often bullous and extended to the macula. Diagnosis is often clinical but handheld optical coherence tomography can be helpful in atypical forms. Complications requiring surgical management are frequent.
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Macula Lutea , Descolamento Retiniano , Retinosquise , Pré-Escolar , Humanos , Lactente , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/etiologia , Descolamento Retiniano/cirurgia , Retinosquise/diagnóstico , Retinosquise/genética , Retinosquise/cirurgia , Estudos Retrospectivos , Tomografia de Coerência Óptica , Idade de Início , Resultado do TratamentoRESUMO
AIM: Preterm children are highly vulnerable to sensorial impairments through Retinopathy Of Prematurity (ROP). The objective was to determine whether some cases of ROP requiring surgery could be secondary to deficiencies in care pathways. METHODS: Descriptive study of neonatal characteristics and the screening/treatment pathways of children treated for stage ≥4A ROP from 2009 to 2020 in a referral unit in France. RESULTS: Twenty-five preterm children (44 eyes) were included: median gestational age was 25 weeks, and median birthweight was 700 grams. Eighty-four per cent had received at least one fundus examination, 50% of which were completed on time. At the time of retinal detachment diagnosis, only 36% of the children had received laser or anti-vascular endothelial growth factor (VEGF) intra-vitreal injection. ROP stage was only reported in 8%, and the zone or type was reported in 16% of the files. CONCLUSION: The risk of blindness and the effectiveness of laser or anti-VEGF treatment highlight the need to enhance screening and treatment practices in France.
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Descolamento Retiniano , Retinopatia da Prematuridade , Recém-Nascido , Criança , Humanos , Lactente , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/etiologia , Descolamento Retiniano/terapia , Retinopatia da Prematuridade/terapia , Retinopatia da Prematuridade/cirurgia , Recém-Nascido Prematuro , Peso ao Nascer , Idade Gestacional , Fotocoagulação a Laser , Estudos RetrospectivosRESUMO
PURPOSE: To investigate the morphologic changes on optical coherence tomography angiography (OCTA) of treatment-naive Type 3 neovascularization secondary to exudative age-related macular degeneration after 1 year of anti-vascular endothelial growth factor therapy. METHODS: Consecutive patients diagnosed with treatment-naive early-stage Type 3 neovascularization were enrolled in this retrospective study. All patients underwent color fundus photographs/MultiColor (Heidelberg Engineering) imaging, fluorescein angiography, indocyanine green angiography, structural spectral domain OCT, and OCTA Optovue RTVue XR Avanti (Optovue) at baseline, and repeated OCTA and structural spectral domain OCT at Month 12. Qualitative analysis of the 3 × 3 OCTA examinations at baseline and Month 12 was then compared, to assess changes after anti-vascular endothelial growth factor therapy. RESULTS: A total of 15 treatment-naive eyes of 15 consecutive patients were included in the analysis. At 12-month follow-up after pro-re-data anti-vascular endothelial growth factor therapy (5.75 ± 1.48 injections of ranibizumab, and injections of 6.33 ± 1.21 of aflibercept), OCTA demonstrated persistence of the deep capillary plexus abnormalities in 13/15 eyes. In the outer retina and choriocapillaris, the initial lesion became undetectable in 7/15 cases, accompanied by choriocapillaris atrophy. The abnormal vascular complex persisted in the form of a tuft-shaped lesion in the outer retinal segmentation in 9/15 eyes, which in the choriocapillaris segmentation was associated with sub-retinal pigment epithelium neovascularization in 8 cases. CONCLUSION: Optical coherence tomography angiography showed that the tuft-shaped abnormal outer retinal lesion, frequently associated with a small clew-like flow signal in the choriocapillaris, after 1 year of anti-vascular endothelial growth factor therapy, either becomes undetectable or develops sub-retinal pigment epithelium neovascularization.
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Corioide/patologia , Neovascularização de Coroide/tratamento farmacológico , Angiofluoresceinografia/métodos , Ranibizumab/administração & dosagem , Receptores de Fatores de Crescimento do Endotélio Vascular/administração & dosagem , Proteínas Recombinantes de Fusão/administração & dosagem , Tomografia de Coerência Óptica/métodos , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidores , Inibidores da Angiogênese/administração & dosagem , Corioide/irrigação sanguínea , Neovascularização de Coroide/diagnóstico , Neovascularização de Coroide/metabolismo , Feminino , Seguimentos , Fundo de Olho , Humanos , Injeções Intravítreas , Masculino , Epitélio Pigmentado da Retina/patologia , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento , Acuidade VisualRESUMO
PURPOSE: To report on the anatomical and functional outcomes of a modified limbal lensectomy-vitrectomy (LV) approach for stages 4B and 5 retinopathy of prematurity (ROP) as defined in the International Classification of Retinopathy of Prematurity, 3rd Edition (ICROP 3). DESIGN: Retrospective, monocentric, consecutive case series. PATIENTS: Infants with ROP that underwent limbal LV for diffuse retrolental fibroplasia. METHODS: Clinical charts and Retcam photographs were reviewed. Surgical approach consisted of a limbal LV through peripheral iridectomies with centripetal dissection of the preretinal fibrosis. MAIN OUTCOME MEASURES: Anatomical success and visual function at last follow-up were evaluated. Multivariate logistic regression was used to explore potential prognostic factors affecting the anatomical outcome. RESULTS: A total of 128 eyes of 81 patients with a mean gestational age of 28.7 ± 3.0 weeks and a mean birthweight of 1244 ± 429 g were included. Eighteen eyes (14.1%) had a stage 4B, 24 (18.8%) a stage 5B, and 86 a stage 5C (67.2%) ROP. Mean age at surgery was 57.4 ± 36.3 weeks and mean postoperative follow-up was 22.7 ± 20.4 months. Only 5 eyes (3.9%) had prior peripheral retinal ablation. Macular reattachment was achieved in 74 eyes (57.8%). Controlling for other baseline factors, a stage 5C (versus stage 4B, odds ratio [OR] = 6.9 [1.5-32.1], P = 0.01 and versus stage 5B, OR = 7.4 [1.5-37.1], P = 0.02), the presence of vascular activity (OR = 6.4 [2.3-18.1], P < 0.001), and the presence of Schlieren sign (OR = 13.0 [2.1-82.2], P = 0.006) were associated with a failure of macular reattachment. Visual acuity was assessed in 92 eyes (71.9%), among which 59 eyes (64.1%) had light perception or better. CONCLUSIONS: Modified limbal LV resulted in macular reattachment in more than half of eyes with ROP-related retinal detachment and diffuse retrolental fibrosis. A stage 5C based on ICROP 3, the presence of vascular activity, and a Schlieren sign were significantly associated with a failure of macular reattachment. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
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Idade Gestacional , Retinopatia da Prematuridade , Acuidade Visual , Vitrectomia , Humanos , Estudos Retrospectivos , Retinopatia da Prematuridade/cirurgia , Retinopatia da Prematuridade/diagnóstico , Retinopatia da Prematuridade/complicações , Vitrectomia/métodos , Feminino , Masculino , Recém-Nascido , Seguimentos , Cristalino/cirurgia , Resultado do Tratamento , Limbo da Córnea/cirurgia , LactenteRESUMO
PURPOSE: To determine retinal detachment (RD) risk factors after lens removal surgery in children with Marfan syndrome (MS). DESIGN: Retrospective, case control study. METHODS: This was an institutional case series including children (age <18 years) with MS who underwent lens removal surgery. Clinical and surgical characteristics were extracted from the children's electronic files: age, axial length (AL), gender, number of surgeries received, intraocular lens (IOL) implantation at the first surgery, complete removal of the capsular bag, and final best-corrected visual acuity. Risk factors associated with RD occurrence were identified. RESULTS: Among 158 eyes included (85 children), 35 eyes (22.2%) developed RD during follow-up. Bilateral detachment occurred in 11 patients (45.8%). Age at the time of the lens removal surgery was not different between groups. Children in the RD group had a higher AL (P < .001), longer follow-up, IOL implantation, and capsular residue. Multivariate analysis identified capsular residue (odds ratio, 16.8; 95% CI, 1.9-148.8; P = .01) and AL (odds ratio, 1.3; 95% CI, 1.01-1.7; P = .03) as predictors for RD. CONCLUSIONS: Children with MS and increased AL were more likely to develop an RD after lens surgery. When considering lens removal surgery in a pediatric population presenting with MS, a complete capsular removal seemed to be the safer option regarding RD risk.
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PURPOSE: To the describe OCT imaging characteristics of a cohort of patients showing spontaneously closing degenerative or mixed type lamellar macular holes (LMH) and to compare them to the ones of a sex and age matched group showing stable lesions. METHODS: Patients diagnosed with degenerative and mixed type LMHs showing OCT-documented spontaneous anatomical closure were retrospectively selected from 3 specialized retina centres. An equal number of age and sex matching subjects were randomly selected among patients with anatomically stable lesions. RESULTS: Eleven (11) spontaneously closing (SC group) and 11 stable (ST group) degenerative LMH with a mean follow up of 4 years were recruited. Hyperreflective inner border (HIB) and linear hyperreflectivity in the outer plexiform layer (LHOP) at baseline were significantly more prevalent in SC group in processed images (respectively p = 0.007 and p = 0.003). A borderline significance in lamellar hole associated epiretinal proliferation (LHEP) at last follow up was detected (p = 0.085). As for mixed type LMH, 10 patients for SC group and 10 for ST group were recruited. LHOP at baseline in processed images was significantly more prevalent in SC group (p = 0.005). CONCLUSIONS: Spontaneously closing LMHs show higher prevalence of HIB and LHOP at the beginning of the closing process, a difference which is enhanced by image processing. These signs might be a signal of microglial and Muller cells coordinated activation.
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Membrana Epirretiniana , Perfurações Retinianas , Humanos , Perfurações Retinianas/diagnóstico por imagem , Membrana Epirretiniana/diagnóstico , Estudos Retrospectivos , Acuidade Visual , Tomografia de Coerência Óptica/métodos , SeguimentosRESUMO
PURPOSE: To elaborate a deep learning (DL) model for automatic prediction of late recurrence (LR) of rhegmatogenous retinal detachment (RRD) using pseudocolor and fundus autofluorescence (AF) ultra-wide field (UWF) images obtained preoperatively and postoperatively. MATERIALS AND METHODS: We retrospectively included patients >18 years who underwent either scleral buckling (SB) or pars plana vitrectomy (PPV) for primary or recurrent RRD with a post-operative follow-up >2 years. Records of RRD recurrence between 6 weeks and 2 years after surgery served as a ground truth for the training of the deep learning (DL) models. Four separate DL models were trained to predict LR within the 2 postoperative years (binary outputs) using, respectively, UWF preoperative and postoperative pseudocolor images and UWF preoperative and postoperative AF images. RESULTS: A total of 412 eyes were included in the study (332 eyes treated with PPV and 80 eyes with SB). The mean follow-up was 4.0 ± 2.1 years. The DL models based on preoperative and postoperative pseudocolor UWF imaging predicted recurrence with 85.6% (sensitivity 86.7%, specificity 85.4%) and 90.2% accuracy (sensitivity 87.0%, specificity 90.8%) in PPV-treated eyes, and 87.0% (sensitivity 86.7%, specificity 87.0%) and 91.1% (sensitivity 88.2%, specificity 91.9%) in SB-treated eyes, respectively. The DL models using preoperative and postoperative AF-UWF imaging predicted recurrence with 87.6% (sensitivity 84.0% and specificity 88.3%) and 91.0% (sensitivity 88.9%, specificity 91.5%) accuracy in PPV eyes, and 86.5% (sensitivity 87.5%; specificity 86.2%) and 90.6% (sensitivity 90.0%, specificity 90.7%) in SB eyes, respectively. Among the risk factors detected with visualisation methods, potential novel ones were extensive laser retinopexy and asymmetric staphyloma. CONCLUSIONS: DL can accurately predict the LR of RRD based on UWF images (especially postoperative ones), which can help refine follow-up strategies. Saliency maps might provide further insight into the dynamics of RRD recurrence.
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PURPOSE: Nonpowder toy guns (NPTGs) are responsible for many ocular traumas. This study aims to detail the outcomes of these injuries depending on the causative NPTG. DESIGN: Retrospective, observational case series. METHODS: Cases of NPTG-associated ocular trauma managed in a Parisian eye emergency department between August 1, 2010, and January 1, 2023, were reviewed. The date of trauma, causative NPTG, patient demographics, initial and follow-up eye examinations, any surgical procedure, and visual outcomes for each ocular trauma were analyzed. RESULTS: Over 12 years, NPTGs were responsible for 324 eye injuries and 980 visits. Patients were mostly male (77.5%), and mean age at trauma was 16.2 years. Foam bullets or foam dart blasters accounted for 54.9% of traumas and were mainly responsible for corneal injuries and hyphema (30.9% and 27%, respectively). BB guns and airsoft guns were frequently responsible for anterior segment lesions, as well as intravitreal hemorrhages (14.7%) and commotio retinae (21.1%). Paintball guns accounted for the largest proportion of posterior segment lesions (eg, intraretinal or subretinal hemorrhages leading to macular atrophy/contusion maculopathy), and one-third of casualties had undergone ocular surgery. Among all traumas, final visual acuity was lower than 20/200 in 6.5% of cases. Phthisis occurred in 8 cases: Two were related to foam bullets or foam dart blaster injuries (1 contusion and 1 rupture), 2 other cases followed a rupture due to BB guns/airsoft guns, 1 case occurred after a rupture related to a paintball gunshot, and 3 others were due to other types of compressed air guns (1 rupture, 1 intraocular foreign body, and 1 total retinal detachment). CONCLUSIONS: NPTG-related ocular trauma outcomes differ according to the causative toy. Paintball guns and BB guns/airsoft gun-related traumas were more likely to be associated with severe lesions, but an increasing number of ocular injuries related to the use of foam bullets or foam dart blasters are reported in younger and younger children. Public health policies should promote the use of protective eyewear.
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Traumatismos Oculares , Jogos e Brinquedos , Acuidade Visual , Humanos , Estudos Retrospectivos , Masculino , Feminino , Jogos e Brinquedos/lesões , Adulto , Adolescente , Incidência , Criança , Traumatismos Oculares/epidemiologia , Traumatismos Oculares/etiologia , Acuidade Visual/fisiologia , Adulto Jovem , Pessoa de Meia-Idade , Pré-Escolar , Recreação , Idoso , Armas de Fogo , Ferimentos por Arma de Fogo/epidemiologia , SeguimentosRESUMO
PURPOSE: To describe a new surgical technique and outcomes of lens-sparing vitrectomy and retrolental stalk dissection in posterior persistent fetal vasculature (PFV). DESIGN: Retrospective interventional case series. METHODS: RESULTS: Among the 21 included eyes, 8 (38%) had no macular involvement and 4 (19%) presented with microphthalmia. Median age at the first surgery was 8 months (range: 1-113 months). Surgical success was obtained in 71.4% of cases (15 of 21). In the remaining cases, the lens was removed because of capsular rupture in 2 cases (9.5%) and a large capsular opacity after stalk removal or an adherent stalk impossible to dissect in 4 cases (19.1%). In the capsular bag, IOL implantation was accomplished for all but 1 eye. None of the eyes developed retinal detachment or required glaucoma surgery. Endophthalmitis occurred in 1 eye. Secondary lens aspiration was needed in 3 eyes after a mean interval of 10.7 months following initial surgery. At last follow-up, half of the eyes remained phakic. CONCLUSION: Lens-sparing vitrectomy is a useful approach to addressing the retrolental stalk in selected cases of persistent fetal vasculature syndrome. By delaying or avoiding lens extraction, this approach allows preservation of accommodation, reduction of the risk of aphakia, glaucoma, and development of secondary lens reproliferation.
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INTRODUCTION: Epidemiological data on the use of eye-related emergency services by children are limited. The objective of this study was to determine how COVID-19 affected the epidemiological trends of pediatric ocular emergencies. METHODS: We performed a retrospective chart review of children under the age of 18 years who visited our eye-related emergency department between March 17 and June 7, 2020 and between March 18 and June 9, 2019. This was a descriptive and comparative analysis of the two study periods based on the demographic characteristics of patients and the diagnosis reported by the ophthalmologist in the digital medical charts. One of the investigators performed a second reading of the files to homogenize the diagnosis classification based on the most frequently found items. RESULTS: In total, 754 children were seen in our eye-related emergency department during the 2020 study period versus 1399 in 2019, representing a 46% decrease. In 2019, the four main diagnoses were traumatic injury (30%), allergic conjunctivitis (15%), infectious conjunctivitis (12%), and chalazion/blepharitis (12%). In the 2020 study period there was a significant decrease in the proportion of patients presenting with traumatic injuries (p < 0.001), infectious conjunctivitis (p = 0.03), and chalazion/blepharitis (p < 0.001). Consultations for chalazion/blepharitis were the most affected by the pandemic, followed by traumatic injuries (-72% and -64%, respectively). The proportion of patients who required surgery after trauma was higher in 2020 than in 2019 (p < 0.01), but the absolute number of severe trauma cases remained stable. CONCLUSIONS: The COVID-19 pandemic was accompanied by a decrease in the overall use of a pediatric eye-related emergency services in Paris. Visits due to benign causes and ocular trauma also decreased, but visits for more severe pathologies were not affected. Longer-term epidemiological studies may confirm or refute a change in eye emergency department use habits.
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Blefarite , COVID-19 , Calázio , Conjuntivite , Criança , Humanos , Adolescente , Estudos Retrospectivos , Paris/epidemiologia , Emergências , Pandemias , COVID-19/epidemiologia , Serviço Hospitalar de Emergência , Conjuntivite/epidemiologiaRESUMO
Purpose: to quantitatively analyze choriocapillaris perfusion using swept-source optical coherence tomography angiography (SS-OCTA) in eyes presenting with angioid streaks in comparison with control eyes. Methods: Macular 6 × 6 mm SS-OCTA scans were retrospectively analyzed in eyes with angioid streaks and in control eyes. En face choriocapillaris flow images were compensated with en face choriocapillaris structure images, followed by the Phansalkar local thresholding method (with a window radius of four and eight pixels). Quantitative analysis was performed in the four peripheral 1 × 1 mm corners of the 6 × 6 mm SS-OCTA image to include equidistant and comparable regions. The percentage of flow deficits (FD%), the number and size of the flow deficits (FDs) and the total area of FDs were then calculated. Results: 54 eyes of 31 patients were included in the study: 27 eyes diagnosed with angioid streaks and 27 controls. Analysis of the four 1 × 1 mm peripheral corners of the 6 × 6 mm SS-OCTA image showed that eyes with angioid streaks had a higher FD% compared to the control group (47.62 ± 8.06 versus 38.90 ± 6.38 using a radius of four pixels (p < 0.001); 48.37 ± 7.65 versus 39.66 ± 6.51 using a radius of eight pixels (p < 0.001). The average size of FDs as well as the total area size of the FDs were significantly higher in eyes with angioid streaks compared to control eyes (p < 0.001). Eyes with angioid streaks present reduced choriocapillaris flow compared to control eyes. Decreased choriocapillaris perfusion may contribute, among other factors, to the development of neovascularization and atrophy in patients with angioid streaks.
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Purpose: To compare clinical characteristics at presentation and outcomes of Coats disease between females and males. Methods: In this retrospective, consecutive case series we included all children diagnosed with Coats disease in a single tertiary referral center. Initial clinical presentation, treatment and outcomes were collected. Results: A total of 158 children were included, of whom 29 (18.3%) were females and 11 (6.9%) had bilateral involvement. Age at diagnosis and disease stage were similar between females and males. Females had more bilateral involvement (p < 0.001) and tended to have a worse visual acuity at diagnosis (p = 0.05). At last follow-up, visual acuity and anatomical outcome after treatment were similar between genders. Conclusion: Female patients with Coats disease had more bilateral involvement and tended to have worse visual acuity at presentation. Clinical presentation and outcomes seemed to be similar between genders.
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PURPOSE: To evaluate the current French screening guidelines for retinopathy of prematurity (ROP) and to suggest modifications to it. METHODS: 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. RESULTS: 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). CONCLUSION: 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.
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Retinopatia da Prematuridade , Peso ao Nascer , Criança , Idade Gestacional , Humanos , Incidência , Lactente , Recém-Nascido , Triagem Neonatal , Retinopatia da Prematuridade/diagnóstico , Retinopatia da Prematuridade/epidemiologia , Estudos Retrospectivos , Fatores de RiscoRESUMO
PURPOSE: Distinguishing posterior persistent fetal vasculature (PFV) from retinal detachment (RD) may be very challenging clinically and ultrasonographically, as they share common morphological features. However, it is crucial, considering their substantially distinct management and treatment. We aimed to assess the relevance of quantitative colour Doppler flow imaging to distinguish PFV from RD in children. METHODS: This retrospective bi-centre study included 66 children (30 females and 36 males, mean age: 244 ± 257 days) with a clinically suspected diagnosis of RD or posterior PFV. All children underwent systematic and standardized conventional ultrasonography and colour Doppler flow imaging under general anaesthesia with a qualitative and quantitative analysis of the retrolental tissue's vascularization. Peak systolic velocity, end-diastolic velocity and resistive index were recorded for analysis. Whenever available, surgical findings were deemed gold standard for diagnosis. A Mann-Whitney U-test was used to compare quantitative colour Doppler flow imaging data. RESULTS: Peak systolic velocity and end-diastolic velocity were significantly lower in children with PFV versus RD: 2.7 (IQR: 0.5) versus 5.1 (IQR: 2.8), p < 0.001, and 0.0 (IQR: 0.0) versus 2.0 (IQR: 1.2), p < 0.001, respectively. Resistive index was significantly higher in children with PFV versus RD: 1 (IQR: 0) versus 0.6 (IQR: 0.1), p < 0.001. Area under curves (AUCs) were of 0.94, 0.99 and 1, respectively. No differences between PFV and RD were observed on structural ultrasound or qualitative analysis of colour Doppler. CONCLUSION: Quantitative colour Doppler flow imaging has an excellent accuracy in distinguishing PFV from RD in children. It may help to improve management and treatment.
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Vítreo Primário Hiperplásico Persistente/diagnóstico por imagem , Descolamento Retiniano/diagnóstico por imagem , Ultrassonografia Doppler em Cores/normas , Velocidade do Fluxo Sanguíneo , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Masculino , Vítreo Primário Hiperplásico Persistente/patologia , Curva ROC , Descolamento Retiniano/patologia , Estudos RetrospectivosRESUMO
We report the 51/2 year prevalence of visual and oculomotor impairments in preterm children born at 24−34 weeks' gestation (WG) using the population-based cohort study EPIPAGE-2, set in France, 2011. The main outcomes were imputed prevalence of refractive errors (REs), strabismus, and binocular visual acuity (VA). Children were clinically assessed by specially trained pediatricians. The population was also analyzed in terms of cerebral palsy at 51/2 years (no CP, stage 1, stage 2, or stage 3−5) and retinopathy of prematurity in the neonatal period (no ROP, stage 1 or 2, or severe ROP). Among the 4441 children included, 2718 (weighted percentage 58.7%) were clinically assessed. REs were reported in 43.1% (95% confidence interval 37.6−48.4), 35.2% (32.7−37.6), and 28.4% (25.0−31.8) of children born at 24−26, 27−31, and 32−34 WG (p < 0.01), respectively; strabismus rates were 19.5% (14.6−24.4), 14.8% (12.9−16.7), and 8.3% (6.2−10.4) (p < 0.001), respectively. Moderate/severe visual deficiencies (VA < 3.2/10) were present in 1.7% (0.2−3.3) of children born at 24−26 WG, and in less than 1% in other groups. A suboptimal VA 5/10−6.3/10 was measured in 40.6% (35.3−45.8) of children born at 24−26 WG, 35.8% (33.5−38.1) at 27−31 WG, and 33.7% (30.4−37.0) at 32−34 WG. CP and ROP were associated with strabismus and RE. The association between CP and VA was strong, while it was not observed for ROP. In this large cohort of preterm-born children, we found a high prevalence of RE and strabismus regardless of WG, supporting the need for specific attention in this population. High prevalence of suboptimal VA could be challenging for these children at the age of reading and writing acquisition.
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This study aims to assess the prevalence and characteristics of preterm infants with retinopathy of prematurity (ROP) treated outside the recommended guidelines. In this retrospective monocentric cohort, we included all premature children treated in our department for ROP by laser photoablation or anti-VEGF intravitreal injection. The main outcome was treatment of both eyes for ROP less severe than pre-threshold type 1, treated outside ETROP guidelines. A total of 114 children received treatment for ROP in our department, among whom 32 (28.1%) children received treatment for indications outside the ETROP guidelines for both eyes. The indications outside the guidelines were persistent stage 2 or 3 ROP that showed no evidence of regression after 41 weeks of corrected gestational age (11 children; 34.4%), pre-plus stage (11; 34.4%), difficulties in disease staging (7; 21.9%), type 2 ROP with plus disease (2; 6.2%), and treatment due to logistical difficulties (1; 3.1%; hospitalized in neonatal units hundreds of miles away from our department, with no fundus examination possible in the neonatal unit). To resume, in our cohort, 28.1% of children received treatment for ROP less severe than pre-threshold type 1 both eyes. The main indications for off-label treatment were the persistence of active ROP during follow-up and the presence of pre-plus-stage disease. Our data suggest the need to update ROP treatment criteria to reflect real-life practices. Additional studies are required in order to evaluate the long-term benefits and side effects of treatments outside the recommended indications, and to establish revised treatment guidelines.
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INTRODUCTION: Retinopathy of prematurity (ROP) is a blinding disease that requires screening by retinal examination. Screening practices are rarely evaluated. We aimed to determine the prevalence of ROP screening in very preterm infants and individual- and center-related factors associated with ROP screening. METHODS: Data were extracted from the EPIPAGE-2 cohort, a French prospective population-based study of premature births in 2011. Children born before 32 weeks' gestation (WG) without severe malformation and alive at the recommended time for ROP screening were included. Outcome measures were achievement of ROP screening and compliance with recommended screening timeline. Individual- and center-related factors associated with both measures were studied using mixed models. RESULTS: Among 3,077 eligible infants, 2,169 (70.5%) had a ROP screening, ranging from 96% at 24 WG to 50% at 31 WG. Large variability among units was observed. Individual characteristics associated with screening were low gestational age, low birth weight, severe bronchopulmonary dysplasia or neurological lesions, and transfer between neonatal units during the screening period. Odds of screening were higher in neonatal units using wide-angle imaging (odds ratio 2.65 [95% confidence interval 1.17-6.01]) but decreased in units without a local protocol for ROP screening (0.03 [0.01-0.09]). Among screened children, 1,641/2,169 (75.7%) were screened according to recommended timeline. Delayed screening was associated with low gestational age, severe bronchopulmonary dysplasia or necrotizing enterocolitis, and absence of local protocol for ROP screening. DISCUSSION/CONCLUSIONS: In this large cohort study of infants born very preterm, almost one-third were not screened for ROP. Children most at risk for ROP were the best screened but often with delay. The higher compliance of neonatal units using wide-angle imaging systems supports its use.