The Impact of Early Photoscreening on Medium-term Visual Acuity: A Population-Based Study.

PURPOSE: To analyze the outcomes of an early ophthalmological intervention in children included in the pilot project of the "Rastreio de Saúde Visual Infantil" (RSVI) visual screening program in Portugal. METHODS: This was a retrospective analysis of the medical records of all children included in the RSVI from April 1, 2016 to December 31, 2016, and who were referred to an ophthalmology appointment. Data of refractive errors, anisometropia, amblyopia, instituted treatments, and visual acuity at the end of the ophthalmological intervention were collected. RESULTS: Two hundred sixty-seven (18.2%) 2-year-old children from the Centro Hospitalar Universitário do Porto reference area had a positive screening result and were subsequently referred to an ophthalmology appointment. Glasses were prescribed to 31.1% of the patients who attended. Presumed amblyopia was diagnosed in 2.5% and occlusion was prescribed. At the end of a median follow-up of 3 years, of those who wore glasses without occlusion, 94.3% had a visual acuity of 20/25 or better in both eyes with an asymmetry of two lines or less between eyes. Of the 4 children who wore glasses with occlusion, 3 of them had a visual acuity of 20/25 or better in both eyes with an asymmetry of two lines or less between eyes. CONCLUSIONS: This study demonstrates the effectiveness of an early intervention in the prevention and treatment of amblyopia, because after a median follow-up of 3 years after treatment none of the referred children met criteria for amblyopia and 94.3% of the referred children who had an intervention had a normal visual acuity. [J Pediatr Ophthalmol Strabismus. 2023;60(3):178-183.].

Pupillometry: An objective test to assess endocular hereditary transthyretin amyloidosis.

OBJECTIVE: To automatically study the pupillary light reflex in patients with hereditary transthyretin-associated amyloidosis (hATTR). METHODS: Prospective cross-sectional observational study in patients with hATTR with unilateral scalloped iris. Pupillary light reflex of scalloped iris eyes (21 eyes) were compared with non-scalloped iris eyes (21 eyes, paired eyes of the same patients) and also with a control group of 20 healthy eyes, using static and dynamic pupillometry with the Metrovision® MonPack One. RESULTS: No patient presented evident neurological involvment of the cranial nerves. No significant differences were found in the pupillary diameters under standardized lighting conditions (static pupillometry) among groups. In dynamic pupillometry, the amplitude of contraction, the velocity of contraction and the velocity of dilation were statistically significantly lower in eyes with scalloped iris, comparing both with the contralateral non-scalloped iris eyes (p < 0.001 for all) and with eyes from healthy subjects (p < 0.05 for all). CONCLUSION: A scalloped iris reflects a more advanced endocular hATTR and it is associated with an altered pupillary light reflex. Pupillometry may be a quick, simple, and portable test to objectively evaluate ocular amyloid deposition in hATTR eyes. Pupillary light reflex may not be reliable to evaluate neurological dysfunction in these patients.

Intravitreal Ranibizumab or Aflibercept After Bevacizumab in Diabetic Macular Edema: Exploratory Retrospective Analysis.

AIM: To evaluate the efficacy of switching from bevacizumab to ranibizumab or aflibercept in eyes with diabetic macular edema (DME) unresponsive to bevacizumab. METHODS: Single-center retrospective comparative study of patients with DME unresponsive to intravitreal bevacizumab that was switched to ranibizumab or aflibercept. Best-corrected visual acuity (BCVA) and central foveal thickness (CFT) were analysed prior to and 4 months after the switch. Ocular coherence tomography (OCT) biomarkers were also analysed. RESULTS: Fifty-six eyes from 40 patients were included in the study, 33 eyes switched to ranibizumab and 23 to aflibercept. A significant median CFT decrease was observed in both groups (p<0.001), with no between-group differences. BCVA gain was only significant in the ranibizumab group (p<0.001). None of the pre-baseline or baseline parameters were associated with the response to ranibizumab or aflibercept. CONCLUSION: In persistent DME unresponsive to bevacizumab, both anatomical and functional improvements were observed with ranibizumab whereas aflibercept only showed an anatomical improvement. Clinicaltrials.gov NCT04018833.

OPTICAL COHERENCE TOMOGRAPHY AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY PARAMETERS IN PATIENTS WITH PHACOMATOSIS.

PURPOSE: To compare the retinal vasculature characteristics between eyes of patients with and without phacomatosis. METHODS: Case-control observational study with retinal vasculature evaluation by optical coherence tomography and optical coherence tomography angiography of the macula and disk. RESULTS: The study included 80 eyes. Neurofibromatosis Type 1 patients presented with a higher central macular thickness (P = 0.007), a lower optical disk nervous fiber layer (P = 0.006), a lower perimeter, area, and circularity of the foveal avascular zone (P < 0.05), a higher vascular density of macular avascular layer (AMVD) (P = 0.004), and a lower papillary vascular density of superficial capillary plexus (SPVD) (P = 0.048). Patients with tuberous sclerosis presented with an increase in central macular thickness (P = 0.024) and in vascular densities (P < 0.05) [except for macular vascular density of deep capillary plexus (PMVD), AMVD, and SPVD]. Patients with Sturge-Weber syndrome showed a decrease in optical disk nervous fiber layer (P < 0.001), subfoveal choroid thickness (P = 0.011), macular vascular density of superficial capillary plexus (SMVD) (P = 0.036), and SPVD (P < 0.001). CONCLUSION: Phacomatosis patients showed statistically significant differences of retinal vasculature characteristics, compared to eyes without pathology. Further studies are needed to determine when and if these parameters change with the course of the disease and if they can be used as biomarkers for disease severity or progression.

Dealing with rhegmatogenous retinal detachment in patients under 40 years old: a tertiary center results.

PURPOSE: The risk of rhegmatogenous retinal detachment (RRD) increases with age, but some studies report a secondary peak in younger patients. Since visual recovery in RRD depends on surgical treatment and, considering the personal, social and economic burden of low vision in the working-age population, our purpose was to analyze the features and outcomes of RRD in young patients. METHODS: Clinical data of patients under 40 years old submitted to surgery for first time RRD, consecutively selected between 2016 and 2019, was analyzed. Patients with less than 3 months follow-up were excluded. RESULTS: Eighty-nine eyes from 89 patients were included. Mean age was 31.2 ± 7.8 years (minimum 10 years) and 56% were female gender. Most patients (63%) had high myopia. Pars plana vitrectomy (79%) alone, combined with scleral buckling (1%) or scleral buckling alone (20%) was performed. Primary anatomical success was 72%, and final anatomical success was 91%. Final visual acuity of 20/40 or better was achieved in 29% of cases, but 28% remained under 20/400. The presence of myopia (p = 0.022), localized RRD (p = 0.007) and attached macula at presentation (p < 0.001) was associated with a better final visual acuity. CONCLUSION: Management of RRD in young patients must be thorough. In younger patients, anatomical outcomes may be worse than in older patients. Myopia may be recognized as a major risk factor for RRD in this age group, but also as a protective factor for retinal function after surgery.

Comparison of Ocular Ultrasound Versus SD-OCT for Imaging of the Posterior Vitreous Status in Patients With DME.

BACKGROUND AND OBJECTIVES: To assess the percentage of vitreous adherence to the posterior pole in patients with diabetic macular edema (DME) with ocular ultrasonography (US) and establish a comparison with spectral-domain optical coherence tomography (SD-OCT). PATIENTS AND METHODS: Cross-sectional consecutive analysis of patients followed in a diabetic retinopathy consultation. Vitrectomized eyes and patients with epiretinal membranes were excluded. A comparison between macular SD-OCT 20 × 20°, SD-OCT 55 × 35°, and ocular US for the vitreous status was performed. A subanalysis of the percentage of eyes with thickened posterior hyaloid and focal vitreous macular adhesion (VMA) was determined with SD-OCT 20 × 20° and SD-OCT 55 × 35°. RESULTS: From 78 eyes of 39 patients, 55 eyes were included. All patients had type 2 diabetes mellitus with a median duration of 20 years (range: 3 to 40 years); 60% were phakic, and 61.8% were male. Previous treatments included intravitreal injections in 54.5% eyes, macular laser in 67.3%, and panretinal photocoagulation in 56.4%. All eyes had a non-posterior vitreous detachment (PVD) status on US. The 55 × 35° SD-OCT detected a non-PVD status in 96.4% (100% in video display mode) and a VMA in 87.3%. The 20 × 20° SD-OCT only detected a VMA in 43.6% of cases, with a thickened posterior hyaloid in 40% and a focal VMA in 18.2%. CONCLUSIONS: In the authors' DME patients, vitreous adherence to the posterior pole was highly prevalent, with a total agreement between US and SD-OCT 55 × 35° video display mode. SD-OCT 20 × 20° is not an accurate method to diagnose VMA compared to SD-OCT 55 × 35°. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:S50-S53.].

Phakic Intraocular Lens Implantation After Deep Anterior Lamellar Keratoplasty: Retrospective Case Series Analysis With Long-Term Follow-Up.

PURPOSE: To report outcomes of phakic intraocular lens (IOL) implantation after deep anterior lamellar keratoplasty (DALK) to correct high ametropia. SETTING: Centro Hospitalar Universitário do Porto, Portugal. METHODS: Retrospective case series with 11 eyes submitted to phakic IOL implantation after DALK. Main outcomes measured were uncorrected and corrected distance visual acuity (UDVA and CDVA), refractive error components, tomographic parameters and endothelial cell density (ECD). The minimum follow-up was 3 years for all cases. RESULTS: Mean ECD loss was 8.7±6.7% at 1 year, 13.1±8.6% at 3 years (n=11; p=0.016, p=0.007, respectively) and 14.0±20.4% at 5 years (n=5, p=0.212). Mean logMAR UDVA increased from 1.27±0.90logMAR preoperatively to 0.16±0.15logMAR postoperatively (p≤0.001) and no statistically significant differences were registered during follow-up. All patients gained at least 5 lines of UDVA. 54.5% of the eyes gained 1 line in CDVA postoperative and only one eye lost one CDVA line through follow-up. Efficacy and safety indexes at 1 and 3 years were 1.01-0.97 and 1.24-1.21, respectively. Mean spherical equivalent was reduced from -7.84±4.63 D preoperatively to -1.05±1.07 D postoperatively (p=0.001). Mean percentage of reduction in refractive cylinder and spherical error was 83.8±15.8% and 73.1±31.5%, respectively, p≤0.001 for both. In one eye there was a significantly gradual ECD loss over 5 years follow-up and the patient will be submitted to IOL explant. CONCLUSION: Phakic IOLs were effective for correction high ametropia after DALK, showing high efficacy and safety indexes with stability over time. However, it was registered a continuing endothelial cell loss postoperatively, which assumed to be higher than those reported in eyes without DALK.

Real-world retrospective comparison of 0.19 mg fluocinolone acetonide and 0.7 mg dexamethasone intravitreal implants for the treatment of diabetic macular edema in vitrectomized eyes.

PURPOSE: The aim of this study was to evaluate the long-term real-world effectiveness of FAc and DEX implants in vitrectomized DME eyes in a real-world setting. METHODS: This was a non-interventional, retrospective, comparative study of 46 vitrectomized eyes in 33 patients with persistent or recurrent DME quantified best-corrected visual acuity (BCVA), central foveal thickness (CFT) and intraocular pressure (IOP) over up to 37 months. RESULTS: Both FAc and DEX treatment led to statistically and clinically significant improvements in BCVA and CFT. FAc >10-letter improvement on the Early Treatment Diabetic Retinopathy Study [ETDRS] chart over months 3-24 and a sustained ~200 µm CFT reduction over months 1-24; DEX: >5-letter improvement on the ETDRS chart at months 1 and 3 and >100 µm CFT reduction at month 1. FAc demonstrated sustained, stable and predictable effects on BCVA and CFT over 24 months and also improved BCVA and decreased CFT in a cohort of DME eyes that was refractory to DEX over 6 months. CONCLUSION: This real-world study demonstrates long-term effectiveness of FAc in vitrectomized DME eyes and sustained effectiveness in DME eyes that did not respond to DEX therapy.

Aplicação do modelo WINROP no rastreio de Retinopatia de Prematuridade (ROP) numa amostra de prematuros portugueses / Application of the WINROP model in Retinopathy of Prematurity (ROP) screening in a Portuguese cohort of premature infants

Resumo Objetivo: Avaliar a sensibilidade e especificidade do algoritmo WINROP na deteção de retinopatia de prematuridade (ROP) numa amostra de prematuros portugueses. Métodos: Estudo retrospetivo que incluiu todos os recém-nascidos prematuros (RN) submetidos a rastreio de ROP no Centro Hospital Universitário do Porto entre Janeiro de 2010 a Maio de 2016. A idade gestacional (IG), peso à nascença e os pesos semanais dos RN, até uma idade pós-menstrual de 35-36 semanas, foram introduzidos na aplicação online do WINROP, que sinaliza com uma mensagem de alarme os RN em risco de desenvolver ROP tipo 1, que requer tratamento. Resultados: De um total de 496 RN submetidos a rastreio de ROP, 20 foram excluídos do estudo por registos incompletos e 101 por IG≥32 semanas. Dos RN com uma IG≥ 32 semanas, um desenvolveu ROP tipo 1 e foi submetido a tratamento. Dos 375 RN introduzidos no modelo WINROP, 231 (62%) registaram um sinal de alarme. Todos os RN com ROP tipo 1 foram identificados pelo sinal de alarme. O tempo médio entre o sinal de alarme e o tratamento foi de 11 semanas. O algoritmo WINROP apresentou uma sensibilidade e um valor preditivo negativo de 100% e uma especificidade de 42%. Conclusão: O modelo WINROP demonstrou ser sensível na deteção de prematuros com necessidade de tratamento. Embora com um valor de especificidade menor, a aplicação do algoritmo pode ajudar a reduzir substancialmente o número de exames realizados. Uma das limitações do modelo consiste na exclusão de RN com IG≥32 semanas.

Abstract Objective: To evaluate the sensitivity and specificity of the WINROP algorithm for predicting retinopathy of prematurity (ROP) in a population of Portuguese preterm infants. Methods: Retrospective study of all newborns who underwent ROP screening at Hospital Universitário do Porto from January 2010 to May 2016. Gestational age (GA), birthweight and weekly postnatal weight measurements of the newborns, up to a postmenstrual age of 35-36 weeks, were entered in the online application of WINROP, which signals with an alarm the newborns who are at risk of developing ROP type 1, which requires treatment. Results: Of a total of 496 RN undergoing ROP screening, 20 were excluded from the study by incomplete records and 101 by GA ≥32 weeks. Of the newborns with a GA≥32 weeks, one developed ROP type 1 and was treated. Of the 375 newborns introduced in the WINROP application, 231 recorded an alarm signal. All the newborns with type 1 ROP were identified by the alarm signal. The WINROP algorithm presented a sensitivity and a negative predictive value of 100% and a specificity of 41%. The mean time between the alarm signal and the treatment was 10 weeks plus 6 days. Conclusion: The WINROP model was 100% sensitive in the detection of preterm infants requiring treatment for ROP. Besides its lower specificity, WINROP application can reduce substantially the number of exams in ROP screening. One of the limitations of the model is the exclusion of newborns with GA≥32 weeks.

Latest Clinical Approaches in the Ocular Management of Cystinosis: A Review of Current Practice and Opinion from the Ophthalmology Cystinosis Forum.

Cystinosis, a rare autosomal recessive disease caused by intracellular cystine accumulation, occurs in an estimated 1/100,000-200,000 live births. Ocular non-nephropathic cystinosis is typically diagnosed during adulthood, when patients present with corneal crystal deposition and no systemic involvement. Due to the rarity of the condition, diagnosis is often delayed and can have a significant impact on the overall prognosis of the disease. Early diagnosis is therefore imperative to ensure successful treatment and improve quality of life, as most of its clinical manifestations can be prevented or delayed. Early detection strategies and practical approaches for the ocular management of cystinosis were discussed during the Ophthalmology Cystinosis Forum, a 1-day meeting held in Berlin, Germany during June 2017. Recommendations for early detection comprise ophthalmic assessment, including self- and clinician-assessed recording of photophobia, and visual acuity, slit-lamp examination and tonometry ophthalmic examinations. In vivo confocal microscopy and anterior segment optical coherence tomography were highlighted as valuable techniques in evaluating cystine crystals in the cornea, in vivo and non-invasively. The mainstay of ocular cystinosis treatment is the cystine-depleting aminothiol cysteamine. Indeed, early treatment with and strict adherence to cysteamine therapy has a considerable impact on the long-term prognosis of ocular cystinosis. In rare diseases such as ocular cystinosis, standardised guidelines and recommendations for detection, patient care and follow-up assessments are essential. Such guidelines provide a support tool for healthcare professionals caring for ocular cystinosis patients. Multidisciplinary teams (MDTs) are essential for delivering gold standard care and improving quality of life for patients and their families. This review paper highlights current early detection policies, clinical treatment strategies and practical approaches for the ocular management of cystinosis, including implementing a cystinosis MDT. Additionally, discussions of the Ophthalmology Cystinosis Forum held in 2017 are summarised. FUNDING: Orphan Europe. Plain language summary available for this article.

Implantation of intracorneal ring segments in pediatric patients: long-term follow-up.

PURPOSE: To analyze the long-term outcomes of intracorneal ring segments (ICRS) implantation for keratoconus management in pediatric patients. METHODS: Retrospective case series review of the long-term (>5 years) outcomes of Intacs® ICRS implantation for keratoconus in pediatric patients (age <18 years old at the time of surgery) between January 2008 and December 2011 at Ophthalmology Department of Hospital de Santo António. Demographic data, follow-up time, preoperative and postoperative uncorrected visual acuity (UCVA) and best-corrected visual acuity (BCVA) in decimal scale, and corneal topography were evaluated. Statistical analysis was done using SPSS for windows (version 24). Significance was set at p<0.0125. RESULTS: Fourteen eyes of 14 patients, with a mean age of 15.36 years (range 10-18 years), were included in this study. All patients had been diagnosed with keratoconus with reported progression in the 6 months prior to surgery. Follow-up time was 6.36±0.97 years. UCVA and BCVA improved after ICRS implantation (p<0.0125). Keratometry (K) minimum (Kmin) and K maximum (Kmax) decreased after surgery (p<0.0125). During follow-up, UCVA, BCVA, Kmin, and Kmax values ranged, showing a tendency to worsen at the end of follow-up. However, statistically significant differences were not observed. CONCLUSION: ICRS implantation showed good visual and topographic results in pediatric patients. Long-term follow-up suggests that, despite ICRS implantation, there is still progression of keratoconus. To the best of our knowledge, there are no reports regarding the long-term efficacy of ICRS implantation in pediatric patients.