Advanced Surface Ablation in a Patient With Suspect Topography: A Case Report.

The purpose of this clinical report is to describe a 10-year clinical outcome of advanced surface ablation with photorefractive keratectomy (PRK) in a patient who had been previously incorrectly diagnosed with keratoconus (KC). Corneal ectasia is a rare but extremely relevant complication of laser vision correction, and KC represents a major contraindication for these procedures. Nonetheless, some surface ablation procedures, such as PRK, might be a valid option for particular patients with atypical corneal topography or subclinical or mild forms of KC. Patient education and complete preoperative refractive multimodal imaging are essential for a more conscious therapeutic decision, minimizing iatrogenic ectasia, as well as decreasing the number of patients who are incorrectly denied refractive surgery, as was the patient presented in this study.

Nationwide consensus on quality indicators to assess glaucoma care: A modified Delphi approach.

PURPOSE: Performance assessments are essential to tracking and improving quality in health care systems. Key aspects of the care process that act as indicators must be measured in order to gain an in-depth understanding of a care unit's operation. Without standardized quality indicators (QIs), it is difficult to characterize and compare the abilities of institutions to achieve excellence. The aim of this study is to reach a consensus among glaucoma specialists concerning the development of a set of QIs to assess the performance of glaucoma care units. METHODS: A two-round Delphi technique was performed among glaucoma specialists in Portugal, using a 7-point Likert scale. Fifty-three initial statements (comprising process, structure, and outcome indicators) were evaluated and participants had to agree on which ones would be part of the final set of QIs. RESULTS: By the end of both rounds, 28 glaucoma specialists reached consensus on 30/53 (57%) statements, including 19 (63%) process indicators (mainly relating to the proper implementation of complementary exams and the setting of follow-up intervals), 6 (20%) structure indicators, and 5 (17%) outcome indicators. Of the indicators that were part of the final list, functional and structural aspects of glaucoma progression and the availability of surgical/laser procedures were the most prevalent. CONCLUSIONS: A set of 30 QIs for measuring the performance of glaucoma units was developed using a consensus methodology involving experts in the field. Their use as measurement standards would provide important information about unit operations and allow further implementation of quality improvements.

Effect of Repeated Intravitreal Injections in Glaucoma Spectrum Diseases.

Purpose: To evaluate whether repeated intravitreal injections (IVI) with an anti-vascular endothelial growth factor (anti-VEGF) agent are associated with glaucomatous progression in eyes with glaucoma spectrum diseases (GSD). Methods: Single-center, retrospective, longitudinal study of patients with bilateral and similar GSD who: (1) received ≥8 IVI in only one eye during the study period; (2) had ≥2 retinal nerve fiber layer thickness (RNFL) measurements obtained by spectral-domain optical coherence tomography (SD-OCT) at least 12 months apart. The primary outcome was the absolute RNFL thickness change, comparing injected and fellow uninjected eyes. Linear mixed effects models were constructed, including a multivariable model. Results: Sixty-eight eyes from 34 patients were included, 34 injected and 34 fellow uninjected eyes. Average baseline age was 67.68±21.77 years with a follow-up of 3.66±1.89 years and 25.12±14.49 IVI. RNFL thickness decreased significantly from 80.92±15.78 to 77.20±17.35 µm (p<0.001; -1.18±1.93 µm/year) in injected eyes and from 79.95±17.91 to 76.61±17.97 µm (p<0.001; -1.07±0.98 µm/year) in uninjected eyes. In a multivariable linear mixed model of injected eyes, only higher baseline RNFL thickness (p < 0.001) significantly predicted higher absolute RNFL thickness loss. Neither absolute RNFL thickness variation (p=0.716) nor RNFL rate (p=0.779) was significantly different between paired injected and uninjected eyes. Absolute IOP variation was not significantly different between groups (16.62±4.77 to 15.09±4.34 mmHg in injected eyes and 17.68±5.01 to 14.50±3.39 mmHg in fellow uninjected eyes; p=0.248). The proportion of eyes receiving glaucoma medical treatment increased significantly in both groups (55.9% to 76.5% in injected eyes; p=0.039; 58.8% to 76.5% in uninjected eyes; p = 0.031). The number of glaucoma medications also increased significantly in both groups (1.03±1.11 to 1.59±1.18 glaucoma medications in injected eyes; p=0.003; 1.09±1.11 to 1.56±1.19 glaucoma medications in uninjected eyes; p=0.003). Conclusion: Repeated IVI do not seem to accelerate glaucomatous progression. Future studies with a longer follow-up are needed.

Terson Syndrome - Clinical Presentation, Management, and Visual Outcomes in a Tertiary Centre.

Purpose: The purpose of this study was to characterize the clinical presentation, management strategy and visual outcomes of patients diagnosed with Terson syndrome and followed in a tertiary centre in Portugal. Patients and Methods: A single-centre retrospective study was performed, based on the survey review of the medical records of every consecutive patient diagnosed with Terson syndrome and followed from January 2018 to August 2021. The change in best-corrected visual acuity (BCVA) from baseline to the final evaluation was the primary outcome. Results: Fifteen eyes from 8 patients (50% female) were included. The mean age at diagnosis was 55±7 years. The neurological event was traumatic brain injury in 37.5% (n=3) and subarachnoid haemorrhage in 62.5% of the patients (n=5). Bilateral intraocular haemorrhage occurred in 875% (n=7) of the patients. Vitreous and preretinal haemorrhages occurred each in 66.7% (n=10), intraretinal in 30% (n=3) and subretinal in 13.3% (n=2) of the eyes. In 40% of the eyes (n=6), spontaneous resolution of intraocular haemorrhage occurred, while PPV was performed in the remaining 60% (n=9). Ocular haemorrhage detection occurred 58.47 ± 40.94 days after the neurological event (range 11 to 121 days). Baseline BCVA was 1.11 ± 1.01 logMAR and improved to 0.32 ± 0.69 logMAR in the follow-up period (p=0.004). A positive correlation was found between initial and final BCVA (Spearman's rho = 0.643, p=0.01). Baseline BCVA of eyes undergoing PPV was lower than of those conservatively managed (1.84±0.72 vs 0.20±0.28 logMAR, p<0.001). However, there were no statistically significant differences in final BCVA after surgery or observation (0.56 ± 0.90 vs 0.04 ± 0.04 logMAR, p=0.149). Longer periods between the neurological and the ophthalmological diagnosis were correlated with worse final BCVA (Spearman's rho = 0.688, p=0.005). Conclusion: Terson syndrome is a potential cause of irreversible visual loss. Diagnosis delay may affect visual prognosis. PPV is indicated when intraocular haemorrhage is dense and does not resolve spontaneously or when visual acuity at presentation is low, allowing for good visual outcomes with minimal complications.

Intravitreal Fluocinolone Acetonide Implant (FAc, 0.19 mg, ILUVIEN®) in the Treatment of Patients with Recurrent Cystoid Macular Edema After Pars Plana Vitrectomy.

INTRODUCTION: Postoperative cystoid macular edema (PCME) is a complication of several ocular procedures, including pars plana vitrectomy (PPV), due to the activation of the inflammatory cascade. The purpose of this case series is to evaluate the effectiveness and safety of fluocinolone acetonide intravitreal implant (FAc, 0.2 µg/day; ILUVIEN®) in the treatment of refractory PCME after successful PPV. METHODS: This retrospective observational case series includes consecutive eyes of patients with recurrent PCME after PPV and treated with a single FAc implant at Centro Hospitalar Universitário de São João, Porto, Portugal. Previous treatments, best-corrected visual acuity (BCVA, ETDRS letters), central macular thickness (CMT, µm), intraocular pressure (IOP, mmHg), and IOP-lowering medication needed were recorded at baseline and during follow-up. Total macular edema resolution was defined as CMT less than 300 µm or a reduction of greater than 20%, and partial macular edema resolution was defined as a reduction of greater than 10%. RESULTS: Nine eyes from nine patients were included. Before FAc implant, all eyes received intravitreal short-action corticosteroids (triamcinolone and dexamethasone implant), with a good response but relapse 1-5 months later. At baseline, BCVA was 55.0 ± 10.6 letters, CMT was 514.9 ± 165.6 µm, and IOP was 15.4 ± 2.4 mmHg with four eyes under IOP-lowering medication. After FAc implant, all eyes achieved edema resolution (eight total and one partial) with a peak gain of 17.2 letters and a maximum decrease of 208.2 µm in CMT. During follow-up (44.0 ± 14.8 months), 66.7% of the eyes kept their macula dry and three showed recurrence after 11, 14, and 28 months, respectively. The maximum IOP registered was 17.0 ± 6.0 mmHg. IOP-lowering regimen was increased in one eye and two additional eyes started hypotensive drops. CONCLUSION: FAc implant can be considered a therapeutic alternative in PCME refractory to other therapies in vitrectomized eyes, reducing the need for repeated treatments.

Retinal and choroidal vasoreactivity in central serous chorioretinopathy.

PURPOSE: This study aims to investigate retinal and choroidal vascular reactivity to carbogen in central serous chorioretinopathy (CSC) patients. METHODS: An experimental pilot study including 68 eyes from 20 CSC patients and 14 age and sex-matched controls was performed. The participants inhaled carbogen (5% CO2 + 95% O2) for 2 min through a high-concentration disposable mask. A 30° disc-centered fundus imaging using infra-red (IR) and macular spectral domain optical coherence tomography (SD-OCT) using the enhanced depth imaging (EDI) technique was performed, both at baseline and after a 2-min gas exposure. A parametric model fitting-based approach for automatic retinal blood vessel caliber estimation was used to assess the mean variation in both arterial and venous vasculature. Choroidal thickness was measured in two different ways: the subfoveal choroidal thickness (SFCT) was calculated using a manual caliper and the mean central choroidal thickness (MCCT) was assessed using an automatic software. RESULTS: No significant differences were detected in baseline hemodynamic parameters between both groups. A significant positive correlation was found between the participants' age and arterial diameter variation (p < 0.001, r = 0.447), meaning that younger participants presented a more vasoconstrictive response (negative variation) than older ones. No significant differences were detected in the vasoreactive response between CSC and controls for both arterial and venous vessels (p = 0.63 and p = 0.85, respectively). Although the vascular reactivity was not related to the activity of CSC, it was related to the time of disease, for both the arterial (p = 0.02, r = 0.381) and venous (p = 0.001, r = 0.530) beds. SFCT and MCCT were highly correlated (r = 0.830, p < 0.001). Both SFCT and MCCT significantly increased in CSC patients (p < 0.001 and p < 0.001) but not in controls (p = 0.059 and 0.247). A significant negative correlation between CSC patients' age and MCCT variation (r = - 0.340, p = 0.049) was detected. In CSC patients, the choroidal thickness variation was not related to the activity state, time of disease, or previous photodynamic treatment. CONCLUSION: Vasoreactivity to carbogen was similar in the retinal vessels but significantly higher in the choroidal vessels of CSC patients when compared to controls, strengthening the hypothesis of a choroidal regulation dysfunction in this pathology.

Cerebral Neurovascular Coupling Impairment in Central Serous Chorioretinopathy.

BACKGROUND: Central serous chorioretinopathy (CSCR) is a chorioretinal disorder resulting from choroidal hyperpermeability. Its comorbidities as hypertension, coronary disease, and psychological stress, suggest that it might reflect a more generalized vascular dysfunction. OBJECTIVES: The aim of the study was to assess the cerebrovascular regulation integrity, using cerebral autoregulation (CA), carbon dioxide vasoreactivity (VR), and neurovascular coupling (NVC) in CSCR. METHODS: This observational pilot study included 20 CSCR patients and 14 age- and sex-matched controls. A State-Trait Anxiety Inventory (STAI) inquiry was full-filled. Continuous measurement of cerebral blood flow velocity (CBFV), arterial blood pressure, heart rate, and end-tidal carbon dioxide was performed. VR was assessed during hypercapnia (inhaling carbogen gas) and hypnocapnia (hyperventilation). For NVC, the CBFV relative increase during mental activation using the N-Back Task was calculated. RESULTS: No significant differences in systemic hemodynamic parameters, CA or VR, were found between both groups. During the NVC performance, the average CBFV rise during mental stress was significantly lower in CSCR (p = 0.011). A significant negative correlation was found between STAI scores and NVC. CONCLUSIONS: CSCR patients presented a significantly impaired cerebral NVC compared to controls, supporting the theory of a potential systemic vascular dysfunction. Stress could be related to this NVC impairment.

Cone dystrophy with supernormal rod responses: A rare KCNV2 gene variant.

PURPOSE: To describe the clinical, electrophysiological, and genetic findings of three Portuguese families with a rare variant in the KCNV2 gene resulting in "cone dystrophy with supernormal rod responses" (CDSRR). METHODS: Retrospective clinical revision of five individuals from three unrelated families with CDSRR. Ophthalmological examination was described in all patients and included color vision testing, fundus photography, fundus autofluorescence (FAF) imaging, spectral domain-optical coherence tomography (SD-OCT), pattern electroretinogram (ERG), and full-field ERG. The mutational screening of the KCNV2 gene was performed with Sanger and Next Generation Sequencing. RESULTS: All patients showed childhood-onset photophobia and progressive visual acuity loss with varying degrees of severity. In multimodal imaging, various degrees of retinal pigment epithelium disturbances and outer retinal atrophy, which tend to be worst with advancing age, were observed. Molecular screening identified a rare presumed truncating variant (p.Glu209Ter) in homozygosity in two families and in compound heterozygosity in a third family. Three patients showed ERG changes characteristic of CDSRR, however, two patients presented with incomplete electrophysiological features of the disease. CONCLUSION: A rare variant in the KCNV2 gene was identified in five patients from three Portuguese families. This variant often leads to a severe and progressive form of retinopathy. Considerable variability in the ERG responses among patients with this KCNV2 variant was observed.

Optical coherence tomography angiography changes in cardiovascular systemic diseases and risk factors: A Review.

Cardiovascular (CV) disease (CVD) is the main cause of death around the world, and assessing a patient's CV risk factors (CVRF) can play a major role in its prevention. Since it has been shown that retinal vascular alterations may reflect several systemic processes such as CVRF, we conducted a systematic review in order to summarize which ocular microvasculature changes can be found using Optical Coherence Tomography Angiography (OCTA) in patients without ocular diseases and with systemic pathologies/conditions that affect the CV system when compared to healthy subjects. We searched on online databases, namely PubMed, Scopus, Cochrane and Web of Science, and obtained additional studies through citation tracking. Case reports and review articles were excluded. A total of 47 articles were included in our review. We describe that patients with hypertension, diabetes mellitus, kidney disease, preeclampsia, coronary artery disease, carotid artery stenosis and obstructive sleep apnoea syndrome have, in general, lower retinal and choroidal Vessel Density (VD) and Length (VL), as well as an increased foveal avascular zone area and perimeter. Additionally, several characteristics and/or conditions in healthy subjects, such as smoking status, hyper or hypoxia conditions, race, among others, are also related to ocular vascular changes and should be accounted for. We concluded that OCTA could be a useful tool to assess a patient's CV risk profile in a non-invasive way, possibly integrating the diagnostic and prognostic algorithms of the most prevalent CV diseases in the future.

Longitudinal multimodal functional macular analysis after half-dose photodynamic therapy for central serous chorioretinopathy.

BACKGROUND: Half-dose photodynamic therapy (HD-PDT) has been widely used for central serous chorioretinopathy (CSC) with good anatomical results. However, long-term functional outcomes after this treatment remain uncertain. This study aimed a longitudinal multimodal macular assessment, correlating functional and anatomical outcomes. METHODS: This is a retrospective study performed in a tertiary referral center including 111 eyes from 95 CSC patients. Data on best corrected visual acuity (BCVA), central macular thickness (CMT), central retinal sensitivity (CRS) using microperimetry (MP) and multifocal electroretinography (mfERG) at baseline and 3, 6, 12, 18, 24, 36, 48 and 60 months after treatment were registered. A correlation analysis was performed. RESULTS: Mean follow-up was 34.5 ± 26.3 months. A significant improvement in BCVA and CMT was registered in all the visits. CRS significantly improved until 24 months (p < 0.001 at 12 months, p < 0.05 at 24 months), worsening afterwards. The mfERG amplitude of N1 and P1 waves significantly improved in the first 12 months, aggravating afterwards. The implicit time improved until 24 months, deteriorating after 48 months. This long-term decline was also described in some inactive untreated fellow eyes CONCLUSIONS: A multimodal longitudinal analysis of CSC patients after HD-PDT shows that, after the first 12 to 24 months, the significant sustained improvement in BCVA and CMT is not paired by a sustained improvement in macular sensitivity or electrical response. This long-term functional deterioration might result from the disease itself and not directly from the treatment .

Cross-sectional study investigating the prevalence and causes of vision impairment in Northwest Portugal using capture-recapture.

OBJECTIVES: The aim of this study was to estimate the prevalence and causes of vision impairment (VI) in Portugal. SETTING: Information about people with VI was obtained from primary care centres, blind association (ACAPO) and from hospitals (the PCVIP study) in the Northwest of Portugal during a period spanning years 2014-2015. Causes of VI were obtained from hospitals. PARTICIPANTS: Administrative and medical records of people with visual acuity in the better seeing eye of 0.5 decimal (0.30logMAR) or worse and/or visual field less than 20° were investigated. Capture-recapture with log-linear models was applied to estimate the number of individuals missing from lists of cases obtained from available sources. PRIMARY AND SECONDARY OUTCOME MEASURES: Log-linear models were used to estimate the crude prevalence and the category specific prevalence of VI. RESULTS: Crude prevalence of VI was 1.97% (95% CI 1.56% to 2.54%), and standardised prevalence was 1% (95% CI 0.78% to 1.27%). The age-specific prevalence was 3.27% (95% CI 2.36% to 4.90%), older than 64 years, 0.64% (95% CI 0.49% to 0.88%), aged 25-64 years, and 0.07% (95% CI 0.045% to 0.13%), aged less than 25 years. The female-to-male ratio was 1.3, that is, higher prevalence among females. The five leading causes of VI were diabetic retinopathy, cataract, age-related macular degeneration, glaucoma and disorders of the globe. CONCLUSIONS: The prevalence of VI in Portugal was within the expected range and in line with other European countries. A significant number of cases of VI might be due to preventable cases and, therefore, a reduction of the prevalence of VI in Portugal seems possible. Women and old people were more likely to have VI and, therefore, these groups require extra attention. Future studies are necessary to characterise temporal changes in prevalence of VI in Portugal.

Scleral Fixation of Akreos AO60 Intraocular Lens Using Gore-Tex Suture: An Eye on Visual Outcomes and Postoperative Complications.

PURPOSE: "In-the-bag" placement of an IOL is the Holy Grail for any cataract surgeon. However, in the absence of capsular integrity, alternative surgical options to place the IOL must be sought. We aim to report the clinical outcomes and safety profile of scleral-fixated Akreos AO60 intraocular lens implantation using Gore-Tex suture, combined with pars plana vitrectomy. METHODS: This is a single-center, retrospective case series descriptive study. Electronic clinical records of all patients subjected to scleral fixation of a Bausch and Lomb Akreos AO60 IOL combined with pars plana vitrectomy, between April 1, 2017, and August 1, 2021, were reviewed. Data concerning age, sex, laterality, past ophthalmological history, pre- and postoperative best-available visual acuity, surgical indication, and intra- and postoperative complications were collected. Measured outcomes were the differences in best-available visual acuity and frequency of postoperative complications. RESULTS: A total of 37 eyes (20 right eyes and 17 left eyes) from 36 patients (16 females and 20 males) were included in the statistical analysis. The mean age at time of surgery was 72.0 ± 12.4 years. The mean follow-up period was 548.9 days (range 39-1564 days). Globally, the mean best-available logMAR visual acuity improved from 1.61 preoperatively (0.025 decimal equivalent) to 0.57 postoperatively (0.3 decimal equivalent), this difference being statistically significant (P < 0.001). Indications for surgery included aphakia due to complicated cataract surgery (24.3%; n = 9); subluxated IOL due to closed trauma (21.6%; n = 8); PEX-related subluxated IOL (16.2%; n = 6); non-traumatic, non-PEX-related subluxated IOL (18.9%; n = 7); subluxated crystalline lens due to closed trauma (8.1%; n = 3); aphakia due to open-globe injury (5.4%; n = 2); silicone-induced IOL opacification (2.7%; n = 1); and aphakia post-endophthalmitis (2.7%; n = 1). Postoperative complications included transient ocular hypertension (27.0%; n = 10), transient corneal edema (18.9%; n = 7), cystoid macular edema (18.9%, n = 7), self-limited hypotension (5.4%, n = 2), self-limited vitreous hemorrhage (2.7%, n = 1), central retinal vein occlusion (2.7%, n = 1), late retinal detachment (2.7%, n = 1), and Akreos IOL opacification (2.7%, n = 1). No suture-related complications were observed. CONCLUSION: There was a statistically significant improvement in visual acuity after scleral fixation of Akreos AO60 intraocular lens using Gore-Tex suture, with no suture-related problems recorded. This procedure seems to be a valuable alternative for posterior chamber IOL placement when secondary IOL implantation is required.

Endophthalmitis following Intravitreal Injection, Cataract Surgery, and Vitrectomy: Clinical Features and Visual Outcomes.

PURPOSE: To describe and compare the clinical features and visual outcomes of endophthalmitis following intravitreal injections (IVI), cataract surgery, and pars plana vitrectomy (PPV). METHODS: This is a single-centre, retrospective study. All included patients had acute postoperative endophthalmitis secondary to one of these three procedures. Visual acuity (VA), comorbidities, time to presentation, and treatment were assessed. The primary outcome was visual outcome. A poor outcome was considered if final VA was worse than or equal to counting fingers (CF) and a good outcome was classified as VA better than CF. RESULTS: Over 12 years, a total of 61 patients were included. Twenty-seven cases were post-cataract endophthalmitis; twenty-five were post-IVI and nine post-PPV. Endophthalmitis post-PPV had a worse visual outcome (88.9% of patients with VA worse than or equal to CF 95% CI 51.3 to 100.0%) than endophthalmitis following cataract surgery (25.9% of patients with VA worse than or equal to CF 95% CI 11.0 to 39.9%) and the IVI subgroup (44.0% of VA worse than or equal to CF 95% CI 24.0 to 67.0%) (p=0.001 and p=0.047). There were no significant differences in the proportion of patients with a poor visual outcome between endophthalmitis following cataract surgery and IVI (p=0.171). CONCLUSIONS: The number of patients with poor visual outcomes following acute endophthalmitis was similar in endophthalmitis following IVI and cataract surgery, but better than endophthalmitis following vitrectomy.

Prone Positioning Covid-19 Patients: A Double-Edged Sword-A Case Report of a Devastating Ocular Complication.

In the late 1970s, prone positioning was established as an efficient treatment for acute respiratory distress syndrome (ARDS). Currently, with the world facing a global health crisis due to the COVID-19 pandemic, it has become an accepted routine practice in intensive care units dealing with critically ill COVID-19 patients. Ophthalmic complications associated with the prone position are not a novelty in clinical practice. Indeed, it is estimated that in patients undergoing spine surgery, prone positioning carries a tenfold increased risk of eye injury when compared to supine and lateral positioning. The majority of these complications are treatable ocular surface disorders, but irreversible sight-threatening conditions also occur. We report a unique and dramatic case of a ruptured globe in a COVID-19 patient placed in prolonged prone position, emphasizing its difficult diagnosis and management while focusing on life-saving support.

A Scoping Review of Process Indicators for Measuring Quality of Care in Glaucoma.

PRCIS: There are no standardized process quality indicators (QIs) in glaucoma care. Although they can be inferred from guidelines and trials, they should be designed and standardized to allow better assessment of the quality of care. PURPOSE: QIs are crucial for assessing the performance of any health care system. To allow efficiency, effectiveness, and patient-centeredness, there is a need for prompt acquisition of up-to-date information. Among the available QIs, process indicators have the highest sensitivity to frequent changes and could better reflect the implementation outcomes of novel ideas and technology. This study aimed to map the available information regarding process QIs in glaucoma care, identify the current development stage of these indicators, and systematically synthesize them. MATERIALS AND METHODS: We performed a scoping review of 4 electronic bibliographic databases for studies reporting on process QIs in glaucoma. We retrieved 7502 references and created a domain list reflecting the core idea underlying each indicator. RESULTS: We summarized information from 18 documents and listed 20 domains. The most mentioned domains were follow-up, optic nerve head assessment, visual field test, and intraocular pressure. Indicators regarding the quality of life assessment, patient assistance, or presence of written protocols were less frequently mentioned. CONCLUSIONS: There are notable variations among process QIs in glaucoma and significant heterogeneity in their descriptions in published studies. Although novel indicators can be inferred from guidelines and trials, they should be designed and standardized for better assessment of performance in health systems to improve their quality.

Child with a mild phenotype of Incontinentia Pigmenti and inner retinal dysfunction.

PURPOSE: To describe a case of a child with mild phenotype of Incontinentia Pigmenti (IP), with changes in Spectral-Domain Optical Coherence Tomography (SD-OCT) and Optical Coherence Tomography Angiography (OCT-A) and an electronegative dark-adapted (DA) 3.0 electroretinogram (ERG), suggestive of inner retinal dysfunction. CASE REPORT: We described a 7-year-old female child with IP. Her best corrected acuity was 8/10 in the right eye and 6/10 in the left eye. Biomicroscopy, intraocular pressure and fundoscopy were normal. The electroretinography findings showed an electronegative DA 3.0 ERG with a normal a-wave but a b-wave that did not elevate above baseline. SD-OCT identified irregularities in the outer plexiform layer in both eyes, and OCT-A assessment revealed at the superficial capillary plexus, areas of decrease in the flow in parafoveal and perifoveal regions. CONCLUSION: Classically, IP affects the peripheral retina; however, vascular and structural changes in macula can occur as well. To our knowledge, we report the first electronegative electroretinogram in a patient with IP.

Long term outer retinal changes in central serous chorioretinopathy submitted to half-dose photodynamic therapy.

PURPOSE: To evaluate long-term changes in the foveal and parafoveal outer retina after half-dose photodynamic therapy (HD-PDT) in central serous chorioretinopathy (CSC). METHODS: Retrospective study including CSC patients submitted to HD-PDT. Best corrected visual acuity (BCVA) was evaluated. Spectral-domain optical coherence tomography automatic segmentation algorithm was used and data on retinal, inner retinal, outer retinal and outer nuclear layers (ONL) in both foveal 1 mm (C) and parafoveal 3 mm ETDRS circles for the superior, nasal, inferior and temporal sectors, were obtained at baseline and 3, 12 and 24 months post-treatment. Subfoveal choroidal thickness, photoreceptors' outer segment thickness, subretinal fluid (SRF) height and width were also measured. RESULTS: Twenty-one eyes of 15 patients were included. At baseline, the mean ONL thickness in the foveal area was significantly thinner in affected eyes compared to their fellow unaffected ones (55,50 ± 32,75 µm vs 93,00 ± 17,0 µm; p = 0,011), and was negatively correlated to logMAR BCVA (R=-0,601, p = 0,008) ONL thickness increased by 10,94 ± 11,88 µm at 24 months in the foveal area, and all the parafoveal sectors presented a similar increase. Baseline SRF width was significantly correlated with baseline BCVA (R1 = 0,483, p = 0,036), and with ONL thickness in all sectors. CONCLUSION: In our study we found a significant long-term increase in foveal and parafoveal ONL thickness in CSC after HD-PDT, suggesting that this seems to be a safe treatment for the outer retina. This is the first study mapping the outer retinal changes in the macular area to 24 months follow up.

Incidence of endophthalmitis after intravitreal injection with and without topical antibiotic prophylaxis.

PURPOSE: The purpose was to compare the incidence of endophthalmitis after intravitreal injection with and without topical antibiotic prophylaxis. METHODS: This is a single-center, retrospective case-control study. All patients treated with intravitreal injection of ranibizumab, bevacizumab, aflibercept, or corticosteroids for a variety of retinal vascular diseases between 1 October 2014 and 30 November 2018 were included. The total number of patients and injections were determined from a review of billing code and practice management records. Endophthalmitis cases were determined from billing records and then confirmed with chart review. A 24-month period when topical antibiotics were prescribed after intravitreal injection was compared with a 24-month period when topical antibiotics were not prescribed. RESULTS: Between 1 October 2014 and 30 November 2018, a total of 33,515 intravitreal injections were performed and 13 cases of post-intravitreal injection endophthalmitis were identified (incidence rate of 0.0388%; 95% confidence interval, 0.0217%-0.0644%) or approximately 1 case for every 2578 intravitreal injections. Between 1 October 2014 and 31 October 2016, while topical antibiotic prophylaxis was used postoperatively, 14,828 intravitreal injections were performed and 5 cases of endophthalmitis were reported (0.0337%; 95% confidence interval, 0.0129%-0.0739%). Between 1 November 2016 and 30 November 2018, while no prophylaxis was used, 18,687 intravitreal injections were performed and 8 cases of endophthalmitis were identified (0.0428%; 95% confidence interval, 0.0202%-0.0808%). There were no statistical differences in the incidence rates between the two groups (p = 0.675). CONCLUSION: The incidence rate of endophthalmitis in the group with topical antibiotic prophylaxis after intravitreal injection was similar to the group with no prophylaxis. Changing the current clinical practice to no antibiotic prophylaxis had no effect on the incidence of endophthalmitis.

Metabolomic profiling of aqueous humor from glaucoma patients - The metabolomics in surgical ophthalmological patients (MISO) study.

Glaucoma is still a poorly understood disease with a clear need for new biomarkers to help in diagnosis and potentially offer new therapeutic targets. We aimed to determine if the metabolic profile of aqueous humor (AH) as determined by nuclear magnetic resonance (NMR) spectroscopy allows the distinction between primary open-angle glaucoma patients and control subjects, and to distinguish between high-tension (POAG) and normal-tension glaucoma (NTG). We analysed the AH of patients with POAG, NTG and control subjects (n = 30/group). 1H NMR spectra were acquired using a 400 MHz spectrometer. Principle component analysis (PCA), machine learning algorithms and descriptive statistics were applied to analyse the metabolic variance between groups, identify the spectral regions, and hereby potential metabolites that can act as biomarkers for glaucoma. According to PCA, fourteen regions of the NMR spectra were significant in explaining the metabolic variance between the glaucoma and control groups, with no differences found between POAG and NTG groups. These regions were further used in building a classifier for separating glaucoma from control patients, which achieved an AUC of 0.93. Peak integration was performed on these regions and a statistical analysis, after false discovery rate correction and adjustment for the different perioperative topical drug regimen, revealed that five of them were significantly different between groups. The glaucoma group showed a higher content in regions typical for betaine and taurine, possibly linked to neuroprotective mechanisms, and also a higher content in regions that are typical for glutamate, which can indicate damaged neurons and oxidative stress. These results show how aqueous humor metabolomics based on NMR spectroscopy can distinguish glaucoma patients from controls with a high accuracy. Further studies are needed to validate these results in order to incorporate them in clinical practice.