Design and Development of a Web-Based Prospective Nationwide Registry for Ocular Inflammatory Diseases: UVEITE.PT - The Portuguese Ocular Inflammation Registry.

Uveitis is a heterogeneous collection of infrequent diseases, which poses significant challenges to cost-effective research in the field. Medical registries are being increasingly recognized as crucial tools to provide high-quality data, thus enabling prospective clinical research. This paper describes the design and technical structure development of an innovative countrywide electronic medical record for uveitis, Uveite.pt, and gives an overview of the cohort registered since its foundation, March 2020.Uveite.pt is an electronic medical record platform developed by the Portuguese Ocular Inflammation Group (POIG), a scientific committee of the Portuguese Ophthalmology Society. This is a nationwide customized web-based platform for uveitis patients useful for both clinical practice and real-world-based research, working as a central repository and reporting tool for uveitis. This paper describes the technical principles, the design and the development of a web-based interoperable registry for uveitis in Portugal and provides an overview of more than 400 patients registered in the first 18 months since inception.In infrequent diseases, the existence of registries enables to gather evidence and increase research possibilities to clinicians. The adoption of this platform enables standardization and improvement of clinical practice in uveitis. It is useful to apprehend the repercussion of medical and surgical treatments in uveitis and scleritis, supporting clinicians in the strict monitoring of drug adverse reactions and surgical outcomes.

Bull's Eye Maculopathy in Near-Infrared Reflectance as An Early Sign of Hydroxychloroquine Toxicity.

Hydroxychloroquine (HCQ) ocular toxicity is rare but severe, and progression can occur even after termination of therapy. Case reports have suggested that a bull's eye maculopathy detected by near-infrared reflectance (NIR) may indicate early HCQ toxicity. This retrospective cross-sectional study evaluated patients treated with HCQ who underwent routine screening with optical coherence tomography (OCT), fundus autofluorescence (FAF) and 10-2 perimetry. NIR images captured alongside OCT were subsequently graded independently by 2 masked graders for the presence of bull's eye maculopathy, and the result was compared to the outcome of the screening. A total of 123 participants (246 eyes) were included, and 101 (90%) were female. The patients' mean age was 55.2 ± 13.8 years. The mean time of HCQ usage was 84.0 ± 72.3 months, and the mean weekly dose was 2327 ± 650 mg. Two eyes showed toxicity in all 3 routine screening exams, with one patient suspending HCQ. The prevalence of bull´s eye lesions in NIR was 13% (33 eyes) with substantial intergrader agreement, a 71.3% specificity and 88.0% negative predictive value for HCQ toxicity. We suggest that NIR changes may be a sign of early HCQ toxicity. The detection of NIR bull´s eye lesions may warrant an increased screening frequency.

Long-Term Effect of Anti-Vascular Endothelial Growth Factor (Anti-VEGF) Injections in Choroidal Neovascularization Secondary to Angioid Streaks.

Purpose: This study aimed to evaluate the long-term effectiveness of intravitreal anti-vascular endothelial growth factor (VEGF) injections in the treatment of choroidal neovascularization (CNV) associated with angioid streaks. Methods: Multicenter retrospective cohort study, including eyes with CNV secondary to angioid streaks treated with anti-VEGF injections, were performed. Best-corrected visual acuity (BCVA) in ETDRS letters; qualitative and quantitative (foveal thickness) OCT parameters; anti-VEGF type; and number of injections were collected at baseline and at 3, 6, 12, 24, 36, 48, 60, and 72 months. Results: Thirty-nine eyes from 29 patients, 17 (58.6%) females, were included. The mean follow-up time was 69.4 ± 34.5 months. BCVA was 59.3 ± 23.3 letters at baseline and 63.7 ± 21.9 letters at 48 months. At 3 months, BCVA improved 6.9 ± 11.7 letters (P=0.003). Then, BCVA remained stable. The mean foveal thickness decreased from 343.3 ± 120.2 µm at baseline to 268.3 ± 65.4 at 48 months (P=0.021). The mean number of injections was 4.6 ± 2.1 at 12 months, decreasing to 1.7 ± 2.4 injections between 36 and 48 months (P=0.093). Conclusion: This real-world study suggests that the functional and morphologic response to anti-VEGF therapy for CNV related to angioid streaks is generally satisfactory and maintained in the long term.

A Non-Infectious Uveitis Multidisciplinary Clinic in a Tertiary Referral Center: Clinical Impact and Added Value.

Non-infectious uveitis (NIU) is a group of sight-threatening diseases that generates significant burden for the healthcare systems due to its adverse outcomes, irreversible structural complications in the eye with loss of visual function, limited clinical expertise and low-grade evidence for best practice. The usefulness of multidisciplinary care, specifically close collaboration between Rheumatologists and Ophthalmologists in NIU, has been emphasized in the literature. In this paper, the assessment tools and protocols used in our clinic are depicted and an overview of our activity with a brief description of the patients included in our registry, between 2018 and 2020 is provided. The cohort of 290 patients assessed in our NIU clinic, their demographics, sources of referral, details about immunosuppression treatment, and internal and external collaborations is described. This experience-based manuscript aims to describe the general functioning of our multidisciplinary NIU clinic, highlighting the benefits and drawbacks of multidisciplinary team management in patients with NIU, ultimately initiating a dialogue on what an NIU clinic should be and providing information for newly NIU clinics start-up. In conclusion, establishing a standardized and multidisciplinary clinic in NIU allows to systematically observe and follow-up this infrequent disease at a tertiary hospital level, thus improving quality of care delivery and research avenues.

A standardized protocol of laser suture lysis in postoperative management in trabeculectomy with mitomycin C: One-year study.

AIM: To analyse the efficacy and safety of a standardized laser suture lysis protocol following trabeculectomy. MATERIALS AND METHODS: Prospective interventional study conducted at a tertiary centre, between June 2016 and July 2017. Consecutive patients undergoing primary trabeculectomy with mitomycin C (0.4 mg/mL) for open angle glaucoma were enrolled. According to study protocol, a first laser suture lysis was performed routinely when intraocular pressure was greater than 10 mmHg, starting at postoperative day 8, and a second laser suture lysis was done whenever the intraocular pressure was newly greater than 10 mmHg, with a minimum interval of 1 week after the first laser suture lysis. Primary outcome was intraocular pressure (mean value, and mean difference from baseline in percentage). Safety parameters were also analysed. RESULTS: Thirty-three eyes of 28 patients (13 males) were enrolled with a mean age of 69.6 ± 15.1 years. Pre-operative intraocular pressure was 24.9 ± 8.6 mmHg. All eyes underwent a laser suture lysis before the 12th postoperative week, with a mean intraocular pressure-lowering efficacy of 42.4% (p < 0.01). A second laser suture lysis procedure, when performed (n = 14), had an additional intraocular pressure-lowering efficacy of 26.7% (p = 0.02). Mean intraocular pressure at 1-month follow-up was 12.9 ± 8.2 mmHg, and during this period, two cases of self-limited choroidal detachments were noticed. Ten patients needed additional bleb needling. Mean intraocular pressure at 1-year follow-up was 13.8 ± 8.0 mmHg under an average of 0.4 topical intraocular pressure-lowering drugs (p < 0.01). CONCLUSION: In our study, laser suture lysis was an effective tool to lower intraocular pressure during the early postoperative period, with an acceptable safety profile.

Optic disc drusen in children: morphologic features using EDI-OCT.

AIMS: This study aimed to investigate morphologic features of optic disc drusen (ODD) and peripapillary hyperreflective ovoid mass-like structures (PHOMS) in children, using enhanced depth imaging optical coherence tomography (EDI-OCT). It also assessed if the presence of these features were associated with decreased peripapillary retinal nerve fibre layer (RNFL) thickness. METHODS: Retrospective observational study of children with ODD. All subjects underwent complete ophthalmic examination and multimodal imaging. ODD were identified on EDI-OCT as circumscribed hyporeflective spheroidal elements located in front of lamina cribrosa, fully or partially surrounded by a hyperreflective border. PHOMS were identified as hyperreflective ovoid structures located in the peripapillary circumference. Both associations between ODD and RNFL loss and PHOMS and RNFL loss were tested using chi-squared test. RESULTS: In total, 38 eyes of 20 children were analysed. PHOMS were present in 90% of patients. ODD and PHOMS were predominantly found in the nasal, superonasal and inferonasal sectors. A significant positive association was found between ODD and decreased RNFL thickness in the nasal (p = 0.02), superonasal (p = 0.05) and inferotemporal (p = 0.04) sectors. There was no significant association found with the presence of PHOMS. CONCLUSION: EDI-OCT allowed morphological analysis of ODD and PHOMS in children. Drusen were found to be distinct from PHOMS both in their appearance and impact on the RNFL. ODD are hyporeflective and appear on the ONH above the lamina cribrosa and were associated with decreased thickness of the RNFL. On the contrary, PHOMS are hyperreflective structures located around the ONH and were not associated with RNFL loss.

Internal retinal layer thickness and macular migration after internal limiting membrane peeling in macular hole surgery.

PURPOSE: To provide a spectral-domain optical coherence tomography (SD-OCT)-based analysis of retinal layers thickness and nasal displacement of closed macular hole after internal limiting membrane peeling in macular hole surgery. METHODS: In this nonrandomized prospective interventional study, 36 eyes of 32 patients were subjected to pars plana vitrectomy and 3.5 mm diameter internal limiting membrane (ILM) peeling for idiopathic macular hole (IMH). Nasal and temporal internal retinal layer thickness were assessed with SD-OCT. Each scan included optic disc border so that distance between optic disc border and fovea were measured. RESULTS: Thirty-six eyes had a successful surgery with macular hole closure. Total nasal retinal thickening (p<0.001) and total temporal retinal thinning (p<0.0001) were observed. Outer retinal layers increased thickness after surgery (nasal p<0.05 and temporal p<0.01). Middle part of inner retinal layers (mIRL) had nasal thickening (p<0.001) and temporal thinning (p<0.05). The mIRL was obtained by deducting ganglion cell layer (GCL) and retinal nerve fiber layer (RNFL) thickness from overall thickness of the inner retinal layer. Papillofoveal distance was shorter after ILM peeling in macular hole surgery (3,651 ± 323 µm preoperatively and 3,361 ± 279 µm at 6 months; p<0.0001). CONCLUSIONS: Internal limiting membrane peel is associated with important alteration in inner retinal layer architecture, with thickening of mIRL and shortening of papillofoveal distance. These factors may contribute to recovery of disrupted foveal photoreceptor and vision improvement after IMH closure.

Tomographic Structural Changes of Retinal Layers after Internal Limiting Membrane Peeling for Macular Hole Surgery.

PURPOSE: To highlight tomographic structural changes of retinal layers after internal limiting membrane (ILM) peeling in macular hole surgery. METHODS: Nonrandomized prospective, interventional study in 38 eyes (34 patients) subjected to pars plana vitrectomy and ILM peeling for idiopathic macular hole. Retinal layers were assessed in nasal and temporal regions before and 6 months after surgery using spectral domain optical coherence tomography. RESULTS: Total retinal thickness increased in the nasal region and decreased in the temporal region. The retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), and inner plexiform layer (IPL) showed thinning on both nasal and temporal sides of the fovea. The thickness of the outer plexiform layer (OPL) increased. The outer nuclear layer (ONL) and outer retinal layers (ORL) increased in thickness after surgery in both nasal and temporal regions. CONCLUSION: ILM peeling is associated with important alterations in the inner retinal layer architecture, with thinning of the RNFL-GCL-IPL complex and thickening of OPL, ONL, and ORL. These structural alterations can help explain functional outcome and could give indications regarding the extent of ILM peeling, even though peeling seems important for higher rate of hole closure.