Axenfeld-Rieger syndrome combined with a foveal anomaly in a three-generation family: a case report.

BACKGROUND: Axenfeld-Rieger syndrome (ARS) is a rare autosomal dominant eye disorder that can also affect other organs of the human body. The condition is primarily characterized by the anterior segmental abnormalities of the eye. Here, we present an observational case series of a three-generation family with ARS and unexpected foveal anomaly. CASE PRESENTATION: A 33-year-old woman was admitted to an Ophthalmology Clinic in Bialystok for left eye congenital cataract surgery. The patient (proband) was diagnosed with visual deterioration, multiple defects of iris, corectopia, displacement of the Schwalbe's line, and phenotypic characteristics of ARS. A perimetric examination indicated peripheral visual field loss and signs typical for glaucoma. Based on the phenotypic symptoms and genetic test, the patient was diagnosed with Axenfeld Rieger Syndrome. However, the optical coherence tomography of the macula showed foveal anomaly (absence of the physiological pit), which is not typically associated with this genetic disorder. The patient's family history revealed that her two daughters were undergoing treatment for congenital glaucoma, and one of the daughters also had foveal anomaly the same as her mother. Interestingly, an examination of the patient's mother showed typical phenotypic features of ARS such as a defect of the iris, posterior embryotoxon, and coloboma, as well as foveal anomaly. A genetic test confirmed PITX2 mutation in both, proband's two daughters and mother. CONCLUSIONS: This study highlights the occurrence of ARS with unusual ophthalmic features such as foveal anomaly (absence of the physiological pit) in a three-generation family. Although ARS is known to represent the developmental defects of the anterior segment of the eye, it is very important to perform fundus evaluation to identify associated posterior segment anomalies that may affect visual acuity. The presence of ocular defects not typically associated with ARS suggests a wide spectrum of mutations within PITX2 gene which are required to identify in order to determine genotype- phenotype correlation in ARS affected individuals.

An Approach to Automatic Hard Exudate Detection in Retina Color Images by a Telemedicine System Based on the d-Eye Sensor and Image Processing Algorithms.

Hard exudates are one of the most characteristic and dangerous signs of diabetic retinopathy. They can be marked during the routine ophthalmological examination and seen in color fundus photographs (i.e., using a fundus camera). The purpose of this paper is to introduce an algorithm that can extract pathological changes (i.e., hard exudates) in diabetic retinopathy. This was a retrospective, nonrandomized study. A total of 100 photos were included in the analysis-50 sick and 50 normal eyes. Small lesions in diabetic retinopathy could be automatically diagnosed by the system with an accuracy of 98%. During the experiments, the authors used classical image processing methods such as binarization or median filtration, and data was read from the d-Eye sensor. Sixty-seven patients (39 females and 28 males with ages ranging between 50 and 64) were examined. The results have shown that the proposed solution accuracy level equals 98%. Moreover, the algorithm returns correct classification decisions for high quality images and low quality samples. Furthermore, we consider taking retina photos using mobile phones rather than fundus cameras, which is more practical. The paper presents an innovative approach. The results are introduced and the algorithm is described.

Minimally invasive bleb surgery versus minimally invasive glaucoma surgery: a 12-month retrospective study.

This study aimed to compare the IOP-lowering effectiveness and safety of standalone Preserflo MicroShunt and iStent 1st generation implantation combined with phacoemulsification in Caucasian patients with a 12-month follow-up period. This retrospective study analyzed the medical histories of patients undergoing antiglaucoma surgery at the Department of Ophthalmology, Medical University of Bialystok, between January 2019 and January 2022. The main outcome measures were success rates (complete: proportion of eyes with IOP < 18 mmHg (criterion A) and < 15 mmHg (criterion B) or 20% reduction in IOP without any glaucoma medication; qualified: proportion of eyes achieving IOPs < 18 mmHg and < 15 mmHg or 20% reduction in IOP from baseline with or without medications), mean reduction (%) in IOP, medication burden, number of complications and additional interventions. In both groups, a significant decrease in IOP and medication burden were observed at 6 and 12 months when compared with baseline. At 12 months, qualified surgical success in criterion A was recorded in 67.4% and 85.7% of patients in the Preserfo and iStent groups, respectively (p = 0.045). Complete surgical success in criterion B at 12 months accounted to 61.4% of patients from Prserflo group and 32.7% patients in iStent group (p = 0.04). Surgical failure at 12 months was documented in 30.2% and 6.3% of patients, respectively (p = 0.003). There was a significant difference between groups in %IOP reduction after 12 months. Greater reduction was observed in Preserflo group, MD = - 8.41 CI95 [- 15.88; - 0.95], p = 0.028, (- 33.49% ± 21.59 vs - 25.07% ± 14.15 in iStent group). Both procedures effectively reduced IOP and postoperative use of antiglaucoma medications in glaucoma patients.

The PreserFlo MicroShunt in the Context of Minimally Invasive Glaucoma Surgery: A Narrative Review.

Recently, the quest for novel glaucoma surgical techniques and devices has been underway. Trabeculectomy remains the gold standard, but it requires the implantation of glaucoma drainage devices and frequent follow-ups, and it also carries a high risk of serious complications. The need for less invasive and safer procedures has led to the development of minimally invasive glaucoma surgery (MIGS), particularly for patients with mild-to-moderate disease. Among them, minimally invasive bleb surgery seems to be effective in classical glaucoma surgery, while maintaining MIGS benefits. The relatively new PreserFlo® MicroShunt (Santen, Osaka, Japan) is registered in Europe. It was released in 2019 for the treatment of patients with early-to-advanced open-angle glaucoma, where intraocular pressure (IOP) remains uncontrolled while on maximum tolerated medication and/or where glaucoma progression warrants surgery. This review focuses on the place of the PreserFlo MicroShunt, characterized by ab externo implantation, among MIGS procedures, discussing its advantages and disadvantages. The mechanisms of action, technical aspects, efficacy, and safety issues are summarized. The surgical technique, its efficacy, and safety profile are described, and directions for future studies are indicated. The PreserFlo MicroShunt ensures a high safety profile, minimal anatomical disruption, meaningful IOP-lowering effect, and ease of use for patients and physicians.

Changes to glaucoma surgery patterns during the coronavirus disease 2019 pandemic: a shift towards less invasive procedures.

PURPOSE: The aim of the study was to compare the quantity, type of glaucoma surgeries, and the disease stage before and during the coronavirus disease 2019 (COVID-19) pandemic. METHODS: This was a retrospective, single-centre consecutive case series that included medical records of patients who underwent glaucoma surgery at the University Hospital in Bialystok between 4 September, 2018, and 3 March, 2020 (pre-pandemic group) and compared it with patients treated between 4 March, 2020, and 4 September, 2021 (pandemic group). Adult patients with primary or secondary open-angle or closed-angle glaucoma who underwent surgery were included in this study. Finally, 534 operated eyes (362 and 172 eyes operated on before and during the pandemic, respectively) were examined. RESULTS: The number of glaucoma surgeries dropped by 50% during the pandemic compared to a similar pre-pandemic period, with a significant difference in the kind of procedure between the two groups (p < 0.001). The most common procedures in the pre-pandemic group were Ex-Press implantation (33.7%) and trabeculectomy (31.5%). Within the pandemic group, half of the eyes underwent trabeculectomy (50.0%), followed by Preserflo microshunt (11.6%), iStent (8.7%), and transscleral cyclophotocoagulation (TSCP) (8.7%). A significant difference in the average intraocular pressure was revealed among patients who qualified for surgery. CONCLUSION: The COVID-19 pandemic is associated with a decrease in the number of extended antiglaucoma procedures and an increase in the number of short procedures performed, such as TSCP and minimally invasive glaucoma surgery.Key MessagesOur study has shown the negative impact of the COVID-19 pandemic in reducing the number of antiglaucoma procedures.The number of glaucoma surgeries dropped by 50% during the pandemic compared to those in a similar pre-pandemic period, and the type of performed procedures has changed.The COVID-19 pandemic is associated with a decrease in the number of combined antiglaucoma procedures, in opposite: the number of minimally invasive glaucoma surgeries increased due to safety reasons.

Early Complications and Results of Preserflo MicroShunt in the Management of Uncontrolled Open-Angle Glaucoma: A Case Series.

We analyze the surgical outcomes and early complications with their management of the Preserflo MicroShunt (Santen Pharmaceutical Co., Ltd., Osaka, Japan) at six-month follow-up. The study is conducted between March 2021 and May 2022. Best-corrected visual acuity (BCVA) logMAR, intraocular pressure (IOP), and changes in glaucoma medications are assessed. Thirty eyes of 30 patients (22 women [73.3%] and 8 men [26.7%]) are included. They are augmented with mitomycin C (MMC) 0.5 mg/mL (8 subjects) or MMC 0.2 mg/mL (22 subjects) intraoperatively. BCVA is significantly higher one day after the treatment than before the treatment (MD with 95% CI = 0.05 (<0.01; 0.30); p = 0.045) when analyzing all patients. Such dependency is not observed when analyzing only patients treated with MMC 0.2 or 0.5 mg/mL (p > 0.050 for both analyses). No other statistically significant differences are detected in the level of BCVA before and after treatment. Among the patients, overall IOP is significantly lower at each time point after treatment than before surgery (p < 0.001 for all analyses). Among patients augmented with MMC 0.2 mg/mL, the IOP level is also significantly lower at each time point after treatment than before treatment (p ≤ 0.001 for all analyses). The same differences are observed among patients with MMC = 0.5­the IOP level is significantly lower at each time point after treatment than before treatment (p < 0.050 for all analyses). Five subjects (16.7%) require anti-glaucoma medications three months after the procedure. Early complications (hypotony, choroidal effusion, keratitis, hyphema, and bleb fibrosis) are observed in 46.7% of cases. Our early results show that Preserflo MicroShunt is safe and effective for lowering IOP; however, it is not free from transient complications.

Recurrent monocular exudative retinal detachment as the first manifestation of squamous cell lung cancer: A case report.

RATIONALE: In this report, we present an extremely rare case of recurrent monocular exudative retinal detachment without concomitant ocular metastases. This turned out to be the first symptom of squamous cell lung cancer. PATIENT CONCERNS: A 63-year-old woman was referred to our ophthalmology clinic by her primary care physician with a complaint of deteriorating vision in her right eye that had started four months prior, without concomitant pain. DIAGNOSES: We observed a detachment in the lower part of the retina during her ophthalmoscopy. We did not find any tears, holes, or degenerative changes in the periphery of the retina of the right eye during the surgery. In addition, plaques, tumor masses, and metastases were absent. Therefore, we diagnosed her with unilateral paraneoplastic exudative retinal detachment. Imaging tests performed before surgery revealed perihilar density with a visible air bronchogram in the middle field of the left lung. This turned out to be squamous cell carcinoma. INTERVENTIONS: Patient underwent pars plana vitrectomy and routine laboratory and imaging tests before the procedure that utilized 20-gauge instrumentation. The subretinal fluid and was drained and a tamponade using Densiron (Fluoron Co, Neu-Ulm, Germany) was applied. After ophthalmic treatment, patient underwent complex oncological treatment based on chemotherapy and radiotherapy. OUTCOMES: Despite the application of heavy silicone oil (Densiron) into the vitreous chamber, we observed a recurrence of retinal detachment in the right eye during the follow-up visit, 13 months after the first ophthalmic surgery. Following subsequent pars plana vitrectomy, the Densiron and subretinal membranes were removed. Despite oncological treatment, the patient died, twenty months after the appearance of the first ocular symptoms. LESSONS: Exudative retinal detachment without tumor metastasis to the eyeball can be one of the first signs of lung cancer in rare cases. Multidisciplinary care and imaging methods with greater accuracy will provide comprehensive care to the patients. It will not only facilitate timely detection and treatment of lung tumors but also for a plethora of oncological diseases.

Phacotrabeculectomy versus Phaco with Implantation of the Ex-PRESS Device: Surgical and Refractive Outcomes-A Randomized Controlled Trial.

The aim of this study was to compare surgical and refractive outcomes between phacotrabeculectomy (P-Trab) and phaco with Ex-PRESS (P-Ex-PRESS) for glaucoma at a 6-month follow-up. This prospective randomized controlled trial included 81 eyes; 43 eyes (53%) and 38 eyes (47%) were assigned to the P-Ex-PRESS and P-Trab groups, respectively. Refraction, intraocular pressure (IOP), and best-corrected visual acuity were measured. Refractive change was analyzed using the cylinder's magnitude, and polar analysis assessed the change in the trend of astigmatism [with-the-rule, against-the-rule (ATR), oblique (OBL)], evaluating mean astigmatism in centroid form. All patients showed a statistically significant postoperative decrease in IOP (P < 0.05). There were no differences between the groups in terms of postoperative IOP and visual outcomes or in astigmatism preoperatively or postoperatively (P = 0.61, P = 0.74). In both groups, the mean preoperative and postoperative astigmatism were ATR and OBL, respectively. Preoperative and postoperative centroids in the P-Ex-PRESS group were 0.44 ± 1.32 D at 177° and 0.35 ± 1 D at 8°, respectively, (P = 0.5) and in the P-Trab group were 0.16 ± 1.5 D at 141° and 0.39 ± 1.38 D at 29°, respectively (P = 0.38). Both P-Ex-PRESS and P-Trab showed comparable antihypertensive efficacy in treating open-angle glaucoma over 6 months. Preoperative and postoperative astigmatism did not differ between groups. The groups showed comparable results for final visual acuity.

Evaluation of the Effect of the First Generation iStent on Corneal Endothelial Cell Loss-A Match Case-Control Study.

Glaucoma is the leading cause of irreversible blindness worldwide. The only proven factor in slowing the progression of glaucomatous neuropathy is lower intraocular pressure (IOP), which can be achieved with pharmacology, laser therapy, or surgery. However, these treatments are associated with various adverse effects, including corneal endothelial cell loss (CECL). In recent years, several novel surgeries for reducing the IOP, collectively referred to as minimally invasive glaucoma surgery (MIGS), have been developed, one of which is the iStent. However, the long-term effects of such surgeries remain unknown. We compared a group of patients with open-angle glaucoma and cataract who underwent phacoemulsification alone with a group of patients with similar demographic and clinical characteristics who underwent simultaneous phacoemulsification and iStent implantation. Overall, 26 eyes of 22 subjects who underwent a combined phacoemulsification-iStent procedure and 26 eyes of 24 subjects who underwent cataract surgery were included. Before surgery, endothelial cells accounted to 2228.65 ± 474.99 in iStent group and 2253.96 ± 404.76 in the control group (p = 0.836). After surgery, their number declined to 1389.77 ± 433.26 and 1475.31 ± 556.45, respectively (p = 0.509). There was no statistically significant difference in CECL between the two groups 18-24 months after surgery, despite increased manipulation in the anterior chamber and the presence of an implant in the trabecular meshwork in those with an iStent implant. Thus, iStent bypass implantation is a safe treatment in terms of CECL for mild-to-moderate open-angle glaucoma.

Foveal Avascular Zone Does Not Correspond to Choroidal Characteristics in Patients with Diabetic Retinopathy: A Single-Center Cross-Sectional Analysis.

PURPOSE: The aim of the study was to compare two non-overlapping blood supply systems of the retina to obtain a better insight into the relation between diabetic macular retinopathy and choroidopathy. Specifically, the study focused on the relationships between (1) retinal vascular changes around the fovea in fluorescein angiography (FA) and (2) choroidal thickness, volume and other parameters assessed by optical coherence tomography (OCT). PATIENTS AND METHODS: The retrospective cross-sectional single-center study included 210 eyes from 152 patients with diabetic retinopathy (mean age 60.7±12.4 years, 49.3% of women; foveal avascular zone [FAZ] outline: 44.3% grade ≤2, 55.7% grade ≥3). The outline of FAZ, a measure of capillary loss due to ischemic processes, was analyzed on FA according to the Early Treatment Diabetic Retinopathy Study Research Group (ETDRS) standards. The eyes were stratified according to the FAZ outline and size and the presence of clinically significant diabetic macular edema (CSME). Then, resultant groups were compared in terms of the spectral domain OCT parameters: choroidal thickness and volume (within ETDRS subfields), luminal, stromal and total choroidal areas and choroidal vascularity index (based on the foveal scan). Statistical analysis was based on univariate models with the choroidal parameters as independent variables, and age, sex, panretinal photocoagulation, the severity of diabetic retinopathy and CSME as covariates. RESULTS: No significant relationships were found between the FAZ outline and area and choroidal characteristics of patients with diabetic retinopathy. In patients without CSME, no correlation was observed between the FAZ area and choroidal characteristics. In patients with CSME, no correlation was found between the FAZ area and choroidal characteristics other than the choroidal vascularity index. CONCLUSION: In patients with diabetic retinopathy, damage to the macular retinal vasculature (FAZ) does not seem to be associated with changes in the choroidal vasculature, and these two processes appear to occur independently.

The Impact of Three Waves of the COVID-19 Pandemic on the Characteristics of Primary Rhegmatogenous Retinal Detachments at a Tertiary Referral Centre.

PURPOSE: This study assessed the effect of three waves of the COVID-19 pandemic primarily on the number and timing of referrals due to rhegmatogenous retinal detachment (RRD) and secondarily on the demographic or clinical characteristics of patients. PATIENTS AND METHODS: A retrospective single-centre analysis of medical records included 247 eyes from 247 patients who underwent primary RRD repair with (phaco) vitrectomy between January 1, 2019, and May 31, 2021. RESULTS: The percentage of referrals due to primary RRD during the first year of pandemic (March 2020-February 2021) was 16.5% (p=0.179) lower than a year earlier. The percentage of referrals during the first, second and third wave of the pandemic was 48.4%, 18.5% and 26.1% (p=0.029, 0.475, 0.343) lower than in the corresponding months of 2019. A rebound effect was observed only after the first wave. The numbers of referrals in April and May 2021 were similar as in the corresponding months of 2019. No significant differences were observed in the demographic and clinical characteristics of patients admitted before and during the COVID-19 pandemic, other than a tendency towards a higher representation of women, younger persons, longer duration of symptoms but better visual acuity in the latter group. Silicone oil tamponade was used more frequently during the pandemic than before. CONCLUSION: In this study, the COVID-19 pandemic affected the number and timing of referrals due to RRD and the clinical but not demographic characteristics of the patients. The effect lessened with the duration of the pandemic. Previously raised concerns regarding the delay in RRD referrals may no longer be valid during a potential fourth wave of the pandemic. The impact of the pandemic's waves should be analysed separately, as such an approach provides a better insight into the fluctuations in the number of referrals due to RRD than a year-to-year comparison.

Iris-Claw Lens Implantation in a Patient with Iridoschisis.

BACKGROUND Iridoschisis is a rare condition defined as a separation of anterior iris stroma from the posterior stroma and muscle layers. The etiopathogenesis of iridoschisis is not fully understood. We report the case of uveitis-glaucoma-hyphema (UGH) syndrome related to the iris-claw lens implantation in a patient with iridoschisis, and propose an alternative approach to aphakia correction. CASE REPORT A 47-year-old male was referred to our department with bilateral iridoschisis, associated lens subluxation, mature cataract, and secondary glaucoma. The patient underwent bilateral surgery, with entirely different anterior segment results depending on the method of artificial lens implantation. To the best of our knowledge, iris-claw implantation in iridoschisis and the potential association of iridoschisis with increased risk of UGH syndrome, have not been reported previously. CONCLUSIONS Due to the possibly increased risk of UGH syndrome in patients with iridoschisis, one may consider treating aphakia by implantation of scleral fixated lenses, rather than iris-claw lenses.

Iridoschisis-A Systematic Review.

Iridoschisis is a rare condition defined as a separation of the anterior iris stroma from the posterior stroma and muscle layers. In this paper, we review current data about the epidemiology, pathophysiology, clinical characteristics and differential diagnoses of this condition and discuss the specificity of surgical treatment of concomitant ocular diseases in iridoschisis patients. Iridoschisis may pose a challenge for both an ophthalmologist in an outpatient setting and an ophthalmic surgeon. Glaucoma, primarily angle-closure glaucoma, is the most often described condition concomitant to iridoschisis. Other ocular abnormalities found relatively often in iridoschisis patients include cataract, lens subluxation and corneal abnormalities. Special attention has been paid to potential complications of cataract surgery and prevention thereof. Beside addressing the practical aspects, we point to discrepancies and suggest topics for further investigation.

Comparison of ExPress Mini-Device Implantation Alone or Combined with Phacoemulsification for the Treatment of Open-Angle Glaucoma.

We propose comparative assessment of the effectiveness of two surgical methods for the treatment of open-angle glaucoma: (1) ExPress mini-device implantation combined with phacoemulsification and (2) ExPress mini-device implantation alone. In this prospective study, 81 patients (88 phakic eyes) with uncontrolled open-angle glaucoma enrolled for surgery. They were assigned two groups, those with coexisting cataracts (46 eyes; P-ExPress group) and those with glaucoma alone (42 eyes; ExPress group). The follow-up period was 12.9 ± 0.4 months in P-ExPress and 12.2 ± 0.6 months in ExPress group. In both groups the following parameters were measured: best corrected visual acuity (BCVA), intraocular pressure (IOP), number of complications and necessary postoperative interventions, and number of glaucoma medications. The IOP at the end of follow-up was similar in both groups (18.8 ± 5.9 versus 18.1 ± 4.8 mmHg; P = 0.814). There were no statistical differences in the average number of glaucoma medications between ExPress and P-ExPress groups (0.9 ± 1.65 versus 1.3 ± 1.7; P = 0.419) as well as in the number of postoperative complications (26 versus 21%; P = 0.179 in the P-ExPress and ExPress groups, resp.). Both methods are safe and effective for the surgical treatment of open-angle glaucoma. Coexistence of cataracts does not constitute a compelling contraindication for combined surgery.

Prospective Randomized Study Comparing Combined Phaco-ExPress and Phacotrabeculectomy in Open Angle Glaucoma Treatment: 12-Month Follow-Up.

Purpose of the Study. To compare the efficacy and safety of phacotrabeculectomy (P-Trab) and phacoemulsification with the ExPress (P-ExPress) mini glaucoma shunt implantation. Study Plan. Prospective randomized study. Material and Methods. 85 eyes with cataract and unregulated open angle glaucoma. There were 46 eyes in the P-ExPress and 39 the P-Trab group. Intraocular pressure (IOP), the number of antiglaucoma medications, qualified and complete surgical success (defined as IOP ≤ 18.0 mmHg), visual acuity (CDVA), the number of endothelial cells, and postoperative complications and additional procedures were assessed. Results. After 12 months of observation, the average IOP in the P-Express group went from 26.4 ± 9.3 down to 17.1 ± 5 mmHg (P < 0.05) and from 27.9 ± 12.9 down to 15.9 ± 2.7 mmHg in the P-Trab group (P < 0.05). No significant differences in the amount of medications used after surgery and CDVA were discovered between the groups. In the P-ExPress group, greater loss of endothelial cells was noted (CDloss%), compared to the P-Trab group. Conclusions. Both P-ExPress and P-Trab have comparable efficacy and similar early postoperative complication profile. The presence of additional implant (as is the case of the ExPress mini glaucoma shunt implantation) may cause progressive loss of endothelial cells.