Purpose: To determine the 6-month effect of conventional (CXL30) and accelerated cross-linking with a UVA intensity of 9 mW/cm² (CXL10) on corneal stability and to investigate whether there was a difference in ABCD grading system parameters regarding the two different procedures. Methods: Twenty-eight eyes of 28 patients with a documented keratoconus (KN) progression were included. Patients were selected to undergo either epi off CXL30 or CXL10. At the baseline and the follow-up visits after one (V1), three (V2), and six months (V3), the patients underwent complete ophthalmic examination and corneal tomography. Results: In the CXL30 group, all the parameters from the ABCD grading system significantly changed from baseline to V3; parameter A decreased (p = 0.048), B and C increased (p = 0.010, p < 0.001), and D decreased (p < 0.001). In the CXL10 group, there were no changes in parameters A (p = 0.247) and B (p = 0.933), though parameter C increased (p = 0.001) and D decreased (p < 0.001). After an initial decline after one month, visual acuity (VA) recovered on V2 and V3 (p < 0.001), and median maximal keratometry (Kmax) decreased in both groups (p = 0.001, p = 0.035). In the CXL30 group, there were significant changes in other parameters; average pachymetric progression index (p < 0.001), Ambrósio relational thickness maximum (ARTmax) (p = 0.008), front and back mean keratometry (p < 0.001), pachymetry apex (PA) (p < 0.001), and front elevation (p = 0.042). However, in the CXL10 group, there were significant changes only in ARTmax (p = 0.019) and PA (p < 0.001). Conclusion: Both epi-off CXL protocols showed similar short-term efficacy in improving VA and Kmax, halting the progression of KN, and both similarly changed tomographic parameters. However, the conventional protocol modified the cornea more significantly. Full article

Background and Objectives: The aim of the study is to assess macular ganglion cell and inner plexiform layer (mGCIPL) thickness in deprivational amblyopic eyes (AE), fellow non-amblyopic eyes (FE) and normal eyes (NE) using spectral. domain optical coherence tomography (SD-OCT). Materials and Methods: Twenty two children (64% boys) who underwent surgical removal of unilateral congenital or developmental cataracts and exhibited visual impairment despite postoperative visual rehabilitation were included in the study. Cataract surgery was performed in patients aged 55.82 ± 35.85 months (range 6 to 114 months). The mean age of the study group was 9.73 ± 2.85 years (range 5 to 15 years). The comparison group consisted of 22 healthy age- and gender-matched children. The best corrected visual acuity (BCVA) after surgery was: 0.75 ± 0.27 (range 0.3 to 1.3) in AE, 0.1 ± 0.13 (range 0 to 0.5) in FE and 0.04 ± 0.07 (range 0 to 0.2) in NE. OCT scans were performed in all patients and subsequently corrected for axial length related magnification errors. Results: The average thickness of mGCIPL was 70.6 ± 11.28 μm in AE; 77.50 ± 6.72 μm in FE and 81.73 ± 5.18 μm in NE. We found that mGCIPL was statistically significantly thinner in deprivation AE compared to FE (p = 0.038) and NE (p = 0.0005). The minimum thickness of mGCIPL was respectively: 62.68 ± 13.2 μm, 70.3 ± 7.61 μm, and 74.5 ± 5.47, and also differed between AE and FE (p = 0.023) and AE and NE (p = 0.0004). Also, measurements in the inferior, inferotemporal, and superotemporal sectors showed thinning of mGCIPL in AE compared to NE. Conclusions: This analysis may suggest that deprivational amblyopia caused by unilateral congenital or developmental cataract in children may be associated with mGCIPL thinning. Full article

Background and Objectives: We aimed to analyze and compare the outcomes of conventional ectropion surgery procedures with and without concurrent bicanalicular nasolacrimal duct intubation to identify if the combination of procedures could serve as a novel surgical approach to treat lower eyelid ectropion. Materials and Methods: A retrospective review of all patients who underwent surgical correction for lower eyelid ectropion at the Cantonal Hospital of Aarau between January 2019 and December 2020 was performed. Patient medical records were examined for etiology, surgical correction technique and intra- and postoperative complications. The postoperative punctal position, the pre- and postoperative epiphora and reoperation rate were also documented. Two study groups consisting of cases with isolated and combined procedures were compared, with respect to postoperative punctual and lower lid position. Results: A total of 53 lower eyelids (35 patients) were included in this study. Six months postoperatively, the correct punctum position (p = 0.1188) and improvement of epiphora (p = 0.7739) did not significantly differ between the two groups. More complications were seen in the nasolacrimal duct intubation group (p = 0.0041), which consisted of cheese wiring and one tube dislocation. Conclusion: In our study, bicanalicular nasolacrimal intubation during ectropion surgery does not seem to improve the outcome of ectropion surgery and is, therefore, not recommended on a routine basis. Full article

Background and objectives: To analyze demographic and clinical features of pattern strabismus patients and assess the relationship among these clinical variables and risk factors. Materials and Methods: Medical records of pattern strabismus patients who had undergone strabismus surgery at our center between 2014 and 2019 were retrospectively reviewed. Data collected included gender, age at onset, age at surgery, refraction, Cobb angle, pre- and post-operative deviations in the primary position, up- and downgaze, angle of ocular torsion, type/amount of pattern, grade of oblique muscle function and presence/grade of binocular function. To verify the clinical significance of the Cobb angle, 666 patients who had undergone surgery within one week after ocular trauma between 2015 and 2021 were enrolled as controls. Results: Of the 8738 patients with horizontal strabismus, 905 (507 males and 398 females) had pattern strabismus, accounting for 10.36%. Among these 905 patients, 313 showed an A-pattern and 592 showed a V-pattern. The predominant subtype was V-exotropia, followed by A-exotropia, V-esotropia and A-esotropia. Over half of these patients (54.6%) manifested an A- or V-pattern in childhood. The overall mean ± SD Cobb angle was 5.03 ± 4.06° and the prevalence of thoracic scoliosis was 12.4%, both of which were higher than that observed in normal controls (4.26 ± 3.36° and 7.8%). Within A-pattern patients, 80.2% had SOOA and 81.5% an intorsion, while in V-pattern patients, 81.5% had IOOA and 73.4% an extorsion. Patients with binocular function showed decreases in all of these percent values. Only 126 (13.9%) had binocular function, while 11.8% of A-pattern and 15.1% of V-pattern patients still maintained binocular function. Pre-operative horizontal deviation was negatively correlated with binocular function (r = −0.223, p < 0.0001), while the grade of oblique muscle overaction was positively correlated with the amount of pattern (r = 0.768, p < 0.0001) and ocular torsion (r = 0.794, p < 0.0001). There were no significant correlations between the Cobb angle and any of the other clinical variables. There were 724 patients (80.0%) who had received an oblique muscle procedure and 181 (20.0%) who received horizontal rectus muscle surgery. The most commonly used procedure consisted of horizontal rectus surgery plus inferior oblique myectomy (n = 293, 32.4%), followed by isolated horizontal rectus surgery (n = 122, 13.4%). Reductions of pattern were 14.67 ± 6.93 PD in response to horizontal rectus surgery and 18.26 ± 7.49 PD following oblique muscle surgery. Post-operative deviations were less in V- versus A-pattern strabismus. Post-operative binocular function was obtained in 276 of these patients (30.5%), which represented a 16.6% increase over that of pre-operative levels. The number of patients with binocular function in V-pattern strabismus was greater than that of A-pattern strabismus (p = 0.048). Conclusions: Of patients receiving horizontal strabismus surgery, 10.36% showed pattern strabismus. In these patients, 54.6% manifested an A- or V-pattern in childhood, and V-exotropia was the most frequent subtype. Pattern strabismus patients showed a high risk for developing scoliosis. Cyclovertical muscle surgery was performed in 724 of these patients (80.0%), and horizontal rectus surgery was effective in correcting relatively small levels of patterns. Binocular function represented an important factor as being involved with affecting the occurrence and development of pattern strabismus. Full article

Age-related macular degeneration (AMD) is an eye disease that leads to progressive vision loss. Its prevalence has been increasing due to population aging. Previously, it was commonly believed that the disease affects the central retina, that is, the macula. However, recent studies have shown that it also involves the peripheral retina. Novel imaging techniques revealed various degenerative lesions that extend beyond the central macula. While their prevalence remains unknown, they seem to be more frequent in patients with late AMD. These findings suggest that the term “age-related retinal dysfunction” might be more adequate to describe some cases of AMD. They also raise the question about the role of electroretinography (ERG) as an objective measure of retinal function. The most common types of ERG tests used in AMD are multifocal (mfERG) and full-field ERG (ffERG). mfERG is more sensitive to macular changes, but the test is difficult to perform when fixation is unstable. On the other hand, ffERG reflects the function of the entire retina, not only the macular area. It helps assess the impact of peripheral retinal lesions and overall retinal function in patients with AMD. As ffERG results are normal in early-stage AMD, any abnormalities indicate that the disease is more severe and affects the entire retina. Anti-vascular endothelial growth factor injections improve retinal function in patients with neovascular AMD, as demonstrated by an increase in their ERG responses. More research is needed to assess the association between local and general retinal dysfunction. In this review, ffERG findings in patients with AMD are described and the usefulness of ffERG is discussed based on previous studies and cases from our own clinical practice. Full article

Background: Cone–rod dystrophies (CRDs) are a heterogeneous group of inherited retinal diseases (IRDs) characterized by cone photoreceptor loss, that is followed by subsequent rod photoreceptor impairment. Case presentation: A 49-year-old man complaining of diminution of vision in both eyes (OU) was referred to our outpatient clinic. He reported visual loss for 5 years, but it was most progressive during the last few months. The best-corrected visual acuity (BCVA) at presentation was 0.4 in the right eye (RE) and 1.0 in the left eye (LE). Fundus fluorescein angiography (FFA) revealed granular hyperfluorescence in the macula and concomitant areas of capillary atrophy. Flash full-field electroretinography (ffERG) showed lowering of a and b waves as well as prolonged peak time in light-adapted conditions. However, outcomes of dark-adapted ERGs were within normal limits. Based on the constellation of clinical, angiographic, and electrophysiological tests findings, a diagnosis of IRD was suspected. Genetic testing showed a homozygous, pathogenic c.783G>A mutation in the cadherin-related family member 1 (CDHR1) gene, which confirmed CRD type 15 (CRD15). Conclusions: We demonstrate the clinical characteristics, retinal imaging outcomes, and genetic test results of a patient with CRD15. Our case contributes to expanding our knowledge of the clinical involvement of the pathogenic mutation c.783G>A in CDHR1 variants. Full article

Background: Mowat-Wilson syndrome (MWS) is extremely rare multisystemic autosomal dominant disorder caused by mutations in the Zinc Finger E-Box Binding Homeobox 2 (ZEB2) gene. Ocular pathologies are one of the symptoms that appear in the clinical picture of MWS individuals, but not many have been described so far. Pathologies such as optic nerve or retinal epithelium atrophy, iris or optic disc coloboma as well as congenital cataracts have been most frequently described until now. Therefore, we would like to report the first case of bilateral developmental cataract in a 9-year-old girl with MWS who underwent successful cataract surgery with intraocular lens implantation. Case Presentation: A 9-year-old girl, diagnosed with p.Gln694Ter mutation in ZEB2 gene and suspicion of MWS was referred to the Children’s Outpatient Ophthalmology Clinic for ophthalmological evaluation. Her previous assessments revealed abnormalities of the optic nerve discs. The patient was diagnosed with atrophy of the optic nerves, convergent strabismus, and with-the-rule astigmatism. One year later, during the follow-up visit, the patient was presented with decreased visual acuity (VA), developmental total cataract in the right eye and a developmental partial cataract in the left eye. This resulted in decreased VA confirmed by deteriorated responses in visual evoked potential (VEP) test. The girl underwent a two-stage procedure of cataract removal, first of one eye and then of the other eye with artificial lens implants. In the 2 years following the operation, no complications were observed and, most remarkably, VA improved significantly. Conclusions: The ZEB2 gene is primarily responsible for encoding the Smad interaction protein 1 (SIP1), which is involved in the proper development of various eye components. When mutated, it results in multilevel abnormalities, also in the proper lens formation, that prevent the child from normal vision development. This typically results in the formation of congenital cataracts in children with MWS syndrome, however, our case shows that it also leads to the formation of developmental cataracts. This is presumably due to the effect of the lack of SIP1 on other genes, altering their downstream expression and is a novel insight into the importance of the SIP1 in the occurrence of ocular pathologies. To the best of our knowledge, this is the first case of bilateral developmental cataract in the context of MWS. Moreover, a novel mutation (p.Gln694Ter) in the ZEB2 gene was found corresponding to this syndrome entity. This report allows us to gain a more comprehensive insight into the genetic spectrum and the corresponding phenotypic features in MWS syndrome patients. Full article

Background: Despite its proven effectiveness and safety profile, the XEN gel stent (Allergan Inc., CA, USA) for minimally invasive glaucoma surgery (MIGS) has a probability of postoperative complications, including postoperative hypotony, hyphema, stent migration, stent obstruction, bleb fibrosis, and fibrin formation. In particular, the use of adjunctive Mitomycin-C (MMC) might be associated with bleb-related complications, including conjunctival erosion, XEN gel stent exposure, and blebitis. However, there are few studies on XEN gel stent exposure and its management. We describe a case of XEN gel stent exposure with conjunctival erosion 18 months postoperatively, which resolved effectively after combination treatment with a rotational conjunctival flap and amniotic membrane transplantation. Case presentation: A 74-year-old Korean male patient with diabetes and hypertension underwent uncomplicated ab interno XEN gel stent implantation with a subconjunctival injection of 0.1 cc of 0.02% MMC and presented with low intraocular pressure (IOP) with a well-functioning filtering bleb. Periocular pain and tearing developed 18 months after the initial operation, with mild deterioration of visual acuity to 20/100. Despite conservative medical treatment, the conjunctival erosion was not relieved. Anterior segment optical coherence tomography (AS-OCT) revealed an exposed XEN gel stent with conjunctival erosion. We performed bleb revision surgery using a rotational conjunctival flap and amniotic membrane transplantation. Slit-lamp examination and AS-OCT showed a well-formed moderate bleb without leakage, and IOP continued to be well controlled (14 mm Hg with latanoprost) until six months after bleb revision. Conclusions: This case report highlights the importance of careful examination, including slit-lamp examination, the Seidel test, and AS-OCT, to identify accurate anatomical positioning and to monitor ocular surface changes after XEN gel stent implantation with MMC or 5-FU. Combination treatment (rotational conjunctival flap and amniotic membrane transplantation) may be relatively safe for persistent XEN gel stent exposure. Full article

Central serous chorioretinopathy (CSC) is a common chorioretinal disorder. It has been postulated that impaired retinal pigment epithelium and hyperpermeability of the choriocapillaris may be involved in the development of CSC, but the exact pathomechanism has not been established. We report an unusual case of a middle-aged man who developed CSC after triamcinolone acetonide injection for macular edema. Edema developed as a late complication of radiation retinopathy after brachytherapy for childhood retinoblastoma. Steroid treatment is an important risk factor for CSC, but the underlying causative mechanisms have not been fully elucidated. It is important to increase the awareness of this link among clinicians who prescribe exogenous corticosteroids, irrespective of the route of administration. Full article