Neurotrophic Keratopathy in Marfan Syndrome Patient After Micropulse Transscleral Cyclophotocoagulation: A Call for Risk Stratification.

BACKGROUND Micropulse transscleral cyclophotocoagulation is a non-invasive, widely employed procedure that uses diode laser to target the ciliary body to lower the intraocular pressure. Despite its acknowledged efficacy, certain complications are recognized, with neurotrophic keratopathy being a rare yet serious consequence. This report seeks to shed light on a potential high-risk subgroup susceptible to neurotrophic keratopathy, exemplified by our patient with Marfan syndrome, a condition characterized by thin sclera. CASE REPORT Our patient, who was confirmed to have Marfan syndrome with pseudophakic glaucoma, underwent micropulse transscleral cyclophotocoagulation due to high intraocular pressure bilaterally and subsequently quickly manifested neurotrophic keratopathy in both eyes postoperatively. Swift initiation of management involved a comprehensive approach, including topical antibiotics, preservative-free lubrication, medroxyprogesterone acetate 1%, serum balanced salt solution (BSS) 50%, and the application of bandage contact lenses to expedite healing. Fortunately, the left eye demonstrated resolution within 10 days, while the right eye exhibited delayed healing, leading to subsequent scarring. CONCLUSIONS This report highlights the critical importance of recognizing populations predisposed to neurotrophic keratopathy before subjecting them to micropulse transscleral cyclophotocoagulation. Such awareness allows for the fine-tuning of procedural parameters, offering a strategic approach to mitigate the risk of neurotrophic keratopathy development. By further exploring and recognizing potential risk factors, clinicians can enhance patient outcomes and refine the safety profile of micropulse transscleral cyclophotocoagulation.

Late-Onset Chronic Corneal Fistula Following Phototherapeutic Keratectomy: A Case Report and Importance of Early Detection.

BACKGROUND We report a case of late-onset chronic fistula in a decompensated cornea after multiple ocular surgeries and a recent phototherapeutic keratectomy (PTK). CASE REPORT A 73-year-old woman presented to our service with a past ocular history of bilateral chronic angle closure glaucoma and pseudophakic bullous keratopathy in the left eye. Given a history of long-term uncontrolled glaucoma with advanced disc cupping and poor visual potential, the patient underwent multiple palliative procedures, including, most recently, a PTK. Few years later she presented with a spontaneous late onset of slowly appearing corneal leak on fluorescein staining upon routine clinical examination. Corrected distance visual acuity was hand motion and intraocular pressure (IOP) was 40 mmHg in the affected eye. Serial anterior segment optical coherence tomography (AS-OCT) sections were obtained, which aided in understanding the current presentation and revealed distinctive multilayer corneal changes during the healing process. The patient was successfully managed with cyanoacrylate corneal gluing and ocular hypotensive medications, which halted the corneal leak. CONCLUSIONS We report a case of a rare finding of corneal fistula in an eye with multiple previous ocular surgeries, and provide an explanation of the possible etiopathogenesis. We also highlight the pivotal role of AS-OCT for evaluating such cases and stress the importance of early detection of similar subtle leaks in the setting of a formed anterior chamber, which can often be missed, carrying a risk of infection.

Visual outcomes and recurrence rate of macular corneal dystrophy following phototherapeutic keratectomy in Saudi Arabia.

PURPOSE: The purpose of the study was to evaluate the visual outcomes and recurrence rate of patients with macular corneal dystrophy (MCD) who have undergone phototherapeutic keratectomy (PTK). METHODS: This retrospective, single-centered study enrolled patients from King Khaled Eye Specialist Hospital who had undergone PTK for MCD between 2000 and 2020. Pre-, intra-, and post-operative data were collected, and the primary outcome measures included uncorrected visual acuity, best-corrected visual acuity (BCVA), spherical equivalent, central keratometry, keratometric astigmatism, recurrence rate, and necessity of subsequent surgery. RESULTS: This study evaluated 42 eyes of 29 patients. BCVA improved from 0.41 (±0.17) preoperatively to 0.30 (±0.16) postoperatively. Sixteen out of 42 eyes (38%) had the recurrence of macular dystrophy deposits within the stroma; the average time from PTK to recurrence was 37 months (range: 5.5-115.4 months). The overall success rate of PTK at 2 years was 44.8%. Eleven eyes (26%) required subsequent surgery following PTK at an average of 43 months postoperatively. Deeper ablation and longer application of mitomycin C (MMC) were both found to be statistically significant factors associated with visual improvement and lower recurrence rate following PTK. CONCLUSION: PTK can be considered a treatment modality for younger patients to defer keratoplasty to a later stage. Deeper ablation is associated with improved postoperative visual acuity, and longer application of MMC is associated with lower recurrence rates of MCD.

Scleral melt and uveal prolapse following 23-gauge pars plana vitrectomy.

Purpose: To report a case of uncontrolled juvenile open angle glaucoma (JOAG), with secondary high axial myopia who presented with scleral melting and uveal prolapse post pars plana-vitrectomy. Observations: A 17-year-old male juvenile glaucoma patient, not known to have any systemic disease underwent a right eye 23-gauge-pars plana vitrectomy for retinal detachment repair. Three weeks following his surgery he presented complaining of tearing and photophobia in the operated eye in the absence of any ocular pain. Clinical exam revealed enlarged sclerotomy sites with localized conjunctival and episcleral injection, scleral thinning and uveal prolapse. Infectious and serologic work up were obtained to rule out an infectious etiology or underlying autoimmune disease. Patient was successfully managed with topical steroids and a donor scleral patch graft. Conclusion and importance: This report addresses multiple risk factors for a devastating complication that may occur in predisposed patients, with primary or secondary axial myopia and a compromised sclera. This group of patients can be at risk of post-operative scleral melting and thinning and should be identified pre-operatively and monitored closely during their post-operative course.

Risk Factors for Glaucoma Drainage Device Exposure in Children: A Case-Control Study.

PURPOSE: To identify the risk factors for glaucoma drainage device (GDD) exposure in children. DESIGN: Retrospective case-control study. METHODS: The study population comprised children (one eye each) who presented with a history of GDD exposure to a tertiary care eye hospital over the period January 2014 to January 2020. Consecutive children (age <18 years) were included. A control group from the same time period (children without GDD exposure) were included in the ratio of 5 controls to every 1 case of exposure. The main outcome measures included risk factors for exposure (univariate and multivariate analysis). RESULTS: A total of 21 eyes (of 21 children with implant exposure) and 115 eyes (of 115 children without exposure) were included in this study. During the same study period, a total of 494 eyes had undergone GDD implantation, giving an estimated incidence of exposure of 4.25%). In the bivariate analysis, GDD exposure was associated with multiple previous ocular surgeries (P = .001), longer follow-up duration (P < .001), combined procedure at the time of primary implantation (P = .002), and a younger age at the time of primary implantation (P = .006). The former 3 risk factors continued to prove a statistically significant association on multivariable regression analysis. Postoperative use of eye lubricants was more common among children in the control group (P = .007). CONCLUSION: In pediatric glaucoma patients, younger age and combined procedure at the time of primary GDD implantation, in addition to multiple previous ocular surgeries, were associated with greater risk of implant exposure. Postoperative use of eye lubricants may be protective.

Retinal arterial macroaneurysms with supravalvular pulmonic stenosis syndrome can be associated with coronary and major systemic arterial disease.

Purpose: To report novel life-threatening coronary and systemic arterial disease associated with Retinal Arterial Macroaneurysms with Supravalvular Pulmonic Stenosis (RAMSVPS) syndrome, previously known as Familial Retinal Arterial Macroaneurysms (FRAM). Observations: A 29-years old woman with longstanding poor vision in her right eye presented with acute myocardial infarction and subclavian bruit. Her polyangiogram showed peculiar ostial coronary aneurysms, left anterior descending coronary artery stenosis, occlusion of the left subclavian artery, stenosis of both renal arteries, irregularities in the mesenteric artery and tapering of the aorta. Takayasu arteritis was initially presumed, however fundus examination revealed beading and macroaneurysms along major retinal arteries, intraretinal exudation and hemorrhages, retinal arterial sheathing and stenosis, Coats'-like features and submacular gliosis in the right eye, vitreous hemorrhage in the left eye, and persistent hyaloid artery remnant in both eyes. These features evoked RAMSVPS syndrome. Genetic testing identified the same homozygous IGFBP7 c.830-1G > A mutation reported with RAMSVPS syndrome, rectifying the systemic diagnosis. Conclusion and importance: RAMSVPS syndrome can be associated with more life-threatening coronary and widespread major arterial disease than previously recognized. It is crucial for ophthalmologists to recognize RAMSVPS syndrome and refer patients for a thorough cardiovascular evaluation. Likewise, a careful retinal examination and the possibility of an IGFBP7 mutation should be considered in the setting of systemic arterial or cardiac disease.

Intermediate-term Visual Outcomes and Complications of Type 1 Boston Keratoprosthesis With and Without Glaucoma Surgery.

PURPOSE: Glaucoma is a cause of comorbidity in patients receiving the Boston keratoprosthesis (KPro). The aim of this study was to report the outcomes of the Boston KPro with or without glaucoma surgery. METHODS: This was a retrospective single-center cohort study. Patients who underwent Boston KPro from March 2009 to February 2019 were included. One eye per patient (the first surgery) was included in this study. Patients were classified into 2 groups: KPro only (group 1) and KPro with any form of glaucoma procedure (group 2). Main outcome measures were Best-corrected visual acuity (BCVA), functional success (BCVA 20/200 or better), anatomical success (retention of KPro at the last follow-up), and complications. RESULTS: Seventy-one eyes were included: 27 eyes (38%) in group 1 and 44 (62%) in group 2. There was no statistically significant difference in BCVA between groups 1 and 2 at each time point. Of the eyes in group 1, 11% lost light perception vision and 4.5% in group 2 ( P = 0.293). There was no difference in anatomical success with 70% in group 1 and 77% in group 2 ( P = 0.703) at the last follow-up, with a median failure time of 18 months. The functional success was 48% for group 1 and 50% for group 2 ( P = 0.541). CONCLUSIONS: Eyes undergoing KPro with glaucoma surgery before or at the same time carry a similar functional and anatomical success to eyes without glaucoma surgery.

Perception of Ophthalmologists of COVID-19 Using the Health Belief Model.

Purpose To assess ophthalmologists' preparedness in such a critical period in the history of pandemics, a logical socio-psychological framework assessment using the health belief model (HBM) is essential to evaluate their risk perception, their willingness to actively participate in engaging in protective health behavior and acknowledge its benefits, and their capability to perform adequate successful methods for limiting the spread of coronavirus disease 2019 (COVID-19) and overcome the barriers they might encounter while implementing such precautions. Methods A cross-sectional study conducted at King Khaled Eye Specialist Hospital using a questionnaire-based (HBM) was distributed to 135 ophthalmologists in the institute to evaluate their risk perception of COVID-19 and determine which components of the HBM contribute to preventive health behavior related to the COVID-19 infection. Results The questionnaire had a reasonable response rate (79.3%, 107 ophthalmologists, including 48 consultants, 51 fellows, and 36 residents). The study demonstrated that this model is useful and mapped how several components were significantly correlated to actions. Most significantly, perceived susceptibility was the most important predictor of action. The second most important determinant of action was the perceived benefit. Conclusion Pandemics such as COVID-19 are likely to happen again in the future. Explicit attention to factors influencing motivation such as threat perception to adopt appropriate health-related behavior to limit the spread of communicable diseases is necessary. This study has successfully represented preparedness and risk behavior perception of ophthalmologists of the novel COVID-19 pandemic in one of the largest tertiary eye hospitals in the Middle East using the Health Belief Model.

Combined cilioretinal artery and hemi-retinal vein occlusion in Sturge Weber syndrome: Expanding the clinical spectrum.

A 9-year-old boy with the diagnosis of Sturge-Weber Syndrome, and port-wine stain involving the right side of the face, presented with acute visual loss of the right eye. Examination revealed a high intraocular pressure and a combined cilioretinal artery and inferior hemi-retinal vein occlusion in the right eye. Systemic work-up was negative. After controlling the intraocular pressure, gradual improvement of vision was noticed with simultaneous disappearance of signs of vascular occlusion.

High-Mobility Group Box-1 Protein Mediates the Regulation of Signal Transducer and Activator of Transcription-3 in the Diabetic Retina and in Human Retinal Müller Cells.

PURPOSE: The expression of high-mobility group box-1 (HMGB1) and signal transducer and activator of transcription-3 (STAT-3) is upregulated in the diabetic retina. We hypothesized that the activation of STAT-3 is under the control of HMGB1. METHODS: Retinas from 1-month-old diabetic rats and from normal rats intravitreally injected with HMGB1 and human retinal Müller glial cells (MIO-M1) stimulated with HMGB1 or high glucose were studied by Western blot analysis and immunofluorescence. We also studied the effect of the HMGB1 inhibitor glycyrrhizin (GA) on high-glucose-induced pSTAT-3 nuclear translocation and upregulation in Müller cells and on pSTAT-3 expression in the retinas of diabetic rats (n = 7-10 in each group). In addition, we studied the effect of STAT-3 inhibitor on the HMGB1-induced induction of vascular endothelial growth factor (VEGF) by Müller cells and human retinal microvascular endothelial cell (HRMEC) migration. RESULTS: Treatment of retinal Müller cells with recombinant HMGB1 induced nuclear translocation of pSTAT-3 but did not alter pSTAT-3 expression. High glucose induced a significant upregulation of HMGB1 and pSTAT-3 upregulation and nuclear translocation in retinal Müller cells. GA co-treatment normalized the high-glucose-induced upregulation of HMGB1 and pSTAT-3 upregulation and nuclear translocation in Müller cells. Intravitreal administration of HMGB1 in normal and diabetic rats upregulated pSTAT-3 expression in the retina. GA attenuated the diabetes-induced upregulation of pSTAT-3 in the retina. The STAT-3 inhibitor attenuated HMGB1-induced VEGF upregulation by Müller cells and HRMEC migration. CONCLUSIONS: The results suggest a role for HMGB1 in the modulation of STAT-3 expression in the diabetic retina.