Foveal photoreceptor atrophy, persistent fetal vasculature, congenital cataracts, and microphthalmia in a pediatric patient with BCOR-associated oculo-facio-cardio-dental (OFCD) syndrome.

Purpose: To report a case of oculo-facio-cardio-dental (OFCD) syndrome secondary to a novel BCOR variant in a pediatric patient with congenital cataracts, microphthalmia, persistent fetal vasculature (PFV), focal chorioretinal hyperpigmentation, peripheral retinal avascularity, and foveal photoreceptor atrophy. Observations: A 3-month-old female patient was referred for bilateral congenital cataracts with microphthalmia. Her past medical history was significant for syndactyly of the toes, left bifid rib, atrial septal defect, patent ductus arteriosus, mitral regurgitation, pulmonary hypertension, anemia of prematurity, vesicoureteral reflux, and duodenal atresia. Examination under anesthesia revealed persistent fetal vasculature (PFV) with peripheral avascularity, foveal photoreceptor atrophy, and focal chorioretinal hyperpigmentation. A bilateral lensectomy with anterior vitrectomy and posterior capsulotomy were performed. Genetic testing identified a novel heterozygous pathogenic variant in the BCOR gene (c.1612C > T (p.Gln538Ter)), confirming a diagnosis of OFCD syndrome. Conclusions and importance: This case describes novel posterior segment findings in a patient with OFCD. A detailed examination of both anterior and posterior segments in combination with multimodal imaging should be performed in patients suspected of having OFCD, as this may be critical in determining visual potential and appropriate surgical management.

Compliance with web content accessibility guidelines in ophthalmology social media posts.

This is a cross-sectional exploratory analysis of publicly available Internet data to examine compliance to web content accessibility guidelines (WCAG) on patient education social media posts in ophthalmology. WCAG ensures web content accessibility for those with disabilities (including visual impairment). A total of 100 social media posts were sampled from ten ophthalmology patient education social media pages and ten non-ophthalmology (cardiopulmonary) pages as the comparison group. Three independent graders evaluated the selected posts based on the WCAG 2 checklist by WebAIM, a non-profit affiliated with Utah State University, after its adaptation for social media posts. Validated accessibility standard labels: "0" for not meeting any standards, "1" or "A" for meeting bare minimum accessibility requirements, "2" or "AA" for meeting legal accessibility requirements, or "3" or "AAA" for exceeding accessibility requirements. There was not enough evidence to detect a difference in WCAG scores between ophthalmology and non-ophthalmology posts (p = 0.80). Forty-nine percent of scores for ophthalmology social media posts showed no compliance with any WCAG. The most common reasons that ophthalmology posts failed to meet criteria were due to color and contrast issues (39%). Most ophthalmology social media posts had low WCAG scores, indicating poor compliance to WCAG. Because social media is highly visual, reduced compliance to WCAG may create barriers for low vision individuals to successfully access patient education social media content.

Using Large Language Models to Generate Educational Materials on Childhood Glaucoma.

PURPOSE: To evaluate the quality, readability, and accuracy of large language model (LLM)-generated patient education materials (PEMs) on childhood glaucoma, and their ability to improve existing the readability of online information. DESIGN: Cross-sectional comparative study. METHODS: We evaluated responses of ChatGPT-3.5, ChatGPT-4, and Bard to 3 separate prompts requesting that they write PEMs on "childhood glaucoma." Prompt A required PEMs be "easily understandable by the average American." Prompt B required that PEMs be written "at a 6th-grade level using Simple Measure of Gobbledygook (SMOG) readability formula." We then compared responses' quality (DISCERN questionnaire, Patient Education Materials Assessment Tool [PEMAT]), readability (SMOG, Flesch-Kincaid Grade Level [FKGL]), and accuracy (Likert Misinformation scale). To assess the improvement of readability for existing online information, Prompt C requested that LLM rewrite 20 resources from a Google search of keyword "childhood glaucoma" to the American Medical Association-recommended "6th-grade level." Rewrites were compared on key metrics such as readability, complex words (≥3 syllables), and sentence count. RESULTS: All 3 LLMs generated PEMs that were of high quality, understandability, and accuracy (DISCERN ≥4, ≥70% PEMAT understandability, Misinformation score = 1). Prompt B responses were more readable than Prompt A responses for all 3 LLM (P ≤ .001). ChatGPT-4 generated the most readable PEMs compared to ChatGPT-3.5 and Bard (P ≤ .001). Although Prompt C responses showed consistent reduction of mean SMOG and FKGL scores, only ChatGPT-4 achieved the specified 6th-grade reading level (4.8 ± 0.8 and 3.7 ± 1.9, respectively). CONCLUSIONS: LLMs can serve as strong supplemental tools in generating high-quality, accurate, and novel PEMs, and improving the readability of existing PEMs on childhood glaucoma.

Continuous Wave Transscleral Cyclophotocoagulation and Endoscopic Cyclophotocoagulation in Childhood Glaucoma: A Meta-Analysis.

PRCIS: Transscleral cyclophotocoagulation (TS-CPC) and endoscopic cyclophotocoagulation (ECP) were effective in reducing intraocular pressure (IOP) and glaucoma medications in childhood glaucoma. OBJECTIVE: To report the outcomes of continuous wave TS-CPC and ECP in childhood glaucoma. MATERIALS AND METHODS: We performed a systematic search of relevant databases. We collected data on age, follow-up duration, type of glaucoma, previous surgical interventions, preoperative and postoperative IOP, preoperative and postoperative number of glaucoma medications, adverse events, number of sessions, and success rates at different time points. The main outcome measures are the amount of IOP and glaucoma medication reduction. RESULTS: We included 17 studies studying 526 patients (658 eyes); 11 evaluated the effectiveness of TS-CPC (268 patients, 337 eyes), 5 evaluated ECP (159 patients, 197 eyes), and one study compared both techniques (56 patients, 72 eyes for TS-CPC vs 43 patients, 52 eyes for ECP). The median duration of follow-up was 28 months in the TS-CPC group and 34.4 months in the ECP group. The mean number of treatment sessions was 1.7 in the TS-CPC and 1.3 in the ECP. In the TS-CPC group, the mean IOP was significantly reduced from 31.2 ± 8 to 20.8 ± 8 mm Hg at the last follow-up ( P < 0.001). The mean number of glaucoma medications was reduced from 2.3 ± 1.3 to 2.2 ± 1.3 ( P = 0.37). In the ECP group, there was also a significant reduction in the mean IOP from 32.9 ± 8 mm Hg with a mean of 1.7 ± 0.7 glaucoma medications to 22.6 ± 9.8 mm Hg ( P < 0.0001) on 1.2 ± 1.1 medications ( P = 0.009) at the last follow-up. CONCLUSION: Both TS-CPC and ECP were effective in reducing the IOP and glaucoma medications in childhood glaucoma. Multiple treatment sessions were required.

A patient with concurrent Axenfeld-Rieger and Stickler syndromes verified by molecular genetics.

Purpose: To report a case of Axenfeld-Rieger and Stickler Syndrome in a pediatric patient. Observations: A 3-month-old male was referred to the glaucoma clinic after he was noted to have elevated intraocular pressures in both eyes. His family history was notable for infantile glaucoma on his maternal side and retinal detachment on his paternal side. He was found to have anterior segment dysgenesis with iris strands, iridocorneal adhesions, and corectopia, as well as veil-like vitreous in both eyes. He required trabeculotomy, goniotomy, and multiple Baerveldt glaucoma implants in both eyes to achieve intraocular pressure control. Furthermore, the patient later developed macula-involving retinal detachments in both eyes, requiring pars plana vitrectomy with silicone oil tamponade. Genetic analysis confirmed heterozygous pathogenic variants in both the FOXC1 and COL2A1 genes, leading to the concurrent diagnoses of Axenfeld-Rieger and Stickler syndromes. Conclusions and importance: This is a rare case of a patient with concurrent Axenfeld-Rieger and Stickler syndromes. The severity of pathology in both the anterior and posterior segments required a collaborative multidisciplinary approach. In the diagnostic evaluation of congenital eye diseases, if there is strong family history of atypical findings for a given diagnosis, concurrent syndromes should be considered and ruled out. A comprehensive eye genetics panel may be a useful tool in these cases.

Tube Versus Trabeculectomy IRIS Registry Study: 1-Year Secondary Treatment Outcomes.

PURPOSE: To describe 1-year secondary outcomes in the Tube Versus Trabeculectomy IRIS® (Intelligent Registry In Sight) Registry Study (TVTIRIS), and to compare to the TVT randomized controlled trial (TVTRCT). DESIGN: TVTIRIS was a retrospective cohort study. METHODS: The 2013-2017 IRIS Registry was used to identify eyes that received a tube shunt (tube) or trabeculectomy after a previous trabeculectomy and/or cataract surgery and had 1 year of follow-up. The TVTRCT compared a Baerveldt 350-mm2 glaucoma implant to trabeculectomy in similar eyes. RESULTS: In the TVTIRIS cohort, the tube (n = 236, 56.3%) and trabeculectomy (n = 183, 43.7%) groups had similar and significant reductions in intraocular pressure (IOP) from baseline to 1 year. In the tube group, IOP (mean ± SD) decreased from 26.6 ± 6.5 mm Hg at baseline to 14.3 ± 4.8 mm Hg at 1 year. In the trabeculectomy group, IOP decreased from 25.3 ± 6.4 mm Hg at baseline to 13.5 ± 5.2 mm Hg at 1 year. The trabeculectomy groups from both studies had similar 1-year IOP reduction (P = .18), although the TVTRCT cohort used fewer medications at all time points (P < .01). There were more pronounced differences in the mean IOP and medications between the tube groups in the 2 studies, presumably due to the inclusion of valved tubes in TVTIRIS. More reoperations occurred in TVTIRIS. CONCLUSIONS: The TVTIRIS tube and trabeculectomy groups had comparable 1-year IOP reduction, although trabeculectomy eyes used fewer glaucoma medications. The trabeculectomy group in TVTIRIS and TVTRCT had similar IOP and medication reduction at 1 year. Randomized controlled trials and electronic health record data both provide invaluable insight into surgical outcomes.

Ahmed and Baerveldt Glaucoma Drainage Devices in Childhood Glaucoma: A Meta-Analysis.

PRCIS: The effectiveness of Ahmed glaucoma valve (AGV) and Baerveldt glaucoma implant (BGI) was comparable in the management of childhood glaucoma over the long term despite initial better success rate with BGI. There were higher tube block and retraction rates in the BGI group and higher tube exposure rates in the AGV group. PURPOSE: To evaluate the outcomes and safety of AGV and BGI in childhood glaucoma. MATERIALS AND METHODS: We performed a systematic literature review of publications from 1990 to 2022 in PubMed, EMBASE, ClinicalTrials.gov, Ovid MEDLINE, Cochrane CENTRAL, and google scholar for studies evaluating AGV and BGI in childhood glaucoma. Primary outcome measures were intraocular pressure (IOP) reduction and glaucoma medication reduction. The secondary outcome measures were the success rates and incidence of postoperative complications. We conducted a meta-analysis using a random effects model. RESULTS: Thirty-two studies met the inclusion criteria. A total of 1480 eyes were included. The mean IOP reduction was 15.08 mm Hg ( P < 0.00001) for AGV and 14.62 ( P < 0.00001) for the BGI group. The mean difference between pre and postoperative glaucoma medications was 1 ( P < 0.00001) fewer medications in the AGV group and 0.95 ( P < 0.0001) fewer medications in the BGI group. There was a lower success rate in the AGV versus BGI groups at 2 years [63% vs 83%, respectively ( P < 0.0001) and 3 years (43% vs 79%, respectively ( P < 0.0001)]; however, the success was higher for AGV at 5 years (63% vs 56% in the BGI group, P < 0.001). The incidence of postoperative complications was comparable in the AGV and BGI groups, with rates of 28% and 27%, respectively. CONCLUSIONS: The IOP and glaucoma medication reduction, success rates, and incidence of postoperative complications were comparable in Ahmed and Baerveldt groups. Most literature comes from retrospective low-quality studies on refractory childhood glaucoma. Further larger cohort studies are needed.

Cost analysis of childhood glaucoma surgeries using the US Medicaire allowable costs.

AIM: To analyze and calculate the relative cost of various childhood glaucoma surgical interventions per mm Hg intraocular pressure (IOP) reduction ($/mm Hg). METHODS: Representative index studies were reviewed to quantitate the reduction of mean IOP and glaucoma medications for each surgical intervention in childhood glaucoma. A US perspective was adopted, using Medicare allowable costs to calculate cost/mm Hg IOP reduction ($/mm Hg) at 1y postoperatively. RESULTS: At 1y postoperatively, the cost/mm Hg IOP reduction was $226/mm Hg for microcatheter-assisted circumferential trabeculotomy, $284/mm Hg for cyclophotocoagulation, $288/mm Hg for conventional ab-externo trabeculotomy, $338/mm Hg for Ahmed glaucoma valve, $350/mm Hg for Baerveldt glaucoma implant, $351/mm Hg for goniotomy, and $400/mm Hg for trabeculectomy. CONCLUSION: Microcatheter-assisted circumferential trabeculotomy is the most cost-efficient surgical method to lower IOP in childhood glaucoma, while trabeculectomy is the least cost-efficient surgical method.

Transscleral vs endoscopic cyclophotocoagulation: safety and efficacy when combined with phacoemulsification.

PURPOSE: To compare the effectiveness and safety of phacoemulsification combined with endoscopic cyclophotocoagulation (phaco/ECP), phacoemulsification combined with MicroPulse transscleral cyclophotocoagulation (phaco/MP-TSCPC), and phacoemulsification alone (phaco) in the treatment of coexisting cataract and glaucoma. METHODS: Retrospective cohort study of consecutive cases at Massachusetts Eye & Ear. The main outcome measures were the cumulative probabilities of failure between the phaco/ECP group, phaco/MP-TSCPC group, and the phaco alone group with failure defined as reaching NLP vision at any point postoperatively, undergoing additional glaucoma surgery, or the inability to maintain ≥ 20% IOP reduction from baseline with IOP between 5-18 mmHg while maintaining ≤ baseline medications. Additional outcome measures included changes in average IOP, number of glaucoma medications, and complication rates. RESULTS: Sixty-four eyes from 64 patients (25 phaco/ECP, 20 phaco/MPTSCPC, 19 phaco alone) were included in this study. The groups did not differ in age (mean 71.04 ± 6.7 years) or length of follow-up time. Baseline IOPs were significantly different between groups (15.78 ± 4.7 mmHg phaco/ECP, 18.37 ± 4.6 mmHg phaco/MP-TSCPC, 14.30 ± 4.2 mmHg phaco alone, p = 0.02). Primary open-angle glaucoma was the most common type of glaucoma in the phaco alone (42%) and phaco/ECP (48%) groups while mixed-mechanism glaucoma was the most common type in the phaco/MP-TSCPC group (40%). Surgical failure was less likely in eyes in the phaco/MP-TSCPC (3.40 times, p = 0.005) and phaco/ECP (1.40 times, p = 0.044) groups compared to phaco alone based on the Kaplan-Meier survival criteria. These differences maintained statistical significance when differences in preoperative IOP were taken into account using the Cox PH model (p = 0.011 and p = 0.004, respectively). Additionally, surgical failure was 1.98 times less likely following phaco/MP-TSCPC compared to phaco/ECP (p = 0.038). This difference only approached significance once differences in preoperative IOP were accounted for (p = 0.052). There was no significant difference in IOP reduction at 1 year between groups. Mean IOP reductions at 1 year were 3.07 ± 5.3 mmHg from a baseline of 15.78 ± 4.7 in the phaco/ECP group, 6.0 ± 4.3 mmHg from a baseline of 18.37 ± 4.6 in the phaco/MP-TSCPC group and 1.0 ± 1.6 from a baseline of 14.30 ± 4.2 mmHg in the phaco alone group. There were no differences in complication rates among the three groups. CONCLUSIONS: Both Phaco/MP-TSCPC and phaco/ECP appear to provide superior efficacy for IOP control when compared to phaco alone. All three procedures had similar safety profiles.

Superior Visual Field Testing Using Virtual Reality With and Without Eye Tracking for Functional Upper Eyelid Surgery Evaluation: A Pilot Study.

PURPOSE: To assess the sensitivity and specificity of superior visual field tests administered in virtual reality (VR) with eye tracking (VR-ET) and without eye tracking (VR 0 ) for the fulfillment of insurance coverage criteria for functional upper eyelid surgery as compared with standard automated perimetry (SAP). METHODS: This prospective cross-sectional study included 78 eyes from 41 patients with ptosis, brow ptosis, and dermatochalasis undergoing functional upper eyelid surgery evaluation. Participants underwent serial superior visual field tests using SAP and VR 0 or VR-ET in randomized order. Fulfillment of insurance coverage criteria for blepharoplasty was defined as a 30% increase in the grid seen from the untaped to the taped state. The main outcome measure was the sensitivity and specificity of VR 0 , VR-ET, and overall VR in meeting insurance coverage criteria as compared with SAP. RESULTS: VR had a sensitivity of 84.1% and specificity of 67.6%, with no significant difference between VR 0 and VR-ET. SAP agreed on insurance coverage criteria fulfillment with VR 0 in 28 (71.8%) eyes and with VR-ET in 32 (82.1%) eyes. Insurance coverage criteria fulfillment rates varied significantly by diagnosis on SAP ( p = 0.012) but not VR ( p = 0.059). CONCLUSIONS: VR may be an alternative to SAP for functional upper eyelid surgery evaluation. Future studies are needed to determine differences in patient satisfaction, testing and waiting time, and test-retest reliability between VR and SAP.

Professional Quality of Life and Associated Factors Among Pediatric Glaucoma Providers.

PURPOSE: Even though physician burnout can negatively impact physical/mental health, ophthalmologists' quality of life (QOL) is understudied. Although managing high-risk diseases like pediatric glaucoma may lead to compassion satisfaction (CS), the responsibility of caring for very sick, demanding patients has been linked to higher burnout. Burnout and secondary traumatic stress (STS) constitute compassion fatigue, the negative effects resulting from helping those who experience stress. We assessed professional QOL, including CS, burnout, and STS, along with associated factors among pediatric glaucoma providers managing potentially blinding disease in children. DESIGN: Cross-sectional survey study. PARTICIPANTS: Pediatric glaucoma providers in the Childhood Glaucoma Research Network, American Glaucoma Society, and Indian Paediatric Glaucoma Society. METHODS: Participants were sent an electronic survey of a validated instrument (Professional Quality of Life-Version 5) designed to measure burnout, STS, and CS, as well as questions related to demographics, lifestyle, training, and practice. Burnout, STS, and CS scores range from 10 to 50 and are classified into low (≤ 22), moderate (23-41), and high (≥ 42) subgroups. MAIN OUTCOME MEASURES: Burnout, STS, and CS. RESULTS: Seventy-six pediatric glaucoma providers completed the survey with resulting burnout (22.6 ± 6.3), STS (22.7 ± 6.1), and CS (42.3 ± 6.7) scores. Most respondents had low burnout (43, 56.6%), low STS (44, 57.9%) and high CS (48, 63.2%), though more than a third reported moderate burnout (33, 43.4%), STS (31, 40.8%), and CS (27, 35.6%). Older age and more years in practice correlated positively with CS, and age correlated negatively with STS (P < 0.05 for all). Frequent workday fatigue correlated positively with burnout and STS and negatively with CS, and frequent caffeine consumption correlated positively with burnout and negatively with CS (P < 0.05 for all). Members of a married or unmarried couple had significantly lower CS scores than single, divorced, or separated respondents (P = 0.022). CONCLUSIONS: Pediatric glaucoma providers derive a high level of professional satisfaction from their work, though many report moderate burnout and STS. To comprehensively address provider QOL, CS, and both components of compassion fatigue must be considered. Initial efforts may be focused on younger, early-career providers as this group had lower professional QOL scores than their older, late-career counterparts. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

LGBTQ+ Identity and Ophthalmologist Burnout.

PURPOSE: To evaluate lesbian, gay, bisexual, transgender, questioning, and other sexual/gender minority (LGBTQ+) orientation as a burnout risk factor among an international ophthalmologist cohort. METHODS: An anonymous, cross-sectional electronic survey was distributed via an Internet platform to characterize the relationship among demographic factors, including LGBTQ+ orientation, and burnout as measured by the Copenhagen Burnout Inventory (CBI). Univariable data analysis (linear) by sexual orientation was performed and variables with an association with a P value of <0.15 in univariable analysis were included in the multiple linear regression modeling. RESULTS: A total of 403 ophthalmologists participated in the survey. The majority self-identified as "White" (69.2%), were from North America (72.0% United States, 18.6% Canada) and were evenly distributed between age of 30 and 65 years. Overall, 13.2% of participants identified as LGBTQ+ and 98.2% as cisgender. Approximately 12% had witnessed or experienced LGBTQ+-related workplace discrimination or harassment. The personal and work-related burnout scores and confidence limits of persons identified as LGBTQ+ were higher and nonoverlapping compared with those reported as non-LGBTQ+. Multivariable analysis identified significant risk factors for higher personal and work-related burnout scores: LGBTQ+ (11.8 and 11.1, P = .0005 and .0023), female gender (5.36 and 4.83, P = .0153 and .0434), older age (19.1 and 19.2, P = .0173 and .0273). and caretaker stress (6.42 and 5.97, P = .0085 and .0239). CONCLUSIONS: LGBTQ+ orientation is a burnout risk factor among ophthalmologists, and LGBTQ+ workplace discrimination may be a contributing factor. Support from ophthalmology organizations to address LGBTQ+-, gender-, and age-related workplace discrimination may decrease burnout. NOTE: Publication of this article is sponsored by the American Ophthalmological Society.

Diagnostic yield of next generation sequencing gene panel assays for early-onset glaucoma in an ethnically diverse population.

BACKGROUND: Early-onset glaucoma is a potentially sight-threatening condition with high heritability. Next generation sequencing is a cost-effective alternative to individual gene screening that could expedite its diagnosis. However, the diagnostic yield of multigene panel assays for early-onset glaucoma varies according to the tested population. The purpose of this study was to ascertain the diagnostic yield of next generation sequencing panels in our cohort and to identify population characteristics that increase such yield. METHODS: We conducted a retrospective review of the medical records of consecutive patients from November 2016 to August 2021 who were evaluated at our clinics for early-onset glaucoma and had undergone next generation sequencing panels for molecular diagnosis. RESULTS: A total of 118 patients were included, in 22 of whom (19%) a causative variant was identified. Diagnostic yield varied by age of onset: of 60 patients with onset at <3 years of age, 19 (32%) had such variants identified. In contrast, of 58 patients with later-onset glaucoma, 3 (5%) had said variants identified (P = 0.0003). Other metrics that increased diagnostic yield were presence of additional ocular anomalies (P = 0.0092) and identifying ethnicity as White (compared with non-White, P = 0.0001). CONCLUSIONS: In childhood glaucoma, earlier age of onset is correlated with higher likelihood of pathogenic variant identification. The large proportion of unsolved cases indicates a robust opportunity for gene discovery and genetic therapy targets in early-onset glaucoma patients.

Placement and Extension of Glaucoma Drainage Devices Using Polypropylene Suture in Pediatric Patients.

Glaucoma drainage device tube placement and extension in pediatric patients may be complicated by abnormal anterior segment anatomy and corneal opacities. The authors describe two techniques for placement and extension of non-valved glaucoma drainage device tubes using polypropylene suture as a guidewire and stabilizer, respectively. [J Pediatr Ophthalmol Strabismus. 2022;59(5):e62-e65.].

A Novel Technique for Anterior Segment Imaging Using B-scan Ultrasonography When Ultrasound Biomicroscopy Is Unavailable.

Ultrasound biomicroscopy offers high-resolution images of anterior segment anatomy, essential for determining surgical intervention in special circumstances, yet is sometimes unavailable. The authors describe a novel technique to obtain gross anterior segment visualization using a commonly available instrument, B-scan ultrasonography, and compare it to ultrasound biomicroscopy imaging from two pediatric cases. [J Pediatr Ophthalmol Strabismus. 2022;59(5):e58-e61.].

Glaucoma Cascade Screening in a High Risk Afro-Caribbean Haitian Population: A Pilot Study.

PRCIS: Glaucoma cascade screening in first-degree relatives (FDRs) of young Haitian glaucoma patients had high yield for diagnosing manifest and suspected glaucoma in 30.8% of those screened despite modest participation. PURPOSE: To evaluate the outcomes of glaucoma cascade screening in FDRs (parents, siblings, and offspring) of Haitian juvenile open-angle glaucoma (JOAG) patients. PATIENTS AND METHODS: Consecutive index patients (Haitians with JOAG) were identified, and the number/type of FDRs residing in South Florida were recorded. These FDRs were invited for free glaucoma screening, which included a comprehensive ophthalmic exam, gonioscopy, automated visual field testing and optical coherence tomographic analysis of the retinal nerve fiber layers. FDR characteristics and clinical findings from screening are reported. RESULTS: A total of 77 FDRs were invited, 26 (33.8%) agreed to undergo screening (18 females, 9 males), which revealed 2 (7.7%) with manifest glaucoma (mean age 77.5 y; one of whom was previously unaware of his glaucoma diagnosis), 6 (23.1%) with suspected glaucoma (mean age 29.8±18.3 y), and 18 (69.2%) without manifest or suspected glaucoma (mean age 37.2±21.8 y). Siblings of index patients were least likely to participate in cascade glaucoma screening when compared with index patients' parents or offspring. FDR eyes with manifest glaucoma had significantly worse best-corrected visual acuities, higher intraocular pressures, thinner central corneal thicknesses, and thinner circumferential papillary retinal nerve fiber layer thicknesses than those without glaucoma. CONCLUSION: Glaucoma cascade screening of Haitian JOAG patients' FDRs revealed that 30.8% had suspected or manifest glaucoma. Future efforts centered on provider-initiated recruitment and improving public glaucoma awareness and education may increase screening participation.

Outcomes of phacoemulsification and endoscopic cyclophotocoagulation performed with dual blade ab interno trabeculectomy or trabecular micro-bypass stent insertion.

BACKGROUND/OBJECTIVE: To report the initial outcomes of phacoemulsification, endoscopic cyclophotocoagulation, and dual blade ab interno trabeculectomy (PEcK), and compare them to those of phacoemulsification, endoscopic cyclophotocoagulation, and trabecular micro-bypass stent insertion (ICE-1). SUBJECTS/METHODS: Patients from January 2018 to December 2019 that underwent PEcK or ICE-1 at a tertiary referral centre were included in this retrospective comparative case series. Patients were excluded if they had additional concomitant procedures, less than 6 weeks (42 days) of follow-up or were not at least 18 years old. Intraocular pressure (IOP), number of glaucoma medications, and best-corrected visual acuity were collected preoperatively and postoperatively at 6 weeks, 3, 6, and 12 months. Kaplan-Meier survival analysis and Cox proportional-hazards regression were conducted to elucidate any factors associated with survival time. RESULTS: The mean preoperative IOP was 18.3 ± 5.9 mmHg in the PEcK group (53 eyes) and 14.7 ± 4.3 mmHg in the ICE-1 group (23 eyes) (p = 0.004) on 3.3 ± 1.3 and 1.7 ± 0.93 glaucoma medications (p < 0.001), respectively. Twelve months postoperatively the mean IOP reduction was 5.1 ± 4.4 mmHg and 2.3 ± 4.0 mmHg (p = 0.08), and the mean medication reduction was 1.6 ± 1.5 and 0.97 ± 0.66 (p = 0.10), in the PEcK and ICE-1 groups, respectively. Kaplan-Meier survival analysis did not reveal any differences in treatment survival. CONCLUSIONS: Both PEcK and ICE-1 provide clinically relevant reductions in IOP and glaucoma medication burden, however the PEcK procedure may confer greater reductions in IOP. The procedures did not differ with regard to Kaplan-Meier survival probability.

Evaluation of a computer-based facial dysmorphology analysis algorithm (Face2Gene) using standardized textbook photos.

BACKGROUND: Genetic syndromes often have ocular involvement. Ophthalmologists may have difficulty identifying dysmorphic features in genetic syndrome evaluations. We investigated the sensitivity and specificity of Face2Gene (F2G), a digital image analysis software trained on integrating dysmorphic features, by analysing patient photos from genetics textbooks. METHODS: We analysed all clear facial photos contained within the textbooks Smith's Recognizable Patterns of Human Malformation and Genetic Diseases of the Eye using F2G under standard lighting conditions. Variables captured include colour versus grey scale photo, the gender of the patient (if known), age of the patient (if known), disease categories, diagnosis as listed in the textbook, and whether the disease has ophthalmic involvement (as described in the textbook entries). Any photos rejected by F2G were excluded. We analysed the data for accuracy, sensitivity, and specificity based on disease categories as outlined in Smith's Recognizable Patterns of Malformation. RESULTS: We analysed 353 photos found within two textbooks. The exact book diagnosis was identified by F2G in 150 (42.5%) entries, and was included in the top three differential diagnoses in 191 (54.1%) entries. F2G is highly sensitive for craniosynostosis syndromes (point estimate [PE] 80.0%, 95% confidence interval [CI] 56.3-94.3%, P = 0.0118) and syndromes with facial defects as a major feature (PE 77.8%, 95% CI 52.4-93.6%, P = 0.0309). F2G was highly specific (PE > 83percentage with P < 0.001) for all disease categories. CONCLUSIONS: F2G is a useful tool for paediatric ophthalmologists to help build a differential diagnosis when evaluating children with dysmorphic facial features.

Effects of Postoperative Intravitreal Injections on Outcomes of Traditional Glaucoma Surgery in Patients with Preoperative Intravitreal Injections.

PURPOSE: To compare outcomes of glaucoma drainage device (GDD) implantation and trabeculectomies with and without postoperative intravitreal injections (IVIs) in glaucoma patients with a history of preoperative IVIs. DESIGN: Retrospective cohort study. PARTICIPANTS: A total of 133 eyes of 133 glaucoma patients who underwent GDD implantation or trabeculectomy with at least 1 IVI preoperatively between January 2005 and October 2020 at Massachusetts Eye and Ear. METHODS: Chart review of glaucoma patients with traditional glaucoma surgery and at least 1 IVI before surgery. All statistical analyses were conducted with R statistical programming software. MAIN OUTCOME MEASURES: Intraocular pressure (IOP), medication burden, best-corrected visual acuity (BCVA), Kaplan-Meier success rates, adjusted hazard ratios (HRs), and complications. RESULTS: Baseline demographics were similar between the groups with and without postoperative IVIs. The group with postoperative IVIs had a higher proportion of diabetic retinopathy and retinal vascular occlusions than the group without postoperative IVIs, which had more eyes with age-related macular degeneration. Intraocular pressure, medication burden, and visual acuity were similar between groups at all time points except for IOP at 6 weeks, which was lower in the group with postoperative IVIs. The group with postoperative IVIs had significantly more preoperative IVIs than the group without postoperative IVIs (6.6 vs. 3.3, P = 0.017). For success defined as IOP reduction ≥ 20% with 5 < IOP ≤ 21 mmHg, Kaplan-Meier analyses demonstrated similar success rates between groups with and without IVIs. When stratified by the number of IVIs, success rates for the group with 7 or more IVIs were significantly higher than the success rates for the group with 0-6 IVIs (P = 0.005). Each additional postoperative IVI resulted in a 7.2% decrease in the hazard of failure to achieve our stated success criteria. With regard to late complications, the group with postoperative IVIs had a higher incidence of vitreous hemorrhage (18.5% vs. 3.2%, P = 0.039) than the group without postoperative IVIs. CONCLUSIONS: A higher number of postoperative IVIs, specifically 7 or more IVIs, may be associated with improved success rates of traditional glaucoma surgery in glaucoma patients who received IVIs before surgery.

Netarsudil as an Adjunctive Therapy: Efficacy and Factors Contributing to a Favorable IOP-Lowering Effect.

Purpose: The aim of the study is to assess netarsudil's intraocular pressure (IOP)-lowering potential when prescribed as an adjunctive agent, to examine the effect of baseline IOP on patients' response to netarsudil, and to explore patients' characteristics predictive of pronounced responses to netarsudil. Methods: This is a single-center, multiprovider retrospective cohort study set at Massachusetts Eye and Ear. Patients with a diagnosis of glaucoma or ocular hypertension on netarsudil and at least one other hypotensive agent for glaucoma who had at least one month of follow-up were included. Patients with additional procedures or glaucoma medication changes were excluded. The main outcome measures were IOP reduction, Kaplan-Meier survival analyses, netarsudil responder type, and complication rates. Results: 236 eyes of 236 patients were included. The mean baseline IOP was 19.06 ± 4.6 mmHg on an average of 4 ocular hypotensive medications. 196 (83.1%) patients experienced IOP reduction at the first follow-up visit of 2.84 ± 0.30 mmHg at 55.66 ± 51.89 days. IOP reduction at the second visit among these patients was 3.01 ± 0.44 mmHg at 133.24 ± 77.63 days. After starting netarsudil, 59% had a sustained response (median duration of 315 days), 25% had a robust response (>20% IOP reduction for at least 80% of visits), and 10% had a super response (>20% and >10 mmHg IOP reduction). Netarsudil was effective as an adjunctive therapy across all baseline IOP categories with greater relative IOP reduction in higher baseline IOP groups. Conclusions: Netarsudil is an effective adjunctive glaucoma therapy. IOP reductions between 2 and 3 mmHg are typical, but a minority had more pronounced and sustained effects (>10 mmHg). Further analysis is needed to assess specific demographic and clinical factors predictive of these robust responses.