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Purpose: To evaluate frequency of surgery and post-treatment outcomes in glaucoma following cataract surgery (GFCS)., Methods: The medical records of patients with GFCS were reviewed retrospectively. Lensectomy and glaucoma surgery details and final examination findings were collected. Inclusion criteria included history of lensectomy at <1 year of age, diagnosis of glaucoma, and at least 1 year of follow-up., Results: Of 169 eyes of 127 GFCS patients (66 male, 58 bilateral cases), 88 eyes (52%) of 73 (57%) patients underwent glaucoma surgery (median, 3.5 years of age at first glaucoma surgery; median of two glaucoma surgeries). At final follow-up (mean, 13.6 ± 7.0 years), eyes requiring glaucoma surgery had worse visual acuity (P = 0.01) and greater cup:disk ratio (P < 0.01). GFCS patients with history of bilateral congenital cataracts had better visual acuity in affected eyes than those with history of unilateral congenital cataract (P < 0.01). Angle surgery (n = 56), Baerveldt devices (n = 38), Ahmed valves (n = 19), and cycloablation (n = 21) showed 1-year survival rates between 64% and 75%. Baerveldt implants showed the highest 5- and 10-year survival rates, at 65% and 43%, respectively., Conclusions: More than 50% of eyes with GFCS in our study cohort required at least one glaucoma surgery. Glaucoma surgery and history of unilateral cataract were associated with worse visual acuity outcomes., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose: To identify specific factors and outcomes associated with corneal edema and Haabs striae in primary congenital glaucoma (PCG)., Methods: The medical records of patients with PCG from 2011 to 2023 with >3 months' follow-up were reviewed retrospectively. Preoperative details and final outcomes were compared between eyes with and without corneal findings. The right eye of bilateral cases and the affected eye in unilateral cases were included., Results: A total of 58 patients (104 eyes, 69% male) underwent initial angle surgery at an average age of 297 ± 368 (median, 134) days. Corneal edema and Haabs striae were present preoperatively in 72 (69%) eyes of 41 patients and 68 (65%) eyes of 39 patients, respectively. Patients with corneal edema presented at a younger age (P < 0.0001) and with shorter axial length (P = 0.01) than those without edema. Univariate analysis showed that corneal edema was associated with worse visual acuity at final follow-up (OR = 4.4; 95% CI, 1.2-25.3). Patients with Haabs striae were older than those without striae (P = 0.04). After angle surgery, corneal edema was present at 1 month in 71% (95% CI, 52-84), at 2 months in 26% (95% CI, 12-42), at 3 months in 16% (95% CI, 6-30), and at 1 year in 3% (95% CI, 0-13). Corneal opacification did not resolve in 4 eyes of 3 patients after >4 years of follow-up., Conclusions: In our study cohort, corneal edema resolved in the majority of PCG cases within 2-3 months of initial angle surgery but was associated with younger age at presentation and worse visual acuity at final follow-up., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Determine outcomes of concurrent strabismus surgery with placement of a glaucoma drainage device (GDD) in children., Methods: Retrospective review of pediatric patients who underwent simultaneous lateral rectus (LR) muscle surgery with superotemporal GDD placement. Strabismus and GDD success were defined as residual horizontal misalignment < 10 prism diopter (PD) and intraocular pressure (IOP) < 21 mmHg, no visually devastating complications, and no additional IOP-lowering surgeries., Results: Fifteen eyes of 13 patients (69% male) underwent LR surgery (14 recessions, 1 resection) for exotropia or esotropia simultaneous with GDD placement (13 Baerveldt, 2 Ahmed) at 8.34 ± 5.26 years. Preoperative visual acuity (VA) in operative eye (0.89 ± 0.54) was worse than non-operative eye (0.23 ± 0.44, p = 0.0032). Preoperative horizontal deviation was 38.3 ± 9.4 PD and LR recession was 7.4 ± 1.1 mm. At final follow-up, VA in operative eye (0.87 ± 0.52) was unchanged from preoperative (p = 0.4062). Final IOP was significantly decreased (12.4 ± 4.7 mmHg vs. 31.1 ± 11.4 mmHg, p = 0.0001) as was number of glaucoma medications (2.7 ± 1.7 vs. 1.1 ± 1.3, p = 0.0037). Five (38%) and 9 patients (69%) met criteria for strabismus and GDD success, respectively. Two eyes required tube revision and endoscopic cyclophotocoagulation and 2 eyes had additional strabismus surgery., Conclusions: Concurrent strabismus and GDD surgery decreased horizontal deviation and obtained IOP control. It is important to consider correction of strabismus at time of GDD placement to maximize visual development and improve cosmesis in children with glaucoma., (© 2024. The Author(s).)

Background: There is no consensus and few reports as to the surgical management of encapsulated Ahmed glaucoma drainage devices (GDD) which no longer control intraocular pressure (IOP), especially within the pediatric population. The purpose of this study was to report outcomes of exchanging the Ahmed GDD for a Baerveldt GDD in children with refractory glaucoma., Methods: Retrospective review of children (< 18yrs) who underwent removal of Ahmed FP7 and placement of Baerveldt 350 (2016-2021) with ≥ 3-month follow-up. Surgical success was defined as IOP 5-20 mmHg without additional IOP-lowering surgeries or visually devastating complications. Outcomes included change in best-corrected visual acuity (BCVA), intraocular pressure (IOP), and number of glaucoma medications., Results: Twelve eyes of 10 patients underwent superotemporal Ahmed FP7 to Baerveldt 350 GDD exchange at 8.8 ± 3.6 years. Time to Ahmed failure was 2.7 ± 1.9 years with 1-, 3-, and 5-year survival rates of 83% with a 95% CI[48,95], 33% with a 95% CI[10, 59], and 8% with a 95% CI[0, 30]. At final follow-up (2.5 ± 1.8 years), success rate for Baerveldt 350 GDDs was 75% (9 of 12 eyes) with 1 and 3-yr survival rates of 100% and 71% with 95% CI[25,92], respectively. IOP (24.1 ± 2.9 vs. 14.9 ± 3.1 mmHg) and number of glaucoma medications (3.7 ± 0.7 vs. 2.7 ± 1.1) were significantly decreased (p < 0.004). BCVA remained stable. Two eyes required cycloablation and 1 eye developed a retinal detachment., Conclusions: Ahmed removal with Baerveldt placement can improve IOP control with fewer medications in cases of refractory pediatric glaucoma. However, more eyes with greater follow-up are required to determine long-term outcomes., (© 2023. The Author(s).)

Purpose: To determine the visual outcomes and effectiveness of glaucoma surgeries in congenital ectropion uvea., Design: Retrospective interventional case series., Methods: Surgeries and examination findings were collected on 11 eyes of 8 patients with congenital ectropion uvea at 2 academic sites from 2001 to 2021. Visual outcomes, surgical success (intraocular pressure [IOP]: 5-20 mm Hg, no additional IOP-lowering surgery, no visually devastating complications), and survival rates of glaucoma surgeries were assessed., Results: Glaucoma in bilateral congenital ectropion uvea was diagnosed at an earlier age (0.02 ± 0.01 years) than unilateral disease (8.9 ± 5.3 years, P = .002). All eyes required glaucoma surgery with 91% requiring multiple surgeries (3.5 ± 2.1, median 3 surgeries per eye). Trabeculotomy (8 eyes) showed 13% success rate. Although none of the 4 eyes that underwent trabeculectomy with mitomycin C needed repeat trabeculectomy, glaucoma drainage device placement, or cycloablation, 75% required bleb revision surgery. Glaucoma drainage devices (7 eyes) had a 57% success rate with 3 eyes requiring subsequent cycloablation (2) or trabeculectomy (1). At the final follow-up (8.5 ± 6.6 years, median: 7.9 years), all eyes achieved IOP control, and IOP was lower compared with presentation (13.2 ± 2.6 mm Hg vs 32.9 ± 9.9 mm Hg, P = .002). Best-corrected logarithm of the minimum angle of resolution visual acuity at the final follow-up was 0.2 ± 0.2., Conclusions: Bilateral congenital ectropion uvea presents with glaucoma earlier than unilateral cases. The majority of eyes required multiple glaucoma surgeries. Angle surgery was less effective than trabeculectomy or glaucoma drainage devices. IOP control was obtained in all eyes and affected individuals had good visual outcomes., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Prcis: In children, Baerveldt implants showed 84% success at 1 year, but decreased to 32% at 8 years. Age, race, and glaucoma type were not risk factors for failure. Concurrent intraocular surgery was associated with complications., Purpose: Evaluate success and risk factors for failure and complications of Baerveldt glaucoma implants in children., Methods: Retrospective case series of children who underwent Baerveldt implant placement (2012-2019 by single surgeon) with ≥1 year follow-up. Ocular examination and surgical details were collected. Failure defined as intraocular pressure (IOP) <5 mm Hg or >21 mm Hg for 2 consecutive visits, need for IOP related surgery, or visually significant complication., Results: One hundred-six eyes of 76 patients underwent 110 Baerveldt placement at median 6.4 years. Baerveldt placement was combined with additional procedures in 49% with vitrectomy most common (30%). Success of first Baerveldt (per patient) was 64% at final follow-up (median 4.7 y). One-, 5-, and 8-year survival rates were 84%, 60%, and 32%, respectively. There was no difference (P=0.97) in survival between first Baerveldt and all Baerveldt surgeries. Failure of first Baerveldt was not associated with sex, age, ethnicity, prior IOP-lowering surgery, concurrent intraocular surgery, or glaucoma type. Complications occurred in 14% and were associated with concurrent surgery. Twenty-six percent required additional IOP-lowering surgery. At final follow-up, IOP and glaucoma medications were significantly decreased (P<0.0001). Eyes underwent an average of 3.8±2.3 ocular surgeries and 3.0±2.0 glaucoma surgeries., Conclusions: Baerveldt implants showed good success initially, but survival rates declined over time. No risk factors for failure of first implanted Baerveldt were identified. Concurrent surgery was associated with complications. Majority of eyes required multiple surgeries to achieve IOP control and preserve vision., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.)

Background: We previously showed that Ologen increased success and survival rates of Ahmed glaucoma devices in a small number of children. The current study analyzed the same surgical technique in an expanded cohort., Methods: Retrospective interventional case series of children who underwent Ologen augmentation of Ahmed glaucoma device (OAGD) from 2018 to 2021 with ≥6 months' postoperative follow-up. Complete success was defined as intraocular pressure (IOP) of 5-20 mm Hg without glaucoma medications or additional IOP-lowering surgeries. Complete or qualified success was defined as above, except that IOP control was maintained with or without glaucoma medications., Results: A total of 26 eyes of 18 patients underwent OAGD at a median age of 2.0 years. Diagnoses included primary congenital glaucoma (5 eyes) and glaucoma secondary to nonacquired ocular anomalies (9 eyes), nonacquired systemic anomalies (8 eyes), and acquired conditions (4 eyes). Seventeen eyes had ≥1 prior eye surgery (average, 1.6 ± 0.9 surgeries per eye). Preoperative IOP was 29.4 ± 9.9 mm Hg on an average of 2.7 ± 1.0 glaucoma medications. At final follow-up (1.3 ± 1.0 years; median 1.0), IOP (13.4 ± 4.7 mm Hg) and number of glaucoma medications (0.3 ± 0.7, median 0) were significantly decreased (P < 0.0001). Complete success was achieved in 77% of eyes (20/26); Kaplan Meier analysis showed 1- and 3-year survival rates of 82% (95% CI, 59-93) and 60% (95% CI, 25-83), respectively. Complete or qualified success was achieved in 100% of eyes (26/26) at final follow-up. There were no visually devastating complications., Conclusions: OAGD showed a high rate of success defined by decreased IOP and medication dependency in our study cohort of pediatric glaucoma patients., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Evaluate outcomes and identify prognostic factors in congenital aniridia., Methods: Retrospective interventional case series of patients with congenital aniridia treated between 2012-2020. Ocular examination and surgical details were collected. Surgical failure was defined as disease progression or need for additional surgery for same/related indication. Kaplan-Meier survival curves, Wilcoxon test, and univariate and multivariate linear regression analyses were performed., Results: Ninety-four patients with congenital aniridia presented at median 19.0 years. Two-thirds of patients underwent ≥ 1intraocular surgery, with average of 1.7 ± 2.3 surgeries/eye. At final follow-up (median 4.0 years), 45% of eyes had undergone lensectomy. Aphakic eyes showed worse visual acuity (VA) than phakic or pseudophakic eyes. Glaucoma affected 52% of eyes, of which half required IOP-lowering surgery. Glaucoma drainage devices showed the highest success rate (71%) at 14.2 ± 15.4 years of follow-up. Keratopathy affected 65% of eyes and one-third underwent corneal surgery. Keratoprosthesis had the longest survival rates at 10-years (64% with 95% CI [32,84]). LogMAR VA at presentation and final follow-up were not statistically different. Half of patients were legally blind at final follow-up. Final VA was associated with presenting VA, glaucoma diagnosis, and cataract or keratopathy at presentation. Penetrating keratoplasty and keratoprosthesis implantation correlated with worse BCVA., Conclusions: Most aniridic patients in this large US-based cohort underwent at least 1 intraocular surgery. Cataract, glaucoma, and keratopathy were associated with worse VA and are important prognostic factors to consider when managing congenital aniridia., (© 2022. The Author(s).)

Monogenic syndromic disorders frequently feature ocular manifestations, one of which is glaucoma. In many cases, glaucoma in children may go undetected, especially in those that have other severe systemic conditions that affect other parts of the eye and the body. Similarly, glaucoma may be the first presenting sign of a systemic syndrome. Awareness of syndromes associated with glaucoma is thus critical both for medical geneticists and ophthalmologists. In this review, we highlight six categories of disorders that feature glaucoma and other ocular or systemic manifestations: anterior segment dysgenesis syndromes, aniridia, metabolic disorders, collagen/vascular disorders, immunogenetic disorders, and nanophthalmos. The genetics, ocular and systemic features, and current and future treatment strategies are discussed. Findings from rare diseases also uncover important genes and pathways that may be involved in more common forms of glaucoma, and potential novel therapeutic strategies to target these pathways.

Background: Limited data exists on the effectiveness of the collagen matrix, Ologen, on increasing Ahmed glaucoma valve (AGV) success in childhood glaucomas., Methods: Ocular examination and surgical details of pediatric patients who underwent AGV placement ± Ologen augmentation between 2012 and 2020. Complete success was defined as intraocular pressure (IOP) between 5 and 20 mmHg without glaucoma medications and additional IOP-lowering surgeries. Qualified success was defined as above, except IOP control maintained with or without glaucoma medications., Results: Twenty-two eyes of 16 patients underwent AGV placement of which 6 eyes had Ologen-augmentation (OAGV) and 16 eyes had conventional surgery (CAGV). Average age was 6.4 ± 5.1 years with 4.2 ± 2.5 follow-up years. There was no difference in age, number of previous surgeries, and preoperative IOP and glaucoma medications. At final follow-up, success rate was 100% (5 eyes complete, 6 eyes qualified) in the OAGV group compared to 31% (0 eyes complete, 5 eyes qualified) in the CAGV group. One and two-year survival rates were 100% for OAGV compared to 62 and 38% for CAGV. Postoperative IOP was significantly lower at 1-month and final follow-up (p = 0.02) as was the number of glaucoma medications at 3, 6, 12-months and final follow-up (p < 0.05) in the OAGV group., Conclusions: Ologen-augmentation increased the success and survival rates of AGVs in childhood glaucomas. Further, Ologen mitigated the hypertensive phase and decreased medication dependency. Longer follow-up with a greater number of eyes is required to fully evaluate the effectiveness of OAGV.